title: Rare Cause of Infranodal Block.
abstract: The patient exhibits multiple features suggestive of Timothy syndrome, which is a multisystem autosomal-dominant condition with findings that include prolonged QT interval, hand and foot abnormalities, dysmorphic facial features, and mental retardation. A 2:1 infranodal atrioventricular block may occasionally be seen in the setting of severely prolonged QT interval. Functional nature of atrioventricular block is demonstrated by resumption of 1:1 conduction with changes in heart rate.
authors.affiliation: Aurora Sinai/Aurora St. Luke's Medical Centers, 2801 W Kinnickinnic River Parkway, Suite 777, Milwaukee, WI 53215, USA; Children's Hospital of Wisconsin-Milwaukee Campus, The Medical College of Wisconsin, 8915 W. Connell Ct, Milwaukee, WI 53226, USA. Electronic address: publishing30@aurora.org.
authors.forename: Anwer A
authors.initials: AA
authors.lastname: Dhala
authors.affiliation: Children's Hospital of Wisconsin-Milwaukee Campus, The Medical College of Wisconsin, 8915 W. Connell Ct, Milwaukee, WI 53226, USA.
authors.forename: Anoop K
authors.initials: AK
authors.lastname: Singh
mesh_terms: D054537:Atrioventricular Block; D001321:Autistic Disorder; D004562:Electrocardiography; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008133:Long QT Syndrome; D013576:Syndactyly
keywords: Infranodal block; Long QT syndrome; Timothy syndrome