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Objectives The aim of this study was to compare left ventricular longitudinal strain (LS) evaluated by 2-dimensional echocardiography with cardiac magnetic resonance (CMR) in cardiac amyloidosis (CA), establish correlations between histological and imaging findings, and assess the prognostic usefulness of LS measurement and CMR. Background CA is a condition with a poor prognosis due chiefly to 3 forms of amyloidosis: light-chain amyloidosis (AL), hereditary transthyretin (M-TTR), and wild-type transthyretin (WT-TTR). Two-dimensional echocardiography measurement of LS has been reported to detect early left ventricular systolic dysfunction. The pathophysiological underpinnings, regional distribution, and prognostic significance of LS in CA are unclear. Methods All patients underwent echocardiography, and 53 underwent CMR. The native hearts of the 3 patients who received heart transplants were subjected to histological examination. For each of the 17 left ventricular segments in the American Heart Association model, we evaluated LS, late gadolinium enhancement (LGE) by CMR, and cardiac amyloid deposition. Univariate and multivariate analyses were performed at 6 months to identify variables associated with major adverse cardiac events (MACE). Results We studied 79 patients with CA; 26 had AL, 36 M-TTR, and 17 WT-TTR. Mean LS was −10 ± 4%. Both LS and amyloid deposits showed a basal-to-apical gradient. The mean LS and number of segments with LGE were similar across the 3 CA types. LS correlated with LGE and amyloid burden (r = 0.72). LGE was seen in the 6 basal segments in all WT-TTR patients. During the median follow-up of 11 months (range 4 to 17 months), 36 (46%) patients experienced MACE. Independent predictors of MACE were apical LS (cutoff, −14.5%), N-terminal pro–B-type natriuretic peptide (cutoff, 4,000 ng/l), and New York Heart Association functional class III to IV heart failure. Conclusions Basal-to-apical LS abnormalities are similar across CA types and reflect the amyloid burden. Apical LS independently predicts MACE.

Cardiac involvement is common in sickle cell disease (SCD). Studies are needed to establish haematological determinants of this involvement and prognostic markers. The aim of the study was to identify haematological factors associated with cardiac involvement in SCD and their impact on prognosis.This longitudinal observational study was performed on 1780 SCD patients with SS or S-β(0)-thalassemia referred to our centre. Six hundred fifty-six met our inclusion criteria (availability of a blood-workup and echocardiogram obtained1 year apart, no heart valve surgery and no current pregnancy). Median age was 31 (interquartile range, 25-40) years, and median haemoglobin (Hb) was 87 (80-95)g/L. Left ventricular (LV) dilation, left atrial dilation, cardiac index (CI)4 L/min/m(2), LV ejection fraction55%, and tricuspid regurgitant velocity (TRV) ≥2.5 m/s were found in 35, 78, 23, 8.5, and 17% of patients, respectively. Compared with other patients, those in the fourth quartiles (Q4) of LV end-diastolic dimension index (LVEDDind) and left atrial dimension index (LADind) and those with high CI had significantly lower Hb, % foetal Hb (HbF), and red blood cell (RBC) counts; and significantly higher lactate dehydrogenase, bilirubin, and %dense RBCs. Independent haematologic determinants of Q4 LVEDDind and LADind were low RBC count and %HbF; high %dense RBCs were associated with LADind. Low %HbF and RBC count were associated with high CI. High %dense RBCs or no α-thalassemia gene deletion was associated with greater severity of anaemia and cardiac dilation and with higher CI. During the median follow-up of 48 (32-59) months, 50 (7.6%) patients died. Tricuspid regurgitant velocity ≥ 2.5 m/s was a predictor of mortality. The risk of death increased four-fold when left ventricular ejection fraction55% was present also (P = 0.0001).Cardiac dilation and CI elevation in patients with SCD are associated with haematologic variables reflecting haemolysis, RBC rigidity, and blood viscosity. Tricuspid regurgitant velocity ≥ 2.5 and LV dysfunction (even mild) predict mortality.

