Pulmonary arterial hypertension in adults with congenital heart disease: markers of disease severity, management of advanced heart failure and transplantation
File(s)
Accepted version
Author(s)
Type
Journal Article
Abstract
Introduction:
Pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) is a progressive, life-limiting disease.
Areas covered:
In this paper, we review the classification and pathophysiology of PAH-CHD, including the mechanisms of disease progression and multisystem effects of disease. We evaluate current strategies of risk stratification and the use of biological markers of disease severity, and review principles of management of PAH-CHD. The indications, timing and the content of advanced heart failure assessment and transplant listing are discussed, along with a review of the types of transplant and other forms of available circulatory support in this group of patients. Finally, the integral role of advance care planning and palliative care is discussed.
Expert opinion/commentary:
All patients with PAH-CHD should be followed up in expert centers, where they can receive appropriate risk assessment, PAH therapy and supportive care. Referral for transplant assessment should be considered if there continue to be clinical high-risk features, persistent symptoms or acute heart failure decompensation despite appropriate PAH specific therapy. Expert management of PAH-CHD patients, therefore, requires vigilance for these features, along with a close relationship with local advanced heart failure services and a working knowledge of listing criteria, which may disadvantage congenital heart disease patients.
Pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) is a progressive, life-limiting disease.
Areas covered:
In this paper, we review the classification and pathophysiology of PAH-CHD, including the mechanisms of disease progression and multisystem effects of disease. We evaluate current strategies of risk stratification and the use of biological markers of disease severity, and review principles of management of PAH-CHD. The indications, timing and the content of advanced heart failure assessment and transplant listing are discussed, along with a review of the types of transplant and other forms of available circulatory support in this group of patients. Finally, the integral role of advance care planning and palliative care is discussed.
Expert opinion/commentary:
All patients with PAH-CHD should be followed up in expert centers, where they can receive appropriate risk assessment, PAH therapy and supportive care. Referral for transplant assessment should be considered if there continue to be clinical high-risk features, persistent symptoms or acute heart failure decompensation despite appropriate PAH specific therapy. Expert management of PAH-CHD patients, therefore, requires vigilance for these features, along with a close relationship with local advanced heart failure services and a working knowledge of listing criteria, which may disadvantage congenital heart disease patients.
Date Issued
2021-09-20
Date Acceptance
2021-08-03
Citation
Expert Review of Cardiovascular Therapy, 2021, 19 (9), pp.837-855
ISSN
1477-9072
Publisher
Taylor and Francis
Start Page
837
End Page
855
Journal / Book Title
Expert Review of Cardiovascular Therapy
Volume
19
Issue
9
Copyright Statement
© 2021 Taylor & Francis. This is an Accepted Manuscript of an article published by Taylor & Francis in Expert Review of Cardiovascular Therapy on 20 Sep 2021, available online: https://doi.org/10.1080/14779072.2021.1977124
Identifier
https://www.tandfonline.com/doi/full/10.1080/14779072.2021.1977124
Subjects
Science & Technology
Life Sciences & Biomedicine
Cardiac & Cardiovascular Systems
Cardiovascular System & Cardiology
Pulmonary arterial hypertension
pulmonary vascular disease
congenital heart disease
eisenmenger syndrome
advanced heart failure
transplantation
heart-lung transplant
ventricular assist device
extra-corporeal membrane oxygenation
VENTRICULAR ASSIST DEVICE
QUALITY-OF-LIFE
EXTRACORPOREAL MEMBRANE-OXYGENATION
SINGLE-LUNG TRANSPLANTATION
EISENMENGER-SYNDROME
INTERNATIONAL SOCIETY
COMBINATION THERAPY
PREGNANCY OUTCOMES
EXERCISE CAPACITY
INHALED ILOPROST
Pulmonary arterial hypertension
advanced heart failure
congenital heart disease
eisenmenger syndrome
extra-corporeal membrane oxygenation
heart-lung transplant
pulmonary vascular disease
transplantation
ventricular assist device
Adult
Biomarkers
Heart Defects, Congenital
Heart Failure
Humans
Hypertension, Pulmonary
Pulmonary Arterial Hypertension
Severity of Illness Index
1102 Cardiorespiratory Medicine and Haematology
1117 Public Health and Health Services
Cardiovascular System & Hematology
Publication Status
Published
Date Publish Online
2021-09-20