2024 AHA/ACC/AMSSM/HRS/PACES/SCMR Guideline for the Management of Hypertrophic Cardiomyopathy: A Report of the American Heart Association/American College of Cardiology Joint Committee on Clinical Practice Guidelines.

AIM: The "2024 AHA/ACC/AMSSM/HRS/PACES/SCMR Guideline for the Management of Hypertrophic Cardiomyopathy" provides recommendations to guide clinicians in the management of patients with hypertrophic cardiomyopathy. METHODS: A comprehensive literature search was conducted from September 14, 2022, to November 22, 2022, encompassing studies, reviews, and other evidence on human subjects that were published in English from PubMed, EMBASE, the Cochrane Library, the Agency for Healthcare Research and Quality, and other selected databases relevant to this guideline. Additional relevant studies, published through May 23, 2023, during the guideline writing process, were also considered by the writing committee and added to the evidence tables, where appropriate. STRUCTURE: Hypertrophic cardiomyopathy remains a common genetic heart disease reported in populations globally. Recommendations from the "2020 AHA/ACC Guideline for the Diagnosis and Treatment of Patients With Hypertrophic Cardiomyopathy" have been updated with new evidence to guide clinicians.

Del nido versus conventional blood cardioplegia in patients with obstructive hypertrophic cardiomyopathy.

BACKGROUND: Myocardial protection is essential in cardiac surgery with cardiopulmonary bypass The Del Nido cardioplegia which was initially used in pediatric cardiac surgery, has been increasingly used in adult cardiac surgery recently. However, no literature has reported the efficacy of DNC in hypertrophic obstructive cardiomyopathy. METHODS: This retrospective study involved elective patients who underwent extended surgical myectomy with or without concomitant cardiac surgical procedures between September 2017 and June 2022. Patients were distributed into two groups, the DNC and the CBC group. The primary outcome was high-sensitivity cardiac troponin I (hs-TnI) and creatine kinase-MB (CK-MB) levels at the 0, 1, and 2 postoperative days. The secondary outcomes contained: intraoperative LVEF, return to spontaneous rhythm; postoperative myocardial infarction, worsening or deteriorating of EF, mechanical circulatory support; new-onset atrial fibrillation; mechanical ventilation duration; intensive care unit hours; in-hospital days. RESULTS: Fifty-nine patients were included and divided into the CBC (n = 15) and the DNC group (n = 44). There was no statistical difference in patients' demographics and preoperative parameters between the two groups. No in-hospital mortality. The total cardioplegia volume [21.93(18.36,26.07) vs. 25.68(23.17,37.12), p = 0.012] and infusion times [1(1,1) vs. 2(2,3), p = 0.000] were less and the incidence of return to spontaneous rhythm after declamping was higher in the DNC group [97.7% vs. 73.3%, p = 0.013]. Postoperative hs-TnI and CK-MB levels were comparable between the two groups. A longer DNC infusion interval was associated with higher levels of CK-MB on postoperative day 1 and day 2 (p = 0.009 and p = 0.011, respectively). CONCLUSIONS: The use of DNC in extended surgical myectomy procedure was as safe and effective as CBC. However, DNC infusion interval over 60 minutes was associated with increased postoperative CK-MB levels.

2020 AHA/ACC Guideline for the Diagnosis and Treatment of Patients With Hypertrophic Cardiomyopathy: Executive Summary: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines.

Aim This executive summary of the hypertrophic cardiomyopathy clinical practice guideline provides recommendations and algorithms for clinicians to diagnose and manage hypertrophic cardiomyopathy in adult and pediatric patients as well as supporting documentation to encourage their use. Methods A comprehensive literature search was conducted from January 1, 2010, to April 30, 2020, encompassing studies, reviews, and other evidence conducted on human subjects that were published in English from PubMed, EMBASE, the Cochrane Collaboration, Agency for Healthcare Research and Quality reports, and other relevant databases. Structure Many recommendations from the earlier hypertrophic cardiomyopathy guidelines have been updated with new evidence or a better understanding of earlier evidence. This summary operationalizes the recommendations from the full guideline and presents a combination of diagnostic work-up, genetic and family screening, risk stratification approaches, lifestyle modifications, surgical and catheter interventions, and medications that constitute components of guideline directed medical therapy. For both guideline-directed medical therapy and other recommended drug treatment regimens, the reader is advised to follow dosing, contraindications and drug-drug interactions based on product insert materials.

