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Cardiomiopatía Dilatada , Corazón Auxiliar , Lamina Tipo A , Mutación , Humanos , Cardiomiopatía Dilatada/genética , Cardiomiopatía Dilatada/complicaciones , Cardiomiopatía Dilatada/terapia , Cardiomiopatía Dilatada/fisiopatología , Lamina Tipo A/genética , Masculino , Adulto , Persona de Mediana Edad , ElectrocardiografíaRESUMEN
Pathologic left ventricular remodeling and valvular heart disease may contribute to the clinical presentation and outcomes of patients presenting with heart failure, and limit the effectiveness of guideline-directed medical therapy. Although surgical interventions including surgical ventricular restoration techniques and valve repair or replacement are effective therapies, there is growing evidence that transcatheter interventions may be options for patients with persistent symptoms of heart failure despite optimal medical therapy, where surgical options may be limited. This scientific statement will review the current available and investigational percutaneous strategies for the management of structural contributors to heart failure: dilated left ventricular cardiomyopathies and valvular heart disease.
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Insuficiencia Cardíaca , Humanos , Insuficiencia Cardíaca/terapia , Enfermedades de las Válvulas Cardíacas/cirugía , Enfermedades de las Válvulas Cardíacas/terapia , Procedimientos Quirúrgicos Cardíacos/métodos , Cardiomiopatía Dilatada/terapia , Cardiomiopatía Dilatada/fisiopatologíaRESUMEN
Objective: To summarize the clinical characteristics of patients with end-stage heart failure who receive heart transplant under extracorporeal membrane oxygenation (ECMO) support. Methods: The clinical data of 12 pediatric patients who received heart transplant with ECMO support in the Seventh Medical Center of Chinese People's Liberation Army General Hospital and Guangdong Provincial People's Hospital, from January 2019 to December 2023 was collected. The data included sex, age, weight, diagnosis, pre-ECMO lactate level, left ventricular ejection fraction (LVEF), vasoactive-inotropic score (VIS), and preoperative ECMO running time. Surgical data included cold ischemia time of the donor heart, cardiopulmonary bypass time, intraoperative use of immunosuppressant, postoperative use of ECMO, duration of postoperative ECMO, rate of successful weaning from ECMO, and survival discharge rate. The paired t-test was performed to compare cardiac function indices before and after left ventricular decompression. Results: The 12 patients ranged in age from 1.1 to 15.8 years, and weighted from 8 to 63 kg. Ten children were diagnosed with dilated cardiomyopathy, one with myocardial underdensification, and one with a novel heterozygous mutation of the SCN5A gene causing overlap syndrome complicated by fatal arrhythmia. Before ECMO, the lactate ranged from 0.6 to>15.0 mmol/L, the LVEF from 6.5% to 43%, and VIS from 3 to 108. Four patients underwent left ventricular decompression supported by preoperative ECMO, and their pulse pressure was significantly increased after decompression ((17.8±2.1) vs. (9.8±1.5) mmHg, 1 mmHg=0.133 kPa, t=11.31, P=0.001), while there was no apparent change in LVEF ((26.8±4.4)% vs. (24.9±4.9)%, t=1.75, P=0.178). A total of 7 children received a second run of ECMO after surgery and 3 of them successfully weaned off ECMO and survived to discharge. In the entire cohort, 10 were successfully weaned from ECMO and 8 survived to discharge. Conclusions: For children with end-stage heart failure supported by ECMO, left ventricular decompression can significantly improve pulse pressure. These patients will eventually require heart transplantation.
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Oxigenación por Membrana Extracorpórea , Insuficiencia Cardíaca , Trasplante de Corazón , Humanos , Oxigenación por Membrana Extracorpórea/métodos , Niño , Masculino , Lactante , Femenino , Adolescente , Preescolar , Insuficiencia Cardíaca/terapia , Cardiomiopatía Dilatada/terapia , Cardiomiopatía Dilatada/cirugía , Función Ventricular Izquierda , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
Arrhythmias frequently accompany heart failure and left ventricular dysfunction. Tachycardias, atrial fibrillation, and premature ventricular contractions can induce a reversible form of dilated cardiomyopathy (CM) known as arrhythmia-induced CM (AiCM). The intriguing question is why certain individuals are more susceptible to AiCM, despite similar arrhythmia burdens. The primary challenge is determining the extent of arrhythmias' contribution to left ventricular systolic dysfunction. AiCM should be considered in patients with a mean heart rate of >100 beats/min, atrial fibrillation, or a PVC burden of >10%. Confirmation of AiCM occurs when CM reverses upon eliminating the responsible arrhythmia. Therapy choice depends on the specific arrhythmia, patient comorbidities, and preferences. After left ventricular function is restored, ongoing follow-up is essential if an abnormal myocardial substrate persists. Accurate diagnosis and treatment of AiCM have the potential to enhance patients' quality of life, improve clinical outcomes, and reduce hospital admissions and overall health care costs.
