期刊
CURRENT OPINION IN CARDIOLOGY
卷 23, 期 6, 页码 545-554出版社
LIPPINCOTT WILLIAMS & WILKINS
DOI: 10.1097/HCO.0b013e3283126954
关键词
advanced therapies; congenital heart disease; pulmonary hypertension
资金
- European Society of Cardiology
- Hellenic Cardiological Society
- Propondis' Foundation
- Cardiological Society of Northern Greece
- Clinical Research Committee
- Royal Brompton Hospital
- London and the British Heart Foundation
Purpose of review To present the available data on pathophysiology, clinical presentation, prognosis, and especially management strategies for adult patients with congenital heart disease (CHID) 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. Recent findings Pulmonary vascular disease in adults with CHID 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. Summary 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.
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