Cardiac Sarcoidosis Multi-Center Prospective Cohort Study
试验速览
- 阶段
- 不适用
- 状态
- 招募中
- 入组人数
- 1,500
- 试验地点
- 28
- 主要终点
- Clinically Silent and Control Patients
研究概览
简要总结
Recent data has shown that sarcoidosis, presenting initially with cardiac manifestations (CS) of either conduction system disease or cardiomyopathy and sustained VT, is not uncommon. A Canadian physician survey found that most physicians do not investigate for CS as a possibility in these situations. Thus many patients with clinically important CS are going un-diagnosed. A study from Finland showed that missing the diagnosis of CS in these patients' leads to significant mortality and morbidity.
There are no published clinical consensus guidelines on treatment of CS. Corticosteroid therapy is advocated by most experts. This is based on very modest data from small retrospective observational studies using variable definitions of clinical response. The effect of corticosteroid treatment on the clinical course of CS has not been studied in prospective studies and will be one of the aims of this project. Recent physician surveys regarding CS, in Canada and the US, found that current clinical practice varies widely. The 2008 American College of Cardiology/American Heart Association/Heart Rhythm society guidelines recommend implantation of a defibrillator (Class IIa recommendation) to prevent sudden cardiac death. The most recent Canadian device therapy guidelines do not mention CS.
A multi-center collaborative approach to study CS is greatly needed." The investigators propose exactly that i.e. a multi-center prospective cohort to start to answer clinical questions. The investigators have formed the CANADIAN CARDIAC SARCOIDOSIS RESEARCH GROUP. The group includes respirologists with an interest in sarcoidosis, cardiac electrophysiologists, cardiac imaging specialists with extensive experience in imaging of sarcoidosis and biostatisticians. The research will be in two phases; a registry of current diagnostic approaches, treatment and prognosis, and a randomized clinical trial of the effect of corticosteroid treatment on the clinical course of cardiac sarcoidosis.
详细描述
Baseline assessment of Clinically Manifest CS patients consists of: history, echocardiogram, ECG, chest CT scan, FDG-PET scan, blood for biomarkers within 2 months of the PET scan, cardiac MRI and possibly a signal average ECG and biopsy (encouraged-either endomyocardial or extra-cardiac).
Follow-up and clinical management of clinically manifest patients diagnosed with CS will occur at 3-6 months with a repeat FDG-PET scan and blood biomarkers. Follow-up will then be annually with an echo and ECG. Treatment with steroids/immunosuppressants and device therapy will be at the discretion of the treating physician.
Baseline assessment of patients diagnosed with extra-cardiac sarcoidosis and being screened for CS consists of: history, echocardiogram, ECG, holter, chest CT scan, biopsy, and cardiac MRI (CMR). If the CMR is suggestive of CS the patient will be have a FDG-PET scan done and be followed as a Clinically Silent patient. They will be contacted every 2 years. If the CMR is negative the patient will be followed as a extra-cardiac sarcoidosis patient with no evidence of CS and be in the control group. They will be contacted at 5 years and at the time of study completion.
All patients will be followed until the last patient recruited has been followed for 4 years.
The occurrence of the primary and secondary outcomes will be assessed in treated and untreated patients.
研究设计
- 研究类型
- Observational
- 观察模型
- Cohort
- 时间视角
- Prospective
入排标准
- 年龄范围
- 18 Years 至 99 Years（Adult, Older Adult）
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •To diagnose Clinically Manifest CS all following criteria must be met:
- •(i) Positive biopsy* for Sarcoid (either EMB or extra-cardiac) AND/OR (ii) CT Chest highly suggestive of pulmonary sarcoidosis AND (iii) one or more of the following clinical features:
- •advanced conduction system disease (sustained Mobitz II AV block or third degree AV block)
- •non- sustained or sustained ventricular arrhythmia
- •ventricular dysfunction (LVEF < 50% and/or RVEF < 40%) AND (iv) No alternative explanation for clinical features AND (v) FDG-PET suggestive of active CS
- •To diagnose clinically silent CS all of the following criteria must be met
- •(i) Biopsy proven extra-cardiac sarcoidosis
- •AND/OR (ii) CT Chest highly suggestive of pulmonary sarcoidosis
- •AND (iii) CMR suggestive of cardiac sarcoidosis
- •AND (iv) Does not have criteria for clinically manifest CS ie. should not have any of following
- •advanced conduction system disease (sustained Mobitz II AV block or third degree AV block)
- •non- sustained or sustained ventricular arrhythmia
- •ventricular dysfunction (LVEF < 50% and/or RVEF < 40%)
- •Patients with negative CMR will be designated as 'extra-cardiac sarcoidosis with no evidence of CS' and followed as control
排除标准
- •unable or unwilling to provide informed consent
- •patients who are pregnant or lactating
- •patients with known claustrophobia
- •age < 18 years
结局指标
主要结局
Clinically Silent and Control Patients
时间窗: 9 years
Cardiac death or cardiac transplantation or sustained VT/VF or sustained second or third degree AV block or development of clinical congestive heart failure (with documented LVEF \< 50%).
Clinically Manifest Patients
时间窗: On active therapy for 6 months
"Clinically improved" if they are alive and not had a heart transplant and have not had heart failure hospitalization and no sustained VT/VF and meet one or more of following (i) No sustained VT (if presented with sustained VT) (ii) Improvement in LV function (defined as 10% decrease in LV end systolic volume or 5% absolute increase in LVEF) (iii) Resolution of conduction system disease (if presented with sustained heart block) failure hospitalization and have not had sustained VT and one or both of: a. LV function improvement (defined as 10% decrease in LV end systolic volume) b. Resolution of conduction system disease.
次要结局
- cardiovascular mortality(6 months and 60 months)
- total mortality(6 months and 60 months)
- change in disease activity as assessed by PET imaging(6 months and 60 months)
- change in LVEF from baseline(6 months and 60 months)
- heart failure hospitalization(6 months and 60 months)
- Atrial Fibrillation burden(6 months and 60 months)
- Ventricular arrhythmia burden(6 months and 60 months)
- % of ventricular pacing(6 months and 60 months)