jksronline.org
eISSN 2951-0805
    J Korean Soc Radiol. 2018 Mar;78(3):170-178. English.
    Published online Mar 02, 2018.
    https://doi.org/10.3348/jksr.2018.78.3.170
    Copyrights © 2018 The Korean Society of Radiology
    Original Article

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

    Se Jin Cho, MD, Dong Hyun Yang, MD, Joon-Won Kang, MD and Tae-Hwan Lim, MD
      • Department of Radiology and Research Institute of Radiology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.
    • Corresponding author: Dong Hyun Yang, MD. Department of Radiology and Research Institute of Radiology, Asan Medical Center, University of Ulsan College of Medicine, 88 Olympic-ro 43-gil, Songpa-gu, Seoul 05505, Korea. Tel. 82-2-3010-5820, Fax. 82-2-2045-4127, Email: donghyun.yang@gmail.com
    Received April 11, 2017; Revised August 10, 2017; Accepted October 01, 2017.

    This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

    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.

    Keywords
    Cardiomyopathies; Magnetic Resonance; Multidetector Computed Tomography; Echocardiography

    INTRODUCTION

    Left ventricular noncompaction (LVNC) is a myocardial disorder characterized by numerous prominent trabeculations and increased depth of inter-trabecular recesses that could contain thrombi (1). Congenital developmental arrest during the first trimester leading to the formation of two layers of the myocardium is the most accepted theory for the onset of LVNC (1, 2, 3). However, congenital developmental arrest alone is not sufficient to explain the etiology of LVNC, as suggested by some reports of acquired LVNC cases and even of reversible trabeculation in pregnant women (4, 5).

    Although several diagnostic criteria for this disorder have been suggested, a global standard diagnosis for LVNC has yet to be established. Variable clinical manifestations and prognoses for LVNC have also been reported because patient population and imaging criteria have differed between previous studies (1, 6, 7, 8, 9). We considered that a description of clinical characteristics and imaging features in an LVNC series, even using retrospective data from a single center, would increase our understanding of this rare disease entity. In our current study, we described imaging and clinical findings for a LVNC in a cohort of 63 adult patients.

    MATERIALS AND METHODS

    Patients

    The Institutional Review Board of our hospital approved this retrospective study, and waived the requirement to obtain informed consent (2015-0570). Using the research-dedicated database system (ABLE, Asan Medical Center, Seoul, Korea) of our institution, which contains cardiac CT, cardiac MRI, and transthoracic echocardiography (TTE) reports, patients with LVNC and aged above 18 years were identified using the keywords noncompaction, spongy myocardium, or hypertrabeculation. Among the 72 searched patients, from 2000 to 2014, 66 patients were diagnosed with LVNC at our hospital based on cardiac imaging. Three cases without image data or for whom the image quality was suboptimal were excluded. Finally, 17 CTs, 18 MRIs, and 51 TTEs of 63 LVNC patients were enrolled. Total 43 patients were diagnosed by just one image modality (34 only by TTEs, 6 only by CTs, and 3only by MRs). We divided the study patients into two groups: LVNC without cardiac anomaly (isolated disease group, n = 32) and LVNC with cardiac anomaly (combined disease group, n = 31) (Table 1). Combined cardiac anomalies included dilated cardiomyopathy (n = 15, 24%) followed by other types of congenital heart disease (n = 12, 19%) including coarctation of the aorta (CoA), tetralogy of Fallot (TOF), Ebstein anomaly, and transposition of the great arteries (TGA). Clinical manifestations at diagnosis included symptoms such as neck vein distention, rales, acute pulmonary edema, nocturnal dyspnea, and other manifestations of congestive heart failure (CHF) based on the criteria of the Framingham Heart Study (10). All detectable symptoms in our study patients were described at their initial presentation. Incidental detection was defined as diagnosis of LVNC on imaging that was conducted to evaluate other cardiac diseases without symptoms of CHF or on screening echocardiography (ECG).