Review on sudden death risk reduction after septal reduction therapies in hypertrophic obstructive cardiomyopathy.

Treatment of left ventricular outflow tract (LVOT) obstruction in hypertrophic obstructive cardiomyopathy (HOCM) with septal reduction, either with myectomy or alcohol septal ablation, is aiming to reduce the LVOT gradient and improve symptoms in patients who are refractory to or do not tolerate medical treatment. Apart from contributing to the evolution to heart failure, LVOT obstruction is considered a risk factor for sudden cardiac death (SCD). Both septal reduction treatments have been proven effective in reducing symptoms and seem to improve survival, which has been shown equal to the expected in the normal population. SCD is probably reduced after septal reduction, implying that LVOT obstruction is a major factor predisposing to ventricular tachyarrhythmias. Although available algorithms for SCD stratification have not been tested in patients after septal reduction treatments, effective treatment improves SCD risk profile substantially. Furthermore, high-risk patients with already implanted implantable cardioverter defibrillators (ICDs) before septal reduction show very low appropriate ICD shock rate after effective treatment. It should be noted, however, that the best outcomes for septal myectomy or ablation have been reported in HOCM patients treated in high-volume centres, which substantiates the need to refer patients to centres with high procedural expertise.

Transcatheter septal ablation in hypertrophic obstructive cardiomyopathy: a technical guide and review of published results.

Transcatheter alcohol septal ablation (ASA) treatment of symptomatic patients with hypertrophic obstructive cardiomyopathy (HOCM) is based on the existence and degree of intraventricular obstruction. Patients with significant gradient and symptoms who do not respond to optimal medical therapy are eligible to gradient reduction through a surgical (septal myectomy) or a transcatheter (alcohol septal ablation) septal reduction. The latter encompasses occlusion of a septal branch perfusing the hypertrophied septum, which is involved in the generation of obstruction, by injecting ethanol into the supplying septal branch(es). ASA has been established as a highly effective and safe method and has outnumbered the surgical gold standard. Although the technique is straightforward, patient selection and some technical details may influence the efficacy and safety of the procedure. The technique is based on echocardiographic contrast guidance, which allows accurate target septal branch selection and optimisation of the result. Published long-term results from high-volume centres have confirmed the effectiveness of ASA and have shown excellent survival, which is comparable to that in the general population. Choice and performance of the surgical or interventional treatment should be implemented in highly specialised centres in terms of a heart-team approach, taking notice of anatomic characteristics as well as comorbidities. Involvement of all cases in international registries may reveal the individual merits and indications for the surgical and interventional treatment in HOCM.

Underappreciated occurrence of discrete subaortic membranes producing left ventricular outflow obstruction in hypertrophic cardiomyopathy.

Subaortic obstruction due to systolic anterior motion (SAM) of the mitral valve with ventricular septal contact is a major cause of progressive heart failure symptoms in patients with hypertrophic cardiomyopathy (HCM). However, we have recently observed a unique, but not uncommon subgroup of HCM patients with outflow tract obstruction due only to discrete subaortic membrane or who have a membrane in addition to SAM-septal contact. HCM patients with subaortic membranes may be at increased risk for developing progressive heart failure symptoms. Identification requires a high index of suspicion and raises important management considerations, including need for surgical myectomy for definitive relief of obstruction with associated excellent outcomes, as the alternative invasive treatment option with percutaneous alcohol septal ablation would be ineffective.

Measurements of the Interventricular Septum and Mitral Leaflet Length in Hypertrophic Cardiomyopathy Patients Who Underwent Surgical Myectomy: A Prospective Comparative Multimodality Imaging Study.

