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CHAPTER 37 The Fetal Heart 1273

TABLE 37.2 Recurrence Risks

in Siblings for Any Congenital

Heart Defect a

Defect

SUGGESTED RISK

(%)

If One

Sibling

If Two

Siblings

Fibroelastosis 4 12

Ventricular septal defect 3 10

Patent ductus arteriosus 3 10

Atrioventricular septal defect 3 10

Atrial septal defect 2.5 8

Tetralogy of Fallot 2.5 8

Pulmonary stenosis 2 6

Coarctation of aorta 2 6

Aortic stenosis 2 6

Hypoplastic left heart 2 6

Transposition 1.5 5

Tricuspid atresia 1 3

Ebstein anomaly 1 3

Truncus 1 3

Pulmonary atresia 1 3

a Combined data published during two decades from European and

North American populations.

With permission from Nora J. 252

TABLE 37.3 Suggested Offspring

Recurrence Risk (%) for Congenital Heart

Defects Given One Affected Parent

Defect

AFFECTED PARENT

Father

Mother

Aortic stenosis 3 13-18

Atrial septal defect 1.5 4-4.5

Atrioventricular septal defect 1 14

Coarctation of aorta 2 4

Pulmonary stenosis 2 4-6.5

Tetralogy of Fallot 1.5 2.5

Ventricular septal defect 2 6-10

With permission from Nora J. 252

with literature suggesting a ninefold increase in the incidence

of CHD in monochorionic-diamniotic twin gestations. 19 Lastly,

there appears to be a slightly increased risk of CHD with in vitro

fertilization (IVF), although at least some of this increased risk

appears to be related to the increased incidence of twin pregnancies

associated with IVF. 20 Most fetuses with CHD have no known

risk factors, which underscores the importance of a meticulous

evaluation of the four-chamber heart views and outlow tracts

on all routine obstetric ultrasound examinations. When severe

structural cardiac anomalies are identiied before viability, termination

may be ofered. Certainly, one of the most important

aspects of fetal echocardiography is the psychological relief it

afords parents whenever normal cardiac anatomy and function

are documented in a fetus at risk.

NORMAL FETAL CARDIAC ANATOMY

AND SCANNING TECHNIQUES

he fetal heart is similar to that of the adult, with several anatomic

and physiologic diferences. he long axis of the fetal heart is

perpendicular to the long axis of the body, such that a transverse

section through the fetal thorax demonstrates the four cardiac

chambers in a single view. he adult heart, in contrast, is obliquely

oriented with its long axis along a line between the let hip and

the right shoulder. he four-chamber view is important because

10% to 96% of structural anomalies are detectable on this

view. 21-26

Common Indications for Fetal

Echocardiography

Abnormal heart on routine ultrasound

Hydrops

Polyhydramnios

Fetal arrhythmia

Fetal chromosomal anomalies

Fetal extracardiac anomalies

Family history (congenital heart disease [CHD], syndromes

associated with CHD)

Maternal disease (diabetes, collagen vascular,

phenylketonuria)

Maternal infection (rubella)

Teratogen exposure

Increased nuchal translucency on irst-trimester screening

In vitro fertilization

Monochorionic twins

Monitoring response to intrauterine therapy

Monitoring fetus at risk for decompensation (persistent

tachyarrhythmia, hydrops)

Cardiac axis and position are normally such that the apex of

the heart points to the let and the bulk of the heart is in the let

side of the chest (Fig. 37.1A). his is levocardia. In mesocardia

the heart is central with the apex pointing anteriorly. In dextrocardia

the apex is directed rightward, and the heart is primarily

in the right side of the chest. his abnormality must be distinguished

from dextroposition (Fig. 37.1B), in which the heart

maintains a normal axis but is displaced to the right by an external

process, such as a let chest mass or pleural efusion. Abnormal

cardiac axis is associated with a 50% mortality and abnormal

cardiac position with an 81% mortality. 27

he fetal cardiovascular system contains several unique shunts:

the ductus venosus, foramen ovale, and ductus arteriosus (Fig.

37.2). Antenatally, the placenta rather than the lungs is the fetus’s

sole source of oxygen. Oxygenated blood leaves the placenta

through the umbilical vein and travels through the hepatic

vasculature and ductus venosus to the inferior vena cava (IVC)

and then into the fetal right atrium. As a result of increased

velocity, blood entering the IVC via the ductus venosus is shunted

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