Aorto-right ventricular tunnel causing functional tricuspid atresia.

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Stiller
Barbara E.U. Burkhardt, Raoul Arnold, Christian Schlensak, Regina Bökenkamp and Brigitte
Right Ventricular Tunnel Causing Functional Tricuspid Atresia
−
Aorto
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Images in Cardiovascular Medicine
Aorto–Right Ventricular Tunnel Causing Functional
Tricuspid Atresia
Barbara E.U. Burkhardt, MD; Raoul Arnold, MD, PhD; Christian Schlensak, MD, PhD;
Regina Bo ¨kenkamp, MD; Brigitte Stiller, MD, PhD
A
primary cesarean section to a 27-year-old primiparous
woman. Fetal echocardiography had shown a nearly atretic
tricuspid valve. Postnatally, the child stabilized rapidly but
girl with symmetrical intrauterine growth retardation
and a birth weight of 2240 g was delivered at term by
showed signs of heart failure with tachypnea and general
weakness, as well as cyanosis (transcutaneous oxygen
saturation, 85%).
The presence of an aorto–right ventricular (RV) tunnel
(ARVT) was confirmed by transthoracic echocardiography,
Figure 1. Echocardiographic views. A, Apical
4-chamber view. Arrow indicates the minimal
tricuspid valve opening during diastole. B, Aor-
to–right ventricular tunnel (ARVT). C, Diastolic
left-to-right shunt through the ARVT (arrow). RA
indicates enlarged right atrium; RV, right ventricle;
and Ao, aorta.
From the Department of Congenital Heart Disease and Pediatric Cardiology (B.E.U.B., R.A., B.S.) and Department of Cardiovascular Surgery (C.S.),
University Hospital, Freiburg, Germany, and Leids University Medisch Centrum, Leiden, the Netherlands (R.B.).
The online-only Data Supplement is available with this article at http://circ.ahajournals.org/lookup/suppl/doi:10.1161/CIRCULATIONAHA.111.062570/-/
DC1.
Correspondence to Barbara E.U. Burkhardt, MD, Department of Congenital Heart Disease and Pediatric Cardiology, University Hospital Freiburg,
Mathildenstraße 1, 79106 Freiburg, Germany. E-mail barbara.burkhardt@uniklinik-freiburg.de
(Circulation. 2012;125:e591-e594.)
© 2012 American Heart Association, Inc.
Circulation is available at http://circ.ahajournals.orgDOI: 10.1161/CIRCULATIONAHA.111.062570
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which showed a 5 to 6?15–mm structure extending from the
proximal ascending aorta to the outflow tract of the RV, with the
left coronary artery originating from the aortic end of the tunnel.
The ARVT caused a strong left-to-right shunt during diastole
(Figure 1 and Movies I and II in the online-only Data
Supplement).
Both ventricles were well developed, with the RV
showing distinct hypertrophy. Tricuspid valve diameter
was 10 mm, with ?3 mm of maximum diastolic opening
and fibrotic, misshapen chordae. Blood flow across the
tricuspid valve was minimal and mainly regurgitant, giving
the appearance of nearly tricuspid atresia. There was a
5-mm-wide right-to-left shunt across a secundum-type
atrial septal defect.
The mitral and semilunar valves were intact; the aortic and
pulmonary valve annular diameters were 8 and 10 mm,
respectively; and the ascending aorta was dilated to 16 mm.
Chest x-ray revealed severe cardiomegaly and pulmonary
hyperperfusion (Figure 2).
Retrograde catheterization of the ascending aorta and
contrast injection proximal to the orifice of the tunnel
confirmed a tunnel length of 15 mm, width of up to 6 to
7 mm, and normal origin of the right coronary artery from
the right coronary sinus. The left coronary artery could not
be visualized (Figure 3 and Movie III in the online-only
Data Supplement).
After retrogradely passing the ARVT, the RV showed
systemic pressure. Injection of contrast into the RV revealed
mild tricuspid regurgitation and normal antegrade emptying
into the pulmonary artery.
Surgery was carried out by opening the ascending aorta.
Because of the intratunnular origin of the left coronary artery,
the aortic orifice was left open, and a right ventriculotomy
and patch closure of the right ventricular orifice of the tunnel
were performed.
The hypertrophied RV showed marked endocardial fibro-
elastosis. Muscular resection was necessary to improve RV
Figure 2. Preoperative (A) chest x-ray
showing severe cardiomegaly and 3
weeks postoperatively (B).
