ArticlePDF Available

Recent advances in managing septal defects: ventricular septal defects and atrioventricular septal defects

Authors:

Abstract

This review discusses the management of ventricular septal defects (VSDs) and atrioventricular septal defects (AVSDs). There are several types of VSDs: perimembranous, supracristal, atrioventricular septal, and muscular. The indications for closure are moderate to large VSDs with enlarged left atrium and left ventricle or elevated pulmonary artery pressure (or both) and a pulmonary-to-systemic flow ratio greater than 2:1. Surgical closure is recommended for large perimembranous VSDs, supracristal VSDs, and VSDs with aortic valve prolapse. Large muscular VSDs may be closed by percutaneous techniques. A large number of devices have been used in the past for VSD occlusion, but currently Amplatzer Muscular VSD Occluder is the only device approved by the US Food and Drug Administration for clinical use. A hybrid approach may be used for large muscular VSDs in small babies. Timely intervention to prevent pulmonary vascular obstructive disease (PVOD) is germane in the management of these babies. There are several types of AVSDs: partial, transitional, intermediate, and complete. Complete AVSDs are also classified as balanced and unbalanced. All intermediate and complete balanced AVSDs require surgical correction, and early repair is needed to prevent the onset of PVOD. Surgical correction with closure of atrial septal defect and VSD, along with repair and reconstruction of atrioventricular valves, is recommended. Palliative pulmonary artery banding may be considered in babies weighing less than 5 kg and those with significant co-morbidities. The management of unbalanced AVSDs is more complex, and staged single-ventricle palliation is the common management strategy. However, recent data suggest that achieving two-ventricle repair may be a better option in patients with suitable anatomy, particularly in patients in whom outcomes of single-ventricle palliation are less than optimal. The majority of treatment modes in the management of VSDs and AVSDs are safe and effective and prevent the development of PVOD and cardiac dysfunction.
Open Peer Review
F1000FacultyReviewsarecommissioned
frommembersoftheprestigiousF1000
.InordertomakethesereviewsasFaculty
comprehensiveandaccessibleaspossible,
peerreviewtakesplacebeforepublication;the
refereesarelistedbelow,buttheirreportsare
notformallypublished.
Discuss this article
(0)Comments
REVIEW
Recent advances in managing septal defects: ventricular septal
defects and atrioventricular septal defects [version 1; referees: 3
approved]
PSyamasundarRao ,AndreaDHarris2
UniversityofTexas-HoustonMcGovernMedicalSchool,ChildrenMemorialHermannHospital,Houston,USA
PediatrixCardiologyAssociatesofNewMexico,Albuquerque,USA
Abstract
Thisreviewdiscussesthemanagementofventricularseptaldefects(VSDs)
andatrioventricularseptaldefects(AVSDs).ThereareseveraltypesofVSDs:
perimembranous,supracristal,atrioventricularseptal,andmuscular.The
indicationsforclosurearemoderatetolargeVSDswithenlargedleftatriumand
leftventricleorelevatedpulmonaryarterypressure(orboth)anda
pulmonary-to-systemicflowratiogreaterthan2:1.Surgicalclosureis
recommendedforlargeperimembranousVSDs,supracristalVSDs,andVSDs
withaorticvalveprolapse.LargemuscularVSDsmaybeclosedby
percutaneoustechniques.Alargenumberofdeviceshavebeenusedinthe
pastforVSDocclusion,butcurrentlyAmplatzerMuscularVSDOccluderisthe
onlydeviceapprovedbytheUSFoodandDrugAdministrationforclinicaluse.
AhybridapproachmaybeusedforlargemuscularVSDsinsmallbabies.
Timelyinterventiontopreventpulmonaryvascularobstructivedisease(PVOD)
isgermaneinthemanagementofthesebabies.Thereareseveraltypesof
AVSDs:partial,transitional,intermediate,andcomplete.CompleteAVSDsare
alsoclassifiedasbalancedandunbalanced.Allintermediateandcomplete
balancedAVSDsrequiresurgicalcorrection,andearlyrepairisneededto
preventtheonsetofPVOD.Surgicalcorrectionwithclosureofatrialseptal
defectandVSD,alongwithrepairandreconstructionofatrioventricularvalves,
isrecommended.Palliativepulmonaryarterybandingmaybeconsideredin
babiesweighinglessthan5kgandthosewithsignificantco-morbidities.The
managementofunbalancedAVSDsismorecomplex,andstaged
single-ventriclepalliationisthecommonmanagementstrategy.However,
recentdatasuggestthatachievingtwo-ventriclerepairmaybeabetteroptionin
patientswithsuitableanatomy,particularlyinpatientsinwhomoutcomesof
single-ventriclepalliationarelessthanoptimal.Themajorityoftreatment
modesinthemanagementofVSDsandAVSDsaresafeandeffectiveand
preventthedevelopmentofPVODandcardiacdysfunction.
Keywords
Ventricularseptaldefect,atrioventricularseptaldefect,surgery,percutaneous
treatment,hybridprocedure
1 2
1
2

Referee Status:
 InvitedReferees
version 1
published
26Apr2018
 
123
,SilesianCenterforJacek Bialkowski
HeartDiseases,MedicalUniversityof
Silesia,Poland
1
,JohnHopkinsUniversity,Monesha Gupta
USA
2
,Children'sHospitalBostonMeena Nathan
andHarvardMedicalSchool,USA
3
26Apr2018, (F1000FacultyRev):498(doi:First published: 7
)10.12688/f1000research.14102.1
26Apr2018, (F1000FacultyRev):498(doi:Latest published: 7
)10.12688/f1000research.14102.1
v1
Page 1 of 16
F1000Research 2018, 7(F1000 Faculty Rev):498 Last updated: 26 APR 2018
PSyamasundarRao( )Corresponding author: P.Syamasundar.Rao@uth.tmc.edu
 :Conceptualization,FormalAnalysis,Investigation,Methodology,ProjectAdministration,Supervision,Writing–Review&Author roles: Rao PS
Editing; :Writing–OriginalDraftPreparationHarris AD
Nocompetinginterestsweredisclosed.Competing interests:
RaoPSandHarrisAD.How to cite this article: Recent advances in managing septal defects: ventricular septal defects and
2018, (F1000FacultyRev):498(doi:atrioventricular septal defects [version 1; referees: 3 approved] F1000Research 7
)10.12688/f1000research.14102.1
©2018RaoPSandHarrisAD.ThisisanopenaccessarticledistributedunderthetermsoftheCopyright: CreativeCommonsAttributionLicence
,whichpermitsunrestricteduse,distribution,andreproductioninanymedium,providedtheoriginalworkisproperlycited.
Theauthor(s)declaredthatnograntswereinvolvedinsupportingthiswork.Grant information:
26Apr2018, (F1000FacultyRev):498(doi: )First published: 7 10.12688/f1000research.14102.1
Page 2 of 16
F1000Research 2018, 7(F1000 Faculty Rev):498 Last updated: 26 APR 2018
Introduction
Septal defects are the most common types of congenital heart
defects (CHDs) with the exception of bicuspid aortic valve.
Advances in the management of atrial septal defects (ASDs) have
been addressed in an earlier review1. In the current article, ven-
tricular septal defects (VSDs) and atrioventricular septal defects
(AVSDs) will be discussed. Whereas transcatheter (percutane-
ous) approaches are the mainstay in the management of secun-
dum ASDs, the VSDs and AVSDs are at present managed largely
by surgical methodology since a limited number of lesions are
amenable for transcatheter and hybrid approaches. In this
review, we will present a classification of the VSDs and AVSDs,
indications for repair, and a discussion of surgical, transcatheter,
and hybrid methodologies with a particular focus on advances in
the management of these defects.
Ventricular septal defects
Isolated VSDs are the most common CHDs (provided that
subjects with bicuspid aortic valve are excluded) and constitute
20 to 25% of all CHDs. They are most commonly classified on the
basis of their location in the ventricular septum and are divided
into perimembranous (situated in the membranous ventricular
septum in the subaortic region), supracristal (found in the conal
septum in the subpulmonary region), atrioventricular (AV) septal
(defect located in the posterior septum), and muscular (located
in the muscular and apical areas of the ventricular septum)25.
The membranous defects are most common among the VSDs
(80% prevalence), and supracristal (5 to 7%), AV septal (8%),
and muscular (5 to 20%) defects are much less common. These
defects may be large, medium, or small in size. Most of the
defects are single; however, multiple defects may be present in
the muscular septum, described as the “Swiss cheese” type of
VSDs.
Left-to-right shunt across the VSD produces dilatation of the
left atrium and left ventricle (LV). Owing to high pulmonary
vascular resistance (PVR), this shunt may not manifest in the
neonate and during the first weeks of life. As the pulmonary
arterioles involute, PVR falls with resultant increase in left-to-
right shunt. The right ventricle (RV) and main and branch
pulmonary arteries may also be dilated in moderate to large
defects. Whereas pulmonary vascular obstructive disease
(PVOD) does not manifest until adulthood in patients with
ASD, patients with VSD are likely to develop PVOD as early as
18 months to 2 years of age if a large VSD is left unrepaired.
Discussion of VSDs seen in association with tetralogy of
Fallot, pulmonary atresia/stenosis, transposition of the great
arteries, tricuspid and mitral atresia, and double-outlet RV and
heterotaxy (asplenia and polysplenia) syndromes will not be
included in this article. Similarly, post-traumatic and post-
myocardial infarction VSDs will not be addressed in this review.
Indications for ventricular septal defect closure
The indications for intervention depend, to a large degree,
on the size and type of the VSD26. Closure of the VSD is not
necessary in patients with a small VSD. Assurance of the
parents and perhaps subacute bacterial endocarditis prophylaxis
and occasional clinical follow-up are suggested. However, if the
VSD has become smaller because of its closure by prolapsed
aortic valve cusp into the defect with resultant aortic insuffi-
ciency, surgical closure of the defect with resuspension of the
aortic valve leaflets is recommended. The development of aortic
insufficiency is seen in both membranous and supracristal
VSDs.
In moderate-sized VSDs, congestive heart failure (CHF), if present,
should be treated. In the presence of failure to thrive, markedly
enlarged left atrium and LV or elevated pulmonary artery pres-
sures (or both), closure of the defect is generally recommended. An
additional criterion is a pulmonary-to-systemic flow ratio (Qp:Qs)
greater than 2:1.
In large VSDs with systolic pressures in the RV and pulmo-
nary artery close to left ventricular and aortic systolic pressures,
closure should be undertaken. This should be done prior to
6 to 12 months of age (certainly no later than 18 months of
age) irrespective of control of heart failure and adequacy of
weight gain. The reason for this recommendation is to prevent
irreversible PVOD. In babies with Down syndrome, such closure
should be undertaken prior to six months of age since these
patients tend to develop PVOD sooner than non-Down babies.
In present-day practice, primary surgical correction is preferred
in contradistinction to initial pulmonary artery banding followed
by surgical closure of the VSD, a common practice in an earlier
era. However, such a staged approach may be considered for the
Swiss-cheese variety of muscular VSDs. Some clinicians may
also use pulmonary artery banding in low-weight infants who
may have an increased risk of heart block with repair, although the
current trend is toward early complete repair.
In cases with elevated PVR, most authorities would suggest
intervention if the calculated PVR index is less than 6 Wood units
or the pulmonary-to-systemic vascular resistance ratio (Rp:Rs)
is less than 0.35 (or both) with a Qp:Qs greater than 1.568.
In patients with higher PVR values, pulmonary vascular
reactivity testing with oxygen and nitric oxide (NO) should be
performed6,8,9. If the PVR index drops below 6 to 8 units with
oxygen or NO, then these patients become candidates for VSD
closure.
Patients with large VSD and severely elevated PVR (that is,
irreversible PVOD) are not suitable for VSD closure. These
patients eventually may become candidates for lung transplanta-
tion.
Management
The treatment of the VSD, as mentioned above, is largely
dependent on its size and the clinical status of the patient. Several
methods of available treatment options will be reviewed.
Medical management. Infants with moderate to large VSDs
with signs of CHF should receive aggressive treatment with
anti-congestive measures to include digoxin, diuretics (furosem-
ide and aldactone), and afterload reducing agents (angiotensin-
converting enzyme inhibitors: captopril/enalopril and others).
Page 3 of 16
F1000Research 2018, 7(F1000 Faculty Rev):498 Last updated: 26 APR 2018
Although the above order of drug administration has been used
for a long time, recent doubts about the usefulness of digoxin
have changed the order of administration to diuretics, afterload
reducing agents, and digoxin (in that order). We continue to
believe that digoxin is useful in the management of CHF in
infants and children. When chronic administration of furosem-
ide is required, aldactone may be added for its potassium-sparing
effect. Optimization of nutrition, maintenance of adequate
hemoglobin level, and appropriately addressing the associated
respiratory symptoms should be a part of overall management of
these babies.
Clinical improvement may occur with adequate medical
therapy; such improvement may be related to spontaneous
closure or diminution in the size of the VSD, development of
right ventricular outflow tract obstruction (Gasul’s transforma-
tion), or increased PVR. Careful clinical and echocardiographic
follow-up evaluation and, when necessary, cardiac catheteriza-
tion should be undertaken to ensure that the improvement is
not secondary to elevation of PVR. As mentioned in the preced-
ing section, VSD closure should be undertaken prior to 18 months
of age to prevent the development of irreversible PVOD.
As also mentioned in the previous section, large VSDs with
severe increase of PVR (irreversible PVOD) should not have
their VSDs closed. Recently described pulmonary vasodilators
(prostacyclins, sildenafil, bosentan, and others), which may
improve symptoms, may be used. If severe polycythemia is
present, erythropheresis to treat symptoms of polycythemia
should be performed. Transcatheter creation of atrial commu-
nication may relieve symptoms of PVOD. Discussion of the
pulmonary vasodilators and management of PVOD is beyond
the scope of this article. Ultimately, patients with PVOD may need
lung transplantation.
Although it is extremely important that every effort be made
to prevent PVOD, it should be recognized that 40% of the VSDs
close spontaneously and an additional 25 to 30% of defects
become small enough not to require intervention. Defects in
the muscular septum tend to close more often than the defects
in the perimembranous region. Small defects are likely to
close more often than large VSDs (60% versus 20%). It is well
documented that even large defects producing CHF or requiring
pulmonary artery banding during infancy close spontaneously.
Whereas most of the defects close by two years of age, the
process of spontaneous closure continues through childhood,
adolescence, and adulthood. These considerations should be kept
in mind when decisions to recommend surgical or transcatheter
closure of VSDs are being made.
Surgical repair. Following the description of cardiopulmo-
nary bypass techniques by Gibbon, Lillehei, and Kirklin in the
1950s to successfully close ASDs1, surgical techniques for repair
of other CHDs, including VSDs, were soon developed. Median
sternotomy incision is performed under general anesthesia
and the aorta and vena cavae are cannulated to institute cardi-
opulmonary bypass. The majority of perimembranous VSDs
are closed with a Dacron patch via right atriatomy with or
without detachment of the tricuspid valve leaflets. Supracristal
defects are addressed via the pulmonary valve. VSDs with
associated aortic insufficiency, though small, should be closed to
prevent progression of aortic insufficiency10. Moderate to severe
aortic valve prolapse may require re-suspension of the aortic
valve or other valvuloplasty techniques or both11,12.
