Surprise, surprise: Idiopathic, isolated complete atrioventricular block may be heritable

摘要

Baruteau and colleagues present findings that further the potential for a genetic basis for nonimmune isolated atrioventricular (AV) block. 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 approx=1 in 20 000 live-born infants and is commonly associated with an immune-related cause associated with maternal collagen vascular disease or structural cardiac disease. Other causes of CAVB have been described, including infections, myopa-thies, and genetic disorders such as the Hunter and Hurler syndromes. Still, the specific cause of 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 phe-notypes that include cardiac conduction abnormalities.