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1.
Am J Cardiol ; 125(11): 1738-1744, 2020 Jun 01.
Artigo em Inglês | MEDLINE | ID: mdl-32295701

RESUMO

Transvenous neurostimulation of the phrenic nerve (PNS) is a potentially improved and unique approach to the treatment of central sleep apnea (CSA). There have been multiple studies with limited individuals evaluating the efficacy of PNS. Our aim was to review and pool those studies to better understand whether phrenic nerve stimulation is efficacious in the treatment of CSA. The initial search on Pubmed retrieved a total of 97 articles and after screening all articles, only 5 could be included in our quantitative analysis. Pooling of data from 5 studies with a total of 204 patients demonstrated a reduction of mean apnea hypopnea index with PNS compared to controls by -26.7 events/hour with 95% confidence interval and P value of [CI (-31.99, -21.46), I2 85, p 0.00]. The mean difference in central apnea index was -22.47 [CI (-25.19, -19.76), I2 0, p 0.00]. The mean reduction in the oxygen desaturation index of 4% or more demonstrated a decrease in PNS group by -24.16 events/hour [(CI -26.20, -22.12), I2 0, p 0.00] compared with controls. PNS resulted in mean reduction in arousal index of -13.77 [CI (-16.15, -11.40), I2 0, p 0.00]. The mean change in percent of time spent in rapid eye movement sleep demonstrated a nonsignificant increase in PNS group by 1.01 % [CI (-5.67, 7.86), I293, p 0.75]. In conclusion, PNS therapy for treating CSA demonstrated positive outcomes but larger randomized studies are needed to evaluate the safety and clinical outcomes.

2.
Am J Cardiol ; 2020 Apr 02.
Artigo em Inglês | MEDLINE | ID: mdl-32305220

RESUMO

Patients with hypertrophic cardiomyopathy (HC) may require higher energies to terminate ventricular fibrillation (VF); thus, dual coil defibrillation leads are often implanted. However, single coil leads may be preferred in young patients. All patients with HCM implanted with a transvenous ICD from years 2000 to 2014 were included. Of 249 patients, 223 underwent VF testing including 150 with a dual coil lead and 73 a single coil. Patients tested with dual coil compared with single coil had lower successful VF energies (15.7 ± 6.1 joule to 20.2 ± 7.9 joule (p <0.0001)). Adequate safety margin for defibrillation was noted in 97.3% of patients. Notably, 6 (4 with single coil leads) had inadequate safety margins (defined as ≥10 joule). Three of these 6 patients required replacement of a single coil lead with a dual coil lead. The remaining 3 underwent waveform tilt alteration, higher energy ICD, or removal of the can from the shock vector. There were no clinical or implant predictors of inadequate safety margins. In follow-up of 16 ± 30 months (range 0 to 170), there were 24 arrhythmias including 13 VF, all successfully terminated. In conclusion, in HC patients undergoing ICD implantation, single coil leads can provide adequate safety margins. In conclusion, defibrillation testing should be considered in all HC patients undergoing ICD implantation, and should be performed in those undergoing implantation with a single coil lead.

3.
Clin Lung Cancer ; 21(1): 21-27.e5, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31780402

RESUMO

BACKGROUND: Concomitant medication use, including agents that prolong the corrected QT (QTc) interval, can result in the exclusion of patients with cancer from clinical trials. To estimate the potential effects on accrual, we determined the prevalence of QTc-prolonging medication prescriptions in a national patient cohort. PATIENTS AND METHODS: We identified adult patients in the Veterans Affairs system with a diagnosis of lung cancer from 2003 to 2016. The use of QTc interval-prolonging medications and risk category were obtained from CredibleMeds. We calculated the prevalence of prescriptions for QTc-prolonging medications with a known or possible risk of torsade de pointes in the 3 months up to and including the date of cancer diagnosis. The rates across patient groups were compared using χ2 test. RESULTS: A total of 280,068 patients were included in the present study. The mean age was 70 years, 98% were male, and 72% were white. Overall, 28.4% had been prescribed a QTc-prolonging medication, and 7.3% had been prescribed ≥2 in the 3 months before the cancer diagnosis. The most commonly prescribed QTc-prolonging medications were antimicrobial agents (14.0%), psychiatric agents (10.2%), antiemetic agents (2.6%), and cardiac medications (1.7%). Excluding the antimicrobial agents, 18.4% of the patients had been prescribed a QTc-prolonging medication. CONCLUSIONS: A substantial proportion of individuals with lung cancer will be prescribed QTc-prolonging medications. These prescriptions can limit patients' eligibility for clinical trials and complicate the administration of standard cancer therapies. Further research into the actual clinical risks and optimal management of QTc-prolonging medications in cancer populations is warranted.

