[Cardiomyopathies: a practical approach to the assessment and management of patients and their families]. / Kardiomyopathien: ein praktischer Ansatz für Abklärung und Management der betroffenen Patienten und deren Familien.

INTRODUCTION: The new 2023 European Society of Cardiology (ESC) Guidelines for the management of cardiomyopathies addresses all cardiomyopathies in a single document for the first time. The focus is on a phenotype-oriented diagnostic approach, multimodal imaging and genetic testing to establish the most accurate diagnosis possible. Additionally, new recommendations for risk stratification for sudden cardiac death in various cardiomyopathy phenotypes are provided. MRI and genetic testing have significantly gained importance in this context. Recommendations for comprehensive clinical and genetic cascade screening in relatives of individuals with cardiomyopathies have been revised. This article presents the most important innovations of these guidelines in a practice-oriented approach.

Prevention of sudden death in hypertrophic cardiomyopathy: bridging the gaps in knowledge.

Sudden cardiac death (SCD) is the most devastating complication of hypertrophic cardiomyopathy (HCM). Although the annual rate of SCD in the general HCM population is <1% per year according to contemporary series, there is still a small subset of patients who are at increased risk of SCD. The greatest challenge in the management of HCM is identifying those at increased risk as an implantable cardioverter defibrillator is a potentially life-saving therapy. In this review, we sought to summarize the available data on SCD in HCM and provide a clinical perspective on the current differing and somewhat conflicting European and American recommendations on risk stratification, with balanced guidance with regards to rational clinical decision making. Additionally, we sought to learn more on the actual implementation of the guidelines by HCM experts worldwide.

Prognostic value of quantitative contrast-enhanced cardiovascular magnetic resonance for the evaluation of sudden death risk in patients with hypertrophic cardiomyopathy.

BACKGROUND: Hypertrophic cardiomyopathy (HCM) is the most common cause of sudden death in the young, although not all patients eligible for sudden death prevention with an implantable cardioverter-defibrillator are identified. Contrast-enhanced cardiovascular magnetic resonance with late gadolinium enhancement (LGE) has emerged as an in vivo marker of myocardial fibrosis, although its role in stratifying sudden death risk in subgroups of HCM patients remains incompletely understood. METHODS AND RESULTS: We assessed the relation between LGE and cardiovascular outcomes in 1293 HCM patients referred for cardiovascular magnetic resonance and followed up for a median of 3.3 years. Sudden cardiac death (SCD) events (including appropriate defibrillator interventions) occurred in 37 patients (3%). A continuous relationship was evident between LGE by percent left ventricular mass and SCD event risk in HCM patients (P=0.001). Extent of LGE was associated with an increased risk of SCD events (adjusted hazard ratio, 1.46/10% increase in LGE; P=0.002), even after adjustment for other relevant disease variables. LGE of ≥15% of LV mass demonstrated a 2-fold increase in SCD event risk in those patients otherwise considered to be at lower risk, with an estimated likelihood for SCD events of 6% at 5 years. Performance of the SCD event risk model was enhanced by LGE (net reclassification index, 12.9%; 95% confidence interval, 0.3-38.3). Absence of LGE was associated with lower risk for SCD events (adjusted hazard ratio, 0.39; P=0.02). Extent of LGE also predicted the development of end-stage HCM with systolic dysfunction (adjusted hazard ratio, 1.80/10% increase in LGE; P<0.03). CONCLUSIONS: Extensive LGE measured by quantitative contrast enhanced CMR provides additional information for assessing SCD event risk among HCM patients, particularly patients otherwise judged to be at low risk.

Quantification of mitral regurgitation by real time three-dimensional color Doppler flow echocardiography pre- and post-percutaneous mitral valve repair.

