Treatment Of Hypertrophic Obstructive Cardiomyopathy Research Articles

Man With Progressive Exertional Dyspnea.

A 65-year-old man with a history of hypertension and left ventricular hypertrophy presented with progressive exertional dyspnea for one month. Physical examination showed a blood pressure of 155/82 mmHg, a pulse of 102 beats/min, mild icteric sclera, and a high-pitched midsystolic murmur at the left lower sternal border. Laboratory tests reported a normal hemoglobin level. Electrocardiography was typical for left ventricular hypertrophy, and chest radiography showed mild cardiomegaly without active lung infiltrate (Figure 1). Ultrasonography with the parasternal long-axis view of the heart revealed increased thickness of the interventricular septum and an obvious aortic valve fluttering when the leaflets were open (Figure 2 and Video E1). The aortic valve fluttering during the systolic phase was confirmed by the M-mode echocardiogram (Figure 3).Figure 2Ultrasonography with the parasternal long-axis view of the heart revealed increased thickness of the interventricular septum (asterisk) and an obvious aortic valve fluttering when the leaflets were open (arrow).View Large Image Figure ViewerDownload Hi-res image Download (PPT)Figure 3The aortic valve fluttering during the systolic phase (arrows) was shown on the M-mode tracing at the aortic valve in the parasternal long-axis view of the heart.View Large Image Figure ViewerDownload Hi-res image Download (PPT) Hypertrophic obstructive cardiomyopathy. After consultation with cardiovascular specialists, the patient received an elective left ventricular myomectomy for hypertrophic obstructive cardiomyopathy. The fluttering of the cusps of the aortic valve is mostly reported during diastole, and it is associated with flail cusps with significant aortic regurgitation.1Qamar S. Ammar K.A. Paterick T.E. et al.Diastolic fluttering of the aortic valve: pathognomonic M-mode features of flail cusp.Eur Heart J Cardiovasc Imaging. 2013; 14: 204Crossref PubMed Scopus (1) Google Scholar Systolic fluttering of the aortic valve has been reported, which was explained by turbulent systolic flow downstream of an aortic valve.2Becsek B. Pietrasanta L. Obrist D. Turbulent systolic flow downstream of a bioprosthetic aortic valve: velocity spectra, wall shear stresses, and turbulent dissipation rates.Front Physiol. 2020; 11577188Crossref PubMed Scopus (12) Google Scholar Before the widespread use of Doppler echocardiogram, systolic fluttering was used to diagnose a patient with severe aortic stenosis.3Chin M.L. Bernstein R.F. Child J.S. et al.Aortic valve systolic flutter as a screening test for severe aortic stenosis.Am J Cardiol. 1983; 51: 981-985Abstract Full Text PDF PubMed Scopus (8) Google Scholar Systolic fluttering of the aortic valve indicates a large pressure gradient during the systole, which is pathognomonic for hypertrophic obstructive cardiomyopathy.4Pollick C. Shmueli H. Maalouf N. et al.Left ventricular cavity obliteration: mechanism of the intracavitary gradient and differentiation from hypertrophic obstructive cardiomyopathy.Echocardiography. 2020; 37: 822-831Crossref PubMed Scopus (1) Google Scholar Since left ventricular hypertrophy is common in the elderly, recognizing the aortic valve fluttering by clinicians facilitates the correlation of clinical symptoms, consultation, and timely treatment for hypertrophic obstructive cardiomyopathy. eyJraWQiOiI4ZjUxYWNhY2IzYjhiNjNlNzFlYmIzYWFmYTU5NmZmYyIsImFsZyI6IlJTMjU2In0.eyJzdWIiOiI1YzAxZjJmZDVmYjg2ZTM4ZGFjM2EwMjc4ZDUyMGQ4YiIsImtpZCI6IjhmNTFhY2FjYjNiOGI2M2U3MWViYjNhYWZhNTk2ZmZjIiwiZXhwIjoxNjc1NzYwNDg4fQ.l8n6GzpnK5MPpC1H1vaaZNBSV9NXyo8dNwHvQgC-HV8KCQK6B7P0Eb2wyRB1OKRFVvXIu8cVL4pTqPEIchOdRO7GgaY6-MTWE-ZZZ8wFG9AaDLZGd3SK_NVRyPuThLWyjmXP5gQXvoFcqAmT3XqT3VZVCRfD5JbkLNTd8kms7lZw2PGWPRexp3Q2d_Dw5dWL8eBCZ5NaEBEAmvhio4GuUNjVoHR7tfEV8q06Hqur4PFADzFXVcNGWW87aGJxI9HcWgK6F86W17-3k0DTUt4BPMMDatDekNdROGq9X_ekBZ_Yv53pq652T6lVJYliLhMQNt19sqJR5r6mY2rzWF6O5A Download .mp4 (2.98 MB) Help with .mp4 files Video E1Ultrasonography with the parasternal long-axis view of the heart revealed a thick interventricular septum indicating left ventricular hypertrophy. In the brightness mode, a remarkable aortic valve fluttering in the systolic phase was noted (white arrow, 00:05). Color Doppler (00:09 to 00:13) demonstrated turbulent flows originating from the left ventricular outflow tract (black arrowheads, 00:10), which was compatible with hypertrophic obstructive cardiomyopathy. (Used with permission of Nin-Chieh Hsu, Taipei City Hospital, Taiwan.)

