# Chronic kidney disease is a major risk factor for mortality in triglyceride deposit cardiomyovasculopathy patients

## TL;DR

Chronic kidney disease significantly increases the risk of death in patients with a rare heart condition called triglyceride deposit cardiomyovasculopathy.

## Contribution

This study identifies chronic kidney disease as a major mortality risk factor specific to TGCV patients.

## Key findings

- CKD patients with TGCV had significantly lower 3- and 5-year survival rates compared to non-CKD patients.
- CKD was independently associated with higher mortality risk in TGCV patients, even after adjusting for age.
- Common risk factors like diabetes and hypertension did not increase mortality in TGCV patients.

## Abstract

Triglyceride deposit cardiomyovasculopathy (TGCV) is a rare cardiovascular disorder caused by defective intracellular lipolysis of triglyceride, resulting in heart failure and diffuse narrowing atherosclerosis. Recently, the registry of TGCV patients in Japan revealed that the 3-year overall survival rate was 80.1% and the 5-year overall survival rate was 71.8%. In this study, the effect on mortality of chronic kidney disease (CKD), diabetes malleus (DM), hypertension (HT), and dyslipidemia (DL) was analyzed using this retrospective registry of TGCV patients. The 3-year survival rate was 71.3% in the CKD group and 91.7% in the non-CKD group, and the 5-year survival rate was 61.8% in CKD group and 84.4% in the non-CKD group. The Kaplan–Meier analysis revealed that CKD is a risk factor for mortality in TGCV patients (p = 0.006). Although TGCV patients with CKD were older than those without CKD, Cox proportional hazard model analyses including age indicated that CKD has a significant association of the prognosis of TGCV patients (hazard ratio 2.33 [1.12–4.86], p = 0.024). DM, HT, and DL did not increase mortality in TGCV patients, although these risk factors were established in the general population. TGCV might cause cardiac disorders and kidney disease at the same time, because podocyte foot process disorder in the glomeruli might be caused by TGCV itself, while CKD should be a risk factor for mortality in TGCV patients as is true in the general population. In conclusion, CKD is a major risk factor for mortality in TGCV patients and thus should be paid attention to in these patients.

The online version contains supplementary material available at 10.1007/s10157-024-02618-z.