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Progressive pulmonary hypertension in cyanotic congenital heart disease with severe pulmonary stenosis

Published online by Cambridge University Press:  19 August 2008

Yiu-Fai Cheung ,
Maurice P. Leung  and
E. P. Wang
Yiu-Fai Cheung
Affiliation:
Cardiological Division of the Department of Paediatrics, Grantham Hospital, University of Hong Kong, Hong Kong
Maurice P. Leung*
Affiliation:
Cardiological Division of the Department of Paediatrics, Grantham Hospital, University of Hong Kong, Hong Kong
E. P. Wang
Affiliation:
Department of Pathology, Grantham Hospital, Hong Kong
*
Dr. Maurice P Leung, 5/F, University Paediatrics Unit, Grantham Hospital, 125 Wong Chuk Hang Road, Aberdeen, Hong Kong. Tel. 852-25182629; Fax. 852-25539491.
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Abstract

We report the progressive development of pulmonary hypertension despite the presence of severe pulmonary stenosis in three patients with cyanotic congenital heart disease. The associated intracardiac lesions were complete transposition with a ventricular septal defect, double outlet right ventricle, and a heart with univentricular atrioventricular connection, respectively. Serial measurements on hemodynamics and histological findings documented the progression of pulmonary hypertension and pulmonary vascular diseases. The underlying etiology remains speculative. In view of the possibility of development of pulmonary hypertension despite a ‘protected’ pulmonary vasculature, corrective surgery should be contemplated as soon as technically feasible.

Keywords

Pulmonary hypertensionpulmonary stenosiscyanotic heart disease
Type
Original Manuscripts
Information
Cardiology in the Young , Volume 6 , Issue 3 , July 1996 , pp. 228 - 231
Copyright
Copyright © Cambridge University Press 1996

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