Anomalous branch of pulmonary artery from the aorta and tetralogy of Fallot: morphology, surgical techniques and results.

Objectives: Tetralogy of Fallot (TOF) with hemitruncus (HT) is a rare entity. In this report, we present our experience with this condition over the last 20 years.
Methods: Between January 1994 and June 2013, 11 patients with HT and TOF underwent surgery at the All India Institute of Medical Sciences, New Delhi, India. All available clinical, radiographic, echocardiographic, cardiac catheterization, operative and follow-up data were reviewed.
Results: The mean age was 73 ± 7.1 months (range 7 months to 18 years) and the mean weight was 15.7 ± 1.2 kg. The mean preoperative saturation was 79.3 ± 11.7% (range 62-92%). Six patients had anomalous left pulmonary artery (PA), whereas 5 had an anomalous right PA arising from the aorta. Surgical procedures consisted of complete intracardiac repair of TOF with direct implantation of the anomalous PA into the main PA (n = 7), intracardiac repair of TOF with an interposition saphenous vein graft between the right PA and main PA (n = 1), and reconstruction of the left PA with autologous pericardium with intracardiac repair of TOF (n = 1), direct implantation of the anomalous PA into the main PA with an innominate to right pulmonary artery shunt (n = 1) and a right PA banding with innominate to left PA shunt (n = 1). There were two early deaths. Follow-up ranged from 3 to 73 months. All survivors are in NYHA Class I and follow-up echocardiograms did not show any residual lesions.
Conclusions: Surgical repair of HT with TOF results in acceptable early outcomes. The surgical strategy needs to be individualized to the anatomy of the patient.
(© The Author 2014. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)