Surgery for patients with obstructive hypertrophic cardiomyopathy: challenge and future

After more than 60 years of development, with the deepening of the pathophysiological understanding of obstructive hypertrophic cardiomyopathy, the extent and resection thickness of myectomy have increased significantly. Myectomy combined with the correction of anomalies of the mitral valve apparatus has become the standard treatment of obstructive hypertrophic cardiomyopathy. Only a few centers worldwide can routinely perform it due to the difficulty. Because of the advances of new drugs and interventional therapy, the development of surgical treatment faces many challenges. At the same time, generations of cardiovascular surgeons are constantly trying to promote septal myectomy, including developing devices and the surgical field, as well as improving surgical planning by advanced technology. At present, the superior long-term efficacy of septal myectomy has been confirmed. It is necessary to work together to promote the treatment of hypertrophic obstructive cardiomyopathy, so as to guard people's health.

Effect analysis of myectomy guided by personalized three-dimensional reconstruction and printing in the treatment of obstructive hypertrophic cardiomyopathy

Objective: To examine the clinical efficacy of myectomy guided by personalized three-dimensional reconstruction and printing for patients with obstructive hypertrophic cardiomyopathy. Methods: The clinical data of 28 patients with obstructive hypertrophic cardiomyopathy, who underwent septal myectomy guided by personalized three-dimensional reconstruction and printing in the Department of Cardiaovascular Surgery, Guangdong Provincial People's Hospital from May 2020 to December 2021, were retrospectively analyzed. There were 14 males and 14 females, aging (51.1±14.0) years (range: 18 to 72 years). Enhanced cardiac computed tomography images were imported into Mimics software for preoperative three-dimensional reconstruction. The direction of the short axial plane of each segment was marked perpendicularly to the interventricular septum on the long axial plane of the digital cardiac model, then the thickness was measured on each short axial plane. A figurative digital model was used to determine the extent of resection and to visualize mitral valve and papillary muscle abnormalities. Correlation between the length, width, thickness, and volume of the predicted resected myocardium and those of the surgically resected myocardium was assessed by Pearson correlation analysis or Spearman correlation analysis. The accuracy of detecting mitral valve and papillary muscle abnormalities of transthoracic echocardiography and three-dimensional reconstruction was also compared. Results: There was no death or serious complications like permanent pacemaker implantation, re-sternotomy for bleeding, low cardiac output syndrome, stroke, or multiple organ dysfunction syndromes in the whole group. Namely, the obstruction of the left ventricular outflow tract was effectively relieved. The systolic anterior motion of the anterior mitral valve leaflet was absent in all patients after myectomy. The length, width, and thickness of the predicted resected myocardium by three-dimensional reconstruction were significantly positively correlated with the length (R=0.65, 95%CI: 0.37 to 0.82, P<0.01), width (R=0.39, 95%CI: 0.02 to 0.67, P<0.01), and thickness (R=0.82, 95%CI: 0.65 to 0.92, P<0.01) of the surgically resected myocardium, while the relation of the volume of the predicted resected myocardium and the volume of the surgically resected myocardium was a strong positive correlation (R=0.88, 95%CI: 0.76 to 0.94, P<0.01). Importantly, the interventricular septal myocardial thickness measured by preoperative transthoracic echocardiography showed a moderate positive correlation with the volume of surgically resected myocardium (R=0.52, 95%CI: 0.19 to 0.75, P<0.01). During a follow-up of (14.4±6.8) months (range: 3 to 22 months), no death occurred, and 1 patient was readmitted for endocardial radiofrequency ablation due to atrial fibrillation. Conclusion: Personalized three-dimensional reconstruction and printing can not only visualize the intracardiac structure but also guide septal myectomy by predicting the thickness, volume, and extent of resected myocardium to achieve ideal resection.