Small muscular VSDs are likely to close spontaneously and
do not require surgery. In infants, large muscular VSDs, par-
ticularly of the “Swiss cheese” variety, are difficult to close from
the right ventricular side. Pulmonary artery banding initially to
control CHF and reduce the pulmonary artery pressures is per-
formed in young (up to three months of age) babies. Closure of
the VSD via an apical left ventriculotomy may be performed later
during childhood13,14. At that time, the pulmonary artery band
is removed and the pulmonary artery is repaired, if necessary,
to ensure that there is no residual stenosis at the prior band site.
The senior author (PSR) observed several patients in whom
the muscular VSD closed spontaneously following pulmonary
artery band placement, and repeat thoracotomy to remove the
pulmonary band was required. Absorbable pulmonary artery
band may be a good option in such situations. The polydiox-
anone band (absorbable) reduces pulmonary blood flow and pres-
sure initially and helps decrease symptoms of CHF. When the
VSD closes spontaneously, the pulmonary artery band is also
resorbed and will not require additional surgery to remove it.
The principles are similar to those advocated for patients with
tricuspid atresia with a large VSD15,16. Unfortunately, however,
most surgeons are reluctant to use an absorbable pulmonary
artery band in this situation.
Results
Surgical closure of VSDs is safe; the mortality rate is less than
3%. The long-term outlook following surgery in both the early
era17 and more recent times18 is generally good with rare resid-
ual shunts, frequent right bundle branch block, occasional
pulmonary hypertension, infrequent heart block or sinus node
dysfunction, and modest progression of aortic insufficiency.
Following VSD closure, the left ventricular volume and mass
return to normal with preservation of normal left ventricular
function19.
Percutaneous closure. Percutaneous closure of VSDs in ani-
mal models was first reported by Rashkind in the early 1970s20.
He used hooked, single-disc, and double-disc Rashkind devices.
Afterwards, other cardiologists followed his lead and used
Rashkind’s double-disc patent ductus arteriosus (PDA) umbrella,
Rashkind’s ASD double-umbrella, and clamshell devices for
transcatheter occlusion of VSDs2126. Subsequently, other devices
were used to percutaneously occlude the VSDs: buttoned device
(Custom Medical Devices, Amarillo, TX, USA)27,28, CardioSEAL
and STARFlex devices (Nitinol Medical Technologies, Inc.,
Boston, MA, USA)29, Nit-Occlud device (pfm - Produkte für
die Medizin AG, Köln, Germany)3033, Amplatzer Muscular
VSD Occluder device (St. Jude Medical, Inc., St. Paul, MN,
USA)3439, detachable steel coils (Cook, Bloomington, IN,
USA)40, Gianturco coils (Cook)41, flipper, 0.052˝ Gianturco,
or 0.035˝ platinum coils (Cook)42, wireless devices such as
Page 4 of 16
F1000Research 2018, 7(F1000 Faculty Rev):498 Last updated: 26 APR 2018
detachable balloon and transcatheter patch (Custom Medical
Devices)43, Amplatzer Duct Occluder (St. Jude Medical, Inc.)44,
Shanghai symmetrical perimembranous VSD occluder (Shape
Memory Alloy Ltd., Shanghai, China)45, Amplatzer Duct Occluder
II (St. Jude Medical, Inc.)4656, Cera devices (Lifetech Scientific
Co. Ltd., Shenzhen, China)57, and perhaps other devices (not
known to the authors).
The majority of the above mentioned (with exception of coils
and Amplatzer Duct Occluder) are double-disc devices and
require septal rims to hold the device in place. Therefore, they
can be used only in occluding muscular VSDs and perimembra-
nous defects with a good-sized aortic rim. Owing to lack of aortic
rim and proximity of the aortic valve to the defect, it may not be
feasible to close the more common perimembranous VSDs. In
an attempt to address this challenge, the device was redesigned58
so that the aortic end of the left ventricular disc is made to be
shorter (0.5 mm) while the other end is designed to be longer
(5.5 mm). The lower pole of the left ventricular disc was impreg-
nated with a platinum marker in order to aid appropriate device
positioning during deployment of the device. This redesigned
device, now called Amplatzer Membranous VSD Occluder
(St. Jude Medical, Inc.), was used in patients with small- to
medium-sized VSDs5968, including in US Food and Drug
Administration (FDA)-approved US clinical trials67. The results
were generally considered acceptable. In addition to the usual
complications seen with complex procedures, complete heart
block6065,6776 was detected both immediately after and during
follow-up after implantation of Amplatzer Membranous VSD
occluders, raising concerns77,78 regarding the advisability of
using this device.
The connecting waist of most double-disc devices is located
within the VSD, is small, and is not likely to stretch the defect.
The mechanism of defect closure is by stop-flow by the discs
on both sides of the VSD. However, the Amplatzer Membra-
nous VSD Occluder indeed “stents” the defect and, with time,
stretches the defect since the device size is generally larger than
the size of the VSD. Since the conduction system is situated
along the rims of the VSD, the device possibly exerts pressure
on the conduction system78. This is likely to be the mechanism
for reported development of heart block6065,6776 and conduction
abnormalities6365,71,72. The described incidence of complete heart
block varied between 1 and 22% and many of them required
pacemaker therapy6065,6776. This is in contradistinction to 1%
incidence of heart block following surgical closure79. Therefore,
it is difficult to justify the use of membranous VSD Occluder to
transcatheter-occlude the VSDs. It has been suggested that
the device be redesigned to make its edges supple or soft such
that less or no pressure is exerted on the conduction system78.
However, to the best of authors’ knowledge, no such modifica-
tions seem to have been undertaken by the device manufacturer.
Most of the devices described above had experimental device
closures in animal models. Clinical trials in human sub-
jects followed with local institutional review board, CR Mark
(in Europe), or FDA (in the USA) approval. Feasibility, safety,
and effectiveness of occluding the VSDs have been shown for the
majority of the devices. However, at this time, only the Amplatzer
Muscular VSD Occluder has received approval for clinical use
by the FDA. Although CardioSEAL Septal Occlusion System
with QwikLoad and the STARFlex Septal Occlusion System
have received FDA approval in the past, they are not widely used
at present. Free and detachable Gianturco coils41,42, Nit-Occlud
device3033,75, Amplatzer Duct Occluder44, and Amplatzer Duct
Occluder II4656 are also being used on an off-label basis. Discus-
sion of devices used on an off-label basis will not be included
in this review; the interested reader may review the respective
publications3033,41,42,44,4656,75.
Amplatzer muscular ventricular septal defect occluder.
Description of the device
The device is constructed with 0.004˝ to 0.005˝ Nitinol
(nickel–titanium compound) wire with shape memory and
consists of two equal-sized discs connected with a 7-mm-long
waist. The discs on either side of the waist are 4 mm longer than
the waist. Dacron polyester patches are sewn into both discs.
The device size is determined by the diameter of the connecting
waist. Available device sizes are 4 to 18 mm. The device is easily
retrieved and redeployed.
Method of implantation
Following recording of the usual hemodynamic data, a biplane
left ventricular cine-angiogram is performed in a sitting-up
(60° long-axis oblique and 30° cranial) and lateral views to define
the size and location of the VSD. Balloon sizing of the VSD,
performed at the beginning, is no longer routinely performed;
instead, transesophageal echocardiographic (TEE) and angi-
ographic diameters are used to measure the VSD size. Additional
angiography in other views is obtained if the required data on
the VSD size and location could not be clearly defined. Initially,
a right coronary artery, multipurpose, or balloon wedge catheter
is advanced from the LV into the RV via the VSD with the aid
of a soft-tipped guide wire, and an exchange-length guide wire
(Noodle wire, St. Jude Medical, Inc.) is passed and its tip is
positioned either in the superior vena cava or in the pulmonary
artery. The tip of the wire is snared from the femoral venous
route and the wire exteriorized, establishing an arterio-venous
guide-wire loop. Now, the selected delivery sheath is advanced
from the femoral vein and positioned into the left ventricular
apical region and the guide wire and catheters are removed. A
right internal jugular venous approach may be required in some
cases, especially when kinking of the sheath introduced via the
femoral vein occurs. Alternatively, the device may be delivered
from the arterial route. A pigtail catheter is placed retrogradely
into the LV. An Amplatzer Muscular VSD Occluder that is 1 to 2
mm larger than the diameter of the VSD is selected for deploy-
ment. The device is screwed onto a delivery wire and loaded
into the delivery sheath while taking precautions to eliminate air
bubbles in the system. The device is advanced within the
delivery sheath, and the left ventricular disc is delivered into the
LV under fluoroscopic and TEE guidance. After making sure that
the device does not impinge on the mitral valve apparatus, the
left ventricular disc is pulled back against the ventricular septum
under fluoroscopic and TEE guidance. If necessary, left ven-
tricular angiography is performed to verify the position of the
Page 5 of 16
F1000Research 2018, 7(F1000 Faculty Rev):498 Last updated: 26 APR 2018
device. The waist of the device is delivered into the defect. Then
the right ventricular disc is deployed on the right side of the
ventricular septum by slow withdrawal of the sheath, again while
monitoring by fluoroscopy and TEE. Left ventricular angiogra-
phy and TEE are repeated to ensure good position of the device
which is still connected to the delivery wire. At this point, the
device is still retrievable back into the delivery sheath and
redeployed as seen fit. Once the device position is satisfactory,
the device is disconnected from the delivery wire. A final left
ventricular angiogram and TEE are performed prior to removal
of the catheters and sheaths. If additional muscular VSDs are
present, they may also be occluded by placement of additional
Amplatzer Muscular VSD Occluders as previously described
elsewhere37,80. Administration of heparin (100 units/kg and
additional doses to maintain activated clotting times above
200 seconds), antibiotics (usually Ancef, three doses eight
hours apart) and anti-platelet therapy (aspirin or clopidogrel or
both), in accordance with institutional protocol for intracardiac
device implantations, is undertaken.
Results
Immediate results of implantation of Amplatzer Muscular VSD
Occluders in 1 to 119 patients have been reported3439,74,8083.
Most reports involving single-institution studies stated that
some degree of contrast foaming through the device was seen
immediately after device deployment with almost complete
resolution of shunt by the next day. However, the multi-
institutional US Registry study80 reports a complete closure
rate of 47% at 24 hours after the procedure, which increased
to 70% at 6 months and 92% at 12 months after the procedure.
Reported complications include transient complete left
bundle branch block; transient junctional rhythm; complete heart
block, both transient and permanent (the latter requiring pace-
maker implantation); tamponade resulting in death; cardiac per-
foration; device embolization (some retrieved by transcatheter
methodology and others by surgery), device malposition
requiring surgical removal; severe hemolysis (some causing renal
failure); and death34,36,38,74,8084; however, these complications are
rare. Follow-up results were reported in a few studies but with no
additional complications.
Hybrid (perventricular) device delivery. Because of inherent
limitations of both surgical and percutaneous device closure of
muscular VSDs in small infants, a hybrid approach, also called
“perventricular closure”, was introduced85,86. The initial descrip-
tions included six infants85 and seven mini pigs86. Feasibility
of this approach was demonstrated in both young infants and
animal models without cardiopulmonary bypass85,86. A number of
other investigators87101 subsequently adopted this technique.
The procedure
Under general anesthesia, a median sternotomy or a subxiphoid
incision is made and the right ventricular free wall is needle-
punctured via a purse-string suture. The soft end of a guide wire
is introduced across the VSD into the LV under TEE guidance.
The needle is removed and an appropriate-size delivery sheath
with a dilator is advanced over the guide wire into the LV and the
dilator and guide wire are withdrawn, leaving the tip of the
sheath in the LV. An appropriate-size device (1 to 2 mm larger
than the TEE diameter of the VSD) is advanced through the
sheath. Once the device reaches the tip of the sheath, the sheath is
retracted to extrude the left ventricular disc in the mid LV. Then
the entire system is slowly withdrawn so that the LV disc is
apposed to the interventricular septum. Further slow retraction
of the sheath is undertaken, delivering the device waist within
the defect and the right ventricular disc on the right side of the
ventricular septum—all while the procedure is being monitored
by TEE and while the heart is beating. Once the position of the
device is satisfactory as evaluated by TEE, the delivery wire
is disconnected and the sheath is removed while the purse-
string suture is tightened. A final TEE is performed to ensure
appropriate position of the device and to detect any additional
VSDs.
Results
Immediate results of hybrid procedures to close perimembrano-
sus and muscular VSDs have been reported85101. The number
of subjects included was anywhere between 6 and 408 patients.
Successful device implantation is reported from 82 to 100%
of patients. In most studies, unsuccessful implantations were
converted to conventional open-heart surgical repair, irrespec-
tive of the reason for failure. Residual shunts were not significant.
Complications were few and the occasional pericardial effusion
occurred.
In one large study involving 408 patients with perimembranous
VSDs with a mean age of 3.1 years (± 1.7 years), an age range
of 5 months to 15 years, and a weight range of 4.5 to 26 kg,
successful closure was accomplished in 393 patients (96.3%)90.
The devices used were similar to Amplatzer VSD devices but
manufactured in China by the Shanghai Alloy Material Corpora-
tion; 213 (54.2%) were symmetric devices and 180 (45.8%) were
asymmetric devices. Follow-up from three months to two years
after the procedure exhibited no residual shunts, stable device
position, and no increase in aortic insufficiency.
In a multicenter retrospective study from Europe, investigators
reported closure of muscular VSDs in 21 patients; the device
was successfully implanted in 89% of patients91. In the remain-
ing patients, the device was removed because of arrhythmia,
malposition, or additional defects. At a mean follow-up of
1.4 years, only one patient had more than trivial shunt. No other
complications occurred.
Since the perimembranous VSD closure with devices is likely
to result in heart block as described in the “Percutaneous
closure” section, we do not advocate their closure by a hybrid
procedure. Detailed review of these hybrid procedure reports
reveals that many patients had small VSDs (<5 mm) or aneu-
rysmal formation of membranous ventricular septum, a natural
attempt for spontaneous closure, and the authors of this article
question the advisability of closure of such VSDs.
This hybrid technique is especially helpful in small babies with
large muscular VSDs in whom there is a higher prevalence of
adverse events for percutaneous device closure when compared
with older children.
Page 6 of 16
F1000Research 2018, 7(F1000 Faculty Rev):498 Last updated: 26 APR 2018
Comparison of various methods of intervention to occlude
ventricular septal defects
There are a limited number of studies comparing one method
of VSD closure with the other.
Percutaneous versus surgical closure. In one study, the results
of percutaneous closure in 852 patients were compared with
1,326 patients who had conventional surgical repair102. Proce-
dure success rates and prevalence of major complications were
similar. However, the rates of minor complications (6.4% versus
0.6%) and requirement of blood transfusions (10.3% versus
0%) and duration of hospitalization (12.9 versus 3.2 days) were
higher in the surgical than in the percutaneous group. The authors
concluded that the percutaneous approach is an effective and
reasonable alternative to surgery in the management of patients
with VSD102.
