ABSTRACT
Aortopulmonary septal defect [or window] is an uncommon congenital cardiac defect characterized by a deficiency in the septum between the aorta and the pulmonary artery. It is a rare cardiac defect comprising about 0.1-0.3% of congenital heart diseases in children. In about one half of affected patients, it presents as an isolated defect and in the other half, presents as a more complex heart disease in conjuction with another associated cardiac defect. A patent ductus arteriosus is seen in almost three fourths of patients and an interrupted aortic arch or severe coarctation is present in 10-15%. There have been few reports of aortopulmonary window presenting in patients affected by other syndromes, such as VACTERL association. If left untreated, it results in irreversible pulmonary vascular changes and early mortality. The prognosis for isolated aortopulmonary septal defect, with early surgical intervention, is good. In the presence of associated cardiac disease, the prognosis will depend on the nature and severity of the other lesions. An aortopulmonary window does not spontaneously close, and surgical repair is mandatory to prevent the development of pulmonary vascular obstructive disease. Most centers use techniques that involve cardiopulmonary bypass. Aortopulmonary septal defect is a rare defect, and its occurrence in association with extracardiac malformations [such as in VACTERL association] is even rarer. Here we report a case of aortopulmonary septal defect associated with unilateral renal agenesis. A five day old Indian male neonate with unilateral renal agenesis presented in the ER with cardiogenic shock. He was brought by his parents with complaints of poor feeding. A two-dimensional echocardiography revealed an aortopulmonary septal defect with coarctation of the aorta