Atorvastatin Ameliorates Doxorubicin-Induced Cardiomyopathy by Regulating the Autophagy-Lysosome Pathway and Its Upstream Regulatory Factor Transcription Factor EB.

ABSTRACT: Doxorubicin is a widely used anticancer drug in clinical practice, and its myocardial toxicity is the main concern in oncotherapy. Statins are commonly used as hypolipidemic drugs. Recent studies have also focused on the effects of statins on autophagy. Autophagy is a process in which cells consume their own cytoplasm or organelles after stimulation and finally degrade the phagosome in the lysosome. Transcription factor EB (TFEB) is the main factor regulating lysosomal gene transcription and function. We found that atorvastatin (ATO) increased TFEB protein levels and the ratio of lysosomal-associated membrane protein 2/LC3B in the myocardial tissue of mice with doxorubicin-induced cardiomyopathy (DIC). Therefore, we speculated that ATO may improve cardiac function in mice with DIC by increasing the expression of TFEB to enhance lysosomal function and autophagy. This study explored the role of TFEB in DIC and the possible mechanism of ATO in improving DIC and used statins to prevent and treat DIC; various dilated cardiomyopathy and heart failure diseases provide more experimental evidence. All relevant data are within the article and its supporting information files.

Atorvastatin Improves Doxorubicin-Induced Cardiac Dysfunction by Modulating Hsp70, Akt, and MAPK Signaling Pathways.

Atorvastatin is a lipid-regulating drug that is commonly used in clinical practice and can stabilize plaques. Increasing evidence shows that statins have anti-heart failure (HF) effects, but their specific mechanism is not clear. The purpose of this study was to investigate the cardioprotective effects of atorvastatin on HF in rats and its mechanism. Continuous intraperitoneal injection of 2.5 mg/kg/w doxorubicin for 6 weeks, with a cumulative dose of 15 mg/kg, was used to induce a rat model of HF. Then, the rats were treated with low-dose atorvastatin, high-dose atorvastatin, or saline for 4 weeks. In the DOX-treated groups, echocardiography showed decreases in left ventricular ejection fraction and fractional shortening and increases in left ventricular end-diastolic diameter and left ventricular posterior wall thickness compared with those in the control group, and increased levels of brain natriuretic peptide and Hsp70 were also found in the doxorubicin-treated groups. Compared with saline intervention, atorvastatin ameliorated left ventricular ejection fraction, fractional shortening, left ventricular end-diastolic diameter, and left ventricular posterior wall thickness (a significant difference was observed only in the high-dose group) and reduced serum brain natriuretic peptide. Hematoxylin and eosin staining showed that atorvastatin ameliorated myocardial injury. The improvement in cardiac function induced by atorvastatin was accompanied by increased Hsp70 expression, decreased p-ERK and p-JNK expression, and a reduction in myocardial fibrosis shown by Masson staining. In addition, atorvastatin had a protective effect on the myocardial apoptosis signaling pathway, with increased p-Akt expression and downregulated cleaved caspase-3 expression, and the reduction in myocardial apoptosis was confirmed by a TUNEL assay. Therefore, our experiments demonstrated that atorvastatin may protect cardiac function by modulating Hsp70, p-Akt, p-ERK, and p-JNK signaling to reduce myocardial fibrosis and myocardial apoptosis.

Ox-LDL induces endothelial cell apoptosis and macrophage migration by regulating caveolin-1 phosphorylation.

Oxidative low-density lipoprotein (ox-LDL) is a risk factor for atherosclerosis. Ox-LDL leads to endothelial injury in the initial stage of atherosclerosis. In this study, we investigated the role of ox-LDL in endothelial injury and macrophage recruitment. We demonstrated that ox-LDL promoted a dose-dependent phosphorylation of caveolin-1 in human umbilical vein endothelial cells. Phosphorylated caveolin-1 increased ox-LDL uptake. Intracellular accumulation of ox-LDL induced NF-κB p65 phosphorylation, promoted HMGB1 translocation from nucleus to cytoplasm and cytochrome C release from mitochondria to cytoplasm, and activated caspase 3, resulting in cell apoptosis. NF-κB activation also facilitated cavolin-1 phosphorylation and HMGB1 expression. In addition, caveolin-1 phosphorylation favored HMGB1 release and nuclear translocation of EGR1. Nuclear translocation of EGR1 contributed to cytoplasmic translocation of HMGB1. The extracellular HMGB1 induced the migration of PMBC-derived macrophages toward HUVECs in a TLR4-dependent manner. Our results suggested that ox-LDL promoted HUVECs apoptosis and macrophage recruitment by regulating caveolin-1 phosphorylation.

