Congenital heart block
You don't need to be Editor-In-Chief to add or edit content to WikiDoc. You can begin to add to or edit text on this WikiDoc page by clicking on the edit button at the top of this page. Next enter or edit the information that you would like to appear here. Once you are done editing, scroll down and click the Save page button at the bottom of the page.
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Phone:617-632-7753
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
Electrocardiography | |
|---|---|
| Overview | History of the EKG • EKG interpretation basics • Normal sinus rhythm |
| EKG Complexes | P wave • QRS complex • ST Segment • T wave • U wave Osborn wave • H wave • K wave • Delta wave |
| EKG Intervals | PR Interval • QRS Interval • ST Interval • QT Interval |
| Conduction System & Bradycardia | Cardiac pacemaker • SA node • AV node• Bundle of His • Purkinje fibers • Sinus bradycardia • First Degree AV Block • Second Degree AV Block • Complete or Third-Degree AV Block • Concealed conduction • AV Junctional Rhythms • LBBB • LAHB • LPHB • RBBB • Trifascicular block |
| Atrial Arrhythmias | Sinus tachycardia • Premature Atrial Contractions (PACs) • Ectopic Atrial Rhythm • Paroxysmal Atrial Tachycardia (PAT) • Paroxysmal Atrial Tachycardia (PAT) with Block • Multifocal Atrial Tachycardia (MAT) • Atrial Flutter • Atrial Fibrillation • Wandering atrial pacemaker |
| Ventricular Arrhythmias | Differential Diagnosis of Tachycardia with a Wide QRS Complex • Accelerated Idioventricular Rhythm • Ventricular Parasystole • Premature Ventricular Contractions (PVCs) • Ventricular tachycardia • Ventricular Fibrillation • Sudden cardiac death |
| EKG Abnormalities in Disease States | Hypertrophy & Dilatation • Right atrial enlargement • Left atrial enlargement • Biatrial enlargement • Left Ventricular Hypertrophy • Right Ventricular Hypertrophy • Biventricular Hypertrophy •
Acute myocardial infarction • NSTEMI • STEMI • Right ventricular myocardial infarction • Atrial infarction Pre-excitation Syndromes • Wolff-Parkinson-White Syndrome • Lown Ganong Levine Syndrome • Mahaim Type Preexcitation Cardiomyopathies • Arrhythmogenic Right Ventricular Dysplasia • Dilated Cardiomyopathy • Hypertrophic Cardiomyopathy Drug Effects on the EKG • Adenosine • β-blockers • Digitalis • Quinidine • Procainamide • Disopyramide • Lidocaine • Tocainide and Mexiletine • Phenytoin • Encainide, Flecainide and Propafenone • Amiodarone • Bretylium • Ca Channel Blockers • Phenothiazines • Tricyclic Antidepressants • Lithium Congenital Heart Disease • Dextrocardia • Atrial Septal Defect • Ventricular Septal Defect • Tetralogy of Fallot • Conjoined Twins or Siamese Twins • Congenital heart block Electrolyte Disturbances • Hyperkalemia • Hypokalemia • Hypercalcemia • Hypocalcemia • Nonspecific Changes Other Heart Diseases • Pericarditis • Myocarditis • Tamponade • Heart Transplantation • Sick Sinus Syndrome • Long QT Syndrome Inherited Disease • Brugada Syndrome Systemic Diseases • CNS Disease • Cardiac Tumors Heart Transplantation • EKG Changes in patient with Heart Transplantation Exogenous Effects • Hypothermia • Chest Trauma • Insect Bites • Electric Injuries |
| Technical Issues and Potential Errors in Interpretation | Artifacts • Lead Placement Errors • The EKG in a Patient with a Pacemaker • EKG in athletes |
Circulatory system pathology (I, 390-459) | |
|---|---|
| Hypertension | Hypertensive heart disease - Hypertensive nephropathy - Secondary hypertension (Renovascular hypertension) |
| Ischaemic heart disease | Angina pectoris (Prinzmetal's angina) - Myocardial infarction (heart attack) - Dressler's syndrome |
| Pulmonary circulation | Pulmonary embolism - Cor pulmonale |
| Pericardium | Pericarditis - Pericardial effusion - Cardiac tamponade |
| Endocardium/heart valves | Endocarditis - mitral valve (regurgitation, prolapse, stenosis) - aortic valve (stenosis, insufficiency) - pulmonary valve (stenosis, insufficiency) - tricuspid valve (stenosis, insufficiency) |
| Myocardium | Myocarditis - Cardiomyopathy (Dilated cardiomyopathy, Hypertrophic cardiomyopathy, Loeffler endocarditis, Restrictive cardiomyopathy) - Arrhythmogenic right ventricular dysplasia |
| Electrical conduction system of the heart | Heart block: AV block (First degree, Second degree, Third degree) - Bundle branch block (Left, Right) - Bifascicular block - Trifascicular block Pre-excitation syndrome (Wolff-Parkinson-White, Lown-Ganong-Levine) - Long QT syndrome - Adams-Stokes syndrome - Cardiac arrest - Sudden cardiac death Arrhythmia: Paroxysmal tachycardia (Supraventricular, AV nodal reentrant, Ventricular) - Atrial flutter - Atrial fibrillation (Familial) - Ventricular fibrillation - Premature contraction (Atrial, Ventricular) - Ectopic pacemaker - Sick sinus syndrome |
