TY - JOUR
T1 - Congenital and childhood atrioventricular blocks
T2 - pathophysiology and contemporary management
AU - Baruteau, Alban Elouen
AU - Pass, Robert H.
AU - Thambo, Jean Benoit
AU - Behaghel, Albin
AU - Le Pennec, Solène
AU - Perdreau, Elodie
AU - Combes, Nicolas
AU - Liberman, Leonardo
AU - McLeod, Christopher J.
N1 - Publisher Copyright:
© 2016, The Author(s).
PY - 2016/9/1
Y1 - 2016/9/1
N2 - Atrioventricular block is classified as congenital if diagnosed in utero, at birth, or within the first month of life. The pathophysiological process is believed to be due to immune-mediated injury of the conduction system, which occurs as a result of transplacental passage of maternal anti-SSA/Ro-SSB/La antibodies. Childhood atrioventricular block is therefore diagnosed between the first month and the 18th year of life. Genetic variants in multiple genes have been described to date in the pathogenesis of inherited progressive cardiac conduction disorders. Indications and techniques of cardiac pacing have also evolved to allow safe permanent cardiac pacing in almost all patients, including those with structural heart abnormalities. Conclusion: Early diagnosis and appropriate management are critical in many cases in order to prevent sudden death, and this review critically assesses our current understanding of the pathogenetic mechanisms, clinical course, and optimal management of congenital and childhood AV block.What is Known:• Prevalence of congenital heart block of 1 per 15,000 to 20,000 live births. AV block is defined as congenital if diagnosed in utero, at birth, or within the first month of life, whereas childhood AV block is diagnosed between the first month and the 18th year of life. As a result of several different etiologies, congenital and childhood atrioventricular block may occur in an entirely structurally normal heart or in association with concomitant congenital heart disease. Cardiac pacing is indicated in symptomatic patients and has several prophylactic indications in asymptomatic patients to prevent sudden death.• Autoimmune, congenital AV block is associated with a high neonatal mortality rate and development of dilated cardiomyopathy in 5 to 30 % cases.What is New:• Several genes including SCN5A have been implicated in autosomal dominant forms of familial progressive cardiac conduction disorders.• Leadless pacemaker technology and gene therapy for biological pacing are promising research fields. In utero percutaneous pacing appears to be at high risk and needs further development before it can be adopted into routine clinical practice. Cardiac resynchronization therapy is of proven value in case of pacing-induced cardiomyopathy.
AB - Atrioventricular block is classified as congenital if diagnosed in utero, at birth, or within the first month of life. The pathophysiological process is believed to be due to immune-mediated injury of the conduction system, which occurs as a result of transplacental passage of maternal anti-SSA/Ro-SSB/La antibodies. Childhood atrioventricular block is therefore diagnosed between the first month and the 18th year of life. Genetic variants in multiple genes have been described to date in the pathogenesis of inherited progressive cardiac conduction disorders. Indications and techniques of cardiac pacing have also evolved to allow safe permanent cardiac pacing in almost all patients, including those with structural heart abnormalities. Conclusion: Early diagnosis and appropriate management are critical in many cases in order to prevent sudden death, and this review critically assesses our current understanding of the pathogenetic mechanisms, clinical course, and optimal management of congenital and childhood AV block.What is Known:• Prevalence of congenital heart block of 1 per 15,000 to 20,000 live births. AV block is defined as congenital if diagnosed in utero, at birth, or within the first month of life, whereas childhood AV block is diagnosed between the first month and the 18th year of life. As a result of several different etiologies, congenital and childhood atrioventricular block may occur in an entirely structurally normal heart or in association with concomitant congenital heart disease. Cardiac pacing is indicated in symptomatic patients and has several prophylactic indications in asymptomatic patients to prevent sudden death.• Autoimmune, congenital AV block is associated with a high neonatal mortality rate and development of dilated cardiomyopathy in 5 to 30 % cases.What is New:• Several genes including SCN5A have been implicated in autosomal dominant forms of familial progressive cardiac conduction disorders.• Leadless pacemaker technology and gene therapy for biological pacing are promising research fields. In utero percutaneous pacing appears to be at high risk and needs further development before it can be adopted into routine clinical practice. Cardiac resynchronization therapy is of proven value in case of pacing-induced cardiomyopathy.
KW - Congenital heart disease
KW - Heart block
KW - Outcomes
KW - Pacemaker
KW - Pathophysiology
UR - http://www.scopus.com/inward/record.url?scp=84976351388&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84976351388&partnerID=8YFLogxK
U2 - 10.1007/s00431-016-2748-0
DO - 10.1007/s00431-016-2748-0
M3 - Review article
C2 - 27351174
AN - SCOPUS:84976351388
SN - 0340-6199
VL - 175
SP - 1235
EP - 1248
JO - European Journal of Pediatrics
JF - European Journal of Pediatrics
IS - 9
ER -