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

Abstract

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.