4.
Heart Rhythm ; 17(2): 305-312, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31521807

RESUMO

BACKGROUND: Sudden cardiac death due to malignant arrhythmias is a common cause of death in dilated cardiomyopathy (DCM). Whether genetic variants increase the risk of arrhythmias in DCM is unknown. OBJECTIVE: The purpose of this study was to investigate the genetic causes of arrhythmias in DCM patients. METHODS: Whole-exome sequencing and high-depth targeted next-generation sequencing (142-gene panel) were used. Eight specific DCM pedigrees with arrhythmias and 2 separate cohorts of 1232 consecutive unrelated sporadic DCM patients from 3 medical centers (550 in the discovery cohort, 682 in the replication cohort) were analyzed; 470 (250 in the discovery cohort, 220 in the replication cohort) suffered from arrhythmias (DCM-A group) and 762 (300 in the discovery cohort, 462 in the replication cohort) did not (DCM-NA group). All identified causative variants were Sanger sequenced to eliminate false-positive results and then screened in 700 unrelated matched arrhythmia- and DCM-free healthy controls. RESULTS: We identified long QT syndrome (LQTS)-causative variants that independently cosegregated in 2 unrelated DCM-LQTS pedigrees. Pathogenic variants in arrhythmia-related genes (ion channelopathies) were identified in 4.9% (23/470) of sporadic DCM-A patients (4.0% in the discovery cohort, 5.9% in the replication cohort) but only 0.1% (1/762) of sporadic DCM-NA patients (P = 2.16 × 10-9). These arrhythmia-related pathogenic variants included long QT syndrome, atrial fibrillation, sick sinus syndrome, cardiac conduction disease, and Brugada syndrome. CONCLUSION: Some arrhythmias in DCM patients are caused by arrhythmia-related pathogenic variants. For DCM patients with explicit arrhythmias, arrhythmia-causative genetic screening may help to explain the etiology and decision-making.

5.
Artigo em Inglês | MEDLINE | ID: mdl-31862214

RESUMO

Hypertrophic cardiomyopathy (HCM) has a variable clinical presentation due to the diversity of causative genetic mutations. Animal models allow in vivo study of genotypic expression through non-invasive imaging, pathologic sampling, and force analysis. This review focuses on the spontaneous and induced mutations in various animal models affecting mainly sarcomere proteins. The sarcomere is comprised of thick (myosin) filaments and related proteins including myosin heavy chain and myosin binding protein-C; thin (actin) filament proteins and their associated regulators including tropomyosin, troponin I, troponin C, and troponin T. The regulatory milieu including transcription factors and cell signaling also play a significant role. Animal models provide a layered approach of understanding beginning with the causative mutation as a foundation. The functional consequences of protein energy utilization and calcium sensitivity in vivo and ex vivo can be studied. Beyond pathophysiologic disruption of sarcomere function, these models demonstrate the clinical sequalae of diastolic dysfunction, heart failure, and arrhythmogenic death. Through this cascade of understanding the mutation followed by their functional significance, targeted therapies have been developed and are briefly discussed.

