CT Chapter 11 - Congenital

Question 1

Describe the findings:

Answer 1

Tricuspid atresia

• 3rd most common form of cyanotic congenital heart disease (0.3-3.7%)
• Absent TV → underdeveloped (hypoplastic) / absent RV → single LV physiology
• Requires ASD to be compatible with life
• Concomitant pulmonary atresia / stenosis also present

Question 2

Describe the findings:

Answer 2

Unroofed coronary sinus ASD

• Diagnosed by lack of contrast proximal to the lesion and the relative lack of contrast in the RA
• L → R shunt at the level of the coronary sinus and the LA

Question 3

What are common associations?

• unroofed coronary sinus

Answer 3

• persistent left SVC
  
  • agitated saline may help establish if unroofed coronary sinus also present

Question 4

Describe the findings:

Answer 4

PDA (large)

• normal prenatal channel between the aortic arch and the pulmonary artery remains open
• L-to-R shunting of blood

Question 5

What are common associations:

• PDA

Answer 5

• hypoplastic left heart
• D-transposition of Great Vessels
• Pulmonary atresia

Question 6

Describe the findings:

Answer 6

A. Partial anomalous pulmonary venous return (PAPVR)

• anomaly resulting in some of the pulmonary veins draining into systemic veins rather than the LA

B. Sinus Venosus ASD

• superiorly located

Question 7

Where do PAPVR drain?

• Right
• Left

Answer 7

• Right
  
  • azygous vein
  • RA
  • SVC
  • Coronary sinus
• Left
  
  • SVC (left sided)
  • Left brachiocephalic vein

Question 8

What is Scimitar sydrome?

Answer 8

• pulmonary vein drains below the diaphragm into the IVC
• Associations:
  
  • hypoplasia of the right lung
  • sinus venosus ASD

Question 9

Describe the findings:

Answer 9

Muscular VSD

• superior right ventricular insertion point
  *

Question 10

Describe the findings:

Answer 10

Scimitar Syndrome

• “congenital pulmonary venolobar” sydrome
• PAPVR in which venous drainage from all or some part of the particular lung (majority right lung) occurs into the IVC
• Association:
  
  • hypoplastic right lung

Question 11

What syndrome is most likely to be associated with this abnormality?

Answer 11

Shone Complex

• supravalvar mitral ring, parachute mitral valve, subaortic stenosis, aortic coarctation
• Bicuspid AV present in 84-89% of these patients

Question 12

What are the associated syndromes?

• Bicuspid aortic valve

Answer 12

• Shone complex → 84-89%
• Coarctation → 36-70%
• Turner and Williams Syndrome → less common

Question 13

Describe the findings:

Answer 13

L-transposition of the great arteries with Dextrocardia

Question 14

Describe the findings:

Answer 14

PDA

• extends from the descending aorta - superior aspect of the pulmonary artery near the pulmonary bifurcation

Question 15

Describe the findings:

Answer 15

Cor triatriatum sinister (double chambered left atrium)

• prominent septation → double chambered LA
• Common association:
  
  • anomalous pulmonary venous return
  • persistent L SVC (not seen)
  • unroofed coronary sinus (not seen)

Question 16

Describe the findings:

Answer 16

Superior sinus venosus defect / LV dilatation not expected

• creates an ASD with left-to-right shunting
• Common associations:
  
  • anomalous pulmonary venous return
  • persistent L SVC
  • Right sided dilatation
• Images:
  
  • RV dilatation
  • left panel white arrow → dilated coronary sinus
  • center panel white arrow → continuation of coronary sinus into a persistent L SVC without contrast due to a right upper extremity contrast injection
  • left panel white arrow → anomalous pulmonary venous return at level of sinus venosus defect

Question 17

Describe the findings:

Answer 17

Secundum ASD (large)

• severe R sided chamber dilation
• dense contrast pooling that extends form the RA to the LA which raises concern for Eisenmenger’s syndrome

Question 18

Describe the findings:

Answer 18

Hypoplastic left heart syndrome

• star - VSD
• Fontan procedure - surgically closed systemic venous to right atrial fenestration

Question 19

Describe the findings:

Answer 19

D-Transposition of the great arteries

Question 20

Describe the findings and common associations:

Answer 20

Absence of Pulmonic Valve

• Common associations:
  
  • Tetralogy of Fallot (TOF) should be suspected
    
    • Pulmonary stenosis
    • RV hypertrophy
    • Overriding Aorta
    • VSD

Question 21

Describe the findings:

Answer 21

Membranous VSD

Question 22

Describe the findings:

Answer 22

Unroofed coronary sinus

• Panel A
  
  • dextrocardia
  • dilated RA/RV → raise suspicion for a shunt from LA to RA
  • LV normal size → VSD unlikely
• Panel B
  
  • white arrow → persistent L SVC
    
    • common association → unroofed coronary sinus (variant of ASD as communication from LA to RA via coronary sinus)
• Panel C
  
  • axial projection showing the dilated coronary sinus
• Panel D
  
  • coronal projection showing the coronary sinus adjacent to the LA
  • typically the CS is inferior to the LA but is superior due to anatomic distortion

Question 23

Describe the findings:

Answer 23

Right sided SVC communicating to LA

• bright contrast in the IVC suggests that there is a SVC communicating to the CS and RA
• rare congenital abnormality
• ICD seen in LV apex rather than RV apex
  *

Question 24

What type of ASD was likely repaired?

Answer 24

Primum ASD / Complete AV canal defect

• AV canal defects / endocardial cushion defects
• Common association:
  
  • cleft mitral valve