Number Of Non-compacted Segments Research Articles

Analysis of left ventricular systolic function in patients with isolated ventricular noncompaction by cardiac MRI

Objective To compare cardiac magnetic resonance imaging (MRI) features between patients with normal and decreased left ventricular (LV) systolic function in isolated ventricular noncompaction (IVNC) and to analyze the related factors of LV systolic function. Methods In a retrospective blinded study, 62 IVNC patients were divided into two subgroups: those with normal LV systolic function [LV ejection fraction (LVEF) ≥55%) ] and those with decreased LV systolic function (LVEF< 55%). A total of 30 healthy volunteers were enrolled as controls. The LVEF, LV volume, number of non-compacted segments, ratio of non-compacted to compacted myocardium (NC/C ratio) , and delayed enhancement were assessed using cardiac MRI. One-way analysis of variance, independent t test and χ2 test were used. The correlation between LVEF and the number of non-compacted segments or NC/C ratio were calculated with Spearman and Pearson correlation analysis. Results The LVEF of patients with normal LVEF [ (58.8±3.8) %] and patients with decreased LVEF [ (31.1±11.1) %] were less than that of control group [ (66.1±6.0) %], which demonstrated significant differences (F=140.789, P 0.05). The patients with decreased LVEF had significantly greater LV volume compared with patients with normal LVEF and control group (P<0.05). The number of non-compacted segments in patients with decreased LVEF (7.5±1.0) was more than that of the patients with normal LVEF (5.6± 1.0) , and the NC/C ratio in patients with decreased LVEF (4.4±0.9) was higher than that of patients with normal LVEF (3.4±0.7) , which demonstrated significant differences (t=7.248, 4.472, P<0.01). There was significant difference in the rate of delayed enhancement between patients with decreased LVEF (18/42) and patients with normal LVEF (1/20) (χ2=9.136, P<0.01). The non-compacted segments were more common in apex, apical and mid lateral segments in two IVNC groups, and there were no involvement of basal septal, mid septal and mid inferior segments. Some basal segments were involved in patients with decreased LVEF, while there were no basal segments involved in patients with normal LVEF. The number of non-compacted segments and NC/C ratio were correlated negatively with LVEF (r=- 0.690, - 0.591, respectively,P<0.01). Conclusions The dilation, remodeling and myocardial delayed enhancement of LV in IVNC patients can lead to decreased LV systolic function. In addition, the extent and severity of non-compacted myocardium in IVNC patients are correlated negatively with LVEF. Key words: Cardiomypathies; Ventricular dysfunction, left; Magnetic resonance imaging

Effects of cardiac resynchronization therapy on left ventricular remodeling and dyssynchrony in patients with left ventricular noncompaction and heart failure.

Left ventricular noncompaction (LVNC) is a rare cardiomyopathy with high incidence of heart failure (HF). It is unclear whether LVNC patients with desynchronized HF would benefit from cardiac resynchronization therapy (CRT). In order to evaluate the effect of CRT on LVNC, this study explored left ventricular (LV) remodeling and mechanical synchronicity before and after CRT in LVNC patients, and compare with that in idiopathic dilated cardiomyopathy (DCM) patients. We collected 15 LVNC and 30 matched DCM patients. All the patients underwent clinical evaluation,electrocardiogram and echocardiography before CRT and ≥6 months later. LV response was defined as ≥15 % decrease in LV end-systolic volume (LVESV). Longitudinal synchronicity was quantified by YU-index using tissue Doppler imaging. The time delay of peak radial strain from anteroseptal to posterior wall, which derived from speckle tracking imaging, was used to quantify radial synchronicity. In LVNC group, LV ejection fraction increased from 27.6 ± 5.5 to 39.1 ± 7.0 % (P < 0.01) during follow-up, but LV volumes did not change significantly (both P > 0.05). Five LVNC patients (33.3 %) responded to CRT, and all of them were super-responders (reduction in LVESV > 30 %). In addition, the number of noncompacted segments and the thickness ratio of noncompacted to compacted myocardium decreased (both P < 0.05). Inter-ventricular, longitudinal and radial intra-ventricular dyssynchrony also reduced significantly (all P < 0.05). Compared with DCM group, there was no significant difference in LV response rate (33.3 vs. 60.0 %, P = 0.092), improvement of LV function and dyssynchrony index (all P < 0.05). In conclusion, CRT improved heart function, morphology and mechanical dyssynchrony in LVNC patients.

