United States CTEPH Registry
试验速览
- 阶段
- 不适用
- 状态
- 已完成
- 入组人数
- 754
- 试验地点
- 1
- 主要终点
- Describe the patterns of evaluation of CTEPH Patients
研究概览
简要总结
The U.S. CTEPH Registry is a multicenter, observational, U.S.-based study of the clinical course and treatment of patients diagnosed with chronic thromboembolic pulmonary hypertension (CTEPH), WHO Group IV Classification for Pulmonary Hypertension. The mission of the Registry will be to promote a greater understanding of the prevalence, pathophysiology, evaluation, and treatment of patients with CTEPH through shared information, education, and collaborative investigation among pulmonary hypertension (PH) centers of excellence throughout the U.S.
详细描述
Chronic thromboembolic pulmonary hypertension (CTEPH) is a subset of pulmonary hypertension (PH) characterized by the obstruction of pulmonary arteries with fibrotic material and vascular remodeling that leads to increased pulmonary arterial pressure and right ventricular failure.
A United States CTEPH Registry has been established under the guidance of a Steering Committee consisting of recognized physicians and scientists with expertise in CTEPH and related diseases. The mission of the Registry will be to promote a greater understanding of the prevalence, pathophysiology, evaluation, and treatment of patients with CTEPH through shared information, education, and collaborative investigation among PH centers of excellence throughout the U.S. The development of a national CTEPH registry will be an important element in the advancement of the understanding of CTEPH and improvement in the care of patients who suffer from this debilitating disease.
All consecutive consenting patients recently diagnosed (< 6 months) with CTEPH at participating study sites will be enrolled. At the time of study entry, data on prior history and evaluation will be obtained from patient interview and review of medical records. Patients will be followed biannually after enrollment until the conclusion of the study (minimum of 1 year follow-up for final patients enrolled).
Much of the data gathered by the Registry will be presented as descriptive statistics. The Registry will seek the assistance of a biostatistician to develop a statistical plan for within patient and between patient cohort analyses.
Approximately 750 patients with newly diagnosed (< 6 months) CTEPH will be enrolled.
研究设计
- 研究类型
- Observational
- 观察模型
- Cohort
- 时间视角
- Prospective
入排标准
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •Patients must be a permanent resident of the United States
- •Documentation of the following hemodynamic parameters by right heart catheterization
- •Mean pulmonary arterial pressure (PAPm) ≥ 25 mm Hg at rest and,
- •Pulmonary artery wedge pressure (PAWP) ≤ 15 mm Hg (or > 15 mmHg if justified)
- •Radiologic confirmation that chronic thromboembolic disease is the cause of the pulmonary hypertension by
- •One or more mismatched perfusion defect(s) by lung ventilation/perfusion scan, and
- •Confirmation of chronic thromboembolic disease by evidence of bands/webs, vessel narrowing or occlusion seen on CT pulmonary angiogram (CTA), conventional angiography or MR angiography (MRA).
- •Patients must be diagnosed with CTEPH within 6 months of being considered for study eligibility (signing of consent to participate). The date of diagnosis will be defined as when both hemodynamic criteria have been met and chronic thromboembolic disease is confirmed to be the cause of the pulmonary hypertension by an abnormal V/Q scan and the presence of chronic thromboembolic disease on CTA, MRA or pulmonary angiography. Hemodynamic and radiologic criteria can be met at separate time points; the most recently met criteria time point will be defined as the date of diagnosis.
- •Prior to enrollment all subjects must have the diagnosis of CTEPH confirmed by the Adjudication Committee.
排除标准
- •Patients unwilling or unable to provide written consent for participation in the study. Appropriate surrogate consent will be obtained for pediatric patients as defined by each investigational site's IRB.
- •Patients with an underlying medical disorder with an anticipated life expectancy less than 2 years.
- •Patients who do not meet inclusion criteria including:
- •Have not had documentation of hemodynamic criteria by right heart catheterization as outlined in the inclusion criteria
- •Do not have radiologic confirmation of chronic thromboembolic disease as outlined in the inclusion criteria
- •Meet the criteria for inclusion into WHO Groups I, II, III, or V
结局指标
主要结局
Describe the patterns of evaluation of CTEPH Patients
时间窗: up to 5 years
Describe the patters of risk factors and clinical presentation of CTEPH Patients
时间窗: up to 5 years
Outcomes of medical and surgical therapy of CTEPH patients as assessed by hemodynamics, quality of life, and functional status
时间窗: up to 5 years, at 6 month intervals
次要结局
未报告次要终点
研究者
Kim Kerr
Clinical Professor of Medicine
University of California, San Diego