Abstract
Prematurity and low birthweight are associated with increased mortality in infants undergoing cardiac surgery. Pulmonary atresia with intact ventricular septum and right ventricle-dependent coronary circulation carries one of the highest risks of mortality. We present a patient who was born at 28 weeks of gestation at 1.2 kg, with pulmonary atresia intact ventricular septum, right ventricle-dependent coronary circulation, coronary artery atresia, and discontinuous pulmonary arteries, who successfully underwent palliation with a modified Blalock-Taussig shunt, pulmonary arterioplasty, and subsequently a bidirectional Glenn.
Highlights
Pulmonary atresia intact ventricular septum comprises 1% of CHDs.[1]
At the most severe end of the spectrum are those with severe right ventricle hypoplasia, right ventricle-dependent coronary circulation, and proximal coronary atresia
We present a case with twin gestation, very preterm birth, very low birthweight, with pulmonary atresia intact ventricular septum, right ventricle-dependent coronary circulation, coronary atresia, and discontinuous pulmonary artery
Summary
Pulmonary atresia intact ventricular septum comprises 1% of CHDs.[1]. It occurs as a spectrum, defined by complete obstruction of the pulmonary valve, with varying degree of tricuspid and right ventricular hypoplasia. Patients undergoing single ventricle palliation demonstrate good outcome, with reported 80% survival at discharge and post-Fontan survival up to 96%.2,3 right ventricle-dependent coronary circulation is a risk factor for mortality, especially when associated with coronary ostial atresia.[3–5] Confounding the outcome of these infants is the presence of prematurity and low birthweight, both of which are associated with increased mortality and morbidity in infants undergoing cardiac surgery.[6–8]
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