宋昌鹏
首都医科大学附属北京天坛医院
BACKGROUND:Myocardial bridging (MB) is common in patients with hypertrophic cardiomyopathy (HCM). There are sparse data on the impact of MB on myocardial fibrosis in HCM. This study was designed to evaluate the relationship between MB and myocardial fibrosis in patients with obstructive HCM.METHODS:In this cohort study, retrospective data were collected from a high-volume HCM center. Patients with obstructive HCM who underwent septal myectomy and preoperative cardiac magnetic resonance (CMR) were screened from 2011 to 2018.RESULTS:Finally, 492 patients were included in this study, with an average age of 45.7 years. Of these patients, 76 patients had MB. MB occurred mostly in the left anterior descending artery (73/76). The global extent of late gadolinium enhancement (LGE) was correlated with the degree of systolic compression (r = 0.33, p = 0.003). Multivariable linear regression analysis revealed that the degree of systolic compression was an independent risk factor for LGE (β = 0.292, p = 0.007). The LGE fraction of basal and mid anteroseptal segments in patients with severe MB (compression ratio ≥ 80%) was significantly greater than that in patients with mild to moderate MB (compression ratio < 80%). During a median follow-up of 28 (IQR: 15-52) months, 15 patients died. Kaplan-Meier analysis did not identify differences in all-cause death (log-rank p = 0.63) or cardiovascular death (log-rank p = 0.72) between patients undergoing MB-related surgery and those without MB.CONCLUSIONS:MB with severe systolic compression was significantly associated with a high extent of fibrosis in patients with obstructive HCM. Concomitant myotomy or coronary artery bypass grafting might provide excellent survival similar to that of patients without MB. Identification of patients with severe MB and providing comprehensive management might help improve the prognosis of patients with HCM.
BMC medicine 2024
Background Sarcomere gene mutation and myocardial fibrosis are both associated with poorer clinical outcomes in patients with hypertrophic cardiomyopathy (HCM). The aim of this study was to determine the relationship between sarcomere gene mutation and myocardial fibrosis measured by both histopathology and cardiac magnetic resonance (CMR). Methods and Results Two hundred twenty-seven patients with HCM who underwent surgical treatment, genetic testing, and CMR were enrolled. We retrospectively analyzed basic characteristics, sarcomere gene mutation, and myocardial fibrosis measured by CMR and histopathology. In our study, the mean age was 43 years, and 152 patients (67.0%) were men. A total of 107 patients (47.1%) carried a positive sarcomere gene mutation. The myocardial fibrosis ratio was significantly higher in the late gadolinium enhancement (LGE)+ group (LGE+ 14.3±7.5% versus LGE- 9.0±4.3%; P=0.001). Patients with HCM with SARC+ showed a high probability of fibrosis both in histopathology (myocardial fibrosis ratio 15.3±8.0% versus 12.4±6.5%; P=0.003) and CMR examination (LGE+ 98.1% versus 84.2%; P<0.001; LGE quantification 8.3% versus 5.8%; P<0.001). Linear regression analysis showed that sarcomere gene mutation (B=2.661; P=0.005) and left atrial diameter (B=0.240; P=0.001) were related factors for histopathological myocardial fibrosis. Also, the myocardial fibrosis ratio was significantly higher in the MYH7 (myosin heavy chain) group (MYH7 18.1±9.6% versus MYBPC3 [myosin binding protein C] 13.1±5.2%; P=0.019). Conclusions Patients with HCM with positive sarcomere gene mutation had a higher myocardial fibrosis extent than patients without mutation, and a significant difference in myocardial fibrosis was also observed between the MYBPC3 and MYH7 groups. In addition, a high consistency was found between CMR-LGE and histopathological myocardial fibrosis in patients with HCM.
Journal of the American Heart Association 2023
Background Obesity is an established cardiovascular risk factor in patients with hypertrophic cardiomyopathy. Postoperative atrial fibrillation (POAF) is one of the most common complications after surgery in patients with obstructive hypertrophic cardiomyopathy (OHCM). We aimed to determine the impact of body mass index (BMI) on the occurrence of POAF in patients with OHCM who underwent septal myectomy. Methods and Results In all, 712 OHCM patients without previous atrial fibrillation who underwent septal myectomy were identified. Patients were stratified into 3 groups based on BMI. Of these, 224 (31.5%) had normal weight (BMI<24 kg/m2), 339 (47.6%) were overweight (BMI, 24 to <28 kg/m2), and 149 (20.9%) were obese (BMI≥28 kg/m2). Overweight and obese patients had increased levels of left atrial diameter (P<0.001) and left ventricular end-diastolic diameter (P<0.001), compared with patients with normal weight. Among 184 patients (25.8%) developing POAF, 32 cases (14.3%) occurred in the normal weight group, 100 cases (29.5%) occurred in the overweight group, and 52 cases (34.9%) occurred in the obese group (P<0.001). Logistic regression analysis indicated that overweight (odds ratio [OR]: 2.161, 95% CI, 1.333-3.503; P=0.002) or obesity (OR, 2.803; 95% CI, 1.589-4.944; P<0.001), age (OR, 1.037; 95% CI, 1.018-1.057; P<0.001), and left atrial diameter (OR, 1.060; 95% CI, 1.027-1.095; P<0.001) were independently associated with the occurrence of POAF in patients with OHCM. Conclusions Overweight and obesity are strong predictors of POAF in patients with OHCM. Strategies aimed at lowering BMI may be a potential way to prevent POAF.
