Teaching Cases JAN BOGAERT Cases 21-40 CLINICAL CARDIAC MRI SECOND EDITION Cases 21-40 Apical MI with mural thrombus Cardiac rhabdomyoma Iatrogenic aortic valve rupture Bronchogenic cyst Acute anteroapical MI Cardiac hemangioma (2 patients) Cor pulmonale Coarctation + AI ALCAPA + MI Idiopathic DCM Cor triatriatum Tachycardia-induced CMP Adverse post-infarction LV remodeling Double-chambered RV Idiopathic DCM with secondary VHD ASD (type II) Cardiac sarcoidosis (2 patients) Lateral MI (acute phase - follow up) Cardiac transplantation with humoral rejection Athlete’s heart 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 / 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. Apical MI with Mural Thrombus 55-year-old woman presenting with cardiac decompensation secondary to recent anterior MI. Cardiac catheterization shows occlusion of mid LAD / positive cardiac enzymes / Q-waves in II, III and aVF. Cardiac MRI shows dysfunctional, moderately dilated LV (EDV 203 ml - EF 16%) with dyskinetic, aneurysmally thinned LV apex. Late Gd imaging shows transmural enhancement in LV apex with mural thrombus. Presence of breast implant. See Fig. 38 Ischemic Heart Disease Iatrogenic Aortic Valve Rupture (1) 38-year-old woman presenting with increasing dyspnea post-partum. History of minimal invasive surgery for MV prolapse with 4/4. Referred to MRI to exclude post-partum CMP. Cardiac MRI shows severely dilated LV (EDV 392 ml – EF 31% - SV 123 ml) with diffuse severe hypokinesia. Normovolemic and normocontractile RV. The repaired MV seems to function with small MR (1/4). Diastolic regurgitant jet is visible in LVOT. Iatrogenic Aortic Valve Rupture (2) Specific views along the LVOT and flow measurements at the level of the AV show an important regurgitant jet caused by a rupture of the non-coronary cusp, causing a severe LV volume overload. PC-MRI shows a regurgitant flow of 42 ml/heart beat. At surgery a perforation of 8 mm in the non-coronary cusp is found, treated with a Carpentier Edwards bioprothesis. Though new-onset AR (2-3/4) was reported early post MV surgery on cardiac US, it took about two years to become symptomatic. Symptoms were probably boosted by pregnancy. Acute Anteroapical MI 42-year-old man admitted with ST-elevation MI treated with PCI in mid LAD. Cardiac MRI: LV EDV 235 ml - EF 46% / akinesia of anteroseptal anterior wall and LV apex. Diffuse edema in LV anteroseptal wall – apex with transmural enhancement on late Gd imaging. Presence of small apical thrombus. The extent of dysfunction correlates well with the extent of enhanced myocardium of short-axis cine images (obtained post-contrast administration). Note the small non-enhancing thrombus in the most apical short-axis slices. See Figs. 7 and 8 Ischemic Heart Disease Cor Triatriatum (1) 75-year-old man presenting with decreased exercise tolerance, dyspnea grade III. On invasive coronary angiography no significant CAD. Cardiac MRI shows normovolemic (EDV 161 ml) and normocontractile (EF 70%) LV / no abnormal findings on late Gd imaging. Incidental finding of membrane in LA separating this chamber in a dorsal and a ventral one: cor triatriatum. Cor Triatriatum (2) The thin membrane in the left atrium (arrowhead) can be well appreaciated on cardiac MRI and CT. A narrow communication (*, right panel) between the dorsal and ventral chamber can lead to increased pulmonary venous pressures. Tachycardia-induced CMP (1) 25-year-old man with continuous runs of atrial tachycardia (160/min) and tachycardia-induced cardiomyopathy. Pre-ablation cardiac MRI shows severely dilated and dysfunctional LV (EDV 504 ml – EF 9%). Image quality deteriorated by fast, irregular heart rithm. See Fig. 49 Heart Muscle Diseases Tachycardia-induced CMP (2) Follow-up cardiac MRI (9 months post-ablation) shows significant decrease in LV EDV 236 ml (vs 504 ml) and improvement in EF 49% (vs 9%). Residual region of lower contractility in LV mid inferolateral wall, corresponding to focal subendocardial enhancement. Though invasive coronary angiography revealed no abnormalities, the nature of enhancement is most suggestive of ischemic, possibly emboligenic. See Fig 19 Ischemic Heart Disease and Fig. 49 Heart Muscle Diseases Adverse Post-Infarction LV Remodeling 46-year-old man with large transmural anteroseptoapical MI (infarct size by late Gd imaging: 49 g). Cardiac MRI performed at 1week (left), 4 months (middle left), 1 year (middle right) and 5 years (right). Concomitant severe concentric LV hypertrophy (LVM 270g – WT 21 mm) at baseline. FU shows thinning of the infarct area (see LV apex), with progressive increase in LV EDV, i.e., 167 ml, 180 ml, 230 ml, 257 ml, respectively. Thinning of the non-infarcted myocardial walls at FU. Findings of adverse LV remodeling that may ultimately progess towards ischemic heart