Effects of ambrisentan in treatment of pulmonary arterial hypertension: a pilot study with 15 patients / 中华心血管病杂志

<p><b>OBJECTIVE</b>To investigate the preliminary efficacy and safety of ambrisentan, a selective endothelin receptor antagonist, in patients with pulmonary arterial hypertension (PAH).</p><p><b>METHODS</b>A total of 15 patients with PAH, including 10 patients with idiopathic PAH and 5 patients with associated connective-tissue disease, received 2.5 mg or 5 mg of ambrisentan once daily for 12 weeks. Before and after 12 weeks treatment, 6-minute walk test (6-MWD), WHO functional classification (WHO FC) and N-terminal pro-brain natriuretic peptide (NT-proBNP) were measured.</p><p><b>RESULTS</b>After 12 weeks treatment, the 6-MWD was significantly increased [(376.5 ± 108.2) m vs.(460.3 ± 95.7) m, P = 0.021] and the systolic pulmonary artery was significantly decreased [(85.0 ± 33.3) mm Hg (1 mm Hg = 0.133 kPa)vs. (70.5 ± 30.5) mm Hg,P = 0.015] and NT-proBNP was significantly reduced [892.0 (99.0-2245.0) ng/L vs. 205.0 (56.0-534.0) ng/L, P = 0.026] than before treatment. WHO FC was improved in 4 patients after 12 weeks treatment. No patient was withdrawn from this study for safety reasons.</p><p><b>CONCLUSIONS</b>Ambrisentan treatment can effectively improve the exercise capacity, and reduce systolic pulmonary artery pressure and NT-proBNP in PAH patients. Ambrisentan use is safe and could be well tolerated in Chinese PAH patients.</p>

Clinical characteristics of 161 Chinese patients with idiopathic pulmonary arterial hypertension / 中华心血管病杂志

<p><b>OBJECTIVE</b>To investigate the clinical characteristics of patients with idiopathic pulmonary arterial hypertension (IPAH) in China.</p><p><b>METHODS</b>A total of 161 patients diagnosed as IPAH in Shanghai Pulmonary Hospital from June 2008 to June 2010 were retrospective analyzed.</p><p><b>RESULTS</b>The mean diagnostic age was (33 ± 15) years old and 70.2% patients were female. The median duration from symptoms onset to diagnostic right heart catheterization was 12 months. Incidence of NYHA class III to IV was 56.5% at diagnosis and the mean six minutes walk distance was limited to (398 ± 108) meters. Incidence of mild obstructive, restrictive and diffusing impairment in pulmonary function test was 7.8%, 42.2% and 82.2% patients with IPAH, respectively. Right heart catheterization demonstrated severe elevated mean pulmonary arterial pressure [(63 ± 17) mm Hg (1 mm Hg = 0.133 kPa)] and pulmonary vascular resistance index [(25 ± 12) Wood U/m(2)] in this patient cohort. The response rate of acute pulmonary vasoreactivity testing was 8.7% in this cohort. Compared with non-responders, responders to acute pulmonary vasoreactivity testing were younger and with less severe pulmonary hypertension. Among non-responders, 89% patients were treated by one specific anti-pulmonary arterial hypertension drug and 27% patients received combined anti-pulmonary arterial hypertension medications.</p><p><b>CONCLUSIONS</b>Young female was predominantly involved in patients with IPAH in China. The diagnosis of IPAH is often made at advanced disease stage and majority patients with IPAH received specific anti-pulmonary arterial hypertension therapies in this patient cohort.</p>

Clinical characteristics and survival of patients with pulmonary veno-occlusive disease / 中华心血管病杂志

