Validation of the MEDIA echo score for the prognosis of heart failure with preserved ejection fraction.

There is currently no widely used prognostic score in heart failure (HF) with preserved ejection fraction (HFpEF). The MEDIA echo score, including four variables (pulmonary arterial systolic pressure > 40 mmHg, inferior vena cava collapsibility index < 50%, average E/e' > 9, and lateral mitral annular s' < 7 cm/s), has been proposed as a useful risk stratification tool. This study aimed at further validating the MEDIA echo score in both hospitalised and ambulatory HFpEF patients. The MEDIA echo score ranges from 0 to 4 (each criterion scores 1 point). The associations between MEDIA echo score and cardiovascular outcomes were assessed in two independent HFpEF cohorts, namely patients hospitalised for worsening HFpEF (N = 242, mean age 78 ± 11), and stable ambulatory HFpEF patients (N = 76, mean age 65 ± 8). Using multivariable Cox models, in the worsening HFpEF cohort, patients with a MEDIA echo score of 3-4 displayed a significant increased risk of death (HR 2.10, 95%CI 1.02-4.33, P = 0.043, score 0-1 as reference). In the ambulatory HFpEF cohort, patients with a MEDIA echo score of 2 had a significantly higher risk of death or HF hospitalisation (HR 3.44, 95%CI 1.27-9.30, P = 0.015, score 0 as reference), driven by HF hospitalisation; in that cohort, adding the MEDIA echo score to the clinical model significantly improved reclassification for the combined endpoint (integrated discrimination improvement 6.2%, P = 0.006). The MEDIA echo score significantly predicted the outcome of HFpEF patients in both hospital and ambulatory settings; its use may help refine routine risk stratification on top of well-established prognosticators in stable HFpEF patients.

Detection of acute myocarditis by ECG-triggered PET imaging of somatostatin receptors compared to cardiac magnetic resonance: preliminary results.

Somatostatin receptors are overexpressed by inflammatory cells but not by cardiac cells, under normal conditions. This study assesses the detection of acute myocarditis by the ECG-triggered digital-PET imaging of somatostatin receptors (68Ga-DOTATOC-PET), as compared to Cardiac Magnetic Resonance (CMR) imaging, which is the reference diagnostic method in this setting. METHODS: Fourteen CMR-defined acute myocarditis patients had a first 15-minutes ECG-triggered 68Ga-DOTATOC PET recording, 4.4 ± 3.0 days from peak troponin, and 10 had a second 4.3 ± 0.3 months later. Myocardial/blood SUVmax ratio was analyzed relative to the normal upper limit of 2.18, which had been previously determined from oncology 68Ga-DOTATOC-PET recordings of patients with a similar age range as the myocarditis patients. RESULTS: An increased myocardial 68Ga-DOTATOC uptake relative to blood activity was invariably observed during the acute phase. SUVmax ratio exceeded 2.18 in all patients during the acute phase but also in 3/10 patients at 4-months, at a time when there were no more signs of active inflammation on CMR. A residual myocardial 68Ga-DOTATOC uptake was still observed on all gated-PET cine loops at 4-months. CONCLUSION: These preliminary results suggest that 68Ga-DOTATOC ECG-triggered digital-PET may be as sensitive as CMR at detecting myocarditis during the acute phase and more sensitive at later stages.

Outcomes of culture-negative vs. culture-positive infective endocarditis: the ESC-EORP EURO-ENDO registry.

AIM: Fatality of infective endocarditis (IE) is high worldwide, and its diagnosis remains a challenge. The objective of the present study was to compare the clinical characteristics and outcomes of patients with culture-positive (CPIE) vs. culture-negative IE (CNIE). METHODS AND RESULTS: This was an ancillary analysis of the ESC-EORP EURO-ENDO registry. Overall, 3113 patients who were diagnosed with IE during the study period were included in the present study. Of these, 2590 (83.2%) had CPIE, whereas 523 (16.8%) had CNIE. As many as 1488 (48.1%) patients underwent cardiac surgery during the index hospitalization, 1259 (48.8%) with CPIE and 229 (44.5%) with CNIE. The CNIE was a predictor of 1-year mortality [hazard ratio (HR) 1.28, 95% confidence interval (CI) 1.04-1.56], whereas surgery was significantly associated with survival (HR 0.49, 95% CI 0.41-0.58). The 1-year mortality was significantly higher in CNIE than CPIE patients in the medical subgroup, but it was not significantly different in CNIE vs. CPIE patients who underwent surgery. CONCLUSION: The present analysis of the EURO-ENDO registry confirms a higher long-term mortality in patients with CNIE compared with patients with CPIE. This difference was present in patients receiving medical therapy alone and not in those who underwent surgery, with surgery being associated with reduced mortality. Additional efforts are required both to improve the aetiological diagnosis of IE and identify CNIE cases early before progressive disease potentially contraindicates surgery.

