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Objectives: Hypertrophic cardiomyopathy with left ventricular apical aneurysm is a phenotype associated with a 4-fold increase in the risk for sudden cardiac death. In this study, we describe the surgical outcome of concomitant apical aneurysm repair in patients undergoing transapical myectomy for hypertrophic cardiomyopathy., Methods: We identified 67 patients with left ventricular apical aneurysms who underwent transapical myectomy and apical aneurysm repair between July 2000 and August 2020. Long-term survival was compared with that of 2746 consecutive patients undergoing transaortic septal myectomy for obstructive hypertrophic cardiomyopathy with subaortic obstruction., Results: Transapical myectomy was indicated for midventricular obstruction (n = 44) or left ventricular remodeling for diastolic heart failure (n = 29). Preoperatively, 74.6% (n = 50) of patients were in New York Heart Association class III/IV heart failure, and 34.3% (n = 23) of patients had experienced syncope or presyncope. Atrial fibrillation was documented in 22 patients (32.8%), and episodes of ventricular arrhythmias were recorded in 30 patients (44.8%). Thrombus was present in the apical aneurysm in 6 patients. During a median (interquartile range) follow-up of 4.9 (1.8-7.6) years, the estimated 1- and 5-year survivals were 98.5% and 94.5%, respectively, which were not significantly different from that of patients undergoing transaortic septal myectomy for obstructive hypertrophic cardiomyopathy (P = .52) or an age- and sex-matched US general population (P = .40)., Conclusions: Apical aneurysm repair in conjunction with septal myectomy is a safe procedure, and the good long-term survival of patients suggests that the procedure may reduce cardiac-related death in this high-risk hypertrophic cardiomyopathy population., (Copyright © 2023 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Objectives: Patients with hypertrophic cardiomyopathy often have concomitant pulmonary hypertension, which has a negative prognostic effect in patients undergoing myectomy. Our objective was to investigate the effect of myectomy on pulmonary artery pressure obtained via Swan-Ganz catheter and characterize how changes in pulmonary artery systolic pressure may indicate outcomes in these patients., Methods: We performed a single-center retrospective analysis of 271 patients with recordings of intraoperative pulmonary artery pressures during surgical myectomy. We analyzed primary composite outcomes as 30-day or in-hospital major cardiopulmonary adverse events., Results: There was a 5.17% adverse event rate. Patients with adverse events were older, were more likely to be female, had chronic obstructive pulmonary disease, and had longer cardiopulmonary bypass times. Some 35.7% of those with adverse events had moderate to severe pulmonary hypertension (pulmonary artery systolic pressure ≥50 mm Hg) on postbypass stress test, compared with 4.3% of those without adverse events (P < .001). Further, 21.4% of patients with adverse events had pulmonary artery systolic pressure 50 mm Hg or greater at the end of surgery, compared with 1.9% of patients without adverse events (P < .001). The pulmonary artery systolic pressure decrease after surgery in those without adverse events was on average 5 mm Hg more than in those with adverse events., Conclusions: Postoperative pulmonary hypertension was associated with a higher rate of adverse cardiopulmonary events. This may influence the decision to use Swan-Ganz catheters in patients undergoing septal myectomy in monitoring pulmonary artery pressures to better risk stratify and manage these patients postoperatively., (Copyright © 2022. Published by Elsevier Inc.)

Objectives: Pulmonary hypertension (PH) is an independent predictor of all-cause mortality among patients with obstructive and nonobstructive hypertrophic cardiomyopathy (HCM). However, there is little information on the influence of coexisting PH on long-term survival following septal myectomy. This study investigates the prevalence of PH among patients with obstructive HCM undergoing septal myectomy and analyzes patient survival and the course of PH after operation., Methods: We included 1342 patients with obstructive HCM who had Doppler echocardiographic estimates of the right ventricular systolic pressure (RVSP) before and after transaortic septal myectomy. PH was defined as RVSP ≥35 mm Hg, with ≥50 mm Hg categorized as moderate-to-severe PH. A multivariable Cox proportional hazards model was used to identify characteristics associated with survival, and longitudinal trends in RVSP were modeled with generalized least squares analysis., Results: Patients underwent operations from 1989 to 2019. The median age was 57.9 years (interquartile range, 47.4-66.7 years); 49.5% were women. Preoperatively, PH was present in 47.8% of patients, and 14.4% had moderate-to-severe PH. Higher preoperative RVSP was independently associated with overall mortality in the multivariable Cox model. Among patients with moderate to severe preoperative RVSP elevation, postoperative RVSP decreased from baseline by a median of 12 mm Hg., Conclusions: Preoperative PH is independently associated with late mortality following septal myectomy, and the magnitude of preoperative RVSP was associated with a postoperative decrease in pulmonary pressure. The influence of PH on late postoperative survival may influence the timing of operation in patients who are candidates for septal myectomy., (Copyright © 2022 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

