Objective To assess right ventricular(RV) regional and global function in patients with repaired tetralogy of Fallot (TOF) by speckle tracking imaging (STI).Methods 36 asymptomatic patients with repaired TOF whose postoperative period is more than one year and 35 healthy controls were studied.RV longitudinal peak systolic strain (ε),strain rate (SRs) were measured in RV free wall and interventricular septum(IVS) for basal,mid and apical segments by STI from the apical 4-chamber view,RV global longitudinal peak systolic strain (GLS),strain rate (GLSRs),early diastolic strain rate (GLSRe) and late diastolic strain rate (GLSRa) were measured from the above view.The correlations between RV systolic functional parameters and the postoperative period,degree of pulmonary regurgitation (PR) and QRS duration were explored.Results ①Compared with controls,εand SRs of RV free wall and IVS for most segments,GLS,GLSRs,GLSRe,and GLSRa were significantly impaired in patients with repaired TOF (P ＜ 0.05 for all).Both GLS and GLSRs were lower in postoperative patients with transannular patch than in those with RV out tract patch.②In comparison with controls,GLS,GLSRs,GLSRe and GLSRa were decreased in postoperative patients with mild,mid and severe PR,Whereas,there was no difference in RV global function among the patients with varying degree of PR.③GLSRs of RV was correlated inversely with postoperative period and QRS duration(r1--0.431,P1 =0.009 ; r2 ==-0.469,P2-0.004),RV GLS and GLSRs had no correlation with PR.Conclusions RV regional and global function in patients with late repaired TOF are decreased,STI can early detect right ventricular dysfunction in asymptomatic patients with repaired TOF.The type of operation impacts on late prognosis,RV abnormal deformations are associated with electrical depolarization abnormalities.

OBJECTIVE: To report a patient with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) manifesting as lumbago, hunchback and Parkinson's syndrome. METHODS: A 49-years-old male CADASIL patient was reported. Results of clinical examination, neuroimaging and genetic testing were analyzed. His family members were also subjected to genetic testing. Related literature was reviewed. RESULTS: The patient had no typical symptoms of CADASIL such as headache, repeated stroke, dementia and emotional disorders, but progressive Parkinson's syndrome, late onset lumbago, hunchback, dysphagia, and diplopia. Brain MRI showed left basal ganglia and external capsule lacunar infarction. Genetic testing revealed a point mutation c.1630C>T (p.R544C) in exon 11 of the NOTCH3 gene. A heterozygous mutation was detected in the same gene in his mother, elder sister and younger brother, all of whom showed different clinical phenotypes. CONCLUSION The clinical features of CADASIL are heterogeneous. Lumbago, humpback, and Parkinson's syndrome may be a rare clinical phenotype of CADASIL.
CADASIL ; complications ; genetics ; Humans ; Low Back Pain ; etiology ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Mutation ; Parkinson Disease ; etiology ; Receptor, Notch3 ; genetics

