Left Ventricular Noncompaction Patients Research Articles

Long-term prognostic value of thyroid hormones in left ventricular noncompaction.

Thyroid function is closely related to the prognosis of cardiovascular diseases. This study aimed to explore the predictive value of thyroid hormones for adverse cardiovascular outcomes in left ventricular noncompaction (LVNC). This longitudinal cohort study enrolled 388 consecutive LVNC patients with complete thyroid function profiles and comprehensive cardiovascular assessment. Potential predictors for adverse outcomes were thoroughly evaluated. Over a median follow-up of 5.22years, primary outcome (the combination of cardiovascular mortality and heart transplantation) occurred in 98 (25.3%) patients. For secondary outcomes, 75 (19.3%) patients died and 130 (33.5%) patients experienced major adverse cardiovascular events (MACE). Multivariable Cox analysis identified that free triiodothyronine (FT3) was independently associated with both primary (HR 0.455, 95%CI 0.313-0.664) and secondary (HR 0.547, 95%CI 0.349-0.858; HR 0.663, 95%CI 0.475-0.925) outcomes. Restricted cubic spline analysis illustrated that the risk for adverse outcomes increased significantly with the decline of serum FT3. The LVNC cohort was further stratified according to tertiles of FT3 levels. Individuals with lower FT3 levels in the tertile 1 group suffered from severe cardiac dysfunction and remodeling, resulting in higher incidence of mortality and MACE (Log-rank P < 0.001). Subgroup analysis revealed that lower concentration of FT3 was linked to worse prognosis, particularly for patients with left atrial diameter ≥ 40mm or left ventricular ejection fraction ≤ 35%. Adding FT3 to the pre-existing risk score for MACE in LVNC improved its predictive performance. Through the long-term investigation on a large LVNC cohort, we demonstrated that low FT3 level was an independent predictor for adverse cardiovascular outcomes.

The Trouble with Trabeculation: How Genetics Can Help to Unravel a Complex and Controversial Phenotype.

Excessive trabeculation of the cardiac left ventricular wall is a complex phenotypic substrate associated with various physiological and pathological processes. There has been considerable conjecture as to whether hypertrabeculation contributes to disease and whether left ventricular non-compaction (LVNC) cardiomyopathy is a distinct pathology. Building on recent insights into the genetic basis of LVNC cardiomyopathy, in particular three meta-analysis studies exploring genotype-phenotype associations using different methodologies, this review examines how genetic research can advance our understanding of trabeculation. Three groups of genes implicated in LVNC are described-those associated with other cardiomyopathies, other cardiac/syndromic conditions and putatively with isolated LVNC cardiomyopathy-demonstrating how these findings can inform the underlying pathologies in LVNC patients and aid differential diagnosis and management in clinical practice despite the limited utility suggested for LVNC genetic testing in recent guidelines. The outstanding questions and future research priorities for exploring the genetics of hypertrabeculation are discussed.

Right ventricular global longitudinal strain is associated with cardiovascular outcome in left ventricular non-compaction phenotype

Abstract Introduction Speckle-tracking echocardiography is an increasingly important tool for assessing left ventricular non-compaction (LVNC) phenotype. This study aims to analyse right (RV) and left (LV) ventricular global longitudinal strain (GLS) in patients with LVNC and explore their association with CV outcome during long-term follow-up. Methods From our prospective LVNC cohort study, 40 patients were identified with a comprehensive echocardiographic examination including strain analysis of both ventricles. Strain was determined using TomTec ImageArena v.4.6. A combined endpoint (CV event) was defined to include: atrial flutter/fibrillation, sustained ventricular arrhythmias, cardiac syncope, thromboembolic event, heart failure progression, aborted cardiac arrest, cardiovascular death or heart transplantation. Results Over a median follow-up duration of 8 [6–10] years, 23 (58%) of the patients experienced one or more CV event (event group); while the rest (17 [42%]) did not (no event group). Baseline clinical characteristics of the two groups were comparable except for a significantly older age (P&amp;lt;0.01) and a higher BMI (P=0.033) in the event group. RVGLS (Figure 1) was significantly lower in the event group (-13.9[-17.1 – -10.3]%) compared to no-event group (-17.3[-18.8 – -15.1]%, P=0.019). While LVGLS did not show a significant difference (P=0.112), LVEF was significantly lower (P=0.011) in the event group. Other echocardiographic parameters were comparable between the groups. RVGLS &amp;gt; -14.3% (sens. 61%, spec. 88%, AUC 72%; P = 0.010) was applied as cut-off to identify patients with a higher risk of CV events during follow-up. Kaplan-Meier curves revealed a higher probability of CV events when RVGLS was &amp;gt; -14.3% (ꭓ2 = 10.28, P = 0.001; Figure 2). Univariable regression models showed that RVGLS (HR 1.10; P = 0.013), LVGLS (HR 1.23; P = 0.045), and LVEF (HR 0.95; P = 0.005) were associated with an increased risk of CV events. The association of RVGLS (OR 1.19, P = 0.047) remained independent of LV GLS and LVEF in a multivariable logistic regression analysis. When RVGLS was included to the LV function model, a significant improvement was noted (ꭓ2 from 7.31 to 11.58; ANOVA P = 0.009). Conclusions RVGLS differentiated LVNC patients suffering a CV event from those who did not and provided incremental value to the association of LV function (LVEF and LVGLS) with an increased risk of CV events. These findings highlight the potential value of RVGLS for evaluating long-term outcome during follow-up of LVNC patients.