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/m 2 ; 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., (Copyright © 2022 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Introduction: To investigate cardiovascular characteristics and progressions of hypertrophic cardiomyopathy (HCM) and pulmonary stenosis (PS) and determine whether any genotype-phenotype correlations exist in patients with gene-confirmed RASopathy syndrome., Study Design: Eighty patients (male, 55%) confirmed as having RASopathy syndrome by genetic testing at a single tertiary center were enrolled. Subjects' medical and echocardiography records were reviewed and the changes in the z scores of left ventricular wall thickness (LVWT) and the degree of PS over time were examined during follow-up of 5.7 ± 3.1 and 7.5 ± 5.2 years, respectively., Results: The most common RASopathy gene identified was PTPN11 (56%), followed by RAF1 (10%). Eighty-five percent of patients had cardiovascular diseases, wherein 42% had HCM, and 38% PS. Mean maximal LVWT z score on the initial echocardiography (mean age 5.0 ± 6.0 years) was 3.4 ± 1.3 (median 2.8, range 2.1-6.6) in the HCM group. Overall, the maximal LVWT increased with time, especially in the HCM group (z = 3.4 ± 1.3 to 3.7 ± 1.6, P = .008) and RAF1-variant group (z = 3.7 ± 1.7 to 4.6 ± 1.8, P = .031). Five patients newly developed HCM during the study period. Genotype-phenotype correlation was significant for HCM (P = .002); 31% of patients with PTPN11 and 88% with RAF1 variants had HCM. PS did not progress in this study cohort., Conclusions: In this study, progression of ventricular hypertrophy was seen in a significant number of patients with genotype correlation. Thus, long-term follow up of cardiovascular problems in patients with RASopathy is necessary., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Background: Septal myectomy is the gold standard treatment for refractory hypertrophic obstructive cardiomyopathy. The present study characterized the association of septal myectomy volume and cardiac surgery volume with outcomes after septal myectomy., Methods: Adults undergoing septal myectomy for hypertrophic obstructive cardiomyopathy were identified in the 2016 to 2019 Nationwide Readmissions Database. Centers were grouped into low-, medium-, and high-volume hospitals by tertiles based on institutional septal myectomy caseload. Overall cardiac surgery volume was similarly assessed. Generalized linear models were used to test the association between hospital septal myectomy or cardiac surgery volume and in-hospital mortality, mitral valve repair, and 90-day non-elective readmission., Results: Of 3,337 patients, 30.8% underwent septal myectomy at high-volume hospitals, whereas 39.1% were managed at low-volume hospitals. Patients at high-volume hospitals had a similar burden of comorbidities at low-volume hospitals, although congestive heart failure was more prevalent at high-volume hospitals. Despite comparable rates of mitral regurgitation, patients more commonly avoided mitral valve intervention at high-volume hospitals compared with low-volume hospitals (72.9% vs 68.3%; P = .007). After risk adjustment, high-volume hospital status was associated with reduced odds of mortality (0.24; 95% CI, 0.08-0.77) and readmission (0.59; 95% CI, 0.3-0.97). Among cases requiring mitral intervention, high-volume hospital status was associated with greater odds of valve repair (5.33; 95% CI, 2.54-11.13) relative to low-volume hospitals. Overall cardiac surgery volume was not associated with any studied outcome., Conclusion: Greater septal myectomy volume, but not overall cardiac surgery volume, was associated with reduced mortality and greater mitral valve repair relative to replacement after septal myectomy. These findings suggest that septal myectomy for hypertrophic obstructive cardiomyopathy should be performed at centers with expertise in this operation., (Copyright © 2023 The Author(s). Published by Elsevier Inc. All rights reserved.)

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., (Copyright © 2021 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

The most prevalent cause of mortality in children with hypertrophic cardiomyopathy (HCM) is sudden cardiac death (SCD), which happens more frequently than in adult patients. Risk stratification tactics have generally been drawn from adult practice, however emerging data has revealed significant disparities between children and adult cohorts, implying the need for pediatric-specific risk stratification methodologies. We conducted an all-language literature search on Medline, Cochrane, Embase, and Google Scholar until October 2021. The following search strings and Medical Subject Heading (MeSH) terms were used: "HCM," "SCD," "Sudden Cardiac Death," and "Childhood Onset HCM." We explored the literature on the risk of SCD in HCM for its epidemiology, pathophysiology, the role of various genes and their influence, associated complications leading to SCD and preventive and treatment modalities. Childhood-onset HCM is linked to significant life-long morbidity and mortality, including a higher SCD rate in children than in adults. The present focus is on symptom relief and avoiding illness-related consequences, but the prospect of future disease-modifying medicines offers an intriguing opportunity to alter disease expression and outcomes in these young individuals. Current preventive recommendations promote implantable cardioverter defibrillator placement based on cumulative risk factor thresholds, although they have been demonstrated to have weak discriminating capacity. This article addresses questions and discusses the etiology, risk factors, and method to risk stratification for SCD in children with HCM., Competing Interests: Conflicts of Interest & Source of Funding In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Introduction: Inducible laryngeal obstruction is a common and challenging cause of exertional dyspnea. We report a case of an unanticipated cardiac condition that presented with symptoms suggestive of inducible laryngeal obstruction., Discussion: A 55-year-old man was evaluated for progressive exertional dyspnea and throat tightness, unexplained after multiple medical evaluations. Resting laryngeal examination was suspicious for laryngopharyngeal reflux and mild vocal fold adduction during quiet expiration. Given progressive and refractory symptoms, maximal cardiopulmonary exercise testing with intermittent laryngeal examination was performed. This study excluded laryngeal causes of exercise limitation and led to an unexpected diagnosis of persistent atrial flutter and hypertrophic cardiomyopathy., Conclusion: Cardiopulmonary exercise testing with laryngeal examination can identify unexpected and life-threatening mimics of inducible laryngeal obstruction that may be missed by unmonitored exercise challenges. Suspicion for inducible laryngeal obstruction at rest may not predict the true nature of exercise limitation on cardiopulmonary exercise testing., (Copyright © 2020 The Voice Foundation. All rights reserved.)

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., (Copyright © 2021 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

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 &lt;30 mm Hg, provoked gradient &gt;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 &lt; .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 &lt; .001). There was no difference in early (&lt;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., (Copyright © 2020 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Background: Hypertrophic cardiomyopathy (HCM) is a common and clinically heterogeneous inherited cardiac disease. Quality of life (QOL) and physical functioning are important clinically but are underexplored in diverse populations with HCM., Objectives: To examine predictors for and compare QOL and physical functioning in Black and White adults with HCM., Methods: We analyzed a sub-sample from a longitudinal prospective study on HCM. Eligibility criteria included self-identified Black and White adults (≥18 years) with clinical HCM. QOL was measured with the Minnesota Living with Heart Failure Questionnaire (MLWHF);physical functioning included age-adjusted exercise capacity and NYHA class. Covariates included HCM structural characteristics and common comorbidities. We analyzed data from 434 individuals, 57 (13.1%) of whom self-identified as Black/African American., Results: In this sample, the Black cohort had higher MLWHF scores, 31.2 (27.2) v. 23.9 (22.1), p=0.042, signifying worse QOL, but there were no intergroup differences when QOL was dichotomized. Mean metabolic equivalents (METs) on symptom-limited stress testing were similar, though the Black cohort was younger, 54.6 (13.4) v.62.5 (14.8) years, p=0.001. No one from the Black cohort achieved an "excellent-for-age" exercise capacity, and 64.1% had a "below-average-for-age" exercise capacity vs 47% in the White cohort, though this was not statistically significant, p=0.058. There was no difference between groups in advanced NYHA class. Female gender was associated with worse QOL and physical functioning irrespective of covariates., Conclusions: This study is a starting point that underscores the need for a more comprehensive examination of well-being and physical functioning in Black populations with HCM., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Background: Studies on risk factors of sudden cardiac death (CD) or CD in children with hypertrophic cardiomyopathy (HCM) are lacking., Objectives: To assess factors associated with the risk of sudden CD or CD in HCM children., Methods: Pubmed, Embase, Cochrane Library, and Web of Science databases were searched., Results: The results indicated that children with previous adverse cardiac events during childhood and with a history of syncope had an increased risk of sudden CD or CD. Non-sustained ventricular tachycardia (VT) in HCM children was associated with sudden CD or CD. Children with left ventricular hypertrophy (LVH) were at higher risk of sudden CD or CD. And left ventricular outflow tract (LVOT) obstruction was a potential risk factor for sudden CD in children with HCM (all P<0.05)., Conclusion: Optimal care and appropriate monitoring is necessary for HCM children with higher risk of sudden CD or CD., Competing Interests: Declaration of Competing Interest None., (Copyright © 2021 The Authors. Published by Elsevier Inc. All rights reserved.)

