Midterm Outcome After Septal Myectomy and Medical Therapy in Mildly Symptomatic Patients With Hypertrophic Obstructive Cardiomyopathy.

Objective: The purpose of this study was mainly to determine the midterm outcome of septal myectomy (SM) and medical therapy (MT) in mildly symptomatic patients (NYHA class II) with hypertrophic obstructive cardiomyopathy (HOCM). Methods: The study cohort consisted of 184 mildly symptomatic patients with HOCM evaluated in Beijing Anzhen Hospital, Capital Medical University between March 2001 and December 2017, including 82 patients in the SM group and 102 patients in the MT group. Overall survival and HCM-related survival were mainly observed. Results: The average follow-up time was 5.0 years. Compared to patients accepting MT, patients treated with SM were associated with comparable overall survival (96.5% and 93.1% vs. 92.9% and 83.0% at 5 and 10 years, respectively; P = 0.197) and HCM-related survival (98.7% and 98.7% vs. 94.2% and 86.1% at 5 and 10 years, respectively; P = 0.063). However, compared to MT, SM was superior at improvement of NYHA class (1.3 ± 0.6 vs. 2.1 ± 0.5, P < 0.001) and mean reduction of resting left ventricular outflow (LVOT) gradient (78.5 ± 18.6% vs. 28.3 ± 18.4%, P < 0.001). Multivariate analysis suggested that resting LVOT gradient in the last clinical examination was an independent predictor of all-cause mortality (HR = 1.017, 95%CI: 1.000-1.034, P = 0.045) and HCM-related mortality (HR = 1.024, 95%CI: 1.005-1.043, P = 0.012) in the entire cohort. Conclusion: Compared with MT, SM had comparable overall survival and HCM-related survival in mildly symptomatic HOCM patients, but SM had advantages on improving clinical symptoms and reducing resting LVOT gradient. Resting LVOT gradient in the last clinical examination was an independent predictor of all-cause mortality and HCM-related mortality.

Outcome of alcohol septal ablation in mildly symptomatic patients with hypertrophic obstructive cardiomyopathy: A comparison with medical therapy.

OBJECTIVE: The aim of this study was primarily to determine efficacy after alcohol septal ablation (ASA) in mildly symptomatic patients (NYHA class II) with hypertrophic obstructive cardiomyopathy (HOCM), as compared to medical therapy. METHODS: This retrospective study included 163 mildly symptomatic patients with HOCM evaluated in Beijing Anzhen Hospital between March 2001 and August 2019, consisting of the medical group (n = 105) and the ASA group (n = 58). All-cause mortality and HCM-related death were mainly observed. RESULTS: Follow-up was completed in 161 patients and the median follow-up was 6.0 years. Compared to medically treated patients, patients post-ASA had comparable survival free of all-cause mortality (98.3% and 95.1% vs. 93.0% and 83.1% at 5 and 10 years, respectively; p = 0.374). Survival free of HCM-related death was also similar between ASA and medical groups (98.3% and 95.1% vs. 94.3% and 86.2% at 5 and 10 years, respectively; p = 0.608). However, compared to medical therapy, ASA had advantages on the improvement of NYHA class (1.4 ± 0.6 vs. 2.1 ± 0.5, p = .000) and lower occurrence of new-onset atrial fibrillation (AF) (7.8% vs. 20.4%, p = .048). Multivariate analysis demonstrated that resting LVOT gradient at the last clinical check-up was an independent predictor of all-cause mortality (HR = 1.021, 95%CI 1.002-1.040, p = .027). CONCLUSION: This registry suggests that mildly symptomatic patients with HOCM treated with ASA have comparable survival to that of medically treated patients, with the improvement of NYHA class and lower occurrence of new-onset AF. All-cause mortality is independently associated with resting LVOT gradient at the last clinical check-up.

Comparison of Clinical Effects between Percutaneous Transluminal Septal Myocardial Ablation and Modified Morrow Septal Myectomy on Patients with Hypertrophic Cardiomyopathy.

