Converging Pathways: A Review of Pulmonary Hypertension in Interstitial Lung Disease.

Pulmonary hypertension (PH) in interstitial lung disease (ILD) is relatively common, affecting up to 50% of patients with idiopathic pulmonary fibrosis (IPF). It occurs more frequently in advanced fibrotic ILD, although it may also complicate milder disease and carries significant clinical implications in terms of morbidity and mortality. Key pathological processes driving ILD-PH include hypoxic pulmonary vasoconstriction and pulmonary vascular remodelling.

Pulmonary Hypertension and the Gut Microbiome.

The gut microbiome and its associated metabolites are integral to the maintenance of gut integrity and function. There is increasing evidence that its alteration, referred to as dysbiosis, is involved in the development of a systemic conditions such as cardiovascular disease (e.g.

Cardioprotective medication in Duchenne muscular dystrophy: a single-centre cohort study.

Background: Duchenne muscular dystrophy (DMD) is a neuromuscular disorder characterised by progressive muscle wasting impacting mobility, ventilation and cardiac function. Associated neuromuscular cardiomyopathy remains a major cause of morbidity and mortality. We investigated the effects of cardioprotective medications [angiotensin-converting enzyme inhibitors (ACE-I), beta-blockers] on clinical outcomes in DMD patients.

Diagnosis and management of pulmonary hypertension related to chronic respiratory disease.

Pulmonary hypertension (PH) is a recognised and significant complication of chronic lung disease (CLD) and hypoxia (referred to as group 3 PH) that is associated with increased morbidity, decreased quality of life and worse survival. The prevalence and severity of group 3 PH varies within the current literature, with the majority of CLD-PH patients tending to have non-severe disease. The aetiology of this condition is multifactorial and complex, while the prevailing pathogenetic mechanisms include hypoxic vasoconstriction, parenchymal lung (and vascular bed) destruction, vascular remodelling and inflammation.

Perioperative management of patients with pulmonary hypertension undergoing non-cardiothoracic, non-obstetric surgery: a systematic review and expert consensus statement.

Background: The risk of complications, including death, is substantially increased in patients with pulmonary hypertension (PH) undergoing anaesthesia for surgical procedures, especially in those with pulmonary arterial hypertension (PAH) and chronic thromboembolic PH (CTEPH). Sedation also poses a risk to patients with PH. Physiological changes including tachycardia, hypotension, fluid shifts, and an increase in pulmonary vascular resistance (PH crisis) can precipitate acute right ventricular decompensation and death.

Narrative review of sleep and pulmonary hypertension.

Pulmonary hypertension (PH) is a condition of raised pulmonary artery pressure (PAP), which may be secondary to a number of causes, one of which is sleep disordered breathing (SDB). When PH complicates SDB, it carries a significant burden of morbidity and mortality due to the risk of progression to right ventricular failure over time. This narrative review will cover the definition and classification of PH, and explore the epidemiology of PH in SDB.

Long-term adherence to home mechanical ventilation: a 10-year retrospective, single-centre cohort study.

Background: Sleep-disordered breathing (SBD) can be associated with hypercapnic respiratory failure (HRF). Home Mechanical Ventilation (HMV) is the preferred long-term treatment for patients with chronic HRF. We reviewed the database of a large tertiary referral centre for HMV to study the long-term adherence to HMV in chronic hypercapnic patients.

Long-term survival following initiation of home non-invasive ventilation: a European study.

Introduction: Although home non-invasive ventilation (NIV) is increasingly used to manage patients with chronic ventilatory failure, there are limited data on the long-term outcome of these patients. Our aim was to report on home NIV populations and the long-term outcome from two European centres.

Methods: Cohort analysis including all patients established on home NIV from two European centres between 2008 and 2014.

Time-to-death in chronic respiratory failure on home mechanical ventilation: A cohort study.

Background And Objective: Home mechanical ventilation (HMV) is used in heterogeneous conditions underlying chronic hypercapnic respiratory failure, but there are sparse data on long-term clinical outcomes. The aim was to systematically analyse the time and the circumstances of death on HMV.

Methods: All-cause mortality data of HMV patients were prospectively collected between 2008 and 2018 in a large tertiary centre.

Neural respiratory drive and cardiac function in patients with obesity hypoventilation syndrome following initiation of non-invasive ventilation.

Background: Chronic hypercapnic respiratory failure (HRF) in obesity hypoventilation syndrome (OHS) is commonly treated using non-invasive ventilation (NIV). We hypothesised that treatment of OHS would improve neural respiratory drive index (NRDI) and cardiac function.

Methods: Fourteen patients (8 females) with OHS, who were admitted for initiation of domiciliary NIV, were prospectively studied.

The CRASH report: emergency management dilemmas facing acute physicians in patients with pulmonary arterial hypertension.

Treatment of acute emergencies in patients with pulmonary arterial hypertension (PAH) can be challenging. In the UK and Ireland, management of adult patients with PAH is centred in eight nationally designated pulmonary hypertension (PH) centres. However, many patients live far from these centres and physicians in local hospitals are often required to manage PAH emergencies.

Association of haemodynamic changes measured by serial central venous saturation during ultrafiltration for acutely decompensated heart failure with diuretic resistance and change in renal function.

Background: Patients with acute decompensated heart failure with diuretic resistance (ADHF-DR) have a poor prognosis. The aim of this study was to assess in patients with ADHF-DR, whether haemodynamic changes during ultrafiltration (UF) are associated with changes in renal function (Δcreatinine) and whether Δcreatinine post UF is associated with mortality.

