A New Perspective on the Management of Primary Immunodeficiencies: Evaluation of Arrhythmia and Cardiac Diseases.

INTRODUCTION

Primary immunodeficiency diseases (PIDs) are a growing group of rarely seen diseases. Various clinical conditions like autoimmunity, lymphoproliferative/malignant diseases, chronic lung, and gastrointestinal system diseases have been identified which accompanies PIDs besides recurrent infections. However, there is a lack of information about accompanying cardiovascular diseases. We aimed to determine the frequency of cardiovascular diseases and arrhythmias in PID patients.

METHODS

Forty-eight PID patients and 48 control group patients were included to this single-center, prospective controlled study. All patients underwent resting electrocardiogram, echocardiogram and 7-lead 24-h ambulatory electrocardiogram (Holter) monitoring assessed by an experienced cardiologist.

RESULTS

Both supraventricular and ventricular extrasystoles were found to be statistically significantly higher in patient group in terms of frequency and sustained, non-sustained, and runs compared to control group. The median of total supraventricular extrasystoles was 8 (0-65) in patient group which was 0.5 (0-4.5) in control group (p < 0.001) while the median of total ventricular extrasystoles was 2 (0-45.5) and 0 (0-2) in two groups, respectively (p = 0.022). Eighteen patients (37.5%) had supraventricular and/or ventricular arrhythmias. The patient group had a statistically significantly higher systolic pulmonary artery pressure value compared to control group (20 [16-28] vs. 17.5 [15-25]; p = 0.036). We found 7 patients had 13 structural heart diseases including second degree or above valve pathologies in patient group whereas none of the control group patients had these diseases (p = 0.013).

CONCLUSION

With the positive findings of higher frequency and risk of arrhythmias and various structural heart diseases, we hope that our study will provide a new perspective on the management of PID patients, contributing positively to their survival and early prevention of cardiovascular comorbidities.