Noncompaction Cardiomyopathy Patients Research Articles

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.

Prevalence and clinical correlates of ascending aortic dilatation in patients with noncompaction cardiomyopathy

Ascending aortic (AoAsc) dilatation can lead to acute aortic syndromes and has been described in various familial cardiac diseases. Its prevalence and clinical significance in patients with noncompaction cardiomyopathy (NCCM) are however unknown. Establishing the prevalence can facilitate recommendations on routine screening in NCCM. In this cross-sectional cohort study based on the Rijnmond Heart Failure/Cardiomyopathy Registry, the patient were enrolment between 2014 and 2021. All NCCM patients (n = 109) were age and sex matched with 109 dilated cardiomyopathy (DCM) patients as controls. The aortic diameters were measured through the parasternal long-axis transthoracic echocardiographic view at the sinuses of valsalva (SoV-Ao), sinotubular junction (STJ) and ascending aorta (AscAo). Dilatation was defined using published criteria adjusted for body surface area (BSA), sex, and age. Median age of age-sex matched NCCM and DCM patients was 45[31–56] vs. 45 [31–55] years with 53% males in both groups. NCCM patients had more familial hereditary patterns and genetic variants (55% vs. 24%, p < 0.001). DCM patients had more heart failure and left ventricular dysfunction (ejection fraction 34 ± 11 vs. 41 ± 12, p = 0.001). Ascending aortic dilatation was present in 8(7%) patients with NCCM and 5(5%) patients with DCM (p = 0.46). All dilatations were classified as mild. In conclusion, in this cross-sectional cohort study the prevalence of ascending aortic dilatation in NCCM patients was 7%, which were only mild dilatations and not significantly different from an age-sex matched cohort of DCM patients. Routine aortic dilatation screening therefore does not seem warranted in patients with NCCM.

Abstract 10980: Echocardiographic Evaluation of the Left Ventricular Function in Patients With Noncompaction Cardiomyopathy: Biplane Method, Wall Motion Score or Global Longitudinal Strain?

Introduction: Noncompaction cardiomyopathy (NCCM) is characterized by left ventricular (LV) hypertrabeculation. Therefore, endocardial tracing / quantification of LV function could be cumbersome. Hypothesis: Accurate assessment of the (subclinical) LV dysfunction in NCCM patients could improve non-invasive monitoring of disease progression, risk stratification, and treatment. Method: We reviewed the echocardiographic images of 67 patients (54% male, median age 48 year [22-73]. LV function was assessed by conventional biplane disk summation, wall motion score (WMS), and global longitudinal strain (GLS). LV function was considered abnormal if LV ejection fraction (LVEF) &lt;50% and GLS &gt;-18,9%. Results: LV function measured by biplane vs WMS showed significantly lower LV EF with WMS (p-value: 0,0016; Figure ), while the average GLS in all patients was -11,0%, SD ±3,8 (normal values: -18,9%; p-value: p&lt;0,001). By dichotomizing the group into LVEF &lt;50% vs. ≥50%, biplane EF was abnormal in 68,7%, while this was with WMS 88,1% en GLS in 100% patients. In patient with EF &lt;50% compared to ≥ 50% GLS in low biplane EF group was: -9.3% vs -14.5% (p&lt;0.001). In the WMS EF was this respectively -10,3±3,6% versus -15.5± 1.5 (p&lt;0.001). Conclusion: The systolic LV dysfunction in patients with NCCM noncompaction cardiomyopathy found by WMS and GLS was significantly lower compared to routine biplane EF measurement suggesting that WMS and GLS measurement are much more potential prognostic tool. Given the correct execution of WMS is limited by the assessor's experience, estimation of the LV function with GLS are probably most reliable. Future research should expel whether these results are representative in a larger group of patients and correlates with the clinically relevant endpoints.

Abstract 15678: The Introduction of Novel Heart Failure Therapy in Patients With Non-Compaction Cardiomyopathy Leads to an Improvement of Heart Failure

Introduction: In patients with noncompaction cardiomyopathy (NCC) no heart failure (HF) therapy has been shown to improve the left ventricle ejection fraction (LVEF). Sudden cardiac death (SCD) or need for heart transplantation (HTx) or left ventricle assist device (LVAD) implantation have been described. The effect of novel HF therapy, such as angiotensin receptor and neprilysin inhibitor (ARNI) and sodium glucose co-transporter 2 inhibitors (SGLT2i) remains unclear. Hypothesis: We sought to analyze a potential recovery of cardiac function in patients with NCC after introducing the novel HF therapy. Methods: We have prospectively enrolled 41 (33 male, 8 female) patients with NCC, confirmed by cardiac MRI from 2018 to 2021. The average age was 49±16.3 years. At inclusion, echocardiography was performed to determine the left ventricle end-diastolic diameter and volume (EDD and EDV) and LVEF; serum levels of NT-proBNP have also been obtained. ARNI and SGLT2i have been introduced into patients’ therapy and up titrated to maximal tolerated dose. After 1 year follow up investigations, performed at patients' inclusion, have been repeated. Results: Of 41 patients enrolled 23 patients (56%) had echocardiographic evidence of myocardial recovery (Group A), defined by improvement of LVEF above 40% (LVEF increased from 31±6.6% to 48±5.2%. 9 patients (22%) showed no significant LVEF improvement (Group B, LVEF increased from 26±8.0% to 33±2.6%). 9 patients were excluded (2 due to SCD, 7 had HTx or LVAD implantation performed). The two groups did not differ in sex (male 41% vs. 39% in Group B, P=0.51), age (51±17.2 years vs. 46±15.2 years, P=0.72), sodium (140±2.6mEq/L vs. 138±1.8mEq/mL, P=0.22), creatinine (82±24μmol/L vs. 87±18 μmol/L, P=0.51) or bilirubin levels (17±12μmol/L vs. 21±12μmol/L, P=0.42). When compared to Group B at inclusion, Group A had significantly higher LVEF (31,6±6,6% vs. 26,5±8,0% in Group B, P=0.033) and lower NTproBNP levels (1619±1505pg/mL vs. 4902±3795pg/mL, P&lt;0.001). Conclusions: In patients with NCC, the introduction of novel HF therapy seems to be connected with significant reverse remodelation of the myocardium. The improvement has been shown to be more pronounced in NCC patients with better LVEF and lower neurohormonal activation.