In another study, the results of percutaneous versus surgical
closure of perimembranous VSDs were compared by examining
the results of seven previously published articles; there were
1,312 percutaneous device implantations and 1,822 surgical
closures103. The patients in the percutaneous group were older
than those in the surgical group (mean age of 12.2 versus
5.5 years), but the VSD sizes were similar. Procedural success
rates, major complications such as need for reoperation, early
deaths, and requirement for permanent pacemaker implanta-
tion were similar as were the residual shunts, significant aortic
and tricuspid insufficiency, and advanced heart block at follow-
up. The higher requirement for blood transfusion and longer
duration of hospital stay were seen in the surgical group. The
authors concluded that percutaneous intervention and surgical
closure of perimembranous VSDs had similar procedural success
rates and that percutaneous closure did not manifest higher
rates of valvar insufficiency and heart block than surgical
closure103.
Hybrid versus surgical closure. A comparison of 49 hybrid
device closures with 41 surgical closures was made98. The ages
were similar, but the VSDs were slightly larger (6.03 versus
5.03 mm) in the surgical group. Major complications, namely
death, severe valvar insufficiency, significant residual shunts,
and lethal arrhythmias, did not occur in either group. Complete
closure rates immediately after procedure and at follow-up were
similar for the two groups. The requirement for blood transfu-
sion was higher and the length of the stay in intensive care unit
was longer in the surgical than in the hybrid group. The authors
conclude that hybrid VSD closure may be an alternative to
surgical closure98.
Comments on comparisons. Although the authors of the
above studies have made considerable efforts to compare
various groups, all studies appear to be retrospective and non-
randomized and may not be as definitive as the authors would
like us to believe. In addition, it should be observed that the
above-mentioned comparative studies do not compare identical
patient populations and there is a selection bias in the patients
managed with device closure because only ideal candidates are
managed with device closures, and more complex VSDs were
referred for surgery. Additionally, they do not take into account
that repair under cardiopulmonary bypass will entail a period of
ventilation and intensive care stay.
Conclusions
When one carefully examines the data of patients undergo-
ing VSD closure, many VSDs are less than 5 mm in size and the
Qp:Qs was less than 2:1. The availability of less invasive tran-
scatheter approaches should not, in the authors’ opinion, relax the
indications for closure and these indications should be the same
as those used for standard surgical closure. In addition, the natural
history studies indicate that spontaneous closure occurs in VSDs
and such closures continue to occur during childhood, adoles-
cence, and adulthood. The pediatricians and the general pediatric
cardiologists should serve as gatekeepers to prevent interven-
tion by pediatric cardiac surgeons and interventional pediatric
cardiologists for “small” VSDs that do not strictly fit estab-
lished criteria for closure, irrespective of the type of intervention,
whether it is conventional surgical, percutaneous, or hybrid.
Based on clinical experience for over 45 years in caring for
patients with VSDs by the senior author (PSR) and an extensive
review of the subject at the time of this writing, the follow-
ing recommendations may be made. Open-heart surgical closure
remains the main treatment option for large and non-restrictive
perimembranous VSDs. Timely intervention to prevent PVOD
is highly important and is in the purview of the pediatrician
and pediatric cardiologist caring for the child. Percutaneous
closure seems to be a valuable option for closure of large mus-
cular VSDs. A hybrid procedure is a good option for large
muscular VSDs in small infants. When that is not possible, par-
ticularly in babies with the Swiss-cheese type of VSDs, banding
of the pulmonary artery as an initial palliative procedure
with later closure of VSD is likely to be beneficial to these
infants.
Atrioventricular septal defects
What were formerly known as AV canals and endocardial
cushion defects are now termed as atrioventricular septal
defects (AVSDs). The characteristic features of this CHD include
defects in atrial and ventricular septa along with deficiency in
one or both AV valves. The AVSDs constitute 4 to 5% of all
CHDs. The AVSD is the most common defect in babies with
Down syndrome.
The AVSDs are classified into partial, transitional, intermediate,
and complete forms104. The partial form usually had a large ASD
in the anterior portion of the lower part of the atrial septum along
with a cleft in the anterior leaflet of the mitral valve. A cleft in the
septal leaflet of the tricuspid valve may also be present in some
patients. In the transitional form, there is an additional small inlet
VSD and the physiology is similar to that of the partial form.
The management of the partial form, also called ostium primum
ASD, and transitional form is similar and was discussed in our
previous review1 and will not be repeated here. The complete
form has one AV valve annulus, a large ostium primum ASD,
and a contiguous large inlet VSD. The intermediate form is
similar to the complete form with the exception that the single
Page 7 of 16
F1000Research 2018, 7(F1000 Faculty Rev):498 Last updated: 26 APR 2018
AV annulus is divided into two orifices by a tongue of tissue. The
complete forms are further divided into Rastelli types A, B,
and C on the basis of the characteristics of the anterior bridging
leaflet105. Another classification based on relative ventricu-
lar sizes is balanced and unbalanced AVSDs; the unbalanced
defects constitute 10 to 15% of all complete forms of AVSDs. The
unbalanced forms may be LV-dominant (large LV and small RV)
or RV-dominant (large RV and small LV); the RV-dominant
AVSDs are more common. The unbalanced AVSDs require
different types of surgical approaches.
Hemodynamic abnormalities in patients with AVSD are second-
ary to the left-to-right shunt across the ASD and VSD components
and mitral valve insufficiency. As mentioned in the “Ventricular
septal defects” section, the left-to-right shunt across the ASD
and VSD may not manifest in the neonate and young infant and
this is due to high PVR. As the pulmonary arterioles involute and
PVR falls, left-to-right shunt occurs, resulting in dilatation of the
left atrium and LV; this may take several weeks unless there is
an anomalous and rapid fall in PVR. Because the ASD and VSD
components are large, there is dilatation of the right atrium, RV,
and main and branch pulmonary arteries. If there is moderate to
severe mitral insufficiency, further dilatation of the left atrium
and LV ensues. PVOD may develop as early as six months to one
year of age in patients with complete AVSDs and even earlier in
babies with Down syndrome.
Discussion of AVSDs seen in association with tetralogy of
Fallot, pulmonary atresia/stenosis, transposition of the great
arteries, and double-outlet RV will not be included in this
review.
Indications for atrioventricular septal defect repair
Most, if not all, complete and intermediate types of AVSD have
large ASD and VSD components and therefore all patients with
AVSD should have their defects repaired. Alternatively, pulmo-
nary artery banding106 may be performed. At present, primary
complete repair is performed at most institutions. Pulmonary
artery banding may be considered for babies weighing less than
5 kg in whom the CHF could not be controlled and babies
with other significant co-morbidities107109.
In patients with elevated PVR, the considerations for
operability68 and pulmonary vascular reactivity testing6,8,9 are
similar to those described in the “Ventricular septal defects”
section and will not be repeated. Patients with AVSDs and
severely elevated PVR (that is, irreversible PVOD) are not suit-
able for AVSD repair. These patients eventually may become
candidates for lung transplantation.
Management
Medical and surgical treatment options for balanced and
unbalanced AVSDs will be reviewed separately.
Balanced atrioventricular septal defects (complete and
intermediate).
Medical management
Neonates with AVSDs with no signs of CHF do not need
treatment in the newborn period; however, we usually begin
treatment with infrequent doses of furosemide (once every other
day) since a predictable decrease of PVR with time occurs with
a resultant increase in pulmonary blood flow and development
of CHF. In a few weeks, these infants will develop CHF; at that
time, they should be managed with aggressive anticongestive
treatment, as reviewed in the “Ventricular septal defects”
section above, including optimization of nutrition, maintenance
of adequate hemoglobin level, and appropriately addressing the
associated respiratory symptoms.
Similar to what was discussed in the preceding section on
VSDs, patients with a severe increase of PVR (irreversible
PVOD) should not have their AVSDs closed. The management
of PVOD associated with AVSDs is the same as that discussed in
the VSD section.
Surgical management
In order to prevent the development of irreversible PVOD,
timely surgical repair, preferably prior to six months of age,
should be performed. Although it is our general impression
that babies with Down syndrome develop PVOD earlier than
non-Down babies, histological studies do not seem to indi-
cate any difference in pulmonary vascular changes between
Down and non-Down children110. Pulmonary alveolar and cap-
illary hypoplasia111 and chronic upper airway obstruction with
resultant hypoventilation producing hypoxia and hypercarbia
may be responsible in part for higher PVR112 and early PVOD
in babies with Down syndrome.
Corrective surgery. Surgical correction is performed after
placing the patient on cardiopulmonary bypass and consists of
patch closure of atrial and ventricular septal defects along with
repair and reconstruction of AV valves. Closure of ASD and VSD
components may be performed by using either a single-patch
technique (pericardial) or two-patch technique (pericardial patch
for primum ASD closure and pericardial or Dacron patch for the
VSD). In all instances, the common AV valve is separated into
left and right components; the left AV valve cleft is completely or
partially closed on the basis of subvalvar anatomy, and the right
AV valve cleft may be closed as indicated. In some instances,
the Australian repair (primary closure of VSD component with
septation of the common AV valve, closure of AV valve cleft,
primum ASD closure with pericardial patch) may be considered.
A recent metal analysis comparing single-patch with two-patch
technique demonstrated no significant difference between two
groups with comparable outcomes113; however, the cardiopul-
monary bypass and aortic cross-clamp times were shorter in the
single-patch method of closure of the defect113. AV valves are
Page 8 of 16
F1000Research 2018, 7(F1000 Faculty Rev):498 Last updated: 26 APR 2018
repaired/reconstructed by using the techniques described by
Carpentier114, Puga and McGoon115, or Chopra et al.116. Associ-
ated defects such as patent ductus arteriosus and left ventricu-
lar outflow tract obstruction (usually fibromuscular membrane)
should be addressed at the same sitting. In the present day, TEE
is performed to evaluate mitral and tricuspid valve function
(mitral/tricuspid insufficiency and stenosis) and residual shunts
prior to decannuation from cardiopulmonary bypass. Re-repair
of mitral (or, in rare cases, tricuspid) valve is generally undertaken
if the degree of insufficiency or stenosis is more than mild.
Pulmonary artery banding. As mentioned above, banding of
the pulmonary artery may be performed in babies weighing less
than 5 kg in whom the CHF could not be controlled and when
associated with significant co-morbidities. The banding should
be tight enough to produce near normal pressures distal to the
band, but care should be taken to avoid any deterioration in LV
function based on intra-operative transesophageal echocar-
diogram. Corrective surgery along with the removal of the band
may be performed a few months later when the infant improves
clinically. However, the trend is for complete repair at most
institutions.
Surgical results
A large number of investigators reported results of surgi-
cal repair of AVSDs. We examined the results of some of
these studies116125, which included 37 to 1,917 patients. The
immediate mortality rates varied between 2 and 62%116125.
Some studies compared the results of the earlier era with those
of the present time, these comparisons have consistently shown
improvement of mortality rates in recent years. More recent stud-
ies demonstrated a substantial fall in mortality rates (down to 2 to
3%)122,124,125. Although there are differences in risk factors for
surgical mortality from one study to the next, the most commonly
found risk factors were preoperative severity of mitral regurgita-
tion and New York Heart Association (NYHA) functional class.
Other risk factors identified in some studies were very young
age, small size of the babies, postoperative residual mitral valve
regurgitation, ventricular hypoplasia (unbalanced ventricular
sizes), additional muscular VSDs, and double-orifice mitral valve.
There is some early concern regarding poor tolerance for
surgery in the patients with Down syndrome. One relatively
recent study126 compared the outcomes and complications
between Down and non-Down babies. There was no postopera-
tive mortality in either group. Cardiac complications such as the
prevalence of junctional ectopic tachycardia, the need for early
reoperation, and the requirement for insertion of a permanent
pacemaker for complete heart block were similar in the two
groups. However, there was higher prevalence of non-cardiac
complications such as pneumothorax, pleural effusions, and
infections in infants with Down syndrome than in those without
Down syndrome. However, these non-cardiac complications
did not increase duration of stay in the pediatric intensive care
unit. These data affirm our thinking that babies with Down
syndrome should receive the same treatment as those without.
In fact, non-Down babies tend to have more abnormal left AV
valve morphology, and AVSD repair in children with Down
syndrome is more straightforward than that in babies without
Down syndrome. In the current era, surgery is the therapy of
choice in all infants and ideally is performed between three and
six months of age.
In studies in which long-term follow-up data were available,
actuarial survival rates were reported be 91%, 91%, and 89%
at 1, 5, and 15 years, respectively123, and 85%, 82%, and 71% at
10, 20, and 30 years, respectively124. The estimated rates of
freedom from late reoperation were 96% at 1 year, 89% at 5 years,
and 89% at 15 years123. Reoperation may be required in 10 to
15% of patients; mitral valve regurgitation (7 to 10%) is the most
common reason for late reoperation, followed by left ventricular
outflow tract obstruction (3.5%). A small percentage of patients
may require surgery for tricuspid regurgitation, mitral stenosis,
or residual shunts. Risk factors for late reoperation were other
cardiovascular anomalies, mitral valve dysplasia, and absence of
mitral cleft closure during the initial surgery123.
Unbalanced atrioventricular septal defects.
Medical management
The medical management at presentation is similar to that
described for balanced AVSDs.
Surgical management
Several surgical options are in use for treating unbalanced
AVSDs and will be reviewed.
Single-ventricle palliation (Fontan)
The reparative techniques described above for balanced AVSDs
cannot be used in unbalanced AVSDs because of hypoplasia of
the RVs or LVs which after the repair may not be able to support
the pulmonary or systemic circulations, respectively. Marginal-
sized RVs may be addressed with a combination of biventricu-
lar repair and bidirectional Glenn procedure. By and large,
these patients require a staged Fontan surgery similar to that
described for tricuspid atresia and other single-ventricle (SV)
lesions125131. Remarkable changes in the concepts and methods
of surgery have occurred since the initial description of right
ventricular bypass by a complex procedure by Fontan125 and a
simpler atriopulmonary anastamosis by Kruetzer126. The
descriptions of bidirectional Glenn132134, total cavopulmonary
connection135, extracardiac non-valved conduits136,137, staged
procedures133,134, creation of fenestration138140, and device closure
of fenestration139 have resulted in the evolution of the procedure
such that it is now a staged (three-stage) total cavopulmonary
connection with an extracardiac conduit and fenestration with
subsequent device closure of the fenestration129131.
Following control of CHF, as described above in the “Balanced
atrioventricular septal defects” section, pulmonary artery band-
ing is performed to limit the pulmonary blood flow, control CHF,
and normalize the pulmonary artery pressures (stage I of Fontan).
At about six months of age, a bidirectional Glenn procedure is
performed (stage II of Fontan). Prior to the procedure, one must
be assured of normal pulmonary artery pressures; if this cannot
be done by echo-Doppler studies, cardiac catheterization may
be required. In the presence of a persistent left superior vena
Page 9 of 16
F1000Research 2018, 7(F1000 Faculty Rev):498 Last updated: 26 APR 2018
cava, a bilateral, bidirectional Glenn procedure may be required,
especially if the bridging left innominate vein is absent or small.
If there is significant AV valve regurgitation or other hemody-
namically significant abnormalities, they should be appropriately
addressed at this stage. One year after the bidirectional Glenn (or
between one and four years of age), the inferior vena caval flow
is diverted into the pulmonary artery by either a lateral tunnel or
an extracardiac non-valved conduit (stage IIIA); most surgeons
seem to prefer extracardiac conduit with fenestration. Cardiac
catheterization and selective cine-angiography are recommended
(to evaluate pulmonary artery anatomy and pressures, transpul-
monary gradient, PVR, and left ventricular end-diastolic pressure)
prior to stages II and III to ensure suitability for proceeding with
the next stage of surgery. Six months to one year after stage
IIIA, the fenestration is closed percutaneously with a device
(stage IIIB). Detailed angiographic anatomy at each Fontan stage
is demonstrated in a prior publication131, and the interested reader
is referred to that article.
