Heart Rate Variability is Related to Disease Severity in Children and Young Adults with Pulmonary Hypertension.

Background: Pulmonary hypertension (PH) is frequently associated with an increase in sympathetic tone. This may adversely affect cardiac autonomic control. Knowledge about the clinical impact of autonomic dysfunction in patients with PH is limited.

Poor sleep quality is associated with exercise limitation in precapillary pulmonary hypertension.

Background: Patients with precapillary pulmonary hypertension (PH) have been reported to suffer from poor sleep quality, however, if this is related to physical exercise performance has not yet been thoroughly investigated.

Methods: Clinically stable out-patients with idiopathic pulmonary arterial hypertension (IPAH, n = 52) and chronic thromboembolic PH (CTEPH, n = 64) in NYHA classes II and III were prospectively enrolled. 54 healthy volunteers matched for anthropometric variables served as a control group.

Vascular receptor autoantibodies in pulmonary arterial hypertension associated with systemic sclerosis.

Rationale: Systemic sclerosis (SSc)-associated pulmonary arterial hypertension (PAH) portends worse outcome than other forms of PAH. Vasoconstrictive and vascular remodeling actions of endothelin (ET) 1 and angiotensin (Ang) II via endothelin receptor type A (ETAR) and Ang receptor type-1 (AT1R) activation are implicated in PAH pathogenesis.

Objectives: We hypothesized that stimulating autoantibodies (Abs) targeting and activating AT1R and ETAR may contribute to SSc-PAH pathogenesis, and tested their functional and biomarker relevance.

[Mental distress and wish for psychosomatic treatment of patients with pulmonary hypertension].

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.

The prognostic significance of inspiratory capacity in pulmonary arterial hypertension.

Background: Patients with pulmonary arterial hypertension (PAH) present with an altered inspiratory capacity (IC) reflecting dynamic hyperinflation (DH) that leads to mechanical constraints and excessive ventilatory demand, particularly during exercise, resulting in exertional dyspnea.

Objectives: Assessment of the long-term consequences of altered IC and DH in PAH.

Methods: 50 patients with newly diagnosed PAH were prospectively recruited.

Short-term improvement in pulmonary hemodynamics is strongly predictive of long-term survival in patients with pulmonary arterial hypertension.

Abstract Hemodynamic measurements provide important parameters for determining prognosis and therapy in patients with pulmonary arterial hypertension (PAH). Current guidelines do not incorporate the possible predictive value of individual changes in hemodynamic variables during the disease time course, and there is no consensus about the time point for hemodynamic reevaluation. We aimed to assess the long-term prognostic value of short-term changes in hemodynamic parameters.

Economic evaluation of exercise training in patients with pulmonary hypertension.

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).

Anticoagulation and survival in pulmonary arterial hypertension: results from the Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension (COMPERA).

Background: For almost 30 years, anticoagulation has been recommended for patients with idiopathic pulmonary arterial hypertension (IPAH). Supporting evidence, however, is limited, and it is unclear whether this recommendation is still justified in the modern management era and whether it should be extended to patients with other forms of pulmonary arterial hypertension (PAH).

Methods And Results: We analyzed data from Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension (COMPERA), an ongoing European pulmonary hypertension registry.

Assessment and prognostic relevance of right ventricular contractile reserve in patients with severe pulmonary hypertension.

Background: This study sought to analyze a new approach to assess exercise-induced pulmonary artery systolic pressure (PASP) increase by means of stress Doppler echocardiography as a possible measure of right ventricular contractile reserve in patients with severe pulmonary hypertension and right heart failure.

Methods And Results: In this prospective study, patients with invasively diagnosed pulmonary arterial hypertension or inoperable chronic thromboembolic pulmonary hypertension and impaired right ventricular pump function despite a stable targeted pulmonary arterial hypertension medication underwent a broad panel of noninvasive assessments, including stress echocardiography and cardiopulmonary exercise testing. On the basis of the assumption that exercise-induced PASP is a measure of right ventricular contractile reserve, patients were classified into 2 groups according to an exercise-induced PASP increase above or below the median.

Cardiovocal Syndrome (Ortner's Syndrome) Associated with Chronic Thromboembolic Pulmonary Hypertension and Giant Pulmonary Artery Aneurysm: Case Report and Review of the Literature.

Cardiovocal syndrome or Ortner's syndrome is hoarseness due to left recurrent laryngeal nerve palsy caused by mechanical affection of the nerve from enlarged cardiovascular structures. Chronic thromboembolic pulmonary hypertension is extremely rarely found to cause this syndrome. We describe a case of a 56-year-old patient with sudden onset of hoarseness.