Replacement Myocardial Fibrosis in Patients With Mitral Valve Prolapse: Relation to Mitral Regurgitation, Ventricular Remodeling, and Arrhythmia.

BACKGROUND: Mitral valve prolapse (MVP) is a frequent disease that can be complicated by mitral regurgitation (MR), heart failure, arterial embolism, rhythm disorders, and death. Left ventricular (LV) replacement myocardial fibrosis, a marker of maladaptive remodeling, has been described in patients with MVP, but the implications of this finding remain scarcely explored. We aimed at assessing the prevalence, pathophysiological and prognostic significance of LV replacement myocardial fibrosis through late gadolinium enhancement (LGE) by cardiac magnetic resonance in patients with MVP. METHODS: Four hundred patients (53±15 years of age, 55% male) with MVP (trace to severe MR by echocardiography) from 2 centers, who underwent a comprehensive echocardiography and LGE cardiac magnetic resonance, were included. Correlates of replacement myocardial fibrosis (LGE+), influence of MR degree, and ventricular arrhythmia were assessed. The primary outcome was a composite of cardiovascular events (cardiac death, heart failure, new-onset atrial fibrillation, arterial embolism, and life-threatening ventricular arrhythmia). RESULTS: Replacement myocardial fibrosis (LGE+) was observed in 110 patients (28%; 91 with myocardial wall including 71 with basal inferolateral wall, 29 with papillary muscle). LGE+ prevalence was 13% in trace-mild MR, 28% in moderate MR, and 37% in severe MR, and was associated with specific features of mitral valve apparatus, more dilated LV and more frequent ventricular arrhythmias (45% versus 26%, P<0.0001). In trace-mild MR, despite the absence of significant volume overload, abnormal LV dilatation was observed in 16% of patients and ventricular arrhythmia in 25%. Correlates of LGE+ in multivariable analysis were LV mass (odds ratio, 1.01 [95% CI, 1.002-1.017], P=0.009) and moderate-severe MR (odds ratio, 2.28 [95% CI, 1.21-4.31], P=0.011). LGE+ was associated with worse 4-year cardiovascular event-free survival (49.6±11.7 in LGE+ versus 73.3±6.5% in LGE-, P<0.0001). In a stepwise multivariable Cox model, MR volume and LGE+ (hazard ratio, 2.6 [1.4-4.9], P=0.002) were associated with poor outcome. CONCLUSIONS: LV replacement myocardial fibrosis is frequent in patients with MVP; is associated with mitral valve apparatus alteration, more dilated LV, MR grade, and ventricular arrhythmia; and is independently associated with cardiovascular events. These findings suggest an MVP-related myocardial disease. Last, cardiac magnetic resonance provides additional information to echocardiography in MVP.

Correction: Diagnostic performance of congestion score index evaluated from chest radiography for acute heart failure in the emergency department: A retrospective analysis from the PARADISE cohort.

[This corrects the article DOI: 10.1371/journal.pmed.1003419.].

Phenotype/Genotype Relationship in Left Ventricular Noncompaction: Ion Channel Gene Mutations Are Associated With Preserved Left Ventricular Systolic Function and Biventricular Noncompaction: Phenotype/Genotype of Noncompaction.

BACKGROUND: Few data exist concerning genotype-phenotype relationships in left ventricular noncompaction (LVNC). METHODS AND RESULTS: From a multicenter French Registry, we report the genetic and clinical spectrum of 95 patients with LVNC, and their genotype-phenotype relationship. Among the 95 LVNC, 45 had at least 1 mutation, including 14 cases of mutation in ion channel genes. In a complementary analysis including 16 additional patients with ion channel gene mutations, for a total of 30 patients with ion channel gene mutation, we found that those patients had higher median LV ejection fraction (60% vs 40%; P < .001) and more biventricular noncompaction (53.1% vs 18.5%; P < .001) than the 81 other patients with LVNC. Among them, both the 19 patients with an HCN4 mutation and the 11 patients with an RYR2 mutation presented with a higher LV ejection fraction and more frequent biventricular noncompaction than the 81 patients with LVNC but with no mutation in the ion channel gene, but only patients with HCN4 mutation presented with a lower heart rate. CONCLUSIONS: Ion channel gene mutations should be searched systematically in patients with LVNC associated with either bradycardia or biventricular noncompaction, particularly when LV systolic function is preserved. Identifying causative mutations is of utmost importance for genetic counselling of at-risk relatives of patients affected by LVNC.

