Myocardial non compaction in at least two members of 4 families: cardio-rm and echocardiography.
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1 Myocardial non compaction in at least two members of 4 families: cardio-rm and echocardiography. Poster No.: C-0270 Congress: ECR 2011 Type: Educational Exhibit Authors: I. Sansoni, M. Banci, F. Papetti, I. Di Giampietro, C. L Piccolo, B. Beomonte Zobel ; Rome/IT, Roma/IT Keywords: Diagnostic procedure, MR, Cardiac DOI: /ecr2011/C-0270 Any information contained in this pdf file is automatically generated from digital material submitted to EPOS by third parties in the form of scientific presentations. References to any names, marks, products, or services of third parties or hypertext links to thirdparty sites or information are provided solely as a convenience to you and do not in any way constitute or imply ECR's endorsement, sponsorship or recommendation of the third party, information, product or service. ECR is not responsible for the content of these pages and does not make any representations regarding the content or accuracy of material in this file. As per copyright regulations, any unauthorised use of the material or parts thereof as well as commercial reproduction or multiple distribution by any traditional or electronically based reproduction/publication method ist strictly prohibited. You agree to defend, indemnify, and hold ECR harmless from and against any and all claims, damages, costs, and expenses, including attorneys' fees, arising from or related to your use of these pages. Please note: Links to movies, ppt slideshows and any other multimedia files are not available in the pdf version of presentations. Page 1 of 43
2 Learning objectives To show morphologic, kinetic and perfusion findings in MR of related patients with myocardial non compaction in 4 families. Page 2 of 43
3 Background Myocardial non compaction is a condition that is now being defined and is more frequent than expected, as in the past it has often been misunderstood because of inadequate diagnostic instruments. The development of echocardiography and highly performing MR sequences allow an accurate study of this condition. Infact MR allows is now able to define: - morphology: volume of cardiac chambers; - cardiac function: total/sectoral contractility - perfusion: in condition of rest and stress - late enhancement: necrosis/fibrosis areas. Echocardiographics criteria for Non-compacted myocardium are: - Absence of coexistent cardiac anomalies - Wall left ventricle segmental thickening composed by two layers; uncompacted/ compacted myocardium ratio >2:1 in endsystole with wall myocardium thickening; - Disease main localization in middle-side apical and middle-lower regions of left ventricle; - In color doppler examination presence of blood flow through deep recesses normally perfused. Some authors proposed different criteria for MR diagnosis of non-compacted myocardium; Petersen suggested as the main criteria segmental thickening of left ventricle wall composed by two layers; trabeculae/compacted layer ratio > 2,3 in enddiastole; main localization of trabeculae in distal segments, from anterior wall in clockwise manner. Jacquier instead proposed other criteria: left ventricle % of trabeculation 3 times higher in NCM (32 +/- 10%) than DCM (11 +/- 4%), HCM (12 +/- 4%) and healthy patients (12 +/- 5%); trabeculation mass >20% than LV total mass predictive of non-compacted myocardium (sensitivity 93,7% e specificity 93,7%). Pujadas took into consideration diastolic and systolic disfunction as diagnostic criteria. Page 3 of 43
4 Other authors considered also the possibility to evaluate the relative hypoperfusion and myocardic fibrosis. Considering all these premises, our image analisys includes the following diagnostic criteria: - Left ventricle wall segmental thickening composed by two layers; - trabeculation/compacted layer>2,3 in end-diastole. - Evaluation of hypoperfusion - extension of myocardic fibrosis - main localization in distal segments, from anterior wall in clockwise. (Accessory:) (- LV % of trabeculation 3 times higher in MNC) (- trabeculation mass>20% of whole LV mass) In conclusion, comparing echocardiography and MR examinations in terms of advantages and disadvantages, we evidence that: - Echocardiography has low cost, is more accessible, doesn't use ionizing radiation, but is operator-dependant, sometimes not practicable because of inadequate acoustic window, it performes a geometric calculation of FE. - On the other hand, MR also doesn't use ionizing radiation, allows perfusion and late enhancement evaluation, is highly feasible and is reliable in quantification of FEV, provides panoramic evaluation and contrast resolution, but is expensive, has long duration of examination, and sometimes is controindicated (PMK, calustrophobia,...). Page 4 of 43
