Sarcopenia in Fontan patients: a sign of frailty-associated premature ageing?

Abstract

On November 10, 1942, after winning the battle of Alamein, Winston Churchill delivered these famous words: "Now this is not the end. It is not even the beginning of the end. But it is, perhaps, the end of the beginning."Adults living with CHD are a growing population, as they are now surviving into adulthood and even reaching geriatric ages thanks to the emergence of surgical and medical innovations. 1 In all adults with CHD, the fastest-growing population is the one with complex congenital lesions. 1 Fontan patients are among the most resource-intensive populations from a health service perspective. They experience multiple extracardiac complications that are mainly related to sustained decreased cardiac output and increased systemic venous pressure. 2 This means that Fontan patients are living with a lifespan state of compensated or decompensated heart failure. 2 The functional consequences of their singular cardiovascular physiology have been expressed as both a reduced life expectancy when compared to other CHD population 3 and a decreased exercise capacity. 4 Moreover, skeletal muscle strength has been shown to be similar to that of patients with advanced heart failure, 5 since they have increased adiposity and, as shown by Cordina et al., Fontan-associated myopenia. 6 The advent of the Fontan operation has certainly improved outcomes for patients with univentricular heart, but given the ensuing complications, treating physicians feel this is "the end of the beginning," to use Churchill's words.Psoas muscle area, an indicator of sarcopenia, has proven to be an important marker of worse cardiovascular outcomes and of increased risk of mortality in patients with long-standing heart failure and increased cardiovascular burden. [7][8][9][10][11] It has also been shown to be correlated with indicators of physical frailty such as low handgrip strength and short physical performance battery. 12 Frailty is a broad multidimensional syndrome that reflects a patient's vulnerability. It encompasses multiple clinical elements such as muscle mass, strength, malnutrition, and functional performance. 13 Sarcopenia is therefore a crucial element in the assessment of frailty. We hypothesised that Fontan patients, despite their young age, exhibit sarcopenia resembling that more often seen in elderly patients with cardiovascular comorbidities rather than in healthy agematched controls.At the McGill Adult Unit for Congenital Heart Disease (MAUDE clinic, Montreal, Canada), a total of 67 patients with Fontan are actively followed. Multiple imaging modalities are used as part of the routine screening for Fontan-associated liver disease, including abdominal ultrasound, CT, and magnetic resonance. In this setting, 17 out of 67 Fontan patients had a CT of the abdomen performed and thus were included in the study. The psoas muscle areas were measured on the CT scan using the validated web-based software Coreslicer.com. 14 Measurements were done in the axial plane, at the highest level of the L4 vertebrae. There is no gold standard numb...

Born to Age: When Adult Congenital Heart Disease Converges With Geroscience

“… 91 , 92 In a single-center study of sarcopenia, 67 Fontan patients, of whom 17 had abdominal CT scans, were compared to 125 age-matched healthy controls and 425 elderly patients undergoing a transaortic valve replacement (TAVR). 95 The male subgroup with a mean age of 24 years with Fontan has a psoas muscle area that was not significantly different from that of the TAVR group with a mean age of 85 years but was significantly lower than that of the healthy controls with a mean age of 30 years. 95 …”

“… 95 The male subgroup with a mean age of 24 years with Fontan has a psoas muscle area that was not significantly different from that of the TAVR group with a mean age of 85 years but was significantly lower than that of the healthy controls with a mean age of 30 years. 95 …”

Nutritional considerations for people living with a Fontan circulation: a narrative review

“…Although their diagnostic criteria are similar, sarcopenia is characterised by a progressive muscle loss associated with ageing, in contrast to myopenia that may, at least in part, be developmental or "intrinsic." 8,9,23,37,50,51,[54][55][56][57] Fontan-associated myopenia is likely due to a myriad of factors, which are poorly characterised. These factors include, but are not limited to, physical inactivity, increased protein turnover in the myocardium and skeletal muscle, increased energy requirements of the peripheral pump with exercise, metabolic alteration of substrates (i.e., glucose, lipids, amino acids) secondary to body composition changes, inadequate dietary intake compared to nutritional requirements, malabsorption, nutrition-impact symptoms, feeding difficulties during childhood, and reduced appetite secondary to low levels of the appetite hormone ghrelin.…”

Medical Therapy for Heart Failure in Adult Congenital Heart Disease Patients

“… 72 Medications thought to improve exercise capacity and quality of life may be confounded by both increased frailty within ACHD patients and discordant self-estimation of FC. 73 , 74 , 75 …”