Abstract
Background
Isolated left ventricular non-compaction has been reported extensively. However, apical non-compaction of both ventricles and the interventricular septum (IVS) is not often reported in the literature. The objective of our study is to evolve the echocardiographic diagnostic criteria and to assess the types and impact of the associated lesions in “apical non-compaction”.
Methods and results
Seventy consecutive cases that fulfilled standard echocardiographic criteria for non-compaction of the left ventricle and, in addition, N/C ratio of >3 for the right ventricle and apical IVS formed the material. The age of patients ranged from 3 days to 35 years, with 37 males and 33 females. The associated lesions were present in all 70 cases: 62 had acyanotic (88.6 %) and 8 had cyanotic congenital heart diseases (11.4 %). Of the 70 cases, 18 had pump failure (25.7 %): 8 cases had left ventricular dysfunction, 7 had right ventricular dysfunction, and 3 had biventricular dysfunction; 33 (47.1 %) had pulmonary hypertension, 2 (2.9 %) had thrombus, and 1 (1.4 %) had tachyarrhythmia. Pump failure was worsened by volume overload in 33.9 % and by pressure overload in 8.1 % of cases. Some very rare lesions were detected. All 70 cases had Swiss cheese appearance of the apical half of the IVS, looking like the delta of a river.
Conclusion
All of the apical non-compaction syndrome cases had associated lesions, mostly acyanotic congenital heart disease with volume overload, rather than obstructive lesions. Transthoracic echocardiography plays an important role in the diagnosis of apical non-compaction syndrome and associated lesions that worsen the pump failure.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Junga G, Kneifel S, Von Smekal A, et al. Myocardial ischaemia in children with isolated ventricular non-compaction. Eur Heart J. 1999;20:910–6.
Chin TK, Perloff JK, Williams RG, et al. Isolated noncompaction of left ventricular myocardium. A study of eight cases. Circulation. 1990;82:507–13.
Jenni R, Oechslin E, Schneider J, et al. Echocardiographic and pathoanatomical characteristics of isolated left ventricular non-compaction: a step towards classification as a distinct cardiomyopathy. Heart. 2001;86:666–71.
Sarma RJ, Chana A, Elkayam U. Left ventricular noncompaction. Prog Cardiovasc Dis. 2010;52:264–73.
Anderson RH. Anatomy. In: Anderson RH, Baker EJ, Macartney FJ, Rigby ML, Shinebourne EA, Tynan M, editors. Paediatric cardiology. 2nd ed. London: Churchill Livingstone; 2002. p. 17–35.
Ben-Shachar G, Arcilla RA, Lucas RV, et al. Ventricular trabeculations in the chick embryo heart and their contribution to ventricular and muscular septal development. Circ Res. 1985;57:759–66.
Anderson RH. Ventricular non-compaction—a frequently ignored finding? Eur Heart J. 2008;29:10–1.
Burke A, Mont E, Kutys R, et al. Left ventricular noncompaction: a pathological study of 14 cases. Hum Pathol. 2005;36:403–11.
Weiford BC, Subbarao VD, Mulhern KM. Noncompaction of the ventricular myocardium. Circulation. 2004;109:2965–71.
Ozturk O, Ozturk U, Toprak N. Biventricular myocardial noncompaction. Dicle Tip Dergisi. 2006;33:95–7.
Stöllberger C, Finsterer J. Septal hypertrabeculation/noncompaction: cardiac and neurologic implications. Int J Cardiol. 2009;20(132):173–5.
Ichida F, Hamamichi Y, Miyawaki T, et al. Clinical features of isolated noncompaction of the ventricular myocardium: long-term clinical course, hemodynamic properties, and genetic background. J Am Coll Cardiol. 1999;34:233–40.
Talwar S, Nair VV, Kothari SS, et al. Aortico-right ventricular tunnel with anomalous right coronary artery. J Card Surg. 2011;26:521–6.
Singh SK, Dwivedi SK, Kumar A, et al. Aneurysmal aorto-right ventricular tunnel. Ann Thorac Surg. 2012;93:e21–2.
Vijayalakshmi IB, Narasimhan C, Agarwal A. Closure of aorto-right ventricular tunnel with Amplatzer duct occluder II. J Invasive Cardiol. 2013;25:E75–7.
Sinkovec M, Kozelj M, Podnar T. Familial biventricular myocardial noncompaction associated with Ebstein’s malformation. Int J Cardiol. 2005;102:297–302.
Saleeb SF, Margossian R, Spencer CT, et al. Reproducibility of echocardiographic diagnosis of left ventricular noncompaction. J Am Soc Echocardiogr. 2012;25:194–202.
Sedmera D, Pexieder T, Rychterova V, et al. Remodeling of chick embryonic ventricular myoarchitecture under experimentally changed loading conditions. Anat Rec. 1999;254:238–52.
Acknowledgments
We are grateful to Dr. Bob Anderson who gave a masterly insight into apical non-compaction and generated more interest in us to take up this study. We thank Dr. Ravindran, postgraduate student at our institute, and Mr P. Madhusudhan for the schematic diagram.
Conflict of interest
Dr. I. B. Vijayalakshmi, Dr. Chitra Narasimhan, Dr. Anuspandana Mahapatra, and Dr. C. N. Manjunath declare that they have no conflict of interest.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Vijayalakshmi, I.B., Narasimhan, C., Mahapatra, A. et al. Echocardiography in the diagnosis of apical non-compaction associated with congenital heart diseases. J Echocardiogr 12, 31–39 (2014). https://doi.org/10.1007/s12574-013-0202-0
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s12574-013-0202-0