Atrial flutter and fibrillation in patients with pulmonary hypertension

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Abstract

Background

Atrial flutter and fibrillation are being increasingly reported in patients with pulmonary hypertension but little is known about their clinical implications. We sought to determine the incidence and clinical impact of these arrhythmias in patients with pulmonary hypertension.

Methods

In a 5-year, prospective study, we assessed the incidence of new-onset atrial flutter and fibrillation as well as risk factors, clinical consequences, management, and impact on survival in patients with pulmonary arterial hypertension (PAH, n = 157) or inoperable chronic thromboembolic pulmonary hypertension (CTEPH, n = 82).

Results

The cumulative 5-year incidence of new-onset atrial flutter and fibrillation was 25.1% (95% confidence interval, 13.8–35.4%). The development of these arrhythmias was frequently accompanied by clinical worsening (80%) and signs of right heart failure (30%). Stable sinus rhythm was successfully re-established in 21/24 (88%) of patients initially presenting with atrial flutter and in 16/24 (67%) of patients initially presenting with atrial fibrillation. New-onset atrial flutter and fibrillation were an independent risk factor of death (p = 0.04, simple Cox regression analysis) with a higher mortality in patients with persistent atrial fibrillation when compared to patients in whom sinus rhythm was restored (estimated survival at 1, 2 and 3 years 64%, 55%, and 27% versus 97%, 80%, and 57%, respectively; p = 0.01, log rank analysis).

Conclusions

Atrial flutter and fibrillation develop in a sizable number of patients with PAH or inoperable CTEPH and often lead to clinical deterioration and right heart failure. Mortality is high when sinus rhythm cannot be restored.

Introduction

Pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) are diseases characterized by an obliterative pulmonary vasculopathy and a progressive increase in pulmonary vascular resistance eventually leading to right heart failure and death, if not effectively treated [1], [2]. Despite therapeutic advances, PAH remains an incurable disease. The same is true for CTEPH, at least for those patients who cannot undergo pulmonary endarterectomy surgery [3], [4]. With the introduction of active medical therapies, however, patients with PAH and inoperable CTEPH now have a better life expectancy than in earlier years [5], [6], [7], but most of them live with chronic right ventricular dysfunction.

The right ventricle has a variable capacity to adapt to an increased afterload but progressive pulmonary vasculopathy invariably results in cardiac remodeling. Elevated right-sided filling pressures and structural changes, primarily dilatation of the right atrium and right ventricle with subsequent tricuspid regurgitation may trigger atrial fibrillation and atrial flutter [8], [9], [10]. Case reports and observational studies suggest that these arrhythmias may have profound implications including a high risk of clinical deterioration, right heart failure, and death [8], [10], [11]. To the best of our knowledge, the incidence and clinical consequences of atrial flutter and fibrillation in patients with pulmonary hypertension have not been systematically evaluated and no studies have assessed the management of these arrhythmias in this patient population.

In order to further evaluate the incidence and clinical impact of new-onset atrial flutter and fibrillation in patients with chronic pulmonary hypertension, we conducted a prospective study of patients with PAH or inoperable CTEPH focusing on incidence, risk factors, clinical consequences, treatment patterns and survival.

Section snippets

Methods

This study enrolled all consecutive patients ≥ 18 years treated for PAH or inoperable CTEPH between January 2005 and December 2010. The follow-up was completed June 30th, 2011. To be eligible, patients had to have a complete diagnostic work-up according to contemporary standards [12], [13] including right heart catheterization demonstrating precapillary pulmonary hypertension with a mean pulmonary artery pressure (PAPm)  25 mm Hg and a pulmonary arterial occlusion pressure (PAOP)  15 mm Hg. For

Results

A total of 239 patients were enrolled; 157 with PAH and 82 with inoperable CTEPH. Forty-eight (20%) patients had at least one episode of atrial flutter or atrial fibrillation. Both types of arrhythmia were equally common (24 episodes each). Of the 24 cases of atrial flutter, 9 (37.5%) had a typical and 15 (62.5%) an atypical ECG pattern. Table 1 shows the baseline characteristics of the overall patient population and a comparison of the baseline characteristics of patients who did and did not

Discussion

The main findings of the present study are (i) the relatively high incidence of new-onset atrial flutter or fibrillation in patients with advanced pulmonary hypertension; (ii) the fact that the majority of these episodes were associated with marked clinical deterioration and signs of right heart failure; (iii) the observation that sinus rhythm could be successfully restored in the majority of patients resulting in clinical recovery; and (iv) the finding that the development of permanent atrial

Author contributions

Dr. Olsson: contributed to study design, collection, analysis and interpretation of data, drafting and critical review of the manuscript; and has seen and approved the final version.

Dr. Nickel: contributed to analysis and interpretation of data, critical review of the manuscript; and has seen and approved the final version.

Dr. Tongers: contributed to analysis and interpretation of data, critical review of the manuscript; and has seen and approved the final version.

Dr. Hoeper: contributed to

Disclosures

KMO has received speaker fees from Actelion and Pfizer; MMH has received fees for consultations and/or speaking at conferences from Actelion, Bayer, Gilead, GSK, Lilly, Novartis and Pfizer. The other authors have no conflicts of interest.

Funding

None.

Acknowledgement

The authors of this manuscript have certified that they comply with the principles of Ethical Publishing in the International Journal of Cardiology.

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    The first two authors contributed equally to this work.

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