failure. See Fig. 3 Ischemic Heart Disease Double-Chambered RV 11-year-old asymptomatic boy presenting with a loud systolic precordial murmur, having a history of a spontaneously regressed RV rhabdomyoma. Presence of fibro-muscular web dividing the RV in a high-pressure inlet. chamber and a low-pressure outlet. Orifice 1.5cm2 with pressure gradient of 77 mm Hg (see right panels) Surgical resection of the web and enucleation of small residual rhabdomyoma in ventricular septum. ASD (type II) 42-year-old man presenting with severe PAHT (55 mm Hg), MRI performed to exclude CHD. Severely dilated RV (EDV 477 ml – EF 45%) with inversion and paradoxical motion of ventricular septum. Dilated RA. Presence of a large ASD. Normal entrance of pulmonary veins in LA. Severely dilated pulmonary arteries. PCMRI shows aortic flow (63 ml/hb) and pulmonary trunk (231 ml/hb), yielding Qp/Qs of 3.6. See similar case Fig. 14 Congenital Heart Disease Lateral MI (Acute Phase) 49-year-old man admitted with ST-elevation MI, occlusion of 1st lateral branch LCx. Cardiac MRI performed at day 3 post-PCI shows dysfunctional LV (EDV 167 ml - EF 22%) with akinesia of the LV lateral wall. Images post contrast administration shows transmural enhancement in LV lateral wall with presence of large no-reflow zone. See similar case Fig. 34 Ischemic Heart Disease Lateral MI (Healed Phase) Follow up study one year post-infarction. LV EDV 255 ml – EF 36%. Infarct healing with fibrotic repair and thinning of the lateral wall (strongly enhancing on late Gd imaging). Negative LV remodeling with significant increase in LV volumes and loss in EF. See similar case Fig. 34 Ischemic Heart Disease Athlete’s Heart 26-year-old man – semi-professional soccer player. LV EDV 243 ml – SV 176 ml – EF 72% / RV EDV 230 ml - SV 170 ml - EF 73 %. Septal WT 12.4 mm – lateral WT 12 mm, normal regional contractility. PC-MRI shows normal inflow physiology. Findings compatible with exercise-related (physiological) biventricular remodeling. Cardiac Rhabdomyoma One-month-old girl presenting with a large paracardiac mass on cardiac ultrasound. Cardiac mass shows a large well-defined mass along the left ventricle, clearly distinguishable from the adjacent myocardium. dynamic (cine) imaging shows that the myocardial contractility is not affected. See Fig. 13 Cardiac Masses Bronchogenic Cyst 48-year-old man presenting with repeated symptoms of malaise, retrosternal chest and fever. Incidental finding of infracarinal mass. Cardiac MRI performed for further investigation. Presence of a well-defined structure between ascending aorta and thoracic spine, compressing the SVC and LA. An intra-lesional fluid-fluid level is clearly visible. At surgery, a chocolate-colored fluid, proteinaceous fluid was found. Histology showed presence of respiratory epithelium, cartilage islands, and hair cells: findings compatible with a bronchogenic cyst. See Fig. 31 Cardiac Masses Cardiac Hemangioma (patient 2) One-day-old girl with prenatal diagnosis of pericardial effusion and RA mass. MRI performed after urgent pericardiocentesis because of cardiac tamponade. Large mass attached to RA wall extending to RA appendage. First-pass perfusion MRI shows strong enhancement similar to the enhancement of the surrounding blood, suggestive of benign highly vascular tumor (type hemangioma). Surgery shows capillary hemangioma (2g). See Fig. 17 Cardiac Masses Cardiac Hemangioma (patient 2) 50-year-old man presenting with a thickened appearance of the LV basal anteroseptal wall, maximal wall thickness of 29 mm. Though findings are compatible with asymmetric septal form of HCM, first pass perfusion MRI (panel, middle right) shows very strong, well-defined enhancement of the thickened myocardium. Invasive coronary angiography shows a network of small arterial branches supplied by the LAD coronary artery. Findings primarily suspected of cardiac hemangioma. See Fig. 18 Cardiac Masses Cor Pulmonale 29-year-old man presenting with dyspnea (NYHA grade III-IV). Severe pulmonary arterial hypertension (mPAP 59 mm Hg, PCWP 19 mm Hg, no nitric-oxide response). Obesitas (BMI: 40) and obstructive sleep apnea syndrome. Cardiac MRI: LV EDV 153 ml – EF 49% / RV EDV 216 ml – EF 35% hypertrophic and hypokinetic RV wall. Early diastolic inversion of ventricular septum. Dilated pulmonary trunk (42 mm) and arteries: findings compatible with severe cor pulmonale with RV pressure overload, diastolic and systolic dysfunction. See similar case Fig. 1 Pulmonary Hypertension Endocarditis + Aortic Coarctation (1) 19-year-old man presenting palpitations and decreased exercise tolerance since 1.5 years, admitted with fever, weight loss (-10 kg), hemorrhagic sputa. Cardiac US (TEE) shows bicuspid aortic valve with prolapse and a 1cm floating structure attached: findings compatible