Skip to main content
Top
Published in: European Radiology 12/2012

Open Access 01-12-2012 | Cardiac

Value of cardiovascular MR in diagnosing left ventricular non-compaction cardiomyopathy and in discriminating between other cardiomyopathies

Authors: Matthias Grothoff, Milena Pachowsky, Janine Hoffmann, Maximilian Posch, Sabine Klaassen, Lukas Lehmkuhl, Matthias Gutberlet

Published in: European Radiology | Issue 12/2012

Login to get access

Abstract

Objectives

To analyse the value of cardiovascular magnetic resonance (CMR)-derived myocardial parameters to differentiate left ventricular non-compaction cardiomyopathy (LVNC) from other cardiomyopathies and controls.

Methods

We retrospectively analysed 12 patients with LVNC, 11 with dilated and 10 with hypertrophic cardiomyopathy and compared them to 24 controls. LVNC patients had to fulfil standard echocardiographic criteria as well as additional clinical and imaging criteria. Cine steady-state free precession and late gadolinium enhancement (LGE) imaging was performed. The total LV myocardial mass index (LV-MMI), compacted (LV-MMIcompacted), non-compacted (LV-MMInon-compacted), percentage LV-MMnon-compacted, ventricular volumes and function were calculated. Data were compared using analysis of variance and Dunnett’s test. Additionally, semi-quantitative segmental analyses of the occurrence of increased trabeculation were performed.

Results

Total LV-MMInon-compacted and percentage LV-MMnon-compacted were discriminators between patients with LVCN, healthy controls and those with other cardiomyopathies with cut-offs of 15 g/m2 and 25 %, respectively. Furthermore, trabeculation in basal segments and a ratio of non-compacted/compacted myocardium of ≥3:1 were criteria for LVNC. A combination of these criteria provided sensitivities and specificities of up to 100 %. None of the LVNC patients demonstrated LGE.

Conclusions

Absolute CMR quantification of the LV-MMInon-compacted or the percentage LV-MMnon-compacted and increased trabeculation in basal segments allows one to reliably diagnose LVNC and to differentiate it from other cardiomyopathies.

