An Elderly Lady with Unexpected Oligosymptomatic Aortopulmonary Window Associated with Atrial Septal Defect: Rare Case from Bangladesh

In aortopulmonary window (APW) (also known as aortopulmonary septal defect, aortopulmonary fenestration), a large defect is present between intrapericardial portion of the ascending aorta and the main pulmonary artery. This condition results from failure of the spiral septum to completely divide the embryonic truncus arteriosus. AP window is a rare lesion that can mimic patent ductus arteriosus (PDA) clinically and comprises only 0.1% to 0.6% of congenital heart defects. The defect is usually large; therefore, the likelihood of established pulmonary hypertension in the adult patient is high unless closure took place early in childhood. About 10% of aortopulmonary windows are relatively small and restrictive, in terms of pressure, and, therefore, not susceptible to early pulmonary hypertension. Aortopulmonary windows are commonly associated with other cardiac lesions such as ventricular septal defect (VSD), tetralogy of Fallot, subaortic stenosis, atrial septal defect (ASD), or PDA and thus can be easily overlooked. Occasionally, the right and rarely the left coronary arteries are transposed to the pulmonary trunk, and this must be taken into consideration in the surgical planning. Fifty percent of patients usually have no other heart defects. Here we describe an elderly Bangladeshi lady with chronic exertional dyspnoea for years. She was found to have aorto pulmonary window with atrial septal defect on echocardiography with mild pulmonary hypertension. She was referred to a higher center for surgical management.

Keywords:Aortopulmonary window; Congenital heart defects; Atrial septal defect; Pulmonary hypertension