BACKGROUND: Percutaneous transluminal septal myocardial ablation (PTSMA) and modified Morrow septal myectomy (MMSM) are two invasive strategies used to relieve obstruction in patients with hypertrophic cardiomyopathy (HCM). This study aimed to determine the clinical outcome of these two strategies. METHODS: From January 2011 to January 2015, 226 patients with HCM were treated, 68 by PTSMA and 158 by MMSM. Both ultrasonic cardiograms and heart functional class were recorded before, after operations and in the follow-up. Categorical variables were compared using Chi-square or Fisher's exact tests. Quantitative variables were compared using the paired samples t-test. RESULTS: Interventricular septal thickness was significantly reduced in both groups (21.27 ± 4.43 mm vs. 18.72 ± 4.13 mm for PTSMA, t = 3.469, P < 0.001, and 21.83 ± 5.03 mm vs. 16.57 ± 3.95 mm for MMSM, t = 10.349, P < 0.001, respectively). The left ventricular outflow tract (LVOT) pressure gradient (PG) significantly decreased after the operations in two groups (70.30 ± 44.79 mmHg vs. 39.78 ± 22.07 mmHg for PTSMA, t = 5.041, P < 0.001, and 74.58 ± 45.52 mmHg vs. 13.95 ± 9.94 mmHg for MMSM, t = 16.357, P < 0.001, respectively). Seven patients (10.29%) in the PTSMA group required a repeat operation in the follow-up. Eight (11.76%) patients were evaluated for New York Heart Association (NYHA) III/IV in the PTSMA group, which was significantly more than the five (3.16%) in the same NYHA classes for the MMSM group at follow-up. Less than 15% of patients in the PTSMA group and none of the patients in the MMSM group complained of chest pain during follow-up. CONCLUSIONS: Both strategies can not only relieve LVOT PG but also improve heart function in patients with HCM. However, MMSM might provide a more reliable reduction in gradients compared to PTSMA.

Transvenous versus open chest lead placement for resynchronization therapy in patients with heart failure: comparison of ventricular electromechanical synchronicity.

BACKGROUND: Transvenous lead placement is the standard approach for left ventricular (LV) pacing in cardiac resynchronization therapy (CRT), while the open chest access epicardial lead placement is currently the most frequently used second choice. Our study aimed to compare the ventricular electromechanical synchronicity in patients with heart failure after CRT with these two different LV pacing techniques. METHODS: We enrolled 33 consecutive patients with refractory heart failure secondly to dilated cardiomyopathy who were eligible for CRT in this study. Nineteen patients received transvenous (TV group) while 14 received open chest (OP group) LV lead pacing. Intra- and inter-ventricular electromechanical synchronicity was assessed by tissue Doppler imaging (TDI) before and one year after CRT procedure. RESULTS: Before CRT procedure, the mean QRS-duration, maximum time difference to systolic peak velocity among 12 left ventricle segments (LV Ts-12), standard deviation of time difference to systolic peak velocity of 12 left ventricle segments (LV Ts-SD), and inter-ventricular mechanical delay (IVMD) in OP and TV group were 166 ± 17 ms and 170 ± 21 ms, 391 ± 42 ms and 397 ± 36 ms, 144 ± 30 ms and 148 ± 22 ms, 58 ± 25 ms and 60 ± 36 ms, respectively (all P > 0.05). At one year after the CRT, the mean QRS-duration, LV Ts-12, LV Ts-SD, and IVMD in TV and OP group were 128 ± 14 ms and 141 ± 22 ms (P = 0.031), 136 ± 37 ms and 294 ± 119 ms (P = 0.023), 50 ± 22 ms and 96 ± 34 ms (P = 0.015), 27 ± 11 ms and 27 ± 26 ms (P = 0.86), respectively. The LV lead implantation procedure time was 53.4 ± 16.3 min for OP group and 136 ± 35.1 min for TV group (P = 0.016). The mean LV pacing threshold increased significantly from 1.7 ± 0.6 V/0.5 ms to 2.3 ± 1.6 V/0.5 ms (P < 0.05) in TV group while it remained stable in the OP group. CONCLUSIONS: Compared to conventional endovascular approach, open chest access of LV pacing for CRT leads to better improvement of the intraventricular synchronization.

