Early Detection of Cardiac Impairment and Prediction of RV Hypertrophy in Patients With CTD
Condition: Connective Tissue Diseases; Right Ventricular Hypertrophy
MeSH terms: Hypertrophy, Right Ventricular; Connective Tissue Diseases; Hypertrophy
Study type: Observational

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.
    

Intervention name(s): CMR examination
Intervention type(s): Diagnostic Test
Intervention description(s): 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.
Observational model: Cohort
Time perspective: Prospective
Sampling method: Probability Sample
Study population: 
        Consecutive subjects were prospectively enrolled into 3 cohorts between July 2014 and Nov
        2016. The cohorts were divided as follows: the CTD patients with RVH, the CTD patients
        without RVH and the control group.
      
Keywords: Connective Tissue Diseases; Extracellular Volume; Myocardial Deformation; Cardiovascular Magnetic Resonance