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ANSWERS
CASE 37
Placental Infarct
1. A, B, C, and D
2. C
3. B
4. B
References
Levine AB, Frieden FJ, Stein JL, et al: Prenatal sonographic diagnosis of
placental infarction in association with elevated maternal serum alpha-
fetoprotein. J Ultrasound Med 1993; 12(3):169-171.
http://www.ncbi.nlm.nih.gov/pubmed/7684089 (Accessed on June 6, 2012.)
Polat P, Suma S, Kantarcy M, et al: Color Doppler US in the evaluation of
uterine vascular abnormalities. Radiographics 2002; 22(1):47-53.
http://www.ncbi.nlm.nih.gov/pubmed/11796897 (Accessed on June 6,
2012.)
Sepulveda W, Alcalde JL, Schnapp C, et al: Perinatal outcome after prenatal
diagnosis of placental chorioangioma. Obstet Gynecol 2003; 102(5 Pt 1):
1028-1033.
http://www.ncbi.nlm.nih.gov/pubmed/14672481 (Accessed on June 6,
2012.)
Cross-Reference
Ultrasound: The REQUISITES, 2nd ed, pp 510-511.
Comment
Incidence of Placental Infarct
Small placental infarcts, as shown in this case (Figure A), are
common and occur in approximately 25% of pregnancies.
Larger infarcts are associated with complications, including
intrauterine growth restriction (IUGR) and increased perinatal mortality. These infarcts are more common in pregnancies
complicated by maternal preeclampsia or essential hypertension. Placental infarcts are associated with an elevated maternal
alpha-fetoprotein.
Ultrasound Appearance
The ultrasound appearance of a placental infarct has been
described in several studies, and the descriptions vary. Harris et al. reported that placental infarcts are not visible with
ultrasound unless they are complicated by hemorrhage. Other
series showed that infarcts can be seen, can be hyperechoic
acutely and become isoechoic with time, or can manifest as
hypoechoic lesions. Placental infarcts typically occur along the
maternal plate of the placenta (Figure A).
Differential Diagnosis of Large Placental Infarcts
The differential diagnosis of larger infarcts includes hemorrhage or hematoma, which decreases in size and changes in
appearance with time. Three additional focal placental abnormalities include brin deposition, chorioangioma (Figure B),
and intervillous thrombosis. Fibrin deposition is more common on the fetal side of the placenta. Chorioangiomas are
benign vascular masses with arterial and venous ow of mixed
echogenicity with numerous cystic spaces (Figure B) that usually manifest in the rst half of pregnancy. A chorioangioma
is the most common abnormality affecting the placenta. The
most common complications from this entity are polyhydramnios and preterm labor. Later in gestation, intervillous
thrombosis may be detected as a hypoechoic region with slow,
turbulent ow.
76
CASE 38
Used with permission from McGahan JP, et al: Fetal Heart. In McGahan JP,
Goldberg BB [eds]: Diagnostic Ultrasound, 2nd ed. New York: Informa
Healthcare USA, 2008; 1275.
History: A patient with a fetal cardiac abnormality identied on an ultrasound scan performed at an outside institution
is referred for a level II examination.
1. What should be included in the differential diagnosis?
(Choose all that apply.)
A. Tricuspid atresia
B. Coarctation of the aorta
C. Hypoplastic left heart syndrome (HLHS)
D. Interrupted aortic arch
E. Pulmonic atresia
2. Which of the following is not a feature of HLHS?
A. Ventricular septal defect
B. Hypoplasia of the ascending aorta
C. Small mitral valve with poor mitral valve motion
D. Small aortic valve and aortic valve motion
3. Which of the following is not an anomaly associated with
HLHS?
A. Endocardial broelastosis
B. High frequency of extracardiac malformation
C. Increased risk of chromosomal abnormalities
D. Ductus-dependent anomaly
4. Which of the following statements concerning HLHS is
false?
A. HLHS is associated with greater than 50% death rate in
utero.
B. HLHS is the most common cardiac cause of death
within the rst month of life.
C. If HLHS is left untreated, 95% of live-born infants die
in the rst month of life.
D. Heart transplant can be considered a denitive treat-
ment in the neonate.
77
ANSWERS
CASE 38
Hypoplastic Left Heart Syndrome
1. B, C, and D
2. A
a small left ventricle and ow into the descending aorta retrograde through the ductus from the pulmonary artery. HLHS,
severe coarctation of the aorta, and interrupted aortic arch all
could be considered in the differential diagnosis.
