The Impact on Parents of Diagnosing PCD in Young Children.

Primary ciliary dyskinesia (PCD) is an incurable, rare, inherited, chronic condition. Treatment includes the regular clearing of airway mucus, aggressive treatment of infections and management of hearing loss. Caregiver burden has not been explored, hence we interviewed 18 mothers and 6 fathers of children under 6 years to understand the impact of diagnostic testing and implications of a positive diagnosis.

Mid- and long-term outcomes of thoracic endovascular aortic repair in acute and subacute uncomplicated type B aortic dissection.

Background: Uncomplicated type B aortic dissection (un-TBAD) has been managed conservatively with medical therapy to control the heart rate and blood pressure to limit disease progression, in addition to radiological follow-up. However, several trials and observational studies have investigated the use of thoracic endovascular aortic repair (TEVAR) in un-TBAD and suggested that TEVAR provides a survival benefit over medical therapy. Outcomes of TEVAR have also been linked with the timing of intervention.

Catheter Ablation for Atrial Fibrillation in Patients with Heart Failure with Preserved Ejection Fraction: A Systematic Review and Meta-Analysis.

Background: Catheter ablation (CA) for atrial fibrillation (AF) has been proposed as a means of improving outcomes among patients with heart failure and reduced ejection fraction (HFrEF) who are otherwise receiving appropriate treatment. Unlike HFrEF, treatment options are more limited in patients with preserved ejection fraction (HFpEF) and the data pertaining to the management of AF in these patients are controversial. The aim of this systematic review and meta-analysis was to investigate the effects of CA on outcomes of patients with AF and HFpEF, such as functional status, post-procedural complications, hospitalization, morbidity and mortality, based on data from observational studies.

What is the optimal timing for thoracic endovascular aortic repair in uncomplicated Type B aortic dissection?

Background: Uncomplicated Stanford Type B aortic dissection (un-TBAD) is characterized by a tear in the aorta distal to the left subclavian artery without ascending aorta and arch involvement. Optimized cardiovascular control (blood pressure and heart rate) is the current gold standard treatment according to current international guidelines. However, emerging evidence indicates that thoracic endovascular aortic repair (TEVAR) is both safe and effective in the treatment of un-TBAD with improved long-term survival outcomes in combination with optimal medical therapy (OMT) relative to OMT alone.

Retrosternal goiter in thoracic surgical practice.

Objective: To evaluate and optimize surgical approaches to the management of retrosternal goiter.

Material And Methods: A cohort retrospective single-center study included 56 patients aged 68.3±9.

Diagnostic and Prognostic Value of Cardiovascular Magnetic Resonance in Neuromuscular Cardiomyopathies.

Neuromuscular diseases (NMD) encompass a broad spectrum of diseases with variable type of cardiac involvement and there is lack of clinical data on Cardiovascular Magnetic Resonance (CMR) phenotypes or even prognostic value of CMR in NMD. We explored the diagnostic and prognostic value of CMR in NMD-related cardiomyopathies. The study included retrospective analysis of a cohort of 111 patients with various forms of NMD; mitochondrial: n = 14, Friedreich's ataxia (FA): n = 27, myotonic dystrophy: n = 27, Becker/Duchenne's muscular dystrophy (BMD/DMD): n = 15, Duchenne's carriers: n = 6, other: n = 22.

Report from the 2018 consensus conference on immunomodulating agents in thoracic transplantation: Access, formulations, generics, therapeutic drug monitoring, and special populations.

In 2009, the International Society for Heart and Lung Transplantation recognized the importance and challenges surrounding generic drug immunosuppression. As experience with generics has expanded and comfort has increased, substantial issues have arisen since that time with other aspects of immunomodulation that have not been addressed, such as access to medicines, alternative immunosuppression formulations, additional generics, implications on therapeutic drug monitoring, and implications for special populations such as pediatrics and older adults. The aim of this consensus document is to address critically each of these concerns, expand on the challenges and barriers, and provide therapeutic considerations for practitioners who manage patients who need to undergo or have undergone cardiothoracic transplantation.

Supravalvular mitral remnant: The other side of the… valve.