First-in-man application of Liwen RF™ ablation system in the treatment of drug-resistant hypertrophic obstructive cardiomyopathy.

This study sought to evaluate the clinical applicability of the Liwen Liu RF™ ablation system for percutaneous intramyocardial septal radiofrequency ablation (PIMSRA). Data on new cardiac radiofrequency ablation devices for the treatment of hypertrophic obstructive cardiomyopathy (HOCM) are limited. From July 2019 to July 2020, a total of 68 patients with drug-resistant HOCM, who underwent PIMSRA with the Liwen RF™ ablation system, which has an ablation electrode of stepless adjustable length, were prospectively enrolled. Safety endpoints included, amongst others, the occurrence of pericardial effusion and/or hemorrhage, cardiac arrhythmias, device failure and procedural death. The reduction in left ventricular outflow tract (LVOT) gradients at 12 months follow-up were used as a surrogate marker for device efficacy. All procedures were technically successful. The total energy output time of the system was 75.8 (IQR: 30.0) min, and the average power was 43.61 ± 13.34 watts. No ablation system error occurred. The incidence of pericardial effusion or hemorrhage, transient arrhythmia and resuscitation was 8.8, 39.7, and 1.5% during procedure, respectively. None of the patients died. During 30-day follow-up, there were no complications with the exception of a pericardial effusion in one patient (1.5%). No further complications were reported after 30 days. The patients' resting [baseline: 75 (IQR: 48) vs. 12-months: 12 (IQR: 19) mmHg, p < 0.001] and provoked [baseline: 122 (IQR: 53) vs. 12-months: 41 (IQR: 59) mmHg, p < 0.001] LVOT gradients decreased significantly during follow-up. In this study, we demonstrate the safety and feasibility of the Liwen RF™ ablation system to treat HOCM. The system allows for significant and sustainable LVOT gradient reduction during 12-months of follow-up. Hence, the Liwen RF™ ablation system is a promising new device that has the potential to become an alternative to existing septal reduction concepts in HOCM patients.

Abstract 11462: Alcohol Septal Ablation vs Surgical Myectomy for Hypertrophic Cardiomyopathy: A Systematic Review and Meta-Analysis

Introduction: Surgical Myectomy (SM) is the gold standard treatment for hypertrophic obstructive cardiomyopathy (HOCM). However, alcohol septal ablation (ASA) has emerged as an alternative option for selected patients. Nonetheless, the long-term efficacy and safety of ASA have been debated in recent years. The aim of this metanalysis is to evaluate the long-term outcomes of ASA vs SM in HOCM patients. Hypothesis: ASA is a safe and effective alternative to SM in HOCM. Methods: : Unrestricted searches of the PubMed, EMBASE, and Cochrane databases from inception till June 1, 2022, for studies comparing long-term outcomes of ASA with SM in HOCM patients. Relevant data were extracted and analyzed using Revman 5.3 software. Odds Ratio (OR) and 95% Confidence interval (CI) were calculated using the random-effects model. Results: : A total of 12 retrospective studies were included examining 7,599 HOCM patients (2,010 ASA vs 5,589 SM). After a mean follow-up of 5.04 years, all-cause mortality was similar between the two groups (OR 1.18; 95% CI 0.60-2.29). However, ASA was associated with high rates of reinterventions (OR 15.68; 95% CI 6.71-36.61), and pacemaker insertion (OR 2.74; 95% CI 1.39-5.41). Conclusions: Although there was no difference in mortality between ASA and SM, ASA was associated with higher rates of reinterventions and pacemaker insertion in long-term follow-up. Therefore, the selection of septal reduction therapy in HOCM should be individualized and should be performed in a comprehensive center after detailed risk and benefits discussions with an experienced team.

EE457 Long-Term Cost Effectiveness of Mavacamten for Treatment of Hypertrophic Obstructive Cardiomyopathy (HOCM)

Mavacamten is a first-in-class cardiac myosin modulator for HOCM, which is a genetic disorder of the cardiac sarcomere. This study assessed the cost effectiveness of mavacamten in addition to standard of care (SoC) compared with SoC alone, as well as disopyramide, myectomy, and septal ablation each in addition to SoC.

Septal Thickness Does Not Impact Outcome After Hypertrophic Obstructive Cardiomyopathy Surgery (Septal Myectomy and Subvalvular Mitral Apparatus Remodeling): A 15-Years of Experience.

