Impact of composite clinical worsening events on outcome of patients with pulmonary arterial hypertension associated with congenital heart disease / 中华心血管病杂志

Objective: To explore the impact of composite clinical worsening (cCW) events and its components on the prognosis of patients with pulmonary arterial hypertension associated with congenital heart disease (CHD-PAH). Methods: This is a retrospective study. Patients who were diagnosed with CHD-PAH in Beijing Anzhen Hospital between January 2007 and July 2018, were included, and their baseline clinical data including demographic, clinical manifestations and New York Heart Association (NYHA) classification were collected retrospectively. All-cause deaths and clinical worsening events were recorded, which included syncope, PAH related hospitalization, NYHA classification deterioration and ≥ 2 PAH related clinical symptoms (dyspnea, hemoptysis, edema, chest pain, palpitations, cyanosis) appearance/progress. Three kinds of cCW events were defined: cCW1 (included PAH related hospitalization, NYHA classification deterioration), cCW2 (increased syncope on the basis of cCW1) and cCW3 (increased ≥ 2 PAH related clinical symptoms appearance/progress on the basis of cCW2). The Kaplan-Meier survival curve was used to analyze the long-term survival of the included patients. Univariate and multivariate Cox regression models were used to evaluate the impact of cCW events and their components on the risk of all-cause mortality. Results: A total of 525 patients with CHD-PAH were included in this study. The median age at diagnosis was 20.7 (11.2, 30.3) years. There were 43.8% children (<18 years), and 68.8% female patients. There were 431 patients (82.1%) with NYHA classification II. A total of 180 patients had PAH symptoms at diagnosis. The median follow-up time was 4.5 (2.6, 6.7) years. Forty-seven patients (9.0%) died during the follow-up period. Survival rates at 1, 5 and 10 years after diagnosis of PAH were 98.0%, 89.9% and 84.4%, respectively. Cox multivariate analysis showed that NYHA classification deterioration (HR=3.901, 95%CI 1.863-8.169, P<0.001), ≥2 PAH symptoms appearance/progress (HR=4.458, 95%CI 1.870-10.625, P<0.001), PAH-related hospitalization (HR=4.058, 95%CI 1.851-8.896, P<0.001) and syncope (HR=11.313, 95%CI 4.860-26.332, P<0.001) were independent predictors of increased risk of death. All 3 kinds of cCW events were significantly associated with the significantly increased risk of death, and cCW2 was highly predictive to increased risk of death (HR=15.476, 95%CI 4.346-37.576, P<0.001). Conclusions: The overall long-term prognosis of CHD-PAH patients in this study is relatively good. cCW events and its components (NYHA classification deterioration, ≥2 PAH symptoms occurrence/worsening, PAH-related hospitalization and syncope) have adverse influence on all-cause death in this patient cohort.

Risk factors for death and the clinical features of different subtypes of patients with pulmonary arterial hypertension related to congenital heart disease / 中华心血管病杂志

Objective: To explore the risk factors for death in patients with pulmonary arterial hypertension related to congenital heart disease (PAH-CHD) and the clinical characteristics of different subtypes in patients with PAH-CHD. Methods: It was a retrospective study. A total of 507 PAH-CHD patients, who were hospitalized in the Department of Pediatric Cardiology of Beijing Anzhen Hospital between September 2005 and May 2019, were included. Patients were divided into 4 subgroups: (1) Eisenmenger syndrome(ES) group. (2) PAH associated with prevalent systemic-to-pulmonary shunts(SP) group. (3) PAH associated with small defects(SD) group. (4) PAH after defect correction(CD) group. According to the complexity of cardiac malformation, patients were divided into simple-medium complex malformation group and complex malformation group. According to the location of shunts, patients were divided into pre-tricuspid group, post-tricuspid group, and mixed group or complex deformity group. Baseline clinical data of patients were collected from the electronic medical record system. Demographic data (age, gender, etc.), percutaneous oxygen saturation(SPO(2)), New York Heart Association(NYHA) cardiac function classification, 6 minutes walking distance(6MWD), and B type natriuretic peptide(BNP), systolic pulmonary arterial pressure(sPAP) estimated by echocardiography and mean pulmonary artery pressure (mPAP), mean right atrial pressure(mRAP), cardiac index(CI), and calculated pulmonary vascular resistance (PVR) estimated by right heart catheterization were compared among various groups. The results of regular follow-up of all enrolled patients were collected, including the status of monotherapy or combination of PAH-targeted drugs during the follow-up period, cardiac-related adverse events(hemopysis, syncope, edema, arrhythmia, etc.) and primary endpoint event(all-cause death) were obtained and analyzed. Risk factors for all-cause death were analyzed using univariate and multivariate Cox regression analysis model. Results: The median age at diagnosis was 23.1(13.9,32.1) years, 345 cases(68.0%) were female. Two hunderds and thirty-five cases(46.4%) were diagnosed with ES; 193 cases(38.1%) were diagnosed with CD, 47 cases (9.3%) were diagnosed with SD. Among them, 32 cases(6.3%) were in the SP group. All 507 patients underwent echocardiography examination, there were significant differences in sPAP among different clinical subgroups(P<0.001). A total of 289 patients(57.0%) received right heart catheterization examination, the results showed that the ES group had the highest mPAP and PVR and the lowest mRAP(all P<0.001), the CD group had the highest mRAP and CI(both P<0.001). The 6MWD in the ES group was significantly shorter than that in the SP, SD, and CD groups(all P<0.001). The proportion of patients with NYHA class Ⅲ/Ⅳ was higher in SD group than in SP group(P<0.001), which was similar between SD, ES and CD groups (P values were 0.077 and 0.072, respectively). At admission, the proportion of patients with NYHA class Ⅰ/Ⅱwas the highest in SP group(96.9% (31/32) ), followed by CD group (85.5%(165/193)) and the ES group(85.1%(200/235)), and the SD group(75.0%(35/47)). The BNP level at admission was also higher in SD group than in SP, ES and CD groups(P<0.001). Of the 507 patients, 379(74.8%) patients received PAH-targeted drug therapy at the last follow-up, and the treatment plan was mainly monotherapy(75.7%(287/379)). The median follow-up time was 3.6(2.0, 5.6) years and 37(7.3%) patients died, including 13 in the CD group, 17 in the ES group, and 7 in the SD group. No deaths occurred in the SP group. Right heart failure was the most common cause of death(11(29.7%)), followed by severe hemoptysis dyspnea(7(18.9%)), sudden cardiac death(6(16.2%)), and pulmonary hypertensive crisis(4(10.8%)). Kaplan-Meier curve showed that survival rates of end-point-free events at 1, 3, 5 and 10 years after diagnosis of PAH were 98.0%, 95.4%, 89.9%, and 84.4%, respectively; there were statistically significant differences in survival among the subgroups(P=0.026); there was no significant difference in the survival rate between the ES group and the CD group(P=0.918), and both were higher than the SD group(P values were 0.011 and 0.013, respectively). Univariate Cox regression analysis showed that NYHA class Ⅲ/Ⅳ and BNP>100 ng/L at admission were the risk factors for all-cause death in patients with PAH-CHD(HR=6.452, 95%CI 3.378-12.346, P<0.001, and HR=2.481, 95%CI 1.225-5.025, P=0.012). Multivariate Cox regression analysis showed that NYHA class Ⅲ/Ⅳ was an independent risk factor for all-cause death in patients with PAH-CHD(HR=4.998, 95%CI 1.246-20.055, P=0.023). Conclusions: PAH-CHD patients with different clinical subtypes have different clinical symptoms, cardiac functional class, hemodynamic characteristics, and mid to long-term survival rates. SP patients have the best prognosis, outcome of ES and CD patients is similar, and SD patients have the worst prognosis. NYHA class Ⅲ/Ⅳ is an independent risk factor for all-cause death in patients with PAH-CHD.

