25/01/2021
💠AORTOPULMONARY SEPTAL DEFECT
🔷Pathology and Pathophysiology
In aortopulmonary septal defect (also known as aortopulmonary [AP] window), a large defect is present between the ascending aorta and the main PA. This condition results from failure of the spiral septum to completely divide the embryonic truncus arteriosus. Unlike persistent truncus arteriosus, two separate semilunar valves are present in this condition.
🔷Clinical Manifestations
1. Clinical manifestations are similar to those of persistent truncus arteriosus and are more severe than those of PDA. CHF and pulmonary hypertension appear in early infancy. Peripheral pulses are bounding, but the heart murmur is usually of the systolic ejection type (rather than continuous murmur) at the base.
2. The natural history of this defect is similar to that of a large untreated PDA, with development of pulmonary vascular obstructive disease in surviving patients.
🔷Management
Prompt surgical closure of the defect under cardiopulmonary bypass is indicated. The surgical mortality rate is very low.
➡Congenitally complete or dextro-transposition of the great arteries (d-TGA) with (a) and without (b) ventricular septal defect (VSD). The aorta (Ao) arises from the right ventricle (RV) and lies anterior and to the right of the pulmonary trunk (PT), whereas the PT arises from the left ventricle (LV). PDA, patent ductus arteriosus; LA, left atrium; RA, right atrium.⬇