J Korean Soc Radiol.  2018 Mar;78(3):170-178. 10.3348/jksr.2018.78.3.170.

Left Ventricular Noncompaction in Adults: Imaging and Clinical Findings in 63 Patients

• Cho SJ
• Yang DH
• Kang JW
• Lim TH

• KMID: 2405719
• DOI: http://doi.org/10.3348/jksr.2018.78.3.170

Abstract

PURPOSE
To describe imaging and clinical findings for a left ventricular noncompaction (LVNC) in the adult.
MATERIALS AND METHODS
From 2000 to 2014, 63 patients were diagnosed with LVNC by echocardiography, computed tomography, and magnetic resonance imaging at our hospital. Baseline characteristics, clinical manifestations, combined cardiac or systemic anomalies, and imaging findings were reviewed. We made a comparison between the isolated and combined disease groups.
RESULTS
Among 63 patients with LVNC, 32 (51%) patients did not have combined cardiac anomalies (isolated disease group). The mean age at the initial diagnosis was higher in the isolated than in the combined disease group (54.2 years vs. 40.2 years, p < 0.001). The combined disease group presented symptoms more frequently at initial diagnosis than the isolated disease group (94% vs. 75%, p = 0.082). Heart failure symptoms were the most common adverse events (60.3% in all patients). Thromboembolic events developed in 20 patients, and were more frequent in the combined disease group than in the isolated disease group (39% vs. 26%, p = 0.279). The most common cardiac abnormality was dilated cardiomyopathy (n = 15, 24%). There was no significant difference in the mean noncompacted/compacted ratios between both of the disease groups.
CONCLUSION
Isolated and combined LVNC disease groups showed differences in age at diagnosis and clinical manifestations. The clinical and imaging findings may be helpful to better understand LVNC.

MeSH Terms

Adult*
Cardiomyopathies
Cardiomyopathy, Dilated
Diagnosis
Echocardiography
Heart Failure
Humans
Magnetic Resonance Imaging
Multidetector Computed Tomography

Figure

• 

  Fig. 1. 63 years old male with typical image findings of left ventricular noncompaction. Long and short axis view on echocardiography at end systolic phase of a 63 years old male hospitalized for dyspnea (A, B). Note that extensive trabeculations, two inner compacted (A, marking with bidirectional arrow end) and outer noncompacted layer (A, marking with bidirectional circle end) with different echogenicity, increased the non-compacted layer thickness over the compacted layer thickness ratio, and visible low echogenic inter-trabecular recess (B, arrow). The CT (C) and MRI (D) images at end diastolic phase of a 34 years old female with prominent inner noncompacted layer, especially in mid to apical wall of left ventricle.

• 

  Fig. 2. 31 years old female with LVNC: correlation between the autopsy specimen and image findings. The gross morphology of autopsy specimen (A), TTE finding (B), and MRI findings (C, D) of a 31years old female LVNC patient who underwent heart transplantation. There was delayed myocardial enhancement at mid anterior, mid septal, and mid inferior wall on MRI examination (D). LVNC = left ventricular noncompaction

Reference

References

1. Chin TK, Perloff JK, Williams RG, Jue K, Mohrmann R. Isolated noncompaction of left ventricular myocardium. A study of eight cases. Circulation. 1990; 82:507–513.

2. Benjamin MM, Khetan RA, Kowal RC, Schussler JM. Diagnosis of left ventricular noncompaction by computed tomography. Proc (Bayl Univ Med Cent). 2012; 25:354–356.

3. Udeoji DU, Philip KJ, Morrissey RP, Phan A, Schwarz ER. Left ventricular noncompaction cardiomyopathy: updated review. Ther Adv Cardiovasc Dis. 2013; 7:260–273.

4. Hofer M, Stöllberger C, Finsterer J. Acquired noncompaction associated with myopathy. Int J Cardiol. 2007; 121:296–297.

5. Gati S, Papadakis M, Papamichael ND, Zaidi A, Sheikh N, Reed M, et al. Reversible de novo left ventricular trabecu-lations in pregnant women: implications for the diagnosis of left ventricular noncompaction in low-risk populations. Circulation. 2014; 130:475–483.

6. Jenni R, Oechslin E, Schneider J, Attenhofer Jost C, Kaufmann PA. Echocardiographic and pathoanatomical characteristics of isolated left ventricular noncompaction: a step towards classification as a distinct cardiomyopathy. Heart. 2001; 86:666–671.