Journal of the American Heart Association 2022
BACKGROUND:Red blood cell distribution width (RDW) is associated with increased morbidity and mortality in several cardiovascular diseases. However, the prognostic significance of RDW in patients with hypertrophic obstructive cardiomyopathy (HOCM) who underwent septal myectomy remains unclear as no studies have been conducted on this topic. This study aimed to assess the prognostic significance of RDW in these patients.METHODS:A total of 867 adults with HOCM who underwent septal myectomy at Fuwai Hospital from 2011 to 2017 were retrospectively studied. All patients were assessed comprehensively, including their medical history, echocardiograms, and blood test results.RESULTS:The median age of patients was 47.9 [interquartile range (IQR), 37.0-56.0] years and 61.5% of patients were men. During a median follow-up period of 32 (IQR, 17-53) months, 26 patients died and 23 had a cardiovascular death during follow-up. Compared to patients in the lowest RDW quartile, those in the highest quartile had a significantly lower 5-year survival free from all-cause and cardiovascular death (95.9% vs. 87.6%, P<0.001; 95.9% vs. 89.9%, P<0.001). Compared with lower RDW, higher RDW was significantly associated with all-cause and cardiovascular death after adjustment for age, sex, body mass index, and relevant clinical risk factors [per RDW standard deviation (SD) hazard ratio (HR) increase =1.76, 95% confidence interval (CI): 1.54-2.05, P<0.001; per RDW SD HR =1.91, 95% CI: 1.63-2.22, P for trend <0.001].CONCLUSIONS:Higher RDW is independently associated with all-cause and cardiovascular death in patients with HOCM after septal myectomy. Therefore, this readily available biomarker could be considered as an additive biomarker for risk stratification in these patients.
Journal of thoracic disease 2021
BACKGROUND:Ventricular late potentials (VLPs) detected by signal-averaged electrocardiogram are considered as an indicator of electrical instability in diseased myocardium. Little information exists about the VLPs and their effects on clinical prognosis in patients with obstructive hypertrophic cardiomyopathy (OHCM) undergoing septal myectomy.METHODS:Patients with OHCM who underwent septal myectomy from January 2019 to December 2019 were prospectively enrolled. All patients underwent signal-averaged electrocardiogram. Patients were subsequently divided into two groups based on the presence or absence of VLPs. All patients were followed up after surgery to obtain information of survival status and adverse events, including all-cause mortality, congestive heart failure requiring hospitalization, new-onset stroke, and unexplained syncope.RESULTS:In total, 128 patients (47.5±12.8 years, 57.8% male) were enrolled. There were 21 (16.4%) individuals in the VLPs-positive group and 107 (83.6%) individuals in the VLPs-negative group. There were no statistically significant differences in non-sustained ventricular tachycardia and late gadolinium enhancement on cardiovascular magnetic resonance images between the two groups. Eight adverse events were reported at the 14.9±4.1 months follow-up. The rates of adverse events in groups of VLPs-positive and VLPs-negative were 23.8% (5/21) and 2.8% (3/107) of cases, respectively. These events included three patients hospitalized for heart failure, two who experienced ischemic strokes, and three with unexplained syncope, respectively. Patients in the VLPs-positive group had a higher risk of adverse events than those in the VLPs-negative group (P<0.001). The presence of positive VLPs (hazard ratio =9.095, 95% confidence intervals: 2.080-39.776, P=0.003) was a strong independent risk factor of adverse events by multivariate Cox regression analysis. Cardiac function Class III or IV, as defined by the New York Heart Association classification, was also an independent risk factor of adverse events (hazard ratio =13.756, 95% confidence intervals: 1.667-113.510, P=0.015).CONCLUSIONS:VLPs may increase the risk of adverse events in patients with OHCM after septal myectomy, which may be used as a screening test for further risk stratification.
Annals of palliative medicine 2021
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.