Objective:To evaluate the efficacy of the domestic D-Shant device for the treatment of patients with chronic heart failure (CHF) using echocardiography.Methods:Twenty-four CHF patients who were treated with domestic D-Shant device in Union Hospital, Tongji Medical College, Huazhong University of Science and Technology from September 2020 to December 2021 were enrolled in the study. Pulmonary capillary wedge pressure (PCWP)/ left atrial pressure (LAP), right atrial pressure (RAP), pulmonary artery pressure, interatrial septal gradient pressure, cardiac index and pulmonary/systemic blood flow ratio (Qp/Qs) were measured before and after implantation using right heart catheterization.Left atrial end-diastolic area index (LAEDAI), left atrial end-diastolic volume index (LAEDVI), left ventricular end-diastolic volume index (LVEDVI), left ventricular end-systolic volume index (LVESVI), left ventricular ejection fraction (LVEF), right atrial end-diastolic diameter, right ventricular end-diastolic diameter, tricuspid annular plane systolic excursion (TAPSE), right ventricular tractional area change (RVFAC), device shunt aperture, velocity and pressure, together with mitral and tricuspid regurgitation severity were measured using echocardiography before, and 1 month as well as 3 months after D-Shant device implantation. Clinical data were collected and analyzed including 6-minute walking test (6MWT), New York Heart Association (NYHA) classification and the Kansas City Cardiomyopathy Questionnaire (KCCQ). Spearman correlation analysis was used to determine the relation between the changes in PCWP/LAP as well as echocardiographic parameters before and 3 months after implantation and NYHA classification. Binary Logistic regression analysis was performed to determine the predictive factors of NYHA classification improvement at 3-month follow-up after D-Shant device implantation.Results:①D-Shant devices were successfully implanted in all patients. ②Compared with preoperative values, invasive PCWP/LAP systolic, diastolic and mean pressures, transatrial septal gradient, and pulmonary systolic, diastolic and mean pressures decreased significantly after implantation(all P<0.001); Qp/Qs increased significantly after implantation( P<0.001). ③Compared with preoperative values, TAPSE, RVFAC and pulmonary artery flow velocity increased at 1 month after implantation(all P<0.05), whereas a significant reduction in mitral regurgitation grade, and an increase in LVEF and pulmonary artery flow velocity at 3 months after implantation(all P<0.05). Right atrial end-diastolic diameter, right ventricular end-diastolic diameter, LAEDAI, LAEDVI, LVEDVI, LVESVI, ratio of early to late diastolic peak velocities of mitral inflow(E/A), systolic peak velocity of mitral annulus at septal site(S′), ratio of early diastolic peak velocity of mitral inflow to diastolic peak velocity of mitral annulus(E/e′), pulmonary artery diameter, inferior vena cava diameter and degree of tricuspid regurgitation did not change among before, and 1 month as well as 3 months after implantation. There were no significant changes in the device shunt aperture, velocity and pressure between 1 month and 3 months after implantation(all P>0.05). ④The significant improvements in NYHA classification, KCCQ scores and 6MWT were observed at 1 and 3 months after implantation compared with preoperative values (all P<0.01). ⑤NYHA classification at 3 months after implantation was correlated with LVEF pre-post, PCWP/LAP pre-post, TAPSE pre-post and RVFAC pre-post ( rs=0.738, -0.730, 0.738, 0.723; all P<0.001). Logistic regression analysis showed that LVEF pre-post was an independent predictor for NYHA classification improvement at 3 months after implantation ( OR=0.687, 95% CI=0.475-0.992, P=0.045) . Conclusions:Domestic D-Shant device can effectively improve the cardiac function and clinical symptoms in patients with CHF. Echocardiography is a feasible and effective method to evaluate the benefits of domestic D-Shant device for the treatment of CHF.

Objective:To investigate the effect of preoperative pulmonary hypertension (PH) on right ventricular function in patients with heart transplantation(HTx) one year after surgery.Methods:A total of 120 patients who underwent HTx in Union Hospital, Tongji Medical College, Huazhong University of Science and Technology from January 2017 to January 2020 were retrospectively recruited.According to the mean pulmonary arterial pressure (mPAP) obtained by preoperative right heart catheterization, the research subjects were divided into the pulmonary hypertension group (PH group, n=81) and without pulmonary hypertension group (NPH group, n=39). Conventional echocardiographic indices of right ventricular function such as right ventricular area change (RV-FAC), tricuspid annular plane systolic excursion (TAPSE), tricuspid lateral annular systolic velocity (S′), and two-dimensional speckle tracking imaging (2D-STI) strain parameters including right ventricular global longitudinal strain (RV-GLS), right ventricular free wall longitudinal strain (RV-FWLS) were obtained to assess the right ventricular function of grafted hearts. The echocardiographic parameters one year after the operation of the two groups were analyzed to compare the differences in right ventricular function and their correlation with preoperative mPAP. Results:The grafted heart RV-GLS and RV-FWLS were significantly decreased in the PH group (all P<0.01), while RV-FAC, TAPSE, and S′ were similar between the two groups (all P>0.05). RV-FWLS and RV-GLS correlated with preoperative hemodynamic parameter mPAP( rs=-0.46, -0.54; all P<0.05)while RV-FAC, TAPSE, and S′ were not significantly correlated with mPAP (all P>0.05). Conclusions:Preoperative PH correlates with right ventricular function in HTx patients 1 year after the operation. The absolute values of RV-FWLS and RV-GLS in HTx patients with preoperative PH decrease 1 year after the operation. 2D-STI is more sensitive than conventional echocardiography to monitor the changes in right ventricular function in HTx patients after the operation.

