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*If left untreated, [#%] of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
*If left untreated, [#%] of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
*Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
*Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
*Prognosis is generally [excellent/good/poor], and the [1/5/10­year mortality/survival rate] of patients with [disease name] is approximately [#%].
*Prognosis is generally [excellent/good/poor], and the [1/5/10­year mortality/survival rate] of patients with [disease name] is approximately [#%]


== Diagnosis ==
== Diagnosis ==

Revision as of 14:48, 20 February 2020

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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

Overview

Congenital heart block is a rare congenital heart disease caused by defects in the heart conduction system diagnosed on or before 28 days of life. It can lead to slowed heart rate.

Historical Perspective

  • Congenital heart block was first discovered by [scientist name], a [nationality + occupation], in [year] during/following [event].
  • In [year], [gene] mutations were first identified in the pathogenesis of [disease name].
  • In [year], the first [discovery] was developed by [scientist] to treat/diagnose [disease name].

Classification

  • Congenital Heart Block may be classified according to cause into 3 groups:[1]
  • First Degree Congenital Heart Block
  • Second Degree Congenital Heart Block
  • Third Degree Congenital (Complete) Heart Block

Pathophysiology

The normal physiology of congenital heart block can be understood as follows:[2][3][4][5]

  • Mother's antibodies cross from the fetus placenta and damage of cardiac conduction system. The majority of anti bodies are from mother to response an infectionin or in response to an autoimmune disorder (SLE).
  • Genetic disorde (autosomal recessive/dominant genetic trait).
  • In some patients, congenital heart block is a secondary characteristic disorders or heart muscle tumors (myocardium).
  • Fibrous tissue that either replaces the atrioventricular (AV) node and its surrounding tissue or by an interruption between the atrial myocardium and the AV nod.

Causes

The most common cause of congenital heart block is neonatal lupus.[6]

cardiac conduction system is damaged by antibodies that cross from the mother to the fetus via the placenta

It accounts for almost all cases presenting in the intrauterine and neonatal period. Other causes include:[7][8][9]

Differentiating [disease name] from other Diseases

Epidemiology and Demographics

  • The prevalence of congenital heart block is approximately 1 per 22,00 live births individuals worldwide. [12][13]
  • In [year], the incidence of [disease name] was estimated to be [number or range] cases per 100,000 individuals in [location].

Age

  • Congential heart block is diagnosed among patients aged from brith to 28 days old.
  • Congential heart block is observed among children.

Gender

  • Congenital heart block affects men and women equally.
  • [Gender 1] are more commonly affected with [disease name] than [gender 2].
  • The [gender 1] to [Gender 2] ratio is approximately [number > 1] to 1.

Race

  • There is no racial predilection for congenital heart block.

Risk Factors

  • One form of congenital heart block occurs in babies whose mothers have autoimmune diseases, such as lupus.[14]
  • Patients with congenital heart block make proteins called antibodies that attack and damage the body's tissues or cells.[14]

Natural History, Complications, Prognosis

Prognosis

The damaged heart may beat extremely slowly. In some cases, the heart rate is so slow that it is fatal in nearly 20% of affected babies (with most deaths occurring as fetal demises). Patients presenting as fetuses or at birth have significantly higher morbidity and mortality rates than do patients presenting later in childhood.


  • The majority of patients with [disease name] remain asymptomatic for [duration/years].
  • Early clinical features include skin rash, liver
  • If left untreated, [#%] of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
  • Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
  • Prognosis is generally [excellent/good/poor], and the [1/5/10­year mortality/survival rate] of patients with [disease name] is approximately [#%]

Diagnosis


Symptoms

Physical Examination

  • Patients with congenital heart block usually appear :[20]
  • Annular lesions (Face, particularly around eyes)
  • Photosensitive

Laboratory Findings

  • There are no specific laboratory findings associated with congenital heart block.[21]
  • May be autoantibodies to Ro/SSA and La/SSB ribonucleoproteins using ELISAW are helpful when when congenital heart block detected.[22]
  • Other laboratory findings consistent with the diagnosis of congenital heart block include Sera, ELISA, counter-immunoelectrophoresis (CIE) method.[22][23][24]

Imaging Findings

  • There are no [imaging study] findings associated with [disease name].
  • [Imaging study 1] is the imaging modality of choice for [disease name].
  • On [imaging study 1], [disease name] is characterized by [finding 1], [finding 2], and [finding 3].
  • [Imaging study 2] may demonstrate [finding 1], [finding 2], and [finding 3].