Electrocardiographic findings in correlation to magnetic resonance imaging patterns in African patients with isolated ventricular noncompaction.

Objective:Isolated ventricular noncompaction is a rare primary genetic cardiomyopathy characterized by persistent embryonic myocardial morphology without any other cardiac anomalies. Arrhythmias are frequently present, including both tachyarrhythmia and conduction disturbance. Our study aimed to describe the electrocardiographic findings and to correlate them with the clinical presentation and cardiac magnetic resonance imaging findings.Methods:We retrospectively reviewed 24 patients diagnosed with isolated ventricular noncompaction (IVNC) by cardiac magnetic resonance imaging. Correlations were investigated between arrhythmias and the site of ventricular noncompaction, number of noncompacted segments, presence of fibrosis, and left ventricular dysfunction.Results:The mean age was 42.7±13.1 years. Patients were first presented with heart failure in 41.7% and arrhythmia in 45.8%. Electrocardiogram was abnormal in 91.6% of patients; the most common anomaly was left bundle branch block (LBBB) (41.7%), followed by supraventricular arrhythmias (29.1%), repolarization abnormalities (29.1%), and ventricular tachycardia (20.8%). A normal left ventricular systolic function was frequently observed in patients who first presented with rhythm disorders than heart failure (p=0.008). There was also a delayed diagnosis of IVNC when presented with arrhythmia versus heart failure (p=0.02). We found no correlation between arrhythmias and the noncompaction site or fibrosis, except for LBBB, which was associated to left ventricle lateral wall involvement (p=0.028). No correlation between systolic dysfunction and the number of noncompacted segments, fibrosis, or arrhythmia was demonstrated.Conclusion:While electrocardiographic abnormalities are frequent in isolated ventricular noncompactison, no specific patterns were identified. More large studies are needed for stratification of arrhythmic risk of this highly arrhythmogenic substrate.

095: Segmental and global peak systolic longitudinal strain using speckle myocardial echocardiography in isolated left ventricular noncompaction. Description of 28 cases

Isolated left ventricular noncompaction (ILVNC) is a rare cardiomyopathy characterized by deep trabeculations in the ventricular wall which define recesses with the main chamber. The aim of our study was to compare segmental and global peak systolic longitudinal strain (pSSL) in ILVNC considering various left ventricular ejection fraction (LVEF). 28 patients (17-74 years old, mean age 46±16) with ILVNC were retrospectively enrolled and compared to 40 controls (group 1). Patients were divided into two groups according to LVEF group 2=LVEF>50% (n=15) and group 3 = LVEF<50% (n=13). pSSL was compared in each 17 left ventricular segments of the 3 groups. Number of non compacted segments was higher in group 3 than in group 2 (5.9±1.7 vs 4.6 ±1.3, p=0.008). Tissue Doppler Imaging E’ was lower in group 3 compared with group 1 (p<0.001) whereas E’ was not different between groups 1 and 2. Global pSSL (-15.75±4.8 and -22.18±1.9 p<0.001), basal mean (p<0.001), middle mean (p<0.001) and apex mean (p<0.001) were lower in patients with ILVNC compared to group 1. Basal mean (p=0.013), middle mean (p<0.001), apex mean (p<0.001) and global pSSL (-19.07±2.3 and -22.18±1.9 p<0.001) were lower in group 2 compared to controls. Basal pSSL of normally compacted basal segment in ILVNC were lower compared to basal pSSL in controls (septum p<0.001; lateral, p=0.009; inferior, p=0.008; anterior, p=0.009). There was a significant correlation between the number of non-compacted segments and the global pSSL (r=-0.418, p=0.027). Global left ventricular pSSL is reduced in patients with ILVNC even those with normal LVEF. Even in basal normally compacted segments, segmental correspondent pSSL is lower in patients with ILVNC compared to controls. This finding suggests that ILVNC is a diffuse left ventricular cardiomyopathy not only affected non compacted segments. Our results suggest that pSSL could be an early predictor of left ventricular systolic dysfunction in ILVNC.