Characteristics of the right ventricle in left ventricular noncompaction with reduced ejection fraction in the light of dilated cardiomyopathy.

Reports of left ventricular noncompaction (LVNC) rarely include descriptions of the right ventricle (RV). This study aimed to describe the characteristics of the RV in LVNC patients with reduced LV function (LVNC-R) compared with patients with dilated cardiomyopathy (DCM) and subjects with LVNC with normal left ventricular ejection fraction (LV-EF) (LVNC-N). Forty-four LVNC-R patients, 44 LVNC-N participants, and 31 DCM patients were included in this retrospective study (LV-EF: LVNC-R: 33.4±10.2%; LVNC-N: 65.0±5.9%; DCM: 34.6±7.9%). Each group was divided into two subgroups by the amount of RV trabeculation. There was no difference in the RV-EF between the groups, and the RV trabecular mass correlated positively with the RV volume and negatively with the RV-EF in all the groups. All the measured parameters were comparable between the groups with decreased LV function. The hypertrabeculated RV subgroups showed significantly higher RV volumes and lower RV-EF only in the decreased-LV-function groups. The correlation of LV and RV trabeculation was observed only in the LVNC-N group, while LV trabeculation correlated with RV volumes in both noncompacted groups. Both decreased-LV-function groups had worse RV strain values than the LVNC-N group; however, RV strain values correlated with RV trabeculation predominantly in the LVNC-R group. The presence and characteristics of RV hypertrabeculation and the correlations between LV trabeculation and RV parameters raise the possibility of RV involvement in noncompaction; moreover, RV strain values might be helpful in the early detection of RV function deterioration.

Long-Term Prognosis of Different Subtypes of Left Ventricular Noncompaction Cardiomyopathy Patients: A Retrospective Study in China.

Left ventricular noncompaction (LVNC) is a heterogeneous cardiomyopathy that can be classified into different subtypes based on morphologic and functional features. However, the prognosis of the dilated and isolated subtypes of non-pediatric LVNC remains unknown. We retrospectively studied 101 patients with LVNC diagnosed at Peking Union Medical College Hospital from 2006 to 2022 using the Jenni criteria of transthoracic echocardiography. The patients were grouped into those with dilated LVNC (n = 64) or isolated LVNC (n = 37), and 88 patients (54 with dilated LVNC and 34 with isolated LVNC) were followed up successfully. The primary outcome was major adverse cardiovascular events (a composite of cardiovascular mortality, heart failure, severe ventricular arrhythmia, and systolic embolism). The median follow-up time was 5.24 years. The incidence of major adverse cardiovascular events was 43.2%; patients with dilated LVNC had a higher risk (adjusted hazard ratio, 4.43; 95% confidence interval, 1.24-15.81; p = 0.02) than those with isolated LVNC. None of the isolated LVNC patients had cardiovascular deaths or severe ventricular arrhythmias. The risk of systemic embolism was similar between patients with dilated and isolated LVNC. Our findings indicate that transthoracic echocardiography is a useful tool for classifying LVNC into subtypes with distinct clinical outcomes. Dilated LVNC is associated with a poor prognosis, while the isolated subtype is probably a physiological condition.

