Table 1.

Participant Characteristics

	All Fontan Participants (n=28)	Normal Skeletal Muscle Mass (n=9)	Skeletal Muscle Deficit (n=19)	P Valuea
Sex (female/male), n/n	15/13	7/2	8/11	0.11
Age, y	26±7	24±7	27±6	0.33
BMI, kg/m2	22.4 [20.4–27.3]	27.2±5.7	22.1±3.4	0.03
BMI Z score	0.38±1.32	1.32±1.28	−0.06 ±1.11	0.007
Height, m	1.67±0.12	1.65±0.09	1.68±0.12	0.55
Weight, kg	62.7 [54.5–72.3]	68.1 [60.4–91.9]	60.2 [51.2–71.6]	0.09
BSA, m2	1.7±0.2	1.8±0.2	1.7±0.2	0.21
Predominant ventricular morphology, n (%)				1.00b
Left	16 (57)	6 (67)	10 (53)
Right	8 (29)	2 (22)	6 (32)
Univentricle	2 (7)	1 (11)	1 (5)
Indeterminate	2 (7)	0 (0)	2 (11)
Type of Fontan, n (%)				0.23c
Extracardiac	14 (50)	4 (44)	10 (53)
Intracardiac	11 (39)	3 (33)	8 (42)
Atriopulmonary	3 (11)	2 (22)	1 (5)
Fenestration	9 (32)	3 (33)	6 (32)	1.00
Systolic function, n (%)				1.00d
Normal	22 (79)	9 (100)	13 (68)
Mildly impaired	5 (18)	0 (0)	5 (26)
Moderately impaired	1 (4)	0 (0)	1 (5)
Severely impaired	0 (0)	0 (0)	0 (0)
AV valve regurgitation, n (%)				0.14e
None	10 (36)	4 (44)	6 (32)
Mild	13 (46)	5 (56)	8 (42)
Moderate	4 (14)	0 (0)	4 (21)
Severe	1 (4)	0 (0)	1 (5)
Systemic valve regurgitation, n (%)				1.00f
None	23 (82)	9 (100)	14 (74)
Mild	4 (14)	0 (0)	4 (21)
Moderate	1 (4)	0 (0)	1 (5)
Severe	0 (0)	0 (0)	0 (0)
Medications, n (%)
Antiplatelets	16 (57)	6 (67)	10 (53)	0.7
Warfarin/DOACs	10 (36)	3 (33)	7 (37)	1.00
ACEI/ARB	6 (21)	2 (22)	4 (21)	1.00
β‐Blocker	2 (7)	1 (11)	1 (5)	1.00
Digoxin	1 (4)	0 (0)	1 (5)	1.00
Diuretics	5 (18)	3 (33)	2 (11)	0.29
Antiarrhythmic	4 (14)	2 (22)	2 (11)	0.57
NYHA class, n (%)				0.65g
Class I	21 (75)	6 (67)	15 (79)
Class II	7 (25)	3 (33)	4 (21)
Pacemaker	6 (21)	2 (22)	4 (21)	1.00

Data shown are mean±SD or median [interquartile range] except as noted. ARB indicates angiotensin II receptor blocker; ACEI, angiotensin‐converting enzyme inhibitor; AV, atrioventricular; BMI, body mass index; BSA, body surface area; DOAC, direct‐acting anticoagulant; and NYHA, New York Heart Association.

^a P value for normal skeletal muscle mass compared with skeletal muscle mass deficit groups.

^bRight ventricle morphology vs left ventricle, univentricular and indeterminate ventricle morphology.

^cExtra‐ and intracardiac vs atriopulmonary connection.

^dNormal or mildly impaired systolic function vs moderate or severe systolic function.

^eNo or mild AV valve regurgitation vs moderate or severe AV valve regurgitation.

^fNo or mild systolic valve regurgitation vs moderate and severe systolic valve regurgitation.

^gNYHA class I vs NYHA class II.