Management of patients with pulmonary arterial hypertension due to congenital heart disease: recent advances and future directions
Pulmonary arterial hypertension is a serious complication of adult congenital heart disease associated with systemic-to-pulmonary shunts. Although early shunt closure restricts development of pulmonary arterial hypertension, patients remain at risk even after repair. The development of pulmonary art...
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Published in | Expert review of cardiovascular therapy Vol. 13; no. 12; p. 1377 |
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Main Authors | , , , |
Format | Journal Article |
Language | English |
Published |
England
02.12.2015
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Subjects | |
Online Access | Get more information |
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Summary: | Pulmonary arterial hypertension is a serious complication of adult congenital heart disease associated with systemic-to-pulmonary shunts. Although early shunt closure restricts development of pulmonary arterial hypertension, patients remain at risk even after repair. The development of pulmonary arterial hypertension is associated with a markedly increased morbidity and mortality. It is important to identify patients with a poor prognosis using disease specific markers. Echocardiography and biomarkers arise as practical tools to determine the risk of mortality. Although pulmonary arterial hypertension cannot be cured, four classes of disease-targeting therapies are currently available and several promising therapies are being studied. There is a shift in drug studies towards more clinically relevant endpoints such as time to clinical worsening and morbidity and mortality events. |
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ISSN: | 1744-8344 |
DOI: | 10.1586/14779072.2015.1101341 |