Teaching Cases Jan Bogaert Cases 61-80 ClinicalCardiac MRI SecondEdition
Cases 61-80 • 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 MAPCA’s • TOF + myocardial infarction • LV dysfunction in TOF patient • Ebstein’s Disease • Complex CHD • Partial non-expansion or agenesis RV • Giant aneurysm of sinus of Valsalva • Fontan circulation • Persistent ductusarteriosus • Situsinversustotalis • Incidental finding of CHD in patient with severe AS • Scimitar vein
Abbreviations Ao, aorta / AR, aorticregurgitation / AS, aortic stenosis / ARVC-D, arrhythmogenic RV cardiomyopathy-dysplasia / ASD, atrial septal defect / AV, aorticvalve / CAD, coronaryarterydisease / CMP, cardiomyopathy / CT, computedtomography / DCM, dilated cardiomyopathy / DILV, double inlet LV / EDV, end-diastolic volume / EF, ejectionfraction / ESV, end-systolic volume / HCM, hypertrophic cardiomyopathy / ICD, intracardiacdevice / IVC, inferiorvenacava / LA, left atrium / LV, leftventricle / LVM, leftventricularmass / LVNC, leftventricularnon-compaction / LVOT, LV outflowtract / MAPCA, major aorticpulmonarycollateralartery / MI, myocardialinfarction / MPI, myocardialperfusionimaging / MR, mitral regurgitation / MV, mitral valve / MVL, mitral valveleaflet / PAHT, pulmonaryarterialhypertension / PAPVR – partialanomalouspulmonaryvenous return / PC-MRI, phase-contrast MRI / PCI, percutaneous coronaryintervention / PR, pulmonaryregurgitation / PS, pulmonary stenosis / PT, pulmonarytrunk / PV, pulmonaryvalve / RA, right atrium / RFA, radiofrequencyablation / RV, right ventricle / RVOT, RV outflowtract / STEMI, ST-elevation MI / SVC, superior venacava / TGA, transposition of the great arteries / TOF, tetralogy of Fallot / TR, tricuspidregurgitation / US, ultrasound / UVH, univentricularheart / VSD, ventricular septal defect / WT, wallthickness.
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%. FlowAo57 ml/hb – PT178 ml/hb – Qp/Qs > 3. DilatedPT. Large,multifenestratedASD. Normalentrance of pulmonary veins in LA • MRI post ASD closure(right panel): LV EDV 127 ml – EF 75% / RV EDV 143 ml – EF 61%. FlowAo86 ml/hb – PT91 ml/hb. Qp/Qs = 1. Seesimilar 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%, globallywellpreservedmyocardialcontractility. PS (PC-MRI: 56 mm Hg) with post-stenotic dilation of PT (57 mm).
Membranous VSD 20-year-old womanwithknownmembranous VSD, cardiac US shows gradient of 80mm Hg over VSD, noevidence of PAHT. Cardiac MRI: LV EDV 197 ml – SV 129 ml – EF 66% / RV EDV 105 ml – SV 65 ml – EF 60%. PC-MRI, Aoflow57 ml/hb – PT 135 ml/hb, yieldingQp/Qs of 2.36. Presence of membranous VSD withsystolic 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. Seealso Fig.16 and Table 5 Congenital Heart Disease
Muscular VSD 73-year-old womanwithhistory of mitral and tricuspidvalverepair for MR / TR, persistence and gradualincrease of dyspnea. Cardiac MRI: LV EDV 180 ml – EF 57% - SV 103 ml / RV EDV 245 ml – EF 22% - SV 54 ml. LR-shunt, flowascending aorta 53 ml/hb – pulmonarytrunk 112 ml/hb (Qp/Qs = ±2). Thinned appearance of apical ventricular septum with multi-fenestrated VSD (seedark-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 womanwith tetralogy of Fallot, pulmonaryvalveatresia, VSD, right aorticarch, major aorticpulmonarycollateral 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 womanknownwith Waterston anastomosis for tetralogy of Fallot, presenting with Eisenmenger syndrome, right heartfailure, cardiaccirrhosis and chronicrenalinsufficiency. Cardiac MRI: LV EDV 128 ml – EF 47% / RV EDV 260 ml – EF 47% - severe RV hypertrophy. Largemembranous VSD. Hypoplastic RVOT – hypoplastic PV. Severe TR (PC-MRI: 80 ml) with RA/IVC dilatation. Atrial septal aneurysm. Dilatedaortic root. Right aorticarchwithmirror-imagebranching. Waterston anatomosis with bidirectional flow (video right panels).
Tetralogy of FallotwithSevere PR 22-year-old womanwithsurgicallycorrected tetralogy of Fallot (infundibularresection) send for cardiac MRI study to evaluatesevere PR and RV volumes/function. LV EDV 162 ml – EF 47% / RV EDV 300 ml – EF 35 % - diffuse hypokineticwall motion. Diastolicflattening of ventricularseptum. Severe PR (PC-MRI, right panel: regurgitant volume 45 ml).
