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To evaluate the real-life use of a modified Gillmore algorithm with a "one-stop-shop" approach, bone scintigraphy (BS), a monoclonal gammopathy test (GT), a salivary gland biopsy (SGB), and genetic testing performed at the same time for the diagnosis of cardiac amyloidosis at the French National Reference Centre for Cardiac Amyloidosis (Henri Mondor Hospital, Créteil, France).This retrospective cohort study included a total of 1222 patients with suspected amyloidosis who underwent BS and GT between June 2008 and May 2019.Of 1222 patients, 349 had no cardiac uptake on BS and negative GT (BS-/GT-), 276 were BS-/GT positive (GT+), 420 patients were BS+/GT-, and 177 were BS+/GT+. Our one-stop-shop check-up enabled us to diagnose 892 (72.9%) patients; 330 (27.0%) patients required additional examinations, such as mass spectrometry and/or a cardiac biopsy. This subset notably included 112 patients with amyloid light chain amyloidosis. More than 64% of the patients with transthyretin amyloidosis or another type of amyloidosis were diagnosed during the one-stop shop visit. Sensitivity and specificity of BS for transthyretin amyloidosis diagnosis was 99% and 96%, respectively. For amyloid light chain diagnosis, sensitivity and specificity were 100% and 76%, respectively, for GT and 54% and 100%, respectively, for SGB. Of 910 transthyretin genetic tests, 205 (17%) detected mutations.The results of our real-life cohort study confirmed the ability of a one-stop-shop approach with a modified Gillmore algorithm to diagnose cardiac amyloidosis and the interest of simultaneous testing for earlier diagnosis. The SGB has diagnostic value because it is easy, quick, and less invasive than a cardiac biopsy.

Introduction Hereditary (ATTRv) and senile (ATTRwt) transthyretin amyloidosis are severe and fatal systemic diseases, characterised by amyloid fibrillar accumulation principally in the heart or peripheral nerves (or both). Since 2012, tafamidis is used in France to treat patients with ATTRv with neuropathy (alone or with cardiomyopathy). Recently, the Phase III ATTRACT trial showed that tafamidis decreased the relative risk of mortality in ATTR amyloidosis with cardiomyopathy. Purpose The aims of this study were to assess the clinical characteristics of ATTR amyloidosis in a real-life population in comparison to the population included in the ATTRACT trial and to assess the impact of tafamidis on major cardiovascular outcome-free (MCO-free) survival time without cardiac decompensation, heart transplant, or death. Methods From June 2008 to November 2018, 648 patients with ATTR amyloidosis (423 ATTRwt and 225 ATTRv) consecutively referred to the French Referral Center for cardiac amyloidosis were included. 467 (72%) patients matched the inclusion criteria of the ATTRACT study. Results For the 631 patients with cardiomyopathy, median MCO-free survival time increased with tafamidis treatment (N = 98): 1565 (1010–2400) days vs. 771 (686–895) days without treatment (log-rank P Conclusion In a large cohort of ATTRwt and ATTRv patients, representative of the inclusion criteria of the ATTRACT trial, the present results confirm the positive impact of tafamidis treatment on preventing the occurrence of cardiovascular outcome in a real-life population.

Background The prevalence of small-fiber neuropathy (SFN) is high in patients with hereditary transthyretin amyloidosis (ATTRv) but remains unknown in transthyretin wild-type cardiac amyloidosis (ATTRwt-CA). Objective To determine the prevalence of SFN in patients with ATTRwt-CA using Sudoscan®, a non-invasive method used to provide evidence of SFN in clinical practice and based on measurement of electrochemical skin conductance at the hands (hESC) and feet (fESC). Methods A series of 62 non-diabetic patients with ATTRwt-CA was prospectively included over 2 years and compared to healthy elderly subjects, matched on age, gender and body mass index. The presence of SFN was defined as hESC Results Mean hESC and fESC were significantly lower in patients with ATTRwt-CA than in elderly controls: 61.5 vs. 70.0 μS (P = 0.006) and 68.3 vs. 76.9 μS respectively (P Conclusion A reduced fESC was observed in almost 50% of patients with ATTRwt-CA and was associated with a worse prognosis. Sudoscan® could be used easily to screen ATTRwt-CA patients for the presence of SFN and identify patients at higher risk of outcome.