6.
Heart Rhythm ; 16(11): e373-e407, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31676023

RESUMO

Arrhythmogenic cardiomyopathy (ACM) is an arrhythmogenic disorder of the myocardium not secondary to ischemic, hypertensive, or valvular heart disease. ACM incorporates a broad spectrum of genetic, systemic, infectious, and inflammatory disorders. This designation includes, but is not limited to, arrhythmogenic right/left ventricular cardiomyopathy, cardiac amyloidosis, sarcoidosis, Chagas disease, and left ventricular noncompaction. The ACM phenotype overlaps with other cardiomyopathies, particularly dilated cardiomyopathy with arrhythmia presentation that may be associated with ventricular dilatation and/or impaired systolic function. This expert consensus statement provides the clinician with guidance on evaluation and management of ACM and includes clinically relevant information on genetics and disease mechanisms. PICO questions were utilized to evaluate contemporary evidence and provide clinical guidance related to exercise in arrhythmogenic right ventricular cardiomyopathy. Recommendations were developed and approved by an expert writing group, after a systematic literature search with evidence tables, and discussion of their own clinical experience, to present the current knowledge in the field. Each recommendation is presented using the Class of Recommendation and Level of Evidence system formulated by the American College of Cardiology and the American Heart Association and is accompanied by references and explanatory text to provide essential context. The ongoing recognition of the genetic basis of ACM provides the opportunity to examine the diverse triggers and potential common pathway for the development of disease and arrhythmia.

7.
Circulation ; 140(24): e881-e894, 2019 Dec 10.
Artigo em Inglês | MEDLINE | ID: mdl-31722552

RESUMO

The fundamentals of cardiac resuscitation include the immediate provision of high-quality cardiopulmonary resuscitation combined with rapid defibrillation (as appropriate). These mainstays of therapy set the groundwork for other possible interventions such as medications, advanced airways, extracorporeal cardiopulmonary resuscitation, and post-cardiac arrest care, including targeted temperature management, cardiorespiratory support, and percutaneous coronary intervention. Since 2015, an increased number of studies have been published evaluating some of these interventions, requiring a reassessment of their use and impact on survival from cardiac arrest. This 2019 focused update to the American Heart Association advanced cardiovascular life support guidelines summarizes the most recent published evidence for and recommendations on the use of advanced airways, vasopressors, and extracorporeal cardiopulmonary resuscitation during cardiac arrest. It includes revised recommendations for all 3 areas, including the choice of advanced airway devices and strategies during cardiac arrest (eg, bag-mask ventilation, supraglottic airway, or endotracheal intubation), the training and retraining required, the administration of standard-dose epinephrine, and the decisions involved in the application of extracorporeal cardiopulmonary resuscitation and its potential impact on cardiac arrest survival.

8.
Circulation ; 140(24): e895-e903, 2019 Dec 10.
Artigo em Inglês | MEDLINE | ID: mdl-31722563

RESUMO

Survival after out-of-hospital cardiac arrest requires an integrated system of care (chain of survival) between the community elements responding to an event and the healthcare professionals who continue to care for and transport the patient for appropriate interventions. As a result of the dynamic nature of the prehospital setting, coordination and communication can be challenging, and identification of methods to optimize care is essential. This 2019 focused update to the American Heart Association systems of care guidelines summarizes the most recent published evidence for and recommendations on the use of dispatcher-assisted cardiopulmonary resuscitation and cardiac arrest centers. This article includes the revised recommendations that emergency dispatch centers should offer and instruct bystanders in cardiopulmonary resuscitation during out-of-hospital cardiac arrest and that a regionalized approach to post-cardiac arrest care may be reasonable when comprehensive postarrest care is not available at local facilities.