Intravenous Mineralocorticoid Receptor Antagonist Use in Acutely Decompensated Heart Failure with Diuretic Resistance.

Intravenous mineralocorticoid receptor antagonists (MRAs) have been used in some centers for decades to reduce the risk of hypokalemia and boost diuresis in acutely decompensated heart failure (ADHF). We report the well-tolerated use of intravenous MRAs as a rescue procedure in 3 patients admitted for ADHF with important diuretic resistance. Undertaking trials evaluating the effect of this therapeutic strategy in ADHF could represent a promising avenue.

Diagnostic performance of congestion score index evaluated from chest radiography for acute heart failure in the emergency department: A retrospective analysis from the PARADISE cohort.

BACKGROUND: Congestion score index (CSI), a semiquantitative evaluation of congestion on chest radiography (CXR), is associated with outcome in patients with heart failure (HF). However, its diagnostic value in patients admitted for acute dyspnea has yet to be evaluated. METHODS AND FINDINGS: The diagnostic value of CSI for acute HF (AHF; adjudicated from patients' discharge files) was studied in the Pathway of dyspneic patients in Emergency (PARADISE) cohort, including patients aged 18 years or older admitted for acute dyspnea in the emergency department (ED) of the Nancy University Hospital (France) between January 1, 2015 and December 31, 2015. CSI (ranging from 0 to 3) was evaluated using a semiquantitative method on CXR in consecutive patients admitted for acute dyspnea in the ED. Results were validated in independent cohorts (N = 224). Of 1,333 patients, mean (standard deviation [SD]) age was 72.0 (18.5) years, 686 (51.5%) were men, and mean (SD) CSI was 1.42 (0.79). Patients with higher CSI had more cardiovascular comorbidities, more severe congestion, higher b-type natriuretic peptide (BNP), poorer renal function, and more respiratory acidosis. AHF was diagnosed in 289 (21.7%) patients. CSI was significantly associated with AHF diagnosis (adjusted odds ratio [OR] for 0.1 unit CSI increase 1.19, 95% CI 1.16-1.22, p < 0.001) after adjustment for clinical-based diagnostic score including age, comorbidity burden, dyspnea, and clinical congestion. The diagnostic accuracy of CSI for AHF was >0.80, whether alone (area under the receiver operating characteristic curve [AUROC] 0.84, 95% CI 0.82-0.86) or in addition to the clinical model (AUROC 0.87, 95% CI 0.85-0.90). CSI improved diagnostic accuracy on top of clinical variables (net reclassification improvement [NRI] = 94.9%) and clinical variables plus BNP (NRI = 55.0%). Similar diagnostic accuracy was observed in the validation cohorts (AUROC 0.75, 95% CI 0.68-0.82). The key limitation of our derivation cohort was its single-center and retrospective nature, which was counterbalanced by the validation in the independent cohorts. CONCLUSIONS: In this study, we observed that a systematic semiquantified assessment of radiographic pulmonary congestion showed high diagnostic value for AHF in dyspneic patients. Better use of CXR may provide an inexpensive, widely, and readily available method for AHF triage in the ED.

Prognostic influence of acute decompensated heart failure in patients planned for transcatheter aortic valve implantation.

