Cardiac Conduction System Disease

Diseases characterized by pathological irregularities in the HEART CONDUCTION SYSTEM. They may be associated with other heart diseases and syndromes (e.g., BRUGADA SYNDROME; NEUROMUSCULAR DISEASE, HEART BLOCKS), isolated or may result from injuries. You can have a conduction disorder without having an arrhythmia, but some arrhythmias arise from conduction disorders. OMIM: 601144.

Also Known As:

Networked: 93 relevant articles (3 outcomes, 1 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Muscle Weakness
2.	Chronic Kidney Failure (Chronic Renal Failure)
3.	Tachycardia (Tachyarrhythmias)
4.	Brugada Syndrome
5.	Myotonic Dystrophy (Dystrophia Myotonica)

Experts

1.	Mahadevan, Mani S: 4 articles (01/2020 - 09/2006)
2.	Yadava, Ramesh S: 4 articles (01/2020 - 09/2006)
3.	Yu, Qing: 4 articles (01/2020 - 09/2006)
4.	Wolf, Cordula M: 3 articles (01/2020 - 04/2006)
5.	Fishman, Glenn I: 2 articles (01/2022 - 02/2008)
6.	McNally, Elizabeth M: 2 articles (01/2022 - 07/2003)
7.	Choquet, Caroline: 2 articles (08/2021 - 12/2019)
8.	Miquerol, Lucile: 2 articles (08/2021 - 12/2019)
9.	Mandal, Mahua: 2 articles (01/2020 - 01/2019)
10.	Nakamori, Masayuki: 2 articles (12/2019 - 03/2014)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Cardiac Conduction System Disease:

