The efficacy and safety of bosentan therapy for Chinese patients with idiopathic pulmonary arterial hypertension: an open-label, prospective multicenter study / 中华心血管病杂志

<p><b>OBJECTIVE</b>To investigate the efficacy, safety and tolerance of bosentan, a dual endothelin receptor antagonist, in Chinese patients with idiopathic pulmonary arterial hypertension (IPAH).</p><p><b>METHODS</b>Totally 79 IPAH patients (hemodynamic criteria confirmed by right heart catheterization) were included in this open-label, prospective multicenter study. Patients received 62.5 mg of bosentan twice daily for the first 4 weeks, and then up-titrated to 125 mg twice daily for another 12 weeks. The primary end point was the change in exercise capacity showed by six-minute walk distance (6MWD) from baseline to 16 weeks. Secondary end points included the change in World Health Organization (WHO) functional class, Borg dyspnoea scale and systolic pulmonary artery pressure measured by echocardiography.</p><p><b>RESULTS</b>The 6MWD increased from (343.7 ± 93.7) meters at baseline to (397.5 ± 104.4) meters after 16 weeks (P < 0.01), WHO functional class and Borg dyspnoea scale were also significantly improved after 16 weeks therapy compared to baseline levels (all P < 0.01). Furthermore, the systolic pulmonary artery pressure was significantly decreased from (97.8 ± 25.2) mm Hg (1 mm Hg = 0.133 kPa) to (92.8 ± 29.5) mm Hg (P < 0.05) after 16 weeks bosentan treatment. There was no patient withdrawal from this study for safety consideration.</p><p><b>CONCLUSION</b>Bosentan therapy is well tolerated and can improve the exercise capacity and WHO functional class in Chinese IPAH patients.</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>