A proposed strategy for anticoagulation therapy in noncompaction cardiomyopathy.

Noncompaction cardiomyopathy (NCCM) is a rare condition characterized by prominent trabeculae, deep intertrabecular recesses, and a left ventricular myocardium with a two‐layered structure, characterized by a spongy endocardial layer and a thinner and compacted epicardial one. NCCM can be isolated or associated with other congenital heart diseases and complex syndromes involving neuromuscular disorders and facial dysmorphisms. To date, more than 40 genes coding for sarcomeric, cytoskeletal, ion channels, and desmosomal proteins have been identified. Clinical presentation is also highly variable, ranging from no symptoms to end‐stage heart failure (HF), lethal arrhythmias, sudden cardiac death, or thromboembolic events. In particular, the prevalence of thromboembolism in NCCM patients appears to be higher than that of a similar, age‐matched population without NCCM. Thromboembolism has a multifactorial aetiology, which is linked to genetic, as well as traditional cardiovascular risk factors. In previous studies, atrial fibrillation (AF) was observed in approximately 25–30% of adult NCCM patients and embolism had a cardiac source in ~63–69% of cases; therefore, AF represents a strong predictor of adverse events, especially if associated to HF and neuromuscular disorders. Left ventricular dysfunction is another risk factor for thromboembolism, as a result of blood stagnation and local myocardial injury. Moreover, it is not completely clarified if the presence of deep intertrabecular recesses causing stagnant blood flow can constitute per se a thrombogenic substrate even in absence of ventricular dysfunction. For the clinical management of NCCM patients, an appropriate stratification of the thromboembolic risk is of utmost importance for a timely initiation of anticoagulant therapy. The aim of the present study is to review the available literature on NCCM with particular attention on thromboembolic risk stratification and prevention and the current evidence for oral anticoagulation therapy. The use of direct oral anticoagulants vs. vitamin K antagonists is also discussed with important implications for patient treatment and prognosis.

Molecular analysis of dilated and left ventricular noncompaction cardiomyopathies in Egyptian children.

Paediatric cardiomyopathy is a progressive, often lethal disorder and the most common cause of heart failure in children. Despite its severe outcomes, the genetic aetiology is still poorly characterised. High-throughput sequencing offers a great opportunity for a better understanding of the genetic causes of cardiomyopathy. The current study aimed to elucidate the genetic background of cardiomyopathy in Egyptian children. This hospital-based study involved 68 patients; 58 idiopathic primary dilated cardiomyopathy and 10 left ventricular noncompaction cardiomyopathy. Cardiomyopathy-associated genes were investigated using targeted next-generation sequencing. Consanguinity was positive in 53 and 70% of dilated cardiomyopathy and left ventricular noncompaction cardiomyopathy patients, respectively. Positive family history of cardiomyopathy was present in 28% of dilated cardiomyopathy and 10% of the left ventricular noncompaction cardiomyopathy patients. In 25 patients, 29 rare variants were detected; 2 likely pathogenic variants in TNNI3 and TTN and 27 variants of uncertain significance explaining 2.9% of patients. The low genetic detection rate suggests that novel genes or variants might underlie paediatric cardiomyopathy in Egypt, especially with the high burden of consanguinity. Being the first national and regional report, our study could be a reference for future genetic testing in Egyptian cardiomyopathy children. Genome-wide tests (whole exome/genome sequencing) might be more suitable than the targeted sequencing to investigate the primary cardiomyopathy patients. Molecular characterisation of cardiomyopathies in different ethnicities will allow for global comparative studies that could result in understanding the pathophysiology and heterogeneity of cardiomyopathies.

