Congenital heart block

A congenital condition of unknown etiology. It may be associated with maternal AUTOIMMUNE DISEASE or viral infection during pregnancy. OMIM: 234700

Also Known As:

Congenital Heart Block, Isolated; Congenital complete heart block; Familial congenital heart block; Heart Block, Congenital; Isolated Congenital Heart Block; Isolated Heart Block, Congenital

Networked: 446 relevant articles (10 outcomes, 26 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Heart Block
2.	Cardiotoxicity
3.	Heart Failure
4.	Premature Birth (Birth, Premature)
5.	Type 2 Diabetes Mellitus (MODY)

Experts

1.	Buyon, Jill P: 47 articles (10/2020 - 01/2002)
2.	Clancy, Robert M: 29 articles (01/2019 - 08/2002)
3.	Wahren-Herlenius, Marie: 20 articles (01/2022 - 05/2002)
4.	Sonesson, Sven-Erik: 15 articles (01/2022 - 01/2005)
5.	Friedman, Deborah M: 13 articles (10/2020 - 06/2002)
6.	Brucato, Antonio: 13 articles (01/2019 - 01/2002)
7.	Ruffatti, Amelia: 12 articles (01/2022 - 08/2009)
8.	Costedoat-Chalumeau, Nathalie: 11 articles (01/2021 - 10/2004)
9.	Cimaz, Rolando: 10 articles (01/2019 - 01/2002)
10.	Hoxha, Ariela: 9 articles (01/2022 - 07/2015)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Congenital heart block:

1.	Hydroxychloroquine (Plaquenil)FDA LinkGeneric 01/01/2020 - "(Preventive Approach to Congenital Heart Block With Hydroxychloroquine [PATCH]; NCT01379573)." 01/01/2020 - "Hydroxychloroquine to Prevent Recurrent Congenital Heart Block in Fetuses of Anti-SSA/Ro-Positive Mothers." 01/01/2019 - "Hydroxychloroquine (HCQ) appears protective against flares in pregnancy, neonatal congenital heart block and preterm birth. " 03/01/2014 - "Two retrospective analyses suggest that hydroxychloroquine may prevent congenital heart block in pregnancies exposed to SSA/Ro antibodies. " 10/01/2020 - "To provide data regarding any potential fetal/neonatal cardiotoxicity, we leveraged a unique opportunity in which neonatal ECGs and hydroxychloroquine blood levels were available in a recently completed study evaluating the efficacy of hydroxychloroquine 400 mg daily to prevent the recurrence of congenital heart block associated with anti-SSA/Ro (anti-Sjögren's Syndrome A/Ro) antibodies. "
2.	AntibodiesIBA 07/01/2013 - "Remission of congenital complete heart block without anti-Ro/La antibodies: A case report." 09/01/2006 - "Impaired clearance of apoptotic cardiocytes is linked to anti-SSA/Ro and -SSB/La antibodies in the pathogenesis of congenital heart block." 01/01/2016 - "We reviewed previous as well as most recent studies with the subject heading Sjogren in combination with antibodies and congenital heart block (CHB). " 12/01/2006 - "Recent studies have broadened our understanding of the immunological mechanism underlying congenital heart block induced by anti-Ro/La antibodies. " 03/01/2004 - "To allow detailed studies of the antibodies associated with congenital heart block, B-cell derived combinatorial antibody libraries from patients were screened for Ro52 and p200 specific antibody clones. "
3.	Intravenous Immunoglobulins (IVIG)FDA Link 09/01/2011 - "Association of the idiotype:antiidiotype antibody ratio with the efficacy of intravenous immunoglobulin treatment for the prevention of recurrent autoimmune-associated congenital heart block." 11/01/2015 - "Prevention of recurrent congenital heart block in a SSA/SSB positive mother using high dose maternal IVIG from 16-week gestation." 04/01/2010 - "Failure of intravenous immunoglobulin to prevent congenital heart block: Findings of a multicenter, prospective, observational study." 04/01/2010 - "Intravenous immunoglobulin does not prevent recurrence of congenital heart block in children of SSA/Ro-positive mothers." 04/01/2010 - "Evaluation of fetuses in a study of intravenous immunoglobulin as preventive therapy for congenital heart block: Results of a multicenter, prospective, open-label clinical trial."
4.	SS-A antibodiesIBA 05/01/2017 - "We traced the evolution of two anti-Ro52 autoantibodies isolated from circulating IgG1-switched B-cells from an asymptomatic mother of a child with third degree congenital heart block. " 01/01/2017 - "Anti-SS-A antibodies are associated with congenital heart block. " 07/01/2016 - "To determine the maternal predictive factors for fetal congenital heart block (CHB) in pregnancy in mothers positive for anti-SS-A antibodies. " 07/01/2016 - "Maternal predictive factors for fetal congenital heart block in pregnant mothers positive for anti-SS-A antibodies." 04/01/2016 - "Congenital heart block (CHB) may develop in fetuses of women with anti-Ro/SSA autoantibodies, and carries substantial morbidity and mortality. "
5.	Dexamethasone (Maxidex)FDA LinkGeneric 04/15/2009 - "We evaluated the efficacy of dexamethasone (DEX) in anti-SSA/Ro-exposed fetuses newly diagnosed with congenital heart block. " 04/15/2009 - "Prospective evaluation of fetuses with autoimmune-associated congenital heart block followed in the PR Interval and Dexamethasone Evaluation (PRIDE) Study." 01/29/2008 - "Utility of cardiac monitoring in fetuses at risk for congenital heart block: the PR Interval and Dexamethasone Evaluation (PRIDE) prospective study." 11/01/2006 - "Normal neuropsychological development in children with congenital complete heart block who may or may not be exposed to high-dose dexamethasone in utero." 10/01/2003 - "Questions about dexamethasone use for the prevention of anti-SSA related congenital heart block."
6.	Ritodrine (Yutopar)FDA Link 11/01/2002 - "Successful prenatal treatment of congenital heart block with ritodrine administered transplacentally." 01/01/1997 - "Fetal ventricular rate in case of congenital complete heart block is increased by ritodrine. " 01/01/2010 - "A case study of a pregnant patient with a congenital heart block accompanied by left isomerism and uncontrolled type 2 diabetes who was treated successfully with ritodrine."
7.	Antinuclear AntibodiesIBA 06/25/2021 - "Testing for antinuclear antibodies should be considered in every pregnant woman since early treatment with hydroxychloroquine or intravenous immunoglobulin (IVIG) has proven to be effective in preventing congenital heart block." 11/01/1989 - "We have examined the relationships between maternal connective tissue disease (CTD), fetal and neonatal health, and the presence of specific autoantibodies: antinuclear antibodies (ANA), anti-Ro, antiLa, anti-Sm, anti-RNP, anti-DNA, and anticardiolipin (ACL) in 27 mothers with CTD (Group A), and 10 asymptomatic mothers of babies with complete congenital heart block (CCHB), or cardiac arrhythmias (Group B). "
8.	Adrenergic Agents (Adrenergic Drugs)IBA 01/01/2001 - "Maternal administration of the beta-adrenergic agent terbutaline is suggested to be effective for improving fetal congenital heart block in order to prevent heart failure in utero."
9.	Terbutaline (Brethaire)FDA LinkGeneric 01/01/2001 - "Treatment of fetal congenital complete heart block with maternal administration of beta-sympathomimetics (terbutaline): a case report." 01/01/2001 - "Maternal administration of the beta-adrenergic agent terbutaline is suggested to be effective for improving fetal congenital heart block in order to prevent heart failure in utero."
10.	AutoantibodiesIBA 06/01/2014 - "The main aim of this study was to identify foetal susceptibility genes on chromosome six for Ro/SSA autoantibody-mediated congenital heart block. " 03/01/1994 - "Autoantibody, HLA studies and C4 phenotypes were performed on twins discordant for isolated congenital heart block. " 11/01/1993 - "To study the long-term outcome of mothers of children with isolated congenital heart block (CHB) and to characterize the maternal autoantibody response to SS-A/Ro and SS-B/La. " 09/01/2023 - "Our work, also, includes a detailed review of the accumulated evidence regarding the association between autoantibodies and congenital heart block, the available screening modalities of the condition, and its potential therapeutic interventions." 12/24/2021 - "[Detection and prevention of maternal autoantibody-related congenital heart block]."

