Congenital heart block: Difference between revisions

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==Classification==
==Classification==
*Congenital Heart Block may be classified according to cause into 3 groups:<ref name="BaruteauPass2016">{{cite journal|last1=Baruteau|first1=Alban-Elouen|last2=Pass|first2=Robert H.|last3=Thambo|first3=Jean-Benoit|last4=Behaghel|first4=Albin|last5=Le Pennec|first5=Solène|last6=Perdreau|first6=Elodie|last7=Combes|first7=Nicolas|last8=Liberman|first8=Leonardo|last9=McLeod|first9=Christopher J.|title=Congenital and childhood atrioventricular blocks: pathophysiology and contemporary management|journal=European Journal of Pediatrics|volume=175|issue=9|year=2016|pages=1235–1248|issn=0340-6199|doi=10.1007/s00431-016-2748-0}}</ref>
*Congenital Heart Block may be classified according to cause into 3 groups:


:*First Degree Congenital Heart Block
:*First Degree Congenital Heart Block
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==Pathophysiology==
==Pathophysiology==
The normal physiology of congenital heart block can be understood as follows:<ref name="pmid29176145">{{cite journal |vauthors=Zhou KY, Hua YM |title=Autoimmune-associated Congenital Heart Block: A New Insight in Fetal Life |journal=Chin. Med. J. |volume=130 |issue=23 |pages=2863–2871 |date=December 2017 |pmid=29176145 |pmc=5717867 |doi=10.4103/0366-6999.219160 |url=}}</ref><ref name="pmid23410880">{{cite journal |vauthors=Fahed AC, Gelb BD, Seidman JG, Seidman CE |title=Genetics of congenital heart disease: the glass half empty |journal=Circ. Res. |volume=112 |issue=4 |pages=707–20 |date=February 2013 |pmid=23410880 |pmc=3827691 |doi=10.1161/CIRCRESAHA.112.300853 |url=}}</ref><ref name="pmid221830632">{{cite journal |vauthors=Capone C, Buyon JP, Friedman DM, Frishman WH |title=Cardiac manifestations of neonatal lupus: a review of autoantibody-associated congenital heart block and its impact in an adult population |journal=Cardiol Rev |volume=20 |issue=2 |pages=72–6 |date=2012 |pmid=22183063 |pmc=3275696 |doi=10.1097/CRD.0b013e31823c808b |url=}}</ref><ref name="SharmaLinden2010">{{cite journal|last1=Sharma|first1=Gaurav|last2=Linden|first2=Michael D.|last3=Schultz|first3=Daniel S.|last4=Inamdar|first4=Kedar V.|title=Cystic tumor of the atrioventricular node: an unexpected finding in an explanted heart|journal=Cardiovascular Pathology|volume=19|issue=3|year=2010|pages=e75–e78|issn=10548807|doi=10.1016/j.carpath.2008.10.011}}</ref>
The normal physiology of congenital heart block can be understood as follows:


*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).
*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).
* Genetic disorde (autosomal recessive/dominant genetic trait).
* In some patients, congenital heart block is a secondary characteristic disorders or heart muscle tumors (myocardium).
* In some patients, congenital heart block is a secondary characteristic disorders or [[heart]] [[muscle]] [[Tumor|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.
*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==
==Causes==
The most common cause of congenital  heart block is neonatal lupus.<ref name="VinetPineau2015">{{cite journal|last1=Vinet|first1=Évelyne|last2=Pineau|first2=Christian A.|last3=Scott|first3=Susan|last4=Clarke|first4=Ann E.|last5=Platt|first5=Robert W.|last6=Bernatsky|first6=Sasha|title=Increased Congenital Heart Defects in Children Born to Women With Systemic Lupus Erythematosus|journal=Circulation|volume=131|issue=2|year=2015|pages=149–156|issn=0009-7322|doi=10.1161/CIRCULATIONAHA.114.010027}}</ref>
The most common cause of congenital  heart block is neonatal lupus.


