In this episode of In the Thick of It, Dr. John Fritzlen joins leading electrophysiologist Dr. Mark Link and Hannah and Bethany from Heart Charged to discuss the critical role of implantable cardioverter-defibrillators (ICDs) in managing hypertrophic cardiomyopathy. The group navigates the complex clinical landscape of ICD indications, including the importance of MRI findings and specific risk factors like non-sustained ventricular tachycardia (NSVT). Dr. Link provides an expert comparison of transvenous, subcutaneous (S-ICD), and the latest extravascular (EV-ICD) technologies, focusing on device durability and suitability for active patients. Throughout the conversation, the "Bionic Babes" of Heart Charged offer invaluable patient perspectives on life with a defibrillator, the necessity of shared decision-making, and the evolving guidelines that now encourage vigorous exercise for those living with the condition.

In this episode of In the Thick of It, Dr. Floridian Rader and Dr. Ahmad Masri discuss the transformative results of the MAPLE trial, a head-to-head study comparing aficamten—a cardiac myosin inhibitor—to metoprolol in patients with symptomatic obstructive hypertrophic cardiomyopathy1. The trial utilized a rigorous "double-dummy" design to ensure blinding while testing both medications at therapeutic doses2. Dr. Masri highlights unexpected findings, including the significant reduction in peak $VO_2$ and the worsening of cardiac biomarkers like NT-proBNP in the metoprolol group3. The conversation explores how these results challenge decades of reliance on beta-blockers as a first-line therapy and demonstrate the efficacy of aficamten as a potent upfront monotherapy for improving patient function and cardiac remodeling4.

In this episode of In the Thick of It, Dr. Madeline Smoot talks with Dr. Alex McNamara to discuss the complexities of managing refractory symptoms in patients with hypertrophic cardiomyopathy (HCM). The conversation highlights the distinction between obstructive and non-obstructive symptoms, emphasizing the importance of provocative testing—such as cardiopulmonary exercise testing (CPET) and gold-directed Valsalva maneuvers—to unmask "labile" obstruction that resting echoes might miss. They explore the evolving role of cardiac myosin inhibitors and the potential impact of GLP-1 agonists on obesity-related symptom burden. Additionally, Dr. McNamara provides insights into identifying red flags beyond obstruction, including primary mitral valve disease and the early stages of restrictive or "burnt-out" HCM.

In this episode of In the Thick of It, genetic counselor Matthew Thomas sits down with Dr. Roddy Walsh and Dr. Juan Pablo Ochoa to discuss their groundbreaking research on the genetic architecture of Hypertrophic Cardiomyopathy (HCM). The conversation centers on "Intermediate Effect Variants" (IEVs)—genetic markers that fall between rare, high-impact Mendelian mutations and common, low-impact polygenic risk factors. By utilizing a massive dataset of over 20,000 patients and robust ancestry-matched controls, the researchers demonstrate how these variants contribute to disease severity, age of onset, and wall thickness. This episode explores the practical implications for clinical genetic testing, the necessity of diverse genomic data, and how these findings are shifting the field toward a more quantitative and comprehensive understanding of HCM risk.

References: https://www.ahajournals.org/doi/10.1161/CIRCULATIONAHA.125.074529

In this episode of In the Thick of It, Dr. Betty Raman interviews Dr. Srihari Naidu, Director of the Hypertrophic Cardiomyopathy Medical Center at Westchester Medical Center, to explore what truly defines a "Center of Excellence". Dr. Naidu shares how his philosophy has evolved from a structural focus on advanced imaging and surgical tools to a patient-centered approach that prioritizes individualized care and a multidisciplinary "heart team". The discussion highlights the critical role of specialized centers in navigating complex differential diagnoses—such as amyloidosis or mitochondrial HCM—and the importance of shared decision-making in sudden cardiac death risk stratification. Dr. Naidu also provides a practical roadmap for clinicians looking to establish regional HCM services, emphasizing the necessity of robust echo protocols and strong collaborative networks for advanced procedures.

In this episode of In the Thick of It, hosts Dr. Betty Raman and Dr. James MacNamara welcome Professor Martin Maron, Director of the Hypertrophic Cardiomyopathy Center at Lahey Hospital & Medical Center, to discuss the innovative FORTITUDE-HCM study. This Phase 2b trial focuses on the symptomatic non-obstructive HCM population—a group considered to have one of the greatest unmet treatment needs in the field. The conversation explores the unique mechanism of ninerafaxstat, a metabolic modulator that shifts energy utilization from fatty acids to glucose to improve cardiac energetics and diastolic relaxation. Professor Maron highlights the promising results from the earlier IMPROVE-HCM trial, details the 12-week design of the current study, and speculates on a future where "combination therapy" becomes the standard of care for complex HCM cases.

In this episode of In the Thick of It, Dr. James MacNamara sits down with Dr. Sharlene Day, Director of Translational Research at the University of Pennsylvania, to discuss the groundbreaking SONATA trial. This double-blind, placebo-controlled clinical trial is evaluating the safety and efficacy of sotagliflozin—a dual SGLT1 and SGLT2 inhibitor—in patients with both obstructive and non-obstructive hypertrophic cardiomyopathy. Dr. Day details the trial's unique design, its primary endpoint of improving the Kansas City Cardiomyopathy Questionnaire (KCCQ) score, and the fascinating metabolic mechanisms that may allow these drugs to reduce the heart's workload. The conversation also covers the safety protocols for monitoring outflow tract obstruction and the potential pleiotropic benefits of SGLT2 inhibitors beyond traditional heart failure management.

Dr. James MacNamara and Dr. Betty Raman recap the latest annual Hypertrophic Cardiomyopathy Society (HCMS) meeting held in New Orleans. The discussion emphasizes that HCM care is a "team sport," involving a multidisciplinary coalition of physicians, pharmacists, and advanced practice providers. Key highlights include a deep dive into Professor Hugh Watkins' lecture on the future of gene therapy and the impact of polygenic risk scores on disease penetrance. They also explore the evolving landscape of HCM treatment—comparing the roles of cardiac myosin inhibitors versus surgical interventions—and share promising new data on the safety and efficacy of moderate-intensity exercise for non-obstructive patients.

How can echocardiographers improve the accuracy and confidence of hypertrophic cardiomyopathy diagnosis? In this episode of In the Thick of It, sonographers Amy Dillenbeck and Brad Mehl explore the essential role of echocardiography in evaluating HCM, sharing best practices for optimizing imaging techniques, Doppler assessment, and contrast use. They discuss the most common challenges in detecting HCM, how to train sonographers to recognize subtle diagnostic clues, and strategies to avoid misinterpretation. Whether you're a seasoned clinician or new to HCM imaging, this episode offers valuable insights into refining echocardiographic evaluation for better patient outcomes.

Season 2 of In the Thick of It is brought to you by the HCM Society, is supported by sponsorship from Cytokinetics, and is produced by Earfluence.

How do we determine which hypertrophic cardiomyopathy patients are at the highest risk for sudden cardiac death? In this episode of In the Thick of It, Dr. Steve Ommen and Dr. Mark Link take a deep dive into the role of non-sustained ventricular tachycardia (NSVT) in risk assessment and ICD decision-making. They discuss how age, VT duration, and other clinical factors influence risk stratification, as well as the evolving guidelines on ICD placement. The conversation also covers the latest advancements in device technology and the nuances of selecting the right ICD for each patient. Don’t miss this essential discussion on refining risk assessment and improving outcomes in HCM care.

Season 2 of In the Thick of It is brought to you by the HCM Society, is supported by sponsorship from Cytokinetics, and is produced by Earfluence.