OBJECTIVE: The aim of our study was to evaluate the outcome of patients with severe aortic stenosis presenting with acute decompensated heart failure (ADHF) and planned for transcatheter aortic valve implantation (TAVI) and to study the variables influencing their prognosis. METHODS: Our retrospective study included 801 patients planned for TAVI in our center. Seven hundred and fifty-six underwent TAVI and were categorized according to ADHF as the initial clinical presentation into two groups: ADHF group (n = 261) and no-ADHF group (n = 495). Pre as well as periprocedural outcomes and 1 year mortality were analyzed. RESULTS: Among the patients planned for the TAVI procedure, 45 patients remained untreated: 35 patients died while waiting to undergo TAVI which represented 20% of all deaths in our study, ADHF was observed in 23 of 45 (51%) these untreated patients. The 1-year all-cause mortality rate was significantly higher in the ADHF group versus the no-ADHF group (27% vs. 15%, p < .0001). In multivariate analysis, male gender (odds ratio [OR] =2.5, 95% confidence interval [CI]: 1.37-4.57, p = .03), body mass index <25 kg/m2 (OR = 2.76, 95% CI: 1.51-5.04, p = .0009), and logistic EuroSCORE II ≥20% (OR = 3.04, 95% CI: 1.56-5.94, p = .001) were associated with a higher 1-year mortality in the ADHF group. CONCLUSION: The patients eligible for TAVI presenting with ADHF were associated with a higher mortality for both: while on the waiting list for TAVI as well as at 1-year follow-up and thus asking for clearer criteria to prioritize action in this high-risk TAVI patients.

Cardiovascular Comorbidities Are the Main Predictors of Cardiac Reverse Remodeling following Kidney Transplantation.

BACKGROUND: End-stage renal disease is associated with cardiac remodeling, which is partly reversible after kidney transplantation (KT). We aimed to determine the association of cardiovascular comorbidities or kidney-related factors with cardiac reverse remodeling after KT. METHODS: We performed echocardiography in 56 patients (aged 48 ± 15 years, mean ± SD) before and 24 months after undergoing their first KT. Echocardiograms were reviewed using a standardized process with blinding for the patient characteristics and evaluation timing. Multivariable linear regression analysis was used to evaluate the association between comorbidities and changes in cardiac structure and systolic/diastolic function. RESULTS: Left ventricular mass index (LVMI) and diastolic parameters did not change significantly, while left ventricular ejection fraction (LVEF) increased from 63.9 to 69.6% (p = 0.046). Multivariable analysis revealed associations of histories of valvular heart disease with a smaller reduction in LVMI (ß = -27.3, p = 0.04), of coronary artery disease or heart failure with a smaller increase in LVEF (ß = 7.17, p = 0.02), and of diabetes mellitus with less improvement in E wave (ß = -0.19, p = 0.05), e' (ß = 4.15, p = 0.046), and E/e' (ß = -5.00, p < 0.01). CONCLUSION: Cardiovascular comorbidities were -associated with less improvement in cardiac structure and function following KT. Our findings suggest that patients with CV comorbidities may experience limited "favorable" reverse cardiac remodeling following KT.

Exercise Hemodynamics in the Prognosis of Patients with Pulmonary Arterial Hypertension.

BACKGROUND: Assessment of prognosis is of major importance when deciding on a therapeutic strategy in patients with pulmonary arterial hypertension (PAH). OBJECTIVES: The aim of this study was to investigate the prognostic value of pulmonary hemodynamics during exercise and changes during treatment in patients with PAH. METHODS: Consecutive incident patients (n = 49) with PAH undergoing right heart catheterization at rest and during a constant workload cycle exercise in supine position were included. Predictors of survival were identified at baseline using Cox proportional hazard regression models in a univariate analysis unadjusted and adjusted for age and gender. RESULTS: During a median follow-up period of 42 months, 13 (27%) of the 49 patients studied died. Two predictors of death were found: rest-to-exercise changes in heart rate and systolic pulmonary artery pressure. Adjusted hazard ratios were 0.92 (95% CI 0.86-0.99) and 0.93 (95% CI 0.88-0.99), respectively. These 2 variables were correlated with each other (r = 0.55, p < 0.001). CONCLUSIONS: Rest-to-exercise changes in heart rate and systolic pulmonary artery pressure measured at diagnosis are predictors of survival in patients with PAH. These measurements taken from an exercise test reflect right ventricular function.

Targeted panel sequencing in adult patients with left ventricular non-compaction reveals a large genetic heterogeneity.