12.
Resuscitation ; 141: 69-72, 2019 08.
Artigo em Inglês | MEDLINE | ID: mdl-31201884

RESUMO

BACKGROUND: Cardiac troponin is routinely tested in the post-cardiac arrest setting, but its utility in identifying ischaemic aetiology and predicting left ventricular systolic dysfunction (LVSD) and survival is not known. METHODS: In a retrospective single center registry, we identified 145 consecutive patients who had achieved return of spontaneous circulation after cardiac arrest and had undergone serial cardiac troponin T (cTnT) testing, echocardiogram, and expert adjudication of aetiology. Initial and peak cTnT were evaluated for assessing ischaemic aetiology, LVSD, and survival to discharge using area under the receiver operating characteristic curve (AUROC). RESULTS: Mean age was 61 ±â€¯14 years and 71% were men. Of the 145 arrests, 19% had an ischaemic aetiology, 68% had LVSD post-arrest, and 55% survived to discharge. All patients had a positive initial cTnT at 0.01 ng/mL (clinical cut-off). Even at higher cut-offs of 10×, 100× and 1000×, initial cTnT performed poorly (AUROC 0.57, 0.56, and 0.56) and peak cTnT performed modestly (AUROC 0.55, 0.61, and 0.62) as diagnostic tests for ischaemic aetiology. Similarly, even at higher cut-offs, initial (AUROC 0.60, 0.62, 0.55) and peak (AUROC 0.57, 0.61, and 0.62) cTnT performed poorly to modestly at predicting LVSD. The test performed poorly for predicting survival to discharge (AUROC for all <0.6). CONCLUSIONS: At both current and several-fold higher thresholds, cTnT does not perform sufficiently well to guide clinical decision-making or predict patient outcomes. Routine post-cardiac arrest testing of cTnT should be reevaluated.

13.
JAMA Cardiol ; 4(7): 644-657, 2019 Jul 01.
Artigo em Inglês | MEDLINE | ID: mdl-31116360

RESUMO

Importance: Strategies for reliable selection of high-risk patients with hypertrophic cardiomyopathy (HCM) for prevention of sudden cardiac death (SCD) with implantable cardioverter/defibrillators (ICDs) are incompletely resolved. Objective: To assess the reliability of SCD prediction methods leading to prophylactic ICD recommendations to reduce the number of SCDs occurring in patients with HCM. Design, Setting, and Participants: In this observational longitudinal study, 2094 predominantly adult patients with HCM consecutively evaluated over 17 years in a large HCM clinical center were studied. All patients underwent prospective ICD decision making relying on individual major risk markers derived from the HCM literature and an enhanced American College of Cardiology/American Heart Association (ACC/AHA) guidelines-based risk factor algorithm with complete clinical outcome follow-up. Data were collected from June 2017 to February 2018, and data were analyzed from February to July 2018. Main Outcomes and Measures: Arrhythmic SCD or appropriate ICD intervention for ventricular tachycardia or ventricular fibrillation. Results: Of the 2094 study patients, 1313 (62.7%) were male, and the mean (SD) age was 51 (17) years. Of 527 patients with primary prevention ICDs implanted based on 1 or more major risk markers, 82 (15.6%) experienced device therapy-terminated ventricular tachycardia or ventricular fibrillation episodes, which exceeded the 5 HCM-related SCDs occurring among 1567 patients without ICDs (0.3%), including 2 who declined device therapy, by 49-fold (95% CI, 20-119; P = .001). Cumulative 5-year probability of an appropriate ICD intervention was 10.5% (95% CI, 8.0-13.5). The enhanced ACC/AHA clinical risk factor strategy was highly sensitive for predicting SCD events (range, 87%-95%) but less specific for identifying patients without SCD events (78%). The C statistic calculated for enhanced ACC/AHA guidelines was 0.81 (95% CI, 0.77-0.85), demonstrating good discrimination between patients who did or did not experience an SCD event. Compared with enhanced ACC/AHA risk factors, the European Society of Cardiology risk score retrospectively applied to the study patients was much less sensitive than the ACC/AHA criteria (34% [95% CI, 22-44] vs 95% [95% CI, 89-99]), consistent with recognizing fewer high-risk patients. Conclusions and Relevance: A systematic enhanced ACC/AHA guideline and practice-based risk factor strategy prospectively predicted SCD events in nearly all at-risk patients with HCM, resulting in prophylactically implanted ICDs that prevented many catastrophic arrhythmic events in this at-risk population.