Journal of cardiac surgery 2021
BACKGROUND:Type 2 diabetes mellitus is common in cardiovascular disease. It is associated with adverse clinical outcomes for patients who had undergone coronary artery bypass and valve operations. The aim of this study was to evaluate the impact of type 2 diabetes mellitus on the midterm outcomes of patients with hypertrophic cardiomyopathy who underwent septal myectomy.METHODS:We retrospectively analyzed the data of 67 hypertrophic cardiomyopathy patients with type 2 diabetes mellitus who underwent septal myectomy from two medical centers in China from 2011 to 2018. A propensity score-matched cohort of 134 patients without type 2 diabetes mellitus was also analyzed.RESULTS:During a median follow-up of 28.0 (interquartile range: 13.0-3.0) months, 9 patients died. The cause of death of all of these patients was cardiovascular, particularly sudden cardiac death in 3 patients. Patients with type 2 diabetes mellitus had a higher rate of sudden cardiac death (4.5% vs. 0.0%, p = 0.04). The Kaplan-Meier survival analysis revealed that the rates of predicted 3-year survival free from cardiovascular death (98.1% vs. 95.1%, p = 0.14) were similar between the two groups. However, the rates of predicted 3-year survival free from sudden cardiac death (100% vs. 96.7%, p = 0.01) were significantly higher in hypertrophic cardiomyopathy patients without type 2 diabetes mellitus than in those with type 2 diabetes mellitus. Furthermore, after adjustment for age and sex, only N-terminal pro-brain natriuretic peptide (hazards ratio: 1.002, 95% confidence interval: 1.000-1.005, p = 0.02) and glomerular filtration rate ≤ 80 ml/min (hazards ratio: 3.23, 95% confidence interval: 1.34-7.24, p = 0.047) were independent risk factors for hypertrophic cardiomyopathy patients with type 2 diabetes mellitus.CONCLUSIONS:Hypertrophic cardiomyopathy patients with and without type 2 diabetes mellitus have similar 3-year cardiovascular mortality after septal myectomy. However, type 2 diabetes mellitus is associated with higher sudden cardiac death rate in these patients. In addition, N-terminal pro-brain natriuretic peptide and glomerular filtration rate ≤ 80 ml/min were independent risk factors among hypertrophic cardiomyopathy patients with type 2 diabetes mellitus.
Cardiovascular diabetology 2020
BACKGROUND:Postoperative atrial fibrillation (POAF) is a common complication in patients with obstructive hypertrophic cardiomyopathy (HOCM) who undergo surgical myectomy. POAF is associated with poor outcome. The role of plasma big endothelin-1 level in predicting atrial fibrillation after surgical septal myectomy in HOCM patients has not well been studied.METHODS:A total of 118 patients with HOCM who underwent surgical septal myectomy were recruited in this study. Plasma big endothelin-1 level was measured. The heart rhythm was continuously monitored during hospital stay. Preoperative, intraoperative, and postoperative variables were collected.RESULTS:POAF developed among 26 of the 118 patients (22%) in this study. Compared with those without POAF, patients with POAF were significantly older (53.5 ± 10.6 vs. 47.3 ± 13.6 years, P = 0.033), more likely to undergo mitral valve surgery (38.5% vs. 18.5%, P = 0.032), and had higher plasma big endothelin-1 levels (0.41 ± 0.19 vs. 0.27 ± 0.14 pmol/l, P = 0.001), longer hospital stay (9.1 ± 3.7 vs. 7.5 ± 2.8 days, P = 0.022), larger preoperative left atria (48.0 ± 5.2 vs. 44.1 ± 5.9 mm; P = 0.003). In the receiver operating characteristic curve analysis, the area under the curve for big endothelin-1 was 0.734 (95% CI, 0.634 to 0.834, P<0.001). In multivariate logistic regression analysis, preoperative big endothelin-1 level (OR 100.7, 95%CI: 5.0-2020.0, P = 0.003) and left atrial diameter (OR 1.106, 95%CI: 1.015-1.205, P = 0.022) were independent predictors of POAF.CONCLUSION:Elevated preoperative plasma big endothelin-1 level is an independent predictor of POAF in HOCM patients undergoing surgical septal myectomy.
BMC cardiovascular disorders 2019
Background The prognostic value of N-terminal pro-brain natriuretic peptide ( NT -pro BNP ) in patients with hypertrophic cardiomyopathy who underwent septal myectomy has not been well studied. Methods and Results We retrospectively evaluated NT -pro BNP levels in 758 patients (46.1±13.8 years; median follow-up, 936 days) who underwent septal myectomy in our center between March 2011 and April 2018. The median NT -pro BNP level was 1450.5 (interquartile range 682.6-2649.5) pg/mL. Overall, 22 (2.9%) patients died during follow-up; of these, 86.4% were cardiovascular deaths. The 3-year survival free from all-cause mortality by tertile was 95.2% (95% CI 91.1% to 97.4%; NT -pro BNP >2080 pg/mL), 98.3% (95% CI 94.6% to 99.5%; NT -pro BNP , 947-2080 pg/mL), and 99.2% (95% CI , 94.4% to 99.9%; NT -pro BNP <947 pg/mL). The 3-year survival rate free from cardiovascular mortality by tertiles was 95.2% in the highest tertile, 98.8% in the middle tertile, and 99.2% in the lowest tertile. Cox regression analysis indicated that Ln( NT -pro BNP ) was a significantly independent predictor of all-cause mortality (hazard ratio 2.380, 95% CI 1.356-4.178, P=0.003) and cardiovascular mortality (hazard ratio 2.788, 95% CI 1.450-5.362, P=0.002). In addition, concomitant coronary artery bypass grafting for coronary artery disease was also an independent predictor of cardiovascular mortality (hazard ratio 5.178, 95% CI 1.597-16.789, P=0.006). Conclusions Increased preoperative NT -pro BNP level is a strong predictor of midterm mortality in patients undergoing septal myectomy.
Journal of the American Heart Association 2019
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.
Heart rhythm 2019