BACKGROUND: Echocardiographic quantification of mitral regurgitation (MR) can be challenging if the valve geometry is significantly altered. Our aim was to compare the quantification of MR by the recently developed real time three-dimensional (3D) volume color flow Doppler (RT-VCFD) method to the conventional two-dimensional (2D) echocardiographic methods during the MitraClip procedure. METHODS: Twenty-seven patients (mean age 76 ± 8 years, 56% male) were prospectively enrolled and severity of MR was assessed before and after the MitraClip procedure in the operating room by 3 different methods: (1) by integrative visual approach by transesophageal echocardiography, (2) by transthoracic 2D pulsed-wave Doppler-based calculation of aortic stroke volumes (SV) and mitral inflow allowing calculation of regurgitant volume, and (3) by transthoracic 3D RT-VCFD-based calculation of regurgitant volume. RESULTS: We found moderate agreement between the integrative visual approach and the 3D RT-VCFD method for assessment of MR severity before (κ = 0.4, P < 0.05) and after MitraClip (κ = 0.5, P < 0.05). Relevant MR (3+ and 4+) was detected by visual approach in 27/27 and by 3D-VCFD method in 24/27 patients before and in 1 patient by both methods after the MitraClip procedure. In contrast, MR quantification by 2D SV method did not agree with the integrative visual approach or with the 3D RT-VCFD method. CONCLUSIONS: Quantification of MR before and after percutaneous MV repair by 3D RT-VCFD is comparable to the integrative visual assessment and more reliable than the 2D SV method in this small study population. Further automation of 3D RT-VCFD is needed to improve the accuracy of peri-interventional MR quantification.

The role of echocardiography in hypertrophic cardiomyopathy.

Hypertrophic cardiomyopathy (HCM) is a disease with a constellation of unique clinical, hemodynamic and echocardiographic features. Over the past decades, echocardiography has played a pivotal role in our understanding of the epidemiology, pathophysiology and prognosis of the condition. Recent advances in echocardiographic techniques have not only expanded our knowledge base but have also extrapolated the role of imaging from that of diagnosis, screening and non-invasive serial assessment to identification of poor prognostic markers, differentiation from phenocopies of HCM and guidance of intraprocedural decision-making in both septal ethanol ablation and surgical myectomy. This article summarizes the recent advances in echocardiography pertaining to HCM.

Significance of left ventricular apical-basal muscle bundle identified by cardiovascular magnetic resonance imaging in patients with hypertrophic cardiomyopathy.

AIMS: Cardiovascular magnetic resonance (CMR) has improved diagnostic and management strategies in hypertrophic cardiomyopathy (HCM) by expanding our appreciation for the diverse phenotypic expression. We sought to characterize the prevalence and clinical significance of a recently identified accessory left ventricular (LV) muscle bundle extending from the apex to the basal septum or anterior wall (i.e. apical-basal). METHODS AND RESULTS: CMR was performed in 230 genotyped HCM patients (48 ± 15 years, 69% male), 30 genotype-positive/phenotype-negative (G+/P-) family members (32 ± 15 years, 30% male), and 126 controls. Left ventricular apical-basal muscle bundle was identified in 145 of 230 (63%) HCM patients, 18 of 30 (60%) G+/P- family members, and 12 of 126 (10%) controls (G+/P- vs. controls; P < 0.01). In HCM patients, the prevalence of an apical-basal muscle bundle was similar among those with disease-causing sarcomere mutations compared with patients without mutation (64 vs. 62%; P = 0.88). The presence of an LV apical-basal muscle bundle was not associated with LV outflow tract obstruction (P = 0.61). In follow-up, 33 patients underwent surgical myectomy of whom 22 (67%) were identified to have an accessory LV apical-basal muscle bundle, which was resected in all patients. CONCLUSION: Apical-basal muscle bundles are a unique myocardial structure commonly present in HCM patients as well as in G+/P- family members and may represent an additional morphologic marker for HCM diagnosis in genotype-positive status.

Fabry disease: development and progression of left ventricular hypertrophy despite long-term enzyme replacement therapy.