We report a challenging clinical case of an atypical supravalvular mitral remnant in recent mitral and aortic valve replacement with mechanical valve prostheses, associated with postoperative recurrent inflammatory episodes overlapped with difficult anticoagulation. Negative myocardial scintigraphy was associated with persistence of negative blood cultures. Serial echocardiographic evaluation was performed before and after antimicrobial treatment, and at 3 months follow-up a transesophageal echocardiography showed the persistence of the mass.

An observational study of the lung clearance index throughout childhood in cystic fibrosis: early years matter.

https://bit.ly/2yKyMbM

Minimal change in structural, functional and inflammatory markers of lung disease in newborn screened infants with cystic fibrosis at one year.

Background: With the widespread introduction of newborn screening for cystic fibrosis (CF), there has been considerable emphasis on the need to develop objective markers of lung health that can be used during infancy. We hypothesised that in a newborn screened (NBS) UK cohort, evidence of airway inflammation and infection at one year would be associated with adverse structural and functional outcomes at the same age.

Methods: Infants underwent lung function testing, chest CT scan and bronchoscopy with bronchoalveolar lavage (BAL) at 1 year of age when clinically well.

Treatment of Allergic Rhinitis as a Strategy for Preventing Asthma.

Purpose Of Review: To evaluate the impact of allergic rhinitis (AR) on the development of asthma and to update readers on recent literature suggesting that early treatment of allergic subjects with immunotherapy may prevent asthma onset.

Recent Findings: AR is frequently associated with asthma, leading to the concept that these two conditions are different aspects of the same disease. There is increasing evidence that AR precedes the onset of asthmatic symptoms and current treatment strategies are beneficial in symptom control with no impact prevention.

Surgical Treatment of Retrosternal Goitre.

This study aims to evaluate surgical approaches to the management of retrosternal goitre. Between 2004 and 2014, 35 patients (eight males; mean age 67.4 ± 10.

Mid-term clinical outcomes of ABSORB bioresorbable vascular scaffold versus everolimus-eluting stent for coronary bifurcation lesions.

Background: Data regarding bioresorbable vascular scaffolds (BVS) use in coronary bifurcation lesions are limited. The aim of this study was to compare mid-term clinical outcomes of all-comer patients treated with BVS versus everolimus-eluting stents (EES) for bifurcation lesions.

Methods: A total of 351 non-left-main bifurcation lesions in 323 all-comer patients were treated either with BVS (166 bifurcations in 147 patients) or EES (185 bifurcations in 176 patients).

An observational study of Donor Ex Vivo Lung Perfusion in UK lung transplantation: DEVELOP-UK.

Background: Many patients awaiting lung transplantation die before a donor organ becomes available. Ex vivo lung perfusion (EVLP) allows initially unusable donor lungs to be assessed and reconditioned for clinical use.

Objective: The objective of the Donor Ex Vivo Lung Perfusion in UK lung transplantation study was to evaluate the clinical effectiveness and cost-effectiveness of EVLP in increasing UK lung transplant activity.

Percutaneous left atrial appendage occlusion using different technologies in the United Kingdom: A multicenter registry.

Objectives: This study aimed at assessing the feasibility and long-term efficacy of left atrial appendage occlusion (LAAO) in a "real world" setting.

Background: Although LAAO has recently emerged as an alternative to oral anticoagulants in patients with atrial fibrillation for the prevention of thromboembolic stroke, "real world" data about the procedure with different devices are lacking.

Methods: Eight centers in the United Kingdom contributed to a retrospective registry for LAAO procedures undertaken between July 2009 and November 2014.

Diagnostic Value of Circulating CXC Chemokines in Non-small Cell Lung Cancer.

Background/aim: To evaluate the diagnostic value of circulating CXC chemokines as biomarkers for non-small cell lung cancer and compare them against a standard panel of already existing cancer biomarkers.

Materials And Methods: A total of 90 individuals were enrolled in the study. We analyzed 30 patients with stage IA-IIB carcinoma of the lung who underwent pulmonary resection, 30 patients with metastatic NSCLC, and 30 healthy volunteers.