with endocarditis. Cardiac MRI shows dilated LV (EDV 377 ml / EF 42%), a functionally bicuspid aortic valve with an important AI (54% regurgitant fraction), a moderate pericardial effusion. The valvular vegetation can be suspected on dynamic cine images. Cardiac US: courtesy of Prof. Dr. JU Voigt Endocarditis + Aortic Coarctation (2) As an incidental finding, the patient had an aortic coarctation (suggested by cardiac US). The minimal diameter is 8 mm, presence of important collateral vessels. Patient underwent aortic valve replacement (homograft), at surgery 2 valvular vegetations were found with presence of abscesses in the subaortic curtain. The aortic coarctation was treated with a balloon dilatation and stenting. Follow up cardiac MRI 3 years later (right panel) shows normalization of LV EDV (161 ml) but persistence of LV dysfunction (EF 39%). ALCAPA with secondary MI (1) 68-year-old woman presenting with atypical angina and dyspnea (NYHA II). Invasive coronary angiography shows tortuous heavily dilated coronary arteries with a suspicion of LAD originating from pulmonary trunk. Cardiac MRI: both the RCA and LCx have a normal origin but show heavily tortous and dilated (max. diameter 10 mm). The LAD, however, arises from the pulmonary trunk and has dilated and tortuous appearance. Findings of (partial) anomalous left coronary artery arising from pulmonary artery (ALCAPA syndrome). See Fig. 16 Coronary Artery Disease – See similar case Fig. 45 Congenital Heart Disease ALCAPA with secondary MI (2) LV EDV 379 ml, EF 25% / thinning and a-to dyskinesia of the LV apical anteroseptal wall showing >50% transmural enhancement on late Gd imaging. The extensive infarcisation in the LAD perfusion territory is most likely secondary to chronic hypo-oxygenation. Conservative treatment for heart failure symptoms. See Fig. 16 Coronary Artery Disease – See similar case Fig. 45 Congenital Heart Disease Idiopathic DCM 11-year-old girl presenting with DCM. Cardiac MRI shows severely dilated and dysfunctional ventricles (LV EDV 324 ml - EF 9% / RV EDV 520 ml – EF 7%) with left-sided cardiac rotation / dilated atria and dilated IVC / mild pericardial effusion. Late Gd imaging shows diffuse strong enhancement of epicardial LV borders and diffuse RV enhancement. Myocardial biopsy shows myocardial replacement fibrosis. See Fig. 27 Heart Muscle Diseases Idiopathic DCM with secondary VHD 58-year-old man presenting with cardiac decompensation, history of idiopathic DCM with secondary MR and AR, treated with AV replacement and MV plasty. Aneurysmal dilation of ascending aorta (53 mm). Cardiac MRI shows severe dilated LV (EDV 470 ml – EF 19%), regional moderate to severe hypokinesia, moderately severe MR with LA enlargement, and right-sided pleural effusion. Late Gd imaging shows midwall septal enhancement, suggesting replacement fibrosis as seen in DCM. Cardiac Sarcoidosis (patient 1) 47-year-old man presenting with cardiomyopathy of unknown origin, cardiac US shows low EF (40%), mediastinal and hilar lymph nodes on chest CT. Cardiac MR image quality limited by arrhythmias. Real-time cine MRI shows dilated dysfunctional ventricles (LV EDV 193 ml – EF 35% / RV EDV 270 ml EF 25%), with flattening of ventricular septum. Dilated RA. Late Gd imaging shows diffuse patchy subepicardial enhancement in LV, and diffuse RV enhancement. Cardiac and skin biopsy show granulomatous inflammation (sarcoidosis). See Fig. 60 Heart Muscle Diseases Cardiac Sarcoidosis (patient 2) 42-year-old man with sarcoidosis (positive lymph node biopsy) referred for cardiac MRI because cardiac US shows borderline to mild LV hypertrophy, diastolic dysfunction with moderately dilated LA and 1st degree AV block (PR 248 ms). Cardiac MRI: EDV 184 ml – EF 58%. Thnning of mid anteroseptal wall, apical inferior wall (segments 7,8,11,15), while segment 14 is mildy thickened. Hypokinesia apicoinferior (segments 14,16). Late Gd imaging shows patchy enhancement in segments 2,3,8,9,14,15,16,17 – focal enhancement of RV anterior/inferior wall. Findings of biventricular involvement of sarcoidosis. Cardiac Transplantation with Humoral Rejection 26-year-old man with cardiac transplant (2005) for familial cmp, admitted with progressive graft deterioration and dysfunction. Cardiac MRI shows biventricular systolic dysfunction (LV EDV 216 ml – EF 37% / RV EDV 171 ml – EF 42%) with thickened walls (LVM 252 g – septum 19 mm). Moderate pericardial effusion. Strong, diffuse enhancement in LV and RV wall. Although cardiac MRI findings are compatible with cardiac amyloidosis, in the clinical context findings are suspected of diffuse fibrose/inflammation. Myocardial biopsy is negative for cellular rejection, but CD4 staining and HLA-AS are positive suggesting humoral rejection. See Fig 9 Heart Failure and Heart Transplantation