Entropy as a novel predictor of cardiovascular events in patients with left ventricular noncompaction

BackgroundThe risk stratification of left ventricular noncompaction (LVNC) remains ambiguous. LV entropy derived from late gadolinium enhancement (LGE) in cardiac magnetic resonance (CMR) as a novel measurement of myocardial heterogeneity may serve as the substrate of major adverse cardiovascular events (MACEs). This retrospective study aimed to investigate the value of LV entropy for predicting MACEs in LVNC patients. MethodsConsecutive patients who underwent CMR and met the diagnosis criteria of LVNC were included. All patients were follow-up for MACEs (cardiac death, ventricular arrhythmia requiring therapy or heart failure hospitalization), and their LV entropy derived from the distribution of pixel signal intensities in the LGE of the LV myocardium was analyzed. ResultsOne hundred and forty-three patients (mean age 40 years, 64.3% male) were followed for a median of 3.2 years, and forty-two (29.4%) experienced MACEs. Presenting of symptoms, left ventricular end-diastolic diameter (LVEDD), LV end-diastolic volume (LVEDV) index, LV end-systolic volume (LVESV) index, LV ejection fraction (LVEF), LGE extent, and LV entropy showed association with MACEs. LV entropy maintained independent association with MACEs (HR: 4.76, 95%CI 3.68–5.15, p < 0.001) in multivariable analysis. Entropy was also strong independent predictor of MACEs in patients with and without LGE (HR: 5.89, 95% CI4.18–7.73, p < 0.001; HR: 3.06, 95% CI:1.53–4.80, p = 0.013, respectively). ConclusionsLV entropy can predict MACEs in LVNC patients and provide incremental prognostic value on top of LVEF and LGE. Also, LV entropy may help risk stratification in LGE-negative LVNC patients.

The impact of fragmented QRS on clinical findings and outcomes in children with dilated cardiomyopathy with or without left ventricular non-compaction.

The aim of this study is to investigate the frequency of fragmented QRS and its associations with clinical findings and prognosis in children diagnosed with dilated cardiomyopathy with or without left ventricular non-compaction. This retrospective study was conducted between 2010 and 2020. Patients with dilated cardiomyopathy were classified into two groups according to the presence of left ventricular non-compaction: Dilated cardiomyopathy with left ventricular non-compaction and dilated cardiomyopathy without left ventricular non-compaction. Patients were also divided into two groups according to the presence of fragmented QRS (fragmented QRS group and non-fragmented QRS group). Twenty-three of 44 patients (52.3%) were male. Among left ventricular non-compaction patients, the fragmented QRS group had more complex ventricular arrhythmias (p = 0.003). Patients with fragmented QRS had a significantly higher rate of major adverse cardiac events and/or cardiac death in both cardiomyopathy groups (p = 0.003 and p = 0.005). However, the rate of major adverse cardiac events and/or cardiac death was similar between dilated cardiomyopathy patients with and without left ventricular non-compaction. Multivariate logistic regression analysis showed that the presence of fragmented QRS strongly predicts major adverse cardiac events and/or cardiac death (odds ratio, 31.186; 95% confidence interval, 2.347-414.307). Although the survival rates between cardiomyopathy groups were similar, patients with fragmented QRS had a markedly lower survival rate during the follow-up period, as mean of 15 months (p = 0.001). Our study showed that the presence of fragmented QRS may be an important ECG sign predicting an major adverse cardiac event and/or cardiac death in patients with dilated cardiomyopathy. We believe that recognising fragmented QRS could be valuable in forecasting patient prognosis and identifying high-risk patients who require additional support.

Prognostic value of cardiac magnetic resonance imaging parameters in left ventricular noncompaction with left ventricular dysfunction

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.

Abstract 10124: Characteristics of the Right Ventricle in Left Ventricular Noncompaction With Reduced Ejection Fraction

Introduction: Reports of left ventricular noncompaction (LVNC) rarely include descriptions of the right ventricle (RV). Hypothesis: This study aimed to describe the characteristics of the RV in LVNC patients with reduced LV function (LVNC-R) compared with patients with dilated cardiomyopathy (DCM) and subjects with LVNC with normal left ventricular ejection fraction (LV-EF) (LVNC-N). Methods: Forty-four LVNC-R patients, 44 LVNC-N participants, and 31 DCM patients were included in this retrospective study (LV-EF: LVNC-R: 33.4±10.2%; LVNC-N: 65.0±5.9%; DCM: 34.6±7.9%). Each group was further divided into two subgroups by the amount of RV trabeculation. Results: There was no difference in the RV-EF between the groups, and the RV trabecular mass correlated positively with the RV volume and negatively with the RV-EF in all the groups. All the measured parameters were comparable between the groups with decreased LV function. Subgroups with RV hypertrabeculation showed significantly higher RV volumes and lower RV-EF in the decreased-LV-function groups than patients with normal RV trabeculation. LV trabeculation correlated with RV volumes in both noncompacted groups, while the correlation with RV trabeculation was observed only in the LVNC-N group (r=0.469, p=0.001). Both decreased-LV-function groups had worse RV strain values than the LVNC-N group; however, RV strain values correlated with RV trabeculation predominantly in the LVNC-R group. Conclusions: Correlations between LV trabeculation and RV parameters and the presence and characteristics of RV hypertrabeculation raise the possibility of RV involvement in noncompaction; moreover, RV strain values might be helpful in the detection of early subclinical changes of the RV.