Objective: To report a patient with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) manifesting as lumbago, hunchback and Parkinson’s syndrome. Methods: A 49-years-old male CADASIL patient was reported. Results of clinical examination, neuroimaging and genetic testing were analyzed. His family members were also subjected to genetic testing. Related literature was reviewed. Results: The patient had no typical symptoms of CADASIL such as headache, repeated stroke, dementia and emotional disorders, but progressive Parkinson’s syndrome, late onset lumbago, hunchback, dysphagia, and diplopia. Brain MRI showed left basal ganglia and external capsule lacunar infarction. Genetic testing revealed a point mutation c. 1630C>T (p.R544C) in exon 11 of the NOTCH3 gene. A heterozygous mutation was detected in the same gene in his mother, elder sister and younger brother, all of whom showed different clinical phenotypes. Conclusion The clinical features of CADASIL are heterogeneous. Lumbago, humpback, and Parkinson’s syndrome may be a rare clinical phenotype of CADASIL.

Drug-associated reward memories are conducive to intense craving and often trigger relapse. Simvastatin has been shown to regulate lipids that are involved in memory formation but its influence on other cognitive processes is elusive. Here, we used a mass spectrometry-based lipidomic method to evaluate the impact of simvastatin on the mouse brain in a cocaine-induced reinstatement paradigm. We found that simvastatin blocked the reinstatement of cocaine-induced conditioned place preference (CPP) without affecting CPP acquisition. Specifically, only simvastatin administered during extinction prevented cocaine-primed reinstatement. Global lipidome analysis showed that the nucleus accumbens was the region with the greatest degree of change caused by simvastatin. The metabolism of fatty-acids, phospholipids, and triacylglycerol was profoundly affected. Simvastatin reversed most of the effects on phospholipids induced by cocaine. The correlation matrix showed that cocaine and simvastatin significantly reshaped the lipid metabolic pathways in specific brain regions. Furthermore, simvastatin almost reversed all changes in the fatty acyl profile and unsaturation caused by cocaine. In summary, pre-extinction treatment with simvastatin facilitates cocaine extinction and prevents cocaine relapse with brain lipidome remodeling.

Drug-associated reward memories are conducive to intense craving and often trigger relapse. Simvastatin has been shown to regulate lipids that are involved in memory formation but its influence on other cognitive processes is elusive. Here, we used a mass spectrometry-based lipidomic method to evaluate the impact of simvastatin on the mouse brain in a cocaine-induced reinstatement paradigm. We found that simvastatin blocked the reinstatement of cocaine-induced conditioned place preference (CPP) without affecting CPP acquisition. Specifically, only simvastatin administered during extinction prevented cocaine-primed reinstatement. Global lipidome analysis showed that the nucleus accumbens was the region with the greatest degree of change caused by simvastatin. The metabolism of fatty-acids, phospholipids, and triacylglycerol was profoundly affected. Simvastatin reversed most of the effects on phospholipids induced by cocaine. The correlation matrix showed that cocaine and simvastatin significantly reshaped the lipid metabolic pathways in specific brain regions. Furthermore, simvastatin almost reversed all changes in the fatty acyl profile and unsaturation caused by cocaine. In summary, pre-extinction treatment with simvastatin facilitates cocaine extinction and prevents cocaine relapse with brain lipidome remodeling.
Animals ; Brain ; Cocaine ; Conditioning, Operant ; Extinction, Psychological ; Lipidomics ; Male ; Mice ; Simvastatin/therapeutic use*