3 Clinical Trials for Various Conditions
This is a novel preliminary study of biomarkers of pathologic pre-operative pulmonary vascular development, elevated pre-operative Pulmonary Vascular Resistance Index (PVRi), and complications associated with decreased post-operative pulmonary blood flow in single ventricle patients undergoing superior cavo-pulmonary anastomosis (SCPA). The study will utilize a combined targeted and untargeted approach to both optimize translation of a promising existing biomarker and efficiently identify novel biomarkers and potential therapeutic targets in this population.
This study is a prospective, randomized study of patients with single ventricle heart disease who are to undergo superior cavo-pulmonary anastomosis, or "Glenn" operation. Such patients have historically undergone cardiac catheterization to ensure suitability for the procedure. Cardiac magnetic resonance imaging (cardiac MRI) is a newer technology that provides excellent anatomic and functional imaging of the heart. This study is designed to demonstrate our hypothesis that cardiac magnetic resonance imaging will provide comparable information to catheterization, with less side effects.
Patients with single ventricle anatomy undergo staged surgical palliation. The result is an "in series" circulation with pulmonary blood flow and cardiac output directly related to pulmonary vascular resistance. While surgical outcomes have improved, the physiology of the single ventricle palliation results in continued long term attrition. Elevated pulmonary vascular resistance and impaired systemic ventricular function are important risk factors for failure of single ventricle palliation. Sildenafil is a pulmonary vasodilator and has been shown to improve cardiac contractility in the pressure overloaded right ventricle. The investigators will assess the safety, pharmacokinetics and hemodynamic efficacy of sildenafil in single ventricle patients following stage II and III surgical palliation.