Table 1 Patients’ characteristics of Primary and Idiopathic TGCV
From: Triglyceride deposit cardiomyovasculopathy: a rare cardiovascular disorder
| Â | 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) |