Selexipag for the treatment of children with pulmonary arterial hypertension: first multicenter experience in drug safety and efficacy

The European Pediatric Pulmonary Vascular Disease Network (EPPVDN) investigated safety and efficacy of add-on selexipag, an oral IP prostacyclin receptor agonist approved for pulmonary arterial hypertension (PAH) in adults, in the largest, exploratory pediatric cohort to date.
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Tags: Feature Article Source Type: research

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CONCLUSIONS: PH-NF1 is characterized by a female predominance, a low DLCO and severe functional and hemodynamic impairment. Despite a potential benefit of PAH treatment, prognosis remains poor, and double-lung transplantation is an option for eligible patients. PMID: 32437637 [PubMed - as supplied by publisher]
Source: American Journal of Respiratory and Critical Care Medicine - Category: Respiratory Medicine Authors: Tags: Am J Respir Crit Care Med Source Type: research
This study provides direct evidence for the contribution of gut microbiota to the cognitive decline during normal aging and suggests that restoring microbiota homeostasis in the elderly may improve cognitive function. On Nutraceutical Senolytics https://www.fightaging.org/archives/2020/05/on-nutraceutical-senolytics/ Nutraceuticals are compounds derived from foods, usually plants. In principle one can find useful therapies in the natural world, taking the approach of identifying interesting molecules and refining them to a greater potency than naturally occurs in order to produce a usefully large therape...
Source: Fight Aging! - Category: Research Authors: Tags: Newsletters Source Type: blogs
The age of patients with pulmonary arterial hypertension (PAH) has increased, with registries now reporting mean ages of 50-65 years old. Limited data exist on age-related differences in hemodynamic and functional assessments in PAH.
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Source Type: research
Introduction Sarcoidosis-associated pulmonary hypertension (SAPH) is associated with reduced survival in single-centre studies. The international Registry for SAPH (ReSAPH) with long-term follow-up was established to enrich our knowledge of this complication of sarcoidosis. This analysis aims to elucidate factors associated with reduced transplant-free survival in SAPH patients. Methods ReSAPH contains prospectively collected outcomes of SAPH patients since the time of registry enrolment. Information analysed includes right heart catheterisation data, pulmonary function testing, chest radiography, Scadding stage and 6-min...
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Pulmonary vascular disease Original Articles: Pulmonary hypertension Source Type: research
Conclusion PAH due to TBX4 mutations may occur with or without skeletal abnormalities across a broad age range from birth to late adulthood. PAH is usually severe and associated with bronchial and parenchymal abnormalities.
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Genetics, Pulmonary vascular disease Original Articles: Pulmonary hypertension and genetics Source Type: research
ConclusionsA clear trend towards worse outcome with larger TRV or TR% was shown; however, the number of events was insufficient for significant outcome differences. Prognostic value of quantitative TR should be investigated in a larger multicentre cohort. Effective RV ejection fraction may be considered an improved measure of RV function in PAH.
Source: ESC Heart Failure - Category: Cardiology Authors: Tags: Original Research Article Source Type: research
An 11-year-old girl presented with pulmonary hypertension (PH), World Health Organization (WHO) functional class (FC) III associated with sickle cell disease (SCD) and history of a congenital diaphragmatic hernia (CDH) repair after birth. Her past medical history was significant for multiple episodes of pneumonia and pain crises. At age 7 years, her mean pulmonary artery pressure (mPAP) was 32 mmHg and pulmonary vascular resistance index (PVRi) was 3.6 Wood units x m2 by right heart cardiac catheterization (RHC) at another institute.
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Tags: Case Anecdotes, Comments and Opinions Source Type: research
Background: Screening and early diagnosis of pulmonary hypertension (PH) is critical for managing progression and preventing associated mortality, there are no tools for this purpose. We developed and validated an artificial intelligence (AI) algorithm for predicting PH using electrocardiography (ECG).Methods: This historical cohort study included data from consecutive patients from two hospitals. The patients in one hospital were divided into derivation (56,670 ECGs from 24,202 patients) and internal validation (3,174 ECGs from 3,174 patients) dataset, while the patients in the other hospital were included in only an exte...
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Tags: Original Clinical Science Source Type: research
A 61-year-old African American man with history of hypertension, coronary artery disease, end-stage renal disease (on hemodialysis since 2014) and end-stage heart failure secondary to arrhythmogenic right ventricular cardiomyopathy (ARVC) underwent dual organ heart-kidney transplantation in May, 2019. Approximately two months after transplant mycophenolate was discontinued due to episodes of pancreatitis, leukopenia and detectable BK polyoma virus. Since then, the patient was maintained on tacrolimus (goal level 8 ng/mL) and low-dose prednisone (5mg/day).
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Source Type: research
CoronaVirus Disease-19 (COVID-19) poses a threat to individuals with chronic health conditions who are more likely to develop severe pneumonia and death. Those with pulmonary arterial hypertension represent such a high-risk group. Severe COVID-19 presents with respiratory failure secondary to immuno-pathologic injury likely due to a combination of direct cytopathic effects of the virus in concert with an aberrant immune response. The interplay between these two components has been recently better understood.
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Tags: Case Anecdotes, Comments and Opinions Source Type: research
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