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Arritmias Cardíacas , Humanos , Arritmias Cardíacas/etiología , Arritmias Cardíacas/terapia , Arritmias Cardíacas/fisiopatología , Cardiomiopatías/etiología , Cardiomiopatías/terapia , Cardiomiopatías/fisiopatología , Cardiomiopatías/diagnóstico , Cardiomiopatía Dilatada/terapia , Cardiomiopatía Dilatada/fisiopatología , Cardiomiopatía Dilatada/etiologíaRESUMEN
BACKGROUND: Dilated cardiomyopathy (DCM) is one of the most common causes of heart failure. Multiple identified mutations in nexilin (NEXN) have been suggested to be linked with severe DCM. However, the exact association between multiple mutations of Nexn and DCM remains unclear. Moreover, it is critical for the development of precise and effective therapeutics in treatments of DCM. RESULTS: In our study, Nexn global knockout mice and mice carrying human equivalent G645del mutation are studied using functional gene rescue assays. AAV-mediated gene delivery is conducted through systemic intravenous injections at the neonatal stage. Heart tissues are analyzed by immunoblots, and functions are assessed by echocardiography. Here, we identify functional components of Nexilin and demonstrate that exogenous introduction could rescue the cardiac function and extend the lifespan of Nexn knockout mouse models. Similar therapeutic effects are also obtained in G645del mice, providing a promising intervention for future clinical therapeutics. CONCLUSIONS: In summary, we demonstrated that a single injection of AAV-Nexn was capable to restore the functions of cardiomyocytes and extended the lifespan of Nexn knockout and G645del mice. Our study represented a long-term gene replacement therapy for DCM that potentially covers all forms of loss-of-function mutations in NEXN.
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Cardiomiopatía Dilatada , Terapia Genética , Ratones Noqueados , Animales , Cardiomiopatía Dilatada/genética , Cardiomiopatía Dilatada/terapia , Ratones , Humanos , Dependovirus/genética , Miocitos Cardíacos/metabolismo , Modelos Animales de Enfermedad , Mutación , Vectores Genéticos/administración & dosificación , Técnicas de Transferencia de GenRESUMEN
AIMS: CD4+ T cells are activated during inflammatory dilated cardiomyopathy (iDCM) development to induce immunogenic responses that damage the myocardium. Low-intensity pulsed ultrasound (LIPUS), a novel physiotherapy for cardiovascular diseases, has recently been shown to modulate inflammatory responses. However, its efficacy in iDCM remains unknown. Here, we investigated whether LIPUS could improve the severity of iDCM by orchestrating immune responses and explored its therapeutic mechanisms. METHODS AND RESULTS: In iDCM mice, LIPUS treatment reduced cardiac remodelling and dysfunction. Additionally, CD4+ T-cell inflammatory responses were suppressed. LIPUS increased Treg cells while decreasing Th17 cells. LIPUS mechanically stimulates endothelial cells, resulting in increased secretion of extracellular vesicles (EVs), which are taken up by CD4+ T cells and alter their differentiation and metabolic patterns. Moreover, EVs selectively loaded with microRNA (miR)-99a are responsible for the therapeutic effects of LIPUS. The hnRNPA2B1 translocation from the nucleus to the cytoplasm and binding to caveolin-1 and miR-99a confirmed the upstream mechanism of miR-99a transport. This complex is loaded into EVs and taken up by CD4+ T cells, which further suppress mTOR and TRIB2 expression to modulate cellular differentiation. CONCLUSION: Our findings revealed that LIPUS uses an EVs-dependent molecular mechanism to protect against iDCM progression. Therefore, LIPUS is a promising new treatment option for iDCM.
