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Pulmonary Hypertension in Adult Congenital Heart Disease pp 341–361Cite as

Palliative Care and End-of-Life Considerations in Patients with PAH–CHD

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Part of the book series: Congenital Heart Disease in Adolescents and Adults ((CHDAA))

Abstract

Pulmonary arterial hypertension (PAH) related to congenital heart disease (PAH–CHD) in most of its forms is a progressive condition with high morbidity and mortality. Despite huge leaps in managing this condition over the last three decades, there remains no cure, and significant morbidity and mortality persist. Clinical problems encountered include arrhythmias, endocarditis, cerebral abscess, renal dysfunction, restrictive ventilatory defects, obstructive or central sleep apnoea, need for long-term oxygen therapy (LTOT) and osteopathy. Ultimately, these lead to detraining, pain, discomfort, cardiac cachexia and a negative impact on prognosis. In this Chapter, we provide a detailed overview of the palliative and end-of life care needs of patients with PAH-CHD.

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Abbreviations

ACP:

Advance care planning

AICD:

Automatic implantable cardioverter-defibrillator

BNP:

Brain natriuretic peptide

CAMPHOR:

Cambridge Pulmonary Hypertension Outcome Review

CCGs:

Clinical commissioning groups

CHD:

Congenital heart disease

CI:

Cardiac index

CMR:

Cardiac magnetic resonance

CPR:

Cardiopulmonary resuscitation

DNACPR:

Do not attempt cardiopulmonary resuscitation

EP:

Electrophysiology

EpaCCS:

Electronic Palliative Care Coordination System

ESC:

European Society of Cardiology

GMC:

General Medical Council

GP:

General practitioner

HF:

Heart failure

IV:

Intravenous

LASA:

Linear Analogue Self-Assessment

LTOT:

Long-term oxygen therapy

NHS:

National Health System

NSAID:

Non-steroidal anti-inflammatory drug

PAH:

Pulmonary arterial hypertension

PAH-CHD:

Pulmonary arterial hypertension related to congenital heart disease

PH:

Pulmonary hypertension

RA:

Right atrium

RAP:

Right atrial pressure

RCT:

Randomized controlled trial

RV:

Right ventricle

SOB:

Shortness of breath

VCO2 :

Carbon dioxide production

VE:

Ventilation

VO2 :

Oxygen consumption

VT:

Ventricular tachycardia

WHO:

World Health Organization

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Authors and Affiliations

  1. Royal Brompton Hospital & Imperial College, London, UK

    Laura C. Price, Edith Ubogagu, Laura Bernstein, Jenny Wright & Konstantinos Dimopoulos

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  1. Laura C. Price
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  2. Edith Ubogagu
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  3. Laura Bernstein
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  4. Jenny Wright
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  5. Konstantinos Dimopoulos
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Correspondence to Konstantinos Dimopoulos .

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Editors and Affiliations

  1. Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital and Imperial College, London, United Kingdom

    Konstantinos Dimopoulos

  2. University Hospital, Münster, Germany

    Gerhard-Paul Diller

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Price, L.C., Ubogagu, E., Bernstein, L., Wright, J., Dimopoulos, K. (2017). Palliative Care and End-of-Life Considerations in Patients with PAH–CHD. In: Dimopoulos, K., Diller, GP. (eds) Pulmonary Hypertension in Adult Congenital Heart Disease. Congenital Heart Disease in Adolescents and Adults. Springer, Cham. https://doi.org/10.1007/978-3-319-46028-4_23

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  • DOI: https://doi.org/10.1007/978-3-319-46028-4_23

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