3. B
4. A
References
Brown DW, Connor JA, Pigula FA, et al: Variation in preoperative and intra-
operative care for rst-stage palliation of single-ventricle heart disease: a
report from the Joint Council on Congenital Heart Disease National Quality Improvement Collaborative. Congenit Heart Dis 2011; 6(2):108-115.
http://www.ncbi.nlm.nih.gov/pubmed/21426524 (Accessed on June 6,
2012.)
Galindo A, Nieto O, Villagrá S, et al: Hypoplastic left heart syndrome diag-
nosed in fetal life: associated ndings, pregnancy outcome and results of
palliative surgery. Ultrasound Obstet Gynecol 2009; 33(5):560-566.
http://www.ncbi.nlm.nih.gov/pubmed/19367583 (Accessed on June 6,
2012.)
Stumper O: Hypoplastic left heart syndrome. Postgrad Med J 2010;
86(1013):183-188.
http://www.ncbi.nlm.nih.gov/pubmed/20237013 (Accessed on June 6,
2012.)
Cross-Reference
Ultrasound: The REQUISITES, 2nd ed, pp 416-417.
Comment
Differential Diagnosis
HLHS accounts for less than 10% of major structural congenital heart defects. A spectrum of anomalies is associated with
HLHS, including small left ventricle, mitral and aortic atresia,
little or no communication between the left atrium and left
ventricle, and small ascending aorta. The ventricular septum is
usually intact. However, there may be different forms of hypoplasia of the left side of the heart, ranging from critical aortic
stenosis to severe coarctation and interrupted aortic arch.
Although these aortic arch anomalies are considered different
from HLHS structurally, they often have the same result with
Ultrasound Findings
The most common nding identied on a four-chamber view
of the heart is a very small left ventricle (see Figure). In some
cases, the left ventricle may be so small that it is almost nonexistent. In some cases, the left ventricle has increased echogenicity, suggesting that there are endocardial broelastosis.
In the classic form of HLHS, there are associated mitral and
aortic atresia and lack of motion in these valves. There is also
hypoplasia of the ascending aorta. This hypoplasia can be recognized on the outow tract views. A four-chamber view of
the heart shows discrepancies in size between the smaller left
ventricle and aorta and the larger right ventricle and pulmonary artery (see Figure). With severe obstruction of the left
ventricle, the aortic arch is supplied in a retrograde fashion
through the ductus arteriosus, from the pulmonary artery; this
can be identied by color Doppler.
Prognosis and Management
The prognosis of HLHS is very poor. Although only approximately 5% of all fetuses die in utero, 95% of live-born infants
die within the rst month of life. This high neonatal death
rate is due to the fact that this anomaly is ductus dependent,
and once the ductus arteriosus closes, neonatal death results.
If critical care is to be given to newborns with HLHS, delivery should occur within a tertiary care hospital. Various bypass
procedures have been helpful, including numerous staged palliative procedures, with more denitive surgeries performed
later in childhood. Heart transplant can be performed in some
cases. Donor availability for newborns with HLHS is often
problematic. Even with prenatal diagnosis and neonatal therapy, the mortality rate is still high.
78
CASE 39
A
Courtesy of Roy Filly, MD.
B
Courtesy of Roy Filly, MD.
C
Courtesy of Roy Filly, MD.
History: The patient has a history of prior pregnancy with
heart defect.
1. What should be included in the differential diagnosis in this
case of a large vessel overriding a ventricular septal defect
(VSD) as seen in Figure A? (Choose all that apply.)
A. Hypoplastic left heart syndrome
B. Endocardial cushion defect
C. Tetralogy of Fallot
D. Complete transposition of the great arteries
E. Truncus arteriosus
2. Which of the following is not one of the four components
of tetralogy of Fallot?
A. Infundibular pulmonic stenosis
B. Subaortic VSD
C. Overriding aorta
D. Atrial septal defect
3. In cases of tetralogy of Fallot, obstetric management and
outcome include all of the following except:
A. Fetal karyotype
B. Cesarean section
C. Hydrops and polyhydramnios
D. Low surgical mortality
4. Which of the following is not an anomaly associated with
tetralogy of Fallot?
