201

History: A patient presents with cyanosis.

1. What lesions characteristically manifest with
decreased vascularity on radiography? (Choose all
that apply.)
A. Ebstein anomaly
B. Truncus arteriosus
C. Tetralogy of Fallot
D. Pulmonary atresia with ventricular septal defect
2. What is the most likely diagnosis in this patient?
A. Ebstein anomaly
B. Truncus arteriosus
C. Tetralogy of Fallot
D. Pulmonary atresia with ventricular septal defect
3. Which valve is most likely abnormal?
A. Mitral
B. Aortic
C. Tricuspid
D. Pulmonary
4. Which imaging modality best assesses the valve
leaets?
A. Echocardiography
B. CT
C. MRI
D. Angiography
CASE 99
A
B
202
Ebstein Anomaly
1. A, C, and D
2. A
3. C
4. A
Reference
Higgins CB: Radiography of congenital heart disease. In Webb WR,
Higgins CB, editors: Thoracic imaging: pulmonary and cardiovas-
cular radiology, ed 2, Philadelphia, 2010, Lippincott Williams &
Wilkins.
Cross-Reference
Cardiac Imaging: The REQUISITES, ed 3, pp 206207.
Comment
Etiology and Physiology
Ebstein anomaly is a congenital lesion characterized by
an abnormality of the tricuspid valve; the septal and
posterior leaets are displaced apically, causing tricuspid
regurgitation. A portion of the right ventricle is function-
ally incorporated into the atrium, which is known as
atrialization of the right ventricle, although the atrial-
ized portion of the ventricle contracts during ventricular
systole. Tricuspid regurgitation causes marked enlarge-
ment of the right ventricle and atrium. Tricuspid regur-
gitation with a coexisting atrial septal defect results in a
right-to-left shunt. Pulmonary vascularity is decreased,
and cyanosis can develop. Ebstein anomaly is commonly
associated with either an atrial septal defect or a patent
foramen ovale. Less common associations include supra-
ventricular tachycardia (25% to 50%), pulmonary atresia
or stenosis (25%), and Wolff-Parkinson-White syndrome
(10%). Maternal lithium use during the rst trimester may
result in Ebstein anomaly.
Imaging
Chest radiography characteristically demonstrates
marked enlargement of the heart, particularly the right
atrium and ventricle (Figs. A and B). Echocardiography
is used for further evaluation. MRI can also be performed
to assess the anatomy and to quantify the severity of
regurgitation. MRI provides the same information as
echocardiography and gives more reliable and reproduc-
ible information in patients with limited acoustic
windows. It can assess right ventricular size and function
and accurately quantitate the volume of tricuspid regur-
gitation, which has a direct impact on the timing of
valvular surgery in these patients.
Differential Diagnosis
A few other rare conditions may manifest with cyano-
sis, cardiomegaly, and reduced pulmonary vascularity,
including (1) severe pulmonic stenosis with a restrictive
atrial septal defect, (2) tricuspid atresia with a restric-
tive atrial septal defect, and (3) pulmonary atresia with
a restrictive ventricular septal defect.
ANSWERS
CASE 99
Notes