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Cardiomiopatía Dilatada , Modelos Animales de Enfermedad , Vesículas Extracelulares , Ratones Endogámicos C57BL , MicroARNs , Transducción de Señal , Terapia por Ultrasonido , Función Ventricular Izquierda , Animales , Vesículas Extracelulares/metabolismo , Vesículas Extracelulares/trasplante , Cardiomiopatía Dilatada/metabolismo , Cardiomiopatía Dilatada/terapia , Cardiomiopatía Dilatada/patología , Cardiomiopatía Dilatada/genética , Cardiomiopatía Dilatada/inmunología , Cardiomiopatía Dilatada/fisiopatología , MicroARNs/metabolismo , MicroARNs/genética , Ondas Ultrasónicas , Remodelación Ventricular , Masculino , Células Th17/inmunología , Células Th17/metabolismo , Linfocitos T Reguladores/inmunología , Linfocitos T Reguladores/metabolismo , Caveolina 1/metabolismo , Caveolina 1/genética , Serina-Treonina Quinasas TOR/metabolismo , Células Cultivadas , Humanos , RatonesRESUMEN
BACKGROUND: Patients with nonischemic dilated cardiomyopathy (DCM), severe left ventricular (LV) dysfunction, and complete left bundle branch block benefit from cardiac resynchronization therapy (CRT). However, a large heterogeneity of response to CRT is described. Several predictors of response to CRT have been identified, but the role of the underlying genetic background is still poorly explored. OBJECTIVES: In the present study, the authors sought to define differences in LV remodeling and outcome prediction after CRT when stratifying patients according to the presence or absence of DCM-causing genetic background. METHODS: From our center, 74 patients with DCM subjected to CRT and available genetic testing were retrospectively enrolled. Carriers of causative monogenic variants in validated DCM-causing genes, and/or with documented family history of DCM, were classified as affected by genetically determined disease (GEN+DCM) (n = 25). Alternatively, by idiopathic dilated cardiomyopathy (idDCM) (n = 49). The primary outcome was long-term LV remodeling and prevalence of super response to CRT (evaluated at 24-48 months after CRT); the secondary outcome was heart failure-related death/heart transplant/LV assist device. RESULTS: GEN+DCM and idDCM patients were homogeneous at baseline with the exception of QRS duration, longer in idDCM. The median follow-up was 55 months. Long-term LV reverse remodeling and the prevalence of super response were significantly higher in the idDCM group (27% in idDCM vs 5% in GEN+DCM; P = 0.025). The heart failure-related death/heart transplant/LV assist device outcome occurred more frequently in patients with GEN+DCM (53% vs 24% in idDCM; P = 0.028). CONCLUSIONS: Genotyping contributes to the risk stratification of patients with DCM undergoing CRT implantation in terms of LV remodeling and outcomes.
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Terapia de Resincronización Cardíaca , Cardiomiopatía Dilatada , Remodelación Ventricular , Humanos , Femenino , Masculino , Cardiomiopatía Dilatada/genética , Cardiomiopatía Dilatada/terapia , Cardiomiopatía Dilatada/fisiopatología , Persona de Mediana Edad , Estudios Retrospectivos , Remodelación Ventricular/genética , Remodelación Ventricular/fisiología , Anciano , Resultado del Tratamiento , Insuficiencia Cardíaca/genética , Insuficiencia Cardíaca/terapia , Insuficiencia Cardíaca/fisiopatología , Adulto , Disfunción Ventricular Izquierda/genética , Disfunción Ventricular Izquierda/fisiopatología , Disfunción Ventricular Izquierda/terapia , Bloqueo de Rama/genética , Bloqueo de Rama/terapia , Bloqueo de Rama/fisiopatologíaRESUMEN
Myocardial work (MW) derived from pressure-strain loops is a novel non-invasive tool to assess left ventricular (LV) function, incorporating global longitudinal strain (GLS) by speckle tracking echocardiography and non-invasively assessed blood pressure. Studies on the role of MW in dilated cardiomyopathy (DCM) are still limited. Therefore, the aim of this study was to evaluate the potential value of MW for predicting adverse outcomes in patients with DCM. 116 consecutive patients with DCM who underwent heart catheterization were retrospectively recruited from June 2009 to July 2014. 34 patients (30%) met the composite endpoints for major adverse cardiac events (MACE) of cardiac transplantation, need for implantable cardioverter-defibrillator (ICD) therapy, heart failure hospitalization and all-cause mortality. Patients with DCM were followed up for a mean of 5.1 years (IQR: 2.2-9.1 years). Global work index (GWI) and global constructive work (GCW) were not only independent predictors but also provided incremental predictive values (Integrated discrimination improvement [IDI] > 0) of MACE in multivariate Cox models. Furthermore, Patients with GWI < 788 mm Hg% (HR 5.46, 95%CI 1.66-17.92, p = 0.005) and GCW < 1,238 mm Hg% (HR 4.46, 95%CI 1.53-12.98, p = 0.006) had higher risks of MACE. GWI and GCW assessed by strain imaging echocardiography may have an additional value beyond LV-EF and GLS for predicting adverse outcomes in DCM.
