PMC5875143_01
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
JCE-28-69-g001.jpg
PMC5875143_01_JCE-28-69-g001.jpg
F1
His echocardiogram showed hypoplastic ascending aorta and pulmonary arteries, severe calcification of mitral valve, aortic valve, aorta, basal ventricular and atrial septum, severe valvular mitral and aortic stenosis, moderate mitral regurgitation, mild aortic regurgitation, rupture of anterior tricuspid leaflet chordae with severe tricuspid regurgitation, with the right ventricular (RV) systolic pressure of 41 mmHg and good biventricular function [Figure 1].
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
JCE-28-69-g002.jpg
PMC5875143_01_JCE-28-69-g002.jpg
F2
In addition, there was severe noncompaction of the right ventricle with multiple trabeculae and deep intertrabecular spaces in the entire free wall, apex as well as RV side of septum [Figure 2].
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
JCE-28-69-g003.jpg
PMC5875143_01_JCE-28-69-g003.jpg
F3
Computed tomography scan showed diffuse hypoplasia of aorta with extensive calcifications involving thoracic aorta and its major branches [Figure 3].