Treatment

Medical Therapy

Surgery

Prevention

  • There are no primary preventive measures available for Congenital Heart Disease.
  • Fetus with second or third degree heart block are less in women who take HCQ.[34][35]

References

  1. ↑ Baruteau, Alban-Elouen; Pass, Robert H.; Thambo, Jean-Benoit; Behaghel, Albin; Le Pennec, Solène; Perdreau, Elodie; Combes, Nicolas; Liberman, Leonardo; McLeod, Christopher J. (2016). "Congenital and childhood atrioventricular blocks: pathophysiology and contemporary management". European Journal of Pediatrics. 175 (9): 1235–1248. doi:10.1007/s00431-016-2748-0. ISSN 0340-6199.
  2. ↑ Zhou KY, Hua YM (December 2017). "Autoimmune-associated Congenital Heart Block: A New Insight in Fetal Life". Chin. Med. J. 130 (23): 2863–2871. doi:10.4103/0366-6999.219160. PMC 5717867. PMID 29176145.
  3. ↑ 3.0 3.1 Fahed AC, Gelb BD, Seidman JG, Seidman CE (February 2013). "Genetics of congenital heart disease: the glass half empty". Circ. Res. 112 (4): 707–20. doi:10.1161/CIRCRESAHA.112.300853. PMC 3827691. PMID 23410880.
  4. ↑ Capone C, Buyon JP, Friedman DM, Frishman WH (2012). "Cardiac manifestations of neonatal lupus: a review of autoantibody-associated congenital heart block and its impact in an adult population". Cardiol Rev. 20 (2): 72–6. doi:10.1097/CRD.0b013e31823c808b. PMC 3275696. PMID 22183063.
  5. ↑ Sharma, Gaurav; Linden, Michael D.; Schultz, Daniel S.; Inamdar, Kedar V. (2010). "Cystic tumor of the atrioventricular node: an unexpected finding in an explanted heart". Cardiovascular Pathology. 19 (3): e75–e78. doi:10.1016/j.carpath.2008.10.011. ISSN 1054-8807.
  6. ↑ Vinet, Évelyne; Pineau, Christian A.; Scott, Susan; Clarke, Ann E.; Platt, Robert W.; Bernatsky, Sasha (2015). "Increased Congenital Heart Defects in Children Born to Women With Systemic Lupus Erythematosus". Circulation. 131 (2): 149–156. doi:10.1161/CIRCULATIONAHA.114.010027. ISSN 0009-7322.
  7. ↑ Zhou KY, Hua YM (December 2017). "Autoimmune-associated Congenital Heart Block: A New Insight in Fetal Life". Chin. Med. J. 130 (23): 2863–2871. doi:10.4103/0366-6999.219160. PMC 5717867. PMID 29176145.
  8. ↑ Lee LA (2004). "Neonatal lupus: clinical features and management". Paediatr Drugs. 6 (2): 71–8. doi:10.2165/00148581-200406020-00001. PMID 15035648.
  9. ↑ Yousuf T, Kramer J, Jones B, Keshmiri H, Dia M (2016). "Pulmonary Hypertension in a Patient With Congenital Heart Defects and Heterotaxy Syndrome". Ochsner J. 16 (3): 309–11. PMC 5024815. PMID 27660582.
  10. ↑ Capone C, Buyon JP, Friedman DM, Frishman WH (2012). "Cardiac manifestations of neonatal lupus: a review of autoantibody-associated congenital heart block and its impact in an adult population". Cardiol Rev. 20 (2): 72–6. doi:10.1097/CRD.0b013e31823c808b. PMC 3275696. PMID 22183063.
  11. ↑ Lipshultz, Steven E.; Law, Yuk M.; Asante-Korang, Alfred; Austin, Eric D.; Dipchand, Anne I.; Everitt, Melanie D.; Hsu, Daphne T.; Lin, Kimberly Y.; Price, Jack F.; Wilkinson, James D.; Colan, Steven D. (2019). "Cardiomyopathy in Children: Classification and Diagnosis: A Scientific Statement From the American Heart Association". Circulation. 140 (1). doi:10.1161/CIR.0000000000000682. ISSN 0009-7322.
