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

Ahmed K. Mahmoud; Mohammed Tiseer Abbas; Moaz A. Kamel; Juan M. Farina; Milagros Pereyra; Isabel G. Scalia; Timothy Barry; Chieh Ju Chao; Francois Marcotte; Chadi Ayoub; Robert L. Scott; David S. Majdalany; Reza Arsanjani

doi:10.3390/jpm14010005

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

Ahmed K. Mahmoud, Mohammed Tiseer Abbas, Moaz A. Kamel, Juan M. Farina, Milagros Pereyra, Isabel G. Scalia, Timothy Barry, Chieh Ju Chao, Francois Marcotte, Chadi Ayoub, Robert L. Scott, David S. Majdalany, Reza Arsanjani

Cardiovascular Diseases

Research output: Contribution to journal › Review article › peer-review

Abstract

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.

Original language	English (US)
Article number	5
Journal	Journal of Personalized Medicine
Volume	14
Issue number	1
DOIs	https://doi.org/10.3390/jpm14010005
State	Published - Jan 2024

Keywords

bosentan
congenital heart disease
epoprostenol
macitentan
ongoing trials
pulmonary hypertension
selexipag
sildenafil
sotatercept

ASJC Scopus subject areas

Medicine (miscellaneous)

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10.3390/jpm14010005

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Mahmoud, A. K., Abbas, M. T., Kamel, M. A., Farina, J. M., Pereyra, M., Scalia, I. G., Barry, T., Chao, C. J., Marcotte, F., Ayoub, C., Scott, R. L., Majdalany, D. S., & Arsanjani, R. (2024). Current Management and Future Directions for Pulmonary Arterial Hypertension Associated with Congenital Heart Disease. Journal of Personalized Medicine, 14(1), Article 5. https://doi.org/10.3390/jpm14010005

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title = "Current Management and Future Directions for Pulmonary Arterial Hypertension Associated with Congenital Heart Disease",

abstract = "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.",

keywords = "bosentan, congenital heart disease, epoprostenol, macitentan, ongoing trials, pulmonary hypertension, selexipag, sildenafil, sotatercept",

author = "Mahmoud, {Ahmed K.} and Abbas, {Mohammed Tiseer} and Kamel, {Moaz A.} and Farina, {Juan M.} and Milagros Pereyra and Scalia, {Isabel G.} and Timothy Barry and Chao, {Chieh Ju} and Francois Marcotte and Chadi Ayoub and Scott, {Robert L.} and Majdalany, {David S.} and Reza Arsanjani",

note = "Publisher Copyright: {\textcopyright} 2023 by the authors.",

year = "2024",

month = jan,

doi = "10.3390/jpm14010005",

language = "English (US)",

volume = "14",

journal = "Journal of Personalized Medicine",

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T1 - Current Management and Future Directions for Pulmonary Arterial Hypertension Associated with Congenital Heart Disease

AU - Mahmoud, Ahmed K.

AU - Abbas, Mohammed Tiseer

AU - Kamel, Moaz A.

AU - Farina, Juan M.

AU - Pereyra, Milagros

AU - Scalia, Isabel G.

AU - Barry, Timothy

AU - Chao, Chieh Ju

AU - Marcotte, Francois

AU - Ayoub, Chadi

AU - Scott, Robert L.

AU - Majdalany, David S.

AU - Arsanjani, Reza

PY - 2024/1

Y1 - 2024/1

N2 - 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.

AB - 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.

KW - bosentan

KW - congenital heart disease

KW - epoprostenol

KW - macitentan

KW - ongoing trials

KW - pulmonary hypertension

KW - selexipag

KW - sildenafil

KW - sotatercept

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DO - 10.3390/jpm14010005

M3 - Review article

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JO - Journal of Personalized Medicine

JF - Journal of Personalized Medicine

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ER -

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

Abstract

Keywords

ASJC Scopus subject areas

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