Atrial fibrillation concerning worse prognosis in patients with left ventricular noncompaction

Abstract Purpose This study aims to investigate the relationship between atrial fibrillation (AF) and the prognosis of patients with left ventricular noncompaction (LVNC). Methods This retrospective study enrolled 302 patients (median age 46 years, 65.9% male) diagnosed as LVNC through echocardiography or cardiac nuclear magnetic according to Jenni criteria1 and Petersen criteria2, respectively. The primary endpoint is major cardiovascular adverse events (MACEs) which is a composite endpoint including heart transplantation, deaths in-hospital, stroke, system embolism, malignant ventricular arrhythmia, and rehospitalization for cardiovascular reasons. The second endpoints are thromboembolism events including system embolism and stroke. Results The patients with LVNC were divided into 2 groups: with AF group (n=56) and without AF group (n=246). In the group of patients combined with AF and LVNC, the proportion of CHA2DS2-VASc score≥2 was 66.07%. The rate of receiving oral anticoagulant therapy in LVNC patients with AF was significantly higher than without the AF group (69.64% vs. 19.51%, p&amp;lt;0.01). After a median follow-up of 4.3 years, a total of 69 MACEs (22.85%) and 19 thromboembolism events (6.3%) occurred. Among them, 49 MACEs in the non-AF group (19.92%) while 20 cases (35.71%) occurred MACEs in the AF group (p=0.01). Moreover, the rate of thromboembolism in the AF group was notably higher compared with the non-AF group (16.1% vs. 4.1%, p=0.01). The Kaplan-Meier curves (Figure 1) compared by the log-rank test showed that the AF group was associated with a higher risk of thromboembolism events than the non-AF group and the difference between the 2 groups was significant (p&amp;lt;0.05). Crude Logistic regression model results revealed that AF was associated with increased risk of MACEs in patients with LVNC [Crude odds ratio (OR) 2.23, 95% confidence interval (CI) 1.19–4.19, p=0.01]. Adjusted Logistic regression model indicated that AF was still an independent risk factor (adjusted OR 2.43, 95% CI 1.08–5.45, p=0.03) for MACEs after adjusting for other risk factors including age, heart rate, syncope, heart failure, NYHA class, cardio-pulmonary resuscitation history, hypertension, prior stroke, prior system embolism, family history of cardiomyopathy, levels of NT-pro-BNP, left atrial diameter, left ventricular ejection fraction, oral anticoagulant therapy and antiplatelet therapy. Conclusion This current study indicated that AF is associated with a worse prognosis in patients with LVNC. Extra attention is needed in clinical practice for LVNC patients with AF. Funding Acknowledgement Type of funding sources: None.

The predictive value of left atrial diameter for thrombotic risk in patients with left ventricular noncompaction

