樊朝美
中国医学科学院阜外医院 心血管内科
OBJECTIVE:To investigate the prognostic value of elevated urinary albumin concentration (UAC) in hospitalized acute decompensated heart failure (ADHF) patients.METHODS:We measured UAC at baseline in 1818 hospitalized ADHF patients who were admitted to our Heart Failure Center. All patients were followed up for a median period of 937.5 days. The primary endpoint was a composite of all-cause death or heart transplantation (HTx) or left ventricular assist device (LVAD) implantation.RESULTS:In total, 41.5% of ADHF patients had albuminuria (UAC ≥ 20 mg/L). The median value of UAC was 15.5 mg/L. A total of 679 patients died or underwent HTx/LVAD during follow-up. The median UAC was significantly lower in non-HTx/LVAD survivors (14.3 mg/L) than in those who died or underwent HTx/LVAD (18.0 mg/L, P < 0.001). Compared with patients without albuminuria (reference, n = 1064), those with albuminuria had a 1.47-fold higher risk of all-cause death or HTx/LVAD (95% confidence interval [CI]:1.26-1.71, P < 0.001), with hazard ratios (HRs) of 1.42 (95% CI: 1.21-1.66) and 1.74 (95% CI: 1.33-2.26) in patients with microalbuminuria (20 mg/L ≤ UAC < 200 mg/L, n = 617) and macroalbuminuria (UAC ≥ 200 mg/L, n = 137), respectively (both P < 0.001). After adjustment for significant clinical risk factors, the albuminuria group had a higher risk of primary adverse events than the non-albuminuria group (HR = 1.28, 95% CI: 1.09-1.50, P = 0.003), with HRs of 1.27 [95% CI: 1.07-1.49] and 1.36 [95% CI: 1.01-1.84] in patients with microalbuminuria and macroalbuminuria, respectively (P = 0.006 and P = 0.041). The adjusted risk of primary adverse events also increased with the degree of albuminuria in the test for trend (HR = 1.21, 95% CI: 1.06-1.37, P for trend = 0.004). In the subgroup analysis, albuminuria had a significantly greater prognostic value for patients with left ventricular ejection fraction ≥ 40%, eGFR ≥ 60 mL/min/1.73 m2 , BUN/creatinine ratio ≥ 20 or NT-proBNP < 2098 pg/mL.CONCLUSION:The presence of albuminuria evaluated by UAC predicts adverse clinical outcomes in hospitalized ADHF patients.
ESC heart failure 2021
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.
PloS one 2018
INTRODUCTION:Severe right ventricular hypertrophy (SRVH) is a rare phenotype in hypertrophic cardiomyopathy (HCM) for which limited information is available. This study was undertaken to investigate the clinical, prognostic and genetic characteristics of HCM patients with SRVH.METHODS:HCM with SRVH was defined as HCM with a maximum right ventricular wall thickness ≥10 mm. Whole-genome sequencing (WGS) was performed in HCM patients with SRVH. Multivariate Cox proportional hazards regression models were used to identify risk factors for cardiac death and events in HCM with SRVH. Patients with apical hypertrophic cardiomyopathy (ApHCM) were selected as a comparison group. The clinical features and outcomes of 34 HCM patients with SRVH and 273 ApHCM patients were compared.RESULTS:Compared with the ApHCM group, the HCM with SRVH group included younger patients and a higher proportion of female patients and also displayed higher cardiovascular morbidity and mortality. The multivariate Cox proportional hazards regression models identified 2 independent predictors of cardiovascular death in HCM patients with SRVH, a New York Heart Association class ≥III (hazard ratio [HR] = 8.7, 95% confidence interval (CI): 1.43-52.87, p = 0.019) and an age at the time of HCM diagnosis ≤18 (HR = 5.5, 95% CI: 1.24-28.36, p = 0.026). Among the 11 HCM patients with SRVH who underwent WGS, 10 (90.9%) were identified as carriers of at least one specific sarcomere gene mutation. MYH7 and TTN mutations were the most common sarcomere mutations noted in this study. Two or more HCM-related gene mutations were observed in 9 (82%) patients, and mutations in either other cardiomyopathy-related genes or ion-channel disease-related genes were found in 8 (73%) patients.CONCLUSIONS:HCM patients with SRVH were characterized by poor clinical outcomes and the presentation of multiple gene mutations.
PloS one 2017
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.
Cardiology 2017
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.
Scientific reports 2017
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.
Medicine 2017
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.
Clinical cardiology 2017
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.
Heart and vessels 2016
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.
Cardiology 2016
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.
Journal of interventional cardiology 2016