[Influence of coronary CT angiography findings on prevention strategies of coronary artery disease].

OBJECTIVE: To explore impact of coronary CT angiography findings on preventive medical treatment and control of coronary artery disease (CAD) risk factors. METHODS: Consecutive patients with atherosclerotic plaque detected by coronary CT angiography were enrolled in our study from September 2013 to December 2014, grouped as <50% stenosis and ≥ 50% stenosis.Baseline and follow-up data were recorded.Comparative analysis was performed both between stenosis groups and pre- and post-CT angiography data.Multivariable Logistic regression were preformed to investigate association between coronary CT angiography findings and subsequent medical therapies. RESULTS: Totally 160 patients were enrolled in our study, 99 were <50% stenosis and 61 were ≥ 50% stenosis.Significant reduction of total cholesterol (5.06 ± 1.04 vs 4.54 ± 1.09 mmol/L, P<0.01), low-density lipoprotein cholesterol (3.16 ± 0.95 vs 2.60 ± 0.88 mmol/L, P<0.01), and triglyceride (1.66 (1.14, 2.28) vs 1.55(1.07, 2.05) mmol/L, P=0.004) were observed Pre- versus post-CT angiography. Compared to patients with <50% stenosis, patients with ≥ 50% stenosis demonstrated more significant reduction with regard to total cholesterol (-0.70 ± 0.94 vs -0.42 ± 0.96 mmol/L, P=0.035) and low-density lipoprotein cholesterol (-0.78 ± 0.99 vs -0.43 ± 0.79 mmol/L, P=0.016). After CT angiography, aspirin (13.8% vs 65.6%, P<0.01) and statin (20.0% vs 71.9%, P<0.01) use were significantly increased, blood pressure medication (53.1% vs 63.1%, P=0.07) use showed no statistical differences. Adjusted for baseline risk factors and pretest medications, CT angiography findings were independently associated with increased post-CT angiography use of aspirin (adjusted OR (95% CI) : 3.58 (1.61-7.99), P=0.002) and statin (adjusted OR (95% CI) : 15.01 (4.40-51.22), P<0.01). CONCLUSION: Coronary CT angiography findings demonstrated direct impact on subsequent medical therapies and control of CAD risk factors, and offered important guidance for prevention strategies of CAD.

[Application feasibility of low tube voltage and lower iodine concentration contrast medium as well as iterative reconstruction in coronary computed tomography angiography].

OBJECTIVE: To investigate the feasibility of using lower iodine concentration (270 mgI/ml) contrast medium, lower X-ray tube voltage (100 kVp) and iterative reconstruction (IR) to reduce both iodine load and radiation dose but keep the image quality of coronary CT angiography (CCTA). METHODS: A total of 80 consecutive patients with suspected coronary artery disease were prospectively assigned to one of two groups via computer central system from January to May 2013. The control group (n = 40) was scanned using dual-source CCTA protocols of 120 kV, 370 mgI/ml Iopromide and filtered back projection reconstruction with a vascular algorithm (B26f). The study group (n = 40) was scanned using 100 kV, 270 mgI/ml Iodixanol and sinogram affirmed iterative reconstruction with a vascular algorithm (I26f). Other scan parameters and contrast injection protocol were similar between the two groups. Attenuation in the ascending aorta and coronary arteries along with image noise were measured. Images were reconstructed, measured and graded, and iodine load and effective radiation dose were calculated. RESULTS: The body mass index ((25.3 ± 3.0) kg/m² vs. (25.4 ± 3.0)kg/m², P = 0.852), image quality scores (4.70 ± 0.52 vs. 4.63 ± 0.59, P = 0.545), mean signal-to-noise ratios (22.2 ± 5.5 vs. 23.6 ± 5.8, P = 0.277), and contrast-to-noise ratios (35.6 ± 17.6 vs. 41.1 ± 17.6, P = 0.163) were similar between the control group and study group. Mean iodine loads were significantly reduced in the study group ((18.49 ± 0.75)g) compared to control group ((25.27 ± 0.94)g), P< 0.001). Mean effective radiation doses were also significantly reduced in the study group ((2.31 ± 0.73) mSv) compared to that in control group ((3.52 ± 1.16) mSv), P< 0.001). CONCLUSION: Use of low X-ray tube voltage and iterative reconstruction allows lower iodine load and effective radiation dose application at CCTA without image quality reduction.

