Cardiac characteristics and natural progression in Taiwanese patients with mucopolysaccharidosis III

Table 2 Baseline clinical, echocardiographic and electrocardiographic features of the 26 patients with MPS III

No.	Gender	MPS type	Age (years)	MS	MR	AS	AR	MVP	Thick IVS	ASH	Left ventricular remodeling pattern	Electrocardiographic features
1	M	IIIB	1.8	0	0	0	0	–	+	+	Concentric remodeling	Normal
2	M	IIIB	2.2	0	0	0	0	–	–	–	Normal	Normal
3	F	IIIA	3.6	0	0	0	0	–	+	–	Concentric remodeling	NA
4	M	IIIB	4.2	0	0	0	0	–	–	–	Normal	Normal
5	M	IIIB	4.3	0	0	0	0	–	–	–	Normal	Sinus bradycardia, sinus arrhythmia
6	F	IIIB	4.9	0	1	1	0	–	–	+	Normal	Normal sinus rhythm, borderline QTc
7	M	IIIB	5.1	0	0	0	0	–	–	–	Normal	NA
8	M	IIIB	5.1	0	0	0	0	–	–	–	Normal	NA
9	F	IIIB	5.2	0	0	0	0	–	–	–	Normal	Sinus arrhythmia
10	F	IIIB	5.2	0	1	0	0	+	–	–	Normal	Sinus tachycardia
11	M	IIIB	5.4	0	0	0	0	–	–	–	Normal	NA
12	F	IIIB	6.0	0	0	0	0	+	–	–	Normal	NA
13	M	IIIB	7.2	1	0	0	0	–	+	+	Normal	NA
14	M	IIIB	7.5	0	0	0	0	+	–	–	Concentric remodeling	NA
15	M	IIIB	7.8	0	0	0	0	–	+	–	Normal	NA
16	M	IIIA	9.9	0	0	0	0	–	–	–	Normal	Sinus arrhythmia
17	F	IIIB	10.5	0	1	0	2	–	–	–	Normal	NA
18	F	IIIA	11.0	0	1	0	0	–	–	–	Normal	NA
19	F	IIIB	11.4	0	0	0	0	–	+	+	Concentric remodeling	NA
20	F	IIIB	11.5	0	0	0	1	–	–	–	Normal	NA
21	F	IIIB	12.3	0	0	0	0	–	–	+	Normal	Sinus bradycardia, short PR interval
22	F	IIIB	12.9	0	0.5	0	0.5	–	–	–	Normal	NA
23	M	IIIA	13.6	0	1	0	3	–	–	–	Normal	NA
24	M	IIIA	16.4	0	0.5	0	0	–	–	–	Normal	NA
25	F	IIIB	18.5	1	1	0	2	–	–	–	Normal	Normal
26	M	IIIC	26.5	0	0	1	0	–	–	–	Normal	Normal

MPS Mucopolysaccharidosis, MS Mitral stenosis, MR Mitral regurgitation, AS Aortic stenosis, AR Aortic regurgitation, MVP Mitral valve prolapse, IVS Interventricular septum, ASH Asymmetric septal hypertrophy, NA Not available. Severity of valvular stenosis and regurgitation (MS, MR, AS, AR) were estimated and graded on the following scores: 0 (none), 1 (mild), 2 (moderate), and 3 (severe)

ISSN: 1750-1172