Therapies and Procedures

1.	Therapeutics 01/01/2022 - "The short and long-term outcomes of children with anti-Ro/La-related congenital heart block treated with a combined maternal-neonatal therapy protocol were compared with those of controls treated with other therapies. " 01/01/2022 - "Short and long-term outcomes of children with autoimmune congenital heart block treated with a combined maternal-neonatal therapy. " 01/01/2018 - "Most asymptomatic patients with congenital complete heart block will eventually become symptomatic and require pacemakers at some point in their life but the definitive answer for the ideal time to initiate pacemaker therapy in such population has not been established. " 06/01/2010 - "Because of the variety of the clinical presentations, especially in regard to pacing therapy, it is mandatory to refer patients with congenital complete heart block to specialized centers with adequate resources and experienced personnel." 06/01/2010 - "Controversies in the therapy of congenital complete heart block are reviewed in terms of the timing of pacemaker implantation, the type and complications of pacing and its role in the presence of myocardial dysfunction. "
2.	Plasmapheresis 07/01/2016 - "Plasmapheresis, intravenous immunoglobulins and bethametasone - a combined protocol to treat autoimmune congenital heart block: a prospective cohort study." 08/01/2001 - "[Therapeutic plasmapheresis for the prevention of congenital complete heart block associated with anti-SS-A/Ro antibody and anti-SS-B/La antibody]." 08/01/1994 - "They should be closely followed during pregnancy to enable early detection of the development of congenital heart block, as prevention by plasmapheresis plus dexamethasone may be possible at an early stage." 01/01/2007 - "Effect of steroid administration and plasmapheresis to prevent fetal congenital heart block in patients with systemic lupus erythematosus and/or Sjögren's syndrome." 08/01/1992 - "Pregnant women at high risk with known connective tissue disease, previous children with congenital heart block, high titers of SS-A-/SS-B-antibodies and immunogenetic predisposition (HLA-DR3) have to be monitored intensively because of the possibility of a prophylaxis with dexamethasone and, in some selected cases, plasmapheresis."
3.	Cardiac Resynchronization Therapy 07/01/2023 - "An 8-year-old girl with a history of single-chamber epicardial pacemaker during infancy and cardiac resynchronization therapy with a His bundle pacing lead implantation six months earlier, presented with congenital complete heart block. " 10/01/2021 - "Ventricular pacing and myocardial function in patients with congenital heart block: Is it time to consider de novo biventricular pacing?" 05/01/2017 - "Patients with an implantable cardioverter-defibrillator, cardiac resynchronization therapy, or iatrogenic and/or congenital heart block were excluded. " 02/01/2008 - "Finally, cardiac resynchronization therapy may apply to children with congenital heart block and cardiomyopathy, as well as to the population with CHD. "
4.	Cesarean Section (Caesarean Section) 01/01/2012 - "Herein, we present a case of congenital complete heart block posted for elective caesarean section for an obstetric indication. " 01/01/2012 - "Congenital complete heart block and spinal anaesthesia for caesarean section." 11/01/2020 - "Congenital complete heart block in the setting of severe pre-eclampsia requiring urgent cesarean section." 04/01/2013 - "On bivariate analysis, severe preeclampsia, Caesarean section, newborn resuscitation for > 3 minutes, respiratory distress syndrome, assisted ventilation, bronchopulmonary dysplasia, patent ductus arteriosus, mild to moderate intraventricular hemorrhage, retinopathy of prematurity, and congenital heart block in neonates were significantly more frequent in the women with SLE. "
5.	Thoracotomy 01/01/2019 - "Congenital complete heart block requires pacemaker implantation at birth through thoracotomy, which can result in scoliosis. " 07/01/1994 - "Epicardial fetal pacing via thoracotomy has the potential of being a safer and more reliable procedure to treat congenital complete heart block (CHB) associated with fetal hydrops refractory to medical therapy. "