Slide 1

JAN BOGAERT Teaching Cases CLINICAL CARDIAC MRI SECOND EDITION Cases 61-80

Slide 2

ASD type secundum Univentricular heart Membranous VSD Muscular VSD Uncorrected TOF TOF + Waterston Anastomosis Corrected TOF with severe PR TOF+ severe subvalvular PS Decompensated TOF TOF with MAPCAs TOF + myocardial infarction LV dysfunction in TOF patient Ebsteins Disease Complex CHD Partial non-expansion or agenesis RV Giant aneurysm of sinus of Valsalva Fontan circulation Persistent ductus arteriosus Situs inversus totalis Incidental finding of CHD in patient with severe AS Scimitar vein

Slide 3

Abbreviations Ao, aorta / AR, aortic regurgitation / AS, aortic stenosis / ARVC-D, arrhythmogenic RV cardiomyopathy-dysplasia / ASD, atrial septal defect / AV, aortic valve / CAD, coronary artery disease / CMP, cardiomyopathy / CT, computed tomography / DCM, dilated cardiomyopathy / DILV, double inlet LV / EDV, end-diastolic volume / EF, ejection fraction / ESV, end-systolic volume / HCM, hypertrophic cardiomyopathy / ICD, intracardiac device / IVC, inferior vena cava / LA, left atrium / LV, left ventricle / LVM, left ventricular mass / LVNC, left ventricular non-compaction / LVOT, LV outflow tract / MAPCA, major aortic pulmonary collateral artery / MI, myocardial infarction / MPI, myocardial perfusion imaging / MR, mitral regurgitation / MV, mitral valve / MVL, mitral valve leaflet / PAHT, pulmonary arterial hypertension / PAPVR partial anomalous pulmonary venous return / PC- MRI, phase-contrast MRI / PCI, percutaneous coronary intervention / PR, pulmonary regurgitation / PS, pulmonary stenosis / PT, pulmonary trunk / PV, pulmonary valve / RA, right atrium / RFA, radiofrequency ablation / RV, right ventricle / RVOT, RV outflow tract / STEMI, ST-elevation MI / SVC, superior vena cava / TGA, transposition of the great arteries / TOF, tetralogy of Fallot / TR, tricuspid regurgitation / US, ultrasound / UVH, univentricular heart / VSD, ventricular septal defect / WT, wall thickness.

Slide 4

ASD Type Secundum 33-year-old woman with large type secundum ASD. Baseline cardiac MRI (left panel): LV EDV 96 ml SV 54 ml EF 56% / RV EDV 319 ml SV 159 ml EF 49%. Flow Ao 57 ml/hb PT 178 ml/hb Qp/Qs > 3. Dilated PT. Large, multifenestrated ASD. Normal entrance of pulmonary veins in LA MRI post ASD closure(right panel): LV EDV 127 ml EF 75% / RV EDV 143 ml EF 61%. Flow Ao 86 ml/hb PT 91 ml/hb. Qp/Qs = 1. See similar case Fig. 13 Congenital Heart Disease

Slide 5

Univentricular Heart 39-year-old man with complex CHD: congenitally corrected TGA (L-TGA), double inlet left ventricle (DILV), VSD, non-restrictive univentricular heart (UVH), dominant LV, rudimentary RV, and PS. Cardiac MRI : UVH EDV 405 ml EF 59%, globally well preserved myocardial contractility. PS (PC-MRI: 56 mm Hg) with post-stenotic dilation of PT (57 mm).

Slide 6

Membranous VSD 20-year-old woman with known membranous VSD, cardiac US shows gradient of 80mm Hg over VSD, no evidence of PAHT. Cardiac MRI: LV EDV 197 ml SV 129 ml EF 66% / RV EDV 105 ml SV 65 ml EF 60%. PC-MRI, Ao flow 57 ml/hb PT 135 ml/hb, yielding Qp/Qs of 2.36. Presence of membranous VSD with systolic jet (arrow, right panel). The RV is non-dilated and non-hypertrophied while the LV EDV and SV are more than two-fold the EDV and SV of RV. See also Fig.16 and Table 5 Congenital Heart Disease

Slide 7

Muscular VSD 73-year-old woman with history of mitral and tricuspid valve repair for MR / TR, persistence and gradual increase of dyspnea. Cardiac MRI: LV EDV 180 ml EF 57% - SV 103 ml / RV EDV 245 ml EF 22% - SV 54 ml. LR-shunt, flow ascending aorta 53 ml/hb pulmonary trunk 112 ml/hb (Qp/Qs = 2). Thinned appearance of apical ventricular septum with multi-fenestrated VSD (see dark-flow zones PC-MRI, right middle panel). Though the etiology of the muscular VSD in this elderly woman is uncertain, a myocardial infarction is the most likely hypothesis.

