Short term outcomes of extremely low birth weight infants from a multicenter cohort study in Guangdong of China.

With the increase in extremely low birth weight (ELBW) infants, their outcome attracted worldwide attention. However, in China, the related studies are rare. The hospitalized records of ELBW infants discharged from twenty-six neonatal intensive care units in Guangdong Province of China during 2008-2017 were analyzed. A total of 2575 ELBW infants were enrolled and the overall survival rate was 55.11%. From 2008 to 2017, the number of ELBW infants increased rapidly from 91 to 466, and the survival rate improved steadily from 41.76% to 62.02%. Increased survival is closely related to birth weight (BW), regional economic development, and specialized hospital. The incidence of complications was neonatal respiratory distress syndrome (85.2%), oxygen dependency at 28 days (63.7%), retinopathy of prematurity (39.3%), intraventricular hemorrhage (29.4%), necrotizing enterocolitis (12.0%), and periventricular leukomalacia (8.0%). Among the 1156 nonsurvivors, 90.0% of infants died during the neonatal period (≤ 28 days). A total of 768 ELBW infants died after treatment withdrawal, for reasons of economic and/or poor outcome. The number of ELBW infants is increasing in Guangdong Province of China, and the overall survival rate is improving steadily.

Clinical treatment outcomes and their changes in extremely preterm twins: a multicenter retrospective study in Guangdong Province, China. / è¶ æœªæˆç†ŸåŒèƒŽå„¿ä¸´åºŠæ•‘æ²»ç»“å±€åŠå˜åŒ–åˆ†æžï¼šä¸€é¡¹å¹¿ä¸œçœå¤šä¸­å¿ƒå›žé¡¾æ€§ç ”ç©¶.

OBJECTIVES: To investigate the clinical treatment outcomes and the changes of the outcomes over time in extremely preterm twins in Guangdong Province, China. METHODS: A retrospective analysis was performed for 269 pairs of extremely preterm twins with a gestational age of <28 weeks who were admitted to the department of neonatology in 26 grade A tertiary hospitals in Guangdong Province from January 2008 to December 2017. According to the admission time, they were divided into two groups: 2008-2012 and 2013-2017. Besides, each pair of twins was divided into the heavier infant and the lighter infant subgroups according to birth weight. The perinatal data of mothers and hospitalization data of neonates were collected. The survival rate of twins and the incidence rate of complications were compared between the 2008-2012 and 2013-2017 groups. RESULTS: Compared with the 2008-2012 group, the 2013-2017 group (both the heavier infant and lighter infant subgroups) had lower incidence rates of severe asphyxia and smaller head circumference at birth (P<0.05). The mortality rates of both of the twins, the heavier infant of the twins, and the lighter infant of the twins were lower in the 2013-2017 group compared with the 2008-2012 group (P<0.05). Compared with the 2008-2012 group, the 2013-2017 group (both the heavier infant and lighter infant subgroups) had lower incidence rates of pulmonary hemorrhage, patent ductus arteriosus (PDA), periventricular-intraventricular hemorrhage (P-IVH), and neonatal respiratory distress syndrome (NRDS) and a higher incidence rate of bronchopulmonary dysplasia (P<0.05). CONCLUSIONS: There is a significant increase in the survival rate over time in extremely preterm twins with a gestational age of <28 weeks in the 26 grade A tertiary hospitals in Guangdong Province. The incidences of severe asphyxia, pulmonary hemorrhage, PDA, P-IVH, and NRDS decrease in both the heavier and lighter infants of the twins, but the incidence of bronchopulmonary dysplasia increases. With the improvement of diagnosis and treatment, the multidisciplinary collaboration between different fields of fetal medicine including prenatal diagnosis, obstetrics, and neonatology is needed in the future to jointly develop management strategies for twin pregnancy.

Analyses of risk factors and prognosis for new-onset atrial fibrillation in elderly patients after dual-chamber pacemaker implantation.