A. Ebstein anomaly
B. Infundibular pulmonic stenosis
C. Atrial septal defect
D. Extracardiac defects
79
ANSWERS
CASE 39
Tetralogy of Fallot
1. C and E
2. D
3. B
4. A
References
Hirji A, Bernasconi A, McCrindle BW, et al: Outcomes of prenatally
diagnosed tetralogy of Fallot: implications for valve-sparing repair versus
transannular patch. Can J Cardiol 2010; 26(1):e1-e6.
http://www.ncbi.nlm.nih.gov/pubmed/20101358 (Accessed on June 6,
2012.)
Martínez JM, Gómez O, Bennasar M, et al: The ‘question mark’ sign as a
new ultrasound marker of tetralogy of Fallot in the fetus. Ultrasound Obstet
Gynecol 2010; 36(5):556-560.
http://www.ncbi.nlm.nih.gov/pubmed/20205151 (Accessed on June 6,
2012.)
Yagel S, Cohen SM, Achiron R: Examination of the fetal heart by ve
short-axis views: a proposed screening method for comprehensive cardiac
evaluation. Ultrasound Obstet Gynecol 2001; 17(5):367-369.
http://www.ncbi.nlm.nih.gov/pubmed/11380958 (Accessed on June 6,
2012.)
Cross-Reference
Ultrasound: The REQUISITES, 2nd ed, pp 416-420.
Comment
Differential Diagnosis
Differential diagnosis in this case would include any conotruncal abnormality in which there is hypoplasia of one of the
great arteries with an associated VSD. Tetralogy of Fallot
would be considered in this differential diagnosis. Truncus
arteriosus is another abnormality that could be considered in
the differential diagnosis. However, there would be a distinction between truncus arteriosus, in which usually a single arterial vessel gives rise to both the systemic and the pulmonary
circulation, and tetralogy of Fallot, in which the pulmonary
artery and the aorta are separated. In both of these abnormalities, usually the largest of the arteries overrides a VSD.
In tetralogy of Fallot there is pulmonary atresia, whereas in
truncus arteriosus the pulmonary arteries arise from a common large arterial trunk.
Ultrasound Findings
Typical ultrasound features of tetralogy of Fallot include a
VSD, an overriding aorta, infundibular pulmonic stenosis, and
hypertrophy of the right ventricle (Figures A and B). Hypertrophy of the right ventricle is often not evident in utero because
it usually develops after birth. However, there may be other
associated anomalies, including subaortic VSD, atrial septal
defect, or a complete defect of the atrioventricular septum.
There may be a right aortic arch in tetralogy of Fallot. VSD
associated with tetralogy of Fallot is often not visualized on the
four-chamber view but may be better identied on the longaxis view of the left ventricular outow tract. There may also
be increased rotation of the cardiac axis, usually greater than
60 degrees. An inverse relationship usually exists between the
size of the ascending aorta and the pulmonary artery. In the
present case, there is a disproportionate increase in the size of
the ascending aorta compared with the small size of the pulmonary artery (Figure C). Color Doppler also can be helpful to
show this and the direction of ow. In the three-vessel view of
the heart, the aorta may be enlarged and curved; this has been
called the “question mark” sign because the aorta resembles a
question mark on axial views. This was a helpful sign in 50% of
cases in which it was used. This sign can be seen when a threevessel view is performed on examination of the fetal heart by
use of ve short-axis views as described by Yagel et al.
Prognosis and Management
Obstetric management usually includes fetal karyotyping and
a comprehensive fetal ultrasound examination. Tetralogy of
Fallot may be associated with many different chromosomal
abnormalities, including trisomy 18, trisomy 21, and 22q11
microdeletion syndrome. Isolated tetralogy of Fallot usually
does not alter the standard of obstetric management unless
there is associated hydrops or polyhydramnios.
The prognosis of isolated tetralogy of Fallot is usually
of Fallot has a very low operative mortality rate, usually less
than 5%. The prognosis during childhood and adolescence is
good, but long-term problems, including pulmonary insufciency and right ventricular dysfunction, can occur later in life.
80
CASE 40
A
Used with permission from McGahan JP, Benacerraf BR: Fetal heart.
In McGahan JP, Goldberg BB [eds]: Diagnostic Ultrasound, 2nd ed.
New York: Informa Healthcare USA, 2008, 1279.
History: A patient undergoes a routine anatomic ultrasound
scan at 19 weeks’ gestation.
1. What should be included in the differential diagnosis?
(Choose all that apply.)
A. Hypoplastic left heart syndrome
B. Pulmonary atresia
C. VSD
D. Coarctation of the aorta
E. Endocardial cushion defect
2. Four different types of ventricular defects are identied
based on location. Which of the following is not one of
these types?
