Author + information
- Received May 1, 2013
- Accepted May 7, 2013
- Published online November 5, 2013.
- Pairoj Chattranukulchai, MD∗,†,
- Sudarat Satitthummanid, MD∗,†,
- Sarinya Puwanant, MD∗,†,
- Suphot Srimahachota, MD∗,†,
- Seri Singhatanadgige, MD†,‡ and
- Smonporn Boonyaratavej, MD∗,†
- ∗Division of Cardiology, Department of Medicine, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand
- †Cardiac Center, King Chulalongkorn Memorial Hospital, Thai Red Cross Society, Bangkok, Thailand
- ‡Division of Cardiothoracic Surgery, Department of Surgery, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand
A 26-year-old man without known medical history presented with progressive exertional dyspnea. He had central cyanosis (saturation of 88%) and signs of pulmonary hypertension. Chest radiograph showed markedly enlarged pulmonary trunk (A, arrowheads). Echocardiography demonstrated severe pulmonary hypertension and a large communication (B) between ascending aorta (Ao) and pulmonary trunk (PT) with predominantly right to left bidirectional shunting from color Doppler (C, Online Video 1) and contrast echocardiogram (D, Online Video 2).
Magnetic resonance confirmed a large aortopulmonary (AP) window (E, short axis, Online Video 3). The defect was 4.5 cm in axial diameter (F, Online Video 4) and longitudinal lengths of 6 cm (coronal views, G, H). Axial contrast-enhanced computed tomography also delineated the confluence of great vessels (I, Online Video 5) without other cardiac anomalies. Cardiac catheterization disclosed irreversible severe pulmonary hypertension. The patient was on a heart-lung transplantation waiting list.
AP window is a very rare congenital aortic septation. Early diagnosis followed by timely repair is crucial. To our knowledge, this is the largest AP window size in an untreated adult.
- Received May 1, 2013.
- Accepted May 7, 2013.
- American College of Cardiology Foundation