BackgroundThe aim of this study was to assess the impact of septal thickness on long-term outcomes of surgical treatment for hypertrophic obstructive cardiomyopathy (HOCM) and correction of mitral subvalvular anomalies.MethodsSixty-six consecutive patients (58 ± 12 years, 56% female) undergoing extended septal myectomy and subvalvular mitral apparatus remodeling from 2007 to 2021 were retrospectively reviewed. Patients were divided into 2 groups according to septal thickness: moderate [< 18 mm, 29 patients (44%)] and severe [≥ 18 mm, 37 patients (56%)]. End points included survival, symptom improvement, reduction of left ventricle outflow tract (LVOT) gradient, resolution of mitral regurgitation (MR), and reoperation.ResultsThe mean interventricular septal thickness was 19 ± 3 mm, 15.8 ± 0.8 mm in patients with moderate and 21.4 ± 3.2 mm in those with severe hypertrophy. Preoperative data, intraoperative variables, postoperative complication rates, pre-discharge echocardiographic and clinical parameters did not differ between the two study groups [except for procedures involving the posterior mitral leaflet (p = 0.033) and septal thickness after myectomy (p = 0.0001)]. Subvalvular apparatus remodeling (secondary chordae of mitral valve resection and papillary muscle and muscularis trabecula procedures including resection, splitting, and elongation) was invariably added to septal myectomy (100%). Four (6%) procedures involved the posterior mitral leaflets. Mitral valve replacement was carried out in two patients (3%, p = 0.4). Reoperation for persistent MR was necessary in one patient (1%, p = 0.4). Neither iatrogenic ventricular septal defect nor in-hospital mortality occurred. During follow-up (mean 4.8 ± 3.8 years), two deaths occurred. NYHA class was reduced from 2.9 ± 0.7 to 1.6 ± 0.6 (p < 0.0001), the LVOT gradient from 89.7 ± 34.5 to 16.3 ± 8.8 mmHg (p < 0.0001), mitral valve regurgitation grade from 2.5 ± 1 to 1.2 ± 0.5 (p < 0.0001), and septal thickness from 18.9 ± 3.7 to 13.9 ± 2.7 mm (p < 0.0001).ConclusionsRegardless of septal thickness, subvalvular apparatus remodeling with concomitant septal myectomy can provide satisfactory long-term outcomes in terms of symptom improvement, LVOT obstruction relief, and MR resolution (without mitral valve replacement in most cases) in patients with HOCM.

Review of Contemporary Invasive Treatment Approaches and Critical Appraisal of Guidelines on Hypertrophic Obstructive Cardiomyopathy: State-of-the-Art Review.

Background: Hypertrophic obstructive cardiomyopathy (HOCM) is a heterogeneous disease with different clinical presentations, albeit producing similar dismal long-term outcomes if left untreated. Several approaches are available for the treatment of HOCM; e.g., alcohol septal ablation (ASA) and surgical myectomy (SM). The objectives of the current review were to (1) discuss the place of the standard invasive treatment modalities (ASA and SM) for HOCM; (2) summarize and compare novel techniques for the management of HOCM; (3) analyze current guidelines addressing HOCM management; and (4) offer suggestions for the treatment of complex HOCM presentations. Methods: We searched the literature and attempted to gather the most relevant and impactful available evidence on ASA, SM, and other invasive means of treatment of HOCM. The literature search yielded thousands of results, and 103 significant publications were ultimately included. Results: We critically analyzed available guidelines and provided context in the setting of patient selection for standard and novel treatment modalities. This review offers the most comprehensive analysis to-date of available invasive treatments for HOCM. These include the standard treatments, SM and ASA, as well as novel treatments such as dual-chamber pacing and radiofrequency catheter ablation. We also account for complex pathoanatomic presentations and current guidelines to offer suggestions for tailored care of patients with HOCM. Finally, we consider promising future therapies for HOCM. Conclusions: HOCM is a heterogeneous disease associated with poor outcomes if left untreated. Several strategies for treatment of HOCM are available but patient selection for the procedure is crucial.

Transaortic Shallow Septal Myectomy and Cutting of Secondary Fibrotic Mitral Valve Chordae-A 5-Year Single-Center Experience in the Treatment of Hypertrophic Obstructive Cardiomyopathy.

