A successfully novel ICD implantation and medical treatment in a child with LQT syndrome and self-limiting ventricular fibrillation

2007 ◽  
Vol 118 (3) ◽  
pp. e108-e112 ◽  
Author(s):  
Fabrizio Drago ◽  
Giovanni Fazio ◽  
Massimo Stefano Silvetti ◽  
Gianluca Oricchio ◽  
Guido Michelon
2001 ◽  
Vol 47 (3) ◽  
pp. 459-463 ◽  
Author(s):  
Thomas Schlüter ◽  
Hannsjörg Baum ◽  
Andreas Plewan ◽  
Dieter Neumeier

Abstract Background: Implantable cardioverter defibrillator (ICD) implantation is a common approach in patients at high risk of sudden cardiac death. To check for normal function, it is necessary to test the ICD. For this purpose, repetitive induction and termination of ventricular fibrillation by direct current shocks is required. This may lead to minor myocardial damage. Cardiac troponin T (cTnT) and I (cTnI) are specific markers for the detection of myocardial injury. Because these proteins usually are undetectable in healthy individuals, they are excellent markers for detecting minimal myocardial damage. The objective of this study was to evaluate the effect of defibrillation of induced ventricular fibrillation on markers of myocardial damage. Methods: This study included 14 patients who underwent ICD implantation and intraoperative testing. We measured cTnT, cTnI, creatine kinase MB (CK-MB) mass, CK activity, and myoglobin before and at definite times after intraoperative shock application. Results: Depending on the effectiveness of shocks and the energy applied, the cardiac-specific markers cTnT and cTnI, as well as CK-MB mass, showed a significant increase compared with the baseline value before testing and peaked for the most part 4 h after shock application. In contrast, the increases in CK activity and myoglobin were predominantly detectable in patients who received additional external shocks. Conclusions: ICD implantation and testing leads to a short release of cardiac markers into the circulation. This release seems to be of cytoplasmic origin and depends on the number and effectiveness of the shocks applied.


Author(s):  
Omar Rezk Alshaer ◽  
Abdullah Obaid Binobaid ◽  
Saeed Dawas Alwadai ◽  
Wejdan Hani Alhakeem ◽  
Khalid Abdullah Al-Attas ◽  
...  

Studies have shown that many manifestations can be observed for patients suffering from long QT syndrome (LQTS), as it is a common cause of syncope and mortality among younger patients, in addition to convulsions. Many management modalities for LQTS have been described in the literature including medical treatment modalities and lifestyle modification approaches. However, evidence regarding the outcomes of these approaches is continuously updating. In this study, we have reviewed findings from the current studies in the literature about the medical treatment and lifestyle modifications for patients suffering from LQTS. Furthermore, studies have shown that β-blockers are effective modalities and should be used at a maximum dose. However, the potential side events should be considered and adequately managed and patient compliance should be maintained along the course of treatment. Implantable cardioverter-defibrillator (ICD) implantation and LCSD were also discussed in the present study with their favorable indications. Additionally, lifestyle modifications were also important and have been reported with favorable events and therefore, these should be considered in such situations. However, evidence regarding some approaches as limiting competitive exercises is still conflicting, indicating the need for future investigations.


EP Europace ◽  
2001 ◽  
Vol 2 (Supplement_1) ◽  
pp. A2-A2
Author(s):  
P. Scipione ◽  
R. Renzi ◽  
F. Capestro ◽  
G. P. Perna

Author(s):  
Guangchen Zou ◽  
Mukul Khanna ◽  
Saliha Zahid ◽  
Samir Dengle ◽  
Bhavna Matta ◽  
...  

Abstract Background Pharmacologic challenge test is often used to diagnose Brugada syndrome (BrS) when spontaneous ECGs do not show type I Brugada pattern but reported sensitivity varies. The role of exercise stress test in diagnosing Brugada syndrome is not well-established. Case Summary A patient had a type I Brugada pattern ECG during the recovery phase of exercise stress test but had a negative procainamide challenge test. He had a loop recorder implanted and later survived a ventricular fibrillation (VF) arrest provoked by COVID-19. ECG on arrival showed type 1 Brugada pattern. He was discharged after implantable cardioverter-defibrillator (ICD) implantation. He later underwent genetic testing and was found to be heterozygous for c.844C>G (p.Arg282Gly) mutation in the SCN5A gene. Discussion Type 1 Brugada pattern ECG may be unmasked by ST segment augmentation during recovery from exercise. Exercise stress test may play a role in diagnosis of Brugada syndrome when suspicion for Brugada syndrome remains after a negative procainamide challenge test or if the patient has exercise related symptoms. COVID-19 can unmask BrS and trigger a VF cardiac arrest.


Resuscitation ◽  
2008 ◽  
Vol 78 (1) ◽  
pp. 38-45 ◽  
Author(s):  
Julia H. Indik ◽  
Craig M. Peters ◽  
Richard L. Donnerstein ◽  
Peter Ott ◽  
Karl B. Kern ◽  
...  