14.
Prog Cardiovasc Dis ; 62(3): 235-241, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31075279

RESUMO

Sudden cardiac death (SCD) is a major cause of mortality in patients with nonischemic cardiomyopathy (NICM). Identifying patients who are at highest risk for SCD is an ongoing challenge. At present, guidelines recommend the use of an implantable cardioverter-defibrillator (ICD) in patients with NICM with a reduced left ventricular ejection fraction (LVEF) and heart failure (HF) symptoms. Some recent data, however, suggest that ICDs may not increase longevity in this population. Conversely, community-based studies have demonstrated that many at-risk individuals who may benefit from ICD therapy remain unprotected. Current recommendations for ICD implantation are continually debated, justifying comprehensive individualized risk assessment. Various promising techniques for further risk stratification are under evaluation, including cardiac magnetic resonance imaging, electrocardiographic assessment of electrical instability, and genetic testing. However, none of these strategies has been fully adapted into guidelines. Hence, clinical risk stratification practice today depends on LVEF and HF symptoms, which have poor sensitivity and specificity for predicting SCD risk.


Assuntos
Cardiomiopatias/complicações , Morte Súbita Cardíaca/prevenção & controle , Desfibriladores Implantáveis , Cardiomiopatias/diagnóstico , Cardiomiopatias/fisiopatologia , Cardiomiopatias/terapia , Morte Súbita Cardíaca/etiologia , Ecocardiografia , Insuficiência Cardíaca/complicações , Humanos , Imagem por Ressonância Magnética , Medição de Risco , Sensibilidade e Especificidade , Função Ventricular Esquerda
15.
Prog Cardiovasc Dis ; 62(3): 272-278, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31078561

RESUMO

Therapeutic hypothermia, or targeted temperature management (TTM), is a strategy of reducing the core body temperature of survivors of sudden cardiac arrest (SCA) to minimize neurological damage caused by severe hypoxia. Initial clinical trials examining this technique demonstrated significant improvement in neurological function among survivors of out-of-hospital SCA with an initial shockable rhythm. Since then, TTM has become an integral part of the care provided to comatose survivors of SCA. However, multiple questions persist regarding the target cooling temperature, duration of cooling, and utility of TTM in patient populations such as survivors of out-of-hospital SCA with non-shockable rhythms or in-hospital SCA. This review article summarizes the current evidence regarding optimal application of TTM and compares the recommendations for TTM in current guidelines.


Assuntos
Parada Cardíaca/terapia , Hipotermia Induzida/métodos , Humanos , Guias de Prática Clínica como Assunto
16.
Prog Cardiovasc Dis ; 62(3): 227-234, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31078562

RESUMO

Sudden cardiac death (SCD) accounts for 230,000 to 350,000 deaths per year in the United States. While many who suffer SCD possess underlying structural heart disease, inherited arrhythmia syndromes are also important contributors to SCD. In patients without structural heart disease, inherited arrhythmia syndromes are identified in >50% of the remaining patients. In this review, we will focus on the presentation and management of three major inherited syndromes that lead to SCD in patients without structural heart disease: long QT syndrome (LQTS), Brugada syndrome, and catecholaminergic polymorphic ventricular tachycardia (CPVT). All these syndromes can present in patients who are asymptomatic or, at the other extreme, with syncope and even SCD. LQTS syndrome and Brugada are the most common inherited arrhythmogenic syndromes, while CPVT is much rarer. Determining which patients need pharmacologic treatment and those who would benefit from more aggressive treatment such as sympathectomies and implantable defibrillators is not always clear.


