Pulmonary Atresia with Intact Ventricular Septum

Abstract

The pathology of pulmonary atresia with intact ventricular septum ranges between two extremes. One extreme is that of a hypoplastic right ventricle (RV) with no significant regurgitation of tricuspid valve with RV to coronary arterial sinusoids. The other extreme is that of a dilated right ventricle with tricuspid regurgitation and typically no RV to coronary sinusoids. Holosystolic murmur in patients with pulmonary atresia suggests severe tricuspid regurgitation. The right ventricle in such cases is typically dilated. Patients with hypoplastic right ventricle and competent tricuspid valves do not exhibit holosystolic murmur. Abnormal coronary arteries with supply from the right ventricular cavity and stenosis constitute high risk patients with possibilities of sudden death due to myocardial ischemia and ventricular arrhythmias. Ductus arteriosus patency is needed preoperatively as it provides the only source of pulmonary blood flow. After surgical or interventional cardiac catheterization repair, patency of ductus arteriosus is still needed till forward flow across the right heart and pulmonary valve is established; this may require several days or weeks to achieve.