Background: Anomalies of the mitral apparatus have been shown to contribute to left ventricular outflow obstruction in patients with hypertrophic cardiomyopathy (HCM). We report our 5-year single-center experience with a shallow myectomy procedure associated with transaortic mitral valve repair in a cohort of HCM patients. Methods: We studied 83 consecutive patients who underwent surgical treatment of symptomatic left ventricular outflow obstruction. In all study patients, a transaortic shallow septal myectomy was performed. Fibrous or muscular structures connecting the papillary muscles to the septum or free wall were resected, and fibrotic secondary chordae of the anterior mitral valve were cut selectively. Results: We report one death (1.2%) during hospitalization, no iatrogenic ventricular septal defects, and two (2.4%) mitral valve replacements. At discharge, no patients were in New York Heart Association (NYHA) Class III/IV, from 49 (59%) preoperatively. Mean maximal septal thickness decreased from 24 ± 6 to 16 ± 3 mm. Mean outflow gradient decreased from 93 ± 33 to 13 ± 11 mmHg. Grade 3 or 4 mitral regurgitation was noticed in one patient postoperatively, from 32 (39%) before surgery. Conclusions: Shallow septal myectomy associated with secondary mitral valve chordal cutting and papillary muscle mobilization provided excellent results offering adequate treatment of outflow obstruction.

P291 SUBVALVULAR MITRAL APPARATUS REMODELING DURING HYPERTROPHIC OBSTRUCTIVE CARDIOMYOPATHY SURGERY: A 15–YEARS OF EXPERIENCE

Abstract Background and Aims To assess the role of the mitral valve apparatus (leaflets, chordae and papillary muscles (PM) in left ventricle outflow tract (LVOT) obstruction, and results of the surgical treatment for hypertrophic obstructive cardiomyopathy (HOCM) in a retrospective observational study. Methods Seventy consecutive patients (58±12 years, M/F ratio: 41/29) undergoing HOCM surgery from 2007 to 2021 at our institute were reviewed. End points included the involvement of the mitral valve in LVOT obstruction, mortality, and change in clinical and echocardiographic characteristics after HOCM surgery. Results Secondary chordae tendineae tractioning the anterior mitral leaflet to the interventricular septum were detected in the majority of the patients. Anomalous, hypertrophied, and fused PM with muscularis trabeculae hypertrophy were a common findings. Four patients had posterior leaflet redundancy. Secondary chordae (90%), PM and muscularis trabeculae resection and PM splitting and elongation (77%) were added variably to septal myectomy (100%). Nine procedures (6%) on mitral valve leaflets were performed, involving 6 posterior and 3 anterior mitral leaflets. Long–term follow up was 4.6±3.7 years. There was no hospital mortality, and NYHA was reduced from 3±0.6 to 1±0.7 (p &amp;lt; 0.0001), the LVOT gradient from 91±35 to 21±17 mmHg (p &amp;lt; 0.0001), mitral valve regurgitation from grade 3±1 to 1±0.6 (p &amp;lt; 0.0001), and septum thickness from 19±3 to 14±2 mm (p &amp;lt; 0.0001). Conclusions The mitral valve apparatus with all its components contribute variably to LVOT dynamic obstruction thus, surgical correction in addition to extended myectomy is recommended to achieve the best outcome.

Surgical treatment of obstructive hypertrophic cardiomyopathy in children with Noonan syndrome

About 80-90% of patients with Noonan syndrome have changes in the cardiovascular system, 30% of which include cardiomyopathy, hypertrophic phenotype in particular. The treatment of cardiomyopathy patients with Noonan syndrome is carried out according to the clinical recommendations for the treatment of chronic heart failure (CHF) and cardiomyopathy. Surgical treatment of progressive nature of heart failure with left ventricular outflow tract obstruction and mitral regurgitation can be one of the possible methods. The review presents our experience with septal myectomy (SM) in children suffered from hypertrophic obstructive cardiomyopathy with Noonan syndrome. Objective. To assess the efficiency of septal myectomy in children suffered from hypertrophic obstructive cardiomyopathy with Noonan syndrome. Materials and methods. Clinical examination (family history, patient examination), lab tests (NT-proBNP level), echocardiography, electrocardiography, Holter monitoring, Chest X-rays and molecular genetic methods using mass parallel sequencing (NGS) and direct automatic Sanger sequencing. The indications for the SM were determined according to the clinical guidelines for the diagnostic and treatment of hypertrophic cardiomyopathy. Results. 7 patients out of 48 with Noonan syndrome underwent SM and 1 with Noonan syndrome with multiple lentigo (LEOPARD). Two of them required the removal of obstruction of the outflow tracts of both ventricles, followed by plastic removal of the right ventricle and pulmonary artery valve. Related surgical interventions included aortic (n = 1) and mitral valves (n = 2), permanent pacemaker (n = 1) in the early postoperative period. After a year, all children recorded a decline of NT-proBNP from the average of 16,198 to 3,865 pg/ml, a significant improvement in health, disappearance of shortness of breath, increased physical activity tolerance, improved physical (weight, growth) and speech development. When assessing the dynamics of the Echo parameters 1 year after the SM, normalization of the size of both atria in 4 cases, reduction of severity of isolated dilation of the left atrium in 2 cases was noted. No cases of repeated obstruction have been reported. Conclusion. Hypertrophic cardiomyopathy (HCM) can be in structure of hereditary syndromes such as Noonan syndrome. Septal myectomy (SM) can now be used in the case of progressive nature of heart failure with left ventricular outflow tract obstruction in children with Noonan syndrome.