Varied distributions of late gadolinium enhancement found among patients meeting cardiovascular magnetic resonance criteria for isolated left ventricular non-compaction.

BackgroundLate gadolinium enhancement (LGE) is identified frequently in LVNC. However, the features of this findings are limited. The purpose of the present study was to describe the frequency and distribution of LGE in patients meeting criteria for left ventricular non-compaction (LVNC), as assessed by cardiovascular magnetic resonance (CMR).MethodsForty-seven patients (37 males and 10 females; mean age, 39 ± 18 years) considered to meet standard CMR criteria for LVNC were studied. The LGE images were obtained 15 ± 5 min after the injection of 0.2 mmol/kg of gadolinium-DTPA using an inversion-recovery sequence, and analyzed using a 17-segment model.ResultsMean number of non-compacted segments per patient was 7.4 ± 2.5 and the NC:C was 3.2 ± 0.7. Non-compaction was most commonly noted in the apical segments in all patients. LGE was present in 19 of the 47 patients (40%), and most often located in the ventricular septum. The distribution of LGE was subendocardial (n = 5; 6%), mid-myocardial (n = 61; 68%), subepicardial (n = 10; 11%), and transmural (n = 14; 15%) in total of 90 LGE (+) segments.ConclusionsIn patients considered to meet criteria for LVNC, LGE distributions visible were strikingly heterogeneous with appearances potentially attributable to three or more distinct cardiomyopathic processes. This may be in keeping with previous suggestions that the criteria may be of low specificity. Further work is needed to determine whether conditions such as dilated cardiomyopathy, previous myocardidtis or ischaemic heart disease increase the apparent depth of non-compact relative to compact myocardium.

Insights from magnetic resonance imaging of left ventricular non-compaction in adults of North African descent

BackgroundLeft ventricular non-compaction (LVNC) is a recently recognized rare disorder. Magnetic resonance imaging (MRI) may help to clarify the uncertainties related to this genetic cardiomyopathy. Despite the fact that many articles have been published concerning the use of MRI in the study of LVNC, there is a lack of data describing the disease in the North African population. The aim of our study is to clarify MRI findings of LVNC in North African patients.MethodsIn our retrospective cohort, twelve patients (7 male, mean age 53 ± 8 years) underwent MRI for suspected LVNC. Correlations were investigated between the number of non-compacted segments per patient and left ventricular ejection fraction (LVEF), then between the number of non-compacted segments and left ventricular end diastolic diameter. The presence or absence of late gadolinium enhancement (LGE) was qualitatively determined for each left ventricular myocardial segment.ResultsNon-compaction was more commonly observed at the apex, the anterior and the lateral walls, especially on their apical and mid-cavity segments. 83% of patients had impaired LVEF. There was no correlation between the number of non-compacted segments per patient and LVEF (r = -0.361; p = 0.263), nor between the number of non-compacted segments per patient and left ventricular end diastolic diameter (r = 0.280; p = 0.377). LGE was observed in 22 left ventricular segments. No association was found between the pattern of fibrosis and non-compaction distribution (OR = 2.2, CI [0.91-5.55], p = 0.076).ConclusionThe distribution of LVNC in North African patients does not differ from other populations. Ventricular dysfunction is independent from the number of non-compacted segments. Myocardial fibrosis is not limited to non-compacted areas but can extend to compacted segments.