Aims Sleep-disordered breathing (SDB) is highly prevalent and of adverse prognostic significance in patients with chronic heart failure (CHF). Polygraphy is used for diagnosing SDB but polygraphy resources fall short of needs. Here, our aim was to develop a score for SDB screening in patients with CHF. Methods and results Consecutive patients with stable chronic CHF referred to our CHF clinic for a scheduled follow-up evaluation were included prospectively between 2000 and 2012. SDB was defined as an apnoea–hypopnoea index ≥ 5/h as assessed by routine polygraphy. A screening score was developed as a linear combination of factors independently associated with SDB by multivariate logistic regression. Calibration and discrimination were evaluated using the Hosmer–Lemeshow (HL) test and area under the receiver-operating characteristics curve (AUC), respectively. Bootstrapping was performed to assess internal validity. Of 450 included patients (mean age, 59.5 ± 13.7 years), 397 (88%) had SDB. An easy-to-use score was based on age (2 points if ≥65 years), body mass index (2 points if ≥25 kg/m 2 ), New York Heart Association (NYHA) class (2 points if ≥3 or 4) and male gender (3 points). A score cut-off of 5 was 78.9% sensitive and 61.5% specific for SDB . The final model exhibited adequate calibration ( p HL ≥ 0.3) and discrimination (AUC, 0.737; 95% confidence interval, 0.663; 0.810). Conclusion An easy-to-use clinical score combining age, body mass index, NYHA class, and gender may help to identify those CHF patients most likely to have SDB, thereby improving the allocation of scarce polygraphy resources and early diagnosis of SDB.

To evaluate myocardial enhancement of patients with cardiac amyloidosis (CA) using computed tomography (CT). Thirteen patients with CA and 11 control patients were examined with first-pass and delayed CT acquisition. A qualitative and quantitative analysis of images was performed. Myocardial attenuation, myocardial signal-to-noise ratio (SNRmyoc), blood pool SNR (SNRblood), contrast-to-noise ratio between blood pool and myocardium (CNRblood-myoc) and relative attenuation index (RAI) defined as variation of myocardial attenuation between delayed and first-pass acquisitions were calculated. Two false negative cases (15 %) and three false positive cases (27 %) were detected on qualitative analysis. SNRmyoc of patients with CA was significantly (p

Senile systemic amyloidosis (SSA) is characterized by infiltration of amyloid transthyretin fibrils in the myocardium. SSA occurs mainly (but not always) in elderly men. SSA leads to hypertrophic and/or restrictive cardiomyopathy complicated by conduction disturbances, atrial arrhythmia and systemic embolization (stroke…). That is why SSA needs a special care and to be diagnosed. Cardiac SSA diagnosis needs to exclude two other forms of cardiac amyloidosis: AL amyloidosis (light chain) and hereditary transthyretin amyloidosis (genetic testing). Scintigraphic 99mTc-DPD heart retention is observed in cardiac amyloidosis. DPD heart retention is more frequent in cardiac transthyretin amyloidosis than in cardiac AL amyloidosis. Specific treatments of cardiac TTR amyloidosis are in development.

Purpose To detect if a difference of T2 ratio, defined as the signal intensity (SI) of the myocardium divided by the SI of the skeletal muscle on T2-weigthed cardiac magnetic resonance (CMR) imaging, exists between patients with systemic amyloidosis, by comparison to control subjects. To determine if a relationship exists between T2 ratio and the overall mortality. Materials and methods CMR imaging examinations of 73 consecutive patients (48 men, 25 women; mean age, 63 years ±15 [SD]) with amyloidosis and suspicion of CA and 27 control subjects were retrospectively analyzed after institutional review board approval. Final diagnosis of CA was retained in case of histological confirmation of CA, typical pattern of CA on imaging and/or positivity of 99Technetium-hydroxymethylene diphosphonate scintigraphy. Patients were divided in 2 groups according to the presence or the absence of CA. T2 ratios were calculated in patients with and those without CA and in control subjects with using analysis of variance. Prognostic value of T2 ratio was studied with a Kaplan–Meier curve. Results Thirty-five patients (51%) had CA and 33 (49%) were free from CA. T2 ratio was lower in patients with CA (1.18 ± 0.29) than in patients without cardiac involvement (1.37 ± 0.35) (P = 0.03) and control subjects (1.45 ± 0.24) (P = 0.004). A T2 ratio of 1.36 was the best threshold value for predicting CA with a sensitivity of 63% and a specificity of 73%. Kaplan–Meier analysis showed a significant relationship between a shortened overall survival and a T2 ratio Conclusion Patients with CA exhibit lower T2 ratio on CMR imaging by comparison with patients free of CA and control subjects.

