Vaccination against coronavirus disease 2019 in patients with pulmonary hypertension: A national prospective cohort study.

BACKGROUND: Coronavirus disease 2019 (COVID-19) has potential risks for both clinically worsening pulmonary hypertension (PH) and increasing mortality. However, the data regarding the protective role of vaccination in this population are still lacking. This study aimed to assess the safety of approved vaccination for patients with PH. METHODS: In this national prospective cohort study, patients diagnosed with PH (World Health Organization [WHO] groups 1 and 4) were enrolled from October 2021 to April 2022. The primary outcome was the composite of PH-related major adverse events. We used an inverse probability weighting (IPW) approach to control for possible confounding factors in the baseline characteristics of patients. RESULTS: In total, 706 patients with PH participated in this study (mean age, 40.3 years; mean duration after diagnosis of PH, 8.2 years). All patients received standardized treatment for PH in accordance with guidelines for the diagnosis and treatment of PH in China. Among them, 278 patients did not receive vaccination, whereas 428 patients completed the vaccination series. None of the participants were infected with COVID-19 during our study period. Overall, 398 patients received inactivated severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccine, whereas 30 received recombinant protein subunit vaccine. After adjusting for baseline covariates using the IPW approach, the odds of any adverse events due to PH in the vaccinated group did not statistically significantly increase (27/428 [6.3%] vs. 24/278 [8.6%], odds ratio = 0.72, P = 0.302). Approximately half of the vaccinated patients reported at least one post-vaccination side effects, most of which were mild, including pain at the injection site (159/428, 37.1%), fever (11/428, 2.6%), and fatigue (26/428, 6.1%). CONCLUSIONS: COVID-19 vaccination did not significantly augment the PH-related major adverse events for patients with WHO groups 1 and 4 PH, although there were some tolerable side effects. A large-scale randomized controlled trial is warranted to confirm this finding. The final approval of the COVID-19 vaccination for patients with PH as a public health strategy is promising.

Clonal Hematopoiesis of Indeterminate Potential in Chronic Thromboembolic Pulmonary Hypertension: A Multicenter Study.

BACKGROUND: The pathogenesis of chronic thromboembolic pulmonary hypertension (CTEPH) is multifactorial and growing evidence has indicated that hematological disorders are involved. Clonal hematopoiesis of indeterminate potential (CHIP) has recently been associated with an increased risk of both hematological malignancies and cardiovascular diseases. However, the prevalence and clinical relevance of CHIP in patients with CTEPH remains unclear. METHODS: Using stepwise calling on next-generation sequencing data from 499 patients with CTEPH referred to 3 centers between October 2006 and December 2021, CHIP mutations were identified. We associated CHIP with all-cause mortality in patients with CTEPH. To provide insights into potential mechanisms, the associations between CHIP and inflammatory markers were also determined. RESULTS: In total, 47 (9.4%) patients with CTEPH carried at least 1 CHIP mutation at a variant allele frequency of ≥2%. The most common mutations were in DNMT3A, TET2, RUNX1, and ASXL1. During follow-up (mean, 55 months), deaths occurred in 22 (46.8%) and 104 (23.0%) patients in the CHIP and non-CHIP groups, respectively (P<0.001, log-rank test). The association of CHIP with mortality remained robust in the fully adjusted model (hazard ratio, 2.190 [95% CI, 1.257-3.816]; P=0.006). Moreover, patients with CHIP mutations showed higher circulating interleukin-1ß and interleukin-6 and lower interleukin-4 and IgG galactosylation levels. CONCLUSIONS: This is the first study to show that CHIP mutations occurred in 9.4% of patients with CTEPH are associated with a severe inflammatory state and confer a poorer prognosis in long-term follow-up.

Percutaneous transluminal pulmonary angioplasty for Takayasu arteritis-associated pulmonary hypertension: A single-arm meta-analysis.

