Primary antibody deficiencies are characterized by defective antibody production and recurrent infections. Patients usually present with recurrent respiratory tract infections with consequent chronic pulmonary damage and bronchiectasis, which could potentially influence cardiac function. Our aim was to assess noninvasively the cardiac complications due to pulmonary disease in patients with primary antibody deficiency.
A cross-sectional series of patients with primary antibody deficiency syndromes from our referral immunology center were recruited. Individuals undergoing high-resolution computed tomography (HRCT) and transthoracic echocardiography were reviewed.
Thirty primary immunodeficient patients aged 5–55 years of age (21 males and 9 females) were enrolled in this study. Half of the patients (50%) were found to have bronchiectasis in HRCT imaging. In echocardiographic examination, 20 patients (67%) had at least one abnormality; among which pulmonary hypertension was the most common (33%). Patients with bronchiectasis had higher pulmonary artery pressures and HRCT bronchiectasis score was strongly correlated with pulmonary artery pressure (regression R = 0.59, P value = 0.001).
Echocardiographic evaluation of right ventricular function and noninvasive estimation of pulmonary artery pressure could have an important diagnostic role in the follow-up and therapeutic management of patients with primary immune deficiency.
aGrowth and Development Research Center, Iran
bDepartment of Pediatrics, Pediatrics Center of Excellence, Iran
cDepartment of Pediatric Cardiology, Iran
dDepartment of Pediatric Radiology, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran
Received 28 June, 2009
Revised 4 October, 2009
Accepted 22 October, 2009
Correspondence to Asghar Aghamohammadi, MD, Children's Medical Center, 62 Qarib Street, Keshavarz Blvd., Tehran 14194, Iran Tel: +98 21 6694 9662; fax: +98 21 6692 3054; e-mail: email@example.com