Key Points

Cardiac magnetic resonance imaging can reliably diagnose left ventricular non-compaction cardiomyopathy.
Differentiation of LVNC from other cardiomyopathies and normal hearts is possible.
The best diagnostic performance can be achieved if combined MRI criteria for the diagnosis are used.
Literature
1.
go back to reference Breckenridge RA, Anderson RH, Elliott PM (2007) Isolated left ventricular non-compaction: the case for abnormal myocardial development. Cardiol Young 17:124–9PubMedCrossRef Breckenridge RA, Anderson RH, Elliott PM (2007) Isolated left ventricular non-compaction: the case for abnormal myocardial development. Cardiol Young 17:124–9PubMedCrossRef
2.
go back to reference Jenni R, Oechslin E, Schneider J, Attenhofer Jost CH, Kaufmann PA (2001) Echocardiographic and pathoanatomical characteristics of isolated LV non-compaction: a step towards classification as a distinct cardiomyopathy. Heart 86:666–71PubMedCrossRef Jenni R, Oechslin E, Schneider J, Attenhofer Jost CH, Kaufmann PA (2001) Echocardiographic and pathoanatomical characteristics of isolated LV non-compaction: a step towards classification as a distinct cardiomyopathy. Heart 86:666–71PubMedCrossRef
3.
go back to reference Weiford BC, Subbarao VD, Mulhern KM (2004) Noncompaction of the ventricular myocardium. Circulation 109:2965–71PubMedCrossRef Weiford BC, Subbarao VD, Mulhern KM (2004) Noncompaction of the ventricular myocardium. Circulation 109:2965–71PubMedCrossRef
4.
go back to reference Angelini A, Melacini P, Barbero F, Thiene G (1999) Evolutionary persistence of spongy myocardium in humans. Circulation 99:2475PubMedCrossRef Angelini A, Melacini P, Barbero F, Thiene G (1999) Evolutionary persistence of spongy myocardium in humans. Circulation 99:2475PubMedCrossRef
5.
go back to reference Chin TK, Perloff JK, Williams RG, Jue K, Mohrmann R (1990) Isolated noncompaction of LV myocardium. A study of eight cases. Circulation 82:507–13PubMedCrossRef Chin TK, Perloff JK, Williams RG, Jue K, Mohrmann R (1990) Isolated noncompaction of LV myocardium. A study of eight cases. Circulation 82:507–13PubMedCrossRef
6.
go back to reference Ichida F, Tsubata S, Bowles KR et al (2001) Novel gene mutations in patients with LV noncompaction or Barth syndrome. Circulation 103:1256–63PubMedCrossRef Ichida F, Tsubata S, Bowles KR et al (2001) Novel gene mutations in patients with LV noncompaction or Barth syndrome. Circulation 103:1256–63PubMedCrossRef
7.
go back to reference Klaassen S, Probst S, Oechslin E et al (2008) Mutations in sarcomere protein genes in LV noncompaction. Circulation 117:2893–901PubMedCrossRef Klaassen S, Probst S, Oechslin E et al (2008) Mutations in sarcomere protein genes in LV noncompaction. Circulation 117:2893–901PubMedCrossRef
8.
go back to reference Sasse-Klaassen S, Gerull B, Oechslin E, Jenni R, Thierfelder L (2003) Isolated noncompaction of the LV myocardium in the adult is an autosomal dominant disorder in the majority of patients. Am J Med Genet A 119A:162–7PubMedCrossRef Sasse-Klaassen S, Gerull B, Oechslin E, Jenni R, Thierfelder L (2003) Isolated noncompaction of the LV myocardium in the adult is an autosomal dominant disorder in the majority of patients. Am J Med Genet A 119A:162–7PubMedCrossRef
9.
go back to reference Sasse-Klaassen S, Probst S, Gerull B et al (2004) Novel gene locus for autosomal dominant LV noncompaction maps to chromosome 11p15. Circulation 109:2720–3PubMedCrossRef Sasse-Klaassen S, Probst S, Gerull B et al (2004) Novel gene locus for autosomal dominant LV noncompaction maps to chromosome 11p15. Circulation 109:2720–3PubMedCrossRef
10.
go back to reference Martin M, Barriales V, Corros C, Santamaria E (2011) Usefulness of cardiac magnetic resonance imaging in LV non-compaction cardiomyopathy. Eur J Heart Fail 13:177–85CrossRef Martin M, Barriales V, Corros C, Santamaria E (2011) Usefulness of cardiac magnetic resonance imaging in LV non-compaction cardiomyopathy. Eur J Heart Fail 13:177–85CrossRef
11.
go back to reference Petersen SE, Selvanayagam JB, Wiesmann F et al (2005) LV non-compaction: insights from cardiovascular magnetic resonance imaging. J Am Coll Cardiol 46:101–5PubMedCrossRef Petersen SE, Selvanayagam JB, Wiesmann F et al (2005) LV non-compaction: insights from cardiovascular magnetic resonance imaging. J Am Coll Cardiol 46:101–5PubMedCrossRef
12.
go back to reference Jacquier A, Thuny F, Jop B et al (2010) Measurement of trabeculated LV mass using cardiac magnetic resonance imaging in the diagnosis of LV non-compaction. Eur Heart J 31:1098–104PubMedCrossRef Jacquier A, Thuny F, Jop B et al (2010) Measurement of trabeculated LV mass using cardiac magnetic resonance imaging in the diagnosis of LV non-compaction. Eur Heart J 31:1098–104PubMedCrossRef
13.