SYNTAX Score-II Predicts Long-Term Mortality in Patients Who Underwent Left Main Percutaneous Coronary Intervention Treated With Second-Generation Drug-Eluting Stents.

The aim of this study was to evaluate the capacity of the SYNTAX Score-II (SS-II) to predict long-term mortality in patients undergoing left main percutaneous coronary intervention (LM-PCI) treated with second-generation drug-eluting stents (DES).Data from 487 consecutive patients with de novo left main coronary artery disease undergoing PCI were retrospectively studied. The patients were divided into tertiles according to the SS-II: low SS-II tertile (SS-II ≤ 22), intermediate SS-II tertile (SS-II of 23 to 30), and high SS-II tertile (SS-II ≥ 30). The survival curves were estimated by the Kaplan-Meier method. Univariate and multivariate Cox proportional hazard regression analyses were performed to evaluate the possible associations between the SS-II and the rates of long-term mortality. The predictive ability of the SS-II for mortality was assessed and compared with the SYNTAX score (SS) alone by an area under the receiver operator curve (AUC).The overall SS-II was 27.3 ± 9.1. At a mean follow-up of 5.1 years, the long-term mortality was 6.0%. The rates of mortality were 2.4%, 3.4%, and 11.6%, respectively (P < 0.0001) in the low, intermediate, and high SS-II tertiles. The cardiac mortality rates were 1.8%, 1.4%, and 8.1%, respectively (P = 0.002) among patients in the 3 groups. By multivariate analysis, SS-II was an independent predictor of the long-term mortality (hazard ratio: 1.56, 95% confidence interval: 1.05 to 2.32; P = 0.03). The AUC demonstrated a substantially higher predictive accuracy of the SS-II for mortality compared with the SS alone (AUC was 0.689 and 0.596, respectively).In patients with LM-PCI treated with a second-generation DES, the SS-II is an independent predictor of long-term mortality and demonstrates a superior predictability compared with the SS alone.

Screening Mutations of MYBPC3 in 114 Unrelated Patients with Hypertrophic Cardiomyopathy by Targeted Capture and Next-generation Sequencing.

Hypertrophic cardiomyopathy (HCM) is a major cause of sudden cardiac death. Mutations in the MYBPC3 gene represent the cause of HCM in ~35% of patients with HCM. However, genetic testing in clinic setting has been limited due to the cost and relatively time-consuming by Sanger sequencing. Here, we developed a HCM Molecular Diagnostic Kit enabling ultra-low-cost targeted gene resequencing in a large cohort and investigated the mutation spectrum of MYBPC3. In a cohort of 114 patients with HCM, a total of 20 different mutations (8 novel and 12 known mutations) of MYBPC3 were identified from 25 patients (21.9%). We demonstrated that the power of targeted resequencing in a cohort of HCM patients, and found that MYBPC3 is a common HCM-causing gene in Chinese patients. Phenotype-genotype analyses showed that the patients with double mutations (n = 2) or premature termination codon mutations (n = 12) showed more severe manifestations, compared with patients with missense mutations (n = 11). Particularly, we identified a recurrent truncation mutation (p.Y842X) in four unrelated cases (4/25, 16%), who showed severe phenotypes, and suggest that the p.Y842X is a frequent mutation in Chinese HCM patients with severe phenotypes.

[Relationship between estimated glomerular filtration rate level and clinical characteristics and outcome in patients with angiographic coronary artery disease and normal serum creatinine].

