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Semin Respir Crit Care Med 2009; 30(4): 421-428
DOI: 10.1055/s-0029-1233311
© Thieme Medical Publishers

Pulmonary Arterial Hypertension in Congenital Heart Diseases

Maurice Beghetti1 , Cecile Tissot1
  • 1Pediatric Cardiology Unit, Department of the Child and Adolescent, Children's University Hospital, Geneva, Switzerland
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Publication History

Publication Date:
24 July 2009 (online)

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ABSTRACT

Pulmonary hypertension complicates the course of many children and adults with congenital heart diseases (CHDs). The increase in pulmonary pressure associated with CHD is secondary to either increased pulmonary blood flow or increased postcapillary pressures. Pulmonary arterial hypertension is in the vast majority associated with congenital cardiac shunts. Despite major advances in the understanding of the regulation of the pulmonary vascular bed and the pulmonary endothelial lesions leading to pulmonary vascular disease, despite the advances in surgical repair and the discovery of potential therapies in the pre- and postoperative period, pulmonary hypertension still carries a significant mortality and morbidity in patients with CHD. The recent introduction of targeted therapies in other forms of pulmonary arterial hypertension has led to a renewed interest in pulmonary hypertension associated with CHD and this particularly for the most advanced form, the so-called Eisenmenger syndrome (ES). This review summarizes the current knowledge on pulmonary hypertension associated with CHD, focusing on the pathophysiology and treatment of ES.

KEYWORDS

Hypertension - pulmonary - heart defect - congenital - Eisenmenger syndrome

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Maurice BeghettiM.D. 

Pediatric Cardiology Unit, Department of the Child and Adolescent, Hôpital des Enfants

6 rue Willy Donzé, 1211 Geneva, 14 Switzerland

Email: maurice.beghetti@hcuge.ch

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