  12. ↑ Dm Friedman, Lj Duncanson, J. Glickstein & Jp Buyon (2003). "A review of congenital heart block". Images in paediatric cardiology. 5 (3): 36–48. PMID 22368629. Unknown parameter |month= ignored (help)
  13. ↑ Pilar Brito-Zeron, Peter M. Izmirly, Manuel Ramos-Casals, Jill P. Buyon & Munther A. Khamashta (2015). "The clinical spectrum of autoimmune congenital heart block". Nature reviews. Rheumatology. 11 (5): 301–312. doi:10.1038/nrrheum.2015.29. PMID 25800217. Unknown parameter |month= ignored (help)
  14. ↑ 14.0 14.1 Friedman D, Duncanson L, Glickstein J, Buyon J (July 2003). "A review of congenital heart block". Images Paediatr Cardiol. 5 (3): 36–48. PMC 3232542. PMID 22368629. Vancouver style error: initials (help)
  15. ↑ Friedman D, Duncanson L, Glickstein J, Buyon J (July 2003). "A review of congenital heart block". Images Paediatr Cardiol. 5 (3): 36–48. PMC 3232542. PMID 22368629. Vancouver style error: initials (help)
  16. ↑ Hunter LE, Seale AN (September 2018). "EDUCATIONAL SERIES IN CONGENITAL HEART DISEASE: Prenatal diagnosis of congenital heart disease". Echo Res Pract. 5 (3): R81–R100. doi:10.1530/ERP-18-0027. PMC 6107762. PMID 30012852.
  17. ↑ Friedman D, Duncanson L, Glickstein J, Buyon J (July 2003). "A review of congenital heart block". Images Paediatr Cardiol. 5 (3): 36–48. PMC 3232542. PMID 22368629. Vancouver style error: initials (help)
  18. ↑ Donofrio, Mary T.; Moon-Grady, Anita J.; Hornberger, Lisa K.; Copel, Joshua A.; Sklansky, Mark S.; Abuhamad, Alfred; Cuneo, Bettina F.; Huhta, James C.; Jonas, Richard A.; Krishnan, Anita; Lacey, Stephanie; Lee, Wesley; Michelfelder, Erik C.; Rempel, Gwen R.; Silverman, Norman H.; Spray, Thomas L.; Strasburger, Janette F.; Tworetzky, Wayne; Rychik, Jack (2014). "Diagnosis and Treatment of Fetal Cardiac Disease". Circulation. 129 (21): 2183–2242. doi:10.1161/01.cir.0000437597.44550.5d. ISSN 0009-7322.
  19. ↑ 19.0 19.1 Kusumoto, Fred M.; Schoenfeld, Mark H.; Barrett, Coletta; Edgerton, James R.; Ellenbogen, Kenneth A.; Gold, Michael R.; Goldschlager, Nora F.; Hamilton, Robert M.; Joglar, José A.; Kim, Robert J.; Lee, Richard; Marine, Joseph E.; McLeod, Christopher J.; Oken, Keith R.; Patton, Kristen K.; Pellegrini, Cara N.; Selzman, Kimberly A.; Thompson, Annemarie; Varosy, Paul D. (2019). "2018 ACC/AHA/HRS Guideline on the Evaluation and Management of Patients With Bradycardia and Cardiac Conduction Delay". Journal of the American College of Cardiology. 74 (7): e51–e156. doi:10.1016/j.jacc.2018.10.044. ISSN 0735-1097.
  20. ↑ Friedman D, Duncanson L, Glickstein J, Buyon J (July 2003). "A review of congenital heart block". Images Paediatr Cardiol. 5 (3): 36–48. PMC 3232542. PMID 22368629. Vancouver style error: initials (help)
  21. ↑ Brucato, Antonio; Grava, Chiara; Bortolati, Maria; Ikeda, Keigo; Milanesi, Ornella; Cimaz, Rolando; Ramoni, Veronique; Vignati, Gabriele; Martinelli, Stefano; Sadou, Youcef; Borghi, Adele; Tincani, Angela; Chan, Edward K.L.; Ruffatti, Amelia (2009). "Congenital Heart Block Not Associated with Anti-Ro/La Antibodies: Comparison with Anti-Ro/La-positive Cases". The Journal of Rheumatology. 36 (8): 1744–1748. doi:10.3899/jrheum.080737. ISSN 0315-162X.
