Williams Syndrome and Right Ventricular Noncompaction: A Rare Association

Figures

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  Figure 1. Echocardiogram showing severe calcification of mitral valve, aortic valve, basal ventricular septum and ascending aorta in a patient with Williams syndrome. Continues – wave Doppler examination demonstrating severe valvular aortic stenosis with mean gradient of 58 mmHg and severe valvular mitral stenosis with mean gradient of 20 mmHg. Continues – wave Doppler examination of tricuspid regurgitation jet illustrating moderate pulmonary hypertension. LA = Left atrium, AO = Aorta

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  Figure 2. Echocardiogram showing RV noncompaction in a patient with Williams syndrome. The apical 4-chamber view echocardiogram (a) and (b) showing prominent trabeculations and intertrabecular recesses in the RV free wall, apex and RV aspect of interventricular septum (arrow heads). Color Doppler displayed flow within the intertrabecular recesses (arrow heads) (c). The short-axis view of left ventricle (d) showing trabeculations of RV side of mid-septum with intertrabecular recesses (arrow heads). LA = Left atrium, RA = Right atrium, RV = Right ventricle, LV = Left ventricle

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  Figure 3. Computed tomography angiogram of aorta in a patient with Williams syndrome showing diffuse hypoplasia of aorta (left arrowheads) with extensive calcifications involving thoracic aorta (right arrowheads) AO = Aorta