<p><b>OBJECTIVE</b>To investigate the clinical presentation, diagnosis, treatment and outcome of patients with pulmonary veno-occlusive disease (PVOD).</p><p><b>METHODS</b>Data from patients diagnosed as PVOD from May 2008 to May 2011 in Shanghai Pulmonary Hospital, Tongji University were retrospectively reviewed.</p><p><b>RESULTS</b>During this period, 5 patients [4 female, aged from 12 to 42 (22 ± 12) years old] were diagnosed as PVOD. The durations from symptoms onset to PVOD diagnosis was 2 to 50 (16 ± 20) months and four of them were previously diagnosed as idiopathic pulmonary arterial hypertension. All patients at the time of PVOD diagnosis had a severely impaired WHO pulmonary hypertension functional class (3 in class III and 2 in class IV). Furthermore, all patients characterized by a typical sign of centrilobular ground-glass opacities in high-resolution computed tomography, a markedly reduction of diffusing capacity of the lung for carbon monoxide [(38 ± 12)% of predicted value] in pulmonary functional test and severely compromised cardio-pulmonary hemodynamics identified by right heart catheterization. All patients received conventional and pulmonary arterial hypertension specific therapies, and then followed-up regularly. Up to now, 4 out of 5 patients died due to refractory right heart failure. The durations from symptoms onset to death and from PVOD establish to death were 5 - 65 (27 ± 26) months and 1 - 16 (9 ± 9) months, respectively.</p><p><b>CONCLUSIONS</b>PVOD is a rare and malignant cardio-pulmonary disorder that often be misdiagnosed as idiopathic pulmonary arterial hypertension. Given the poor responses to modern pulmonary arterial hypertension specific therapies, lung transplantation remains the treatment of choice.</p>

Clinical efficacy of intravenous L-carnitine in patients with right-sided heart failure induced by pulmonary arterial hypertension / 中华心血管病杂志

<p><b>OBJECTIVE</b>To investigate the efficacy of L-carnitine in patients with right-sided heart failure induced by pulmonary arterial hypertension (PAH).</p><p><b>METHODS</b>A total of 66 pulmonary arterial hypertension patients (14 idiopathic, 36 congenital heart disease associated and 16 connective-tissue disease associated PAH, WHO heart functional class III, n = 38 or IV, n = 28) were enrolled in this study and divided into control group (26 cases) and L-carnitine group (40 cases). All patients received conventional treatment according to guideline for treatment of right-sided heart failure. The patients in L-carnitine group received 5 g/d L-carnitine intravenously for seven days. Six-minute walking distance, WHO heart functional class, physical examination, and serum markers were evaluated at baseline and 7 days after enrollment.</p><p><b>RESULTS</b>Compared to the baseline, six-minute walking distance was significantly increased (75 m vs. 45 m, P < 0.05), WHO heart functional class significantly improved (improved 2 classes in 16 patients, improved 1 class in 13, no improved in 6, worsen in 5 vs. 3, 8, 9, 6 respectively in the control, P = 0.04), BNP level significant decreased (58.16 ng/L vs. 33.29 ng/L, P = 0.01) and systolic blood pressure significantly increased [8.1 mm Hg vs. 2.4 mm Hg (1 mm Hg = 0.133 kPa), P = 0.03] in L-carnitine group compared with those in control group. No patient was withdrawn from this study for safety reasons.</p><p><b>CONCLUSIONS</b>L-carnitine could improve short-term exercise capacity and WHO heart functional class in right-sided heart failure patients induced by PAH.</p>

Diagnosis value of electrocardiogram in patients with pulmonary artery hypertension / 中华心血管病杂志

<p><b>OBJECTIVES</b>To analyze the diagnostic value of electrocardiogram (ECG) in patients with pulmonary artery hypertension (PAH) confirmed by right-heart catheterization (RHC).</p><p><b>METHODS</b>A total of 64 patients with suspected PAH [sPAP > or = 36 mm Hg (1 mm Hg = 0.133 kPa) estimated by echocardiography] were enrolled in this study. All patients were examined by 12-lead ECG within half an hour before RHC.</p><p><b>RESULTS</b>PAH was excluded in 26 patients and confirmed in 38 patients. ECG analysis showed that S amplitude > 0.21 mV in lead I, QRS axis > 87 degrees , R(V1) + S(V5) > 0.76 mV were good parameters for diagnosing PAH with sensibility and specificity of 89%, 81%; 86%, 92%; 84%, 83%, respectively. QRS axis was positively correlated with mean pulmonary artery pressure (mPAP) (r = 0.75, P < 0.001), R(V1) + S(V5) was positively correlated with pulmonary vascular resistance (PVR) (r = 0.74, P < 0.001), R(V1) + S(V5) and S amplitude in lead I was negatively correlated with cardiac index (CI) (r = -0.62, P < 0.001).</p><p><b>CONCLUSION</b>ECG combined with echocardiography are adequate screening tools to rule out the presence of PAH. QRS axis, R(V1) + S(V5) and S amplitude in lead I were significantly correlated with hemodynamic parameters derived from RHC in PAH patients.</p>

