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Dilated cardiomyopathy in isolated congenital complete atrioventricular block: early and long-term risk in children.

Publication ,  Journal Article
Udink ten Cate, FE; Breur, JM; Cohen, MI; Boramanand, N; Kapusta, L; Crosson, JE; Brenner, JI; Lubbers, LJ; Friedman, AH; Vetter, VL; Meijboom, EJ
Published in: J Am Coll Cardiol
March 15, 2001

OBJECTIVES: We sought to identify the risk factors predicting the development of dilated cardiomyopathy (DCM) in patients with isolated congenital complete atrioventricular block (CCAVB). BACKGROUND: Recently evidence has emerged that a subset of patients with CCAVB develop DCM. METHODS: This was a retrospective study of 149 patients with CCAVB who had heart size and left ventricular (LV) function assessed by echocardiography and chest radiography over a follow-up period of 10 +/- 7 years. RESULTS: Nine patients developed DCM at the age of 6.5 +/- 5 years. No definite cause could be identified. In these nine patients, CCAVB was diagnosed in eight at 23 +/- 2.3 weeks gestation and in one at birth. Maternal SSA/SSB antibodies were confirmed in seven of the nine patients. Pacemakers were implanted in eight patients in the first month and in one patient at five years of age. The initial left ventricular end-diastolic dimension (LVEDD) was in the 96th +/- 2.6 percentile and the cardiothoracic (CT) ratio was 64 +/- 3.8% in the nine patients who developed DCM, and differed significantly in patients with CCAVB (p < 0.005) who did not develop DCM. The LVEDD and CT ratio did not decrease in the patients with CCAVB and DCM, but decreased significantly in the patients with CCAVB without DCM (p < 0.001) once pacing was initiated. Two patients with DCM died within two months of diagnosis; one patient is neurologically compromised; two patients received a heart transplant; and four patients are listed for heart transplantation. CONCLUSIONS: Isolated CCAVB is associated with a long-term risk for the development of DCM. Risk factors may be SSA/SSB antibodies, increased heart size at initial evaluation and the absence of pacemaker-associated improvement.

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Published In

J Am Coll Cardiol

DOI

ISSN

0735-1097

Publication Date

March 15, 2001

Volume

37

Issue

4

Start / End Page

1129 / 1134

Location

United States

Related Subject Headings

  • Ventricular Function, Left
  • Risk Factors
  • Retrospective Studies
  • Radiography, Thoracic
  • Prognosis
  • Pacemaker, Artificial
  • Male
  • Infant
  • Humans
  • Heart Block
 

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Udink ten Cate, F. E., Breur, J. M., Cohen, M. I., Boramanand, N., Kapusta, L., Crosson, J. E., … Meijboom, E. J. (2001). Dilated cardiomyopathy in isolated congenital complete atrioventricular block: early and long-term risk in children. J Am Coll Cardiol, 37(4), 1129–1134. https://doi.org/10.1016/s0735-1097(00)01209-2
Udink ten Cate, F. E., J. M. Breur, M. I. Cohen, N. Boramanand, L. Kapusta, J. E. Crosson, J. I. Brenner, et al. “Dilated cardiomyopathy in isolated congenital complete atrioventricular block: early and long-term risk in children.J Am Coll Cardiol 37, no. 4 (March 15, 2001): 1129–34. https://doi.org/10.1016/s0735-1097(00)01209-2.
Udink ten Cate FE, Breur JM, Cohen MI, Boramanand N, Kapusta L, Crosson JE, et al. Dilated cardiomyopathy in isolated congenital complete atrioventricular block: early and long-term risk in children. J Am Coll Cardiol. 2001 Mar 15;37(4):1129–34.
Udink ten Cate, F. E., et al. “Dilated cardiomyopathy in isolated congenital complete atrioventricular block: early and long-term risk in children.J Am Coll Cardiol, vol. 37, no. 4, Mar. 2001, pp. 1129–34. Pubmed, doi:10.1016/s0735-1097(00)01209-2.
Udink ten Cate FE, Breur JM, Cohen MI, Boramanand N, Kapusta L, Crosson JE, Brenner JI, Lubbers LJ, Friedman AH, Vetter VL, Meijboom EJ. Dilated cardiomyopathy in isolated congenital complete atrioventricular block: early and long-term risk in children. J Am Coll Cardiol. 2001 Mar 15;37(4):1129–1134.
Journal cover image

Published In

J Am Coll Cardiol

DOI

ISSN

0735-1097

Publication Date

March 15, 2001

Volume

37

Issue

4

Start / End Page

1129 / 1134

Location

United States

Related Subject Headings

  • Ventricular Function, Left
  • Risk Factors
  • Retrospective Studies
  • Radiography, Thoracic
  • Prognosis
  • Pacemaker, Artificial
  • Male
  • Infant
  • Humans
  • Heart Block