Abstract Purpose Patients with left ventricular noncompaction (LVNC) are associated with increased risk of thromboembolism including system embolism (SE) and stroke. Moreover, left atrial (LA) diameter was proved to be a strong predictor for stroke and SE [1]. This study aimed to evaluate the predictive value of left atrial diameter on the risk of thromboembolism in patients with LVNC. Methods This retrospective longitudinal study enrolled 302 patients (median age 46 years, 65.9% male) diagnosed as LVNC through echocardiography or cardiac nuclear magnetic according to Jenni criteria [2] and Petersen criteria [3], respectively. The primary endpoint was thromboembolism events including SE and stroke. Results The 302 patients with LVNC were divided into 2 groups: large LA group (LA diameter ≥43mm, n=155) and small LA group (LA diameter&amp;lt;43mm, n=147), according to the median of LA diameter. After a median follow-up of 4.3 years, 19 cases (6.3%) patients occurred thromboembolism events. In the large LA group, 16 patients (10.3%) occurred thromboembolism events while there were 3 cases (2.0%) suffering from SE/stroke in the small LA group (p&amp;lt;0.05). The Kaplan-Meier curves (Figure 1) compared by the log-rank test showed that the large LA group was associated with a higher risk of thromboembolism events and the difference between the 2 groups was significant (p&amp;lt;0.05). Crude Cox regression model results revealed that LA diameter was an independent risk factor for thromboembolism events in patients with LVNC (Crude HR 1.07, 95% CI 1.02–1.12, p=0.004). After adjusting for prior heart failure, NYHA class, atrial fibrillation, prior stroke, coronary artery disease, pulmonary arterial hypertension, family history of cardiomyopathy, LA diameter was still associated with increased thrombotic risk (Adjusted HR 4.98, 95% CI 1.07–23.04, p=0.04). Moreover, the ROC curves (Figure 2) showed that the AUC of LA diameter was 0.692 and even better than the CHA2DS2-VASc score (AUC=0.576). Conclusion The current study indicated that LA diameter is an effective predictor of thrombotic risk in patients with LVNC. Moreover, extra attention is needed in LVNC patients with larger LA diameters for the risk of thromboembolism. Funding Acknowledgement Type of funding sources: None.

Prognostic factors associated with left ventricular non-compaction: A PRISMA-compliant meta-analysis.

Background:Left ventricular non-compaction (LVNC) is a rare disease with a poor prognosis. Efforts to improve prognosis are limited by the quality and scope of the available evidence on prognostic factors.Methods:Pubmed, Embase, China National Knowledge Infrastructure, Cochrane Library, Wanfang, and Baidu Scholar were searched and all relevant studies that examined factors related to LVNC prognosis, published before January 2021, were retrieved. Study quality evaluation and data extraction were independently completed by two authors. Statistical analyses were performed using STATA 15.0 software.Results:A total of 20 cohort studies were included in this study, with a total of 1910 patients. The results of the meta-analysis are as follows: New York Heart Function Association (NYHA) class III/IV (hazard ratio [HR] = 3.93, 95% confidence interval [CI]: 1.66–9.29), (NT-proBNP) increased (HR = 1.98, 95% CI: 1.10–3.58), left ventricular ejection fraction (LVEF) decreased (HR = 1.04, 95% CI: 1.03–1.06), left ventricular end-diastolic diameter (LVEDD) increased (HR = 1.03, 95% CI: 1.01–1.06) was an independent poor prognostic factor, and body mass index (HR = 0.80, 95% CI: 0.64–0.98) was an independent protective factor. Creatinine (CR) level (HR = 1.09, 95% CI: 0.95–1.25) and late gadolinium-enhanced (LGE) imaging (HR = 3.1, 95% CI: 0.85–11.31) has no statistical significance in the prognosis of LVNC.Conclusion:In LVNC patients, NYHA class III/IV, elevated N-terminal pro-brain natriuretic peptide (NT-proBNP) levels, decreased LVEF, and increased LVEDD may lead to poor prognosis, and increased body mass index may improve the prognosis of LVNC. Further clinical research with large sample sizes and long-term follow-ups should be conducted.PROSPERO Registration Number:42020152706

Left Ventricular Noncompaction Cardiomyopathy: Left Ventricular Dilation and Dysfunction at Baseline Portend the Risk of Death or Heart Transplantation.

Left ventricular noncompaction (LVNC) is associated with genetic and phenotypic variability that influences outcomes. We aimed to identify risk factors for death or heart transplantation (HTx) in a paediatric LVNC cohort. We reviewed patients < 18 years of age (2001-2018) with LVNC, either isolated (I-LVNC) or with dilated phenotype (D-LVNC), and at least mildly reduced left ventricular ejection fraction (EF). Patients with dilated cardiomyopathy (DCM) were included as control subjects. Descriptive statistics, multivariate analysis, and time-to-event analysis were used. We included 188 patients, 34 (18%) with I-LVNC, 37 (20%) with D-LVNC, and 117 (62%) with DCM. Overall median age at diagnosis was 1.08 years (interquartile range [IQR] 0.22-10.65) and median follow-up was 1.4 years (IQR 0.2-5.2) years. I-LVNC patients' median baseline LVEF was 47%, compared with 33% for D-LVNC, and 21% for DCM (P < 0.0001); 62% of I-LVNC patients developed moderate to severe LV dysfunction during follow-up. The incidence of death or transplantation was 43.6% in the overall cohort. Freedom from death or HTx at 10 years after diagnosis was 88.6% (95% CI 76%-100%) for I-LVNC, 47% (95% CI 29%-65%) for D-LVNC, and 42.3% (95% CI 33%-52%) for DCM. On multivariable analysis, baseline LVEF and LV end-diastolic diameter (LVEDD) z-score were associated with death or transplantation. Patients with a baseline LVEDD z-score > 4 and moderate to severe LV dysfunction had a transplantation-free survival of 38%. Baseline LV dilation and systolic dysfunction were independently associated with progression to death or HTx in LVNC patients.