OBJECTIVE: To explore the relationship between estimated glomerular filtration rate level and clinical characteristics and outcome in coronary artery disease (CAD) patients with normal serum creatinine. METHOD: A total of 548 hospitalized and angiographic CAD patients with normal fasting serum creatinine were enrolled. The kidney function was estimated by using the abbreviated modification of diet in renal disease (MDRD) study equation. Patients were divided into three groups according to eGFR tertiles: high eGFR group (eGFR > 88.15 ml×min(-1)×1.73 m(-2), n = 184); intermediate group ZU(70.30 ml× min(-1)×1.73 m(-2) < eGFR ≤ 88.15 ml×min(-1)×1.73 m(-2), n = 187); low eGFR group (eGFR ≤ 70.30 ml × min(-1)×1.73 m(-2), n = 177). Clinical data and cardiovascular risk factors were recorded after admission and during (14.02 ± 8.31) months follow up. The primary end point was combined major adverse cardiovascular and cerebral events (MACCE) including death, targeted vascular revascularization, non-fatal myocardial infarction, rehospitalization due to unstable angina and heart failure, and transient ischemic attack (TIA) and stroke. RESULTS: Patients in intermediate and low eGFR groups were older, more males, had more severe coronary artery disease, higher level of hsCRP, higher incidence of hypertension, and lower smoking rate than those in high eGFR group (all P < 0.05). A total of 89 MACCE were recorded during follow up. The level of eGFR was significantly lower in patients with MACCE than patients without MACCE [(73.76 ± 19.81) ml×min(-1)×1.73 m(-2) vs. (84.97 ± 23.42) ml×min(-1)×1.73 m(-2), P < 0.05]. Univariate and multivariate Cox regression analysis showed that eGFR was an independent predictor of MACCE in patients with CAD (univariate analysis: RR = 0.99, 95%CI:0.973-0.997, P < 0.05; multivariate analysis: RR = 0.98, 95%CI:0.976-0.998, P < 0.05). Kaplan-Meier survival analysis suggested that patients with low eGFR was linked with a decreased event free survival ratio (log-rank χ(2) = 7.271, P < 0.05). CONCLUSIONS: eGFR level in CAD patients with normal serum creatinine is associated with coronary artery severity, inflammation level and serves as an independent predictor for MACCE in this patient cohort.

[Clinical characterization of patients with Danon disease].

OBJECTIVE: To analyze the clinical characterization of Danon disease caused by the mutation of lysosome-associated membrane protein-2 (LAMP-2) gene. METHODS: The clinical features, serum biochemical index, electrocardiogram and echocardiography data were retrospectively reviewed in 5 patients with genetically confirmed Danon disease. Mean follow-up period was (56 ± 6) months. RESULTS: Five patients including 2 men and 3 women in 2 unrelated families with 2 novel mutations in the exon 3 (c.189-190TGdel) and 8 (c.1205Cdel) of the LAMP-2 gene were identified. All patients had cardiomyopathy, 1 patient (1/5) had skeletal myopathy, and none of the patients had mental retardation. The two male patients presented cardiac symptoms at the age of 9 and 10 years, respectively, and all female patients were asymptomatic. Biochemical analysis showed that serum creatine kinase and liver transaminase enzyme were increased in 2 patients (2/5). Abnormal electrocardiogram was observed in all patients, and 2 patients (2/5) had ventricular preexcitation. During the follow-up. One male patient died of cardiac failure at the age of 18 years and three months, and the symptoms of the other male patients rapidly developed with the evolution from hypertrophic cardiomyopathy into dilated cardiomyopathy. However, all female patients remained asymptomatic, and repeat echocardiography indicated only mild ventricular hypertrophy during follow up. CONCLUSION: Patients with Danon disease mainly present hypertrophic cardiomyopathy, and sometimes presents with skeletal myopathy. The disorder occurs at early, age and progresses quickly and ends with poor prognosis in male patients. Other clinical features include elevations of serum creatine kinase and liver transaminase enzyme, ventricular preexcitation on electrocardiogram, and ventricular hypertrophy detected by echocardiography. Female patients remain asymptomatic till now in our cohort.

Assessment of myocardial fibrosis and coronary arteries in hypertrophic cardiomyopathy using combined arterial and delayed enhanced CT: comparison with MR and coronary angiography.

