Conditions: Heart Disease
Keywords: Hypoplastic left heart syndrome Hypoplastic right heart syndrome Superior cavopulmonary anastomosis Total cavopulmonary anastomosis Children with single ventricle anatomy
Allocation: NA | Intervention model: SINGLE_GROUP
Enrollment: 21
Healthy volunteers: no

Brief summary:
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.

Detailed description:

Arms:
arm 1: Pharmacokinetic and hemodynamic evaluation following sildenafil administration

Interventions: intervention 1: Sildenafil by injection intervention 2: Sildenafil by injection intervention 3: Sildenafil by injection intervention 4: Sildenafil by injection
Intervention descriptions: intervention 1: Sildenafil 0.125mg/kg injection over 20min intervention 2: Sildenafil 0.25mg/kg injection over 20min intervention 3: Sildenafil 0.35mg/kg by injection over 20min intervention 4: Sildenafil 0.45mg/kg by injection over 20min

Protocol document: