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Objective: To examine the relationship among age, septal morphological subtype, and presence of hypertrophic cardiomyopathy (HCM)-associated myofilament mutations. Patients and Methods: Comprehensive mutation analysis of the 8 HCM susceptibility genes that encode the myofilaments of the cardiac sarcomere was performed previously in 382 unrelated patients with HCM. Blinded to genotype status, we used echocardiography to characterize the left ventricular morphological features. Multivariate regression was used to assess the relationship among morphological subtypes, clinical data, and genetic variables. Results: The mean +/- SD age of the patients was 41.6+/-19.0 years, with 126 patients 50 years or older at initial diagnosis. The septal morphological subtype was sigmold in 181 (47%), reverse in 132 (35%), apical variant in 37 (10%), and neutral in 32 (8%). The HCM-associated myofilament mutations were Identified in 143 patients (37%). Multivariate analysis showed that the reverse curvature septal morphological subtype was a strong predictor of genotype-positive status (odds ratio, 21; P<.001). Overall, the yield of HCM genetic testing was 79% in the setting of reverse curvature HCM but only 8% in sigmold septal HCM. Conclusion: In stark contrast to HCM in young patients, elderly patients with HCM display a predominantly sigmoid septal morphological subtype and uncommonly have perturbations of known HCM susceptibility genes. Independent of age, septal morphological subtype strongly predicts the presence or absence of HCM-associated myofilament mutations and may enable echocardiography-guided genetic testing for HCM. [ABSTRACT FROM AUTHOR]

OBJECTIVE: To determine the clinical parameters of hypertrophic cardiomyopathy (HCM) that correlated significantly with the presence of an identifiable sarcomeric mutation. PATIENTS AND METHODS: Previous comprehensive mutational analyses of all protein-coding exons of 8 sarcomeric genes revealed pathogenic mutations in 147 (38%) of 389 unrelated patients seen at the HCM outpatient clinic at the Mayo Clinic in Rochester, Minn, between April 1997 and December 2001. Clinical data, extracted from patient records and blinded to patient genotype, were maintained in a custom database. RESULTS: In 389 unrelated patients, younger age at diagnosis, family history of HCM, and Increasing left ventricular wall thickness were all associated with Increased likelihood of identifying an HCM-associated sarcomeric mutation. In contrast, family history of sudden cardiac death, myectomy status, and anatomical subtype did not correlate significantly with genotype-positive status. With use of a simple scoring system based on age at diagnosis, left ventricular wall thickness, and family history of HCM, the likelihood of a sarcomeric mutation could be estimated. CONCLUSION: Clinical predictors of positive genotype, such as the presence of an implantable cardioverter-defibrillator, age at diagnosis, degree of left ventricular wall hypertrophy, and family history of HCM, may aid in patient selection for genetic testing and increase the yield of cardiac sarcomere gene screening. [ABSTRACT FROM AUTHOR]

OBJECTIVE: To pool results from studies of patients with hypertrophic cardiomyopathy (HCM) to elucidate important phenotypic differences among genotypes. MATERIAL AND METHODS: Data published from November 1998 through November 2004 were gathered and compared from unrelated study population genotyping studies from the Mayo Clinic (Rochester, Minn), Harvard Medical School (Boston, Mass), France, Germany, Sweden, Finland, and Spain. Standard statistical analysis techniques were used to pool and compare data across genotypes with respect to frequency of mutations, age at diagnosis, and degree of hypertrophy (left ventricular wall thickness). RESULTS: The French study population harbored the highest frequency of mutations (61%), followed by the Mayo Clinic (38%), Harvard Medical School (36%), and Swedish (30%) study populations. For every study population, mutations in myosin binding protein C (MYBPC3) were the most common cause of HCM. Patients with a family history of HCM had mutations more frequently than those without. This pooled analysis revealed no statistically significant differences in left ventricular wall thickness or in mean age at diagnosis across all genotypes. CONCLUSIONS: Differentiation of sarcomeric genotypes, such as MYBPC3-HCM and MYH7-HCM, is not possible on the basis of currently reported phenotypic data. A myriad of genetic and/or environmental modifiers in addition to the primary disease-causing genetic substrate must play an important role in determining a patient's particular phenotype. [ABSTRACT FROM AUTHOR]

Objectives: This investigation examined the risk of sudden cardiac death and other mortality in adult patients with hypertrophic cardiomyopathy (HCM) who have myocardial bridging diagnosed at coronary angiography.Background: Several reports have associated myocardial bridging with an adverse prognosis in pediatric HCM patients, but the prognosis of myocardial bridging in adult patients with HCM is unknown.Methods: The coronary angiograms of 425 patients with HCM (mean age 60 +/- 15 years [range 18 to 89 years]) at the Mayo Clinic were examined for the presence of myocardial bridging. Clinical follow-up was conducted to assess mortality. Survival of patients with bridging was compared with HCM patients who also underwent angiography but who did not have evidence of bridging.Results: A total of 64 patients (15%) had myocardial bridging. The mean follow-up for the entire study was 6.8 +/- 5.4 years. There was no difference in survival free of all-cause mortality (5-year estimate, bridging vs. no bridging, 91% vs. 85%; p = 0.42), all cardiac death (93% vs. 89%; p = 0.60), and sudden cardiac death (95% vs. 97%; p = 0.72). Univariate and multivariate proportional hazards models also did not identify the presence of bridging or specific characteristics of the degree or extent of bridging with a poor outcome.Conclusions: This study observed no increased risk of death, including sudden cardiac death, among adult patients with HCM who had myocardial bridging diagnosed at coronary angiography. [ABSTRACT FROM AUTHOR]

A 29-year-old man with history of antiphospholipid antibody syndrome (APS) and two prior episodes of acute rheumatic fever developed a third episode of acute rheumatic fever. This was complicated by acute myocardial infarction due to spontaneous arterial thrombosis of the left anterior descending and right coronary arteries. We postulate that rheumatic pancarditis created an inflammatory, thrombogenic environment that facilitated coronary artery thrombosis secondary to APS, a novel association. Our patient was left with significant left ventricular dysfunction at a young age, and cases such as this emphasize the need for early recognition and proper treatment of APS. Further, the pathogenesis of thrombosis in APS is incompletely understood, and the cellular and molecular basis for this, including the role of 'second hits', are areas needing further investigation. [ABSTRACT FROM AUTHOR]

Objective: To characterize the prognostic implications of exercise echocardiography in patients who have reduced exercise capacity at the time of testing. Patients and Methods: We examined the outcomes of 941 patients at the Mayo Clinic in Rochester, Minn, between January 1, 1990, and December 31, 1995, who had reduced exercise capacity on exercise echocardiography (women, <5 metabolic equivalents; men, <7 metabolic equivalents) and evaluated the potential association between clinical, electrocardiographic, and echocardiographic variables and outcomes for patients with normal vs abnormal exercise echocardiograms. We used variables of independent prognostic value to estimate cardiac risk. Results: For patients with normal exercise echocardiograms (n=282), the rate of cardiac death or nonfatal myocardial infarction was 09% per person-year of follow-up, and previous coronary revascularization was the only predictor of the time to cardiac event. For patients with abnormal exercise echocardiograms (n=659), the cardiac event rate was 4.4%. Independent predictors of outcome were exercise left ventricular (LV) ejection fraction (risk ratio, 1.44 per 10% decrement; 95% confidence interval, 1.2-1.7; P<.001) and an increase or no change in LV end-systolic size in response to exercise (risk ratio, 2.22; 95% confidence interval, 1.2-4.1; P=.01). Conclusion: Exercise echocardiographic findings have important prognostic implications for patients who have reduced exercise capacity on testing. Echocardiographic descriptors of LV systolic function and dysfunction obtained immediately after exercise can be used to stratify cardiac risk of patients who do not achieve a level of exercise ordinarily considered to be of "diagnostic" value. [ABSTRACT FROM AUTHOR]

Distinguishing the pathologic hypertrophy of hypertrophic cardiomyopathy (HC) from the physiologic hypertrophy of professional football players (PFP) can be challenging when septal wall thickness falls within a "gray zone" between 12 and 16 mm. It was hypothesized that 2-dimensional and speckle-tracking strain (ε) echocardiography could differentiate the hearts of PFPs from those of patients with HC with similar wall thicknesses. Sixty-six subjects, including 28 professional American football players and 21 patients with HC, with septal wall thicknesses of 12 to 16 mm, along with 17 normal controls, were studied using 2-dimensional echocardiography. Echocardiographic parameters, including modified relative wall thickness (RWT; septal wall thickness + posterior wall thickness/left ventricular end-diastolic diameter) and early diastolic annular tissue velocity (e'), were measured. Two-dimensional ε was analyzed by speckle tracking to measure endocardial and epicardial longitudinal ε and circumferential ε and radial cardiac ε. Septal wall thickness was higher in patients with HC than in PFPs (14.7 ± 1.1 vs 12.9 ± 0.9 mm, respectively, p <0.001), while posterior wall thickness showed no difference. RWT was larger in patients with HC than in PFPs (0.68 ± 0.10 vs 0.48 ± 0.06, p <0.001). Longitudinal endocardial ε and radial cardiac ε were significantly higher in PFPs than in patients with HC, while circumferential endocardial ε was no different. RWT was the parameter that most accurately differentiated PFPs from patients with HC. An RWT cut point of 0.6 differentiated PFPs from patients with HC, with an area under the curve of 0.97. In conclusion, a 2-dimensional echocardiographic measure of RWT (septal wall + posterior wall thickness/left ventricular end-diastolic dimension) accurately differentiated PFPs' hearts from those of patients with HC when septal wall thickness was in the gray zone of 12 to 16 mm. Two-dimensional strain analysis identifies variations in myocardial deformation between PFPs and patients with HC with gray-zone hypertrophy. [ABSTRACT FROM AUTHOR]

OBJECTIVE: To review our institution's experience with patients who failed to benefit from septal artery ablation, which necessitated subsequent septal myectomy, and to examine reasons for ablation failure and outcome of myectomy after ablation. PARTICIPANTS AND METHODS: Of 550 patients who underwent septal myectomy at Mayo Clinic Rochester between January 1, 1999, and December 31, 2006, 16 (3%) had had a total of 22 previous septal artery ablations. This subset of 16 patients was analyzed and compared with a reference group of 120 patients whose septal artery ablations were performed at our institution during this period. Angiograms obtained during septal ablation were available for 13 (81%) of 16 patients in this series and were reviewed by 2 interventional cardiologists (R.A.N. and S.R.O.). These cardiologists also reviewed preoperative and postoperative echocardiography data, hospital course, and follow-up data to compile a list of characteristics that could have contributed to failed ablation. RESULTS: The median age of the patients at operation was 65 years (interquartile range [IQR], 52-72 years), and interval between ablation and myectomy was 409 days (IQR, 162-568 days). Angiograms revealed 2 failed procedures secondary to technical error. One patient had a relatively large first septal perforator with a large resting gradient. In 10 patients no septal perforators supplying the proximal septum were identified. Postoperatively, mitral regurgitation decreased from 3.00 to 1.00 (P less than .001), and left ventricular outflow tract gradient decreased from 75 mm Hg to 0 mm Hg (IQR, 0-29 mm Hg; P less than .001). Two patients died after surgery: 1 patient developed multiple-organ system failure on postoperative day 7, and 1 patient developed arrhythmia on postoperative day 21. Patients with previous septal artery ablation were older (P=.04), were more likely to have preoperative permanent pacemakers or implantable cardioverter-defibrillators (P=.05), were more likely to require postoperative pacemaker placement (P less than .001), and had higher operative mortality (P less than .001) than control patients. Fourteen patients survived the early recovery phase; 9 were followed up at a median of 1.88 years (IQR, 306 days to 3.3 years). All patients' symptoms improved. Median gradient of the left ventricular outflow tract was 13 mm Hg (IQR, 0-15 mm Hg) at follow-up with mild to moderate (1.6) mitral regurgitation. CONCLUSION: Septal myectomy performed after failed ablation improves gradient and provides excellent relief of symptoms but is associated with a higher incidence of morbidity and mortality. [ABSTRACT FROM AUTHOR]

Objectives: The aim of this study was to assess the prognostic value of a noninvasive measure of left ventricular diastolic pressure (LVDP) early after acute myocardial infarction (MI). Background: The early diastolic velocity of the mitral valve annulus (e') reflects the rate of myocardial relaxation. When combined with measurement of the early transmitral flow velocity (E), the resultant ratio (E/e') correlates well with mean LVDP. In particular, an E/e' ratio >15 is an excellent predictor of an elevated mean LVDP. We hypothesized that an E/e' ratio >15 would predict poorer survival after acute MI. Methods: Echocardiograms were obtained in 250 unselected patients 1.6 days after admission for MI. Patients were followed for a median of 13 months. The end point was all-cause mortality. Results: Seventy-three patients (29%) had an E/e' >15. This was associated with excess mortality (log-rank statistic 21.3, p < 0.0001) and was the most powerful independent predictor of survival (risk ratio 4.8, 95% confidence interval 2.1 to 10.8, p = 0.0002). The addition of E/e' >15 improved the prognostic utility of a model containing clinical variables and conventional echocardiographic indexes of left ventricular systolic and diastolic function (p = 0.001). Conclusions: E/e' is a powerful predictor of survival after acute MI. An E/e' ratio >15 is superior, in this respect, to other clinical or echocardiographic features. Furthermore, it provides prognostic information incremental to these parameters. [ABSTRACT FROM AUTHOR]

Inflammation is increasingly recognized as having an important role in patients with acute coronary syndromes. We sought to determine whether an elevated white blood cell (WBC) count would predict subsequent mortality and whether revascularization would have a protective effect. We analyzed data from 10,480 patients with acute coronary syndromes enrolled in the PURSUIT trial who had a WBC count measured on admission. WBC count values were stratified by quartiles, and death rates at 6 months were examined in univariate and multivariate analyses. Propensity analysis was performed to assess the effect of revascularization on the relation between WBC count and mortality. In the lowest quartile of WBC count, mortality was 4.0%; it was 5.8% in the second quartile, 6.7% in the third quartile, and 8.0% in the fourth quartile (p <0.001). In a multivariable model incorporating baseline demographic and clinical variables, an increasing WBC count was a significant predictor of death, with a hazard ratio of 1.07 per 1,000/microl increment in WBC count (p <0.001). Furthermore, the interaction term between mortality due to an elevated WBC count and benefit of in-hospital revascularization was significant (hazard ratio 0.94, p = 0.032), suggesting that the excess risk due to an elevated WBC count was attenuated by revascularization. An elevated WBC count at hospital admission, although only a crude index of inflammation, nevertheless is an independent predictor of death at 6 months in patients with acute coronary syndromes. This finding supports a pivotal role for inflammation in acute coronary syndromes. Importantly, this study suggests that in-hospital revascularization may mitigate some of the excess risk due to inflammation. [ABSTRACT FROM AUTHOR]

Background: Hypertrophic cardiomyopathy (HCM) is a common genetic heart disease. Women with HCM tend to have a later onset but more severe disease course. However, the underlying pathobiological mechanisms for these differences remain unknown., Methods: Myectomy samples from 97 patients (53 males/44 females) with symptomatic obstructive HCM and 23 control cardiac tissues were included in this study. RNA-sequencing was performed on all samples. Mass spectrometry-based proteomics and phosphoproteomics was performed on a representative subset of samples., Results: The transcriptome, proteome, and phosphoproteome was similar between sexes and did not separate on PCA plotting. Overall, there were 482 differentially expressed genes (DEGs) between control females and control males while there were only 53 DEGs between HCM females and HCM males. There were 1983 DEGs between HCM females and control females compared to 1064 DEGs between HCM males and control males. Additionally, there was increased transcriptional downregulation of hypertrophy pathways in HCM females and in HCM males. HCM females had 119 differentially expressed proteins compared to control females while HCM males only had 27 compared to control males. Finally, the phosphoproteome showed females had 341 differentially phosphorylated proteins (DPPs) compared to controls while males only had 184. Interestingly, there was hypophosphorylation and inactivation of hypertrophy pathways in females but hyperphosphorylation and activation in males., Conclusion: There are subtle, but biologically relevant differences in the multi-omics profile of HCM. This study provides the most comprehensive atlas of sex-specific differences in the transcriptome, proteome, and phosphoproteome present at the time of surgical myectomy for obstructive HCM., (Copyright © 2024 Elsevier Ltd. All rights reserved.)

Introduction: We have previously reported that genetically positive patients have a more profound early decrease in provocable left ventricular outflow tract gradient compared to genetically negative patients utilizing mavacamten in the first 12 weeks of therapy., Methods and Results: In this current analysis, we found that genetically positive patients have less favorable remodeling as measured by left ventricular wall thickness regression when evaluated long-term as compared to genetically negative patients, despite an overall better early response to mavacamten. The majority of genetically positive patients were maintained on only 2.5 mg of mavacamten due to early robust response., Conclusion: We hypothesize that this lower dosing attenuated the long-term benefit of mavacamten in genetically positive patients. We believe that the long-term benefit of mavacamten on positive cardiac remodeling is dose-dependent and not solely related to the magnitude of left ventricular outflow gradient decrease., Competing Interests: Conflict of Interest Nothing to report in relation to this specific project., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Objectives: Surgical septal reduction is sometimes avoided in older adults due to anticipated high operative risk. The study objectives were to compare the clinical and echocardiographic characteristics of young and older patients undergoing septal myectomy for obstructive hypertrophic cardiomyopathy and assess differences in early and late postoperative outcomes., Methods: A total of 2663 patients with obstructive hypertrophic cardiomyopathy underwent transaortic septal myectomy between 2000 and 2021 and were categorized by age: 18 to 64 years, 65 to 74 years, and 75 years or more., Results: Median age at the time of surgery increased over the study interval. Female sex (P < .001), hypertension P < .001), and diabetes (P = .004) were more prevalent in older patients, but extent of functional limitation (New York Heart Association) was similar (P = .092). Elderly patients had thinner septal and posterior walls (P < .001, P = .006) and less prominent asymmetry (P < .001). They are less likely to have positive genetic testing. Hospital mortality was 0.2%, 0.5%, and 1.3% in patients aged less than 65 years, 65 to 74 years, and 75 years or more, respectively (P = .06), and 5-year survivals were 97%, 93%, and 91%, respectively. Septal-to-posterior wall thickness ratio significantly correlated with increased mortality in patients aged more than 65 years, but not in patients aged less than 65 years (P = .92). Most of the patients reported improved quality of life after myectomy., Conclusions: Clinical characteristics of obstructive hypertrophic cardiomyopathy in older patients differ from those in younger patients. More symmetric but less extensive ventricular hypertrophy and less positive genetic testing suggests that hypertrophic cardiomyopathy has distinct clinical and morphological variants in the elderly. Septal myectomy is safe in older patients, but the presence of left ventricular wall asymmetry portends a poorer prognosis., Competing Interests: Conflict of Interest Statement The authors reported no conflicts of interest. The Journal policy requires editors and reviewers to disclose conflicts of interest and to decline handling or reviewing manuscripts for which they may have a conflict of interest. The editors and reviewers of this article have no conflicts of interest., (Copyright © 2024 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Competing Interests: Disclosures Dr Ackerman is a consultant for Abbott, BioMarin Pharmaceuticals, Boston Scientific, Bristol Myers Squibb, Daiichi Sankyo, Illumina, Invitae, Medtronic, Solid Biosciences, Tenaya Therapeutics, and UpToDate. Dr Ackerman and Mayo Clinic have license agreements with AliveCor, Anumana, ARMGO Pharma, Pfizer, and Thryv Therapeutics. Dr Siontis is a consultant for AskBio and Bristol Myers Squibb (fees paid to institution). Dr Geske is a consultant for Cytokinetics and is the principal investigator for clinical trials funded by Bristol Myers Squibb and Cytokinetics. Dr Giudicessi is a consultant for Avidity Therapeutics and serves as a principal investigator for clinical trials sponsored by Tenaya Therapeutics. Drs Ackerman, Siontis, Geske, and Giudicessi and Mayo Clinic have an equity sharing agreement with Prolaio. However, none of these entities participated in this work. Other authors have no relevant conflicts of interest to declare.

