Arrhythmia Associated With Higher Mortality in Congenital Heart Disease-Related PAH

Arrhythmia was a significant predictor of mortality in adult patients with congenital heart disease and pulmonary arterial hypertension.

The presence of arrhythmia in patients with pulmonary arterial hypertension (PAH) and congenital heart disease (CHD) is associated with worse long-term outcomes, according to a study published in Heart.

In this retrospective study, investigators evaluated outcomes data of patients with PAH-CHD who attended a tertiary center between January 2007 and December 2015 (n=310; mean age, 34.9±12.3 years). Investigators followed patients for new-onset atrial or ventricular arrhythmia, as well as supraventricular arrhythmias, during a median follow-up period of 6.1 years. A supraventricular arrhythmia included atrial fibrillation and supraventricular tachycardia (eg, atrial ectopic tachycardia, atrial flutter, atrioventricular re-entrant tachycardia, and atrioventricular nodal re-entrant tachycardia).

The presence of arrhythmia was identified using follow-up electrocardiograms (ECGs) as well as ECGs during hospital stay, ambulatory ECG recordings, implantable loop recordings, and/or device interrogation.

At baseline analysis, the investigators noted a prior history of arrhythmia in 14.2% of patients (n=44). In the majority of these patients (86.4%), supraventricular arrhythmia was found, followed by atrial fibrillation (36.4%) and supraventricular tachycardia (50%). During the median follow-up period, a total of 64 patients had developed ≥1 new arrhythmic episode, which represented an incidence rate of 3.47% per year in patients with PAH-CHD.

The majority of the new arrhythmic episodes were attributable to supraventricular tachycardia or atrial fibrillation (78.1%). Overall, arrhythmia was a significant predictor of mortality after adjustment for various demographic and clinical variables (hazard ratio, 3.41; 95% CI, 2.10-5.53; P <.0001).

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A limitation of this analysis included its retrospective design, which may have resulted in the underestimation of arrhythmia incidence in this patient population.

The investigators suggested that “the occurrence of an arrhythmia may prompt physicians to consider uptitration and optimisation of PAH therapy” when pursuing a goal-oriented therapeutic strategy.


Drakopoulou M, Nashat H, Kempny A, et al. Arrhythmias in adult patients with congenital heart disease and pulmonary arterial hypertension [published online May 18, 2018]. Heart. doi:10.1136/heartjnl-2017-312881