. Author manuscript; available in PMC: 2021 Jul 28.

Published in final edited form as: J Am Coll Cardiol. 2020 Jul 28;76(4):419–431. doi: 10.1016/j.jacc.2020.05.059

TABLE 1.

Anatomic Diagnosis in Fontan Patients

Diagnosis	n (%)	Ventricle Morphology
Diagnosis	n (%)	RV	LV
HLHS	484 (42)	484	0
TA	181 (16)	0	181
DILV	159 (14)	0	159
DORV/MA	94 (8)	94	0
Heterotaxy	84 (7)	66	18
PAIVS	78 (7)	0	78
uAVSD	41 (4)	26	15
Others	41 (4)	8	33
Total	1,162	678	484

The most common diagnosis was HLHS (n = 484, 42%). Fifty-five of 84 patients with heterotaxy had common atrioventricular valve morphology. A total of 96 patients with common atrioventricular valve morphology were detected, with 71 having a right dominant valve and 25 having a left dominant valve. Other diagnoses included variant HLHS, d-transposition of great arteries, truncus arteriosus, and Ebstein anomaly.

DILV = double inlet left ventricle; DORV = double outlet right ventricle; HLHS = hypoplastic left heart syndrome; MA = mitral atresia; PAIVS = pulmonary atresia with intact ventricular septum; TA = tricuspid atresia; uAVSD = unbalanced atrioventricular septal defect.