title: Anomalous origin of the pulmonary artery from the aorta: early diagnosis and repair leading to immediate physiological correction.
abstract: BACKGROUND
  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.
  
  
  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: Department of Cardiac Surgery, Schneider Children's Medical Center of Israel, Petach Tikva, Israel. GabrielA@clalit.org.il
authors: Gabriel
authors: G
authors: Amir
authors: Georgy
authors: G
authors: Frenkel
authors: Elhanan
authors: E
authors: Bruckheimer
authors: Tamir
authors: T
authors: Dagan
authors: Jacob
authors: J
authors: Katz
authors: Michael
authors: M
authors: Berant
authors: Bernardo
authors: B
authors: Vidne
authors: Einat
authors: E
authors: Birk
mesh_terms: D000714:Anastomosis, Surgical; D001011:Aorta / Q000002:abnormalities* / Q000601:surgery*; D006328:Cardiac Catheterization; D013504:Cardiovascular Surgical Procedures / Q000379:methods; D003937:Diagnosis, Differential; D004452:Echocardiography; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology; D007223:Infant; D007231:Infant, Newborn; D011651:Pulmonary Artery / Q000002:abnormalities* / Q000601:surgery*