Congenital heart block

Development of late-onset cardiomyopathy, a previously underappreciated sequela

Jeffrey P. Moak, Karyl S. Barron, Thomas J. Hougen, Henry B Wiles, Seshadri Balaji, Narayanswami Sreeram, Mark H. Cohen, Aaron Nordenberg, George F. Van Hare, Richard A. Friedman, Maria Perez, Frank Cecchin, Daniel S. Schneider, Rodrigo A. Nehgme, Jill P. Buyon

Research output: Contribution to journalArticle

265 Citations (Scopus)

Abstract

OBJECTIVES: We report 16 infants with complete congenital heart block (CHB) who developed late-onset dilated cardiomyopathy despite early institution of cardiac pacing. BACKGROUND. Isolated CHB has an excellent prognosis following pacemaker implantation. Most early deaths result from delayed initiation of pacing therapy or hemodynamic abnormalities associated with congenital heart defects. METHODS: A multi-institutional study was performed to identify common clinical features and possible risk factors associated with late-onset dilated cardiomyopathy in patients born with congenital CHB. RESULTS: Congenital heart block was diagnosed in utero in 12 patients and at birth in four patients. Ten of 16 patients had serologic findings consistent with neonatal lupus syndrome (NLS). A pericardial effusion was evident on fetal ultrasound in six patients. In utero determination of left ventricular (LV) function was normal in all. Following birth, one infant exhibited a rash consistent with NLS and two had elevated hepatic transaminases and transient thrombocytopenia. In the early postnatal period, LV function was normal in 15 patients (shortening fraction [SF] = 34 ± 7%) and was decreased in one (SF = 20%). A cardiac pacemaker was implanted during the first two weeks of life in 15 patients and at seven months in one patient. Left ventricular function significantly decreased during follow-up (14 days to 9.3 years, SF = 9% ± 5%). Twelve of 16 patients developed congestive heart failure before age 24 months. Myocardial biopsy revealed hypertrophy in 11 patients, interstitial fibrosis in 11 patients, and myocyte degeneration in two patients. Clinical status during follow-up was guarded: four patients died from congestive heart failure; seven required cardiac transplantation; one was awaiting cardiac transplantation; and four exhibited recovery of SF (31 ± 2%). CONCLUSIONS: Despite early institution of cardiac pacing, some infants with CHB develop LV cardiomyopathy. Patients with CHB require close follow-up not only of their cardiac rate and rhythm, but also ventricular function.

Original languageEnglish (US)
Pages (from-to)238-242
Number of pages5
JournalJournal of the American College of Cardiology
Volume37
Issue number1
DOIs
StatePublished - Jan 27 2001
Externally publishedYes

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Cardiomyopathies
Left Ventricular Function
Dilated Cardiomyopathy
Heart Transplantation
Congenital heart block
Heart Failure
Parturition
Ventricular Function
Pericardial Effusion
Congenital Heart Defects
Transaminases
Exanthema
Thrombocytopenia
Muscle Cells
Hypertrophy
Fibrosis
Hemodynamics

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

Cite this

Congenital heart block : Development of late-onset cardiomyopathy, a previously underappreciated sequela. / Moak, Jeffrey P.; Barron, Karyl S.; Hougen, Thomas J.; Wiles, Henry B; Balaji, Seshadri; Sreeram, Narayanswami; Cohen, Mark H.; Nordenberg, Aaron; Van Hare, George F.; Friedman, Richard A.; Perez, Maria; Cecchin, Frank; Schneider, Daniel S.; Nehgme, Rodrigo A.; Buyon, Jill P.

In: Journal of the American College of Cardiology, Vol. 37, No. 1, 27.01.2001, p. 238-242.

Research output: Contribution to journalArticle

Moak, JP, Barron, KS, Hougen, TJ, Wiles, HB, Balaji, S, Sreeram, N, Cohen, MH, Nordenberg, A, Van Hare, GF, Friedman, RA, Perez, M, Cecchin, F, Schneider, DS, Nehgme, RA & Buyon, JP 2001, 'Congenital heart block: Development of late-onset cardiomyopathy, a previously underappreciated sequela', Journal of the American College of Cardiology, vol. 37, no. 1, pp. 238-242. https://doi.org/10.1016/S0735-1097(00)01048-2
Moak, Jeffrey P. ; Barron, Karyl S. ; Hougen, Thomas J. ; Wiles, Henry B ; Balaji, Seshadri ; Sreeram, Narayanswami ; Cohen, Mark H. ; Nordenberg, Aaron ; Van Hare, George F. ; Friedman, Richard A. ; Perez, Maria ; Cecchin, Frank ; Schneider, Daniel S. ; Nehgme, Rodrigo A. ; Buyon, Jill P. / Congenital heart block : Development of late-onset cardiomyopathy, a previously underappreciated sequela. In: Journal of the American College of Cardiology. 2001 ; Vol. 37, No. 1. pp. 238-242.
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abstract = "OBJECTIVES: We report 16 infants with complete congenital heart block (CHB) who developed late-onset dilated cardiomyopathy despite early institution of cardiac pacing. BACKGROUND. Isolated CHB has an excellent prognosis following pacemaker implantation. Most early deaths result from delayed initiation of pacing therapy or hemodynamic abnormalities associated with congenital heart defects. METHODS: A multi-institutional study was performed to identify common clinical features and possible risk factors associated with late-onset dilated cardiomyopathy in patients born with congenital CHB. RESULTS: Congenital heart block was diagnosed in utero in 12 patients and at birth in four patients. Ten of 16 patients had serologic findings consistent with neonatal lupus syndrome (NLS). A pericardial effusion was evident on fetal ultrasound in six patients. In utero determination of left ventricular (LV) function was normal in all. Following birth, one infant exhibited a rash consistent with NLS and two had elevated hepatic transaminases and transient thrombocytopenia. In the early postnatal period, LV function was normal in 15 patients (shortening fraction [SF] = 34 ± 7{\%}) and was decreased in one (SF = 20{\%}). A cardiac pacemaker was implanted during the first two weeks of life in 15 patients and at seven months in one patient. Left ventricular function significantly decreased during follow-up (14 days to 9.3 years, SF = 9{\%} ± 5{\%}). Twelve of 16 patients developed congestive heart failure before age 24 months. Myocardial biopsy revealed hypertrophy in 11 patients, interstitial fibrosis in 11 patients, and myocyte degeneration in two patients. Clinical status during follow-up was guarded: four patients died from congestive heart failure; seven required cardiac transplantation; one was awaiting cardiac transplantation; and four exhibited recovery of SF (31 ± 2{\%}). CONCLUSIONS: Despite early institution of cardiac pacing, some infants with CHB develop LV cardiomyopathy. Patients with CHB require close follow-up not only of their cardiac rate and rhythm, but also ventricular function.",
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T2 - Development of late-onset cardiomyopathy, a previously underappreciated sequela

