Abstract
Pulmonary arterial hypertension (PAH) is a devastating, life-limiting disease driven by small vessel vascular remodeling leading to a rise in pulmonary vascular resistance (PVR). Patients present with a range of symptoms including shortness of breath, exercise intolerance, palpitations or syncope. Symptoms may be related to vascular disease progression or arrhythmia secondary to the adaptation of the right heart to pressure overload. Arrhythmic burden is high in patients with left heart disease and guideline-based treatment of arrhythmias improves quality of life and prognosis. In PAH the incidence and prevalence of arrhythmias is less well-defined and there are no PAH-specific guidelines for arrhythmia management. We undertook a literature search identifying 13 relevant papers; detection of arrhythmias was acquired from 12-lead electrocardiogram (ECG) or Holter monitors. In all forms of pulmonary hypertension (PH) the prevalence of supraventricular arrhythmias (SVA) was 26–31%, ventricular arrhythmias (VA) 24% and a 5-year incidence of SVA ~13.2–25.1%. Prevalence and incidence of arrhythmias in PAH is less clear due to limited study numbers and the heterogenous nature of the patient population studied. For arrhythmia treatment, only single-arm studies of therapeutic strategies were reported using antiarrhythmic drugs (AAD), direct current cardioversion (DCCV) and ablation. Periods between ECG or Holter have not been investigated, highlighting the possibility that significant arrhythmias may be undetected. Advances in monitoring allow long-term surveillance via implanted/non-invasive monitors. Use of such technologies may provide an accurate estimate of incidence and prevalence of arrhythmias in patients with PAH, further defining relationships to adverse outcomes, and therapeutic options.
Highlights
The recent World Symposium classifies pulmonary hypertension into five groups based on underlying cause (1)
Five-year survival in Pulmonary arterial hypertension (PAH)/inoperable CTEPH was 68% with a fall to 58% if the patient developed a transient supraventricular arrhythmias (SVA) and reduced further to 47% in permanent SVA. They found that atrial fibrillation (AF) was more resistant to treatment than atrial flutter. Consistent with these findings, Ruiz-Cano et al (16) found that after the first episode of SVA 46.4% patients required an increase in PAH-specific medications secondary to progressive right heart failure (RHF), an interesting observation but not guideline directed
It highlighted that the use of Holter monitoring in this cohort was beneficial for arrhythmia diagnosis and this may be a useful tool in PAH patients
Summary
The recent World Symposium classifies pulmonary hypertension into five groups based on underlying cause (1). Olsson et al (20) diagnosed patients with SVA on 12-lead ECG and largely from patients presenting with deterioration of PAH/RHF symptoms rather than symptoms of arrhythmia [as per Cannillo et al (18)] Those with atrial flutter received ablation earlier with the concurrent use of AAD’s with or without synchronized DCCV. They found that AF was more resistant to treatment than atrial flutter (similar to non-PAH disease) Consistent with these findings, Ruiz-Cano et al (16) found that after the first episode of SVA (despite restoration of NSR/adequate rate control) 46.4% patients required an increase in PAH-specific medications secondary to progressive RHF, an interesting observation but not guideline directed. As in left heart disease NSR was easier to restore in atrial flutter vs. AF and all Frontiers in Medicine | www.frontiersin.org
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