Background Light-chain amyloidosis (AL) with cardiac involvement carries a poor prognosis; median untreated survival is Purpose Our study evaluates the effect on mortality of first-line chemotherapy administered in combined or in sequential in patients with cardiac AL. Methods Two hundred and sixty-two patients diagnosed with AL amyloidosis were retrospectively studied, since 2008. Among these patients, 34% already received chemotherapy and 15% were not treated or treated in another hospital. 51% patients newly diagnosed had a medical follow-up in our center, of whom 3% died before receiving chemotherapy. A total of 131 patients were analyzed. Initial chemotherapies administered were classified into 4 groups according to the sequence of administration: combination of Velcade (Bortzezomib)/Cyclophosphamide/Dexamethasone (VADcomb), sequential administration of Dexamethasone/Velcade/Cyclophosphamide (DCVseq) or Cyclophosphamide/Velcade/Dexamethasone (CVDseq) and other chemotherapy. Survival was assessed using a log-rank test ( Fig. 1 , Table 1 ). Results Among 131 treated patients (65,5years ± 11.6, 63% male, 73% lambda subtype), 57 received CVDseq, 18 DCVseq, 38 VADcomb and 18 other chemotherapy. At 1 month, mortality was 16.5%, and mortality per group was 17% in CVDseq group, 16.7% in DCVseq group, 16.7% in other chemotherapy group and 15.8% in VADcomb group. Overall mortality is 45% and mortality per group is 40% with CVDseq, 44% with DCV seq, 50% with VADcomb and 50% with other chemotherapy. Conclusion Overall mortality and mortality at 1 month were not significantly different between the 4 groups (respectively P = 0.785 and P = 0.99). There is no difference of mortality in the cohort whether the chemotherapy is administered sequentially or in combination.

Background Left atrial (LA) dysfunction (LAD) and thrombi have been described in systemic light chain (AL) cardiac amyloidosis (CA). The causes and consequences of these alterations are unclear. Purpose To (1) demonstrate that LA 2D-strain echocardiography (2D-SE) is useful to analyze LAD; (2) explore causes, consequences and association of this dysfunction with thromboembolic events (TE) in the three major types of CA. Methods and results Seventy-six patients with CA and in sinus-rhythm were included. 21 had AL, 42 hereditary transthyretin (m-TTR) and 13 wild-type (wt-TTR) CA. History of TE was recorded at baseline. Global and regional LA longitudinal strain (LS) were measured using 2D-SE. Peak atrial LS (PALS-4C) was negatively correlated to NT-proBNP (r = −0.6, P Conclusions Atrial function estimated by 2D-SE is useful to identify patients at risk of death and might be helpful to select those that may benefit from preventive anticoagulant treatment.

Introduction Cardiac amyloidosis (CA) is a severe disease, often lethal. Little is known about cause of death during the natural course of CA. Identification of cause of death during CA is of particular interest to design clinical trials and identify therapeutic targets in this pathology. We aimed to describe mode of death in CA patients during long-term follow-up, according to the type of amyloidosis. Material and methods All patients consecutively referred to the French referral center for light-chain (AL), transthyretin hereditary (hTTR) or wild-type (WT-TTR) CA were consecutively included between 2010 and 2016 were included. Mode of death was centrally adjudicated by two blinded clinical committees, using multiple sources, and classified as cardiovascular (including worsening heart failure, sudden death, stroke) or non-cardiovascular (1). Results From the 565 patients included, 187 patients had AL amyloidosis, 220 had hTTR and 158 had WT-TTR amyloidosis. One hundred thirty-nine patients (25%) died during a follow-up of 864 patients-years, with median follow-up in survivors 14.6 months (IQR 3.7–33.9). One-year survival was 67% in AL patients, 92% in hTTR patients and 89% in WT-TTR patients (logrank Discussion and Conclusions Mortality is high during natural course of cardiac amyloidosis, significantly different according to CA type. Main causes of death were cardiovascular (mostly worsening heart failure and sudden death), opening room for optimal prevention and management.

