CMR for Congenital Heart Disease

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1 CMR for Congenital Heart Disease * Second-line tool after TTE * Strengths of CMR : tissue characterisation, comprehensive access and coverage, relatively accurate measurements of biventricular function/ volume flow, non-radiation exposure * Follow-up CHD case Fontan operation, repairs of TOF/ aortic coarctation/ TGA/ complex congenital lesions

2 Sequential Segmental Analysis * Atrial arrangement : situs solitus, situs inversus, isomerism * Atrioventricular connections concordant/ discordant * Ventriculo-arterial connections concordant/ discordant

3 TIPS : Differentiation of Anatomy of RV & LV RV * Presence of a moderator band * Free wall and apical regions of the RV are normally extensively trabeculated, with relatively little compact myocardium. * Atrioventricular valve TV (apical displacement of septal insertion) LV * Less trabeculated and smooth cavity esp. septum with more compact myocardium * Atrioventricular valve - MV

4 Congenitally Corrected TGA (CCTGA)

5 Quiz 5 * A 6 y.o. Thai boy * Known congenital defects with mild hypoxemia * Fc I * TTE : normal LV/ RV function, positive saline agitation test (suspected right to left shunt) No ASD, PFO, VSD detected v Request for CMR

6

7 Persistent Left SVC LSVC LSVC LA

8 Summary of Case 5 * Persistent of left SVC directly emptying into LA chamber (absence of unroofed CS) * Bridging vein connecting from right SVC to left SVC (diameter 9-10 mm) * No evidence of shunts, Coronary sinus is not dilated and emptying into RA chamber * LV normal size, LVEF 44% * RV normal size, RVEF 51%

9 Quiz 6 * A 20 y.o. Thai female * History of TOF s/p total corrective surgery at age 7 * Request for CMR to follow up after surgery

10 Q flow Ao Q flow PA

11 Summary of Case 6 * RV - Severe RVE, EDV386 cc, EDVI 297 cc/m2, ESV 180 cc, ESVI 28 cc/m2; RVEF 53% normal wall thickness * LV LVEF 60%, normal size * Valve severe free PR (PR RF 72%) resulting from PV resection, mild TR, * Qp/Qs = 1 * Right-sided aortic arch

12 Aim of CMR follow up in TOF (post total correction) * Assess RV volume : RV dysfunction, RVOT aneurysm * Assess PV function : PR severity*, RVOT diameter* * Recognition of other complication : VSD, TR, aortic root dilatation, ARdue to VSD repair, stenosis of any RV pulmonary artery conduit, and possible anomalous origin of the coronary arteries (in 5% 12% of patients with TOF)

13 Quiz 7 * A 32 y.o Thai female * History of RV hypoplasia (single ventricle) s/p classic Fontan operation during childhood. * She had progressive DOE and more degree of central cyanosis. * Request for CMR to follow up after surgery

14 Fontan operation - Atriopulmonary (Fontan or modifications) anastomoses or total cavopulmonary connections. - Systemic venous return is channeled directly into the pulmonary arteries, by means of an intraatrial tunnel or extracardiac conduit.

15 Cine

16 Patch leakage (right to left shunt) TV MV RA VSD

17 Patched RVOT & hypoplasia of PV Large VSD (opening to rudimentary hypoplastic RV)

18 IVC RA - MPA connection Patch leakage RA MPA

19 SVC-RPA connection

20 Summary of Case 7 * There was a small defect (6 mm) at patch between RA and RV causing right to left shunt via large VSD * No thrombosis or stenosis of lateral tunnel along RA to MPA * SVC was surgically connected to RPA * RV hypoplasia, TV is presence with mild TR * LV (as functional single ventricle) is moderate dilatation, normal function * Moderate RAE

21 Aim of CMR for F/U Fontan * 1. Systemic ventricular dysfunction and/or significant atrioventricular valve regurgitation * 2. Enlargement of cardiac chambers and possible compression of neighboring structures (rt. PV) * 3. Significant pulmonary artery or Fontan circuit stenosis * 4. Thrombus formation

22 Quiz 8 * A 45 y.o. Thai male * History of IE of AV * TTE : moderate AR due to perforated RCC, intracardiac shunt with dynamic RVOT obstruction * RHC : mean PAP 12 mmhg, and suspected shunt

23 Cine 2C

24 Muscular ridge inserted from upper RV sepum to mid RV free wall Mid RV obstruction

25 Subarterial type VSD; 7 mm

26 Bicuspid AV

27 Summary of Case 8 * Moderate size VSD (subarterial type) with left to right shunt * LV moderate dilatataion, LVEF 50%, diastolic & systolic D-shape * RV concentric RVH, prominent muscular ridge inserted from upper RV septum to mid RV free wall causing intracavitary RV gradient * Moderate AR; bicuspid AV (fusion of NCC-RCC) * Qp/Qs = 1

28 Strength of CMR in VSD * Associated with other extracardiac defects or more complex lesions * Determine the site, size, number of defects, degree of shunting, and ventricular function and help identify any associated valvular dysfunction * Shunt calculation : comparing flow measurements obtained in phase-contrast studies within the pulmonary trunk and ascending aorta, which reflect the pulmonary-to-systemic flow ratio (reverse in PDA)

29 ASD 4 types u Primum ASD u Secundum ASD u Sinus venosus ASD u Unroofed coronary sinus +/- persistent left SVC

30 Primum ASD (large) Sinus venosus ASD PAPVR

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