Novel Oral Anticoagulants for Primary Stroke Prevention in Hypertrophic Cardiomyopathy Patients With Atrial Fibrillation.

Background and Purpose- Hypertrophic cardiomyopathy patients with atrial fibrillation are at increased risk of stroke, and anticoagulation is strongly recommended. However, limited data are available regarding the safety and effectiveness of non-vitamin K antagonist oral anticoagulants (NOACs) for primary prevention of stroke. Methods- Using the Korean Health Insurance Review and Assessment Service database, we identified 2397 patients with hypertrophic cardiomyopathy and nonvalvular atrial fibrillation on oral anticoagulation from 2013 to 2016 without history of ischemic stroke, intracranial hemorrhage (ICH), or gastrointestinal bleeding (992 on warfarin and 1405 on NOACs). Inverse probability of treatment weighting with propensity scores was used to balance covariates between treatment groups. Risk for ischemic stroke, ICH, gastrointestinal bleeding, death, and their composite outcome associated with NOAC use was assessed with warfarin use as the reference. Results- During a mean follow-up of 1.6 years, the incidence rates of ischemic stroke, ICH, gastrointestinal bleeding, death, and composite outcome were all significantly lower in the NOAC than in the warfarin group (stroke, 2.8 versus 5.0; ICH, 0.5 versus 1.3; gastrointestinal bleeding, 2.3 versus 3.0; death, 3.0 versus 5.1; composite, 7.5 versus 12.5 events per 100 person-years). NOACs were associated with significantly lower risks of ischemic stroke (hazard ratio [HR], 0.47; 95% CI, 0.32-0.68), ICH (HR, 0.31; 95% CI, 0.14-0.69), gastrointestinal bleeding (HR, 0.62; 95% CI, 0.40-0.96), death (HR, 0.45; 95% CI, 0.31-0.65), and the composite outcome (HR, 0.48; 95% CI, 0.38-0.61) than warfarin. The same trend was observed regardless of the NOAC dose and across various high-risk subgroups. In analysis of individual NOACs, all NOACs were associated with lower risks of ischemic stroke and composite outcome. Conclusions- NOACs showed superior effectiveness and safety versus warfarin in the primary prevention of stroke versus warfarin in real-world Asian hypertrophic cardiomyopathy with atrial fibrillation.

Improvement in gastrointestinal bleeding after septal myectomy for hypertrophic cardiomyopathy.

OBJECTIVE: Patients with obstructive hypertrophic cardiomyopathy may have occult gastrointestinal bleeding. In this study, we analyzed outcomes of septal myectomy in patients who had a history of gastrointestinal bleeding preoperatively to understand patient characteristics and impact of septal reduction on recurrent gastrointestinal bleeding. METHODS: We analyzed 73 adult patients who had a history of gastrointestinal bleeding before transaortic septal myectomy for obstructive hypertrophic cardiomyopathy and compared outcomes to 219 patients without gastrointestinal bleeding preoperatively. RESULTS: Patients with preoperative history of gastrointestinal bleeding were older (median (IQR) age, 65 (59-69) years, P < .001) and were more likely to have systemic hypertension (70% vs 53%, P = .020) and coronary artery disease (25% vs 13%, P = .026). Preoperatively, patients with gastrointestinal bleeding had a larger left atrial volume index (median, 53 mL/m2; interquartile range, 42-67; P = .006) and greater right ventricular systolic pressure (median, 36 mm Hg; interquartile range, 32-49; mm Hg, P = .005) but no significant difference in severity of outflow tract obstruction (P = .368). There were no perioperative deaths. The estimated 5- and 10-year survivals were 96.6% and 81.8%, respectively. At a median of 3.4 (interquartile range, 1.9-9.1) years after septal myectomy, 11 patients (15%) had recurrence of gastrointestinal bleeding, which was attributed to angiodysplasia or unknown causes in 6 patients (8%). CONCLUSIONS: Patients with a preoperative history of gastrointestinal bleeding have favorable short- and long-term outcomes after septal myectomy for obstructive hypertrophic cardiomyopathy. Remission of gastrointestinal bleeding was observed in 85% of patients postprocedure, and only 8% of the patients had recurrent gastrointestinal bleeding due to angiodysplasia or unknown causes.

