Cardiac magnetic resonance in the assessment of hypertrophic cardiomyopathy phenotypes and stages – pictorial review

Figures

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  Figure 1. A 67-year-old woman with paroxysmal atrial fibrillation and hypertrophic cardiomyopathy diagnosed by echocardiography presented with congestive heart failure, EF – 30%. Her coronary angiogram was normal. Cardiac magnetic resonance 4-chamber view: A) cine image demonstrating interventricular septum thickening, B) LGE image with suppressed blood pool and transmural enhancement of myocardium typical for amyloidosis; endomyocardial biopsy revealed SSA, genetic testing confirmed hereditary TTR amyloid; recurrent hospitalisations due to advanced heart failure during follow-up

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  Figure 2. Most common LGE distribution patterns in hypertrophic cardiomyopathy. A) Extensive midwall LGE in short-axis view (arrows). B, C) Patchy enhancement at insertion points of the RV wall into the anterior and posterior interventricular septum (arrows): B) short-axis view, C) modified 2-chamber view, D) patchy mid-wall delayed enhancement in the hypertrophied segments (arrows), E) cine image corresponding with D

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  Figure 3. SSFP cine images – examples of findings characteristic for hypertrophic cardiomyopathy (HCM) – causing mutation carriers. A) Cardiac magnetic resonance imaging (CMR) image at end – diastole, 2-chamber view, showing myocardial crypt perpendicular to the endocardial border at inferior wall (arrow). B, C, D) 40-year-old woman with a family history of HCM presented with chest pain, dyspnoea, reduced exercise tolerance, palpitations, and anaemia. CMR SSFP cine images revealed abnormal papillary muscle (arrows) and a mild hypertrophy; B) 4-chamber view, C) short-axis view, D) 2-chamber view

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  Figure 4. Distribution of hypertrophy – basal anteroseptal. A 29-year-old woman who presented with palpitations. A) Two-chamber SSFP cine cardiac magnetic resonance imaging image at end-diastole shows anterior hypertrophy (asterisk). B) Short-axis cine view demonstrates marked anteroseptal hypertrophy (asterisks). C) Corresponding LGE image showing the area of delayed enhancement (arrow)

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  Figure 5. Distribution of hypertrophy – reversed curvature with apical aneurysm. A 53-year-old man referred for cardiac magnetic resonance imaging (CMR) with a suspicion of hypertrophic cardiomyopathy (HCM), EF – 75%. A) Systolic 4-chamber SSFP cine image demonstrates a “dumbbell” configuration of the LV cavity, mid-cavity constriction by hypertrophied segments (asterisks) and a thin-walled apical aneurysm (arrow), not visible in echo. B) LGE image in diastole showing hypertrophy (asterisks) and a subtle delayed enhancement thinned apical aneurysm (arrow). A 57-year-old woman with recurrent syncope and nSVT, suspected hypertrophic cardiomyopathy: C) systolic cine 4-chamber view, demonstrating hypertrophy (asterisks), thin-walled apical aneurysm (arrow), and relatively thin lateral wall (arrow), D) systolic cine 2-chamber view, demonstrating hypertrophy (asterisks) and thin-walled apical aneurysm (arrow), E) LGE 2-chamber view showing mid-wall, patchy delayed enhancement of the hypertrophied segments (thick arrows) and no enhancement of the aneurysm (thin arrow); EF was in the adverse remodelling range – 58%. The aneurysm was not visible in the echo examination. The patient refused ICD and…

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  Figure 6. Distribution of hypertrophy – apical involvement, 4-chamber views. A, B) A 61-year-old woman previously diagnosed with hypertrophic cardiomyopathy (HCM) presented with palpitations – paroxysmal AF and nsVT, EF – 64%; cardiac magnetic resonance imaging (CMR) revealed mild hypertrophy of the apex (asterisks), please note large left atrium and B) no LGE; the patient received ICD and underwent two cryoablations because of recurrent AF and was hospitalised because of heart failure due to arrhythmia during the follow-up. C, D) A 58-year-old man referred for CMR because of suspected HCM in echo, apical hypertrophy more pronounced than in A, B. C) Cine image demonstrate spade-like appearance of LV (triangle). D) LGE image showing enhancement in hypertrophied apical segments; uneventful follow-up

