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. 2013 Jan;28(1):126-9.
doi: 10.1007/s00380-012-0235-8. Epub 2012 Feb 22.

A left ventricular noncompaction in a patient with long QT syndrome caused by a KCNQ1 mutation: a case report

Kentaro Nakashima  1 , Isao Kusakawa, Tsuyoshi Yamamoto, Shinsuke Hirabayashi, Ryohta Hosoya, Wataru Shimizu, Naokata Sumitomo
Affiliations

A left ventricular noncompaction in a patient with long QT syndrome caused by a KCNQ1 mutation: a case report

Kentaro Nakashima et al. Heart Vessels. 2013 Jan.

Abstract

A 5-year-old girl developed cardiopulmonary arrest after crying. From the electrocardiogram and echocardiography, a left ventricular noncompaction (LVNC) with long QT syndrome (LQT) was suspected as the cause of the cardiopulmonary arrest, and treatment with a β-blocker and a calcium antagonist was then begun. A genetic screening of LQT-related genes revealed a previously reported heterozygous KCNQ1 mutation. The association of LVNC and LQT is an extremely rare condition, and long-term treatment based on the characteristics of both disorders is required. Also, the association of cardiomyopathy and LQT could become a new clinical entity in the future.

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