Impact of Race and Ethnicity on Use and Outcomes of Septal Reduction Therapies for Obstructive Hypertrophic Cardiomyopathy.

Background Information on impact of race and ethnicity on use and early outcomes of septal reduction therapies (SRTs) for obstructive hypertrophic obstructive cardiomyopathy are limited. Methods and Results Using the National Inpatient Sample from January 2012 through December 2019, we identified all adult admissions with a primary diagnosis of obstructive hypertrophic cardiomyopathy and those undergoing SRT. Predictors of receiving SRT and outcomes including in-hospital mortality, complications, and resource use were evaluated in racial and ethnic groups. Among a total of 18 895 adult admissions with obstructive hypertrophic cardiomyopathy, SRT was performed in 7255 (38.4%) admissions. Septal myectomy was performed in 4930 (26.1%), while alcohol septal ablation was performed in 2325 (12.3%). In adjusted analysis, Black patient (versus White patient adjusted odds ratio, 0.65 [95% CI, 0.57-0.73]; P<0.001) and Hispanic patient admissions (versus White adjusted odds ratio, 0.78 [95% CI, 0.66-0.92]; P=0.003) were less likely to receive SRT. Among admissions undergoing SRT, in-hospital mortality was significantly higher for Hispanic (adjusted odds ratio, 3.38 [95% CI, 1.81-6.30], P<0.001) and other racial and ethnic groups (adjusted odds ratio 2.02 [95% CI, 1.00-4.11]; P=0.05) compared with White patient admissions, whereas Black patient admissions had comparable mortality. Black, Hispanic, and other ethnic group patients had higher rates of SRT complications and more frequent dismissals to acute care facilities. Conclusions Among obstructive hypertrophic cardiomyopathy hospitalizations, minoritized racial groups were less likely to receive SRT. Importantly, hospitalizations of Hispanic and other ethnic patients undergoing SRT had significantly higher in-hospital mortality and complication rates. Further studies with granular echocardiographic information to assess indications for SRT are needed to better understand these differences.

Preoperative left ventricular longitudinal strain predicts outcome of septal myectomy for obstructive hypertrophic cardiomyopathy.

OBJECTIVE: The objective of this study was to determine the characteristics of longitudinal strain and its effect on outcomes in patients with obstructive hypertrophic cardiomyopathy (HCM) who underwent septal myectomy. METHODS: We reviewed patients with obstructive HCM who underwent septal myectomy at our clinic from 2007 to 2016. Data of those who had strain echocardiography within 6 months before isolated myectomy were analyzed. RESULTS: The median age of the 857 patients studied was 55 (interquartile range [IQR], 44-63) years, and 451 (52.6%) were male. Left ventricular ejection fraction was 71% (IQR, 67%-74%), and the resting peak outflow tract gradient was 58 (IQR, 27-85) mm Hg. The median global longitudinal strain (GLS) was -14.6% (IQR, -12.0% to -17.3%). Regional longitudinal strain was nonuniform as reflected by more normal values in apical segments and more abnormal in basal segments. Moreover, GLS correlated poorly with ejection fraction and outflow tract gradient. In 64 patients who had postoperative strain echocardiography, GLS was comparable before and after septal myectomy, but regional strain was more uniform after myectomy. Over a follow-up of 8.3 (IQR, 6.5-10.3) years, when patients were equally stratified according to GLS (cutoff, -14.64%), the group with worse GLS had significantly poorer survival compared with the better GLS group (P = .002). Left ventricular ejection fraction had no association with survival. CONCLUSIONS: Left ventricular longitudinal strain is nonuniform and might be significantly reduced in patients with obstructive HCM. Septal myectomy does not impair GLS but is associated with more uniform regional strains. Most importantly, reduced GLS preoperatively is strongly and independently associated with increased all-cause mortality after septal myectomy for obstructive HCM.

Is anterior mitral valve leaflet length important in outcome of septal myectomy for obstructive hypertrophic cardiomyopathy?