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  Figure 7. Distribution of hypertrophy with RV involvement. A, B) A 39-year-old gentleman with massive biventricular hypertrophy shown in SSFP cine images, arrow indicates free right ventricular wall, EF – 73%. C, D) A 36-year-old gentleman, referred for routine cardiac magnetic resonance imaging after alcohol septal ablation, EF – 59%. C) SSFP cine end-diastolic four-chamber image demonstrates IVS (asterisk) and free RV wall (arrow) hypertrophy. D) 4-chamber LGE image demonstrates patchy areas of delayed enhancement present also in hypertrophied RV wall (arrow) and probably post-ablation septal scar

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  Figure 8. Left ventricular outflow tract obstruction: a 56-year-old gentleman diagnosed with obstructive hypertrophic cardiomyopathy referred for routine cardiac magnetic resonance imaging. A) Three-chamber diastolic cine image show anteroseptal hypertrophy and elongated anterior mitral leaflet (arrow), B) mid-systolic image show SAM and turbulent-velocity jet (arrow) within the LVOT, C) late-systolic image demonstrates posterior jet of dynamic mitral regurgitation caused by SAM; note enlarged left atrium, D) in-plane phase velocity image showing LVOTO high-velocity jet (arrow). Maximal LVOT velocity was 5.5 m/s, mitral regurgitation volume – 52 ml

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  Figure 9. Adverse remodelling. A 71-year-old man admitted after cardiac arrest caused by VT/VF, with persistent AF. He was diagnosed with hypertrophic cardiomyopathy (HCM) 1 year earlier, with an EF 55-60%, SAM, LVOTO, normal coronary arteries. Cardiac magnetic resonance images: A) short-axis SSFP cine view showing septal hypertrophy (asterisk) and lateral wall thinning (arrow), B) same level LGE image demonstrates large midwall area of delayed enhancement in hypertrophied septum (asterisk), C) SSFP cine 4-chamber view showing septal hypertrophy (asterisk) and lateral wall thinning (arrow), D) corresponding LGE image. During CMR study no LVOTO was demonstrated, EF equalled 51%, LAVI – 75 ml/m2, LAEF – 1%, LGE 12%. The patient received ICD as primary prevention, during the follow up successful shocks were delivered, due to recurrent electric storms with congestive heart failure he required frequent hospitalizations

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  Figure 10. Overt dysfunction with hypokinetic-dilated form, D-ES. A 56-year-old man presented with decompensated CHF, low EF – 32%, recent infection, suspected myocarditis. Cardiac magnetic resonance imaging revealed EF 24%, LVEDV – 263 ml, LVESV – 200 ml, LAEF – 38%, LAVI – 54 ml/m2, LGE 2%. A, C) SSFP end-diastolic cine images demonstrated IVS hypertrophy inconsistent with dilated cardiomyopathy and LA enlargement. B, D) Corresponding LGE images, small, patchy areas of delayed enhancement – arrows. The patient received ICD, during follow-up 2 successful shocks were delivered, he required frequent hospitalizations due to recurrent heart failure

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  Figure 11. Overt disfunction hypokinetic-restrictive, N-ES. A 61-year-old woman with persistent AF, nsVT, with a history of ICD explantation because of infective endocarditis presented with palpitations and dyspnoea. Cardiac magnetic resonance imaging (CMR) revealed LAEF – 39%, LAVI – 64 ml/m2, LVEDV – 159 ml, LVESV – 87 ml, EF – 45%, LGE 17%. A) SSFP cine 4-chamber view showing septal hypertrophy (asterisk) and lateral wall thinning (arrow), note biatrial enlargement. B) corresponding LGE image showing extensive, almost transmural delayed enhancement areas (arrows). C) Cine short-axis view showing again septal hypertrophy (asterisk) and lateral wall thinning (arrow). D) Corresponding LGE image showing extensive delayed enhancement areas (arrows). Her HCM risk score was 5%, she refused another ICD implantation and required recurrent hospitalizations because of heart failure during the follow-up