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@article{Jansen:2021:10.1080/14779072.2021.1977124,
author = {Jansen, K and Constantine, A and Condliffe, R and Tulloh, R and Clift, P and Moledina, S and Wort, SJ and Dimopoulos, K},
doi = {10.1080/14779072.2021.1977124},
journal = {Expert Review of Cardiovascular Therapy},
pages = {837--855},
title = {Pulmonary arterial hypertension in adults with congenital heart disease: markers of disease severity, management of advanced heart failure and transplantation},
url = {http://dx.doi.org/10.1080/14779072.2021.1977124},
volume = {19},
year = {2021}
}

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TY  - JOUR
AB  - 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.
AU  - Jansen,K
AU  - Constantine,A
AU  - Condliffe,R
AU  - Tulloh,R
AU  - Clift,P
AU  - Moledina,S
AU  - Wort,SJ
AU  - Dimopoulos,K
DO  - 10.1080/14779072.2021.1977124
EP  - 855
PY  - 2021///
SN  - 1477-9072
SP  - 837
TI  - Pulmonary arterial hypertension in adults with congenital heart disease: markers of disease severity, management of advanced heart failure and transplantation
T2  - Expert Review of Cardiovascular Therapy
UR  - http://dx.doi.org/10.1080/14779072.2021.1977124
UR  - https://www.tandfonline.com/doi/full/10.1080/14779072.2021.1977124
UR  - http://hdl.handle.net/10044/1/90975
VL  - 19
ER  -

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