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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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References

1. Fraentzel, O. Ein Fall Von Abnormer communication Der Aorta mit der Arteria Pulmonalis. Arch Pathol Anatom 1868; 43: 420–426.CrossRefGoogle Scholar
2. Prifti, E, Bonacchi, M, Murzi, B, et al. . Anomalous origin of the right pulmonary artery from the ascending aorta. J Cardiac Surg 2004; 19: 103–112.CrossRefGoogle ScholarPubMed
3. Pool, PE, Vogel, JH, Blount, SG Jr. Congenital unilateral absence of a pulmonary artery. The importance of flow in pulmonary hypertension. Am J cardiol 1962; 10: 706–732.CrossRefGoogle ScholarPubMed
4. Amer, RM, Shumaker, HB, Klatte, EC. Origin of the right pulmonary artery from the ascending aorta. Primary surgical repair in infancy. Circulation 1961; 24: 662–668.CrossRefGoogle Scholar
5. Kutsche, LM, Van Mierop, LH. Anomalous origin of a pulmonary artery from the ascending aorta: associated anomalies and pathogenesis. Am J Cardiol 1988; 61: 850–856.CrossRefGoogle ScholarPubMed
6. Abu-Sulaiman, RM, Hashmi, A, McCrindle, BW, Williams, WG, Freedom, RM. Anomalous origin of one pulmonary artery from the ascending aorta: 36 years’ experience from one centre. Cardiol Young 1998; 8: 449–454.CrossRefGoogle ScholarPubMed
7. Nathan, M, Rimmer, D, Piercey, G, et al. . Early repair of hemitruncus: excellent early and late outcomes. J Thorac Cardiovasc Surg 2007; 133: 1329–1335.CrossRefGoogle ScholarPubMed
8. Peng, EW, Shanmugam, G, Macarthur, KJ, Pollock, JC. Ascending aortic origin of a branch pulmonary artery – surgical management and long-term outcome. Eur J Cardiothorac Surg 2004; 26: 762–766.CrossRefGoogle ScholarPubMed
9. Fontana, GP, Spach, MS, Effmann, EL, Sabiston, DC Jr. Origin of the right pulmonary artery from the ascending aorta. Ann Surg 1987; 206: 102–113.CrossRefGoogle ScholarPubMed
10. Trapali, CJ, Thanopoulos, BD. Severe right ventricular dysfunction in a neonate with aortic origin of the RPA. Pediatr Cardiol 1998; 19: 425–427.CrossRefGoogle Scholar
11. Pool, PE, Averill, KH, Vogel, JH. Effect of ligation of left pulmonary artery at birth on maturation of pulmonary vascular bed. Med Thorac 1962; 19: 362–369.Google ScholarPubMed
12. Ayed, AK, Owayed, A. Pulmonary resection in infants for congenital pulmonary malformation. Chest 2003; 124: 98–101.CrossRefGoogle ScholarPubMed
13. Schwartz, MZ, Ramachandran, P. Congenital malformations of the lung and mediastinum – a quarter century of experience from a single institution. J Pediatr Surg 1997; 32: 44–47.CrossRefGoogle ScholarPubMed
14. Foroulis, CN, Kotoulas, CS, Kakouros, S, et al. . Study on the late effect of pneumonectomy on right heart pressures using Doppler echocardiography. Eur J Cardiothorac Surg 2004; 26: 508–514.CrossRefGoogle Scholar
15. Aslam, M, Christou, HA. Intrauterine closure of the ductus arteriosus: implications for the neonatologist. Am J Perinatol 2009; 26: 473–478.CrossRefGoogle ScholarPubMed
16. Downing, GJ, Thibeault, DW. Pulmonary vasculature changes associated with idiopathic closure of the ductus arteriosus and hydrops fetalis. Pediatr Cardiol 1994; 15: 71–75.CrossRefGoogle ScholarPubMed