Endocardial Radiofrequency Ablation vs. Septal Myectomy in Patients With Hypertrophic Obstructive Cardiomyopathy: A Systematic Review and Meta-Analysis

BackgroundSeptal myectomy (SM) has been the gold standard therapy for most patients with hypertrophic obstructive cardiomyopathy (HOCM). Endocardial radiofrequency ablation of septal hypertrophy (ERASH) is a novel treatment for septal reduction. We aimed to assess the efficacy and safety between two treatment strategies.MethodsWe searched PubMed, Web of Science, Cochrane Library, and Embase databases to identify relevant studies published up to March 2021. Random-effect models were used to calculate standardized mean difference (SMD) and 95% confidence intervals (CIs) for resting left ventricular outflow tract gradient (LVOTG) and septal thickness.ResultsTwenty-five studies are included in this review, eighteen studies for SM and seven studies for ERASH. During follow-up, there were significant reductions of the mean resting LVOTG in adults (SM groups: SMD = −3.03, 95% CI [−3.62 to −2.44]; ERASH groups: SMD = −1.95, 95% CI [−2.45 to −1.45]) and children (SM groups: SMD = −2.67, 95% CI [−3.21 to −2.12]; ERASH groups: SMD= −2.37, 95% CI [−3.02 to −1.73]) after the septal reduction therapies. For adults, SM groups contributed to more obvious reduction than ERASH groups in interventricular septal thickness (SM groups: SMD = −1.82, 95% CI [−2.29 to −1.34]; ERASH groups: SMD = −0.43, 95% CI [−1.00 to 0.13]). The improvement of the New York Heart Association class was similar in the two groups (SM groups: 46.4%; ERASH groups: 46.7%). The periprocedural mortality in SM and ERASH were 1.1 and 1.8%, respectively.ConclusionsThis systematic review suggests that SM is superior to ERASH in the treatment of HOCM. But for the patients who are at risk for open cardiac surgeries or prefer a less invasive approach, ERASH might be an optional approach.

Minimally invasive right infra-axillary thoracotomy for transaortic septal myectomy.

Extended left ventricular septal myectomy remains the gold standard for the treatment of hypertrophic obstructive cardiomyopathy (HOCM) with refractory symptoms. On the basis of traditional modified transaortic Morrow myectomy, we innovatively performed a minimally invasive, video-assisted single-port thoracotomy through the right infra-axillary region. Our procedure can provide good visualization of the left ventricular outflow tract and hypertrophic ventricular septum for accurate resection. It also ensures optimal exposure of the mitral valve in the presence of complex mitral subvalvular structures.

Surgical treatment of hypertrophic obstructive cardiomyopathy in relatively elderly patients: Short- and long-term outcomes.

Our goal was to assess the short- and long-term outcomes of surgical treatment for hypertrophic obstructive cardiomyopathy in patients ≥65 years of age compared to patients &lt; 65 years of age. Sixty-four patients aged ≥65 years, surgically treated for symptomatic hypertrophic obstructive cardiomyopathy, were compared to a control group of 125 patients &lt;65 years. Patients aged ≥65 years were less frequently male (36% vs 68%, P &lt; 0.001) and had higher EuroSCORE II scores [1.4 (1.1-2.2) vs 0.8 (0.7-1.2), P &lt; 0.001], lower risk of sudden death, higher pulmonary artery pressure [40 (30-50) vs 30 (30-43), P = 0.04) and more mitral annulus calcifications (44% vs 14%, P &lt; 0.001) compared to younger patients.Hospital death was 1%, with no difference between the 2 groups (1.5% vs 0.8%, P = 0.9).Patients aged ≥65 years had more concomitant coronary bypass grafting (12% vs 5%, P = 0.05) and a higher incidence of blood transfusions (50% vs 17%, P &lt; 0.001) and postoperative atrial fibrillation (19% vs 8%, P = 0.02).Follow-up was 98% complete [median 8.3 (5.3-12.8) years]. The 13-year survival in the group aged ≥65 was 54 (SD: 9) % vs 83 (SD: 5) % in the control group (P &lt; 0.001), but it was comparable to that expected in the age-sex matched general national population.At 13 years, the cumulative incidence function of cardiac death in the elderly group was 19 (SD: 7)%, mostly unrelated to hypertrophic cardiomyopathy causes.At the last follow-up, 90% of patients were in New York Heart Association functional class I-II and 68% were in sinus rhythm. Selected elderly symptomatic patients with hypertrophic obstructive cardiomyopathy can benefit from surgery, with low hospital mortality and morbidity, relief of symptoms and late survival comparable to that expected in the age-sex matched general population.

