Saves the questions and what to expect into your notes, next to the visit they belong to.
Keep this study
Lead
The Hospital for Sick Children
Age
8–17
Sex
ALL
Healthy volunteers
Not accepted
You may be eligible if
Patients must be ≥ 8 and ≤ 17.5 years of age at the time of study enrollment.
Patients must have a mean pulmonary artery pressure of greater than 25mmHg at rest in a setting of normal pulmonary arterial wedge pressure of 15mmHg or less with a PVR index greater than 3 Woods units•m2 at last hemodynamic study.
Patients must be diagnosed with any of the following:Idiopathic PAH (IPAH), PAH associated with repaired congenital heart disease, PAH associated with minor congenital heart disease (small interventricular communication, small interarterial communication, small ductus arteriosis)
Patients must be clinically stable (i.e. no treatment changes) for the last 3 months
Patients must have no or minimal evidence of fluid overload or volume depletion judged by clinical evaluation (with or without diuretic treatment)
Written informed consent
You may not be if
Patients who are unable to perform a six minute walk test (6MWT)
Patients with a known history of pulmonary hypertension secondary to venoocclusive disease and/or capillary hemangiomatosis; pulmonary hypertensions owing to left heart disease
Patients who have previously received treatment with an intravenous positive inotropic agent in the last 3 months
Patients who are currently receiving β-blockers
Patients with a known history of reactive airways disease (bronchial asthma or relate bronchospastic conditions)
Patients with chronic obstructive pulmonary disease (COPD)
Patients with a known history of adverse reaction to β-blockers
Patients with a heart block on ECG or resting heart rate \< 60 bpm
Patients with coagulopathy (INR \< 1.5 or platelet count \<50,000/mm3)
Patients with a known history of severe hepatic impairment (defined by the presence of ascites, esophageal varices, jaundice or spider angiomata)
Patients with severe renal insufficiency (defined as creatinine clearance \< 30 mL/min/m2)
Patients with a known malignancy or other co-morbidity expected to limit survival or to limit the ability to complete the study
Patients with trisomy 21
Patients with a known history of sick sinus syndrome
Patients with a known history of moderate or severe primary obstructive valvular heart disease
Patients with a known history of diabetes
Female patients who are pregnant of breast-feeding