BACKGROUND: The efficacy and safety of percutaneous transluminal pulmonary angioplasty (PTPA) for Takayasu arteritis-associated pulmonary hypertension (TA-PH) remain unclear. OBJECTIVES: To examine the efficacy and safety of PTPA in TA-PH. METHODS: PubMed, Embase, and the Cochrane Central Register of Controlled Trials Library were searched from inception to August 18, 2022, for articles investigating the efficacy and safety of PTPA for TA-PH. The primary efficacy outcomes were pulmonary vascular resistance (PVR) changes from baseline to re-evaluation and 6-minute walking distance (6MWD). The safety outcome was procedure-related complications. RESULTS: Five articles comprising 104 patients with TA-PH who underwent PTPA were included. The scores of article quality, as assessed using the methodological index for nonrandomized studies tool, were high, ranging from 13 to 15 points. The pooled treatment effects of PVR (weighted mean difference [WMD]: -4.8 WU; 95% confidence interval [CI]: -6.0 to -3.5 WU; I2 = 0.0%), 6MWD (WMD: 101.9 m; 95% CI: 60.3-143.6 m; I2 = 70.4%) significantly improved. Procedure-related complications, which predominantly present as pulmonary artery injury and pulmonary injury, occurred in 32.0% of the included patients. Periprocedural death occurred in one patient (1.0%, 1/100). CONCLUSIONS: Patients with TA-PH could benefit from PTPA in terms of hemodynamics and exercise tolerance, at the expense of procedure-related complications. PTPA should be encouraged to enhance the treatment response in TA-PH. These findings need to be confirmed by further studies, ideally, randomized controlled trials. REGISTRATION: PROSPERO CRD42022354087.

Non-Ischemic, Non-Hypoxic Myocardial Injury, and Long-Term Mortality in Patients with Coronavirus Disease 2019: A Retrospective Cohort Study.

Objective: Cardiac damage is commonly reported in patients with coronavirus disease 2019 (COVID-19) but its prevalence and impact on the long-term survival of patients remain uncertain. This study aimed to explore the prevalence of myocardial injury and assess its prognostic value in patients with COVID-19. Methods: A single-center, retrospective cohort study was performed at the Affiliated Hospital of Jianghan University. Data from 766 patients with confirmed COVID-19 who were hospitalized from December 27, 2019 to April 25, 2020 were collected. Demographic, clinical, laboratory, electrocardiogram, treatment data and all-cause mortality during follow-up were collected and analyzed. Results: Of the 766 patients with moderate to critically ill COVID-19, 86 (11.2%) died after a mean follow-up of 72.8 days. Myocardial injury occurred in 94 (12.3%) patients. The mortality rate was 64.9% (61/94) and 3.7% (25/672) in patients with and without myocardial injury, respectively. Cox regression showed that myocardial injury was an independent risk factor for mortality (hazard ratio: 8.76, 95% confidence interval: 4.76-16.11, P < 0.001). Of the 90 patients with myocardial injury with electrocardiogram results, sinus tachycardia was present in 29, bundle branch block in 26, low voltage in 10, and abnormal T-wave in 53. Conclusions: COVID-19 not only involves pneumonia but also cardiac damage. Myocardial injury is a common complication and an independent risk factor for mortality in COVID-19 patients.

Percutaneous Pulmonary Angioplasty for Patients With Takayasu Arteritis and Pulmonary Hypertension.