OBJECTIVES: Elongation of mitral valve leaflets is a phenotypic feature of hypertrophic cardiomyopathy, and some surgeons advocate plication of the anterior leaflet at the time of septal myectomy. The present study investigates mitral valve leaflet length and outcomes of patients undergoing septal myectomy for obstructive hypertrophic cardiomyopathy. METHODS: We reviewed the records and echocardiograms of 564 patients who underwent transaortic septal myectomy for obstructive hypertrophic cardiomyopathy between February 2015 and April 2018. Extended septal myectomy without plication of the anterior leaflet was the standard procedure. From intraoperative prebypass transesophageal echocardiograms, we measured anterior and posterior mitral valve leaflets and their coaptation length. For comparison, we performed these mitral valve leaflet measurements in 90 patients who underwent isolated coronary artery bypass grafting and 92 patients undergoing aortic valve replacement in the same period. Among patients with hypertrophic cardiomyopathy undergoing septal myectomy, we assessed left ventricular outflow tract gradient relief and 1-year survival in relation to leaflet length. RESULTS: Median patient age (interquartile range) was 60.3 (50.2-67.7) years, and 54.1% were male. Concomitant mitral valve repair was performed in 36 patients (6.4%), and mitral valve replacement was performed in 8 patients (1.4%), primarily for intrinsic mitral valve disease. Patients in the hypertrophic cardiomyopathy cohort had significantly longer mitral valve leaflet measurements compared with patients undergoing coronary artery bypass grafting or aortic valve replacement (P < .001 for all 3 measurements). Preoperative resting left ventricular outflow tract gradients were not related to leaflet length (<30 mm, median 49 [21, 81.5] mm Hg vs ≥30 mm, 50.5 [21, 77] mm Hg; P = .76). Further, gradient reduction after myectomy was not related to leaflet length; patients with less than 30 mm anterior leaflet length had a median gradient reduction of 33 (69, 6) mm Hg compared with 36.5 (62, 6) mm Hg for patients with leaflet length 30 mm or more (P = .36). Anterior mitral valve leaflet length was not associated with increased 1-year mortality (P = .758). CONCLUSIONS: Our study confirms previous findings that patients with hypertrophic cardiomyopathy have slight (5 mm) elongation of mitral valve leaflets. In contrast to other reports, increased anterior mitral valve leaflet length was not associated with higher left ventricular outflow tract gradients. Importantly, we found no significant relationship between anterior mitral valve leaflet length and postoperative left ventricular outflow tract resting gradients or gradient relief. Thus, in the absence of intrinsic mitral valve disease, transaortic septal myectomy with focus on extending the excision beyond the point of septal contact is sufficient for almost all patients.

Surgical Management of Hypertrophic Cardiomyopathy Complicated by Infective Endocarditis.

BACKGROUND: Infective endocarditis is a serious complication in hypertrophic cardiomyopathy. Cardiac surgery is often necessary, however, literature assessing surgical outcomes is limited. METHODS: From December 1995 to September 2018, 43 patients with a history of hypertrophic cardiomyopathy and native valve infective endocarditis underwent cardiac surgery at our institution. Relevant data were abstracted from medical records and analyzed. RESULTS: Median age was 57 years (interquartile range, 45 to 67); 81% (n = 35) were male. Infective endocarditis was active in 21% of patients (n = 9) at the time of surgery; of these, the suspected origin of infection was orodental in 19% (n = 8). Significant mitral valve regurgitation was detected in 54% of patients (n = 23), and aortic valve regurgitation in 7% (n = 3). Septal myectomy was performed in 95% of patients (n = 41), with concomitant valve surgery in 58% (n = 25), including prosthetic replacement in 28% (n = 12). Two patients underwent double valve replacement without septal myectomy. Outflow gradients improved from a median 67 mm Hg (interquartile range, 34 to 97 mm Hg) to 9 mm Hg (interquartile range, 6 to 22 mm Hg). One inhospital death occurred because of uncontrollable pulmonary edema. As of last follow-up, 7 patients required reoperation, and the 5-year and 10-year cumulative incidences were 11% and 26%, respectively. Ten deaths occurred; overall survival probability at 5 and 10 years was 94% and 78%, respectively. CONCLUSIONS: Valvular complications of infective endocarditis add complexity to surgical management of hypertrophic cardiomyopathy. There is an increased need for concomitant valve repairs, prosthetic replacements, and reoperation. These data underscore the need for recognition of infection, especially after oral procedures, which preceded the majority of the active infective endocarditis cases.