The prevalence of takotsubo cardiomyopathy (TCM) has been on the rise, but co-occurrence with hypertrophic cardiomyopathy (HOCM) remains rare. Although presenting patient demographics were similar to those in TCM, the potential for hemodynamic compromise was significantly compounded by the presence of underlying HOCM. Management was similar to standalone TCM, although use of inotropic agents and mechanical support appears to be more prevalent. Despite the increased potential for complications and the paucity of data regarding management, outcomes appear to be mostly favorable in both the hospitalization period and at follow-up. Interestingly, despite a new diagnosis of HOCM in about half the cases described, which signifies no significant left ventricular outflow tract (LVOT) gradient prior to TCM, half of those patients had a persistently elevated LVOT gradient after resolution of TCM. This poses a question of whether or not TCM can predispose to LVOT obstruction in HOCM patients even after its resolution., Competing Interests: Declaration of Competing Interest None., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Objective: There has been debate on the importance and pathophysiologic effects of the dynamic subaortic pressure gradient in hypertrophic obstructive cardiomyopathy. The study was conducted to elucidate the hemodynamic abnormalities associated with the dynamic pressure gradient in hypertrophic obstructive cardiomyopathy., Methods: Eight patients with hypertrophic obstructive cardiomyopathy and 7 patients with valvular aortic stenosis underwent a detailed hemodynamic study of pressure flow relationships before and after myectomy or aortic valve replacement during operation., Results: In aortic stenosis, the increased gradient after premature ventricular contraction was associated with an increase in peak flow (325 ± 122 mL/s to 428 ± 147 mL/s, P = .002) and stroke volume (75.0 ± 27.3 mL to 88.0 ± 24.0 mL, P = .004), but in hypertrophic obstructive cardiomyopathy peak flow remained unchanged (289 ± 79 mL/s to 299 ± 85 mL/s, P = .334) and stroke volume decreased (45.9 ± 18.7 mL to 38.4 ± 14.4 mL, P = .04) on the postpremature ventricular contraction beat. After myectomy, the capacity to augment stroke volume on the postpremature ventricular contraction beats was restored in patients with hypertrophic obstructive cardiomyopathy (45.6 ± 14.4 mL to 54.4 ± 11.8 mL, P = .002)., Conclusions: The pressure flow relationship in hypertrophic obstructive cardiomyopathy supports the concept of true obstruction to outflow, with a low but continued flow during late systole, when the ventricular-aortic pressure gradient is the highest. Septal myectomy can abolish obstruction and restore the ability to augment stroke volume, which may explain the mechanism of symptomatic improvement after operation., (Copyright © 2019 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Background: It is unknown whether sex-specific differences in mortality observed in HCM are due to older age of women at presentation, or whether women have greater degree of LV myopathy than men., Methods: We retrospectively compared clinical/imaging characteristics and outcomes between women and men in our overall cohort composed of 728 HCM patients, and in an age-matched subgroup comprised of 400 age-matched patients. We examined sex-specific differences in LV myopathy, and dissected the influence of age and sex on outcomes. LV myopathy was assessed by measuring LV mass, LVEF, global peak longitudinal systolic strain (LV-GLS), diastolic function (E/A, E/e'), late gadolinium enhancement (LV-LGE) and myocardial blood flow (MBF) at rest/stress. The primary endpoint was a composite outcome, comprising heart failure (HF), atrial fibrillation (AFib), ventricular tachycardia/fibrillation (VT/VF) and death; individual outcomes were defined as the secondary endpoint., Results: Women in the overall cohort were older by 6 years. Women were more symptomatic and more likely to have obstructive HCM. Women had smaller LV cavity size, stroke volume and LV mass, higher indexed maximum wall thickness (IMWT), more hyperdynamic LVEF and higher/similar LV-GLS. Women had similar LV-LGE and E/A, but higher E/e' and rest/stress MBF. Female sex was independently associated with the composite outcome in the overall cohort, and with HF in the overall cohort and age-matched subgroup after adjusting for obstructive HCM, LA diameter, LV-GLS., Conclusions: Our results suggest that sex-specific differences in LV geometry, hyper-contractility and diastolic function, not greater degree of LV myopathy, contribute to a higher, age-independent risk of diastolic HF in women with HCM., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: Thrill-seeking activities are a favorite pastime for people of all ages. Patients with hypertrophic cardiomyopathy (HCM) are often barred from participation on the basis of danger for arrhythmias. Our aim was to collect information regarding the safety of thrill-seeking activities for HCM patients., Methods: An anonymous online survey invited adult HCM patients to report participation in 11 activities (rollercoaster riding, jet skiing, rafting, bungee jumping, rappelling, paragliding, kayaking/canoeing, motor racing, snowboarding, BASE jumping and skydiving) before and after HCM diagnosis, along with major (ICD shock, syncope) or minor (nausea, dizziness, palpitations, chest pain) adverse events related to participation, and relevant physician advice., Results: Six hundred forty-seven HCM patients completed the survey, with 571 (88.2%) reporting participation in ≥1 TSAs (participant age 50.85 ± 14.21, 56.6% female, 8143 post-diagnosis participations). At time of survey, 457 participants (70.6%) were ICD-carriers or had ≥1 risk factor for sudden cardiac death. Nine (1.5%) participants reported a major event during or immediately after (60 minutes) of surveyed activity. Minor adverse events were reported by 181 participants (31.6%). In addition, 8 participants reported a major adverse event >60 minutes later but within the same day. Regarding physician advice, of the 213 responders (32.9%) receiving specific advice, 56 (26.2%) were told safety data is absent with no definitive recommendation, while 24 (11.2%) and 93 (43.6%) were told TSAs were respectively safe or dangerous., Conclusions: In this cohort, participation in thrill-seeking activities rarely caused major adverse events. This information can be used for shared-decision making between providers and patients., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Traumatic spinal cord injuries are significant contributors to the morbidity and mortality burden of trauma patients worldwide, and consume significant resources in both their acute and rehabilitative care. Another cause of mortality and morbidity is hypertrophic obstructive cardiomyopathy, which can cause syncope or sudden cardiac death in patients with no known prior cardiac disease. This case report describes a unique combination of these two high-risk pathologies in a scene trauma patient, and provides an overview of the pathophysiology and treatment of these high-risk disease processes., (Copyright © 2019 Air Medical Journal Associates. Published by Elsevier Inc. All rights reserved.)

