Exercise-based rehabilitation programmes for pulmonary hypertension.

Background: Individuals with pulmonary hypertension (PH) have reduced exercise capacity and quality of life. Despite initial concerns that exercise training may worsen symptoms in this group, several studies have reported improvements in functional capacity and well-being following exercise-based rehabilitation.

Objectives: To evaluate the benefits and harms of exercise-based rehabilitation for people with PH compared with usual care or no exercise-based rehabilitation.

Survival of Idiopathic Pulmonary Arterial Hypertension Patients in the Modern Era in Australia and New Zealand.

Background: Epidemiology and treatment strategies continue to evolve in pulmonary arterial hypertension (PAH). We sought to define the characteristics and survival of patients with idiopathic, heritable and drug-induced PAH in the current management era.

Methods: Consecutive cases of idiopathic, heritable and drug-induced PAH were prospectively enrolled into an Australian and New Zealand Registry.

Exercise-based rehabilitation programmes for pulmonary hypertension.

Background: Individuals with pulmonary hypertension (PH) have reduced exercise capacity and quality of life. Despite initial concerns that exercise training may worsen symptoms in this group, several studies have reported improvements in functional capacity and well-being following exercise-based rehabilitation in PH.

Objectives: To assess the efficacy and safety of exercise-based rehabilitation for people with PH.

Gas exchange responses during 6-min walk test in patients with pulmonary arterial hypertension.

Background And Objective: The 6-min walk test (6MWT) is the most widely utilized method of assessing exercise capacity in pulmonary arterial hypertension (PAH). Cardiopulmonary exercise testing has the advantage of providing additional physiological information over 6MWT. The goals of our study were to describe the addition of gas exchange measurements to 6MWT and to determine how these parameters were related to the severity of PAH in three major subgroups of PAH (idiopathic (IPAH), connective tissue disease-related (CTPAH) and congenital heart disease-related (CHPAH)).

Prognostic value of right ventricular free wall strain in pulmonary hypertension patients with pseudo-normalized tricuspid annular plane systolic excursion values.

Pulmonary hypertension (PH) is a progressively fatal disease having a significant impact on right ventricular (RV) function, a major determinant of long-term outcome in PH patients. In our clinic we frequently noticed the combination of PH and reduced RV function, but with discordant Tricuspid Annular Plane Systolic Excursion (TAPSE) values. The present study focuses on whether RV free wall strain measured using 2-dimensional speckle-tracking echocardiography is able to predict mortality in this subgroup of PH patients.

Successful use of pre and post-operative ECMO for pulmonary endarterectomy, mitral valve replacement and myomectomy in a patient with chronic thromboembolic pulmonary hypertension and hypertrophic cardiomyopathy.

As a salvage strategy, extracorporeal membrane oxygenation (ECMO) permits the recovery of end-organ perfusion, whilst allowing the surgeon time for patient reassessment and surgical planning. We report upon the first known case in which VA ECMO was instituted as peri-operative supportive therapy for a young patient, in-extremis, with surgically correctable Chronic Thromboembolic Pulmonary Hypertension (CTEPH) and Hypertrophic Cardiomyopathy (HOCM).

RETRACTED: Right to left ventricular volume ratio: A novel marker of disease severity in chronic thromboembolic pulmonary hypertension.

[This retracts the article DOI: 10.1016/j.ijchv.

Time from symptoms to definitive diagnosis of idiopathic pulmonary arterial hypertension: The delay study.

Survival rates for patients with idiopathic pulmonary arterial hypertension (IPAH) have improved with the introduction of PAH-specific therapies. However, the time between patient-reported onset of symptoms and a definitive diagnosis of IPAH is consistently delayed. We conducted a retrospective, multi-center, descriptive investigation in order to (a) understand what factors contribute to persistent diagnostic delays, and (b) examine the time from initial symptom onset to a definitive diagnosis of IPAH.

An Australian tertiary referral center experience of the management of chronic thromboembolic pulmonary hypertension.

