Valve-Sparing Aortic Root Replacement in
Nishant D. Patel, MD, George J. Arnaoutakis, MD, Timothy J. George, MD,
Jeremiah G. Allen, MD, Diane E. Alejo, BA, Harry C. Dietz, MD,
Duke E. Cameron, MD, and Luca A. Vricella, MD
Johns Hopkins Division of Cardiac Surgery, Department of Surgery, The Johns Hopkins Hospital, Baltimore, Maryland
Background. Loeys-Dietz syndrome (LDS) is a recently
recognized aggressive aortic disorder characterized by
root aneurysm, arterial tortuosity, hypertelorism, and
bifid uvula or cleft palate. The results of prophylactic
root replacement using valve-sparing procedures (valve-
sparing root replacement [VSRR]) in patients with LDS
is not known.
Methods. We reviewed all patients with clinical and
genetic (transforming growth factor-? receptor muta-
tions) evidence of LDS who underwent VSRR at our
institution. Echocardiographic and clinical data were
obtained from hospital and follow-up clinic records.
Results. From 2002 to 2009, 31 patients with a firm
diagnosis of LDS underwent VSRR for aortic root aneu-
rysm. Mean age was 15 years, and 24 (77%) were children.
One (3%) patient had a bicuspid aortic valve. Preopera-
tive sinus diameter was 3.9 ? 0.8 cm (z score 7.0 ? 2.9)
and 2 (6%) had greater than 2? aortic insufficiency.
Thirty patients (97%) underwent reimplantation proce-
dures using a Valsalva graft. There were no operative
deaths. Mean follow-up was 3.6 years (range, 0 to 7
years). One patient required late repair of a pseudoaneu-
rysm at the distal aortic anastomosis, and 1 had a conver-
sion to a David reimplantation procedure after a Florida
sleeve operation. No patient suffered thromboembolism
or endocarditis, and 1 (3%) patient experienced greater
than 2? late aortic insufficiency. No patient required late
aortic valve repair or replacement.
Conclusions. Loeys-Dietz syndrome is an aggressive
aortic aneurysm syndrome that can be addressed by
prophylactic aortic root replacement with low operative
risk. Valve-sparing procedures have encouraging early
and midterm results, similar to those in Marfan syn-
drome, and are an attractive option for young patients.
(Ann Thorac Surg 2011;92:556–61)
© 2011 by The Society of Thoracic Surgeons
and conotruncal congenital heart disease and may com-
plicate some cardiovascular surgical operations such as
the Ross procedure, Norwood procedure, and arterial
switch. Aortic root replacement may be indicated in some
children to prevent aneurysm rupture, aortic dissection,
and valvar incompetence.
Traditionally the Bentall procedure  has been the
operation of choice for aortic root disease and has dem-
onstrated excellent long-term outcomes [2–4]. However
an aortic valve with a mechanical prosthesis necessitates
long-term anticoagulation and bioprosthetic valves have
limited durability in young patients. These consider-
ations make valve-sparing aortic root replacement
(VSRR), originally described by Sarsan and Yacoub 
and David and Feindel , an attractive option for chil-
dren with aortic root aneurysms.
Recently Loeys and Dietz described an autosomal-
dominant inherited syndrome resulting from mutations
ortic root aneurysms are rare in children; they are
usually associated with connective tissue disorders
in the receptors for transforming growth factor (TGF)-?
. Loeys-Dietz syndrome (LDS) is characterized by
aggressive vascular disease, arterial tortuosity and aneu-
rysm, hypertelorism, and bifid uvula or cleft palate.
Patients with LDS experience aortic dissection and rup-
ture at smaller aortic diameters and at a younger age than
do patients with Marfan syndrome. We have embarked
on a program of early prophylactic aortic root replace-
ment to prevent vascular catastrophe in LDS. This review
was conducted to evaluate the safety and early results of
this surgical program.
Material and Methods
Study Design, Patient Selection, and Patient Variables
After institutional review board approval, data were
collected retrospectively for all patients with LDS who
underwent VSRR between 2002 and 2009 at the Johns
Hopkins Hospital. Data were obtained from medical and
electronic patient records. A waiver for individual con-
sent was granted by our institutional review board.
All patients who underwent VSRR had transthoracic
echocardiograms to assess preoperative and postopera-
tive aortic valve function. Intraoperative transesophageal
echocardiograms were routinely obtained. Echocardio-
graphic data included maximum aortic sinus diameter,
Accepted for publication April 1, 2011.
