Current developments and future directions in COPD.

The European Respiratory Society journals publish respiratory research and policy documents of the highest quality, offering a platform for the exchange and promotion of scientific knowledge. In this article, focusing on COPD, the third leading cause of death globally, we summarise novel research highlights focusing on the disease's underlying mechanisms, epidemiology and management, with the aim to inform and inspire respiratory clinicians and researchers.

The impact of acute air pollution fluctuations on bronchiectasis pulmonary exacerbation: a case-crossover analysis.

In bronchiectasis, exacerbations are believed to be triggered by infectious agents, but often no pathogen can be identified. We hypothesised that acute air pollution exposure may be associated with bronchiectasis exacerbations.We combined a case-crossover design with distributed lag models in an observational record linkage study.

Distinct genetic architectures for syndromic and nonsyndromic congenital heart defects identified by exome sequencing.

Congenital heart defects (CHDs) have a neonatal incidence of 0.8-1% (refs. 1,2).

Determination of Clinical Outcome in Mitral Regurgitation With Cardiovascular Magnetic Resonance Quantification.

Background: Surgery for severe mitral regurgitation is indicated if symptoms or left ventricular dilation or dysfunction occur. However, prognosis is already reduced by this stage, and earlier surgery on asymptomatic patients has been advocated if valve repair is likely, but identifying suitable patients for early surgery is difficult. Quantifying the regurgitation may help, but evidence for its link with outcome is limited.

Pulmonary arterial hypertension associated with congenital heart disease: recent advances and future directions.

Congenital heart disease (CHD), the most common inborn defect, affects approximately 1% of all newborns worldwide. Advances in its diagnosis and treatment have led to a dramatic improvement in patients' quality of life and long-term survival prospects. However, recently it has been realised that many of these patients are affected by ongoing and life-long cardiac issues, namely residual and progressive haemodynamic lesions, arrhythmia and sudden cardiac death, as well as the development of chronic heart failure and pulmonary arterial hypertension (PAH) - all of which merit tertiary care.

Updated clinical classification of pulmonary hypertension.

In 1998, a clinical classification of pulmonary hypertension (PH) was established, categorizing PH into groups which share similar pathological and hemodynamic characteristics and therapeutic approaches. During the 5th World Symposium held in Nice, France, in 2013, the consensus was reached to maintain the general scheme of previous clinical classifications. However, modifications and updates especially for Group 1 patients (pulmonary arterial hypertension [PAH]) were proposed.

Eisenmenger syndrome in an adult patient with a large patent ductus arteriosus.

This is the case of a young female who was seen at our adult congenital heart disease and pulmonary hypertension service (Royal Brompton Hospital, London, UK) at the age of 17 years. She initially presented at the age of 4 years with increasing shortness of breath. At that time, there was differential cyanosis with clubbing and lower oxygen saturations in the toes (82%) compared with her fingers (95%).

Long-term outcomes of dilated cardiomyopathy diagnosed during childhood: results from a national population-based study of childhood cardiomyopathy.

Background: Existing studies of childhood dilated cardiomyopathy deal mainly with early survival. This population-based study examines long-term outcomes for children with dilated cardiomyopathy.

Methods And Results: The diagnosis of dilated cardiomyopathy was based on clinical, echocardiographic, and pathological findings.

Optimised rate-responsive pacing does not improve either right ventricular haemodynamics or exercise capacity in adults with a systemic right ventricle.

Background: Inappropriate heart rate response to exercise - chronotropic incompetence - and exercise intolerance are common in patients with a systemic right ventricle. We aimed to assess the relationship between heart rate increase, oxygen consumption, and timing of the right ventricular cardiac cycle in this cohort.

Methods: We prospectively studied nine patients with systemic right ventricles and pre-existing pacemakers using Doppler-echocardiography and treadmill exercise testing.

Structural abnormalities of the pulmonary trunk in tetralogy of Fallot and potential clinical implications: a morphological study.

Objectives: The purpose of this study was to determine whether intrinsic histological abnormalities of the pulmonary trunk (PT) are present from birth and interact with palliative surgery and/or repair.

Background: Little is known about PT histology in patients with tetralogy of Fallot (TOF), especially in the era of surgical intervention in childhood.

Methods: We studied 39 formalin-fixed necropsy heart specimens with TOF and compared them with 17 normal control heart specimens.

Pulmonary arterial hypertension in adults with congenital heart disease: distinct differences from other causes of pulmonary arterial hypertension and management implications.

Purpose Of Review: To present the available data on pathophysiology, clinical presentation, prognosis, and especially management strategies for adult patients with congenital heart disease (CHD) and pulmonary arterial hypertension (PAH). Particular emphasis is placed on differences between other types of PAH and CHD-related PAH, in which clinical presentation and management relate to a constellation of factors, both pulmonary and cardiac.

Recent Findings: Pulmonary vascular disease in adults with CHD and especially its extreme expression, the Eisenmenger syndrome, is a chronic disease with slow progression, leading to multiorgan failure and death, decades after its first clinical presentation.

Longer-term bosentan therapy improves functional capacity in Eisenmenger syndrome: results of the BREATHE-5 open-label extension study.

Background: Bosentan, an oral endothelin ET(A)/ET(B) receptor antagonist, improves hemodynamics and exercise capacity in patients with Eisenmenger syndrome but longer-term effects are unknown. This study investigated the efficacy and safety of bosentan up to 40 weeks in these patients.

Methods: Following the 16-week, double blind, placebo-controlled BREATHE-5 study of bosentan in patients with Eisenmenger syndrome, an open-label extension (OLE) was performed.

Doppler-echocardiographic assessment of pulmonary regurgitation in adults with repaired tetralogy of Fallot: comparison with cardiovascular magnetic resonance imaging.

Aims: The purpose of this study was to compare the noninvasive assessment of severity of pulmonary regurgitation with Doppler echocardiography versus cardiovascular magnetic resonance imaging (CMR) in adult patients with repaired tetralogy of Fallot (rTOF).

Methods: We studied 52 (22 females) consecutive patients (aged 32 +/- 2 years, 23 +/- 5 years after rTOF) using Doppler echocardiography and compared these findings with CMR. From the continuous-wave Doppler trace, the duration of pulmonary regurgitation and of total diastole was measured and the ratio between the 2 was defined as pulmonary regurgitation index (PRi).

Risk factors for arrhythmia and sudden cardiac death late after repair of tetralogy of Fallot: a multicentre study.

Background: Ventricular arrhythmia and sudden cardiac death late after repair of tetralogy of Fallot are devastating complications in adult survivors of early surgery, but their prediction remains difficult.

Methods: We examined surgical, electrocardiographic, and late haemodynamic data, and their relation to clinical arrhythmia and sudden death occurring over 10 years, in a multicentre cohort of patients with repaired tetralogy, who were alive in 1985.

Results: Of 793 patients (mean age at repair 8.