Pulmonary arterial hypertension (PAH) is commonly associated with congenital heart disease (CHD) and relates to type of the underlying cardiac defects and repair history. Large systemic to pulmonary shunts may develop PAH if untreated or repaired late. PAH, when present, markedly increases morbidity and mortality in patients with CHD. Significant progress has been made for patients with Eisenmenger syndrome in pathophysiology, prognostication and disease-targeting therapy (DTT), which needs to be applied to routine patient care. Patients with PAH–CHD and systemic to pulmonary shunting may benefit from late defect closure if pulmonary vascular resistance (PVR) is still normal or near normal. Patients with PAH and coincidental defects, or previous repair of CHD should be managed as those with idiopathic PAH. Patients with a Fontan circulation, despite not strictly fulfilling criteria for PAH, may have elevated PVR; recent evidence suggests that they may also benefit from DTT, but more data are required before general recommendations can be made. CHD–PAH is a lifelong, progressive disease; patients should receive tertiary care and benefit from a proactive DTT approach. Novel biomarkers and genetic advances may identify patients with CHD who should be referred for late defect closure and/or patients at high risk of developing PAH despite early closure in childhood. Ongoing vigilance for PAH and further controlled studies are clearly warranted in CHD.
- congenital heart disease
- pulmonary vascular disease
- secondary pulmonary hypertension
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Contributors Both authors contributed equally to the planning, conduct and reporting of the work described in the article; MAG assumes responsibility for the overall content as guarantor.
Funding MB is the recipient of the International Training and Research Fellowship EMAH Stiftung Karla VÖLLM, Krefeld, Germany. MAG has received support from the British Heart Foundation, London, UK, and Actelion, Allschwil, Switzerland.
Competing interests MAG is a steering committee member for Actelion Pharmaceuticals and has received unrestricted educational grants from Actelion Pharmaceuticals, Pfizer and GlaxoSmithKline.
Patient consent Not required.
Provenance and peer review Commissioned; externally peer reviewed.