'Let me get on with it': Teenager, 18, refuses heart and double lung transplant so he can enjoy the time he has left outside hospital

Liam Gawthorpe, 18, from West Yorkshire, was diagnosed with a congenital heart defect and pulmonary hypertension at three-months-old.
Source: the Mail online | Health - Category: Consumer Health News Source Type: news

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Right ventricular failure (RVF) is still associated with an increased mortality in patients with pulmonary hypertension (PH) or congenital heart disease. Transition from right ventricular dysfunction to RVF is not predictable and has not been well understood so far. Several studies in rodents and in piglets showed that impairment of capillary density (CD) in the right ventricle (RV) is associated with right ventricular failure (1, 2). Some evidence suggested that the RV of patients with Eisenmenger Syndrome (ES) has remarkable adaptive capacities to high pulmonary pressures (3), leading to a longer survival in these patien...
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Tags: Research Correspondence Source Type: research
ConclusionsVarious biomarkers are associated with the event-free survival in adults with PH. However, risk stratification exclusively based on single or a combination of biomarkers seems not superior to existing risk scores.
Source: American Heart Journal - Category: Cardiology Source Type: research
Despite the numerous innovations in all diagnostic and treatment aspects of congenital heart disease, the management of pulmonary vein stenosis (PVS), primary or postoperative, continues to be perplexing and 1-2 year survival of patients with progressive PVS seems to have stubbornly plateaued at a low range of 50-60%.1-4 Various medical, percutaneous and surgical treatment modalities for PVS have been described. Nonetheless, all those options continue to be associated with inferior outcomes due to residual lesions, recurrent stenosis secondary to disease progression in the same or other pulmonary veins, development of pulm...
Source: Seminars in Thoracic and Cardiovascular Surgery - Category: Cardiovascular & Thoracic Surgery Authors: Tags: Congenital – Editorial Commentary Source Type: research
Conclusion: LCI can be successfully measured in LTx patients, and changes with BOS grade. Longitudinal assessments will reveal whether abnormal LCI in those with preserved FEV1 predicts future clinical change.
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Clinical respiratory physiology, exercise and functional imaging Source Type: research
This study assesses the utility of the ISWT in patients with non-group 1 PH.Methods: Data were retrieved from the ASPIRE registry (Assessing the Spectrum of Pulmonary hypertension Identified at a REferral centre) for consecutive patients diagnosed with pulmonary hypertension between 2001-10 (2). For inclusion patients were required to have been systematically assessed as Group 2-5 PH and to have a baseline ISWT within 3 months of cardiac catheterization. Patients were stratified according to ISWT distance (ISWD) and ISWD%pred.Results: 479 patients with non-group 1 PH were identified. ISWD and ISWD%pred correlated significa...
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Pulmonary hypertension Source Type: research
Conclusion: Despite the development of treatments, the recent implementation of screening programs, the prognosis for PAH remains reserved.
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Pulmonary hypertension Source Type: research
Background: Pulmonary hypertension (PH) associated with interstitial lung disease (ILD) is associated with significant mortality.Aims: To describe the demographics and outcomes of patients with ILD referred to a National PH service.Methods: All patients with ILD referred for right heart catheterisation (RHC) between 2005 and 2015 were included. Patients were excluded if they had evidence of CTEPH or
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Pulmonary hypertension Source Type: research
Background: Pulmonary hypertension (PH) is often associated with respiratory diseases (R-PH), but pulmonary arterial hypertension (PAH)-specific therapy has not been approved for R-PH. We previously showed an improved survival in patients who received phospphodiesrerase-5 inhibitors for severe R-PH patients.Aim: To evaluate the demographics, survival and prognoses of R-PH patients in pulmonary specialized centers in Japan.Methods: We prospectively registered R-PH patients with confirmed mean pulmonary arterial pressure (PAP) ≥25mmHg by right heart catheterization from 21 participating pulmonary specialty centers between...
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Pulmonary hypertension Source Type: research
Introduction: The presence of pulmonary hypertension (PH) before lung transplantation (LuTx) is a well known risk factor for primary graft dysfunction (PGD). However, the role of the comprehensive cardiopulmonary hemodynamic assessed before LuTx is limited. We aimed to explore if other hemodynamic variables may have a role in PGD, besides absolute PH.Methods: a retrospective study was conducted at Policlinico Hospital of Milan (Italy) on adults who underwent LuTx for any indication other than cystic fibrosis from 2011 to 2017. PGD was defined as PaO2/FIO2
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Transplantation Source Type: research
Authors: Naranjo M, Lo KB, Mezue K, Rangaswami J Abstract Pulmonary hypertension is not uncommon in patients with renal disease and vice versa; therefore, influences treatments and outcomes. There is a large body of literature on pulmonary hypertension in patients with kidney disease, its prognostic implications, economic burden, and management strategies. However, the converse, namely the hemodynamic effects of pulmonary hypertension on kidney function (acute and chronic kidney injury) is less studied and described. There is also increasing interest in the effects of pulmonary hypertension on kidney transplant out...
Source: Current Cardiology Reviews - Category: Cardiology Tags: Curr Cardiol Rev Source Type: research
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