Left ventricular non-compaction (LVNC) is a cardiomyopathy that may be of genetic origin; however, few data are available about the yield of mutation, the spectrum of genes and allelic variations. The aim of this study was to better characterize the genetic spectrum of isolated LVNC in a prospective cohort of 95 unrelated adult patients through the molecular investigation of 107 genes involved in cardiomyopathies and arrhythmias. Fifty-two pathogenic or probably pathogenic variants were identified in 40 patients (42%) including 31 patients (32.5%) with single variant and 9 patients with complex genotypes (9.5%). Mutated patients tended to have younger age at diagnosis than patients with no identified mutation. The most prevalent genes were TTN, then HCN4, MYH7, and RYR2. The distribution includes 13 genes previously reported in LVNC and 10 additional candidate genes. Our results show that LVNC is basically a genetic disease and support genetic counseling and cardiac screening in relatives. There is a large genetic heterogeneity, with predominant TTN null mutations and frequent complex genotypes. The gene spectrum is close to the one observed in dilated cardiomyopathy but with specific genes such as HCN4. We also identified new candidate genes that could be involved in this sub-phenotype of cardiomyopathy.

Impact of microvascular obstruction on left ventricular local remodeling after reperfused myocardial infarction.

PURPOSE: To evaluate by cardiac magnetic resonance imaging (MRI) the impact of microvascular obstruction (MVO) on regional left ventricular (LV) wall characteristics and local remodeling after acute myocardial infarction (AMI). MATERIALS AND METHODS: In all, 114 AMI patients underwent cardiac MRI at 3T within 2-4 days (baseline) and at 6 months (follow-up) after reperfusion. Late gadolinium enhancement and cine sequences were performed. The impact of MVO (ie, presence and extent) on regional wall thickening (WT, %), end-diastolic wall thickness (EDWT, mm), and local cavity change (mm) of LV were quantitatively analyzed. Local cavity change, calculated as surface-to-surface distance from registered endocardial surface meshes of cine imaging datasets acquired initially and at follow-up, was used to assess local remodeling. RESULTS: MVO was detected in 69 patients (60.5%). WT was significantly lower when MVO was present (P < 0.05); and it was inversely related to MVO transmural extent (P < 0.0001). WT improvement was significantly worsened when MVO was present in segments with infarct transmural extent exceeding 50%. Significant wall thinning occurred at follow-up in segments with infarct transmural extent >75% with further thinning by MVO presence; and EDWT decreased with increasing MVO transmural extent (P < 0.0001). LV cavity shrank in patients without MVO, whereas it dilated in those with MVO. Local cavity changes were not significantly different by a region-to-region analysis throughout the LV within each group (P = 0.57 and 0.74, respectively). CONCLUSION: MVO has a significant adverse effect on LV wall characteristics and LV remodeling. Postinfarct remodeling seems to be globally mediated rather than locally mediated during the first 6 months. LEVEL OF EVIDENCE: 2 Technical Efficacy: Stage 3 J. Magn. Reson. Imaging 2018;47:499-510.

Vertebral fracture prevalence and measurement of the scanographic bone attenuation coefficient on CT-scan in patients with systemic sclerosis.

To study vertebral fracture (VF) prevalence and the scanographic bone attenuation coefficient of the first lumbar vertebra (SBAC-L1) on computed tomography scans (CT-scans) in systemic sclerosis patients. This monocentric retrospective study included patients followed from 2000 to 2014 and fulfilling ACR/EULAR 2013 criteria for systemic sclerosis and who underwent a thoracic or thoraco-abdomino-pelvic CT-scan during their follow-up. Clinical characteristics for sclerosis and osteoporosis risk factors were collected. For CT-scan, the VFs were determined according to Genant's classification, the SBAC-L1 was measured in Hounsfield Units (HU), and a SBAC-L1 ≤ 145 HU (fracture threshold) defined patients at VF risk. Predictive factors for SBAC-L1 ≤ 145 HU were studied. A total of 70 patients were included [mean age, 62.3 (± 15.6) years, women 88.5%, diffuse sclerosis 22.9% (n = 16)]. On CT-scans, three VFs were detected in three patients (4.3%). The mean SBAC-L1 was 157.26 HU (± 52.1), and 35 patients (50%) presented a SBAC-L1 ≤ 145 HU. In multivariate analysis, only age (especially patients older than 63 years, OR = 1.08, CI 95% 1.04-1.13, p = 0.001) and calcinosis (OR = 6.04, CI 95% 1.27-28.70, p = 0.02) were independently associated with a SBAC-L1 ≤ 145 HU. On a large sample of patients with systemic sclerosis, the VF prevalence on CT-scan was low (4.3%) while 50% of the patients presented a SBAC-L1 ≤ 145 HU. Interestingly, the presence of calcinosis, periarticular calcifications or acro-osteolysis was linked with low SBAC-L1 and should lead to an osteoporosis screening, especially for patients under 63 years old.