1.	Lamin Type A (Lamin A)IBA 03/01/2017 - "A major gene associated with DCM with cardiac conduction system disease is lamin A/C (LMNA) gene. " 07/10/2003 - "Mutations in the gene encoding lamin A/C can lead to significant cardiac conduction system disease that can be successfully treated with pacemakers and/or defibrillators. " 07/06/2023 - "Like mice with whole body or cardiomyocyte-specific lamin A/C ablation, Lmnaflox/floxSM22αCre mice recapitulated the main hallmarks of human LMNA-DCM, including ventricular systolic dysfunction, cardiac conduction defects, cardiac fibrosis, and premature death. " 02/01/2008 - "Lamin A/C haploinsufficiency causes dilated cardiomyopathy and apoptosis-triggered cardiac conduction system disease." 01/01/2019 - "Emery-Dreifuss muscular dystrophy, caused by mutations in genes such as emerin (EMD) or lamin A/C (LMNA), is a disorder affecting the joints, muscles, and heart, with a wide spectrum of patient phenotypes including muscle wasting and cardiac conduction defects. "
2.	Neuromuscular Agents (Skeletal Muscle Relaxants)IBA 01/01/2018 - "Severe hypermagnesaemia is known to cause cardiac conduction defects, neuromuscular effects and muscle weakness; a slightly elevated Mg has been suggested to be beneficial in patients with end-stage renal disease. "
3.	Sodium Channels (Sodium Channel)IBA 04/01/2006 - "Human genetic studies have identified mutations in the sodium channel SCN5A gene causing tachyarrhythmia disorders, as well as progressive cardiac conduction system diseases, or overlapping syndromes. " 01/01/2007 - "Some mutations of the sodium channel gene Na(V1.5) are multifunctional, causing combinations of LQTS, Brugada syndrome and progressive cardiac conduction system disease (PCCD). " 01/01/2007 - "Sodium channel kinetic changes that produce Brugada syndrome or progressive cardiac conduction system disease." 11/01/2009 - "The mutation carriers show a unique overlap of long-QT syndrome (type 3), Brugada syndrome and progressive cardiac conduction defects attributed to a single mutation in the cardiac sodium channel gene SCN5a. " 05/01/2005 - "Mutations in the cardiac sodium channel, SCN5A, have been associated with one type of long-QT syndrome, with isolated cardiac conduction defects and Brugada syndrome. "
4.	Proteins (Proteins, Gene)FDA Link 02/01/2009 - "Differential protein expression in heart in UT-B null mice with cardiac conduction defects." 01/01/2020 - "The patient is a 49-year-old male with KSS, diagnosed clinically upon the core features, namely, onset before the age 20 of years, pigmentary retinopathy, and ophthalmoparesis, and the complementary features, namely, elevated CSF protein, cardiac conduction defects, and cerebellar ataxia. " 02/15/2015 - "Kearns-Sayre syndrome (KSS) is a mitochondrial disorder characterised by onset before the age of 20years, progressive external ophthalmoplegia, and pigmentary retinopathy, accompanied by either cardiac conduction defects, elevated cerebrospinal fluid protein or cerebellar ataxia. " 02/01/2008 - "Mutations in the lamin A/C (LMNA) gene, which encodes nuclear membrane proteins, cause a variety of human conditions including dilated cardiomyopathy (DCM) with associated cardiac conduction system disease. " 02/01/2015 - "Mutations in the inner nuclear envelope protein emerin cause Emery-Dreifuss muscular dystrophy (EDMD), which is characterized by progressive skeletal muscle wasting, cardiac conduction defects, and tendon contractures. "
5.	Retinaldehyde (Retinal)IBA 10/15/1976 - "Apart from the chronic progressive external ophthalmoplegia the syndrome consists of retinal changes and cardiac conduction defects in all cases, as well as other signs indicating damage to the nervous system. " 11/01/1996 - "We report a 17-year-old girl with short stature, external ophthalmoplegia, atypical retinal pigmentary degeneration, sensorineural hearing loss, and cardiac conduction defect (Kearns-Sayre syndrome). " 04/01/1988 - "The Kearns-Sayre syndrome is a mitochondrial myopathy characterised by ptosis, chronic progressive external ophthalmoplegia, abnormal retinal pigmentation, and cardiac conduction defects. " 11/01/2016 - "Kearns-Sayre syndrome (KSS) is characterized by external ophthalmoplegia, retinal pigmentation and cardiac conduction defects due to mitochondrial DNA (mtDNA) deletions. " 01/01/2007 - "The triad of progressive external ophthalmoplegia, atypical retinal pigmentation and cardiac conduction defects characterizes Kearns-Sayre syndrome (KSS), which is most often caused by a single, large deletion of mitochondrial DNA. "
6.	Ion Channels (Ion Channel)IBA 01/01/2019 - "In the following sections, we describe the genetic disorders associated with primary cardiac conduction defects, primarily caused by mutations in ion channel genes. " 03/01/2014 - "[Myotonia and cardiac conduction defects in myotonic dystrophy and defect in ion channels]." 07/27/2002 - "In the long QT syndrome and Brugada syndrome, mutations in ion channel genes can cause the disease; one of those genes is also implicated in progressive cardiac conduction defect. " 07/01/2008 - "The inherited cardiac arrhythmias including congenital and acquired long QT syndrome (LQTS), Brugada syndrome, progressive cardiac conduction defect, catecholaminergic polymorphic ventricular tachycardia, arrhythmogenic right ventricular cardiomyopathy, familial atrial fibrillation, familial sick sinus syndrome and short QT syndrome, are linked to mutations in genes encoding for ion channels or other membrane components. " 01/01/2006 - "Primary cardiac conduction defects are mainly caused by mutations in genes involved in impulse conduction processes (e.g., gap-junction genes and transcription factors) or repolarisation processes (e.g., ion-channel genes), whereas primary cardiomyopathies are mainly caused by defective force generation or force transmission due to gene mutations in either sarcomeric or cytoskeleton proteins. "
