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 | |