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Idiopathic pulmonary arterial hypertension with coexisting CHD

Published online by Cambridge University Press:  10 January 2018

Thomas G. Day*
Affiliation:
Department of Cardiology, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK Department of Adult Congenital Cardiology, Bart’s Heart Centre, Bart’s Health NHS Trust, London, UK
Marina Hughes
Affiliation:
Department of Cardiology, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK
Bejal Pandya
Affiliation:
Department of Adult Congenital Cardiology, Bart’s Heart Centre, Bart’s Health NHS Trust, London, UK
*
Author for correspondence: Thomas G. Day, Department of Cardiology, Great Ormond Street Hospital for Children NHS Foundation Trust, London, United Kingdom. Tel: 07944326254; E-mail: tomgeorgeday@gmail.com

Abstract

Sinus venosus atrial septal defect can result in an increase in pulmonary blood flow and vascular resistance, leading to pulmonary hypertension. Rarely, the degree of pulmonary hypertension is out of proportion to the degree of intra-cardiac shunting. This case outlines the differences between pulmonary hypertension secondary to CHD and idiopathic pulmonary hypertension, and illustrates the investigation and management strategy used in a patient with features of both.

Type
Brief Report
Copyright
© Cambridge University Press 2018 

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Footnotes

Current address: Department of Cardiology, Great Ormond Street Hospital for Children NHS Foundation Trust, London, United Kingdom.

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