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. 2019 Jan-Mar;9(1):2045894019834890.
doi: 10.1177/2045894019834890.

Pathophysiology, incidence, management, and consequences of cardiac arrhythmia in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension

Meghan M Cirulis  1   2 , John J Ryan  2 , Stephen L Archer  3
Affiliations

Pathophysiology, incidence, management, and consequences of cardiac arrhythmia in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension

Meghan M Cirulis et al. Pulm Circ. 2019 Jan-Mar.

Abstract

Arrhythmias are increasingly recognized as serious, end-stage complications of pre-capillary pulmonary hypertension, including pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH). Although arrhythmias contribute to symptoms, morbidity, in-hospital mortality, and possibly sudden death in PAH/CTEPH, there remains a paucity of epidemiologic, pathophysiologic, and outcome data to guide management of these patients. This review summarizes the most current evidence on the topic: from the molecular mechanisms driving arrhythmia in the hypertrophied or failing right heart, to the clinical aspects of epidemiology, diagnosis, and management.

Keywords: atrial fibrillation; atrial flutter; prognosis; right heart failure; sudden death; supraventricular atrial arrhythmia; survival.

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Figures

Fig. 1.
Fig. 1.
Overall frequency and subtypes of SVA (by study) in PAH/CTEPH. Subtypes: atrial fibrillation, atrial flutter, or other atrial tachycardia. SVA, supraventricular arrhythmia.
Fig. 2.
Fig. 2.
Comparative survival of PAH patients with and without SVA. Kaplan–Meier survival analyses from two prospective studies, examining SVA in PAH. In both studies, permanent SVA was associated with increased mortality compared to transient SVA and never SVA. Included with permission.
Fig. 3.
Fig. 3.
Schematic illustration outlining the development and consequence of arrhythmia in PH. CRT, cardiac resynchronization therapy; ICD, implanted cardiac defibrillator; VT, ventricular tachycardia; VF, ventricular fibrillation; fib, fibrillation; 6WMD, 6-min walk distance; WHO FC, World Health Organization functional class; BNP, brain natriuretic peptide; NT-proBNP, N-terminus-pro brain natriuretic peptide.
See this image and copyright information in PMC

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