Hypertrophic cardiomyopathy is a genetic disorder characterized by marked hypertrophy of the myocardium. It is frequently accompanied by dynamic left ventricular outflow tract obstruction and symptoms of dyspnea, angina, and syncope. The initial therapy for symptomatic patients with obstruction is medical therapy with β-blockers and calcium antagonists. However, there remain a subset of patients who have continued severe symptoms, which are unresponsive to medical therapy. These patients can be treated with septal reduction therapy, either surgical septal myectomy or alcohol septal ablation. When performed by experienced operators working in high-volume centers, septal myectomy is highly effective with a >90% relief of obstruction and improvement in symptoms. The perioperative mortality rate for isolated septal myectomy in most centers is

Hypertrophic Obstructive Cardiomyopathy: Surgical Myectomy and Septal Ablation

Hypertrophic cardiomyopathy (HCM) is the most common genetic cardiovascular disease, which goes along with increased risk for sudden cardiac death (SCD). Despite the knowledge about the different causal genes, the relationship between individual genotypes and phenotypes is incomplete. We retrieved PubMed/Medline literatures on genotype–phenotype associations in patients with HCM and mutations in MYBPC3, MYH7, TNNT2, and TNNI3. Altogether, 51 studies with 7675 HCM patients were included in our meta-analysis. The average frequency of mutations in MYBPC3 (20%) and MYH7 (14%) was higher than TNNT2 and TNNI3 (2% each). The mean age of HCM onset for MYH7 mutation positive patients was the beginning of the fourth decade, significantly earlier than patients without sarcomeric mutations. A high male proportion was observed in TNNT2 (69%), MYBPC3 (62%) and mutation negative group (64%). Cardiac conduction disease, ventricular arrhythmia and heart transplantation (HTx) rate were higher in HCM patients with MYH7 mutations in comparison to MYBPC3 (p < 0.05). Furthermore, SCD was significantly higher in patients with sarcomeric mutations (p < 0.01). A pooled dataset and a comprehensive genotype–phenotype analysis show that the age at disease onset of HCM patients with MYH7 is earlier and leads to a more severe phenotype than in patient without such mutations. Furthermore, patients with sarcomeric mutations are more susceptible to SCD. The present study further supports the clinical interpretation of sarcomeric mutations in HCM patients.

Clinical outcomes associated with sarcomere mutations in hypertrophic cardiomyopathy: a meta-analysis on 7675 individuals

Transcatheter alcohol septal ablation (ASA) treatment of symptomatic patients with hypertrophic obstructive cardiomyopathy (HOCM) is based on the existence and degree of intraventricular obstruction. Patients with significant gradient and symptoms who do not respond to optimal medical therapy are eligible to gradient reduction through a surgical (septal myectomy) or a transcatheter (alcohol septal ablation) septal reduction. The latter encompasses occlusion of a septal branch perfusing the hypertrophied septum, which is involved in the generation of obstruction, by injecting ethanol into the supplying septal branch(es). ASA has been established as a highly effective and safe method and has outnumbered the surgical gold standard. Although the technique is straightforward, patient selection and some technical details may influence the efficacy and safety of the procedure. The technique is based on echocardiographic contrast guidance, which allows accurate target septal branch selection and optimisation of the result. Published long-term results from high-volume centres have confirmed the effectiveness of ASA and have shown excellent survival, which is comparable to that in the general population. Choice and performance of the surgical or interventional treatment should be implemented in highly specialised centres in terms of a heart-team approach, taking notice of anatomic characteristics as well as comorbidities. Involvement of all cases in international registries may reveal the individual merits and indications for the surgical and interventional treatment in HOCM.

Transcatheter septal ablation in hypertrophic obstructive cardiomyopathy: a technical guide and review of published results.

The study sought to assess the prognostic impact of chronic kidney disease (CKD) and renal replacement therapy (RRT) in patients with ventricular tachyarrhythmias and sudden cardiac arrest (SCA) on admission. A large retrospective registry was used including all consecutive patients presenting with ventricular tachycardia (VT), fibrillation (VF) and SCA on admission from 2002 to 2016. Non-CKD vs. “CKD without RRT”, and “CKD without RRT” vs. “CKD with RRT” were compared applying multivariable Cox regression models and propensity-score matching for evaluation of the primary prognostic endpoint defined as long-term all-cause mortality at 2 years. Secondary prognostic endpoints were cardiac death at 24 h, in-hospital death at index and the composite endpoint of recurrent ventricular tachyarrhythmias, appropriate ICD therapies and cardiac death at 24 h. In 2686 unmatched high-risk patients with ventricular tachyarrhythmias and SCA, non-CKD was present in 46%, “CKD without RRT” in 46% and “CKD with RRT” in 8%. Each, VT and VF occurred in about one-third of CKD patients. Multivariable Cox regression models revealed that “CKD without RRT” (HR = 2.118; p = 0.001) and “CKD with RRT” (HR = 3.043; p = 0.001) patients were associated with the primary endpoint of long-term mortality at 2 years, which was also proven after propensity-score matching (non-CKD vs. “CKD without RRT”: 43% vs. 27%, log rank p = 0.001; HR = 1.847; “CKD without RRT” vs. “CKD with RRT”: 74% vs. 51%, log rank p = 0.001; HR = 2.129). The rates of secondary endpoints were higher for cardiac death at 24 h, in-hospital death at index and the composite of recurrent ventricular tachyarrhythmias, appropriate ICD therapies and  cardiac death at 24 h, respectively, for “CKD without RRT” and “CKD with RRT” patients.  In patients presenting with ventricular tachyarrhythmias and aborted SCA on admission, the presence of CKD, especially combined with RRT, is independently associated with an increase of long-term all-cause mortality at 2 years, cardiac death at 24 h, in-hospital death and the composite of recurrent ventricular tachyarrhythmias, appropriate ICD therapies and  cardiac death at 24 h.

Prognostic impact of chronic kidney disease and renal replacement therapy in ventricular tachyarrhythmias and aborted cardiac arrest.

Background and objective: Treatment for patients with hypertrophic obstructive cardiomyopathy (HOCM) can be either conservative or invasive (alcohol septal ablation (ASA) and myectomy). As there is...

Long-term outcome of conservative and invasive treatment in patients with hypertrophic obstructive cardiomyopathy

Percutaneous alcohol septal ablation (PTSMA) is an established treatment for symptomatic patients with hypertrophic obstructive cardiomyopathy (HOCM). However, there is concern of a higher risk for ventricular tachyarrhythmias and sudden death due to the myocardial scar created after PTSMA. We investigated the possibility of increased ventricular arrhythmias and risk of sudden death after PTSMA in a subgroup of patients with an already implanted ICD. Between 2009 and 2012, 239 PTSMAs were performed in 212 patients with HOCM. In 32 of those an ICD had already been implanted before PTSMA for primary (31 patients) or secondary (1 patient) prevention of sudden death. The maximum left ventricular outflow tract gradient (LVOTG) was reduced from 114 ± 39 mmHg before PTSMA to 23 ± 19 mmHg (P  14.3 %). In a selected high-risk patient cohort with HOCM ominous arrhythmic events seem to be rare and predominantly occur in patients with a very high estimated risk of sudden death before PTSMA.

Low occurrence of ventricular arrhythmias after alcohol septal ablation in high-risk patients with hypertrophic obstructive cardiomyopathy

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Low occurrence of ventricular arrhythmias after alcohol septal ablation in high-risk patients with hypertrophic obstructive cardiomyopathy