International audience; 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 \textless 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

Amyloidosis is characterized by extracellular deposits of insoluble proteins that cause tissue damage. The three main types are monoclonal light chain (AL), wild-type transthyretin (wt-TTR) and mutated transthyretin (m-TTR) amyloidosis. Cardiac amyloidosis (CA) raises diagnostic challenges.To assess the diagnostic accuracy of (99m)Tc-HMDP-scintigraphy for typing CA, differentiating CA from non-amyloid left ventricle hypertrophy (LVH), and predicting outcomes.121 patients with suspected CA underwent (99m)Tc-HMDP-scintigraphy in addition to standard investigations.CA was diagnosed in all AL (n = 14) and wt-TTR (n = 21). Among m-TTR (n = 34), 26 had CA, 4 neuropathy without CA and 4 were asymptomatic carriers. Of the 52 patients with non-amyloid heart disease, 37 had LVH and served as controls. (99m)Tc-HMDP cardiac uptake occurred in all wt-TTR, in m-TTR with CA except two and in one AL. A visual score ≥ 2 was 100% specific for diagnosing TTR-CA. Among TTR-CA, heart-to-skull retention (HR/SR) correlated with CA severity (LVEF and NT-proBNP). Median follow-up was 111 days (50;343). In a multivariate Cox model including clinical, echocardiographic and scintigraphic variables, NYHA III-IV and HR/SR 1.94 predicted acute heart failure and/or death.This preliminary study suggests that (99m)Tc-HMDP-scintigraphy may aid differentiation between transthyretin and AL-CA as well as CA from other LVHs. (99m)Tc-HMDP-scintigraphy appears to provide prognostic information in CA.

Sleep disordered breathing (SDB) is common in patients with heart failure with reduced ejection fraction (HFrEF). An increased apnea-hypopnea index (AHI) is associated with poor outcomes. We examined whether an analysis of nocturnal desaturations (NDs) can improve the risk stratification.Three-hundred seventy-six consecutive patients with stable chronic HFrEF and LVEF ≤ 45% were prospectively screened using polygraphy. Sleep apnea (SA) was defined as an AHI ≥ 15. The mean age was 59 ± 13 years, the mean LVEF was 30 ± 6%, and the median AHI was 18 [IQR: 9.33). The composite end-point of death, heart transplantation or LV assistance occurred in 98 patients (26%) within 3 years. Minimal oxygen saturation (MOS) during sleep, the number of desaturations90%/h and the time spent with oxygen saturation90% were significantly associated with adverse events (adjusted HR 1.25 [1.03-1.52], 1.25 [1.03-1.53], and 1.28 [1.04-1.59]), whereas the AHI was not (1.10 [0.86-1.39]). The best MOS cut-off value for poor outcomes was ≤ 88%. The patients with an MOS ≤ 88% had a significantly higher event rate (31.9%) than those with an MOS88% (15.6%; p0.01). The risk assessment using an MOS of ≤ 88% in addition to established prognostic markers yielded a net reclassification index (NRI) of nearly 6% and was particularly useful in the subgroup of patients with events (NRI: 8.4%).In HFrEF patients, ND ≤ 88% appears to be predictive of adverse events, independent of the presence of SA. This suggests that the risk assessment in HFrEF should also include ND in top of AHI.

The present study was undertaken to find potent molecules against the toxicity of nitrogen mustard mechlorethamine (HN2) on respiratory epithelial cells, using a human bronchial epithelial cell line (16HBE14o-) as an in vitro model. The compounds examined included inhibitors of poly(ADP-ribose) polymerase (PARP), sulfhydryl-group donors as nucleophiles, and iron chelators and inhibitors of lipid peroxidation as antioxidants. Their effectiveness was determined upon observance of metabolic dysfunction induced by HN2 following a 4-h exposure, using (3-(4,5-dimethylthiazole-2-yl)-2,5-diphenyl tetrazolium bromide (MTT) reduction and ATP-level assays as indicators. Moreover, the fluorescent probe, monobromobimane (mBBr), and 2',7'-dichlorofluorescin-diacetate (H 2 DCF-DA) were used to assess intracellular sulfhydryl and peroxide level modifications by flow cytometry, respectively, following a 3-h exposure. At last, cell death was assessed by flow cytometry using the propidium iodide (PI)-dye-exclusion assay following 24-h exposure. PARP inhibitors (niacinamide, 3-aminobenzamide, 6(5H)-phenanthridinone), and two sulfhydryl-group donors (N-acetylcysteine, WR-1065) were found to be effective in preventing HN2-induced metabolic dysfunction when added in immediate or delayed treatment with HN2. Only N-acetylcysteine, however, was found to prevent cell death induced by HN2, though it must be present at the time of the HN2 challenge. Flow cytometric measurements of intracellular sulfhydryl levels strongly suggested that N-acetylcysteine and WR-1065 are preventive in alkylation of cellular compounds, mainly by direct extracellular interaction with HN2. PARP inhibitors prevent secondary deleterious effects induced by HN2, considering metabolism dysfunction as the endpoint. Elsewhere, the oxidative stress appears to be a side effect in HN2 toxicity only upon considering the inefficiency of several antioxidants.