Transesophageal echocardiography (TEE) plays an important role for real-time procedural guidance during surgical smyectomy (SM) for hypertrophic obstructive cardiomyopathy (HOCM). We aimed to compare (1) interventricular septum (IVS) thickness using 2- (2D) and 3-dimensional (3D) intraoperative TEE and preoperative cardiac magnetic resonance (CMR) and (2) mitral valve (MV) leaflet length using 2D, 3D TEE, automatic quantification of mitral valve (AMVQ) and preoperative CMR. We prospectively studied 50 patients with HOCM (age 59 ± 12 years, 44% men) who underwent SM during 2018 to 2019. The maximal basal, mid, and distal anteroseptum (AS) and inferoseptum (IS) were measured by multiplanar 3D reconstruction on TEE and by short-axis imaging on preoperative CMR and classified as mild (≤18 mm), moderate (18 to 25 mm), or severe (≥25 mm) groups based on AS and IS thickness on CMR. MV leaflet lengths were evaluated by preoperative CMR and intraprocedural 2D TEE, zoom 3D TEE, and AMVQ (EchoPAC, General Electric, Wisconsin). There was a moderate correlation between AS and IS thickness on 3D TEE and CMR (R2 = 0.46, p <0.01 and R2 = 0.41, p <0.01, respectively), with 3D TEE showing an average overestimation of 3.8 and 4.7 mm versus CMR. The 3D TEE overestimated 14 patients (56%) with mild thickness as moderate and 5 patients (22%) with moderate thickness as severe. Assuming 3D TEE as the gold standard, the closest correlation for anterior mitral leaflet length was with CMR (average overestimation by CMR of 0.5 mm [root mean square deviation (RMSE%) 17]), intermediate correlation with 2D TEE (average deviation of 0.6 mm [RMSE% 21]) and no correlation with AMVQ (average deviation of 0.7 mm [RMSE% 24]). In conclusion, 3D TEE overestimates IVS thickness versus CMR in patients with HOCM who underwent SM, with greater discrepancy in those with thinner IVS. There are significant differences in MV lengths measured using different imaging techniques.

2024 AHA/ACC/AMSSM/HRS/PACES/SCMR Guideline for the Management of Hypertrophic Cardiomyopathy: A Report of the American Heart Association/American College of Cardiology Joint Committee on Clinical Practice Guidelines.

AIM: The "2024 AHA/ACC/AMSSM/HRS/PACES/SCMR Guideline for the Management of Hypertrophic Cardiomyopathy" provides recommendations to guide clinicians in the management of patients with hypertrophic cardiomyopathy. METHODS: A comprehensive literature search was conducted from September 14, 2022, to November 22, 2022, encompassing studies, reviews, and other evidence on human subjects that were published in English from PubMed, EMBASE, the Cochrane Library, the Agency for Healthcare Research and Quality, and other selected databases relevant to this guideline. Additional relevant studies, published through May 23, 2023, during the guideline writing process, were also considered by the writing committee and added to the evidence tables, where appropriate. STRUCTURE: Hypertrophic cardiomyopathy remains a common genetic heart disease reported in populations globally. Recommendations from the "2020 AHA/ACC Guideline for the Diagnosis and Treatment of Patients With Hypertrophic Cardiomyopathy" have been updated with new evidence to guide clinicians.

Hypertrophic obstructive cardiomyopathy caused by Fabry disease: implications for surgical myectomy.

Hypertrophic obstructive cardiomyopathy can be the phenotype of storage disorders as Fabry disease cardiomyopathy. In this instance, its recognition through GLA gene analysis and preventive administration of enzyme replacement therapy may reduce heart failure risk of surgical septal myectomy (SSM). A 59-year-old man was referred for SSM as dyspnoea and low threshold muscle fatigue associated to severe left ventricular outflow obstruction (gradient of 100 mmHg) due to both interventricular septal hypertrophy and mitral leaflet systolic anterior motion were not controlled by metoprolol 100 mg bid. Electrocardiogram showed sinus rhythm and a complete left bundle branch block. Cardiac magnetic resonance imaging showed a preserved left ventricular (LV) contractility (ejection fraction 70%) but failed to reveal reduced T1 mapping and fibrosis of postero-lateral LV wall suggesting Fabry disease cardiomyopathy. Cardiac catheterization and coronary angiography documented increased LV end-diastolic pressure but normal coronary arteries. SSM was followed by acute renal and heart failure with left ventricular ejection fraction declining to 35%. Histology of SSM showed regularly arranged severely enlarged cardiomyocytes containing extensive vacuoles that were intensely positive to immunofluorescence with anti-Gb3 antibodies and appeared at electron microscopy to consist of myelin bodies suggesting the diagnosis of FD. This entity was confirmed by low blood levels of alpha-galactosidase A (0.8 nmol/mL/h; NV > 1), high values of Lyso-Gb3 (5.85 nmol/L; NV < 2.3), and the presence of the pathogenic mutation c.644A>G in the exon 5 of GLA gene. This study emphasizes the importance of a genetic screening for FD before SSM be considered for hypertrophic obstructive cardiomyopathy.

