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Anomalous origin of the pulmonary artery from the aorta: early diagnosis and repair leading to immediate physiological correction.
MedLine Citation:
PMID:  20723270     Owner:  NLM     Status:  In-Process    
Abstract/OtherAbstract:
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 Children'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 operative 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.
Authors:
Gabriel Amir; Georgy Frenkel; Elhanan Bruckheimer; Tamir Dagan; Jacob Katz; Michael Berant; Bernardo Vidne; Einat Birk
Publication Detail:
Type:  Journal Article     Date:  2010-08-20
Journal Detail:
Title:  Cardiology in the young     Volume:  20     ISSN:  1467-1107     ISO Abbreviation:  Cardiol Young     Publication Date:  2010 Dec 
Date Detail:
Created Date:  2010-10-26     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9200019     Medline TA:  Cardiol Young     Country:  England    
Other Details:
Languages:  eng     Pagination:  654-9     Citation Subset:  IM    
Affiliation:
Department of Cardiac Surgery, Schneider Children's Medical Center of Israel, Petach Tikva, Israel. GabrielA@clalit.org.il
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