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Purpose Exercise training as an add-on to medical therapy has been shown to improve exercise capacity, quality of life, and possibly prognosis in patients with pulmonary hypertension (PH). The purpose of this study was to analyze the impact of exercise training on healthcare costs in PH. Methods Estimated healthcare costs have been compared between patients with severe PH under optimized medical therapy only (control group) versus patients who received exercise training as an add-on to medical therapy (training group). Cost-analysis included a cost-estimation model of costs for baseline and follow-up visits and all PH-related healthcare events that occurred within the follow-up period. Time to clinical worsening and survival were assessed by clinical records, phone, and/or control visits. Results At baseline, the training (n = 58) and control group (n = 48) did not differ in age, gender, WHO-functional class, 6-min walking distance, hemodynamic parameters, or PH-targeted medication. During a follow-up of 24 ± 12 months, the training group had significantly better survival rates at 1 and 3 years and less worsening events (death, lung transplantation, hospitalization due to PH, new PAH-targeted medication) than the control group (15 vs. 25 events, p < 0.05), which also led to lower estimated healthcare costs of 657€ within a period of 2 years. Conclusions This is the first study to investigate the cost-effectiveness of exercise training in PH. Due to less worsening events within 2 years, healthcare costs were lower in patients performing exercise training as add-on to medical therapy than in patients with medical treatment only. Further prospective, randomized studies are needed to confirm these findings. Keywords Pulmonary hypertension * Exercise training * Cost-effectiveness * Socioeconomic model * Survival, Introduction Pulmonary hypertension (PH) is a chronic progressive disease that is characterized by an increasing pressure in the pulmonary arteries leading to right heart failure, an impaired quality of life, [...]

Abstract Background The majority of patients with idiopathic pulmonary arterial hypertension (IPAH) in functional classes II and III are currently being treated with non-parenteral therapies, including endothelin receptor antagonists (ERA), phosphodiesterase (PDE)-5 inhibitors, inhaled iloprost or combinations of these substances. If these treatments fail, current guidelines recommend the addition of parenteral prostanoid therapy. There is, however, limited evidence for the efficacy of parenteral prostanoids when added to combinations of non-parenteral therapies. Methods In this retrospective, multicentre study we collected data from consecutive IPAH patients receiving intravenous iloprost in addition to optimized non-parenteral therapy between Jan 2002 and Dec 2009. Analyses included 6 min walk distance (6MWD), functional class, need for transplantation, and survival. Results During the observation period, 50 patients were treated with intravenous iloprost in addition to non-parenteral therapy; 44% of the patients were on dual combination therapy and 52% on triple combination. Three months after initiation of iloprost, functional class had improved in 24% of the patients and the median 6MWD had increased from 289 m to 298 m (n.s.). During the observation period, 22 patients (44%) died and 14 (28%) underwent lung transplantation. The probabilities of LuTx-free survival at 1, 3 and 5 years following iloprost initiation were 38%, 17% and 17%, respectively. A 6MWD < 300 m and persistent functional class IV at 3 months after initiation of intravenous iloprost were predictors of an adverse outcome. Conclusion In essence, late initiation of intravenous iloprost in IPAH patients who previously failed to respond to non-parenteral therapies appears to be of limited efficacy in the majority patients. Alternative therapeutic options are currently not available, underlying the need for the development of new drugs.