Imaging the Right Heart-Pulmonary Circulation Unit: The Role of Ultrasound.

Echocardiography is the first step in imaging the right heart pulmonary circulation unit (RH-PCU), and the only one to allow its complete morphologic, functional, and hemodynamic analysis in all clinical scenarios. Right ventricular (RV) function is not only the consequence of its intrinsic contractile function (morphology and contractility) but also highly dependent on preload, afterload, and ventricular interdependence. Comprehensive echocardiographic examination of RH-PCU allows insight into intrinsic and extrinsic factors of RV function. Newer echocardiographic techniques allow for 3-dimensional evaluation of RV and detailed measurements of regional function using tissue Doppler or speckle tracking-based strain estimates.

Cardiac remodeling following reperfused acute myocardial infarction is linked to the concomitant evolution of vascular function as assessed by cardiovascular magnetic resonance.

BACKGROUND: Left ventricular (LV) remodeling following acute myocardial infarction (MI) is difficult to predict at an individual level although a possible interfering role of vascular function has yet to be considered to date. This study aimed to determine the extent to which this LV remodeling is influenced by the concomitant evolution of vascular function and LV loading conditions, as assessed by phase-contrast Cardiovascular Magnetic Resonance (CMR) of the ascending aorta. METHODS: CMR was performed in 121 patients, 2-4 days after reperfusion of a first ST-segment elevation myocardial infarction and 6 months thereafter. LV remodeling was: (i) assessed by the 6-month increase in end-diastolic volume (EDV) and/or ejection fraction (EF) and (ii) correlated with the indexed aortic stroke volume (mL.m-2), determined by a CMR phase-contrast sequence, along with derived functional vascular parameters (total peripheral vascular resistance (TPVR), total arterial compliance index, effective arterial elastance). RESULTS: At 6 months, most patients were under angiotensin enzyme converting inhibitors (86%) and beta-blockers (84%) and, on average, all functional vascular parameters were improved whereas blood pressure levels were not. An increase in EDV only (EDV+/EF-) was documented in 17% of patients at 6 months, in EF only (EDV-/EF+) in 31%, in both EDV and EF (EDV+/EF+) in 12% and neither EDV nor EF (EDV-/EF-) in 40%. The increase in EF was mainly and independently linked to a concomitant decline in TPVR (6-month change in mmHg.min.m2.L-1, EDV-/EF-: +1 ± 8, EDV+/EF-: +3 ± 9, EDV-/EF+: -7 ± 6, EDV+/EF+: -15 ± 20, p < 0.001) while the absence of any EF improvement was associated with high persisting rates of abnormally high TPVR at 6 months (EDV-/EF-: 31%, EDV+/EF-: 38%, EDV-/EF+: 5%, EDV+/EF+: 13%, p = 0.007). By contrast, the 6-month increase in EDV was mainly dependent on cardiac as opposed to vascular parameters and particularly on the presence of microvascular obstruction at baseline (EDV-/EF-: 37%, EDV+/EF-: 76%, EDV-/EF+: 38%, EDV+/EF+: 73%, p = 0.003). CONCLUSION: LV remodeling following reperfused MI is strongly influenced by the variable decrease in systemic vascular resistance under standard care vasodilating medication. The CMR monitoring of vascular resistance may help to tailor these medications for improving vascular resistance and consequently, LV ejection fraction. TRIAL REGISTRATION: NCT01109225 on ClinicalTrials.gov site (April, 2010).

Prevalence and clinical phenotype of hereditary transthyretin amyloid cardiomyopathy in patients with increased left ventricular wall thickness.

