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Left Ventricular Noncompaction
Franklin Dana, MD; John D. Grizzard, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Diagnostic Checklist

          TERMINOLOGY

          • Abbreviations

            • Left ventricular noncompaction (LVNC)
          • Synonyms

            • Isolated noncompaction of ventricular myocardium
            • LV hypertrabeculation
            • Spongy myocardium
          • Definitions

            • Suspected cardiomyopathy characterized by excessive LC trabeculation
              • American Heart Association classifies LVNC as "genetic cardiomyopathy;" however, European Society of Cardiology defines as "nonclassified entity"
            • Thought to be due to arrest of normal embryologic process of myocardial compaction
            • Results in persistence of multiple prominent ventricular trabeculations and deep intertrabecular recesses
              • LVNC is favored to represent myocardial phenotype with heterogeneous genetic profile and variable course
              • Termed isolated LVNC when unaccompanied by other structural heart disease

          IMAGING

          • General Features

            • Imaging Recommendations

              • MR Findings

                • CT Findings

                  • Radiographic Findings

                    DIFFERENTIAL DIAGNOSIS

                      PATHOLOGY

                      • General Features

                        • Gross Pathologic & Surgical Features

                          • Microscopic Features

                            CLINICAL ISSUES

                            • Presentation

                              • Demographics

                                • Natural History & Prognosis

                                  • Treatment

                                    DIAGNOSTIC CHECKLIST

                                    • Image Interpretation Pearls

                                      • Reporting Tips

                                        Selected References

                                        1. Liu J et al: Cardiac magnetic resonance evaluation of left ventricular functional, morphological, and structural features in children and adolescents vs. young adults with isolated left ventricular non-compaction. Int J Cardiol. 271:402, 2018
                                        2. Andreini D et al: Long-term prognostic value of cardiac magnetic resonance in left ventricle noncompaction: a prospective multicenter study. J Am Coll Cardiol. 68(20):2166-81, 2016
                                        3. Arbustini E et al: Left ventricular noncompaction: a distinct genetic cardiomyopathy? J Am Coll Cardiol. 68(9):949-66, 2016
                                        4. Weir-McCall JR et al: Left ventricular noncompaction: anatomical phenotype or distinct cardiomyopathy? J Am Coll Cardiol. 68(20):2157-65, 2016
                                        5. Sidhu MS et al: Defining left ventricular noncompaction using cardiac computed tomography. J Thorac Imaging. 29(1):60-6, 2014
                                        6. Zemrak F 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. 64(19):1971-80, 2014
                                        7. Grothoff M et al: Value of cardiovascular MR in diagnosing left ventricular non-compaction cardiomyopathy and in discriminating between other cardiomyopathies. Eur Radiol. 22(12):2699-709, 2012
                                        8. Kawel N et al: Trabeculated (noncompacted) and compact myocardium in adults: the multi-ethnic study of atherosclerosis. Circ Cardiovasc Imaging. 5(3):357-66, 2012
                                        9. Nucifora G et al: Myocardial fibrosis in isolated left ventricular non-compaction and its relation to disease severity. Eur J Heart Fail. 13(2):170-6, 2011
                                        10. Oechslin E et al: Left ventricular non-compaction revisited: a distinct phenotype with genetic heterogeneity? Eur Heart J. 32(12):1446-56, 2011
                                        11. Dodd JD et al: Quantification of left ventricular noncompaction and trabecular delayed hyperenhancement with cardiac MRI: correlation with clinical severity. AJR Am J Roentgenol. 189(4):974-80, 2007
                                        12. Weiford BC et al: Noncompaction of the ventricular myocardium. Circulation. 109(24):2965-71, 2004
                                        13. Pignatelli RH et al: Clinical characterization of left ventricular noncompaction in children: a relatively common form of cardiomyopathy. Circulation. 108(21):2672-8, 2003
                                        14. Jenni R et al: Echocardiographic and pathoanatomical characteristics of isolated left ventricular non-compaction: a step towards classification as a distinct cardiomyopathy. Heart. 86(6):666-71, 2001
                                        15. Oechslin EN et al: Long-term follow-up of 34 adults with isolated left ventricular noncompaction: a distinct cardiomyopathy with poor prognosis. J Am Coll Cardiol. 36(2):493-500, 2000
                                        Related Anatomy
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                                        Related Differential Diagnoses
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                                        References
                                        Tables

