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Case Reports
. 2017 Oct 28;4(1):14-17.
doi: 10.1016/j.hrcr.2017.09.001. eCollection 2018 Jan.

Genotype-phenotype-guided medical and surgical intervention in long QT syndrome

Robyn J Hylind  1 , Virginie Beausejour Ladouceur  1 , Francis Fynn-Thompson  2 , Shannon E Hourigan  3 , Vassilios J Bezzerides  1 , Dominic J Abrams  1
Affiliations
Case Reports

Genotype-phenotype-guided medical and surgical intervention in long QT syndrome

Robyn J Hylind et al. HeartRhythm Case Rep. .
No abstract available

Keywords: Arrhythmia; Electrophysiology; Left cardiac sympathetic denervation; Long QT syndrome; Sudden cardiac death.

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Figures

Figure 1
Figure 1
A depiction of the potassium channel, KvLQT1, encoded by the gene KCNQ1, is shown with 6 transmembrane domains (S1–S6) seen spanning the cardiomyocyte membrane. The site of the pathogenic variant identified in the proband—KCNQ1 c.1552C>T; p.R518X—is depicted by the blue circle in the C-terminus.
Figure 2
Figure 2
A multigenerational family pedigree with the proband denoted by an arrow. Males are represented by squares and females by circles. Blue quadrants represent the KCNQ1 R518X genotype, and red quadrants represent long QT syndrome. A&W= alive and well; d. = died; MI = myocardial infarct; yo = years old.
Figure 3
Figure 3
Corrected QT intervals measured in the patient during the recovery period from exercise under the following conditions: A: on 20 mg nadolol therapy, B: on no therapy, C: post left cardiac sympathectomy denervation. The normal range (2nd to 98th percentiles) derived from control subjects is shown in gray.
See this image and copyright information in PMC

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