CLINICAL STUDY: PEDIATRIC CARDIOLOGY
Congenital heart block: development of late-onset cardiomyopathy, a previously underappreciated sequela
Jeffrey P. Moak, MD, FACC*,
Karyl S. Barron, MD ,
Thomas J. Hougen, MD, FACC ,
Henry B. Wiles, MD, FACC ,
Seshadri Balaji, MRCP, FACC,
Narayanswami Sreeram, MRCP, FACC||,
Mark H. Cohen, MD, FACC¶,
Aaron Nordenberg, MD, FAAC#,
George F. Van Hare, MD, FACC**,
Richard A. Friedman, MD, FACC,
Maria Perez, MD,
Frank Cecchin, MD,
Daniel S. Schneider, MD, FACC||||,
Rodrigo A. Nehgme, MD, FACC¶¶ and
Jill P. Buyon, MD##
* Department of Cardiology, Children’s National Medical Center, Washington, D.C., USA
National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, Maryland, USA
Division of Pediatric Cardiology, Georgetown University Hospital, Washington, D.C., USA
The Children’s Heart Center of South Carolina, Medical College of South Carolina, Charleston, South Carolina, USA
|| Wilhelmina Children’s Hospital, Utrecht, Netherlands
¶ Pediatric Cardiology, Geisinger Medical Center, Danville, Pennsylvania, USA
# Pediatric Cardiology, Polyclinic Medical Center, Harrisburg, Pennsylvania, USA
** Division of Pediatric Cardiology, Rainbow Babies and Children’s Hospital, Cleveland, Ohio, USA
Division of Pediatric Cardiology, Texas Children’s Hospital, Houston, Texas, USA
Division of Pediatric Rheumatology, Texas Children’s Hospital, Houston, Texas, USA
Children’s Heart Center, Children’s Hospital and Medical Center, Seattle, Washington, USA
|||| Division of Pediatric Cardiology, Children’s Hospital of the King’s Daughters, Norfolk, Virginia, USA
¶¶ Division of Pediatric Cardiology, Yale-New Haven Medical Center, New Haven, Connecticut, USA
## Department of Rheumatology, Hospital for Joint Diseases, New York, New York, USA
Manuscript received November 3, 1998;
revised manuscript received July 24, 2000,
accepted September 13, 2000.
Reprint requests and correspondence: Dr. Jeffrey P. Moak, Department of Cardiology, Children’s National Medical Center, 111 Michigan Avenue, NW, Washington, D.C. 20010
jmoak{at}CNMC.org
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.
|
Abbreviations and Acronyms
| | AV | = atrioventricular | | CHB | = congenital heart block | | NLS | = neonatal lupus syndrome | | SF | = shortening fraction |
|
This article has been cited by other articles:
|
|
|
|
 
C. S. Chronister
Congenital Complete Atrioventricular Block in a Young Man: A Case Study
Crit. Care Nurse,
October 1, 2009;
29(5):
45 - 56.
[Full Text]
[PDF]
|
|
|
|
|
|
|
J Janousek, R A Gebauer, H Abdul-Khaliq, M Turner, L Kornyei, O Grollmuss, E Rosenthal, E Villain, A Fruh, T Paul, et al.
Cardiac resynchronisation therapy in paediatric and congenital heart disease: differential effects in various anatomical and functional substrates
Heart,
July 15, 2009;
95(14):
1165 - 1171.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
L. M. Lopes, M. A. B. Lopes, M. Zugaib, A. P. Damiano, G. M. P. Tavares, V. D. Aiello, and R. Schultz
Response to Letters Regarding Article, "Perinatal Outcome of Fetal Atrioventricular Block: One-Hundred Sixteen Cases From a Single Institution"
Circulation,
May 26, 2009;
119(20):
e541 - e542.
[Full Text]
[PDF]
|
|
|
|
|
|
|
R. A. Gebauer, V. Tomek, A. Salameh, J. Marek, V. Chaloupecky, R. Gebauer, T. Matejka, P. Vojtovic, and J. Janousek
Predictors of left ventricular remodelling and failure in right ventricular pacing in the young
Eur. Heart J.,
May 1, 2009;
30(9):
1097 - 1104.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
M. O. Sweeney and F. W. Prinzen
Ventricular Pump Function and Pacing: Physiological and Clinical Integration
Circ Arrhythm Electrophysiol,
June 1, 2008;
1(2):
127 - 139.
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. E. Epstein, J. P. DiMarco, K. A. Ellenbogen, N.A. M. Estes III, R. A. Freedman, L. S. Gettes, A. M. Gillinov, G. Gregoratos, S. C. Hammill, D. L. Hayes, et al.
ACC/AHA/HRS 2008 Guidelines for Device-Based Therapy of Cardiac Rhythm Abnormalities: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the ACC/AHA/NASPE 2002 Guideline Update for Implantation of Cardiac Pacemakers and Antiarrhythmia Devices) Developed in Collaboration With the American Association for Thoracic Surgery and Society of Thoracic Surgeons
J. Am. Coll. Cardiol.,
May 27, 2008;
51(21):
e1 - e62.
