Implantable cardioverter–defibrillator in hypertrophic cardiomyopathy

Abstract

Sudden cardiac death (SCD) is the most devastating complication in hypertrophic cardiomyopathy (HCM). The implantable cardioverter–defibrillator (ICD) has proven to be effective in SCD prevention in several clinical scenarios. In HCM population, it has demonstrated to successfully abort life-threatening ventricular arrhythmias despite the extreme morphology characteristic of HCM, often with massive degrees of left ventricular hypertrophy and/or LV outflow tract obstruction. Studies showed a high rate of appropriate intervention in secondary prevention and in primary prevention of patients considered at high risk. This appropriate intervention rate is even more significant considering the young and otherwise healthy patients that compose HCM population. Since SCD incidence in HCM is relatively low, optimal identification of patients at high risk is crucial. Classical strategy of risk stratification based on clinical risk factors has several limitations and has proven to overestimate risk. A new risk prediction model that provides individual 5-year estimated risk appears to be superior to traditional models based on bivariate risk factors. Perioperative complications seem to be similar to those related to the implant of other cardiac devices, while long-term complications have been traditionally in the spotlight. HCM patients are considered more vulnerable to ICD-related complications and inappropriate ICD therapy because of their young age at implant and increased prevalence of atrial fibrillation, but long-term follow-up data on ICD-related complications in general practice is limited. The subcutaneous implantable cardioverter defibrillator seems to be a safe and effective alternative in HCM, although long-term data are scarce.

Genetically Associated Hypertrophic Cardiomyopathy Combined with Persistent Left Superior Vena Cava

“…PLSVC is the most common thoracic congenital malformation. It is an isolated malformation in our case but it can be associated with atrial septal defect, single atrium, ventricular septal defect, tetralogy of Fallot, coarctation of the aorta, pulmonary stenosis and anomalous pulmonary venous return (10). Certain inherited HCM are more prone to develop atrioventricular blocks like PRKAG2.…”

Cardiodesfibrilador implantable: estado del arte

“…La presencia de hipertrofia ventricular izquierda y arritmias ventriculares predispone a estos pacientes a sufrir MSC. Los factores de riesgo reconocidos para muerte súbita en la miocardiopatía hipertrófica se resumen en la tabla 4 23 . El CDI ha mostrado ser efectivo en la prevención de la MSC en varios escenarios clínicos de la miocardiopatía hipertrófica, en prevención tanto primaria como secundaria 24 .…”

Screening for hypertrophic cardiomyopathy

“…Patients who have exercise-induced symptoms such as chest pain and syncope are candidates for pharmacologic treatment 34. The primary goal of medication management is to reduce symptoms and control hypertension 8,35. Medications that have been used in the past include beta-blockers (atenolol, metoprolol, and propranolol), calcium channel blockers (verapamil and diltiazem), HMG-CoA reductase inhibitors (atorvastatin), angiotensin-converting enzyme (ACE) inhibitors or angiotensin II receptor blockers, and antiarrhythmics (disopyramide and amiodarone) 35.…”

“…Undiagnosed HCM may lead to sudden cardiac death, especially in young athletes. Nonsustained ventricular tachycardia and ventricular premature beats are common dysrhythmias in patients with HCM 35. Additionally, supraventricular dysrhythmias also may be present in patients with HCM.…”

“…8,35 Medications that have been used in the past include beta-blockers (atenolol, metoprolol, and propranolol), calcium channel blockers (verapamil and diltiazem), HMG-CoA reductase inhibitors (atorvastatin), angiotensin-converting enzyme (ACE) inhibitors or angiotensin II receptor blockers, and antiarrhythmics (disopyramide and amiodarone). 35 Beta-blockers inhibit sympathetic stimuli, reducing heart rate and myocardial workload. 36 Patients may be candidates for an ICD if their risk of sudden cardiac death is high.…”