    Table 1
    Baseline Characteristics of Left Ventricular Noncompaction Patients

    Imaging Techniques and Analysis

    Electrocardiography-gated cardiac CT was performed using either 16-slice CT, first-generation dual-source CT, or second-generation dual-source CT (Sensation 16, Definition, and Definition FLASH respectively, Siemens Healthcare, Erlangen, Germany). Images were obtained after the injection of 60–80 mL of iomeprol-400 (Iomeron; Bracco Imaging, Milan, Italy) followed by 40 mL of a saline chaser. Body size-adaptive adjustment of tube potential and tube current was performed to reduce the radiation dose. Cardiac MRI was performed using a 1.5-T machine (Intera, Philips, Amsterdam, Netherlands; or Avanto, Siemens Healthcare, Erlangen, Germany). The balanced steadystate free precession sequence applied for cine-MRI data slice thickness of 5–8 mm and imaging matrix 256 × 256 on the short axis, 4-chamber, 2-chamber, and 3-chamber slice position. Delayed enhancement of MRI was performed using a 2D segmented inversion recovery gradient echo sequence 20minutes after the intravenous administration of gadoterate meglumine (Dotarem®, Guerbet, Villepinte, France) (0.4 cc/kg) (11). Seventeen patients with cardiac CT and 18 patients with cardiac MR were also reviewed by two radiologists in consensus based on the criterion of Peterson et al. (8) for cardiac MRI and CT at end diastolic phase (6, 8, 9). The noncompacted layer thickness over the compacted layer thickness (NC/C) ratio was obtained from the most severe portion of trabeculation in the cardiac wall on the sagittal view.

    TTE, which included two-dimensional and Doppler imaging, was performed using commercially available ultrasonographic equipment (Sonos 7500, Philips Medical Systems, Andover, MA, USA; or Vivid 7, GE Healthcare, Waukesha, WI, USA) with a 35 MHz transducer. The echocardiographic diagnosis of LVNC was based on the criterion of Jenni et al. (6). It included an end-systolic NC/C ratio higher than 2, and evidence of inter-trabecular recesses on color Doppler (6). TTE images were reviewed based on the described criterion to define absence or existence of LVNC. NC/C ratio measured on 2 chamber view, left ventricular ejection fraction (LVEF), indexed LV end diastolic/systolic volume, and indexed LV mass were measured.

    Statistical Analysis

    Continuous variables are expressed as mean ± standard deviation, and nominal variables as numbers and percentages. Patient demographics, hemodynamic parameters, and imaging measurements were compared between patients with and those without a combined cardiac anomaly. Continuous variables were compared using the t-test, and categorical variables using the chi-square test or the Fisher exact test. Additional Bland-Altman Analysis was done for the inter-modality difference comparison. We performed statistical analysis using SPSS version 21.0 software (IBM Corp., Armonk, NY, USA). A p-value < 0.05 was considered statistically significant.

    RESULTS

    Among the 63 patients with LVNC analyzed in this study, 32 (51%) did not have a combined cardiac anomaly (isolated disease group) and 31 (49%) had combined cardiac abnormalities (combined disease group). The mean age at initial diagnosis of the isolated disease group was higher than that of the combined disease group (54.3 vs. 40.3 years, p < 0.001) (Table 1). The combined disease group presented with symptoms at initial diagnosis more frequently than the isolated disease group (94% vs. 75%, p = 0.082). The most frequent chief complaint at initial diagnosis was CHF in both groups (n = 38, 60%), which was more frequent in the combined than in the isolated disease group (74% vs. 47%, p = 0.027). Thromboembolic events were more common in the combined than in the isolated disease group, without statistical significance (39% vs. 26%, p = 0.279). ECG findings were heterogeneous, varying from normal sinus rhythm to Wolff-Parkinson-White (WPW) syndrome. Left bundle branch block was the most common finding in the isolated disease group (n = 9, 28%), whereas atrial fibrillation was the most frequent in the combined disease group (n = 8, 26%). The number of patients who underwent cardiac intervention or operation was much higher in the combined than in the isolated disease group (14 vs. 1, respectively). Four patients underwent heart transplantation, 1 in the isolated and 3 in the combined disease group. In the combined disease group, the most common comorbid cardiac abnormality was dilated cardiomyopathy (n = 15, 24%), followed by other congenital heart diseases (n = 12, 19%) including CoA, TOF, Ebstein anomaly, and TGA.

