Download Double-Outlet Left Ventricle with Ebstein Anomaly in a Neonate with

DOLV with Ebstein Anomaly in VACTERL
Case Report
Acta Cardiol Sin 2011;27:65-7
Double-Outlet Left Ventricle with Ebstein Anomaly
in a Neonate with VACTERL Association
Hok-Keong Chang,2 Jieh-Neng Wang,1 Wen-Pin Hung,3 Wen-Lan Yen1 and Jing-Ming Wu1
Double-outlet left ventricle is a very rare congenital cardiac malformation in which both the aorta and the
pulmonary artery arise predominantly or exclusively from the morphologic left ventricle. We describe a neonate
with esophageal atresia with tracheo-esophageal fistula (so-called VACTERL association) and unusual cardiac
anomalies including double-outlet left ventricle, subaortic ventricular septal defect, pulmonary stenosis, and
Ebstein anomaly. Many morphologic variations of cardiac malformation have been described in patients with
VACTERL association, but to the best of our knowledge, double-outlet left ventricle combined with Ebstein
anomaly has never been reported.
Key Words:
Cardiac anomaly · Double-outlet of ventricle · Ebstein anomaly · VACTERL association
INTRODUCTION
antenatal check up. On examination, she presented decreased activity and bubbling salivation from the mouth.
A grade III/VI systolic heart murmur was audible over
the left upper sternum. Chest X-ray showed blinded end
of esophagus. The patient was finally diagnosed with
tracheo-esophageal fistula by computed tomography
scan. Echocardiography revealed a septal leaflet of
tricuspid valve displaced into the right ventricle cavity,
forming an atrialized right ventricle (Figure 1A). There
was a D-loop ventricle with a subaortic ventricle septal
defect (VSD) and pulmonary stenosis with pressure gradient of 51 mmHg. Both aorta and pulmonary artery
originated from the left ventricle, with the aorta right
lateral to the pulmonary trunk (Figure 1B). Cardiac
catheterization with left ventriculogram confirmed the
diagnosis (Figure 2). The patient underwent modified
Blalock-Taussig shunt later.
Double-outlet ventricle is an uncommon cardiac
anomaly that only contributes 1% of congenital heart
disease. Meanwhile, double-outlet left ventricle (DOLV)
accounts for less than 5% in double-outlet ventricle
cases.1-3 Herein, we described a very rare cardiac anomaly of DOLV associated with Ebstein anomaly in a
neonate with VACTERL association.
CASE REPORT
A 1-day-old female baby, small for gestational age
with birth weight 2.2 kg, was found to have maternal
polyhydramnios and decreasing fetal movement during
Received: May 13, 2010
Accepted: June 28, 2010
1
Department of Pediatrics, National Cheng Kung University Hospital;
2
Department of Pediatrics, Madou Sin Lau Hospital; 3Department of
Pediatrics, Kuo General Hospital, Tainan, Taiwan.
Address correspondence and reprint requests to: Dr. Jing-Ming Wu,
Department of Pediatric Cardiology, National Cheng Kung University
Hospital, No. 138 Sheng-Li Road, Tainan 70403, Taiwan. Tel: 8866-235-3535 ext. 4187; Fax: 886-6-275-3083; E-mail: jingming@
mail.ncku.edu.tw
DISCUSSION
Ventriculoarterial connection is considered doubleoutlet ventricle when both of the great arteries mainly
originate from the same ventricle. The occurrence of
DOLV is significantly less than that of double-outlet
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Acta Cardiol Sin 2011;27:65-7
Hok-Keong Chang et al.
A
B
Figure 1. (A) Apical four chamber echocardiogram shows downward displacement of the septal leaflet of tricuspid valve (black double arrow
head) measured 1 cm and an atrialized right ventricle (aRV). RA: right atrium. (B) Subcostal long-axis echocardiogram shows both the aorta (Ao)
and pulmonary trunk (PA) originated from the left ventricle (LV) and a subaortic ventricular septal defect. RV: right ventricle.
tricular valve anomalies were also noted in 30% of
cases, and mostly in the tricuspid valve. Among 109
cases of DOLV reported by Van Praagh et al.,1-2 only one
autopsy case, similar to our case, presented with DOLV,
subaortic VSD, pulmonary stenosis and Ebstein anomaly.
Congenital heart disease is the leading combined
anomaly of VACTERL association in Scott et al.’s series,5-7 with incidence up to 32.1%.The most common
heart anomaly is a VSD, which accounted for 22.3%.5
Cyanotic heart disease was uncommon with only 4.5%
in these patients. Yang et al.6 declared the rate of heart
disease association can be as high as 50%. Though congenital heart disease is a common associated anomaly in
VACTERL, the combination of DOLV and Ebstein
anomaly has never been reported.
Figure 2. Left ventriculogram (antero-posterior view) shows that both
the aorta and pulmonary trunk (PA) originated from the left ventricle
(LV).
REFERENCES
1. Hagler D. Double-outlet right ventricle and double-outlet left
ventricle. In Allen HD, Driscoll DJ, Shaddy RE, Feltes TF. Moss
and Adams’Heart Disease in Infants, Children, and Adolescents,
Vol. 2, Ed. 7. Philadelphia: Lippincott Williams & Wilkins,
2008;1100-27.
2. Van Praagh R, Weinberg PM, Srebro JP. Double-outlet left
ventricle. In: Adams FH, Emmanouilides GC, Riemenschneider
JA, eds. Moss and Adams’ Heart Disease in Infants, Children,
and Adolescents. 4th ed. Baltimore: Williams & Wilkins, 1989;
461-85.
3. Lin CY, Tsai CS, Hong GJ, et al. Long-term results after biventricular repair for double-outlet right ventricle. Acta Cardiol Sin
2009;25,85-90.
right ventricle.1-3 It is difficult to use embryologic basis
to describe the existence of DOLV. Manner et al.4 tried
to explain it by an excessive leftward shift of the embryonic conotruncus, anomalous differential conal
growth, or absorption or malorientation of the subarterial
portion of the ventricular septum above the crista supraventricularis separating the right ventricle infundibulum from both great arteries. DOLV is often associated with a VSD. The VSD may locate in a subaortic,
subpulmonary, doubly committed or remote area. Subaortic VSD is the most common type. 1,2 AtriovenActa Cardiol Sin 2011;27:65-7
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DOLV with Ebstein Anomaly in VACTERL
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