Female subjects, on a reliable method of birth control or not physically able to bear children. Talk with your doctor and family members or friends about deciding to join a study. Patients with moderate to severe pulmonary hypertension undergo heart catheterization to evaluate the severity of hypertension before beginning sildenafil therapy.
Any unstable (chronic or acute) condition that in the opinion of the investigator will prevent completion of the study. Listing a study does not mean it has been evaluated by the u. Risk factors for death in 632 patients with sickle cell disease in the united states and united kingdom.
The relationship between the severity of hemolysis, clinical manifestations and risk of death in 415 patients with sickle cell anemia in the us and europe. This study will examine whether the drug sildenafil can lower blood pressure in the pulmonary artery (the blood vessel that leads from the heart to the lungs) in patients with sickle cell disease and pulmonary hypertension (high blood pressure in the lungs). Screened patients who do not qualify for participation in the main interventional trial may be contacted every 6-12 months for up to 3 years to assess major disease-related complications, including mortality.
Systemic fat embolism and pulmonary hypertension in sickle cell disease. Electrophoretic documentation of sickle cell disease (including, but not limited to ss, sc, sd, or s-beta zero thalassemia). History of sustained priapism requiring medical or surgical treatment, unless currently impotent or on transfusion program within the last two years.
Assessments will include historical and laboratory data, doppler echocardiogram, 6-minute walk test, plasmaserum, and dna for banking. Subjects with systolic blood pressure greater than or equal to 140 mmhg or diastolic blood pressure greater than or equal to 90 mmhg. The primary outcome measure was change in exercise capacity assessed by 6 minute walk distance in meters from baseline to 16 weeks.
In the opinion of the investigator, able to complete the protocol scheduled assessments during the 16-week, double-blind phase. There was a balancing of treatment group assignment across tricuspid regurgitant jet velocity(trv)measured on echo placebo 20mg three times daily for 6 weeks,followed by 40mg three times daily for 4 weeks followed by 80mg three times daily for 6 weeks. . The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Read our (subjects on drug were more likely to have severe pain crises requiring hospitalization.
Tricuspid regurgitant jet velocity was measured by transthoracic doppler echocardiography. Machado rf, barst rj, yovetich na, hassell kl, kato gj, gordeuk vr, gibbs js, little ja, schraufnagel de, krishnamurti l, girgis re, morris cr, rosenzweig eb, badesch db, lanzkron s, onyekwere o, castro ol, sachdev v, waclawiw ma, woolson r, goldsmith jc, gladwin mt walk-phasst investigators and patients. Echocardiographic markers of elevated pulmonary pressure and left ventricular diastolic dysfunction are associated with exercise intolerance in adults and adolescents with homozygous sickle cell anemia in the united states and united kingdom. Subjects who are anticoagulated and have proliferative retinopathy (unless they have had ophthalmologist recommended intervention (e. For general information, males or females, greater than or equal to 12 years of age and less than or equal to 70 years of age.
The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. The randomized, double-blind, placebo controlled phase is designed to determine the effects of 16 weeks of sildenafil therapy on exercise endurance, cardiopulmonary hemodynamic parameters and symptoms in this patient population. Sildenafil has been proven beneficial in pulmonary hypertension (ph) and recent phase iii studies from the intramural national institutes of health (nih) suggest it is well tolerated and efficacious in the scd population. This study will examine whether the drug sildenafil can lower blood pressure in the pulmonary artery (the blood vessel that leads from the heart to the lungs) in patients with sickle cell disease and pulmonary hypertension (high blood pressure in the lungs). The severity is measured on a 10 point scale with 0 nothing at all and 10maximum severity of breathlessness.
At least mild pulmonary hypertension with trv greater than or equal to 2. Left ventricular ejection fraction (lvef) less than 40 percent or clinically significant ischemic, valvular or constrictive heart disease lvef less than 40 percent or sf less than 22 percent. Subjects without a week 16 assessment had their last observation carried forward. Sickle cell disease (scd) is an autosomal recessive disorder and the most common genetic disease affecting african-americans. . Males or females, 12 years of age or older and less than or equal to 70 years of age. Assessments will include historical and laboratory data, doppler echocardiogram, 6-minute walk test, plasmaserum, and dna for banking. Furthermore, a number of recent studies have suggested that nitric oxide (no) based therapies may have a favorable impact on sickle red cells at the molecular level and could improve the abnormal microvascular perfusion that is characteristic of sickle cell anemia. Read our (subjects on drug were more likely to have severe pain crises requiring hospitalization. Acute or chronic impairment (other than dyspnea), limiting the ability to comply with study requirements (in particular with 6mwt), e.PDF on ResearchGate | Severe, chronic pulmonary hypertension in childhood is uncommon, difficult to treat, and carries a poor prognosis. Sildenafil (Viagra, Pfizer) has been used…