OBJECTIVES: We sought to determine the feasibility and accuracy of dual-source computed tomography (DSCT) in assessing coronary artery disease and myocardial fibrosis of hypertrophic cardiomyopathy (HCM) compared with cardiac magnetic resonance (CMR) imaging and coronary angiography (CA). METHODS: Forty-seven consecutive patients with HCM were prospectively enrolled. DSCT images were acquired in the arterial and late phases following intravenous contrast medium. The CMR and CA were performed within 7 days. Independent blinded readers read each study. Patients were classified according to myocardial delayed enhanced (MDE) CMR, coronary artery stenosis by CA, and arterial and MDE-DSCT. The diagnostic accuracy of DSCT in detecting coronary stenosis and MDE was analysed. RESULTS: Wall thickness determined by DSCT was strongly correlated with MR results (r = 0.91). DSCT and CMR MDE showed substantial agreement for the detection of myocardial fibrosis on per-patient and per-segment levels. The CT classification of patients by arterial stenosis and delayed enhancement had excellent agreement with MR and CA methods. CONCLUSIONS: The comprehensive cardiac CT examination provides reliable coronary artery and myocardial assessments. MDE-DSCT is a robust alternative method to MDE-CMR in assessing myocardial fibrosis in HCM particularly in patients with pacemakers or other contraindications to CMR.

[Clinical characteristics of coronary artery disease patients with reduced left ventricular ejection fraction and their prognostic analysis].

OBJECTIVE: To explore the relationship between reduced left ventricular ejection fraction (LVEF) and characteristics of coronary artery disease (CAD) and investigate the association between reduced LVEF and cardiovascular prognosis. METHODS: A total of 677 hospitalized patients with angiographic CAD were enrolled. All patients' clinical data were recorded. LVEF were measured, high sensitive C-reactive protein (hs-CRP), white blood cell (WBC) and classic cardiovascular risk factors were recorded after admission. All patients were followed up from admission. The primary end point was combination occurrence of major adverse cardiovascular and cerebral events (MACCE), including death, targeted vascular revascularization, non-fatal myocardial infarction and rehospitalization due to unstable angina or heart failure, transient ischemic attack or stroke. RESULTS: All patients were tracked for (15±12) months, and patients were divided into normal LVEF group (LVEF≥0.50, n=585) and reduced LVEF group (LVEF<0.50, n=92) according to LVEF level. Compared with normal LVEF group, reduced LVEF group had more severe coronary stenosis (Gensini score: 62.85±41.45 vs. 47.68±33.26, P<0.05), a higher level of WBC and hs-CRP (WBC: 7.60±2.71 ×10(9)/L vs. 7.09±2.13 ×10(9)/L, hs-CRP: 5.68±3.97 mg/L vs. 3.97±3.75 mg/L, both P<0.05). A total of 146 MACCE occurred during follow-up periods. Compared with no-MACCE group, LVEF levels were significantly lower in MACCE group (0.576±0.113 vs. 0.603±0.101) and there were a higher level of hs-CRP and Gensini score in MACCE group (hs-CRP: 5.26±3.99 mg/L vs. 3.91±3.72 mg/L, Gensini score: 53.72±35.50 vs. 48.63±34.59, all P<0.05). Moreover, both of univariate and multivariate Cox regression analysis indicated LVEF be an independent predictor of MACCE in patients with CAD [univariate: relative risk (RR)=0.974, 95% confidence interval (95%CI) 0.960 to 0.988, P=0.000; multivariate: RR=0.979, 95%CI 0.961 to 0.998, P=0.033]. Kaplan-Meier analysis suggested that patients with reduced LVEF had an increased MACCE occurrence (χ(2)=14.56, P<0.05). CONCLUSION: LVEF level may be associated with coronary artery severity, and could be independently predict the prognosis of CAD.

Successful treatment with biventricular pacing in a patient with hypertrophic obstructive cardiomyopathy.

We report the effects of biventricular pacing in a patient with hypertrophic obstructive cardiomyopathy (HOCM) refractory to medical therapy. A 58-year-old man with HOCM had suffered from dyspnea, chest pain and palpitation for 5 years. Cardiac catheterization showed a left ventricular outflow tract (LVOT) gradient of 80 mmHg. He refused septal myomectomy and the septal ablation was not available. Based on intraoperative pressure measurements, he was implanted with biventricular pacing and LVOT gradient decreased to 10 mmHg. During the follow-up period of 6 months, the patient's symptoms were markedly improved. Biventricular pacing may be an alternative therapy for patients with HOCM.

[Efficacy of minimally invasive left ventricular epicardial lead placement for cardiac resynchronization therapy].

