PurposeDiagnostic criteria for left ventricular non-compaction (LVNC) are still a matter of dispute. The aim of our present study was to test the diagnostic value of two novel diagnostic cardiac magnetic resonance (CMR) parameters: proof of non-compact (NC) myocardium blood flow using T2 sequences and changes in geometry of the left ventricle.Materials and MethodsThe study included cases with LVNC and controls, from a data base formed in a period of 3.5 years (n=1890 exams), in which CMR protocol included T2 sequences. Measurement of perpendicular maximal and minimal end diastolic dimensions in the region with NC myocardium from short axis plane was recorded, and calculated as a ratio (MaxMinEDDR), while flow through trabecula was proven by intracavital T2-weighted hyperintensity (ICT2HI). LVNC diagnosis met the following three criteria: thickening of compact (C) layer, NC:C>2.3:1 and NC>20%LV.ResultsThe study included 200 patients; 71 with LVNC (35.5%; i.e., 3.76% of CMRs) and 129 (64.5%) controls. MaxMinEDDR in patients with LVNC was significantly different from that in controls (1.17±0.08 vs. 1.06±0.04, respectively; p<0.001). MaxMinEDDR >1.10 had sensitivity of 91.6% [95% confidence intervals (CI) 82.5–96.8], specificity of 85.3% (95% CI 78.0–90.0), and area under curve (AUC) 0.919 (95% CI 0.872–0.953; p<0.001) for LVNC. Existence of ICT2HI had sensitivity of 100.0% (95% CI 94.9–100.0), specificity of 91.5% (95% CI 85.3–95.7), and AUC 0.957 (95% CI 0.919–0.981; p<0.001) for LVNC.ConclusionTwo additional diagnostic parameters for LVNC were identified in this study. ICT2HI and geometric eccentricity of the ventricle both had relatively high sensitivity and specificity for diagnosing LVNC.

BackgroundThere are still ambiguities existing in regard to left ventricular non-compaction (LVNC) diagnostic imaging. The aim of our study was to analyze diagnostic potential of late gadolinium enhancement (LGE) and ventricle geometry in patients with LVNC and controls.MethodsData on cardiac magnetic resonance imaging (CMR) studies for LVNC were reassessed from the hospital’s database (3.75 years; n=1975 exams). Matching sample of controls included cases with no structural heart disease, hypertrophic or dilative cardiomyopathy, arrhythmogenic right ventricular dysplasia or subacute myocarditis. Eccentricity of the left ventricle was measured at end diastole in the region with pronounced NC and maximal to minimal ratio (MaxMinEDDR) was calculated.ResultsStudy included 255 patients referred for CMR, 100 (39.2%) with LVNC (prevalence in the studied period 5.01%) and 155 (60.8%) controls. Existing LGE had sensitivity of 52.5% (95%-CI:42.3–62.5), specificity of 80.4% (95%-CI:73.2–86.5) for LVNC, area under curve (AUC) 0.664 (95%-CI:0.603–0.722);p<0.001. MaxMinEDDR>1.10 had sensitivity of 95.0% (95%-CI:88.7–98.4), specificity of 82.6% (95%-CI: 75.7–88.2) for LVNC, AUC 0.917 (95%-CI:0.876–0.948); p<0.001. LGE correlated with Max-Min-EDD-R (Rho=0.130; p=0.038) and there was significant difference in ROC analysis ΔAUC0.244 (95%-CI:0.175–0.314); p<0.001. LGE also correlated negatively with stroke volume and systolic function (both p<0.05, respectively).ConclusionsLGE was found to be frequently expressed in patients with LVNC, but without sufficient power to be used as a discriminative diagnostic parameter. Both LGE and eccentricity of the left ventricle were found to be relatively solid diagnostic landmarks of complex infrastructural and functional changes within the failing heart.

BackgroundDilatation and other infrastructural rearrangements of the left ventricle are connected with poor prognosis. The aim of our study was to analyze the overlapping phenotypes and dilatation of the ventricle on impairment of systolic function and existence of late gadolinium enhancement (LGE).Material/MethodsConsecutive sample of cases with dilated left ventricle due to non-ischemic cardiomyopathy and healthy controls were included from our cardiac magnetic resonance imaging (CMR) database for a period of 3 years (n=1551 exams).ResultsThe study included 127 patients; 30 (23.6%) with dilated cardiomyopathy (DCM); 30 (23.6%) with left ventricular non-compaction (LVNC); 13 (10.2%) with hypertrophic cardiomyopathy (HCM), and 50 (39.4%) controls. Overlapping phenotypes were found in 48 (37.8%) of the studied cases. Odds for impairment of systolic function in connection with overlapping phenotypes were estimated at 7.8 (95%-CI: 3.4–17.6), (p<0.001). There were significant differences in geometric parameters for patients with overlapping phenotypes vs. controls, as follows: left ventricle end-diastolic dimension(LVEDD)=6.6±0.8 vs. 5.6±1.0 cm (p<0.001); left ventricular ejection fraction (LVEF)=39.3±14.0 vs. 52.1±16.1 (p<0.001); and existence of LGE 36 (75.0%) vs. 21 (26.6%), (p<0.001), respectively. Overlapping phenotypes correlated with LVEDD (Spearman’s-Rho-CC)=0.521, p<0.001; LVEF (Rho-CC)=−0.447, p<0.001 and LGE (Rho-CC)=0.472, p<0.001.ConclusionsThis study found there are many patients with overlapping phenotypes among NICMPs with dilated left ventricles. Overlapping phenotype was associated with greater LVEDD, lesser systolic function, and commonly existing LGE, which all impose increased cardiovascular risk. Linear midventricular LGE stripe was the most powerfully connected with loss of systolic function.