BACKGROUND: Enzyme replacement therapy (ERT) may halt or attenuate disease progression in patients with Anderson-Fabry disease (AFD). However, whether left ventricular hypertrophy (LVH) can be prevented by early therapy or may still progress despite ERT over a long-term follow-up is still unclear. METHODS: Consecutive patients with AFD from the Independent Swiss-Fabry Cohort receiving ERT who were at least followed up for 5 years were included. Cardiac progression was defined as an increase of >10 g/m2 in left ventricular mass index (LVMI) between the first and the last available follow-up transthoracic echocardiography. RESULTS: 60 patients (35 (23-48) years, 39 (65%) men) were followed up for 10.5 (7.2-12.2) years. 22 had LVH at ERT start (LVMI of 150±38 g/m2). During follow-up, 22 (36%, 34±15 years) had LVMI progression of 12.1 (7-17.6) g/m2 per 100 patient-years, of these 7 (11%, 29±13 years) with no LVH at baseline. Three of them progressed to LVH. LVMI progression occurred mostly in men (17 of 39 (43%) vs 5 of 21 (24%), p<0.01) and after the age of 30 years (17 of 22 (77%)). LVH at ERT start was associated with LVMI progression (OR 1.3, 95% CI 1.1 to 2.6; p=0.02). A total of 19 (31%) patients experienced a major AFD-related event. They were predominantly men (17 of 19, 89%), older (45±11 vs 32±9 years) with baseline LVH (12 of 19, 63%), and 10 of 19 (52%) presented with LVMI progression. CONCLUSIONS: Over a median follow-up of >10 years under ERT, 36% of the patients still had LVMI cardiac progression, and 32%, predominantly older men, experienced major AFD-related events. LVH at treatment initiation was a strong predictor of LVMI progression and adverse events on ERT.

Clinical and CMR characteristics associated with cardiac events in patients with Fabry disease.

BACKGROUND: The assessment of late gadolinium enhancement (LGE) and left ventricular hypertrophy (LVH) by cardiac magnetic resonance (CMR) as diagnostic and prognostic maker in Fabry disease is advancing. We aimed to investigate the impact of clinical characteristics and CMR findings on cardiac outcome in patients with FD. METHODS: In this study 55 patients with genetically confirmed FD and available CMR imaging were included. The primary endpoint was defined as a composite of cardiac events including cardiac death, new occurrence of atrial fibrillation, heart failure, ventricular tachycardia and bradycardia requiring device insertion. RESULTS: During a median follow-up of 4.9 years (IQR 3.7-5.9), 9 patients (16.3%) reached the primary cardiac end point. The global amount of LGE was associated with an increased risk for primary endpoint in the univariate analysis (HR 1.4 per 10% increase in LGE, p = 0.002). However maximal wall thickness (MWT) was the sole independent predictor of the primary endpoint in a stepwise logistic regression model (HR 9.8 per mm increase in MWT, p < 0.0001). Kaplan-Meier analysis revealed significant difference in event free survival rate between patients with and without LVH (Long-rank p = 0.006) and in patients with and without LGE (Long-rank p < 0.001). Patients without LVH and LGE were free of adverse cardiac events. CONCLUSION: LVH and LGE detected by CMR were associated with adverse cardiac events in FD. In particular maximal wall thickness can be useful in cardiac risk stratification of FD patients.

Assessment of treatment response in cardiac sarcoidosis based on myocardial 18F-FDG uptake.

Objective: Immunosuppressive therapy for cardiac sarcoidosis (CS) still largely consists of corticosteroid monotherapy. However, high relapse rates after tapering and insufficient efficacy are significant problems. The objective of this study was to investigate the efficacy and safety of non-biological and biological disease-modifying anti-rheumatic drugs (nb/bDMARDs) considering control of myocardial inflammation assessed by 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) of the heart. Methods: We conducted a retrospective analysis of treatment response to nb/bDMARDs of all CS patients seen in the sarcoidosis center of the University Hospital Zurich between January 2016 and December 2020. Results: We identified 50 patients with CS. Forty-five patients with at least one follow-up PET/CT scan were followed up for a mean of 20.5 ± 12.8 months. Most of the patients were treated with prednisone and concomitant nb/bDMARDs. At the first follow-up PET/CT scan after approximately 6.7 ± 3 months, only adalimumab showed a significant reduction in cardiac metabolic activity. Furthermore, comparing all serial follow-up PET/CT scans (143), tumor necrosis factor inhibitor (TNFi)-based therapies showed statistically significant better suppression of myocardial 18F-FDG uptake compared to other treatment regimens. On the last follow-up, most adalimumab-treated patients were inactive (n = 15, 48%) or remitting (n = 11, 35%), and only five patients (16%) were progressive. TNFi was safe even in patients with severely reduced left ventricular ejection fraction (LVEF), and a significant improvement in LVEF under TNFi treatment was observed. Conclusion: TNFi shows better control of myocardial inflammation compared to nbDMARDs and corticosteroid monotherapies in patients with CS. TNFi was efficient and safe even in patients with severely reduced LVEF.

