Improving Outcomes in Hypertrophic Cardiomyopathy

Improving outcomes in hypertrophic cardiomyopathy (HCM) is focused on the improvement of the therapeutic strategies for patients with HCM. First it demonstrates that individual patient selection in patients with obstructive and symptomatic HCM can lead to near normal life-expectancy; but multidisciplinary decision-making is strongly advocated. Second, further improvement and validation of current models of identification of high risk patients leads to more appropriate ICD therapy. Finally, the improvement of our understanding of genotype-phenotype relations could lead to possible therapeutic strategies in the future

FIRST RESULTS OF MICROSPHERE EMBOLIZATION AS AN ALTERNATIVE FOR ALCOHOL IN PERCUTANEOUS TRANSLUMINAL SEPTAL MYOCARDIAL ABLATION

Farrando Sicilia, Jordi; Fuente Fuente, Carlo

Microsphere embolisation as an alternative for alcohol in percutaneous transluminal septal myocardial ablation

Background Percutaneous transluminal septal myocardial ablation using microsphere embolisation is a new interventional technique to treat patients with hypertrophic obstructive cardiomyopathy. Methods and results In two patients, considered at high risk for myectomy, targeted septal perforators were occluded with microsphere embolisation instead of alcohol ablation to reduce left ventricular outflow gradient. In both cases the left ventricular outflow tract gradient was immediately reduced. No adverse events occurred. Conclusion This is the first clinical experience with Embozene® Microspheres in the Netherlands as an alternative for alcohol septal ablation. In both cases it resulted in immediate improvement in the haemodynamics, without any adverse events

Effectiveness of the 2014 European Society of Cardiology guideline on sudden cardiac death in hypertrophic cardiomyopathy: a systematic review and meta-analysis.

Objective In 2014, the European Society of Cardiology (ESC) recommended the use of a novel risk prediction model (HCM Risk-SCD) to guide use of implantable cardioverter defibrillators (ICD) for the primary prevention of sudden cardiac death (SCD) in patients with hypertrophic cardiomyopathy (HCM). We sought to determine the performance of HCM Risk-SCD by conducting a systematic review and meta-analysis of articles reporting on the prevalence of SCD within 5 years of evaluation in low, intermediate and high-risk patients as defined by the 2014 guidelines (predicted risk <4%, 4%–<6% and ≥6%, respectively). Methods The protocol was registered with PROSPERO (registration number: CRD42017064203). MEDLINE and manual searches for papers published from October 2014 to December 2017 were performed. Longitudinal, observational cohorts of unselected adult patients, without history of cardiac arrest were considered. The original HCM Risk-SCD development study was included a priori. Data were pooled using a random effects model. Results Six (0.9%) out of 653 independent publications identified by the initial search were included. The calculated 5-year risk of SCD was reported in 7291 individuals (70% low, 15% intermediate; 15% high risk) with 184 (2.5%) SCD endpoints within 5 years of baseline evaluation. Most SCD endpoints (68%) occurred in patients with an estimated 5-year risk of ≥4% who formed 30% of the total study cohort. Using the random effects method, the pooled prevalence of SCD endpoints was 1.01% (95% CI 0.52 to 1.61) in low-risk patients, 2.43% (95% CI 1.23 to 3.92) in intermediate and 8.4% (95% CI 6.68 to 10.25) in high-risk patients. Conclusions This meta-analysis demonstrates that HCM Risk-SCD provides accurate risk estimations that can be used to guide ICD therapy in accordance with the 2014 ESC guidelines. Registration number PROSPERO CRD42017064203;Pre-results.pre-print379 K

The Clinical Implications of Body Surface Area as a Poor Proxy for Cardiac Output

Background: Prosthesis-patient mismatch (PPM), routinely used to characterize the degree of hemodynamic obstruction caused by a prosthetic heart valve, is associated with adverse patient outcomes after aortic valve replacement (AVR). In the common definition of PPM, the opening area of the valve is related to the patients’ cardiac output, by indexing effective orifice area (EOA) with body surface area (BSA). The aim of this study is to assess the implications of using BSA as a proxy for cardiac output. Methods: 744 patients with normal LV function underwent echocardiographic assessment after surgical AVR. To validate the use of BSA as a proxy for cardiac output, the relation between these variables was analyzed. The effects of BSA on the classification of PPM (EOAi < 0.85 cm2/m2) and the presence of hemodynamic obstruction (mean gradient ≥ 20 mmHg and/or Doppler velocity index < 0.35) were estimated. Results: There was a weak correlation between BSA and cardiac output (r: 0.29, 95% CI: 0.22;0.35), and cardiac output was not proportional to BSA (Cardiac output = 1.5 x BSA +1.9). As a result, the increased risk of patients with a large BSA to be labelled with PPM (OR: 5.2, 95% CI: 2.5,11 per m2 BSA), was not reflected by a significantly higher risk of hemodynamic obstruction (OR: 1.5, 95% CI: 0.5,4.9 per m2 BSA). Conclusions: The current definition of PPM results in a systematic overestimation of hemodynamic obstruction in patients with a larger BSA, and we recommend cautious use in this subgroup. Abbreviations: AVR: Aortic valve replacement; BMI: Body mass index; BSA: Body surface area; EOA: Effective orifice area; EOAi: Indexed effective orifice area; LVOT: Left ventricular outflow tract; PERIGON: PERIcardial SurGical AOrtic Valve ReplacemeNt Pivotal Trial; PPM: Prosthesis-patient mismatch; TTE: Transthoracic echocardiography; VARC-2: Valve Academic Research Consortium-2