title: Isolated congenital complete heart block in a five-year-old seronegative girl born to a woman seropositive for human immunodeficiency virus: a case report.
abstract: BACKGROUND
  Congenital complete heart block is a life-threatening condition which is highly associated with autoimmune and connective tissue disorders. Presence of maternal autoantibodies for associated conditions increases the risk of delivering a child with congenital complete heart block, however, less than a half of all women with such antibodies are symptomatic even after delivery. Mortality rate is highest during the neonatal period (45 %) and about two-thirds of all cases will require permanent pacing at some point in their lives.
  
  
  METHODS
  We report a case of isolated complete heart block in a 5-year-old HIV-free girl of African descent born to an HIV-infected woman with no prior history of autoimmune disorders. She was referred to us with chief complaints of recurrent syncopal attacks and effort intolerance since birth. A physical examination was unremarkable except for her being small for her age (body mass index 16.3 kg/m2) and bradycardia. Her vital signs were within acceptable range with the exception of her pulse rate, which ranged between 22 and 34 beats/minute. An echocardiogram revealed a sinus bradycardia, otherwise a structurally normal heart. An electrocardiogram showed atrioventricular dissociation in keeping with third-degree atrioventricular block. The child underwent a permanent epicardial pacemaker insertion and has been symptom-free following pacing.
  
  
  CONCLUSIONS
  Despite its infrequency and life-threatening potential, patients with congenital complete heart block have an excellent survival rate with timely diagnosis and intervention. An incidental detection of bradycardia in a fetus during routine obstetrical ultrasound examination should increase the index of suspicion for congenital complete heart block and warrant a screening for associated maternal autoantibodies.
authors.affiliation: Department of Cardiovascular Medicine, The Jakaya Kikwete Cardiac Institute, P.O Box 65141, Dar es Salaam, Tanzania. pedro.pallangyo@gmail.com.
authors.forename: Pedro
authors.initials: P
authors.lastname: Pallangyo
authors.affiliation: Department of Cardiovascular Medicine, The Jakaya Kikwete Cardiac Institute, P.O Box 65141, Dar es Salaam, Tanzania.
authors.forename: Isaac
authors.initials: I
authors.lastname: Mawenya
authors.affiliation: Department of Cardiovascular Medicine, The Jakaya Kikwete Cardiac Institute, P.O Box 65141, Dar es Salaam, Tanzania.
authors.forename: Paulina
authors.initials: P
authors.lastname: Nicholaus
authors.affiliation: Department of Cardiovascular Medicine, The Jakaya Kikwete Cardiac Institute, P.O Box 65141, Dar es Salaam, Tanzania.
authors.forename: Henry
authors.initials: H
authors.lastname: Mayala
authors.affiliation: Department of Pediatric and Child Health, Muhimbili National Hospital, P.O Box 65000, Dar es Salaam, Tanzania.
authors.forename: Amida
authors.initials: A
authors.lastname: Kalombola
authors.affiliation: Department of Pediatric Cardiology, The Jakaya Kikwete Cardiac Institute, P.O Box 65141, Dar es Salaam, Tanzania.
authors.forename: Godwin
authors.initials: G
authors.lastname: Sharau
authors.affiliation: Department of Pediatric Cardiology, The Jakaya Kikwete Cardiac Institute, P.O Box 65141, Dar es Salaam, Tanzania.
authors.forename: Naiz
authors.initials: N
authors.lastname: Majani
authors.affiliation: Department of Cardiovascular Medicine, The Jakaya Kikwete Cardiac Institute, P.O Box 65141, Dar es Salaam, Tanzania.
authors.forename: Mohamed
authors.initials: M
authors.lastname: Janabi
mesh_terms: D054537:Atrioventricular Block / Q000151:congenital / Q000175:diagnosis / Q000628:therapy; D001919:Bradycardia / Q000209:etiology; D002675:Child, Preschool; D004562:Electrocardiography; D005260:Female; D018023:HIV Seronegativity; D006679:HIV Seropositivity; D006327:Heart Block / Q000151:congenital / Q000175:diagnosis* / Q000628:therapy; D006801:Humans; D010138:Pacemaker, Artificial
keywords: Case report; Congenital complete heart block; Congenital third-degree AV block; Isolated congenital heart block; Recurrent syncopal attacks; Symptomatic bradycardia