The present perspective is a synthesis of published investigations in the setting of subvalvular aortic aneurysms. We identified 75 investigations and reviewed the clinical presentation, diagnostic modalities used, surgical techniques employed, and their outcomes. Clinical presentation, radiographic findings, transthoracic and transesophageal echocardiography, electrocardiogram-gated computerized tomography, and magnetic resonance imaging provided the diagnostic information and were used to define the disease entity before surgery. In this article, we have attempted to address several issues concerning establishment of diagnosis, varied clinical presentation, and their management. We submit that an increased appreciation of this disease entity will contribute to improved surgical management.

We performed Dacron patch closure of ventricular septal defect with concomitant aortic valve replacement using a St. Jude Medical mechanical aortic prosthesis for a patient with ventricular septal defect and severely deformed and irreparable aortic valve. We highlight the technical details of the procedure to prevent paravalvular aortic leakage and residual ventricular septal defect.

The atrioventricular septal defects encompass a spectrum of lesions in which the common etiology appears to be abnormal development of the superior and inferior endocardial cushions, resulting in a deficiency or absence of the atrioventricular septum [1-6]. This deficiency of the atrioventricular septum results in an ostium primum defect immediately above the atrioventricular valves and a scooped-out area in the inlet (basal) portion of the ventricular septum. Data from the New England Regional Infant Cardiac Program and the Baltimore-Washington infant study defined a prevalence of 0.118 and 0.352 per 1000 live births [7,8]. Approximately, 50-75% of these patients have trisomy 21 [7-9]. When viewed from a reverse perspective, approximately 35-40% of patients with Down’s syndrome have an atrioventricular septal defect [7-9].

The atrioventricular septal defects encompass a spectrum of lesions in which the common etiology appears to be abnormal development of the superior and inferior endocardial cushions, resulting in a deficiency or absence of the the atrioventricular septum [1,2]. Data from the New England Regional Infant Cardiac Program and the Baltimore-Washington infant study defined a prevalence of 0.118 and 0.352 per 1000 live births [3,4]. Approximately, 50-75% of these patients have trisomy 21 [5]. When viewed from a reverse perspective, approximately 35-40% of patients with Down’s syndrome have an atrioventricular septal defect [5].

A 10-year-old female patient diagnosed with isolated type I aortopulmonary window and severe pulmonary arterial hypertension who underwent repair of the aortopulmonary window via Johansson's transwindow approach using a unidirectional, fenestrated, valved, aorticopulmonary patch. The preoperative pulmonary vascular resistance was 9.0 Woods units/m2 that reduced to 4.0 Woods units/m2 after oxygen (100%) and nitric oxide (80 ppm) administration. The postoperative recovery was uneventful.