MR -specific characteristics of left ventricular noncompaction and dilated cardiomyopathy

BackgroundThe differentiation of dilated cardiomyopathy (DCM) and left ventricular noncompaction (LVNC) is a recurring issue during cardiac imaging processes; thus, we aimed to compare the left ventricular (LV) cardiac MRI characteristics of these patients. MethodsThirty-one nonischemic DCM patients, 42 LVNC patients with reduced ejection fraction and 42 healthy controls were included in this retrospective study. LV volumetric, functional and myocardial mass parameters were measured with a threshold-based technique, while global and segmental strain values and rotational patterns were analyzed with feature-tracking strain analysis. ResultsOf the LV volumetric and myocardial mass parameters, only the trabeculated and papillary muscle mass (TPMi) values differed significantly between the patient groups and were higher in the LVNC group (DCM vs LVNC: 43.2 ± 8.9 vs 51.6 ± 13.6 g/m2, p < 0.002). The global longitudinal and circumferential strains were similar between the patient groups and significantly worse than those of the controls. In comparing the segmental strains between the patient groups, only the circumferential apical strain was significantly lower in the LVNC group (DCM vs LVNC: −30.5 ± 13.5 vs −24.5 ± 12.0%, p < 0.05). There was no difference in the rotational pattern between the patient groups, and both healthy and patient populations showed heterogeneous rotational patterns. ConclusionsDespite the similarities between DCM and LVNC in volumetric, global strain parameters, and rotational patterns, we found some differences between the patient groups, as the TPMi was higher and the apical circumferential strains were significantly lower in LVNC. These minor alterations might be due to the morphological characteristics of LVNC with a trabeculated apical region.

Sacubitril-valsartan therapy in a patient with heart failure due to isolated left ventricular noncompaction: a case report and literature review

Abstract Background: Left ventricular noncompaction (LVNC) is a rare type of cardiomyopathy. The core clinical feature is heart failure that responds poorly to treatments. Case presentation: A 58-year-old woman received various treatments (including metoprolol, benazepril, torasemide, spirolactone, and digoxin) for 4 years for LVNC, but responded poorly. Upon presentation, transthoracic echocardiogram (ECHO) showed 26% left ventricular ejection fraction (LVEF) and class IV diastolic dysfunction. Upon cardiac magnetic resonance imaging (CMRI), the ratio of noncompacted versus compacted myocardium was 3.9. She received guideline-recommended treatments that included sacubitril-valsartan (100 mg/day) in addition to β-blocker, torasemide, spirolactone, digoxin, and isosorbide. Symptoms and signs improved rapidly, and she was discharged 1 week later. Sacubitril-valsartan dosage was adjusted to 200 mg/day 4 weeks later. She remained in relatively good health thereafter. At the last follow-up 16 months later, LVEF was 51% on ECHO. CMRI showed the significantly reduced ratio of 2.8 in noncompacted versus compacted myocardium. Conclusions: Sacubitril-valsartan therapy may result in reverse remodeling and improve long-term outcomes in LVNC patients.

MR -specific characteristics of left ventricular noncompaction and dilated cardiomyopathy