OBJECTIVE: To retrospectively identify risk factors and the prognosis for new-onset atrial fibrillation (AF) after implantation of dual-chamber pacemakers in elderly patients. METHODS: Consecutive patients aged ≥ 65 years who underwent their first implantation of a dual-chamber permanent pacemaker in Beijing Anzhen Hospital from October 2013 to May 2016 were enrolled. Their complete programming and follow-up data were recorded. Follow-up end points included new-onset AF and major adverse cardiovascular and cerebrovascular events. RESULTS: Altogether, 322 patients were enrolled, with new-onset AF observed in 79 (24.5%) during their follow-up. Multivariable analysis identified four independent predictors of new-onset AF in elderly patients after pacemaker implantation: hypertension (HR = 3.040, 95% CI: 1.09-3.05, P = 0.00); age (HR = 1.966, 95% CI: 1.57-3.68, P = 0.01); left atrial enlargement (HR = 1.645, 95% CI: 1.05-1.25, P = 0.03); high ventricular pacing rate (HR = 1.137, 95% CI: 1.01-1.06, P = 0.01). Univariable analysis indicated that the CHA2DS2-VASc score was also a risk factor for AF (HR = 1.368, 95% CI: 1.178-1.589, P = 0.002), whereas multivariable regression analysis did not. Kaplan-Meier survival analysis showed that the risk for ischemic stroke was significantly higher in the new-onset AF group than in the non-AF group (P < 0.05). CONCLUSION: Hypertension, age, left atrial enlargement, and high ventricular pacing rate were independent predictors of new-onset AF in elderly patients after implantation of a permanent pacemaker. New-onset AF increased the risk for ischemic stroke.

Non-invasive ventilation improves hemorheology status in hypoxemic patients with acute myocardial infarction after PCI.

BACKGROUND: Hypoxemia sometimes occurs in the emergency room in the patients with acute myocardial infarction (AMI) after percutaneous coronary intervention (PCI), even in those with administration of conventional high-flow oxygen inhalation. The objective of the present study was to evaluate the effectiveness of non-invasive ventilation (NIV) in improving blood oxygen content and hemorheology in patients with AMI and hypoxemia. METHODS: This prospective study enrolled 50 consecutive eligible patients with AMI (aged 72.3 ± 9.5 years), who had undergone PCI and been administered high-flow oxygen but still had hypoxemia. Blood was taken before NIV and at 0.5, 1, and 2 h after NIV. Blood gases, hemorheological variables including erythrocyte deformability, erythrocyte aggregation, erythrocyte osmotic fragility, membrane fluidity, and oxidative stress level were measured. RESULTS: Blood PaO2 increased to normal by 1 h after NIV. Assessed hemorheological variables had all improved and plasma malondialdehyde concentration decreased significantly after 2 h of NIV. CONCLUSIONS: Our data suggest that NIV can help to improve blood oxygen content, hemorheological status, and minimize plasma lipid peroxidation injury in hypoxemic patients with AMI who have undergone PCI.

Very Long-term Outcomes and Predictors of Percutaneous Coronary Intervention with Drug-eluting Stents Versus Coronary Artery Bypass Grafting for Patients with Unprotected Left Main Coronary Artery Disease.

BACKGROUND: There are limited data on longer-term outcomes (>5 years) for patients with unprotected left main coronary artery (ULMCA) disease who underwent percutaneous coronary intervention (PCI) in the drug-eluting stents (DES) era. This study aimed at comparing the long-term (>5 years) outcomes of patients with ULMCA disease underwent PCI with DES and coronary artery bypass grafting (CABG) and the predictors of adverse events. METHODS: All consecutive patients with ULMCA disease treated with DES implantation versus CABG in our center, between January 2003 and July 2009, were screened for analyzing. A propensity score analysis was carried out to adjust for potential confounding between the two groups. RESULTS: Nine hundred and twenty-two patients with ULMCA disease were enrolled for the analyses (DES = 465 vs. CABG = 457). During the median follow-up of 7.1 years (interquartile range 5.3-8.2 years), no difference was found between PCI and CABG in the occurrence of death (P = 0.282) and the composite endpoint of cardiac death, myocardial infarction (MI) and stroke (P = 0.294). Rates of major adverse cardiac and cerebrovascular events were significantly higher in the PCI group (P = 0.014) in large part because of the significantly higher rate of repeat revascularization (P < 0.001). PCI was correlated with the lower occurrence of stroke (P = 0.004). Multivariate analysis showed ejection fraction (EF) (P = 0.012), creatinine (P = 0.016), and prior stroke (P = 0.031) were independent predictors of the composite endpoint of cardiac death, MI, and stroke in the DES group, while age (P = 0.026) and EF (P = 0.002) were independent predictors in the CABG group. CONCLUSIONS: During a median follow-up of 7.1 years, there was no difference in the rate of death between PCI with DES implantation and CABG in ULMCA lesions in the patient cohort. CABG group was observed to have significantly lower rates of repeat revascularization but higher stroke rates compared with PCI. EF, creatinine, and prior stroke were independent predictors of the composite endpoint of cardiac death, MI, and stroke in the DES group, while age and EF were independent predictors in the CABG group.

