Hypertrophe Kardiomyopathie: Definition, klinische Präsentation, Risikostratifizierung für den plötzlichen Herztod und Therapieoptionen

2016 ◽  
Vol 73 (12) ◽  
pp. 759-765
Author(s):  
Bettina Muggli ◽  
Christiane Gruner
Keyword(s):  
Sudden Cardiac Death ◽  
Cardiac Death ◽  
Hypertrophe Kardiomyopathie ◽  
Linksventrikuläre Hypertrophie ◽  
Orale Antikoagulation ◽  
Diastolische Dysfunktion ◽  
Icd Implantation ◽  
Elektrische Kardioversion ◽  
Systemische Erkrankungen

Zusammenfassung. Die hypertrophe Kardiomyopathie (HCM) ist die häufigste hereditäre Kardiomyopathie und wird definiert durch eine unerklärte linksventrikuläre Hypertrophie mit normal grossen Ventrikeln ohne andere kardiale Ursachen oder systemische Erkrankungen. Die klinische Präsentation ist vielfältig und reicht vom asymptomatischen Familienmitglied bis hin zu Patienten mit Symptomen einer schweren Herzinsuffizienz. Häufige Probleme sind der plötzliche Herztod (’sudden cardiac death’ SCD) und die Risikostratifizierung der Patienten im Hinblick auf eine primärprophylaktische ICD-Implantation. Des Weiteren spielen natürlich die Obstruktion des linksventrikulären Ausflusstrakts und deren Behandlung (medikamentös, kathetertechnisch mittels Septalalkoholablation, chirugische Myektomie), diastolische Dysfunktion, Vorhofflimmern und die Entwicklung einer Herzinsuffizienz mit systolischer linksventrikulärer Dysfunktion eine wichtige Rolle bei der Betreuung von Patienten mit HCM. Beim Vorhofflimmern muss unabhängig vom CHA2DS2Vasc Score eine orale Antikoagulation begonnen werden und aufgrund der diastolischen Dysfunktion sollte wenn möglich wieder ein Sinusrhythmus erreicht werden durch medikamentöse Massnahmen, elektrische Kardioversion und / oder Pulmonalvenenisolation. Kommt es zur Entwicklung einer Herzinsuffizienz ist eine übliche Herzinsuffizienztherapie indiziert. Bei terminaler Herzinsuffizienz sollte frühzeitig eine Herztransplantation evaluiert werden.

scholarly journals LEFT VENTRICULAR LEAD IMPLANTATION FOR DETECTION OF VENTRICULAR ARRHYTHMIAS IN PATIENT WITH IMPLANTABLE CARDIOVERTER DEFIBRILLATOR AND LOW R WAVE IN RIGHT VENTRICLE

2021 ◽  
pp. 17
Author(s):  
Keyword(s):  
Sudden Cardiac Death ◽  
Right Ventricle ◽  
Implantable Cardioverter Defibrillator ◽  
Cardiac Death ◽  
Ventricular Arrhythmias ◽  
Systolic Function ◽  
Left Ventricular ◽  
Icd Implantation ◽  
Cardioverter Defibrillator ◽  
Ventricular Lead

Dilated cardiomyopathy (DCM) is a disease characterised as left ventricular (LV) or biventricular dilatation with impaired systolic function. Regardless of underlying cause patients with DCM have a propensity to ventricular arrhythmias and sudden cardiac death. Implantable Cardioverter Defibrillator (ICD) implantation for these patients results in significant reduction of sudden cardiac death [1-3]. ICD devices may be limited by right ventricle (RV) sensing dysfunction with low RV sensing amplitude. We present a clinical case of patient with DCM, implanted ICD and low R wave sensing on RV lead.

scholarly journals Dealing with Sudden Cardiac Death: Who Deserves Device Implantation

2019 ◽  
Vol 5 (1 (P)) ◽  
pp. 12
Author(s):  
Dicky Armein Hanafy
Keyword(s):  
Heart Failure ◽  
Coronary Heart Disease ◽  
Heart Disease ◽  
Sudden Cardiac Death ◽  
Cardiac Arrhythmias ◽  
Cardiac Death ◽  
Cardiac Insufficiency ◽  
Resynchronization Therapy ◽  
Icd Implantation ◽  
Life Threatening

Sudden cardiac death is one of the leading causes of death in the western industrial nations. Most people are affected by coronary heart disease (coronary heart disease, CHD) or heart muscle (cardiomyopathy). These can lead to life-threatening cardiac arrhythmias. If the heartbeat is too slow due to impulse or conduction disturbances, cardiac pacemakers will be implanted. High-frequency and life-threatening arrhythmias of the ventricles (ventricular tachycardia, flutter or fibrillation) cannot be treated with a pacemaker. In such cases, an implantable cardioverter-defibrillator (ICD) is used, which additionally also provides all functions of a pacemaker. The implantation of a defibrillator is appropriate if a high risk of malignant arrhythmias has been established (primary prevention). If these life-threatening cardiac arrhythmias have occurred before and are not caused by a treatable (reversible) cause, ICD implantation will be used for secondary prevention. The device can stop these life-threatening cardiac arrhythmias by delivering a shock or rapid impulse delivery (antitachycardic pacing) to prevent sudden cardiac death. Another area of application for ICD therapy is advanced heart failure (heart failure), in which both main chambers and / or different wall sections of the left ventricle no longer work synchronously. This form of cardiac insufficiency can be treated by electrical stimulation (cardiac resynchronization therapy, CRT). Since the affected patients are also at increased risk for sudden cardiac death, combination devices are usually implanted, which combine heart failure treatment by resynchronization therapy and the prevention of sudden cardiac death by life-threatening arrhythmia of the heart chambers (CRT-D device). An ICD is implanted subcutaneously or under the pectoral muscle in the area of the left collarbone. Like pacemaker implantation, ICD implantation is a routine, low-complication procedure today.