The role of circumferential strain in the differential diagnosis of cardiomyopathies with left ventricular hypertrabeculation

Abstract Funding Acknowledgements Type of funding sources: Public grant(s) – National budget only. Main funding source(s): Supported by the ÚNKP-19-3-II New National Excellence Program of the Ministry for Innovation and Technology Dilated (DCM), hypertrophic (HCM) and noncompaction cardiomyopathy (NCMP) are genetically and morphologically overlapping diseases, however they differ in clinical manifestation, treatment and prognosis. Cardiac MRI feature-tracking might help to differentiate between these cardiomyopathies with left ventricular (LV) hypertrabeculation. We aimed to describe the differences in the functional and strain parameters of NCMP patients with good LV ejection fraction (EF, NCMP-G) compared with patients with HCM, and NCMP patients with reduced EF (NCMP-R) compared with patients with DCM . We included 62 NCMP patients from which 31 had good LV function and 31 had decreased LV-EF. The NCMP-G group was compared with an HCM population (n = 31) and the NCMP-R group was compared with a DCM group (n = 31) matching in age and sex (age, EF; NCMP-G 46.0 ± 13.0 years, 65.5 ± 5.3% vs. HCM 47.2 ± 14.4 years, 74.8 ± 6.3%; NCMP-R 54.5 ± 12.1 years, 32.8 ± 10.1% vs. DCM 50.8 ± 16.7 years, 34.0 ± 8.2%). 1.5 T Philips Achieva and Siemens Aera MRI machines were used for the scans, Medis Suite program was used for analysis and MedCalc software for statistics, p &amp;lt; 0.05 was considered statistically significant. Significant differences were found between the functional parameters of HCM and NCMP-G patients, while the DCM and NCMP-R groups differed only in the trabecular mass values (LV-trab, NCMP-G vs. HCM: 26.2 ± 7.5 vs. 30.7 ± 7.0 g/m2, NCMP-R vs. DCM: 48.2 ± 13.2 vs. 42.1 ± 10.1 g/m2, p &amp;lt; 0.05). The global longitudinal strain values of the studied populations were not significantly different, however the global circumferential strain (GCS) values were significantly better in patients with HCM and DCM compared with the NCMP groups (GCS, NCMP-G vs. HCM: -31.2 ± 4.9 vs. -43.0 ± 8.4%, NCMP-R vs. DCM: -11.7 ± 7.3 vs. -16.9 ± 6.1%). The average circumferential strain values of the LV basal, mid and apical parts were significantly better in the HCM and DCM groups compared with the NCMP groups (NCMP-G vs. HCM: -35.7 ± 9.5 vs. -50.5 ± 14.1%, NCMP-R vs. DCM: -29.5 ± 13.2 vs. -15.6 ± 6,7%). We assessed the cut-off point of the average LV apical circumferential strain to differentiate the studied populations (HCM vs. NCMP-G cut-off: -47.3% sens.: 83.9%, spec.: 67.7%, AUC: 0.81; DCM vs. NCMP-R cut-off: -19.3% sens.: 83.9%, spec.: 83.9%, AUC: 0.86). The diverse circumferential strain values of the hypertrabeculated LV apical third could help the differential diagnosis of NCMP, DCM and HCM.

Left ventricular hypertrabeculation - an unanswered question

Abstract Funding Acknowledgements Type of funding sources: Other. Main funding source(s): Supported by the ÚNKP-19-3-II New National Excellence Program of the Ministry for Innovation and Technology Left ventricular (LV) hypertrabeculation (H-TRAB) is a morphological description when the LV is highly trabeculated, but it remains below the diagnostic criterion of noncompaction cardiomyopathy (NCMP). Although, it is a frequent finding on cardiac MRI scans its diagnostic and prognostic relevance remains controversial. In this cardiac MRI study, we aimed to describe the LV functional and feature-tracking strain values of healthy subjects with LV H-TRAB to decide whether it is a normal variant or an NCMP-like pattern. We included 189 adult participants with good LV ejection fraction (EF), and without co-morbidities. 63 of them had a morphology of LV H-TRAB (H-TRAB, age: 37.1 ± 14.4 years; EF: 66.4 ± 5.1%), 63 patients were diagnosed with NCMP (age: 36.3 ± 14.0 years, EF: 65.8 ± 5.5%) and the remaining 63 people were healthy controls (38.3 ± 14.0 years, EF: 69.0 ± 4.9%). MR examinations were performed with 1,5T Philips Achieva and Siemens Aera devices. The Medis Suite software was used for post-processing analysis, the MedCalc software for statistics, p &amp;lt; 0.05 was considered statistically significant. No significant differences were found between the functional parameters of the H-TRAB and NCMP groups. However, the EF was decreased, the end-diastolic (EDVi), and end-systolic volume (ESVi), the myocardial mass (LV-massi), and trabeculated muscle mass (LV-trabi) values were increased in both groups compared to controls (H-TRAB vs. control: EF: 6.4 ± 5.4 vs. 69.0 ± 5.0%, EDVi: 74.4 ± 14.3 vs. 69.7 ± 12.8 ml/m2, ESVi: 25.1 ± 6.7 vs. 21.7 ± 5.8 ml/m2, LV-massi: 77.0 ± 15.1 vs. 71.2 ± 12.3 g/m2, LV-trabi: 25.0 ± 5.1 vs. 20.1 ± 4.0 g/m2; NCMP vs. control: EF: 65.8 ± 5.5 vs. 69.0 ± 5.0%, EDVi: 77.8 ± 15.0 vs. 69.7 ± 12.8 ml/m2, ESVi: 26.7 ± 7.9 vs. 21.7 ± 5.8 ml/m2, LV-massi: 76.8 ± 18.0 vs. 71.2 ± 12.3 g/m2, LV-trabi: 25.9 ± 7.5 vs. 20.1 ± 4.0 g/m2; p ≤ 0.05). The global circumferential strain (GCS) differed significantly between these groups: its absolute value was the lowest in the NCMP and the highest in the control group (NCMP vs. H-TRAB vs. control: -30.2% vs. -34.3% vs. -35.9%; p &amp;lt; 0.05). Similar results were found in the comparison of the mean segmental circumferential strain values of the apical-, mid- and basal-parts between the three groups. The absolute values of these parameters were the lowest in the NCMP patients, followed by the H-TRAB, and were the highest in the control group. These results suggest that the LV functional and strain values of the H-TRAB population are more similar to NCMP patients than healthy controls. Further studies are necessary to determine the pathological implications and consequences of this morphology.

