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THE HEART

Fig. 5.25 Hypoplastic left heart syndrome,

15 weeks, four-chamber view (apical), transvaginal

sonography. The right ventricle (rV) appears large in

comparison with the left ventricle (lV), which is

barelyvisible.

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seen for the first time as late as in the third trimester.

Clinical management: Further sonographic

screening. Karyotyping, consultation with pediatric

cardiologist. Vaginal delivery is possible.

Procedure after birth: As this anomaly is associated

with and dependent on the Botallo’s

duct, oxygen administration should not exceed

40–60%. Therapy with prostaglandin should

start immediately after birth.

Prognoses: This is a fatal condition without surgical

intervention. Cardiac surgical procedures

such as the Norwood operation (at least three

surgical corrections)are performed, with survival

rates of 20–70%. Heart transplantation has

also been tried in these children recently; the

success rate has been reported to be as high as

70%. In the long run, the quality of life after these

interventions remains limited. After the Norwood

operation, persistent neurological deficits

have been described in up to 70% of the infants.

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