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
Jansen, Katrijn
Constantine, Andrew
Condliffe, Robin
Tulloh, Robert
Clift, Paul
Moledina, Shahin
Wort, S John
Dimopoulos, Konstantinos
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Affiliation
Newcastle upon Tyne Hospitals NHS Foundation Trust; Newcastle University; Royal Brompton Hospital; Imperial College London; Royal Hallamshire Hospital; University Hospitals Bristol and Weston NHS Foundation Trust; University Hospitals Birmingham NHS Foundation Trust; Great Ormond Street Hospital for Children NHS Foundation Trust; University College London
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Publication date
2021-09-20
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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.
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.
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Article