No abstract

No abstract

In women with pulmonary hypertension, mean QTc and QTcd are positively correlated to mean pulmonary arterial pressure and are significantly increased in patients with severe pulmonary hypertension.

Background: Percutaneous transluminal angioplasty and stent implantation for stenotic lesions of renal arteries and other branches of the aorta in Takayasu's arteritis have been reported to show good outcomes. However, this form of therapy has been reported in few cases with pulmonary artery involvement. Hypothesis: The aim of this study was to evaluate the role of this interventional treatment for pulmonary stenosis due to Takayasu's arteritis. Methods: A total of 4 patients (3 female and 1 male, ages 30-40 yrs) with Takayasu's pulmonary arteritis underwent percutaneous transluminal balloon angioplasty and stent implantation and were followed up for 1 to 4 years. Results: One patient underwent balloon angioplasty alone, 3 patients underwent balloon angioplasty and stent implantation. The stenoses were relieved acutely, oxygen saturation improved immediately due to improvement in lung perfusion and relief of dyspnea. The pressure gradient fell from 58.3 ± 8.7 mm Hg to 14 ± 3.2 mm Hg and mean pulmonary arterial pressure decreased from 48.5 ± 12.0 mm Hg to 37.3 ± 6.0 mm Hg. At a follow-up period of 34.5 ± 15.8 months, the patient with balloon angioplasty alone developed a recurrence of symptoms 18 months after the procedure. The other 3 patients continued to be asymptomatic and the stent remained patent without restenosis after the procedure. Conclusion: Percutaneous transluminal angioplasty and stent implantation is a safe and effective treatment in patients with pulmonary stenosis caused by Takayasu's arteritis.

Background: Soluble suppression of tumorigenicity (sST2) has been proposed to be a marker for biomechanical strain and a possible predictor of mortality in patients with chronic heart failure. The use of sST2 in pulmonary arterial hypertension (PAH) has not been well defined. Hypothesis: Plasma sST2 levels may correlate with the disease severity and predict clinical worsening in PAH. Methods: We performed a cohort study of 40 idiopathic PAH patients with data on demographics, exercise capacity, echocardiographic parameters, laboratory tests, hemodynamics, and medications. Plasma sST2 was assessed with the high-sensitivity ST2 ELISA kit at diagnostic catheterization. All patients were followed up from the date of blood sampling. The endpoint was clinical worsening. Results: sST2 was significantly elevated in patients with idiopathic PAH compared with control subjects (28.9 ± 13.9 vs 20.7 ± 7.5 ng/mL, P = 0.003). Pearson correlation analysis revealed that sST2 levels correlated with cardiac index (r = −0.534, P = 0.000) and pulmonary vascular resistance (r = 0.350, P = 0.027), and could reflect disease severity of PAH. After a mean follow-up of 14 ± 5 months, 12 patients showed clinical worsening. Receiver operating characteristic analysis suggested that sST2 levels >31.4 ng/mL discriminated clinical worsening with a sensitivity and specificity of 83.3% and 78.6%, respectively. Kaplan-Meier analysis showed that higher sST2 levels (>31.4 ng/mL) were associated with poor clinical outcomes (P = 0.008). Multivariate Cox regression analysis showed that sST2 was an independent predictor of clinical worsening (hazard ratio: 6.067, 95% confidence interval: 1.317-27.948, P = 0.021). Conclusions: sST2 correlates with disease severity and is a significant predictor of clinical worsening in patients with PAH.