OBJECTIVE: Ventricular resynchronization might be achieved via minimally invasive left ventricular epicardial lead placement. METHOD: Six patients with congestive heart failure underwent minimally invasive left ventricular epicardial lead placement after failed coronary sinus cannulation were followed up for 1 year, cardiac function and LV lead threshold were evaluated. RESULTS: There were no in-hospital deaths, intraoperative complications and diaphragm stimulation. Correct lead positioning was achieved in all 6 patients. LV lead thresholds remained unchanged [(1.2 ± 0.5) V vs (1.1 ± 0.4) V, P = 0.68] at 12 months follow-up. Improvements on 6 min walking test [(327 ± 77) m vs (267 ± 68) m, P = 0.001], LVEF [(26.1 ± 6.0)% vs (38.2 ± 4.7)%, P = 0.004], and NYHA functional class were evidenced at 12 months follow-up. CONCLUSION: Minimally invasive left ventricular epicardial lead placement is a safe and reliable technique and should be considered as an alternative option in case of difficult coronary venous anatomy and inability to position the lead for resynchronization therapy.

Myocardial fibrosis in desmin-related hypertrophic cardiomyopathy.

Desmin-related myopathy (DRM) is known to cause different types of cardiomyopathy. Late gadolinium enhancement cardiovascular magnetic resonance (CMR) has been shown to identify fibrosis in ischemic and non-ischemic cardiomyopathies. We present a rare case of desmin-related hypertrophic cardiomyopathy, CMR revealed fibrosis in the lateral wall of the left ventricle. CMR is superior to conventional echocardiography for the detection of myocardial fibrosis in desmin-related cardiomyopathy, which may be useful to detect early cardiac involvement and predict the patient prognosis.

[Clinical characteristics and desmin mutations in patients with desminopathy associated cardiomyopathy from 5 Chinese families].

OBJECTIVE: To investigate the clinical and myopathological characteristics and desmin mutations in patients with desminopathy associated cardiomyopathy from 5 Chinese families. METHODS: Thirty-six individuals (18 male, 18 female) were from 4 autosomal dominant inherited families and 1 sporadic case. Nineteen patients manifested myopathy followed by cardiomyopathy; 13 patients presented with isolated cardiomyopathy; 1 patient had isolated myopathy; 3 patients died of cardiac diseases without detailed clinical information. Out of the 23 patients underwent electrocardiogram examinations, 20 patients showed kinds of abnormalities in cardiac conduction block. Echocardiogram revealed dilated cardiomyopathy in one case, hypertrophic cardiomyopathy in one case, and restrictive cardiomyopathy in two cases. Muscle specimens from 7 different patients were performed for histological, immunohistochemistry and ultrastructural examinations. All exons of the desmin gene were screened in 21 patients, 17 asymptomatic family individuals and 50 Chinese controls. RESULTS: Muscle biopsies revealed multiple proteins aggregated in muscle fibers, also supported by immunostaining and electroscopic examinations. Five novel heterogeneous mutations were identified in 4 families and one sporadic case. CONCLUSIONS: Novel mutations of desmin gene were linked with cardiomyopathy in patients from 5 Chinese families with desminopathy.

[Cardiac manifestations of Fabry's disease].

OBJECTIVE: Fabry' s disease is a rare X-linked recessive disease. Its cardiac manifestations are not well recognized. METHODS: The data of 3 patients from different Chinese kindreds with Fabry's disease and cardiac manifestations who seeked medical advice in our department in 2007 were analyzed. The age, sex, family history, main symptoms, ECG and echocardiographic findings were recorded for all the patients. The diagnostic criteria of Fabry's disease was based on alpha-galactosidase (alpha-GAL) quantity in white blood cells. RESULTS: All of the patients were female. Their age was from 41 to 57. Two of them had the typical symptoms of Fabry's disease in their young age. All of them had family history of the disease and cardiac symptoms. ECG showed ST-T change and echocardiography showed hypertrophy of left ventricule of different degrees. Their alpha-galactosidase level in white blood cells was lower than normal. The alpha-galactosidase level in patient 1 was the lowest. Her cardiac symptoms were most serious in the three patients and she had involvement of other organs. CONCLUSION: Patients with Fabry's disease may have cardiac manifestations. Family history, typical symptoms in young age and the characteristics of multisystemic disorder are helpful clues to the diagnosis.