Bizarre parosteal osteochondromatous proliferation and other similar lesions on humerus: a comparative analysis of clinicopathologic and imageologic characteristics / 临床与实验病理学杂志

Purpose To investigate the clinicopathologic and imageologic characteristics of bizarre parosteal osteochondromatous proliferation (BPOP),osteochondroma and chondrosarcoma.Methods One case of BPOP on right humerus,one case of osteochondroma on left humerus,and one case of chondrosarcoma on right humerus were analyzed and compared on their clinical,pathologic and imaging manifestations and followup.Results The patients of BPOP and osteochondroma did not feel pain,but patient with chondrosarcoma felt severe pain.Microscopically,it was composed of different amounts cartilage,bone,and spindle cells with a poorly organized distribution.It appeared bizarre chondrocyte and characterized " blue bone".The mass was arising from the surface of the bone and the lesion was not cortical erosion and continuity with medullary cavity in the imaging findings.BPOP,osteochondroma and chondrosarcoma could be distinguished by the above characteristics.Conclusion BPOP is a rare and benign osteochondroma-like lesion,which may be confused with osteochondroma and chondrosarcoma.BPOP can be diagnosed definitely by combining histology with medical imageology.

Non-invasive assessment of portal hypertension in patients with liver cirrhosis using FibroScan transient elastography / 中华肝脏病杂志

<p><b>OBJECTIVE</b>To investigate the clinical value of FibroScan transient elastography for assessing portal hypertension in liver cirrhosis patients by determining the relationship between the liver or spleen stiffness measurement with the imaging parameters of esophageal varices, portal vein width, spleen volume, and splenic vein width.</p><p><b>METHODS</b>A total of 259 patients with liver cirrhosis underwent FibroScan measurement, ultrasound, computed tomography and routine blood analyses. One-hundred-and-one of those patients also underwent endoscopy to diagnose esophageal varices. Receiver operating characteristic (ROC) curves were generated and the areas under the curves (AUCs) were calculated to assess the accuracy of the FibroScan liver and spleen stiffness measurements to predict esophageal varices. Pearson's correlation analysis was used to assess the relationship between clinical features.</p><p><b>RESULTS</b>The median liver and spleen stiffness of the cirrhotic patients were 24.27 kPa and 44.64 kPa, respectively. Liver and spleen stiffness increased in conjunction with increases in Child-Pugh score. Liver stiffness was positively correlated with spleen stiffness (P less than 0.05). Liver and spleen stiffness were positively correlated with esophageal varices, portal vein width, spleen thickness, spleen volume, and splenic vein width. The correlation of spleen stiffness was higher than that of liver stiffness. Spleen stiffness was also negatively correlated with white blood cell count and platelet count. Liver and spleen stiffness also increased in conjunction with increased severity of esophageal varices. The AUC of spleen stiffness was higher than that of liver stiffness for predicting esophageal varices (0.804 vs. 0.737). The optimal cut-off level of spleen stiffness was 44.5 kPa (sensitivity: 88%; specificity: 68%). The estimated prevalence of esophageal varices was 97.87% and the optimized cut-off level of liver stiffness was 18.0 kPa.</p><p><b>CONCLUSION</b>FibroScan appears to be a clinically valuable non-invasive method to assess portal hypertension in cirrhotic patients. Both liver and spleen stiffness measurements correlated with portal hypertension but the spleen stiffness measurement may be of higher clinical value.</p>

Sinomenine in combination with CsA prolonged survival of rat cardiac allograft / 中华器官移植杂志

0.05).The combined use of sinomenine and CsA could prolong graft survival significantly(groups 4 vs groups 1,2,3,5)(P