A5086418594- Pulmonary Hypertension Research and Treatments
- Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
- Chronic Obstructive Pulmonary Disease (COPD) Research
- Heart Failure Treatment and Management
- Protease and Inhibitor Mechanisms
- Cardiovascular Issues in Pregnancy
- Vascular Anomalies and Treatments
- Cardiovascular Function and Risk Factors
- Obstructive Sleep Apnea Research
- Sarcoidosis and Beryllium Toxicity Research
- Delphi Technique in Research
- Peptidase Inhibition and Analysis
- Systemic Sclerosis and Related Diseases
- Long-Term Effects of COVID-19
- Medical Imaging and Pathology Studies
- Cardiac Arrhythmias and Treatments
- Respiratory Support and Mechanisms
- Congenital Heart Disease Studies
- Poisoning and overdose treatments
- Respiratory viral infections research
- Family and Patient Care in Intensive Care Units
- Health Systems, Economic Evaluations, Quality of Life
- Airway Management and Intubation Techniques
- Lymphatic Disorders and Treatments
- Eosinophilic Disorders and Syndromes
Cleveland Clinic Florida
2015-2025
United Therapeutics (United States)
2025
Triangle
2025
Cleveland Clinic
2015-2025
Mayo Clinic in Florida
2022
Inserm
2021
Université Paris-Saclay
2021
Bicêtre Hospital
2021
Assistance Publique – Hôpitaux de Paris
2021
Methodist Hospital
2020
Abstract Background Systemic sclerosis (SSc) is a rare, complex, connective tissue disorder. Interstitial lung disease (ILD) common in SSc, occurring 35–52% of patients and accounting for 20–40% mortality. Evolution therapeutic options has resulted lack consensus on how to manage this condition. This Delphi study was initiated develop recommendations based expert physician insights regarding screening, progression, treatment criteria, monitoring response, the role recent advances with...
Pulmonary hypertension (PH) is a common complication of interstitial lung disease (ILD) and associated with worse outcomes increased mortality. Evaluation PH recommended in transplant candidates, but there are currently no standardized screening approaches. Trials have identified therapies that effective this setting, providing another rationale to routinely screen patients ILD for PH.What strategies identifying supported by expert consensus?The study convened panel 16 pulmonologists...
Introduction: Alpha-1 antitrypsin deficiency (AATD) is a genetic disease that may be manifested by chronic obstructive pulmonary disease. Despite professional society guidelines recommend broad testing of at-risk individuals, fewer than 10% affected individuals have been identified. The goals this study were to estimate the frequency abnormal AAT genotypes among patients found fixed airflow obstruction and assess feasibility having Pulmonary Function Laboratory personnel administer study....
ABSTRACT Lung transplantation is indicated for selected patients with advanced pulmonary arterial hypertension (PAH). We used a modified Delphi process to develop recommendations on care of PAH undergoing lung transplantation. This panel was recruited from the Pulmonary Vascular Research Institute's Innovative Drug Discovery Initiative ‐ Transplantation Workstream, consisting clinical and research experts in In this process, 29 panelists were given open‐ended questions, querying topics...
Pulmonary arterial hypertension (PAH) is a progressive disease characterized by severe remodeling of the pulmonary artery resulting in increased pressure and right ventricular hypertrophy and, ultimately, failure. Bone marrow-derived progenitor cells play critical role vascular homeostasis have been shown to be involved pathogenesis PAH. A proliferation c-Kit(+) hematopoietic progenitors mast has noted remodeled vessels Imatinib, tyrosine kinase inhibitor that targets c-Kit, beneficial for...
Oral treprostinil was recently labeled for treatment of pulmonary arterial hypertension. Similar to the period immediately after parenteral approved, there is a significant knowledge gap practicing physicians who might prescribe oral treprostinil. Despite its route delivery, use drug challenging because requirement careful titration and management drug‐related adverse effects. We aimed create consensus document combining available evidence with expert opinion provide guidance Following...
The primary aim was to explore the safety and tolerability of inhaled treprostinil when used in patients with pulmonary hypertension (PH) concomitant chronic obstructive disease (COPD). Patients a diagnosis pre-capillary PH (defined as artery mean pressure ≥ 25 mmHg wedge or left ventricular end diastolic ≤ 15 mmHg) who were being initiated on had COPD FEV1/FVC ratio 70% FEV1 40% predicted) considered for inclusion this pilot study. Assessments included adverse events, physical exam, World...
Alpha-1 antitrypsin deficiency (AATD) is under-recognized by clinicians, with long diagnostic delays between patients' first symptom and initial diagnosis. Recent recommendations official societies encourage testing for AATD in all symptomatic adults spirometric evidence of COPD, though compliance this recommendation has been variable. For 6 months, the following physician alert was added to PFT reports patients airflow obstruction GOLD Stage II or higher: "The American Thoracic Society...
Nontuberculous mycobacterial (NTM) diseases are difficult-to-treat infections, especially in lung transplant (LTx) candidates. Currently, there is a paucity of recommendations on the management NTM infections LTx, focusing Mycobacterium avium complex (MAC), M. abscessus and kansasii.Pulmonologists, infectious disease specialists, LTx surgeons Delphi experts with expertise were recruited. A patient representative was also invited. Three questionnaires comprising questions multiple response...
Dual combination therapy with a phosphodiesterase-5 inhibitor (PDE5i) and endothelin receptor antagonist is recommended for most patients intermediate-risk pulmonary arterial hypertension (PAH). The RESPITE REPLACE studies suggest that switching from PDE5i to soluble guanylate cyclase (sGC) activator may provide clinical improvement in this situation. optimal approach escalation or transition of other scenarios not well defined. We developed an expert consensus statement on the sGC treatment...
Purpose: The extent of the survival benefit augmentation therapy for alpha-1 antitrypsin deficiency (AATD) in individuals with advanced COPD is difficult to define. We performed a retrospective analysis using all available data from observational registry severe (AAT) conducted by NHLBI investigators. Patients and Methods: Individuals (N=1129) AAT were evaluated mortality sources stratified 10% increments baseline forced expiratory volume 1 second (FEV1) percent predicted status (ever...
Nintedanib is an approved treatment for idiopathic pulmonary fibrosis (IPF), which slows disease progression. Management of patients with IPF receiving nintedanib can be complicated by tolerability issues, comorbidities, and concomitant medications. We developed consensus recommendations on the management dosing, adverse events comorbidities in treated nintedanib.A modified Delphi process using 3 questionnaires was used to survey 14 pulmonologists experienced nintedanib. Panelists rated...
In patients who require urgent initiation of pulmonary arterial hypertension medications due to disease progression, it is customary start intravenous prostacyclin therapy, typically during a hospital admission. If there are complicating factors or relative contraindications and subcutaneous prostanoids, oral treprostinil provides another pathway prostanoid but this usually requires prolonged titration. We describe the case thirty-six-year-old male with severe contraindication therapy...
Treprostinil is a prostacyclin analogue that targets multiple cellular receptors to treat pulmonary arterial hypertension (PAH). In certain scenarios, patients may require aggressive treprostinil titration. Several studies have demonstrated higher doses of lead greater clinical benefit. Data supports successful transitions from parenteral oral treprostinil; however, administration routes, transition duration, and setting vary in the real-world. The EXPEDITE trial (NCT03497689) prospectively...