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. E. Epstein, J. P. DiMarco, K. A. Ellenbogen, N.A. Mark Estes III, R. A. Freedman, L. S. Gettes, A. M. Gillinov, G. Gregoratos, S. C. Hammill, D. L. Hayes, et al.
ACC/AHA/HRS 2008 Guidelines for Device-Based Therapy of Cardiac Rhythm Abnormalities: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the ACC/AHA/NASPE 2002 Guideline Update for Implantation of Cardiac Pacemakers and Antiarrhythmia Devices) Developed in Collaboration With the American Association for Thoracic Surgery and Society of Thoracic Surgeons
J. Am. Coll. Cardiol.,
May 27, 2008;
51(21):
2085 - 2105.
[Full Text]
[PDF]
|
|
|
|
|
|
|
Writing Committee Members, A. E. Epstein, J. P. DiMarco, K. A. Ellenbogen, N.A. M. Estes III, R. A. Freedman, L. S. Gettes, A. M. Gillinov, G. Gregoratos, S. C. Hammill, et al.
ACC/AHA/HRS 2008 Guidelines for Device-Based Therapy of Cardiac Rhythm Abnormalities: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the ACC/AHA/NASPE 2002 Guideline Update for Implantation of Cardiac Pacemakers and Antiarrhythmia Devices): Developed in Collaboration With the American Association for Thoracic Surgery and Society of Thoracic Surgeons
Circulation,
May 27, 2008;
117(21):
2820 - 2840.
[Full Text]
[PDF]
|
|
|
|
|
|
|
Writing Committee Members, A. E. Epstein, J. P. DiMarco, K. A. Ellenbogen, N.A. M. Estes III, R. A. Freedman, L. S. Gettes, A. M. Gillinov, G. Gregoratos, S. C. Hammill, et al.
ACC/AHA/HRS 2008 Guidelines for Device-Based Therapy of Cardiac Rhythm Abnormalities: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the ACC/AHA/NASPE 2002 Guideline Update for Implantation of Cardiac Pacemakers and Antiarrhythmia Devices): Developed in Collaboration With the American Association for Thoracic Surgery and Society of Thoracic Surgeons
Circulation,
May 27, 2008;
117(21):
e350 - e408.
[Full Text]
[PDF]
|
|
|
|
|
|
|
J. Frankovich, C. Sandborg, P. Barnes, S. Hintz, and E. Chakravarty
Neonatal Lupus and Related Autoimmune Disorders of Infants
NeoReviews,
May 1, 2008;
9(5):
e206 - e217.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
J.P. Buyon and R.M. Clancy
Dying right to live longer: positing apoptosis as a link between maternal autoantibodies and congenital heart block
Lupus,
February 1, 2008;
17(2):
86 - 90.
[Abstract]
[PDF]
|
|
|
|
|
|
|
D. M. Friedman, M. Y. Kim, J. A. Copel, C. Davis, C. K.L. Phoon, J. S. Glickstein, J. P. Buyon, and for the PRIDE Investigators
Utility of Cardiac Monitoring in Fetuses at Risk for Congenital Heart Block: The PR Interval and Dexamethasone Evaluation (PRIDE) Prospective Study
Circulation,
January 29, 2008;
117(4):
485 - 493.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. M. Kelle, C. L. Backer, S. Tsao, R. D. Stewart, W. H. Franklin, B. J. Deal, and C. Mavroudis
Dual-chamber epicardial pacing in neonates with congenital heart block.
J. Thorac. Cardiovasc. Surg.,
November 1, 2007;
134(5):
1188 - 1192.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
P.A. Gordon
Review: Congenital heart block: clinical features and therapeutic approaches
Lupus,
August 1, 2007;
16(8):
642 - 646.
[Abstract]
[PDF]
|
|
|
|
|
|
|
A. G. Tzioufas and H. M. Moutsopoulos
Predicting autoimmune congenital heart block: is it feasible and how?
Rheumatology,
August 1, 2007;
46(8):
1221 - 1222.
[Full Text]
[PDF]
|
|
|
|
|
|
|
R. Vatasescu, T. Shalganov, D. Paprika, L. Kornyei, Z. Prodan, G. Bodor, A. Szatmari, and T. Szili-Torok
Evolution of left ventricular function in paediatric patients with permanent right ventricular pacing for isolated congenital heart block: a medium term follow-up
Europace,
April 1, 2007;
9(4):
228 - 232.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
E. Villain, N. Coastedoat-Chalumeau, E. Marijon, Y. Boudjemline, J.-C. Piette, and D. Bonnet
Presentation and Prognosis of Complete Atrioventricular Block in Childhood, According to Maternal Antibody Status
J. Am. Coll. Cardiol.,
October 17, 2006;
48(8):
1682 - 1687.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. Tincani, C. B. Rebaioli, M. Taglietti, and Y. Shoenfeld
Heart involvement in systemic lupus erythematosus, anti-phospholipid syndrome and neonatal lupus
Rheumatology,
October 1, 2006;
45(suppl_4):
iv8 - iv13.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
R. G. Williams, G. D. Pearson, R. J. Barst, J. S. Child, P. del Nido, W. M. Gersony, K. S. Kuehl, M. J. Landzberg, M. Myerson, S. R. Neish, et al.