Effect of carbodiimide cross-linking of decellularized porcine pulmonary artery valvular leaflets.

Decellularization provides low immunogenicity and is only slightly subject to calcification in tissue engineering. However, the mechanical properties of the tissues are weakened after decellularization. We adopted cross-linking agent 1-ethyl-3-(3-dimethylaminopropyl) carbodiimide (EDC) to treat decellularized porcine pulmonary artery valvular leaflets to improve their mechanical properties. Twenty porcine pulmonary artery valvular leaflets were divided into three groups: the fresh control group A, group B treated with trypsin and Triton X-100 to remove cells, and group C cross-linked with EDC after decellularization. All samples were evaluated the physical and mechanical properties and were then subcutaneously embedded in rabbits. These valvular leaflets were removed after 1, 2, or 4 weeks and checked for pathological changes. The cells of the valvular leaflets were completely removed. The thickness of the valvular leaflets was thinner in group B than in group A (P<0.01). In the subcutaneous embedding of the group B samples, there was mild immunological response after 1-2 weeks, and parts of the scaffolds were degraded. After 4 weeks, fibroblasts had grown into the scaffolds. In group C, there was an increase in the tensile strength and thermal shrinkage temperature in group C compared with group B (P<0.01). In subcutaneous embedding of the group C samples, there was a mild immunological response after 1-2 weeks. The fibroblasts had grown into the samples. The EDC-based cross-linking procedure can enhance the tensile strength of decellularized pulmonary artery valvular leaflets and both decrease the valvular leaflets' rejection and promote tissue regeneration in vivo.

[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.

A novel surgical procedure: scaffold-pulmonary autograft transplantation.

Mitral valve-related operations are easy to perform and show good results, but to prevent severe thromboembolism or a high ratio of prosthetic valve destruction by tissue, lifetime anticoagulant therapy is essential after the operation. Thus, identifying a new type of surgical procedure and prosthetic valve to cure mitral valve diseases is necessary. Pulmonary valve autograft transplantation (Ross II) with the "top hat" transplantation technique was first reported by Ross DN to cure mitral disease. Because the "top hat" procedure has some shortcomings, we designed the scaffold-pulmonary autograft transplantation procedure and performed animal experiments to confirm the feasibility and effectiveness of the procedure. A total of 13 minipigs, weighing 20-25 kg, were employed as experimental animals to undergo scaffold-pulmonary autograft valve transplantation in our surgical animal lab. The surgical procedure was performed under hypothermic general anaesthesia and extracorporeal circulation (or cardiopulmonary bypass, CPB). Briefly, the chest cave was opened through the left intercostal, the pulmonary valve autograft was harvested during on-pump beating heart, and the pulmonary valve autograft was mounted in a self-made pulmonary valve scaffold and transferred to the mitral valve annulus without removing the mitral instruments. Finally, the outflow tract of the right ventricle was re-established with a pig pulmonary homograft. After finishing data collection, all animals were executed 1 hour after removal from the CPB. For the 13 minipigs that underwent the operation, the CPB time was 182.4 ± 23.4 min. Two of the thirteen cases died of bleeding during the operation and of a post-operative pulmonary embolism, and the remaining eleven survived for one hour. The pressure of the left atrium did not increase significantly (P = 1.00), and the ultrasonic cardiograph (UCG) showed good function of the new mitral valves, with mean ejection fraction (EF) values of 63.6%. The mitral valve orifice areas were 1.10 ± 0.13 cm(2) (pre-operation) and 1.01 ± 0.08 cm(2) (post-operation) (P = 0.013). The function and structure of the new mitral valves were normal. We preliminarily consider scaffold-pulmonary autograft valve transplantation to be a new alternative to cure mitral valve disease, but advanced chronic animal experiments will be needed to confirm the long-term results of the operation. The results showed it could be a new alternative to cure mitral valve disease.