Circulation ◽  
2007 ◽  
Vol 116 (suppl_16) ◽  
Author(s):  
Jonas S De Jong ◽  
Roos F Marsman ◽  
José P Henriques ◽  
Arthur A Wilde ◽  
Lukas R Dekker

Introduction : Based on data from the thrombolysis era high mortality rates are ascribed to survivors of primary ventricular fibrillation, i.e. VF within the first 48 hours of acute myocardial infarction (AMI). However, current guidelines do not advice ICD implantation if left ventricular function recovers well. We present new data from a large dataset of primary VF survivors treated with percutaneous coronary intervention (PCI). Methods : Multi-center case-control study with prospective follow-up. Consecutive patients with a first AMI and VF prior to PCI (cases, n =372) were compared with patients with a first AMI without VF (controls, n =305). Primary endpoint is survival > 30 days. Results : Median follow up duration was 3.34 years for cases and 3.69 years for controls ( p = 0.015). VF occurred at a mean of 65 minutes after onset of chest pain and occurred out of hospital in 42.3%, in the ambulance in 23.4% and after hospital admission in 32.7% of cases. Median time to PCI was 160 mins in cases vs 180 mins in controls. All cause mortality > 30 days was 5.9% for cases and 8.2% for controls. Cox regression analysis showed that survival was not influenced by VF. During follow-up 25 cases received an ICD against 3 in controls. Even if all ICD wearers were presumed dead, VF did not reach significance as a predictor for survival. Significant predictors for improved survival were: younger age ( p =0.013) and time to PCI ( p =0.027). Conclusion : VF during AMI does not predict survival in those who survive the first month. These VF survivors have an excellent prognosis with or without an ICD. This study underlines that primary VF by itself does not constitute an indication for ICD implantation.


Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Tetsuji Shinohara ◽  
Mikiko Nakagawa ◽  
Naohiko Takahashi

Introduction: Brugada syndrome (BrS) and idiopathic ventricular fibrillation (VF) associated with infero-lateral early repolarization patterns (early repolarization syndrome (ERS)) are termed “J-wave syndromes”. In such patients, an implantable cardioverter-defibrillator (ICD) is first-line therapy for prevention of sudden cardiac death. However, frequent ICD shocks due to recurrent VF remain serious problems. We investigated if combination therapy using cilostazol and bepridil could suppress recurrent VF. Methods: Our cohort comprised 22 consecutive patients (BrS, 16; ERS, 3; unknown, 3) with J-wave syndromes in whom ICDs were implanted for spontaneous episodes of VF. We enrolled 7 patients with J-wave syndromes who experienced ICD shocks due to recurrent VF after ICD implantation. At first, cilostazol was instituted. In all subjects, palpitations due to sinus tachycardia caused by cilostazol were symptomatic. Addition of bepridil attenuated cilostazol-induced palpitations (87±12 to 66±7 bpm, P<0.01) and maintained the suppressive effect of cilostazol against VF. Results: Prior to the onset of the cilostazol/bepridil combination therapy, all 7 patients had a total of 20 shocks over an accumulated period of 79 months. In contrast, after the onset of therapy, they accumulated a combined follow-up period of 375 months, and during that significantly longer period only one of them received 2 shocks. Three patients underwent replacement of the ICD generator 4-5 years after ICD implantation. Cilostazol was discontinued 2 days before replacement because of its anti-platelet effects. In all 3 patients, temporary discontinuation of cilostazol led to the reappearance of J waves, culminating in VF and an appropriate ICD shock in one of them. J waves disappeared upon re-institution of cilostazol. Conclusions: These data suggest that combination therapy of cilostazol and bepridil may be effective and safe in suppressing VF recurrence in some cases of J-wave syndromes.


Circulation ◽  
2007 ◽  
Vol 116 (suppl_16) ◽  
Author(s):  
Brian M Ramza ◽  
Andrew C Kao ◽  
William C Daniel

Introduction: Specific guidelines exist for identification and treatment of patients at risk for sudden cardiac arrest (SCA); however, little data exist describing how often these patients are accurately identified and appropriately treated. The objectives of this prospective, single center study were to compare the effect of an electronic reminder and protocol on the ability to recognize patients at risk for SCA and the application of the guidelines for therapy. Methods: In this study an electronic reminder and protocol were used at one site, the main clinic (MC), compared to all other sites, regional clinics (RC). The protocol was based on current AHA/ACC and CMS guidelines with practice specific quality measures. The protocol included optimal medical treatment prior to ICD, temporary and absolute exclusions for ICD therapy. MC staff was trained to use a new process designed to better identify patients at risk for SCA. This included screening forms, a standardized patient education video, and a patented reminder tool for EMR systems to continue tracking patients that did not immediately qualify for ICD therapy. An independent review of medical charts for all patients visiting the MC and RC over a 2-month period was then conducted to evaluate the clinics ability to identify patients at risk for SCA and apply the current guidelines for appropriate care. Patients were followed for 6 months. Results: Table 1 . Recognition of patients at risk for SCA improved significantly from 70% at RC to 93% at MC. Application of the guidelines into practice as measured ultimately by optimal medical treatment combined with ICD implantation was significantly improved as well. Conclusions: Recognition rates of patients at risk for SCA and subsequent adoption of guideline-based therapy including optimal medical therapy and ICD implantation was significantly greater with the use of this novel protocol combining AHA/ACC and CMS guidelines with practice specific quality measures. Table 1


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