Report of the National Heart, Lung, and Blood Institute Working Group on Research in Adult Congenital Heart Disease
J. Am. Coll. Cardiol.,
February 21, 2006;
47(4):
701 - 707.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
N Costedoat-Chalumeau, S Georgin-Lavialle, Z Amoura, and J-C Piette
Anti-SSA/Ro and anti-SSB/La antibody-mediated congenital heart block
Lupus,
September 1, 2005;
14(9):
660 - 664.
[Abstract]
[PDF]
|
|
|
|
|
|
|
Y Maeno, W Himeno, A Saito, S Hiraishi, O Hirose, M Ikuma, N Inamura, M Kawataki, A Mizukami, M Ota, et al.
Clinical course of fetal congenital atrioventricular block in the Japanese population: a multicentre experience
Heart,
August 1, 2005;
91(8):
1075 - 1079.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
J.-B. Thambo, P. Bordachar, S. Garrigue, S. Lafitte, P. Sanders, S. Reuter, R. Girardot, D. Crepin, P. Reant, R. Roudaut, et al.
Detrimental Ventricular Remodeling in Patients With Congenital Complete Heart Block and Chronic Right Ventricular Apical Pacing
Circulation,
December 21, 2004;
110(25):
3766 - 3772.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
Z. Li, J. F. Strasburger, B. F. Cuneo, N. L. Gotteiner, and R. T. Wakai
Giant Fetal Magnetocardiogram P Waves in Congenital Atrioventricular Block: A Marker of Cardiovascular Compensation?
Circulation,
October 12, 2004;
110(15):
2097 - 2101.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
E. T. Jaeggi, J.-C. Fouron, E. D. Silverman, G. Ryan, J. Smallhorn, and L. K. Hornberger
Transplacental Fetal Treatment Improves the Outcome of Prenatally Diagnosed Complete Atrioventricular Block Without Structural Heart Disease
Circulation,
September 21, 2004;
110(12):
1542 - 1548.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
M Motta, A Tincani, A Lojacono, D Faden, R Gorla, P Airo, F Neri, A Gasparoni, L Ciardelli, A de Silvestri, et al.
Neonatal outcome in patients with rheumatic disease
Lupus,
September 1, 2004;
13(9):
718 - 723.
[Abstract]
[PDF]
|
|
|
|
|
|
|
R. M. Clancy, C. B. Backer, X. Yin, R. P. Kapur, Y. Molad, and J. P. Buyon
Cytokine Polymorphisms and Histologic Expression in Autopsy Studies: Contribution of TNF-{alpha} and TGF-{beta}1 to the Pathogenesis of Autoimmune-Associated Congenital Heart Block
J. Immunol.,
September 15, 2003;
171(6):
3253 - 3261.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
E Rosenthal
Classification of congenital complete heart block: autoantibody-associated or isolated?
Lupus,
June 1, 2003;
12(6):
425 - 426.
[PDF]
|
|
|
|
|
|
|
A Brucato, A Jonzon, D Friedman, L D Allan, G Vignati, M Gasparini, J I Stein, S Montella, M Michaelsson, and J Buyon
Proposal for a new definition of congenital complete atrioventricular block
Lupus,
June 1, 2003;
12(6):
427 - 435.
[Abstract]
[PDF]
|
|
|
|
|
|
|
Committee Members, G. Gregoratos, J. Abrams, A. E. Epstein, R. A. Freedman, D. L. Hayes, M. A. Hlatky, R. E. Kerber, G. V. Naccarelli, M. H. Schoenfeld, et al.
ACC/AHA/NASPE 2002 Guideline Update for Implantation of Cardiac Pacemakers and Antiarrhythmia Devices--Summary Article: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (ACC/AHA/NASPE Committee to Update the 1998 Pacemaker Guidelines)
J. Am. Coll. Cardiol.,
November 6, 2002;
40(9):
1703 - 1719.
[Full Text]
[PDF]
|
|
|
|
|
|
|
G. Gregoratos, J. Abrams, A. E. Epstein, R. A. Freedman, D. L. Hayes, M. A. Hlatky, R. E. Kerber, G. V. Naccarelli, M. H. Schoenfeld, M. J. Silka, et al.
ACC/AHA/NASPE 2002 Guideline Update for Implantation of Cardiac Pacemakers and Antiarrhythmia Devices: Summary Article: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (ACC/AHA/NASPE Committee to Update the 1998 Pacemaker Guidelines)
Circulation,
October 15, 2002;
106(16):
2145 - 2161.
[Full Text]
[PDF]
|
|
|
|
|
|
|
C. Balmer, M. Fasnacht, M. Rahn, L. Molinari, and U. Bauersfeld
Long-term follow up of children with congenital complete atrioventricular block and the impact of pacemaker therapy
Europace,
January 1, 2002;
4(4):
345 - 349.
[Abstract]
[PDF]
|
|
|
|