cardiac conduction system is damaged by antibodies that cross from the mother to the fetus via the placenta
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:<ref name="pmid291761452">{{cite journal |vauthors=Zhou KY, Hua YM |title=Autoimmune-associated Congenital Heart Block: A New Insight in Fetal Life |journal=Chin. Med. J. |volume=130 |issue=23 |pages=2863–2871 |date=December 2017 |pmid=29176145 |pmc=5717867 |doi=10.4103/0366-6999.219160 |url=}}</ref><ref name="pmid15035648">{{cite journal |vauthors=Lee LA |title=Neonatal lupus: clinical features and management |journal=Paediatr Drugs |volume=6 |issue=2 |pages=71–8 |date=2004 |pmid=15035648 |doi=10.2165/00148581-200406020-00001 |url=}}</ref><ref name="pmid27660582">{{cite journal |vauthors=Yousuf T, Kramer J, Jones B, Keshmiri H, Dia M |title=Pulmonary Hypertension in a Patient With Congenital Heart Defects and Heterotaxy Syndrome |journal=Ochsner J |volume=16 |issue=3 |pages=309–11 |date=2016 |pmid=27660582 |pmc=5024815 |doi= |url=}}</ref>
It accounts for almost all cases presenting in the intrauterine and [[neonatal]] period.  Other causes include:


*[[Myocarditis]]
*[[Myocarditis]]
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*[[Hurler syndrome|Hurler cardiomyopathy]]
*[[Hurler syndrome|Hurler cardiomyopathy]]
*[[Polysplenia]] with atrioventricular canal defect
*[[Polysplenia]] with atrioventricular canal defect
*In some patients, congenital heart block is a secondary characteristic disorders or heart muscle tumors (myocardium).<ref name="pmid22183063">{{cite journal |vauthors=Capone C, Buyon JP, Friedman DM, Frishman WH |title=Cardiac manifestations of neonatal lupus: a review of autoantibody-associated congenital heart block and its impact in an adult population |journal=Cardiol Rev |volume=20 |issue=2 |pages=72–6 |date=2012 |pmid=22183063 |pmc=3275696 |doi=10.1097/CRD.0b013e31823c808b |url=}}</ref>
*In some patients, congenital heart block is a secondary characteristic disorders or heart muscle tumors (myocardium).


==Differentiating [disease name] from other Diseases==
==Differentiating [disease name] from other Diseases==
*Congenital heart block must be differentiated from other [[Disease|diseases]]:<ref name="LipshultzLaw2019">{{cite journal|last1=Lipshultz|first1=Steven E.|last2=Law|first2=Yuk M.|last3=Asante-Korang|first3=Alfred|last4=Austin|first4=Eric D.|last5=Dipchand|first5=Anne I.|last6=Everitt|first6=Melanie D.|last7=Hsu|first7=Daphne T.|last8=Lin|first8=Kimberly Y.|last9=Price|first9=Jack F.|last10=Wilkinson|first10=James D.|last11=Colan|first11=Steven D.|title=Cardiomyopathy in Children: Classification and Diagnosis: A Scientific Statement From the American Heart Association|journal=Circulation|volume=140|issue=1|year=2019|issn=0009-7322|doi=10.1161/CIR.0000000000000682}}</ref>
*Congenital heart block must be differentiated from other [[Disease|diseases]]:
**[[Pediatrics|Pediatric]] [[viral myocarditis]]
**[[Pediatrics|Pediatric]] [[viral myocarditis]]
**[[Transposition of the great vessels|Transposition of the Great Arteries]]
**[[Transposition of the great vessels|Transposition of the Great Arteries]]


==Epidemiology and Demographics==
==Epidemiology and Demographics==
* The prevalence of congenital heart block is approximately 1 per 22,00 live births individuals worldwide. <ref>{{Cite journal
* The prevalence of congenital heart block is approximately 1 per 22,00 live births individuals worldwide.  
| author = [[Dm Friedman]], [[Lj Duncanson]], [[J. Glickstein]] & [[Jp Buyon]]
| title = A review of congenital heart block
| journal = [[Images in paediatric cardiology]]
| volume = 5
| issue = 3
| pages = 36–48
| year = 2003
| month = July
| pmid = 22368629
}}</ref><ref>{{Cite journal
| author = [[Pilar Brito-Zeron]], [[Peter M. Izmirly]], [[Manuel Ramos-Casals]], [[Jill P. Buyon]] & [[Munther A. Khamashta]]
| title = The clinical spectrum of autoimmune congenital heart block
| journal = [[Nature reviews. Rheumatology]]
| volume = 11
| issue = 5
| pages = 301–312
| year = 2015
| month = May
| doi = 10.1038/nrrheum.2015.29
| pmid = 25800217
}}</ref>
* In [year], the incidence of [disease name] was estimated to be [number or range] cases per 100,000 individuals in [location].
* In [year], the incidence of [disease name] was estimated to be [number or range] cases per 100,000 individuals in [location].
   
