Figure 1
Teaching Case
SectionCardiovascular
Case TypeClinical Cases
AuthorsLucio Olivetti 1, Marco Orsatti 1, Sara Ceccomancini 2
Connected authors
42 years, male
A 42-year-old man with heart failure symptoms presented at the Emergency Department. The medical history revealed smoking (30-40 cigarettes/day) and a moderate intake of alcoholic beverages but excluded hypertension, diabetes mellitus and heart disease.
Left ventricular non-compaction (LVNC)
Hypertrophic cardiomyopathy
Dilated cardiomyopathy
Electrocardiogram: sinus tachycardia (115 bpm).
Chest radiograph: Clear both lung fields and costophrenic angles. Left ventricle enlargement. Elevation of the right diaphragm.
Echocardiogram: enlarged and diffusely hypokinetic left ventricle with severely reduction of the contractile function (ejection fraction 27%). Medium-to-severe mitral regurgitation, no signs of pulmonary hypertension.
Cardiac CT: normal right and left main trunk coronary arteries; noncalcified plaque with 50% stenosis of the anterior descending artery.
Cardiac MRI: enlarged left ventricle with increased intraventricular end-diastolic diameter (64 mm) and not compact morphology of the myocardium at the level of apex and the middle third of the lateral wall. Non-compaction/compaction ratio of > 2, 3 in diastole (Fig. 1). Mild delayed enhancement of the left lateral ventricular wall.
After MRI, an echocardiogram with contrast medium injection confirmed the MRI diagnosis showing the contrast filling of multiple, prominent myocardial trabeculations and deep intertrabecular recesses communicating with the LV cavity (Fig. 2).
Chest radiograph: Clear both lung fields and costophrenic angles. Left ventricle enlargement. Elevation of the right diaphragm.
Echocardiogram: enlarged and diffusely hypokinetic left ventricle with severely reduction of the contractile function (ejection fraction 27%). Medium-to-severe mitral regurgitation, no signs of pulmonary hypertension.
Cardiac CT: normal right and left main trunk coronary arteries; noncalcified plaque with 50% stenosis of the anterior descending artery.
Cardiac MRI: enlarged left ventricle with increased intraventricular end-diastolic diameter (64 mm) and not compact morphology of the myocardium at the level of apex and the middle third of the lateral wall. Non-compaction/compaction ratio of > 2, 3 in diastole (Fig. 1). Mild delayed enhancement of the left lateral ventricular wall.
After MRI, an echocardiogram with contrast medium injection confirmed the MRI diagnosis showing the contrast filling of multiple, prominent myocardial trabeculations and deep intertrabecular recesses communicating with the LV cavity (Fig. 2).
Left ventricular non-compaction (LVNC) is characterized by the presence of an extensive non compacted myocardial layer lining the cavity of the left ventricle (LV) and potentially leads to cardiac failure, thromboembolism and malignant arrhythmias [1-3]. It can be isolated or associated with neuromuscular disorders [4] and co-exist with other cardiac malformations such as different types of cardyomyopathy, and other cardiac malformations as Ebstein’s anomaly or complex cyanotic heart disease. In some cases, the right ventricle is also affected [5].
LVNC is considered a distinct cardiomyopathy, which has been categorized as unclassified cardiomyopathy by the WHO [6, 7] and recently has been listed among the group of genetic cardiomypathies [6, 8, 9].
LVNC is found in both pediatric and adult populations. One generally accepted hypothesis is that LVNC is caused by an intrauterine arrest (between week 5 and 8) of the process of compaction of the loosely interwoven meshwork of myocardial fibers, which normally progresses from epi- to endo-cardium and from cardiac base to apex [10, 11]. As a result, LVNC patients present with prominent trabeculations, deep intertrabecular recesses and thin epicardial compacta. A second hypothesis suggests that the prominent trabeculations represent an adaptive mechanism to compensate for abnormally contracting myocardium [12].
Characteristic LVNC morphologic features identified with echocardiography include: (1) thick, bilayered myocardium composed of noncompacted and compacted layers; (2) prominent trabecular outpouchings; and (3) deep endomyocardial recess [13, 14]. Diagnosis is complicated by the fact that LVNC shares morphologic features with hypertrophic and dilated cardiomyopathies. Echocardiography, moreover, poses inherent problems in assessing the left ventricular apex, known to be the most commonly non-compacted area [15]. In our case, the use of contrast medium allowed the echocardiography diagnosis of LVNC missed in the previous examination.
The superior spatial and contrast resolution of MRI makes this technique intrinsically appealing for detection of LVNC. The location and the extent of the trabecular network, and the thickness of the trabeculations and compacta can be well visualized using a combination of cine-MRI in different cardiac imaging planes. These sequences, moreover, allow the impact of morphologic abnormalities on regional and global function to be assessed [6]. Using MRI images, a non-compaction/compaction ratio of > 2, 3 in diastole distinguishes pathological non-compaction, with values for sensivity, specifity, and positive and negative prediction of 86%, 99%, 75% and 99%, respectively [1].
LVNC is considered a distinct cardiomyopathy, which has been categorized as unclassified cardiomyopathy by the WHO [6, 7] and recently has been listed among the group of genetic cardiomypathies [6, 8, 9].
