Turk Kardiyol Dern Ars. 2005; 33(4): 225-228

One-stage repair of aortopulmonary septal defect and interrupted aortic arch in an infant

Gülhis Batmaz¹, Halil Türkoğlu², Ahmet Şaşmazel³, Vedat Bayer², Aydın Aytaç^2
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Aortopulmonary window (APW) is a rare cardiac anomaly. It may be present in conjunction with interrupted aortic arch (IAA). A one-month-old female infant was referred to our institution with suspected aortic coarctation. Physical examination revealed a left parasternal systolic ejection murmur and absence of femoral pulses. Arterial blood pressure was 120 mmHg in upper extremities and 65 mmHg in lower extremities. Transthoracic echocardiography showed a 13-mm communication between the main pulmonary artery and ascending aorta in the subcostal long axis view. There was a 67-mmHg gradient of a coarctation flow pattern across the proximal descending aorta. Surgical intervention was delayed until three months of age due to recurrent pulmonary infections. A type A IAA and a proximal type APW was detected during surgery. Under deep hypothermic circulatory arrest, a single-stage total correction was carried out with end-to-end repair of the IAA and intra-aortic Gore-Tex patch closure of the APW. In the postoperative period, the patient developed recurrent pulmonary infections and transthoracic echocardiography showed persistence of pulmonary hypertension. Three months after surgery, pulmonary hypertension decreased and the general condition of the infant improved. To our knowledge, this is the first reported case of a single-stage repair of APW and IAA in Turkey.

Keywords: Aorta, thoracic/abnormalities/surgery; aortopulmonary septal defect/surgery; cardiopulmonary bypass; echocardiography; heart defects, congenital/diagnosis; infant

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