Abstract
ABSTRACTAortopulmonary window (APW) is a rare congenital heart defect with abnormal communication between the ascending aorta and the pulmonary trunk with two separate semilunar valves. We present an autopsy case report wherein a young primigravida woman presented with progressive breathlessness and central cyanosis at 21 weeks of gestation. Echocardiography performed in the emergency room revealed elevated right-sided cardiac pressures suggestive of severe pulmonary hypertension; however, no structural cardiac defect was discernible. The patient succumbed to congestive cardiac failure and progressive hypoxia within 5 days of hospitalization. The autopsy revealed a Type I aortopulmonary window (2 cm) with patent ductus arteriosus. The lungs showed changes of severe pulmonary hypertension with superadded bronchopneumonia. This report underscores a rare presentation of APW, undiagnosed until pregnancy, leading to the Eisenmenger syndrome and death.
Highlights
Aortopulmonary window (APW) is a rare congenital heart defect that accounts for 0.1-0.2% of all congenital heart defects.[1,2,3]
We describe an adult case of APW associated with patent ductus arteriosus who first presented at 21 weeks of gestation with cyanosis and rapidly succumbed to her illness
The aortopulmonary window arises due to embryonic failure of the fusion of two opposing conotruncal ridge or spiral septum, which separates the truncus arteriosus into the aorta and pulmonary artery
Summary
Aortopulmonary window (APW) is a rare congenital heart defect that accounts for 0.1-0.2% of all congenital heart defects.[1,2,3] It is characterized by abnormal communication between the ascending aorta and pulmonary trunk with two separate aortic and pulmonary valves producing a left-to-right shunt. The presentation time can be variable, ranging from the prenatal period to as late as the seventh decade.[4] Surgical correction at an early stage can prevent the development of irreversible pulmonary hypertension (PHT) and Eisenmenger syndrome.[5] We describe an adult case of APW associated with patent ductus arteriosus who first presented at 21 weeks of gestation with cyanosis and rapidly succumbed to her illness. Examination revealed a respiratory rate of 32/min, a heart rate of 110/min, and SpO2 of 27% on room air. She had bilateral, symmetrical, Grade 2 clubbing fingers and. The patient had persistent hypoxia and refractory shock, to which she succumbed on the third day of hospitalization.
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