BACKGROUND: Familial amyloid polyneuropathy (FAP) mainly targets the peripheral nervous system and heart. Early noninvasive detection of cardiac impairment is critical for therapeutic management. AIM: To assess if amino-terminal pro-brain natriuretic peptide (NT-proBNP) or troponin T (cTnT) can predict echocardiographic left-ventricle (LV) impairment in FAP. METHODS: Thirty-six asymptomatic carriers and patients with FAP had echocardiographic measurement of left-ventricular (LV) systolic function, hypertrophy (LVH) and estimation of filling pressure (FP). RESULTS: Overall, median age, NT-proBNP, and LV ejection fraction were, respectively, 59 years (41-74), 323 pg/ml (58-1960), and 60% (51-66). Twelve patients had increased cTnT. Prevalence of ATTR gene mutations was 53% for Val30Met. Four individuals were asymptomatic, 6 patients had isolated neurological clinical signs, and 26 had echo-LV abnormalities. The ROC curve identified NT-proBNP patients with echo-LV abnormalities (area: 0.92; (0.83-0.99), p = 0.001) at a threshold >82 pg/ml with a sensitivity of 92%, and a specificity of 90%. Increased in NT-proBNP occurred in patients with SD and/or LVH with or without increase in FP. Elevated cTnT (>0.01 ng/ml) was only observed in patients with LVH and systolic dysfunction, with or without FP. CONCLUSION: In FAP, NT-proBNP was associated with cardiac impairment suggesting that NT-proBNP could be used in carriers or in FAP patients with only neurologic symptoms for identifying the appropriate time to start cardiac echocardiographic assessment and follow-up. cTnT identified patients with severe cardiac disease.

Aims To determine whether severity patterns or nocturnal ventilation to treat sleep-disordered breathing (SDB) during chronic heart failure (CHF) is associated with adverse outcomes. Although SDB is frequent during CHF, the relationships between SDB and CHF outcomes are unknown. Methods and results A total of 384 CHF patients (82% men, mean age 59 ± 13 years) with a left ventricular ejection fraction (LVEF) of ≤45% (mean LVEF 29 ± 9%) were assessed by polygraphy in our clinic between 2001 and 2009. Nocturnal ventilation was started according to the severity of SDB. Combined endpoints were death, heart transplant, and implant of a ventricular assist device. The prevalence of obstructive sleep apnoea (OSA), central sleep apnoea (CSA), and Cheyne–Stokes respiration (CSR) was 62, 26, and 29%, respectively. A primary endpoint occurred in 31%. Mean follow-up for survivors was 47 ± 25 months. Those with moderate [apnoea–hypopnoea index (AHI) ≤5–20/h] and severe SDB (AHI ≥20/h), and OSA and CSA, had poor prognoses compared with patients without SDB (P = 0.036, P = 0.003, respectively). A total of 31% of SDB patients were treated with nocturnal ventilation. Treated SDB had a better outcome than untreated severe SDB after adjustment for confounding factors [P = 0.031; hazard ratio (HR) 0.56; 95% confidence interval (CI) 0.33–0.95]. Subgroup analysis that included only OSA showed a similar result after adjustment (P = 0.017; HR 0.40; 95% CI 0.19–0.95). Conclusions In CHF, SDB is associated with a poor prognosis whatever the SDB pattern, and nocturnal ventilation is associated with a better outcome.

