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 sotatercept's effects in this specific group. Participants will be randomly assigned in a 2:1 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.

This research aims to evaluate the effects of steroids on the early postoperative inflammatory response in children undergoing elective congenital heart surgery using cardiopulmonary bypass (CPB). The study focuses on pediatric patients with cyanotic and acyanotic heart disease who require CPB during their first cardiac surgery. The trial is a phase 4 randomized controlled study designed to monitor inflammatory markers and clinical outcomes related to steroid use in this setting. Participants will receive either intravenous methylprednisolone at a dose of 30 mg/kg (up to 500 mg) or intravenous normal saline as a placebo. These treatments are administered during the surgery involving CPB. The study includes two groups: an experimental group receiving methylprednisolone and a placebo comparator group receiving saline. The trial uses triple masking to ensure unbiased evaluation. During the study, blood samples will be collected before the first dose, immediately after protamine infusion, and at 24 and 48 hours following CPB. These samples will measure serum levels of interleukin-6, C-reactive protein (CRP), and creatinine to assess inflammatory and kidney function responses. Participants will be monitored closely in the cardiac intensive care unit during the initial 24 hours and followed up as per protocol. The total duration of participation varies based on these assessments and clinical care needs.

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.

This research investigates hereditary cancer predisposition by identifying novel genes and genetic variants that may contribute to familial cancer. While hereditary factors are known to cause some cancers, many causes remain unknown. The study focuses on families with cancer clustering, aiming to better understand genetic links to improve diagnosis and care for hereditary cancer. Participants provide blood or saliva samples and medical and family history information. Leftover tumor samples may also be collected when available. Samples are stored in a biorepository and studied using next generation sequencing to detect gene changes. Some participants may provide skin samples if they had bone marrow transplants without available pre-transplant DNA. Participants may be contacted yearly for health and family history updates. During the study, researchers analyze DNA and other biological samples to find mutations linked to cancer. This includes sequencing, genetic and cellular studies, and possibly stem cell analyses. Personal information is removed, and samples remain in the repository for future research. The primary outcome is identifying new cancer predisposing genes, with follow-up of up to 20 years from study start.

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.

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 valsartan's 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.