Hypertrophic obstructive cardiomyopathy (HOCM) is the most frequently inherited cardiovascular disease (prevalence in the general population of 1/500) and is characterized by significant left ventricular hypertrophy, especially in the interventricular septum, combined with small-volume cardiac cavities. Transaortic surgical septal myectomy is the most commonly used technique to treat HOCM, and is associated with low operative morbidity and mortality and a reduction of the outflow gradients. The composite operative mortality of only 0.4% (17/3695 patients) from 5 major high-volume centers in North America highlights the role of dedicated HOCM units. The involvement of the mitral valve in the pathophysiology of HOCM has been addressed as systolic anterior motion (SAM)-related left ventricular outflow tract obstruction. Hypertrophic cardiomyopathy mitral malformations include leaflet elongation and a wide array of malformations of the papillary muscles and chordae that can be detected by transthoracic and transesophageal echocardiography and by cardiac magnetic resonance. Because they participate fundamentally in the predisposition to SAM, they have increasingly been repaired surgically.
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http://dx.doi.org/10.1714/3093.30858 | DOI Listing |
Laryngoscope
January 2025
Department of Otorhinolaryngology, Ankara Yıldırım Beyazıt University School of Medicine, Ankara, Turkey.
Objectives: The study aims to investigate the relationship between the presence of laryngopharyngeal reflux (LPR) and obstruction levels identified during drug-induced sleep endoscopy (DISE) in obstructive sleep apnea (OSA) among nonobese patients.
Methods: We conducted a prospective study of 105 adult patients diagnosed with OSA who underwent DISE using propofol sedation from 2019 to 2024 at a tertiary hospital. To control for the confounding impact of obesity on LPR, the study selectively enrolled individuals presenting a body mass index within the normal range.
CPT Pharmacometrics Syst Pharmacol
January 2025
Huashan Hospital, Fudan University, Shanghai, China.
Mavacamten is a cardiac myosin inhibitor for adults with obstructive hypertrophic cardiomyopathy (HCM). Dose optimization is performed 4 weeks after starting mavacamten, guided by periodic echo measurements of Valsalva left ventricular outflow tract gradient (VLVOTg) and left ventricular ejection fraction (LVEF). Previously, a population pharmacokinetic (PPK) model was developed and exposure-response (E-R) of VLVOTg (efficacy) and LVEF (safety) was used to identify the mavacamten titration regimen with the optimal benefit/risk ratio, now included in the US prescribing information.
View Article and Find Full Text PDFPediatr Pulmonol
January 2025
Hôpital Femme Mère Enfant, Hospices Civils de Lyon, 59 Boulevard Pinel, Lyon, France.
Background: New CFTR Modulator triple therapy Elexacaftor-Ivacaftor-Tezacaftor (ETI) prove efficacy in pulmonary outcomes. However, its impact on nasal sinus symptoms in children has not been specifically studied. The aim of this study is to evaluate the impact of this therapy on nasal sinus symptomatology in children aged 6-12 years.
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