BackgroundThe effects of focal hypertrophy on geometry of the left ventricle and systolic function have not been studied in patients with hypertrophic cardiomyopathy (HCM), despite the fact that the former is the most prominent disease characteristic. The aim of our study was to analyze systolic function over ventricle geometry, generating a functional index made from left ventricle end diastolic dimension (LVEDD) divided by end diastolic thickness of the region with maximal extent of hypertrophy and interventricular septum.Material/MethodsOur hospital database of cardiac magnetic resonance was screened for HCM. Geometric functional index (GFI) was calculated for LVEDD over maximal end diastolic thickness (MaxEDT) giving GFI-M, while LVEDD over interventricular septum was expressed as GFI-I. There were 55 consecutive patients with HCM.ResultsThere were 43 males (78.2%) and 12 females (21.8%). The mean age was 52.3±16.7 years (range: 15.5–76.4 years). A significant difference of GFI was found for preserved versus impaired systolic function of the left ventricle (preserved systolic function); GFI-M 2.28±0.60 versus 3.66±0.50 (p<0.001), and GFI-I 2.75±0.88 versus 3.81±0.87 (p<0.001), respectively. Diagnostic value was tested using receiver operating curve (ROC) analyzes, with GFI-M area under curve (AUC)=0.959 (95% CI: 0.868–0.994); (p<0.001) and GFI-I-AUC=0.847 (0.724–0.930); (p<0.001). GFI-M was superior to GFI-I for appraisal of left ventricle systolic dysfunction in HCM; ΔAUC=0.112 (0.018–0.207); (p=0.020).ConclusionsGFI is a simple tool, with high sensitivity and specificity for detecting impairment of systolic function in patients with HCM. Further studies would be necessary to investigate its clinical and prognostic impacts, as well as reproducibility with prospective validation.

LITERATURE 1.Murphy RT, Thaman R, Blanes JG, Ward D, Sevdalis E, Papra E, et al. Natural history and familial characteristics of isolated left ventricular noncompaction. Eur Heart J. Hypertrophic cardiomyopathy (HCM) and left ventricular noncompaction (LVNC) are both genetically determined and familial diseases. Hypertrophic cardiomyopathy (HCM) is defined as hypertrophy of the myocardium more than 1.5cm, without another identifiable cause, such as long-standing hypertension, amyloidosis, aortic stenosis, glycogen storage disease. Many of the mutations associated with HCM involve the cardiac sarcomeric proteins and include actin, myosin, or troponin component of the sarcomere and it is most frequently transmitted as an autosomal dominant trait. Left ventricular noncompaction is a rare congenital cardiomyopathy which is characterized by the presence of a thin, compacted epicardial layer and a non-compacted thicker endocardial layer of myocardium, with prominent trabeculation and deep recesses communicating with the cavity of the left ventricle. The cause of the disorder has been identified as mutations in genes associated with the mitochondrial function, like G4.5 which encodes the protein tafazzin, genes related with the cytoskeleton, like those of alpha-dystrobrevin or dystrophin, genes that code proteins of the Z line of the sarcomere, like LDB3, which codes the protein Cypher/ZASP, genes of the internal nuclear membrane proteins (LMNA, which encodes lamin A/C) and even genes that code sarcomeric proteins like cardiac alpha-actin and the beta-myosin heavy chain and cardiac troponin T. The clinical picture of both diseases, HCM and LVNC, varies from mild forms until severe forms with heart failure and complex ventricular arrhythmias. LVNC and HCM may appear as overlapping entities. Cases of patients sharing both the LVNC and HCM phenotypes have been already published, and it is speculated that mutations in sarcomere protein genes known to cause hypertrophic cardiomyopathy and dilated cardiomyopathy may be associated with left ventricular noncompaction. 1-5 In our case report, we are presenting patient with clear overlapping pheenotyp for LVNC and HCM, using the imaging method cardiac MRI.