  22. ↑ 22.0 22.1 Brucato A, Grava C, Bortolati M, Ikeda K, Milanesi O, Cimaz R, Ramoni V, Vignati G, Martinelli S, Sadou Y, Borghi A, Tincani A, Chan EK, Ruffatti A (August 2009). "Congenital heart block not associated with anti-Ro/La antibodies: comparison with anti-Ro/La-positive cases". J. Rheumatol. 36 (8): 1744–8. doi:10.3899/jrheum.080737. PMC 2798588. PMID 19567621.
  23. ↑ Bernstein RM, Bunn CC, Hughes GR (October 1982). "Identification of antibodies to acidic antigens by counterimmunoelectrophoresis". Ann. Rheum. Dis. 41 (5): 554–5. doi:10.1136/ard.41.5.554. PMC 1001043. PMID 6982030.
  24. ↑ Chan EK, Hamel JC, Buyon JP, Tan EM (January 1991). "Molecular definition and sequence motifs of the 52-kD component of human SS-A/Ro autoantigen". J. Clin. Invest. 87 (1): 68–76. doi:10.1172/JCI115003. PMC 294993. PMID 1985112.
  25. ↑ Clowse, Megan E B; Eudy, Amanda M; Kiernan, Elizabeth; Williams, Matthew R; Bermas, Bonnie; Chakravarty, Eliza; Sammaritano, Lisa R; Chambers, Christina D; Buyon, Jill (2018). "The prevention, screening and treatment of congenital heart block from neonatal lupus: a survey of provider practices". Rheumatology. 57 (suppl_5): v9–v17. doi:10.1093/rheumatology/key141. ISSN 1462-0324.
  26. ↑ Dm Friedman, Lj Duncanson, J. Glickstein & Jp Buyon (2003). "A review of congenital heart block". Images in paediatric cardiology. 5 (3): 36–48. PMID 22368629. Unknown parameter |month= ignored (help)
  27. ↑ Kai-Yu Zhou & Yi-Min Hua (2017). "Autoimmune-associated Congenital Heart Block: A New Insight in Fetal Life". Chinese medical journal. 130 (23): 2863–2871. doi:10.4103/0366-6999.219160. PMID 29176145. Unknown parameter |month= ignored (help)
  28. ↑ Aurelie Ambrosi & Marie Wahren-Herlenius (2012). "Congenital heart block: evidence for a pathogenic role of maternal autoantibodies". Arthritis research & therapy. 14 (2): 208. doi:10.1186/ar3787. PMID 22546326. Unknown parameter |month= ignored (help)
  29. ↑ Dm Friedman, Lj Duncanson, J. Glickstein & Jp Buyon (2003). "A review of congenital heart block". Images in paediatric cardiology. 5 (3): 36–48. PMID 22368629. Unknown parameter |month= ignored (help)
  30. ↑ Dm Friedman, Lj Duncanson, J. Glickstein & Jp Buyon (2003). "A review of congenital heart block". Images in paediatric cardiology. 5 (3): 36–48. PMID 22368629. Unknown parameter |month= ignored (help)
  31. ↑ Dm Friedman, Lj Duncanson, J. Glickstein & Jp Buyon (2003). "A review of congenital heart block". Images in paediatric cardiology. 5 (3): 36–48. PMID 22368629. Unknown parameter |month= ignored (help)
  32. ↑ Kai-Yu Zhou & Yi-Min Hua (2017). "Autoimmune-associated Congenital Heart Block: A New Insight in Fetal Life". Chinese medical journal. 130 (23): 2863–2871. doi:10.4103/0366-6999.219160. PMID 29176145. Unknown parameter |month= ignored (help)
  33. ↑ Kai-Yu Zhou & Yi-Min Hua (2017). "Autoimmune-associated Congenital Heart Block: A New Insight in Fetal Life". Chinese medical journal. 130 (23): 2863–2871. doi:10.4103/0366-6999.219160. PMID 29176145. Unknown parameter |month= ignored (help)
  34. ↑ Tunks, Robert D.; Clowse, Megan E.B.; Miller, Stephen G.; Brancazio, Leo R.; Barker, Piers C.A. (2013). "Maternal autoantibody levels in congenital heart block and potential prophylaxis with antiinflammatory agents". American Journal of Obstetrics and Gynecology. 208 (1): 64.e1–64.e7. doi:10.1016/j.ajog.2012.09.020. ISSN 0002-9378.
