Table 7:

Clinical characteristics of HLHS cohort by CMA class

Cardiac Parameters on Presentation
		Normal	cnVUS	Abnormal
		N=70	N=25	N=10
Parameter		Mean (SD)	Mean (SD)	Mean (SD)	P-value
MS/AS, MS/AA, MA/AA	MA/AA	32 (46.4)	17 (65.4)	3 (33.3)	0.140
	MS/AA	24 (34.8)	6 (23.1)	6 (66.7)	.
	MS/AS	13 (18.8)	3 (11.5)	0	.
	Missing	1 (1.4)	0	0	.
LSVC	No	65 (92.9)	26 (100)	9 (100)	0.436
	Yes	5 (7.1)	0	0	.
PAPVR/TAPVR	No	68 (97.2)	24 (92.3)	9 (100.0)	0.510
	Yes	2 (2.8)	2 (7.7)	0	.
Degree of TR	Trivial/None	40 (57.1)	11 (42.3)	4 (44.4)	0.503
	Mild	23 (32.9)	12 (46.2)	3 (33.3)	.
	Moderate/Severe	7 (10.0)	3 (11.5)	2 (22.2)	.
Mod/Severe TR	No	64 (91.4)	23 (88.5)	7 (77.8)	0.333
	Yes	6 (8.6)	3 (11.5)	2 (22.2)	.
RV function, n (%)	Normal	62 (89.9)	20 (80.0)	5 (55.6)	0.008
	Mildly/Moderately	7 (10.1)	5 (20.0)	4 (44.4)	.
	Depressed
RV moderate dysfunction, n (%)	No	69 (98.6)	24 (92.3)	7 (77.8)	0.016
	Yes	1 (1.4)	2 (7.7)	2 (22.2)	.
Extra cardiac abnormalities	No	66 (94.3)	24 (92.3)	4 (44.4)	<0.001
	Yes	4 (5.7)	2 (7.7)	5 (55.6)	.
Sano, BTS, or Hybrid	BTS	45 (64.3)	17 (65.4)	3 (33.3)	0.010
	Sano	16 (22.9)	9 (34.6)	1 (11.1)	.
	Hybrid	3 (4.3)	0	1 (11.1)	.
	None	6 (8.6)	0	4 (44.4)
Restrictive ASD, n (%)	No	54 (77.1)	22 (84.6)	7 (77.8)	0.743
	Yes	16 (22.9)	4 (15.4)	2 (22.2)	.
Ascending aorta diameter (cm)		0.34 ± 0.28	0.27 ± 0.15	0.24 ± 0.07	0.204

AA, aortic atresia; AS, aortic stenosis; ASD, atrial septal defect; BTS, Blalock-Taussig shunt; LSVC, left superior vena cava; MA, mitral atresia; MS, mitral stenosis; RV, right ventricle; TR, tricuspid regurgitation; PAPVR/TAPVR, partial anomalous pulmonary venous return/total anomalous pulmonary venous return.