Background— Implantable cardioverter-defibrillator indications in Brugada syndrome remain controversial, especially in asymptomatic patients. Previous outcome data are limited by relatively small numbers of patients or short follow-up durations. We report the outcome of patients with Brugada syndrome implanted with an implantable cardioverter-defibrillator in a large multicenter registry.

Methods and Results— A total of 378 patients (310 male; age, 46±13 years) with a type 1 Brugada ECG pattern implanted with an implantable cardioverter-defibrillator (31 for aborted sudden cardiac arrest, 181 for syncope, and 166 asymptomatic) were included. Fifteen patients (4%) were lost to follow-up. During a mean follow-up of 77±42 months, 7 patients (2%) died (1 as a result of an inappropriate shock), and 46 patients (12%) had appropriate device therapy (5±5 shocks per patient). Appropriate device therapy rates at 10 years were 48% for patients whose implantable cardioverter-defibrillator indication was aborted sudden cardiac arrest, 19% for those whose indication was syncope, and 12% for the patients who were asymptomatic at implantation. At 10 years, rates of inappropriate shock and lead failure were 37% and 29%, respectively. Inappropriate shock occurred in 91 patients (24%; 4±4 shocks per patient) because of lead failure (n=38), supraventricular tachycardia (n=20), T-wave oversensing (n=14), or sinus tachycardia (n=12). Importantly, introduction of remote monitoring, programming a high single ventricular fibrillation zone (>210–220 bpm), and a long detection time were associated with a reduced risk of inappropriate shock.

Conclusions— Appropriate therapies are more prevalent in symptomatic Brugada syndrome patients but are not insignificant in asymptomatic patients (1%/y). Optimal implantable cardioverter-defibrillator programming and follow-up dramatically reduce inappropriate shock. However, lead failure remains a major problem in this population.

# Clinical Perspective {#article-title-24}

Outcome After Implantation of a Cardioverter-Defibrillator in Patients With Brugada Syndrome A Multicenter Study

Background—Implantable cardioverter-defibrillator indications in Brugada syndrome remain controversial, especially in asymptomatic patients. Previous outcome data are limited by relatively small nu...

https://tamthuvn.com/Reprints/SacherBrugadaFollowup2006.pdf

Outcome After Implantation of a Cardioverter-Defibrillator in Patients With Brugada Syndrome

https://www.heartrhythmjournal.com/article/S1547-5271(15)01177-7/pdf

Implantable cardioverter-defibrillator harm in young patients with inherited arrhythmia syndromes: A systematic review and meta-analysis of inappropriate shocks and complications

Quinidine, a life-saving medication for Brugada syndrome, is inaccessible in many countries.

Importance Drug-induced hypersensitivity syndrome (DIHS), also known as drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome, is a potentially life-threatening reaction to medications with a mortality rate up to 10%. Standard therapy involves the use of systemic corticosteroids with tapering doses extending up to 9 months after the initial reaction. Alternative treatments for DIHS are needed, especially for patients for whom systemic corticosteroids are contraindicated. Objective To assess a short course of cyclosporine as first-line therapy for DIHS. Design, Setting, and Participants In this case series, 2 patients referred to the dermatology service of an academic tertiary care hospital and subsequently diagnosed as having DIHS were studied from December 1, 2013, through July 31, 2014. Interventions Short course (3-7 days) of cyclosporine. Main Outcomes and Measures Clinical and laboratory indicators were examined to determine the timing and efficacy of cyclosporine treatment. Results Two cases are reported of drug hypersensitivity reaction that were treated with cyclosporine, resulting in rapid and significant clinical improvement. The first case involved a woman in her 40s who developed DIHS after treatment with carbamazepine. A 7-day course of cyclosporine resulted in clinical resolution of the DIHS. The second case was that of a man in his 30s with minocycline-induced DIHS. A 3-day course of cyclosporine resulted in rapid and sustained clinical improvement. Conclusions and Relevance A short course of cyclosporine was of therapeutic benefit in the treatment of 2 patients with DIHS. Short courses of cyclosporine in the acute care setting may be an alternative to longer courses of systemic corticosteroids in the treatment of DIHS.

Cyclosporine Treatment of Drug-Induced Hypersensitivity Syndrome.

Outcome after implantable cardioverter-defibrillator in patients with Brugada syndrome: the Gulf Brugada syndrome registry

Drug rash, eosinophilia, and systemic symptoms (DRESS) syndrome represents one pattern of the cutaneous involvement in type IV hypersensitivity reaction to drugs. It is a severe, delayed, idiosyncratic reaction presented as rash with fever, lymphadenopathy, and visceral involvement. There are several reported cases of sulfasalazine-induced DRESS syndrome, but myocardial involvement was rare. High index of suspicion is needed in every patient receiving these drugs for prompt diagnosis and early management. We report a case of a 56-year-old woman treated with sulfasalazine for ankylosing spondylitis for 3 weeks, which was discontinued after development of DRESS syndrome. Despite treating her with high dose of steroid and cyclosporine, her symptoms persisted, and ultimately, she developed toxic myocarditis with a misleading presentation of acute ST-elevated myocardial infarction. The diagnosis was made based on postmortem histopathologic finding.

Acute myocardial infarction in a 56-year-old female patient treated with sulfasalazine.

All procedures have inherent risk. Our patient endured a sequence of rare life-threatening complications from commonly preformed procedures. The sequence of these complications was; large pericardial effusion post implantable cardioverter-defibrillator (ICD) implantation with echocardiographic signs of tamponade, left main narrowing post radiofrequency ablation, and late stent thrombosis post coronary intervention with a bare metal stent. All these occurred to one unfortunate young man. Furthermore, our patient demonstrated an unintended benefit of ICD which saved his life.

Unintended Harm and Benefit of the Implantable Defibrillator in an Unfortunate 19-Year-Old Male: Featuring a Sequence of Rare Life-threatening Complications of Cardiac Procedures

Abdul-Karim Al-Habib

Papers

Outcome after implantable cardioverter-defibrillator in patients with Brugada syndrome: the Gulf Brugada syndrome registry

Acute myocardial infarction in a 56-year-old female patient treated with sulfasalazine.

Unintended Harm and Benefit of the Implantable Defibrillator in an Unfortunate 19-Year-Old Male: Featuring a Sequence of Rare Life-threatening Complications of Cardiac Procedures