Transcatheter occlusion of azygos/hemiazygos vein in patients with systemic venous collateral development after the bidirectional Glenn procedure.

OBJECTIVES: To elucidate the feasibility and treatment effects of transcatheter occlusion of the azygos/hemiazygos vein in patients with progressive cyanosis after performing the bidirectional Glenn procedure. METHODS: From January 2007 to May 2011, transcatheter closure was performed on 9 patients (7 males and 2 females) aged 5-15 years (median 9 years). RESULTS: A total of 7 azygos veins and 3 hemiazygos veins were occluded successfully. Coils were employed in 4 of the procedures, patent ductus arteriosus (PDA) occluders in 3, atrial septal defect occluders in 2 and a PDA occluder together with coils in 1 procedure. Immediate complete occlusions were achieved in 9 (81.8%) procedures and trivial residual shunt in 2 (18.2%). The femoral artery oxygen saturation (SaO2) rose from 81 [interquartile range (IQR) 75-86%] to 88% (IQR 84-91%). CONCLUSIONS: Patients with superior vena cava azygos vein collateral channels can be treated successfully by transcatheter occlusion with multiple devices. The device for embolization depends on the extent of the severity of the shunt, the size of the lumen of the azygos vein/hemiazygos vein and the candidate location for occlusion.

Association between blood pressure control during aneurysm clipping and functional outcomes in patients with aneurysmal subarachnoid hemorrhage.

Objectives: We explored the relationship between blood pressure variability (BPV) during craniotomy aneurysm clipping and short-term prognosis in patients with aneurysmal subarachnoid hemorrhage to provide a new method to improve prognosis of these patients. Methods: We retrospectively analyzed the differences between patient groups with favorable modified Rankin Scale (mRS ≤ 2) and unfavorable (mRS > 2) prognosis, and examined the association between intraoperative BPV and short-term prognosis. Results: The intraoperative maximum systolic blood pressure (SBPmax, p = 0.005) and the coefficient of variation of diastolic blood pressure (DBPCV, p = 0.029) were significantly higher in the favorable prognosis group. SBPmax (OR 0.88, 95%CI 0.80-0.98) and Neu% (OR 1.22, 95%CI 1.03-1.46) were independent influence factors on prognosis. Patients with higher standard deviations of SBP (82.7% vs. 56.7%; p = 0.030), DBP (82.7% vs. 56.7%; p = 0.030), and DBPCV (82.7% vs. 56.7%; p = 0.030) had more favorable prognosis. Conclusion: Higher SBPmax (≤180 mmHg) during the clipping is an independent protective factor for a 90-day prognosis. These results highlight the importance of blood pressure (BP) control for improved prognosis; higher short-term BPV during clipping may be a precondition for a favorable prognosis.

Hemodynamics, anatomy, and outcomes of quadricuspid aortic valves: Multimodality imaging assessment.

