Experience of a Single Center in the Diagnosis and Classification of Cases of Left Ventricular Noncompaction

Roberta Martinoli¹, Patrizia Saccucci^1*, Federica Papetti², Alessandro Dofcaci², Stefano Piccirilli³, Ilaria Sansoni⁴, Francesca Ianniello⁵, Federica Ferrante⁶, Maria Banci²

¹Department of Biomedicine and Prevention, University of Rome “Tor Vergata”, Rome, Italy.
²Operating Unit of Cardiology, Valmontone Hospital, Rome, Italy.
³Department of Cardiology, University of Rome “Tor Vergata”, Rome, Italy.
⁴Department of Imaging Diagnostic, University Campus Biomedical, Rome, Italy.
⁵Operating Unit of Gynecology, Valmontone Hospital, Rome, Italy.
⁶Operating Unit of Neurology, Valmontone Hospital, Rome, Italy.

DOI: 10.4236/ijcm.2015.64029                

Abstract

Objectives: To analyse the clinical profile of consecutive cases of Left Ventricular Non Compaction (LVNC) with particular interest in non-compacted segments valuation. Methods: There were 18,000 patients seen from 2007 to 2010, with a complete evaluation including family history and personal cardiac history, clinical examination and electrocardiography. Diagnosis was based on three published definitions. Results: The diagnosis of LVNC was placed in 1.4% of cases. Clinical and echo-cardiographic data for the 250 cases of LVNC are presented. Trabecular meshwork was observed predominantly at the apex (91.6%), in the lateral and inferior wall (40.4% and 38.0% respectively), and less frequently in the posterior and anterior wall (21.6% and 9.2% respectively). Conclusions: This study suggests that LVNC is a form of cardiomyopathy with higher prevalence and relatively better prognosis than previously reported.

Keywords

Cardiomyopathy, Endocardial Morphogenesis, Isolated Left Ventricular Noncompaction, Heart Failure, Echocardiography

Conflicts of Interest

The authors declare no conflicts of interest.

