Hypertrophic cardiomyopathy (HCM) is a genetic disease and the most frequent primary cardiomyopathy, affecting 1:500 of the general population. Integrated multimodality imaging, including transthoracic echocardiography, 2- and 3‑dimensional transesophageal echocardiography, stress echocardiography, and cardiac magnetic resonance, has provided answers to questions on the management of HCM, leading to standardized protocols. The late 1990s brought the news of a nonsurgical treatment of obstruction in HCM. It is now increasingly evident that septal ablation cannot address all the mechanisms of the left ventricular outflow tract (LVOT) gradient, especially mitral valve involvement. According to American and European guidelines, surgical septal myectomy is the current gold standard treatment. However, deep septal myectomy requires specific operator and institutional experience; therefore, it should not be performed in small community hospitals but only in centers of excellence for HCM treatment. The so-called Ferrazzi technique involves cutting the fibrotic secondary chordae of the mitral valve (MV) and thus helps avoid a deep myectomy by moving the anterior mitral leaflet and the coaptation point of the MV posteriorly away from the septum. This technique, together with careful mobilization of the papillary muscles, helped us achieve excellent results since November 2015, with no mortality, resolution of the LVOT gradient, and MV preservation in all 72 patients. Owing to recent advances in the surgical treatment of hypertrophic obstructive cardiomyopathy, addressing not only the septum but also the MV, the procedure of a deep myectomy has been simplified and mitral regurgitation adequately corrected.
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http://dx.doi.org/10.33963/KP.14965 | DOI Listing |
J Anat
January 2025
Hannover Medical School, Institute of Functional and Applied Anatomy, Hannover, Germany.
Obesity, along with hypoxia, is known to be a risk factor for pulmonary hypertension (PH), which can lead to right ventricular hypertrophy and eventually heart failure. Both obesity and PH influence the autonomic nervous system (ANS), potentially aggravating changes in the right ventricle (RV). This study investigates the combined effects of obesity and hypoxia on the autonomic innervation of the RV in a mouse model.
View Article and Find Full Text PDFJACC Case Rep
January 2025
Department of Cardiovascular Disease, Mayo Clinic, Rochester, Minnesota, USA.
Left ventricular outflow tract (LVOT) obstruction is mostly caused by hypertrophic obstructive cardiomyopathy and subaortic stenosis. Rarely, malignancy can lead to dynamic LVOT obstruction and has only been sporadically documented. We present the first case of dynamic and/or nearly fixed LVOT obstruction caused by a cardiac myxoid spindle cell sarcoma.
View Article and Find Full Text PDFJACC Case Rep
January 2025
Department of Cardiovascular Medicine, Richmond Heart & Vascular Associates, Richmond, Virginia, USA.
Transcatheter edge-to-edge repair (TEER) is approved for patients with symptomatic severe mitral regurgitation (MR) who are deemed inoperable or at high surgical risk with life expectancy of more than 1 year, but has also been used off-label in patients with hypertrophic obstructive cardiomyopathy (HOCM) for symptomatic relief who are not candidates for septal reduction therapy. An 83-year-old woman with decompensated heart failure was found to have HOCM with systolic anterior motion of the mitral valve and a large P2 flail segment with ruptured cords. TEER was performed resulting in mild MR and resolution of the prior left ventricular outflow tract gradient.
View Article and Find Full Text PDFReports (MDPI)
December 2024
Division of Cardiology, Department of Medicine, University of Washington, Seattle, WA 98195, USA.
Unlabelled: The combination of hypertrophic cardiomyopathy with outflow tract obstruction, severe pre-capillary and post-capillary pulmonary hypertension, and severe primary mitral regurgitation is rare and presents distinct management challenges.
Background And Clinical Significance: Pulmonary hypertension is an independent predictor of all-cause mortality in patients with hypertrophic cardiomyopathy managed medically and often precludes patients from undergoing cardiopulmonary bypass due to increased surgical morbidity and mortality. In studies specifically evaluating surgical myectomy, however, survival is favorable in patients with moderate-to-severe pulmonary hypertension.
Background: Disopyramide is used to treat heart failure symptoms in patients with obstructive hypertrophic cardiomyopathy (HCM) with known medium-term efficacy and safety, while long-term outcomes are unknown.
Methods And Results: A total of 92 consecutive patients with symptomatic obstructive HCM with peak left ventricular outflow tract gradients of ≥30 mm Hg at rest or with provocation who were maintained on disopyramide for ≥5 years at 2 dedicated HCM centers were included: 92 patients; mean age, 62.5 years; 54% women; treated with disopyramide for median 7.
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