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

http://www.google.fr/url?sa=i&rct=j&q=&esrc=s&source=images&cd=&cad=rja&uact=8&ved=2ahUKEwi-7qbW7uPeAhVQXRoKHX8fBk4QjRx6BAgBEAU&url=http://iltascalibro.blogspot.com/2015/04/faccia-faccia-con-mr-emoticon.html&psig=AOvVaw3ESJ0yMcY_3tnNoqigYg23&ust=1542833995690817

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