   
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==Risk Factors==
==Risk Factors==


* One form of congenital heart block occurs in babies whose mothers have autoimmune diseases, such as [[SLE|lupus]].<ref name="pmid223686293">{{cite journal |vauthors=Friedman D, Duncanson Lj, Glickstein J, Buyon J |title=A review of congenital heart block |journal=Images Paediatr Cardiol |volume=5 |issue=3 |pages=36–48 |date=July 2003 |pmid=22368629 |pmc=3232542 |doi= |url=}}</ref>
* One form of congenital heart block occurs in babies whose mothers have autoimmune diseases, such as [[SLE|lupus]].
* Patients with congenital heart block make proteins called antibodies that attack and damage the body's tissues or cells.<ref name="pmid223686293" />
* Patients with congenital heart block make proteins called antibodies that attack and damage the body's tissues or cells.<ref name="pmid223686293" />


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== Diagnosis ==
== Diagnosis ==


*The [[prenatal]] diagnosis of congenital heart block is more common. <ref name="pmid22368629">{{cite journal |vauthors=Friedman D, Duncanson Lj, Glickstein J, Buyon J |title=A review of congenital heart block |journal=Images Paediatr Cardiol |volume=5 |issue=3 |pages=36–48 |date=July 2003 |pmid=22368629 |pmc=3232542 |doi= |url=}}</ref><ref name="pmid30012852">{{cite journal |vauthors=Hunter LE, Seale AN |title=EDUCATIONAL SERIES IN CONGENITAL HEART DISEASE: Prenatal diagnosis of congenital heart disease |journal=Echo Res Pract |volume=5 |issue=3 |pages=R81–R100 |date=September 2018 |pmid=30012852 |pmc=6107762 |doi=10.1530/ERP-18-0027 |url=}}</ref><ref name="pmid223686292">{{cite journal |vauthors=Friedman D, Duncanson Lj, Glickstein J, Buyon J |title=A review of congenital heart block |journal=Images Paediatr Cardiol |volume=5 |issue=3 |pages=36–48 |date=July 2003 |pmid=22368629 |pmc=3232542 |doi= |url=}}</ref>
*The [[prenatal]] diagnosis of congenital heart block is more common.  


:*[[Fetal]] [[echocardiography]] (with [[doppler]] techniques)
:*[[Fetal]] [[echocardiography]] (with [[doppler]] techniques)
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:*[[Cardiac]] [[imaging]] techniques
:*[[Cardiac]] [[imaging]] techniques


