OBJECTIVES: We sought to determine whether the presentation and prognosis of children with complete atrioventricular block (CAVB) were related to maternal antibody status. BACKGROUND: Comparative studies related to the presence or absence of maternal antibodies anti-SSB/La and anti-SSA/Ro are lacking in children with isolated complete CAVB. METHODS: From 1980 to 2004, we screened for maternal antibodies in 111 children <15 years old with CAVB. According to the presence (Ab+) or absence (Ab-) of antibodies, 2 groups of patients were retrospectively compared. RESULTS: The study group included 56 Ab+ and 55 Ab- patients with equal gender distribution. A total of 96% Ab+ patients were diagnosed in utero or within the first month, compared with 24% Ab- patients. Progression from incomplete to complete block was shown in 23 Ab- and 2 Ab+ patients. Echocardiography showed normal heart structures in Ab- patients, but 8 Ab+ patients had ostium secundum or ductus arteriosus. Pacemaker implantation was performed in 105 patients, and age at implantation was younger in the Ab+ group. At follow-up (age 9.7 +/- 6 years), all Ab- patients were alive with normal left ventricular function; dilated cardiomyopathy was diagnosed at diagnosis or during follow-up in 16 Ab+ patients, and 6 of 16 have died. CONCLUSIONS: Patients with antibody-mediated CAVB were diagnosed and underwent pacing earlier in life and had a more severe prognosis than Ab- patients because of a high risk of dilated cardiomyopathy. The absence of antibody suggests a different pathologic mechanism than autoimmunity, and the term congenital may be not appropriate in these cases.
OBJECTIVES: We sought to determine whether the presentation and prognosis of children with complete atrioventricular block (CAVB) were related to maternal antibody status. BACKGROUND: Comparative studies related to the presence or absence of maternal antibodies anti-SSB/La and anti-SSA/Ro are lacking in children with isolated complete CAVB. METHODS: From 1980 to 2004, we screened for maternal antibodies in 111 children <15 years old with CAVB. According to the presence (Ab+) or absence (Ab-) of antibodies, 2 groups of patients were retrospectively compared. RESULTS: The study group included 56 Ab+ and 55 Ab- patients with equal gender distribution. A total of 96% Ab+ patients were diagnosed in utero or within the first month, compared with 24% Ab- patients. Progression from incomplete to complete block was shown in 23 Ab- and 2 Ab+ patients. Echocardiography showed normal heart structures in Ab- patients, but 8 Ab+ patients had ostium secundum or ductus arteriosus. Pacemaker implantation was performed in 105 patients, and age at implantation was younger in the Ab+ group. At follow-up (age 9.7 +/- 6 years), all Ab- patients were alive with normal left ventricular function; dilated cardiomyopathy was diagnosed at diagnosis or during follow-up in 16 Ab+ patients, and 6 of 16 have died. CONCLUSIONS:Patients with antibody-mediated CAVB were diagnosed and underwent pacing earlier in life and had a more severe prognosis than Ab- patients because of a high risk of dilated cardiomyopathy. The absence of antibody suggests a different pathologic mechanism than autoimmunity, and the term congenital may be not appropriate in these cases.
Authors: Peter M Izmirly; Amit Saxena; Mimi Y Kim; Dan Wang; Sara K Sahl; Carolina Llanos; Deborah Friedman; Jill P Buyon Journal: Circulation Date: 2011-10-03 Impact factor: 29.690
Authors: Amit Saxena; Erin McDonnell; Paula S Ramos; Satria Sajuthi; Miranda C Marion; Carl D Langefeld; Jill P Buyon; Robert M Clancy Journal: Arthritis Rheum Date: 2012-03