Hypertrophic cardiomyopathy (HCM) is an extremely heterogeneous genetic disease that affects the left ventricle (LV) and has a varied clinical course and phenotypic expression. Here, we report a case of two sisters with HCM who developed a massive refractory left atrial appendage (LAA) thrombus and recurrent embolism. The older sister, who was at a high surgical risk due to progressive LV systolic dysfunction with an ejection fraction of 19%, underwent LAA plication in combination with implantation of an LV assist device after progression to treatment-refractory heart failure at the age of 49. The younger sister underwent surgical thrombectomy, LAA plication, and Maze surgery before deterioration of heart failure at the age of 47. She was free from embolism and atrial fibrillation for 2years after surgery. Individualized therapeutic approaches targeting the LAA at a relatively early stage are required in the subgroups of HCM patients with left atrial dysfunction., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Objectives: Residual midventricular obstruction after transaortic myectomy may lead to recurrent symptoms and reoperation in patients with hypertrophic cardiomyopathy and long-segment septal hypertrophy. A combined transaortic and transapical approach to septal myectomy during initial operation allows for the complete relief of subaortic and midventricular gradients and may reduce the risk of poor late functional results., Methods: We analyzed the early outcomes of 86 patients aged 18 years or more who underwent combined transaortic and transapical septal myectomy for left ventricular outflow tract obstruction due to systolic anterior motion and midventricular obstruction or cavitary obliteration due to apical hypertrophic cardiomyopathy., Results: Midventricular obstruction was present in 59 patients (68.6%); 12 patients (14.0%) had cavitary obliteration, and 15 patients (17.4%) had a combination of both. Overall, median (25th, 75th percentile) prebypass and postbypass directly measured intracavitary gradients were 85 mm Hg (48, 125) and 4 mm Hg (0, 10.8), respectively; median predischarge transthoracic left ventricular outflow tract and midventricular gradients were 0 mm Hg (0, 0) and 0 mm Hg (0, 8.5). Median crossclamp and perfusion times were 35 minutes (27, 44) and 48.5 minutes (40, 64). The 30-day and 1-year survivals were both 95%, with 2 early deaths. Functional status beyond 30 days postoperatively was available in 42 patients, and 41 patients reported improvement in symptoms and were in New York Heart Association class I/II., Conclusions: Combined transaortic and transapical septal myectomy is an effective and reasonably safe approach for patients with hypertrophic cardiomyopathy with complex septal hypertrophy. This method may prevent residual midventricular obstruction and permits myectomy to augment diastolic filling and improve left ventricular stroke volume in patients with apical hypertrophic cardiomyopathy., (Copyright © 2017. Published by Elsevier Inc.)

Objective: The appearance of atrial fibrillation is associated with significant clinical deterioration in patients with obstructive hypertrophic cardiomyopathy; therefore, maintenance of sinus rhythm is desirable. Guidelines and most articles have reported the results of catheter ablation and pharmacologic atrial fibrillation treatment; nevertheless, data regarding concomitant procedures during septal myectomy are limited. The aim of this study was to assess the outcomes of concomitant atrial fibrillation treatment in patients with obstructive hypertrophic cardiomyopathy., Methods: Between 2010 and 2013 in our clinic, 187 patients with obstructive hypertrophic cardiomyopathy underwent extended myectomy. In 45 cases, concomitant Cox-Maze IV procedure was performed; however, obstructive hypertrophic cardiomyopathy was the primary indication for surgery. Atrial fibrillation was paroxysmal in 26 patients (58%) and nonparoxysmal in 19 patients (42%). The mean age of patients was 52.8 ± 14.2 years (range, 22-74 years). Mean peak gradient was 90.7 ± 24.2 mm Hg, and interventricular septum thickness was 26.1 ± 4.3 mm. Mean atrial fibrillation duration was 17.3 ± 8.5 months., Results: There were no early deaths. No procedure-related complications occurred with regard to ablation procedure. Complete atrioventricular block was achieved in 2 patients (4.0%). Mean crossclamping time was 61 ± 36 minutes. Peak left ventricular outflow tract gradient was 12.6 ± 5.5 mm Hg based on transesophageal echocardiography. The Maze IV procedure was used for ablation in all patients (radiofrequency ablation with bipolar clamp + cryolesion for mitral and tricuspid lines). Because of the atrial wall thickness (5-6 mm), applications were performed 8 to 10 times on each line. There were no cases of pacemaker implantation due to sinus node dysfunction. All patients were discharged in stable sinus rhythm. Mean follow-up was 23.7 ± 1.3 months. The rate of atrial fibrillation freedom was 100% (45 patients) at 6 months, 89% (40 patients) at 1 year, and 78% (35 patients) at 24 months., Conclusions: Concomitant ablation atrial fibrillation during septal myectomy in patients with obstructive hypertrophic cardiomyopathy is a safe and effective procedure and should be considered carefully in this patient group., (Copyright © 2017 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Background: Late gadolinium enhancement (LGE) on cardiac MRI indicates the myocardial fibrosis in hypertrophic cardiomyopathy (HCM), and the prognostic value of LGE in HCM has been described in several studies, but controversy exists given the