BACKGROUND: Normal values and the prognostic significance of right ventricle (RV)-2D strain in chronic heart failure (CHF) patients are unknown. METHODS AND RESULTS: Between 2005 and 2010, we prospectively enrolled 43 controls and 118 stable CHF patients. Standard echocardiographic variables, tricuspid annular plane systolic excursion, peak systolic velocity of tricuspid annular motion using tissue Doppler imaging, and RV and left ventricle (LV) 2D-strain were measured. The primary outcome was death or emergency transplantation or emergency ventricular assist device implantation or acute heart failure. RV-2D strain was measurable in 39 controls (58±17 years, 50% men), whose median value was 30% (95% confidence interval [95%CI], 39%; 20%); and in 104 CHF patients (80% men, mean age 57±11 years, and mean LV ejection fraction 29%±8%), whose median value was 19% (95%CI, 34%; 9%). During the mean follow-up of 37±14 months, 44 experienced the primary outcome. By Cox proportional hazards multivariate analysis, only RV-2D strain and log B-type natriuretic peptide independently predicted experiencing the primary outcome within the first year. The best RV-2D strain cut-off by receiver-operating characteristics analysis was 21%, and patients with values >21% were at greatest risk (χ(2)-log-rank test=14.1, P

Background Transthyretin (TTR) familial amyloid polyneuropathy (FAP) is a fatal autosomal dominant neurodegenerative disease characterized by deposition of transthyretin targeting mainly the peripheral nervous system and the heart. Early noninvasive detection of cardiac impairment is of importance for the therapeutic management. Aim Assess if natriuretic peptide (NT-proBNP) or troponin T (cTnT) are reliable biomarkers to predict echocardiographic left ventricle (LV) impairment in a wide variety of TTR patients. Methods 36 asymptomatic carriers and patients with proven FAP genetic mutation had clinical, biological and echocardiography assessment of left ventricle (LV) systolic function (SD), filling pressure (FP) and hypertrophy (LVH) as marker of amyloid deposition Results In the all cohort, the median (IQR) age, NT-proBNP, LV ejection fraction were respectively 59 (41-74), 323pg/ml (58-1960) and 60% (51-66). 64% were men and 12 had an increased in cTnT. TTR gene mutations prevalence was 50% for Val30Met. 4 patients were asymptomatic, 6 had only neurologic clinical signs and 26 had echo-LV abnormalities with or without neurologic disorders. Their median NT-proBNP value were respectively: 33 (19-50), 54 (37-154) and 747 (253;2840). Using received-operator curve, NTproBNP identified significantly patients with echo-LV abnormalities (Area: 0.92;(0.83-0.99), p=0.001) with a threshold above 82pg/ml and a sensitivity of 92% and specificity of 90%. Elevated cTnT (superior to 0.01ng/ml) was only observed in patients combining impairment of LVH and SD or LVH, SD and FP. Conclusion In TTR amyloidosis, NTproBNP and troponin T are associated with LV impairment measured by echocardiography suggesting that NTproBNP could be useful in FAP carriers to start echocardiographic follow-up whereas troponin would identify patients with severe cardiac disease.

Cardiac amyloidosis (CA) is of poor prognosis and has three main causes: light chain (AL), hereditary (m-TTR) and wild type transthyretin amyloidosis (WT-TTR). Longitudinal 2D-strain (LS) has been proposed for early diagnosis of AL-CA with increased LS, indicating less contractility, in basal segments. LS apical sparing physiopathology and prognosis value are unknown in the 3 types of CA. Aims 1-Compare regional LV LS between the 3 types of CA, 2 – Determine if they correlate to amyloid deposits; 3 – Determine the prognostic value of LV LS. Methods 79 patients were prospectively included. 17 segments LV LS was measured. 53 had cardiac MRI with LGE evaluation for each segment. Amyloid deposition was quantified using Congo red staining in the 3 explanted heart following heart transplant (HT). All patients were followedup. The composite primary endpoint was major adverse cardiac event (MACE) i.e., death, HT, and acute heart failure. MACE predictors were identified by multivariate analysis. Results 26 patients had AL, 36 m-TTR and 17 WT-TTR CA. Global (–10±4%) and regional 17 LV LS were similarly impaired in the 3 types of CA. 91% of MRI had LV LGE enhancement. Both LGE and LS analysis showed a basal to apex gradient. Interestingly LS in the basal free wall (mean – 5±6%) had the best accuracy to predict LGE (AUC=0.97, P=0.002; 95%CI {0.92-0.99}). Amyloid deposits in the 3 HT were more frequent in the basal segments and a strong correlation was found between LGE and LS segments (r=0.72). 46% experienced MACE during the follow-up with median of 13[7;23] months for survivors. Apical LS, NT-proBNP and NYHA III-IV were independently associated with MACE. Cut-off to predict MACE was –14.5% for Apical LS and 4000ng/L for NT-proBNP. Conclusions LS abnormalities are similar between CA types and reflect amyloid deposition which predominates in basal LV segments. Impairment of basal free wall LS predict LV MRI LGE. High value of apical LS is an independent predictor of MACE.

