long QT syndrome 16

A long QT syndrome characterized by perinatal onset of markedly prolonged corrected QT (QTc) interval, 2:1 atrioventricular (AV) block, and bradycardia or ventricular tachycardia (torsades de pointes) that has_material_basis_in heterozygous mutation in the CAL

A long QT syndrome characterized by perinatal onset of markedly prolonged corrected QT (QTc) interval, 2:1 atrioventricular (AV) block, and bradycardia or ventricular tachycardia (torsades de pointes) that has_material_basis_in heterozygous mutation in the CALM3 gene on chromosome 19q13.32. Syncope, cardiac arrest, and sudden death are common.

Signs and symptoms

• T-wave alternans
• Bradycardia
• Second degree atrioventricular block
• Perimembranous ventricular septal defect
• Prolonged QTc interval
• Patent ductus arteriosus after birth at term

Also known as: LQT16