Pulmonary arterial hypertension in paediatric and adult patients with congenital heart disease
Open Access
- 31 August 2009
- journal article
- review article
- Published by European Respiratory Society (ERS) in European Respiratory Review
- Vol. 18 (113), 154-161
- https://doi.org/10.1183/09059180.00003309
Abstract
Pulmonary arterial hypertension (PAH) commonly arises in patients with congenital heart disease (CHD). Greater numbers of patients with PAH associated with CHD (PAH-CHD) are now surviving into adulthood, many with increasingly complex cardiac defects. Patients with cardiac defects which result in left-to-right shunting are at risk of developing PAH, owing to the increased shear stress and circumferential stretch induced by increased pulmonary blood flow, which leads to endothelial dysfunction and progressive vascular remodelling and, thus, increased pulmonary vascular resistance. Although PAH-CHD shares similar lung histology with idiopathic PAH, differences do exist between these aetiologies. Management of PAH-CHD can involve surgical correction of the cardiac defect and/or treatment of the PAH, depending on the underlying cardiac defect and status of disease progression. Transplantation surgery can be curative but is not without limitations. The timing of intervention in patients with PAH-CHD is important, but the optimums are sometimes difficult to define, with limited robust data to inform management decisions. Uncontrolled studies suggest that prostacyclin analogues and phosphodiesterase type-5 inhibitors may have benefits in advanced pulmonary vascular disease. In the only randomised controlled trial dedicated to end-stage PAH-CHD, bosentan significantly reduced pulmonary vascular resistance and significantly increased 6-minute walk distance without compromising peripheral oxygen saturation, in patients with Eisenmenger syndrome. These data suggest that targeted therapies are beneficial in the PAH-CHD population, and warrant further research.Keywords
This publication has 42 references indexed in Scilit:
- Consensus statement on the management of pulmonary hypertension in clinical practice in the UK and IrelandThorax, 2008
- Management of Pulmonary Arterial Hypertension Associated with Congenital Systemic-to-Pulmonary Shunts and Eisenmenger???s SyndromeDrugs, 2008
- Effects of chronic sildenafil in patients with Eisenmenger syndrome versus idiopathic pulmonary arterial hypertensionInternational Journal of Cardiology, 2007
- Pulmonary arterial hypertension in adults born with a heart septal defect: the Euro Heart Survey on adult congenital heart diseaseHeart, 2007
- Epoprostenol treatment in children with severe pulmonary hypertensionHeart, 2007
- Pulmonary arterial hypertension in congenital heart disease: An epidemiologic perspective from a Dutch registryInternational Journal of Cardiology, 2006
- Adult patients with Eisenmenger syndrome report flying safely on commercial airlinesHeart, 2006
- Erwachsene Patienten mit angeborenen Herzfehlern: Gegenwart und ZukunftDeutsche Medizinische Wochenschrift (1946), 2005
- Clinical classification of pulmonary hypertensionJournal of the American College of Cardiology, 2004
- The Pathology of Hypertensive Pulmonary Vascular DiseaseCirculation, 1958