Congenital heart block: Difference between revisions
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Revision as of 17:38, 21 March 2009
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Associate Editor-In-Chief: Cafer Zorkun, M.D., Ph.D. [2]
Contributors: Adam C. Lake
Please Take Over This Page and Apply to be Editor-In-Chief for this topic: There can be one or more than one Editor-In-Chief. You may also apply to be an Associate Editor-In-Chief of one of the subtopics below. Please mail us [3] to indicate your interest in serving either as an Editor-In-Chief of the entire topic or as an Associate Editor-In-Chief for a subtopic. Please be sure to attach your CV and or biographical sketch.
Overview
Congenital heart block is a rare congenital heart disease where a slowed heart rate is caused by defects in the heart conduction system. Scarring of the conduction system (the heart’s own natural “pacemaker”), a consequence of inflammation triggered by the mother’s antibodies, damages or even destroys the cells that allow the heart to beat at a normal rhythm. Instead, the damaged heart beats extremely slowly, to an extent that is fatal to nearly 20% of affected babies (with most deaths occurring as fetal demises). Nearly all surviving children with Congenital heart block require permanent implantation of a pacemaker device. Because it is so difficult to treat or repair the damaged heart, a high-priority strategy is to try to prevent the inflammatory process before irreversible scarring can occur.
Neonatal lupus (NL) is the name given to a group of conditions that can affect the babies of mothers who have certain autoantibodies against components of the body’s cells that are called SSA/Ro and SSB/La.
Neonatal lupus can appear as a temporary rash that usually goes away by the time the baby is 6 months old, or very rarely an abnormal blood or liver condition that also improves with time – or it can cause permanent and often life-threatening damage to the fetal heart, known as congenital heart block (CHB).
In women with anti-Ro/La antibodies who are pregnant for the first time, only about 2% of the babies will develop Congenital heart block. But for a woman who has already had a child with Congenital heart block or Neonatal lupus rash, the risk of Congenital heart block in her next pregnancy is nearly 20%. Unfortunately, once complete (third degree) heart block has been unequivocally identified in a fetus, it has never been reversed with any of the therapies that have been tried to date.
If a child is born to a mother with Lupus, there is a chance that the child will develop neonatal lupus, especially if the mother is Anti-Ro (SS/A) positive. In the majority of cases, the mother's antibodies will clear from the child in 6 months, however, in a few cases, the affected child will develop complete congenital heart block.
Source
Ongoing Clinical Trial
- Preventive IVIG Therapy for Congenital Heart Block (The PITCH Study)
References
- Figa FH, McCrindle BW, Bigras JL, et al. Risk factors for venous obstruction in children with transvenous pacing leads. Pacing Clin Electrophysiol. Aug 1997;20(8 Pt 1):1902-9.
- Michaelsson M, Jonzon A, Riesenfeld T. Isolated congenital complete atrioventricular block in adult life. A prospective study. Circulation. Aug 1 1995;92(3):442-9. [Full Text].
- Boutjdir M, Chen L, Zhang ZH, et al. Arrhythmogenicity of IgG and anti-52-kD SSA/Ro affinity-purified antibodies from mothers of children with congenital heart block. Circ Res. Mar 1997;80(3):354-62.
- Boutjdir M, Chen L, Zhang ZH, et al. Serum and immunoglobulin G from the mother of a child with congenital heart block induce conduction abnormalities and inhibit L-type calcium channels in a rat heart model. Pediatr Res. Jul 1998;44(1):11-9.
- Claus R, Hickstein H, Kulz T, et al. Identification and management of fetuses at risk for, or affected by, congenital heart block associated with autoantibodies to SSA (Ro), SSB (La), or an HsEg5-like autoantigen. Rheumatol Int. Aug 2006;26(10):886-95.
- Copel JA, Buyon JP, Kleinman CS. Successful in utero therapy of fetal heart block. Am J Obstet Gynecol. Nov 1995;173(5):1384-90.
- Costedoat-Chalumeau N, Amoura Z, Villain E, et al. Anti-SSA/Ro antibodies and the heart: more than complete congenital heart block? A review of electrocardiographic and myocardial abnormalities and of treatment options. Arthritis Res Ther. 2005;7(2):69-73.
- Costedoat-Chalumeau N, Georgin-Lavialle S, Amoura Z, et al. Anti-SSA/Ro and anti-SSB/La antibody-mediated congenital heart block. Lupus. 2005;14(9):660-4.
- Cutler NG, Karpawich PP, Cavitt D, et al. Steroid-eluting epicardial pacing electrodes: six year experience of pacing thresholds in a growing pediatric population. Pacing Clin Electrophysiol. Dec 1997;20(12 Pt 1):2943-8.
- Friedman DM, Kim MY, Copel JA, et al. Utility of cardiac monitoring in fetuses at risk for congenital heart block: the PR Interval and Dexamethasone Evaluation (PRIDE) prospective study. Circulation. 2008;117:485-93.
- Friedman DM, Zervoudakis I, Buyon JP. Perinatal monitoring of fetal well-being in the presence of congenital heart block. Am J Perinatol. 1998;15(12):669-73.
- Hamilton R, Gow R, Bahoric B, et al. Steroid-eluting epicardial leads in pediatrics: improved epicardial thresholds in the first year. Pacing Clin Electrophysiol. Nov 1991;14(11 Pt 2):2066-72.
- Hamilton RM, Chiu C, Gow RM, Williams WG. A comparison of two stab-on unipolar epicardial pacing leads in children. Pacing Clin Electrophysiol. Mar 1997;20(3 Pt 1):631-6.
- Jaeggi ET, Hornberger LK, Smallhorn JF, Fouron JC. Prenatal diagnosis of complete atrioventricular block associated with structural heart disease: combined experience of two tertiary care centers and review of the literature. Ultrasound Obstet Gynecol. Jul 2005;26(1):16-21.
- Karpawich PP, Stokes KB, Proctor K, et al. "In-line" bipolar, steroid-eluting, high impedance, epimyocardial pacing lead. Pacing Clin Electrophysiol. Mar 1998;21(3):503-8.
- Karpawich PP, Walters H, Hakimi M. Chronic performance of a transvenous steroid pacing lead used as an epi- intramyocardial electrode. Pacing Clin Electrophysiol. Jul 1998;21(7):1486-8.
- Miranda-Carus ME, Boutjdir M, Tseng CE. Induction of antibodies reactive with SSA/Ro-SSB/La and development of congenital heart block in a murine model. J Immunol. Dec 1 1998;161(11):5886-92.
- Moak JP, Barron KS, Hougen TJ, et al. Congenital heart block: development of late-onset cardiomyopathy, a previously underappreciated sequela. J Am Coll Cardiol. Jan 2001;37(1):238-42.
- Neiman AR, Lee LA, Weston WL, Buyon JP. Cutaneous manifestations of neonatal lupus without heart block: characteristics of mothers and children enrolled in a national registry. J Pediatr. Nov 2000;137(5):674-80.
- Rao V, Williams WG, Hamilton RH, et al. Trends in pediatric cardiac pacing. Can J Cardiol. Dec 1995;11(11):993-9.
- Suarez-Penaranda JM, Munoz JI, Rodriguez-Calvo MS, et al. The Pathology of the heart conduction system in congenital heart block. J Clin Forensic Med. Aug-Nov 2006;13(6-8):341-3.
- Weng KP, Chiou CW, Huang SH, et al. The long-term outcome of children with isolated congenital complete atrioventricular block. Acta Paediatr Taiwan. Sep-Oct 2005;46(5):260-7.
See Also
External Links