*[[Diagnosis]] of congenital heart block is confirmed by [[maternal]] [[fetal]] monitoring (MFM) in during [[infancy]] or early [[childhood]].<ref name="DonofrioMoon-Grady2014">{{cite journal|last1=Donofrio|first1=Mary T.|last2=Moon-Grady|first2=Anita J.|last3=Hornberger|first3=Lisa K.|last4=Copel|first4=Joshua A.|last5=Sklansky|first5=Mark S.|last6=Abuhamad|first6=Alfred|last7=Cuneo|first7=Bettina F.|last8=Huhta|first8=James C.|last9=Jonas|first9=Richard A.|last10=Krishnan|first10=Anita|last11=Lacey|first11=Stephanie|last12=Lee|first12=Wesley|last13=Michelfelder|first13=Erik C.|last14=Rempel|first14=Gwen R.|last15=Silverman|first15=Norman H.|last16=Spray|first16=Thomas L.|last17=Strasburger|first17=Janette F.|last18=Tworetzky|first18=Wayne|last19=Rychik|first19=Jack|title=Diagnosis and Treatment of Fetal Cardiac Disease|journal=Circulation|volume=129|issue=21|year=2014|pages=2183–2242|issn=0009-7322|doi=10.1161/01.cir.0000437597.44550.5d}}</ref>
*[[Diagnosis]] of congenital heart block is confirmed by [[maternal]] [[fetal]] monitoring (MFM) in during [[infancy]] or early [[childhood]].
*First degree:<ref name="KusumotoSchoenfeld2019">{{cite journal|last1=Kusumoto|first1=Fred M.|last2=Schoenfeld|first2=Mark H.|last3=Barrett|first3=Coletta|last4=Edgerton|first4=James R.|last5=Ellenbogen|first5=Kenneth A.|last6=Gold|first6=Michael R.|last7=Goldschlager|first7=Nora F.|last8=Hamilton|first8=Robert M.|last9=Joglar|first9=José A.|last10=Kim|first10=Robert J.|last11=Lee|first11=Richard|last12=Marine|first12=Joseph E.|last13=McLeod|first13=Christopher J.|last14=Oken|first14=Keith R.|last15=Patton|first15=Kristen K.|last16=Pellegrini|first16=Cara N.|last17=Selzman|first17=Kimberly A.|last18=Thompson|first18=Annemarie|last19=Varosy|first19=Paul D.|title=2018 ACC/AHA/HRS Guideline on the Evaluation and Management of Patients With Bradycardia and Cardiac Conduction Delay|journal=Journal of the American College of Cardiology|volume=74|issue=7|year=2019|pages=e51–e156|issn=07351097|doi=10.1016/j.jacc.2018.10.044}}</ref>
*First degree:
**Patient [[asymptomatc]]
**Patient [[asymptomatc]]
*Second degree:
*Second degree:
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=== Symptoms ===
=== Symptoms ===
*[[Signs]] and [[symptoms]] depend on the type of heart block the child has. [[First-degree heart block]] rarely causes [[symptom]]<nowiki/>s.<ref name="BaruteauPass20162">{{cite journal|last1=Baruteau|first1=Alban-Elouen|last2=Pass|first2=Robert H.|last3=Thambo|first3=Jean-Benoit|last4=Behaghel|first4=Albin|last5=Le Pennec|first5=Solène|last6=Perdreau|first6=Elodie|last7=Combes|first7=Nicolas|last8=Liberman|first8=Leonardo|last9=McLeod|first9=Christopher J.|title=Congenital and childhood atrioventricular blocks: pathophysiology and contemporary management|journal=European Journal of Pediatrics|volume=175|issue=9|year=2016|pages=1235–1248|issn=0340-6199|doi=10.1007/s00431-016-2748-0}}</ref>
*[[Signs]] and [[symptoms]] depend on the type of heart block the child has. [[First-degree heart block]] rarely causes [[symptom]]<nowiki/>s.
*[[Symptom|Symptoms]] of [[second-degree heart block|second-]] and [[third-degree heart block]] include:
*[[Symptom|Symptoms]] of [[second-degree heart block|second-]] and [[third-degree heart block]] include:
*First degree:<ref name="KusumotoSchoenfeld2019" />
*First degree:<ref name="KusumotoSchoenfeld2019" />
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=== Physical Examination ===
=== Physical Examination ===
*[[Patient|Patients]] with congenital heart block usually appear :<ref name="pmid223686294">{{cite journal |vauthors=Friedman D, Duncanson Lj, Glickstein J, Buyon J |title=A review of congenital heart block |journal=Images Paediatr Cardiol |volume=5 |issue=3 |pages=36–48 |date=July 2003 |pmid=22368629 |pmc=3232542 |doi= |url=}}</ref>
*[[Patient|Patients]] with congenital heart block usually appear :


