Intravenous L-Citrulline to Treat Children Undergoing Heart Bypass Surgery
Heart DefectsCongenital2 moreThis clinical trial will determine the safety and effectiveness of intravenous L-citrulline in children undergoing cardiopulmonary bypass during heart surgery. Participants will be randomly assigned to either L-citrulline or a placebo (a substance that has no medicine in it). Citrulline is a protein building block in the body that can convert into another substance, nitric oxide (NO), which controls blood pressure in the lungs. Increased blood pressure in the lungs can be an important surgical problem; it may also lead to problems following surgery, such as severe high blood pressure in the lungs (pulmonary hypertension), increased time spent on a breathing machine, and a longer stay in the intensive care unit (ICU). The hypothesis of this study is that perioperative supplementation with intravenous citrulline will increase plasma citrulline, arginine and NO metabolites and prevent elevations in the postoperative PVT leading to a decrease in the duration of postoperative invasive mechanical ventilation.
Impact of Face Masks on 6MWD in Patients With Pulmonary Hypertension
Pulmonary HypertensionPulmonary hypertension (PH) is classified according to the Nice Classification into different etiologies, including pulmonary arterial hypertension (PAH), a disease of the pulmonary arteriolar vasculature (Class I), and forms of pulmonary hypertension associated with left heart disease (Class II), lung disease (Class III), pulmonary artery obstructions including chronic pulmonary embolism (Class IV) or other less common causes (Class V). Patients with PH are at risk in the current COVID 19 pandemic. The course of the disease and the prognosis of the patients are assessed on the basis of various parameters and therapy is adapted accordingly. In addition to clinical, echocardiographic and laboratory examinations, cardiopulmonary performance tests such as the 6-minute walking distance (6MWD) are of particular significance. According to the ESC/ERS guidelines for PH and the recommendations of the Cologne Consensus Conference, exercise performance is a central criterion for prognostication and treatment decisions. During the COVID-19 pandemic, hospitals require the constant use of face masks for patients, in most cases also during the 6 minute walking test. We suspect a performance-reducing effect of face masks, thus impacting the results of the 6MWD. A systematic error in the assessment of cardiopulmonary performance should be revealed by comparing the results of the 6MWD with and without mask (particularly surgical mask and FFP2 mask).
Exercise Therapy After Pulmonary Thromboendarterectomy or Balloon Pulmonary Angioplasty for Chronic...
Chronic Thromboembolic Pulmonary HypertensionThis study is being completed to determine the feasibility and acceptability of completing a home-based, structured, low-to-moderate intensity exercise training program in chronic thromboembolic pulmonary hypertension (CTEPH) patients following surgical or percutaneous intervention. Eligible participants will be enrolled and have a 12 week home based exercise training program. The study team hypothesizes that: The following percentage of participants successfully complete the ramp-up phase of the exercise program: Greater or equal to 70% at end of week 7 Greater or equal to 80% at end of week 10 Greater or equal to 90% at end of week 12 Greater or equal to 80% of participants will both complete ≥1 week of maintenance phase exercise and complete 12 weeks of the exercise intervention. Patients will have no adverse events, defined as syncope, worsening World Health Organization (WHO) functional class, pulmonary hypertension (PH) related hospitalization, or death, caused by the exercise intervention.
The Effect of Remote Ischemic Preconditioning on the Ischemic Reperfusion Injury in Infants With...
Ventricular Septal DefectPulmonary HypertensionIntraoperative myocardial and pulmonary protection is important for better outcome after cardiac surgery. Ischemic preconditioning is one of organ protective strategies against ischemia-reperfusion injury by applying brief ischemia to the target organ before a subsequent critical ischemia, and its effect has been confirmed. However, its clinical application is not easy because ischemic insult may aggravate the function of vulnerable organ. On the other hand, remote ischemic preconditioning (RIPC) is another protective approach by applying ischemia to other less vulnerable organ such as skeletal muscle before critical ischemia-reperfusion injury to heart. The effect of RIPC has been well demonstrated in adults and children. However, Little is known about the effect of remote ischemic precondition on the pediatric myocardium to ischemia and reperfusion injury. The effect of RIPC on the children remains to be further evaluated because the degree of ischemia-reperfusion injury is different according to age, cardiac pathology and cyanosis. In addition, the previous report on children dealt with a diverse range of congenital heart defects with a wide age range. The purpose of this study was to evaluate the effect of RIPC on myocardial and pulmonary protection in infants with pulmonary hypertension who need repair of simple ventricular septal defect.