Objective: In this study, we describe the incidence, sites of valve injury, and the influence of aortic valve regurgitation (AR) on outcomes of septal myectomy for obstructive hypertrophic cardiomyopathy., Methods: We analyzed patients who underwent transaortic septal myectomy for obstructive hypertrophic cardiomyopathy from 2001 to 2022. The primary study end point was incidence of procedure-related AR, defined as the need for an unplanned aortic valve (AV) procedure or new-onset moderate AR on early postoperative echocardiography., Results: There were 2807 patients who underwent transaortic septal myectomy for hypertrophic cardiomyopathy and had pre- and postoperative transthoracic echocardiograms. Procedure-related AR was observed in 55 (2%) patients; 27 (1%) required unplanned AV procedures at the time of myectomy, and 29 (1%) additional patients developed moderate AR postoperatively. During follow-up, 9 total patients underwent late AV reoperation, 1 patient who developed new moderate AR postoperatively required late AVR due to severe calcific AS, and none of the patients who had unplanned AV procedures required late reoperation. The overall cumulative incidence of AV reoperation at 10 and 15 years was 1% and 5%. The predicted probability of moderate or severe AR at 5 and 10 years was 1.4% and 1.8%, respectively. There was no difference in survival comparing patients with or without early postoperative mild or worse AR (P = .69)., Conclusions: Procedure-related AR was observed in 2% of patients undergoing transaortic septal myectomy, and unplanned AV procedures were necessary for half of these patients. Intraoperative identification and valve repair mitigate the impact of cusp injury on late reintervention and survival., (Copyright © 2023 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

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.)