BACKGROUND: Percutaneous transluminal pulmonary angioplasty (PTPA) is a treatment modality for chronic thromboembolic pulmonary hypertension, but whether it can be applied to Takayasu arteritis-associated pulmonary hypertension (TA-PH), another chronic obstructive pulmonary vascular disease, remains unclear. OBJECTIVES: This study sought to investigate the efficacy and safety of PTPA for TA-PH. METHODS: Between January 1, 2016, and December 31, 2019, a total of 50 patients with TA-PH who completed the PTPA procedure (the PTPA group) and 21 patients who refused the PTPA procedure (the non-PTPA group) were prospectively enrolled in this cohort study. The primary outcome was all-cause mortality. The safety outcomes included PTPA procedure-related complications. RESULTS: Baseline characteristics and medical therapies were similar between the PTPA group and the non-PTPA group. During a mean follow-up time of 37 ± 14 months, deaths occurred in 3 patients (6.0%) in the PTPA group and 6 patients (28.6%) in the non-PTPA group, contributing to the 3-year survival rate of 93.7% in the PTPA group and 76.2% in the non-PTPA group (P = 0.0096 for log-rank test). The Cox regression model showed that PTPA was associated with a significantly reduced hazard of all-cause mortality in TA-PH patients (HR: 0.18; 95% CI: 0.05-0.73; P = 0.017). No periprocedural death occurred. Severe complications requiring noninvasive positive pressure ventilation occurred in only 1 of 150 total sessions (0.7%). CONCLUSIONS: PTPA tended to be associated with a reduced risk of all-cause mortality with acceptable safety profiles and seemed to be a promising therapeutic option for TA-PH patients.

Mid-term outcomes of biventricular obstruction and left ventricular outflow tract obstruction after surgery correction in child and adolescent patients with hypertrophic cardiomyopathy.

BACKGROUND: Data on the outcomes of hypertrophic cardiomyopathy (HCM) with biventricular obstruction are limited. OBJECTIVE: Our aim is to compare mid-term outcomes of biventricular outflow tract obstruction (BVOTO) HCM, left ventricular outflow tract obstruction (LVOTO) HCM and nonobstructive hypertrophic cardiomyopathy (NO-HCM) in children and adolescents who were treated with standard medication or surgical resection. METHODS: This retrospective study identified 21 BVOTO patients and recruited 27 LVOTO and 24 NO-HCM patients younger than 18 years presenting at our institution. The primary endpoint was all-cause death, and secondary endpoints were cardiovascular events. RESULTS: More BVOTO patients (61.9%) than LVOTO (19.2%) and NO-HCM patients (25%) exhibited New York Heart Association (NYHA) III/IV status (p < 0.01). Fourteen BVOTO and 16 LVOTO patients obtained a significant reduction of outflow tract pressure gradients after surgery (vs. preoperative baseline, p < 0.001). One of the 14 BVOTO patients died, whereas no deaths occurred among LVOTO patients. Three of 14 BVOTO surgery patients had complete heart block (CHB) and 4 had new right bundle branch block (RBBB), while no CHB or RBBB occurred in the LVOTO surgery patients. The BVOTO patients had a longer duration of aortic cross-clamping and postoperative hospital days than the LVOTO patients (p < 0.05). During a median 42-month follow-up, no deaths occurred among the remaining patients. The primary and secondary endpoint-free survival rates of the BVOTO group were comparable to those of the LVOTO and NO-HCM groups. CONCLUSIONS: In children and adolescents, BVOTO patients were associated with more severe symptoms than LVOTO and NO-HCM patients; however, good mid-term outcomes similar to those of the LVOTO and NO-HCM groups can be achieved with the application of contemporary cardiovascular treatment strategies. Notably, BVOTO surgery was associated with an increased risk of CHB and RBBB compared to LVOTO surgery.

Clinical Outcomes of Myocardial Bridging versus No Myocardial Bridging in Patients with Apical Hypertrophic Cardiomyopathy.

