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Objectives: To explore weather oxygen uptake efficiency slope (OUES) may predict the prognosis in patients with idiopathic pulmonary arterial hypertension (IPAH). Methods: The consecutive newly diagnosed IPAH patients in our hospital from 2010-11 to 2015-06 were prospectively enrolled and regular follow-up study was conducted to record cardiovascular events (death and lung transplantation). Kaplan–Meier curve, uni- and multivariate Cox regression analysis were performed to assess the survival rate in relevant patients. Results: A total of 210 IPAH patients at the mean age of (32±10) years were finished cardiopulmonary exercise test (CPET) and received regular follow-up study including 159 female. There were 31 patients died and 1 received lung transplantation over 41 months follow-up period. OUES was positively related to peak oxygen uptake (VO2)/body weight (r=0.71, P0.52 L/(min?m2) (41.9% vs 89.8%), P<0.0001.Conclusion: OUES as a submaximal CPET parameter may well predict the prognosis in IPAH patients.
ABSTRACT
Mitochondrial cardiomyopathy (MCM) is a series of myocardial conditions characterized by abnormal heart-muscle structure,function,or both,secondary to genetic defects involving the mitochondrial respiratory chain,in the absence of concomitant coronary artery disease,hypertension,valvular disease,or congenital heart disease. MCM patients typically have hypertrophic or dilated cardiomyopathy. Arrhythmias and left ventricular myocardial noncompaction are less common,and heart failure may occur as the first symptom in some patients. Since MCM patients often have symptoms of multiple organ involvement,the symptoms are not specific and the diagnosis can be difficult. Thus,awareness of this disease must be increased in clinical settings. Treatments for MCM are mostly supportive and nonspecific. In this review,we summarize new advances in the diagnosis and management of MCM,with an improve the clinical management of this disease.
ABSTRACT
Mitochondria plays a key role in providing ATP for the energy-consuming cardiac tissues. Mitochondrial cardiomyopathy is a myocardial condition characterized by abnormal heart structure and/or function secondary to genetic defects involving the mitochondrial respiratory chain. The typical cardiac manifestations of mitochondrial cardiomyopathy include hypertrophic and dilated cardiomyopathy,while left ventricular myocardial noncompaction is less common. Recent research has suggested that most mitochondrial diseases result from mitochondrial DNA mutation,which can be found in genes that encode ancillary proteins needed for genetic transcription (tRNA),in genes that encode subunits of the electron transport chain complexes,or in genes that control the activities of the mitochondria called D-loop zone. However,the exact physiological mechanisms remain unclear. This review summarizes the recent advances in the molecular mechanism of mitochondrial cardiomyopathy.
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Objective:To explore the effect of red blood cell distribution width (RDW) on long-term follow-up study in patients with acute pulmonary thromboembolism (APE). Methods:A total of 214 consecutive patients with the first episode of APE admitted in our hospital from 2009-01 to 2012-12 were enrolled. The patients were divided into 2 groups:RDW≤15%group, n=202 and RDW>15%group, n=12. Baseline RDW was measured at admission, the follow-up study was conducted at 3, 6, 12 months thereafter, and then at once per year. The major primary end point was chronic thromboembolic pulmonary hypertension (CTEPH). The independent predictor for CTEPH occurrence was studied by uni-and multivariate logistic regression analysis and the predictive capability of RDWwas evaluated by ROC curve. Results: All patients ifnished the follow-up study at the mean of (31±17) months. The overall occurrence rate of CTEPH was 7.5% (16/214), which was higher in RDW>15% group than that in RDW≤15% group (33.3% vs 5.9%, P=0.002). Multivariate logistic regression analysis indicated that with adjusted clinical data and other predictors, RDW>15%was still the strong predictor for CTEPH occurrence (OR=7.916, 95%CI 1.474-42.500, P=0.016). Adding RDW to the evaluating model, the predictive capability could be signiifcantly improved by ROC curve (AUC increased from 0.856 to 0.901, P Conclusion: Elevated RDW is the independent predictor for CTEPH occurrence in APE patients, which is helpful to estimate the prognosis and treatment strategy in APE patients.
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Objective: To evaluate cardiopulmonary exercise testing (CPET) on sildenaifl effect for treating the patients with pulmonary arterial hypertension (PAH). Methods: A total of 25 PAH patients received sildenaifl treatment in our hospital from 2012-01 to 2014-01 were enrolled as PAH group, in addition, there were a Control group including 24 healthy subjects. The CPET, echocardiography, NYHA function class, 6-mimute walking distance (6MWD) and plasma levels of NT-proBNP at the baseline, (6-12) months and (13-18) months after sildenaifl treatment were assessed and compared between 2 groups. Results: Compared with Control group, PAH group showed decreased aerobic capacity (peakVO?2, Peak O2pulse) and ventilation efifciency (PETCO2@AT, VE?/VC?O2@AT), allP<0.05. At (8±2) months after sildenaifl treatment, aerobic capacity and ventilation efifciency was improved, meanwhile, NYHA function class, 6MWD and plasma levels of NT-proBNP were improved, allP<0.05. At (16±2) months after sildenaifl treatment, 6MWD was similar,P=0.26, while peak VO?2 and peak O2 pulse were decreased than they were at (8±2) months after sildenaifl treatment,P=0.04 and 0.06; the ventilation efifciency was elevated (as presented by increased VE?/VC?O2@AT and decreased PETCO2@AT,P=0.04 and P=0.04); plasma level of NT-proBNP was increased,P=0.05. Conclusion: CPET can effectively evaluate sildenaifl effect for treating PAH patients and therefore and guide the drugs therapy.
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Objective: To explore the effect of calcium channel blocker (CCB) treatment in patients of idiopathic pulmonary arterial hypertension (IPAH) with positive acute pulmonary vasodilator test, and to compare the hemodynamic differences between the positive and negative patients. Methods: A total of 156 consecutive IPAH patients with acute pulmonary vasodilator test were studied. The patients were divided into 2 groups according to the testing result. Positive group, n=23 and Negative group, n=133. The positive patients were followed up by clinical or telephone visit to investigate their CCB dose, WHO PAH cardiac classiifcation and the survival conditions. Kaplan-meier curve was conducted to analyze the living condition and t test was used to compare the hemodynamic differences between the positive and negative patients. Results: There were 43 male and 113 female patients at the male/female ratio of 1: 2.6, and 14.7% (23/156) positive patients. The average follow-up period for Positive group was (50.9 ± 3.8) months. There were 13 patients using diltiazem with the mean dose of (277 ± 108) mg/d at the range of (90-450) mg/d; 3 patients using amlodipine, 1 with the dose of 15mg/d and 2 with the dose of 7.5mg/d. The 1, 2 and 3 years survival rate for the positive patients were for 91.3%, 86.6% and 79.7% respectively. The mean pulmonary arterial pressure and pulmonary vascular resistance were lower, P=0.000, while the mixed venous oxygen saturation was higher in Positive group than Negative group, P=0.009.The NT-pro BNP level was lower in Positive group, P=0.001. Conclusion: IPAH patients has lower ratio of positive acute pulmonary vasodilator test. The positive patients has the higher 1, 3 and 5 years survival rate and better hemodynamic parameters as the mean pulmonary arterial pressure, pulmonary vascular resistance and better level of NT-pro BNP.