LAUSR

Background: Supraventricular arrhythmias (SVA) occurrence in pulmonary arterial hypertension (PAH) patients may cause clinical deterioration and affect overall prognosis. Aim: To assess SVA incidence in idiopathic (IPAH) and systemic sclerosis related PAH (SScPAH) and evaluate risk factors, management, and impact on mortality. Methods: We collected baseline demographics, hemodynamics, six minute walking distance, and NTproBNP from consecutive IPAH or SScPAH patients prospectively enrolled in the Johns Hopkins Pulmonary Hypertension Registry from January 2000 to July 2016. During follow-up visits SVA occurrence, therapies and long-term outcome were recorded. Results: Among 317 patients (201 SScPAH, 116 IPAH, mean follow up of 67±51 months), 42 had SVA (34 atrial fibrillation, 18 atrial flutter and 4 atrial ectopic tachycardia). Incidence was slightly higher in SScPAH (13.9 vs 12.1%, p=0.22). In 64.3% of cases at least a second episode occurred. Most SVA were associated with hospitalization (90.5%). Electrical or pharmacological cardioversion was attempted in most cases. Patients with SVA had higher right atrial pressure, pulmonary wedge pressure, NTproBNP (p Conclusion: SVA occurrence in PAH is a matter of concern, acutely leading to clinical deterioration and ultimately impacting long-term prognosis. Therapeutic strategies aimed at preventing SVA recurrence may be a relevant goal.