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Anomalous origin of the pulmonary artery from the aorta: early diagnosis and repair leading to immediate physiological correction

Published online by Cambridge University Press:  20 August 2010

Gabriel Amir ,
Georgy Frenkel ,
Elhanan Bruckheimer ,
Tamir Dagan ,
Jacob Katz ,
Michael Berant ,
Bernardo Vidne  and
Einat Birk
Gabriel Amir*
Affiliation:
Department of Cardiac Surgery, Division of Pediatric Cardiothoracic Surgery, Schneider Children’s Medical Center of Israel, Petach Tikva, Israel
Georgy Frenkel
Affiliation:
Department of Cardiac Surgery, Division of Pediatric Cardiothoracic Surgery, Schneider Children’s Medical Center of Israel, Petach Tikva, Israel
Elhanan Bruckheimer
Affiliation:
Department of Pediatric Cardiology, Schneider Children’s Medical Center of Israel, Petach Tikva, Israel
Tamir Dagan
Affiliation:
Department of Pediatric Cardiology, Schneider Children’s Medical Center of Israel, Petach Tikva, Israel
Jacob Katz
Affiliation:
Department of Pediatric Anesthesia, Schneider Children’s Medical Center of Israel, Petach Tikva, Israel
Michael Berant
Affiliation:
Department of Pediatric Cardiology, Schneider Children’s Medical Center of Israel, Petach Tikva, Israel
Bernardo Vidne
Affiliation:
Department of Cardiac Surgery, Division of Pediatric Cardiothoracic Surgery, Schneider Children’s Medical Center of Israel, Petach Tikva, Israel
Einat Birk
Affiliation:
Department of Pediatric Cardiology, Schneider Children’s Medical Center of Israel, Petach Tikva, Israel
*
Correspondence to: Dr G. Amir, MD, PhD, Division of Pediatric Cardiac Surgery, Schneider Children’s Medical Center of Israel, 14 Kaplan street, 49100, Petach Tikva, Israel. Tel: 050 405 7199; Fax: 972 3925 3146; E-mail: GabrielA@clalit.org.il
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Abstract

Introduction

Anomalous origin of one pulmonary artery from the ascending aorta is a rare cardiac anomaly in which the pulmonary artery abnormally arises from the ascending aorta. Physiologically, most patients develop signs of cardiac failure due to high flow to both lungs, with systemic or supra-systemic pressures in the normally connected lung. The purpose of this study is to present our experience with this rare anomaly, in which early anatomic repair lead to rapid physiologic correction.

Materials and methods

Retrospective case review of all patients with anomalous origin of one pulmonary artery from the ascending aorta at Schneider Chidren’s Medical center of Israel between 1986 and 2007. All clinical operative and echocardiographic charts were analysed.

Results

Twelve patients were diagnosed as anomalous origin of one pulmonary artery from the ascending aorta. In 10 patients, the right pulmonary artery rose from the ascending aorta, while in two an anomalous origin of the left pulmonary artery was associated with a right aortic arch. Initial diagnoses was made with two-dimensional echocardiography in all patients. In six patients, diagnostic cardiac catheterisation was performed in order to confirm the diagnosis. Age at diagnosis ranged from 5 to 180 days with a median of 15 days, and patient weight ranged from 780 grams to 5 kilograms, with a median of 3 kilograms. Initial echocardiographic evaluation showed systemic (four patients) or supra-systemic (seven patients) pressures in the right ventricle and normally connected lung. All underwent surgical repair. There was no operatrive mortality. All reconstructed patients achieved normal right ventricular pressures within days after surgery. The flow pattern in both pulmonary arteries was normalised.

Conclusions

Early surgical repair of anomalous origin of one pulmonary artery from the ascending aorta is feasible and safe even in newborn and premature babies with complete resolution of the pulmonary hypertension and normalisation of pulmonary vascular resistance.

Keywords

Aortapulmonary arteryanomalous originpulmonary hypertensionphysiologypaediatric
Type
Original Articles
Information
Cardiology in the Young , Volume 20 , Issue 6 , December 2010 , pp. 654 - 659
Copyright
Copyright © Cambridge University Press 2010

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