Sex-based outcomes of surgical myectomy for hypertrophic cardiomyopathy: An analysis from the National Readmission Database.

BACKGROUND: There is a paucity of data on sex differences in outcomes after surgical myectomy (SM) for hypertrophic cardiomyopathy (HCM). METHODS: Patients who received SM for HCM during October 1, 2015, through December 31, 2018, were identified from the US National Readmission Database. The primary end point of this study was in-hospital mortality. The secondary end points were major bleeding, acute kidney injury, new pacemaker implantation, severe disability surrogates (non-home discharge and need for mechanical ventilation), resources utilization surrogates (length of stay and cost of hospitalization), and 30-day outcomes (readmission rate, mortality, and new pacemaker insertion). RESULTS: A total of 3031 patients were included in the current analysis. Using propensity score matching, 2 well matched cohorts were compared (women = 1170 and men = 1127). Women had a higher requirement for new pacemaker insertion compared with men (10.9% vs 6.8%; P = .029), higher number of non-home discharges (13.8% vs 7.9%; P < .01), and longer length of hospital stay (median = 7 [interquartile range, 5-9] days) versus (median = 6 [interquartile range, 5-8] days). There was no difference in in-hospital mortality, major bleeding, blood transfusion, acute kidney injury, or hospitalization costs for women versus men. At 30 days, women continued to show a higher need for pacemaker insertion (11.3% vs 7.1%; P = .03) and had a higher readmission rate than men (10.9% vs 7.1%; P = .02). There was no difference in 30-day mortality between women and men (3% vs 2.4%; P = .54). CONCLUSIONS: Among the HCM cohort who received SM, significant sex-based differences in the outcomes were observed. Women had higher new pacemaker insertion rate, higher non-home discharge rate, and higher rate of 30-day readmission compared with men.

A Narrative Review of Emerging Therapies for Hypertrophic Obstructive Cardiomyopathy.

Hypertrophic obstructive cardiomyopathy is a hereditary condition that affects myocardial contraction. In case of failure of pharmacological treatment, alternative approaches might be used that include surgical myectomy, percutaneous transluminal septal myocardial ablation, and radiofrequency ablation. In respect of long-term advantages, surgical septal myectomy remains the therapy of choice for symptomatic hypertrophic obstructive cardiomyopathy. Alcohol septal ablation has been considered an alternative to surgical myectomy, which confers the benefits of a shorter hospital stay, less discomfort, and fewer complications. However, only expert operators should perform it on carefully chosen patients. Further, radiofrequency septal ablation reduces the left ventricular outflow tract gradient and improves the NYHA functional class of patients with hypertrophic obstructive cardiomyopathy, despite complications like cardiac tamponade and atrioventricular block. Further research with a larger sample size is required to compare the radiofrequency approach with established invasive treatment methods for hypertrophic obstructive cardiomyopathy. Septal myectomy has low morbidity and mortality rates, making it the preferred procedure; however, the efficacy and morbidity remain debatable. Advances in invasive techniques, including percutaneous septal radiofrequency ablation and transcatheter myotomy, have provided alternative approaches for reducing left ventricular outflow tract (LVOT) obstruction in patients who are not candidates for traditional surgical septal myectomy. Candidates for alcohol and radiofrequency septal ablation include patients with symptomatic hypertrophic obstructive cardiomyopathy, older adults, and those with multiple comorbidities.

Long-term outcomes and risk factors for mortality of patients with hypertrophic obstructive cardiomyopathy undergoing septal myectomy.

