The mitral valve (MV) plays an important role in the pathophysiology of hypertrophic obstructive cardiomyopathy (HOCM). Dynamic left ventricular outflow tract (LVOT) obstruction, caused by systolic anterior motion (SAM), is a common occurrence in most patients with hypertrophic cardiomyopathy and is directly associated with the MV apparatus. First line therapy for HOCM patients is pharmacological, and surgical intervention is often indicated for patients who do not respond to medical therapy. Emerging research on mitral disease in HOCM, specifically mitral regurgitation (MR), demonstrates that these patients frequently do not respond to standard therapeutic options, and can benefit from MV interventions. In this review, we describe the involvement of the MV in the pathogenesis of HOCM, discuss medical therapy, and explore available mitral procedures. Surgical myectomy, often combined with various modifications to the MV apparatus, is frequently necessary to achieve a durable resolution of LVOT obstruction and SAM-related MR. Alcohol septal ablation, an alternative to surgical myectomy, will be briefly mentioned. We also emphasize the role of transcatheter edge-to-edge repair (TEER) as a promising and novel therapeutic option for HOCM patients. Over time, TEER has established itself as an effective and safe procedure, demonstrating success across a spectrum of anatomical variations. The leaflet modification and movement restriction achieved through TEER help reduce SAM and, consequently, have the potential to alleviate LVOT obstruction and SAM-related MR. Furthermore, we propose a treatment algorithm for cases where TEER is a potential course of action for patients who are at high risk for other interventions.
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http://dx.doi.org/10.1016/j.cjca.2023.12.009 | DOI Listing |
Eur Heart J Case Rep
January 2025
Department of Pediatric Cardiology, Osaka Women's and Children's Hospital, Izumi, Osaka 594-1101, Japan.
Background: A partial atrioventricular septal defect (AVSD) with a hypoplastic left ventricle and common atrium is a rare combination of cardiac anomalies that can be associated with Ellis-van Creveld (EVC) syndrome.
Case Summary: A female neonate with EVC syndrome was diagnosed with an unbalanced AVSD and hypoplastic left ventricle. Pulmonary artery banding and ductus ligation were performed at 23 days after birth.
Catheter Cardiovasc Interv
January 2025
Department of Cardiac Anesthesia, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
Acute coronary occlusion during transcatheter aortic valve implantation (TAVI) is an unwarranted complication associated with high mortality. The current TAVI practices recommend a multidetector computed tomography (MDCT) evaluation of the aortic valve, the left ventricular outflow tract, and the aortic root to determine the conventional risk factors for coronary obstruction like low-lying coronary ostia and narrow sinuses of Valsalva, mandating prophylactic coronary protection or native valve leaflet modification in high-risk patients. Despite optimal anatomy, acute coronary occlusion can still occur due to multiple mechanisms, one of which is coronary embolism due to thrombus, calcium, or native aortic valve fragments.
View Article and Find Full Text PDFAm J Cardiol
January 2025
Department of Cardiovascular Medicine, Baystate Medical Center and Division of Cardiovascular Medicine, University of Massachusetts-Baystate, Springfield, Massachusetts, USA. Electronic address: https://twitter.com/AGoldsweig.
Introduction: Obstructive hypertrophic cardiomyopathy (oHCM) is a genetic disorder characterized by myocardial hypertrophy, which can obstruct left ventricular outflow. Cardiac myosin inhibitors (CMIs) have emerged as a novel therapeutic agent targeting cardiac muscle hypercontractility.
Objective: To compare the efficacy and safety of CMIs mavacamten and aficamten vs.
Multimed Man Cardiothorac Surg
January 2025
Department of Cardiothoracic Surgery, Faculty of Medicine, Mansoura University Mansoura, Egypt.
The Ross procedure continues to be the best procedure to address unrepairable aortic valve pathology, especially in young adults. The Achilles heel of this procedure has been aortic root dilation and the potential need for a reoperation that may be associated with slightly increased risks in addition to the need for intervention on the pulmonary outflow tract. Modifying the Ross procedure by autograft inclusion inside a Dacron graft seems to have the potential advantage of stabilizing the autograft diameter, which may be associated with improved durability and decrease the need for future intervention.
View Article and Find Full Text PDFHeart
January 2025
Yonsei University College of Medicine, Seoul, The Republic of Korea
Background: High-intensity physical activity has traditionally been discouraged in patients with hypertrophic cardiomyopathy due to concerns about triggering sudden cardiac death. However, current guidelines adopt a more liberal stance, and evidence on risk factors for exercise-related sudden cardiac death remains limited. This study investigated the clinical, morphological and genetic factors associated with high-intensity physical activity-related sudden cardiac death in hypertrophic cardiomyopathy.
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