BACKGROUND: Quadricuspid aortic valve (QAV) is a rare congenital heart disease with a limited body of literature. This retrospective cohort study investigates QAV morphology, function, and clinical outcomes. METHODS: Echocardiography was used to assess valvular function. Morphological characteristics such as phenotypes, raphe, regurgitant orifice area (ROA), and aortic dilation (diameter >40 â€‹mm) were assessed by cardiac CT. Patients were followed up for the combined event of all-cause death and aortic valve replacement (AVR). RESULTS: Ninety QAV patients (screened from 322385 CT scans) were included (mean age 55.2 â€‹± â€‹13.6 years, 61.1 â€‹% male). Isolated significant aortic regurgitation (AR) was present in 75.6 â€‹% of patients. The cohort was dominated by type I (four equal leaflets, 37.8 â€‹%) and type II (3 larger and 1 smaller leaflets, 42.2 â€‹%) QAV. Fused raphe was present in 26.7 â€‹% of patients. ROACT was correlated with AR severity and aortic dilation (41.1 â€‹%, n â€‹= â€‹37). Among patients without AVR at baseline (n â€‹= â€‹60), one died and 17 underwent AVR during a median follow-up of 35.0 months (IQR:17.3-62.8). ROACT was associated with an increasing risk of combined event (as a categorical variable with a cut-off of 21.4 â€‹mm2, HR â€‹= â€‹4.25, 95%CI 1.49-12.17, p â€‹= â€‹0.007; as a continuous variable (per mm2 increment), HR â€‹= â€‹1.04, 95%CI 1.01-1.07, p â€‹= â€‹0.003). Additionally, ROACT had incremental prognostic value when added to the AR severity model (area under the receiver-operating characteristic curve increased from 86.8 to 88.4, p â€‹= â€‹0.004). CONCLUSION: QAV is characterized by variable anatomy, progressive AR, concomitant cusp fusion and aortic enlargement. ROACT may be a potential ancillary prognostic marker in patients with QAV.

Assessment of left ventricular myocardial scar in coronary artery disease by a three-dimensional MR imaging technique.

PURPOSE: To evaluate the feasibility of free-breathing three-dimensional (3D) phase sensitive inversion recovery (PSIR) Turbo FLASH late gadolinium enhancement (LGE) magnetic resonance images (MRI) on left ventricular scar in patients with coronary artery disease (CAD) compared with clinically established breathhold two-dimensional (2D) PSIR Turbo FLASH images. MATERIALS AND METHODS: In 58 consecutive patients with confirmed CAD, LGE MRI using the two sequences have been acquired. Image quality was graded on a four-point scale according to the image appearance. Qualitative evaluation including the distribution area and the transmural extent of the scar based on the American Heart Association's (AHA's) 17-segment model was performed in both of 2D and 3D images. The scar volumes were compared quantitatively between 2D and 3D images. RESULTS: A total of 51 individuals were used for final statistical analysis. No differences were noted in image quality (P = 0.80), scar distribution area (P = 0.17), and scar transmural extent (P = 0.20) between 3D and 2D images. There was strong correlation in scar volume between the 3D and 2D results (r = 0.940; P < 0.001; Y = 0.298 + 1.251X, R(2) = 0.876). But the scar volume derived from 3D images was significantly larger than that derived from 2D images (2D versus 3D, 20.08 ± 9.41 cm(3) versus 25.41 ± 12.57 cm(3) , t = -7.60; P < 0.001). The trend toward a larger scar volume identified by 3D method was indicated through Bland-Altman analysis. CONCLUSION: Free-breathing 3D PSIR Turbo FLASH imaging is another feasible method to identify left ventricular myocardial scar in patients with CAD and detects more scar volume compared with breathhold 2D PSIR Turbo FLASH imaging.

Transcatheter closure of coronary artery fistulae: initial human experience with the Amplatzer Duct Occluder II.

