BACKGROUND: Untreated isolated fetal complete atrioventricular block (CAVB) has a significant mortality rate. A standardized treatment approach, including maternal dexamethasone at CAVB diagnosis and beta-stimulation for fetal heart rates <55 bpm, has been used at our institutions since 1997. The study presents the impact of this approach. METHODS AND RESULTS: Thirty-seven consecutive cases of fetal CAVB since 1990 were studied. Mean age at diagnosis was 25.6+/-5.2 gestational weeks. In 33 patients (92%), CAVB was associated with maternal anti-Ro/La autoantibodies. Patients were separated into those diagnosed between 1990 and 1996 (group 1; n=16) and those diagnosed between 1997 and 2003 (group 2; n=21). The 2 study groups were comparable in the clinical presentation at CAVB diagnosis but did differ in prenatal management (treated patients: group 1, 4/16; group 2, 18/21; P<0.0001). Overall, 22 fetuses were treated, 21 with dexamethasone and 9 with beta-stimulation for a mean of 7.5+/-4.5 weeks. Live-birth and 1-year survival rates of group 1 were 80% and 47%, and these improved to 95% for group 2 patients (P<0.01). The 21 patients treated with dexamethasone had a 1-year survival rate of 90%, compared with 46% without glucocorticoid therapy (P<0.02). Immune-mediated conditions (myocarditis, hepatitis, cardiomyopathy) resulting in postnatal death or heart transplantation were significantly more common in untreated anti-Ro/La antibody-associated pregnancies compared with patients treated with steroids (0/18 versus 4/9 live births; P=0.007). CONCLUSIONS: A standardized treatment approach, including transplacental fetal administration of dexamethasone and beta-stimulation at heart rates <55 bpm, reduced the morbidity and improved the outcome of isolated fetal CAVB.
BACKGROUND: Untreated isolated fetal complete atrioventricular block (CAVB) has a significant mortality rate. A standardized treatment approach, including maternal dexamethasone at CAVB diagnosis and beta-stimulation for fetal heart rates <55 bpm, has been used at our institutions since 1997. The study presents the impact of this approach. METHODS AND RESULTS: Thirty-seven consecutive cases of fetal CAVB since 1990 were studied. Mean age at diagnosis was 25.6+/-5.2 gestational weeks. In 33 patients (92%), CAVB was associated with maternal anti-Ro/La autoantibodies. Patients were separated into those diagnosed between 1990 and 1996 (group 1; n=16) and those diagnosed between 1997 and 2003 (group 2; n=21). The 2 study groups were comparable in the clinical presentation at CAVB diagnosis but did differ in prenatal management (treated patients: group 1, 4/16; group 2, 18/21; P<0.0001). Overall, 22 fetuses were treated, 21 with dexamethasone and 9 with beta-stimulation for a mean of 7.5+/-4.5 weeks. Live-birth and 1-year survival rates of group 1 were 80% and 47%, and these improved to 95% for group 2 patients (P<0.01). The 21 patients treated with dexamethasone had a 1-year survival rate of 90%, compared with 46% without glucocorticoid therapy (P<0.02). Immune-mediated conditions (myocarditis, hepatitis, cardiomyopathy) resulting in postnatal death or heart transplantation were significantly more common in untreated anti-Ro/La antibody-associated pregnancies compared with patients treated with steroids (0/18 versus 4/9 live births; P=0.007). CONCLUSIONS: A standardized treatment approach, including transplacental fetal administration of dexamethasone and beta-stimulation at heart rates <55 bpm, reduced the morbidity and improved the outcome of isolated fetal CAVB.
Authors: Nisha M Fernandes; Glenn P Taylor; Cedric Manlhiot; Brian W McCrindle; Michael Ho; Steven E S Miner; Adelle Atkinson; Edgar T Jaeggi; Lynne E Nield Journal: Pediatr Cardiol Date: 2011-04-12 Impact factor: 1.655