Presented at the Fifty-seventh Annual Meeting of the Southern Thoracic
Surgical Association, Orlando, FL, Nov 3–6, 2010.
Address correspondence to Dr Vricella, Johns Hopkins Medical Institutions,
600 N Wolfe St, Blalock 618, Baltimore, MD 21287; e-mail: lvricella@jhmi.
© 2011 by The Society of Thoracic Surgeons
Published by Elsevier Inc
sinotubular junction diameter, and annular diameter
with respective z scores (number of SDs away from mean
for aortic dimensions based on patient body surface
area), as well as the degree of aortic insufficiency (0 to
4?). Clinical follow-up data, including postoperative aor-
tic insufficiency and need for reoperation on the aortic
root, were obtained by telephone interviews with the
patient, family members, and primary care physicians.
Some patients with LDS and aortic root aneurysm/
dissection were not offered VSRR and instead had root
replacement with composite grafts. Our relative contra-
indications for VSRR include marked leaflet fenestration
and asymmetry, acute aortic dissection in unstable pa-
tients, and a giant root with marked leaflet irregularities.
For these patients, a Bentall procedure was performed. In
addition, we do not routinely perform VSRR on bicuspid
aortic valves with extensive calcification, severe prolapse,
marked fenestrations, or a combination of these
The diagnosis of LDS was confirmed both by genetic
analysis and clinical phenotype in collaboration with
colleagues in the McKusick Department of Medical Ge-
netics and the Connective Tissue Disorders Clinic at the
Johns Hopkins Hospital.
Patients underwent a modified David V procedure in
which the native aortic valve was resuspended within a
Dacron tube graft with prefashioned pseudosinuses
(Gelweave Valsalva graft; Vaskutek, Renfrewshire, Scot-
land, UK). One patient had a reimplantation procedure
using a straight Dacron graft because of unavailability of
an appropriate small-sized Valsalva graft. Our operative
technique has been described in detail elsewhere . All
operations are performed through a median sternotomy.
Patients are placed on cardiopulmonary bypass using
aortic and bicaval venous cannulation. A vent is placed in
the left atrium through the right superior pulmonary
vein. After aortic clamping and cardioplegic arrest, the
aorta is divided above the sinotubular ridge. Using com-
mercial valve sizers, we determine the diameter of the
sinotubular junction that provides optimal leaflet appo-
sition and then select a Valsalva graft that is 2 to 3 mm
larger because the prosthesis fits outside the aortic valve
complex. The base of the aorta is dissected externally
down to a subannular level. This is followed by excision
of sinus tissue, leaving an approximately 4-mm to 5-mm
sinus remnant attached to the annulus. The coronary
arteries are mobilized widely. Three 3-0 braided polyes-
ter pledgetted horizontal mattress sutures are passed
under the annulus at the nadir of each aortic leaflet from
inside outward. Our procedure differs from the original
David I reimplantation technique in that only 3 subannu-
lar sutures are placed. The subannular sutures anchor
the graft and ensure that the entire aortic valve complex
is within the sinus segment of the Valsalva graft. The
proximal collar of the graft is trimmed to 2 rings. The
distal end of the graft is usually trimmed to 4 to 5 rings
but may be left longer if more extensive replacement of
the ascending aorta or arch is necessary. The 3 subannu-
lar sutures are passed inside-out through the bottom of
the Valsalva graft 120 degrees apart from each other. The
stay sutures are drawn up within the graft, which is
lowered around the valve complex, and the 3 subannular
sutures are tied.
The commissures are fixed to the sinotubular junction
of the graft using 3 4-0 polypropylene pledgetted mat-
tress sutures. In most patients the commissure height is
level with the sinotubular ridge of the graft. In patients
with very large roots and tall commissures, fixation can
be just above the ridge. The valve is now properly
positioned within the graft by the “3 below” and the “3
above” fixation sutures. Starting at the bottom of each
sinus and sewing upward to the commissure tops, con-
tinuous 4-0 polypropylene sutures are used to fix the
sinus remnant and annulus within the graft. This is the
hemostatic suture line.