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Cardiomiopatía Dilatada , Valor Predictivo de las Pruebas , Función Ventricular Izquierda , Humanos , Cardiomiopatía Dilatada/fisiopatología , Cardiomiopatía Dilatada/diagnóstico por imagen , Cardiomiopatía Dilatada/mortalidad , Cardiomiopatía Dilatada/terapia , Cardiomiopatía Dilatada/complicaciones , Masculino , Femenino , Persona de Mediana Edad , Estudios Retrospectivos , Factores de Riesgo , Factores de Tiempo , Pronóstico , Anciano , Adulto , Cateterismo Cardíaco/efectos adversos , Trasplante de Corazón , Cardioversión Eléctrica/instrumentación , Cardioversión Eléctrica/efectos adversos , Desfibriladores Implantables , Insuficiencia Cardíaca/fisiopatología , Insuficiencia Cardíaca/mortalidad , Insuficiencia Cardíaca/terapia , Insuficiencia Cardíaca/diagnóstico por imagen , Contracción Miocárdica , Medición de Riesgo , Reproducibilidad de los Resultados , EcocardiografíaRESUMEN
We report a case of temporary Berlin Heart EXCOR® explantation in a pediatric patient with idiopathic dilated cardiomyopathy who suffered an uncontrollable inflow cannulation site infection while on bridge-to-transplantation. Despite failure to thrive and catheter-related infections, once free of the device, the patient was cured of infection using systemic antibiotics and surgical debridement. The patient underwent EXCOR® reimplantation after four months, and is awaiting heart transplantation in stable condition. A life-threatening ventricular assist device-related infection may require device explantation under conditions that may not fulfill conventional explantation criteria despite risks. Temporary explantation can be an effective strategy if isolated systolic dysfunction is managed carefully.
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Cardiomiopatía Dilatada , Remoción de Dispositivos , Corazón Auxiliar , Humanos , Corazón Auxiliar/efectos adversos , Remoción de Dispositivos/métodos , Cardiomiopatía Dilatada/cirugía , Cardiomiopatía Dilatada/terapia , Cardiomiopatía Dilatada/complicaciones , Masculino , Infecciones Relacionadas con Prótesis/cirugía , Trasplante de Corazón , Control de Infecciones/métodos , Antibacterianos/uso terapéutico , NiñoRESUMEN
Cardiac contractility modulation (CCM) is a novel device-based therapy used to treat patients with heart failure with reduced ejection fraction (HFrEF). In both randomized clinical trials and real-life studies, CCM has been shown to improve exercise tolerance and quality of life, reverse left ventricular remodeling, and reduce hospitalization in patients with HFrEF. In this case report, we describe for the first time the use of CCM combined with left bundle branch pacing (LBBP) cardiac resynchronization therapy pacemaker (CRT-P) implantation therapy in a female with a 22-year history of non-ischemic dilated cardiomyopathy. With the optimal medical therapy and cardiac resynchronization therapy (CRT) strategies, the patient's quality of life initially recovered to some extent, but began to deteriorate in the past year. Additionally, heart transplantation was not considered due to economic reasons and late stage systolic heart failure. This is the first case of CCM implantation in Fujian Province and the first report of a combined CCM and left bundle branch pacing CRT-P implantation strategy in a patient with non-ischemic etiology dilated cardiomyopathy in China.
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Terapia de Resincronización Cardíaca , Cardiomiopatía Dilatada , Insuficiencia Cardíaca , Marcapaso Artificial , Disfunción Ventricular Izquierda , Humanos , Femenino , Insuficiencia Cardíaca/terapia , Calidad de Vida , Cardiomiopatía Dilatada/complicaciones , Cardiomiopatía Dilatada/terapia , Volumen Sistólico , Resultado del Tratamiento , Disfunción Ventricular Izquierda/terapia , Electrocardiografía , Función Ventricular IzquierdaRESUMEN
Determination of the prognosis and treatment outcomes of dilated cardiomyopathy is a serious problem due to the lack of valid specific protein markers. Using in-depth proteome discovery analysis, we compared 49 plasma samples from patients suffering from dilated cardiomyopathy with plasma samples from their healthy counterparts. In total, we identified 97 proteins exhibiting statistically significant dysregulation in diseased plasma samples. The functional enrichment analysis of differentially expressed proteins uncovered dysregulation in biological processes like inflammatory response, wound healing, complement cascade, blood coagulation, and lipid metabolism in dilated cardiomyopathy patients. The same proteome approach was employed in order to find protein markers whose expression differs between the patients well-responding to therapy and nonresponders. In this case, 45 plasma proteins revealed statistically significant different expression between these two groups. Of them, fructose-1,6-bisphosphate aldolase seems to be a promising biomarker candidate because it accumulates in plasma samples obtained from patients with insufficient treatment response and with worse or fatal outcome. Data are available via ProteomeXchange with the identifier PXD046288.