Figure 3. Still images from angiography. A, Con-
trast injection into the left ventricle (LV) shows the
tunnel originating from the ascending aorta (Ao)
and leading to the right ventricle (RV). B, Contrast
drains into the RV from the ascending aorta. C, A
catheter is passed from the descending aorta
through the tunnel into the RV, thus demarcating
the position of the tunnel.
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function. The tricuspid valve appeared to open enough to
allow adequate RV filling after tunnel closure.
Postoperatively, antegrade flow across the tricuspid valve
was not accelerated above 1 m/s. RV systolic pressure was
echocardiographically estimated at half the systemic blood
pressure.
Delayed sternal closure was performed on the third post-
operative day, and the patient’s recovery was uneventful. She
receives acetylsalicylic acid for anticoagulation.
Histology of the RV part of the tunnel revealed an elastic
artery with smooth muscle cells and well-organized elastin
lamellae (Figure 4).
ARVT is a rare entity thought to represent abnormal
formation of the arterial valvar sinuses and leaflets during
embryonic development of the outflow tracts,1with only
16 patients reported in the English-language literature to
date (Table).2–4We demonstrate a neonate with ARVT in
whom both preoperative echocardiography and angiogra-
Figure 4. Histology of the right ventricular part of the tunnel showing organized elastin lamellae and few ?-smooth muscle (SM) actin–
positive cells (C); detail below (F). HE indicates hematoxylin/eosin (A); detail below (D); RF, resorcin/fuchsin. (B); detail below (E).
Table. Reported Cases of Aorto–Right Ventricular Tunnel
AuthorYearJournal Age at DiagnosisOther FeaturesOutcome
Bharati et al
Saylam et al
Jureidini et al
Kleikamp et al
1973
1974
1989
1992
Chest6 h
10 y
0 d
0 d
RCA and left circumflex artery from ARVT
Absent LCA ostium
Critical PS
LCA from ARVT, rudimentary valve in ARVT,
persistent ductus arteriosus, persistent
foramen ovale
…
Absent RCA ostium, PS
…
Autopsy case
Survived
Died
Died
Annals of Thoracic Surgery
Pediatric Cardiology
Journal of Thoracic and Cardiovascular
Surgery
Westaby et al
Rosengart et al
Van Son et al
1992
1993
1998
Annals of Thoracic Surgery
Annals of Thoracic Surgery
European Journal of Cardio-Thoracic
Surgery
Annals of Thoracic Surgery
International Journal of Cardiology
Heart
Fetal Diagnosis and Therapy
Journal of Invasive Cardiology
Journal of the American College of
Cardiology
Journal of Cardiac Surgery
Annals of Thoracic Surgery
Circulation
3 mo
3 mo
15 mo
Survived
Died
Survived
Vargas et al
Talwar et al
Hruda et al
Freund et al
Poptani et al
Bratsas et al
1998
1999
2001
2007
2010
2010
7 mo
2 y
10 d
Persistent ductus arteriosus
RCA from ARVT
PS
LCA from ARVT
Single coronary artery
LCA from ARVT
Survived
Died
Survived
Survived
Survived
Adult
Prenatal, 36-wk gestation
5 y
31 y
Talwar et al
Singh et al
Dwivedi et al
2011
2012
2012
6 wk
16 y
15 y
RCA from ARVT
Single coronary artery
Single coronary artery
Survived
Survived
Survived
RCA indicates right coronary artery; ARVT, aorto–right ventricular tunnel; LCA, left coronary artery; and PS, pulmonary stenosis.
Burkhardt et alAorto–Right Ventricular Tunnel
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phy suggested nearly tricuspid atresia, whereas the RV was
well developed and hypertrophic as a result of retrograde
diastolic filling via the ARVT (Figure 5). It is therefore
important to consider that tricuspid valve function cannot
be assessed reliably unless blood flow through the tunnel
has been eliminated. In this patient, the tricuspid valve
opened sufficiently; thus, surgical repair by patch closure
of the ARVT and resection of muscular and endocardial
fibroelastotic material from the RV was successful. Cor-
rective surgery in the early neonatal period was necessary
because of pulmonary hypertension and highly excessive
pulmonary blood flow. Clinicians should be certain of the
origin and course of the coronary arteries before making
surgical decisions.
Disclosures
None.
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