Kyung Jin Ahn1,2, Mi Kyoung Song1, Sang Yun Lee1, Ja Kyoung Yoon3, Gi Beom Kim1, Seil Oh4, Eun Jung Bae5. 1. Department of Pediatrics, Seoul National University Children's Hospital, Seoul National University College of Medicine, Seoul, Korea. 2. Department of Pediatrics, Gachon University Gil Medical Center, Incheon, Korea. 3. Department of Pediatrics, Sejong General Hospital, Bucheon, Korea. 4. Department of Internal Medicine, Seoul National University Children's Hospital, Seoul National University College of Medicine, Seoul, Korea. 5. Department of Pediatrics, Seoul National University Children's Hospital, Seoul National University College of Medicine, Seoul, Korea. eunjbaek@snu.ac.kr.
Abstract
BACKGROUND AND OBJECTIVES: Although long QT syndrome (LQTS) is a potentially life-threatening inherited cardiac channelopathy, studies documenting the long-term clinical data of Korean patients with LQTS are scarce. METHODS: This retrospective cohort study included 105 patients with LQTS (48 women; 45.7%) from a single tertiary center. The clinical outcomes were analyzed for the rate of freedom from breakthrough cardiac events (BCEs), additional treatment needed, and death. RESULTS: LQTS was diagnosed at a median age of 11 (range, 0.003-80) years. Genetic testing was performed on 90 patients (yield, 71.1%). The proportions of genetically confirmed patients with LQTS types 1, 2, 3, and others were 34.4%, 12.2%, 12.2%, and 12.2%, respectively. In the symptomatic group (n=70), aborted cardiac arrest was observed in 30% of the patients. Treatments included medications in 60 patients (85.7%), implantable cardioverter-defibrillators in 27 (38.6%; median age, 17 years; range, 2-79 years), and left cardiac sympathetic denervation surgery in 7 (10%; median age, 13 years; range, 2-34). The 10-year BCE-free survival rate was 73.2%. By genotype, significant differences were observed in BCEs despite medication (p<0.001). The 10-year BCE-free survival rate was the highest in patients with LQTS type 1 (81.8%) and the lowest in those with multiple LQTS-associated mutations (LQTM). All patients with LQTS survived, except for one patient who had LQTM. CONCLUSIONS: Good long-term outcomes can be achieved by using recently developed genetically tailored management strategies for patients with LQTS.
BACKGROUND AND OBJECTIVES: Although long QT syndrome (LQTS) is a potentially life-threatening inherited cardiac channelopathy, studies documenting the long-term clinical data of Korean patients with LQTS are scarce. METHODS: This retrospective cohort study included 105 patients with LQTS (48 women; 45.7%) from a single tertiary center. The clinical outcomes were analyzed for the rate of freedom from breakthrough cardiac events (BCEs), additional treatment needed, and death. RESULTS: LQTS was diagnosed at a median age of 11 (range, 0.003-80) years. Genetic testing was performed on 90 patients (yield, 71.1%). The proportions of genetically confirmed patients with LQTS types 1, 2, 3, and others were 34.4%, 12.2%, 12.2%, and 12.2%, respectively. In the symptomatic group (n=70), aborted cardiac arrest was observed in 30% of the patients. Treatments included medications in 60 patients (85.7%), implantable cardioverter-defibrillators in 27 (38.6%; median age, 17 years; range, 2-79 years), and left cardiac sympathetic denervation surgery in 7 (10%; median age, 13 years; range, 2-34). The 10-year BCE-free survival rate was 73.2%. By genotype, significant differences were observed in BCEs despite medication (p<0.001). The 10-year BCE-free survival rate was the highest in patients with LQTS type 1 (81.8%) and the lowest in those with multiple LQTS-associated mutations (LQTM). All patients with LQTS survived, except for one patient who had LQTM. CONCLUSIONS: Good long-term outcomes can be achieved by using recently developed genetically tailored management strategies for patients with LQTS.
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Authors: P D'Adamo; L Fassone; A Gedeon; E A Janssen; S Bione; P A Bolhuis; P G Barth; M Wilson; E Haan; K H Orstavik; M A Patton; A J Green; E Zammarchi; M A Donati; D Toniolo Journal: Am J Hum Genet Date: 1997-10 Impact factor: 11.025