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Cardiomiopatía Dilatada , Humanos , Cardiomiopatía Dilatada/terapia , Proteoma/genética , Proteómica , Biomarcadores , Coagulación SanguíneaRESUMEN
Programmed ventricular stimulation (PVS), a clinical tool introduced in the early 1980s, aims to prove the electrical vulnerability of the heart and, independent of spontaneous arrhythmia variability, to trigger arrhythmias under controlled conditions. A specific response is the inducibility of monomorphic sustained ventricular tachycardia. This depends on the underlying heart disease, e.g., only for coronary artery disease but not for nonischemic diseases. The value of pharmacologic arrhythmia control as serial electrical testing is uncertain. Up to now there seems to be no prognostic value of PVS concerning sudden cardiac death. PVS is used as a tool to monitor the results of ventricular tachycardia (VT)-catheter ablation in patients who were primarily inducible.
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Cardiomiopatía Dilatada , Enfermedad de la Arteria Coronaria , Taquicardia Ventricular , Humanos , Enfermedad de la Arteria Coronaria/complicaciones , Enfermedad de la Arteria Coronaria/diagnóstico , Enfermedad de la Arteria Coronaria/cirugía , Cardiomiopatía Dilatada/complicaciones , Cardiomiopatía Dilatada/diagnóstico , Cardiomiopatía Dilatada/terapia , Estudios de Seguimiento , Taquicardia Ventricular/diagnóstico , Taquicardia Ventricular/cirugía , Ventrículos Cardíacos , Estimulación Cardíaca ArtificialRESUMEN
Dilated cardiomyopathy (DCM) is a common heart muscle disorder of nonischemic etiology associated with heart failure development and the risk of malignant ventricular arrhythmias and sudden cardiac death. A tailored approach to risk stratification and prevention of sudden cardiac death is required in genetic DCM given its variable presentation and phenotypic severity. Currently, advances in cardiogenetics have shed light on disease mechanisms, the complex genetic architecture of DCM, polygenic contributors to disease susceptibility and the role of environmental triggers. Parallel advances in imaging have also enhanced disease recognition and the identification of the wide spectrum of phenotypes falling under the DCM umbrella. Genotype-phenotype associations have been also established for specific subtypes of DCM, such as DSP (desmoplakin) or FLNC (filamin-C) cardiomyopathy but overall, they remain elusive and not readily identifiable. Also, despite the accumulated knowledge on disease mechanisms, certain aspects remain still unclear, such as which patients with DCM are at risk for disease progression or remission after treatment. Imagenetics, that is, the combination of imaging and genetics, is expected to further advance research in the field and contribute to precision medicine in DCM management and treatment. In the present article, we review the existing literature in the field, summarize the established knowledge and emerging data on the value of genetics and imaging in establishing genotype-phenotype associations in DCM and in clinical decision making for DCM patients.
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Cardiomiopatía Dilatada , Humanos , Cardiomiopatía Dilatada/diagnóstico , Cardiomiopatía Dilatada/genética , Cardiomiopatía Dilatada/terapia , Medicina de Precisión/métodos , Muerte Súbita Cardíaca/etiología , Arritmias Cardíacas/genética , Estudios de Asociación GenéticaRESUMEN
BACKGROUND: Alcoholic cardiomyopathy (ACM) is a form of dilated cardiomyopathy (DCM) occurring secondary to long-standing heavy alcohol use and is associated with poor outcomes, but the cause-specific risks are insufficiently understood. METHOD: Between 1997 and 2018, we identified all patients with a first diagnosis of ACM or DCM. The cumulative incidence of different causes of hospitalisation and mortality in the two groups was calculated using the Fine-Gray and Kaplan-Meier methods. RESULTS: A Total of 1,237 patients with ACM (mean age 56.3±10.1 years, 89% men) and 17,211 individuals with DCM (mean age 63.6±13.8 years, 71% men) were identified. Diabetes (10% vs 15%), hypertension (22% vs 31%), and stroke (8% vs 10%) were less common in ACM than DCM, whereas obstructive lung disease (15% vs 12%) and liver disease (17% vs 2%) were more prevalent (p<0.05). Cumulative 5-year mortality was 49% in ACM vs 33% in DCM, p<0.0001, multivariable adjusted hazards ratio 2.11 (95% confidence interval 1.97-2.26). The distribution of causes of death was similar in ACM and DCM, with the predominance of cardiovascular causes in both groups (42% in ACM vs 44% in DCM). 5-year cumulative incidence of heart failure hospitalisations (48% vs 54%) and any somatic cause (59% vs 65%) were also similar in ACM vs DCM. At 1 year, the use of beta blockers (55% vs 80%) and implantable cardioverter defibrillators (3% vs 14%) were significantly less often used in ACM vs DCM. CONCLUSIONS: Patients with ACM had similar cardiovascular risks and hospitalisation patterns as other forms of DCM, but lower use of guideline-directed cardiovascular therapies and greater mortality.
