Download MR-Imaging in diagnosis and follow-up post corrective

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
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.
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.
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.
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.
Congenital heart disease (CHD)
Classification of CHD:
• Left - to - right shunts (acyanotic conditions)
• Right- to- left shunts (cyanotic conditions)
• Valvular and aortic anomalies
Congenital heart disease
Congenital heart disease
Acyanotic conditions
60%
Ventricular septal defect (VSD)
15 - 30 %
Atrial septal defect (ASD)
5 - 10 %
Patent Ductus Botalli (PDA)
10%
Atrioventricular septal defect
(AVSD)
2-4%
Truncus arteriosus
1%
Aortopulmonal window
1%
Congenital heart disease
Cyanotic conditions
20%
Tetralogy of Fallot
6%
TGA
4%
Tricuspid atresia
2%
Pulmonal atresia
2%
Total anomalous pulmonary venous
2%
return
Univentricular heart
1%
Congenital heart disease
Valvular / aortal anomalies
20%
Pulmonary stenosis
7%
Coarction of aorta
7%
Valvular aortic stenosis
6%
Surgical shunts in CHD
Shunt
Classic Blalock-Taussig
Modified Blalock-Taussig
Procedure
Subclavian artery to pulmonary
artery end-to-side anastomosis
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
Surgical procedures in CHD
Procedure
Surgery
Rastelli Procedure
Transposition with a VSD
Norwood Procedure
Hypoplastic LV syndrome
Mustard Procedure
Arterial Switch (Senning)
TGA:
SVC/IVC baffled to LA/LV/PA,
Pulmonary Veins baffled to
RA/RV/Ao
TGA: aorta and MPA are
switched, coronary arteries are
re-implanted
Imaging technique
• Combination of cross-sectional imaging and CEMRA 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
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)
Atrial Septal Defect (ASD)
Classification:
• Secundum
– most common CHD (6-10%)
– RAD
• Primum
– associated with other endocardial cushion defects (cleft AVvalves, 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
Atrial Septal Defect (ASD)
Treatment:
• Percutaneous Closure:
only for secundum defect
• Surgical Closure:
Good prognosis if closure age < 25 and PA
pressure <40
Atrial Septal Defect (ASD)
Sinus venosous defect
Atrial Septal Defect (ASD)
Four chamber view of Ostium secundum defect ( CINE Sequence )
Atrial Septal Defect (ASD)
Four chamber view of Septum primum defect ( CINE Sequence )
Ebstein´s anomaly and Atrial
Septal Defect (ASD)
Four chamber view ( CINE Sequence )
Ventricular Septal Defect (VSD)
• Most common cardiac defect seen in infants and
children (1.5-3.5 live births, 20% of CHD)
Classification:
• Perimembranous VSD (75-80%)
• Muscular VSD (ca.15%)
• Double commited subarterial VSD (5%)
Ventricular Septal Defect (VSD)
Four chamber view ( phase contrast sequence shows a flow signal traversing
the ventricular septum (arrow) from right to left indicating “Eisenmengerreaction“).
Patent Ductus arteriosus
• Physiologic shunt between MPA and descending
aorta
• DA is substantially closed within 12-24 hours
after birth
• Usually completely sealed after to four three
weeks
Patent Ductus arteriosus
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
Coarctation of the Aorta
• Focal aortic narrowing close to ligamentum
arteriosum (postductal type)
• Presence of collaterals
• in 30 - 50% associated with bicuspid aortic valve
Coarctation of the Aorta
Differential diagnosis:
• tubular hypoplasia (preductal type)
• interrupted aortic arch
• pseudocoarctation
• Takayasu arteritis
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
Coarctation of the Aorta
MIP shows coarctation of the
aorta with subsequent
subtotal stenosis of the
vessel
Coarctation of the Aorta
Coarctation of the Aorta
Coarctation of the Aorta
Coarctation of the Aorta
Sag. MRI post
intervention
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
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
Tetralogy of Fallot
Treatment:
Palliative
• Blalock-Taussig-Shunt:
End-to-side anastomosis between subclavian
artery and ipsilateral pulmonary artery
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
Tetralogy of Fallot
Tetralogy of Fallot
Tetralogy of Fallot
MRI post surgery (CINE ax.)
Tetralogy of Fallot
MRI post surgery (MPR, MIP cor.)
Tetralogy of Fallot
6yo patient with surgically corrected TOF. CE CINE short
axis view shows subvalvular RVOT dilatation.
Transposition of the great arteries
(TGA)
• TGA approximately 5% of all congenital heart
disease,
• CCTGA in approx. 0.6-1.4%.
Classification
• D-TGA
1. Simple TGA (isolated TGA): with intact
ventricular septum
2. Complex TGA: with VSD or other defects
Transposition of the great arteries
(TGA)
Classification:
L-TGA (“congenitally corrected” TGA)
1. Isolated: without other defects
2. CCTGA with other defects:
•
•
•
Tricuspid valve anomalies (90%)
VSD (75%)
Pulmonary valve stenosis (75%)
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
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”
D-TGA post surgery
5yo patient after arterial switch procedure. CE-MRA shows
aneurysm of the ascending aorta.
D-TGA post surgery
5yo patient after arterial switch procedure. MIP shows
aneurysm of the ascending aorta.
L-TGA
•
•
Double discordance:
Discordant atrioventricular and discordant
ventriculoarterial connections
Without associated anomalies asymptomatic
until adulthood
L-TGA
Complications after atrial redirection surgery
(Mustard or Senning procedure):
• Bradyarrhythmia
• Tachyarrhythmia
• Baffle obstruction
• Baffle leak
• Ventricular dysfunction
• Sudden cardiac death
Pulmonary veins / arteries:
•
•
•
•
Anomalous pulmonary venous return (APVR)
- Total APVR (2% of patients with CHD)
- Partial APVR (0.3 - 0.5% of patients with
CHD)
Pulmonary Sequestration
Scimitar Syndrome
Pulmonary Atresia (with and without VSD)
Pulmonary veins / arteries:
• PAPVR
Pulmonary veins / arteries:
• PAPVR post surgery
Note the surgical
connection of the SCV to
the left atrium (arrow).
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.
Pulmonary veins / arteries:
• Anomalous venous
return of upper right
pulmonary vein in the
SCV adjacent to the
drainage of the
acygos vein.
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
Pulmonary veins / arteries:
• Imaging post surgery
- Blalock-Taussig shunt
- Glenn shunt
- Hemi-Fontan
- Kawashima operation
- Fontan
Pulmonary veins / arteries:
Functionally univentricular heart and
Glenn shunt
Pulmonary veins / arteries:
Kawashima operation
Bilateral Glenn shunt
and incorporation of
the azygos vein in
cavopulomonary
anastomosis.
Thank you very much for your attention!