Pharmacologic preconditioning therapy prior to atrial septal defect closure in patients at high risk for acute pulmonary edema.

OBJECTIVES: The aim of this study was to assess whether transient atrial septal defect (ASD) occlusion and, if required, vasodilator therapy would improve the safety of percutaneous ASD closure in high-risk subsets. BACKGROUND: While percutaneous ASD closure is generally considered a low risk intervention, hypertensive and elderly patients may develop pulmonary edema following the procedure because of underlying left ventricular (LV) diastolic dysfunction. METHODS: Fifty-two consecutive patients who underwent successful percutaneous ASD closures were enrolled into a single-center prospective registry. Patients with arterial hypertension and/or >60 years of age (n = 15) were considered at risk for periprocedural pulmonary edema. Those patients were tested for an increase of LV filling pressures during transient ASD occlusion and, if this was the case, treated according to a prespecified algorithm. Clinical and echocardiography data were collected in-hospital and at 6 months follow-up. RESULTS: Shunt size was comparable in high and standard-risk patients (Qp:Qs 2.1 ± 0.8 vs. 2.1 ± 0.7, P = 0.82). High-risk patients had more often pulmonary hypertension (58% vs. 14%, P < 0.05) and were more frequently symptomatic. Among them, 4/15 (27%) demonstrated a significant rise of left-sided filling pressures during transient ASD balloon occlusion and underwent pharmacologic preconditioning prior to ASD closure. None of them developed periprocedural pulmonary edema. At follow-up, patients were less symptomatic (Pre: NYHA II n = 15, NYHA III n = 9; Post: NYHA II n = 15, NYHA III n = 0; P = 0.02) and right ventricular size decreased from 23 ± 5 cm(2) to 17 ± 5 cm(2), P < 0.05. CONCLUSION: Transient ASD occlusion and, if required, pharmacologic preconditioning prior to percutaneous closure may prevent periprocedural pulmonary edema in high-risk patients.

Systolic myocardial mechanics in patients with Anderson-Fabry disease with and without left ventricular hypertrophy and in comparison to nonobstructive hypertrophic cardiomyopathy.

OBJECTIVES: Anderson-Fabry disease (AFD) is a lysosomal storage disease, which can involve the heart, mimicking hypertrophic cardiomyopathy (HCM). The underlying mechanism of disease in AFD is an infiltrative, diffuse process, whereas HCM is a primary heart muscle condition with patchy distribution, which may prompt differences in myocardial mechanics. The aim of this study was to assess myocardial mechanics in AFD according to the presence of left ventricular hypertrophy (LVH) compared to nonobstructive HCM (NHCM) and healthy controls. METHODS AND RESULTS: We carried out a single-center, retrospective study in a small, genetically confirmed AFD cohort, which was divided into a subgroup with LVH (LVH+, n = 19), and without LVH (LVH-, n = 21). Comparison groups were healthy controls (n = 40) and NHCM patients (n = 19). Vector Velocity Imaging was applied to two-dimensional echocardiography studies for assessment of longitudinal strain (LS), circumferential strain (CS), and base-to-apex CS gradients. AFD LVH+ patients had lower global LS than AFD LVH- patients (-14 ± 4% vs -17 ± 3%, P < 0.05), but similarly lowered global CS (-24 ± 5% vs -22 ± 5%, P = ns). AFD LVH+ and NHCM had similarly lowered global LS compared to normals, but significantly lower global CS was observed in AFD LVH+ (-24 ± 5% vs -28 ± 4%, P < 0.05), whereas it was significantly increased in NHCM (-31 ± 2% vs -28 ± 4%, P < 0.05). Unlike NHCM, in both AFD subgroups, patients lost their normal base-to-apex CS gradient. CONCLUSIONS: AFD patients without LVH already show abnormal systolic myocardial mechanics. Relevant differences in myocardial mechanics between AFD patients with LVH compared to NHCM reflect the different underlying mechanisms of disease.