LVNC is found in both pediatric and adult populations. One generally accepted hypothesis is that LVNC is caused by an intrauterine arrest (between week 5 and 8) of the process of compaction of the loosely interwoven meshwork of myocardial fibers, which normally progresses from epi- to endo-cardium and from cardiac base to apex [10, 11]. As a result, LVNC patients present with prominent trabeculations, deep intertrabecular recesses and thin epicardial compacta. A second hypothesis suggests that the prominent trabeculations represent an adaptive mechanism to compensate for abnormally contracting myocardium [12].
Characteristic LVNC morphologic features identified with echocardiography include: (1) thick, bilayered myocardium composed of noncompacted and compacted layers; (2) prominent trabecular outpouchings; and (3) deep endomyocardial recess [13, 14]. Diagnosis is complicated by the fact that LVNC shares morphologic features with hypertrophic and dilated cardiomyopathies. Echocardiography, moreover, poses inherent problems in assessing the left ventricular apex, known to be the most commonly non-compacted area [15]. In our case, the use of contrast medium allowed the echocardiography diagnosis of LVNC missed in the previous examination.
The superior spatial and contrast resolution of MRI makes this technique intrinsically appealing for detection of LVNC. The location and the extent of the trabecular network, and the thickness of the trabeculations and compacta can be well visualized using a combination of cine-MRI in different cardiac imaging planes. These sequences, moreover, allow the impact of morphologic abnormalities on regional and global function to be assessed [6]. Using MRI images, a non-compaction/compaction ratio of > 2, 3 in diastole distinguishes pathological non-compaction, with values for sensivity, specifity, and positive and negative prediction of 86%, 99%, 75% and 99%, respectively [1].
References
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[2] Ritter M, Oechslin E, Sutsch G, Attenhofer C, Schneider J, Jenni R (1997) Isolated noncompaction of the myocardium in adults. Mayo Clin Proc 72: 26–31 (PMID: 9005281)
[3] 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 36:493–500 (PMID: 10933363)
[4] Jenni R, Oechslin E, Schneider J, Jost CA, Kaufmann PA (2001) Echocardiographic and pathoanatomical characteristics of pathological noncompaction, as a distinct cardiomyopathy. Heart 86:666–71 (PMID: 11711464)
[5] Cheng H, Zhao S, Jiang S, Lu M, Yan C, Ling J, Zhang Y, Liu Q, Ma N, Yin G, Wan J, Yang Y, Li L, Jerecic R, He Z (2011) Comparison of cardiac magnetic resonance imaging features of isolated left ventricular non compaction in adults versus dilated cardiomyopathy in adults. Clin Radiol 853-860 (PMID: 21684533)
[6] Bogaert J, Dymarkowski S, Taylor AM, Muthurangu V (2012) Clinical Cardiac MRI. 309-312
[7] Richardson P, McKenna W, Bristow M, Maisch B, Mautner B, O\'Connell J, Olsen E, Thiene G, Goodwin J, Gyarfas I, Martin I, Nordet P (1995) Report of the 1995 World Health organization/International Society and Federation of Cardiology Task Force on the definition and classification of cardiomyopathies. Circulation 93: 841–842 (PMID: 8598070)
[8] Maron BJ, Towbin JA, Thiene G et al (2006) Contemporary definitions and classification of the cardiomyopathies. Circulation 113: 1807–1816 (PMID: 16567565)
[9] Watkins H, Ashrafian H, Redwood C (2011) Inherited cardiomyopathies. N Engl J Med 364:1643–1656 (PMID: 21524215)
[10] Elshershari H, Okutan V, Celiker A (2001) Isolated noncompaction of the ventricular myocardium. Cardiol Young 11: 472–475 (PMID: 11558962)
[11] Pignatelli RH, McMahon CJ, Dreyer WJ, Denfield SW, Price J, Belmont JW, Craigen WJ, Wu J, El Said H, Bezold LI, Clunie S, Fernbach S, Bowles NE, Towbin JA (2003) Clinical characterization of left ventricular noncompaction in children. A relatively common form of cardiomyopathy. Circulation 108: 2672–2678 (PMID: 14623814)
[12] Captur G, Nihoyannopoulos P (2010) Left ventricular noncompaction: genetic heterogeneity, diagnosis and clinical course. Int J Cardiol 140: 145–153 (PMID: 19664830)
[13] Paterick TE, Tajik AJ (2012) Left ventricular noncompaction: a diagnostically challenging cardiomyopathy. Circ J 76: 1556-1562 (PMID: 2266478)
[14] Jenni R, Oechslin E, Schneider J, Attenhofer Jost C, Kaufmann PA (2001) Echocardiographic and pathoanatomical characteristics of isolated left ventricular non-compaction: a step towards classification as a distinct cardiomyopathy. Heart 86: 666 – 671 (PMID: 11711464)
[15] Paterick TE, Gerber TC, Pradhan SR, Lindor NM, Tajik AJ (2010) Left ventricular noncompaction cardiomyopathy: What do we know?. Rev Cardiovasc Med 11: 92 – 99 (PMID: 20700091)
Case information
| URL: | https://www.eurorad.org/case/12220 |
| DOI: | 10.1594/EURORAD/CASE.12220 |
| ISSN: | 1563-4086 |
Figure 2
Figure 2
Ceccomancini S, Department of Cardiology. AO Istituti Ospitalieri di Cremona, Cremona, Italy