OBJECTIVES: To evaluate the efficacy and safety of the new device Amplatzer Duct Occluder II (ADO II) for the closure of coronary artery fistulae (CAF) with coronary anomaly. BACKGROUND: Transcatheter device closure is an alternative treatment for selective patients with CAF. The currently available reports regarding the closure of CAF with the ADO II are limited. METHODS: From April 1, 2011 to July 15, 2012, 5 patients (3 males and 2 females) aged from 3 years to 27 years old (median age 5 years old) underwent CAF closure with the ADO II. The immediate and short-term outcomes were evaluated. RESULTS: ADO II was deployed via the femoral vein (2 cases), femoral artery (1 case), brachial artery (1 case), and radial artery (1 case). There were no complications during all the procedures. The median fluoroscopy and procedural times were 20 and 39 minutes, respectively. Immediate trivial and mild residual shunt was present in one patient, respectively, but disappeared 24 hours after the procedure, and there was no recanalization at a median follow-up of 6 months. CONCLUSIONS: The new device ADO II was safely deployed with complete resolution of CAF shunt with tortuous coronary artery to the drainage. The reduced sheath sizes and softer shape of this device allow for venous or arterial approach. The ADO II might be a preferable alternative device for closure of small-tortuous CAFs.

Varied distributions of late gadolinium enhancement found among patients meeting cardiovascular magnetic resonance criteria for isolated left ventricular non-compaction.

BACKGROUND: Late gadolinium enhancement (LGE) is identified frequently in LVNC. However, the features of this findings are limited. The purpose of the present study was to describe the frequency and distribution of LGE in patients meeting criteria for left ventricular non-compaction (LVNC), as assessed by cardiovascular magnetic resonance (CMR). METHODS: Forty-seven patients (37 males and 10 females; mean age, 39 ± 18 years) considered to meet standard CMR criteria for LVNC were studied. The LGE images were obtained 15 ± 5 min after the injection of 0.2 mmol/kg of gadolinium-DTPA using an inversion-recovery sequence, and analyzed using a 17-segment model. RESULTS: Mean number of non-compacted segments per patient was 7.4 ± 2.5 and the NC:C was 3.2 ± 0.7. Non-compaction was most commonly noted in the apical segments in all patients. LGE was present in 19 of the 47 patients (40%), and most often located in the ventricular septum. The distribution of LGE was subendocardial (n = 5; 6%), mid-myocardial (n = 61; 68%), subepicardial (n = 10; 11%), and transmural (n = 14; 15%) in total of 90 LGE (+) segments. CONCLUSIONS: In patients considered to meet criteria for LVNC, LGE distributions visible were strikingly heterogeneous with appearances potentially attributable to three or more distinct cardiomyopathic processes. This may be in keeping with previous suggestions that the criteria may be of low specificity. Further work is needed to determine whether conditions such as dilated cardiomyopathy, previous myocardidtis or ischaemic heart disease increase the apparent depth of non-compact relative to compact myocardium.

Congenital atresia of the left main coronary artery with noncompaction of the ventricular myocardium in an asymptomatic young child.

Congenital atresia of the left main coronary artery (LMCA) is an extremely rare cardiac anomaly, and no cases have been reported from the mainland of China. The diagnosis for the 20-month-old boy in the reported case highlights the essentiality of comprehensive diagnostic measures. To avoid a misdiagnosis, electrocardiographic and echocardiographic evidence should be vigilantly explored in young children suspected of having dilated cardiomyopathy. This is the first case report of LMCA atresia associated with noncompaction of the left ventricular myocardium.

[Coronary atherosclerosis lesion features in coronary artery disease patients complicating with metabolic syndrome].

PURPOSE: To compare coronary plaque burden, composition, distribution and the degree of coronary artery stenosis in invasive coronary angiography (ICA) diagnosed coronary artery disease (CAD) patients with or without metabolic syndrome (MetS). METHODS: From January 2008 to June 2011, consecutive patients underwent both coronary computed tomography angiography (CCTA) and ICA within three months were enrolled. Patients with history of previous percutaneous coronary interventions (PCI) and coronary artery bypass grafting (CABG) were excluded. Plaque characteristics and maximal luminal diameter stenosis were analyzed on a 16-segment basis as suggested by the American Heart Association classification. RESULTS: The study population consisted of 872 patients [age (60.2 ± 10.0) years, 72.70% males] including 377 patients with MetS and 495 patients without MetS. The median coronary artery calcium score (CACS) was higher in MetS patients than in non-MetS patients [102 (10, 410) vs. 58 (0, 274) , P < 0.01]. Percentage of patients with no coronary artery calcium was significantly lower in MetS group than in non-MetS group [19.63% (74/377) vs. 30.71% (152/495) , P < 0.01], while percentage of patients with severe coronary calcium (CACS ≥ 1000) were significantly higher in MetS than non-MetS group [8.22% (31/377) vs. 4.65% (23/495) , P = 0.03]. The proportion of patients with 1-vessel disease was lower [23.61% (89/377) vs. 36.77% (182/495), P < 0.01], 2-vessel [29.71% (112/377) vs. 22.83% (113/495), P < 0.05] and 3-vessel disease [35.54% (134/377) vs. 24.44% (121/495) , P < 0.01] were higher in MetS group than in non-MetS group. Calcified plaque of LM and the middle and distal coronary artery were significantly higher in MetS group than in non-MetS group (all P < 0.05) . CONCLUSIONS: CAD patients with MetS are associated with severer coronary artery calcium deposition and higher percentage of calcified plaque in the middle and distal coronary arteries and severer obstructive coronary vessels.

