Table 1.

Patients reported in the literature diagnosed with MPS VI who had undergone valvuloplasty

Characteristics			Described cases
		Wilson et al., 1980 [16]	Tan et al., 1992 [17]				Marwick et al., 1992 [18]	Torre et al., 2016 [14]	Current case
		Male	Male	Female	Female	Female	Female	Female	Male
Age at diagnosis with MPS VI			30y	+/−31y	N/A	+/−20y	Childhood	34y	7 m
Age of onset of cardiologic symptoms		43y	28y	+/−28y	25y	+/−20y	+/− 20y	+/−37y	+/−7 m
Age at surgery		N/A	30y	34y	25y	21y	25y	40y	11 m
Pre-operative Echocardiography findings	Aortic Valve	Severe stenosis	Stenosis, with mild regurgitation	Stenosis	–	–	Mildly echo dense, with normal leaflet excursion. Minimal regurgitation	Severe stenosis, calcified cusps, moderate regurgitation	Thick valve, discrete regurgitation
	Mitral Valve	N/A	Thick and stenotic leaflets, with mild regurgitation	Stenosis	–	Severe regurgitation	Stenotic and rigid valve, commissural fusion, resembling a rheumatic valve; mild regurgitation	Thickened, with severe stenosis	Thick, redundant, prolapsed posterior cusp, central valve coaptation failure. Chordae tendon mildly thickened, a broken chordae tendon was not excluded
	Tricuspid Valve	N/A	Thick leaflets, no stenosis	–	–	Moderate regurgitation	–	–	Discrete-moderate regurgitation
	Other findings	N/A	–	–	–	Small left ventricular cavity	–	Mild left ventricular hypertrophy	Patent foramen ovale, large left auricle, Major dilatation of left ventricle
Cardiac Surgery		AVR	AVR SJA 19 mm, MVR SJA 21 mm, aortic root enlargement	AVR SJA 19 mm, MVR SJA 21 mm, aortic root enlargement	None	AVR SJA 19 mm, MVR SJA 21 mm, aortic root enlargement	MVR size 2 M Starr-Edwards 6120 prosthesis.	AVR 19 mm mechanical prosthesis.	Mitral valvuloplasty, annuloplasty with bovine pericardium patch

N/A Not Available, AVR Aortic Valve Replacement, MVR Mitral Valve Replacement, AVR SJA 19 mm = Aortic Valve Replacement with St. Jude aortic prosthesis, size 19 mm; MVR SJA 21 mm = Mitral Valve Replacement with inverted St. Jude aortic prosthesis, size 21 mm