Rare condition has left a teenager with the ‘heart and lungs of a pensioner’
Yasmin Swift, 19, from Ashford, Kent, needs a double lung and heart transplant after being diagnosed with idiopathic pulmonary arterial hypertension in November last year.
Inhalational drug delivery to treat PAH is attractive due to enhanced pulmonary specificity and reduced systemic adverse effects. LIQ861 is a dry powder formulation of Treprostinil (TRE) designed to enhance deep-lung delivery and achieve higher tolerated dose levels than current inhaled therapies. LIQ861 ’s uniform (1µm) particle size with a trefoil, pollen-like shape, enables QID delivery of TRE doses in a convenient, palm-sized, disposable dry powder inhaler (DPI). The primary objective of the INSPIRE study is the safety and tolerability of LIQ861 in subjects transitioning from stable doses of Tyvaso® or ...
We present a series of patients with sarcoidosis associated PH and describe correlation between the pre-transplant hemodynamic data from a right heart catheterization (RHC) and vascular morphologic changes in the native explanted lungs.
Central veno-arterial (VA) ECMO provides reliable cardiopulmonary support in selected patients with end-stage lung diseases and severe pulmonary hypertension or cardiac instability. Ambulation dramatically improves patient outcomes. We investigated the outcomes of a new technique of ambulatory central VA ECMO via tunneled cannula insertion and right thoracotomy.
Pre-transplant irreversible pulmonary hypertension (PH) is a contraindication to heart transplantation (HTx) due to the high risk of post-transplant right ventricular (RV) dysfunction. When ventricular assist devices (VAD) are not available, HTx may be considered in selected patients PH.
There is a lack of evidence to guide appropriate donor sizing in recipients with moderate pulmonary hypertension (PH) awaiting transplant. Best practice suggests to oversize hearts for such recipients to prevent post-operative right ventricular failure.
Cardiac allograft vasculopathy (CAV) is a highly prevalent vaso-occlusive disease that is a leading cause of graft failure and mortality after heart transplantation. While the pathogenesis of CAV remains incompletely understood, histologic evidence suggests that macrophages, which constitute an important part of the innate immune response, may play an important role. Prior studies in pulmonary hypertension have shown that macrophage-derived leukotriene B4 (LTB4) induces proliferation and hypertrophy of human pulmonary artery smooth muscle cells.
The objectives of the study were twofold, to determine the effects of β-receptor blockade on 1) ventricular function; and 2) myocardial sympathetic nervous system (SNS) function in an animal mod el of PAH.
Patients with World Health Organization (WHO) functional class (FC) I/II pulmonary arterial hypertension (PAH) are often considered to be patients whose disease is controlled. However, little is known about their prognosis compared with patients with WHO FC III/IV disease. This meta-analysis evaluated survival outcomes in observational registries by FC, and compared treatment effects observed in randomized controlled trials (RCTs) by FC.
Suppressor of tumorigenicity 2 (ST-2) is a marker of cardiac remodeling and fibrosis that correlates with disease severity in patients with pulmonary arterial hypertension (PAH), and is thought to be a predictor of clinical worsening. We performed a post hoc analysis assessing ST-2 as a potential biomarker of likelihood of response in patients switching from phosphodiesterase type 5 inhibitors (PDE5i) to riociguat, and the association of ST-2 with REVEAL risk score (RRS) in the RESPITE study.
Prior research suggests that PAH prevalence and subtype may vary by race/ethnicity. Although some data suggest that disease severity and clinical outcomes also differ by race/ethnicity, these data are limited and based on single-center analyses. The relationship between race/ethnicity and survival was evaluated in a US prospective multicenter registry of WHO group 1 PAH, the Registry to Evaluate Early and Long-term PAH Disease Management (REVEAL).