Congenital heart disease and pulmonary hypertension.
Many patients with congenital heart disease and systemic-to-pulmonary shunts develop pulmonary arterial hypertension (PAH), particularly if the cardiac defect is left unrepaired. A persistent increase in pulmonary blood flow may lead to obstructive arteriopathy and increased pulmonary vascular resistance, a condition that can lead to reversal of shunt and cyanosis (Eisenmenger syndrome). Cardiac catheterization is crucial to confirm diagnosis and facilitate treatment. Bosentan is the only medication to date to be compared with placebo in a randomized controlled trial specifically targeting congenital heart disease-associated PAH. Lung transplantation with repair of the cardiac defect or combined heart-lung transplantation is reserved for recalcitrant cases.
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Related Subject Headings
- Risk Factors
- Pulmonary Embolism
- Oxygen Inhalation Therapy
- Hypertension, Pulmonary
- Humans
- Hemoptysis
- Heart Defects, Congenital
- Exercise Tolerance
- Exercise Test
- Cardiovascular System & Hematology
Citation
Published In
DOI
ISSN
Publication Date
Volume
Issue
Start / End Page
Location
Related Subject Headings
- Risk Factors
- Pulmonary Embolism
- Oxygen Inhalation Therapy
- Hypertension, Pulmonary
- Humans
- Hemoptysis
- Heart Defects, Congenital
- Exercise Tolerance
- Exercise Test
- Cardiovascular System & Hematology