Right ventricular involvement in left ventricular non-compaction cardiomyopathy.

BACKGROUND: Left ventricular non-compaction cardiomyopathy (LVNC) features extensive trabeculations. Involvement of the right ventricle (RV) has been reported; however, distinction from normal RV trabeculation is difficult. This study aimed at assessing RV morphology and function in LVNC by cardiac magnetic resonance (CMR) and transthoracic echocardiography (TTE). METHODS: Dimensional and functional parameters were assessed according to guidelines. Novel CMR parameters were RV end-diastolic (ED) trabeculated area, RV ED trabeculated volume, and RV ED non-compacted to compacted (NC/N) ratio in short axis (SAX) as well as in four-chamber view (4CH). RESULTS: Twenty patients with LVNC and 20 controls were included. RV size and function were comparable in LVNC and controls and exhibited a good correlation between TTE and CMR. Although RV trabeculated area, RV trabeculated volume, and RV ED NC/C ratio in SAX as well as in 4CH were larger in LVNC, there was a major overlap with values in controls. RV ED NC/C ratio in SAX correlated with LV ED NC/C ratio (not in 4CH). Quantitative assessment of RV non-compaction was not feasible in TTE. CONCLUSIONS: Right ventricle size and function in LVNC can be measured by CMR and TTE, while RV trabeculation can only be quantified by CMR. RV myocardium displays more trabeculations in LVNC; however, overlap with normal individuals is extensive, not allowing separation of patients with LVNC from controls.

Sarcoidosis - a multisystem disease.

Sarcoidosis is a systemic inflammatory disease, characterised by granuloma formation upon an unknown trigger in genetically predisposed individuals. The inflammation is characterised by an activation of both the innate immune system, with macrophages differentiating into epitheloid cells and dendritic cells, and the adaptive immune system, particularly T helper (Th) 1 and Th17 cells. Since all organs can be affected to varying extents, clinical presentation is often diverse. Most commonly, the lungs, lymph nodes, skin and eyes are involved, whereas cardiac, renal and neurological manifestations are less common but associated with higher morbidity. Depending on the clinical symptoms, a detailed evaluation including thorough clinical examination, imaging and laboratory tests should explore all possible organ involvements. In some patients, fatigue manifests as a para-sarcoidosis symptom impacting quality of life, even if sarcoidosis is in remission. Some acute syndromic presentations, such as Löfgren's syndrome, have a good prognosis and are commonly self-limiting. If possible, a topical treatment, for example for cutaneous sarcoidosis or bronchial involvement, should be applied. Treatment of severe cases with persisting disease activity necessitates long-term immunosuppressive drugs, with glucocorticoids as the first-line option. Steroid-sparing and second-line drugs include methotrexate, azathioprine, mycophenolate mofetil and immunomodulators such hydroxychloroquine, with the latter being first-line therapy in cutaneous sarcoidosis. Tumour necrosis factor-alpha inhibitors (particularly adalimumab and infliximab) are used as third-line agents but are administered earlier in cases of persistent disease activity, severe organ-involvement or intolerance to conventional drugs. Treatment decisions should be based on a multidisciplinary approach, depending on organ involvement and treatment tolerability. Para-sarcoidosis manifestations, particularly fatigue, should also be carefully addressed, where the patient could also be enrolled in multidimensional rehabilitation programmes. With various organ involvement and different phenotypes, larger studies including real-world data from registries are necessary to evaluate different sarcoidosis endotypes and preferential treatment pathways.