Abstract Funding Acknowledgements Type of funding sources: Public grant(s) – National budget only. Main funding source(s): Thematic Excellence Programme (Tématerületi Kiválósági Program, 2020-4.1.1.-TKP2020) of the Ministry for Innovation and Technology in Hungary within the framework of the Therapeutic Development and Bioimaging Programs of Semmelweis University Development of Scientific Workshops of Medical, Health Sciences and Pharmaceutical Edication (Project identification number: EFOP- 3.6.3-VEKOP-16- 2017-00009) Project no. NVKP_16-1–2016-0017 (’National Heart Program’) has been implemented with the support provided from the National Research, Development and Innovation Fund of Hungary, financed under the NVKP_16 funding scheme. The research was supported by the Ministry of Innovation and Technology NRDI Office within the framework of the Artificial Intelligence National Laboratory Program. The differentiation of dilated cardiomyopathy (DCM) and left ventricular noncompaction (LVNC) is a recurring issue during cardiac imaging processes; thus, we aimed to compare the left ventricular (LV) cardiac MRI (CMR) characteristics of DCM and LVNC patients. Thirty-one nonischemic DCM patients, 42 LVNC patients with reduced EF and 42 healthy controls were included in this retrospective study. The LV volumetric, functional and myocardial mass parameters were measured with a threshold-based technique, while global and segmental strain values and rotational patterns were analyzed with feature-tracking strain analysis. Of the LV volumetric and myocardial mass parameters, only the trabeculated and papillary muscle mass (TPMi) values differed significantly between the patient groups and were higher in the LVNC group compared to DCM (DCM vs LVNC: 43.2 ± 8.9 vs 51.6 ± 13.6 g/m2, p &amp;lt; 0.002). The global longitudinal and circumferential strains were similar between the patient groups and significantly worse than those of the controls. In comparing the segmental strain values between the patient groups, only the circumferential apical strain was significantly lower in the LVNC group (DCM vs LVNC: -30.5 ± 13.5 vs -24.5 ± 12.0%, p &amp;lt; 0.05). There was no difference in the rotational pattern between the patient groups, and both the healthy and patient populations showed heterogeneous rotational patterns. Despite the similarities of DCM and LVNC in volumetric, global strain parameters, and rotational patterns, we found some morphological and functional differences between the patient groups. These minor alterations might be due to the morphological characteristics of LVNC with a trabeculated apical region.

Left Ventricular Noncompaction Is Associated with Valvular Regurgitation and a Variety of Arrhythmias.

Left ventricular noncompaction (LVNC) is a type of cardiomyopathy characterized anatomically by prominent ventricular trabeculation and deep intertrabecular recesses. The mortality associated with LVNC ranges from 5% to 47%. The etiology of LVNC is yet to be fully understood, although decades have passed since its recognition as a clinical entity globally. Furthermore, critical questions, i.e., whether LVNC represents an acquired pathology or has a congenital origin and whether the reduced contractile function in LVNC patients is a cause or consequence of noncompaction, remain to be addressed. In this study, to answer some of these questions, we analyzed the clinical features of LVNC patients. Out of 9582 subjects screened for abnormal cardiac functions, 45 exhibit the characteristics of LVNC, and 1 presents right ventricular noncompaction (RVNC). We found that 40 patients show valvular regurgitation, 39 manifest reduced systolic contractions, and 46 out of the 46 present different forms of arrhythmias that are not restricted to be caused by the noncompact myocardium. This retrospective examination of LVNC patients reveals some novel findings: LVNC is associated with regurgitation in most patients and arrhythmias in all patients. The thickness ratio of the trabecular layer to compact layer negatively correlates with fractional shortening, and reduced contractility might result from LVNC. This study adds evidence to support a congenital origin of LVNC that might benefit the diagnosis and subsequent characterization of LVNC patients.

Comparison of cardiovascular magnetic resonance characteristics and clinical prognosis in left ventricular noncompaction patients with and without arrhythmia

BackgroundLeft ventricular noncompaction (LVNC) is a rare type of cardiomyopathy, and one of its clinical manifestations is arrhythmia. Cardiovascular magnetic resonance (CMR) is valuable for the diagnosis and prognosis of LVNC. However, studies are lacking on the use of CMR for LVNC patients with arrhythmia. This study aimed to characterize and compare CMR features and prognosis in LVNC patients with and without arrhythmia.MethodsEighty-four LVNC patients diagnosed by CMR were enrolled retrospectively in this study. Clinical data, arrhythmia characteristics, and CMR parameters were collected. Patients were divided into different groups according to the arrhythmia characteristics and CMR manifestations for statistical analysis and comparison. Ventricular tachycardia (VT), ventricular fibrillation (Vf), ventricular flutter (VFL), III° atrioventricular block (III° AVB), Wolff–Parkinson–White syndrome (WPW) and ventricular escape (VE) were defined as malignant arrhythmias and benign arrhythmias included premature ventricular contraction, atrial premature beats, atrial fibrillation, supraventricular tachycardia, supraventricular premature beat, bundle branch block, atrial flutter and sinus tachycardia. The outcome events were defined as a composition event of cardiac death, rehospitalization for heart failure, heart transplantation, and implantation of an implantable cardioverter defibrillator (ICD).ResultsSixty-seven LVNC patients (79.76%) mainly presented with arrhythmia, including premature ventricular beat (33 patients [27.73%]), bundle branch block (14 patients [11.77%]), electrocardiogram waveform changes (18 patients [15.13%]), and ventricular tachycardia (11 patients [9.24%]). The cardiac function and structure parameters had no significant difference among the nonarrhythmia group, benign arrhythmia group, and malignant arrhythmia group. However, the presence of late gadolinium enhancement (LGE) was higher in the malignant arrhythmia group than in the other two groups (p = 0.023). At a mean follow-up of 46 months, cardiac events occurred in twenty-three patients (46.94%). Kaplan–Meier analysis showed that there was no statistically significant difference in prognosis among the nonarrhythmia, benign, and malignant arrhythmia groups, but the patients with arrhythmia and association with LGE + or left ventricular ejection fraction (LVEF) < 30% had a higher risk than patients with LGE- or LVEF > 30% (LGE +, HR = 4.035, 95% CI 1.475–11.035; LVEF < 30%, HR = 8.131, 95% CI 1.805–36.636; P < 0.05).ConclusionsIn LVNC patients, the types of arrhythmias are numerous and unrepresentative, and arrhythmia is not the prognostic factor. Arrhythmia combined with presence of LGE or LVEF < 30% is associated with poor prognosis in LVNC patients.