Holes are cut in the graft opposite the coronary artery
buttons. We encircle the coronary buttons with polytet-
rafluoroethylene (Teflon) felt “lifesaver” pledgets. The
left and right coronary anastomoses are completed with
4-0 polypropylene. Finally the distal graft is anastomosed
to the aorta using continuous 4-0 polypropylene suture
and an external felt strip. Air is evacuated from the heart,
the aortic cross-clamp is removed, and the heart is
Statistical analyses were conducted with Stata version 9.0
software package (Stata Corporation, College Station,
TX). Survival and freedom from reoperation of the aortic
root were estimated using the Kaplan-Meier method. All
continuous variables are presented as mean ? SD, unless
Preoperative Clinical Characteristics
A total of 31 patients with a firm diagnosis of LDS
underwent VSRR between 2002 and 2009. Preoperative
patient characteristics are shown in Table 1. Mean age at
operation was 14.6 ? 12.8 years (median, 12.7 years;
range, 9 months to 60 years), and 14 (45.2%) were female
Table 1. Baseline Clinical Characteristics
VariableN ? 31 (%)
Mean age at operation (years)
Pediatric (?18 years)
Previous cardiac surgery
Bicuspid aortic valve
Preoperative maximum sinus
Preoperative sinotubular junction
Preoperative annular diameter (cm)
Preoperative ? 2? aortic
14.6 ? 12.8
3.9 ? 0.8 (z ? 7.0 ? 2.9)
2.8 ? 0.8 (z ? 4.6 ? 2.5)
2.2 ? 0.5 (z ? 3.8 ? 2.3)
Ann Thorac Surg
PATEL ET AL
VSRR IN LOEYS-DIETZ SYNDROME
patients. Twenty-four (77.4%) were younger than 18
years of age at the time of operation. One (3.2%) had a
bicuspid aortic valve. Five (16.1%) patients had had
previous cardiac surgery. All were New York Heart
Association class I preoperatively.
Preoperative sinus diameter was 3.9 ? 0.8 cm (z score
7.0 ? 2.9), sinotubular junction diameter was 2.8 ? 0.8
cm (z score 4.6 ? 2.5), and annular diameter was 2.2 ?
0.5 cm (z score 3.8 ? 2.3). Two (6.5%) patients had
greater than 2? aortic insufficiency on preoperative
Operative data are displayed in Table 2. One patient
(3.2%) underwent David I reimplantation with a straight
tube Dacron graft, and 30 (96.8%) patients had reimplan-
tation procedures using a Valsalva graft. Mean cardio-
pulmonary bypass and cross-clamp times were 144.4 ?
21.7 and 101.3 ? 17.5 minutes, respectively. The most
common prosthesis size was 24 mm and was used in 13
Fifteen (48.4%) patients had patent foramen ovale
closure at the time of VSRR. Three (9.7%) had concomi-
tant patent ductus arteriosus ligation, 3 (9.7%) had atrial
septal defect closure, and 1 (3.2%) had a ventricular
septal defect closure. Two (6.5%) patients had concomi-
tant aortic valve repair at the time of VSRR.
Survival and Functional Class
There were no operative or late deaths. Kaplan-Meier
survival was 100% at 7 years. All patients were New York
Hospital Association class I at follow-up.
Four (12.9%) patients experienced pneumothorax post-
operatively. Postoperative infection rates were low. Two
(6.5%) patients had postoperative pneumonia. There
were no postoperative urinary tract infections or sternal
Mean follow-up was 3.7 years (range, 0 to 7 years). One
8-year-old female patient who underwent reimplantation
with a 24-mm Valsalva graft required repair of a distal
pseudoaneurysm 29 months postoperatively. Preopera-
tive and postoperative maximum sinus and annular di-
ameters with respective z scores are shown Table 3. A
1-year-old patient who initially underwent a Florida
sleeve procedure experienced a coronary artery button
aneurysm and then had a VSRR with the Valsalva graft 4
years later. One patient experienced greater than 2?
aortic insufficiency 4.3 years after reimplantation with the
Valsalva graft. Freedom from late aortic valve repair or
replacement was 100% at 1, 3, and 5 years after
No patient experienced thromboembolism or endocar-
ditis at follow-up and no patient had coronary artery
Aortic root replacement with a mechanical valve has
been the gold standard of care for patients with aortic
root aneurysms for more than 40 years. It has been a safe,
reproducible, and durable operation, but nonetheless
results in a low, constant risk of thromboembolism,
endocarditis, and anticoagulant-related hemorrhage [2–
4]. Implications for lifestyle modification are not insignif-
icant for many patients. For these reasons, we and others
have adopted and selectively applied alternative valve-
preservation surgical strategies for aortic root replace-
ment over the last 15 years.