| Other heart conditions | Heart failure - Cardiovascular disease - Cardiomegaly - Ventricular hypertrophy (Left, Right) |
| Cerebrovascular diseases | Stroke - Transient ischemic attack - Intracranial hemorrhage/cerebral hemorrhage: Extra-axial hemorrhage (Epidural hemorrhage, Subdural hemorrhage, Subarachnoid hemorrhage) Intra-axial hematoma (Intraventricular hemorrhages, Intraparenchymal hemorrhage) - Anterior spinal artery syndrome - Binswanger's disease - Moyamoya disease |
| Arteries, arterioles and capillaries | Atherosclerosis (Renal artery stenosis) - Aortic dissection/Aortic aneurysm (Abdominal aortic aneurysm) - Aneurysm - Raynaud's phenomenon/Raynaud's disease - Buerger's disease - Vasculitis/Arteritis (Aortitis) - Intermittent claudication - Arteriovenous fistula - Hereditary hemorrhagic telangiectasia - Spider angioma - Dissection (Carotid artery, Vertebral artery) |
| Veins, lymphatic vessels and lymph nodes | Thrombosis/Phlebitis/Thrombophlebitis (Deep vein thrombosis, May-Thurner syndrome, Portal vein thrombosis, Venous thrombosis, Budd-Chiari syndrome, Renal vein thrombosis, Paget-Schroetter disease) - Varicose veins / Portacaval anastomosis (Hemorrhoid, Esophageal varices, Varicocele, Gastric varices, Caput medusae) - Superior vena cava syndrome - Lymph (Lymphadenitis, Lymphedema, Lymphangitis) |
| Other | Hypotension (Orthostatic hypotension) - Rheumatic fever |
| See also congenital (Q20-Q28, 745-747) | |
WikiDoc Research Resources for Congenital heart block | |
|---|---|
| Articles on Congenital heart block | Most recent articles on Congenital heart block • Most cited articles on Congenital heart block • Review articles on Congenital heart block • Articles on Congenital heart block in N Eng J Med, Lancet, BMJ |
| Media (Slides, Video, Images, MP3) on Congenital heart block | Powerpoint slides on Congenital heart block • Images of Congenital heart block • Photos of Congenital heart block • Podcasts & MP3s on Congenital heart block • Videos on Congenital heart block |
| Evidence Based Medicine Regarding Congenital heart block | Cochrane Collaboration on Congenital heart block • Bandolier on Congenital heart block • TRIP on Congenital heart block |
| Cost Effectiveness of Congenital heart block | Cost Effectiveness of Congenital heart block |
| Clinical Trials Involving Congenital heart block | Ongoing Trials on Congenital heart block at Clinical Trials.gov • Trial results on Congenital heart block • Clinical Trials on Congenital heart block at Google |
| Guidelines / Policies / Government Resources (FDA/CDC) Regarding Congenital heart block | US National Guidelines Clearinghouse on Congenital heart block • NICE Guidance on Congenital heart block • NHS PRODIGY Guidance • FDA on Congenital heart block • CDC on Congenital heart block |
| Textbook Information on Congenital heart block | Books and Textbook Information on Congenital heart block |
| Pharmacology Resources on Congenital heart block | Dosing of Congenital heart block • Drug interactions with Congenital heart block • Side effects of Congenital heart block • Allergic reactions to Congenital heart block • Overdose information on Congenital heart block • Carcinogenicity information on Congenital heart block • Congenital heart block in pregnancy • Pharmacokinetics of Congenital heart block • |
| Genetics, Pharmacogenomics, and Proteinomics of Congenital heart block | Genetics of Congenital heart block • Pharmacogenomics of Congenital heart block • Proteomics of Congenital heart block |
| Newstories on Congenital heart block | Congenital heart block in the news • Be alerted to news on Congenital heart block • News trends on Congenital heart block |
| Commentary on Congenital heart block | Blogs on Congenital heart block |
| Patient Resources on Congenital heart block | Patient resources on Congenital heart block • Discussion groups on Congenital heart block • Patient Handouts on Congenital heart block • Directions to Hospitals Treating Congenital heart block • Risk calculators and risk factors for Congenital heart block |
| Healthcare Provider Resources on Congenital heart block | Symptoms of Congenital heart block • Causes & Risk Factors for Congenital heart block • Diagnostic studies for Congenital heart block • Treatment of Congenital heart block |
| Continuing Medical Education (CME) Programs on Congenital heart block | CME Programs on Congenital heart block |
| International Resources on Congenital heart block | Congenital heart block en Espanol • Congenital heart block en Francais |
| Business Resources on Congenital heart block | Congenital heart block in the Marketplace • Patents on Congenital heart block |
| Informatics Resources on Congenital heart block | List of terms related to Congenital heart block |
| ||||
Acknowledgement and Attribution Regarding Sources of Content
Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .
