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Open in new tab Download slide OBJECTIVES This study aimed to assess the effectiveness of three-dimensional printing (3DP) in patients with complex hypertrophic cardiomyopathy requiring combined transaortic and transapical septal myectomy. METHODS We created 3DP models for 7 patients undergoing this surgery approach between June and October 2022 using silicone-like resin and conducted mock operations. The models were compared with echocardiography to identify abnormal muscle bundles and heart structures. These patients were then compared with a 1:2 matched group without 3DP, considering age, sex and additional operations. RESULTS The models mostly presenting with midventricular obstruction showed high consistency with original computed tomography data (r  = 0.978, P  <   0.001). 3DP identified more abnormal muscle bundles than echocardiography, primarily between the interventricular septum and apex. Excised specimens in mock operations mirrored those in actual myectomies. While cardiopulmonary bypass time was not significantly different, a near-20-min decrease was observed in the 3DP group (135.5 ± 31.1 vs 154.4 ± 36.6 min, P  =   0.054). CONCLUSIONS While no significant differences in surgical outcomes were observed, 3DP appeared to enhance the visualization and understanding of spatial structures (average Likert scale score 4.0), potentially contributing to surgical proficiency (overall rating score 3.9). The use of 3DP may offer additional value in the preparation and execution of operations for complex hypertrophic cardiomyopathy cases. [ABSTRACT FROM AUTHOR]

Aims  Many factors cause left ventricular outflow tract obstruction (LVOTO) in hypertrophic cardiomyopathy (HCM). Previous studies reported that left ventricular basal muscle bundle (BMB) may be associated with LVOTO. We aimed to evaluate the role of BMB in LVOTO by echocardiography. Methods and results  Two hundred fifty-six patients diagnosed with HCM were recruited. The morphologic characteristics of left ventricular outflow tract (LVOT) were analysed. BMB was detected in 178 (69.5%) patients by echocardiography. Patients were separated by a resting or provocative LVOT gradient ≥30 mmHg or not. Compared to patients without LVOTO, patients with LVOTO had a significantly thicker basal septum, elongated anterior mitral leaflet (AML), shorter distance between the AML-free margin and the septum or BMB (M-sept/bundle), larger angle between the plane of the mitral valvular orifice and the ascending aorta (MV-AO angle), and higher prevalence of BMB (P < 0.05). According to multivariate analysis, the independent predictors of LVOTO were the presence of BMB, a large basal septum thickness, a short M-sept/bundle, a large MV-AO angle, and a large AML [odds ratio (95% confidence interval): 5.207 (1.381–19.633), 1.386(1.141–1.683), 0.615(0.499–0.756), 1.113(1.054–1.176), and 1.343(1.076–1.677), respectively, P < 0.05]. Of the 256 included patients, 139 underwent surgical myectomy. The transthoracic echocardiography, compared with surgical specimen, showed: sensitivity 98.3%, specificity 82.3%, positive predictive value 97.6%, negative predictive value 87.5%, and accuracy 96.4% to detect BMB. Conclusions BMB is common in HCM. BMB is a risk factor for LVOTO. [ABSTRACT FROM AUTHOR]

Hypertrophic cardiomyopathy (HCM) characterized by asymmetric ventricular septal hypertrophy, is the commonest cause of sudden cardiac death (SCD) in the young. The underlying etiology of HCM in the childhood and adolescent patients is diverse. Moreover, the prognosis of pediatric HCM depends on the age of presentation and etiology. Despite the complexity of children with obstructive HCM, surgical treatment results in a favorable outcome for carefully selected patients in experienced tertiary referral center in contemporary era. Implantable cardioverter-defibrillator (ICD) remains the most effective and reliable treatment to prevent SCD. New pediatric SCD risk prediction model, which has good discrimination and calibration and can distinguish patients who are most benefit from an ICD implantation, is expected to be further refined in the future. [ABSTRACT FROM AUTHOR]

Background: Mid‐ventricular obstruction (MVO) is a rare subtype of hypertrophic cardiomyopathy (HCM) but it is associated with ventricular arrhythmia. The relationship between MVO and non‐sustained ventricular tachycardia (NSVT) in HCM patients is unknown. Hypothesis: The severity of MVO increases the incidence of NSVT in patients with hypertrophic obstructive cardiomyopathy (HOCM). Methods: Five hundred and seventy‐two consecutive patients diagnosed with HOCM in Fuwai Hospital between January 2015 and December 2017 were enrolled in this study. Holter electrocardiographic and clinical parameters were compared between HOCM patients with and without MVO. Results: Seventy‐six (13.3%) of 572 patients were diagnosed with MVO. Compared to patients without MVO, those with MVO were much younger, and had a higher incidence of syncope, greater left ventricular (LV) posterior wall thickness, a higher percentage of LV late gadolinium enhancement, and higher prevalence of NSVT. Furthermore, the prevalence of NSVT increased with the severity of MVO (without, mild, moderate or severe: 11.1%, 18.2%, 25.6%, respectively, p for trend <.01). Similarly, the prevalence of NSVT differed among patients with isolated LV outflow tract (LVOTO), both MVO and LVOTO, and isolated MVO (11.1%, 21.3%, 26.6%, respectively, p for trend =.018). In addition to age, diabetes, left atrial diameter, and maximal wall thickness, multivariate analysis revealed the presence of MVO as an independent risk factor for NSVT (Odds ratio 2.69; 95% confidence interval 1.41 to 5.13, p =.003). Conclusions: The presence and severity of MVO was associated with higher incidence of NSVT in HOCM patients. [ABSTRACT FROM AUTHOR]

Background: Myocardial bridging (MB) is commonly treated in patients with hypertrophic cardiomyopathy. However, whether and how MB should be treated in patients with hypertrophic obstructive cardiomyopathy (HOCM) who underwent septal myectomy remain unclear. Methods: A total of 823 adults with HOCM who underwent septal myectomy at the Fuwai Hospital from 2011 to 2017 were retrospectively studied. Results: Overall, 31 events occurred: 24 patients died and 7 had nonfatal myocardial infarction (MI). The 3‐year cumulative event‐free survival of all‐cause death (97.9% vs. 100% vs. 100% vs. 98.4%, p =.89) and cardiovascular death (98.3% vs. 100% vs. 100% vs. 98.4%, p =.63) were similar among the four groups (non‐MB, coronary artery bypass grafting [CABG], unroofing, untreated, respectively). However, the 3‐year cumulative event‐free survival of nonfatal MI (100% vs. 97.5% vs. 98.0% vs. 89.9%, p <.001) and combined endpoints (97.9% vs. 97.5% vs. 98.0% vs. 88.4%, p =.02) were significantly lowest in untreated MB (non‐MB, CABG, unroofing, untreated, respectively). Cox regression analysis indicated that untreated MB was a significant independent predictor of combined endpoints (hazard ratio: 4.06, 95% confidence interval: 1.60–10.32, p <.001). Moreover, 49 patients underwent coronary artery computed tomography 1 year after surgery. The patency rate of the saphenous vein graft was significantly higher than that of the left internal mammary artery (13.3% vs. 84.2%, p <.001). No MB was detected in the unroofing group. Conclusions: Surgical MB treatment could be beneficial and performed safely during septal myectomy. Myocardial unroofing is the recommended treatment for MB, and unroofing when technically possible may be preferable for long‐term outcomes. [ABSTRACT FROM AUTHOR]

Background and Aim: Recently alcohol septal ablation (ASA) has emerged as an alternative treatment for drug-refractory hypertrophic obstructive cardiomyopathy (HOCM) and a subgroup of HOCM patients with previous ASA may need myectomy. However, subsequent outcome and mechanism of residual obstruction has not been determined. This study aims to determine outcome after myectomy and mechanism of residual obstruction in HOCM patients with previous ASA.Methods: From February 2009 to June 2017, 38 HOCM patients with previous ASA underwent surgical septal myectomy at our institution. Seventy-six patients who underwent surgical septal myectomy initially were included as the comparison group through one-to-two propensity score matching method.Results: Fourteen available cardiac magnetic resonance images revealed inferior location and small area of infarcted myocardium induced by ASA in 12 patients and outside targeted location in two patients. During follow-up (median, 2.4; maximum, 7.8 years), event-free survival at 7 years was 83.2% in the previous ASA group and 94.6% in the comparison group, respectively (P = 0.0378). Multivariable analysis indicated previous ASA (hazard ratio, 4.28; 95% confidence intervals [CI], 1.20-15.26; P = 0.025) and postoperative left ventricular end-diastolic diameter (hazard ratio, 1.14; 95% CI, 1.05-1.23; P = 0.002) were independent predictors of adverse events.Conclusions: This study demonstrated that uncontrollable extent and location of infarcted myocardium induced by ASA may attribute to residual obstruction after previous ASA, and the long-term event-free survival after myectomy was inferior. It may provide special precaution to patient selection and the increased number of ASA practiced worldwide. [ABSTRACT FROM AUTHOR]

Background: Hypertrophic cardiomyopathy (HCM) is associated with arrhythmias and cardiovascular death. Obstructive sleep apnea (OSA) is highly prevalent and independently associated with atrial fibrillation in patients with HCM.Objective: The purpose of this study was to determine the relationship between nonsustained ventricular tachycardia (NSVT) and OSA in hypertrophic obstructive cardiomyopathy (HOCM).Methods: One hundred thirty consecutive patients with a confirmed diagnosis of HOCM in Fuwai Hospital between September 2017 and May 2018 were included. Polysomnography and Holter electrocardiography were performed in all patients.Results: Of 130 patients, 72 (55%) were diagnosed with OSA, including 38 with mild, 21 with moderate, and 13 with severe OSA, and 27 patients (21%) had NSVT. The prevalence of NSVT increased with the severity of OSA (none, mild, moderate, and severe: 12%, 16%, 33%, and 54%, respectively; P < .001 for trend). Compared to patients without NSVT, the apnea-hypopnea index was significantly higher in patients with NSVT among the different OSA groups (mild, moderate, and severe: 12 [11-13] vs 7 [6-8], P = .001; 24 [22-28] vs 19 [17-22], P = .01; and 54 [41-62] vs 34 [31-39], P = .008). In multivariate logistic regression analysis, family history of HCM or sudden cardiac death (odds ratio 6; 95% confidence interval 2-22; P = .005) and apnea-hypopnea index (odds ratio 1.07; 95% confidence interval 1.02-1.12; P = .001) were the only factors associated with NSVT after adjustment for age, sex, and body mass index.Conclusion: The presence and severity of OSA in patients with HOCM is independently associated with NSVT, which is a risk factor for sudden cardiac death and cardiovascular death in this population. [ABSTRACT FROM AUTHOR]

Background: The mid-term outcome of patients with hypertrophic obstructive cardiomyopathy (HOCM) undergoing coronary artery bypass graft (CABG) is unclear.Materials and Methods: We studied 44 patients with HOCM and coronary artery disease (CAD) who underwent septal myectomy and CABG) between 2011 and 2017. The control group was matched in a ratio of 4:1 based on age, sex, body mass index, hypertension, and chest pain.Results: Compared to patients without CAD, patients with CAD had a higher long-term cardiovascular mortality rate (0.6% vs 6.8%, P = 0.03; hazard ratio [HR] = 8.16, 95% confidence interval [CI]: 1.27-74.48, P = 0.03). In addition, 10 out of 176 (5.7%) patients without CAD and nine out of 44 (20.5%) patients with CAD achieved the secondary endpoints (progressive heart failure, unexplained syncope, stroke, atrial fibrillation, and myocardial infarction) (HR = 2.89, 95%CI: 1.03-8.12, P = 0.04). The 5-year survival rate and cardiovascular event-free survival rate were significantly higher in patients without CAD than in those with CAD (97.4% vs 93.9%, P = 0.03; 89.2% vs 80.1%; P = 0.04). In the multivariate analysis, presence of CAD, New York Heart Association class, and left atrial diameter were predictors of combined cardiovascular events when adjusted for age and male sex.Conclusions: The cardiovascular death and cardiovascular events are significantly increased in patients with HOCM and CAD who underwent CABG at the time of septal myectomy. [ABSTRACT FROM AUTHOR]

Background: Filamin C (FLNC) mutation was reported as a cause of HCM, with a high probability of sudden cardiac death. However, the mutation profile of FLNC, and its relationship with phenotypic expression in HCM, remains to be elucidated. Methods: In this study, FLNC gene was sequenced in 540 HCM patients and 307 healthy controls. Results: We found that 39 (7.2%) patients carried FLNC mutations, with a similar frequency to that of controls (4.2%, p = 0.101). Pedigree analysis showed that mutations were not well segregated with HCM. The baseline characteristics between HCM patients, with and without mutations, were comparable. FLNC mutations did not increase the risk for either all‐cause mortality (HR 0.746, 95% CI 0.222–2.295, p = 0.575) or cardiac mortality (HR 0.615, 95% CI 0.153–1.947, p = 0.354) in HCM patients during a follow‐up of 4.7 ± 3.2 years. Moreover, there was no significant difference in survival free from sudden cardiac arrest (HR 0.721, 95% CI 0.128–3.667, p = 0.660) and heart failure (HR 0.757, 95% CI 0.318–1.642, p = 0.447). Conclusions: FLNC mutations were common in both HCM patients and healthy population. The pathogenicity of FLNC mutations detected in HCM patients and its association with the clinical outcomes should be cautiously interpreted. As a previously reported cause of HCM, FLNC mutation is found to be very common in the healthy population and HCM patients in this study. Generally, FLNC mutation does not cause HCM, and it does not increase later mortality and morbidity in HCM patients. [ABSTRACT FROM AUTHOR]