Objective: To characterize changes in ventricular morphology in patients with hypertrophic cardiomyopathy who develop left ventricular (LV) outflow tract obstruction., Methods: We reviewed patients with hypertrophic cardiomyopathy with LV outflow tract obstruction who underwent septal myectomy from May 2012 to June 2023. Among 68 patients initially without obstruction documented up to 7.6 years (interquartile range, 6.3-9.4 years) before the operation, a comparison was made with 78 patients with nonobstructive hypertrophic cardiomyopathy over a similar period. Patients who did not develop obstruction were matched with those who did on sex, age, and maximum septal wall thickness during the initial echocardiography, identifying 41 matched pairs. Echocardiographic data, including 5 measures of angulation, were compared between the groups., Results: The median interval between echocardiographic assessments was 7.5 years (interquartile range, 6.3-8.1 years) among patients with obstruction versus 7.3 years (interquartile range, 6.2-9.0 years) in patients without nonobstruction. Patients with obstruction were more likely to have hypertension at both times. The maximum septal wall thickness increased within both groups (both P values < .001), but the magnitude of increase was not different between groups (P = .130). Patients with obstruction exhibited a greater increase in LV mass (P < .001) compared with patients without obstruction (P = .004). Aortic angulation significantly increased in 4 of the 5 measurements (all P values < .001) in patients with obstruction, whereas patients with no obstruction showed no change. Anterior and posterior mitral valve leaflet lengths and coaptation lengths remained similar in both groups over time., Conclusions: The development of LV outflow tract obstruction in patients with hypertrophic cardiomyopathy was associated with progressive LV outflow tract angulation and increased LV hypertrophy, as reflected by LV mass. Progression to obstruction was not related to changes in the mitral valve leaflet morphology., Competing Interests: Conflict of Interest Statement The authors reported no conflicts of interest. The Journal policy requires editors and reviewers to disclose conflicts of interest and to decline handling or reviewing manuscripts for which they may have a conflict of interest. The editors and reviewers of this article have no conflicts of interest., (Copyright © 2024 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Background: Patients with hypertrophic cardiomyopathy (HCM) are at increased risk of developing cardiac arrhythmias and have a high prevalence of cardiac implantable electronic device (CIED) use. Tricuspid regurgitation (TR) is a potential complication of device leads and can be severe enough to prompt surgical intervention., Methods: We identified 21 consecutive patients who underwent tricuspid valve (TV) surgery for device lead-induced TR late following septal myectomy (SM) for obstructive HCM. The primary endpoint was long-term all-cause mortality., Results: The median patient age was 63 years (range, 55-71 years), 19 patients (91%) had New York Heart Association class III or IV limitation, and all patients were receiving diuretics for right heart failure. The median interval between device implantation and TV surgery was 4 years (range, 1.5-8.5 years). Eight patients (38%) underwent pacemaker implantation due to complete heart block following SM. Preoperatively, TR was severe in 81% of the patients. The primary mechanism of lead-induced TR was leaflet impingement without adherence (n = 15; 75%). Nine patients (43%) underwent TV replacement, and 12 patients (57%) underwent repair. Only 1 patient died early postoperatively. Patients with lead-induced TR had markedly reduced long-term survival compared to the overall population of patients undergoing SM; 5-year survival was 58%, compared to 96% for the contemporary SM group., Conclusions: Late lead-induced TR is a potential complication of CIEDs in patients with HCM who have undergone SM. Although TV repair and replacement can be done with acceptable early mortality, late patient survival is poor., Competing Interests: The authors reported no conflicts of interest. The Journal policy requires editors and reviewers to disclose conflicts of interest and to decline handling or reviewing manuscripts for which they may have a conflict of interest. The editors and reviewers of this article have no conflicts of interest., (© 2024 The Author(s).)

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., (Copyright © 2024 American Heart Association, Inc., and the American College of Cardiology Foundation. Published by Elsevier. Published by Elsevier Inc. All rights reserved.)

