Classic Images in Congenital Heart Disease
Benjamin W. Eidem
Robert E. Shaddy
Paul F. Kantor
Bryan C. Cannon
Frank Cetta
Questions
A. Anomalous origin of left coronary artery from pulmonary artery (ALCAPA)
B. Anomalous origin of right coronary artery from pulmonary artery (ARCAPA)
C. High, anterior, and leftward origin of the right coronary artery
D. Origin of the right coronary artery from the right sinus of Valsalva
E. Origin of the left coronary artery from the left sinus of Valsalva
View Answer
1. (C) See Figure 18.30. Anomalous origin of the RCA (AORCA) from the left sinus or anomalous origin of the LCA (AOLCA) from the right sinus can cause this ring sign. Also, a high, anterior, and leftward origin of the RCA (a benign anomaly) can give the same appearance as the RCA that crosses anteriorly over the aortic root. Answer (D) and (E) are normal.
 FIGURE 18.30 Interarterial course of CA (arrow). Please note: this is a parasternal long axis image with the ring anterior to the right cusp. (Derived from Jureidini, et al. JASE 2003.) |
A. Tetralogy of Fallot
B. Membranous VSD
C. Infracardiac TAPVC
D. TAPVC to the coronary sinus
E. Large secundum ASD
View Answer
2. (C) These are subcostal echo images in the frontal plane which show a classic “whale-tail” sign consistent with TAPVC to the coronary sinus.
3. The patient with the echo image shown in Figure 18.3 was diagnosed with LV noncompaction (LVNC). What would be the next best decision in this patient’s management?
 FIGURE 18.3 |
A. Take a careful family history to assess risk for sudden death
B. Place a Holter monitor
C. Perform MRI to confirm diagnosis of LVNC
D. Perform genetic testing for LVNC
E. Obtain a second opinion on the echo reading
4. Figure 18.4 shows two vessels on either side of a dilated aortic root.
 FIGURE 18.4 |
What congenital cardiac lesion does this patient most likely have?
A. Tetralogy of Fallot
B. d-TGA
C. Bicuspid aortic valve
D. Marfan syndrome
E. Turner syndrome
View Answer
4. (B) This patient had d-TGA and a subsequent arterial switch with a LeCompte maneuver. The two vessels that straddle the ascending aorta are the RPA and LPA. See the horizontal cut from same patient in Figure 18.31.
 FIGURE 18.31 |
A. Classic Blalock-Taussig-Thomas shunt
B. Mustard
C. Baffes
D. Arterial switch
E. Fontan
6. The patient who has the chest radiograph shown in Figure 18.6 has what chance of having congenital heart disease?
 FIGURE 18.6 |
A. >95%
B. 75%
C. 50%
D. 25%
E. <1%
View Answer
6. (E) The chest radiograph demonstrates situs inversus totalis. The risk of CHD is the same as the general population, 0.8%.
7. A 12-year-old male presented with acute stroke-like symptoms. He experienced recurrent fever, unintentional weight loss, and dizziness over the past 3 months. Physical examination discloses the skin findings shown in Figure 18.7.
 FIGURE 18.7 |
What is his most likely genetic disorder?
A. Gorlin syndrome
B. Tuberous sclerosis
C. Neurofibromatosis
D. Carney complex
E. None of the above
View Answer
7. (D) He likely had a stroke due to embolization of an atrial myxoma and has Carney complex. Carney complex is an association of myxomas, spotty pigmentation, and endocrine over-reactivity. Inheritance is autosomal dominant (PRKAR1A [PRKACA, PRKACB]) and 70% of cases are familial. Gorlin syndrome is associated with fibromas. Tuberous sclerosis is associated with rhabdomyomas. Various congenital defects have been reported with neurofibromatosis.
A. I
B. III
C. IV
D. VI
E. X
View Answer
8. (C) This MRI demonstrates a double aortic arch, an issue with persistence of a right fourth arch.
10. Which of the following congenital cardiac lesions is the least likely to cause the chest radiograph finding shown in Figure 18.10?
 FIGURE 18.10 |
A. ASD
B. VSD
C. PDA
D. AP window
E. Bicuspid aortic valve
View Answer
10. (E) The main pulmonary artery shadow is dilated. All of the lesions listed can cause this except an isolated bicuspid aortic valve.
11. You are asked to evaluate a postoperative patient who had chest tubes removed approximately 1 hour ago. He is doing well except for complaining of some mild incisional pain. Oxygen saturation in room air is 100%. Based on this chest radiograph (Figure 18.11), what is the next best course of action?
 FIGURE 18.11 |
A. Obtain stat echocardiogram
B. Reinsert chest tube
C. Prescribe ibuprofen
D. Do nothing, observe
View Answer
11. (D) The patient has a small pneumopericardium shortly after removal of a chest tube. Since he is hemodynamically stable, observation is all that is needed. A repeat chest x-ray in 24 hours would be prudent. Echocardiogram images with a large pneumopericardium will frequently be of poor quality due to air artifact.
12. You are asked to evaluate a postoperative patient who had chest tubes removed approximately 1 hour ago. He is tachypneic and tachycardiac. Oxygen saturation in room air is 88%. Based on this chest radiograph (Figure 18.12), what is the next best course of action?
 FIGURE 18.12 |
A. Obtain stat echocardiogram
B. Reinsert chest tube
C. Prescribe ibuprofen
D. Do nothing, observe
View Answer
12. (B) There is a large right pneumothorax. The patient is asymptomatic and oxygen saturations are reduced. A chest tube should be reinserted.
13. This Doppler signal from the abdominal aorta (Figure 18.13) is most consistent with which of the following:
 FIGURE 18.13 |
A. Mild aortic valve insufficiency
B. Coarctation of the aorta
C. Large patent ductus arteriosus
D. Severe aortic valve stenosis
E. Hypertension
View Answer
13. (C) This Doppler pattern in the abdominal aorta depicts holodiastolic reversal of flow. This pattern is consistent with abnormalities that have a large runoff including a large PDA, severe aortic valve insufficiency, an aortopulmonary window, and a large cerebral arteriovenous malformation.
14. This classic chest x-ray (CXR) (Figure 18.14A,B) is most likely to be found in which of the following cardiac lesions?
 FIGURE 18.14 |
A. Ebstein anomaly
B. Coarctation of the aorta
C. D-transposition of the great arteries
D. Tetralogy of Fallot
E. Total anomalous pulmonary venous connection
View Answer
14. (A) This CXR is from a patient with Ebstein anomaly with severe dilatation of the right atrium and atrialized right ventricle resulting in severe cardiomegaly. Some have compared this classic CXR to a basketball within the chest. Other “classic” CXRs include coarctation of the aorta (figure of 3 sign), D-TGA (an egg on a string), tetralogy of Fallot (a boot shaped heart), and TAPVC (snowman sign).
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