Results of single-ventricle palliation (Fontan). The operative
mortality in patients who had staged, cavopulmonary connec-
tion with fenestration appears to be between 4.5 and 7.5%131,141.
On follow-up, actuarial survival rates were 93% at 5 years and
91% at 10 years142. In another study, the actuarial survival was
85% at 15 years143. The re-intervention (surgical or percutane-
ous) rate was 12.7%. It should be noted that the patients in these
studies included all types of SV lesions, namely tricuspid
atresia, double-inlet left (single) ventricle, hypoplastic left heart
syndrome, mitral atresia with normal aortic root, unbalanced
complete AVSDs, pulmonary atresia with intact ventricular
septum with markedly hypoplastic RV, and any complex heart
defect with one functioning ventricle. When one examines
Fontan procedures in patients with unbalanced AVSDs, the
mortality rates (17 to 31.7%) were higher144,145 and actuarial
survival rates (66.5% at 5 years and 64.4% at 15 years) were
lower146 and there were more surgical and catheter re-
interventions (51.9%)145. Equally poor results with an overall
long-term survival rate of 50% were observed in another study;
these results were poorer than for a cohort with hypoplastic left
heart syndrome patients operated during the same period146.
It should be noted that complications, namely arrhythmias,
Fontan pathway obstruction, cyanosis, paradoxical emboli,
formation of thrombus, collateral vessels, and protein-losing
enteropathy, may be seen on long-term follow-up, although most
of these complication are less common with staged total cavo-
pulmonary connection than with earlier versions of Fontan130,131.
Two-ventricle repair
The AV valves are repaired to create nearly equal-sized AV valves
along with complete (single stage) or partial (staged) closure
of ASD and VSD components144,147151. In the staged procedure,
the residual defects are repaired surgically after demonstration
of growth of the hypoplastic ventricle.
The criteria used to select a given patient to SV versus
two-ventricle (TV) route are not clearly established. Several
investigators examined this issue by evaluating potential echocar-
diographic LV volume after normalization of septal bowing,
ventricular cavity ratio between the two ventricles (estimated
as left ventricular length × width/right ventricular length
× width), AV valve index (left AV valve area/total AV valve area), a
different type of AV valve index (left valve area/right valve area),
ventricular dimensions, LV end-diastolic volume index, RV/LV
inflow angle in systole, left AV valve color diameter at smallest
inflow, left AV valve color diameter at annulus, indexed ventricular
septal defect (defined as the size of the defect in relation to the
common AV valve diameter), left ventricular inflow index
(calculated as the secondary color inflow diameter indexed to the
left AV valve annulus diameter), and other indices145,151159.
At present, no clear-cut, uniform echocardiographic criteria are
established for selecting a given unbalanced AVSD patient for
TV repair. However, some investigators advocate TV repair if AV
valve index (left/right valve area) is more than 0.5149, more than
0.65150, or more than 0.67152 for right dominant unbalanced
AVSDs. Other suggestions for selecting TV repair are indexed
potential LV volume of more than 15 mL/m2151, left ventricu-
lar inflow index greater than 0.5154, and indexed ventricular
septal defect smaller than 0.2158. The last group of investiga-
tors158 also recommended that patients with indexed VSD values
between 0.2 and 0.35 may be considered for TV repair on the
basis of other factors. If the indexed VSD value is between 0.35
and 0.5, SV palliation may be required. Other investigators
examined various echo-Doppler parameters and made a correla-
tion between these parameters and survivals but did not include
definitive cutoff points for TV repair. Another group of investi-
gators used angiographic measures and suggested that patients
with LV-to-RV long axis ratio of more than 0.65 on cine-
angiograms may be suitable for TV repair150. Perhaps a com-
bination of indexed VSD, AV valve index, and RV/LV inflow
angle in systole may guide the selection of patients for TV
repair158,160.
Results of two-ventricle repair. There are limited data with
regard to the results of TV repair. The mortality rates
varied between 10.4 and 18%144,147,153, although it was a higher
(40%) in an earlier series152. Long-term survival was good and
ranged from 88 to 90%144,147,150. However, surgical (17.4 to
34%) or transcatheter (13.4%) re-interventions were frequently
required144,147,150. But improvement of AV valve Z-scores
(−2.8 to −7.4 versus −0.6 to −2.7 )147, ventricular dimension
Z-scores (−1.0 to −7.5 versus −2.0 to +1.8)147, and LV end-
diastolic volume Z-scores (from a median of −3.15 to +0.42)144
occurred at follow-up.
Conversion from single-ventricle to two-ventricle repair
This group involves patients who initially had SV palliation
but were later converted to TV circulation. This approach
was precipitated by suboptimal long-term outcomes after SV
palliation159. Rehabilitation of the left heart is undertaken by
promotion of flow through the LV by relief of inflow or
outflow tract obstructions (or both) and resection of endocardial
fibroelastosis (if present). Restriction of the atrial septum to
promote flow through the LV is also undertaken.
Results of conversion from single-ventricle to two-ventricle
repair. The reported mortality following conversion to TV
circulation is low (1 to 11%)144,161. Surgical (19%) and catheter
Page 10 of 16
F1000Research 2018, 7(F1000 Faculty Rev):498 Last updated: 26 APR 2018
(38%) re-interventions were required during follow-up161.
Although the initial Z-scores of the LV prior to stage I SV
palliation were not significantly different, the LV Z-scores
increased significantly in the SV to TV group but decreased in
the SV palliation group without conversion159. Another study
from the same institution162 demonstrated improvement of left
ventricular end-diastolic volume by echocardiography from 28.1
to 58.5 mL/m2 in a small group of the unbalanced AVSD group
following SV-to-TV conversion162.
A recent study examined mid-term outcomes of primary and
staged biventricular repair and SV-to-TV conversion of unbal-
anced complete AVSDs and concluded that these methods result
in low morbidity and mortality although re-interventions (both
surgical and catheter) in 52% of patients were required145.
Comparison of various methods of intervention for unbalanced
atrioventricular septal defects
Nathan et al. compared the outcomes of patient cohorts who
had SV palliation, TV repair, and conversion from single-
ventricle to two-ventricle (SV to TV)144. The TV repair and
SV-to-TV groups were reasonably similar with regard to
mortality and the need for heart transplantation but were lower
than that seen with the SV palliation cohort during a mean
follow-up of 35 months. Although the surgical re-interventions
were similar in the three groups, catheter re-interventions were
lower in the TV repair group than in the other two groups.
They conclude that TV repair and SV-to-TV conversion may be
accomplished with lower mortality and morbidity rates in chil-
dren with unbalanced AVSDs, although the study was critiqued163
for allowing survivorship bias in favor of TV repair and SV-to-
TV groups. Conversion from SV palliation and biventricular
recruitment remains an important option for children with
Down syndrome and heterotaxy syndrome for physiologic and
anatomic reasons. Patients with Down syndrome tolerate SV
physiology poorly164. Patients with heterotaxy syndrome have
complex and abnormal AV valve anatomy and require complex
and innovative repair techniques. They also tolerate AV valve
regurgitation poorly when palliated via the SV pathway.
Conclusions
Treatment of CHF (if present) followed by surgical repair
with closure of atrial and ventricular septal defects along with
repair and reconstruction of AV valves is a standard approach in
babies with balanced AVSDs. Pulmonary artery banding may be
performed to palliate infants weighing less than 5 kg as well as
those with significant co-morbidities. Timely intervention to
prevent PVOD is exceedingly important. The management of
unbalanced AVSDs is complex; staged SV palliation (Fontan) is
performed at most institutions, but recent data seem to indicate
that primary or staged TV repair or converting SV to TV repair
may be better choices. The management of babies with Down
syndrome should be similar to that used for non-Down infants.
Author contributions
PSR provided conceptualization, methodology, project
administration, and supervision and contributed to the writing of
the article by reviewing and editing the manuscript. ADH
contributed to the writing of the article by preparing the original
draft.
Competing interests
The authors declare that they have no competing interests.
Grant information
The author(s) declared that no grants were involved in supporting
this work.
1. Rao PS, Harris AD: Recent advances in managing septal defects: atrial septal
defects [version 1; referees: 2 approved]. F1000Res. 2017; 6: 2042.
PubMed Abstract
|
Publisher Full Text
|
Free Full Text
2. Fyler DC: Nadas’ Pediatric Cardiology. Hanly & Belfus, Inc., Philadelphia, PA. 1992.
Reference Source
3. Syamasundar Rao P: Diagnosis and management of acyanotic heart disease:
part II -- left-to-right shunt lesions. Indian J Pediatr. 2005; 72(6): 503–12.
PubMed Abstract
|
Publisher Full Text
4. McDaniel NL, Gutgesell P: Ventricular septal defects. In: Allen HD, Driscoll DJ,
Shaddy RE, Felts TF. Eds. Moss & Adams’ Heart Disease in Infants, Children, and
Adolescents: Including the Fetus and Young Adult. 7th ed. Philadelphia, PA: Wolters
Kluwer/Lippincott Williams & Wilkins; 2008; 667–682.
Reference Source
5. Rao PS: Congenital heart defects – A review. In: Rao PS, ed. Congenital Heart
Disease - Selected Aspects. InTech, ISBN 978-953-307-472-6, Rijeka, Croatia,
2011; 3–44.
Reference Source
6. Rao PS: Consensus on timing of intervention for common congenital heart
diseases: part I - acyanotic heart defects. Indian J Pediatr. 2013; 80(1): 32–8.
PubMed Abstract
|
Publisher Full Text
7. Viswanathan S, Kumar RK: Assessment of operability of congenital cardiac
shunts with increased pulmonary vascular resistance. Catheter Cardiovasc
Interv. 2008; 71(5): 665–70.
PubMed Abstract
|
Publisher Full Text
8. Beghetti M: A classification system and treatment guidelines for PAH
associated with congenital heart disease. Adv Pulm Hypertens. 2006; 5(2): 31–5.
Reference Source
9. Balzer DT, Kort HW, Day RW, et al.: Inhaled Nitric Oxide as a Preoperative Test
(INOP Test I): the INOP Test Study Group. Circulation. 2002; 106(12 Suppl 1):
I76–81.
PubMed Abstract
10. Nadas AS, Thilenius OG, Lafarge CG, et al.: Ventricular Septal Defect with Aortic
Regurgitation: Medical and Pathologic Aspects. Circulation. 1964; 29: 862–73.
PubMed Abstract
|
Publisher Full Text
11. Leung MP, Beerman LB, Siewers RD, et al.: Long-term follow-up after aortic
valvuloplasty and defect closure in ventricular septal defect with aortic
regurgitation. Am J Cardiol. 1987; 60(10): 890–4.
PubMed Abstract
|
Publisher Full Text
12. Yacoub MH, Khan H, Stavri G, et al.: Anatomic correction of the syndrome
of prolapsing right coronary aortic cusp, dilatation of the sinus of Valsalva,
and ventricular septal defect. J Thorac Cardiovasc Surg. 1997; 113(2): 253–60;
discussion 261.
PubMed Abstract
|
Publisher Full Text
13. McDaniel N, Gutgesell HP, Nolan SP, et al.: Repair of large muscular ventricular
septal defects in infants employing left ventriculotomy. Ann Thorac Surg. 1989;
47(4): 593–4.
PubMed Abstract
|
Publisher Full Text
14. Hannan RL, McDaniel N, Kron IL: As originally published in 1989: Repair of large
muscular ventricular septal defects in infants employing left ventriculotomy.
Updated in 1996. Ann Thorac Surg. 1997; 63(1): 288–9.
PubMed Abstract
15. Bonnet D, Sidi D, Vouhé PR: Absorbable pulmonary artery banding in tricuspid
atresia. Ann Thorac Surg. 2001; 71(1): 360–1; discussion 361–2.
PubMed Abstract
|
Publisher Full Text
References
F1000 recommended
Page 11 of 16
F1000Research 2018, 7(F1000 Faculty Rev):498 Last updated: 26 APR 2018
16. Rao PS: Absorbable pulmonary artery band in tricuspid atresia (Editorial). Ann
Thorac Surg. 2001; 71(1): 361–2.
Publisher Full Text
17. Moller JH, Patton C, Varco RL, et al.: Late results (30 to 35 years) after operative
closure of isolated ventricular septal defect from 1954 to 1960. Am J Cardiol.
1991; 68(15): 1491–7.
PubMed Abstract
|
Publisher Full Text
18. Roos-Hesselink JW, Meijboom FJ, Spitaels SE, et al.: Outcome of patients after
surgical closure of ventricular septal defect at young age: longitudinal follow-
up of 22–34 years. Eur Heart J. 2004; 25(12): 1057–62.
PubMed Abstract
|
Publisher Full Text
19. Cordell D, Graham TP Jr, Atwood GF, et al.: Left heart volume characteristics
following ventricular septal defect closure in infancy. Circulation. 1976; 54(2):
294–8.
PubMed Abstract
|
Publisher Full Text
20. Rashkind WJ: Experimental transvenous closure of atrial and ventricular septal
defects. Circulation. 1975; 52: II–8.
Reference Source
21. Lock JE, Block PC, McKay RG, et al.: Transcatheter closure of ventricular septal
defects. Circulation. 1988; 78(2): 361–8.
PubMed Abstract
|
Publisher Full Text
22. O’Laughlin MP, Mullins CE: Transcatheter occlusion of ventricular septal defect.
Cathet Cardiovasc Diagn. 1989; 17(3): 175–9.
PubMed Abstract
|
Publisher Full Text
23. Goldstein SA, Perry SB, Keane JF, et al.: Transcatheter closure of congenital
ventricular septal defects. J Am Coll Cardiol. 1990; 15(2): A240.
Publisher Full Text
24. Rigby ML, Redington AN: Primary transcatheter umbrella closure of
perimembranous ventricular septal defect. Br Heart J. 1994; 72(4): 368–71.
PubMed Abstract
|
Publisher Full Text
|
Free Full Text
25. Janorkar S, Goh T, Wilkinson J: Transcatheter closure of ventricular septal
defects using the Rashkind device: initial experience. Catheter Cardiovasc
Interv. 1999; 46(1): 43–8.
PubMed Abstract
|
Publisher Full Text
26. Bridges ND, Perry SB, Keane JF, et al.: Preoperative transcatheter closure of
congenital muscular ventricular septal defects. N Engl J Med. 1991; 324(19):
1312–7.
PubMed Abstract
|
Publisher Full Text
27. Sideris EB, Walsh KP, Haddad JL, et al.: Occlusion of congenital ventricular
septal defects by the buttoned device. “Buttoned device” Clinical Trials
International Register. Heart. 1997; 77(3): 276–9.
PubMed Abstract
|
Publisher Full Text
|
Free Full Text
28. Sideris EB, Haddad J, Rao PS: The Role of the ‘Sideris’ Devices in the
Occlusion of Ventricular Septal Defects. Curr Interv Cardiol Rep. 2001; 3(4):
349–53.
