Pulmonary arterial hypertension in adults with congenital heart disease: distinct differences from other causes of pulmonary arterial hypertension and management implications.
OBJECTIVE
To present the available data on pathophysiology, clinical presentation, prognosis, and especially management strategies for adult patients with congenital heart disease (CHD) and pulmonary arterial hypertension (PAH). Particular emphasis is placed on differences between other types of PAH and CHD-related PAH, in which clinical presentation and management relate to a constellation of factors, both pulmonary and cardiac.


RESULTS
Pulmonary vascular disease in adults with CHD and especially its extreme expression, the Eisenmenger syndrome, is a chronic disease with slow progression, leading to multiorgan failure and death, decades after its first clinical presentation. In the last few years, oral advanced therapies for PAH have emerged and are considered for mono or combination therapy for CHD, though the evidence is limited. Supportive care and prevention of complications seem to be at least as important in the overall care of these patients.


CONCLUSIONS
Although new advanced therapies hold promise in PAH secondary to CHD, long-term data are clearly needed. Advanced therapies should be considered when other causes of functional limitation, such as iron deficiency, have been first addressed. Expertise in CHD as well as PAH is essential for providing adequate care for this patient group with a unique pathophysiology.
Dimopoulos
Konstantinos
K
Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital and the National Heart and Lung Institute, Imperial College, London, UK.
Giannakoulas
Georgios
G
Wort
Stephen John
SJ
Gatzoulis
Michael A
MA
D000328:Adult; D000959:Antihypertensive Agents / Q000627:therapeutic use*; D001794:Blood Pressure; D000077300:Bosentan; D006330:Heart Defects, Congenital / Q000150:complications* / Q000503:physiopathology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology / Q000473:pathology / Q000503:physiopathology; D011379:Prognosis; D011651:Pulmonary Artery / Q000473:pathology*; D013449:Sulfonamides / Q000627:therapeutic use
D000959:Antihypertensive Agents; D013449:Sulfonamides; D000077300:Bosentan