Quantitative evaluation of ischemic myocardium by adenosine tissue Doppler stress echocardiography / 中华心血管病杂志

<p><b>OBJECTIVE</b>To evaluate the value of adenosine tissue Doppler stress echocardiography on ischemic myocardium.</p><p><b>METHODS</b>Routine dosage (140 microgxkg(-1)xmin(-1) IV for 6 min) adenosine stress echocardiography was performed on 40 patients with chest pain for diagnosis of coronary artery disease (CAD). The images of left ventricular myocardial motion were acquired by tissue Doppler imaging (TDI) based on traditional 2D stress echocardiography before and 3 min, 6 min after adenosine stress (GE Vivid 7, USA). The myocardial velocity, strain and strain rate in 16 segments were offline measured and analyzed on ECHOPAC software. The results were compared with that of coronary angiography (CAG).</p><p><b>RESULTS</b>CAG identified 18 CAD and 22 non-CAD patients with 159 ischemic segments and 465 non-ischemic segments. Adenosine significantly increased the systolic velocity (Sm), early diastolic velocity (Em), late diastolic velocity (Am), peak systolic strain (Smax), systolic strain rate (SRs), early diastolic strain rate (SRe) and late diastolic strain rate (SRa) both ischemic and non-ischemic segments (all P < 0.05). The baseline Sm and Em in ischemic segments were significant lower than non-ischemic segments [(3.16 +/- 1.20) cm/s vs (4.03 +/- 1.27) cm/s, P < 0.01; (3.75 +/- 1.67) cm/s vs (4.66 +/- 1.70) cm/s, P < 0.05]. At peak stress the differences in Sm and Em were more significant [(3.98 +/- 1.63) cm/s vs (5.07 +/- 1.52) cm/s; (4.51 +/- 2.32) cm/s vs (6.52 +/- 2.56) cm/s; P < 0.01]. The reductions on Smax and Se were more significant in ischemic segments compared those in non-ischemic segments (16.91% +/- 3.35% vs 19.56% +/- 5.47%, P < 0.01 and 9.53% +/- 2.89% vs 13.06% +/- 4.63%, P < 0.001). The biggest area under curve (AUC) in peak stress was seen in Se by ROC curve analysis (AUC = 0.740, with sensitivity 67% and specificity 83%).</p><p><b>CONCLUSION</b>Parameters derived from TDI offer reliable and accurate information on ischemic myocardium during adenosine stress echocardiography.</p>

Left ventricular dyssynchrony evaluated by echocardiography in chronic heart failure patients with normal and wide QRS duration / 中华心血管病杂志

<p><b>OBJECTIVE</b>The aim of the study is to evaluate the left ventricular (LV) dyssynchrony in chronic heart failure (HF) patients with normal and wide QRS duration.</p><p><b>METHODS</b>Time to peak velocity at peak systolic and early diastolic phase (Ts and Te) were determined in 12 segments of LV by echocardiography (GE Vivid 7) in 54 HF patients (28 with wide and 26 with normal QRS duration) and 15 normal controls to evaluate LV systolic and diastolic dyssynchrony. The risk factors related to LV dyssynchrony were also evaluated.</p><p><b>RESULTS</b>LV end systolic and diastolic volumes were significantly larger and 12 segmental mean Ts and maximal Te difference (Te-diff) were significantly higher in HF patients with wide QRS duration than HF patients with normal QRS duration. Using mean Ts >or= 182 ms as the cut-off value, systolic dyssynchrony was present in 46% HF patients with normal QRS and 71% HF patients with wide QRS. Using Te-diff >or= 79 ms as the cut-off value, diastolic dyssynchrony was seen in 58% HF patients with normal QRS and 89% HF patients with wide QRS. Combined systolic and diastolic dyssynchrony was seen in 31% HF patients with normal QRS and in 64% HF patients with wide QRS. Systolic dyssynchrony was significantly correlated to LV end systolic volume and diastolic dyssynchrony was correlated to end diastolic volume.</p><p><b>CONCLUSION</b>Percentage of LV dyssynchrony was significantly higher in HF patients with wide QRS, especially in HF patients with increased LV end systolic and diastolic volume.</p>