7. Stöllberger C, Finsterer J, Blazek G. Left ventricular hyper-trabeculation/noncompaction and association with additional cardiac abnormalities and neuromuscular disorders. Am J Cardiol. 2002; 90:899–902.

8. Petersen SE, Selvanayagam JB, Wiesmann F, Robson MD, Francis JM, Anderson RH, et al. Left ventricular noncompaction: insights from cardiovascular magnetic resonance imaging. J Am Coll Cardiol. 2005; 46:101–105.

9. Jacquier A, Thuny F, Jop B, Giorgi R, Cohen F, Gaubert JY, et al. Measurement of trabeculated left ventricular mass using cardiac magnetic resonance imaging in the diagnosis of left ventricular noncompaction. Eur Heart J. 2010; 31:1098–1104.

10. Ho KK, Anderson KM, Kannel WB, Grossman W, Levy D. Survival after the onset of congestive heart failure in Framingham Heart Study subjects. Circulation. 1993; 88:107–115.

11. Kramer CM, Barkhausen J, Flamm SD, Kim RJ, Nagel E. Society for Cardiovascular Magnetic Resonance Board of Trustees Task Force on Standardized Protocols. Standardized cardiovascular magnetic resonance (CMR) protocols 2013 update. J Cardiovasc Magn Reson. 2013; 15:91.

12. Ritter M, Oechslin E, Sütsch G, Attenhofer C, Schneider J, Jenni R. Isolated noncompaction of the myocardium in adults. Mayo Clin Proc. 1997; 72:26–31.

13. Ichida F, Hamamichi Y, Miyawaki T, Ono Y, Kamiya T, Akagi T, et al. Clinical features of isolated noncompaction of the ventricular myocardium: longterm clinical course, hemodynamic properties, and genetic background. J Am Coll Cardiol. 1999; 34:233–240.

14. Oechslin EN, Attenhofer Jost CH, Rojas JR, Kaufmann PA, Jenni R. Long-term follow-up of 34 adults with isolated left ventricular noncompaction: a distinct cardiomyopathy with poor prognosis. J Am Coll Cardiol. 2000; 36:493–500.

15. Rigopoulos A, Rizos IK, Aggeli C, Kloufetos P, Papachara-lampous X, Stefanadis C, et al. Isolated left ventricular noncompaction: an unclassified cardiomyopathy with severe prognosis in adults. Cardiology. 2002; 98:25–32.

16. Murphy RT, Thaman R, Blanes JG, Ward D, Sevdalis E, Papra E, et al. Natural history and familial characteristics of isolated left ventricular noncompaction. Eur Heart J. 2005; 26:187–192.

17. Lofiego C, Biagini E, Pasquale F, Ferlito M, Rocchi G, Perugini E, et al. Wide spectrum of presentation and variable outcomes of isolated left ventricular noncompaction. Heart. 2007; 93:65–71.

18. Captur G, Nihoyannopoulos P. Left ventricular noncompaction: genetic heterogeneity, diagnosis and clinical course. Int J Cardiol. 2010; 140:145–153.

19. Habib G, Charron P, Eicher JC, Giorgi R, Donal E, Laperche T, et al. Working Groups ‘Heart Failure and Cardiomyopathies'and ‘Echocardiography'of the French Society of Cardiology. Isolated left ventricular noncompaction in adults: clinical and echocardiographic features in 105 patients. Results from a French registry. Eur J Heart Fail. 2011; 13:177–185.

20. Zemrak F, Ahlman MA, Captur G, Mohiddin SA, Kawel-Boehm N, Prince MR, et al. The relationship of left ventricular trabeculation to ventricular function and structure over a 9.5-year follow-up: the MESA study. J Am Coll Cardiol. 2014; 64:1971–1980.

21. Sarma RJ, Chana A, Elkayam U. Left ventricular noncompaction. Prog Cardiovasc Dis. 2010; 52:264–273.

22. Niemann M, Störk S, Weidemann F. Left ventricular noncompaction cardiomyopathy: an overdiagnosed disease. Circulation. 2012; 126:e240–e243.

23. Towbin JA, Lorts A, Jefferies JL. Left ventricular noncompaction cardiomyopathy. Lancet. 2015; 386:813–825.

24. Stöllberger C, Finsterer J. Pitfalls in the diagnosis of left ventricular hypertrabeculation/noncompaction. Postgrad Med J. 2006; 82:679–683.

25. Gati S, Rajani R, Carr-White GS, Chambers JB. Adult left ventricular noncompaction: reappraisal of current diagnostic imaging modalities. JACC Cardiovasc Imaging. 2014; 7:1266–1275.