Cardiac tamponade caused by effusive-constrictive pericarditis in a patient with common variable immunodeficiency: a...

Common variable immunodeficiency (CVID) is an immunodeficiency syndrome that is marked by impaired antibody formation and chronic infections. However, it is also associated with immune dysregulation and non-infectious inflammatory disorders. Cardiac involvement is not common, but effusive-constrictive pericarditis (ECP) complicated by cardiac tamponade is rarely seen in CVID patients.

A 17-year-old male patient with CVID (diagnosis made in 2022) under regular replacement therapy with monthly intravenous immunoglobulin (IVIG) was admitted due to progressive dyspnea, dry cough, weakness, fatigue, and severe hypoxemia lasting for several days. Previous medical history included frequent lower respiratory tract infections, inflammatory bowel disease, celiac disease, malnutrition, and psoas abscess. Admission examination revealed arterial blood pressure 90/60 mmHg, oxygen saturation 75% in room air, increased jugular venous pressure, dullness in auscultation, pulsus paradoxus, bilateral rales and peripheral edema. Echo showed a 12-15 mm thick pericardial effusion, mobile fibrin-rich mass in pericardium, dilatation of inferior vena cava (23 mm with < 50% collapse), respiratory variation of mitral and tricuspid inflow with septal bounce. Left ventricle ejection fraction was 40%. Pericardiocentesis was done in an emergency manner and 320 ml of turbid serosanguineous effusion with fibrinous clots was drained. Microbiologic investigations of the sample including bacteriological cultures, acid-fast bacilli, mycobacteria, and MTB/RIF showed negative results. There were no malignant cells in cytological and cell-block analysis. After pericardiocentesis, central venous pressure remained elevated and Doppler investigation showed signs of constrictive physiology such as more than 25% variation in mitral inflow, expiratory reverse flow in hepatic veins, plethoric IVC, and septal bounce, which helped to confirm ECP. Ventricular function and constrictive physiology gradually improved after supportive therapy and IVIG and nutrition treatment without pericardiectomy.

ECP with tamponade is a rare life-threatening complication in CVID patients. In the case of unexplained cardiopulmonary failure in an immunocompromised patient, tamponade cannot be considered only due to the infection. Urgent echocardiography, drainage, and investigation of various infectious, tuberculous, malignant and inflammatory causes is crucial.