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Pediatrics
Diagnosis
Cardiac
Cardiomyopathies
Left Ventricular Noncompaction
Left Ventricular Noncompaction
Randy R. Richardson, MD; Ryan A. Moore, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Clinical Issues

        TERMINOLOGY

        • Abbreviations

          • Left ventricular noncompaction cardiomyopathy (LVNC)

        • Synonyms

          • Noncompacted left ventricle (NCLV), left ventricular hypertrabeculation, spongy myocardium

        • Definitions

          • Inherited cardiomyopathy characterized by extensive hypertrabeculation of left ventricular (LV) myocardium
          • 2-layer appearance of LV with sponge-like noncompacted (NC) inner layer & thinner, dense compacted (C) outer layer
          • Primitive appearance of myocardium suggests potential arrest in fetal LV myocardial development

        IMAGING

        • General Features

          • Radiographic Findings

            • CT Findings

              • MR Findings

                • Echocardiographic Findings

                  • Imaging Recommendations

                    DIFFERENTIAL DIAGNOSIS

                      CLINICAL ISSUES

                      • Presentation

                        • Demographics

                          • Natural History & Prognosis

                            • Treatment

                              Selected References

                              1. Di Fusco SA et al: Left ventricular noncompaction: diagnostic approach, prognostic evaluation, and management strategies. Cardiol Rev. 28(3):125-34, 2020
                              2. Rao K et al: The role of multimodality imaging in the diagnosis of left ventricular noncompaction. Eur J Clin Invest. 50(9):e13254, 2020
                              3. Fennira S et al: [Left ventricular non-compaction: what should be known!.] Ann Cardiol Angeiol (Paris). 68(2):120-4, 2019
                              4. Wengrofsky P et al: Left ventricular trabeculation and noncompaction cardiomyopathy: a review. EC Clin Exp Anat. 2(6):267-83, 2019
                              5. Amzulescu MS et al: Prognostic impact of hypertrabeculation and noncompaction phenotype in dilated cardiomyopathy: a CMR study. JACC Cardiovasc Imaging. 8(8):934-46, 2015
                              6. Jefferies JL et al: Cardiomyopathy phenotypes and outcomes for children with left ventricular myocardial noncompaction: results from the pediatric cardiomyopathy registry. J Card Fail. 21(11):877-84, 2015
                              7. Towbin JA et al: Left ventricular non-compaction cardiomyopathy. Lancet. 386(9995):813-25, 2015
                              8. Stacey RB et al: Comparison of systolic and diastolic criteria for isolated LV noncompaction in CMR. JACC Cardiovasc Imaging. 6(9):931-40, 2013
                              Related Anatomy
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                              Related Differential Diagnoses
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                              References
                              Tables

                              Tables

                              KEY FACTS

                              • Terminology

                                • Imaging

                                  • Clinical Issues

                                    TERMINOLOGY

                                    • Abbreviations

                                      • Left ventricular noncompaction cardiomyopathy (LVNC)

                                    • Synonyms

                                      • Noncompacted left ventricle (NCLV), left ventricular hypertrabeculation, spongy myocardium

                                    • Definitions

                                      • Inherited cardiomyopathy characterized by extensive hypertrabeculation of left ventricular (LV) myocardium
                                      • 2-layer appearance of LV with sponge-like noncompacted (NC) inner layer & thinner, dense compacted (C) outer layer
                                      • Primitive appearance of myocardium suggests potential arrest in fetal LV myocardial development

                                    IMAGING

                                    • General Features

                                      • Radiographic Findings

                                        • CT Findings

                                          • MR Findings

                                            • Echocardiographic Findings

                                              • Imaging Recommendations

                                                DIFFERENTIAL DIAGNOSIS

                                                  CLINICAL ISSUES

                                                  • Presentation

                                                    • Demographics

                                                      • Natural History & Prognosis

                                                        • Treatment

                                                          Selected References

                                                          1. Di Fusco SA et al: Left ventricular noncompaction: diagnostic approach, prognostic evaluation, and management strategies. Cardiol Rev. 28(3):125-34, 2020
                                                          2. Rao K et al: The role of multimodality imaging in the diagnosis of left ventricular noncompaction. Eur J Clin Invest. 50(9):e13254, 2020
                                                          3. Fennira S et al: [Left ventricular non-compaction: what should be known!.] Ann Cardiol Angeiol (Paris). 68(2):120-4, 2019
                                                          4. Wengrofsky P et al: Left ventricular trabeculation and noncompaction cardiomyopathy: a review. EC Clin Exp Anat. 2(6):267-83, 2019
                                                          5. Amzulescu MS et al: Prognostic impact of hypertrabeculation and noncompaction phenotype in dilated cardiomyopathy: a CMR study. JACC Cardiovasc Imaging. 8(8):934-46, 2015
                                                          6. Jefferies JL et al: Cardiomyopathy phenotypes and outcomes for children with left ventricular myocardial noncompaction: results from the pediatric cardiomyopathy registry. J Card Fail. 21(11):877-84, 2015
                                                          7. Towbin JA et al: Left ventricular non-compaction cardiomyopathy. Lancet. 386(9995):813-25, 2015
                                                          8. Stacey RB et al: Comparison of systolic and diastolic criteria for isolated LV noncompaction in CMR. JACC Cardiovasc Imaging. 6(9):931-40, 2013