limited power of these studies to predict future adverse cardiac events. The objective of this study was to perform a meta-analysis to systematically evaluate the predictive value of LGE on cardiac magnetic resonance (CMR) for future adverse cardiac events., Methods: We systematically searched multiple database including PubMed, EMBASE, and Cochrane Library for cohort studies of the effects of LGE on clinical outcomes (sudden cardiac death (SCD)/aborted SCD, all cardiac death, and all-cause mortality) in patients with HCM. We performed a meta-analysis to determine pooled odds ratios (OR), weighted average annualized event rates, and summary receiver-operating characteristic (SROC) curves for these clinical events., Results: We identified nine clinical studies, examining 1734 patients with LGE and 2036 without LGE, and an average follow-up of 2.9 years. The weighted average annualized event rates of SCD/aborted SCD in patients with HCM (positive LGE versus negative LGE) was 1.28% versus 0.32% (p < 0.001), and the pooled OR was 3.40 (95% CI: 1.90, 6.08; p < 0.001). The sensitivity and specificity of predicting future cardiac events were 0.83 (95% CI: 0.66, 0.93) and 0.45 (95% CI: 0.31, 0.59), respectively. The 5-year risk of SCD/aborted SCD was 6.4% in patients with LGE. The all cardiac death and all-cause mortality were also significantly increased in patients with LGE. However, the extent of LGE was not significantly related to the risk of SCD/aborted SCD., Conclusions: LGE is significantly associated with SCD/aborted SCD risk, all cardiac death and all-cause mortality in patients with HCM. Implantable cardioverter defibrillators (ICD) can be considered for those patients with LGE., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Objective: Static 3-dimensional printing is used for operative planning in cases that involve difficult anatomy. An interactive 3D print allowing deliberate surgical practice would represent an advance., Methods: Two patients with hypertrophic cardiomyopathy had 3-dimensional prints constructed preoperatively. Stereolithography files were generated by segmentation of chest computed tomographic scans. Prints were made with hydrogel material, yielding tissue-like models that can be surgically manipulated. Septal myectomy of the print was performed preoperatively in the simulation laboratory. Volumetric measures of print and patient resected specimens were compared. An assessment tool was developed and used to rate the utility of this process. Clinical and echocardiographic data were reviewed., Results: There was congruence between volumes of print and patient resection specimens (patient 1, 3.5 cm 3 and 3.0 cm 3 , respectively; patient 2, 4.0 cm 3 and 4.0 cm 3 , respectively). The prints were rated useful (3.5 and 3.6 on a 5-point Likert scale) for preoperative visualization, planning, and practice. Intraoperative echocardiographic assessment showed adequate relief of left ventricular outflow tract obstruction (patient 1, 80 mm Hg to 18 mm Hg; patient 2, 96 mm Hg to 9 mm Hg). Both patients reported symptomatic improvement (New York Heart Association functional class III to class I)., Conclusions: Three-dimensional printing of interactive hypertrophic cardiomyopathy heart models allows for patient-specific preoperative simulation. Resection volume relationships were congruous on both specimens and suggest evidence of construct validity. This model also holds educational promise for simulation of a low-volume, high-risk operation that is traditionally difficult to teach., (Copyright © 2016 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Objectives: To identify the clinical presentation of children and adolescents affected by 1 of 4 cardiac conditions predisposing to sudden cardiac arrest: hypertrophic cardiomyopathy, long QT syndrome (LQTS), catecholaminergic polymorphic ventricular tachycardia (CPVT), and anomalous origin of the left coronary artery from the right sinus of Valsalva (ALCA-R)., Study Design: This was a retrospective review of newly diagnosed pediatric patients with hypertrophic cardiomyopathy, LQTS, CPVT, and ALCA-R referred for cardiac evaluation at 6 US centers from 2008 to 2014., Results: A total of 450 patients (257 male/193 female; median age 10.1 years [3.6-13.8 years, 25th-75th percentiles]) were enrolled. Patient age was ≤13 years for 70.4% of the cohort (n = 317). Sudden cardiac arrest was the initial presentation in 7%; others were referred on the basis of abnormal or suspicious family history, personal symptoms, or physical findings. Patients with LQTS and hypertrophic cardiomyopathy were referred most commonly because of family history concerns. ALCA-R was most likely to have abnormal signs or symptoms (eg, exercise chest pain, syncope, or sudden cardiac arrest). Patients with CPVT had a high incidence of syncope and the greatest incidence of sudden cardiac arrest (45%); 77% exhibited exercise syncope or sudden cardiac arrest. This study demonstrated that suspicious or known family history plays a role in identification of many patients ultimately affected by 1 of the 3 genetic disorders (hypertrophic cardiomyopathy, LQTS, CPVT)., Conclusion: Important patient and family history and physical examination findings may allow medical providers to identify many pediatric patients affected by 4 cardiac disorders predisposing to sudden cardiac arrest., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Objective: Systolic anterior motion of mitral valve (MV) leaflets is a main pathophysiologic feature of left ventricular outflow tract (LVOT) obstruction in hypertrophic obstructive cardiomyopathy. Thus, restricted