Right ventricular non-compaction: myth or reality?

Abstract Funding Acknowledgements Type of funding sources: None. Noncompaction cardiomyopathy (NCMP) is characterized by excessive left ventricular (LV) trabeculation. The involvement of the right ventricle (RV) is still questionable, furthermore, the normal range for RV trabeculation is undefined. Our aim was to describe the RV functional and strain values of patients with NCMP with preserved LV ejection fraction (EF) using cardiac MRI and to compare these parameters with healthy control subjects, furthermore, we aimed to define the normal range for RV trabeculation. We included 81 NCMP patients with good LV-EF (mean age: 37.0 ± 14 years; EF: 69.4 ± 13.2%) and without comorbidities. Their parameters were compared to an age and sex matched control group (mean age: 37.2 ± 13.7 years; EF: 77.2 ± 15.0%). MR examinations were performed with 1,5T Philips Achieva and Siemens Aera devices. The Medis Suite software was used for post-processing analysis, the MedCalc software for statistics, p &amp;lt; 0.05 was considered statistically significant. The RV trabecular mass index (RV-TRABi) was significantly greater and the RV-EF significantly smaller in the NCMP group compared with the controls (NCMP vs. control; TRABi: 20.6 ± 7.0 vs. 16.9 ± 4.2 g/m2; RV-EF: 62.8 ± 5.5 vs. 64.5 ± 4.5%; p &amp;lt; 0.05). As for the RV global longitudinal strain (GLS) the results were close to significant (NCMP vs. control: -25.1 ± 4.0 vs. -26.4 ± 4.3%; p = 0.05). We defined the normal range of RV-TRABi with a lower limit of 8.2 g/m2 (90% confidence interval (CI): 6.8-9.5) and the upper limit of 22.1 g/m2 (90% CI: 23.6-26.5). 27.2% of the NCMP patients exceeded the upper limit of RV-TRABi. The described differences in the RV-EF, RV-TRABi and RV-GLS draws attention to the possibility of the involvement of RV in patients with NCMP. Further follow-up studies would be necessary to evaluate the clinical importance of these alterations.

Speckle tracking echocardiography and left ventricular twist mechanics: predictive capabilities for noncompaction cardiomyopathy in the first degree relatives.

In non-compaction cardiomyopathy (NCCM), there are several echocardiographic and cardiac magnetic resonance (CMR)-based quantitative diagnostic indices, current criteria mainly placed on morphological features, and none of the diagnostic indices includes left ventricular (LV) function. LV function and hemodynamics could be normal in NCCM patients. Evaluation of left ventricular function at the subclinical stage, strain echocardiographic parameters could be used alternative to standard echocardiographic examinations. The aim of this study to evaluate; NCCM patients, their first-degree relatives, ventricular motion patterns, strain characteristics, and the predictive capabilities of these features for early diagnosis of cardiomyopathy. This cross-sectional, case-control study included 32 NCCM patients, 30 first-degree relatives (father, mother, siblings and children) and 31 healthy volunteers. All patients evaluated with baseline echocardiography, strain measurements, and ventricular wall motion pattern. There were no differences between the groups in terms of age, weight, and body surface area. We observed a statistically significant decrease in ejection fraction (EF), fractional shortening (FS), E/E' and global strain values in patients' relatives compared to healthy volunteers (Patients' relatives: LVEF:60.9 ± 7.2%, FS:0.34 ± 0.07, E/E':7.51 ± 1.83, GLS: - 18.6 ± 3.6, GLSr: - 1.1 ± 0.1, GCS: - 17.1 ± 3.1, GCSr: - 1.2 ± 0.1, GRS:37.1 ± 6.2, GRSr:1.7 ± 0.1; all p values< 0.05). 'Rigid Body Rotation (RBR)' movement pattern was also observed in some of the patient's relative's like in the patients. RBR movement pattern determined patients; EF, longitudinal strain-strain rate, and basal layer rotation values were significantly lower, but radial strain values were higher with the RBR movement pattern (for all values p < 0.05). RBR movement pattern, deterioration of strain parameters, and accompanying echocardiographic features like LVEF, fractional shortening (FS), E/E' in patients' relative groups may contribute to reveal the subclinical status of disease and could be predictive for early diagnosis of cardiomyopathy.

