December 1, 2020Journal of Congenital CardiologyOpen Access

Strategies for the management of pulmonary arterial hypertension in patients with congenital heart disease

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Why the study?

Pulmonary hypertension is commonly seen in adults with congenital heart disease, where PAH therapies improve quality of life and prognosis, warranting a review of management and advanced therapies.

Population

Patients with pulmonary arterial hypertension related to congenital heart disease

Design

Review

Authors

NLNathalie Liew ZRZoya Rashid RTRobert TullohPediatric Cardiology

Discussion

Member takes

Implication

Supports targeted PAH therapies in PAH-CHD; leaves open optimal regimens and long-term data in complex unrepaired cases.

Structured PICO

Population

Adults with congenital heart disease (CHD) who have pulmonary arterial hypertension (PAH-CHD), including Eisenmenger syndrome.

Intervention

Advanced PAH-specific therapies including phosphodiesterase type 5 inhibitors (sildenafil, tadalafil), soluble guanylate cyclase stimulators (riociguat), endothelin receptor antagonists (bosentan, macitentan), and prostacyclin analogues/receptor agonists (epoprostenol, iloprost, treprostinil, selexipag).

Advanced PAH-specific therapies targeting the nitric oxide, endothelin, and prostacyclin pathways offer significant clinical benefits for patients with PAH associated with congenital heart disease.

Cite This Study

Liew et al. (2020) studied this question.

synapsesocial.com/papers/6a7cc9b3b7ca9b673b7cd8eb https://doi.org/10.1186/s40949-020-00052-w

View Full Paper

Ask AI

Bookmark

Strategies for the management of pulmonary arterial hypertension in patients with congenital heart disease

Discussion

Member takes

Implication

Structured PICO

Cite This Study

Strategies for the management of pulmonary arterial hypertension in patients with congenital heart disease

Discussion

Member takes

Implication

Structured PICO

Cite This Study

Also Consider

Also Consider