Dataset Information

Peripartum management of patient with long QT3 after successful implantable cardioverter defibrillator device discharge resulting in device failure: a case report.

ABSTRACT:

Background

Long QT3 syndrome type 3 (LQT3) is a gain of function mutation of the SCN5A gene that is inherited in an autosomal dominant fashion. Long QT3 syndrome type 3 results in an increase in arrhythmic events during rest, sleep, and bradycardia by extending the QT interval and inducing Torsades de pointes and sudden cardiac death. Attempting to block the sodium channel with Class I anti-arrhythmics or blocking adrenergic tone with beta-blockers especially in women has shown to be beneficial. There have been few large-scale studies on treating patients with LQT3 due to its lethality and underreported number of cases. Specifically, the safety and efficacy of pharmacologic treatment in pregnant LQT3 patients are unknown.

Case summary

This case demonstrates the safe use of Mexiletine and Propranolol in a 3rd-trimester pregnant LQT3 patient after a presumed ventricular arrhythmia and device-lead electrical short from therapy rendered her implantable cardioverter defibrillator inoperable in a VVI mode (venticular demand pacing). With appropriate medications, the patient was safely monitored through the remainder of her pregnancy and safely delivered at 36 weeks of pregnancy a healthy baby girl. The daughter, heterozygous for LQT3, showed no evidence of intrauterine growth restriction or other side effects from the medications.

Discussion

There are many variants of the SCN5A gene mutations that can lead to different phenotypes and not all mutations are responsive to the same medications. In this case, Mexiletine and Propranolol, both of which have only recently shown to benefit certain variants or LQT3 respectively, were safely started during the 3rd trimester of pregnancy without harming the foetus.

SUBMITTER: Lee MJ

PROVIDER: S-EPMC8672658 | biostudies-literature |

REPOSITORIES: biostudies-literature

ACCESS DATA

Similar Datasets

Project description:Background One third of primary prevention implantable cardioverter-defibrillator patients receive appropriate therapy, but all remain at risk of defibrillator complications. Information on these complications in contemporary cohorts is limited. This study assessed complications and their risk factors after defibrillator implantation in a Dutch nationwide prospective registry cohort and forecasts the potential reduction in complications under distinct scenarios of updated indication criteria. Methods and Results Complications in a prospective multicenter registry cohort of 1442 primary implantable cardioverter-defibrillator implant patients were classified as major or minor. The potential for reducing complications was derived from a newly developed prediction model of appropriate therapy to identify patients with a low probability of benefitting from the implantable cardioverter-defibrillator. During a follow-up of 2.2 years (interquartile range, 2.0-2.6 years), 228 complications occurred in 195 patients (13.6%), with 113 patients (7.8%) experiencing at least one major complication. Most common ones were lead related (n=93) and infection (n=18). Minor complications occurred in 6.8% of patients, with lead-related (n=47) and pocket-related (n=40) complications as the most prevailing ones. A surgical reintervention or additional hospitalization was required in 53% or 61% of complications, respectively. Complications were strongly associated with device type. Application of stricter implant indication results in a comparable proportional reduction of (major) complications. Conclusions One in 13 patients experiences at least one major implantable cardioverter-defibrillator-related complication, and many patients undergo a surgical reintervention. Complications are related to defibrillator implantations, and these should be discussed with the patient. Stricter implant indication criteria and careful selection of device type implanted may have significant clinical and financial benefits.

Project description:ImportanceCardiac resynchronization therapy (CRT) reduces the risk for mortality and heart failure-related events in select patients. Little is known about the use of CRT in combination with an implantable cardioverter defibrillator (ICD) in patients who are eligible for this therapy in clinical practice.ObjectiveTo (1) identify patient, clinician, and hospital characteristics associated with CRT defibrillator (CRT-D) use and (2) determine the extent of hospital-level variation in the use of CRT-D among guideline-eligible patients undergoing ICD placement.Design, setting, and participantsMulticenter retrospective cohort from 1428 hospitals participating in the National Cardiovascular Data Registry ICD Registry between April 1, 2010, and June 30, 2014. Adult patients meeting class I or IIa guideline recommendations for CRT at the time of device implantation were included in this study.Main outcomes and measuresImplantation of an ICD with or without CRT.ResultsA total of 63 506 eligible patients (88.6%) received CRT-D at the time of device implantation. The mean (SD) ages of those in the ICD and CRT-D groups were 67.9 (12.2) years and 68.4 (11.5) years, respectively. In hierarchical multivariable models, black race was independently associated with lower use of CRT-D (odds ratio [OR], 0.77; 95% CI, 0.71-0.83) as was nonprivate insurance (OR, 0.90; 95% CI, 0.85-0.95 for Medicare and OR, 0.73; 95% CI, 0.65-0.82 for Medicaid). Clinician factors associated with greater CRT-D use included clinician implantation volume (OR, 1.01 per 10 additional devices implanted; 95% CI, 1.01-1.01) and electrophysiology training (OR, 3.13 as compared with surgery-boarded clinicians; 95% CI, 2.50-3.85). At the hospital level, the overall median risk-standardized rate of CRT-D use was 79.9% (range, 26.7%-100%; median OR, 2.08; 95% CI, 1.99-2.18).Conclusions and relevanceIn a national cohort of patients eligible for CRT-D at the time of device implantation, nearly 90% received a CRT-D device. However, use of CRT-D differed by race and implanting operator characteristics. After accounting for these factors, the use of CRT-D continued to vary widely by hospital. Addressing disparities and variation in CRT-D use among guideline-eligible patients may improve patient outcomes.