Current management and surgical advances in patients with hypertrophic obstructive cardiomyopathy

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. KEY WORDS hypertrophic cardiomyopathy, secondary chordae of the mitral valve, septal myectomy 11 Tendera M, Wycisk A, Schneeweiss A, et al. Effect of sotalol on arrhythmias and exercise tolerance in patients with hypertrophic cardiomyopathy. Cardiology. 1993; 82: 335-342. 12 Maron MS, Spirito P, Maron BJ. Case for earlier surgical myectomy in patients with obstructive hypertrophic cardiomyopathy. Circulation. 2018; 138: 2076-2078. 13 Maron BJ, Rowin EJ, Casey SA, Maron MS. How hypertrophic cardiomyopathy became a contemporary treatable genetic disease with low mortality: shaped by 50 years of clinical research and practice. JAMA Cardiol. 2016; 1: 98-105. 14 Vriesendrop PA, Schinkel AF, Soliman OI, Kofflard MJ, de Jong PL, van Herwerden LA, et al. A contemporary European experience with surgical septal myectomy and anterior mitral vave extension in obstructive cardiomyopathy. Eur Heart J. 2012; 33: 2080-2087. 15 Kim LK, Swaminathan RV, Looser P, et al. Hospital volume outcomes after septal myectomy and alcohol septal ablation for treatment of obstructive hypertrophic cardiomyopathy: US Nationwide Inpatient Database, 2003-2011. JAMA Cardiol. 2016; 1: 324-332. 16 Desai MY, Smedira NG, Bhonsale A, et al. Symptom assessment and exercise impairment in surgical decision making in hypertrophic obstructive cardiomyopathy: relations...

Mini-invasive surgical instruments in transaortic myectomy for hypertrophic obstructive cardiomyopathy: a single-center experience with 168 cases

“…The high technical difficulty, however, limited clinical application of the extended septal myectomy procedure. Later, Ferrazzi et al in 2015 proposed the transaortic secondary chordae cutting in addition to a shallow septal myectomy for managing HOCM [8]; notably, it was associated with favorable results [8,19]. However, such an approach remained controversial because the secondary chordae maintained ventricular geometry and enhanced wall thickening, which may be helpful in case of left ventricular dilation and systolic dysfunction [20].…”

Mini-invasive surgical instruments in transaortic myectomy for hypertrophic obstructive cardiomyopathy: A single-center experience with 168 cases

“…The high technical di culty, however, limited clinical application of the extended septal myectomy procedure. Later, Ferrazzi et al in 2015 proposed the transaortic secondary chordae cutting in addition to a shallow septal myectomy for managing HOCM [8] ; notably, it was associated with favorable results [8,19] . However, such an approach remained controversial because the secondary chordae maintained ventricular geometry and enhanced wall thickening, which may be helpful in case of left ventricular dilation and systolic dysfunction [20] .…”

Application of mini-invasive surgical instruments in transaortic myectomy for hypertrophic obstructive cardiomyopathy: A single-center experience with 168 cases

“…In 2015, Ferrazzi et al proposed the transaortic secondary chordae cutting in addition to a shallow septal myectomy for the treatment of HOCM [8] . It was reported to be associated with favorable results [8,19] . However, such an approach remained controversial because the secondary chordae maintains ventricular geometry and enhances wall thickening, which may be helpful in the presence of left ventricular dilation and systolic dysfunction [20] .…”