Overview

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Explore the latest developments in hypertrophic cardiomyopathy (HCM) through this comprehensive cardiology grand rounds lecture. Delve into multimodality imaging techniques for accurate HCM diagnosis, including recognition of apical aneurysms and LV strain analysis. Learn about the NIH-sponsored HCMR Registry and the application of machine learning and AI in HCM research. Understand the importance of eliciting left ventricular outflow tract (LVOT) obstruction and recognizing typical and atypical variants of obstructive HCM. Examine sudden cardiac death risk stratification, comparing ESC and American approaches to ICD placement. Discover management strategies for obstructive HCM, including lifestyle considerations and the LIVE-HCM Trial on vigorous exercise. Explore septal reduction therapies, including surgical approaches and alcohol septal ablation. Gain insights into new pharmacological treatments, such as Mavacamten, and their impact on disease phenotype and cardiac remodeling. Discuss gene therapy prospects and the management of arrhythmias in HCM patients. Finally, learn about genetic testing, long-term outcomes from the SHARE registry, and screening protocols for asymptomatic first-degree relatives.

Syllabus

Current and Evolving Understanding in Hypertrophic Cardiomyopa
Hypertrophic Cardiomyopathy: Some simple math
Step 1: Multimodality imaging key in diagnosing HCM
Recognition of Apical aneurysms
LV Strain
NIH Sponsored HCMR Registry
Machine learning. LV shape and insights into pathophysiology
AI, EKG, HCM
Machine learning and HCM
Elicit LVOT Obstruction: Don't look, Won't find, Can't Tre
Stress Echo
Recognize typical and atypical variants of obstructive HC OCM without the H: Abnormal mitral subvalvular morphology
3 different flavors of "Atypical" LVOT obstruction
Differential diagnosis using multimodality imaging
Important to make the correct diagnosis
SCD risk stratification
Comparison of ESC vs. American Approach
ICD placement
HCM LGE Outcomes
Screening Echo vs. CMR in G+P-patients
Management in oHCM
Lifestyle considerations
Vigorous exercise and HCM: LIVE-HCM Trial
In oHCM, there are no prospective RCT (single or multicenter) compar a various FDA-approved medical therapies b invasive therapies vs. FDA-approved medical Rx c myectomy vs. alcohol ablation
Septal Reduction Therapy: One operation does not fit
Cleveland Clinic approach to Surgical LVOT obstructi
Alcohol septal ablation
Guidelines: Invasive therapies
Spectrum of oHCM management in USA
Expert Centers Have Markedly Better SRT Outcomes But Access to Specialty Centers Are Limited
Mavacamten Prevents Disease Phenotype in HCM Mice
Phase III RCTs: Testing Mavacamten vs. Placebo EXPLORER-HCM vs. VALOR-HCM
Mavacamten LTE study
Valor-HCM: 92% NYHA Class III 19 US HCM Centers
Primary endpoint and NYHA Class Improvement
Secondary Efficacy Endpoints
Favorable Cardiac Remodeling
A VALOR Mavacamten Story
LVEF and mavacamten: Implications in clinical practice
Clinical management of Camzyos (Mavacamten)
Gene-therapy: Adeno-associated Virus mediated
These new therapies are going to bring new logistic scenarios, assum long-term safety and efficacy
Symptomatic Nonobstructive HCM
Managing Arrhythmias Atrial fibrillation: A Bad Actor in HCM, especially oHCM
Management of AF
Genetic Testing in HCM
Genetics and long-term outcomes: SHARE registry!
Screening of asymptomatic first-degree relativ EKG and echo
Valsartan in Early Stage HCM: VANISH Trial