Myocardial Ischemia Correlates with Right Ventricular Dysfunction in Patients with Noncompaction Cardiomyopathy

Noncompaction cardiomyopathy (NCM) results from an arrest of the myocardial compaction process in the embryonic period, leading to the impaired formation of the microvasculature. Predominantly left ventricle is affected, however right ventricle (RV) involvement and its functional impact in NCM remains undefined. We sought to analyze a potential correlation between myocardial ischemia and RV failure in this patient cohort. We enrolled 41 NCM patients (28 male, 13 female), aged 21 to 70 years. At baseline we performed echocardiography to measure RVID (RV inflow diastolic diameter), TAPSE (tricuspid annular plane systolic excursion), Sto (systolic tissue Doppler velocity on tricuspid annulus) and RV FAC (fractional area change). Significant myocardial ischemia was defined as summed difference score (SDS)≥2 on myocardial SPECT at rest and on stress. Myocardial ischemia has been shown in 11 patients (27%, Group A), 30 patients showed no significant ischemic changes (73%, Group B). Groups did not differ in sex (82% male vs. 63% male in Group B, P=0.454), age (48±13 years vs. 47±15 years in Group B, P=0.855), kidney function (serum creatinine 76±11µmol/L vs. 81±26µmol/L in Group B, P=0.565) or liver function (serum bilirubine 12.4±7.1µmol/L vs. 13.3±6.5µmol/L in Group B, P=0.686). When compared to Group B, Group A had significantly lower FAC (30.7±7.7% vs. 44.9±10.2%, P<0.001), greater RVID (3.3±0.6cm vs. 2.9±0.5cm, P=0.016), lower TAPSE (1.9±0.6cm vs. 2.5±0.4cm, P<0.001), higher NT-proBNP levels (1691±1883pg/mL vs. 422±877pg/mL, P=0.006). There was a trend toward lower Sto (10.7±2.6cm/s vs. 12.4±2.4cm/s, P=0.081). Overall, higher SDS was associated with lower FAC (r=-0.51, P=0.006), higher RVID (r=0.41, P=0.032), lower TAPSE (r=-0.49, P=0.008) and higher levels of NT-proBNP (r=0.66, P<0.001). In patients with NCM myocardial ischemia appears to correlate with larger RV size and worse RV function.

Impairment of myocardial perfusion correlates with heart failure severity in patients with non-compaction cardiomyopathy.

ABSTRACTAimsNon‐compaction cardiomyopathy (NCM) is a congenital heart disease characterized by an arrest of the myocardial compaction process. Although NCM patients have impaired formation of microvasculature, the functional impact of these changes remains undefined. We sought to analyse a potential correlation between myocardial ischemia and heart failure severity in NCM patients.Methods and resultsWe enrolled 41 NCM patients (28 male and 13 female), aged 21–70 years. In all patients, we have determined left ventricular end‐diastolic volume (LVEDV), left ventricular ejection fraction (LVEF), and global longitudinal strain (GLS) by echocardiography. At the same time, serum levels of N‐terminal pro‐B‐type natriuretic peptide (NT‐proBNP) have been measured, and myocardial single‐photon emission computed tomography at rest and on stress was used to define significant myocardial ischemia defined as summed difference score ≥ 2. Myocardial ischemia has been demonstrated in 11 patients (27%, Group A), and 30 patients showed no significant ischemic changes (73%, Group B). The groups did not differ in sex, age, kidney, or liver function. When compared with Group B, Group A had significantly lower LVEF (35 ± 15% in Group A vs. 53 ± 11% in Group B, P < 0.001), higher LVEDV (188 ± 52 mL vs. 136 ± 52 mL, P = 0.007), lower GLS (−9.9 ± 5.2% vs. −14.5 ± 4.1%, P = 0.001), and higher NT‐proBNP levels (1691 ± 1883 pg/mL vs. 422 ± 877 pg/mL, P = 0.006). Overall, higher summed difference score was associated with lower LVEF (r = −0.48, P = 0.001), higher LVEDV (r = 0.39, P = 0.012), lower GLS (r = 0.352, P = 0.024), and higher levels of NT‐proBNP (r = 0.66, P < 0.001).ConclusionsThe presence of myocardial ischemia in patients with NCM is associated with worse left ventricular function, dilation of the left ventricle, and more pronounced neurohumoral activation.

