Familial long QT syndrome and late development of dilated cardiomyopathy in a child with a KCNQ1 mutation: A case report Kiona Y. Allen, MD, Victoria.

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Familial long QT syndrome and late development of dilated cardiomyopathy in a child with a KCNQ1 mutation: A case report Kiona Y. Allen, MD, Victoria L. Vetter, MD, MPH, Maully J. Shah, MBBS, Matthew J. O’Connor, MD HeartRhythm Case Reports Volume 2, Issue 2, Pages 128-131 (March 2016) DOI: 10.1016/j.hrcr.2015.10.011 Copyright © 2016 Heart Rhythm Society Terms and Conditions

Figure 1 A: Twelve-lead electrocardiogram obtained at 2 days of life showing a corrected QT interval of 495 msec and nonspecific T wave abnormalities. B: Twelve-lead electrocardiogram obtained at time of presentation with heart failure at 9 years of age demonstrating new T wave changes and voltage criteria for left ventricular hypertrophy. HeartRhythm Case Reports 2016 2, 128-131DOI: (10.1016/j.hrcr.2015.10.011) Copyright © 2016 Heart Rhythm Society Terms and Conditions

Figure 2 Transthoracic echocardiogram showing severe dilation and systolic dysfunction of the left ventricle. HeartRhythm Case Reports 2016 2, 128-131DOI: (10.1016/j.hrcr.2015.10.011) Copyright © 2016 Heart Rhythm Society Terms and Conditions

Figure 3 Family pedigree demonstrating mutations in the KCNQ1 and titin genes. The KCNQ1 was inherited maternally and multiple first- and second-degree relatives were affected, including a maternal cousin with sudden cardiac death. The titin gene mutation was inherited paternally and no other overlap with the KCNQ1 gene was identified. HeartRhythm Case Reports 2016 2, 128-131DOI: (10.1016/j.hrcr.2015.10.011) Copyright © 2016 Heart Rhythm Society Terms and Conditions