Current Management and Future Directions for Pulmonary Arterial Hypertension Associated with Congenital Heart Disease

被引：2

作者：

Mahmoud, Ahmed K. ^{[1
]}

Abbas, Mohammed Tiseer ^{[1
]}

Kamel, Moaz A. ^{[1
]}

Farina, Juan M. ^{[1
]}

Pereyra, Milagros ^{[1
]}

Scalia, Isabel G. ^{[1
]}

Barry, Timothy ^{[1
]}

Chao, Chieh-Ju ^{[2
]}

Marcotte, Francois ^{[1
]}

Ayoub, Chadi ^{[1
]}

Scott, Robert L. ^{[1
]}

Majdalany, David S. ^{[1
]}

Arsanjani, Reza ^{[1
]}

机构：

[1] Mayo Clin, Dept Cardiovasc Med, Phoenix, AZ 85054 USA

[2] Mayo Clin, Dept Cardiovasc Med, Rochester, MN 55905 USA

来源：

JOURNAL OF PERSONALIZED MEDICINE | 2024年 / 14卷 / 01期

关键词：

pulmonary hypertension; congenital heart disease; sildenafil; bosentan; macitentan; epoprostenol; selexipag; sotatercept; ongoing trials; SMOOTH-MUSCLE-CELLS; EISENMENGER-SYNDROME; DOUBLE-BLIND; PHOSPHODIESTERASE-5; INHIBITOR; ENDOTHELIAL DYSFUNCTION; BOSENTAN TREATMENT; PROSTACYCLIN; THERAPY; ADULTS; TREPROSTINIL;

D O I：

10.3390/jpm14010005

中图分类号：

R19 [保健组织与事业（卫生事业管理）];

学科分类号：

摘要：

Current management of patients with congenital heart disease has increased their survival into adulthood. This is accompanied by potential cardiac complications, including pulmonary hypertension associated with congenital heart disease (PAH-CHD). PAH-CHD constitutes a challenging subgroup of pulmonary hypertension and requires expert management to improve quality of life and prognosis. Novel agents have shown a significant improvement in morbidity and mortality in patients with pulmonary arterial hypertension. However, the long-term effects of these medications on PAH-CHD patients remain somewhat uncertain, necessitating treatment plans largely founded on the clinical experience of the healthcare providers. The aim of this review is to summarize the current evidence and future perspectives regarding treatment strategies for PAH-CHD to help better guide management of this complex disease.

引用

页数：17

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