P364 The first prospective study of non-compaction cardiomyopathy patients with preserved ejection fraction using cardiac magnetic resonance feature tracking

Abstract Feature tracking (FT) is a new cardiac magnetic resonance (CMR) technique for strain measurement to reveal changes e.g. in noncompaction cardiomyopathy (NCMP) patients with good ejection fraction (EF). Our aim was to describe, first in the literature, the functional and CMR-FT strain values of NCMP patients with good EF and to compare them with their previous scans taken 4 years ago. At the Heart and Vascular Center of Semmelweis University 6743 CMR examinations were done between 2009-2015 and 232 NCMP patients were diagnosed. We followed up 27 patients, who had a previous examination at least 4 years ago, had no co-morbidities and whoes EF were above 50% (mean age: 37 ± 14.4 years, 18 males, mean follow up: 5.7 ± 1.5 years). Their parameters were compared to a matched control (C) group. The Medis Suite software was used for analysis, the MedCalc software for statistics, (p &amp;lt; 0.05). We compared the patient’s previous (PREV) and recent (REC) functional parameters but did not find significant changes. Comparing the global longitudinal and global circumferential strains (GLS, GCS) and rotation (R) no difference was found between the PREV and the REC values. The GCS showed significant difference between NCMP and C groups (-30.2 ± 5.0 vs -35.9 ± 4.5; p &amp;lt; 0.0001). We compared the segmental longitudinal and circumferential strain values of PREV vs. REC groups and NCMP vs C groups and found significant differences just in a few segments. The left ventricular (LV) apical part’s mean longitudinal strain value showed significant decrease on the REC scans compared to the PREV (PREV vs REC: -24.4 ± 7.7 vs -20.6 ± 5.1%; p &amp;lt; 0.05) and a non-significant decrease compared to the C (C vs REC: -22.8 ± 7.5 vs -20.6 ± 5.1%; p= n.s.). The ratio of the average longitudinal strain value of the apical and basal part of the left ventricle was significantly smaller in the REC group compared to the PREV but did not differ from the C subjects ( PREV vs. REC: 1.5 ± 0.8 vs 1.0 ±0.3; C vs REC: 1.5 ± 0.3 vs 1.2 ± 0.5; p &amp;lt; 0.05) We did not find worsening in the functional parameters of NCMP patients with good EF by the end of the follow up period. However, subclinical changes can be detected in the affected apical part of LV when using FT suggesting the need for follow up.

P1824 Feature tracking strain values of noncompaction cardiomyopathy patients: differences in the light of ejection fraction

Abstract Feature tracking (FT) is a new cardiac magnetic resonance (CMR) technique for strain measurement which may give additional information about the progression of noncompaction cardiomyopathy (NCMP). First in the literature, we compared the strain and functional parameters of NCMP patients with reduced (R) and good (G) ejection fraction (EF) using CMR-FT. From the total of 164 CMR proven R NCMP patients, who were examined between 2009 and 2018 at the Heart and Vascular Center of Semmelweis University, we selected 26 non-ischaemic, contrast agent-free examinations (age: 56 ± 10.6 years; EF: 32.7 ± 10.0%; male = 19). According to their EF they were divided into medium (M; EF: 35-50%; n = 10) and significantly reduced EF (SR; EF &amp;lt;35%; n = 16) subgroups. We searched 25 NCMP patients with good EF (age: 37 ± 14.4 years; EF: 65.9 ± 5.3%; male = 16) and 26 control persons (age: 52 ± 10.7 years; EF: 70.8 ± 5.1%; male = 16) to the above mentioned groups. The Medis Suite program was used for analysis and the MedCalc software for statistics, (p &amp;lt; 0.05). Significant differences were found between the functional parameters of R and G patients. The global longitudinal strain (GLS) and global circumferential strain (GCS) values of the R group were decreased significantly compared to G and C groups. (R vs G; GLS: -9.5 ± 4.3 vs -20.9 + 3.2%; GCS: -12.5 ± 6.1 vs -30.5 ± 5.0%, p &amp;lt; 0.0001; R vs C; GLS: -9.5 ± 4.3 vs -23.3 ± 4.4%; GCS: -12.5 ± 6.1 vs -35.6 ± 5.1%, p &amp;lt; 0.0001). All the segmental strain values of R group were decreased compared to G and C groups, moreover this significancy was observed between the M and SR subgroups as well. The strain values of the R group in all the basal, mid and apical part of the left ventricle (LV) were reduced equally. The average longitudinal strain values of the apical part of the LV in the SR subgroup were significantly reduced compared to the average strains of the basal part (apical vs basal: -8.9 ± 3.0 vs -11.9 ± 4.4 %, p &amp;lt; 0.05). In addition, we found positive correlation in the R subgroup between the GCS and the mass of trabeculated muscle (r = 0.58, p = 0.002) . Our study shows that the global and segmental strain values decreased significantly in proportion to the reduction of the EF which describes well the progression of heart failure in NCMP. However, the decreased strain values of the hypertrabeculated apical part might be specific to NCMP.

The pivotal role of cardiovascular imaging in the identification and risk stratification of non-compaction cardiomyopathy patients.

Non-compaction cardiomyopathy (NCM) is a heterogeneous myocardial disease that can finally lead to heart failure, arrhythmias, and/or embolic events. Therefore, early diagnosis and treatment is of paramount importance. Furthermore, genetic assessment and counseling are crucial for individual risk assessment and family planning. Echocardiography is the first-line imaging modality. However, it is hampered by interobserver variability, depends among others on the quality of the acoustic window, cannot assess reliably the right ventricle and the apex, and cannot provide tissue characterization. Cardiovascular magnetic resonance (CMR) provides a 3D approach allowing imaging of the entire heart, including both left and right ventricle, with low operator variability or limitations due to patient's body structure. Furthermore, tissue characterization, using late gadolinium enhancement (LGE), allows the detection of fibrotic areas possibly representing the substrate for potentially lethal arrhythmias, predicts the severity of LV systolic dysfunction, and differentiates apical thrombus from fibrosis. Conversely, besides being associated with high costs, CMR has long acquisition/processing times, lack of expertise among cardiologists/radiologists, and limited availability. Additionally, in cases of respiratory and/or cardiac motion artifacts or arrhythmias, the cine images may be blurred. However, CMR cannot be applied to patients with not CMR-compatible implanted devices and LGE may be not available in patients with severely reduced GFR. Nevertheless, native T1 mapping can provide detailed tissue characterization in such cases. This tremendous potential of CMR makes this modality the ideal tool for better risk stratification of NCM patient, based not only on functional but also on tissue characterization information.

