Table 1 Pulmonary arterial hypertension associated with congenital heart disease (CHD)
Type of defectExample
Left-to-right shuntsAtrial septal defect
Ventricular septal defect
Atrioventricular defects
Patent ductus arteriosus
Left-sided obstructionMitral stenosis
Aortic stenosis
Cyanotic CHD (occurring with increased pulmonary blood flow)Common arterial trunk
Transposition of great vessels
Anomalies of pulmonary arteriesTetralogy of Fallot with pulmonary atresia and MAPCAs
Hemitruncus

  • MAPCA: major aortopulmonary collateral artery. Modified from [8] with permission from the publisher.