Table 3.

Literature Review

Date	First Author	# pts	Ages (yrs)	GSD Type	Type report	Cardiac Clinical Sx	Echocardiogram Findings
2007	Cochrane10	1	39	IIIa	Case report	Yes (CHF; transplant)	LV ejection fraction 20%
							Pulm HTN
2007	Ogimoto25	1 (mentions 6 others)	42	IIIa	Case report	Yes (exertional chest pain; dyspnea)	“Marked hypertrophy”
							Abnormal diastolic function
1997	Akazawa26	1	38	IIIa	Case report	Yes (dyspnea/fatigue w/ exertion; sudden death)	Akinetic & thin portions of LV wall
							Abnormal systolic function (SF24%)
							LVH present (LVmass 225 gm/m2 and septum 18 mm)
1997	Cuspidi13	1	30	IIIa	Case report	Yes (tachypnea, dyspnea)	Severe asymmetric septal hypertrophy, systolic anterior motion of the mitral valve, LV outflow tract obstruction
1997	Shen27	1	4	III	Case report	Yes (sudden death)	LVH present
1997	Lee9	32	0.6 to 31.6 (12 >18 yr)	III	Cross sectional	No symptoms in 5 patients	12/32 w/ LVH (37.5%) (by LV posterior free wall thickness)
1995	Lee28	32	0.6 to 31.6 (12>18 yr)	III	Cross sectional		LV posterior free wall thickness
							SF
							6/18 children w/ LVH (33%)
							6/12 post pubertal (50%)
							12/32 overall w/ LVH (37.5%)
1995	Tada8	1	23	III	Case report	Palpitations	LV dilation and LV concentric hypertrophy; LV wall thinning
						Ventricular tachycardia	Abnormal systolic function (SF9%)
1995	Kobayashi29	1	23	III	Case report		“Marked thickening of interventricular septum”
1994	Talente30	9	18-57	III	Case series		4/6 abnormal echocardiograms (67%)
							LVH in 3, BVH in 1
1993	Carvalho17	23	9 mos to 28	III	Case series	Symptoms uncommon	11/23 (~50%) w/ increased
						2 w/ dyspnea w/ exertion; 3 w/ fatigue; 1 w/ chest pain	LV septal and posterior wall thickness; LV dimensions
							SF all normal
1992	Coleman20	13	4 to 57	IIIa, b, d	Case series	None mentioned	IIIa: 3/7 abnormal echocardiograms (43%) including LVH in one
1991	Labrune18	18	8 mos to 18	III	Case series	None symptomatic	5/16 (30%) w/ increased LV septal and posterior wall thickness
1990	Smit31	50	>10	III	Cross sectional	Not reported	“Cardiomyopathy” in 22/44; no echo details reported
1989	Moses21	20	3-30	III	Cross sectional	19/20 asymptomatic	LVH 13/16
						1/20: Dyspnea, weakness	Mild LV dilation 2/20
							LA dilation 7/20
1984	Olson7	1	25	III	Case report	Yes; orthopnea due to pulmonary edema during pregnancy	“Marked thickening of ventricular myocardium”
							Normal systolic function
1972	Miller6	1	3 mos	III	Case report	Yes (sudden death at 3 mos) Marked LVH at autopsy	No echocardiogram
						“Gross myocardial thickening”

CHF – Congestive Heart Failure; LV – Left ventricle; HTN – Hypertension: LVH – Left Ventricular Hypertrophy; LA – Left atrium