Tetralogy of FallotwithSevereSubvalvular 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 muscularsubvalvular PS with minimal diameter (5.5x9.5mm), and a peakvelocity 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 withsurgicallycorrectedTOF, admittedwithdiastolicheartfailure. Cardiac MRI shows moderatelydilated RV (EDV 240 ml) withpreservedsystolicfunction (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 withuncorrectedTOF, PV atresia, subaortic VSD, MAPCA’s, pulmonaryhypertension, AR (3-4/4) treatedwith AV prosthesis, dilatedascendingAo, right aorticarch. NYHA III. LV EDV 357 ml – EF 41% / RV EDV 252 ml – EF 42% - severe RV hypertrophy. AR 27%, dilatedaortic root 56 mm, complete agenesis RVOT - PV. Multiple MAPCA’soriginatingfromaorticarch and descendingAosupplyingpulmonarycirculation. CT (performed after AV replacement) nicely shows the number, origin, and course of the MAPCA’s. Seesimilar case Fig. 22 Congenital Heart Disease
Tetralogy of Fallot + MyocardialInfarction (1) 43-year-old man with correctedTOF 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.
Tetralogy of Fallot + MyocardialInfarction (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 FallotPatient (1) 35-year-old man withhistory of surgicalrepair for tetralogy of Fallot, presenting with LV dysfunction, and presence of emboli in LCx and distal LAD coronaryartery. Cardiac MRI: LV EDV 198 ml - EF: 37% - hypokinesia in ventricularseptum and in (thinned) lateralwall. 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 extensiveinfarction in twoperfusionterritories. No etiology for the embolifound.
LV Dysfunction in FallotPatient (2) Follow up MRI study, 4 monthsafter, shows adverse RV and LV remodeling, RV EDV 234 ml – EF 38% / LV EDV 239 ml – EF 30%. Thinning of the infarcted areas withdisappearance of microvascular dysfunction. In particular the LV lateralwall has becomedyskinetic (whereasinitiallyseverelyhypokinetic).
Ebstein’sDisease 4-year-old girlwithEbstein’sdisease. Apicalimplantation (41 mm) of septal leaflet of tricuspidvalvewithatrialization of RV. Secondary TR (2/4). Smallperimembranous VSD. Small PFO. Seesimilar case Fig. 35 Valvular Heart Disease
Complex CHD 54-year-old man presenting with complex congenitalheartdisease (CHD): situssolitus, levocardia, double discordance, VSD withbidirectional shunt, Ebsteinmalformation. Severe pulmonary hypertension. LV EDV 391 ml – EF 50% - diffuse hypokinesia / RV EDV 491 ml – EF 26% - diffuse hypokinesia. Severe TR. Massivedilatation of both atria.
PartialNon-ExpansionorAgenesis of RV 32-year-old womanwithEbstein’sdisease and Glenn surgery (SVC to right PA, RV to left PA). Closure of ASD type II. Impression of excluded RV apexbymuscularstructure. Cardiac MRI shows severehypertrophy of trabecular/muscularstructures in RV apex (arrow) withalmost complete obliteration of the cavity. Presence of a notchbetweenbasal and apical part of RV (arrowhead). Severedilatation of RV, IVC and coronary sinus. Important TR (28 ml/heart beat). Surgicalrepair of displastic tricuspidvalve and resection of muscularstructures in RV apex.
GiantAneurysm of Sinus of Valsalva 57-year-old womanwithincidentalfinding of annulo-aorticectasia (max. 56 mm). The patientmentions a history of car accident (eightyearsbefore). Cardiac MRI shows giantaneurysm of sinus of Valsalvaorginatingfromnon-coronarycusp (diameter 58x34 mm) prolabating in RA. No evidence of ruptureof aneurysm. Moderate AI. Surgicalresection of aneurysm and aorticvalvereplacement.
FontanCirculation 40-year-old man withhistory of largeinlet VSD, hypoplastic bipartite RV without apex, TGA, PS, TR. Right aorticarch. Fontantotalcavo-pulmonaryconnection (TCPC) at age 19 years. Cardiac MRI EDV 127 ml – SV 72 ml - EF 57% (combined RV/LV). Dilatedaortic root, flowascending aorta: 68 ml/hb. Inferiorcavopulmonaryconnection (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 DuctusArteriosus 39-year-old man withunsuccessfulclosure of patent ductusarteriosus (age 12 years), currently presenting with Eisenmenger syndrome (Hct 70%, sat. 60%). NYHA class III. Smallintramuscular VSD. Cardiac MRI: LV EDV 116 ml – EF 32% / RV EDV 236 ml – EF 27% - severe RV wallhypertrophy. Inversion of ventricularseptumwithparadoxical motion. Severe TR (39%), moderate MR (26%) and AR (18%). Dilated RA. Patent ductusarterious (22 mm) withbi-directional shunt. Dilatation of proximaldescending aorta (40 mm). Dilatedpulmonarytrunk.
SitusInversusTotalis 37-year-old womanwithdextracardia and situsinversustotalis. Cardiacapexoriented to right side. Right-sidedposition of morphologic LV. Normal atrioventricular and ventriculoarterial concordance. Right-sidedaorticarch. Normalventricular volumes and systolicfunction. Left-sidedposition of liver, right-sidedposition of spleen.
IncidentalFinding of CHD in patientwithsevere AS 50-year-old woman presenting withdyspnea (NYHA II-III), history of aortic coarctation (coarctectomia) and progressive AV stenosis. Cardiac US shows severelydegenerated AV valvewith 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 – pulmonarytrunk: 139 ml/hb (Qp/Qs: 3.25). Bicuspidaorticvalvewithpeakflow of 3.2 m/s (gradient 41 mm Hg). MV prolapse. Persistingleft SVC
ScimitarVein 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.