PMC1190177_01
Anatomy of right ventricle and atrium. A. Dilated coronary sinus. B. Dysplastic tricuspid valve. C. Short thickened chordae tendinae almost implanted into papillary muscle. D. Right ventricular hypertrophy with ventricular wall thickness of 8 mm. E. Pacer wire.
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F1
The heart weighed twice the normal expected weight and there was biventricular hypertrophy, with the right ventricular wall thickness being about four times the normal thickness and the left ventricular wall thickness about twice normal (Figure 1).
Anatomy of left ventricle and atrium. A. Left atrium with endocardial fibroelastosis. B. Short thickened chordae tendinae with direct insertion of posterior mitral valve leaflet into papillary muscle. C. Thickened dysplastic mitral valve with stenosis.
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F2
The right atrium and the coronary sinus were dilated and the left atrium was small with endocardial fibroelastosis (Figure 2).
Histology of Conduction system. VVG stain.
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F3
The membranous portion of the interventricular septum was about three times the normal length and mapping of the junctional tissue revealed that the atrioventricular node was displaced caudally and the bifurcation of the Bundle of His was likewise displaced caudally (Figure 3).
Pulmonary arteriograms. A. Age-matched normal child. B. Kabuki syndrome patient.
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F4
Postmortem pulmonary angiogram revealed severe pruning of the pulmonary vascular tree (Figure 4) and absence of the background 'blush' produced by filling of intracinar arteries.
Histology of the lung. Movat pentachrome stain.
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F5
Microscopic examination revealed marked luminal narrowing or fibrous occlusion of intra-acinar arteries (Figure 5).