Disproportionate Ventricular Size Anomalies

what is Ebstein's anomaly?

-rare CHD

-tricuspid valve is displaced inferiorly in the right ventricle

what causes Ebstein's anomaly?

tricuspid valve leaflets fail to separate from IVS

what is the prognosis of Ebstein's anomaly?

poor (80-90% mortality)

treatment for ebstein's

-evaluate for cardiomegaly and hydrops

-chart to grade severity

-surgery required dependent on severity

differentials for ebstein's

-tricuspid dysplasia

-regurgitation

associations with ebstein's

-mild form may be difficult to diagnose, pericardial effusion may be seen if cardiac function is severely compromised

-maternal lithium use

-pulmonary atresia/stenosis

-arrhythmias

sonographic appearance of ebstein's anomaly

-enlarged right atrium

-inferior displacement of the tricuspid valve

-tricuspid regurgitation

-small right ventricle

-overall enlarged heart

-probable ASD

-bast visualized in apical four chamber view

what is coarctation of the aorta?

-narrowing of the aortic lumen in the region of the isthmus just proximal to the ductus arteriosus

-hemodynamically significant stenosis reduces the volume of blood flow into the aortic arch resulting in arch hypoplasia

what is the prognosis of coarctation of the aorta?

-dependent on severity

treatment for coarctation of the aorta

-foll0w up with fetal echocardiograms to monitor severity of narrowing

-delivered with pediatric cardiology services readily available

-check BP often after birth (may lead to chronic hypertension)

-surgical reconstruction of arch may be required

differentials for coarctation of the aorta

HLHS

associations of coarctation of the aorta

-turner's syndrome

-bicuspid aortic valve

-usually seen in conjunction with other CHDs

-trisomy 18

-DiGeorge syndrome

sonographic appearance of coarctation of the aorta

-narrowed aortic arch

-ventricular disproportion

-ascending aorta normal in diameter until the isthmus

-CD will show narrowing but not aliasing due to prenatal flow may be reversed

-sagittal AA view visualized area of narrowing

-3VV will show narrowed aorta compared to pulmonary artery

-apical four chamber may show ventricular disproportion