Low operative mortality and good midterm results
have been reported after VSRR in patients with aortic
aneurysm [9–18]. In our initial experience, we used the
remodeling technique with scalloped “Dacron tongues”
because of the theoretic advantage of preserved sinuses
[19–23]. However we observed a trend toward late annu-
lar dilatation and aortic insufficiency likely caused by
splaying of the Dacron tongues and loss of leaflet appo-
sition height. This has been observed particularly in
patients with connective tissue disorders. Our protocol
changed to the reimplantation procedure in 2002 when
the Gelweave Valsalva graft, a Dacron graft with prefash-
ioned pseudosinuses, became commercially available.
We believe that the reimplantation technique using the
Valsalva graft preserves sinuses, stabilizes the aortic root
below the nadir of the sinuses, and improves hemostasis.
Results of VSRR in Marfan syndrome have been re-
ported, but such results are lacking for LDS . LDS
results from mutations in the gene coding for receptors
for TGF-?, which lead to abnormal TGF-? signaling and
abnormal collagen deposition in the aortic wall. The
latter, along with elastin disarray, results in a structurally
weak medium that predisposes to aortic aneurysm, dis-
section, and rupture .
Table 2. Operative Data
VariableN ? 31 (%)
Mean cross-clamp time (min)
Mean cardiopulmonary bypass time (min)
Aortic valve repair
Patent foramen ovale closure
Atrial septal defect
Ventricular septal defect
Patent ductus arteriosus ligation
101.3 ? 17.5
144.4 ? 21.7
Table 3. Preoperative and Postoperative Sinus and Annular
VariablePreoperative Postoperative p Value
Annulus z score
Maximum sinus (cm)
Maximum sinus z score
2.2 ? 0.5
3.8 ? 2.3
3.9 ? 0.8
7.0 ? 2.9
2.2 ? 0.4
2.6 ? 3.1
3.0 ? 0.4
2.6 ? 2.6
PATEL ET AL
VSRR IN LOEYS-DIETZ SYNDROME
Ann Thorac Surg
Patients with LDS present with a characteristic pheno-
type of hypertelorism, bifid uvula or cleft palate (or both),
and arterial tortuosity . Like their Marfan counter-
parts, patients with LDS share a predisposition for aneu-
rysm of the aortic root and risk of rupture and dissection,
even in childhood. At our institution patients with LDS
are referred for surgical intervention at a younger age
and smaller aortic root diameters than patients with
Marfan syndrome in concordance with the more aggres-
sive vascular phenotype of the former.
We reported our initial surgical experience in patients
with LDS in 2004 . In that study 14 of the 71 patients
with LDS underwent VSRR at 2 separate institutions
(Johns Hopkins and University of Ghent). One pediatric
patient initially underwent VSRR/arch replacement at
Ghent Hospital, required subsequent Bentall operation,
and died 11 years after the initial VSRR procedure. This
initial experience was instructive in the importance of
early prophylactic root replacement and frequent sur-
veillance of the vascular tree. Of the 71 patients, fatal
intracerebral and aortic catastrophies occurred in 3 pa-
tients younger than 10 years of age. Furthermore, aortic
rupture occurred at diameters less than 4.5 cm, which is
a rare event in Marfan syndrome.
For most adult patients with ascending aortic aneu-
rysm, the threshold for elective aortic root replacement is
an aortic diameter of greater than 5.0 cm, rate of aortic
root growth greater than 1.0 cm/year, or worsening aortic
insufficiency. Indications for VSRR for patients with LDS
are not as well defined. Given the much greater risk of
aortic catastrophe in LDS, our current threshold for aortic
root replacement in adults is an aortic root diameter
greater than 4.0 cm or root expansion greater than 0.5
cm/year. For children with severe craniofacial abnormal-
ities (Loeys-Dietz type I) we advise surgery at an aortic
root z score greater than 3.0 or an aortic root expansion
greater than 0.5 cm/year. For children with mild cranio-
facial abnormalities (Loeys-Dietz type II), we recommend
surgery at an aortic root z score greater than 4.0 or an
aortic root that expands greater than 0.5 cm/year.
