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Echocardiographic abnormalities in primary antibody deficiency

¹ Department of Immunology and Immunogenetics, Southmead Hospital, Bristol, UK
² Department of Cardiology, Southmead Hospital, Bristol, UK
³ Department of Cardiology, John Radcliffe Hospital, Oxford, UK

Correspondence to:
Correspondence to:
Dr Sarah L Johnston
Department of Immunology and Immunogenetics, Southmead Hospital, Westbury-on-Trym, Bristol BS10 5NB, UK; sljoh{at}hotmail.com

Objective: To document cardiac abnormalities secondary to pulmonary disease in primary antibody deficiency.

Patients and methods: A cross sectional audit study of patients from a regional immunology centre. Subjects undergoing two dimensional and Doppler transthoracic echocardiography were reviewed. Ventricular dimensions and function, valvular competence, and estimated pulmonary artery pressure were recorded. Data were compared with clinical variables, pulmonary function tests, and thoracic computed tomography data.

Results: Nineteen patients with common variable immunodeficiency and one with IgG₂ subclass deficiency were included, mean age at diagnosis 37.5 years, mean estimated diagnostic delay 10.94 years. Left ventricular impairment was found in 15% and right heart dilatation in 20%. Pulmonary hypertension (mean pulmonary artery pressure >25 mm Hg) was found in 45% (9/20), graded as moderate (40–60 mm Hg) in 44% of cases. Pulmonary function was obstructive in 47% (9/19). Fifty five percent of the patients with computed tomography data within the last five years (10/18) had confirmed bronchiectasis. Patients with right heart dilatation and/or moderate pulmonary hypertension (n = 6) had a more prolonged diagnostic delay (p = 0.04) and more severe lung disease.

Conclusion: Echocardiographic abnormalities are common in primary antibody deficiency, associated with diagnostic delay and pulmonary complications. Pulmonary hypertension should be considered in those with severe lung disease and can be confirmed by echocardiography.

Keywords: bronchiectasis; common variable immunodeficiency; echocardiography; primary antibody deficiency; pulmonary hypertension

Abbreviations: CVID, common variable immunodeficiency; FEV₁/FVC, forced expiratory volume in one second/forced vital capacity