Article type: Images - Pediatric
Title: Tricuspid atresia type 1B with left superior vena cava in a
4-month-old child: An unusual combination diagnosed by transthoracic
echocardiography
Authors
- Subrahmanya Murti Velamakanni, MD, Resident physician, Department of
Cardiology, Smt NHL Municipal Medical College, Ahmedabad, India
- Aman Tejas Patel, MD, Resident physician, Department of Cardiology,
Smt NHL Municipal Medical College, Ahmedabad, India
- Gajanan Khadkikar, MD, Resident physician, Department of Cardiology,
Smt NHL Municipal Medical College, Ahmedabad, India
- Tejas Patel, DM, Professor and head, Department of Cardiology, Smt NHL
Municipal Medical College, Ahmedabad, India
Corresponding author: Subrahmanya Murti Velamakanni, Room 12060, SVP
Hospital, Ellisbridge, Ahmedabad, India. Email id:
subrahmanyamurti@gmail.com
Keywords: tricuspid atresia, left superior vena cava, cyanotic
congenital heart disease, coronary sinus
Abstract
Tricuspid atresia (TA) is a rare cyanotic congenital heart disease. A
persistent left superior vena cava (LSVC) may be associated with TA. The
presence of LSVC raises important considerations for eventual repair, in
that it may lead to persistent arterial desaturation even after
corrective surgery, if associated with an unroofed coronary sinus. Here,
we present the case of a 4-month-old child who was diagnosed with TA
type 1B, LSVC and a dilated coronary sinus by transthoracic
echocardiography.
Article text
Tricuspid atresia (TA) is a rare cyanotic congenital heart disease with
an estimated incidence of 79 per million live births.1 Tricuspid atresia has been classified according to the modified
Edward-Burchells classification into three major types based on the
relations of the great arteries and degree of pulmonary
stenosis.2 A persistent left superior vena cava (LSVC)
may be associated with TA and raises an important consideration for
eventual repair, in that it may lead to persistent arterial desaturation
even after corrective surgery if associated with an unroofed coronary
sinus.3 Here, we present the case of a cyanosed
4-month-old child who presented with failure to thrive. On examination,
a grade 4/6 pan-systolic murmur was heard in the left lower sternal
border with no other obvious dysmorphic features. On transthoracic
echocardiography, there was situs solitus and levocardia. A large ostium
secondum atrial septal defect (ASD) was noted with predominant right to
left shunting (figure 1). The tricuspid valve was atretic with no flow
across the same. On examination in the parasternal long axis view, a
dilated coronary sinus (CS) was noted (figure 2). A large sub aortic
ventricular septal defect was seen with left to right shunting filling a
hypoplastic right ventricle and the left ventricle was dilated (figure
3A). The pulmonary valve was morphologically normal, main pulmonary
artery was dilated and branch pulmonary arteries were normal. A patent
ductus arteriosus was present with a left to right shunt (figure 3B). On
modified high parasternal views, LSVC was identified (figure 4A). A
right ventricular outflow tract maximum gradient of about 50 mmHg was
seen suggesting moderate sub-pulmonary stenosis (figure 4B).
Atrio-ventricular and ventriculo-arterial concordance was preserved.
Pulmonary venous return was normal. As per the modified
Edwards-Burchells classification, this was a type 1B
TA. Most children with TA require
the Glenn’s or the Fontan’s shunt for correction. An associated LSVC and
unroofed CS with TA has been less reported and assumes importance for eventual corrective surgery.4 Therefore, in all cases of TA, an
LSVC must be sought for, and confirmed using either contrast
echocardiography or cardiac computed tomography (CT) as it may have
important operative implications.
References
- Hoffman JI, Kaplan S. The incidence of congenital heart disease. J Am
Coll Cardiol. 2002 Jun 19;39(12):1890-900
- Tandon R, Edwards JE. Tricuspid atresia. A re-evaluation and
classification. J Thorac Cardiovasc Surg. 1974 Apr;67(4):530-42
- Gupta MD, Girish MP, Ramakrishnan S. Persistent left superior vena
cava draining into an unroofed coronary sinus in tricuspid atresia: an
uncommon anomaly. Pediatr Cardiol. 2011 Apr;32(4):530-1
- Burch M, Stark J, Sullivan I. Unexpected oxygen desaturation after
cardiopulmonary bypass: rapid confirmation of unroofing of the
coronary sinus by intraoperative epicardial echocardiography. Pediatr
Cardiol. 1995 Jul-Aug;16(4):197-200
Legends to figures
Figure 1: A: Showing a subcostal view with color doppler demonstrating
an ostium secondum atrial septal defect with predominant right to left
shunt, B: Subcostal bicaval view showing the atrial septal defect. LA –
left atrium, RA – Right atrium, OS-ASD – Ostium Secondum atrial septal
defect
Figure 2: A: Parasternal long axis view showing a dilated coronary
sinus, B: Foreshortened apical view showing a dilated coronary sinus. LA
– left atrium, LV- left ventricle, RA – Right atrium, RV – Right
ventricle, Ao – Aorta, CS – Coronary sinus
Figure 3: A: Showing an apical four chamber view, B: Color doppler of
the parasternal short axis view showing a patent ductus arteriosus with
left to right shunt. LA – left atrium, LV- left ventricle, RA – Right
atrium, RV – Right ventricle, RVOT – RV outflow tract Ao – Aorta, TV
– Tricuspid Valve, VSD – Ventricular septal defect, PDA – Patent
ductus arteriosus, MPA – Main pulmonary artery, LPA – Left pulmonary
artery
Figure 4: A: Color doppler of a high parasternal view showing a left
superior vena cava, B: Showing a maximum gradient of 50 mmHg on
continuous wave doppler of the right ventricular outflow tract. LV –
Left ventricle, SVC – Superior Vena Cava
Supplementary material: Movie clip for figures 1,2,3,4