Long-term follow up of children with congenital complete atrioventricular block and the impact of pacemaker therapy

Abstract

PM therapy reduces mortality and morbidity in children with CCAVB when compared with natural history data. Although children with PM are free from CCAVB related symptoms limited morbidity remains due to PM system related complications.

Congenital Complete Atrioventricular Block in a Young Man: A Case Study

“…Diagnostic criteria for CCAVB in children include occurrence of complete atrioventricular block (CAVB) in utero or at birth as indicated by echocardiographic or electrocardiographic (ECG) findings or CAVB with some evidence of a slow pulse at a fairly early age with no history of myocarditis that might have caused the condition after birth. 2 The estimated incidence of CCAVB is 1 in 20000 live births, [2][3][4] but in a study by Siren et al, 5 the incidence in Finland during the 1990s had increased to 1 in 11000 births. This increase may be due to improved technological advances and increased prenatal care.…”

“…4 CCAVB patients with structural heart disease have higher mortality than do those without structural heart disease. 2 Neonatal lupus is rare and may lead to CCAVB due to transplacental transfer of autoantibodies from mothers who are positive for antibodies to Ro/SS-A and La, but CCAVB in patients with neonatal lupus is thought to occur in only 3% of infants with antibody-positive mothers. 9 Neonatal lupus syndrome has a 12% to 41% first-year mortality rate 6 and is rarely responsible for previously asymptomatic CCAVB that occurs in childhood or young adulthood.…”

“…9 When tested for CTD, 75% of mothers who had a child with CCAVB had no signs and symptoms of CTD but had antibodies to Ro/SS-A. 2 Other causes of CCAVB (25%-33%), 10 which may or may not have an inherited component, include structural cardiac abnormalities such as L-transposition of great arteries, ventricular inversion, and atrioventricular septal defect in which the atrioventricular node ends blindly. 4 CCAVB patients with structural heart disease have higher mortality than do those without structural heart disease.…”

“…CCAVB is thought to be caused by destruction of the conduction system by myocarditis 2 and is associated with maternal autoimmune disease, 6 structural heart disease, and, when diagnosed in utero, neonatal lupus syndrome. 7 CCAVB is strongly associated with maternal connective tissue disorders (CTDs), especially those involving autoantibodies to Ro/SS-A, which have been detected in maternal sera in up to 98% of cases of CCAVB.…”

Avaliação da estimulação ventricular direita crônica em crianças e adultos jovens com bloqueio atrioventricular congênito isolado

“…Após o implante de marca-passo, em geral, a evolução clínica é muito satisfatória e a diminuição dos diâmetros do ventrículo esquerdo tem sido observada 26 .…”