AIMS: Increased left ventricular wall thickness (LVWT) is a common finding in cardiology. It is not known how often hereditary transthyretin-related familial amyloid cardiomyopathy (mTTR-FAC) is responsible for LVWT. Several therapeutic modalities for mTTR-FAC are currently in clinical trials; thus, it is important to establish the prevalence of TTR mutations (mTTR) and the clinical characteristics of the patients with mTTR-FAC. METHODS AND RESULTS: In a prospective multicentre, cross-sectional study, the TTR gene was sequenced in 298 consecutive patients diagnosed with increased LVWT in primary cardiology clinics in France. Among the included patients, median (25-75th percentiles) age was 62 [50;74]; 74% were men; 23% were of African origin; and 36% were in NYHA Class III-IV. Median LVWT was 18 (16-21) mm. Seventeen (5.7%; 95% confidence interval [CI]: [3.4;9.0]) patients had mTTR of whom 15 (5.0%; 95% CI [2.9;8.2]) had mTTR-FAC. The most frequent mutations were V142I (n = 8), V50M (n = 2), and I127V (n = 2). All mTTR-FAC patients were older than 63 years with a median age of 74 [69;79]. Of the 15 patients with mTTR-FAC, 8 were of African descent while 7 were of European descent. In the African descendants, mTTR-FAC median age was 74 [72;79] vs. 55 [46;65] years in non-mTTR-FAC (P < 0.001). In an adjusted multivariate model, African origin, neuropathy, carpal tunnel syndrome, electrocardiogram (ECG) low voltage, and late gadolinium enhancement (LGE) at cardiac-magnetic resonance imaging were all independently associated with mTTR-FAC. CONCLUSION: Five per cent of patients diagnosed with hypertrophic cardiomyopathy have mTTR-FAC. Mutated transthyretin genetic screening is warranted in elderly subjects with increased LVWT, particularly, those of African descent with neuropathy, carpal tunnel syndrome, ECG low voltage, or LGE.

Hypereosinophilic cardiac disease.

Hypereosinophilia may be due to several aetiologies. Cardiac complications are not uncommon, whatever the causes. Clinical presentations of hypereosinophilic cardiac diseases may vary widely from asymptomatic form to fatal necrotic myocarditis or irreversible restrictive cardiomyopathy. As a major cause of morbidity and mortality, diagnosis of cardiac involvement is crucial. Until recently the diagnosis was exclusively based on cardiac biopsy. Nowadays, multimodal cardiac imaging, including echocardiography and cardiac magnetic resonance imaging, is the cornerstone of the diagnosis. Cardiac magnetic resonance imaging has emerged as a very useful tool for the diagnosis allowing precise tissue characterization. It has even been proposed as a surrogate to cardiac biopsy. It should be systematically performed in case of hypereosinophilia. Early and appropriate treatment is crucial to avoid the evolution toward irreversible cardiac lesions. These treatments are symptomatic but should also be adapted to the underlying diseases.

Myocardial infarct sizing by late gadolinium-enhanced MRI: Comparison of manual, full-width at half-maximum, and n-standard deviation methods.

PURPOSE: To compare three widely used methods for myocardial infarct (MI) sizing on late gadolinium-enhanced (LGE) magnetic resonance (MR) images: manual delineation and two semiautomated techniques (full-width at half-maximum [FWHM] and n-standard deviation [SD]). MATERIALS AND METHODS: 3T phase-sensitive inversion-recovery (PSIR) LGE images of 114 patients after an acute MI (2-4 days and 6 months) were analyzed by two independent observers to determine both total and core infarct sizes (TIS/CIS). Manual delineation served as the reference for determination of optimal thresholds for semiautomated methods after thresholding at multiple values. Reproducibility and accuracy were expressed as overall bias ± 95% limits of agreement. RESULTS: Mean infarct sizes by manual methods were 39.0%/24.4% for the acute MI group (TIS/CIS) and 29.7%/17.3% for the chronic MI group. The optimal thresholds (ie, providing the closest mean value to the manual method) were FWHM30% and 3SD for the TIS measurement and FWHM45% and 6SD for the CIS measurement (paired t-test; all P > 0.05). The best reproducibility was obtained using FWHM. For TIS measurement in the acute MI group, intra-/interobserver agreements, from Bland-Altman analysis, with FWHM30%, 3SD, and manual were -0.02 ± 7.74%/-0.74 ± 5.52%, 0.31 ± 9.78%/2.96 ± 16.62% and -2.12 ± 8.86%/0.18 ± 16.12, respectively; in the chronic MI group, the corresponding values were 0.23 ± 3.5%/-2.28 ± 15.06, -0.29 ± 10.46%/3.12 ± 13.06% and 1.68 ± 6.52%/-2.88 ± 9.62%, respectively. A similar trend for reproducibility was obtained for CIS measurement. However, semiautomated methods produced inconsistent results (variabilities of 24-46%) compared to manual delineation. CONCLUSION: The FWHM technique was the most reproducible method for infarct sizing both in acute and chronic MI. However, both FWHM and n-SD methods showed limited accuracy compared to manual delineation. J. Magn. Reson. Imaging 2016;44:1206-1217.