PubMed Abstract
29. Marshall AC, Perry SB: Cardioseal/Starflex devices. In: Rao PS, Kern MJ (eds).
Catheter Based Devices for the Treatment of Noncoronar y Cardiovascular Disease
in Adults and Children. Lippincott, Williams & Wilkins: Philadelphia, PA, 2003;
253–8.
30. Le T, Vassen P, Freudenthal F, et al.: Nit-Occlud (Nickel-Titanium Spiral Coil). In:
Rao PS, Kern MJ (eds). Catheter Based Devices for the Treatment of Noncoronar y
Cardiovascular Disease in Adults and Children. Lippincott, Williams & Wilkins:
Philadelphia, PA, 2003; 259–64.
31. Odemis E, Saygi M, Guzeltas A, et al.: Transcatheter closure of perimembranous
ventricular septal defects using Nit-Occlud® Lê VSD coil: early and mid-term
results. Pediatr Cardiol. 2014; 35(5): 817–23.
PubMed Abstract
|
Publisher Full Text
32. Haas NA, Kock L, Bertram H, et al.: Interventional VSD-Closure with the
Nit-Occlud®Lê VSD-Coil in 110 Patients: Early and Midterm Results of the
EUREVECO-Registry. Pediatr Cardiol. 2017; 38(2): 215–27.
PubMed Abstract
|
Publisher Full Text
|
F1000 Recommendation
33. Phan QT, Kim SW, Nguyen HL: Percutaneous closure of congenital Gerbode
defect using Nit-Occlud®VSD coil. World J Cardiol. 2017; 9(7): 634–9.
PubMed Abstract
|
Publisher Full Text
|
Free Full Text
|
F1000 Recommendation
34. Thanopoulos BD, Tsaousis GS, Konstadopoulou GN, et al.: Transcatheter closure
of muscular ventricular septal defects with the amplatzer ventricular septal
defect occluder: initial clinical applications in children. J Am Coll Cardiol. 1999;
33(5): 1395–9.
PubMed Abstract
|
Publisher Full Text
35. Tofeig M, Patel RG, Walsh KP: Transcatheter closure of a mid-muscular
ventricular septal defect with an amplatzer VSD occluder device. Heart. 1999;
81(4): 438–40.
PubMed Abstract
|
Publisher Full Text
|
Free Full Text
36. Hijazi ZM, Hakim F, Al-Fadley F, et al.: Transcatheter closure of single muscular
ventricular septal defects using the amplatzer muscular VSD occluder: initial
results and technical considerations. Catheter Cardiovasc Interv. 2000; 49(2):
167–72.
PubMed Abstract
|
Publisher Full Text
37. Waight DJ, Bacha EA, Kahana M, et al.: Catheter therapy of Swiss cheese
ventricular septal defects using the Amplatzer muscular VSD occluder.
Catheter Cardiovasc Interv. 2002; 55(3): 355–61.
PubMed Abstract
|
Publisher Full Text
38. Chessa M, Carminati M, Cao QL, et al.: Transcatheter closure of congenital and
acquired muscular ventricular septal defects using the Amplatzer device.
J Invasive Cardiol. 2002; 14(6): 322–7.
PubMed Abstract
39. Du ZD, Cao QL, Waight D, et al.: [Transcatheter closure of Swiss cheese like
ventricular septal defects in children using the Amplatzer muscular VSD
occluder]. Zhonghua Er Ke Za Zhi. 2003; 41(3): 232–3.
PubMed Abstract
40. Kalra GS, Verma PK, Singh S, et al.: Transcatheter closure of ventricular septal
defect using detachable steel coil. Heart. 1999; 82(3): 395–6.
PubMed Abstract
|
Publisher Full Text
|
Free Full Text
41. Latiff HA, Alwi M, Kandhavel G, et al.: Transcatheter closure of multiple muscular
ventricular septal defects using Gianturco coils. Ann Thorac Surg. 1999; 68(4):
1400–1.
PubMed Abstract
|
Publisher Full Text
42. Nogi S, Haneda N, Tomita H, et al.: Transcatheter coil occlusion of
perimembranous ventricular septal defects. Catheter Cardiovasc Interv. 2008;
72(5): 683–90.
PubMed Abstract
|
Publisher Full Text
43. Sideris EB: Buttoned and other devices. In: Rao PS, Kern MJ (eds): Catheter
Based Devices for the Treatment of Noncoronar y Cardiovascular Disease in Adults
and Children. Lippincott, Williams & Wilkins, Philadelphia, PA, 2003; 239–44.
44. Fraisse A, Agnoletti G, Bonhoeffer P, et al.: [Multicentre study of percutaneous
closure of interventricular muscular defects with the aid of an Amplatzer duct
occluder prosthesis]. Arch Mal Coeur Vaiss. 2004; 97(5): 484–8.
PubMed Abstract
45. Yang J, Yang L, Wan Y, et al.: Transcatheter device closure of
perimembranous ventricular septal defects: mid-term outcomes. Eur Heart J.
2010; 31(18): 2238–45.
PubMed Abstract
|
Publisher Full Text
|
Free Full Text
|
F1000 Recommendation
46. Koneti NR, Penumatsa RR, Kanchi V, et al.: Retrograde transcatheter closure
of ventricular septal defects in children using the Amplatzer Duct Occluder II.
Catheter Cardiovasc Interv. 2011; 77(2): 252–9.
PubMed Abstract
|
Publisher Full Text
47. El Tahlawi M, Kammache I, Fraisse A: Ventricular septal defect closure in a
small children with the Amplatzer Duct Occluder II. Catheter Cardiovasc Interv.
2011; 77(2): 268–71.
PubMed Abstract
|
Publisher Full Text
48. Zhao PJ, Yu ZQ, Gao W, et al.: [Efficacy of the transcatheter closure of
perimembranous and muscular ventricular septal defects with the Amplatzer
duct occluder II]. Zhonghua Xin Xue Guan Bing Za Zhi. 2012; 40(10): 817–20.
PubMed Abstract
|
Publisher Full Text
49. Koneti NR, Sreeram N, Penumatsa RR, et al.: Transcatheter retrograde closure
of perimembranous ventricular septal defects in children with the Amplatzer
duct occluder II device. J Am Coll Cardiol. 2012; 60(23): 2421–2.
PubMed Abstract
|
Publisher Full Text
50. Lee SM, Song JY, Choi JY, et al.: Transcatheter closure of perimembranous
ventricular septal defect using Amplatzer ductal occluder. Catheter Cardiovasc
Interv. 2013; 82(7): 1141–6.
PubMed Abstract
|
Publisher Full Text
51. Kanaan M, Ewert P, Berger F, et al.: Follow-up of patients with interventional
closure of ventricular septal defects with Amplatzer Duct Occluder II. Pediatr
Cardiol. 2015; 36(2): 379–85.
PubMed Abstract
|
Publisher Full Text
52. Vijayalakshmi IB, Natraj Setty HS, Chitra N, et al.: Amplatzer duct occluder II for
closure of congenital Gerbode defects. Catheter Cardiovasc Interv. 2015; 86(6):
1057–62.
PubMed Abstract
|
Publisher Full Text
53. Suligoj B, Cernic N, Zorc M, et al.: Retrograde transcatheter closure of
ventricular septal defect with Amplatzer Duct Occluder II. Postepy Kardiol
Interwencyjnej. 2016; 12(2): 177–8.
PubMed Abstract
|
Publisher Full Text
|
Free Full Text
54. Polat TB, Türkmen E: Transcatheter closure of ventricular septal defects
using the Amplatzer Duct Occluder II device: a single-center experience.
Postepy Kardiol Interwencyjnej. 2016; 12(4): 340–7.
PubMed Abstract
|
Publisher Full Text
|
Free Full Text
|
F1000 Recommendation
55. El-Sisi A, Sobhy R, Jaccoub V, et al.: Perimembranous Ventricular Septal
Defect Device Closure: Choosing Between Amplatzer Duct Occluder I and II.
Pediatr Cardiol. 2017; 38(3): 596–602.
PubMed Abstract
|
Publisher Full Text
|
F1000 Recommendation
56. Pamukcu O, Narin N, Baykan A, et al.: Mid-term results of percutaneous
ventricular septal defect closure with Amplatzer Duct Occluder-II in children.
Cardiol Young. 2017; 27(9): 1726–31.
PubMed Abstract
|
Publisher Full Text
|
F1000 Recommendation
57. Esteves CA, Solarewicz LA, Cassar R, et al.: Occlusion of the perimembranous
ventricular septal defect using CERA® devices. Catheter Cardiovasc Interv.
2012; 80(2): 182–7.
PubMed Abstract
|
Publisher Full Text
58. Gu X, Han YM, Titus JL, et al.: Transcatheter closure of membranous ventricular
Page 12 of 16
F1000Research 2018, 7(F1000 Faculty Rev):498 Last updated: 26 APR 2018
septal defects with a new nitinol prosthesis in a natural swine model. Catheter
Cardiovasc Interv. 2000; 50(4): 502–9.
PubMed Abstract
|
Publisher Full Text
59. Hijazi ZM, Hakim F, Haweleh AA, et al.: Catheter closure of perimembranous
ventricular septal defects using the new Amplatzer membranous VSD occluder:
initial clinical experience. Catheter Cardiovasc Interv. 2002; 56(4): 508–15.
PubMed Abstract
|
Publisher Full Text
60. Bass JL, Kalra GS, Arora R, et al.: Initial human experience with the Amplatzer
perimembranous ventricular septal occluder device. Catheter Cardiovasc Interv.
2003; 58(2): 238–45.
PubMed Abstract
|
Publisher Full Text
61. Pedra CA, Pedra SR, Esteves CA, et al.: Percutaneous closure of
perimembranous ventricular septal defects with the Amplatzer device:
technical and morphological considerations. Catheter Cardiovasc Interv. 2004;
61(3): 403–10.
PubMed Abstract
|
Publisher Full Text
62. Masura J, Gao W, Gavora P, et al.: Percutaneous closure of perimembranous
ventricular septal defects with the eccentric Amplatzer device: multicenter
follow-up study. Pediatr Cardiol. 2005; 26(3): 216–9.
PubMed Abstract
|
Publisher Full Text
63. Carminati M, Butera G, Chessa M, et al.: Transcatheter closure of congenital
ventricular septal defect with Amplatzer septal occluders. Am J Cardiol. 2005;
96(12A): 52L–58L.
PubMed Abstract
|
Publisher Full Text
64. Holzer R, de Giovanni J, Walsh KP, et al.: Transcatheter closure of
perimembranous ventricular septal defects using the amplatzer membranous
VSD occluder: immediate and midterm results of an international registry.
Catheter Cardiovasc Interv. 2006; 68(4): 620–8.
PubMed Abstract
|
Publisher Full Text
65. Thanopoulos BV, Rigby ML, Karanasios E, et al.: Transcatheter closure of
perimembranous ventricular septal defects in infants and children using the
Amplatzer perimembranous ventricular septal defect occluder. Am J Cardiol.
2007; 99(7): 984–9.
PubMed Abstract
|
Publisher Full Text
66. Pinto RJ, Dalvi BV, Sharma S: Transcatheter closure of perimembranous
ventricular septal defects using amplatzer asymmetric ventricular septal
defect occluder: preliminary experience with 18-month follow up. Catheter
Cardiovasc Interv. 2006; 68(1): 145–52.
PubMed Abstract
|
Publisher Full Text
67. Fu YC, Bass J, Amin Z, et al.: Transcatheter closure of perimembranous
ventricular septal defects using the new Amplatzer membranous VSD
occluder: results of the U.S. phase I trial. J Am Coll Cardiol. 2006; 47(2): 319–25.
PubMed Abstract
|
Publisher Full Text
|
F1000 Recommendation
68. Butera G, Carminati M, Chessa M, et al.: Transcatheter closure of
perimembranous ventricular septal defects: early and long-term results.
J Am Coll Cardiol. 2007; 50(12): 1189–95.
PubMed Abstract
|
Publisher Full Text
69. Yip WC, Zimmerman F, Hijazi ZM: Heart block and empirical therapy after
transcatheter closure of perimembranous ventricular septal defect. Catheter
Cardiovasc Interv. 2005; 66(3): 436–41.
PubMed Abstract
|
Publisher Full Text
70. Butera G, Massimo C, Mario C: Late complete atriovenous block after
percutaneous closure of a perimembranous ventricular septal defect. Catheter
Cardiovasc Interv. 2006; 67(6): 938–41.
PubMed Abstract
|
Publisher Full Text
71. Fischer G, Apostolopoulou SC, Rammos S, et al.: The Amplatzer Membranous
VSD Occluder and the vulnerability of the atrioventricular conduction system.
Cardiol Young. 2007; 17(5): 499–504.
PubMed Abstract
|
Publisher Full Text
72. Kramoh EK, Dahdah N, Fournier A, et al.: Invasive measurements of atrioventricular
conduction parameters prior to and following ventricular septal defect closure
with the amplatzer device. J Invasive Cardiol. 2008; 20(5): 212–6.
PubMed Abstract
73. Predescu D, Chaturvedi RR, Friedberg MK, et al.: Complete heart block
associated with device closure of perimembranous ventricular septal defects.
J Thorac Cardiovasc Surg. 2008; 136(5): 1223–8.
PubMed Abstract
|
Publisher Full Text
74. Carminati M, Butera G, Chessa M, et al.: Transcatheter closure of congenital
ventricular septal defects: results of the European Registry. Eur Heart J. 2007;
28(19): 2361–8.
PubMed Abstract
|
Publisher Full Text
75. Chungsomprasong P, Durongpisitkul K, Vijarnsorn C, et al.: The results of
transcatheter closure of VSD using Amplatzer® device and Nit Occlud® Lê
coil. Catheter Cardiovasc Interv. 2011; 78(7): 1032–40.
PubMed Abstract
|
Publisher Full Text
76. Zuo J, Xie J, Yi W, et al.: Results of transcatheter closure of perimembranous
ventricular septal defect. Am J Cardiol. 2010; 106(7): 1034–7.
PubMed Abstract
|
Publisher Full Text
77. Pace Napoleone C, Gargiulo G: Septal defects: surgeons do it better.
J Cardiovasc Med (Hagerstown). 2007; 8(1): 46–9.
PubMed Abstract
|
Publisher Full Text
78. Rao PS: Perimembranous ventricular septal defect closure with the amplatzer
device. J Invasive Cardiol. 2008; 20(5): 217–8.
PubMed Abstract
79. Bonatti V, Agnetti A, Squarcia U: Early and late postoperative complete heart
block in pediatric patients submitted to open-heart surgery for congenital
heart disease. Pediatr Med Chir. 1998; 20(3): 181–6.
PubMed Abstract
80. Holzer R, Balzer D, Cao QL, et al.: Device closure of muscular ventricular septal
defects using the Amplatzer muscular ventricular septal defect occluder:
immediate and mid-term results of a U.S. registry. J Am Coll Cardiol. 2004;
43(7): 1257–63.
PubMed Abstract
|
Publisher Full Text
81. Arora R, Trehan V, Thakur AK, et al.: Transcatheter closure of congenital
muscular ventricular septal defect. J Interv Cardiol. 2004; 17(2): 109–15.
PubMed Abstract
|
Publisher Full Text
82. Djer MM, Latiff HA, Alwi M, et al.: Transcatheter closure of muscular ventricular
septal defect using the Amplatzer devices. Heart Lung Circ. 2006; 15(1): 12–7.