[The clinical characteristics of 5 patients with inherited hypertrophic cardiomyopathy].

OBJECTIVE: To explore the clinical characteristics of patients with inherited hypertrophic cardiomyopathy. METHODS: The clinical characteristics, electrocardiogram, serum chemistry and diagnostic methods were retrospectively investigated in 5 patients with inherited hypertrophic cardiomyopathy. RESULTS: The electrocardiograms of all patients were abnormal, with prominent left ventricular voltage and ST-T changes. One male patient with clinicopathological features of early onset, muscle weakness, ventricular preexcitation, elevations of two serum proteins and intracytoplasmic vacuoles containing autophagic material and glycogen in biceps brachial muscle cells was diagnosed Danon's disease. Mitochondrial cardiomyopathy was diagnosed in one male patient with early onset, short PR interval and biopsy findings of ragged-red fibers in biceps brachial muscle. Three patients were diagnosed as Fabry's disease with clinical characteristics including pain and acroparesthesias, angiokeratoma and decrease of alpha-galactosidase A activity. CONCLUSION: Some of the rare inherited hypertrophic cardiomyopathy might easily be clinically misdiagnosed as hypertrophic cardiomyopathy, systemic and careful case history inquiring and specific relevant examinations would help to make the right diagnosis in these patients.

[Serum troponin I level in patients with hypertrophic cardiomyopathy].

OBJECTIVE: To observe serum troponin I (TNI) level in patients with hypertrophic cardiomyopathy (HCM). METHOD: Six hundreds and twelve HCM patients were analyzed prospectively from January 1990 to November 2007.Ultracardiography were detected for all the patients. The diagnostic criteria of HCM is ventricular wall thickness more than 15 mm. Serum TNI level was measured in 116 patients with HCM. Clinical data including age, gender, history, main symptoms, NYHA grade, coronary angiograph, electrocardiogram and echocardiography were compared between patients with normal and increased TNI levels. RESULTS: In 116 patients who detected TNI, 62 of them (53.4%) had a degree higher than normal. The median TNI value of all these patients is 0.07 ng/ml (0 - 4.38 ng/ml). Sixty-nine patients (59.5%) had undergone coronary angiography. Only 9 of them (13.0%) could be diagnosised as coronary heart disease. The TNI values of HCM patients with or without coronary heart disease were similar. The factors related to a higher TNI value included maximal depth of ventricule (P < 0.05), significant T inversion (P < 0.01) and chest pain (P < 0.05). Compared to all the 612 patients, the ones who detected serum TNI were likely to have chest pain (45.7% vs. 34.5%, P < 0.01) and significant T inversion (75.9% vs. 30.1%, P < 0.01). CONCLUSION: Increased serum TNI could be seen in half of HCM patients, especially in those patients with chest pain or significant T inversion. It is therefore important to different these patients from patients with acute coronary syndrome.

[The incidence and predictors of atrial fibrillation in hypertrophic cardiomyopathy].

OBJECTIVE: To observe the incidence and predictors of atrial fibrillation in hypertrophic cardiomyopathy (HCM). METHODS: 612 HCM patients were analyzed prospectively from July 1990 to November 2007. The age, sex, height, weight, medical history, main symptoms and incidence of atrial fibrillation were recorded. RESULTS: The patients' mean age was (47.8 +/- 14.9). 414 patients (67.6%) were male. 377 patients (61.6%) had left ventricular outflow truct obstruction. 94 patients (15.4%) and atrial fibrillation. 43 patients (6.0%) had sustained and 51 patients (9.4%) had paroxysmal. The patients with atrial fibrillation were older in age and were predominantly female. Their medical history were longer, left atrial diameter (LAD) longer and plasma B-type natriuretic peptide (BNP) higher. logistic regression analysis indicated that the medical history (P = 0.012), LAD (P = 0.0001) and BNP (P = 0.017) were the independent predictors of atrial fibrillation in HCM. Atrial fibrillation was accompanied by a decrease in functional status and an increase in risk of stroke. CONCLUSIONS: The incidence of atrial fibrillation in HCM was high. The medical history, LAD and BNP were the independent predictors of its occurrence.