Sleep apnea in precapillary pulmonary hypertension.

Objectives: Pilot studies have described the occurrence of sleep apnea in patients with precapillary pulmonary hypertension (PH). However, there are no data on the prevalence of sleep-related breathing disorders in larger patient cohorts with PH.

Methods: 169 patients with a diagnosis of PH confirmed by right heart catheterisation and clinically stable in NYHA classes II or III were prospectively investigated by polygraphy (n = 105 females, mean age: 61.

Reduced microRNA-150 is associated with poor survival in pulmonary arterial hypertension.

Rationale: MicroRNAs (miRNAs or miRs) are implicated in the pathogenesis of various cardiovascular diseases, including pulmonary arterial hypertension (PAH).

Objectives: We sought to measure changes in plasma levels of miRNAs in patients with PAH and relate them to the severity of the disease.

Methods: A microarray screen was performed on total plasma RNA from eight patients with PAH and eight healthy control subjects.

Efficacy of exercise training in pulmonary arterial hypertension associated with congenital heart disease.

Background: The objective of this prospective study was to assess the efficacy of exercise training as add-on to medical therapy in patients with congenital heart disease associated pulmonary arterial hypertension (CHD-APAH).

Methods: Patients with invasively confirmed CHD-APAH received in-hospital exercise training for 3 weeks and continued at home. Efficacy parameters were evaluated at baseline, after 3 and 15 weeks.

HbA1c in pulmonary arterial hypertension: a marker of prognostic relevance?

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.

Quality of life in clinically stable adult cystic fibrosis out-patients: associations with daytime sleepiness and sleep quality.

Background: Patients with cystic fibrosis (CF) may suffer from sleep disturbances and reduced health-related quality of life (HRQoL). However, the relationships of daytime sleepiness and sleep quality to HRQoL in CF have not yet been investigated.

Patients And Methods: 55 adult CF out-patients free from a pulmonary exacerbation were prospectively enrolled in this study.

Exercise training in pulmonary arterial hypertension associated with connective tissue diseases.

Introduction: The objective of this prospective study was to assess short- and long-term efficacy of exercise training (ET) as add-on to medical therapy in patients with connective tissue disease-associated pulmonary arterial hypertension (CTD-APAH).

Methods: Patients with invasively confirmed CTD-APAH received ET in-hospital for 3 weeks and continued at home for 12 weeks. Efficacy parameters have been evaluated at baseline and after 15 weeks by blinded-observers.

Pulmonary hypertension: Hemodynamic evaluation. Updated Recommendations of the Cologne Consensus Conference 2011.

The 2009 European Guidelines on Diagnosis and Treatment of Pulmonary Hypertension have been adopted for Germany. The guidelines contain detailed recommendations for the diagnosis of pulmonary hypertension. However, the practical implementation of the European Guidelines in Germany requires the consideration of several country-specific issues and already existing novel data.

Long-term effects of intravenous iloprost in patients with idiopathic pulmonary arterial hypertension deteriorating on non-parenteral therapy.

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.

Dynamic hyperinflation during exercise in patients with precapillary pulmonary hypertension.

Background: Patients with pulmonary arterial hypertension often present with a mild obstructive lung pattern, however, the functional consequences are not known.

Methods: We analysed flow volume loops during exercise in 61 patients with precapillary pulmonary hypertension (PH) (age 55 ± 14 years) in comparison with 21 patients with COPD (60 ± 12 years), 39 patients with pulmonary fibrosis (58 ± 11 years) and 38 healthy controls (HC) (39 ± 15 years). Inspiratory capacity (IC) was measured at rest, and during maximum exercise (max).

Air travel can be safe and well tolerated in patients with clinically stable pulmonary hypertension.

Our aim was to determine what proportion of patients with pulmonary hypertension (PH) has undertaken air travel contrary to the general medical advice and to characterize these patients according to disease severity and medical treatment. In cooperation with Pulmonale Hypertonie e.V.

Usefulness of serial N-terminal pro-B-type natriuretic peptide measurements for determining prognosis in patients with pulmonary arterial hypertension.

Previous studies have shown the prognostic benefit of N-terminal pro-brain natriuretic peptide (NT-pro-BNP) in pulmonary arterial hypertension (PAH) at time of diagnosis. However, there are only limited data on the clinical utility of serial measurements of the inactive peptide NT-pro-BNP in PAH. This study examined the value of serial NT-pro-BNP measurements in predicting prognosis PAH.

Hemodynamic and clinical onset in patients with hereditary pulmonary arterial hypertension and BMPR2 mutations.

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.