leaflet motion that occurs with MV stenosis might be expected to minimize outflow tract obstruction related to systolic anterior motion., Methods: From January 1993 through February 2015, we performed MV replacement and septal myectomy in 12 patients with mitral stenosis and hypertrophic obstructive cardiomyopathy at Mayo Clinic Hospital in Rochester, Minn. Preoperative data, echocardiographic images, operative records, and postoperative outcomes were reviewed., Results: Mean (standard deviation) age was 70 (7.6) years. Preoperative mean (standard deviation) maximal LVOT pressure gradient was 75.0 (35.0) mm Hg; MV gradient was 13.7 (2.8) mm Hg. From echocardiographic images, 4 mechanisms of outflow tract obstruction were identified: systolic anterior motion without severe limitation in MV leaflet excursion, severe limitation in MV leaflet mobility with systolic anterior motion at the tip of the MV anterior leaflet, septal encroachment toward the LVOT, and MV displacement toward the LVOT by calcification. Mitral valve replacement and extended septal myectomy relieved outflow gradients in all patients, with no death or serious morbidity., Conclusions: Patients with mitral stenosis and hypertrophic obstructive cardiomyopathy have multiple LVOT obstruction mechanisms, and MV replacement may not be adequate treatment. We favor septal myectomy and MV replacement in this complex subset of hypertrophic obstructive cardiomyopathy., (Copyright © 2016 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Background: Patients with hypertrophic cardiomyopathy (HCM) have exercise intolerance due to left ventricular outflow tract (LVOT) obstruction, mitral regurgitation, and left ventricular dysfunction. We sought to study predictors of outcomes in HCM patients undergoing cardiopulmonary stress testing (CPT)., Methods: We studied 1,005 HCM patients (50 ± 14 years, 64% men, 77% on β-blockers) who underwent CPT with echocardiography. Clinical, echocardiographic, and exercise variables (peak oxygen consumption [VO2] and heart rate recovery [HRR] at first minute postexercise) were recorded. End point was a composite of death, appropriate defibrillator discharges, resuscitated sudden death, stroke, and heart failure admission., Results: Mean left ventricular ejection fraction (LVEF), postexercise LVOT gradient, and peak VO2 were 62% ± 6%, 92 ± 51 mm Hg, and 21 ± 6 mL kg(-1) min(-1), respectively. Despite 789 patients (78%) being in New York Heart Association classes I to II, only 8% achieved >100% age-gender predicted peak VO2, whereas 77% and 15% achieved 50% to 100% and <50%, respectively. Left ventricular outflow tract gradient ≥30 mm Hg was observed in 83% patients, whereas 23% had abnormal HRR. More than 5.5 ± 4 years, there were 94 (9%) events; 511 (50%) patients underwent surgery for LVOT obstruction. Multivariable Cox proportional analysis demonstrated % age-gender predicted peak VO2 (hazard ratio [HR] 0.96 [0.93-0.98]), normal vs abnormal HRR (HR 0.48 [0.32-0.73]), higher LVEF (HR 0.96 [0.93-0.98]), surgery (0.53 [0.33-0.83]), and atrial fibrillation (HR 1.65 [1.04-2.60]) were associated with outcomes (all P < .05)., Conclusions: In HCM patients undergoing CPT, a higher % of achieved age-gender predicted VO2 and surgical relief of LVOT obstruction were associated with better outcomes, whereas abnormal HRR, atrial fibrillation, and lower LVEF were associated with worse outcomes., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Objective: The aims of the present study were to identify the mechanisms of residual or recurrent left ventricular outflow tract obstruction in patients undergoing repeat septal myectomy for hypertrophic cardiomyopathy and to assess the early and late results of reoperation., Methods: From January 1980 to June 2012, we performed 52 repeat myectomies in 51 patients. We reviewed the medical records and preoperative transthoracic echocardiograms to evaluate the adequacy of the previous resection and mechanism of left ventricular outflow tract obstruction. The complications of previous and repeat myectomy, New York Heart Association class, and survival were analyzed., Results: The mean interval from previous myectomy to reoperation was 43 ± 51 months. In 6 patients (12%) residual or recurrent gradients were caused by isolated midventricular obstruction. In the remaining 46 operations, the mechanism of residual or recurrent gradients was identified as systolic anterior motion of mitral valve-related subaortic obstruction caused by inadequate length of previous subaortic septal excision in 31 patients (59% of the total), both an inadequate length and an inadequate depth of septectomy in 13 patients (25%), and both residual subaortic obstruction due to systolic anterior motion of the mitral valve and midventricular obstruction in 2 patients (4%). Preoperatively, 96% of patients were in New York Heart Association class III or IV; postoperatively, 93.8% were in class I or II (P < .001). The 10-year survival after reoperation was 98% and similar to that of an age- and gender-matched Minnesota population (P = .46)., Conclusions: The most common cause of recurrent left ventricular outflow tract obstruction and symptoms in patients undergoing septal myectomy has been an inadequate length of septal excision. Reoperation is safe, with excellent long-term survival and functional improvement., (Copyright © 2014 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Objective: Patients with hypertrophic obstructive cardiomyopathy due to diffuse hypertrophy extending to or below the papillary muscles are poor candidates for alcohol septal ablation and suboptimal