Slide 8

Uncorrected Tetralogy of Fallot 38-year-old woman with tetralogy of Fallot, pulmonary valve atresia, VSD, right aortic arch, major aortic pulmonary collateral arteries (MAPCAs). LV EDV 260 ml EF 58% / RV EDV 290 ml EF 44% - moderate to severe hypertrophy. Big outlet VSD (22mm). Overriding aorta. Dilated ascending aorta (51x48 mm) with moderate AR (regurgitant fraction 10%) severe TR.

Slide 9

Tetralogy of Fallot + Waterston Anastomosis 41-year-old woman known with Waterston anastomosis for tetralogy of Fallot, presenting with Eisenmenger syndrome, right heart failure, cardiac cirrhosis and chronic renal insufficiency. Cardiac MRI: LV EDV 128 ml EF 47% / RV EDV 260 ml EF 47% - severe RV hypertrophy. Large membranous VSD. Hypoplastic RVOT hypoplastic PV. Severe TR (PC-MRI: 80 ml) with RA/IVC dilatation. Atrial septal aneurysm. Dilated aortic root. Right aortic arch with mirror-image branching. Waterston anatomosis with bidirectional flow (video right panels).

Slide 10

Tetralogy of Fallot with Severe PR 22-year-old woman with surgically corrected tetralogy of Fallot (infundibular resection) send for cardiac MRI study to evaluate severe PR and RV volumes/function. LV EDV 162 ml EF 47% / RV EDV 300 ml EF 35 % - diffuse hypokinetic wall motion. Diastolic flattening of ventricular septum. Severe PR (PC-MRI, right panel: regurgitant volume 45 ml).

Slide 11

Tetralogy of Fallot with Severe Subvalvular PS 11-year-old boy with surgical repair tetralogy of Fallot (infundibular patch), Melody valve for PS, presenting with residual subvalvular PS. Cardiac MRI shows muscular subvalvular PS with minimal diameter (5.5x9.5mm), and a peak velocity of 4.5 m/s (gradient of 64 mm Hg). Severe RV hypertrophy but preserved myocardial contractility (RV EDV 123 ml EF 58%). Presence of Melody valve. Since RFA is unsuccessful, surgical resection of subvalvular PS performed, showing a prominent muscular ring in RVOT.

Slide 12

Decompensated Tetralogy of Fallot 45-year-old man with surgically corrected TOF, admitted with diastolic heart failure. Cardiac MRI shows moderately dilated RV (EDV 240 ml) with preserved systolic function (EF 66%), presence of moderate PR (19%), TI and mild PS. Focally thickened pericardium with some compression of inferolaterobasal wall. Cardiac CT shows heavily calcified pulmonalis homograft, focally thickened and calcified pericardium constricting RV. Surgery: redo pulmonalis homograft, TV plasty and pericardiectomy.

Slide 13

Tetralogy of Fallot + MAPCAs 40-year-old man with uncorrected TOF, PV atresia, subaortic VSD, MAPCAs, pulmonary hypertension, AR (3-4/4) treated with AV prosthesis, dilated ascending Ao, right aortic arch. NYHA III. LV EDV 357 ml EF 41% / RV EDV 252 ml EF 42% - severe RV hypertrophy. AR 27%, dilated aortic root 56 mm, complete agenesis RVOT - PV. Multiple MAPCAs originating from aortic arch and descending Ao supplying pulmonary circulation. CT (performed after AV replacement) nicely shows the number, origin, and course of the MAPCAs. See similar case Fig. 22 Congenital Heart Disease

Slide 14

Tetralogy of Fallot + Myocardial Infarction (1) 43-year-old man with corrected TOF presenting extensive inferior MI (late presentation), secondary MR and heart failure. LV EDV 280 ml EF 32%, thinning of entire LV inferior wall with a-dyskinetic wall motion. RV EDV 279 ml, EF 32%, moderate to severe hypokinesia. Severe MR and TR, small PR. Small LV apical thrombus.

Slide 15

Tetralogy of Fallot + Myocardial Infarction (2) Late Gd imaging shows almost complete transmural enhancement of LV inferior wall (segments 3,4,9,10,15)(arrows, right panels). Enhancement of anterolateral LV papillary muscle (arrowhead right panel) Small apical thrombus (arrowhead, middle panels). Severe MR and TR (arrows, left panel).

Slide 16

LV Dysfunction in Fallot Patient (1) 35-year-old man with history of surgical repair for tetralogy of Fallot, presenting with LV dysfunction, and presence of emboli in LCx and distal LAD coronary artery. Cardiac MRI: LV EDV 198 ml - EF: 37% - hypokinesia in ventricular septum and in (thinned) lateral wall. RV EDV 185 ml EF 47%. Moderate PS (36 mm Hg) and PR (34%). Late Gd imaging shows extensive transmural enhancement in anteroseptal wall (segments 2,89,14,17) and lateral wall (segments 5,6,11,12,16) with presence of microvascular obstruction. Enhancement of the RV anterior free wall. Findings of acute extensive infarction in two perfusion territories. No etiology for the emboli found.