Transapical Ventricular Remodeling for Hypertrophic Cardiomyopathy With Systolic Cavity Obliteration.

BACKGROUND: Some patients with hypertrophic cardiomyopathy (HCM) present with reduced left ventricular (LV) stroke volume and elongated systolic cavity obliteration due to symmetric LV hypertrophy. In this report, we detail our experience with transapical septal myectomy to enlarge the LV volume and to relieve cavity obliteration in this unique subgroup of patients with HCM. METHODS: We analyzed 38 patients with HCM who had extended symmetric LV hypertrophy and underwent transapical septal myectomy to enlarge the LV cavity from February 2001 to May 2021. RESULTS: At the time of evaluation for operation, 84.2% (n = 32) of the patients were in New York Heart Association class III/IV. The peak oxygen consumption was 51.5% (44.0%-58.0%) of the normal predicted values on the preoperative exercise stress test (n = 16). Preoperative left atrial sizes in this cohort were enlarged (left atrial volume index, 39.0 [33.5-51.5] mL/m2), despite only 4 patients with moderate or greater mitral valve regurgitation. All patients underwent transapical septal myectomy to enlarge the LV cavity size. There was no postoperative (within 30 days) death. During a median (interquartile range) follow-up of 3.4 (0.7-6.9) years, the estimated survival rates were 100%, 92%, and 87% at 1, 3, and 5 years, respectively. Follow-up surveys suggested that 16 of the 17 contacted patients experienced improvement in their heart function after the procedure. CONCLUSIONS: Transapical myectomy to enlarge LV cavity volume can be performed safely with good early survival and functional results. This procedure is an important alternative to cardiac transplantation for HCM patients with systolic cavity obliteration and progressive heart failure.

Posterior Wall Thickness Associates With Survival Following Septal Myectomy for Obstructive Hypertrophic Cardiomyopathy.

BACKGROUND: The left ventricular (LV) posterior wall thickness (PWT) is a predictor of sudden cardiac death in pediatric patients with hypertrophic cardiomyopathy (HCM), but the prognostic importance of PWT in adults has not been examined. OBJECTIVES: The goal of this study was to evaluate the association of LV PWT with late survival in adult patients undergoing septal myectomy for obstructive HCM. METHODS: This single-center study reviewed 2,418 patients who underwent transaortic septal myectomy for obstructive HCM. RESULTS: The median preoperative PWT was 13 (IQR: 11-15) mm. Patients with PWT >13 mm tended to have systemic hypertension (55.4% vs 49.1%; P = 0.002) and a larger body mass index (median: 30.8 [IQR: 27.1-35.1] kg/m2 vs 29.6 [IQR: 26.1-33.9] kg/m2; P < 0.001). Preoperatively, PWT >13 mm was associated with increased septal thickness (median: 21 [IQR: 18-24] mm vs 19 [IQR: 17-22] mm; P < 0.001), greater maximum instantaneous left ventricular outflow tract (LVOT) gradient at rest (median: 67 [IQR: 36-96] mm Hg vs 47 [IQR: 19-79] mm Hg), and increased likelihood of moderate or greater mitral valve regurgitation (54.3% vs 47.3%; P = 0.001). However, PWT was not related to the severity of limitations measured by New York Heart Association functional class (P = 0.674). After adjusting for baseline covariates, greater PWT was an independent risk factor for late mortality after septal myectomy (P = 0.003). CONCLUSIONS: PWT is a newly identified predictor of reduced long-term survival after septal myectomy that is independent of septal thickness and severity of LVOT gradient. Future studies are warranted to investigate the mechanisms underlying the association and the potential usefulness of PWT in patient management.

Latent outflow tract obstruction in hypertrophic cardiomyopathy: Clinical characteristics and outcomes of septal myectomy.