OBJECTIVE: To determine the prevalence and clinical effects of myocardial bridging (MB) in patients with apical hypertrophic cardiomyopathy (AHCM). METHODS: Angiograms from 212 AHCM patients were reviewed to identify MB. The patients were classified into 2 groups: AHCM with and AHCM without MB. We reviewed patient records on cardiovascular (CV) risk factors, symptoms, CV events, and CV mortality. RESULTS: In all, 60 patients with MB and 100 without MB were included. Rates of angina (61.7 vs. 40%; p = 0.008), mimicking non-ST-segment elevation myocardial infarction (15 vs. 3%, p = 0.013), and Canadian Cardiovascular Society class III/IV angina (18.3 vs. 4%; p = 0.003) were higher in patients with MB than in those without. Mean follow-up periods (65.5 ± 50.5 vs. 64.4 ± 43.6 months, p = 0.378) and CV mortality (3.3 vs. 1%; p = 0.652) were similar in the 2 groups. Kaplan-Meier estimates demonstrated that CV event-free survival rates were lower in patients with MB than in those without (71.7 vs. 88%; p = 0.022). MB, late gadolinium enhancement, and female sex were independent risk factors for CV events in a multivariate Cox regression analysis adjusted for other risk factors. CONCLUSION: More serious symptoms and a higher risk of CV events were observed in AHCM patients with MB than in those without MB. CV mortality was similar in these 2 groups.

Long-Term Prognosis after Myectomy in Hypertrophic Obstructive Cardiomyopathy with Severe Left Ventricular Hypertrophy.

OBJECTIVES: Patients with hypertrophic obstructive cardiomyopathy (HOCM) and severe left ventricular hypertrophy (maximal left ventricular wall thickness ≥30 mm) are at high risk of sudden cardiac death (SCD). In this study, we aimed to determine whether HOCM patients with severe hypertrophy had a lower incidence of SCD after myectomy. METHODS: HOCM patients with severe hypertrophy were consecutively enrolled from Fuwai Hospital in China between 2000 and 2013. Long-term outcomes were retrospectively compared between the 2 groups, namely the myectomy group and medical group. RESULTS: A total of 244 patients (118 in the myectomy group and 126 in the medical group) were involved. The mean follow-up durations for the myectomy and medical groups were 5.07 ± 3.73 and 6.23 ± 4.15 years, respectively. During the follow-up period, the annual cardiovascular mortality rate was 0.84% in the myectomy group and 2.04% in the medical group (p = 0.041). The annual SCD rate was 0.33% in the myectomy group and 1.40% in the medical group (p = 0.040). Multivariate Cox regression analysis showed that myectomy was independently associated with lower rates of cardiovascular death and SCD. CONCLUSIONS: In HOCM patients with severe hypertrophy, those that underwent myectomy had a lower risk of cardiovascular death and SCD than those treated with medicines only.

Procedural complication and long term outcomes after alcohol septal ablation in patients with obstructive hypertrophic cardiomyopathy: data from China.

Data on procedural complications and long term survival after alcohol septal ablation (ASA) in Chinese patients with obstructive hypertrophic cardiomyopathy (HOCM) are lacking. We aimed to investigate long-term survival of HOCM patients after ASA and compared to the non-obstructive hypertrophic cardiomyopathy(NOHCM). A total of 233 patients with HOCM and a peak pressure gradient of ≥50 mm Hg at rest or with provocation were consecutively enrolled from Fuwai Hospital in China between 2000 and 2012. Another 297 patients without left ventricular outflow tract obstruction were regarded as control group. Periprocedural mortality of ASA were low (0.89%). Periprocedural lethal ventricular arrhythmia occurred in 9 patients (4.0%). Alcohol volume (RR 1.44, 95% CI: 1.03-2.03, P = 0.034) and age ≤40 years old (RR 4.63, 95% CI: 1.07-20.0, P = 0.040) were independent predictors for periprocedural lethal ventricular arrhythmia. The 10- year overall survival was 94.6% in the ASA group, similar with 92.9% in the NOHCM group (P = 0.930). In conclusion, periprocedural mortality and complications were rare in ASA. Long term survival after ASA were satisfactory and comparable to NOHCM. Patients under 40 years old should be more cautious undergoing ASA, for these patients were more likely to endure lethal ventricular arrhythmia during periprocedural period of ASA.

Genetic anticipation in a special form of hypertrophic cardiomyopathy with sudden cardiac death in a family with 74 members across 5 generations.