A. Perimembranous
B. Muscular
C. Subpulmonic
D. Apex
3. VSD is commonly associated with all of the following
anomalies except:
A. Transposition of the great arteries
B. Tetralogy of Fallot
C. Truncus arteriosus
D. Ebstein anomaly
B
Used with permission from McGahan JP, Benacerraf BR: Fetal heart.
In McGahan JP, Goldberg BB [eds]: Diagnostic Ultrasound, 2nd ed.
New York: Informa Healthcare USA, 2008, 1279.
4. Which of the following statements concerning the progno-
sis and management of VSDs is false?
A. Fetal echocardiography should be performed because
there are often associated anomalies.
B. A careful ultrasound examination should be performed
to check for extracardiac malformations.
C. Karyotyping should be considered.
D. Cesarean section should be considered a standard of
care for delivery.
81
ANSWERS
CASE 40
Ventricular Septal Defect
1. C and E
2. D
3. D
4. D
References
Axt-Fliedner R, Schwarze A, Smrcek J, et al: Isolated ventricular septal
defects detected by color Doppler imaging: evolution during fetal and rst
year of postnatal life. Ultrasound Obstet Gynecol 2006; 27(3):266-273.
http://www.ncbi.nlm.nih.gov/pubmed/16485323 (Accessed on June 7,
2012.)
Bahtiyar MO, Dulay AT, Weeks BP, et al: Prenatal course of isolated muscu-
lar ventricular septal defects diagnosed only by color Doppler sonography:
single-institution experience. J Ultrasound Med 2008; 27(5):715-720.
http://www.ncbi.nlm.nih.gov/pubmed/18424646 (Accessed on June 7,
2012.)
Paladini D, Palmieri S, Lamberti A, et al: Characterization and natural history
of ventricular septal defects in the fetus. Ultrasound Obstet Gynecol 2000;
16(2):118-122.
http://www.ncbi.nlm.nih.gov/pubmed/11117079 (Accessed on June 7,
2012.)
Cross-Reference
Ultrasound: The REQUISITES, 2nd ed, pp 416-417.
Comment
Differential Diagnosis
The differential diagnosis is usually small and includes VSD,
atrial septal defect, and endocardial cushion defect (atrioventricular canal). Most VSDs are perimembranous and very small,
whereas endocardial cushion defects are larger and may have
a higher rate of associated cardiac and noncardiac malformations. When a VSD is identied, however, a careful search for
complex cardiac malformations should always be performed.
Identication of VSD may be the rst clue to a more complex
cardiac malformation.
Ultrasound Findings
Small VSDs (1 to 2 mm) may be difcult to detect in utero
and probably account for most septal defects (Figure A). Color
Doppler may enhance diagnosis of small defects by showing
shunting across the septum (Figure B). Perimembranous and
subpulmonic VSDs are best visualized on longitudinal and
occasionally short-axis views of the heart. These views are
helpful because the perimembranous defect is located below
the aortic valve, and the subpulmonic defect is noted anteriorly
underneath the pulmonic valve. Larger VSDs may be more
obvious on in utero scanning.
Prognosis and Management
Most small VSDs (1 to 2 mm) are likely to be missed in utero.
It is also possible that many of these small VSDs may close in
utero. In one large neonatal series, 46% of all defects closed in
utero, 23% closed during the rst year of life, and 31% remain
open.
Obstetric management includes a careful search for associated intracardiac anomalies. When any malformation is
identied, a careful search for extracardiac malformations
is indicated. Fetal karyotyping for a small VSD is not always
necessary but should be considered. An isolated VSD should
not alter standard of care. However, prompt cardiac evaluation including newborn echocardiography is recommended
because other subtle cardiac malformations may not be apparent in utero.
Acknowledgment
Special thanks to Luke Wright, MD, for his contribution to
preparing this case.
82
CASE 41
A
History: A 67-year-old woman presents with vaginal
bleeding.
1. What should be included in the differential diagnosis for
Figures A and B? (Choose all that apply.)
A. Polyp
B. Submucosal broid
C. Endometrial carcinoma
D. Endometrial hyperplasia
2. How does the spectral Doppler image in Figure C aid in the
diagnosis?