7.	Mitochondrial DNA (mtDNA)IBA 02/01/1997 - "In particular, a cardiac conduction defect was present in 12 patients out of 18 with a multisystemic disease associated with a mitochondrial DNA deletion; it was never encountered in 17 patients with a multisystemic disease but no mitochondrial DNA deletion." 11/01/2016 - "Kearns-Sayre syndrome (KSS) is characterized by external ophthalmoplegia, retinal pigmentation and cardiac conduction defects due to mitochondrial DNA (mtDNA) deletions. " 01/01/2007 - "The triad of progressive external ophthalmoplegia, atypical retinal pigmentation and cardiac conduction defects characterizes Kearns-Sayre syndrome (KSS), which is most often caused by a single, large deletion of mitochondrial DNA. " 10/01/2019 - "Kearns-Sayre syndrome (KSS) is a rare mitochondrial DNA (mtDNA) deletion syndrome that typically presents before 20 years of age and is characterized by chronic progressive external ophthalmoplegia, pigmentary retinopathy, and a combination of cardiac conduction defects, cerebellar ataxia, and elevated cerebrospinal fluid protein levels. " 04/01/2013 - "Kearns-Sayre syndrome (KSS) is a rare mitochondrial DNA deletion syndrome defined as the presence of ophthalmoplegia, pigmentary retinopathy, onset less than age 20 years, and one of the following: cardiac conduction defects, cerebellar syndrome, or cerebrospinal fluid protein above 100 mg/dl. "
8.	RNA (Ribonucleic Acid)IBA 12/12/2019 - "Cardiac conduction defects decrease life expectancy in myotonic dystrophy type 1 (DM1), a CTG repeat disorder involving misbalance between two RNA binding factors, MBNL1 and CELF1. " 09/01/2006 - "Reversible model of RNA toxicity and cardiac conduction defects in myotonic dystrophy." 03/01/2014 - "Here, we review the cardiac manifestation and RNA-dominant mechanism of DM, and discuss the possible pathophysiology of cardiac conduction defects by referring to hereditary arrhythmic diseases, such as long QT syndrome and Brugada syndrome." 01/01/2019 - "Using an inducible mouse model of RNA toxicity in which expression of the mutant DMPK 3'UTR results in RNA foci formation, MBNL1 sequestration, splicing defects, myotonia and cardiac conduction defects, we find that MBNL1 overexpression did not rescue skeletal muscle function nor beneficially affect cardiac conduction. "
9.	AutoantibodiesIBA 05/01/2017 - "The detection of cardiac conduction defects in an 18-24 week old foetus in the absence of structural abnormalities predicts with near certainty the presence of autoantibodies against 60kD and 52kD SSA/Ro in the mother regardless of her health status. " 06/01/1992 - "Forty-two babies with different congenital cardiac conduction defects, and in 12 cases the mothers, were tested for autoantibodies to Ro, La, U1RNP and Sm. " 06/01/1992 - "Our findings indicate that all pregnant women at risk for anti-Ro like connective tissue disease or cardiac conduction defects should be tested for these autoantibodies because of the suspicion of cardiac conduction abnormalities in the offspring. " 11/01/1992 - "Neonatal lupus erythematosus (NLE) is a distinct subset of lupus characterized by cutaneous findings (50%), cardiac conduction defects (50%), and autoantibodies to Ro (SS-A) antigen. " 03/01/2012 - "These data for the first time show unambiguously that antibodies specific for amino acids 200-239 of Ro52 can induce cardiac conduction defects in the absence of other autoantibodies, and may therefore be the main initiators of cardiac pathology in the pool of anti-Ro52 antibodies in mothers of children with CHB."
10.	emerinIBA 06/01/2006 - "The Emery-Dreifuss muscular dystrophy is a form of muscular dystrophy that frequently presents early contractures and cardiac conduction defects, caused by emerin deficiency in the inner nuclear membrane of the muscular fibers. " 01/01/2019 - "Emery-Dreifuss muscular dystrophy, caused by mutations in genes such as emerin (EMD) or lamin A/C (LMNA), is a disorder affecting the joints, muscles, and heart, with a wide spectrum of patient phenotypes including muscle wasting and cardiac conduction defects. " 02/01/2015 - "Mutations in the inner nuclear envelope protein emerin cause Emery-Dreifuss muscular dystrophy (EDMD), which is characterized by progressive skeletal muscle wasting, cardiac conduction defects, and tendon contractures. " 05/01/2001 - "New findings have shown that cytoskeletal proteins at the nuclear membrane, such as emerin and lamin AC, cause muscular dystrophy and cardiomyopathy with cardiac conduction system disease. "

Therapies and Procedures

1.	Defibrillators (Defibrillator) 07/10/2003 - "Mutations in the gene encoding lamin A/C can lead to significant cardiac conduction system disease that can be successfully treated with pacemakers and/or defibrillators. "
2.	Therapeutics 11/01/2004 - "Cardiac resynchronisation therapy (CRT) is a novel treatment option for patients with CCF and associated cardiac conduction defects. "
3.	Transcatheter Aortic Valve Replacement 06/01/2017 - "Transcatheter aortic valve implantation (TAVI) is frequently associated with cardiac conduction defects (CCD) requiring permanent pacemaker implantation (PPI). " 11/01/2012 - "Cardiac conduction system disease after transcatheter aortic valve replacement."
4.	Cardiopulmonary Resuscitation (CPR) 01/01/2019 - "The patient presented with a severe clinical phenotype including a markedly prolonged QTc interval (544 ms), recurrent syncope due to Torsade de Pointes tachycardias, survived cardiopulmonary resuscitation, progressive cardiac conduction defect, and atrial fibrillation. "
5.	Transplantation 01/01/2018 - "In vitro platform of allogeneic stem cell-derived cardiomyocyte transplantation for cardiac conduction defects."