Teaching Cases
JAN BOGAERT
Cases 61-80
CLINICAL CARDIAC MRI
SECOND EDITION
Cases 61-80
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ASD type secundum
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TOF + myocardial infarction
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Univentricular heart
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LV dysfunction in TOF patient
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Membranous VSD
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Ebstein’s Disease
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Complex CHD
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Partial non-expansion or agenesis RV
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Muscular VSD
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Uncorrected TOF
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Giant aneurysm of sinus of Valsalva
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TOF + Waterston Anastomosis
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Fontan circulation
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Corrected TOF with severe PR
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Persistent ductus arteriosus
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TOF+ severe subvalvular PS
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Situs inversus totalis
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Incidental finding of CHD in patient
with severe AS
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Scimitar vein
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Decompensated TOF
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TOF with MAPCA’s
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 / PCMRI, 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.
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
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).
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
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.
Uncorrected Tetralogy of Fallot
 38-year-old woman with tetralogy of Fallot, pulmonary valve atresia, VSD,
right aortic arch, major aortic pulmonary collateral arteries (MAPCA’s).
 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.
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).
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).
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.
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.
Tetralogy of Fallot + MAPCA’s
 40-year-old man with uncorrected TOF, PV atresia, subaortic VSD, MAPCA’s,
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
MAPCA’s originating from aortic arch and descending Ao supplying pulmonary
circulation.
 CT (performed after AV replacement) nicely shows the number, origin, and
course of the MAPCA’s.
See similar case Fig. 22 Congenital Heart Disease
Tetralogy of Fallot + Myocardial Infarction (1)
 43-year-old man with corrected TOF presenting extensive inferior MI (late
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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.
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).
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.
LV Dysfunction in Fallot Patient (2)
 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).
Ebstein’s Disease
 4-year-old girl with Ebstein’s 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
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.
Partial Non-Expansion or Agenesis of RV
 32-year-old woman with Ebstein’s 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.
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 noncoronary cusp (diameter 58x34 mm) prolabating in RA. No evidence of rupture
of aneurysm. Moderate AI.
 Surgical resection of aneurysm and aortic valve replacement.
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.
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.
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.
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
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.