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Table 1 Patients’ characteristics of Primary and Idiopathic TGCV

From: Triglyceride deposit cardiomyovasculopathy: a rare cardiovascular disorder

  Primary Idiopathic
n = 7 n = 18
General Status
   Age (years) 55.7 ± 12.7 64.6 ± 14.7
   Sex (female, male) (n) (2, 5) (9, 9)
   BMI (kg/m2) 19.4 ± 3.4 25.4 ± 5.0
   Family history for CVD 7 13
ATGL expression
   PNPLA2 mutation Yes No**
   ATGL activities in leukocytes (nmol/h/mg)* 5.3 ± 8.3 12 ± 9
   (reference value 52 ± 13 nmol/h/mg)   
Vacuole formation in polymorphonuclear leukocytes (%) ~ 100% < 10%
Heart disease
   Mean age of symptom onset (years) 37.7 ± 9.2 55.9 ± 12.5
    Angina at rest (n) 3 10
    Dyspnea or palpitation (n) 4 8
   Clinical diagnosis at registration   
    Angina pectoris 1 13
    (rest, effort) (n) (1,0) (11, 2)
    Heart failure (n) 5 8
    Critical arrhythmia (n) 4 1
    History of myocardial infarction (n) 0 4
   NYHA classification (I, II, III, IV) (n) (1, 1, 2, 3) (2, 5, 11, 0)
Coronary angiography or CT angiogram
   Affected branch (single vessel, multivessels) (n) (0, 5) (4,14)
   Diffuse narrowing (n) 5 18
Washout rate in BMIPP scintigram (%) 一3.2 ± 4.8 1.4 ± 8
   (reference value 19.4 ± 3.2%)   
Treatment history
   Percutaneous coronary intervention (n) 0 7
   Coronary artery bypass grafting (n) 0 5
   Cardiac transplantation (n) 2 0
Comorbidity
   Skin lesions (n) 0 0
   Skeletal myopathy (n) 7 0
   Diabetes mellitus (n) 2 15
Outcome
   Death (n) 5 3
   (before, after registration) (3, 2) (2, 1)
  1. *Three patients with P-TGCV and fourteen with I-TGCV were enrolled
  2. We did not have opportunity for the measurement in the remaing four patients with P-TGCV and four with I-TGCV
  3. **Two patients were dismissed before the genetic analysis
  4. The Japan TGCV study group certified I-TGCV according to the diagnostic guideline
  5. Abbreviations: CT Computed tomography, CVD Cardiovascular disease, TGCV Triglyceride deposit cardiomyovasculopathy