Hypertrophic cardiomyopathy (HCM) is the most common heritable heart disease. The genetic anticipation of HCM and its associated etiology, sudden cardiac death (SCD), remains unclear. The aim of this study was to investigate the mechanism underlying the genetic anticipation of HCM and associated SCD.An HCM family including 5 generations and 74 members was studied. Two-dimensional echocardiography was performed to diagnose HCM. The age of onset of HCM was defined as the age at first diagnosis according to hospital records. The information on SCD was confirmed by verification by ≥2 family members and a review of hospital records. Whole-genome sequencing was performed on 4 HCM subjects and 1 healthy control in the family. The identified mutations were screened in all available family members and 216 unrelated healthy controls by Sanger sequencing.The median ages of onset of HCM were 63.5, 38.5, and 18.0 years in members of the second, third, and fourth generations of the family, respectively, and the differences between the generations were significant (P < 0.001). The age at SCD also decreased with each subsequent generation (P < 0.05). In particular, among the third-generation family members, SCD occurred between 30 and 40 years of age at approximately 8 AM, whereas among the fourth-generation family members, all 5 males who experienced SCD were 16 years of age and died at approximately 8 AM. The sarcomere gene mutations MYH7-A719H and MYOZ2-L169G were detected in the HCM individuals in this pedigree. Increases in the number of mutations and the frequency of multiple gene mutations were observed in the younger generations. Moreover, a structural variant was present in the HCM phenotype-positive subjects but was absent in the HCM phenotype-negative subjects.HCM may exhibit genetic anticipation, with a decreased age of onset and increased severity in successive generations. Multiple gene mutations may contribute to genetic anticipation in HCM and thus may be of prognostic value.

Long-term survival after acute myocardial infarction in patients with hypertrophic cardiomyopathy.

BACKGROUND: Hypertrophic cardiomyopathy (HCM) is associated with poor prognosis. It has been reported that there is no difference in in-hospital mortality after acute myocardial infarction (AMI) between patients with and without HCM. However, whether there is a difference in long-term survival after AMI between patients with and without HCM remains unclear. HYPOTHESIS: Long-term survival after AMI is worse in patients with vs without HCM. METHODS: The clinical profiles of 91 consecutive patients with HCM and AMI (HCM group) and 91 sex- and age-matched patients with AMI without HCM (non-HCM group) were analyzed. The study endpoint was all-cause mortality. RESULTS: During a follow-up period of 4.9 ± 3.6 years, all-cause mortality occurred in 25 patients (27.5%) in the HCM group and 13 patients (14.3%) in the non-HCM group. The survival of the HCM group was inferior to that of the non-HCM group (log-rank P = 0.039). During the first year of follow-up, 3 deaths (3.3%) occurred in the HCM group and 7 deaths (7.7%) occurred in the non-HCM group (log-rank P = 0.177). Among patients who survived beyond the first year of follow-up (172 patients), the annual mortality rates were 6.3% (95% confidence interval: 4.0%-9.3%) in the HCM group and 1.6% (95% confidence interval: 0.6%-3.5%) in the non-HCM group (log-rank P = 0.001). CONCLUSIONS: AMI patients with HCM exhibited worse long-term survival than did AMI patients without HCM.

Comparison of Long-Term Outcome between Apical and Asymmetric Septal Hypertrophic Cardiomyopathy.