Value of the frontal planar QRS-T angle on cardiac dysfunction in patients with old myocardial infarction.

OBJECTIVES: To explore a method of surface electrocardiogram for assessing cardiac dysfunction in patients with old myocardial infarction. METHODS: 1000 patients with old myocardial infarction in Anzhen hospital were analyzed retrospectively. The planar QRS-T angle was calculated automatically according to QRS-wave and T-wave vectors by the electrocardiogram machine. RESULTS: Among these 1000 patients, 822 (82.2%) were male and 178 (17.8%) were female, the mean age was 59.3±10.5 years (34~89 years). The average planar QRS-T angle was 88.5±50.6°. The planar QRS-T angle and LVEF was negative correlation (r=-0.406, p<0.01), the lower the LVEF, the better the relationship with planar QRS-T angle. QRS-T angle>90° had optimal sensitivity and specificity (76%, 74%) in diagnosis of cardiac dysfunction. CONCLUSION: The planar QRS-T angle and LVEF of patients with old myocardial infarction is negative correlation, the larger the planar QRS-T angle, the lower the LVEF. The planar QRS-T angle may be an easier and more feasible index for assessing cardiac dysfunction in patients with old myocardial infarction.

Anticoagulation management of patients with long-term warfarin therapy after valve replacement during the perioperative period of pacemaker implantation.

BACKGROUND: To explore an optimal management of perioperative anticoagulation for patients with long-term warfarin therapy after valve replacement during the perioperative of pacemaker implantation. METHODS: We retrospectively reviewed consecutive patients undergoing pacemaker implantation who received long-term warfarin therapy after valve replacements at our hospital. They were divided into 3 groups: discontinued group, bridging group and continued group. We analyzed the relationship between different anticoagulation methods during the peri-procedure period and hemorrhage and embolism events. RESULTS: 132 patients were enrolled. There was no significant difference concerning the mean age, sex, concomitant diseases, atrial fibrillation and whether performed pacemaker replacement among 3 groups. The incidence of hematomas was irrespective of the perioperative anticoagulation strategy used (P = 0.125). A strategy involving bridging anticoagulation with therapeutic-dose heparin was associated with an incidence of wound errhysis (P = 0.008). There was no thromboembolism event in these three groups. CONCLUSION: The results showed that there was no significant difference in hematoma rate among continued group, discontinued group, and bridging group, but there was much more wound errhysis in the bridging group. Also, the study shows that if warfarin is continued, it will not increase the risk of bleeding when the International Normalized Ratio (INR) is around 1.7 during the procedure.

[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.

A prospective study to evaluate the efficacy of an intracardiac electrogram-based atrioventricular and interventricular intervals optimization method in cardiac resynchronization therapy.

BACKGROUND: Cardiac resynchronization therapy (CRT) with biventricular pacing improves cardiac function, functional capacity and quality of life in selected patients with heart failure. The current study aimed to evaluate the efficacy of the intracardiac electrogram (IEGM)-based optimization method, QuickOpt(TM), in Chinese patients treated with CRT. METHODS: Aortic time velocity integrals (AVTI) achieved at the sensed atrioventricular (AV), paced AV and interventricular (VV) interval settings recommended by both QuickOpt(TM) and standard echocardiographic optimization were measured in 101 patients. Consistency and the strength of the relationship between the two timing cycle optimization methods were assessed by intra-class correlation coefficient (ICC). RESULTS: The ICC showed good agreement and correlation with what the AVTI achieved at the optimal sensed AV (ICC = 0.9683 (0.9535 - 0.9785)), paced AV (ICC = 0.9642 (0.9475 - 0.9757)) and VV (ICC = 0.9730 (0.9602 - 0.9817)) interval settings determined by the two optimization methods. The average time required by echocardiographic optimization and by QuickOpt(TM) were (78.32 ± 32.40) minutes and (1.98 ± 1.64) minutes respectively (P < 0.0001). CONCLUSION: The QuickOpt(TM) algorithm provides a quicker, simpler and reliable alternative to the standard method for timing cycle optimization.