Slide 17

Follow up MRI study, 4 months after, shows adverse RV and LV remodeling, RV EDV 234 ml EF 38% / LV EDV 239 ml EF 30%. Thinning of the infarcted areas with disappearance of microvascular dysfunction. In particular the LV lateral wall has become dyskinetic (whereas initially severely hypokinetic). LV Dysfunction in Fallot Patient (2)

Slide 18

Ebsteins Disease 4-year-old girl with Ebsteins disease. Apical implantation (41 mm) of septal leaflet of tricuspid valve with atrialization of RV. Secondary TR (2/4). Small perimembranous VSD. Small PFO. See similar case Fig. 35 Valvular Heart Disease

Slide 19

Complex CHD 54-year-old man presenting with complex congenital heart disease (CHD): situs solitus, levocardia, double discordance, VSD with bidirectional shunt, Ebstein malformation. Severe pulmonary hypertension. LV EDV 391 ml EF 50% - diffuse hypokinesia / RV EDV 491 ml EF 26% - diffuse hypokinesia. Severe TR. Massive dilatation of both atria.

Slide 20

Partial Non-Expansion or Agenesis of RV 32-year-old woman with Ebsteins disease and Glenn surgery (SVC to right PA, RV to left PA). Closure of ASD type II. Impression of excluded RV apex by muscular structure. Cardiac MRI shows severe hypertrophy of trabecular/muscular structures in RV apex (arrow) with almost complete obliteration of the cavity. Presence of a notch between basal and apical part of RV (arrowhead). Severe dilatation of RV, IVC and coronary sinus. Important TR (28 ml/heart beat). Surgical repair of displastic tricuspid valve and resection of muscular structures in RV apex.

Slide 21

Giant Aneurysm of Sinus of Valsalva 57-year-old woman with incidental finding of annulo-aortic ectasia (max. 56 mm). The patient mentions a history of car accident (eight years before). Cardiac MRI shows giant aneurysm of sinus of Valsalva orginating from non- coronary cusp (diameter 58x34 mm) prolabating in RA. No evidence of rupture of aneurysm. Moderate AI. Surgical resection of aneurysm and aortic valve replacement.

Slide 22

Fontan Circulation 40-year-old man with history of large inlet VSD, hypoplastic bipartite RV without apex, TGA, PS, TR. Right aortic arch. Fontan total cavo-pulmonary connection (TCPC) at age 19 years. Cardiac MRI EDV 127 ml SV 72 ml - EF 57% (combined RV/LV). Dilated aortic root, flow ascending aorta: 68 ml/hb. Inferior cavopulmonary connection (arrow right middle panel): flow 44 ml/hb. Superior connection (arrow, right panel): flow 26 ml/hb. Flow right PA 41 ml/hb left PA 34 ml/hb.

Slide 23

Patent Ductus Arteriosus 39-year-old man with unsuccessful closure of patent ductus arteriosus (age 12 years), currently presenting with Eisenmenger syndrome (Hct 70%, sat. 60%). NYHA class III. Small intramuscular VSD. Cardiac MRI: LV EDV 116 ml EF 32% / RV EDV 236 ml EF 27% - severe RV wall hypertrophy. Inversion of ventricular septum with paradoxical motion. Severe TR (39%), moderate MR (26%) and AR (18%). Dilated RA. Patent ductus arterious (22 mm) with bi-directional shunt. Dilatation of proximal descending aorta (40 mm). Dilated pulmonary trunk.

Slide 24

Situs Inversus Totalis 37-year-old woman with dextracardia and situs inversus totalis. Cardiac apex oriented to right side. Right-sided position of morphologic LV. Normal atrioventricular and ventriculoarterial concordance. Right-sided aortic arch. Normal ventricular volumes and systolic function. Left-sided position of liver, right-sided position of spleen.

Slide 25

Incidental Finding of CHD in patient with severe AS 50-year-old woman presenting with dyspnea (NYHA II-III), history of aortic coarctation (coarctectomia) and progressive AV stenosis. Cardiac US shows severely degenerated AV valve with PIG of 70 mm Hg and calculated AV orifice of 0.5 cm2. Cardiac MRI: dilated RV (EDV 239 ml SV 144 ml EF 60%) / LV EDV 75 ml SV 43 ml EF 57%. Intact appearance of atrial septum but partial abnormal pulmonary venous drainage of right upper/middle lobe vein to SVC. Ascending aorta: 42 ml/hb pulmonary trunk: 139 ml/hb (Qp/Qs: 3.25). Bicuspid aortic valve with peak flow of 3.2 m/s (gradient 41 mm Hg). MV prolapse. Persisting left SVC

Slide 26

Scimitar Vein 9-year-old boy with Scimitar syndrome, PAPVR, dextrocardia, situs solitus. Large vein (Scimitar vein, arrowheads left panel) draining right lower lobe in IVC. Surgical rerouting of abnormal vein toward LA.