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Circ Arrhythm Electrophysiol ; 13(12): e008830, 2020 12.
Artigo em Inglês | MEDLINE | ID: mdl-33198487

RESUMO

BACKGROUND: Videoscopic left cardiac sympathetic denervation (LCSD) is an effective antifibrillatory, minimally invasive therapy for patients with potentially life-threatening arrhythmia syndromes like long QT syndrome (LQTS). Although initially used primarily for treatment intensification following documented LQTS-associated breakthrough cardiac events while on beta-blockers, LCSD as 1-time monotherapy for certain patients with LQTS requires further evaluation. We are presenting our early experience with LCSD monotherapy for carefully selected patients with LQTS. METHODS: Among the 1400 patients evaluated and treated for LQTS, a retrospective review was performed on the 204 patients with LQTS who underwent LCSD at our institution since 2005 to identify the patients where the LCSD served as stand-alone, monotherapy. Clinical data on symptomatic status before diagnosis, clinical, and genetic diagnosis, and breakthrough cardiac events after diagnosis were analyzed to determine efficacy of LCSD monotherapy. RESULT: Overall, 64 of 204 patients (31%) were treated with LCSD alone (37 [58%] female, mean QTc 466±30 ms, 16 [25%] patients were symptomatic before diagnosis with a mean age at diagnosis 17.3±11.8 years, 5 had [8%] ≥1 breakthrough cardiac event after diagnosis, and mean age at LCSD was 21.1±11.4 years). The primary motivation for LCSD monotherapy was an unacceptable quality of life stemming from beta-blocker related side effects (ie, beta-blocker intolerance) in 56/64 patients (88%). The underlying LQTS genotype was LQT1 in 36 (56%) and LQT2 in 20 (31%). There were no significant LCSD-related surgical complications. With a mean follow-up of 2.7±2.4 years so far, only 3 patients have experienced a nonlethal, post-LCSD breakthrough cardiac event in 180 patient-years. CONCLUSIONS: LCSD may be a safe and effective stand-alone therapy for select patients who do not tolerate beta-blockers. However, LCSD is not curative and patient selection will be critical when potentially considering LCSD as monotherapy.


Assuntos
Frequência Cardíaca , Coração/inervação , Síndrome do QT Longo/cirurgia , Síndrome de Romano-Ward/cirurgia , Simpatectomia , Sistema Nervoso Simpático/cirurgia , Cirurgia Vídeoassistida , Adolescente , Adulto , Criança , Pré-Escolar , Tomada de Decisão Clínica , Feminino , Humanos , Síndrome do QT Longo/congênito , Síndrome do QT Longo/diagnóstico , Síndrome do QT Longo/fisiopatologia , Masculino , Recidiva , Estudos Retrospectivos , Síndrome de Romano-Ward/diagnóstico , Síndrome de Romano-Ward/genética , Síndrome de Romano-Ward/fisiopatologia , Simpatectomia/efeitos adversos , Sistema Nervoso Simpático/fisiopatologia , Fatores de Tempo , Resultado do Tratamento , Cirurgia Vídeoassistida/efeitos adversos , Adulto Jovem
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