                                        Tables

                                        KEY FACTS

                                        • Terminology

                                          • Imaging

                                            • Top Differential Diagnoses

                                              • Diagnostic Checklist

                                                TERMINOLOGY

                                                • Abbreviations

                                                  • Left ventricular noncompaction (LVNC)
                                                • Synonyms

                                                  • Isolated noncompaction of ventricular myocardium
                                                  • LV hypertrabeculation
                                                  • Spongy myocardium
                                                • Definitions

                                                  • Suspected cardiomyopathy characterized by excessive LC trabeculation
                                                    • American Heart Association classifies LVNC as "genetic cardiomyopathy;" however, European Society of Cardiology defines as "nonclassified entity"
                                                  • Thought to be due to arrest of normal embryologic process of myocardial compaction
                                                  • Results in persistence of multiple prominent ventricular trabeculations and deep intertrabecular recesses
                                                    • LVNC is favored to represent myocardial phenotype with heterogeneous genetic profile and variable course
                                                    • Termed isolated LVNC when unaccompanied by other structural heart disease

                                                IMAGING

                                                • General Features

                                                  • Imaging Recommendations

                                                    • MR Findings

                                                      • CT Findings

                                                        • Radiographic Findings

                                                          DIFFERENTIAL DIAGNOSIS

                                                            PATHOLOGY

                                                            • General Features

                                                              • Gross Pathologic & Surgical Features

                                                                • Microscopic Features

                                                                  CLINICAL ISSUES

                                                                  • Presentation

                                                                    • Demographics

                                                                      • Natural History & Prognosis

                                                                        • Treatment

                                                                          DIAGNOSTIC CHECKLIST

                                                                          • Image Interpretation Pearls

                                                                            • Reporting Tips

                                                                              Selected References

                                                                              1. Liu J et al: Cardiac magnetic resonance evaluation of left ventricular functional, morphological, and structural features in children and adolescents vs. young adults with isolated left ventricular non-compaction. Int J Cardiol. 271:402, 2018
                                                                              2. Andreini D et al: Long-term prognostic value of cardiac magnetic resonance in left ventricle noncompaction: a prospective multicenter study. J Am Coll Cardiol. 68(20):2166-81, 2016
                                                                              3. Arbustini E et al: Left ventricular noncompaction: a distinct genetic cardiomyopathy? J Am Coll Cardiol. 68(9):949-66, 2016
                                                                              4. Weir-McCall JR et al: Left ventricular noncompaction: anatomical phenotype or distinct cardiomyopathy? J Am Coll Cardiol. 68(20):2157-65, 2016
                                                                              5. Sidhu MS et al: Defining left ventricular noncompaction using cardiac computed tomography. J Thorac Imaging. 29(1):60-6, 2014
                                                                              6. Zemrak F 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. 64(19):1971-80, 2014
                                                                              7. Grothoff M et al: Value of cardiovascular MR in diagnosing left ventricular non-compaction cardiomyopathy and in discriminating between other cardiomyopathies. Eur Radiol. 22(12):2699-709, 2012
                                                                              8. Kawel N et al: Trabeculated (noncompacted) and compact myocardium in adults: the multi-ethnic study of atherosclerosis. Circ Cardiovasc Imaging. 5(3):357-66, 2012
                                                                              9. Nucifora G et al: Myocardial fibrosis in isolated left ventricular non-compaction and its relation to disease severity. Eur J Heart Fail. 13(2):170-6, 2011
                                                                              10. Oechslin E et al: Left ventricular non-compaction revisited: a distinct phenotype with genetic heterogeneity? Eur Heart J. 32(12):1446-56, 2011
                                                                              11. Dodd JD et al: Quantification of left ventricular noncompaction and trabecular delayed hyperenhancement with cardiac MRI: correlation with clinical severity. AJR Am J Roentgenol. 189(4):974-80, 2007
                                                                              12. Weiford BC et al: Noncompaction of the ventricular myocardium. Circulation. 109(24):2965-71, 2004
                                                                              13. Pignatelli RH et al: Clinical characterization of left ventricular noncompaction in children: a relatively common form of cardiomyopathy. Circulation. 108(21):2672-8, 2003
                                                                              14. Jenni R et al: Echocardiographic and pathoanatomical characteristics of isolated left ventricular non-compaction: a step towards classification as a distinct cardiomyopathy. Heart. 86(6):666-71, 2001
                                                                              15. Oechslin EN et al: Long-term follow-up of 34 adults with isolated left ventricular noncompaction: a distinct cardiomyopathy with poor prognosis. J Am Coll Cardiol. 36(2):493-500, 2000