Teaching Cases JAN BOGAERT Cases 1-20 CLINICAL CARDIAC MRI SECOND EDITION Cases 1-20 Cardiac Compression by Depressed Sternum Cardiac amyloidosis (2 patients) DCM (3 patients) ARVC/D (2 patients) Endomyocardial fibrosis Inflammatory-constrictive pericarditis Cardiac angiosarcoma Caseous calcification of MVL Cardiac Chloroma (Epi)-Myocarditis Myocarditis (acute phase + FU) Obstructive HCM (2 patients) Apical HCM LVNC LVNC-Ebstein’s Disease Unclassified CMP Acute MI (Non-STEMI) Restrictive CMP Healed lateral MI 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. Cardiac Compression by Depressed Sternum 17-year-old woman Referred to MRI to evaluate severity of cardiac compression by pectus excavatum (depressed sternum). Minimal distance between sternum and spine is 33 mm. Left-sided displacement of the heart. Compression of RA and RV with restricted outward excursion of RV free wall and RA wall. Normal LV and RV volumes and rest systolic function. Cardiac Amyloidosis (patient 1) 74-year-old man referred to MRI with clinical diagnosis of HCM. Poorly functioning ventricles (LV EF 28% / RV EF 34%). Important concentric hypertrophy (LVM 205 g /max. WT 18 mm). Late Gd MRI shows strong heterogeneous myocardial enhancement (arrows), most pronounced in subepicardium and in RV. Small pericardial effusion. Biopsy: senile cardiac amyloidosis. See similar patient Fig 50 Heart Muscle Diseases Cardiac Amyloidosis (patient 2) 58-year old man presenting with pericardial effusion since CABG (1year ago), cardiac MRI requested to exclude pericardial disease / congestive heart disease. Cardiac MRI: LV EDV 116 ml – EF 43% / RV EDV 132 ml – EF 33%. Biventricular hypertrophy (septal WT 17 mm) with decreased contractility. Enlarged atria. Pericardial and bilateral pleural effusion. Diffuse myocardial enhancement on late Gd imaging (right panel), without possibility to adequate null signal of normal myocardium using a wide range of inversion times. Findings highly suspected of cardiac amyloidosis, confirmed by myocardial biopsy. See similar patient Fig 51 Heart Muscle Diseases DCM (patient 1) 65-year-old man presenting with moderately dilated and severely dysfunctional LV on cardiac US. LV EDV 549 ml – EF 28% - LVM 316 g – diffuse hypokinetic wall motion – dyskinetic motion of ventricular septum (mid/late systole) with phenomenon of apical rocking. Late Gd imaging shows increased myocardial enhancement in subepicardial inferolateral wall (arrows, right panel). MRI findings compatible with dilated cardiomyopathy. DCM (patient 2) 49-year-old woman with clinical history of idiopathic DCM. ECG shows complete left bundle branch block. Cardiac MRI: severe dilated LV (EDV353 ml – EF 26% - LVM 181 g) / normal RV (EDV 135 ml – EF 62%). Dyssynergia with severe septal dyskinesia, and ‘apical rocking’ motion. Late Gd imaging shows mild focal enhancement at the insertion of the RV inferior wall. DCM (patient 3) 63-year-old man with extreme form of DCM of unknown origin. Cardiac MRI shows extremely dilated and dysfunctional LV (EDV 877 ml – EF 12%) / RV EDV 248 ml – EF 42%). Severe dyskinesia with important dyssunergia. Important mitral regurgitation secondary to mitral valve ring dilatation. Regurgitant fraction of 45 ml. Late Gd imaging shows subepicardial enhancement in mid inferior wall, and mid-myocardially in ventricular septum. See Fig 24 Heart Muscle Diseases ARVC/D (patient 1) 51-year old man presenting with retrosternal chest pain, palpitations. ECG: sustained VT with electrical reconversion. Cardiac catheterization: no CAD – inferior hypokinesia. MRI: LV EDV 127ml – EF 64% / RV EDV 211 ml – EF 42%. Severe hypokinesia to akinesia of RV inferior wall, apex and RVOT. Late Gd MRI: focal, strong enhancement of RV free wall. Presumptive diagnosis: ARVC/D. Electrophysiology: positive late potentials. Diagnosis: ARVC/D – Treatment: ICD placement. See Figs. 32 and 35 Heart Muscle Diseases ARVC/D (patient 2) 65-year-old man. Screening for ARVC/D – son has a phakophilin gene deletion (PKP-2 gen) and HCM phenotype. Cardiac US: severe dilated RV with decreased function. MRI: LV EDV 168 ml - EF 65%/ RV EDV 278 ml – EF 32%. Diffuse moderate/severe hypokinesia to akinesia in basal and mid RV free wall and RVOT. Focal wall thinning and increased trabeculations. Late Gd MRI: diffuse RV wall enhancement. Genetic profiling: PKP-2 gen deletion. Tentative diagnosis of ARVC/D. Endomyocardial Fibrosis 78-year-old