Background: We aimed to evaluate the long-term surgical outcomes of patients with hypertrophic obstructive cardiomyopathy and explore the risk factors for mortality, especially those related to atrial fibrillation. Methods: We retrospectively reviewed 150 consecutive patients with hypertrophic obstructive cardiomyopathy who underwent surgical treatment between March 2003 and December 2020. Results: Fifty (33.3%, age 53.7±16.1 years) patients underwent isolated septal myectomy (SM), 79 (52.7%, age 52.3±12.6 years) underwent SM with mitral valve intervention (SM + MVI), and 21 (14.0%, age 57.1±13.5 years) underwent SM with mitral valve replacement (SM + MVR). Overall peak left ventricular outflow tract pressure gradient at rest was significantly decreased from 91.9±43.2 to 13.3±13.0 mmHg (P<0.0001). Survival rates were 96.7%, 89.1%, and 81.5% at 30 days, 5 years, and 10 years, respectively. Patients in the SM + MVI group survived longer than those in SM + MVR or isolated SM groups (94.1% vs. 75.4% vs. 88.0%, respectively, at 5 years, P=0.05). Patients with preoperative atrial fibrillation had a worse 5-year survival rate than those without atrial fibrillation (73.4% vs. 92.8%, respectively, P<0.001). Preoperative atrial fibrillation was an independent risk factor for late mortality in multivariable analysis. Notably, those whose atrial fibrillation was successfully eradicated by surgical ablation had a better 5-year survival rate than other patients (87.7% vs. 28.6%, respectively, P<0.001). Conclusions: Surgical outcomes in hypertrophic obstructive cardiomyopathy are favorable in the long-term, except in patients with preoperative atrial fibrillation. Therefore, intraoperative ablation for preoperative atrial fibrillation in hypertrophic obstructive cardiomyopathy should be actively considered to improve patient outcomes.

Targeting Alcohol Septal Ablation in Patients with Obstructive Hypertrophic Cardiomyopathy Candidates for Surgical Myectomy: Added Value of Three-Dimensional Intracoronary Myocardial Contrast Echocardiography.

BACKGROUND: Myocardial contrast two-dimensional echocardiography (MC-2DE) is widely used to address alcohol septal ablation (ASA) in obstructive hypertrophic cardiomyopathy (HCM). Owing to its limited cut-planes, MC-2DE may inaccurately identify the contrast misplacement associated with an unsuccessful or complicated ASA outcome. OBJECTIVE: The aim of this study was to assess the added value of myocardial contrast three-dimensional echocardiography (MC-3DE) compared with MC-2DE to identify the appropriate matching between the target septal zone (TSZ) and coronary artery branch for safe and long-term effective ASA in HCM patients. METHODS: A consecutive series of 52 symptomatic obstructive HCM patients referred for isolated surgical myectomy (SM) was analyzed with MC-2DE and MC-3DE following injection of echocontrast into one or more septal branches. MC-2DE and MC-3DE patterns were categorized according to complete (Type 1) or incomplete (Type 2) TSZ covering, high-risk (Type 3) exceeding TSZ, or life-threatening outside TSZ distribution (Type 4). RESULTS: MC-2DE per patient analysis showed a Type 1 pattern in 32 patients and Types 2-4 in the remaining 20 patients; subsequent MC-3DE analysis provided a re-phenotyping of MC-2DE findings in 22 of the 52 patients (42%), showing a high-risk Type 2 pattern in 17 of the 32 patients with Type 1, and a new life-threatening Type 4 in three patients with Type 2, respectively. All patients with MC-3DE Type 1 pattern underwent safe and effective ASA with a long-term uneventful follow-up, while the remaining patients underwent SM. CONCLUSIONS: Refining high risk or life-threatening contrast misplacement, MC-3DE is more accurate than conventional MC-2DE to target safe and long-term effective septal reduction with ASA in obstructive HCM patients referred for isolated SM.

Myectomy versus alcohol septal ablation in patients with hypertrophic obstructive cardiomyopathy.