Importance of lead aVR on predicting adverse cardiac events in patients with noncompaction cardiomyopathy.

BackgroundNoncompaction cardiomyopathy (NCCM) is a relatively rare cardiac abnormality with high rates of mortality and morbidity. T‐wave amplitudes during ventricular repolarization in lead aVR (TaVR) have been reported to be associated with the prognosis of various cardiovascular diseases. This study sought to investigate the prevalence and prognostic role of positive TaVR in patients with NCCM.MethodsWe evaluated consecutive 161 patients with NCCM (65.8% men, mean age 42.5 ± 15.2 years old). Presentation electrocardiogram was assessed regarding classical parameters as well as T‐wave amplitudes in lead aVR. The primary endpoint was defined as composite lethal arrhythmic events, including sudden cardiac death, ventricular fibrillation, or sustained ventricular tachycardia or appropriate implantable cardioverter–defibrillator shock. Heart failure requiring hospitalization, cardiovascular death, and all‐cause mortality were also investigated as secondary endpoints.ResultsPatients with positive TaVR showed higher rates for arrhythmic events, hospitalization for heart failure, and death compared with patients without it. In multivariate Cox model, after adjusting for other known clinical and electrocardiographic risk factors, the positive TaVR was found to be a strong independent predictor of primary endpoint (HR: 4.8, 95% CI: 1.2–19.3; p = .025) and all‐cause death (HR: 3.5, 95% CI: 1.0–12.1; p = .045).ConclusionOur findings revealed that positive TaVR is significantly and independently associated with adverse outcomes in NCCM patients. This unique ECG criterion in the often ignored lead provides incremental information beyond what is available with other traditional risk factors.

P4658Left ventricular non-compaction, trait or cardiomyopathy

Abstract Background The diagnosis of cardiac hypertrabecularization has increased considerably in recent years. Whether or not the non-compaction is a pathological condition or a physiological trait is still highly debated. We performed a meta-analysis and systematic review on more than 7,000 adult individuals with left ventricular non-compaction to provide a comprehensive overview on its clinical outcome as well as its genetic background. Methods We first retrieved PubMed/Medline literatures in English language between 2000 to 2018 on clinical outcome and genotype of patients with non-compaction. Altogether, 35 studies with non-compaction cardiomyopathy patients passed the selection criteria and were extensively reviewed and meta-analyzed. Furthermore, we summarized the results of 8 major studies, which investigated the non-compaction in athletes, pregnant women, patients with sickle cell disease, or in individuals from population-based cohorts. Results About 60% of the patients with left-ventricular non-compaction cardiomyopathy were male. The diagnosis was mostly made in the mid of patients' 5th decade. Seven percent of patients had congenital heart diseases (CHD) including atrial/ventricular septum defect or Ebstein anomaly. Around 25% of the patients had positive family history for cardiomyopathy. Frequent phenotypic manifestations were heart rhythm abnormalities including conduction disease (26%), supraventricular tachycardia (17%), and sustained or non-sustained ventricular tachycardia (18%). Neuromuscular disease was a reported comorbidity with a mean frequency of 5%. Three important outcome measures including systemic thromboembolic events (9%), heart transplantation (4%), and adequate ICD therapy (15%) were reported. The genetics of non-compaction cardiomyopathy showed TTN to be the most frequently mutated gene (11%), followed by MYH7 (9%), MYBPC3 (5%), and CASQ2, LDB3 (3% each). TPM1, MIB1, ACTC1, and LMNA mutations had an average frequency of 2% each followed by PLN, HCN4, TAZ, DTNA, TNNT2, and RBM20 (1% each). Eight studies that investigated the occurrence of non-compacted myocardial regions in apparently heart healthy individuals applied different, established imaging-based diagnostic criteria for non-compaction and could confirm its presence in a wide range of 1.3% to 37%. Conclusion This meta-analysis summarizes the clinical presentation of left ventricular non-compaction in a large dataset and indicates that its presence often leads to unfavourable outcome, but can also be observed in heart healthy individuals. Multimodal diagnostic workflows are needed for comprehensive understanding of these individuals and for distinguishing between benign morphological trait and manifest cardiomyopathy.

