A Multiple Dose Titration Study of MK-8892 in Participants With Pulmonary Hypertension and Left...
Pulmonary HypertensionLeft Heart DiseaseThis study will assess multiple doses of MK-8892 administered to participants with pulmonary hypertension "out of proportion" (PHOOP) and heart failure with reduced left ventricular ejection fraction (rEF). It is hypothesized that generally safe and well tolerated multiple doses of MK-8892 will achieve a true reduction from baseline in pulmonary vascular resistance (PVR) greater than 12%. Sixteen participants with PHOOP/rEF were to receive multiple doses of MK-8892 titrated to the highest tolerated dose for each participant (up to 4 mg daily), and to undergo evaluation for safety and systemic hemodynamics and cardiac function. Only 4 participants were enrolled and completed the study due to a strategic business decision by the sponsor to terminate the clinical conduct of all MK-8892 ongoing trials including this trial.
Pilot Study of the Safety and Efficacy of Anakinra (Recombinant Human Interleukin-1 Receptor Antagonist)...
Pulmonary HypertensionThis is an open-label non-randomized, pilot study to evaluate the effect of Interleukin-1 blockade on exercise capacity in patients with pulmonary hypertension. Subjects will undergo cardiopulmonary exercise testing at baseline, and after 4 weeks treatment with Anakinra (recombinant human Interleukin-1 receptor antagonist.
A Study of Patients Having Pulmonary Hypertension Associated With Sickle Cell Disease and Completing...
Pulmonary HypertensionSickle Cell AnemiaThis study will assess the safety and efficacy of bosentan therapy (in a study known as ASSET) for patients who have high blood pressure in the lungs associated with sickle cell disease. That form of hypertension places people at risk for complications, including shortness of breath, pain, pneumonia, and death. Previous studies have shown that bosentan can be helpful in reducing pulmonary hypertension. Patients ages 16 and older who have completed the 16-week treatment in the ASSET 1 or ASSET 2 study and who are not pregnant or breastfeeding may be eligible for this study. The research will be conducted in about 25 hospitals in the United States and Europe. Up to 30 participants will be enrolled. The screening visit will involve a physical examination, blood sample of about 3 teaspoons for laboratory tests, and a pregnancy test. Patients' doctors will give them bosentan tablets (62.5 mg each), to take one in the morning and one in the evening. After 1 month, patients will be told whether the dose should be increased to 125 mg tablets to take twice a day. Two weeks after the increase in dose, a blood test will be done to analyze the drug's effects on the liver. After the start of treatment, patients will return for visits every 6 months, when there will be a 6-minute walking test to measure exercise capacity and evaluate shortness of breath. There will be follow-up for patients up to the end of the study and for 28 days after the last dose of bosentan is taken, to collect information about side effects. Some patients on bosentan have had changes in liver function and red blood cell count. Side effects commonly reported are headache, flushed appearance, inflammation of the throat and nasal passages, and gastrointestinal symptoms. If patients have sudden worsening in breathing in the first few weeks after taking bosentan, they should immediately tell their doctors, because it may be necessary to change the treatment.
Hospital-Based Program for Treatment of Severe Cardiopulmonary Disease With Inhaled Nitric Oxide...
Pulmonary HypertensionLung Disease3 moreThe purpose of this program is to evaluate the logistic issues and patient requirements for chronic pulsed INOmax delivery in ambulatory, home-care patients. To understand patient needs, patients with a variety of underlying diseases will be included. Safety of chronic therapy will be monitored by serial measurements of methemoglobin, platelet function assay and reported adverse events.
Inhaled Nitric Oxide Study for Respiratory Failure in Newborns
InfantNewborn8 moreRespiratory failure in term newborns is associated with increased rates of death and long-term neurodevelopmental problems. This large international multicenter trial randomized newborns who had failed to respond to intensive care, including high levels of ventilator support, to receive either inhaled nitric oxide (iNO) or 100 percent oxygen to test whether iNO would decrease their risk of dying or requiring temporary lung bypass. Infants were followed during their initial hospitalization; their outcome was assessed at 18 to 24 mos of age.
Pulmonary Hypertension in Extremely Preterm Infants
Bronchopulmonary DysplasiaPulmonary Hypertension1 moreExtremely preterm infants are at risk for developing bronchopulmonary dysplasia (BPD) and associated chronic pulmonary hypertension (PH), a consequence of altered pulmonary vasculature. This condition occurs in about 25% of babies with BPD, and the association grows with increasing BPD severity. Other risk factors have been described as well. Morbidity and mortality associated with prematurity and/or BPD increase significantly in the presence of PH. Thus, international guidelines encourage the use of standardized screening protocols for this condition. However, several questions regarding these recommendations are left unanswered, such as a clear definition for PH in this population. The research aim is to prospectively evaluate prevalence, risk factors and clinical course of PH in these children. The investigators aim to identify at-risk infants early on and ultimately improve survival making use of an early targeted intervention.
Improving Treatment Personalization of Pulmonary Hypertension Associated With Diastolic Heart Failure...
Pulmonary HypertensionDiastolic Heart Failure1 moreHeart failure with preserved ejection fraction (HFpEF), is one of the leading causes of pulmonary hypertension (PH). Despite the severity of this disease, no established treatments exist for this class of PH. Nebivolol is a drug used in high blood pressure and heart failure, but not used in patients with PH. Due to some additional properties it possesses, the investigators believe nebivolol will improve disease severity in patients with PH associated with HFpEF. The hypothesis of this research study is that nebivolol improves PH severity in patients with HFpEF, as measured by hemodynamic and clinical parameters.
Quality of Life Assessed With the PAH SYMPACT Questionnaire
Pulmonary HypertensionResearchers are evaluating quality of life in pulmonary hypertension subjects using the Pulmonary Arterial Hypertension-Symptoms and Impact (PAH-SYMPACT) Questionnaire and assessing the questionnaires' performance with regard to relationship to other markers of disease severity, response to treatment, and outcome in a clinical practice setting.
Persistent Pulmonary Hypertension of the Newborn
Persistent Pulmonary Hypertension of the NewbornThe AC-052-391-study is a phase 3 study to investigate whether adding bosentan to inhaled nitric oxide in newborns with persistent pulmonary hypertension of newborns (PPHN) is a supporting and safe therapy and to evaluate the pharmacokinetics of bosentan and its metabolites.
129Xe MRI Cardiopulmonary
Interstitial Lung DiseaseChronic Thromboembolic Pulmonary Hypertension3 moreThe goal of this NIH-sponsored study is to characterize three biomarkers derived from 129Xe gas exchange MRI and to understand how they change in response to interventions.