OBJECTIVES: There is very little evidence comparing the safety and efficacy of alcohol septal ablation versus septal myectomy for a septal reduction in patients with hypertrophic obstructive cardiomyopathy. This study aimed to compare the immediate and long-term outcomes of these procedures. METHODS: Following propensity score matching, we retrospectively analysed outcomes in 105 patients who underwent myectomy and 105 who underwent septal ablation between 2011 and 2017 at 2 reference centres. RESULTS: The mean age was 51.9 ± 14.3 and 52.2 ± 14.3 years in the myectomy and ablation groups, respectively (P = 0.855), and postoperative left ventricular outflow tract gradients were 13 (10-19) mmHg vs 16 (12-26) mmHg; P = 0.025. The 1-year prevalence of the New York Heart Association class III-IV was higher in the ablation group (none vs 6.4%; P = 0.041). The 5-year overall survival rate [96.8% (86.3-99.3) after myectomy and 93.5% (85.9-97.1) after ablation; P = 0.103] and cumulative incidence of sudden cardiac death [0% and 1.9% (0.5-7.5), respectively P = 0.797] did not differ between the groups. The cumulative reoperation rate within 5 years was lower after myectomy than after ablation [2.0% (0.5-7.6) vs 14.6% (8.6-24.1); P = 0.003]. Ablation was associated with a higher reoperation risk (subdistributional hazard ratio = 5.9; 95% confidence interval 1.3-26.3, P = 0.020). At follow-up, left ventricular outflow tract gradient [16 (11-20) vs 23 (15-59) mmHg; P < 0.001] and prevalence of 2+ mitral regurgitation (1.1% vs 10.6%; P = 0.016) were lower after myectomy than after ablation. CONCLUSIONS: Both procedures improved functional capacity; however, myectomy better-resolved classes III-IV of heart failure. Septal ablation was associated with higher reoperation rates. Myectomy demonstrated benefits in gradient relief and mitral regurgitation elimination. The results suggest that decreasing rates of myectomy procedures need to be investigated and reconsidered.

Transapical septal myectomy in the beating heart via a minimally invasive approach: a feasibility study in swine.

OBJECTIVES: The aim of this study was to establish an original transapical septal myectomy procedure that can be performed in the beating heart via a minimally invasive approach for the treatment of hypertrophic obstructive cardiomyopathy. METHODS: We designed an original intracardiac septum resection device to conduct off-pump septal myectomy in swine. A subxiphoid minithoracotomy was performed to access the apex of the heart. This resection device was inserted into the left ventricular outflow tract of the heart via the apex. The basal anteroseptal myocardium beneath the right aortic cusp was identified using a combination of transoesophageal and transthoracic echocardiography and then resected and collected by the device. RESULTS: Six consecutive operations were successfully and accurately performed using the custom-made device under echocardiographic guidance. All pigs survived and appeared to be normal until planned euthanasia 1 week after operation. A 300-700 mg portion of the septal myocardium was resected from the normal swine heart. Echocardiography and electrocardiogram revealed no abnormalities after resection. One exception was the fifth pig, in which mild annular regurgitation of the aortic valve occurred after repetitive resection. Postmortem necropsy demonstrated that all resections were correctly located at the basal anteroseptal septum beneath the right aortic cusp. CONCLUSIONS: Our study provides the first proof-of-concept evidence for a novel beating heart transapical septal myectomy procedure, which showed promising translational potential for the treatment of hypertrophic obstructive cardiomyopathy. This procedure would probably reduce operative risks and improve outcomes and reduce the demanding expertise required to perform conventional surgical myectomy.

From 100 to 0: Endocardial Radiofrequency Ablation of Septal Hypertrophy for Management of Hypertrophic Cardiomyopathy.

Septal reduction techniques can reduce morbidity and mortality in patients with hypertrophic cardiomyopathy. In a patient with hypertrophic cardiomyopathy who was a poor candidate for surgical myectomy and alcohol septal ablation, endocardial radiofrequency ablation of septal hypertrophy provided durable reduction in left ventricular outflow tract gradients and symptomatic improvement. (Level of Difficulty: Intermediate.).

2020 AHA/ACC Guideline for the Diagnosis and Treatment of Patients With Hypertrophic Cardiomyopathy: Executive Summary: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines.