Background: We recently demonstrated that patients with atrial fibrillation (AF) and hypertrophic cardiomyopathy (HCM) have an increased risk of left atrial (LA) thrombus. In this study, we aimed to evaluate thrombus management, thrombus persistence, and thromboembolic events for HCM and non-HCM patients with AF and LA thrombus., Methods: From a cohort of 2,155 AF patients undergoing transesophageal echocardiography (TEE) for any indication, this study included 122 patients with LA thrombus (64 HCM patients and 58 non-HCM controls)., Results: There was no difference in mean CHA2DS2-VASc scores between HCM and control patients (3.9 ± 2.2 vs 3.8 ± 2.0, p = 0.88). Ten (16%) and 4 (7%) patients in the HCM and control groups, respectively, were in sinus rhythm at the time of TEE identifying the LA thrombus (p = 0.13). In all patients, the anticoagulation strategy was modified after the LA thrombus diagnosis. A total of 36 (56%) HCM patients and 34 (59%) control patients had follow-up TEE at median 90 and 62 days, respectively, after index TEE. The HCM group had significantly higher 90-day rates of persistent LA thrombus compared to the control group (88% vs 29%; p < 0.001). In adjusted models, HCM was independently associated with LA thrombus persistence. Among patients with LA thrombus, the 5-year cumulative incidence of thromboembolic events was 11% and 2% in HCM and control groups, respectively (p = 0.22)., Conclusions: Among patients with AF with LA thrombus identified by TEE, those with HCM appear to have a higher risk of LA thrombus persistence than non-HCM patients despite anticoagulation., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Objective: To compare early and late outcomes of septal myectomy in patients with obstructive hypertrophic cardiomyopathy who presented with residual or recurrent left ventricular outflow tract (LVOT) obstruction after previous septal-reduction therapy (SRT)., Methods: From January 1989 to March 2022, 145 patients underwent reintervention by septal myectomy for residual LVOT obstruction after previous SRT; 72 patients had previous alcohol septal ablation (ASA) and 73 had previous surgical septal myectomy. Baseline patient characteristics, echocardiographic parameters, and surgical outcomes were compared between these 2 groups., Results: Patients who had previous ASA were more likely to be male (50.0% vs 30.1%; P = .015), be older (median age 57.5 years vs 48.3 years; P < .001), and have a greater body mass index (32.7 kg/m 2 vs 30.0 kg/m 2 ; P = .011). After repeat SRT by septal myectomy, there was no significant difference in the incidence of postoperative complete heart block, necessitating permanent pacemaker, between the 2 groups (8.3% vs 2.7%; P = .151). One (0.7%) patient died within 30 days of surgery. Over a median follow-up of 7.5 years (interquartile range, 3.0-13.8), there were 20 deaths. Kaplan-Meier 5-, 10-, and 15-year survival rates were 100%, 91%, and 76% for the previous septal myectomy group, and 93%, 81%, and 64% for the previous ASA group (P = .207)., Conclusions: Septal myectomy for residual or recurrent LVOT obstruction in patients who had previous ASA is safe, with an acceptably low rate of postoperative complete heart block. Surgical outcomes and late survival rates in patients with complete heart block ASA were satisfactory and comparable with patients who underwent repeat myectomy., Competing Interests: Conflict of Interest Statement The authors reported no conflicts of interest. The Journal policy requires editors and reviewers to disclose conflicts of interest and to decline handling or reviewing manuscripts for which they may have a conflict of interest. The editors and reviewers of this article have no conflicts of interest., (Copyright © 2024 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

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: To contemporaneously reappraise the incidence-rate, prevalence, and natural history of hypertrophic cardiomyopathy (HCM) in Olmsted County, Minnesota, from 1984 to 2015., Patients and Methods: A validated medical-record linkage system collecting information for residents of Olmsted County was used to identify all cases of HCM between January 1, 1984, and December 31, 2015. After adjudication of records from Mayo Clinic and Olmsted Medical Center, data relating to diagnoses and outcomes were abstracted. The calculated incidence rate and prevalence were standardized to the US 1980 White population (age- and sex-adjusted) and compared with a prior study examining the years 1975-1984., Results: Two hundred seventy subjects with HCM were identified. The age- and sex-adjusted incidence rate was 6.6 per 100,000 person-years, and the point prevalence of HCM on January 1, 2016, was 89 per 100,000 population. The incidence rate and point prevalence of HCM on January 1, 2016, standardized to the US 1980 White population (age- and sex-adjusted), were 6.7 (95% CI, 7.1 to 8.8) per 100,000 person-years and 81.5 per 100,000 population, respectively. The incidence rate of HCM increased each decade since the index study. Individuals with HCM had a higher overall standardized mortality rate than the general population with an observed to expected HR of 1.44 (95% CI, 1.21 to 1.71; P<.001) which improved by each decade., Conclusion: The incidence and prevalence of HCM are higher than rates reported from a prior study in the same community examining the years 1975-1984, but lower than other study cohorts. The risk of mortality in HCM remains higher than expected, albeit with improvement in rates of mortality observed each decade during the study period., (Copyright © 2024. Published by Elsevier Inc.)