Determinants of Sudden Cardiac Death in Adult Patients With Eisenmenger Syndrome

Chiriac, Anca; Russell, Matthew; Moore, Jeremy P.; Vargas, Emily; Hodge, David O.; McLeod, Christopher J.

doi:10.1016/s0735-1097(19)31177-5

Cited by 1 publication

(2 citation statements)

References 19 publications

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“…Previous studies identifying predictors of mortality in PAH‐CHD have not focused on sudden arrhythmic events 55–57 . A recent retrospective study of patients with Eisenmenger syndrome has identified predictors of sudden cardiac death in this subgroup, but identifying patients in the wider PAH‐CHD population who may benefit from automated implantable cardioverter defibrillator (ICD) implantation as primary prevention remains less than straightforward 58 . Moreover, the decision to proceed to ICD implantation, even in secondary prevention, needs to be individualized, to account for age, functional status, life expectancy, and the expected mode of death, that is, arrhythmic sudden cardiac death versus progressive ventricular dysfunction or respiratory failure.…”

Section: Resultsmentioning

confidence: 99%

“…[55][56][57] A recent retrospective study of patients with Eisenmenger syndrome has identified predictors of sudden cardiac death in this subgroup, but identifying patients in the wider PAH-CHD population who may benefit from automated implantable cardioverter defibrillator (ICD) implantation as primary prevention remains less than straightforward. 58 Moreover, the decision to proceed to ICD implantation, even in secondary prevention, needs to be individualized, to account for age, functional status, life expectancy, and the expected mode of death, that is, arrhythmic sudden cardiac death versus progressive ventricular dysfunction or respiratory failure. Finally, ICD implantation can be associated with complications, including a high risk of lead-related complications of up to 25% over 10 years, infective endocarditis, inappropriate shocks, and the need for anticoagulation in patients with large intracardiac A legally binding document regarding a decision to refuse a named treatment (including life-sustaining treatment) in a specific circumstance in the future.…”

Section: Systematic Reviewmentioning

confidence: 99%

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

et al. 2021

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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.

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Section: Resultsmentioning

confidence: 99%