1. MR-Imaging in diagnosis and follow-up post corrective surgery of congenital heart disease
A.G. Opitz¹, P. Fries¹, A. Lindinger², H.-J. Schaefers³, A. Buecker¹, G. Schneider¹
¹Clinic of Diagnostic and Interventional Radiology
²Dept. of Pediatrics
³Dept. of Cardiac and Thoracic Surgery
Saarland University Hospital,
Homburg / Saar, Germany

2. Background
In neonates and infants with congenital heart disease (CHD) detailed imaging of vascular and cardiac anatomy is required for planning treatment and to follow up surgical procedures.

3. Background
One of the well-established applications of MRI is the evaluation of congenital heart disease. Accurate determination of cardiac anatomy and function is crucial for patient management at initial diagnosis as well as during follow-up of patients after repair of cardiovascular malformations.

4. Background
In addition to echocardiography, cardiac MRI is non-invasive diagnostic tool in children with CHD that does not require radiation exposure.
Combination of cross-sectional and functional imaging together with CE MRA studies may give a complete overview on pre- and post surgical status and give important information for patient management.

5. Learning objectives
In this educational exhibit we will review the use of MR imaging in the diagnosis of the different forms of congenital heart disease in pediatric patients.
An overview of common forms of CHD along with treatment procedures will be given.
Special interest is taken on technical issues and particularities in post surgical evaluation will be highlighted.

6. Congenital heart disease (CHD)
Classification of CHD:
Left - to - right shunts (acyanotic conditions)
Right- to- left shunts (cyanotic conditions)
Valvular and aortic anomalies

7. Congenital heart disease

8. Congenital heart disease
Acyanotic conditions
60%
Ventricular septal defect (VSD) %
Atrial septal defect (ASD) %
Patent Ductus Botalli (PDA)
10%
Atrioventricular septal defect (AVSD)
2 - 4 %
Truncus arteriosus 1%
Aortopulmonal window

9. Congenital heart disease
Cyanotic conditions
20%
Tetralogy of Fallot 6%
TGA 4%
Tricuspid atresia 2%
Pulmonal atresia
Total anomalous pulmonary venous return
Univentricular heart 1%

10. Congenital heart disease
Valvular / aortal anomalies
20%
Pulmonary stenosis 7%
Coarction of aorta
Valvular aortic stenosis 6%

11. Surgical shunts in CHD Shunt Procedure Classic Blalock-Taussig
Subclavian artery to pulmonary artery end-to-side anastomosis
Modified Blalock-Taussig
Tube graft from subclavian artery to pulmonry artery
Bidirectional Glenn
SVC to RPA
Fontan Procedure
IVC to RPA to complete venous circulatic to PAS
Waterson
Ascending aorta to RPA
Potts
Descending aorta to LPA

12. Surgical procedures in CHD
Surgery
Rastelli Procedure
Transposition with a VSD
Norwood Procedure
Hypoplastic LV syndrome
Mustard Procedure
TGA:
SVC/IVC baffled to LA/LV/PA,
Pulmonary Veins baffled to RA/RV/Ao
Arterial Switch (Senning)
TGA: aorta and MPA are switched, coronary arteries are re-implanted

13. Imaging technique
Combination of cross-sectional imaging and CE-MRA provides functional evaluation and dynamic information
Examination should include:
- Measurements of biventricular volumes, EF, mass
- RVOT Cine sequences
- Aortic root +/- aortic regurgitation quantification
- Pulmonary regurgitant fraction
- Assessment of LPA and RPA

14. Atrial Septal Defect (ASD)
Most common CHD in adults
All connections between right and left atrium
1/1500 live births
Majority repaired in childhood Asymptomatic / Symptomatic (dyspnea, heart failure, emboli, atrial fibrillation)

15. Atrial Septal Defect (ASD)
Classification:
Secundum
most common CHD (6-10%)
RAD
Primum
associated with other endocardial cushion defects (cleft AV- valves, inlet type, VSD)
LAD
Sinus Venosus
large, associated with anomalous pulmonary venous drainage (usually R superior PV)
Coronary sinus (rare)
associated with unroofed coronary sinus

16. Atrial Septal Defect (ASD)
Treatment:
Percutaneous Closure:
only for secundum defect
Surgical Closure:
Good prognosis if closure age < 25 and PA pressure <40 

17. Atrial Septal Defect (ASD)
Sinus venosous defect

18. Atrial Septal Defect (ASD)
Four chamber view of Ostium secundum defect ( CINE Sequence )

19. Atrial Septal Defect (ASD)
Four chamber view of Septum primum defect ( CINE Sequence )

20. Ebstein´s anomaly and Atrial Septal Defect (ASD)
Four chamber view ( CINE Sequence )

21. Ventricular Septal Defect (VSD)
Most common cardiac defect seen in infants and children ( live births, 20% of CHD)
Classification:
Perimembranous VSD (75-80%)
Muscular VSD (ca.15%)
Double commited subarterial VSD (5%)

22. Ventricular Septal Defect (VSD)
Four chamber view ( phase contrast sequence shows a flow signal traversing the ventricular septum (arrow) from right to left indicating “Eisenmenger-reaction“).