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Cardiomiopatía Alcohólica , Cardiomiopatía Dilatada , Desfibriladores Implantables , Insuficiencia Cardíaca , Masculino , Humanos , Persona de Mediana Edad , Anciano , Femenino , Cardiomiopatía Dilatada/diagnóstico , Cardiomiopatía Dilatada/epidemiología , Cardiomiopatía Dilatada/terapia , Cardiomiopatía Alcohólica/diagnóstico , Cardiomiopatía Alcohólica/epidemiología , Cardiomiopatía Alcohólica/terapia , Desfibriladores Implantables/efectos adversos , IncidenciaAsunto(s)
Cardiomiopatía Dilatada , Humanos , Cardiomiopatía Dilatada/terapia , Cardiomiopatía Dilatada/complicaciones , Cardiomiopatía Dilatada/diagnóstico , Estimulación Cardíaca Artificial/métodos , Electrocardiografía , Masculino , Bloqueo de Rama/diagnóstico , Bloqueo de Rama/terapia , Bloqueo de Rama/etiología , Femenino , Tabiques Cardíacos/diagnóstico por imagen , Anciano , Fascículo Atrioventricular/fisiopatologíaRESUMEN
BACKGROUND: Outcome comparisons among subcutaneous implantable cardioverter-defibrillator (S-ICD) recipients with nonischemic cardiomyopathies are scarce. OBJECTIVE: The aim of this study was to evaluate differences in device-related outcomes among S-ICD recipients with different structural substrates. METHODS: Patients enrolled in the i-SUSI (International SUbcutaneouS Implantable cardioverter defibrillator registry) project were grouped according to the underlying substrate (ischemic vs nonischemic) and subgrouped into dilated cardiomyopathy, hypertrophic cardiomyopathy, Brugada syndrome (BrS), arrhythmogenic right ventricular cardiomyopathy (ARVC). The main outcome of our study was to compare the rates of appropriate and inappropriate shocks and device-related complications. RESULTS: Among 1698 patients, the most common underlying substrate was ischemic (31.7%), followed by dilated cardiomyopathy (20.5%), BrS (10.8%), hypertrophic cardiomyopathy (8.5%), and ARVC (4.4%). S-ICD for primary prevention was more common in the nonischemic cohort (70.9% vs 65.4%; P = .037). Over a median (interquartile range) follow-up of 26.5 (12.6-42.8) months, no differences were observed in appropriate shocks between ischemic and nonischemic patients (4.8%/y vs 3.9%/y; log-rank, P = .282). ARVC (9.0%/y; hazard ratio [HR] 2.492; P = .001) and BrS (1.8%/y; HR 0.396; P = .008) constituted the groups with the highest and lowest rates of appropriate shocks, respectively. Device-related complications did not differ between groups (ischemic: 6.4%/y vs nonischemic: 6.1%/y; log-rank, P = .666), nor among underlying substrates (log-rank, P = .089). Nonischemic patients experienced higher rates of inappropriate shocks than did ischemic S-ICD recipients (4.4%/y vs 3.0%/y; log-rank, P = .043), with patients with ARVC (9.9%/y; P = .001) having the highest risk, even after controlling for confounders (adjusted HR 2.243; confidence interval 1.338-4.267; P = .002). CONCLUSION: Most S-ICD recipients were primary prevention nonischemic cardiomyopathy patients. Among those, patients with ARVC tend to receive the most frequent appropriate and inappropriate shocks and patients with BrS the least frequent appropriate shocks.