Immunoadsorption in Patients With Pulmonary Hypertension
Pulmonary HypertensionPulmonary ResistanceThe purpose of this study is to investigate, if Immunoadsorption of autoantibodies with subsequent substitution of immunoglobulins is able to improve haemodynamics in patients with pulmonary hypertension.
Lung Protective Ventilation in Pulmonary Thromboendarterectomy (PTE) Patients
Chronic Thromboembolic Pulmonary HypertensionAcute Lung InjuryChronic thromboembolic pulmonary hypertension (CTEPH) is caused by unresolved thromboemboli in the pulmonary arteries, which lead to pulmonary hypertension and, left untreated, right heart failure. This disease can be potentially cured by performing a pulmonary thromboendarterectomy (PTE) to remove the blood clots. The surgery is not without risk and the most worrisome complication is the development of a form of acute lung injury called reperfusion lung injury, which occurs in about 40 percent of patients. The landmark publication of the ARDSNET study demonstrated that a low tidal volume strategy of mechanical ventilation, decreased morbidity and mortality in patients who had acute respiratory distress syndrome (ARDS). Since then there have been some studies examining the role of a low tidal volume strategy in all patients who are mechanically ventilated. Some studies have demonstrated a decreased incidence of acute lung injury while others have failed to do the same. In patients at high risk for developing acute lung injury, such as patients undergoing PTE, there may be a benefit to using low tidal volumes to reduce the incidence of reperfusion lung injury. To assess the efficacy of a low tidal volume ventilation strategy in patients undergoing PTE, 134 patients will be randomized at the time of surgery to either low tidal volumes (6ml/kg of ideal body weight), or standard tidal volumes (10ml/kg of ideal body weight). Patients will be followed clinically to assess for the development of reperfusion lung injury. This will be defined as the development of hypoxemia (PaO2/FiO2 ratio less than 300) and chest infiltrates in the area of reperfused lung with no other identifiable etiology within the first 72 hours of surgery. Patients will also be assessed for other factors known to contribute to acute lung injury including: plateau pressures, peak inspiratory pressures, fluid balance, and number of transfusions received. Secondary endpoints of the study will be: time to successful spontaneous breathing trial, ventilator free days, ICU free days, hospital free days, and mortality.
Evaluate Effect of Optison on Pulmonary Artery Systolic Pressure (PASP) and Pulmonary Vascular Resistance...
Pulmonary HypertensionThe design of this study is to conduct a comprehensive safety evaluation of the pulmonary hemodynamic effects of Optison. The study is being conducted in subjects referred for cardiac catheterization for clinical reasons.
Inhaled Nitric Oxide (INO) In Hypoxic Respiratory Failure
Hypoxic Respiratory Failure With Pulmonary HypertensionThis Phase 3, Double-blind, Randomized, Placebo controlled, multicenter study is to confirm the efficacy of inhaled nitric oxide for the management of hypoxic respiratory failure associated with pulmonary hypertension in preterm infants. Study subjects will be administered either inhaled nitric oxide or placebo to determine if there is a change in oxygenation.
Oral Treprostinil in Subjects With Pulmonary Hypertension Associated With Pulmonary Fibrosis
Pulmonary HypertensionPulmonary FibrosisThis multicenter, randomized, open-label study will assess the safety and efficacy of oral treprostinil in subjects diagnosed with pulmonary hypertension associated with pulmonary fibrosis.
A Study of Stellate Ganglion Block and / or Reserpine in Group 2 Pulmonary Hypertension
Pulmonary Artery HypertensionPulmonary hypertension Group 2 (PH 2) associated with left-sided heart disease is relatively common, is associated with a poor prognosis, and unfortunately there are no proven medical treatments beyond attempts at correcting the left sided heart disease. Many PH 2 patients have evidence of active constriction of blood vessels from increased nerve traffic. Use of agents or procedures which produce a reversible chemical blockage of this nerve traffic have not been systematically tested in PH 2. The investigators will test whether acute interruption of sympathetic nervous system tone, which local anesthetic block of the stellate ganglion in the neck, will improve PH2, and also test whether the high blood pressure drug reserpine, which blocks sympathetic nerve activity, will do so during a one month trial period.