Table 3.

Efficacy of enzyme replacement therapy.

	Control Baseline	Control 78 Weeks	Change	ERT Baseline	ERT 78 Weeks	Change	p-value
Echo Data
Ejection fraction	57 [56,61]	58 [56,60]	−1 [−2,3]	61 [58,63]	61 [58,64]	0 [−2,3]	0.80
Ejection fraction <55%	2 (11%)	3 (16%)		3 (6%)	4 (8%)		0.44
Elevated LVMI	1 (6%)	1 (6%)		2 (4%)	4 (9%)		0.71
ECG Data
PR Interval (ms)	160 [140,160]	150 [140,180]	0 [−10,20]	150 [140,160]	150 [140,163]	0 [−10,10]	0.71
Short PR	1 (5%)	3 (14%)		6 (11%)	4 (8%)		0.14
QRS duration (ms)	82 [70,90]	90 [80,100]	7 [0,17]	87 [80,90]	90 [87,100]	5 [0,10]	0.67
Prolonged QRS	1 (5%)	1 (5%)		0 (0)	1 (2%)		0.53
QTc Interval (ms)	400 [387,417]	406 [396,420]	20 [−5,28]	398 [386,413]	413 [404,428]	16 [−1,27]	0.99
Prolonged QTc	0 (0%)	3 (14%)		3 (6%)	7 (13%)		0.85
Left ventricular hypertrophy	3 (14%)	2 (9%)		6 (11%)	5 (9%)		0.72

Data presented are n (%) for categorical variables and median [interquartile range] for continuous variables.

ERT = enzyme replacement therapy, Elevated LVMI = Elevated left ventricular mass index by either 2-dimensional or M-mode measurements. 19 control patients and 49 ERT patients had baseline and 78 week ejection fraction data;16 control patients and 43 ERT patients had baseline and 78 week LMVI data;22 control patients and 53 ERT patients had baseline and 78 week ECG data.