We are also reconsidering our management of the
distal ascending aorta and proximal arch at the time of
root replacement. None of the patients in this series had
an arch or hemiarch replacement because the arch diam-
eters were normal at the time of the valve-sparing pro-
cedure. However several patients experienced dilatated
distal ascending aortas and proximal arches within a few
years postoperatively; this too is an unusual event in
Marfan syndrome. Further experience may suggest that
more aggressive proximal aortic resection is indicated in
some patients with LDS.
In this present study all but 1 patient had a reimplan-
tation procedure using the Valsalva graft. A straight
Dacron graft was used in that 1 patient because the aortic
root diameter was smaller than 24 mm, the smallest
commercially available Valsalva graft.
The absence of operative and late deaths is encourag-
ing, as is the absence of thromboembolic events or
endocarditis at midterm follow-up. However 1 patient
experienced greater than 2? aortic insufficiency 4.3 years
after reimplantation with the Valsalva graft. At the time
of the original operation we noted marked leaflet fenes-
trations, but these were not considered severe enough to
warrant valve replacement. Although this patient will
likely require valve replacement in the years ahead, the
remainder of the postoperative cohort has enjoyed excel-
lent freedom from significant valve regurgitation or
Surgeon experience and patient selection are critical
for good outcomes. Our operative technique for reim-
plantation using the Valsalva graft is simple and repro-
ducible. We recommend a Bentall procedure for patients
with severe aortic insufficiency who have marked leaflet
asymmetry or leaflet fenestrations as well as for patients
with bicuspid valves and significant stenosis, thickening,
prolapse, or fenestrations. Composite grafts are likely to
play a role in the management of patients with LDS; over
the time course of this study, 30 patients with LDS had an
aortic root replacement with a composite graft.
For postoperative surveillance, we recommend that all
patients who receive VSRR undergo echocardiography,
computed tomography (CT), or magnetic resonance im-
aging 6 months postoperatively and annually thereafter
to monitor aortic root diameter and valve competence.
Because of the aggressive nature of LDS and the risk of
rupture and dissection at smaller aortic root diameters,
we recommend echocardiography every 3 to 6 months for
1 year after surgery and every 6 months thereafter. Given
the widespread involvement of the arterial tree in pa-
tients with LDS, we also recommend annual head-to-
pelvis body CT scans.
Valve-sparing root replacement is a safe and effective
surgical option for the management of aortic root aneu-
rysm in patients with LDS. Because of the malignant and
aggressive nature of this aortopathy, early recognition of
the syndrome by clinical phenotype or genetic analysis is
critical. We recommend the reimplantation technique
with the Valsalva graft for prophylactic root replacement
because of improved stabilization of the annulus, better
hemostasis, and preservation of sinuses. Although long-
term results are not yet available, midterm outcomes are
1. Bentall HH, De Bono A. A technique for complete replace-
ment of the ascending aorta. Thorax 1968;23:338–9.
2. Gott VL, Greene PS, Alejo DE, et al. Replacement of the
aortic root in patients with Marfan’s syndrome. N Engl
J Med 1999;340:1307–13.
3. Gott VL, Cameron DE, Alejo DE, et al. Aortic root replace-
ment in 271 Marfan patients: a 24-year experience. Ann
Thorac Surg 2002;73:438–43.
4. Cameron DE, Alejo DE, Patel ND, et al. Aortic root replace-
ment in 372 Marfan patients: evolution of operative repair
over 30 years. Ann Thorac Surg 2009;87: 1344–50.
5. Sarsan MAI, Yacoub M. Remodeling of the aortic valve
annulus. J Thorac Cardiovasc Surg 1993;105:435–8.
6. David TE, Feindel CM. An aortic valve-sparing operation for
patients with aortic incompetence and aneurysm of the
ascending aorta. J Thorac Cardiovasc Surg 1992;103:617–22.
Ann Thorac Surg
PATEL ET AL
VSRR IN LOEYS-DIETZ SYNDROME
7. Loeys BL, Schwarze U, Holm T, et al. Aneurysm syndromes
caused by mutations in the TGF-beta receptor. N Engl J Med
8. Cameron DE, Vricella LA. Valve-sparing aortic root replace-
ment in Marfan syndrome. Semin Thorac Cardiovasc Surg
Pediatr Card Surg Annu 2005;8:103–111.