Comparison of two different speckle tracking software systems: does the method matter?

BACKGROUND: Echocardiographic speckle tracking strain has gained clinical importance. However, the comparability of measurements between different software systems is not well defined. METHODS: In 47 healthy subjects left ventricular (LV) two-dimensional (2D) peak strain and time to peak strain (TTP) generated by EchoPAC (2DS) and velocity vector imaging (VVI) were compared. For each type of strain (longitudinal [LS], circumferential [CS], and radial strain [RS]) we compared global, anatomical level and segmental values. RESULTS: When comparing 2DS to VVI, Pearson correlation coefficients (r) of global LS, CS, and RS were 0.68, 0.44, and 0.59, respectively (all P < 0.05). Correlation of global TTP was higher: 0.81(LS), 0.80 (CS), and 0.68 (RS), all P < 0.01. Segmental peak strain differed significantly between 2DS and VVI in 8/18 (LS), 17/18 (CS), and 15/18 (RS) LV segments (P < 0.05). However, segmental TTP significantly differed only in 5/18 (LS), 7/18 (CS), and 4/18 (RS) of LV segments. Similar strain gradients were found for both systems: apical strain was higher than basal and midventricular strain in LS and CS, with a reversed pattern for RS (P < 0.05). CONCLUSION: TTP strain as well as strain gradients were comparable between VVI and 2DS, but most peak strain values were not. The software-dependency of peak strain values must be considered in clinical application. Further studies comparing the diagnostic and prognostic accuracy of strain values generated by different software systems are mandatory.

Left ventricular mechanics and cardiovascular outcomes in non-compaction phenotype.

AIMS: This study aims at understanding left ventricular (LV) mechanics of non-compaction (LVNC) phenotype using echocardiographic strain analysis and at assessing the association of functional parameters with cardiovascular (CV) outcomes. METHODS AND RESULTS: Longitudinal (GLS) and circumferential strain (GCS) as well as rotation of the LV were analyzed in 55 LVNC patients and 55 matched controls. Cardiovascular outcomes were documented for a median follow-up duration of 6 years. GLS and GCS were impaired in LVNC. Similary, regional longitudinal and circumferential strain as well as twist were reduced. CV events occurred in 28 LVNC patients. Apical peak circumferential strain (APCS), peak systolic rotation of apical segments (APSR), and twist were strongly associated with events. This was independent of and incremental to LVEF and non-compacted to compacted myocardial thickness ratio (NC:C ratio). The association of twist with events was also independent of and slightly superior to GLS. CONCLUSIONS: GLS, GCS, regional strain, and twist were impaired in LVNC. APCS, APSR, and twist exhibited strong association with CV events independent of and incremental to LVEF and NC:C ratio, and in case of twist even GLS. Thus, STE-derived parameters may complement the echocardiographic assessment of LVNC patients in clinical routine.

Differentiating hereditary arrhythmogenic right ventricular cardiomyopathy from cardiac sarcoidosis fulfilling 2010 ARVC Task Force Criteria.

BACKGROUND: The clinical presentation of cardiac sarcoidosis (CS) may resemble that of arrhythmogenic right ventricular cardiomyopathy (ARVC). OBJECTIVE: The purpose of this study was to identify clinical variables to better discriminate between patients with genetically determined ARVC and those with CS fulfilling definite 2010 ARVC Task Force Criteria (TFC). METHODS: In this multicenter study, 10 patients with CS fulfilling definite 2010 ARVC TFC were age and gender matched with 10 genetically proven ARVC patients. A cardiac 18F-fluorodeoxyglucose positron emission tomographic (18F-FDG PET) scan was required for patients to be included in the study. RESULTS: The 2010 ARVC TFC did not reliably differentiate between the 2 diseases. CS patients presented with longer PR intervals, advanced atrioventricular block (AVB), and longer QRS duration (P <.001 and P = .009, respectively), whereas T-wave inversions (TWIs) in the peripheral leads were more common in ARVC patients (P = .009). CS patients presented with more extensive left ventricular involvement and lower left ventricular ejection fraction (LVEF), whereas ARVC patients had a larger right ventricular outflow tract (RVOT) (P = .044). PET scan positivity was only present in CS patients (90% vs 0%). CONCLUSION: The 2010 ARVC TFC do not reliably differentiate between CS patients fulfilling 2010 ARVC TFC and those with hereditary ARVC. Prolonged PR interval, advanced AVB, longer QRS duration, right ventricular apical involvement, reduced LVEF, and positive 18F-FDG PET scan should raise the suspicion of CS, whereas larger RVOT dimensions, subtricuspid involvement and peripheral TWI favor a diagnosis of hereditary ARVC.