go back to reference Nucifora G, Aquaro GD, Pingitore A, Masci PG, Lombardi M (2011) Myocardial fibrosis in isolated left ventricular non-compaction and its relation to disease severity. Eur J Heart Fail 13:170–6PubMedCrossRef Nucifora G, Aquaro GD, Pingitore A, Masci PG, Lombardi M (2011) Myocardial fibrosis in isolated left ventricular non-compaction and its relation to disease severity. Eur J Heart Fail 13:170–6PubMedCrossRef
14.
go back to reference Elliott P, Anderson B, Arbustini E et al (2008) Classification of the cardiomyopathies: a position statement from the European Society of Cardiology working group on myocardial and pericardial diseases. Eur Heart J 29:270–6PubMedCrossRef Elliott P, Anderson B, Arbustini E et al (2008) Classification of the cardiomyopathies: a position statement from the European Society of Cardiology working group on myocardial and pericardial diseases. Eur Heart J 29:270–6PubMedCrossRef
15.
go back to reference Posch MG, Perrot A, Geier C et al (2009) Genetic deletion of arginine 14 in phospholamban causes dilated cardiomyopathy with attenuated electrocardiographic R amplitudes. Hear Rhythm 6:480–6CrossRef Posch MG, Perrot A, Geier C et al (2009) Genetic deletion of arginine 14 in phospholamban causes dilated cardiomyopathy with attenuated electrocardiographic R amplitudes. Hear Rhythm 6:480–6CrossRef
16.
go back to reference Beerbaum P, Barth P, Kropf S et al (2009) Cardiac function by MRI in congenital heart disease: impact of consensus training on interinstitutional variance. J Magn Reson Imaging 30:956–66PubMedCrossRef Beerbaum P, Barth P, Kropf S et al (2009) Cardiac function by MRI in congenital heart disease: impact of consensus training on interinstitutional variance. J Magn Reson Imaging 30:956–66PubMedCrossRef
17.
go back to reference Grothoff M, Spors B, Abdul-Khaliq H, Gutberlet M (2008) Evaluation of postoperative pulmonary regurgitation after surgical repair of tetralogy of Fallot: comparison between Doppler echocardiography and MR velocity mapping. Pediatr Radiol 38:186–91PubMedCrossRef Grothoff M, Spors B, Abdul-Khaliq H, Gutberlet M (2008) Evaluation of postoperative pulmonary regurgitation after surgical repair of tetralogy of Fallot: comparison between Doppler echocardiography and MR velocity mapping. Pediatr Radiol 38:186–91PubMedCrossRef
18.
go back to reference van Geuns RJ, Baks T, Gronenschild EH et al (2006) Automatic quantitative LV analysis of cine MR images by using three-dimensional information for contour detection. Radiology 240:215–21PubMedCrossRef van Geuns RJ, Baks T, Gronenschild EH et al (2006) Automatic quantitative LV analysis of cine MR images by using three-dimensional information for contour detection. Radiology 240:215–21PubMedCrossRef
19.
go back to reference Udupa JK (1996) Fuzzy connectedness and object definition: theory, algorithms and applications in image segmentation. Graph Model Image Proc 58:246–61CrossRef Udupa JK (1996) Fuzzy connectedness and object definition: theory, algorithms and applications in image segmentation. Graph Model Image Proc 58:246–61CrossRef
20.
go back to reference Mosteller RD (1987) Simplified calculation of body-surface area. N Engl J Med 317:1098PubMed Mosteller RD (1987) Simplified calculation of body-surface area. N Engl J Med 317:1098PubMed
21.
go back to reference Cerqueira MD, Weissman NJ, Dilsizian V et al (2002) Standardized myocardial segmentation and nomenclature for tomographic imaging of the heart: a statement for healthcare professionals from the Cardiac Imaging Committee of the Council on Clinical Cardiology of the American Heart Association. J Nucl Cardiol 9:240–5PubMedCrossRef Cerqueira MD, Weissman NJ, Dilsizian V et al (2002) Standardized myocardial segmentation and nomenclature for tomographic imaging of the heart: a statement for healthcare professionals from the Cardiac Imaging Committee of the Council on Clinical Cardiology of the American Heart Association. J Nucl Cardiol 9:240–5PubMedCrossRef
22.
go back to reference Alfakih K, Plein S, Thiele H et al (2003) Normal human left and right ventricular dimensions for MRI as assessed by turbo gradient echo and steady-state free precession imaging sequences. J Magn Res Imaging 17:323–9CrossRef Alfakih K, Plein S, Thiele H et al (2003) Normal human left and right ventricular dimensions for MRI as assessed by turbo gradient echo and steady-state free precession imaging sequences. J Magn Res Imaging 17:323–9CrossRef
23.
go back to reference Kohli SK, Pantazis AA, Shah JS et al (2008) Diagnosis of left-ventricular non-compaction in patients with left-ventricular systolic dysfunction: time for a reappraisal of diagnostic criteria? Eur Heart J 29:89–95PubMedCrossRef Kohli SK, Pantazis AA, Shah JS et al (2008) Diagnosis of left-ventricular non-compaction in patients with left-ventricular systolic dysfunction: time for a reappraisal of diagnostic criteria? Eur Heart J 29:89–95PubMedCrossRef
24.