:*Annular lesions (Face, particularly around eyes)
:*Annular lesions (Face, particularly around eyes)
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=== Laboratory Findings ===
=== Laboratory Findings ===
*There are no specific [[laboratory]] findings associated with congenital heart block.<ref name="BrucatoGrava2009">{{cite journal|last1=Brucato|first1=Antonio|last2=Grava|first2=Chiara|last3=Bortolati|first3=Maria|last4=Ikeda|first4=Keigo|last5=Milanesi|first5=Ornella|last6=Cimaz|first6=Rolando|last7=Ramoni|first7=Veronique|last8=Vignati|first8=Gabriele|last9=Martinelli|first9=Stefano|last10=Sadou|first10=Youcef|last11=Borghi|first11=Adele|last12=Tincani|first12=Angela|last13=Chan|first13=Edward K.L.|last14=Ruffatti|first14=Amelia|title=Congenital Heart Block Not Associated with Anti-Ro/La Antibodies: Comparison with Anti-Ro/La-positive Cases|journal=The Journal of Rheumatology|volume=36|issue=8|year=2009|pages=1744–1748|issn=0315-162X|doi=10.3899/jrheum.080737}}</ref>
*There are no specific [[laboratory]] findings associated with congenital heart block.
*May be [[Autoantibody|autoantibodies]] to Ro/SSA and La/SSB [[Ribonucleoprotein|ribonucleoproteins]] using ELISAW are helpful when when congenital heart block  detected.<ref name="pmid19567621">{{cite journal |vauthors=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 |title=Congenital heart block not associated with anti-Ro/La antibodies: comparison with anti-Ro/La-positive cases |journal=J. Rheumatol. |volume=36 |issue=8 |pages=1744–8 |date=August 2009 |pmid=19567621 |pmc=2798588 |doi=10.3899/jrheum.080737 |url=}}</ref>
*May be [[Autoantibody|autoantibodies]] to Ro/SSA and La/SSB [[Ribonucleoprotein|ribonucleoproteins]] using ELISAW are helpful when when congenital heart block  detected.
*Other laboratory findings consistent with the diagnosis of congenital heart block include Sera, ELISA, counter-immunoelectrophoresis (CIE) method.<ref name="pmid19567621" /><ref name="pmid6982030">{{cite journal |vauthors=Bernstein RM, Bunn CC, Hughes GR |title=Identification of antibodies to acidic antigens by counterimmunoelectrophoresis |journal=Ann. Rheum. Dis. |volume=41 |issue=5 |pages=554–5 |date=October 1982 |pmid=6982030 |pmc=1001043 |doi=10.1136/ard.41.5.554 |url=}}</ref><ref name="pmid1985112">{{cite journal |vauthors=Chan EK, Hamel JC, Buyon JP, Tan EM |title=Molecular definition and sequence motifs of the 52-kD component of human SS-A/Ro autoantigen |journal=J. Clin. Invest. |volume=87 |issue=1 |pages=68–76 |date=January 1991 |pmid=1985112 |pmc=294993 |doi=10.1172/JCI115003 |url=}}</ref>
*Other laboratory findings consistent with the diagnosis of congenital heart block include Sera, ELISA, counter-immunoelectrophoresis (CIE) method.<ref name="pmid19567621" />


== Treatment ==
== Treatment ==
=== Medical Therapy ===
=== Medical Therapy ===
   
   
*Supportive [[therapy]] for congenital heart block includes [[symptomatic]] [[patients]].<ref name="ClowseEudy2018">{{cite journal|last1=Clowse|first1=Megan E B|last2=Eudy|first2=Amanda M|last3=Kiernan|first3=Elizabeth|last4=Williams|first4=Matthew R|last5=Bermas|first5=Bonnie|last6=Chakravarty|first6=Eliza|last7=Sammaritano|first7=Lisa R|last8=Chambers|first8=Christina D|last9=Buyon|first9=Jill|title=The prevention, screening and treatment of congenital heart block from neonatal lupus: a survey of provider practices|journal=Rheumatology|volume=57|issue=suppl_5|year=2018|pages=v9–v17|issn=1462-0324|doi=10.1093/rheumatology/key141}}</ref>
*Supportive [[therapy]] for congenital heart block includes [[symptomatic]] [[patients]].
*The mainstay of [[in utero]] therapy for congenital heart block is [[corticosteroids]].<ref>{{Cite journal
*The mainstay of [[in utero]] therapy for congenital heart block is [[corticosteroids]].
| author = [[Dm Friedman]], [[Lj Duncanson]], [[J. Glickstein]] & [[Jp Buyon]]
*[[Corticosteroids]] act by decreasing [[inflammation]] and damage caused by maternal antibodies.
| title = A review of congenital heart block
| journal = [[Images in paediatric cardiology]]
| volume = 5
| issue = 3
| pages = 36–48
| year = 2003
| month = July
| pmid = 22368629
}}</ref><ref>{{Cite journal
| author = [[Kai-Yu Zhou]] & [[Yi-Min Hua]]
| title = Autoimmune-associated Congenital Heart Block: A New Insight in Fetal Life
| journal = [[Chinese medical journal]]
| volume = 130
| issue = 23
| pages = 2863–2871
| year = 2017
| month = December
| doi = 10.4103/0366-6999.219160
| pmid = 29176145
}}</ref>
*[[Corticosteroids]] act by decreasing [[inflammation]] and damage caused by maternal antibodies.<ref>{{Cite journal
| author = [[Aurelie Ambrosi]] & [[Marie Wahren-Herlenius]]
| title = Congenital heart block: evidence for a pathogenic role of maternal autoantibodies
| journal = [[Arthritis research & therapy]]
| volume = 14
| issue = 2
| pages = 208
| year = 2012
| month = April
| doi = 10.1186/ar3787
| pmid = 22546326
}}</ref>