    From the retrospective review of the data from each image modality, patients with LVNC showed extensive trabeculation, increased NC/C ratio, and inter-trabecular recess (Fig. 1). The mean NC/C ratio was 2.8 ± 0.6 on TTEs in 51 patients, 2.8 ± 0.7 on CTs in 17 patients, and 2.9 ± 0.8 on MRIs in 18 patients. There was no significant difference in the NC/C ratios between the isolated and the combined disease group (NC/C ratio on TTE: 2.8 ± 1.7 vs. 2.8 ± 0.3; p = 0.227) either on TTE or MRI (Table 2). In the 17 patients with CT data, the combined disease group showed a higher NC/C ratio than the isolated disease group (p = 0.010). In the patients who underwent both TTE and CT (n = 9), TTE and MRI (n = 13), and CT and MRI (n = 8), there were no significant inter-modality differences in the NC/C ratios (Table 3). And, the limits of agreement for inter-modality differences in the NC/C ratios on TTE and CT, TTE and MRI, and CT and MRI are 0.11 ± 1.23, 0.12 ± 1.24, and 0.01 ± 0.72, respectively by Bland-Altman Analysis. Among the 18 patients who received MRI, delayed myocardial enhancement was observed in 6 patients, 4 of whom showed a decreased LV ejection fraction of less than 50% at presentation (Fig. 2).

    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 noncompacted 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

    Table 2
    NC/C Ratio on Each Three Image Modality

    Table 3
    Inter-Modality Difference of NC/C between Three Different Image Modality in All Groups

    DISCUSSION

    The major findings from our current analysis of clinical and imaging data in LVNC patients collected over 14 years at a single tertiary center were: 1) the combined form of LVNC shows a younger age at diagnosis, more frequent symptoms, and higher frequency of thromboembolic events than the isolated form; and 2) the mean NC/C ratios of 2.8 ± 0.6 on TTE, 2.7 ± 0.7 on CT, and 2.9 ± 0.8 on MRI indicate no significant inter-modality difference.

    As an unknown myocardial disease, LVNC has shown variable clinical findings and its diagnosis is the subject of some controversy. The clinical characteristics of LVNC have been reported in several studies, showing high variability from an asymptomatic state to sudden cardiac death (1, 7, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21). The most frequent chief complaint at initial diagnosis was heart failure-related symptoms in both groups of our current study (n = 38, 60%), was more frequent in the combined than in the isolated disease group (74% vs. 47%, p = 0.003) (3). In our present analysis, the disease was detected incidentally in the absence of symptoms in only 10 (16%) patients, and these cases were more frequent in the isolated than in the combined LVNC group (25% vs. 7%). Thromboembolic events were evident in 20 patients, consistent with the previously reported incidence range from 0 to 38% (1, 3, 12, 13, 14). In addition, the incidence of this complication was higher in the combined than in the isolated disease group (39% vs. 26%, p = 0.279). Several comorbid ECG abnormal findings have been reported, such as WPW syndrome (3–32%), ventricular tachycardia (15–38%), bundle branch block (5–56%), paroxysmal supraventricular tachycardia and others (1, 3, 12, 13, 14, 22). In our current study, a left bundle branch block was the most common ECG finding in the isolated disease group (n = 9, 28%). Conversely, atrial fibrillation was the most frequent ECG finding in the combined disease group (n = 8, 26%).

    Several reports have recently suggested a relatively benign natural course and lower frequency of symptomatic presentation than the previous notion that NC typically leads to heart systolic dysfunction on follow-up. For example, a recent the MESA-9.5 year follow-up study reported a benign course in the relatively asymptomatic adult population (the so-called “asymptomatic trabeculation”). That report suggested that regular and frequent imaging and clinical follow-up may be unnecessary in subjects with a low pre-test probability of LVNC but with marked trabeculation based on traditional imaging criteria (16, 20, 23). Some reports have also shown that some factors can interfere with the measurement of the NC/C ratio, in particular some mimickers such as a false tendon and aberrant bands (24, 25). On current study population, only 10 among 63 patients (16%) diagnosed as LVNC incidentally. That means the majority of the included population in this study basically focus on the symptomatic patients. We considered that is the main difference about study populations compared to the MESA-9.5 year follow-up study (20).

    This study has some limitations of note. First, definite diagnostic and classification criteria for LVNC are still lacking. According to the classification of cardiomyopathy by the American Heart Association, LVNC is considered to be a genetic cardiomyopathy distinguished from other cardiomyopathies. However, some reports have tried to phenotypically subcategorize LVNC as dilated/ hypertrophic/restricted-type LVNC, or categorize according to the involved chamber (23). To that same purpose, we retrospectively included all LVNC patients with/without combined cardiac abnormalities including congenital heart disease, acquired cardiac disease, and other kinds of cardiomyopathy, regardless of disease category. Therefore, some inclusion bias was unavoidable. Furthermore, the difference of the diagnostic criteria among the image modalities take a role as a limitation. Second, since most cases were diagnosed following hospitalization in a large tertiary hospital, the prevalence of LVNC was hard to evaluate and the true incidental findings were probably underestimated. In addition, there was a limitation in the comparative analysis of imaging findings, since only five patients received all image modality simultaneously. Finally, current study was done with retrospective manners.

