title: Echocardiographic abnormalities and their correlation with bronchiectasis score in primary antibody deficiencies.
abstract: BACKGROUND
  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.
  
  
  METHODS
  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.
  
  
  RESULTS
  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).
  
  
  CONCLUSIONS
  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.
authors.affiliation: Growth and Development Research Center, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran.
authors.forename: Ali Akbar
authors.initials: AA
authors.lastname: Zeinaloo
authors.forename: Asghar
authors.initials: A
authors.lastname: Aghamohammadi
authors.forename: Reza
authors.initials: R
authors.lastname: Shabanian
authors.forename: Ali
authors.initials: A
authors.lastname: Salavati
authors.forename: Sina
authors.initials: S
authors.lastname: Abdollahzade
authors.forename: Nima
authors.initials: N
authors.lastname: Rezaei
authors.forename: Hooman
authors.initials: H
authors.lastname: Alizadeh
authors.forename: Keyhan Sayadpour
authors.initials: KS
authors.lastname: Zanjani
authors.forename: Abdolrazagh
authors.initials: A
authors.lastname: Kiani
mesh_terms: D000293:Adolescent; D000328:Adult; D001987:Bronchiectasis / Q000209:etiology*; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D004452:Echocardiography; D005260:Female; D006331:Heart Diseases / Q000000981:diagnostic imaging / Q000209:etiology*; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000150:complications* / Q000000981:diagnostic imaging; D008171:Lung Diseases / Q000150:complications*; D008297:Male; D008875:Middle Aged; D055815:Young Adult