AIM: This executive summary of the hypertrophic cardiomyopathy clinical practice guideline provides recommendations and algorithms for clinicians to diagnose and manage hypertrophic cardiomyopathy in adult and pediatric patients as well as supporting documentation to encourage their use. METHODS: A comprehensive literature search was conducted from January 1, 2010, to April 30, 2020, encompassing studies, reviews, and other evidence conducted on human subjects that were published in English from PubMed, EMBASE, the Cochrane Collaboration, Agency for Healthcare Research and Quality reports, and other relevant databases. STRUCTURE: Many recommendations from the earlier hypertrophic cardiomyopathy guidelines have been updated with new evidence or a better understanding of earlier evidence. This summary operationalizes the recommendations from the full guideline and presents a combination of diagnostic work-up, genetic and family screening, risk stratification approaches, lifestyle modifications, surgical and catheter interventions, and medications that constitute components of guideline directed medical therapy. For both guideline-directed medical therapy and other recommended drug treatment regimens, the reader is advised to follow dosing, contraindications and drug-drug interactions based on product insert materials.

Guideline-Based Referral for Septal Reduction Therapy in Obstructive Hypertrophic Cardiomyopathy Is Associated With Excellent Clinical Outcomes.

BACKGROUND: The outcome of medically refractory patients with obstructive hypertrophic cardiomyopathy treated according to the American College of Cardiology/American Heart Association consensus guideline recommendations is not known. The objectives of this study were to define the short- and long-term outcomes of medically refractory obstructive hypertrophic cardiomyopathy patients undergoing alcohol septal ablation (ASA) and surgical septal myectomy (SM) with patient management in accordance with these consensus guidelines, as well as to quantify procedural risk and burden of comorbid conditions at the time of treatment. METHODS AND RESULTS: Patients with obstructive hypertrophic cardiomyopathy referred for either ASA or SM from 2004 to 2015 were followed for the primary end point of short- and long-term mortality and compared with respective age- and sex-matched US populations. Of 477 consecutive severely symptomatic patients, 99 underwent ASA and 378 SM. Compared with SM, ASA patients were older ( P<0.001), had a higher burden of comorbid conditions ( P<0.01), and significantly higher predicted surgical mortality ( P<0.005). Procedure-related mortality was 0.3% and similarly low in both groups (0% in ASA and 0.8% in SM). Over 4.0±2.9 years of follow-up, 95% of patients had substantial improvement in heart failure symptoms to New York Heart Association class I/II (96% in SM and 90% in ASA). Long-term mortality was similar between the 2 groups with no difference compared with age- and sex-matched US populations. CONCLUSIONS: Guideline-based referral for ASA and SM leads to excellent outcomes with low procedural mortality, excellent long-term survival, and improvement in symptoms. These outcomes occur in ASA patients despite being an older cohort with significantly more comorbidities.

Impact of Sex on Clinical Course and Survival in the Contemporary Treatment Era for Hypertrophic Cardiomyopathy.

Background The relation of sex to clinical presentation and course in hypertrophic cardiomyopathy (HCM) remains incompletely resolved. We assessed differences in clinical outcomes between men and women within our large HCM cohort. Methods and Results Of 2123 consecutive patients, a minority (38%) were women who were diagnosed with HCM at older ages or referred for subspecialty evaluation later than men (50±19 versus 44±16 and 55±18 versus 49±16; P<0.001). Women more commonly developed advanced New York Heart Association class III/IV symptoms (53% versus 35% in men; P<0.001), predominantly secondary to outflow obstruction. While end-stage heart failure with systolic dysfunction (ejection fraction <50%) was similar in men (5% versus 4% in women; P=0.33), women were 3-fold more likely to develop heart failure with preserved systolic function (7.5% versus 2.6%; P=0.002). Sudden death events terminated by defibrillator therapy were similar in women (0.9%/year) versus men (1.0%/year; hazard ratio, 0.92; 95% CI, 0.6-1.5; P=0.73). HCM mortality was uncommon, with identical rates in both sexes (0.3%/year; hazard ratio, 1.5; 95% CI, 0.7-3.4;, P=0.25). Age-adjusted all-cause mortality also did not differ between women and men (1.7% versus 1.3%/year; hazard ratio, 1.32; 95% CI, 0.92-1.91; P=0.13). Conclusions Survival was not less favorable in women with HCM. Contemporary treatments including surgical myectomy to reverse heart failure and defibrillators to prevent sudden death, were effective in both sexes contributing to low mortality. However, despite more frequent outflow obstruction, women with HCM are underrecognized and referred to centers later than men, often with more advanced heart failure. Greater awareness of HCM in women should lead to earlier diagnosis and treatment, with implications for improved quality of life.