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Anomalous origin of one pulmonary artery from the ascending aorta: 36 years' experience from one centre

Published online by Cambridge University Press:  19 August 2008

Riyadh M. Abu-Sulaiman ,
Aijaz Hashmi ,
Brian W. McCrindle ,
William G. Williams  and
Robert M. Freedom
Riyadh M. Abu-Sulaiman
Affiliation:
Department of Pediatrics, Division of Cardiology, University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada
Aijaz Hashmi
Affiliation:
Department of Pediatrics, Division of Cardiology, University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada
Brian W. McCrindle*
Affiliation:
Department of Pediatrics, Division of Cardiology, University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada
William G. Williams
Affiliation:
Department of Pediatrics, Division of Cardiology, University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada
Robert M. Freedom
Affiliation:
Department of Pediatrics, Division of Cardiology, University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada
*
Brian W McCrindle, MD, The Hospital for Sick Children, 555 University Avenue, Toronto, Ontario, Canada M5G 1X8. Tel: (416) 813-7609; Fax:(416) 813-7547
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Abstract

Anomalous origin of one pulmonary artery from the ascending aorta is an uncommon lesion with an uncertain outcome. We reviewed 16 consecutive children (9 males) presenting with this lesion over a 36 year period at a single institution. Median age at presentation was 2 days (range, birth to 3.2 years). The anomalous pulmonary was the right in 12 and the left in 4, all originating from the proximal ascending aorta, with no patient having stenosis at the origin of the anomalous pulmonary artery. Associated cardiac anomalies were noted in 9 patients. No intervention was attempted in 2 patients: one was deemed inoperable due to complex associated lesions and pulmonary vascular obstructive disease, while the other one died before repair. Surgical intervention was attempted in 14 patients, with 3 intraoperative deaths (21%). Of 11 operative survivors, 8 developed pulmonary arterial stenosis graded severe in 2, moderate in 1 and mild in 5. Patients with severe stenosis required surgical angioplasty, 1 after unsuccessful dilation combined with placement of an endovascular stent. One patient with moderate, and one with mild, stenosis underwent successful transcatheter balloon dilation. The remaining 4 patients with mild stenosis remain unchanged during follow-up. One patient had biopsy evidence of pulmonary vascular obstructive disease at 3.3 years of age. There were no late deaths, giving a total mortality of 25% (4/16).

Conclusion

While early diagnosis and repair of anomalous origin of one pulmonary artery from the ascending aorta is necessary, restenosis of the site of repair is common.

Keywords

Anomalous pulmonary arterypulmonary artery stenosis
Type
Original Articles
Information
Cardiology in the Young , Volume 8 , Issue 4 , October 1998 , pp. 449 - 454
Copyright
Copyright © Cambridge University Press 1998

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