PMC3821707_01
Newborn echocardiogram. (A) Parasternal long axis view showing marked assymetric septal hypertrophy (1.4cm, Z-score 9), a large VSD and aortic malposition. (B) M-mode from parasternal short axis view demonstating marked septal hypertrophy compared to the borderline mild hypertrophy of the free walls. (C) Parasternal short axis view demonstrating the ventricular septal defect (1cm) and anterior deviation of hypertrophied conal tissue and small outflow tract muscle bundles. AO - aorta, LA - left atrium, LV - left ventricle, IVS - intraventricular septum, MV - mitral valve, PV - pulmonary valve, RV - right ventricle, RVOT - right ventricular outflow tract, TCV - tricuspid valve, VSD - ventricualr septal defect.
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An echocardiogram revealed severe asymmetric septal hypertrophy (ASH, 1.4cm, Z-score 9) with borderline left posterior wall hypertrophy (0.5-0.6 cm, Z-score 2-3) and mild right ventricular hypertrophy (Figure 1A, B). There was a large inlet ventricular septal defect (VSD, 1.0 cm) with perimembranous extension (Figure 1A, C), a d-malposed and mildly dilated aortic root, mild dynamic right ventricular outflow obstruction (22 mmHg), a moderate secundum atrial septal defect (ASD, 0.6 cm), and a large patent ductus arteriosus.
Histology staining of surgically excised cardaic tissue. (A) H &E stain: Microscopic examination suggested normal sarcomeric fiber arrangent and myocyte enlargement. The darker pink areas were suspected to represent areas of subendocardial fibrosis (black arrow). (B) Trichrome stain: The trichrome stain identified fibrotic tissue within the subendocardial area (black arow) but no other areas of fibrosis were seen.
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Trichrome (Figure 2B) were applied.