Eisenmenger Syndrome

Researchers are evaluating whether adding sotatercept to standard medicines can help adults with pulmonary arterial hypertension PAH caused by unrepaired congenital heart defects such as atrial or ventricular septal defects and patent ductus arteriosus, including those with Eisenmenger syndrome. This condition affects lung blood vessels and oxygen levels, and the study focuses on adults whose PAH has not improved enough with current vasodilator treatments. The trial is a randomized Phase 4 study conducted in Japan, aiming to understand sotatercepts effects in this specific group. Participants will be randomly assigned in a 21 ratio to receive either sotatercept plus usual vasodilator therapy or vasodilator therapy alone for 24 weeks. Sotatercept is given as subcutaneous injections every 3 weeks, starting with a lower dose and increasing if safety criteria are met, with dose adjustments based on blood counts. All participants continue their stable PAH therapy, and changes are generally discouraged unless needed for safety. During the study, participants will attend visits at the start, week 12, and week 24 to undergo tests including a 6-minute walk test, blood tests, questionnaires, and heart-lung assessments used in routine PAH care. Researchers will monitor changes in walking distance, clinical events, and heart-lung function. Safety monitoring includes regular blood counts to manage potential side effects. The total participation lasts about six months, with study treatments and tests provided at no cost.

Researchers are studying the effects of percutaneous interventions for branch pulmonary artery PA stenosis on exercise capacity in patients with dextro transposition of the great arteries d-TGA, Tetralogy of Fallot ToF, and Truncus Arteriosus TA. This randomized controlled trial also aims to assess the impact of these interventions on right ventricular RV function and to identify early markers to improve the timing of treatment. The study responds to the need for clearer guidelines on when to perform these interventions, as current knowledge about their effects on exercise capacity and heart function is limited. Participants are divided into two groups one undergoing percutaneous stent placement for PA stenosis and a control group receiving conservative management with potential delayed intervention. Both groups will receive standard care evaluations, including transthoracic echocardiogram TTE, cardiopulmonary exercise testing CPET, and cardiac magnetic resonance imaging CMR. The interventional group will have additional low dose dobutamine stress MRI at certain centers and RV pressure measurements during the intervention. Quality of life questionnaires will be collected at baseline and shortly after intervention or corresponding time points in controls. Long-term follow-up with TTE, CPET, and CMR will occur within 2 to 4 years. During the study, participants will undergo examinations at baseline and approximately six months later to measure exercise capacity VO2 max, RV function, and adaptation. Assessments include imaging, exercise testing, and questionnaires to evaluate heart performance and quality of life. Researchers will compare results between groups to determine the effects of the intervention. Safety and technical success will also be monitored through pressure and gradient measurements. The total study duration includes initial assessments, intervention or observation periods, and extended follow-up to understand long-term outcomes.

Researchers are evaluating how changes in right atrial reservoir strain RASr can help identify fluid responsiveness in patients with septic shock. The study focuses on determining if variations in RASr after a fluid challenge or passive leg raising PLR maneuver can predict increases in cardiac output. This is important for managing fluid treatment to avoid unnecessary administration in critically ill septic shock patients. Participants will undergo transthoracic echocardiography TTE at several time points before and after the PLR maneuver, and before and after a fluid challenge. These procedures are routine for septic shock care but will be repeated as part of the study to measure changes in cardiac output and RASr. The TTE is non-invasive, painless, and performed at the bedside with no known risks. During the study, patients will have TTE scans at baseline, and at 10, 30, and 45 minutes after fluid challenge or PLR. The main outcome measured is the change in RASr value after fluid challenge. Secondary outcomes include comparisons of stroke volume, central venous pressure, and right ventricular function. No additional follow-up or contact is required after the final TTE scan. The study concludes once all measurements are collected.

Researchers are studying familial cancer in children and their families to discover new genetic causes. This observational study focuses on families with a history of cancer to identify novel cancer-predisposing genes and genetic variants. The study aims to expand understanding of hereditary cancer predisposition by using next generation sequencing NGS technologies and establishing a linked data registry and biological sample repository. Participants provide blood samples, saliva, skin samples when needed, and occasionally leftover tumor or bone marrow samples. These biological specimens are stored in a biorepository and analyzed to detect gene changes potentially responsible for familial cancer. Participants may also be asked yearly for updated health and family history information if they agree to future contact. During the study, researchers collect medical and family histories along with biological samples to perform DNA sequencing and other genetic analyses. Samples are coded to protect identity and stored for current and future research. The primary outcome is identifying new cancer-related genes over up to 20 years. Participation involves sample collection and periodic updates, with ongoing confidentiality and long-term monitoring.

Researchers are evaluating the feasibility of a larger platform trial called CRAVE to test multiple treatments aimed at improving right ventricular function in people with pulmonary hypertension and right heart failure. This phase 2, randomized, open-label trial will include 30 participants to compare the effects of empagliflozin, ranolazine, both added to standard care, against standard care alone. The study seeks to determine whether a larger trial can be successfully conducted by assessing recruitment, consent, and data collection processes. Participants will be randomly assigned to one of three groups empagliflozin 10 mg once daily plus standard care, ranolazine starting at 500 mg twice daily then increasing to 1000 mg twice daily after two weeks or 500 mg twice daily if taking certain other medications plus standard care, or standard care alone. The treatment period lasts 16 weeks, during which participants will be monitored and their outcomes recorded. During the 16-week study, participants will undergo various assessments including medical record review to track right ventricular function, exercise capacity both virtual and in-person, biomarker levels, hemodynamics, and quality of life using several questionnaires. Researchers will also monitor enrollment rates, loss to follow-up, and ability to gather comprehensive data. This thorough monitoring aims to inform the design of a larger future trial. Participation involves regular follow-up and data collection but no blinding or placebo control.