OBJECTIVE: This investigation analyzed clinical characteristics of patients with hypertrophic cardiomyopathy (HCM) and latent left ventricular outflow tract (LVOT) and outcomes following septal myectomy. METHODS: We reviewed patients with HCM and LVOT obstruction undergoing septal myectomy from 2001 to 2016 at our center. Follow-up data on functional status were obtained through mailed survey questionnaires. RESULTS: There were 629 (31.8%) patients with latent obstruction (resting LVOT gradient <30 mm Hg, provoked gradient >30 mm Hg) among 1981 patients undergoing septal myectomy. Patients with latent obstruction were more likely to be male (65.7% vs 51.8%, P < .001), but there were no important differences in other clinical characteristics. The New York Heart Association functional classes and measured/predicted maximal oxygen consumption (62 [51, 72] vs 60 [48, 72], P = .158) in cardiopulmonary exercise tests were comparable between the 2 groups. Patients with latent obstruction had both lower septal thickness and lower posterior wall thickness. Median intraoperative provoked pressure gradient decreased from 96 (68, 126) mm Hg to 0 (0, 6) mm Hg after myectomy (P < .001). There was no difference in early (<30 days) deaths (3/629 vs 5/1352, P = .726) and long-term survival between patients with latent obstruction and resting obstruction. In follow-up, both general health status and New York Heart Association functional class were significantly improved following septal myectomy. CONCLUSIONS: Patients with HCM and latent LVOT obstruction generally have milder left ventricular hypertrophy but similarly impaired functional capacity compared to those with resting obstruction. Septal myectomy improves functional capacity and symptoms.

Development and implementation of a nurse-based remote patient monitoring program for ambulatory disease management.

Introduction: Numerous factors are intersecting in healthcare resulting in an increased focus on new tools and methods for managing care in patients' homes. Remote patient monitoring (RPM) is an option to provide care at home and maintain a connection between patients and providers to address ongoing medical issues. Methods: Mayo Clinic developed a nurse-led RPM program for disease and post-procedural management to improve patient experience, clinical outcomes, and reduce health care utilization by more directly engaging patients in their health care. Enrolled patients are sent a technology package that includes a digital tablet and peripheral devices for the collection of symptoms and vital signs. The data are transmitted from to a hub integrated within the electronic health record. Care team members coordinate patient needs, respond to vital sign alerts, and utilize the data to inform and provide individualized patient assessment, patient education, medication management, goal setting, and clinical care planning. Results: Since its inception, the RPM program has supported nearly 22,000 patients across 17 programs. Patients who engaged in the COVID-19 RPM program experienced a significantly lower rate of 30-day, all-cause hospitalization (13.7% vs. 18.0%, P = 0.01), prolonged hospitalization >7 days (3.5% vs. 6.7%, P = 0.001), intensive care unit (ICU) admission (2.3% vs. 4.2%, P = 0.01), and mortality (0.5% vs. 1.7%, P = 0.01) when compared with those enrolled and unengaged with the technology. Patients with chronic conditions who were monitored with RPM upon hospital discharge were significantly less likely to experience 30-day readmissions (18.2% vs. 23.7%, P = 0.03) compared with those unmonitored. Ninety-five percent of patients strongly agreed or agreed they were likely to recommend RPM to a friend or family member. Conclusions: The Mayo Clinic RPM program has generated positive clinical outcomes and is satisfying for patients. As technology advances, there are greater opportunities to enhance this clinical care model and it should be extended and expanded to support patients across a broader spectrum of needs. This report can serve as a framework for health care organizations to implement and enhance their RPM programs in addition to identifying areas for further evolution and exploration in developing RPM programs of the future.

Longitudinal Cost of Septal Myectomy Versus Alcohol Septal Ablation for Hypertrophic Cardiomyopathy.

OBJECTIVE: To compare the postprocedural health care utilization and cost of septal myectomy (SM) and alcohol septal ablation (ASA). PATIENTS AND METHODS: Using the OptumLabs Data Warehouse, we analyzed de-identified claims data of adult patients undergoing SM and ASA for obstructive hypertrophic cardiomyopathy from January 1, 2006, through December 31, 2018. We used propensity score weighting to compare the 2-year incidence rates of emergency department visits and rehospitalizations after SM and ASA. RESULTS: We identified 953 patients in total: 660 underwent SM and 293 underwent ASA. There was no difference in the risk (odds ratio, 1.1; 95% CI, 0.6 to 1.8) or frequency (incidence rate ratio, 1.1; 95% CI, 0.8 to 1.5) of emergency department visits, but the annual risk of hospital readmission was 10.8% after SM and 25.9% after ASA during the second postoperative year (P=.004). In those who were ever readmitted, the average length of hospital stay within the first 2 years after ASA was 1.6 times as long as that after SM (incidence rate ratio, 1.6; 95% CI, 1.0 to 2.4). Overall, the 2-year cumulative postprocedural cost was significantly higher after ASA (P<.001). CONCLUSION: Compared with ASA, SM is associated with fewer hospital readmissions and lower 2-year postprocedural health care cost.