  35. ↑ Saxena, Amit; Izmirly, Peter M.; Han, Sung Won; Briassouli, Paraskevi; Rivera, Tania L.; Zhong, Hua; Friedman, Deborah M.; Clancy, Robert M.; Buyon, Jill P. (2015). "Serum Biomarkers of Inflammation, Fibrosis, and Cardiac Function in Facilitating Diagnosis, Prognosis, and Treatment of Anti-SSA/Ro–Associated Cardiac Neonatal Lupus". Journal of the American College of Cardiology. 66 (8): 930–939. doi:10.1016/j.jacc.2015.06.1088. ISSN 0735-1097.
  36. ↑ Dm Friedman, Lj Duncanson, J. Glickstein & Jp Buyon (2003). "A review of congenital heart block". Images in paediatric cardiology. 5 (3): 36–48. PMID 22368629. Unknown parameter |month= ignored (help)
  37. ↑ Pilar Brito-Zeron, Peter M. Izmirly, Manuel Ramos-Casals, Jill P. Buyon & Munther A. Khamashta (2015). "The clinical spectrum of autoimmune congenital heart block". Nature reviews. Rheumatology. 11 (5): 301–312. doi:10.1038/nrrheum.2015.29. PMID 25800217. Unknown parameter |month= ignored (help)
  38. ↑ Dm Friedman, Lj Duncanson, J. Glickstein & Jp Buyon (2003). "A review of congenital heart block". Images in paediatric cardiology. 5 (3): 36–48. PMID 22368629. Unknown parameter |month= ignored (help)
  39. ↑ Pilar Brito-Zeron, Peter M. Izmirly, Manuel Ramos-Casals, Jill P. Buyon & Munther A. Khamashta (2015). "The clinical spectrum of autoimmune congenital heart block". Nature reviews. Rheumatology. 11 (5): 301–312. doi:10.1038/nrrheum.2015.29. PMID 25800217. Unknown parameter |month= ignored (help)
  40. ↑ Kai-Yu Zhou & Yi-Min Hua (2017). "Autoimmune-associated Congenital Heart Block: A New Insight in Fetal Life". Chinese medical journal. 130 (23): 2863–2871. doi:10.4103/0366-6999.219160. PMID 29176145. Unknown parameter |month= ignored (help)
  41. ↑ Dm Friedman, Lj Duncanson, J. Glickstein & Jp Buyon (2003). "A review of congenital heart block". Images in paediatric cardiology. 5 (3): 36–48. PMID 22368629. Unknown parameter |month= ignored (help)
  42. ↑ Pilar Brito-Zeron, Peter M. Izmirly, Manuel Ramos-Casals, Jill P. Buyon & Munther A. Khamashta (2015). "The clinical spectrum of autoimmune congenital heart block". Nature reviews. Rheumatology. 11 (5): 301–312. doi:10.1038/nrrheum.2015.29. PMID 25800217. Unknown parameter |month= ignored (help)


Electrocardiogram

Electrocardiographic findings in congenital heart block depend on the type of block.

Treatment

Surgery

Treatment depends on the type of heart block.

Nearly all surviving children with congenital heart block require permanent implantation of an pacemaker device.

Prevention

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. The mother of the child should consult with a rheumatologist to begin monitoring for possible autoimmune disease. Consultation with a rheumatologist is also advised for the infant, particularly if other manifestations of neonatal lupus erythematosus are present. Genetic consultation is recommended for children with first-degree relatives with structural heart disease or those with storage disorder or cardiomyopathy.

Source

Related Chapters

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.
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  • 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.
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