go back to reference Weiss F, Habermann CR, Lilje C et al (2003) MRI in the diagnosis of non-compacted ventricular myocardium (NCVM) compared to echocardiography. Rofo 175:1214–9PubMedCrossRef Weiss F, Habermann CR, Lilje C et al (2003) MRI in the diagnosis of non-compacted ventricular myocardium (NCVM) compared to echocardiography. Rofo 175:1214–9PubMedCrossRef
25.
go back to reference Probst S, Oechslin E, Schuler P et al (2011) Sarcomere gene mutations in isolated left ventricular noncompaction cardiomyopathy do not predict clinical phenotype. Circ Cardiovasc Genet 4:367–374PubMedCrossRef Probst S, Oechslin E, Schuler P et al (2011) Sarcomere gene mutations in isolated left ventricular noncompaction cardiomyopathy do not predict clinical phenotype. Circ Cardiovasc Genet 4:367–374PubMedCrossRef
26.
go back to reference Oechslin EN, Attenhofer Jost CH, Rojas JR, Kaufmann PA, Jenni R (2000) Long-term follow-up of 34 adults with isolated left ventricular noncompaction: a distinct cardiomyopathy with poor prognosis. J Am Coll Cardiol 36:493–500PubMedCrossRef Oechslin EN, Attenhofer Jost CH, Rojas JR, Kaufmann PA, Jenni R (2000) Long-term follow-up of 34 adults with isolated left ventricular noncompaction: a distinct cardiomyopathy with poor prognosis. J Am Coll Cardiol 36:493–500PubMedCrossRef
27.
go back to reference Junqueira FP, Fernandes FD, Coutinho AC, De Pontes PV, Domingues RC (2009) Case report. Isolated left ventricular myocardium non-compaction: MR imaging findings from three cases. Br J Radiol 82:e37–e41PubMedCrossRef Junqueira FP, Fernandes FD, Coutinho AC, De Pontes PV, Domingues RC (2009) Case report. Isolated left ventricular myocardium non-compaction: MR imaging findings from three cases. Br J Radiol 82:e37–e41PubMedCrossRef
28.
go back to reference Marin RC, Ossaba VS, Maroto AE, Sanchez AM (2010) Lack of MR late-enhancement in left ventricular non-compaction in infants and young children. Radiologia 52:138–43CrossRef Marin RC, Ossaba VS, Maroto AE, Sanchez AM (2010) Lack of MR late-enhancement in left ventricular non-compaction in infants and young children. Radiologia 52:138–43CrossRef
29.
go back to reference Dodd JD, Holmvang G, Hoffmann U (2007) Quantification of left ventricular noncompaction and trabecular delayed hyperenhancement with cardiac MRI: correlation with clinical severity. Am J Roentgenol 189:974–80CrossRef Dodd JD, Holmvang G, Hoffmann U (2007) Quantification of left ventricular noncompaction and trabecular delayed hyperenhancement with cardiac MRI: correlation with clinical severity. Am J Roentgenol 189:974–80CrossRef
30.
go back to reference Eitel I, Fürnau G, Walther C et al (2008) Delayed enhancement magnetic resonance imaging in isolated noncompaction of ventricular myocardium. Clin Res Cardiol 97:277–9PubMedCrossRef Eitel I, Fürnau G, Walther C et al (2008) Delayed enhancement magnetic resonance imaging in isolated noncompaction of ventricular myocardium. Clin Res Cardiol 97:277–9PubMedCrossRef
31.
go back to reference Fazio G, Novo G, Casalicchio C et al (2009) Left ventricular non-compaction cardiomyopathy in children: is segmental fibrosis the cause of tissue Doppler alterations and of EF reduction? Int J Cardiol 132:278–80PubMedCrossRef Fazio G, Novo G, Casalicchio C et al (2009) Left ventricular non-compaction cardiomyopathy in children: is segmental fibrosis the cause of tissue Doppler alterations and of EF reduction? Int J Cardiol 132:278–80PubMedCrossRef
32.
go back to reference Huber AM, Schoenberg SO, Hayes C et al (2005) Phase-sensitive inversion-recovery MR imaging in the detection of myocardial infarction. Radiology 237:854–60PubMedCrossRef Huber AM, Schoenberg SO, Hayes C et al (2005) Phase-sensitive inversion-recovery MR imaging in the detection of myocardial infarction. Radiology 237:854–60PubMedCrossRef
33.
go back to reference Kellman P, Arai AE, McVeigh ER, Aletras AH (2002) Phase-sensitive inversion recovery for detecting myocardial infarction using gadolinium-delayed hyperenhancement. Magn Reson Med 47:372–83PubMedCrossRef Kellman P, Arai AE, McVeigh ER, Aletras AH (2002) Phase-sensitive inversion recovery for detecting myocardial infarction using gadolinium-delayed hyperenhancement. Magn Reson Med 47:372–83PubMedCrossRef
34.
go back to reference Stoellberger C, Finsterer J (2004) Left ventricular hypertrabeculation/noncompaction. J Am Soc Echocardiography 17:91–100CrossRef Stoellberger C, Finsterer J (2004) Left ventricular hypertrabeculation/noncompaction. J Am Soc Echocardiography 17:91–100CrossRef
Metadata
Title
Value of cardiovascular MR in diagnosing left ventricular non-compaction cardiomyopathy and in discriminating between other cardiomyopathies
Authors
Matthias Grothoff
Milena Pachowsky
Janine Hoffmann
Maximilian Posch
Sabine Klaassen
Lukas Lehmkuhl
Matthias Gutberlet
Publication date
01-12-2012
Publisher
Springer-Verlag
Published in
European Radiology / Issue 12/2012
Print ISSN: 0938-7994
Electronic ISSN: 1432-1084
DOI
https://doi.org/10.1007/s00330-012-2554-7

Other articles of this Issue 12/2012

European Radiology 12/2012 Go to the issue