woman presenting with hypereosinophilia and findings on cardiac US of apical HCM. MRI shows important thickening of LV apex obliterating the cavity, deformation of papillary muscles. First-pass perfusion MRI shows extensive perfusion defect suspected of mural thrombus. Late Gd MRI shows focal enhancement in thickened myocardium, representing myocardial fibrosis. MRI findings of endomyocardial fibrosis with mural thrombus. See Fig. 56 Heart Muscle Diseases Inflammatory - Constrictive Pericarditis 59-year-old man with recent extensive anterior MI. First MRI study (left panel) shows thickened, irregularly delineated pericardial layers, mild pericardial effusion, and restricted RV expansion. Follow up MRI (10 days later) (right panel) shows increased pericardial fluid (note presence of diffuse intrapericardial fibrinous strands), signs of RA collapse, increased compression on RV, and increased septal shift. Pericardiectomy: heavily thickened, inflamed and constrictive pericardium. Histology: active, chronic pericardial inflammation See Fig. 13 Pericardial Disease Cardiac Angiosarcoma 54-year-old woman presenting with dyspnea, chest pain, dry cough. Chest film shows cardiomegaly with diffuse hazy-defined pulmonary nodules suspected of diffuse pulmonary metastases. Extensive mass involving the entire RA wall, extending to the RV free wall and pericardium. Presence of several nodular appearing pericardial masses. Some of them present a fluid-fluid level. Presumptive diagnosis of angiosarcoma with diffuse pulmonary metastasis. Histology: angiosarcoma See Fig. 21 Cardiac Masses Caseous Calcification of Mitral Valve Leaflet (1) 85-year-old woman admitted with retrosternal chest pain and dyspnea. Chest film: presence of dense well-defined ovaloid retrocardiac mass. Cardiac US: large, mildly hyperechogenic ovaloid mass on ventricular side of PMVL, moderately severe MR (3/4). Cardiac catheterization: severe 3vessel CAD – calcified ovaloid mass. See Fig. 44 Cardiac Masses Caseous Calcification of Mitral Valve Leaflet (2) Presence of extensive hypo-intense mass in LV infero- and laterobasal wall, compressing the surrounding myocardium, and narrowing the mitral valve area. Presence of a thin wall strongly enhancing following contrast administration. Presence of bilateral pleural effusion. Findings compatible with caseous calcification of posterior mitral valve leaflet (also called ‘liquefaction necrosis of mitral annulus calcification’). See Fig. 44 Cardiac Masses Cardiac Chloroma 56-year-old man with history of acute myeloid leukemia (AML) treated with allogenic bone marrow transplantation (2001), having a decreased exercise capacity since one year. Referred to MRI because of increasing pericardial fluid and 2nd degree AV-block (type Wenckebach). Presence of a soft-tissue like structure diffusely involving the right atrioventricular groove. Invasion of LV inferior /inferseptal wall, having a thickened appearance and exhibiting impaired contractility. Presence of an important pericardial effusion with evidence of cardiac tamponade. Cardiac biopsy: AML recurrence (‘cardiac chloroma’). See Fig. 27 Cardiac Masses (Epi)-Myocarditis 22-year-old man presenting with arrhythmias since 2 years: ventricular extrasystoles worsening during exercise, and incomplete right bundle branch block. Cardiac US shows slightly dilated and dysfunctional LV. MRI: mildly dilated and moderately dysfunctional LV (EDV 190ml - EF 39%) with regional mild to severe hypokinesia most pronounced in lateral wall. Strong subepicardial enhancement in LV anterior-lateral and inferior wall extending to adjacent epicardial fat. Focal midwall enhancement in basal part of ventricular septum. Tentative diagnosis of chronic (epi)myocarditis. See Fig. 44 Heart Muscle Diseases Myocarditis (acute phase) 41-year-old woman presenting with chest pain, irradiating to back, decreased appetite and generalized weakness since 4 days. Recent history of respiratory infection. Temporary inotropic support needed. Cardiac MRI: moderately dysfunctional LV (EF: 43%) with thickened, hypocontractile walls. Moderate pericardial effusion, without hemodynamic compromise. T2w-imaging: diffuse myocardial edema, late Gd imaging: mild subepicardial enhancement in LV lateral wall and midwall ventricular septum. Myocardial biopsy: fulminant myocarditis. See Fig. 45 Heart Muscle Diseases Myocarditis (10 months FU) Follow up MRI 10 months after the