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