OBJECTIVES This study aims to assess the impact of morphological type on the prognosis of obstructive hypertrophic cardiomyopathy patients who underwent myectomy. METHODS We recruited 469 obstructive hypertrophic cardiomyopathy patients refractory to medicinal treatment who have undergone surgical myectomy at a nationwide referral centre. All patients were divided into 3 groups based on the morphological classification of left ventricular hypertrophy and were followed up by telephone every year. The primary end-points were sudden cardiac death, aborted sudden cardiac death and death due to heart failure or stroke. The secondary end-points were non-fatal cardiovascular events. RESULTS Typical hypertrophy limited to the basal septum, hypertrophy of the whole ventricular septum and hypertrophy that involved the whole left ventricle were presented in 248 (52.9%), 141 (30.1%) and 80 (17.0%) patients, respectively. During the follow-up of 2.5 ± 1.4 years after myectomy, 10 (2.1%) and 43 (9.2%) patients met the primary end-point and secondary end-points, respectively. The maximal thickness of the ventricular septum, the left ventricular mass and the presence of extensive late gadolinium enhancement were lower in patients with hypertrophy limited to the basal septum than in other patients. Patients with the basal septum hypertrophy showed better survival after myectomy compared with other patients. For the secondary and composite end-points, there was no significant difference between the 3 groups. CONCLUSIONS Patients with hypertrophy limited to the basal septum represented a special clinical subtype of obstructive hypertrophic cardiomyopathy showing better clinical outcomes, while diffuse hypertrophy of the ventricular septum and free wall indicated lower survival after surgical myectomy. [ABSTRACT FROM AUTHOR]

An amendment to this paper has been published and can be accessed via the original article. [ABSTRACT FROM AUTHOR]

Hypertrophic cardiomyopathy (HCM) represents one of the most common heritable heart diseases. However, the signalling pathways and regulatory networks underlying the pathogenesis of HCM remain largely unknown. Here, we present a strand-specific RNA-seq dataset for both coding and lncRNA profiling in myocardial tissues from 28 HCM patients and 9 healthy donors. This dataset constitutes a valuable resource for the community to examine the dysregulated coding and lncRNA genes in HCM versus normal conditions. Design Type(s) transcription profiling design • disease state design • sequence analysis objective Measurement Type(s) transcription profiling assay Technology Type(s) RNA sequencing Factor Type(s) experimental condition Sample Characteristic(s) Homo sapiens • heart Machine-accessible metadata file describing the reported data (ISA-Tab format) [ABSTRACT FROM AUTHOR]

Background: The prevalence of obstructive sleep apnoea (OSA) is high in patients with hypertrophic cardiomyopathy. The effect of septal myectomy on OSA is not clear. This study aimed to examine the association between hypertrophic obstructive cardiomyopathy and OSA before and after septal myectomy.Method: We included 85 consecutive patents with a confirmed diagnosis of hypertrophic obstructive cardiomyopathy who underwent septal myectomy. Polysomnography was performed in all patients before and 3 months after the surgery.Results: Of the 85 patients, 49 (58%) were diagnosed with OSA. Patients with OSA were significantly older than those without OSA. The incidence of atrial fibrillation significantly increased during the perioperative period in patients with OSA (p = 0.03). The severity of OSA significantly increased 3 months after surgery, as determined by the apnoea-hypopnoea index (AHI; p < 0.001), obstructive apnoea index (p = 0.024), and hypopnoea index (p = 0.003), whereas central apnoea index was decreased (p = 0.008). In the multivariate linear regression analysis, mean oxygen desaturation and time% with SpO2 <90% during sleep before surgery were significantly associated with increased AHI, independently of body mass index and sex (p = 0.026 and p = 0.007, respectively; adjusted R2 = 0.365).Conclusions: The severity of OSA significantly increased 3 months after septal myectomy as determined by AHI, obstructive apnoea index, and hypopnoea index. Mean oxygen saturation and time% with SpO2 <90% during sleep before surgery were independently associated with the increase of AHI. However, the specific mechanism of such deterioration of OSA after septal myectomy needs to be determined in detail. [ABSTRACT FROM AUTHOR]