OBJECTIVES: As reported, diagnostic age, gender and presence of outflow tract obstruction have an impact on prognosis in patients with hypertrophic cardiomyopathy. The aim of this study was to compare the long-term outcome between apical hypertrophic cardiomyopathy (ApHCM) and asymmetric septal hypertrophic cardiomyopathy (ASHCM) after the exclusion of these factors. METHODS: A total of 540 patients (270 with ApHCM and 270 with ASHCM) identified in a consecutive single-center cohort were retrospectively studied. The two groups were matched by diagnostic age, gender and the presence of outflow tract obstruction. Clinical characteristics and long-term outcomes were compared. RESULTS: The mean follow-up duration in ASHCM and ApHCM were 6.6 ± 5.5 and 7.6 ± 4.1 years, respectively. During follow-up, 16 patients experienced cardiovascular death in the ASHCM group, while 2 patients experienced cardiovascular death in the ApHCM group (6.3 vs. 0.7%, p < 0.01). Cardiovascular morbidity in the ASHCM and ApHCM groups were 39.9 and 18.5% (p < 0.01). In the multivariate Cox regression analysis late gadolinium enhancement (LGE; HR 4.81, 95% CI 1.28-78.0, p = 0.03) and unexplained syncope (HR 9.68, 95% CI 1.9-17.2, p < 0.01) were independent predictors for cardiovascular mortality. Unexplained syncope was independently associated with a higher risk for sudden cardiac death (HR 4.3, 95% CI 1.2-15.3, p = 0.02). CONCLUSIONS: After eliminating the interference of diagnostic age, gender and outflow tract obstruction, ASHCM represented a worse prognosis with a higher incidence of cardiovascular mortality and morbidity than ApHCM. LGE was a strong predictor for cardiovascular death.

Effectiveness of Alcohol Septal Ablation Versus Transaortic Extended Myectomy in Hypertrophic Cardiomyopathy with Midventricular Obstruction.

OBJECTIVES: Investigate the effectiveness of alcohol septal ablation (ASA) and transaortic extended myectomy (TEM) in hypertrophic cardiomyopathy (HCM) with midventricular obstruction (MVO). BACKGROUND: MVO is less common than subaortic obstruction. Data on the effectiveness of ASA and TEM in MVO are lacking. METHODS: The clinical profiles of 22 patients undergoing ASA and 37 patients undergoing TEM were compared. No patient had apical aneurysm, abnormal chordae, mitral valve replacement or repair. RESULTS: Baseline midventricular pressure gradient and symptoms were comparable between the ASA and TEM groups. During follow-up, both groups demonstrated substantial reduction in pressure gradient (the ASA group: 79.7 ± 21.2 mm Hg to 43.7 ± 28.9 mm Hg, P < 0.001; the TEM group: 69.0 ± 23.9 mm Hg to 15.0 ± 16.9 mm Hg, P < 0.001). The reduction in pressure gradient was greater (78.9 ± 18.6% vs. 46.4 ± 33.4%, P < 0.001) and the residual pressure gradient was lower after TEM versus ASA (P < 0.001). Patients with New York Heart Association class III/IV dyspnea decreased from 59.1 to 18.2% (P = 0.022) in the ASA group and from 56.8 to 5.6% (P < 0.001) in the TEM group. Patients with Canadian Cardiovascular Society class III/IV angina decreased from 40.9 to 9.1% (P = 0.016) in the ASA group and from 32.4 to 0% (P < 0.001) in the TEM group. CONCLUSIONS: While ASA and TEM both improve gradients and symptoms, TEM may provide a more reliable reduction in gradients compared to ASA.

Effectiveness of Alcohol Septal Ablation in Obstructive Hypertrophic Cardiomyopathy With Versus Without Extreme Septal Hypertrophy.

AIMS: Data on the effectiveness of alcohol septal ablation (ASA) in patients with hypertrophic cardiomyopathy (HCM) and extreme septal hypertrophy (ESH) are lacking. This study aimed to compare the effectiveness of ASA in patients with vs without ESH. METHODS: Clinical profiles of 17 patients with ESH and 256 patients without ESH were compared. RESULTS: Baseline pressure gradient and limiting symptoms were comparable between patients with and without ESH. At median 1.1 years of follow-up after ASA, pressure gradient was 48.5 ± 40.4 mm Hg in the ESH group and 40.9 ± 35.2 mm Hg in the non-ESH (N-ESH) group (P=.33). Patients with New York Heart Association class III/IV represented 5.9% of the ESH group and 16.9% of the N-ESH group (P=.39). Patients with Canadian Cardiovascular Society class III/IV represented 5.9% of the ESH group and 10.2% of the N-ESH group (P=.87). CONCLUSION: The effectiveness of ASA seems comparable between patients with and without ESH.

