Table 1. Diagnostic Criteria 2020 for triglyceride deposit cardiomyovasculopathy.

Items	Clinical findings
1. Essential items	Impaired LCFA metabolism or TG deposition in myocardium
	1) Decreased washout rate (<10%) in myocardial 123I-BMIPP SPECT
	2) Myocardial TG deposition by biopsy specimens (a)
	3) Myocardial TG deposition by CT or MR spectroscopy
2. Major items	1) Decreased left ventricular ejection fraction (<40%)
	2) Diffuse narrowing of coronary arteries documented by CAG and/or coronary CT angiography (b)
	3) Typical Jordans' anomaly (apparent vacuoles >1 μm in size) of polymorphonuclear leucocytes in peripheral blood smear (c)

Diagnosis

 Definite TGCV: One or more essential items and one or more major items are met.

 Probable TGCV: At least one essential item is met.

Supportive items (d)

1. Diabetes mellitus (e)

2. Hemodialysis

(a) For tissue TG contents examination, frozen sections with osmium fixation, but not paraffin sections, should be used for prevention of lipid elution

(b) The presence or absence of a significant stenosis is not considered

(c) For difficult cases, May-Giemsa staining slides of peripheral blood smear will be evaluated by the Japan TGCV Study Group

(d) These items are commonly present according to autopsy heart analysis and small-scale cohort analysis performed by the Japan TGCV Study Group but not proven to have diagnostic accuracy or causal relationship.

(e) According to the diagnostic criteria of diabetes mellitus by the Japan Diabetes Society.

If imaging protocols for ¹²³I-BMIPP myocardial scintigraphy, cardiac CT and cardiac MR spectroscopy are needed, contact Japan TGCV Study Group (E-mail: inffo@tgcv.org)

The classification algorithms for primary TGCV and idiopathic TGCV are described in Figure 1.

Abbreviations: TG: triglyceride, LCFA: long chain fatty acid, ¹²³I-BMIPP: iodine-123-β-methyl iodophenyl-pentadecanoic acid, SPECT: single-photon emission computed tomography, CT: computed tomography, MR: magnetic resonance, CAG: coronary angiography, TGCV: triglyceride deposit cardiomyovasculopathy