[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.

[Electrophysiological characterization and efficacy of radiofrequency ablation of focal atrial tachycardia originating from the left atrial appendage].

OBJECTIVE: To analyze the electrophysiological characteristics and efficacy of radiofrequency catheter ablation (RFA) of focal atrial tachycardia (AT) originating from the left atrial appendage (LAA). METHODS: Electrophysiologic study and RFA were performed in 9 patients (4 female) with focal AT originating from the LAA. Atrial appendage angiography was performed to identify the origin of AT. P waves were classified as negative, positive, isoelectric, or biphasic. RESULTS: The mean age was (21 +/- 9) years. AT occurred spontaneously or was induced by isoproterenol infusion rather than programmed extrastimulation and burst atrial pacing. A characteristic P-wave morphology and endocardial activation pattern were observed. Positive P-wave in inferior leads was seen in all patients, upright or biphasic (+/-) component P wave was observed in lead V1, isoelectric component or an upright component P wave with low amplitude ( < 0.1 mV) was seen in lead V2-V6. Earliest endocardial activity occurred at the distal coronary sinus (CS) in all patients. The earliest endocardial activation at the successful RFA site occurred (36.7 +/- 7.9) ms before the onset of P wave. RFA was successful in all 9 patients immediately post procedure. AT reoccurred in 2 patients within 1 month post RFA and AT disappeared post the 2nd-RFA. AT reoccurred in 1 patient and terminated after the 3rd RFA. At the final follow-up (12 +/ 5) months, all 9 patients were free of arrhythmias without antiarrhythmic drugs. CONCLUSIONS: The LAA is an uncommon site of origin for focal AT. The characteristic P wave and activation timing are suggestive for focal AT originating from the LAA. LAA focal ablation is safe and effective for patients with focal AT originating from the LAA.

[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.

[The primary observation on the effect of pravastatin to non-ischemic heart failure].

OBJECTIVE: To investigate the efficacy and safety of adding pravastatin (Pra) on top of standard therapy in non-ischemic heart failure patients. METHODS: A total of 61 patients hospitalized in our hospital from Jan 2005 to Jul 2006 were randomly divided into pravastatin group (Pra 20 mg/d on top of standard therapy, n = 30) and control group (standard therapy, n = 31) and followed 6 months. The changes on cardiac function, flow-mediated vasodilatation (FMD) of brachial artery, plasma TNF-alpha level, liver and kidney function were observed. RESULTS: In Pra treated patients, FMD of brachial artery significantly increased after 3 months treatments and NYHA stage significantly improved, plasma BNP, TNF-alpha levels and left ventricular end-diastolic dimension significantly decreased, LVEF significantly increased significantly 6 months post therapy compared to baseline (all P < 0.01). In control group, the patients' NYHA stage also significantly improved (P < 0.05) and LVEF tended to be higher (P = 0.052) while FMD, plasma BNP and TNF-alpha levels remained unchanged at 6 months post therapy compared to baseline. In Pra group, the level of TC (P < 0.05) and LDL-C (P = 0.051) also significantly decreased while HDL-C remained unchanged 6 months post therapy. One patient in Pra group discontinued the study drug because of anaphylaxis. No event on liver and kidney dysfunction was noticed. CONCLUSION: Pravastatin was effective and safe in treating non-ischemic heart failure patients and can significantly improve left ventricular remodeling, endothelial and cardiac functions as well as reduce the levels of inflammatory factors.