PubMed Abstract
|
Publisher Full Text
83. Szkutnik M, Kusa J, Białkowski J: Percutaneous closure of post-traumatic and
congenital muscular ventricular septal defects with the Amplatzer Muscular
VSD Occluder. Kardiol Pol. 2008; 66(7): 715–20; discussion 721.
PubMed Abstract
84. Spence MS, Thomson JD, Weber N, et al.: Transient renal failure due to hemolysis
following transcatheter closure of a muscular VSD using an Amplatzer
muscular VSD occluder. Catheter Cardiovasc Interv. 2006; 67(5): 663–7.
PubMed Abstract
|
Publisher Full Text
85. Bacha EA, Cao QL, Starr JP, et al.: Perventricular device closure of muscular
ventricular septal defects on the beating heart: technique and results. J Thorac
Cardiovasc Surg. 2003; 126(6): 1718–23.
PubMed Abstract
|
Publisher Full Text
86. Amin Z, Danford DA, Lof J, et al.: Intraoperative device closure of
perimembranous ventricular septal defects without cardiopulmonary bypass:
preliminary results with the perventricular technique. J Thorac Cardiovasc Surg.
2004; 127(1): 234–41.
PubMed Abstract
|
Publisher Full Text
87. Amin Z, Cao QL, Hijazi ZM: Closure of muscular ventricular septal defects:
Transcatheter and hybrid techniques. Catheter Cardiovasc Interv. 2008; 72(1):
102–11.
PubMed Abstract
|
Publisher Full Text
88. Zeng XJ, Sun SQ, Chen XF, et al.: Device closure of perimembranous
ventricular septal defects with a minimally invasive technique in 12 patients.
Ann Thorac Surg. 2008; 85(1): 192–4.
PubMed Abstract
|
Publisher Full Text
89. Quansheng X, Silin P, Zhongyun Z, et al.: Minimally invasive perventricular
device closure of an isolated perimembranous ventricular septal defect with a
newly designed delivery system: preliminary experience. J Thorac Cardiovasc
Surg. 2009; 137(3): 556–9.
PubMed Abstract
|
Publisher Full Text
90. Xing Q, Pan S, An Q, et al.: Minimally invasive perventricular device closure of
perimembranous ventricular septal defect without cardiopulmonary bypass:
multicenter experience and mid-term follow-up. J Thorac Cardiovasc Surg. 2010;
139(6): 1409–15.
PubMed Abstract
|
Publisher Full Text
91. Michel-Behnke I, Ewert P, Koch A, et al.: Device closure of ventricular septal
defects by hybrid procedures: a multicenter retrospective study. Catheter
Cardiovasc Interv. 2011; 77(2): 242–51.
PubMed Abstract
|
Publisher Full Text
92. Xing Q, Wu Q, Pan S, et al.: Transthoracic device closure of ventricular septal
defects without cardiopulmonary bypass: experience in infants weighting less
than 8 kg. Eur J Cardiothorac Surg. 2011; 40(3): 591–7.
PubMed Abstract
|
Publisher Full Text
93. Pan S, Xing Q, Cao Q, et al.: Perventricular device closure of doubly committed
subarterial ventral septal defect through left anterior minithoracotomy on
beating hearts. Ann Thorac Surg. 2012; 94(6): 2070–5.
PubMed Abstract
|
Publisher Full Text
94. Zhu D, Gan C, Li X, et al.: Perventricular device closure of perimembranous
ventricular septal defect in pediatric patients: technical and morphological
considerations. Thorac Cardiovasc Surg. 2013; 61(4): 300–6.
PubMed Abstract
|
Publisher Full Text
95. Lin K, Zhu D, Tao K, et al.: Hybrid perventricular device closure of doubly
committed subarterial ventricular septal defects: mid-term results. Catheter
Cardiovasc Interv. 2013; 82(3): E225–32.
PubMed Abstract
|
Publisher Full Text
96. Wang S, Zhuang Z, Zhang H, et al.: Perventricular closure of perimembranous
ventricular septal defects using the concentric occluder device. Pediatr Cardiol.
2014; 35(4): 580–6.
PubMed Abstract
|
Publisher Full Text
97. Yin S, Zhu D, Lin K, et al.: Perventricular device closure of congenital
ventricular septal defects. J Card Surg. 2014; 29(3): 390–400.
PubMed Abstract
|
Publisher Full Text
98. Zhu D, Lin K, Tang ML, et al.: Midterm results of hybrid perventricular closure of
Page 13 of 16
F1000Research 2018, 7(F1000 Faculty Rev):498 Last updated: 26 APR 2018
doubly committed subarterial ventricular septal defects in pediatric patients.
J Card Surg. 2014; 29(4): 546–53.
PubMed Abstract
|
Publisher Full Text
99. Zhang S, Zhu D, An Q, et al.: Minimally invasive perventricular device closure of
doubly committed sub-arterial ventricular septal defects: single center long-
term follow-up results. J Cardiothorac Surg. 2015; 10: 119.
PubMed Abstract
|
Publisher Full Text
|
Free Full Text
100. Hongxin L, Wenbin G, Liang F, et al.: Perventricular device closure of a doubly
committed juxtaarterial ventricular septal defect through a left parasternal
approach: midterm follow-up results. J Cardiothorac Surg. 2015; 10: 175.
PubMed Abstract
|
Publisher Full Text
|
Free Full Text
101. Gan C, Peng L, Liang Z, et al.: Percutaneous Perventricular Device Closure
of Ventricular Septal Defect: From Incision to Pinhole. Ann Thorac Surg. 2017;
103(1): 172–7.
PubMed Abstract
|
Publisher Full Text
|
F1000 Recommendation
102. Zheng Q, Zhao Z, Zuo J, et al.: A comparative study: Early results and
complications of percutaneous and surgical closure of ventricular septal
defect. Cardiology. 2009; 114(4): 238–43.
PubMed Abstract
|
Publisher Full Text
103. Saurav A, Kaushik M, Mahesh Alla V, et al.: Comparison of percutaneous
device closure versus surgical closure of peri-membranous ventricular septal
defects: A systematic review and meta-analysis. Catheter Cardiovasc Interv.
2015; 86(6): 1048–56.
PubMed Abstract
|
Publisher Full Text
104. Titus JL, Rastelli GC: Anatomic features of persistent common atrioventricular
canal. In: Feldt RH, ed. Artioventricular Canal Defects. Philadelphia, PA: W.B.
Saunders; 1976; 13–35.
105. Rastelli G, Kirklin JW, Titus JL: Anatomic observations on complete form
of persistent common atrioventricular canal with special reference to
atrioventricular valves. Mayo Clin Proc. 1966; 41(5): 296–308.
PubMed Abstract
106. MULLER WH Jr, DANIMANN JF Jr: The treatment of certain congenital
malformations of the heart by the creation of pulmonic stenosis to reduce
pulmonary hypertension and excessive pulmonary blood flow; a preliminary
report. Surg Gynecol Obstet. 1952; 95(2): 213–9.
PubMed Abstract
107. Epstein ML, Moller JH, Amplatz K, et al.: Pulmonary artery banding in infants
with complete atrioventricular canal. J Thorac Cardiovasc Surg. 1979; 78(1):
28–31.
PubMed Abstract
108. Silverman N, Levitsky S, Fisher E, et al.: Efficacy of pulmonary artery banding
in infants with complete atrioventricular canal. Circulation. 1983; 68(3 Pt 2):
II148–53.
PubMed Abstract
109. Williams WH, Guyton RA, Michalik RE, et al.: Individualized surgical
management of complete atrioventricular canal. J Thorac Cardiovasc Surg.
1983; 86(6): 838–44.
PubMed Abstract
110. Newfeld EA, Sher M, Paul MH, et al.: Pulmonary vascular disease in complete
atrioventricular canal defect. Am J Cardiol. 1977; 39(5): 721–6.
PubMed Abstract
|
Publisher Full Text
111. Cooney TP, Thurlbeck WM: Pulmonary hypoplasia in Down’s syndrome.
N Engl J Med. 1982; 307(19): 1170–3.
PubMed Abstract
|
Publisher Full Text
112. Hals J, Hagemo PS, Thaulow E, et al.: Pulmonary vascular resistance in
complete atrioventricular septal defect. A comparison between children with
and without Down’s syndrome. Acta Paediatr. 1993; 82(6–7): 595–8.
PubMed Abstract
|
Publisher Full Text
113. Li D, Fan Q, Iwase T, et al.: Modified Single-Patch Technique Versus Two-
Patch Technique for the Repair of Complete Atrioventricular Septal Defect: A
Meta-Analysis. Pediatr Cardiol. 2017; 38(7): 1456–64.
PubMed Abstract
|
Publisher Full Text
|
F1000 Recommendation
114. Carpentier A: Surgical anatomy and surgical management of the mitral
component of the atrioventicular canal defects. In: Anderson RH, Shineborn EA.
(eds). Paediatric Cardiology. Edinburgh: Churchill Livengstone, 1978; 477–90.
115. Puga FJ, McGoon DC: Surgical treatment of atrioventicular canal. Mod Technics
Surg. 1980; 86: 1–14.
116. Chopra PS, Kantamneni V, Wilson AD, et al.: Surgical repair of common
atrioventricular septal defects - Long-term results using single-patch
technique. Presented at the 1st World Congress of Pediatric Cardiology and
Cardiac Surgery, Paris, France, June 21–25, 1993. Cardiol Young.1993; 3: 48.
117. Chin AJ, Keane JF, Norwood WI, et al.: Repair of complete common
atrioventricular canal in infancy. J Thorac Cardiovasc Surg. 1982; 84(3): 437–45.
PubMed Abstract
118. Studer M, Blackstone EH, Kirklin JW, et al.: Determinants of early and late results
of repair of atrioventricular septal (canal) defects. J Thorac Cardiovasc Surg.
1982; 84(4): 523–42.
PubMed Abstract
119. Ross DA, Nanton M, Gillis DA, et al.: Atrioventricular canal defects: results of
repair in the current era. J Card Surg. 1991; 6(3): 367–72.
PubMed Abstract
|
Publisher Full Text
120. Hanley FL, Fenton KN, Jonas RA, et al.: Surgical repair of complete
atrioventricular canal defects in infancy. Twenty-year trends. J Thorac
Cardiovasc Surg. 1993; 106(3): 387–94; discussion 394–7.
PubMed Abstract
121. Tweddell JS, Litwin SB, Berger S, et al.: Twenty-year experience with repair of
complete atrioventricular septal defects. Ann Thorac Surg. 1996; 62(2): 419–24.
PubMed Abstract
|
Publisher Full Text
122. Jacobs JP, Jacobs ML, Mavroudis C, et al.: Atrioventricular septal defects:
lessons learned about patterns of practice and outcomes from the congenital
heart surgery database of the society of thoracic surgeons. World J Pediatr
Congenit Heart Surg. 2010; 1(1): 68–77.
PubMed Abstract
|
Publisher Full Text
123. Hoohenkerk GJ, Bruggemans EF, Rijlaarsdam M, et al.: More than 30 years’
experience with surgical correction of atrioventricular septal defects. Ann
Thorac Surg. 2010; 90(5): 1554–61.
PubMed Abstract
|
Publisher Full Text
124. Ginde S, Lam J, Hill GD, et al.: Long-term outcomes after surgical repair of
complete atrioventricular septal defect. J Thorac Cardiovasc Surg. 2015; 150(2):
369–74.
PubMed Abstract
|
Publisher Full Text
125. Vida VL, Tessari C, Castaldi B, et al.: Early Correction of Common
Atrioventricular Septal Defects: A Single-Center 20-Year Experience. Ann
Thorac Surg. 2016; 102(6): 2044–51.
PubMed Abstract
|
Publisher Full Text
|
F1000 Recommendation
126. Desai AR, Branco RG, Comitis GA, et al.: Early postoperative outcomes
following surgical repair of complete atrioventricular septal defects: is down
syndrome a risk factor? Pediatr Crit Care Med. 2014; 15(1): 35–41.
PubMed Abstract
|
Publisher Full Text
127. Fontan F, Baudet E: Surgical repair of tricuspid atresia. Thorax. 1971; 26(3):
240–8.
PubMed Abstract
|
Publisher Full Text
|
Free Full Text
128. Kreutzer G, Bono H, Galindez E: Una operacion para la correccion de la atresia
tricuspidea. Ninth Argent Congress of Cardiology; Buenos Aires, Argentina.
October 31–November 6, 1971.
129. Rao PS: Tricuspid atresia. In: Pediatric Cardiovascular Medicine. 2nd Edition, Moller
JH, Hoffman JIE (eds.), Wiley-Blackwell/A John Wiley & Sons Ltd., Oxford, UK,
2012; 487–508, Chapter 35.
Publisher Full Text
130. Rao PS: Pediatric Tricuspid Atresia. Medscape Drugs & Diseases. Updated
January 5, 2016.
Reference Source
131. Rao PS: Fontan Operation: Indications, Short and Long Term Outcomes. Indian
J Pediatr. 2015; 82(12): 1147–56.
PubMed Abstract
|
Publisher Full Text
132. Azzolina G, Eufrate S, Pensa P: Tricuspid atresia: experience in surgical
management with a modified cavopulmonary anastomosis. Thorax. 1972;
27(1): 111–5.
PubMed Abstract
|
Publisher Full Text
|
Free Full Text
133. Hopkins RA, Armstrong BE, Serwer GA, et al.: Physiological rationale for a
bidirectional cavopulmonary shunt. A versatile complement to the Fontan
principle. J Thorac Cardiovasc Surg. 1985; 90(3): 391–8.
PubMed Abstract
134. Pridjian AK, Mendelsohn AM, Lupinetti FM, et al.: Usefulness of the bidirectional
Glenn procedure as staged reconstruction for the functional single ventricle.
Am J Cardiol. 1993; 71(11): 959–62.
PubMed Abstract
|
Publisher Full Text
135. de Leval MR, Kilner P, Gewillig M, et al.: Total cavopulmonary connection:
a logical alternative to atriopulmonary connection for complex Fontan
operations. Experimental studies and early clinical experience. J Thorac
Cardiovasc Surg. 1988; 96(5): 682–95.
PubMed Abstract
136. Marcelletti C, Corno A, Giannico S, et al.: Inferior vena cava-pulmonary artery
extracardiac conduit. A new form of right heart bypass. J Thorac Cardiovasc
Surg. 1990; 100(2): 228–32.
PubMed Abstract
137. Kumar SP, Rubinstein CS, Simsic JM, et al.: Lateral tunnel versus extracardiac
conduit Fontan procedure: a concurrent comparison. Ann Thorac Surg. 2003;
76(5): 1389–96; discussion 1396–7.
PubMed Abstract
|
Publisher Full Text
138. Billingsley AM, Laks H, Boyce SW, et al.: Definitive repair in patients with
pulmonary atresia and intact ventricular septum. J Thorac Cardiovasc Surg.
1989; 97(5): 746–54.
PubMed Abstract
139. Bridges ND, Lock JE, Castaneda AR: Baffle fenestration with subsequent
transcatheter closure. Modification of the Fontan operation for patients at
increased risk. Circulation. 1990; 82(5): 1681–9.
PubMed Abstract
|
Publisher Full Text
140. Laks H, Pearl JM, Haas GS, et al.: Partial Fontan: advantages of an adjustable
interatrial communication. Ann Thorac Surg. 1991; 52(5): 1084–94; discussion
1094–5.