Abstract 11447: Low Incidence of Appropriate Therapy Following Defibrillator Implantation for Primary Prevention of Sudden Cardiac Death in Hypertrophic Cardiomyopathy

Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Amalie C Thavikulwat ◽  
Todd T Tomson ◽  
Bradley P Knight ◽  
Robert O Bonow ◽  
Lubna Choudhury
Keyword(s):  
Risk Factors ◽  
Risk Factor ◽  
Hypertrophic Cardiomyopathy ◽  
Primary Prevention ◽  
Sudden Cardiac Death ◽  
Secondary Prevention ◽  
Cardiac Death ◽  
Icd Implantation ◽  
Icd Therapy ◽  
Appropriate Therapy

Introduction: Hypertrophic cardiomyopathy (HCM) is a leading cause of sudden cardiac death (SCD) in young adults. Implantable cardioverter defibrillators (ICD) effectively terminate ventricular tachycardia (VT) and fibrillation (VF) that cause SCD, but the reported prevalence of and patient characteristics leading to appropriate ICD therapy in HCM have been variable. Hypothesis: We hypothesized that some risk factors may be more prevalent than others in patients with HCM who receive appropriate ICD therapy and that the overall incidence of appropriate therapy may be lower than that reported previously. Methods: We retrospectively studied all patients with HCM who were treated with ICDs at our referral center from 2000-2013 to determine the rates of appropriate and inappropriate ICD therapies. Results: Of 1136 patients with HCM, we identified 135 who underwent ICD implantation (125 for primary and 10 for secondary prevention), aged 18-81 years (mean 48±17) at the time of implantation. The mean follow-up time was 5.2±4.5 years. Appropriate ICD intervention occurred in 20 of 135 patients (2.8%/year) by providing a shock or antitachycardia pacing in response to VT or VF. The annual rate of appropriate ICD therapy was 2.4%/year for primary and 7.2%/year for secondary prevention devices. Commonly used risk factors were equally prevalent among patients who received appropriate therapy and those who did not; furthermore, the likelihood of receiving appropriate therapy in the presence of each risk factor was similar (Figure). Inappropriate ICD therapy occurred in 27 patients (3.8%/year). Conclusions: ICDs provide clear benefit to patients who experience life-threatening arrhythmias, particularly those being treated for secondary prevention. However, the appropriate therapy rate for primary prevention was lower than previously reported, and no single risk factor appeared to have stronger association with appropriate ICD therapy than others.

Hypertrophic cardiomyopathy: prevention of sudden cardiac death

2018 ◽  
Author(s):  
Constantinos O’Mahony
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Sudden Cardiac Death ◽  
Risk Stratification ◽  
Cardiac Death ◽  
Clinical Care ◽  
Psychological Impact ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Icd Implantation ◽  
European Society Of Cardiology

Sudden cardiac death (SCD) secondary to ventricular arrhythmias is the most common mode of death in hypertrophic cardiomyopathy (HCM) and can be effectively prevented with an implantable cardioverter defibrillator (ICD). The risk of SCD in HCM relates to the severity of the phenotype and regular risk stratification is an integral part of routine clinical care. For the primary prevention of SCD, risk stratification involves the assessment of seven readily available clinical parameters (age, maximal left ventricular wall thickness, left atrial diameter, left ventricular outflow tract gradient, non-sustained ventricular tachycardia, unexplained syncope, and family history of SCD) which are used to estimate the risk of SCD within 5 years of clinical evaluation using a statistical risk prediction model (HCM Risk-SCD). The 2014 European Society of Cardiology Guidelines provide a framework to aid clinical decisions and consider patients with a 5-year risk of SCD of less than 4% as low risk and recommend regular assessment while those with a risk of 6% or higher should be considered for an ICD. In patients with an intermediate risk (4% to <6%) ICD implantation may also be considered after taking into account age, co-morbid conditions, socioeconomic factors, and the psychological impact of therapy. Survivors of ventricular fibrillation arrest should receive an ICD for secondary prevention unless their life expectancy is less than 1 year. Following device implantation, patients should be followed up for device- and disease-related complications, particularly heart failure and cerebrovascular disease.

scholarly journals Aborted Sudden Cardiac Death in a Female Patient Presenting with Takotsubo-Like Cardiomyopathy due to Epicardial Coronary Vasospasm

