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Left ventricular noncompaction: a distinct cardiomyopathy or a trait shared by different cardiac diseases?

ABSTRACT: Whether left ventricular noncompaction (LVNC) is a distinct cardiomyopathy or a morphologic trait shared by different cardiomyopathies remains controversial. Current guidelines from professional organizations recommend different strategies for diagnosing and treating patients with LVNC. This state-of-the-art review discusses new insights into the basic mechanisms leading to LVNC, its clinical manifestations, treatment modalities, anatomy and pathology, embryology, genetics, epidemiology, and imaging. Three markers currently define LVNC: prominent left ventricular trabeculae, deep intertrabecular recesses, and a thin compacted layer. Although new genetic data from mice and humans supports LVNC as a distinct cardiomyopathy, evidence for LVNC as a shared morphological trait is not ruled out. Criteria supporting LVNC as a shared morphological trait may depend on consensus guidelines from the multiple professional organizations. Enhanced imaging and increased use of genetics are both predicted to significantly impact our overall understanding of the basic mechanisms causing LVNC and its optimal management.

SUBMITTER: Arbustini E

PROVIDER: S-EPMC10352990 | biostudies-literature | 2014 Oct

REPOSITORIES: biostudies-literature

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Left ventricular noncompaction: a distinct cardiomyopathy or a trait shared by different cardiac diseases?

Arbustini Eloisa E Weidemann Frank F Hall Jennifer L JL

Journal of the American College of Cardiology 20141021 17

Whether left ventricular noncompaction (LVNC) is a distinct cardiomyopathy or a morphologic trait shared by different cardiomyopathies remains controversial. Current guidelines from professional organizations recommend different strategies for diagnosing and treating patients with LVNC. This state-of-the-art review discusses new insights into the basic mechanisms leading to LVNC, its clinical manifestations, treatment modalities, anatomy and pathology, embryology, genetics, epidemiology, and ima ...[more]

PMID: 25443708

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Project description:BackgroundCardiac magnetic resonance (CMR) has been used to diagnose and risk-stratify patients with left ventricular noncompaction (LVNC). The prognostic value of CMR parameters for LVNC, especially feature tracking (CMR-FT), is not well known in LVNC patients with left ventricular dysfunction. The present study aimed to investigate whether the combination of CMR-FT with traditional CMR parameters can increase the prognostic value of CMR for LVNC patients with reduced left ventricular ejection fraction (LVEF).MethodsA total of 123 candidates were retrospectively included in this multicenter study and 55 LVNC patients (mean age, 45.7 ± 16.2 years; 61.8% men) remained after applying the exclusion criteria. Clinical features, left ventricular (LV) function parameters, global and segment myocardial strain, and late gadolinium enhancement (LGE) were evaluated. The outcomes include the composite events of cardiovascular death, heart transplantation, hospitalization for heart failure, thromboembolic events, and ventricular arrhythmias.ResultsAfter a median follow-up of 5.17 years (interquartile range: 0.17 to 10.58 years), 24 (36.8%) patients experienced at least one major adverse cardiovascular event (MACE). The myocardial strain parameters of patients with events were lower than those of patients without events. In the univariable Cox analysis, LVEF, the presence of LGE, global longitudinal strain (GLS) and segmental strains, including longitudinal strain at the apical level and radial and circumferential strain at the basal level, were significantly associated with MACEs. In the multivariate analysis, LGE (hazard ratio (HR) 3.452, 95% CI 1.133 to 10.518, p = 0.029) was a strong predictor of MACEs and significantly improved the predictive value (chi-square of the model after adding LGE: 7.51 vs. 13.47, p = 0.009). However, myocardial strain parameters were not statistically significant for the prediction of MACEs after adjusting for age, body mass index, LVEF and the presence of LGE and did not increase the prognostic value (chi-square of the model after adding GLS: 13.47 vs. 14.14, p = 0.411) in the multivariate model.ConclusionsThe combination of CMR-FT with traditional CMR parameters may not increase the prognostic value of CMR in LVNC patients with reduced LVEF, while the presence of LGE was a strong independent predictor of MACEs and significantly improved the predictive value.

Project description:Background This study explored the value of myocardial strain in the differential diagnosis of isolated left ventricular myocardial noncompaction (ILVNC) and dilated cardiomyopathy (DCM) using cardiac magnetic resonance (CMR) feature tracking technology. Methods This retrospective analysis was performed on consecutive patients (25 with ILVNC, 30 with DCM, and 30 healthy controls) presenting to Shanxi Cardiovascular Hospital. All ILVNC patients met echocardiographic and CMR criteria for ventricular non-compaction. All patients with DCM met the 2016 American Heart Association and 2018 Chinese Medical Association Cardiovascular Branch diagnostic criteria. cvi42 software (Circle Cardiovascular Imaging) was used to measure radial, circumferential, and longitudinal strain (LS) globally and in segments of the left ventricle. Analysis of variance was used to compare strains among groups and among different segments within the same group. Receiver operating characteristic (ROC) curves were used to evaluate the diagnostic efficacy of different parameters in ILVNC and DCM. Results Basal circumferential strain was lower in the DCM than in the ILVNC group (P=0.05). Both median and apical LS were lower in the ILVNC than in DCM group (P=0.02 and P=0.01, respectively). ROC curves showed that apical LS was the most effective in distinguishing ILVNC from DCM [area under the curve (AUC) =0.883; P<0.001; 95% CI: 0.850–0.977]. Comparing strains among different segments within the same group revealed that in DCM, the circumferential and LS of the apex were higher than those of the basal segment, which is consistent with the pattern in healthy controls; however, has no such regular pattern was seen in ILVNC. Conclusions Myocardial strain parameters are of considerable value in the differential diagnosis of ILVNC and DCM. Differences in patterns between ILVNC and DCM can be sensitively identified, providing more comprehensive information for early clinical diagnosis.

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Left ventricular noncompaction: a distinct cardiomyopathy or a trait shared by different cardiac diseases?

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Left ventricular noncompaction: a distinct cardiomyopathy or a trait shared by different cardiac diseases?

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