Connective Tissue Diseases, Right Ventricular Hypertrophy
Conditions
Keywords
Connective Tissue Diseases, Extracellular Volume, Myocardial Deformation, Cardiovascular Magnetic Resonance
Brief summary
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
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.
Interventions
DIAGNOSTIC_TESTCMR examination
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.
Sponsors
RenJi Hospital
Study design
Observational model
COHORT
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
18 Years to 80 Years
Healthy volunteers
Yes
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.
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Composite endpoint of cardiac condition | within 2 days of CMR scan | Compose of ventricular mass (g), volume (mL), ejection fraction (%) and strain (%) of both left and right ventricles. |
| Composite endpoint of quantitative fibrosis assessment | within 2 days of CMR scan | Compose of percentage of extracellular volume (%) and positive rate of late gadolinium enhancement (%). |
Countries
China
Outcome results
None listed