9. David TE, Ivanov J, Armstrong S, Feindel CM, Webb GD.
Aortic valve-sparing operations in patients with aneurysms
of the aortic root and ascending aorta. Ann Thorac Surg
10. Kallenbach K, Hagl C, Walles T, et al. Results of valve-
sparing aortic root reconstruction in 158 consecutive pa-
tients. Ann Thorac Surg 2002;74:2026–32.
11. Kallenbach K, Karck M, Pak D, et al. Decade of aortic valve
sparing reimplantation: are we pushing the limits too far?
Circulation 2005;112(9 suppl ): I253–259.
12. Settepani F, Szeto WY, Pacini D, et al. Reimplantation
valve-sparing aortic root replacement in Marfan syndrome
using the Valsalva conduit: an intercontinental multicenter
study. Ann Thorac Surg 2007;83:S769–73.
13. Patel ND, Williams JA, Barreiro CJ, et al. Valve-sparing aortic
root replacement: early experience with the De Paulis Valsalva
graft in 51 patients. Ann Thorac Surg 2006;82:548–53.
14. Bethea BT, Fitton TP, Alejo DE, et al. Results of aortic
valve-sparing operations: experience with remodeling and
reimplantation procedures in 65 patients. Ann Thorac Surg
15. Pacini D, Settepani F, De Paulis R, et al. Early results of
valve-sparing reimplantation procedure using the Valsalva
conduit: a multicenter study. Ann Thorac Surg 2006;82:865–71.
16. Patel ND, Weiss ES, Alejo DE, et al. Aortic root operations
for Marfan syndrome: a comparison of the Bentall and
valve-sparing procedures. Ann Thorac Surg 2008;85:2003–11.
17. de Oliveira NC, David TE, Ivanov J, et al. Results of surgery
for aortic root aneurysm in patients with Marfan syndrome.
J Thorac Cardiovasc Surg 2003;125:789–96.
18. David TE, Feindel CM, Webb GD, Colman JM, Armstrong S,
Maganti M. Aortic valve preservation in patients with aortic
root aneurysm: results of the reimplantation technique. Ann
Thorac Surg 2007;83:S732–5.
19. Leyh RG, Schmidtke C, Sievers HH, Yacoub MH. Opening
and closing characteristics of the aortic valve after different
types of valve-preserving surgery. Circulation 1999;100:
20. Bellhouse BJ. Velocity and pressure distributions in the
aortic valve. J Fluid Mechanics 1969;37:587–600.
21. Thubrikar MJ, Nolan SP, Aouad J, Deck JD. Stress sharing
between the sinus and leaflets of canine aortic valve. Ann
Thorac Surg 1986;42:434–40.
22. Grande-Allen KJ, Cochran RP, Reinhall PG, Kunzelman KS.
Recreation of sinuses is important for sparing the aortic
valve: a finite element study. J Thorac Cardiovasc Surg
23. De Paulis R, De Matteis GM, Nardi P, Scaffa R, Bassano C,
Chiariello L. Analysis of valve motion after the reimplanta-
tion type of valve-sparing procedure (David I) with a new
aortic root conduit. Ann Thorac Surg 2002;74:53–7.
24. Loeys BL, Chen J, Neptune ER, et al. A syndrome of altered
cardiovascular, craniofacial, neurocognitive and skeletal de-
velopment caused by mutations in TGFBR1 or TGFBR2. Nat
25. Williams JA, Loeys BL, Nwakanma LU, et al. Early surgical
experience with Loeys-Dietz: a new syndrome of thoracic
aortic aneurysm disease. Ann Thorac Surg 2007;83:S757–
DR THORALF SUNDT (Rochester, MN): I hate to shake every-
body up twice in the same day between the picture of me from
earlier this morning in Keith’s address and now my appearance
in a congenital session, but I appreciate the invitation from the
program committee nonetheless. I have a long-standing interest
in surgery for congenital heart disease, and the valve-sparing
root procedure is one of my favorite operations as well.
So congratulations Dr Patel. It was a beautiful presentation. In
addition, I should point out that the Tiki Award as originally
conceived can be given for outstanding slides as well, and this is
one of the most clear, beautiful presentations I have seen in a
long time. So I would recommend that the Tiki Committee
consider giving the award to somebody who did it right and not
always for somebody who did it wrong.
Congratulations to you, congratulations to Duke, to Luca, and
to Bill for mentoring you and bringing you along. It is critical to
the health of our specialty that residents in general surgery be
brought along to become interested in this specialty.
My first questions for you are, “How many patients during
this period of time with Loeys-Dietz did not have a valve-
sparing root operation? What percentage, roughly, of patients
with Loeys-Dietz are going to turn out to be candidates for
valve-sparing operations?” Duke has mentioned before that he
has reasonably strict criteria about the amount of leaflet fenes-
tration that he will allow in a patient in whom he will do a
valve-sparing root. What percentage of Loeys-Dietz are candi-
dates do you think?
DR PATEL: Thank you very much for your comments. Over the
eight-year time period, from 2002 to 2009, 31 patients had a
valve-sparing operation and 30 had a composite root replace-
ment, so roughly 50:50.
DR SUNDT: The next question relates to the uniqueness of this
disease, a disease that your group is most familiar with but
others are probably less so. It really gets to the question “Why
write this paper at all?” The implication of performing the
analysis is that in some way these patients are going to be
different from Marfan patients, for example, so share with us
more of your thinking today about this disease. Are there some
special concerns you think about when you are doing a valve-
sparing root procedure in Loeys-Dietz syndrome? Given the
uniqueness of the tissues and such, do you modify the opera-
tion? For example, are you more aggressive with the distal
anastomosis? Do you do a hemiarch replacement or even a total
arch replacement with the idea that these patients are subject to
subsequent aneurysmal dilatation or dissection of the arch?
What is your attitude about the leaflets? My experience with
some patients with Marfan syndrome is that there is a spectrum
of how “stretchy” the leaflets are. Is this a concern in Loeys-
Dietz syndrome? What keeps your group up at night worried
about late outcomes.
Congratulations on a really beautiful presentation.
DR PATEL: Thank you again for those very important questions.
With regard to why we did this study and what we were
anticipating with the results for this study, we do believe that
these patients have very, very malignant tissues. Our initial
experience back in 2004, which we reported in the Annals of
Thoracic Surgery, was 71 patients we described who had Loeys-
Dietz syndrome; roughly half of them were from the University
PATEL ET AL
VSRR IN LOEYS-DIETZ SYNDROME
Ann Thorac Surg
of Ghent, where Dr Loeys is located, and the other half were Download full-text
from our institution with Hal Dietz. Of those 71 patients, 5
children less than 10 years of age with roots less than 4 cm died
of a fatal aortic catastrophe, so given that very early data, we
decided to be very aggressive with proceeding with root replace-
ment and monitoring them postoperatively.
Typically for Marfan syndrome we will obtain echocardio-
grams at 6 months and then annually thereafter. These patients
are followed every 3 months with echocardiography and then
every year with CT imaging from the head all the way down to
With regard to replacing the arch, none of the patients in this
series have had abnormal arch diameters and none of them have
had prophylactic arch replacement, but we have seen some of
our composites come back into the clinic with some arch and
descending thoracic aneurysms. At this time we are hesitant to
replace the arch and proceed with a more aggressive descending
thoracic resection because we will be subjecting these patients to
a much more difficult operation as well as subjecting their very
friable tissues to more suture lines. What we are hoping is that
with more long-term data we will be able to identify specific
patients who may benefit from a more aggressive surgical
DR ROBERT STEWART (Cleveland, OH): I echo Dr Sundt’s
compliments on your wonderful presentation. My question
relates to a patient with Loeys-Dietz syndrome who requires a
cardiac operation for a lesion other than in their aorta. If such a
patient has aortic dilatation, but it does not quite meet your
criteria for repair, do you feel that continued dilatation is
inevitable and if so, would you consider replacing the aortic root
when you are fixing the other lesion?
DR PATEL: Well, that is interesting in this set of patients
because as you saw in my clinical characteristics slide there,
there were 2 patients who were fairly elderly when they received
their operation; 1 was 60 years of age. We do believe that these
patients will eventually have dilatated aortic roots. At present, I
would say, from what Dr Cameron and Dr Vricella have told me,
that we should proceed with replacing their roots given these
very early criteria. These criteria that we have developed aren’t
based on any data, unfortunately, because we really don’t have
that data available to us yet. A lot of the natural history of this
disease is still unknown. Hopefully with more long-term fol-
low-up with these patients, as well as patients who have not
undergone surgery, we will be able to better answer that
Ann Thorac Surg
PATEL ET AL
VSRR IN LOEYS-DIETZ SYNDROME