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INTÉRÊT DE L’IRM CARDIAQUE DANS LES
CARDIOPATHIES CONGÉNITALES
L. IACUZIO, N. HUGUES, F. CIVAIA
• 47y , woman, from Gabon • Malaria • Asthenia • ETT PAPs 35 mmHg • LVEF 64 %, EDVI 104 ml/m², SV 66 ml/m² • RVEF 43 %, EDVI 79 ml/m², SV 33 ml/m².
• Left-right shunt • Qp/Qs = 0,5.
Patent ductus arteriosus • Left-right shunt • Qp/Qs = 0,5.
CMR - Advantages
• High spatial and temporal resolution
• Exhaustive anatomical and functional study in any desired plane LV, RV, aorta, pulmonary veins and arteries, flow
• Diagnosis is less observer-dependent No “acoustic window” limitations
• Non invasive and no X-ray : follow up GUCH !
Contraindications to CMR (pacemaker non MRI compatible, metallic splinters, cerebral clips) Patient’s limitations: AF, extrasystoles, claustrophobia, artefacts
from metallic device/stents Need of patient collaboration: long scan, apnoea New sequences in free breathing
Operator training: complex anatomy, complex exam
Limitations
• morphological-ciné sequences anatomy • phase contrast sequences velocity max, regurgitant fraction
• (4D flow)
• 3D angiography Gd free : morphology, diameters
• CE-Angiography (Gadolinium)
Technique
Diagnosis echocardiography, CMR
Follow up GUCH echocardiography CMR
Congenital heart disease
16y boy: f-up of complex congenital disease
16y boy: f-up of complex congenital disease
16y boy: f-up of complex congenital disease
16y boy: f-up of complex congenital disease
Situs inversus - double discordance (CCTGA)
IVC - SVC
RA
RV
PA
pulmonary veins
aorta
LA
LV
• Bicuspid aortic valve • ASD – atrial septal defect • VSD – ventricular septal defect • Tetralogy of Fallot (TOF) • Transposition of the Great Arteries (TGA) • Pulmonary atresia / stenosis • Single ventricle • Double inlet LV • Double outlet RV • Ebstein’s anomaly • Hypoplastic LH/RH syndrome • Aortic coarctation • Patent ductus arteriosus
Congenital heart disease
Valve morphology (type 0, 1, 2)
Stenosis and regurgitation quantification LV volumes and mass
Thoracic aorta
Bicuspid valve (1-2% general population)
Valve morphology (type 0, 1, 2)
Quadricuspide
Aortic stenosis
0,4 cm²/m²
area Max velocity
V max 4 m/s
Aortic regurgitation
Regurgitant fraction 63%
LV volumes
EF 60% EDVI 156 ml/m²
LV volumes and mass
Angiography +/- gadolinium injection
3D Angio without Gd 3D Angio with Gd
Pre Post
Coarctation of the aorta
• Dob 1972 • 1982 tube dacron
• Dob 1972 • 1982 tube dacron • 2007 ascending-descending
connection
Coarctation of the aorta
Coarctation of the aorta
4D flow
Veine cave supérieure gauche
Tetralogy of Fallot : follow up
• Pulmonary regurgitation quantification
• Right ventricle volumes
• Infundibulum stenosis
• Pulmonary arteries stenosis / flow distribution
ToF operated f-up Pulmonary flow distribution
Regurgitant fraction 51%
RPA 65 ml LPA 55 ml
ToF operated f-up RV volumes
Timing of pulmonary valve replacement RV EDVI > 150-180 ml/m²
RVEF 51 %, EDVI 192 ml/m² ESVI 95 ml/m²
EF 52 % EDVI 112 ml/m² ESVI 53 ml/m²
Pre Post 15y, boy
Pulmonary valve replacement
ToF
Infundibular anevrysme
Vmax à 3,4 m/s
Pulmonary stenosis
ToF operated f-up RPA stenosis
ToF operated f-up - RV fibrosis
Ventricular Fibrosis suggested by Cadiovascular Magnetic Resonance in Adult With Repaired Tetralogy of Fallot and Its Relationship to Adverse Markers of Clinical Outcome – Sonya V. Babu-Narayan – 2006 - circulation
ToF operated f-up LV myocardial infarction
13 y, boy 2y TOF correction 12y PV remplacement occlusion CX
• RV volumes • Visualization direction shunt • Qp/Qs
ASD type sinus venosus
ASD type sinus venosus - abnormal pulmonary vein return with overridding of superior vena cava
total abnormal pulmonary venous return
Ant Post
ASD
Sténose valvulaire pulmonaire
V max 3,1 m/s Vmax 2,5 m/s Vmax 3,3 m/s Area 0,6 cm²
Infundibular stenosis
V max 3,8 m/s
TGA : arterial Switch
15y boy
manoeuvre de lecompte
TGA : venous Switch (Mustard /Senning correction)
SVC - LA
IVC - LA
PV - RA
Coronaries anomalies
Ebstein’s anomaly
apical displacement of the septal and posterior tricuspid valve leaflets
Ebstein’s anomaly
Double Discordance (CCTGA)
« RV » EF 50 % EDVI 217 ml/m²
Systemic RV
LV or RV?
single ventricle – 13y girl
Fontan procedure
single ventricle – 13y girl
Double outlet RV – 12y boy • RV is enlarged with huge VSD in
the inlet segment
• Double outlet right ventricle
• Mitral valve regurgitation
• A-V concordance
• No overriding or straddling of TV and MV
Qp/Qs 1,7
80y, woman - CAD screnning
Qp/Qs 2,7
dd ARDV normal systolic fx!!
20y, man, athlete RV dilatation, ESV
Qp/Qs 2,5
85y woman: RV dysfunction
65 y, man - Aortic stenosis
Conclusion
• CMR for GUCH follow up
Conclusion
• CMR for GUCH follow up
• Collaboration pediatric cardiologist and remnographer
Conclusion
• CMR for GUCH follow up
• Collaboration pediatric cardiologist and remnographer
• Anamnesis, surgical history
Conclusion
• CMR for GUCH follow up
• Collaboration pediatric cardiologist and remnographer
• Anamnesis, surgical history
• Systematic analysis of anatomy
Conclusion
• CMR for GUCH follow up
• Collaboration pediatric cardiologist and remnographer
• Anamnesis, surgical history
• Systematic analysis of anatomy
• Think about!
I CMR