PubMed Abstract
|
Publisher Full Text
Page 14 of 16
F1000Research 2018, 7(F1000 Faculty Rev):498 Last updated: 26 APR 2018
141. Gentles TL, Mayer JE Jr, Gauvreau K, et al.: Fontan operation in five hundred
consecutive patients: factors influencing early and late outcome. J Thorac
Cardiovasc Surg. 1997; 114(3): 376–91.
PubMed Abstract
|
Publisher Full Text
142. Stamm C, Friehs I, Mayer JE Jr, et al.: Long-term results of the lateral tunnel
Fontan operation. J Thorac Cardiovasc Surg. 2001; 121(1): 28–41.
PubMed Abstract
|
Publisher Full Text
143. Giannico S, Hammad F, Amodeo A, et al.: Clinical outcome of 193 extracardiac
Fontan patients: the first 15 years. J Am Coll Cardiol. 2006; 47(10): 2065–73.
PubMed Abstract
|
Publisher Full Text
144. Nathan M, Emani S, IJsselhof R, et al.: Mid-term outcomes in unbalanced
complete atrioventricular septal defect: role of biventricular conversion from
single-ventricle palliation. Eur J Cardiothorac Surg. 2017; 52(3): 565–72.
PubMed Abstract
|
Publisher Full Text
|
F1000 Recommendation
145. Buratto E, Ye XT, King G, et al.: Long-term outcomes of single-ventricle
palliation for unbalanced atrioventricular septal defects: Fontan survivors do
better than previously thought. J Thorac Cardiovasc Surg. 2017; 153(2): 430–8.
PubMed Abstract
|
Publisher Full Text
|
F1000 Recommendation
146. Owens GE, Gomez-Fifer C, Gelehrter S, et al.: Outcomes for patients with
unbalanced atrioventricular septal defects. Pediatr Cardiol. 2009; 30(4): 431–5.
PubMed Abstract
|
Publisher Full Text
147. Foker JE, Berry JM, Harvey BA, et al.: Mitral and tricuspid valve repair and
growth in unbalanced atrial ventricular canal defects. J Thorac Cardiovasc Surg.
2012; 143(4 Suppl): S29–32.
PubMed Abstract
|
Publisher Full Text
148. Cohen MS, Spray TL: Surgical management of unbalanced atrioventricular
canal defect. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2005;
8(1):135–44.
PubMed Abstract
|
Publisher Full Text
149. De Oliveira NC, Sittiwangkul R, McCrindle BW, et al.: Biventricular repair in
children with atrioventricular septal defects and a small right ventricle:
anatomic and surgical considerations. J Thorac Cardiovasc Surg. 2005; 130(2):
250–7.
PubMed Abstract
|
Publisher Full Text
150. Delmo Walter EM, Ewert P, Hetzer R, et al.: Biventricular repair in children
with complete atrioventricular septal defect and a small left ventricle. Eur J
Cardiothorac Surg. 2008; 33(1): 40–7.
PubMed Abstract
|
Publisher Full Text
151. van Son JA, Phoon CK, Silverman NH, et al.: Predicting feasibility of
biventricular repair of right-dominant unbalanced atrioventricular canal. Ann
Thorac Surg. 1997; 63(6): 1657–63.
PubMed Abstract
|
Publisher Full Text
152. Cohen MS, Jacobs ML, Weinberg PM, et al.: Morphometric analysis of
unbalanced common atrioventricular canal using two-dimensional
echocardiography. J Am Coll Cardiol. 1996; 28(4): 1017–23.
PubMed Abstract
|
Publisher Full Text
153. Jegatheeswaran A, Pizarro C, Caldarone CA, et al.: Echocardiographic definition
and surgical decision-making in unbalanced atrioventricular septal defect: a
Congenital Heart Surgeons’ Society multiinstitutional study. Circulation. 2010;
122(11 Suppl): S209–15.
PubMed Abstract
|
Publisher Full Text
154. Szwast AL, Marino BS, Rychik J, et al.: Usefulness of left ventricular inflow
index to predict successful biventricular repair in right-dominant unbalanced
atrioventricular canal. Am J Cardiol. 2011; 107(1): 103–9.
PubMed Abstract
|
Publisher Full Text
155. Overman DM, Dummer KB, Moga FX, et al.: Unbalanced atrioventricular septal
defect: defining the limits of biventricular repair. Semin Thorac Cardiovasc Surg
Pediatr Card Surg Annu. 2013; 16(1): 32–6.
PubMed Abstract
|
Publisher Full Text
156. Cohen MS, Jegatheeswaran A, Baffa JM, et al.: Echocardiographic features
defining right dominant unbalanced atrioventricular septal defect: a multi-
institutional Congenital Heart Surgeons’ Society study. Circ Cardiovasc
Imaging. 2013; 6(4): 508–13.
PubMed Abstract
|
Publisher Full Text
157. Arunamata A, Balasubramanian S, Mainwaring R, et al.: Right-Dominant
Unbalanced Atrioventricular Septal Defect: Echocardiography in Surgical
Decision Making. J Am Soc Echocardiogr. 2017; 30(3): 216–26.
PubMed Abstract
|
Publisher Full Text
|
F1000 Recommendation
158. Lugones I, Biancolini MF, Biancolini JC, et al.: Feasibility of Biventricular
Repair in Right Dominant Unbalanced Atrioventricular Septal Defect: A New
Echocardiographic Metric to Refine Surgical Decision-Making. World J Pediatr
Congenit Heart Surg. 2017; 8(4): 460–7.
PubMed Abstract
|
Publisher Full Text
|
F1000 Recommendation
159. Emani SM, McElhinney DB, Tworetzky W, et al.: Staged left ventricular
recruitment after single-ventricle palliation in patients with borderline left
heart hypoplasia. J Am Coll Cardiol. 2012; 60(19): 1966–74.
PubMed Abstract
|
Publisher Full Text
160. Overman DM: Decision-Making in Unbalanced Atrioventricular Septal Defect:
Examining Another Piece of the Puzzle. World J Pediatr Congenit Heart Surg.
2017; 8(4): 468–9.
PubMed Abstract
|
Publisher Full Text
161. Nathan M, Liu H, Pigula FA, et al.: Biventricular conversion after single-ventricle
palliation in unbalanced atrioventricular canal defects. Ann Thorac Surg. 2013;
95(6): 2086–95; discussion 2095–6.
PubMed Abstract
|
Publisher Full Text
162. Kalish BT, Banka P, Lafranchi T, et al.: Biventricular conversion after single
ventricle palliation in patients with small left heart structures: short-term
outcomes. Ann Thorac Surg. 2013; 96(4): 1406–12.
PubMed Abstract
|
Publisher Full Text
163. Buratto E, Khoo B, Ye XT, et al.: Does biventricular conversion bring survival
benefits to patients with an unbalanced atrioventricular septal defect? Eur J
Cardiothorac Surg. 2018.
PubMed Abstract
|
Publisher Full Text
164. Gupta-Malhotra M, Larson VE, Rosengart RM, et al.: Mortality after total
cavopulmonary connection in children with the down syndrome. Am J Cardiol.
2010; 105(6): 865–8.
PubMed Abstract
|
Publisher Full Text
Page 15 of 16
F1000Research 2018, 7(F1000 Faculty Rev):498 Last updated: 26 APR 2018
Open Peer Review
Current Referee Status:
Editorial Note on the Review Process
arecommissionedfrommembersoftheprestigious andareeditedasaF1000FacultyReviews F1000Faculty
servicetoreaders.Inordertomakethesereviewsascomprehensiveandaccessibleaspossible,thereferees
provideinputbeforepublicationandonlythefinal,revisedversionispublished.Therefereeswhoapprovedthe
finalversionarelistedwiththeirnamesandaffiliationsbutwithouttheirreportsonearlierversions(anycomments
willalreadyhavebeenaddressedinthepublishedversion).
The referees who approved this article are:
Version 1
ThebenefitsofpublishingwithF1000Research:
Yourarticleispublishedwithindays,withnoeditorialbias
Youcanpublishtraditionalarticles,null/negativeresults,casereports,datanotesandmore
Thepeerreviewprocessistransparentandcollaborative
YourarticleisindexedinPubMedafterpassingpeerreview
Dedicatedcustomersupportateverystage
Forpre-submissionenquiries,contact research@f1000.com
DepartmentofCardiacSurgery,Children'sHospitalBostonandHarvardMedicalSchool,Meena Nathan
Boston,USA
Nocompetinginterestsweredisclosed.Competing Interests:
1
JohnHopkinsUniversity,Baltimore,USAMonesha Gupta
Nocompetinginterestsweredisclosed.Competing Interests:
1
DepartmentofCongenitalHeartDiseasesandPediatricCardiology,SilesianCenterforJacek Bialkowski
HeartDiseases,MedicalUniversityofSilesia,Zabrze,Poland
Nocompetinginterestsweredisclosed.Competing Interests:
1
Page 16 of 16
F1000Research 2018, 7(F1000 Faculty Rev):498 Last updated: 26 APR 2018
... Initially, the pathologic, pathophysiologic, clinical, echocardiogrphic, and angiographic features of ventricular septal defects (VSDs) were examined. This is followed by description of historical aspects of development of percutaneous closure of VSDs [2,6,7,70,71] and indications (moderate to large VSDs with enlarged left atrium and left ventricle and/or elevated pulmonary artery pressure and a Qp:Qs greater than 2:1) for VSD closure [8,[71][72][73]. VSD occlusion with buttoned and Amplatzer devices were separately reviewed. ...
... Initially, the pathologic, pathophysiologic, clinical, echocardiogrphic, and angiographic features of ventricular septal defects (VSDs) were examined. This is followed by description of historical aspects of development of percutaneous closure of VSDs [2,6,7,70,71] and indications (moderate to large VSDs with enlarged left atrium and left ventricle and/or elevated pulmonary artery pressure and a Qp:Qs greater than 2:1) for VSD closure [8,[71][72][73]. VSD occlusion with buttoned and Amplatzer devices were separately reviewed. ...
... Change of the murmur of PDA with postural variation in three children was demonstrated; the disappearance of PDA murmur in upright position was ascribed to kinking of the ductus. The utility of auscultation in [84] was reviewed elsewhere [1,71,84], and were generally considered acceptable. Apart from the usual complications seen with complex procedures, complete heart block (as detailed in our review [85]) developed in a significant percentage of patients both immediately after and during follow-up after implantation of Amplatzer Membranous VSD occluders. ...
... Transcatheter therapy offers unbeatable advantages when compared to cardiac surgery [1][2][3]. Several devices have been designed and tested over the years to percutaneously close PmVSDs [4][5][6][7]. However, the Amplatzer asymmetrical device has been associated with a high risk of CAVB, prompting many to abandon this intervention and pushing other interventionists to use devices designed for other applications in an off-label basis complication was more frequently seen in children, occurred acutely (transiently, during the procedure, or permanently) or quite unpredictably months to years after the procedure but more permanently. ...
... However, the Amplatzer asymmetrical device has been associated with a high risk of CAVB, prompting many to abandon this intervention and pushing other interventionists to use devices designed for other applications in an off-label basis complication was more frequently seen in children, occurred acutely (transiently, during the procedure, or permanently) or quite unpredictably months to years after the procedure but more permanently. The exact mechanism of CAVB remains unclear [4][5][6][7]. It has been postulated that the device stretches the defect and possibly exerts pressure on the nearby conduction system with possible impingement against its vascular supply system. ...
... Moreover, the alteration in systemic hemodynamics related to non-corrected cardiac defects can influence both the systemic and pulmonary vascular performance and theoretically promote the development of endothelial dysfunction and morphological vascular wall [8][9][10][11][12]. The debate remains ongoing as the challenges are present, and the pitfalls are numerous [1][2][3][4][5][6]. Yet, advancements in device technology are major and we are very close to the re-birth of the ideal device dedicated for PmVSD closure [6,13,14]. ...
Article
Full-text available
Percutaneous closure is the standard therapy for muscular Ventricular Septal Defects (VSDs) beyond infancy with a low rate of major complications. The story is somewhat different for Perimembranous Ventricular Septal Defects (PmVSDs) where surgery remains in 2021 the preferable treatment approach in some centers, due to the historical incidence of Complete Atrioventricular Block (CAVB) that has been associated with the asymmetrical Amplatzer Membranous VSD Occluder. It is certain that transcatheter closure of PmVSD is one of the most complex cardiac interventions and have stringent demands on device design due to several challenging considerations. Despite that, experienced interventionists have been continuously reporting successful experiences with PmVSD closure using a variety of device occluders in an off-label indication. Recent meta-analyses confirmed the very good outcomes of this approach and the non-inferiority compared to surgery. However, these devices represent a compromise, as they are not specifically designed to be placed in the perimembranous position. To date, no device achieved market approval in the United States. The need for a device dedicated to PmVSD transcatheter closure is mandatory to standardize the technique and we are very close to achieving this goal. The most recent KONAR-Multifunctional Occluder (MFO) has been smartly designed, combining technical features of previous devices, to tackle encountered difficulties and the outcomes of emerging clinical reports are consecutively encouraging. The MFO specifications are particular but limitations are present and need to be highlighted. This continuous advancement in device technology through continuous physician input will lead to the birth of the ideal device for this intervention.
... Transitional and partial forms are also described, and are similar to or the same as ostium primum ASDs. Complete AVSDs are also categorized based on the ventricular sizes, namely balanced and unbalanced ( Figure 5) [17]. Unbalanced AVSDs comprise 10-15% of all complete AVSDs. ...
... The unbalanced types may be LV-dominant with a large LV and small RV, or RV-dominant with a large RV and small LV ( Figure 5). RV-dominant AVSDs are more frequently seen [17]. ...
... The criteria used for selection of patients for primary biventricular repair are not clearly established. A number of echocardiographic, MRI, and cine-angiographic criteria have been examined as reviewed elsewhere [17,116]. A combination of LV Z scores and LV volumes seems to help decide on such a selection. ...
Article
Full-text available
In this paper, the author enumerates cardiac defects with a functionally single ventricle, summarizes single ventricle physiology, presents a summary of management strategies to address the single ventricle defects, goes over the steps of staged total cavo-pulmonary connection, cites the prevalence of inter-stage mortality, names the causes of inter-stage mortality, discusses strategies to address the inter-stage mortality, reviews post-Fontan issues, and introduces alternative approaches to Fontan circulation.
... This defect results in fibrous continuity between the tricuspid and the aortic valve (right and non-coronary) leaflets. The conduction system usually courses postero-inferior to the defect [37,38] . ...
Article
Full-text available
Septal defects together account for the majority of the congenital heart defects (CHD); these can occur in isolation or associated with other CHDs. Hemodynamic manifestations are dependent upon the size, location, and the number of the defects, along with the associated lesions. For example, atrial septal defects result in the right ventricular volume overload, whereas the ventricular septal defect (VSD) results in the left heart volume overload. Knowledge of septal anatomy is crucial to understanding these lesions, their hemodynamic significance, and thus better plan management, including interventions. Noninvasive imaging of simple septal defects by various modalities will be reviewed; atrioventricular septal defects, anomalous pulmonary venous connections, patent ductus arteriosus, and complex cardiac conditions with VSD will not be discussed in this chapter.