Transcatheter closure of perimembranous ventricular septal defect using a novel fully bioabsorbable occluder: multicenter randomized controlled trial.

Although the use of bioabsorbable occluder is expected to reduce the risk of metal occluder-related complications, it has not been approved due to incomplete degradation and new complications. Novel fully bioabsorbable occluders were designed to overcome such limitations. The aim of this study was to investigate the efficacy and safety of a fully biodegradable occluder in patients with ventricular septal defects. 125 patients with perimembranous ventricular septal defect (VSD) larger than 3 mm were screened from April 2019 to January 2020 in seven centers. 108 patients were enrolled and randomized into the bioabsorbable occluder group (n = 54 patients) and nitinol occluder group (n = 54). A non-inferiority design was utilized and all patients underwent transcatheter device occlusion. Outcomes were analyzed with a 24-month follow-up. All patients were successfully implanted and completed the trial. No residual shunt >2 mm was observed during follow-up. Transthoracic echocardiography showed a hyperechoic area corresponding to the bioabsorbable occluder which decreased primarily during the first year after implantation and disappeared within 24 months. Postprocedural arrhythmia was the only occluder-related complication with an incidence of 5.56% and 14.81% for the bioabsorbable and nitinol groups, respectively (P = 0.112). The incidence of sustained conduction block was lower in the bioabsorbable occluder group (0/54 vs. 6/54, P = 0.036) at 24-month follow-up. In conclusion, the novel fully bioabsorbable occluder can be successfully and safely implanted under echocardiography guidance and reduce the incidence of sustained postprocedural arrythmia. The efficacy and safety of this fully biodegradable occluder are non-inferior to that of a traditional nitinol one.

Diagnostic accuracy of dual-source CT coronary angiography with prospective ECG-triggering on different heart rate patients.

OBJECTIVE: To evaluate the diagnostic accuracy of dual-source CT (DSCT) prospective ECG-triggering coronary angiography in patients with different heart rate (HR). METHODS: 103 patients with suspected coronary artery disease underwent DSCT prospective ECG-triggered coronary angiography and invasive coronary angiography (ICA). The patients were grouped by HR during CT scans: low HR (≤60 bpm, n = 34); medium HR (60 < HR ≤ 70 bpm, n = 36) and high HR (>70 bpm, n = 33). The sensitivity and specificity of DSCT in detecting ≥50% stenosis were compared among subgroups where ICA was the gold standard. Image quality was scored using a 4-point scale. RESULTS: A total of 1,580 (95.9%) coronary artery segments were evaluable. Sensitivity and specificity were 82.8% and 98.4%, 88.3% and 98.7%, and 80.3% and 98.6% for different subgroups (all p > 0.05). The overall area under the curve of the receiver-operating characteristic analysis was 0.94. The image quality scores were 3.1 ± 0.3, 3.1 ± 0.3 and 3.0 ± 0.4 for subgroups (p > 0.05). The overall average effective radiation dose was 3.60 ± 1.60 mSv. CONCLUSION: DSCT coronary angiography with prospective ECG-triggering could be just as accurate in patients with medium to high HR compared to those with low HR.