Abstract Background Mutations in the bone morphogenetic protein receptor 2 (BMPR2) gene can lead to idiopathic pulmonary arterial hypertension (IPAH). This study prospectively screened for BMPR2 mutations in a large cohort of PAH-patients and compared clinical features between BMPR2 mutation carriers and non-carriers. Methods Patients have been assessed by right heart catheterization and genetic testing. In all patients a detailed family history and pedigree analysis have been obtained. We compared age at diagnosis and hemodynamic parameters between carriers and non-carriers of BMPR2 mutations. In non-carriers with familial aggregation of PAH further genes/gene regions as the BMPR2 promoter region, the ACVRL1, Endoglin, and SMAD8 genes have been analysed. Results Of the 231 index patients 22 revealed a confirmed familial aggregation of the disease (HPAH), 209 patients had sporadic IPAH. In 49 patients (86.3% of patients with familial aggregation and 14.3% of sporadic IPAH) mutations of the BMPR2 gene have been identified. Twelve BMPR2 mutations and 3 unclassified sequence variants have not yet been described before. Mutation carriers were significantly younger at diagnosis than non-carriers (38.53 ± 12.38 vs. 45.78 ± 11.32 years, p < 0.001) and had a more severe hemodynamic compromise. No gene defects have been detected in 3 patients with HPAH. Conclusion This study identified in a large prospectively assessed cohort of PAH- patients new BMPR2 mutations, which have not been described before and confirmed previous findings that mutation carriers are younger at diagnosis with a more severe hemodynamic compromise. Thus, screening for BMPR2 mutations may be clinically useful.

Background: Patients with pulmonary arterial hypertension (PAH) exhibit impaired glucose metabolism and increased insulin resistance. The clinical consequences of these metabolic changes are not known. Methods: We assessed HbA1c levels in 115 patients newly diagnosed with PAH (79 females and 36 males; mean age 49.2 years; idiopathic n = 67, collagen vascular disease n = 16, congenital heart defect n = 19, pulmonary veno-occlusive disease n = 8, portopulmonary n = 5). No patients had diabetes or were receiving anti-diabetic medication or systemic steroids. After initiation of pulmonary vasoactive treatment, patients remained in long-term follow-up. Results: Initially, patients were in an advanced stage of disease (mean pulmonary arterial pressure 53 ± 18 mm Hg, cardiac index 2.3 ± 0.8 liters/min/m2) with a 6-minute-walk distance of 337 ± 123 meters, and in NYHA Functional Class 3.0 ± 0.7. The HbA1c was 5.73 ± 0.75%. A moderate but statistically significant positive correlation was observed between HbA1c levels and BNP (r p = 0.41, p = 0.014), but no correlation was found with hemodynamics or 6-minute-walk distance. The 5-year survival rate for the entire group was 68%. Kaplan–Meier analysis and multivariate Cox proportional hazard models correcting for demographic and clinical covariates revealed that patients with HbA1c <5.7% had a significantly better 5-year survival compared with those having higher initial values (85.1% vs 55.9%; log rank p = 0.002). HbA1c was a predictor of all-cause mortality with a hazard ratio of 2.23 (95% CI 1.06 to 4.70; p = 0.034) per 1-unit increase of HbA1c. Conclusions: In patients with pulmonary arterial hypertension, the HbA1c level at time of diagnosis is an independent predictor of long-term prognosis. [ABSTRACT FROM AUTHOR]

Background: The study investigated the level of mental distress in patients with pulmonary hypertension (PH) and assessed the use of and the wish for psychosomatic treatment., Methods: A total sample of n=187 outpatients participated in the cross-sectional survey. The short form of the Patient Health Questionnaire (PHQ-D), the EuroQol (EQ-5D) and a questionnaire assessing the wish for psychosomatic treatment were applied., Results: 50.6% of the patients exhibited depressive symptoms of varying degrees, 19.2% showed symptoms of major depression. 14.8% of the pa-tients reported panic attacks, and 7.1% demonstrated symptoms of a panic syndrome. Quality of life was low (EQ-5D VAS M=60). Experience with outpatient or inpatient psychotherapy was reported by 23.4% and 8.6% of the patients, respectively. 56.5% reported a wish for psychosomatic treatment., Conclusions: PH-Patients are more likely to suffer from mild or subthreshold depressive syndromes, but are very interested in psychosomatic treatment. The implementation of psychosomatic interventions into clinical practice would be desirable., (© Georg Thieme Verlag KG Stuttgart · New York.)