Diagnostic criteria for left ventricular non-compaction in cardiac computed tomography.

PURPOSE: Left ventricular non-compaction (LVNC) is characterized by a 2-layered myocardium composed of a noncompacted (NC) and a compacted (C) layer. The echocardiographic NC:C ratio is difficult to assess in many patients. The aim of the study was to assess the value of cardiac computed tomography (CCT) for the diagnosis of LVNC. METHODS: In this prospective controlled study, segmental analysis of transthoracic echocardiography (TTE) and prospective ECG-triggered CCT was performed in 17 patients with LVNC and 19 healthy controls. In TTE maximal NC and C thickness was measured at enddiastole and endsystole in the segment with most prominent trabeculation in short axis views. In CCT, maximal segmental NC and C thickness was measured during diastole, and NC:C ratio was determined. Spearman's correlation coefficient and receiver operating characteristic curves were calculated. RESULTS: The median [IQR] radiation dose was 1.3[1.2-1.5]mSv. The CCT thickness of the C layer was significantly lower in patients with LVNC as compared to controls in the inferolateral, midventricular, lateral-, inferior-, and septal-apical segments. The CCT NC:C ratio differed significantly between LVNC and controls in the inferior-midventricular and all the apical segments. NC:C ratio correlated significantly between TTE and CCT at enddiastole (σ = 0.8) and endsystole (σ = 0.9). Using a CCT NC:C ratio ≥1.8, all LVNC patients could be identified. CONCLUSION: LVNC can be diagnosed with ECG-triggered low-dose CCT and discriminated from normal individuals using a NC:C ratio of ≥1.8 in diastole. There is a very good correlation of NC:C ratio in TTE and CCT.

Cardiovascular magnetic resonance T2* mapping for the assessment of cardiovascular events in hypertrophic cardiomyopathy.

Background: Hypertrophic cardiomyopathy (HCM) is associated with an increased risk of adverse cardiac events. Beyond classic risk factors, relative myocardial ischaemia and succeeding myocardial alterations, which can be detected using either contrast agents or parametric mapping in cardiovascular magnetic resonance (CMR) imaging, have shown an impact on outcome in HCM. CMR may help to risk stratify using parametric T2* mapping. Therefore, the aim of the present study was to evaluate the association of T2* values or fibrosis with cardiovascular events in HCM. Methods: The relationship between T2* with supraventricular, ventricular arrhythmia or heart failure was retrospectively assessed in 91 patients with HCM referred for CMR on a 1.5T MR imaging system. Fibrosis as a reference was added to the model. Patients were subdivided into groups according to T2* value quartiles. Results: 47 patients experienced an event of ventricular arrhythmia, 25 of atrial fibrillation/flutter and 17 of heart failure. T2*≤28.7 ms yielded no association with ventricular events in the whole HCM cohort. T2* of non-obstructive HCM showed a significant association with ventricular events in univariate analysis, but not in multivariate analysis. For the combined endpoint of arrhythmic events, there was already an association for the whole HCM cohort, but again only in univariate analyses. Fibrosis stayed the strongest predictor in all analyses. There was no association for T2* and fibrosis with heart failure. Conclusions: Decreased T2* values by CMR only provide a small association with arrhythmic events in HCM, especially in non-obstructive HCM. No information is added for heart failure.