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Triglyceride deposit cardiomyovasculopathy (TGCV) is a phenotype primarily reported in patients carrying genetic mutations in PNPLA2 encoding adipose triglyceride lipase (ATGL) which releases long chain fatty acid (LCFA) as a major energy source by the intracellular TG hydrolysis. These patients suffered from intractable heart failure requiring cardiac transplantation. Moreover, we identified TGCV patients without PNPLA2 mutations based on pathological and clinical studies . We provided the diagnostic criteria, in which TGCV with and without PNPLA2 mutations were designated as primary TGCV (P-TGCV) and idiopathic TGCV (I-TGCV), respectively. We hereby report clinical profiles of TGCV patients. Between 2014 and 2018, 7 P-TGCV and 18 I-TGCV Japanese patients have been registered in the International Registry. Patients with I-TGCV, of which etiologies and causes are not known yet, suffered from adult-onset severe heart disease, including heart failure and coronary artery disease, associated with a marked reduction in ATGL activity and myocardial washout rate of LCFA tracer, as similar to those with P-TGCV. The present first registry-based study showed that TGCV is an intractable, at least at the moment, and heterogeneous cardiovascular disorder.

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quickly and easily and is less invasive than a VAD, which requires a surgical operation. 4,5 VA-ECMO is considered a short-term but effective and useful circulatory support technique in urgent clinical situations. 6 Previous reports have shown that approximately 50-70% of FM patients experience spontaneous recovery during VA-ECMO support. 4,7-11However, VA-ECMO cannot reduce the left ventricular afterload and may cause pulmonary congestion, which is a disadvantage for cardiac recovery. 5 Additionally, it has insufficient power for circulatory support compared with F ulminant myocarditis (FM) is characterized by rapidly progressive myocardial inflammation leading to circulatory collapse. 1,2 A certain number of patients cannot be adequately supported by conventional inotropic therapy, 3 and despite recent progress in both medical knowledge and technology, treatment of these patients depends on mechanical circulatory support (MCS), including venoarterial extracorporeal membrane oxygenation (VA-ECMO) and ventricular assist devices (VADs). Background: Cardiac recovery and prevention of end-organ damage are the cornerstones of establishing successful bridge to recovery (BTR) in patients with fulminant myocarditis (FM) supported with percutaneous venoarterial extracorporeal membrane oxygenation (VA-ECMO). However, the timing and method of successful BTR prediction still remain unclear. We aimed to develop a prediction model for successful BTR in patients with FM supported with percutaneous VA-ECMO.

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