Assuntos
Síndrome de Brugada , Morte Súbita Cardíaca/etiologia , Síndrome do QT Longo , Taquicardia Ventricular , Síndrome de Brugada/complicações , Síndrome de Brugada/diagnóstico , Síndrome de Brugada/terapia , Desfibriladores Implantáveis , Eletrocardiografia , Humanos , Síndrome do QT Longo/complicações , Síndrome do QT Longo/diagnóstico , Síndrome do QT Longo/terapia , Medição de Risco , Taquicardia Ventricular/complicações , Taquicardia Ventricular/diagnóstico , Taquicardia Ventricular/terapia
17.
Heart Rhythm ; 16(11): e301-e372, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31078652

RESUMO

Arrhythmogenic cardiomyopathy (ACM) is an arrhythmogenic disorder of the myocardium not secondary to ischemic, hypertensive, or valvular heart disease. ACM incorporates a broad spectrum of genetic, systemic, infectious, and inflammatory disorders. This designation includes, but is not limited to, arrhythmogenic right/left ventricular cardiomyopathy, cardiac amyloidosis, sarcoidosis, Chagas disease, and left ventricular noncompaction. The ACM phenotype overlaps with other cardiomyopathies, particularly dilated cardiomyopathy with arrhythmia presentation that may be associated with ventricular dilatation and/or impaired systolic function. This expert consensus statement provides the clinician with guidance on evaluation and management of ACM and includes clinically relevant information on genetics and disease mechanisms. PICO questions were utilized to evaluate contemporary evidence and provide clinical guidance related to exercise in arrhythmogenic right ventricular cardiomyopathy. Recommendations were developed and approved by an expert writing group, after a systematic literature search with evidence tables, and discussion of their own clinical experience, to present the current knowledge in the field. Each recommendation is presented using the Class of Recommendation and Level of Evidence system formulated by the American College of Cardiology and the American Heart Association and is accompanied by references and explanatory text to provide essential context. The ongoing recognition of the genetic basis of ACM provides the opportunity to examine the diverse triggers and potential common pathway for the development of disease and arrhythmia.

18.
Circulation ; 140(5): 370-378, 2019 Jul 30.
Artigo em Inglês | MEDLINE | ID: mdl-31006260

RESUMO

BACKGROUND: Cardiopulmonary resuscitation (CPR) is initiated in hospitalized children with bradycardia and poor perfusion. However, their rate of progression to pulseless cardiac arrest despite CPR and the differences in survival compared with initially pulseless arrest are unknown. We examined the prevalence and predictors of survival of children who progress from bradycardia to pulseless in-hospital cardiac arrest despite CPR. METHODS: Pediatric patients >30 days and <18 years of age who received CPR at hospitals participating in Get With The Guidelines-Resuscitation during 2000 to 2016 were included. Each CPR event was classified as bradycardia with pulse, bradycardia with subsequent pulselessness, and initial pulseless cardiac arrest. We assessed risk-adjusted rates of survival to hospital discharge using multilevel Poisson regression models. RESULTS: Overall, 5592 pediatric patients were treated with CPR, of whom 2799 (50.1%) received CPR for bradycardia with poor perfusion and 2793 (49.9%) for initial pulseless cardiac arrest. Among those with bradycardia, 869 (31.0%, or 15.5% of cohort) became pulseless after a median of 3 minutes of CPR (interquartile range, 1-9 minutes). Rates of survival to discharge were 70.0% (1351 of 1930) for bradycardia with pulse, 30.1% (262 of 869) for bradycardia progressing to pulselessness, and 37.5% (1046 of 2793) for initial pulseless cardiac arrest (P for difference across groups <0.001). Children who became pulseless despite CPR for bradycardia had a 19% lower likelihood (risk ratio, 0.81 [95% CI, 0.70, 0.93]; P=0.004) of surviving to hospital discharge than those who were initially pulseless. Among children who progressed to pulselessness despite CPR for bradycardia, a longer interval between CPR and pulselessness was a predictor of lower survival (reference, <2 minutes; for 2-5 minutes, risk ratio, 0.54 [95% CI, 0.41-0.70]; for >5 minutes, risk ratio, 0.41 [95% CI, 0.32-0.53]). CONCLUSIONS: Among hospitalized children in whom CPR is initiated, half have bradycardia with poor perfusion at the initiation of chest compressions, and nearly one-third of these progress to pulseless in-hospital cardiac arrest despite CPR. Survival was significantly lower for children who progress to pulselessness despite CPR compared with those who were initially pulseless. These findings suggest that pediatric patients who lose their pulse despite resuscitation attempts are at particularly high risk and require a renewed focus on postresuscitation care.