*Response to [[Corticosteroids]] can be monitored with fetal [[echocardiogram]] every few weeks.<ref>{{Cite journal
*Response to [[Corticosteroids]] can be monitored with fetal [[echocardiogram]] every few weeks.
| author = [[Dm Friedman]], [[Lj Duncanson]], [[J. Glickstein]] & [[Jp Buyon]]
| title = A review of congenital heart block
| journal = [[Images in paediatric cardiology]]
| volume = 5
| issue = 3
| pages = 36–48
| year = 2003
| month = July
| pmid = 22368629
}}</ref>


=== Surgery ===
=== Surgery ===
*Surgery is the mainstay of therapy for Congenital Heart Block.<ref>{{Cite journal
*Surgery is the mainstay of therapy for Congenital Heart Block.
| author = [[Dm Friedman]], [[Lj Duncanson]], [[J. Glickstein]] & [[Jp Buyon]]
*[[Cardiac pacemaker]] implantation in conjunction with [[Corticosteroids]] is the most common approach to the treatment of Congenital Heart Disease.
| title = A review of congenital heart block
*[[Cardiac pacemaker]] implantation can only be performed for patients with [[Mobitz Type II]] and above Congenital Heart Block.
| journal = [[Images in paediatric cardiology]]
| volume = 5
| issue = 3
| pages = 36–48
| year = 2003
| month = July
| pmid = 22368629
}}</ref>
*[[Cardiac pacemaker]] implantation in conjunction with [[Corticosteroids]] is the most common approach to the treatment of Congenital Heart Disease.<ref>{{Cite journal
| author = [[Dm Friedman]], [[Lj Duncanson]], [[J. Glickstein]] & [[Jp Buyon]]
| title = A review of congenital heart block
| journal = [[Images in paediatric cardiology]]
| volume = 5
| issue = 3
| pages = 36–48
| year = 2003
| month = July
| pmid = 22368629
}}</ref><ref>{{Cite journal
| author = [[Kai-Yu Zhou]] & [[Yi-Min Hua]]
| title = Autoimmune-associated Congenital Heart Block: A New Insight in Fetal Life
| journal = [[Chinese medical journal]]
| volume = 130
| issue = 23
| pages = 2863–2871
| year = 2017
| month = December
| doi = 10.4103/0366-6999.219160
| pmid = 29176145
}}</ref>
*[[Cardiac pacemaker]] implantation can only be performed for patients with [[Mobitz Type II]] and above Congenital Heart Block.<ref>{{Cite journal
| author = [[Kai-Yu Zhou]] & [[Yi-Min Hua]]
| title = Autoimmune-associated Congenital Heart Block: A New Insight in Fetal Life
| journal = [[Chinese medical journal]]
| volume = 130
| issue = 23
| pages = 2863–2871
| year = 2017
| month = December
| doi = 10.4103/0366-6999.219160
| pmid = 29176145
}}</ref>
=== Prevention ===
=== Prevention ===
*There are no primary preventive measures available for Congenital Heart Disease.<ref name="JenkinsBotto2019">{{cite journal|last1=Jenkins|first1=Kathy J.|last2=Botto|first2=Lorenzo D.|last3=Correa|first3=Adolfo|last4=Foster|first4=Elyse|last5=Kupiec|first5=Jennifer K.|last6=Marino|first6=Bradley S.|last7=Oster|first7=Matthew E.|last8=Stout|first8=Karen K.|last9=Honein|first9=Margaret A.|title=Public Health Approach to Improve Outcomes for Congenital Heart Disease Across the Life Span|journal=Journal of the American Heart Association|volume=8|issue=8|year=2019|issn=2047-9980|doi=10.1161/JAHA.118.009450}}</ref>
*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.<ref name="TunksClowse2013">{{cite journal|last1=Tunks|first1=Robert D.|last2=Clowse|first2=Megan E.B.|last3=Miller|first3=Stephen G.|last4=Brancazio|first4=Leo R.|last5=Barker|first5=Piers C.A.|title=Maternal autoantibody levels in congenital heart block and potential prophylaxis with antiinflammatory agents|journal=American Journal of Obstetrics and Gynecology|volume=208|issue=1|year=2013|pages=64.e1–64.e7|issn=00029378|doi=10.1016/j.ajog.2012.09.020}}</ref><ref name="SaxenaIzmirly2015">{{cite journal|last1=Saxena|first1=Amit|last2=Izmirly|first2=Peter M.|last3=Han|first3=Sung Won|last4=Briassouli|first4=Paraskevi|last5=Rivera|first5=Tania L.|last6=Zhong|first6=Hua|last7=Friedman|first7=Deborah M.|last8=Clancy|first8=Robert M.|last9=Buyon|first9=Jill P.|title=Serum Biomarkers of Inflammation, Fibrosis, and Cardiac Function in Facilitating Diagnosis, Prognosis, and Treatment of Anti-SSA/Ro–Associated Cardiac Neonatal Lupus|journal=Journal of the American College of Cardiology|volume=66|issue=8|year=2015|pages=930–939|issn=07351097|doi=10.1016/j.jacc.2015.06.1088}}</ref>
*Fetus with second or third degree heart block are less in women who take HCQ.