Comparison of cardiac magnetic resonance imaging features of isolated left ventricular non-compaction in adults versus dilated cardiomyopathy in adults

To compare cardiac magnetic resonance imaging (MRI) features between isolated left ventricular non-compaction (IVNC) and dilated cardiomyopathy (DCM) in adults. A consecutive series of 50 patients with IVNC from a single institution were reviewed. During the same period, 50 patients with DCM who had prominent trabeculations, who were matched for age, gender, and body surface area, were prospectively included. Left ventricular (LV) morphology and function were assessed using cardiac MRI. Compared with patients with DCM, patients with IVNC had a significantly lower LV sphericity index and end-diastolic volume index (LVEDVI) and a greater LV ejection fraction (LVEF), number of trabeculated segments, and ratio of non-compacted to compacted myocardium (NC/C ratio). There were no significant differences in stroke volume index, cardiac output, and cardiac index between the two patient groups. In patients with IVNC, the number of trabeculated segments and the NC/C ratio correlated positively with LVEDVI (r=0.626 and r=0.559, respectively) and negatively with LVEF (r=-0.647 and r=-0.521, respectively, p<0.001 for all). In patients with DCM, the number of non-compacted segments and the NC/C ratio had no correlation with either the LVEDVI (r=-0.082 and r=-0.135, respectively) or the LVEF (r=0.097 and r=0.205, respectively). There are demonstrable morphological and functional differences between IVNC and DCM at LV assessment using cardiac MRI. The occurrence of trabeculated myocardium might be due to a different pathophysiological mechanism.

Cardiac magnetic resonance imaging characteristics of isolated left ventricular noncompaction in a Chinese adult Han population

To analyze cardiac magnetic resonance imaging (CMR) characteristics in patients with isolated left ventricular noncompaction (IVNC) and assess its value in the diagnosis of IVNC in a Chinese adult Han population. We collected a consecutive series of 30 patients with IVNC from January 1, 2007, to December 31, 2008. During the same period, we prospectively included patients drawn from groups given a potential differential diagnosis for IVNC. All magnetic resonance images were analyzed using 17-segment model. Left ventricular ejection fraction was significantly lower for patients with DCM (16.2±5.2%, P<0.001) and higher in AR (47.6±16.2%, P=0.009), AS (54.6±21.1%, P=0.001) and HHD (62.4±6.8%, P<0.001) compared with IVNC (33.0±14.1%). The two-layered structure was most frequently seen at the apical segments, followed by the mid-cavity and basal segments in patients with INVC. The anterior and lateral walls were more commonly involved in patients with IVNC. The number of noncompacted segments and end-diastolic ratio of non-compacted to compacted myocardium (NC/C ratio) was greater in patients with IVNC than in other five groups. The end-diastolic NC/C ratio of >2.5 had 96.4% sensitivity and 97.4% specificity for identifying patients with IVNC. CMR provides an accurate and reliable evaluation of the localization and extent of noncompacted myocardium at end-diastole. The end-diastolic NC/C ratio of >2.5 had high diagnostic accuracy for IVNC in a Chinese adult Han population.

Ventricular dysfunction and number of non compacted segments in non compaction: Non-independent predictors

Background Isolated ventricular noncompaction (IVNC) is characterized by multiple prominent trabeculations and deep intertrabecular recesses. Some reports prove that the chronic heart failure may occur in approximately half of the patients. In this report we investigate the correlation between the number of non compacted segments and entity of systolic dysfunction from the registry and subregistries of the SIEC. Method To identify the correlation between ventricular dysfunction and number of segments involved in non compaction we evaluated a consecutive series of 238 patients affected by non compaction, from the SIEC (Società Italiana di Ecografia Cardiovascolare) registry. The average age of patients was 41.5 years (range: 1–92 years), 137 were males and 101 females. In 122 cases we found ventricular systolic dysfunctions with an EF average of 34.6%. The number of affected segments by non-compactation and diastolic dysfunction were found to be non-independent predictors of LV systolic dysfunction. Conclusion From the analyses we carried out, it seems that ventricular dysfunction seems to be completely independent from the segment numbers of non compacted segments.