19.
Am J Cardiol ; 124(1): 113-121, 2019 07 01.
Artigo em Inglês | MEDLINE | ID: mdl-31027655

RESUMO

Advances in treatment options for hypertrophic cardiomyopathy (HC) have proven effective in many patients for promoting favorable long-term outcomes. Whether this expectation is similar for patients with the most extreme expression of massive left ventricular (LV) hypertrophy, a particularly aggressive form of the disease is unresolved. Of 1,766 consecutive HC patients presenting to Tufts HC Institute (2004 to 2015), 92 were identified with extreme LV wall thickness (30 to 48 mm), and compared with 1,674 HC patients with less marked hypertrophy (13 to 29 mm). Follow-up assessment was over 5.3 ± 3.4 years. Patients with massive LV hypertrophy (n = 92) had higher sudden death event rates (3.0%/year) than did patients with lesser hypertrophy (0.8%/year; p <0.001). In 16 of the 92 patients (17%), potentially lethal ventricular tachyarrhythmia were successfully aborted by primary prevention implantable cardioverter defibrillator (ICD) therapy at 30 ± 13 years (n = 11), or by resuscitated cardiac arrest with external defibrillation (n = 5) and later by secondary prevention interventions (n = 3); no patient experienced arrhythmic sudden death. Aborted sudden death events (3.0%/year) exceeded HC-related mortality by 7-fold (n = 2; 0.4%/year; p <0.001). European Society of Cardiology risk score would have failed to identify 60% of patients with arrhythmic sudden death events, leaving them exposed to sudden death without ICDs. In addition, 35 patients required surgical myectomy for progressive heart failure due to LV outflow obstruction (improved to NYHA I/II in 30). Eighty-eight (96%) of the 92 patients have survived to age 38 ± 14 years (23% ≥ 50 years). All-cause mortality did not differ from an age and gender-matched general population (p = 0.62). In conclusion, in this referral-based population, patients with the most extreme expression of HC are at increased arrhythmic sudden death risk reliably prevented with prophylactic ICDs. Progressive heart failure secondary to outflow obstruction was reversible with surgical myectomy. Despite extreme phenotypic expression, with contemporary treatment interventions young HC patients have an opportunity to achieve extended survival with good quality of life.


Assuntos
Cardiomiopatia Hipertrófica/patologia , Cardiomiopatia Hipertrófica/terapia , Adolescente , Adulto , Cardiomiopatia Hipertrófica/mortalidade , Criança , Estudos de Coortes , Gerenciamento Clínico , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Fenótipo , Índice de Gravidade de Doença , Taxa de Sobrevida , Resultado do Tratamento , Adulto Jovem
20.
J Cardiovasc Electrophysiol ; 30(6): 886-895, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30847997

RESUMO

INTRODUCTION: Catheter ablation of ventricular tachycardia (VT) can be an effective therapy to reduce VT burden, but often it is limited by the potential for hemodynamic instability. Percutaneous left ventricular assist devices (pLVADs) have been used to maintain hemodynamic support during VT ablation but the evidence regarding its clinical impact has been inconclusive. METHODS AND RESULTS: We sought to assess the clinical impact of pLVAD when used in VT ablation by conducting a meta-analysis of the current evidence. We searched Pubmed and found nine observational studies that compared clinical outcomes of VT ablation in patients with pLVAD support to controls with no pLVAD support. The pooled data did not show a significant difference in mortality between both groups, nor a difference in acute procedural success or in recurrence of VT. There was also no difference in the number of patients receiving a cardiac transplant or being enrolled in the transplant list. Although there was no difference in the ablation time between the groups, patients in the pLVAD group had a longer total procedural time and more procedure-related adverse effects. CONCLUSION: This meta-analysis did not show clinical benefits from using pLVAD support during VT ablation, whereas it was associated with longer procedure times and more complications. This study was, however, limited by the observational nature of the data. In view of these data, the risk and benefit of pLVAD support during VT ablation should be considered on an individual basis.

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