Is there referral bias in outcomes of septal myectomy for hypertrophic cardiomyopathy?

PURPOSE: To determine the potential impact of referral bias on short- and long-term outcomes following septal myectomy for hypertrophic cardiomyopathy. METHODS: We reviewed 2303 adult patients who underwent transaortic septal myectomy for obstructive hypertrophic cardiomyopathy from January 1993 to April 2016. Patients were divided into 3 groups according to their permanent address: local (state) residents (n = 324), regional (surrounding 5 states) patients (n = 515), and national (outside 5 states) patients (n = 1464). RESULTS: Patient groups were similar for age, sex, preoperative New York Heart Association class, and left ventricular ejection fraction. Local patients had increased prevalence of diabetes mellitus (13%, 11%, 8%; P = .006), coronary artery disease (25%, 21%, 19%; P = .031), severe chronic lung disease (2.3%, 1.9%, 0.4%; P < .001), and atrial fibrillation (24%, 18%, 19%; P = .045) when compared with regional and national patients. Echocardiographic features did not differ between the 3 groups, including prevalence of moderate or greater mitral regurgitation (59%, 61%, 56%; P = .161). Local and regional patients were more likely to undergo concomitant procedures than national patients (P < .001). Mitral valve surgery was performed in 9.6% of the patients, more commonly in local and regional patients (12%, 12%, 8%; P = .018). There were 11 operative deaths (0.5%), and early mortality was similar among the groups. Geographic origin did not impact overall late survival. CONCLUSIONS: Compared with distant referrals, local patients who undergo septal myectomy at our institution have more comorbid conditions, and require more concomitant surgical procedures. Despite these differences, referral patterns did not impact early or late outcomes following transaortic septal myectomy.

Survival Following Alcohol Septal Ablation or Septal Myectomy for Patients With Obstructive Hypertrophic Cardiomyopathy.

BACKGROUND: There is little information regarding long-term mortality comparing the 2 most common procedures for septal reduction for obstructive hypertrophic cardiomyopathy (HCM), alcohol septal ablation (ASA), and septal myectomy. OBJECTIVES: This study sought to compare the long-term mortality of patients with obstructive HCM following septal myectomy or ASA. METHODS: We evaluated outcomes of 3,859 patients who underwent ASA or septal myectomy in 3 specialized HCM centers. All-cause mortality was the primary endpoint of the study. RESULTS: In the study cohort, 585 (15.2%) patients underwent ASA, and 3,274 (84.8%) underwent septal myectomy. Patients undergoing ASA were significantly older (median age: 63.0 years [IQR: 52.7-72.8 years] vs 53.7 years [IQR: 44.9-62.8 years]; P < 0.001) and had smaller septal thickness (19.0 mm [IQR: 17.0-22.0 mm] vs 20.0 mm [IQR: 17.0-23.0 mm]; P = 0.007). Patients undergoing ASA also had more comorbidities, including renal failure, diabetes, hypertension, and coronary artery disease. There were 4 (0.7%) early deaths in the ASA group and 9 (0.3%) in the myectomy group. Over a median follow-up of 6.4 years (IQR: 3.6-10.2 years), the 10-year all-cause mortality rate was 26.1% in the ASA group and 8.2% in the myectomy group. After adjustment for age, sex, and comorbidities, the mortality remained greater in patients having septal reduction by ASA (HR: 1.68; 95% CI: 1.29-2.19; P < 0.001). CONCLUSIONS: In patients with obstructive hypertrophic cardiomyopathy, ASA is associated with increased long-term all-cause mortality compared with septal myectomy. This impact on survival is independent of other known factors but may be influenced by unmeasured confounding patient characteristics.