Efficacy and safety of endocardial radiofrequency catheter ablation of interventricular septal hypertrophy in the treatment of hypertrophic obstructive cardiomyopathy

Hypertrofická obstrukční kardiomyopatie je charakterizována ztluštěním stěn a zvětšením masy myokardu nedilatované levé komory srdeční bez vysvětlujících hemodynamických příčin a přítomnosti obstrukce ve výtokovém traktu levé komory. V minulosti byla invazivní nefarmakologickou metodou volby k redukci septální hypertrofie chirurgická myektomie, kterou v devadesátých letech vystřídala alkoholová septální ablace, nyní všeobecně užívaná, bezpečná a účinná metoda v redukci septální hypertrofie. Toto review se zabývá novou metodu používanou v redukci septální hypertrofie, kterou je endokardiální radiofrekvenční ablace septální hypertrofie (ERASH). Naše práce shrnuje dosud publikované studie a srovnává jejich výsledky s jinými metodami redukce septální hypertrofie. ERASH se jeví jako srovnatelně účinná a bezpečná metoda jako alkoholová septální ablace se srovnatelným poklesem gradientu ve výtokovém traktu levé komory a subjektivním zlepšením stavu pacienta charakterizovaným poklesem v třídě NYHA. &#169; 2022, ČKS.

Image-Based Computational Hemodynamics Analysis of Systolic Obstruction in Hypertrophic Cardiomyopathy.

Hypertrophic Cardiomyopathy (HCM) is a pathological condition characterized by an abnormal thickening of the myocardium. When affecting the medio-basal portion of the septum, it is named Hypertrophic Obstructive Cardiomyopathy (HOCM) because it induces a flow obstruction in the left ventricular outflow tract. In any type of HCM, the myocardial function can become compromised, possibly resulting in cardiac death. In this study, we investigated with computational analysis the hemodynamics of patients with different types of HCM. The aim was quantifying the effects of this pathology on the intraventricular blood flow and pressure gradients, and providing information potentially useful to guide the indication and the modality of the surgical treatment (septal myectomy). We employed an image-based computational approach, integrating fluid dynamics simulations with geometric and functional data, reconstructed from standard cardiac cine-MRI acquisitions. We showed that with our approach we can better understand the patho-physiological behavior of intraventricular blood flow dynamics due to the abnormal morphological and functional aspect of the left ventricle. The main results of our investigation are: (a) a detailed patient-specific analysis of the blood velocity, pressure and stress distribution associated to HCM; (b) a computation-based classification of patients affected by HCM that can complement the current clinical guidelines for the diagnosis and treatment of HOCM.

Surgical treatment of obstructive hypertrophic cardiomyopathy combined with muscle bridging of the left anterior descending artery

Myocardial bridging is often detected during diagnostic coronary angiography in patients with congenital and acquired heart defects. In most cases, muscle bridging does not require surgical treatment. Myocardial bridging and tunnels causing compression of one or more major coronary arteries is a potential cause of myocardial ischemia in patients with hypertrophic cardiomyopathy. The authors report surgical treatment of a patient with obstructive hypertrophic cardiomyopathy and myocardial bridging of the left anterior descending artery.

Rare case of aortopathy revealing hypertrophic cardiomyopathy

Hypertrophic cardiomyopathy (HCM) is the most prevalent (0.2%) heritable, genetic cardiovascular disease. Aortopathies also represent a spectrum of familial inheritance with prevalence of ~4%. Previous research evaluating characteristics of the aorta in HCM patients have demonstrated increased vascular stiffness and differences in aortic elastic properties compared to healthy controls. However, a prevalence of aortopathy in HCM patients has not been previously described and a potential relationship between HCM and aortic dilation has not been established limited by relatively small patient cohorts. We report the case of a 64 years old patient who was admitted to our cardiology department for the treatment of obstructive hypertrophic cardiomyopathy associated with anevrysmal dilation of the ascending aorta.

