Surprise, Surprise: Idiopathic, Isolated Complete Atrio-Ventricular Block May Be Heritable
Baruteau and colleagues present findings that further the potential for a genetic basis for non-immune isolated atrio-ventricular block1. Their observations warrant further consideration in the evaluation and management of patients with no definitive cause of their cardiac conduction defect.
Congenital complete AV block (CAVB) affects about 1 in 20,000 live-born infants2 and is associated commonly with an immune related cause related to maternal collagen vascular disease or structural cardiac disease.3 Other causes of CAVB have been described including infections, myopathies, and genetic disorders including Hunter and Hurler syndromes. Still, the specific cause for CAVB remains elusive for a significant number of patients raising the possibility that a portion of idiopathic CAVB stems from CAVB-susceptibility genes. Currently, mutations in transcription factors and cardiac channels yield electrocardiographic phenotypes that include cardiac conduction abnormalities. (SELECT FULL TEXT TO CONTINUE)
- Received August 6, 2012.
- Accepted August 7, 2012.
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