Reduced regional systolic function is not confined to the noncompacted segments in noncompaction cardiomyopathy

Background Isolated ventricular noncompaction (IVNC) is a relatively rare genetic primary cardiomyopathy. The aim of the present study was to investigate with regional real-time three-dimensional echocardiographic analysis whether there is a difference between the contribution of noncompacted and compacted left ventricular (LV) segments to global LV dysfunction in patients with IVNC. Methods The study comprised 289 segments of 17 patients with stringent diagnostic criteria for IVNC. Their results were compared to 153 segments of 9 control subjects. The systolic performance of compacted and noncompacted LV segments was assessed using the wall motion score during 2D echocardiography. The 3D images were acquired with a RT3DE system with X4 matrix-array transducer and were used for the regional volume measurements. Results Wall motion score index was markedly abnormal in the compacted LV segments of IVNC patients but significantly less abnormal compared to the noncompacted segments (2.21 ± 0.63 vs. 2.01 ± 0.74, p < 0.05). No relationship was found between the number of noncompacted segments per patient and LV ejection fraction or end-diastolic volume. In the IVNC patients, noncompacted and compacted LV segments had comparable increased 3D regional volumes and reduced systolic function. Conclusions These results suggest that systolic LV dysfunction observed in IVNC is not confined to noncompacted LV segments.

Alterations in aortic elasticity in noncompaction cardiomyopathy

BackgroundNoncompaction cardiomyopathy (NCCM) is a recently recognized disorder frequently associated with systolic and diastolic heart failures. This study was designed to examine aortic stiffness in NCCM patients and to compare these results to age- and gender-matched controls.MethodsA total of 20 patients with typical echocardiographic features of NCCM (age 38 ± 16 years, eight males) were investigated. Their results were compared to 20 age- and gender-matched controls. All subjects underwent a complete two-dimensional transthoracic echocardiographic examination. Systolic (SD) and diastolic (DD) ascending aortic diameters were recorded in M-mode at a level of 3 cm above the aortic valve from a parasternal long-axis view. Aortic stiffness index (β) was calculated as a characteristic of aortic elasticity, as ln(SBP/DBP)/[(SD - DD)/DD], where SBP and DBP are the systolic and diastolic blood pressures, respectively, and ln is the natural logarithm.ResultsThe number of noncompacted segments in the NCCM patients was 4.6 ± 2.0. NCCM patients had significantly increased left ventricular dimensions and reduced left ventricular ejection fraction. Compared to controls, aortic stiffness index (β) was significantly increased in NCCM patients (8.3 ± 5.2 vs. 3.5 ± 1.1, p < 0.001).ConclusionIncreased aortic stiffness can be observed in patients with NCCM with moderate to severe heart failure. These alterations may be due to neurohormonal changes in heart failure.

Paradoxical Contributions of Non-Compacted and Compacted Segments to Global Left Ventricular Dysfunction in Isolated Left Ventricular Noncompaction

The pathophysiologic mechanisms of left ventricular (LV) dysfunction in isolated ventricular noncompaction (IVNC) remain unclear. Evaluating global and segmental systolic LV function in 65 patients with IVNC, this study found that normal wall motion was more common in noncompacted than in compacted segments. The number of noncompacted segments per patient correlated positively with the LV ejection fraction and negatively with LV end-diastolic volume index. These paradoxical findings support the concept that noncompaction represents a marker of a more generalized (cardio)myopathy rather than the direct pathophysiologic substrate of this still little-understood disease.