Palliative care in pulmonary hypertension associated with congenital heart disease: systematic review and expert opinion

Andrew Constantine, Robin Condliffe, Paul Clift, Robert Tulloh, Konstantinos Dimopoulos, CHAMPION Steering Committee, Andrew Constantine, Robin Condliffe, Paul Clift, Robert Tulloh, Konstantinos Dimopoulos, CHAMPION Steering Committee

Abstract

Aims: Pulmonary arterial hypertension (PAH) is common amongst patients with congenital heart disease (CHD). It is a severe and complex condition that adversely affects quality of life and prognosis. While quality of life questionnaires are routinely used in clinical pulmonary hypertension practice, little is known on how to interpret their results and manage PAH-CHD patients with evidence of impaired health-related quality of life, especially those with advanced disease and palliative care needs.

Methods and results: We performed a systematic review of studies concerning palliative care for people with PAH-CHD, also reviewing the health-related quality of life literature pertaining to these patients. Of 330 papers identified through initial screening, 17 were selected for inclusion. Underutilization of advance care planning and palliative care resources was common. Where palliative care input was sought, this was frequently late in the course of the disease. No studies provided evidence-based clinical criteria for triggering referral to palliative care, a framework for providing tailored care in this patient group, or how to manage the risk of sudden cardiac death and implantable cardioverter defibrillators in advanced PAH-CHD. We synthesize this information into eight important areas, including the impact of PAH-CHD on quality of life, barriers to and benefits of palliative care involvement, advance care planning discussions, and end-of-life care issues in this complex patient group, and provide expert consensus on best practice in this field.

Conclusions: This paper presents the results of a systematic review and expert statements on the preferred palliative care strategy for patients with PAH-CHD.

Keywords: Advance care planning; Congenital heart defects; End-of-life care; Palliative care; Pulmonary hypertension; Systematic review.

Conflict of interest statement

All authors have been consultants to and have received grant support and personal fees from Janssen‐Cilag Limited during the conduct of the study. Dr Condliffe has received personal fees from Bayer and GlaxoSmithKline. Dr Clift has received personal fees from Bayer. Professor Tulloh has received personal fees from Pfizer, Abbott International, GlaxoSmithKline, and Bayer. Professor Dimopoulos has received grants and personal fees from Pfizer, GlaxoSmithKline, and Bayer/MSD.

© 2021 The Authors. ESC Heart Failure published by John Wiley & Sons Ltd on behalf of European Society of Cardiology.

Figures

Figure 1
Figure 1
The palliative care framework for patients with pulmonary arterial hypertension associated with congenital heart disease (PAH‐CHD) should take into account the unique characteristics and natural history of this condition. PAH‐CHD can be a rapidly progressive disease, affecting both quality of life (QoL) and prognosis. PAH therapies have now become integral to the management of most patients and are often escalated, aiming at a reduction in morbidity and mortality, and improvement in QoL. Despite this, patients can remain highly symptomatic; the early introduction of advance care planning (ACP) and palliative care can help to alleviate the impact of the disease and agree treatment goals with patients. The onset of congestive heart failure (HF) and/or progression of symptoms should further prompt palliative care involvement in parallel to escalation of PAH therapies (if appropriate) and transplant assessment. The palliative care framework and resources for PAH‐CHD patients should reflect the natural history of this disease, integrating components of acquired HF and lung disease care, but accounting for important differences: PAH‐CHD is an often‐aggressive disease with early onset of symptoms, especially in ES patients, and a high prevalence of multiorgan involvement. Moreover, PAH‐CHD patients are younger, with a different impact of the disease on their lives compared with older patients (school/studies/work/sport, etc.).
Figure 2
Figure 2
Barriers to effective palliative care involvement, involving different stakeholders of PAH‐CHD care, and recommendations for adapting care towards successful integration and provision of palliative care. +Misconceptions surrounding palliative care include the belief that palliative care is equivalent to end‐of‐life or hospice care, that is, it equates to ‘giving up’ or ‘losing hope’, is incompatible with active PAH therapy and is exclusively the remit of palliative care specialists. ACP, advanced care planning; EOL, end‐of‐life; PAH‐CHD, pulmonary arterial hypertension associated with congenital heart disease; PC, palliative care.
Figure 3
Figure 3
Multidimensional facets and goals of palliative care involvement. QoL, quality of life.

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