vi medicine informatics




Romano-Ward and Jervell and Lange-Nielsen are two syndromes of congenital long QT syndrome. Romano-Ward syndrome has pure cardiac phenotype and has autosomal dominant inheritance. It is caused by mutations in ANK2 gene causing disruption in ion channels and electrical currents in cardiac myocytes. Clinical symptoms include syncope, seizures, development of torsades de pointes and sudden cardiac death. Diagnosis is based on family history of sudden cardiac death, ECG findings, and exercise test. Treatment is aimed at decreasing the incidence of cardiac arrhythmias by using β-blockers and heart ganglion blocks.

tags: oneliner