The objective of this study was to report the outcome of pulmonary endarterectomy (PEA) surgery performed for chronic thromboembolic pulmonary hypertension (CTEPH) at a single tertiary center. The prospective study consisted of 35 patients with surgically amenable CTEPH undergoing PEA between September 2004 and September 2010. The main outcome measures were Functional (New York Heart Association [NYHA] class, 6-Minute Walk Distance), hemodynamic (echocardiography, right heart catheterization, and cardiac MRI), and outcome data (morbidity and mortality).

Oral treprostinil for the treatment of pulmonary arterial hypertension in patients on background endothelin receptor antagonist and/or phosphodiesterase type 5 inhibitor therapy (the FREEDOM-C study): a randomized controlled trial.

Background: Infused and inhaled treprostinil are effective for treatment of pulmonary arterial hypertension (PAH), although their administration routes have limitations. This study assessed the efficacy and safety of bid oral sustained-release treprostinil in the treatment of PAH with a concomitant endothelin receptor antagonist (ERA) and/or phosphodiesterase type 5 inhibitor.

Methods: A 16-week, multicenter, double-blind, placebo-controlled study was conducted in 350 patients with PAH randomized to placebo or oral treprostinil.

A novel approach to the assessment of lymphocytic bronchiolitis after lung transplantation--transbronchial brush.

Background: Lymphocytic bronchiolitis (LB) is the strongest risk factor for subsequent allograft loss due to bronchiolitis obliterative syndrome (BOS); however, it is poorly assessed by transbronchial biopsy (TBBx) because of sampling error, interpretation error and the presence of non-alloimmune airway inflammation. We hypothesized that flow cytometric evaluation of bronchiolar brushings (transbronchial brush, TBBr) may be a better approach.

Methods: Transbronchial brushings (2 to 3 cm from the pleural surface under radiologic guidance) were obtained prior to TBBx in 32 patients and analyzed by flow cytometry.

Oil red O stain of alveolar macrophages is an effective screening test for gastroesophageal reflux disease in lung transplant recipients.

Background: Gastroesophageal reflux disease (GORD) and microaspiration may be associated with acute graft dysfunction and development of obliterative bronchiolitis (OB) after lung transplantation. The "gold standard" for diagnosis of GORD is the 24-hour esophageal pH-monitoring study, although no simple, non-invasive screening test is routinely employed. Oil red O staining of alveolar macrophages in bronchoalveolar lavage (BAL) fluid identifies exogenous lipid and may be a surrogate marker for microaspiration.

Human metapneumovirus in lung transplant recipients and comparison to respiratory syncytial virus.

Rationale: Human metapneumovirus is a newly described virus isolated in 2001 from children with acute respiratory viral infection. It has subsequently been reported globally, although there are limited data in lung transplant recipients.

Objectives: To prospectively analyze whether human metapneumovirus was circulating in our adult lung transplant community and assess the morbidity of this infection and to compare the clinical presentation and outcome after intravenous ribavirin of human metapneumovirus with that of respiratory syncytial virus (RSV).

Unexplained chronic anemia and leukopenia in lung transplant recipients secondary to parvovirus B19 infection.

Parvovirus B19 (PVB) is a non-enveloped, single-stranded DNA virus of the Parvoviridae family pathogenic to humans. Despite numerous case reports of pure red cell aplasia in lung transplant (LT) recipients after PVB infection, the epidemiology remains poorly outlined. Over a 3-year period, 3 of 54 LT patients with unexplained anemia tested positive for circulating PVB by a nested polymerase chain reaction (PCR) assay.

Bosentan for the treatment of pulmonary arterial hypertension associated with congenital cardiac disease.

Aims: Bosentan is efficacious in idiopathic pulmonary arterial hypertension, and the variants associated with connective tissue disease, but not currently approved for treatment of pulmonary arterial hypertension due to Eisenmenger's syndrome. We sought to evaluate its effect in adults with Eisenmenger's syndrome.

Methods: We administered bosentan on the basis of compassionate use in 23 patients with Eisenmenger's syndrome, aged 37 plus or minus 14 years.