Projecting the Long-term Clinical Value of Mavacamten for the Treatment of Obstructive Hypertrophic Cardiomyopathy in the United States: An Assessment of Net Health Benefit

PurposeThe aim of the study was to project the long-term net health benefits of mavacamten for the treatment of symptomatic obstructive hypertrophic cardiomyopathy (HCM) in the United States. MethodsA Markov model with 4 mutually exclusive health states (New York Heart Association [NYHA] functional classes I, II, and III/IV and death) was developed to project the life-years (LYs) and quality-adjusted life-years (QALYs) over a lifetime horizon for patients with symptomatic obstructive HCM receiving mavacamten with or without β-blocker (BB) or calcium channel blocker (CCB) monotherapy or placebo with or without BB or CCB monotherapy. The model simulated a patient cohort with a starting age of 59 years and 41% women. Transition probabilities across NYHA functional classes were estimated using data from the Phase III Clinical Study to Evaluate Mavacamten (MYK-461) in Adults With Symptomatic Obstructive Hypertrophic Cardiomyopathy (EXPLORER-HCM) and the EXPLORER long-term extension (EXPLORER-LTE) cohort from the Long-term Safety Extension Study of Mavacamten in Adults who Have Completed MAVERICK-HCM or EXPLORER-HCM (MAVA-LTE) trial and were extrapolated after week 30. The mortality risks of NYHA functional class I were assumed to be the age- and sex-specific mortality risks of the US general population. The mortality risks for NYHA class II and III/IV were estimated using those for class I in conjunction with the relative mortality risks derived using patients with obstructive HCM from a large real-world registry. Health state utilities for each treatment were estimated from EXPLORER-HCM. Both LYs and QALYs were aggregated over a lifetime for each treatment arm, discounted at 3% annually, and compared between the 2 arms. Sensitivity analyses were conducted to evaluate the robustness of the model findings. FindingsOver a lifetime, treatment with mavacamten with or without BB or CCB monotherapy was associated with 3.67 incremental LYs compared with placebo with or without BB or CCB monotherapy (13.00 vs 9.33 LYs). Compared with individuals in the placebo group, patients in the mavacamten group were projected to spend 6.17 additional LYs in NYHA functional class I and 0.04 and 2.46 fewer LYs in NYHA functional classes II and III/IV, respectively. With utilities incorporated, mavacamten with or without BB or CCB monotherapy was associated with 4.17 additional QALYs compared with placebo with or without BB or CCB monotherapy (11.74 vs 7.57 QALYs). In the sensitivity analyses, incremental benefits ranged from 1.55 to 6.21 LYs and from 2.48 to 6.19 QALYs across the scenarios. ImplicationsThis model projected substantial net health benefits associated with mavacamten for symptomatic obstructive HCM owing to improved patient survival and quality of life. The projected QALY gain underscored the likely long-term clinical value of mavacamten in symptomatic obstructive HCM.

Abstract 10713: Long-Term Impact of Alcohol Septal Ablation on Left Ventricular Remodeling and Diastolic Function: Retrospective Echocardiographic Assessment

Introduction: Alcohol septal ablation (ASA) is a percutaneous procedure for patients with refractory hypertrophic obstructive cardiomyopathy (HOCM). Asymmetric septal hypertrophy (ASH) and systolic anterior motion of the mitral valve (SAM) results in a dynamic left ventricular outflow tract gradient (LVOTG). LVOTG leads to concentric left ventricular hypertrophy and diastolic dysfunction. Hypothesis: We tested the hypothesis that patients who underwent ASA for treatment of HOCM would experience left ventricular remodeling and long-term improvement in diastolic function. Methods: Using a patient database maintained internally we analyzed the echocardiograms of 96 patients (36:60 M:F)(Age=60.5±13.2 years) who underwent successful ASA and had a follow-up echocardiogram at least 270 days following the procedure. Parameters used to describe diastology included interventricular septal thickness (IVSd), LV mass index (LVd mass index), LV diastolic internal diameter (LVIDd), LV end diastolic volume index (LVEDVi), left atrial end systolic volume index (LAESVi), early and late velocities across the mitral valve (MV E and A, respectively), and the MV E/A ratio. A paired t-test was used to compare baseline and follow-up echocardiogram values within individual subjects. Results: The time between ASA and follow-up echocardiogram was 653.9±350.4 days (mean±SD). At follow-up, the resting and provoked gradients were significantly reduced. Baseline and follow-up echocardiogram values were compared, revealing a significant difference in IVSd, LVPWd, LVd mass index, LVEDVi, LAESVi, MV E velocity, MV A velocity and MV E/A ratio. (Figure 1) Conclusions: Alcohol septal ablation induces significant regression of left ventricular hypertrophy and improvement in diastolic function. These changes likely contribute to long-term symptomatic improvement in patients who undergo alcohol septal ablation for treatment of hypertrophic obstructive cardiomyopathy.