AU - Moak, Jeffrey P.

AU - Barron, Karyl S.

AU - Hougen, Thomas J.

AU - Wiles, Henry B

AU - Balaji, Seshadri

AU - Sreeram, Narayanswami

AU - Cohen, Mark H.

AU - Nordenberg, Aaron

AU - Van Hare, George F.

AU - Friedman, Richard A.

AU - Perez, Maria

AU - Cecchin, Frank

AU - Schneider, Daniel S.

AU - Nehgme, Rodrigo A.

AU - Buyon, Jill P.

PY - 2001/1/27

Y1 - 2001/1/27

N2 - OBJECTIVES: We report 16 infants with complete congenital heart block (CHB) who developed late-onset dilated cardiomyopathy despite early institution of cardiac pacing. BACKGROUND. Isolated CHB has an excellent prognosis following pacemaker implantation. Most early deaths result from delayed initiation of pacing therapy or hemodynamic abnormalities associated with congenital heart defects. METHODS: A multi-institutional study was performed to identify common clinical features and possible risk factors associated with late-onset dilated cardiomyopathy in patients born with congenital CHB. RESULTS: Congenital heart block was diagnosed in utero in 12 patients and at birth in four patients. Ten of 16 patients had serologic findings consistent with neonatal lupus syndrome (NLS). A pericardial effusion was evident on fetal ultrasound in six patients. In utero determination of left ventricular (LV) function was normal in all. Following birth, one infant exhibited a rash consistent with NLS and two had elevated hepatic transaminases and transient thrombocytopenia. In the early postnatal period, LV function was normal in 15 patients (shortening fraction [SF] = 34 ± 7%) and was decreased in one (SF = 20%). A cardiac pacemaker was implanted during the first two weeks of life in 15 patients and at seven months in one patient. Left ventricular function significantly decreased during follow-up (14 days to 9.3 years, SF = 9% ± 5%). Twelve of 16 patients developed congestive heart failure before age 24 months. Myocardial biopsy revealed hypertrophy in 11 patients, interstitial fibrosis in 11 patients, and myocyte degeneration in two patients. Clinical status during follow-up was guarded: four patients died from congestive heart failure; seven required cardiac transplantation; one was awaiting cardiac transplantation; and four exhibited recovery of SF (31 ± 2%). CONCLUSIONS: Despite early institution of cardiac pacing, some infants with CHB develop LV cardiomyopathy. Patients with CHB require close follow-up not only of their cardiac rate and rhythm, but also ventricular function.

AB - OBJECTIVES: We report 16 infants with complete congenital heart block (CHB) who developed late-onset dilated cardiomyopathy despite early institution of cardiac pacing. BACKGROUND. Isolated CHB has an excellent prognosis following pacemaker implantation. Most early deaths result from delayed initiation of pacing therapy or hemodynamic abnormalities associated with congenital heart defects. METHODS: A multi-institutional study was performed to identify common clinical features and possible risk factors associated with late-onset dilated cardiomyopathy in patients born with congenital CHB. RESULTS: Congenital heart block was diagnosed in utero in 12 patients and at birth in four patients. Ten of 16 patients had serologic findings consistent with neonatal lupus syndrome (NLS). A pericardial effusion was evident on fetal ultrasound in six patients. In utero determination of left ventricular (LV) function was normal in all. Following birth, one infant exhibited a rash consistent with NLS and two had elevated hepatic transaminases and transient thrombocytopenia. In the early postnatal period, LV function was normal in 15 patients (shortening fraction [SF] = 34 ± 7%) and was decreased in one (SF = 20%). A cardiac pacemaker was implanted during the first two weeks of life in 15 patients and at seven months in one patient. Left ventricular function significantly decreased during follow-up (14 days to 9.3 years, SF = 9% ± 5%). Twelve of 16 patients developed congestive heart failure before age 24 months. Myocardial biopsy revealed hypertrophy in 11 patients, interstitial fibrosis in 11 patients, and myocyte degeneration in two patients. Clinical status during follow-up was guarded: four patients died from congestive heart failure; seven required cardiac transplantation; one was awaiting cardiac transplantation; and four exhibited recovery of SF (31 ± 2%). CONCLUSIONS: Despite early institution of cardiac pacing, some infants with CHB develop LV cardiomyopathy. Patients with CHB require close follow-up not only of their cardiac rate and rhythm, but also ventricular function.

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