BackgroundSleep disordered breathing (SDB) is common in patients with heart failure with reduced ejection fraction (HFrEF). Increased apnoeahypopnoea index (AHI) is predictive of poor outcome. Nocturnal desaturation (ND) is associated with increase in NT-proBNP in HFrEF. The prognostic value of ND as compared to AHI is still unknown.Methods and ResultsThree-hundred seventy six consecutive patients with stable chronic HFrEF and left ventricular ejection fraction (LVEF) ≤45% were prospectively screened for SDB between 2005 and 2010 by polygraphy. SDB was defined by an AHI≥5 and sleep apnea (SA) by an AHI≥15. Mean age was 59±13 years, mean LVEF 30%±6%, and mean AHI 18±10; 310 patients (82%) had SDB. The predefined composite primary end-point (death, heart transplantation or left ventricular assistance) occurred in 98 patients (26%) within 3 years. Minimal oxygen saturation (MOS) during sleep, number of desaturations 88% (p

BackgroundAccuracy of 99mTc-HMDP scintigraphy (sHMDP) to diagnose cardiac amyloidosis (CA) and to discriminate between the different etiologies i.e hereditary TTR amyloidosis (hTTR), senile amyloidosis (wt-TTR) and light chain amyloidosis (AL) are unknown.Methods122 patients referred for suspected CA were prospectively studied with sHMDP. HMDP fixation was evaluated by a visual score (0 up to 3). Heart-to-whole-body (H/WB) and heart-to-skull (H/S) ratio were calculated on planar images. Diagnosis of amyloidosis was biopsy proven for all AL. Diagnosis of CA for hTTR and wt-TTR was established by an experts consensus based on echocardiography, cardiac MRI (cardiac wall thickness ≥12mm), genetic testing and biopsy whenever ethically possible.ResultsThe mean age was 69 (59; 78), 71% were men; of whom, 14 (11%) were considered having AL, 34 (28%) hTTR, 21 (17%) wt-TTR and 53 (43%) had cardiac hypertrophy from other causes. Of the 61 (50%) patients with CA, 46 (75%) had a HMDP visual scoring ≥1; of whom 1/14 (7%) had AL, 24/26 (92%) hTTR and 21/21 (100%) wt-TTR.Cardiac HMPD uptake was absent among all patients without CA (n=61). Visual score was stronger in TTR-CA versus AL-CA (p

International audience; Background Hereditary transthyretin cardiac amyloidosis (mTTR-CA) is a hypertrophic cardiomyopathy with challenging diagnosis and poor prognosis. The prevalence of m-TTR in patients with increased left ventricular wall thickness (LVWT) is unknown. Methods Prospective and consecutive multicenter study with systematic genetic screening for mTTR in adult patients with LVWT ≥15mm included in cardiology primary clinics. Results 298 patients were genotyped of whom 23% were African descendant. The median (IQR) age was 62(50,74), 74% were men and 36% were in NYHA class III-IV. The median of maximal LV thickness was 18 (16, 21)mm.17 patients had TTR mutation (5.7%) of whom 15 (5.0) had confirmed mTTR-CA. All the mTTR-CA were ≥55years meaning that the prevalence of mTTR-CA was 8.3% above this age. Of the 15 with mTTR-CA, 8 were Africans and 6 Caucasians. In Africans ≥55 years, the prevalence was 22% and reached 35% in those over 65 years. The most frequent mutations were V142I (8), V50M (2) and I127V (2). When adjusted to age, neuropathy (OR=20.1; 95%-CI, 5.86-69.4; P