    In conclusion, isolated and combined groups of LVNC showed differences in the age at diagnosis and in the clinical manifestations. The clinical and imaging findings of LVNC presented in this study may assist in the better understanding of LVNC.

    Acknowledgments

    The work was supported by the National Research Foundation of Korea (NRF) grant funded by the Korea government (MSIP) (NRF-2016R1A1A1A05921207 and NRF-2015R1A2A2A04003034) and a grant (2017-7208) from the Asan Institute for Life Sciences, Asan Medical Centre, Seoul, Korea.

    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.
      1. 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.
      1. Udeoji DU, Philip KJ, Morrissey RP, Phan A, Schwarz ER. Left ventricular noncompaction cardiomyopathy: updated review. Ther Adv Cardiovasc Dis 2013;7:260–273.
      1. Hofer M, Stöllberger C, Finsterer J. Acquired noncompaction associated with myopathy. Int J Cardiol 2007;121:296–297.
      1. Gati S, Papadakis M, Papamichael ND, Zaidi A, Sheikh N, Reed M, et al. Reversible de novo left ventricular trabeculations in pregnant women: implications for the diagnosis of left ventricular noncompaction in low-risk populations. Circulation 2014;130:475–483.
      1. Jenni R, Oechslin E, Schneider J, Attenhofer Jost C, Kaufmann PA. Echocardiographic and pathoanatomical characteristics of isolated left ventricular non-compaction: a step towards classification as a distinct cardiomyopathy. Heart 2001;86:666–671.
      1. Stöllberger C, Finsterer J, Blazek G. Left ventricular hypertrabeculation/ noncompaction and association with additional cardiac abnormalities and neuromuscular disorders. Am J Cardiol 2002;90:899–902.
      1. Petersen SE, Selvanayagam JB, Wiesmann F, Robson MD, Francis JM, Anderson RH, et al. Left ventricular non-compaction: insights from cardiovascular magnetic resonance imaging. J Am Coll Cardiol 2005;46:101–105.
      1. 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 non-compaction. Eur Heart J 2010;31:1098–1104.
      1. 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.
      1. 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.
      1. 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.
      1. Ichida F, Hamamichi Y, Miyawaki T, Ono Y, Kamiya T, Akagi 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;34:233–240.
      1. 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.
      1. Rigopoulos A, Rizos IK, Aggeli C, Kloufetos P, Papacharalampous X, Stefanadis C, et al. Isolated left ventricular noncompaction: an unclassified cardiomyopathy with severe prognosis in adults. Cardiology 2002;98:25–32.
      1. Murphy RT, Thaman R, Blanes JG, Ward D, Sevdalis E, Papra E, et al. Natural history and familial characteristics of isolated left ventricular non-compaction. Eur Heart J 2005;26:187–192.
      1. 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 non-compaction. Heart 2007;93:65–71.
      1. Captur G, Nihoyannopoulos P. Left ventricular non-compaction: genetic heterogeneity, diagnosis and clinical course. Int J Cardiol 2010;140:145–153.
      1. 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 non-compaction in adults: clinical and echocardiographic features in 105 patients. Results from a French registry. Eur J Heart Fail 2011;13:177–185.
      1. 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.
      1. Sarma RJ, Chana A, Elkayam U. Left ventricular noncompaction. Prog Cardiovasc Dis 2010;52:264–273.
      1. Niemann M, Störk S, Weidemann F. Left ventricular noncompaction cardiomyopathy: an overdiagnosed disease. Circulation 2012;126:e240–e243.
      1. Towbin JA, Lorts A, Jefferies JL. Left ventricular non-compaction cardiomyopathy. Lancet 2015;386:813–825.
      1. Stöllberger C, Finsterer J. Pitfalls in the diagnosis of left ventricular hypertrabeculation/non-compaction. Postgrad Med J 2006;82:679–683.
      1. 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.
    MeSH Terms
    Adult*
    Cardiomyopathies
    Cardiomyopathy, Dilated
    Diagnosis
    Echocardiography
    Heart Failure
    Humans
    Magnetic Resonance Imaging
    Multidetector Computed Tomography
    Metrics
    Share
    Figures
    slide
    slide

    1 / 2

    Tables
    slide
    slide
    slide

    1 / 3

    Funding Information
    PERMALINK