Publication

Pulmonary arterial hypertension in adults with congenital heart disease: markers of disease severity, management of advanced heart failure and transplantation

Jansen, Katrijn
; 
Constantine, Andrew
; 
Condliffe, Robin
; 
Tulloh, Robert
; 
Clift, Paul
; 
Moledina, Shahin
; 
Wort, S John
; 
Dimopoulos, Konstantinos
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.
MIDER Authors
Clift, Paul
Citations
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Date
2021-09-20
Type
Article
Subject
Cardiology
 Transplantation
 Vascular diseases
 Respiratory medicine
Collections
UHB Cardiology
Patient Information Leaflets
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Citation
Jansen K, Constantine A, Condliffe R, Tulloh R, Clift P, Moledina S, Wort SJ, Dimopoulos K. Pulmonary arterial hypertension in adults with congenital heart disease: markers of disease severity, management of advanced heart failure and transplantation. Expert Rev Cardiovasc Ther. 2021 Sep;19(9):837-855. doi: 10.1080/14779072.2021.1977124. Epub 2021 Sep 20.
Journal / Source Title
Expert Review of Cardiovascular Therapy
DOI
10.1080/14779072.2021.1977124
URI
http://hdl.handle.net/20.500.14200/6329
PMID
34511015
Publisher
Taylor & Francis
Publisher’s URL
https://www.tandfonline.com/journals/ierk20
Publisher’s statement
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