acute event, shows normalization of LV function (EF 67%) and myocardial wall thicknesses, disappearance of myocardial edema but persistence of mild myocardial enhancement and subtle pericardial effusion. Despite favorable cardiac evolution, persistence of fatigue, clinically fitting in a chronic fatigue syndrome. See Fig. 45 Heart Muscle Diseases Obstructive HCM (Patient 1) 57-year-old woman with HCM with LVOT obstruction and secondary MR. MRI shows asymmetrical septal HCM (24 mm) with LVOT narrowing. Flow acceleration during onset of systole (signal void on cine imaging in LVOT). Complete SAM of anterior MVL with secondary MR. PC-MRI in LVOT shows high velocities in early systole with complete occlusion of LVOT during mid systole. Alcoholization of 1st septal perforator with decrease LVOT gradient (82 to 18 mm Hg). See Figs. 15-16 Heart Muscle Diseases Obstructive HCM (Patient 2) 17-year-old man, familial history of obstructive HCM, NYHA II-III. Severely thickened ventricular septum (38 mm) with involvement of apical half of RV wall, narrowing of the LVOT (resting gradient of 167 mm Hg), SAM with moderately severe MR and LA dilatation. Late Gd MRI shows increased enhancement in the thickened myocardium at the level of the anterior/posterior insertion of the RV. Apical Hypertrophic Cardiomyopathy 27-year-old man. Familial history of HCM. Suspicion of apical HCM on cardiac US. Important apical thickening of LV wall (thickness: 17 mm). Apposition of apical walls in late systole with complete obliteration of LV cavity. See Fig. 12 Heart Muscle Diseases Left Ventricular Non Compaction CMP (LVNC) 26-year-old man with LVNC cardiomyopathy presenting with diastolic dysfunction. LV EDV 190 ml – EF 57% / RV EDV 192 ml – EF 51%. Presence of a prominent trabecular network allong the entire LV lateral wall and apex. Also more prominent appearance of trabeculations in RV lateral wall. Moderate MR. Restrictive inflow physiology on PC-MRI. Dilated LA with aneurysmal bulging of atrial septum. Findings of LVNC with most likely also limited RV involvement. See Fig. 38 Heart Muscle Diseases LVNC and Ebstein’s Disease 55-year-old man presenting with heart failure, and suspicion of LVNC on cardiac US. Cardiac MRI shows presence of an extensive trabecular network, most pronounced in LV apex. The trabeculations have a thickened appearanced and the wall is moderately to severely hypokinetic. Incidental finding: obliquely implanted tricuspid valve with atrialization of the RV: Ebstein’s malformation. See Fig. 39 Heart Muscle Diseases Unclassified CMP 53-year-old woman referred for MRI with history of HCM. Cardiac MRI and cardiac catherization show mixture of wall thickening and deep muscular clefts in LV, and hypertrabeculations in RV and LV. LV EDV 143 ml - LVEF 48%. The mixture of hypertrophic myocardium and non-compacted myocardium suggests existence of mixed forms of HCM and LVNC, fitting with novel cardiomyopathies such as ‘saw-tooth’ CMP. See Fig. 41 Heart Muscle Diseases Acute MI (Non-STEMI) 36-year-old man admitted with retrosternal pain, nicotine abuse. ECG: non-STEMI / troponin I : 16 mg/l. Occlusion 1st lateral branch. Cardiac MRI: LV EDV 219 ml - EF 57%, mild hypokinesia in lateral wall, T2wimaging: edema in anterolateral wall (arrows, still frames upper row), with strong transmural enhancement on late Gd MRI (arrows, lower panels). Restrictive CMP 77-year-old man presenting with cardiac failure of unknown origin, chronic atrial fibrillation, ECG: low-voltages. MRI: LV EDV 138 ml - EF 63%/ RV EDV 156 ml - EF 53% / moderate to severe TR / moderate pericardial effusion / dilated atria and IVC / PC-MRI shows restrictive inflow physiology / real-time MRI during respiration shows no inspiratory septal flattening / late Gd imaging shows no abnormal myocardial enhancement / normal T2* myocardium. Findings of (idiopathic) restrictive cardiomyopathy. Healed Lateral MI 58-year-old woman with ischemic cardiomyopathy, presenting with mild symptoms of heart failure (NYHA I-II). MRI: LV EDV 259 ml - EF 26% / RV EDV 110 ml - EF 58%. Important wall thinning of mid and lateral wall showing severe hypokinesia – akinesia / moderate hypokinesia in non-thinned segments. Late Gd imaging shows nearly complete transmural enhancement in thinned lateral wall. Although anigography yielded no coronary stenoses, MRI findings are those of a healed lateral MI with adverse LV remodeling/severe LV dysfunction.