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Investigating the Pathogenesis of MYH7 Mutation Gly823Glu in Familial Hypertrophic Cardiomyopathy using a Mouse Model
Published on: August 8, 2022
Current management and surgical advances in patients with hypertrophic obstructive cardiomyopathy
Lucian Dorobantu1, Razvan Ticulescu2, Maria Greavu2
1Cardiomyopathy Center, Monza Hospital, Bucharest, Romania.
Insights
Hypertrophic cardiomyopathy (HCM) treatment has advanced. A modified surgical technique addresses both the septum and mitral valve (MV), simplifying deep septal myectomy and correcting mitral regurgitation effectively.
Area of Science:
- Cardiology
- Genetics
- Surgical Innovation
Background:
- Hypertrophic cardiomyopathy (HCM) is a common genetic heart disease affecting 1:500 individuals.
- Multimodality imaging has improved HCM management, but challenges remain in treating left ventricular outflow tract (LVOT) obstruction, particularly mitral valve involvement.
- Surgical septal myectomy is the gold standard, but requires specialized expertise and may not fully address all obstruction mechanisms.
Purpose of the Study:
- To evaluate the efficacy of a surgical approach combining deep septal myectomy with mitral valve (MV) intervention for hypertrophic obstructive cardiomyopathy (HOCM).
- To assess the safety and outcomes of the Ferrazzi technique in managing HOCM with LVOT obstruction and MV issues.
Main Methods:
- A modified surgical technique, including the Ferrazzi approach to address mitral valve (MV) pathology and careful papillary muscle mobilization, was employed.
- Deep septal myectomy was performed in conjunction with MV repair/preservation.
- Outcomes were assessed in 72 patients undergoing the procedure since November 2015.
Main Results:
- The modified surgical technique resulted in no mortality in 72 patients.
- Complete resolution of the left ventricular outflow tract (LVOT) gradient was achieved in all patients.
- Mitral valve (MV) preservation and adequate correction of mitral regurgitation were accomplished in all cases.
Conclusions:
- Recent surgical advances for hypertrophic obstructive cardiomyopathy (HOCM) effectively address both septal hypertrophy and mitral valve (MV) pathology.
- This integrated approach simplifies deep septal myectomy and adequately corrects mitral regurgitation, offering excellent outcomes.
Abstract:
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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