The Prevalence and Long-Term Outcomes of Extreme Right versus Extreme Left Ventricular Hypertrophic Cardiomyopathy.

OBJECTIVES: Extreme left ventricular hypertrophy (LVH) is a known risk factor for sudden cardiac death in hypertrophic cardiomyopathy (HCM). Extreme right ventricular hypertrophy (RVH) is rare, and whether it is linked to a poor outcome is unknown. This study was designed to investigate differences between HCM patients with extreme RVH and those with extreme LVH. METHODS: Among 2,413 HCM patients, 31 with extreme RVH (maximum right ventricular wall thickness ≥ 10 mm) and 194 with extreme LVH (maximum left ventricular wall thickness ≥ 30 mm) were investigated. The main clinical features and natural history were compared between the 2 groups. RESULTS: The prevalence of extreme RVH and extreme LVH was 1.3 and 8.0%, respectively. Patients with extreme RVH tended to be younger and female (p < 0.01). Cardiovascular-related mortality and morbidity within 10 years were significantly greater in the extreme RVH group (p < 0.05). Multivariate analysis demonstrated 3 independent predictors for cardiovascular mortality - extreme RVH, left ventricular end-diastolic dimension ≥ 50 mm, and age ≤ 18 years at baseline - and 2 for morbidity - extreme RVH and presyncope. CONCLUSIONS: Compared with extreme LVH, extreme RVH was quite uncommon in HCM and had a worse prognosis. A right ventricle examination should be performed in routine HCM evaluation.

Incidence of ischemic stroke and systemic embolism in patients with hypertrophic cardiomyopathy, nonvalvular atrial fibrillation, CHA2DS2-VASc score of ≤1 and without anticoagulant therapy.

Data on the risk of ischemic stroke and systemic embolism (iSSE) events in patients with nonvalvular atrial fibrillation (NVAF), a CHA2DS2-VASc score of ≤1, hypertrophic cardiomyopathy (HCM), and without anticoagulant therapy are still lacking. The aim of this study was to investigate the incidence of iSSE events in these patients. We consecutively screened medical records of patients with HCM and NVAF referred to Fuwai Hospital between January 1994 and March 2014. The primary end point was iSSE events, defined as a composite of ischemic stroke and systemic embolism. Follow-up was carried out to ascertain end point status. Medical records of 522 patients with NVAF and HCM were screened. A total of 108 patients (20.7 %) with a CHA2DS2-VASc score of ≤1 and without anticoagulant therapy were enrolled and constituted our study population. After a median follow-up of 2.4 years (range 0.6-14.1 years; 376.2 patient-years), ischemic stroke occurred in 2 patients, resulting in death of 1 patient in the first year and paralysis of the other patient in the fourth year. No other iSSE events occurred. The incidence of iSSE was 0.9 % [95 % confidence interval (CI) 0.0-5.0 %] in the first year, and 0.5 % per 100 patient-years (95 % CI 0.1-1.9 %). The risk of iSSE events seems low in patients with NVAF, a CHA2DS2-VASc score of ≤1, HCM, and without anticoagulant therapy. Multicenter studies with sizeable study populations are needed to validate the risk of iSSE events in these patients.

[Clinical features and long-term outcome comparison of patients with midventricular obstructive hypertrophic cardiomyopathy and apical hypertrophic cardiomyopathy].

OBJECTIVE: To compare the clinical features and long-term outcome of patients with midventricular obstructive hypertrophic cardiomyopathy (MVOHCM) and patients with apical hypertrophic cardiomyopathy (AHCM) in China. METHODS: This retrospective study analyzed clinical data of 66 patients with MVOHCM and 263 patients with AHCM from a consecutive single-center cohort consisting of 2 413 patients with HCM. The clinical features, cardiovascular mortality and morbidity were compared between the two groups. RESULTS: Compared with the AHCM, patients in the MVOHCM group was younger and more likely to be symptomatic over a mean follow-up of 7 years. The proportion of MVOHCM and AHCM were 2.7% (66/2 413) and 10.9% (263/2 413) (P < 0.001), respectively, in this cohort. Cardiovascular mortality of the two groups were 13.6% (9/66) and 0.8% (2/263) (P < 0.001), and cardiovascular morbidity of the two groups were 53.0% (35/66) and 14.4% (38/263) (P < 0.001). CONCLUSION: MVOHCM is rarer, but the clinical manifestations and long-term outcomes are worse compared with AHCM in this patient cohort.

