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496 D. Maulik<br />

Fig. 33.14 A,B. Fetal echocardiographic assessment of Ebstein's<br />

anomaly. RA right atrium, RV right ventricle, TV tricuspid<br />

valve. A Two-dimensional image shows four-chamber<br />

view of the fetal heart. Note the apical displacement<br />

of the septal leaflet of the tricuspid valve with an enlarged<br />

right atrium and a diminutive right ventricle. B Color flow<br />

mapping of Epstein's anomaly. Note the impressive regurgitant<br />

flow projecting retrograde into the right atrium across<br />

the thickened tricuspid valve<br />

raphy. They include ASD, pulmonary stenosis, absence<br />

of semilunar valves resulting in regurgitant<br />

flow, AVSD, and other malformations. The prognosis<br />

depends on the severity of the malformations. Severe<br />

dysplasia and abnormal distal tethering directly contribute<br />

to the functional and hemodynamic abnormalities.<br />

In general, neonatal survival has been poor.<br />

Advances in pediatric cardiac surgery have contributed<br />

to the relatively improved outcome.<br />

Hypoplastic Left Heart Syndrome<br />

Hypoplastic left heart syndrome is characterized by a<br />

hypoplastic or completely obliterated left ventricular<br />

chamber, mitral atresia, and aortic valvular atresia or<br />

Fig. 33.15A±C. Fetal echocardiogram shows the sonographic<br />

characteristics of the hypoplastic left heart syndrome.<br />

LA left atrium, RA right atrium, LV left ventricle, RV<br />

right ventricle. A Lateral four-chamber view of the fetal<br />

heart shows virtual absence of the left ventricular chamber<br />

and a small left atrial chamber. Flow in the right heart extends<br />

from the atrium to the apex of the ventricle. B M-<br />

mode depiction of the hypoplastic left atrium, a relatively<br />

enlarged right atrium (left), and a grossly hypoplastic left<br />

ventricle. C Top left: Doppler sample volume in the ventricle<br />

just distal to the valve. C Bottom left: Mitral Doppler<br />

waveform. C Top right: Doppler interrogation of the tricuspid<br />

flow. C Bottom right: Tricuspid Doppler waveforms

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