Congenital Heart Block and Dilated Ascending Aorta
Congenital Complete heart block has a well known association with maternal systemic lupus erythematosus. We present a case series of 5 consecutive patients of CCHB from Nov 2006 to August 2008. Three out of the five cases had severe dilatation of the ascending aorta.
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There is a strong association between CCHB and maternal lupus. Congenital AV block occurs in association with maternal antibodies in 1/15000-20000 live births.Specific antibodies directed against ribonuclear proteins are transmitted across the placenta, often in absence of maternal symptoms.However little is known about CCHB in association with connective tissue disorders in the child. The presence of significantly dilated ascending aorta in 3 out of 5 patients possibly indicates a connective tissue disorder in the children. To the best of our knowledge, ours is only the second report of occurrence of dilated ascending aorta in children with CCHB. A detailed echocardiogram is warranted in every case of CCHB. A dilated ascending aorta may prove to be a marker of morbidity and mortality in such children and may necessitate further work up.
| Age | Ventricular rate (per min) | Mother's Antibody Status | Initial LVIDd (z score) | FS% | CHD | Initial Ascending Aorta ( z score) | Pacemaker | Mosst recent Follow Up |
|---|---|---|---|---|---|---|---|---|
| 1 day | 50 | Anti Ro La negative, ANA positive, 1:320; speckled | 2.1cm(0.67) | 34 | PDA | 1.3cm(+4) | epicardial VVIR | Dilated Cardiomyopathy, asc aorta 1.5cm |
| 3day | 50 | Anti Ro/LA positive | 2cm(0.5) | 45 | PDA/ASD | normal | Epicardial VVIR | lost to follow up |
| 3.5mo | 80 | Anti Ro/La postive, ANA positive (1:10000) speckled nucleolar | 2.2cm(0.8) | 36 | PDA | normal | - | 2nd degree heart block |
| 4.5yrs | 50 | ANA postive | 3.5cm(1.23) | 23 | ASD | 2.1cm(+4.3) | - | posted for pacemaker |
| 13mo | 45 | ANA positive | 3.3cm(+3.9) | 36 | ASD | 2.6cm(+12.8) | - | posted for pacemaker |