23. Patent Ductus arteriosus
Physiologic shunt between MPA and descending aorta
DA is substantially closed within hours after birth
Usually completely sealed after to four three weeks

24. Patent Ductus arteriosus

25. Patent Ductus arteriosus
If DA remains patent, left to right shunt occurs
Untreated risk of Eisenmenger´s reaction
In complex heart disease (TGA) PDA is the only way to supply oxygenated blood

26. Coarctation of the Aorta
Focal aortic narrowing close to ligamentum arteriosum (postductal type)
Presence of collaterals
in % associated with bicuspid aortic valve

27. Coarctation of the Aorta
Differential diagnosis:
tubular hypoplasia (preductal type)
interrupted aortic arch  
pseudocoarctation
Takayasu arteritis   

28. Coarctation of the Aorta
Operative technique
Comment
Resection with end-to-end anastomosis
patients older than 1 year
Resection with extended anastomosis
enlargement of hypoplastic aortic arch
Prosthetic patch aortoplasty
high incidence of late aneurysms
Interposition (tube) graft
if long segment of CoA is present
Subclavian flap aortoplasty
primarily used in younger children

29. Coarctation of the Aorta   
VRT shows coarctation of the aorta with subsequent subtotal stenosis of the vessel

30. Coarctation of the Aorta
Note the marked dilatation of the internal thoracic artery (arrow).

31. Coarctation of the Aorta   

32. Tetralogy of Fallot  
Most commom cyanotic condition,composed of four features:
subpulmonary infundibular stenosis
ventricular septal defect (VSD)
overrriding of the aortic valve
right ventricular hypertrophy
Variability correlates with degree of RVOT obstruction and size/anatomy of PA

33. Tetralogy of Fallot Treatment Reparative takedown of prior shunt
closing the VSD
relieving the RVOT: pulmonary valvotomy, resecion of infundibular muscle, transannular or RVOT patch

34. Tetralogy of Fallot Treatment: Palliative Blalock-Taussig-Shunt:
End-to-side anastomosis between subclavian artery and ipsilateral pulmonary artery

35. Tetralogy of Fallot Complications following surgery:
Shunt obstruction or stenosis
Pseudoaneurysms
Pulmonary hypertension and/or left ventricular dysfunction
"Pseudo RVOT" from endothelial overgrowth and obstruction of the conduit 

36. Tetralogy of Fallot
Note the surgical connection of the ascending aorta to the right pulmonary artery with consecutive hyperperfusion of the right lung.

37. Tetralogy of Fallot
Consecutive coronal MPRs in the same patient.

38. Tetralogy of Fallot
MRI post surgery (CINE ax.)

39. Tetralogy of Fallot
MRI post surgery (MPR, MIP cor.)

40. Tetralogy of Fallot
6yo patient with surgically corrected TOF. CE CINE short axis view shows subvalvular RVOT dilatation.

41. Transposition of the great arteries (TGA)
TGA approximately 5% of all congenital heart disease,
CCTGA in approx %.
Classification
D-TGA
Simple TGA (isolated TGA): with intact ventricular septum
Complex TGA: with VSD or other defects

42. Transposition of the great arteries (TGA)
Classification:
L-TGA (“congenitally corrected” TGA)
Isolated: without other defects
CCTGA with other defects:
Tricuspid valve anomalies (90%)
VSD (75%)
Pulmonary valve stenosis (75%)

43. D-TGA Systemic and pulmonary circulations
consist of two parallel circuits:
IVC+SVC→ RA→ RV→ transposed aorta→ systemic circulation→ IVC+SVC
PV→ LA→ LV→ transposed PA→ pulmonary circulation

44. D-TGA
Cocordant atrioventricular and disocordant ventriculoarterial connections
PA from LV, Aorta from RV and anterior/right of PA
corrected initially with prostaglandin to keep DA open and balloon, atrial septostomy to improve systemic saturation
repair via “atrial switch” Mustard procedure
SVC/IVC baffled to LA/LV/PA
Pulmonary Veins baffled to RA/RV/Ao
repair via “arterial switch”

45. D-TGA post surgery
5yo patient after arterial switch procedure. CE-MRA shows aneurysm of the ascending aorta.

46. D-TGA post surgery
5yo patient after arterial switch procedure. MIP shows aneurysm of the ascending aorta.

47. L-TGA Double discordance:
Discordant atrioventricular and discordant ventriculoarterial connections
Without associated anomalies asymptomatic until adulthood

48. L-TGA
Complications after atrial redirection surgery (Mustard or Senning procedure):
Bradyarrhythmia
Tachyarrhythmia
Baffle obstruction
Baffle leak
Ventricular dysfunction
Sudden cardiac death

49. Pulmonary veins / arteries:
Anomalous pulmonary venous return (APVR)
- Total APVR (2% of patients with CHD)
- Partial APVR ( % of patients with CHD)
Pulmonary Sequestration
• Scimitar Syndrome
Pulmonary Atresia (with and without VSD)

50. Pulmonary veins / arteries:
PAPVR

51. Pulmonary veins / arteries:
PAPVR post surgery
Note the surgical connection of the SCV to the left atrium (arrow).

52. Pulmonary veins / arteries:
Coronary MIP shows partial anomalous venous return. Note on the left side a so-called vertical vein draining to the superior caval vein and on the right side the superior pulmonary vein draining to the SCV adjacent to the acygos vein.

53. Pulmonary veins / arteries:
Anomalous venous return of upper right pulmonary vein in the SCV adjacent to the drainage of the acygos vein.

54. Pulmonary veins / arteries:
Pulmonary Atresia
with ventricular septal defect (VSD) and
multiple aortopulmonary collateral arteries
(MAPCAs), hypoplastic pulmonary arteries
with intact VS, normal sized pulmonary
arteries supplied by ductus arteriosus
and patent foramen ovale

55. Pulmonary veins / arteries:
Imaging post surgery
- Blalock-Taussig shunt
- Glenn shunt
- Hemi-Fontan
- Kawashima operation
- Fontan

56. Pulmonary veins / arteries:
Functionally univentricular heart and Glenn shunt

57. Pulmonary veins / arteries:
Kawashima operation
Bilateral Glenn shunt and incorporation of the azygos vein in cavopulomonary anastomosis.

58. Thank you very much for your attention!