The relative atrial volume ratio and late gadolinium enhancement provide additive information to differentiate constrictive pericarditis from restrictive cardiomyopathy.

BACKGROUND: The differentiation of constrictive pericarditis (CP) from restrictive cardiomyopathy (RCM) is often difficult. This study sought to determine the clinical utility of cardiovascular magnetic resonance imaging (CMR) for differentiating both these disorders. METHODS: Twenty-three patients with surgically documented CP, 22 patients with RCM and 25 normal subjects were included in the study. CMR yielded information about cardiac morphology, function and tissue characteristics. The left (LA) and right atrial (RA) volume was calculated using the area-length method. The relative atrial volume ratio (RAR) was defined as the LA volume divided by RA volume. Receiver operating characteristic curve analysis was used to test the ability of different variables in differentiating CP from RCM. RESULTS: The maximal pericardial thickness in CP patients was significantly larger than in normal subjects and RCM patients. The RA volume index in RCM patients (90.5 ± 35.3 mL/m2) was significantly larger than in CP patients (71.4 ± 15.7 mL/m2, p = 0.006) and normal subjects (38.1 ± 9.0 mL/m2, p < 0.001). The LA volume index in RCM (96.0 ± 37.0 mL/m2) and CP patients (105.6 ± 25.1 mL/m2) was significantly larger than in normal subjects (39.5 ± 9.5 mL/m2, p < 0.001 for all). The RAR in CP patients (1.50 ± 0.29) was significantly larger than in RCM patients (1.12 ± 0.33, p < 0.001) and normal subjects (1.06 ± 0.20, p < 0.001). There were no differences between RCM patients and normal subjects in the RAR (p = 0.452). At a cut-off value of 1.32 for the RAR, the sensitivity was 82.6%, and the specificity was 86.4% in the detection of CP. Septal bounce was identified in 95.7% CP patients, in none of RCM patients and normal subjects. Late gadolinium enhancement (LGE) was present in 31.8% RCM patients and absence in all CP patients and normal subjects. CONCLUSIONS: CMR with LGE and RAR can facilitate differentiation of CP from RCM.

[Clinical features of patients with pulmonary artery hypertension associated with hereditary hemorrhagic telangiectasia].

OBJECTIVE: To investigate the clinical manifestations of patients with pulmonary artery hypertension (PAH) associated with hereditary hemorrhagic telangiectasia (HHT). METHODS: This retrospective analysis summarized the clinical features of 6 patients with PAH associated with HHT hospitalized at department of cardiology in Cardiovascular Institute and Fuwai Hospital between January 2006 and May 2009. RESULTS: The mean age of the 6 patients (3 male) was 34 years (8 - 67 years). Recurrent epistaxis were present in all patients, there were 4 patients with severe PAH and 2 patients with moderate PAH. All of the six patients with PAH associated with HHT were misdiagnosed at the first hospital visit. Clinical symptoms were significantly improved in 4 patients and remained unchanged in 2 patients combined hepatic venous malformation post medical therapy. CONCLUSIONS: Misdiagnosis for patients with PAH associated with HHT is a common phenomenon in daily clinical practice. Patients could benefit from the corresponding medical therapy after the establishment of the correct diagnosis.

[Clinical characteristics of fat replacement of left ventricular myocardium].