BackgroundFew published studies have evaluated the power of the oxygen uptake efficiency slope (OUES) to predict outcomes in patients with idiopathic pulmonary arterial hypertension (IPAH), who typically die of right‐sided heart failure. Our study sought to evaluate the power of OUES to predict clinical worsening and mortality in patients with IPAH.Methods and ResultsPatients with newly diagnosed IPAH who underwent symptom‐limited cardiopulmonary exercise testing from November 11, 2010, to June 25, 2015, in our hospital were prospectively enrolled and followed for up to 66 months. Clinical worsening and mortality were recorded. A total of 210 patients with IPAH (159 women; mean age, 32±10 years) were studied with a median follow‐up of 41 months. Thirty‐one patients died, 1 patient underwent lung transplantation, and 85 patients presented with clinical worsening. The univariate analysis revealed that OUES, OUESI (OUESI=OUES/body surface area), peak oxygen uptake (normalV˙normalO2), peak normalV˙normalO2/kg, ventilation (normalV˙E)/carbon dioxide output (normalV˙CO2) slope, peak systolic blood pressure, heart rate recovery, pulmonary vascular resistance, cardiac index, N‐terminal prohormone brain natriuretic peptide, and World Health Organization functional class were all predictive of clinical worsening and mortality (all P<0.05). Multivariate analysis demonstrated that OUESI and cardiac index were independently predictive of clinical worsening, and OUESI and N‐terminal prohormone brain natriuretic peptide were independently predictive of mortality. Patients with OUESI ≤0.52 m−2 had a worse 5‐year survival rate than patients with OUESI >0.52 m−2 (41.9% versus 89.8%, P<0.0001).ConclusionsThe OUES, a submaximal parameter obtained from cardiopulmonary exercise testing, provides prognostic information for predicting clinical worsening and mortality in patients with IPAH.

This study measured glucose uptake in the right ventricle (RV) of patients with pulmonary hypertension and investigated the relationship to hemodynamics and survival. Myocardial 18F-fluorodeoxy-glucose (FDG) uptake was measured using single-photon positron emission tomography (SPECT) in 24 patients with idiopathic pulmonary arterial hypertension (IPAH) and 43 patients with congenital heart disease (CHD). In both IPAH and CHD-PAH, RV FDG uptake (RV/LV ratio) was associated with pulmonary vascular resistance (PVR). A second SPECT scan was performed in nine patients after 6 months treatment with sildenafil. PVR decreased from 1683±426 to 1207±383 dyn s-1 cm-5 (P < 0.05) and cardiac index improved from 2.2±0.2 to 2.8±0.5 L/min/m2 (P < 0.01). RV/LV FDG uptake decreased from 1.28±0.32 before treatment to 0.99±0.23 (P < 0.05). Survival in the IPAH group with a baseline RV/LV FDG uptake greater than the median value of 1.20 was significantly lower than that of the group with RV/LV FDG uptake below 1.20 (log-rank test, P < 0.05). In contrast, baseline RV/LV FDG was of little informative value in CHD. FDG uptake by the RV reflects the severity of PVR in PAH. Increased RV FDG uptake is a marker of poor prognosis in IPAH and is reduced in patients receiving effective therapy. It could prove useful in the early clinical assessment of novel therapies for PAH.

Background:The difference in underlying pathophysiology in different congenital heart disease (CHD) may have an influence on clinical outcome. It remains unclear whether the effect of sildenafil on pulmonary arterial hypertension (PAH) varies in different types of CHD. Hypothesis: The potential effect of sildenafil on pulmonary arterial hypertension related to CHD may be associated with shunt location. Methods: In this 12-week, prospective, open label, multicenter trial, 55 patients with CHD were divided into the 3 groups: atrial septal defects group (ASD, n = 15), ventricular septal defects group (VSD, n = 24), and patent ductus arteriosus group (PDA, n = 16). Exercise capacity, hemodynamic parameters, and arterial oxygen saturation were assessed at baseline and after sildenafil therapy (25 mg, 3 times daily). Results: Six-minute walk distance significantly increased from 377.2 ± 68.7 m to 436.0 ± 70.4 m in patients with ASD, from 371.2 ± 66.0 m to 413.7 ± 83.1 m in VSD, and from 384.3 ± 90.2 m to 440.9 ± 71.8 m in PDA (P < 0.01, respectively). Moreover, sildenafil also improved the pulmonary vascular resistance and pulmonary blood flow index in the 3 groups, whereas no significant changes in systemic vascular resistance and systemic arterial pressure were observed. However, arterial oxygen saturation was significantly improved in the ASD group only. The incidence of adverse events was similar among the 3 groups. Conclusions: Sildenafil therapy seems to be effective and safe for PAH secondary to ASD, VSD, and PDA, although some clinical and hemodynamic parameters were changed in a different manner among the 3 groups.