Ventricular Septal Myectomy for Obstructive Hypertrophic Cardiomyopathy (Analysis Spanning 60 Years Of Practice): AJC Expert Panel.

Surgical myectomy remains the time-honored primary treatment for hypertrophic cardiomyopathy patients with drug refractory limiting symptoms due to LV outflow obstruction. Based on >50 years experience, surgery reliably reverses disabling heart failure by permanently abolishing mechanical outflow impedance and mitral regurgitation, with normalization of LV pressures and preserved systolic function. A consortium of 10 international currently active myectomy centers report about 11,000 operations, increasing significantly in number over the most recent 15 years. Performed in experienced multidisciplinary institutions, perioperative mortality for myectomy has declined to 0.6%, becoming one of the safest currently performed open-heart procedures. Extended myectomy relieves symptoms in >90% of patients by ≥ 1 NYHA functional class, returning most to normal daily activity, and also with a long-term survival benefit; concomitant Cox-Maze procedure can reduce the number of atrial fibrillation episodes. Surgery, preferably performed in high volume clinical environments, continues to flourish as a guideline-based and preferred high benefit: low treatment risk option for adults and children with drug refractory disabling symptoms from obstruction, despite prior challenges: higher operative mortality/skepticism in 1960s/1970s; dual-chamber pacing in 1990s, alcohol ablation in 2000s, and now introduction of novel negative inotropic drugs potentially useful for symptom management.

Septal Myectomy in patients with previous coronary revascularization - hypertrophic cardiomyopathy masquerading as ischemic heart disease.

BACKGROUND: Patients with obstructive hypertrophic cardiomyopathy (HCM) may have symptoms mimicking ischemic heart disease, including chest pain and shortness of breath. Some patients undergo coronary revascularization which may not lead to symptomatic improvement. This study assesses clinical presentations and outcomes of patients with previous coronary revascularization undergoing septal myectomy. METHOD: From 08/1996 to 07/2017, 166 adult patients with obstructive HCM underwent septal myectomy at our Clinic with a history of percutaneous coronary intervention (PCI, N = 153) or coronary artery bypass grafting (CABG, N = 13). We assessed their functional status before and after coronary intervention and outcomes following myectomy. RESULTS: The median (IQR) age was 65 (59-71) years, and 106 (64%) were male. Among 150 patients whose extent of disease was known, single vessel disease was identified in 109 (73%) who had PCI and 1 (9%) who had CABG. Following revascularization, many (59%) reported no improvement in shortness of breath from preoperative status. Myectomy was performed at a median of 3.2 (1.0-6.3) years following coronary revascularization, and 40 (25%) required myectomy within 1 year. Patients whose shortness of breath persisted after PCI/CABG (N = 90) underwent myectomy earlier than those whose symptoms initially improved (N = 63) after coronary revascularization (1.4 [0.6-4.0] years vs. 5.1 [2.8-9.5] years, p < .001). CONCLUSION: Almost 25% of patient's required septal myectomy within 1 year of coronary intervention for continued symptoms originally thought to be due to ischemic heart disease. These findings highlight the overlap of obstruction and ischemic symptoms and the importance of complete evaluation for dynamic obstruction in HCM.

Substrate and procedural predictors of outcomes after catheter ablation for atrial fibrillation in patients with hypertrophic cardiomyopathy.

BACKGROUND: Hypertrophic cardiomyopathy (HCM) is often accompanied by atrial fibrillation (AF) due to diastolic dysfunction, elevated left atrial pressure, and enlargement. Although catheter ablation for drug-refractory AF is an effective treatment, the efficacy in HCM remains to be established. METHODS: Thirty-three consecutive patients (25 male, age 51 +/- 11 years) with HCM underwent pulmonary vein (PV) isolation (n = 8) or wide area circumferential ablation with additional linear ablation (n = 25) for drug-refractory AF. Twelve-lead and 24-hour ambulating ECGs, echocardiograms, event monitor strips, and SF 36 quality of life (QOL) surveys were obtained before ablation and for routine follow-up. RESULTS: Twenty-one (64%) patients had paroxysmal AF and 12 (36%) had persistent/permanent AF for 6.2 +/- 5.2 years. The average ejection fraction was 0.63 +/- 0.12. The average left atrial volume index was 70 +/- 24 mL/m(2). Over a follow-up of 1.5 +/- 1.2 years, 1-year survival with AF elimination was 62%(Confidence Interval [CI]: 66-84) and with AF control was 75%(CI: 66-84). AF control was less likely in patients with a persistent/chronic AF, larger left atrial volumes, and more advanced diastolic disease. Additional linear ablation may improve outcomes in patient with severe left atrial enlargement and more advanced diastolic dysfunction. Two patients had a periprocedureal TIA, one PV stenosis, and one died after mitral valve replacement from prosthetic valve thrombosis. QOL scores improved from baseline at 3 and 12 months. CONCLUSION: Outcomes after AF ablation in patients with HCM are favorable. Diastolic dysfunction, left atrial enlargement, and AF subtype influence outcomes. Future studies of rhythm management approaches in HCM patients are required to clarify the optimal clinical approach.

