Early Detection of Cardiac Impairment and Prediction of RV Hypertrophy in Patients With CTD

Study Overview
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Brief Summary
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There have been reports suggesting that progressive RV failure and death in connective tissue disease (CTD) are related to right ventricular hypertrophy (RVH) and dilation, irrespective of pulmonary arterial hypertension (PAH). The investigators aim to identify cardiac markers that occur before RVH and to investigate predictors of RVH.

Detailed Description
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Patients with connective tissue disease (CTD) frequently exhibit multi-organ pathophysiological and functional damage. The heart, one of the leading causes of CTD mortality, has attracted increasing attention. However, most patients with CTD present with nonspecific cardiac symptoms, normal ECG, and preserved left ventricular ejection fraction (LVEF) and therefore do not receive an early cardiac diagnosis. Pulmonary arterial hypertension (PAH), right ventricular (RV) dilatation and hypertrophy are the first and the most frequent cardiac findings. However, these are late-stage phenomena, which can eventually lead to death or right heart failure in CTD.Right ventricle abnormalities is associated with the risk of heart failure and cardiovascular death. RV dilation has long been considered a direct consequence of pulmonary arterial hypertension (PAH), but recently, physicians have observed RVH in CTD patients as well. RV dilation and RVH are not necessarily found in the same patient. The pathophysiology behind these issues is less well-understood. RVH progression continues even as CTD-associated PAH alleviates. This finding implies PAH might not be the sole index that leads to RVH. It would be interesting to explore factors that can predict the presence of RVH, which may reduce major adversecardiovascular events in patients with CTD. Cardiovascular magnetic resonance (CMR) is able to depict myocardial characteristics from structure to tissue properties using cine and late gadolinium enhancement (LGE) sequences. Newly developed imaging studies to date include T1 mapping and T1-derived Manuscript ECV estimation.All the previous studies in CTD have been restricted to patients with advanced cardiac involvement. Together with clinical assessment and multi-imaging tests, the aim of the present study was to find markers to detect cardiac involvement before RVH presented, which could be important for guiding treatment decisions such as the timing and choice of pharmaceutical treatment. The combination of myocardial functional and tissue changes may offer further insight into the pathophysiology of CTD.

Official Title
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Early Detection of Cardiac Impairment and Prediction of Right Ventricular Hypertrophy in Patients With Connective Tissue Disease

Conditions
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Connective Tissue Diseases, Right Ventricular Hypertrophy

Intervention / Treatment
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* Diagnostic Test: CMR examination


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria for CTD with RVH Age between 18-80 years old. Definite connective tissue diseases diagnosis. Echocardiography demonstration (later confirmed by CMR) of a hypertrophic RV when maximal end-diastole RV wall thickness >4 mm due to CTD Inclusion Criteria for CTD without RVH Age between 18-80 years old. Definite connective tissue diseases diagnosis. Echocardiography demonstration (later confirmed by CMR) that maximal end-diastole RV wall thickness ≤4 mm Inclusion Criteria for Control group: Absence of known systemic diseases Normal examinations Age between 18-80 years old. Providing written informed consent Exclusion Criteria: Age <18 years old or >80 years old Documented coronary artery disease or prior angiography for coronary artery disease (>50% stenosis). Patients with known congenital heart disease or other systemic diseases that might induce RVH. Patients with standard metallic contraindications to CMR or an estimated glomerular filtration rate < 30 ml/min/1.73 m2.

Ages Eligible for Study
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Minimum Age: 18 Years
Maximum Age: 80 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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Accepts Healthy Volunteers

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| CTD with RVH<br>The diagnosis of CTD was made based on the clinical classification criteria. The RVH patient was diagnosed by an echocardiography demonstration (later confirmed by CMR) of a hypertrophic RV (maximal end-diastole RV wall thickness >4 mm) due to CTD. | Diagnostic Test: CMR examination<br>* After recruiting participants and collecting the baseline information, a CMR scan and a post-processed imaging procedure will be carried on in order to detect the cardiac impairment.<br>|
| CTD without RVH<br>The diagnosis of CTD was made based on the clinical classification criteria.The subjects were enrolled as having non-RVH if their RV wall thickness was ≤ 4 mm (later confirmed by CMR). | Diagnostic Test: CMR examination<br>* After recruiting participants and collecting the baseline information, a CMR scan and a post-processed imaging procedure will be carried on in order to detect the cardiac impairment.<br>|
| Control group<br>The controls were healthy volunteers who have normal electrocardiographic and echocardiographic results and normal CMR findings | Diagnostic Test: CMR examination<br>* After recruiting participants and collecting the baseline information, a CMR scan and a post-processed imaging procedure will be carried on in order to detect the cardiac impairment.<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Composite endpoint of cardiac condition | Compose of ventricular mass (g), volume (mL), ejection fraction (%) and strain (%) of both left and right ventricles. | within 2 days of CMR scan | 
| Composite endpoint of quantitative fibrosis assessment | Compose of percentage of extracellular volume (%) and positive rate of late gadolinium enhancement (%). | within 2 days of CMR scan | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Connective Tissue Diseases, Extracellular Volume, Myocardial Deformation, Cardiovascular Magnetic Resonance