A. High diastolic ow suggests a malignancy.
B. High systolic ow suggests a malignancy.
C. Spectral Doppler ow does not aid in the diagnosis.
3. What is an important question to ask a patient with a thickened endometrium?
A. Date of last menstrual period
B. Number of pregnancies she has had
C. Date of menarche
D. Age at rst pregnancy
4. What is the signicance of endometrial hyperplasia?
A. It is malignant.
B. Atypical hyperplasia is premalignant.
C. Simple hyperplasia is premalignant.
B
C
ANSWERS
CASE 41
Endometrial Cancer
1. C and D
2. A
3. A
4. B
References
Gull B, Karlsson B, Milsom I, et al: Can ultrasound replace dilation and
curettage? A longitudinal evaluation of postmenopausal bleeding and
transvaginal sonographic measurement of the endometrium as predictors
of endometrial cancer. Am J Obstet Gynecol 2003; 188(2):401-408.
http://www.ncbi.nlm.nih.gov/pubmed/12592247 (Accessed on June 7,
2012.)
Smith-Blindman R, Weiss E, Feldstein V, et al: How thick is too thick? When
endometrial thickness should prompt biopsy in postmenopausal women
without vaginal bleeding. Obstet Gynecol 2004; 24(5):558-565.
http://www.ncbi.nlm.nih.gov/pubmed/15386607 (Accessed on June 7,
2012.)
Cross-Reference
Ultrasound: The REQUISITES, 2nd ed, pp 540-544.
Comment
Clinical Findings in Endometrial Hyperplasia
Endometrial cancer usually occurs in women older than 50
years. Most patients present with postmenopausal bleeding.
Ultrasound Findings
Thickening of the endometrium is the rst indicator on ultrasound that an endometrial abnormality is present. A threshold
value of endometrial thickness for considering an endometrial
biopsy in a postmenopausal woman without vaginal bleeding
is 11 mm. Endometrial cancer often causes greater degrees of
endometrial thickening than benign etiologies (Figures A to
C), although hyperplasia can cause a very thick endometrium.
One series using transvaginal ultrasound showed that the
endometrium in endometrial cancer was almost always thicker
than 10 mm (90% of cases) and usually thicker than 20 mm.
The thickened endometrium is usually hyperechoic or heterogeneous and is rarely hypoechoic. Vascularity can be seen in
or around the tumor in most cases; the arterial ow often has
a low resistive index (<0.4) and elevated peak systolic velocity
(Figure C). Color Doppler ultrasound can conrm myometrial
invasion by detecting an interruption of the zone of decreased
echogenicity in the subendometrial region.
In young women, the normal endometrium can measure
15 mm, depending on the phase of the menstrual cycle. Postmenopausal women should have a double-layer thickness of
less than 5 mm. In postmenopausal women who had this thin
endometrial lining and vaginal bleeding, the incidence of endometrial cancer was shown to be about 0.07%; the incidence
was 7.3% if the endometrial lining was thicker. Postmenopausal women without vaginal bleeding and an endometrial
stripe of less than or equal to 11 mm had a risk of endometrial
cancer of about 0.002%, and the incidence was 6.7% if the
endometrial stripe was greater than 11 mm. The history of
vaginal bleeding is important in assessing the risk of endometrial cancer. In one publication, postmenopausal bleeding
was associated with a 64-fold increased risk for endometrial
cancer. Studies in recent years showed that patients receiving
tamoxifen therapy and women taking hormone supplements
postmenopausally and not bleeding may be allowed a normal
thickness of 8 to 10 mm, depending on the patient population.
The decision to perform a biopsy in this group is at the discretion of the clinician.
84
CASE 42
1
A
C
History: A patient presents with a mid–second-trimester
fetus that has a small head for gestational age.
1. What should be included in the differential diagnosis of
multiple echogenic foci in the fetal brain? (Choose all that
apply.)
A. Cytomegalovirus (CMV)
B. Varicella
C. Human immunodeciency virus (HIV)
D. Toxoplasmosis
2. Which of the following anomalies is not related to intrauterine infection?
A. Microcephaly
B. Porencephaly
C. Hydrocephalus
D. Choroid plexus cysts
3. Which of the following statements about intrauterine infection is true?
B
A. The infection is usually clinical in the mother.
B. The infection is usually subclinical in the mother.
C. The virus is almost never isolated in maternal urine or
cervix.
D. Serum antibodies in the mother are not helpful.
4. Which is not a nding in the fetus with TORCH (toxoplasmosis, other infections, rubella, CMV, herpes human immunodeciency virus [HIV] infection)?
A. Hepatosplenomegaly
B. Hepatic and periventricular calcications
C. Cardiomegaly
D. Small placenta
85