Hypertrophic cardiomyopathy: present and future, with translation into contemporary cardiovascular medicine.

Hypertrophic cardiomyopathy (HCM) is a common inherited heart disease with diverse phenotypic and genetic expression, clinical presentation, and natural history. HCM has been recognized for 55 years, but recently substantial advances in diagnosis and treatment options have evolved, as well as increased recognition of the disease in clinical practice. Nevertheless, most genetically and clinically affected individuals probably remain undiagnosed, largely free from disease-related complications, although HCM may progress along 1 or more of its major disease pathways (i.e., arrhythmic sudden death risk; progressive heart failure [HF] due to dynamic left ventricular [LV] outflow obstruction or due to systolic dysfunction in the absence of obstruction; or atrial fibrillation with risk of stroke). Effective treatments are available for each adverse HCM complication, including implantable cardioverter-defibrillators (ICDs) for sudden death prevention, heart transplantation for end-stage failure, surgical myectomy (or selectively, alcohol septal ablation) to alleviate HF symptoms by abolishing outflow obstruction, and catheter-based procedures to control atrial fibrillation. These and other strategies have now resulted in a low disease-related mortality rate of <1%/year. Therefore, HCM has emerged from an era of misunderstanding, stigma, and pessimism, experiencing vast changes in its clinical profile, and acquiring an effective and diverse management armamentarium. These advances have changed its natural history, with prevention of sudden death and reversal of HF, thereby restoring quality of life with extended (if not normal) longevity for most patients, and transforming HCM into a contemporary treatable cardiovascular disease.

Transapical approach to myectomy for midventricular obstruction in hypertrophic cardiomyopathy.

BACKGROUND: Midventricular obstruction in hypertrophic cardiomyopathy (HCM) is less common than subaortic obstruction, and there are few data on outcomes after surgical treatment. METHODS: We reviewed 56 consecutive patients (28 men) with HCM and midventricular obstruction who underwent myectomy between February 1997 and June 2012. Five patients had prior myectomy for subaortic obstruction. Mean age was 42 ± 17 years. Preoperatively, 51% of patients had dyspnea, and the remaining had palpitations (25%), angina (5%), or syncope (9%). RESULTS: Midventricular obstruction was relieved by means of a transaortic myectomy in 5 patients, a transapical approach in 32 patients, and combined transaortic and transapical incisions in 19 patients. In 13 patients, an apical aneurysm or pouch was repaired at the time of midventricular myectomy. There were no early deaths. Intraoperative intraventricular gradients were reduced from 64 ± 32 mm Hg before myectomy to 6 ± 12 mm Hg postoperatively (p ≤ 0.0001). Early complications included atrial arrhythmias in 5 patients and reoperation for bleeding in 4 patients. Fifty patients had follow-up beyond 30 days (median, 1.6 years; range, 33 days to 13 years). Survival at 1 and 5 years was 100% and 95%, and average New York Heart Association class improved from 2.9 ± 0.7 preoperatively to 1.3 ± 0.6 postoperatively (p = 0.0001). There were no aneurysms related to the apical incision; 2 patients had late reoperation, 1 for resection of right atrial mass to prevent embolus. CONCLUSIONS: A transapical approach allows excellent exposure for midventricular myectomy and relief of intraventricular gradients and related symptoms. There were no complications unique to the apical incision, and 5-year survival was similar to expected survival (95% versus 97%).