Prognostic Significance of Heart Rate Turbulence Parameters in Patients with Noncompaction Cardiomyopathy

Background: Noncompaction cardiomyopathy (NC) is a rare congenital heart disease characterized by progressive heart failure and life-threatening arrhythmias. Heart rate turbulence (HRT) has been defined as a noninvasive prognostic method to reveal the cardiac death risk in high-risk patients. Objectives: We aimed to assess the cardiac autonomic functions and their relations to the mortality in NC patients. Methods: A total of 60 NC patients and 70 healthy controls were included in this study. All participants underwent 24-h Holter recording to assess the HRT parameters, included turbulence onset (TO), turbulence slope (TS), standard deviation of NN intervals of all normal beats (SDNN) and mean RR interval. Results: NC patients had higher levels of TO than the control group (0.43 ± 4.66% vs. –1.82 ± 2.19%, p = 0.024), but the TS levels of NC patients were lower than those of the control group (3.43 ± 3.28 vs. 4.94 ± 2.86 ms/RR, p = 0.024). Thirteen patients died during follow-up (mean 83.3 ± 32.5 months). TS was the strongest univariate mortality predictor (hazard ratio 10.01 [95% CI 2.22–42.52]; p = 0.004) in univariate Cox regression analysis. In multivariate analysis, LVEF ≤0.40 and TS ≤2.5 ms/RR interval were the only independent predictors of mortality (hazard ratio 5.29; p = 0.004, hazard ratio 13.45; p = 0.001, respectively). Conclusions: Patients with NC showed abnormal HRV and HRT parameters when compared to healthy subjects. Furthermore, impaired HRT reaction in NC is an independent predictor of mortality.

Myocardial Ischemia Correlates with Left Ventricular Systolic Dysfunction in Patients with Noncompaction Cardiomyopathy

Purpose Noncompaction cardiomyopathy (NCC) results as an arrest of the myocardial compaction process in the embryonic period, leading to the impaired formation of the microvasculature. Myocardial perfusion abnormalities have already been described, however, the functional impact in NCC patients remains undefined. We sought to analyze a potential correlation between myocardial ischemia and heart failure progression in patients with NCC. Methods We enrolled 37 (24 male, 13 female) patients with NCC, average age 47±15 years. Echocardiography determined left ventricular end diastolic diameter and volume (EDD and EDV) and ejection fraction (LVEF). Global longitudinal strain (GLS) postprocessing has been performed. Serum levels of NT-proBNP have been measured. Cardiac MRI has been performed to confirm the diagnosis. Myocardial SPECT was performed at rest and on stress, determining summed difference score (SDS). Significant myocardial ischemia was defined as SDS≥2. Results Myocardial ischemia has been shown in 15 patients (41%, Group A), 22 patients (59%) showed no significant ischemic changes (Group B). The two groups did not differ in sex (male 73% vs. 59% in Group B), age (48±15 years vs. 46±14 years, P=0.65), creatinine (76±14 μmol/L vs. 76±16 μmol/L, P=0.98) or bilirubin (11±6 μmol/L vs. 12±5 μmol/L, P=0.46). When compared to Group B, Group A had significantly lower LVEF (38±15% vs. 55±13% in Group B, P=0.001), higher EDD (6.3±0.9 cm vs. 5.2±0.7 cm, P Conclusion In patients with NCC myocardial ischemia correlates with lower ejection fraction and dilation of the left ventricle, leading to greater neurohumoral activation. As medical heart failure therapy has not been shown to be successful in NCC patients, progressive LV systolic dysfunction due to myocardial ischemia often warrants heart transplantation or left ventricular assist device implantation. Treatment approaches targeting myocardial ischemia, such as CD34+ cell therapy, could slow the progression of disease in NCC patients.

PROGNOSTIC SIGNIFICANCE OF HEART RATE TURBULENCE PARAMETERS IN PATIENTS WITH NONCOMPACTION CARDIOMYOPATHY

Background: Noncompaction cardiomyopathy (NC) is a rare congenital heart disease characterized by progressive heart failure and life-threatening arrhythmias. Heart rate turbulence (HRT) has been defined as a noninvasive prognostic method to reveal the cardiac death risk in high-risk patients. Objectives: We aimed to assess the cardiac autonomic functions and their relations to the mortality in NC patients. Methods: A total of 60 NC patients and 70 healthy controls were included in this study. All participants underwent 24-h Holter recording to assess the HRT parameters, included turbulence onset (TO), turbulence slope (TS), standard deviation of NN intervals of all normal beats (SDNN) and mean RR interval. Results: NC patients had higher levels of TO than the control group (0.43 ± 4.66% vs. –1.82 ± 2.19%, p = 0.024), but the TS levels of NC patients were lower than those of the control group (3.43 ± 3.28 vs. 4.94 ± 2.86 ms/RR, p = 0.024). Thirteen patients died during follow-up (mean 83.3 ± 32.5 months). TS was the strongest univariate mortality predictor (hazard ratio 10.01 [95% CI 2.22–42.52]; p = 0.004) in univariate Cox regression analysis. In multivariate analysis, LVEF ≤0.40 and TS ≤2.5 ms/RR interval were the only independent predictors of mortality (hazard ratio 5.29; p = 0.004, hazard ratio 13.45; p = 0.001, respectively). Conclusions: Patients with NC showed abnormal HRV and HRT parameters when compared to healthy subjects. Furthermore, impaired HRT reaction in NC is an independent predictor of mortality.