... About 30% of the congenital heart morbidity is a part of genetic syndromes like Down syndrome, Turner syndrome, and 22q11 deletion syndrome [13]. Ventricular septal defect and atrial septal defects of less than 5 mm should close by themselves within the first year of life as seen in the majority of cases [14,15]. Medications like valproic acid during the initial months of pregnancy, smoking, hyperglycemia due to gestational diabetes mellitus, and deficiency of folic acid in mothers are some established risk factors for neural tube defects in newborns [16]. ...
Article
We report a case of a one-day-old female with congenital facial nerve palsy, bilateral microtia, congenital heart disease, spina bifida, and congenital cholesteatoma. The newborn was brought by the mother with complaints of abnormally looking ear and a facial droop toward the left side, following which a two-dimensional echocardiography was done showing the atrial septal defect and ventricular septal defect. Computed tomography of the temporal bone showed the presence of congenital cholesteatoma in the left ear. MRI of the lumbosacral spine was suggestive of spina bifida occulta. Brainstem evoked response audiometry was suggestive of sensorineural hearing loss. Such a combination of symptoms is very rare, and therefore this case is being reported.
... 2,18 Palliative pulmonary artery banding is suggested in infants weighing < 5 kg to prevent progressive pulmonary congestion and congestive heart failure. 19 Percutaneous transcatheter closure of VSDs under fluoroscopic and echocardiographic guidance has become the optimal option in the treatment of VSDs in children. [20][21][22][23] However, low body weight, younger age, the use of an overlarge device, and unfavorable surrounding structures are risk factors for complications during transcatheter closure of VSDs. ...
Article
Background: Failure to thrive and poor weight gain are the main problems associated with ventricular septal defects complicated by heart failure in pediatric patients. Recent advances in transcatheter closure have enabled safe and effective interventions in these patients. Objectives: The purpose of this study was to describe our experience with transcatheter closure of ventricular septal defects in young children with low weight. Methods: Pediatric patients weighing < 15 kg who underwent transcatheter closure of ventricular septal defects between January 2018 and December 2019 at our hospital were retrospectively enrolled. Results: Twelve patients were enrolled: one with a muscular defect, two with outlet defects, and nine with perimembranous defects. Their median age was 24 (7-60) months, and their median weight before the procedure was 11.8 kg (4.7-14.9 kg; mean Z-score: -1.3). The median precordial echocardiographic defect diameter was 5.6 (2.0-9.3) mm. Successful transcatheter closure was achieved in 11 cases. The mean weight at 1-month follow-up after defect closure was 13.5 kg (6.2-19.8 kg; mean Z-score: -0.2). The mean length of hospitalization was 2.7 days. Conclusions: This study highlights the potential safety and therapeutic efficacy of transcatheter ventricular septal defect closure in infants with low weight. Considerable weight gain and heart failure symptom attenuation at 1 month after transcatheter closure were observed.
Article
Full-text available
Background Paravalvular leak (PVL) is uncommon but can lead to severe complications after surgical or transcatheter aortic valve implantation. Conditions associated with PVLs such as heart failure, hemolysis, and infective endocarditis can lead to catastrophic results if not treated promptly; the therapeutic goals differ according to the presentation. It is vital that PVLs are diagnosed early using various imaging modalities. Different approaches have been studied in managing PVLs; there is an increased interest in the transcatheter aortic valve closure procedure as it is minimally invasive and decreases the occurrence of further reinterventions. Aim To discuss the classification of PVLs, diagnostic approaches, and available management options. Method A literature review was performed using 28 studies. Results This review evaluated the relationship between the time of diagnosis, management of PVL and the resulting outcomes. Discussion Patients with PVL should be assessed through a multidisciplinary team approach and a patient‐selective plan should be in place. Conclusion Open surgical intervention is reserved for complex cases where minimally invasive techniques cannot be utilized.
Chapter
Ventricular septal defects (VSDs) account for up to 30% of all congenital cardiac anomalies and are one of the most common lesions encountered in day-to-day practice. The etiology is thought to be multifactorial inheritance but it is sometimes associated with chromosomal abnormalities such as aneuploidies and microdeletions. Most of these defects, close spontaneously and do not require treatment. Symptoms are primarily dependent upon the degree of shunt across the ventricles. Echocardiography remains the main modality of definitive diagnosis for isolated defects. Surgical repair is recommended in hemodynamically significant shunts or if there is aortic prolapse and regurgitation. Prognosis after surgical repair remains excellent especially with isolated defects but complete atrioventricular block or worsening valve regurgitation may occur in some patients. Newer techniques involving catheter based or hybrid device closures are being used in select cases such as muscular defects. Large unrepaired shunts, although uncommon in the developed world, may cause irreversible changes in pulmonary vasculature leading to Eisenmenger’s syndrome.
Article
Ventricular septal defects (VSDs) are the most common forms of acyanotic congenital heart disease accounting for 37% of congenital heart disease in children. A VSD is defined by parts of the ventricular septum involved. There are four major types of VSDs: perimembranous, muscular, outlet, and inlet VSDs. Echocardiography is the most important clinical tool to help diagnose and characterize a VSD. Although most VSDs are clinically nonsignificant or close on their own, echocardiography with Doppler and color flow mapping can be used to provide accurate anatomic and hemodynamic evaluation of VSDs in order to determine if surgical or transcatheter‐based intervention is needed. Hence, understanding how to use echocardiography to characterize VSDs is of crucial importance when caring for patients with adult congenital heart disease.
Article
Background Medical advancements have encouraged minimally invasive surgical repair of congenital heart defects such as ventricular septal defects (VSDs), and the diagnostic process can now be carried out using non-traditional techniques such as pulse oximetry. This, in turn, has improved clinical outcomes with reduced complication rates post-surgery. However, the variations in type of VSDs, age of patient, comorbidities, and access to closure devices may limit the efficacy of surgical advancements. Methods Articles were identified amongst Scopus, MEDLINE, and PubMed using various relevant search strings using PRISMA guidelines. Of the 115 articles initially extracted, 10 were eventually reviewed after duplicates and irrelevant studies were removed. Results Of the 24 eligible articles, 10 papers were selected for analysis. Minimally invasive approaches to VSD repair was associated with satisfactory short-term outcomes when compared to open repair. For diagnosis of congenital VSD, whilst recent advances such as pulse oximetry method and genome analysis are more sensitive, the limited availability and access to such investigatory methods must be recognised. Conclusion Pulse oximetry and fetal echocardiography are established non-invasive diagnostic tools for VSD. The recent advances in minimally invasive treatment options including periventricular approach and transcatheter techniques have improved patient outcomes, yet at the expense of higher residual rates. Careful patient selection for each technique and follow-up should be planned through multidisciplinary team meetings.
Article
Full-text available
The purpose of this review is to discuss the management of atrial septal defects (ASD), paying particular attention to the most recent developments. There are four types of ASDs: ostium secundum, ostium primum, sinus venosus, and coronary sinus defects. The fifth type, patent foramen ovale—which is present in 25 to 30% of normal individuals and considered a normal variant, although it may be the seat of paradoxical embolism, particularly in adults—is not addressed in this review. The indication for closure of the ASDs, by and large, is the presence of right ventricular volume overload. In asymptomatic patients, the closure is usually performed at four to five years of age. While there was some earlier controversy regarding ASD closure in adult patients, currently it is recommended that the ASD be closed at the time of presentation. Each of the four defects is briefly described followed by presentation of management, whether by surgical or percutaneous approach, as the case may be. Of the four types of ASDs, only the ostium secundum defect is amenable to percutaneous occlusion. For ostium secundum defects, transcatheter closure has been shown to be as effective as surgical closure but with the added benefits of decreased hospital stay, avoidance of a sternotomy, lower cost, and more rapid recovery. There are several FDA-approved devices in use today for percutaneous closure, including the Amplatzer® Septal Occluder (ASO), Amplatzer® Cribriform device, and Gore HELEX® device. The ASO is most commonly used for ostium secundum ASDs, the Gore HELEX® is useful for small to medium-sized defects, and the cribriform device is utilized for fenestrated ASDs. The remaining types of ASDs usually require surgical correction. All of the available treatment modes are safe and effective and prevent the development of further cardiac complications.
Article
Full-text available
Background: Unbalanced forms of atrioventricular septal defect continue to be challenging and present poor surgical outcomes. Echocardiographic indicators such as atrioventricular valve index, right ventricle/left ventricle inflow angle, and size of the ventricular septal defect have been identified as relevant discriminators that may guide surgical strategy. Our purpose is to describe another metric to refine surgical decision-making. Methods: We outline a geometrical description of the anatomic features of atrioventricular septal defect and describe equations that help explain the interplay between the main echocardiographic variables. Results: A new metric called "indexed ventricular septal defect" is defined as the size of the defect in relation to the valve diameter. We derive a final equation relating this index with the atrioventricular valve index and the right ventricle/left ventricle inflow angle. In the light of that equation, we discuss the interdependence of variables and employ data from a Congenital Heart Surgeons' Society study to set the limits of the new index. Conclusion: Combined use of indexed ventricular septal defect and atrioventricular valve index might help clarify surgical decision-making in patients with mild and moderate unbalance (modified atrioventricular valve index between 0.2 and 0.39). For indexed ventricular septal defect smaller than 0.2, biventricular repair may be recommended. Between 0.2 and 0.35, this strategy could probably be achieved depending on other factors. However, other strategies should be considered for those patients showing an indexed ventricular septal defect between 0.35 and 0.5. For values above 0.5 to 0.55, univentricular palliation might be a reasonable strategy.
Article
Full-text available
We present a case report about percutaneous closure of a congenital Gerbode defect using Nit-Occlud(®) Lê VSD coil. The patient was referred to our hospital with a diagnosis of ventricular septal defect (VSD) and severe pulmonary arterial hypertension. But transthoracic echocardiography revealed a communication between the left ventricle (LV) and the right atrial (RA), called Gerbode defect. Catheterization confirmed the shunt from the LV to the RA. We successfully closed the defect with a VSD coil. After uneventful 6 mo follow-up, the patient was out of dyspnea, the symptom urged him to have medical attention. This case report is to discuss the diagnosis and percutaneous treatment approach for this rare congenital heart disease.
Article
Full-text available
Technical selection for surgical repair of complete atrioventricular septal defect (CAVSD) still remains controversial. This meta-analysis aimed to compare the modified single-patch (MP) technique with the two-patch (TP) technique for patients with CAVSD. Relevant studies comparing the MP technique with the TP technique were identified through a literature search using MEDLINE, EMBASE, Google Scholar, Cochrane Library, and the China National Knowledge Infrastructure databases. The variables were ventricular septal defect (VSD) size, cardiopulmonary bypass (CBP) time, aortic cross-clamp (ACC) time, intensive care unit stay, hospital stay, and other outcomes involving mortality, left ventricular outflow tract obstruction, atrioventricular valve regurgitation, residual septal shunt, atrioventricular block, and reoperation. A random-effect/fixed-effect model was used to summarize the estimates of mean difference/odds ratio with 95% confidence interval. Subgroup analysis stratified by region was performed. Fifteen publications involving 1034 patients were included. This meta-analysis demonstrated that (1) VSD size in the MP group was significantly smaller; (2) CBP time, ACC time, and hospital stay in the MP group experienced improvement; (3) Other postoperative outcomes showed no significant differences between two groups; and (4) The trends in China and other countries were close. The MP and TP techniques had comparable outcomes; however, the MP technique was performed with significantly shorter CBP and ACC times in patients with smaller VSDs. Given this limitation of data, the results of comparison of the two techniques in patients with larger VSDs remain unknown. Further studies are needed.
Article
The original Fontan procedure included a classic superior vena cava-to–right pulmonary artery (Glenn) shunt Subsequent experience demonstrated that this anastomosis was not essential and was an unnecessary commitment of the larger right pulmonary circulation to the smaller blood volume of the superior vena caval return. With application of the Fontan principle to more complex cardiac malformations, there has been a reconsideration of possible benefits of a cavopulmonary shunt in selected patients. A modified shunt from the divided end of the superior vena cava to the side of the undivided right pulmonary artery utilized in 21 patients is described. This shunt is designed to allow bidirectional pulmonary arterial distribution of both superior vena caval inflow and right atrial outflow after completion of the Fontan procedure. Twelve patients had the bidirectional shunt performed prior to a Fontan operation; five of these had a subsequent atriopulmonary connection and seven await operation. Eight patients had construction of this shunt at the time of their Fontan procedure. One patient had a bidirectional shunt constructed following atriopulmonary anastomosis to help relieve right atrial outflow obstruction. Two patients with univentricular heart undergoing simultaneous Fontan procedure and a bidirectional shunt died while in the hospital. The remaining 19 patients have been followed up for 2 months to 9 years with one late sudden death at 9 years. There have been no bidirectional cavopulmonary shunt failures, stenoses, kinks, or recognized pulmonary arteriovenous malformations. Postoperatively, eight patients had assessment of pulmonary distribution of shunt blood flow by angiography. Seven of these patients were also evaluated by radionuclide angiography. Superior vena caval blood flow via the bidirectional cavopulmonary shunt tended to be greater to the right lung, but bilateral pulmonary flow was documented in all but one patient After Fontan operation, six of seven patients tested also demonstrated bilateral distribution of atriopulmonary flow. We concluded from our experience that this modified shunt (1) provides excellent relief of cyanosis, (2) allows bidirectional pulmonary distribution of both superior vena caval return and also the right atrial blood flow after atriopulmonary connection, and (3) may be done before, with, or after a Fontan procedure and is compatible with all currently recommended modifications. Perioperative hemodynamic adjustments to the Fontan procedure may be improved by reducing atrial volume, and this may also be of potential benefit in the long-term adaptation to Fontan physiology by minimizing atrial distention.
Article
Congenital heart defects are among the most common congenital malformations at birth, with an incidence of approximately 8/1000 live births. These defects are characterized by a heterogeneous group of abnormal communications and connections between the cardiac chambers and great vessels with different hemodynamic consequences and hence, varying need for follow-up and interventions. The most common forms are congenital cardiac systemic to pulmonary shunts (ie, ventricular septal defects, atrial septal defects, patent ductus arteriosus) that account for almost 60 % of congenital cardiac malformations.
Article
Aim The aim of this study was to share the mid-term results of percutaneous ventricular septal defect closure using Amplatzer Duct Occluder-II in children. Background Nowadays, percutaneous ventricular septal defect closure is accepted as an alternative to surgery, but so far no ideal device has been developed for this procedure. Methods In the study centre, between April, 2011 and October, 2016, the ventricular septal defect of 49 patients was closed percutaneously using the Amplatzer Duct Occluder-II device, and seven of them were <1 year old. Results The mean age of patients was 86.8±52.6 months. The mean weight of the patients was 24.3±16 kg. The mean diameter of the defect was 3.7±1.4 mm. Mean fluoroscopy time and total procedure time were 37±19.3 and 74.1±27 minutes, respectively. The types of ventricular septal defects were muscular in six patients, and were perimembranous in the rest of them. We did not face any major complications during the procedure, but one patient was admitted with a complete atrioventricular block in the 6th month of follow-up. The total follow-up period was 66 months. Conclusion To our knowledge, our study includes the largest series of paediatric patients whose ventricular septal defect was closed using Amplatzer Duct Occluder-II percutaneously. When the complications during the 66-month follow-up period are taken into consideration, we can state that Amplatzer Duct Occluder-II is a safe and effective device, even in infants aged <1 year.