Sarcopenia is a loss of muscle mass and function [1]. This phenomenon occurs not only in elderly people but also in patients with chronic illnesses such as, chronic heart failure (HF), liver dysfunction, and kidney dysfunction [1-5], which is called secondary sarcopenia. Possible explanations for sarcopenia include an abnormal energy metabolism coupled with mitochondrial dysfunction as well as a change in the structure of the myofibers, malnutrition, systemic inflammation, and oxidative stress [6][7][8][9].Recent studies have reported that secondary sarcopenia is not rare in adults with congenital heart disease (CHD) [10,11]. Sarcopenia is one of the important predictors of HF in non-CHD [12][13][14]; whereas, it remains unclear whether sarcopenia is also relevant to prognosis in adults with CHD. In particular, skeletal muscle function is important in Fontan circulation because a passive blood flow in a Fontan route can be maintained by both skeletal muscle pumping and the change of intrapleural pressure (ventilatory pumping) [15][16][17]. However, it is challenging to measure the entire skeletal muscle mass; until now, dual energy X-ray absorptiometry or bioelectrical impedance analysis (BIA) has been used to measure it [1,2]. The former method needs special equipment, and the latter is not available to patients after pacemaker implantation. The biggest issue in adults with Fontan circulation is that most patients have already undergone pacemaker implantation; therefore,