Survival after alcohol septal ablation versus conservative therapy in obstructive hypertrophic cardiomyopathy.

BACKGROUND: The impact of alcohol septal ablation (ASA) on the survival of patients with drug-refractory obstructive hypertrophic cardiomyopathy (HCM) remains unresolved. The aim of this study was to compare survival after ASA vs. conservative therapy. METHODS: We studied a consecutive cohort of 274 patients with severe drug-refractory obstructive HCM, 229 in ASA group and 45 in conservative group. The primary endpoint was a composite of all-cause mortality and aborted cardiac arrest. RESULTS: With a median follow-up of 4.3 years, primary endpoint occurred in 13 (5.7%) patients in the ASA group, and 8 (17.8%) patients in the conservative group. The 5- and 10-year survival free from primary endpoint of the ASA group (94.5% and 93.0%, respectively) was significantly better than that of the conservative group (78.3% and 72.2%, respectively, log-rank p = 0.009). Independent determinants of primary endpoint were ASA therapy (hazard ratio [HR] 0.22; 95% confidence interval [CI] 0.08-0.60; p = 0.003) and maximal septal thickness (HR 1.14; 95% CI 1.03-1.27; p = 0.011). CONCLUSIONS: In patients with severe drug-refractory obstructive HCM, survival after ASA is favorable and better than that of conservative therapy. ASA seems to improve survival.

Clinical characteristics and prognosis of 60 patients with midventricular obstructive hypertrophic cardiomyopathy.

BACKGROUND: Midventricular obstructive hypertrophic cardiomyopathy (MVOHCM) is a rare form of hypertrophic cardiomyopathy. Knowledge regarding the diagnosis, morbidity and cardiovascular mortality is limited. In this study, we aimed to describe the long-term outcomes of patients with MVOHCM followed in a tertiary referral centre.Methods A retrospective study of 60 patients with MVOHCM diagnosed at FuWai Hospital was performed. Clinical features, mortality and cardiovascular morbidity were analysed. RESULTS: The 60 patients with MVOHCM represented 2.9% of all the hypertrophic cardiomyopathy cases (n = 2068). At diagnosis, the mean age was 40.2 ±â€Š15.0 years. During 7.1 ±â€Š6.3 years of follow-up after diagnosis, the cardiovascular mortality was 15.0%. The probability of survival at 10 years was 77.0 ±â€Š8.0%. The following two predictors of cardiovascular mortality were identified: severe ventricular septal hypertrophy at least 30  mm (hazard ratio, 3.19; P = 0.031) and unexplained syncope (hazard ratio, 4.59; P = 0.002) at baseline. Thirty patients (50.0%) had one or more morbid events, and the most frequent was nonsustained ventricular tachycardia. Apical aneurysm formation was identified in 20% of patients, and the patients with apical aneurysms were more inclined to experience nonsustained ventricular tachycardia than patients without apical aneurysm (58.3 vs. 16.7%; P = 0.003). Peak pressure gradient at least 70  mm Hg (hazard ratio, 3.00; P = 0.01) at baseline was identified as the only predictor of apical aneurysm. CONCLUSION: In Chinese patients, MVOHCM is associated with an unfavourable prognosis of cardiovascular mortality. One-half of these patients experience major cardiovascular events, and 20% develop an apical aneurysm, which significantly increases arrhythmia events. These data warrant measures to ensure the early recognition of MVOHCM followed by appropriate therapeutic interventions.