2017 ◽  
Vol 2017 ◽  
pp. 1-4 ◽  
Author(s):  
Sorin Giusca ◽  
Tom Eisele ◽  
Peter Nunninger ◽  
Benedikt Münz ◽  
Grigorios Korosoglou
Keyword(s):  
Coronary Artery ◽  
Sudden Cardiac Death ◽  
Takotsubo Cardiomyopathy ◽  
Cardiac Death ◽  
Elderly Women ◽  
Apical Ballooning ◽  
Late Enhancement ◽  
Lv Function ◽  
Icd Implantation ◽  
Lv Dysfunction

Takotsubo cardiomyopathy is characterized by apical ballooning of the left ventricle (LV) in the absence of relevant coronary artery stenosis, which typically occurs in elderly women after emotional stress. Catecholamine cardiotoxicity, metabolic disturbance, and coronary microvascular impairment have previously been proposed as underlying pathophysiologic mechanisms of takotsubo cardiomyopathy, whereas myocardial stunning resulting from epicardial coronary artery vasospasm is not generally accepted as a cause of takotsubo cardiomyopathy. The prognosis of takotsubo cardiomyopathy is generally more favourable compared to myocardial infarction; however, severe complications such as rupture of the LV and life-threatening arrhythmias may occur. Herein, we describe a case of an 84-year-old female, who presented with aborted sudden cardiac death due to ventricular fibrillation. Echocardiography suggested LV apical ballooning with severely impaired LV-function, so that takotsubo cardiomyopathy was suspected. However, coronary angiography revealed epicardial spasm of the left anterior ascending, which resolved after intracoronary injection of 0.2 mg nitroglycerine. Cardiac magnetic resonance exhibited subendocardial late enhancement and echocardiography showed normalization of LV dysfunction during follow-up. The patient was put on conservative treatment with nitrates and calcium inhibitors and ICD implantation were deferred.

Hypertrophic cardiomyopathy: prevention of sudden cardiac death

ESC CardioMed ◽  
2018 ◽  
pp. 1462-1466
Author(s):  
Constantinos O’Mahony
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Sudden Cardiac Death ◽  
Risk Stratification ◽  
Cardiac Death ◽  
Clinical Care ◽  
Psychological Impact ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Icd Implantation ◽  
European Society Of Cardiology

Sudden cardiac death (SCD) secondary to ventricular arrhythmias is the most common mode of death in hypertrophic cardiomyopathy (HCM) and can be effectively prevented with an implantable cardioverter defibrillator (ICD). The risk of SCD in HCM relates to the severity of the phenotype and regular risk stratification is an integral part of routine clinical care. For the primary prevention of SCD, risk stratification involves the assessment of seven readily available clinical parameters (age, maximal left ventricular wall thickness, left atrial diameter, left ventricular outflow tract gradient, non-sustained ventricular tachycardia, unexplained syncope, and family history of SCD) which are used to estimate the risk of SCD within 5 years of clinical evaluation using a statistical risk prediction model (HCM Risk-SCD). The 2014 European Society of Cardiology Guidelines provide a framework to aid clinical decisions and consider patients with a 5-year risk of SCD of less than 4% as low risk and recommend regular assessment while those with a risk of 6% or higher should be considered for an ICD. In patients with an intermediate risk (4% to <6%) ICD implantation may also be considered after taking into account age, co-morbid conditions, socioeconomic factors, and the psychological impact of therapy. Survivors of ventricular fibrillation arrest should receive an ICD for secondary prevention unless their life expectancy is less than 1 year. Following device implantation, patients should be followed up for device- and disease-related complications, particularly heart failure and cerebrovascular disease.

scholarly journals Aborted sudden cardiac death due to ventricular fibrillation in a female patient with mitral valve prolapse

2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Sofien Ayed ◽  
Rainer Hoffmann
Keyword(s):  
Sudden Cardiac Death ◽  
Mitral Valve ◽  
Female Patient ◽  
Mitral Valve Prolapse ◽  
Cardiac Death ◽  
Secondary Prophylaxis ◽  
Icd Implantation ◽  
Wide Range ◽  
Patients At Risk ◽  
The 1960S

Abstract Background Mitral valve prolapse is the most frequent valvular defect associated with a wide range of electro-hemodynamic abnormalities, leading to heart failure, arrhythmias and sudden cardiac death. Mitral valve prolapse, first described from Barlow in the 1960s, is defined as displacement of mitral leaflet tissue into the left atrium past the mitral annular plane during systole. The correlation between mitral valve prolapse and sudden cardiac death has been investigated and clarified by various studies in recent years. However, identifying patients at risk and applying measures to prevent those from sudden cardiac death is challenging. Case presentation We report on a 61-year-old female patient who had undergone an aborted sudden cardiac death. An arrythmogenic mitral valve prolapse was diagnosed. In addition, electrocardiographically and morphologically risk markers for sudden cardiac death were found in this case. We performed an ICD implantation as secondary prophylaxis and intended to reconstruct the mitral valve. Conclusion This article examines the association of mitral valve prolapse with sudden cardiac death, the underlying pathophysiological mechanisms and the strategies leading to identify the risk group.

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