candidates for transaortic septal myectomy. In addition, the outflow obstruction is often aggravated by an abnormal mitral valve and subvalvular apparatus., Methods: We performed transatrial myectomy in 12 patients with diffuse hypertrophy, who were highly symptomatic despite maximal medical therapy. All had at least moderate mitral regurgitation and systolic anterior motion. The anterior mitral leaflet (AML) was detached from commissure to commissure, allowing an easy myectomy through this AML toward the base of the anterior papillary muscle, with mobility fully restored. The abnormal chordae from the septum to the anterior papillary muscle and AML were divided. The continuity of this AML was restored with augmentation using an autologous pericardial patch. The height of the posterior mitral leaflet was reduced and the repair completed using an oversized annuloplasty ring., Results: The peak intraventricular gradients decreased spectacularly from 98.8 ± 6.29 to 19.2 ± 13.4 mm Hg (P < .001), and the systolic anterior motion and mitral regurgitation disappeared. One patient died of left ventricular diastolic dysfunction. All other patients left the hospital in New York Heart Association class I or II., Conclusions: We believe that this technique is preferable for patients with hypertrophic obstructive cardiomyopathy and diffuse hypertrophy extending to the midportion of the left ventricle or beyond. It results in disappearance of outflow tract gradients and allows correction of the mitral valve abnormality., (Copyright © 2014 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Objective: Asymmetric septal hypertrophy frequently coexists with severe aortic stenosis and can be unmasked after successful aortic valve replacement (AVR), jeopardizing the clinical and echocardiographic results. The aim of our study was to investigate, at 5 years postoperatively, the effectiveness of myectomy associated with AVR on left ventricular (LV) mass regression and LV diastolic function., Methods: From 1997 and 2004, 86 patients with a diagnosis of severe aortic stenosis and asymmetric septal hypertrophy consecutively underwent AVR (group A) or AVR and concomitant myectomy (group B). To assess the improvement in LV mass and LV diastolic function, we studied the 52 survivors (23 in group A and 29 in group B) who had the same prosthesis type (beleaflet mechanical), the same size (21 mm), and the same follow-up length., Results: In group A, the LV mass index regressed from 119.2 ± 22.0 to 113.8 ± 21.8, and in group B, it regressed from 121.6 ± 20.8 to 112.7 ± 20.0 (P < .0005). In group A, the E/E' ratio improved from 15.3 ± 3.0 to 11.8 ± 3.0, and in group B, it improved from 16.2 ± 3.2 to 12.1 ± 3.3 (P = .02)., Conclusions: Surgeons should inspect the LV outflow tract at AVR. Concomitant myectomy at AVR is a safe and effective procedure that improves LV mass regression and LV diastolic function., (Copyright © 2013 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Objective: Many patients with symptomatic hypertrophic cardiomyopathy have minimal left ventricular outflow tract gradients, and there is uncertainty whether their limitation is due to diastolic dysfunction or labile outflow tract obstruction. The purpose of this study was to characterize the clinical presentation and outcome of septal myectomy in patients with hypertrophic cardiomyopathy and latent obstruction., Methods: Among 749 patients who underwent septal myectomy, 249 had latent obstruction with minimal (0-30 mm Hg) resting gradients preoperatively. All were symptomatic and had more severe left ventricular outflow tract obstruction provoked by Valsalva maneuver or amyl nitrite inhalation during Doppler echocardiography or by stimulation with isoproterenol during catheterization. Clinical characteristics, survival, and functional outcome of these patients were compared with those of 500 patients with more severe resting left ventricular outflow tract obstruction who underwent myectomy during the same period., Results: Compared with those with severe obstruction, more patients with latent obstruction were male (63% vs 52%, P < .003), but ages were similar (53 ± 14 years vs 52 ± 15 years). Preoperative symptoms and functional limitation were similar in the 2 groups with 86% and 85%, respectively, having New York Heart Association class III or IV disability. Among patients with latent obstruction, mixed venous oxygen saturation was 61.6% ± 19.0% of predicted compared with 56.8% ± 17.3% for those with severe resting obstruction (P < .008). Septal thickness was less in patients with latent obstruction (20 ± 9 mm vs 22 ± 15 mm, P < .001). Early mortality was 1% in each group, and survival at 5 and 10 years was 93% and 87%, respectively, for patients with latent obstruction compared with 93% and 74%, respectively, for patients with severe resting obstruction preoperatively (P = .34). Self-reported late functional status was similar; 3 to 5 years postoperatively, 81% of patients with latent obstruction preoperatively were in New York Heart Association class I or II compared with 77% of patients with severe resting obstruction., Conclusions: Patients with obstructive hypertrophic cardiomyopathy who have low resting gradients and latent obstruction may have limiting symptoms comparable to those of patients with more severe resting gradients. Septal myectomy should be offered to these patients because survival and symptom relief are excellent, suggesting that dynamic obstruction is the major hemodynamic problem rather than diastolic dysfunction., (Copyright © 2012 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)