OBJECTIVE: To evaluate the clinical characteristics of left ventricular fat replacement. METHODS: We identified 45 patients [28M/17F, mean age (51.9 ± 14.7) years] with left ventricular myocardial fat replacement (CT value ≤ -30 Hu) by cardiovascular CT. RESULTS: Among 45 patients, 25 patients [20M/5F, mean age (61.2 ± 10.4) years]were diagnosed as coronary artery disease (CAD). There was 56%single-vessel disease, 20% double-vessel disease and 24%triple-vessel disease, true left ventricular aneurysm was detected in 3 patients and left ventricular thrombi in 1 patient, the dimension of left ventricle was (54.5 ± 9.4) mm and the LVEF was (51.8 ± 13)% in CAD group. In this group, fat replacement occurred in the region of myocardial infarction and presented as curvilinear band in subendocardial region. The left ventricular wall thickness was lower than 5 mm in 21 cases. The location of fat replacement in CAD group is as follows: apical region in 18 patients, distal septal in 15 patients, distal anterior in 11 patients, mid-septal in 7 patients, mid-anterior in 7 patients and basal in 1 patients. The age of remaining 20 patients (8M/12F) without CAD were (57.8 ± 13.3) years. In the group of non-CAD, dilated cardiomyopathy was diagnosed in 3 patients, atrial septal defect in 1 patient, rheumatic heart disease in 1 patient, there was no structural heart disease in the remaining 15 patients. The dimension of left ventricle was (51.1 ± 9.1) mm and the LVEF was (59.4 ± 13.9)%. In non-CAD group, fat replacement mainly occurred in septal region, presented as curvilinear band in 17 patients and patch in 3 patients. The location of fat replacement in this group is as follows: mid-septal region in 11 patients, distal-septal in 10 patients and apical in 9 patients. The intramural fat replacement was detected in 14 patients: subendocardial fat replacement in 10 patients and both intramural and subendocardial fat replacement in 4 patients. CONCLUSIONS: Left ventricular fat replacement could be documented in CAD patients, non-CAD cardiomyopathy patients and in patients without structural heart disease. Left ventricular fat replacement often positioned in apical region in CAD patients as a consequence of infarct healing while mostly positioned in septal region in non-CAD patients, the definite clinical implication of left ventricular fat replacement in non-CAD patients remains to be clarified.

[Differentiation of true from false left ventricular aneurysm with magnetic resonance imaging in patients after myocardial infarction].

OBJECTIVE: To observe the value of cardiac magnetic resonance imaging (MRI) for differentiation of true from false left ventricular aneurysm in patients after myocardial infraction (MI). METHODS: Twenty-six patients [22 males/4 females, mean age (59.3 ± 9.3) years] with left ventricular aneurysm after MI were imaged with MRI, echocardiography and coronary angiography. The respective findings were compared with surgical pathology results. RESULTS: There were 24 patients with dyspnea and 15 patients with hypertension. LVEF measured by echocardiography was 36.9% ± 9.1% in this patient cohort. Cardiac MRI showed that the left ventricular end diastolic wall thickness was thinner than 5.5 mm in 24 cases, and between 5.5 to 8 mm in 2 cases. The dimension of left ventricle was (67.8 ± 9.3) mm. Dyskinesia presented in 24 cases, and akinesia in 2 cases. Delayed enhancement was shown in all cases by MRI. Cardiac MRI detected left ventricular true aneurysm in 23 cases, false aneurysm in 3 case and left ventricular thrombi in 7 cases. The diagnosis by magnetic resonance imaging corresponded well to pathological findings. Echocardiography misdiagnosed pseudoaneurysm in 1 patient, and failed to detected left ventricular thrombi in 2 cases. CONCLUSION: Cardiac MRI could correctly differentiate true from false left ventricular aneurysm in patients after MI.

[Clinic diagnosis and treatment of patients with Cantrell syndrome].

OBJECTIVE: To analyze the diagnostic feature, treatment and prognosis of patients with Cantrell syndrome. METHODS: Clinical manifestation, diagnosis, operation and follow-up data of 5 patients with Cantrell syndrome were summarized in this retrospective analysis. RESULTS: The age of the 5 patients was 7 days-76 years, definite diagnosis was made in 3 cases and 2 cases presented feature of incomplete Cantrell syndrome. Three patients with full Cantrell syndrome were correctly diagnosed before operation and confirmed by operation. One patient with incomplete Cantrell syndrome (two-vessel stenosis) received bypass surgery. Another asymptomatic patient with incomplete Cantrell syndrome (apical diverticulum of the left ventricle) does not need operation and is under observation. During follow-up, 1 patient died at 60 months after operation and the remaining 4 patients are alive and well. CONCLUSIONS: With the development of modern imaging technology, it becomes easy to make correct diagnose Cantrell syndrome before operation. Prognosis is fine post timely operation and related intervention.