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Aim To investigate the correlation between maternal autoantibodies and age at diagnosis of isolated complete atrioventricular ( AV) block ( CAVB) and to study signs of late progression of foetal immune-mediated insults in cases of postnatally diagnosed CAVB. Methods Patients with CAVB (n = 190) identified in a population-based manner were included. Maternal autoantibody profile was correlated with age at CAVB diagnosis. A structured review of medical records was performed if a late CAVB diagnosis (>27 days post-partum) was associated with a sero-positive mother. Results Maternal Ro/La autoantibodies were observed in 88% of cases with a congenital diagnosis. Thirteen cases with a sero-positive mother and late CAVB diagnosis were found (age-range: 4 months-43 years). In two cases, CAVB was diagnosed in conjunction with infections, one case had a family history of cardiomyopathy and two cases had nontypical clinical presentations, indicating alternative pathogenetic mechanisms. In the remaining eight cases, no likely factors inducing CAVB, other than maternal autoantibodies, could be identified. Conclusion Our observations support the hypothesis that late progression to CAVB can be the result of an immune-mediated pathogenetic mechanism during foetal life. An autoantibody-associated diagnosis after the neonatal period is therefore possible, and testing of maternal serology at the time of diagnosis is recommended. [ABSTRACT FROM AUTHOR]

Aim To analyse growth of children with and without congenital heart block ( CHB) born to anti-Ro/ SSA positive mothers from birth to 18 years of age, using a population-based cohort of Swedish CHB patients. Methods Medical records for siblings with (n = 72) and without (n = 60) CHB born 1973-2009 to anti-Ro/ SSA positive mothers were retrieved from child healthcare centres and school health services and used to extract data on growth from birth to 18 years. Results Compared with reference standards, children with CHB were retarded in weight by 0.75-1.0 SD from birth to 2-3 years of age. Thereafter, the CHB children started to catch up, reaching the reference standards at 9-11 years of age. Pacemaker treatment was not correlated with the catch-up in growth. Individuals with CHB were retarded in both weight and height from birth to 9-11 years of age when compared to siblings without CHB, who did not demonstrate restriction in these measurements. Conclusion Presence of CHB is a more important predictor of growth restriction than maternal rheumatic disease and foetal anti-Ro/ SSA exposure. The restriction persists for several years after birth, despite pacemaker treatment, which highlights the importance of follow-up of children with CHB regarding nutrition and growth. [ABSTRACT FROM AUTHOR]

Objective To define factors influencing neurodevelopment in children with and without complete congenital heart block ( CHB) born to mothers with Ro/ SSA autoantibodies. Patients and methods Medical records of a population-based cohort of siblings with (n = 60) and without (n = 54) CHB born 1974-2009 to anti-Ro/ SSA-positive mothers were retrieved from children primary healthcare centres and school health services and used to extract data on neurodevelopment. Results Impaired neurodevelopment was reported in 16% of the children (18/114) during the follow-up time of 13.0 (8.2-17.5) years, median (quartiles). Reported problems included speech (9%), motor (8%) and learning (8%) impairment, attention deficit (5%) and behavioural impairment (4%). Impairment in motor skill development was more common in boys (p < 0.001) if the child was born preterm (p < 0.001). Learning impairment was significantly influenced by maternal SLE (p < 0.005), while attention deficits was influenced by both maternal SLE (p < 0.05) and CHB in the child (p < 0.05). Conclusions Our data indicate that in addition to well-established factors such as male sex and being born preterm, both maternal SLE and CHB may influence neurodevelopment. Follow-up of neurodevelopment should therefore be considered for children with CHB, especially if the mother is diagnosed with SLE. [ABSTRACT FROM AUTHOR]

Objective: To study the autoimmune response in mothers of children with congenital heart block (CHB) diagnosed at different ages and with different clinical manifestations.Patients and Methods: Clinical data and sera for the determination of immunological tests were available from 104 mothers of 113 children born between 1950 and 2000 and diagnosed with CHB before the age of 16 y. Prenatal diagnosis was performed in 74 (65%) children of 65 mothers, and 39 (35%) children had postnatal diagnosis of CHB. Maternal antibodies to 52 kd and 60 kd SS-A, and to 48 kd SS-B were determined by time-resolved fluoroimmunoassay (TR-FIA) and to antinuclear antibodies (ANA) by immunoflurescense (IF).Results: Out of the 65 mothers of children with in utero diagnosed CHB, 88% had antibodies to 52 kd SS-A and 83% had ANA. Antibodies to 60 kd SS-A and 48 kd SS-B were less frequently present, in 48% and in 54% of the mothers, respectively. Seven (11%) of the mothers were negative by all immunoassays. Of the 13 mothers of children with in-infancy diagnosed CHB, one mother had high-titer ANA. After 1 y of age, CHB was diagnosed in 26 children; at 1 to 6 y in 16 and after 7 y in 10 children; 1/16 and 1/10 patients had positive antibodies. In all twin pregnancies (n = 4) and in all families with recurring cases of CHB (n = 5), maternal antibodies were positive in at least one assay. The titer of 48 kd anti-SS-B antibodies was significantly higher in children with cutaneous neonatal lupus (98.1 vs 41.0; p = 0.02). All mothers whose children died before the age of 4 y (n = 8) and 85% (11/13) of mothers whose children developed cardiomyopathy had elevated antibody titers in at least one assay. However, we could not find any prognostic value of maternal antibody levels or specificities on the clinical outcome of the children with CHB.Conclusions: Although rare, late detection or postnatal progression of CHB in antibody-mediated CHB should be taken into consideration. Maternal antibody levels or specificities have prognostic effect neither on the clinical outcome of the child with CHB nor on the risk of reappearance in the same family. [ABSTRACT FROM AUTHOR]