*Women of childbearing age with systemic autoimmune disorders should be tested fo anti-Ro and anti-La antibodies.<ref>{{Cite journal
*Women of childbearing age with systemic autoimmune disorders should be tested fo anti-Ro and anti-La antibodies.
| author = [[Dm Friedman]], [[Lj Duncanson]], [[J. Glickstein]] & [[Jp Buyon]]
*Effective measures to decrease the severity of Congenital Heart Block include [[in utero]] administration of [[Corticosteroids]] and [[plasmapheresis]].  
| title = A review of congenital heart block
*Once diagnosed and successfully treated, patients with Congenital Heart Block are followed-up regularly. Follow-up testing includes fetal [[echocardiogram]], [[electcrocardiogram]], and serologic [[antibodies]].
| journal = [[Images in paediatric cardiology]]
| volume = 5
| issue = 3
| pages = 36–48
| year = 2003
| month = July
| pmid = 22368629
}}</ref><ref>{{Cite journal
| author = [[Pilar Brito-Zeron]], [[Peter M. Izmirly]], [[Manuel Ramos-Casals]], [[Jill P. Buyon]] & [[Munther A. Khamashta]]
| title = The clinical spectrum of autoimmune congenital heart block
| journal = [[Nature reviews. Rheumatology]]
| volume = 11
| issue = 5
| pages = 301–312
| year = 2015
| month = May
| doi = 10.1038/nrrheum.2015.29
| pmid = 25800217
}}</ref>
*Effective measures to decrease the severity of Congenital Heart Block include [[in utero]] administration of [[Corticosteroids]] and [[plasmapheresis]].<ref>{{Cite journal
| author = [[Dm Friedman]], [[Lj Duncanson]], [[J. Glickstein]] & [[Jp Buyon]]
| title = A review of congenital heart block
| journal = [[Images in paediatric cardiology]]
| volume = 5
| issue = 3
| pages = 36–48
| year = 2003
| month = July
| pmid = 22368629
}}</ref><ref>{{Cite journal
| author = [[Pilar Brito-Zeron]], [[Peter M. Izmirly]], [[Manuel Ramos-Casals]], [[Jill P. Buyon]] & [[Munther A. Khamashta]]
| title = The clinical spectrum of autoimmune congenital heart block
| journal = [[Nature reviews. Rheumatology]]
| volume = 11
| issue = 5
| pages = 301–312
| year = 2015
| month = May
| doi = 10.1038/nrrheum.2015.29
| pmid = 25800217
}}</ref> <ref>{{Cite journal
| author = [[Kai-Yu Zhou]] & [[Yi-Min Hua]]
| title = Autoimmune-associated Congenital Heart Block: A New Insight in Fetal Life
| journal = [[Chinese medical journal]]
| volume = 130
| issue = 23
| pages = 2863–2871
| year = 2017
| month = December
| doi = 10.4103/0366-6999.219160
| pmid = 29176145
}}</ref>
*Once diagnosed and successfully treated, patients with Congenital Heart Block are followed-up regularly. Follow-up testing includes fetal [[echocardiogram]], [[electcrocardiogram]], and serologic [[antibodies]].<ref>{{Cite journal
| author = [[Dm Friedman]], [[Lj Duncanson]], [[J. Glickstein]] & [[Jp Buyon]]
| title = A review of congenital heart block
| journal = [[Images in paediatric cardiology]]
| volume = 5
| issue = 3
| pages = 36–48
| year = 2003
| month = July
| pmid = 22368629
}}</ref><ref>{{Cite journal
| author = [[Pilar Brito-Zeron]], [[Peter M. Izmirly]], [[Manuel Ramos-Casals]], [[Jill P. Buyon]] & [[Munther A. Khamashta]]
| title = The clinical spectrum of autoimmune congenital heart block
| journal = [[Nature reviews. Rheumatology]]
| volume = 11
| issue = 5
| pages = 301–312
| year = 2015
| month = May
| doi = 10.1038/nrrheum.2015.29
| pmid = 25800217
}}</ref>