This observational study aims to collect real-world data on the Abiomed Impella RP Flex, a device designed to support the right side of the heart. The study focuses on patients with right ventricular dysfunction who have received this mechanical circulatory support. The purpose is to understand how the device performs outside of controlled clinical trial settings. Participants will have received the right-sided ventricular assist device called the Impella RP Flex, which is a percutaneous device used to support right heart function. There are no separate treatment groups or placebo comparisons since this is an observational registry of patients already implanted with the device. During the study, researchers will monitor patient outcomes up to one year after the device implant. They will collect data related to the objectives set for this time frame, focusing on safety and performance as observed in routine clinical use. Participants ongoing health and device function will be tracked through standard medical follow-up and data collection methods.

This research focuses on patients undergoing cardiac surgery with cardiopulmonary bypass CPB who have pulmonary hypertension PH or right ventricular dysfunction. The study is a randomized, double-blind, controlled clinical trial evaluating whether inhaled epoprostenol combined with milrinone can improve outcomes during the transition off CPB. Pulmonary hypertension and right ventricular dysfunction are linked to increased risks such as longer mechanical ventilation, extended hospital stays, and higher mortality, making improved management important. Participants will be randomly assigned to receive either a combination of inhaled epoprostenol and milrinone or a placebo before the start of CPB. The inhaled treatments are delivered via an ultrasonic nebulizer over 20 minutes. The experimental group receives 4mg of milrinone and 60 mcg of epoprostenol, while the control group receives saline. The study aims to compare the rate of difficult CPB weaning, defined by the need for inotropes, vasopressors, mechanical support, or return to bypass. During the trial, patients will be monitored for hemodynamic stability and clinical outcomes including vasopressor and inotrope use, duration of organ dysfunction, and length of ICU stay. Various hemodynamic parameters and postoperative complications will be assessed for up to one year. The total participant involvement covers the perioperative and postoperative period to evaluate the impact of the inhaled combination therapy on cardiac surgery recovery.

Researchers are studying Eisenmenger syndrome, the most advanced form of pulmonary arterial hypertension linked to congenital heart disease. This condition causes chronic low oxygen levels and increased red blood cells, which raises the risk of blood clots. The study aims to see if taking pentoxifylline, a drug that may reduce blood cell clumping and inflammation, raises levels of thrombomodulin, a natural protein that helps prevent clotting and inflammation in these patients. This is a prospective, single-center, randomized study including 48 adults with Eisenmenger syndrome already on standard treatments for pulmonary arterial hypertension. Participants are randomly assigned to either continue their routine therapy or receive pentoxifylline alongside their usual treatment. Pentoxifylline is taken orally at 400 mg per day for the first 30 days, then increased to 800 mg per day for the next five months, completing a six-month treatment period. During the study, researchers will measure blood levels of thrombomodulin and other clotting markers at 3 and 6 months. They will also assess changes in monocyte proteins related to clotting, physical capacity, oxygen levels, blood counts, and heart function. The study will monitor clinical events and safety throughout. All participants maintain their regular pulmonary arterial hypertension treatment during the trial, which is planned to end in September 2026.

Researchers are studying heart function in people with chronic obstructive pulmonary disease COPD to better understand how the right side of the heart responds during rest and different levels of exercise. This study focuses on identifying patterns of right ventricular dysfunction, which may help guide improved treatments for COPD patients in the future. Participants will undergo testing while resting and during moderate- and high-intensity exercise. After a brief warmup, they will perform about 5 minutes of moderate exercise followed by roughly 2 minutes of high-intensity exercise. These activities help identify how the right ventricle of the heart responds under different conditions. During the study, heart function will be measured using transthoracic echocardiography and, in some cases, conductance catheter techniques. Researchers will also assess participants quality of life through questionnaires, measure maximum oxygen consumption, analyze blood samples for specific compounds, and monitor physical activity over a week. Each assessment session lasts up to 1 hour, with physical activity tracked for 7 days. The study is sponsored by the University of Colorado, Denver, and participation involves these tests and monitoring to understand heart response in COPD.

Pulmonary arterial hypertension PAH causes strain on the right side of the heart, which can lead to right heart failure. Unlike left heart failure, there are currently no treatments directly targeting right heart function. Researchers are evaluating valsartan, a medication typically used for high blood pressure and left heart failure, to see if it can help strengthen the right heart in adults with PAH. This is a Phase 2 randomized placebo-controlled trial studying valsartans safety and clinical effect over 24 weeks. Participants will be randomly assigned to take either valsartan 40 mg capsules or placebo capsules twice daily for 24 weeks. Study visits will occur at weeks 0, 2, 12, and 24, with most visits lasting 20 to 30 minutes in addition to usual clinic appointments. While visits at weeks 2 and 12 may be done remotely, echocardiograms will be done at the start and end of the study. Some participants may choose to undergo optional exercise testing or right heart catheterization at weeks 0 and 24. During the study, participants will be monitored through clinical assessments including echocardiograms and questionnaires about heart function and quality of life. The main outcome measured is the six-minute walk distance over 24 weeks. Secondary outcomes include changes in heart function measurements, heart failure classification, biomarkers, quality of life scores, and frequency of increased pulmonary hypertension treatments. The total participation time is 24 weeks, with safety and clinical data collected throughout.