Electrical impedance tomography in pulmonary arterial hypertension.

The characterization of pulmonary arterial hypertension (PAH) relies mainly on right heart catheterization (RHC). Electrical impedance tomography (EIT) provides a non-invasive estimation of lung perfusion that could complement the hemodynamic information from RHC. To assess the association between impedance variation of lung perfusion (ΔZQ) and hemodynamic profile, severity, and prognosis, suspected of PAH or worsening PAH patients were submitted simultaneously to RHC and EIT. Measurements of ΔZQ were obtained. Based on the results of the RHC, 35 patients composed the PAH group, and eight patients, the normopressoric (NP) group. PAH patients showed a significantly reduced ΔZQ compared to the NP group. There was a significant correlation between ΔZQ and hemodynamic parameters, particularly with stroke volume (SV) (r = 0.76; P < 0.001). At 60 months, 15 patients died (43%) and 1 received lung transplantation; at baseline they had worse hemodynamics, and reduced ΔZQ when compared to survivors. Patients with low ΔZQ (≤154.6%.Kg) presented significantly worse survival (P = 0.033). ΔZQ is associated with hemodynamic status of PAH patients, with disease severity and survival, demonstrating EIT as a promising tool for monitoring patients with pulmonary vascular disease.

Inhaled iloprost as third add-on therapy in idiopathic pulmonary arterial hypertension.

Triple combination therapy is suggested in current pulmonary arterial hypertension guidelines in case of unsatisfactory treatment with oral double combination therapy. However, there is a lack of evidence concerning some of the drug combinations currently employed. We demonstrate the clinical and hemodynamical benefits of inhaled iloprost as third add-on therapy in idiopathic pulmonary arterial hypertension.

Cancer-associated thrombosis: the when, how and why.

Cancer-associated thrombosis (CAT) is a condition in which relevance has been increasingly recognised both for physicians that deal with venous thromboembolism (VTE) and for oncologists. It is currently estimated that the annual incidence of VTE in patients with cancer is 0.5% compared to 0.1% in the general population. Active cancer accounts for 20% of the overall incidence of VTE. Of note, VTE is the second most prevalent cause of death in cancer, second only to the progression of the disease, and cancer is the most prevalent cause of deaths in VTE patients. Nevertheless, CAT presents several peculiarities that distinguish it from other VTE, both in pathophysiology mechanisms, risk factors and especially in treatment, which need to be considered. CAT data will be reviewed in this review.

Quality of life as a prognostic marker in pulmonary arterial hypertension.

BACKGROUND: Improvement in quality of life together with better survival are the ultimate goals in the treatment of pulmonary arterial hypertension (PAH) patients. The objective of this study was to evaluate the health-related quality of life (HRQL) of pulmonary arterial hypertension (PAH) patients with the SF-36 generic questionnaire and to identify the prognostic implication of this assessment. METHODS: Fifty-four consecutive newly diagnosed PAH patients (WHO classification group I) in a single PAH reference center were included. Patients were evaluated at baseline for clinical and hemodynamic parameters, and they subsequently received first-line therapy with either an endothelin receptor antagonist or a phosphodiesterase-5 inhibitor. After 16 weeks of specific PAH therapy, all patients were re-evaluated using a 6MWT and a SF 36 questionnaire, and then they were followed up for at least 36 months. RESULTS: After treatment, the patients demonstrated an improved 6MWT (414 ± 124 m vs. 440 ± 113 m, p = 0.001). Specific PAH therapy also improved the HRQL scores.Patients with a baseline Physical Component Score (PCS) higher than 32 had a better survival rate than those who had a score under 32 (p = 0.04). Similarly, patients with a PCS of at least a 38 after the 16 week therapy period had a better survival rate when compared with those who did not achieve this value (p = 0.016). Unlike the absolute PCS values, the post-treatment PCS variability was unable to predict better survival rates (p = 0.58). CONCLUSIONS: Our findings suggest that HRQL is associated with prognosis in PAH. Furthermore, achieving pre-determined PCS scores might represent a specific goal to be reached in treatment-to-target strategies.

The role of target therapies in schistosomiasis-associated pulmonary arterial hypertension.

BACKGROUND: Schistosomiasis-associated pulmonary arterial hypertension (Sch-PAH) may be one of the most prevalent forms of pulmonary arterial hypertension (PAH) worldwide. However, the clinical and hemodynamical response to specific PAH therapy in Sch-PAH is not known. METHODS: We retrospectively analyzed the charts of all patients with Sch-PAH who initiated specific PAH treatment between June 2003 and June 2010 in a single PAH reference center in São Paulo, Brazil. Clinical and hemodynamical data were retrospectively collected and evaluated in two periods: baseline and posttreatment. RESULTS: The study population consisted of 12 patients with Sch-PAH. They were treated with phosphodiseterase-5 inhibitors (seven patients), endothelin receptor antagonists (four patients), or combination therapy (one patient). Mean treatment period was 34.9 ± 15.5 months. Patients with Sch-PAH presented significant improvements in terms of functional class, 6-min walk test distance (439 ± 85 to 492 ± 79 m, P = .032), cardiac index (2.66 ± 0.59 to 3.08 ± 0.68 L/min/m(2), P = .028), and indexed pulmonary vascular resistance (20.7 ± 11.6 to 15.9 ± 9 W/m(2), P = .038) with the introduction of specific PAH treatment. CONCLUSIONS: We conclude that specific PAH therapy may be of benefit to patients with Sch-PAH, considering clinical, functional, and hemodynamic parameters.