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Revision as of 17:50, 24 February 2020

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sogand Goudarzi, MD [2] Cafer Zorkun, M.D., Ph.D. [3]; 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:
  • 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:

  • 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.

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:

  • Myocarditis
  • Mother's antibodies cross from the fetus placenta and damage of cardiac conduction system
  • Genetic disorde (autosomal recessive/dominant genetic trait)[1]
  • Levo transposition of great arteries
  • Atrioventricular discordance
  • Hurler cardiomyopathy
  • Polysplenia with atrioventricular canal defect
  • In some patients, congenital heart block is a secondary characteristic disorders or heart muscle tumors (myocardium).

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.
  • 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.
  • Patients with congenital heart block make proteins called antibodies that attack and damage the body's tissues or cells.[2]

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.


Diagnosis

  • The prenatal diagnosis of congenital heart block is more common.


Symptoms

Physical Examination

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

Laboratory Findings

  • There are no specific laboratory findings associated with congenital heart block.
  • May be autoantibodies to Ro/SSA and La/SSB ribonucleoproteins using ELISAW are helpful when when congenital heart block detected.
  • Other laboratory findings consistent with the diagnosis of congenital heart block include Sera, ELISA, counter-immunoelectrophoresis (CIE) method.[4]

Treatment

Medical Therapy

Surgery

  • Surgery is the mainstay of therapy for Congenital Heart Block.
  • Cardiac pacemaker implantation in conjunction with Corticosteroids is the most common approach to the treatment of Congenital Heart Disease.
  • Cardiac pacemaker implantation can only be performed for patients with Mobitz Type II and above Congenital Heart Block.

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.
  • Women of childbearing age with systemic autoimmune disorders should be tested fo anti-Ro and anti-La antibodies.
  • Effective measures to decrease the severity of Congenital Heart Block include in utero administration of Corticosteroids and plasmapheresis.
  • Once diagnosed and successfully treated, patients with Congenital Heart Block are followed-up regularly. Follow-up testing includes fetal echocardiogram, electcrocardiogram, and serologic antibodies.


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.
  • 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.

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  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.
  2. ↑
  3. ↑
  4. ↑
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