Cardiac geometry, function and mechanics in left ventricular non-compaction cardiomyopathy with preserved ejection fraction

Left ventricular non-compaction (LVNC) cardiomyopathy in adults has primarily been studied with a phenotypic expression of low ejection fraction (EF) and dilated cardiomyopathy; however, data on LVNC with preserved EF is scarce. The present study aimed to evaluate cardiac geometry and mechanics in LVNC patients with preserved EF. A retrospective cohort study of patients diagnosed with LVNC and a preserved EF between 2008 and 2019 was performed. LVNC was defined according to the presence of established transthoracic 2D echocardiographic (TTE) criteria as follows: (1) prominent LV trabeculations with deep recesses; (2) bi-layered myocardial appearance; and, (3) systolic non-compacted:compacted ratio≥ 2. Subjects were matched 1:1 to controls without LVNC referred for routine TTE. Geometric, functional and mechanics parameters were analyzed in the two cohorts using 2D and speckle-tracking TTE. Seventeen patients with LVNC and preserved EF were identified. Compared with controls, patients with LVNC had similar LV systolic function and chamber dimensions, but a larger mass and relative wall thickness, and more abnormal LV geometry (76% vs. 18%, p = 0.002), LA remodeling, and pulmonary hypertension. Global longitudinal strain was significantly decreased (-15.4 ± 3.2 vs. -18.9 ± 2.8%, p = < 0.01) and the prevalence of rigid body rotation was significantly increased (57% vs. 14%, p = 0.05) in the LVNC population. The peak twist values were comparable in both cohorts. Impaired LV geometry and longitudinal mechanics, as well as increased myocardial stiffness as expressed by rigid body rotation, characterize LVNC with preserved EF when compared with controls.

Clinical Characteristics and Prognosis of Fetal Left Ventricular Noncompaction in Japan

Left ventricular noncompaction (LVNC) is morphologically characterized by numerous prominent trabeculations and a severely thickened, two-layered myocardium. The fetal onset of LVNC has rarely been described.Methods and Results:We conducted nationwide retrospective surveys on fetal cardiomyopathy (CM) in Japan from 2010 to 2016, from which 38 fetal patients with CM were enrolled, including 16 patients with LVNC. The rate of diagnostic concordance was 56.3% between fetal and postnatal visits in LVNC patients. The increase in the ratio of noncompacted to compacted (N/C) myocardium was time-dependent throughout the fetal period till birth (LV lateral: 1.6±0.1 to 2.8±0.2; LV apex: 2.0±0.1 to 3.2±0.2). Of all fetuses, 16 (42.1%) died or underwent heart transplantation (HT), with 3 intrauterine deaths. Lower fetal cardiovascular profile score (odds ratio, 26.9; P=0.0266) was a risk factor for death or HT. N/C ratio ≥1.6 at the apex at the first visit was a significant predictor of LVNC (odds ratio, 47.8; P=0.0113).This is the first study to reveal the etiology of fetal CM based on results from a nationwide survey in Japan, highlighting the difficulty of diagnosing LVNC in fetal patients. To better understand and manage fetal CM, novel diagnostic criteria of LVNC in fetus should be established.