References

[1]	Chin, T.K., Perloff, J.K., Williams, R.G., Jue, K. and Mohrmann, R. (1990) Isolated Noncompaction of Left Ventricular Myocardium: A Study of Eight Cases. Circulation, 82, 507-513. http://dx.doi.org/10.1161/01.CIR.82.2.507
[2]	Oechslin, E.N., Attenhofer Jost, C.H., Rojas, J.R., Kaugmann, P.A. and Jenni, R. (2000) Long-Term Follow-Up of 34 Adults with Isolated Left Ventricular Noncompaction: A Distinct Cardiomyopathy with Poor Prognosis. Journal of the American College of Cardiology, 36, 493-500. http://dx.doi.org/10.1016/S0735-1097(00)00755-5
[3]	Ichida, F., Hamamichi, Y., Miyawaki, T., Ono, Y., Kamiya, T., Akagi, T., Hamada, H., Hirose, O., Isobe, T., Yamada, K., Kurotobi, S., Mito, H., Miyake, T., Murakami, Y., Nishi, T., Shinohara, M., Seguchi, M., Tashiro, S. and Tominatsu, H. (1999) Clinical Features of Iisolated Isolated Noncompaction of the Ventricular Myocardium: Long-Term Clinical Course, Hemodynamic Properties, and Genetic Background. Journal of the American College of Cardiology, 34, 233-240. http://dx.doi.org/10.1016/S0735-1097(99)00170-9
[4]	Ritter, M., Oechslin, E., Sütsch, G., Attenhofer, C., Schneider, J. and Jenni, R. (1997) Isolated Noncompaction of the Myocardium in Adults. Mayo Clinic Proceedings, 72, 26-31. http://dx.doi.org/10.4065/72.1.26
[5]	Shah, C.P., Nagi, K.S., Thakur, R.K., Boughner, D.R. and Xie, B. (1998) Spongy Left Ventricular Myocardium in Adults. Texas Heart Institute Journal, 25, 150-151.
[6]	Udeoji, D.U., Philip, K.J., Morrissey, R.P., Phan, A. and Schwarz, E.R. (2013) Left Ventricular Noncompaction Cardiomyopathy: Updated Review. Therapeutic Advances in Cardiovascular Disease, 7, 260-273. http://dx.doi.org/10.1177/1753944713504639
[7]	Jenni, R., Oechslin, E., Schneider, J., Attenhofer Jost, C. and Kaufmann, P.A. (2001) Echocardiographic and Pathoanatomic Characteristics of Isolated Left Ventricular Noncompaction: A Step Toward Classification as a Distinct Cardiomyopathy. Heart, 86, 666-671. http://dx.doi.org/10.1136/heart.86.6.666
[8]	Jenni, R., Oechslin, E.N. and van der Loo, B. (2007) Isolated Ventricular Non-Compaction of the Myocardium in Adults. Heart, 93, 11-15. http://dx.doi.org/10.1136/hrt.2005.082271
[9]	Stollberger, C., Finsterer, J. and Blazek, G. (2001) Prognosis of Left Ventricular Hypertrabeculation/Noncompaction and Association with Additional Cardiac Abnormalities and Neuromuscolar Neuromuscular Comorbidity. American Journal of Cardiology, 90, 899-902. http://dx.doi.org/10.1016/S0002-9149(02)02723-6
[10]	Lang, R.M., Bierig, M., Devereux, R.B., Flachskampf, F.A., Foster, E., Pellikka, P.A., Picard, M.H., Roman, M.J., Seward, J., Shanewise, J.S., Solomon, S.D., Spencer, K.T., Sutton, M.S. and Stewart, W.J. (2005) Recommendations for Chamber Quantification: A Report from the American Society of Echocardiography’s Guidelines and Standards Committee and the Chamber Quantification Writing Group, Developed in Conjunction with the European Association of Echocardiography, a Branch of the European Society of Cardiology. Journal of the American Society of Echocardiography, 18, 1440-1463. http://dx.doi.org/10.1016/j.echo.2005.10.005
[11]	Stollberger, C., Finsterer, J. and Blazek, G. (2002) Left Ventricular Hypertrabeculation, Noncompaction and Association with Additional Cardiac Abnormalities and Neuromuscular Disorders. American Journal of Cardiology, 90, 899-902. http://dx.doi.org/10.1016/S0002-9149(02)02723-6
[12]	World Health Organization (2006) Obesity and Overweight. Fact Sheet Number 311. World Health Organization, Geneva.
[13]	Mancia, G., Fagard, R., Narkiewicz, K., Redón, J., Zanchetti, A., Bohm, M., Christiaens, T., Cifkova, R., De Backer, G., Dominiczak, A., Galderisi, M., Grobbee, D.E., Jaarsma, T., Kirchhof, P., Kjeldsen, S.E., Laurent, S., Manolis, A.J., Nilsson, P.M., Ruilope, L.M., Schmieder, R.E., Sirnes, P.A., Sleight, P., Viigimaa, M., Waeber, B. and Zannad, F., Task Force Members (2013) 2013 ESH/ESC Guidelines for the Management of Arterial Hypertension: The Task Force for the Management of Arterial Hypertension of the European Society of Hypertension (ESH) and of European Society of Cardiology (ESC). Journal of Hypertension, 31, 1281-1357. http://dx.doi.org/10.1097/01.hjh.0000431740.32696.cc
[14]	Stollberger, C., Blazek, G., Winkler-Dworak, M. and Finsterer, J. (2008) Sex Differences in Left Ventricular Noncompaction in Patients with and without Neuromuscular Disorders. Revista Espanola de Cardiología, 61, 130-136. http://dx.doi.org/10.1157/13116200
[15]	Stollberger, C., Finsterer, J. and Blazek, G. (2002) Left Ventricular Hypertrabeculation/Noncompaction and Association with Additional Cardiac Abnormalities and Neuromuscular Disorders. American Journal of Cardiology, 90, 899-902. http://dx.doi.org/10.1016/S0002-9149(02)02723-6
[16]	Finsterer, J., Stollberger, C. and Blazek, G. (2006) Neuromuscular Implications in Left Ventricular Hypertrabeculation/ Noncompaction. International Journal of Cardiology, 110, 288-300. http://dx.doi.org/10.1016/j.ijcard.2005.10.028
[17]	Kramer, C.M. (2010) Cardiac Magnetic Resonance Imaging Identification of Myocardial Fibrosis: The Need for Standardization and Therapies. Journal of the American College of Cardiology, 56, 288-289. http://dx.doi.org/10.1016/j.jacc.2010.01.063