Echocardiographic changes related to pulmonary hypertension in humoral inmunodeficiencies

Introduction Primary humoral immunodeficiencies leads to recurring infections primarily in mucosal surfaces like the respiratory tract, often causing bronchiectasis and lung issues. This study aimed to gauge the occurrence of pulmonary hypertension-related echocardiographic changes in humoral immunodeficiency patients. Materials and Methods: This observational, prospective, cross-sectional, and descriptive study was conducted on a series of patients with humoral immunodeficiencies belonging to the primary immunodeficiencies clinic of National Medical Center. Transthoracic echocardiography was performed to detect changes related to pulmonary hypertension, in order to identify the frequency of pulmonary hypertension in these patients. The obtained data were compared with clinical variables to establish a relationship between primary humoral immunodeficiencies and pulmonary hypertension. Results: The retrospective and prospective observational study included a total of 28 patients with humoral immunodeficiencies. Females: 16 (57.1%) Males: 12 (42.9%). The median age of the patients was 32.5 years [20-92 years]. Diagnoses included Common Variable Immunodeficiency (CVID) 22 (78.6%), ALX: 3 patients (10.7%), Selective IgG2 Deficiency: 1 (3.6%), Hyper IgM Syndrome: 1 (3.6%), and Non-Severe Combined Immunodeficiency: 1 (3.6%). Comorbidities were observed, with various clinical conditions noted, the most common being the presence of bronchiectasis (n:22 [78.6%]). Sinopulmonary complications were notable, followed by gastrointestinal, autoimmune, and dermatological complications. Findings in order of frequency included Decreased velocity time integral in the right ventricular outflow tract (VTI RVOT) (15, 53.6%), mean Pulmonary Arterial Pressure (mPAP) >25mmHg (10, 34.5%), Increased Tei Index (>0.32) (9, 31%), Tricuspid regurgitant velocity (TRV) (>2.2m/s) (, 27.6%), Pulmonary Arterial Hypertension (PAH) (Systolic Arterial Pressure >35 mmHg) (5, 17.2%), among other findings. Additionally, comparative analyses were conducted which did not show significant associations between PAH and comorbidities. Conclusion: This study highlights the prevalence of pulmonary arterial hypertension and its implications in patients with humoral immunodeficiencies. The findings support the importance of constant cardiac monitoring for the management.

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