5 Images for this section: Fig. 0: LV non compaction plus marked hypertrabeculation of right ventricle. Page 5 of 43
6 Fig. 0: T2-weighted fat-saturation, black-blood. Page 6 of 43
7 Fig. 0 Page 7 of 43
8 Fig. 0: Ratio uncompacted/compacted layer in telediastole. Page 8 of 43
9 Imaging findings OR Procedure details We studied 55 patients with suspected myocardial non compaction, among whose we found 4 families with at least 1 parent and 1 son affected by this condition, mainly located in lateral walls and medium-distal tracts of left ventricle; most of those patients showed associated contractile dysfunction of several type and grade; not always the entity of non compaction is directly related to the entity of contractile dysfunction. Right ventricle is often site of myocardial hypertrabeculation, only partially associated to kinetic alterations. We are going to present images from two/four families observed: - Family 1: mother, son and doughter - Family 2: father and two sons Page 9 of 43
10 Images for this section: Fig. 0: Family 1 - Mother HASTE black blood Page 10 of 43
11 Fig. 0: Family 1 - Mother T2-weighted fat-saturation black blood Fig. 0: Family 1 - Mother HASTE black blood Short Axis Page 11 of 43
12 Fig. 0: Family 1 - Mother True-FISP post-gadolinium Fig. 10: Family 1 - Daughter Cine True-FISP Page 12 of 43
13 Fig. 9: Family 1 - Daughter Cine True-FISP Page 13 of 43
14 Fig. 8: Family 1 - Daughter Cine True-FISP Page 14 of 43
15 Fig. 0: Family 1 - Daughter HASTE black blood Page 15 of 43
16 Fig. 0: Family 1 - Daughter HASTE black blood Fig. 0: Family 1 - Daughter True-FISP post gadolinium Page 16 of 43
17 Fig. 7: Family 1 - Mother Cine True-FISP Page 17 of 43
18 Fig. 5: Family 1 - Mother Cine True-FISP Page 18 of 43
19 Fig. 6: Family 1 - Mother Cine True-FISP Page 19 of 43
20 Fig. 0: Family 1 - Son HASTE black blood Page 20 of 43
21 Fig. 0: Family 1 - Son HASTE black blood Page 21 of 43
22 Fig. 0: Family 1 - Son T2-weighted fat-saturation black blood Page 22 of 43
23 Fig. 3: Family 1 - Son Cine True-FISP Page 23 of 43
24 Fig. 4: Family 1 - Son Cine True-FISP Page 24 of 43
25 Fig. 0: Family 2 - Father HASTE black blood Page 25 of 43
26 Fig. 0: Family 2 - Father T2-weighted fat-saturation black blood Page 26 of 43
27 Fig. 0: Family 2 - Father True-FISP Page 27 of 43
28 Fig. 0: Family 2 - Father True-FISP Short axis Page 28 of 43
29 Fig. 0: Family 2 - Father True-FISP Page 29 of 43
30 Fig. 1: Family 2 - Father Cine True-FISP Page 30 of 43
31 Fig. 2: Family 2 - Father Cine True-FISP Page 31 of 43
32 Fig. 0: Family 2 - Son 1 HASTE black blood Page 32 of 43
33 Fig. 0: Family 2 - Son 1 True-FISP Page 33 of 43
34 Fig. 0: Family 2 - Son 1 True-FISP Page 34 of 43
35 Fig. 0: Family 2 - Son 1 True-FISP Short Axis Page 35 of 43
36 Fig. 0: Family 2 - Son 2 True-FISP Page 36 of 43
37 Fig. 0: Family 2 - Son 2 True-FISP Page 37 of 43
38 Fig. 0: Family 2 - Son 2 True-FISP Page 38 of 43
39 Fig. 0: Family 2 - Son 2 HASTE black blood Page 39 of 43
40 Fig. 0: Family 2 - Son 2 True-FISP Page 40 of 43
41 Conclusion Echocardiography and MR are indispensable techniques to individuate myocardial non compaction, which is an often misdiagnosed condition even nowadays and whose early diagnosis allows to attempt to protect cardiac functional reservoir. Page 41 of 43
42 Personal Information Page 42 of 43
43 References -Noncompaction Syndrome of the Myocardium: Pathophysiology and Imaging Pearls. Zenooz NA, Zahka KG, Siwik ES, Gilkeson RC. J Thorac Imaging Mar 8. -Left ventricular noncompaction. Sarma RJ, Chana A, Elkayam U. Prog Cardiovasc Dis Jan-Feb;52(4): Review.PMID: [Lack of MR late-enhancement in left ventricular non-compaction in infants and young children.] Marín Rodríguez C, Ossaba Vélez S, Maroto Álvaro E, Sánchez Alegre ML. Radiologia Dec 15. Page 43 of 43
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