Methods: Seventeen patients with ADHF-DR underwent 20 treatments with UF.

Prospective observational cohort study of patients with weaning failure admitted to a specialist weaning, rehabilitation and home mechanical ventilation centre.

Objectives: According to National Health Service England (NHSE) specialist respiratory commissioning specification for complex home ventilation, patients with weaning failure should be referred to a specialist centre. However, there are limited data reporting the clinical outcomes from such centres.

Setting: Prospective observational cohort study of patients admitted to a UK specialist weaning, rehabilitation and home mechanical ventilation centre between February 2005 and July 2013.

Physiological differences between various types of Eisenmenger syndrome and relation to outcome.

Background: Eisenmenger syndrome (ES) is the most advanced form of pulmonary arterial hypertension (PAH) related to congenital heart disease. Several studies have suggested that the presence and location of the shunt defines the natural history of these patients by influencing right ventricular adaptation to PAH. We aimed to echocardiographically assess differences in cardiac physiology and outcome between various types of ES.

Donation after circulatory death: a national survey of current practice in England in 2012.

Objective: In recent years, England has seen renewed interest in donation after circulatory death. Many national and local initiatives have been implemented to encourage and support donation after circulatory death. To assess whether practice is in line with published guidance, we conducted a national survey with regard to current donation after circulatory death practices, local guidelines, and views on the need to further develop a national standardized protocol for donation after circulatory death.

Treatment of sarcoidosis-associated pulmonary hypertension: A single centre retrospective experience using targeted therapies.

Background: Pulmonary hypertension (PH), an increasingly recognised complication of pulmonary sarcoidosis, is associated with increased morbidity and mortality Evidence of benefit with targeted therapies in sarcoidosis associated pulmonary hypertension (SAPH) is limited.

Methods: We conducted a retrospective review of patients with sarcoidosis and right heart catheter proven PH who received treatment with targeted therapies (phosphodiesterase-5 inhibitors, endothelin receptor antagonists, or combination) at our hospital. Six minute walk test (6MWT), World Health Organisation (WHO) functional class, echocardiography, pulmonary function test (PFT) and serum brain natriuretic peptide (BNP) data were collected at baseline and during follow-up.

Bosentan in pulmonary hypertension associated with fibrotic idiopathic interstitial pneumonia.

Rationale: Pulmonary hypertension (PH) associated with fibrotic idiopathic interstitial pneumonia (IIP; idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia) confers important additional morbidity and mortality.

Objectives: To evaluate the safety and clinical efficacy of the dual endothelin-1 receptor antagonist bosentan in this patient group.

Methods: In a randomized, double-blind, placebo-controlled study, 60 patients with fibrotic IIP and right heart catheter confirmed PH were randomized 2:1 to bosentan (n = 40) or placebo (n = 20).

C-reactive protein in adults with pulmonary arterial hypertension associated with congenital heart disease and its prognostic value.

Objectives: To assess the relationship of C-reactive protein (CRP) to clinical outcome and mortality in adults with pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD-PAH).

Background: Approximately 5-10% of adults with congenital heart disease (ACHD) develop PAH, which in turn is associated with substantial morbidity and mortality. Although CRP is known to predict outcome in idiopathic PAH, little is known regarding its prognostic value in CHD-PAH.

Six-minute walk test distance and resting oxygen saturations but not functional class predict outcome in adult patients with Eisenmenger syndrome.

Background: Eisenmenger syndrome (ES) represents the extreme manifestation of pulmonary arterial hypertension in patients with congenital heart disease, associated with significant exercise intolerance and mortality. Even though of six-minute-walk-test (6MWT) is routinely used in these patients, little is known about its prognostic value in comparison to functional class.

Methods And Results: We included 210 adult patients with ES who underwent a total of 822 6MWTs.

Abnormal lung function in adults with congenital heart disease: prevalence, relation to cardiac anatomy, and association with survival.

Background: Restrictive lung defects are associated with higher mortality in patients with acquired chronic heart failure. We investigated the prevalence of abnormal lung function, its relation to severity of underlying cardiac defect, its surgical history, and its impact on outcome across the spectrum of adult congenital heart disease.

Methods And Results: A total of 1188 patients with adult congenital heart disease (age, 33.

Echocardiographic predictors of outcome in eisenmenger syndrome.

Background: Eisenmenger syndrome differs significantly from other types of pulmonary arterial hypertension in its physiology and prognosis. We sought to assess the relationship between the echocardiographic characteristics of patients with Eisenmenger syndrome and mortality.

Methods And Results: Clinical and echocardiographic variables were assessed in 181 consecutive patients with Eisenmenger syndrome, excluding those with complex congenital heart disease.

Disease targeting therapies in patients with Eisenmenger syndrome: response to treatment and long-term efficiency.

Objectives: To examine long-term efficacy of disease targeting therapies (DTT) in patients with Eisenmenger syndrome.

Methods: All adult patients with Eisenmenger syndrome treated with DTT at our institution were included. Functional class (FC), oxygen saturation and 6-minute walk test distance (6 MWTd) were analysed retrospectively.

Pathophysiology of pulmonary hypertension in acute lung injury.

Acute lung injury (ALI) and acute respiratory distress syndrome are characterized by protein rich alveolar edema, reduced lung compliance, and acute severe hypoxemia. A degree of pulmonary hypertension (PH) is also characteristic, higher levels of which are associated with increased morbidity and mortality. The increase in right ventricular (RV) afterload causes RV dysfunction and failure in some patients, with associated adverse effects on oxygen delivery.