Introduction Amyloidosis is a severe disease in which proteins (amyloid fibrils) build up in tissues. Numerous organs can be affected, resulting in multiple vascular, cardiac, neuropathic, digestive and kidney dysfunctions. Three forms of amyloidosis particularly affect the heart: AL amyloidosis (hematologic) and hereditary or senile transthyretin-related amyloidosis. These dysfunctions disable the patient and induce a loss of independence and a decline of his quality of life. Objectives are to improving global treatment for patients suffering from amyloidosis by integrating it to a therapeutic education approach. Maintaining their quality of life by offering them knowledge and skills to better handle their disease. Methods Carrying out a global therapeutic education program for patients suffering from amyloidosis, with the participation of ‘resource patients’ and of the French Association Against Amyloidosis (from conception until final evaluation). Results Twenty-one therapeutic education workshops have been created for patients and their helpers (cf. Fig. 1 below) and organized by type of dysfunction, of treatment, of therapeutic approach or support of which 5 work groups prevent denutrition and adapt to the type of denutrition. Creating a self-completed ‘AmyloAFFECT’ questionnaire, coupled with an initial check-up, makes it possible to assess patients’ needs and direct them towards work groups. The program is evaluated through assessments of acquired skills and whether or not quality of life is maintained (ODD, AmyloBURDEN). Conclusions Amylo-PEP has been created two years ago and is validated by the Regional Health Authority since 2017. Evaluating this program will assess if to some extent it has improved or stabilized quality of life for patients suffering from cardiac amyloidosis, as well as if it has had a positive impact on knowledge and understanding, on the patient's global treatment and on communication within the multidisciplinary team.

Background Care pathways are developed to improve the treatment of heart failure (HF), with uncomplete successful because of lack of time and human medical resources. New technology, numerical tools, and cooperation between doctors and nurses could be answers. Objectives (1) get going medication titration, (2) provide a quick answer to early cardiac decompensation, (3) improve in-hospital pathway and discharge coordination, (4) promote home care, and (5) reduce readmission rate. Methods We transfer knowledge and skills from cardiologists to specialized nurses (cooperation work) in the field of chronic HF: medication titration protocol, telemonitoring, and acute HF management. Medical treatment for HF will be optimized at regular intervals, fitting with European guidelines, by HF nurse under the responsibility of a cardiologist as part of a cooperation protocol. At home, symptoms will be monitored by connected scale and tablet transmitting daily weight and answers to targeted questions. Presence of signs of acute HF sets off an immediate evaluation firstly by phone, then by a face-to-face consultation if needed. HF nurse will be in the front line for this early evaluation in order to reduce the delay between symptoms and therapeutic adaptations, and to refer the patient to the most appropriate structure (GP, cardiologist, hospitalisation or home care), without passing through emergency units, while promoting ambulatory care and improving in-hospital pathway. This cooperation protocol aims to be nationally transposed, to all interested heart failure team, in the hospital or in the office. Perspectives With this new model of cooperation, transfer of skills to HF nurses will enhance the quality of care for HF patients, while medical time will be redistributed to complex cases. Inscription to a specific formation for HF nurses will be possible for academic year 2018-2019, and accession to the cooperation protocol is expected for 2019.

Introduction Cardiac Amyloidosis (CA) is caused by the extracellular accumulation of insoluble fibrillar proteins. The most common types of CA are AL amyloidosis (light-chain), transthyretin amyloidosis: Hereditary (hTTR) or wild-type (wTTR). Without treatment and adequate care management, the prognosis for CA is poor. Heart transplantation is remains the final cardiac therapeutic option. Methods This is a retrospective study that included all patients who had a heart transplant for CA from 2005 to 2018 (n = 18). We report the baseline and post-transplant data follow-up. Results Eighteen patients had a heart transplant: 13 (72%) had AL amyloidosis and 5 had hTTR (28%). The average age was 56 years (43 to 66 years), there were 8 women (44%). Four patients had combined heart and liver transplant (all of them were hTTR). Four patients had combined heart and kidney transplant (all of them were AL). All AL patients received at least one cycle of chemotherapy before the surgery and 8 (62%) were in partial remission. Six patients (33%) were urgently transplanted. The 30-day survival is 100%, the one-year survival was 75%. The deaths occurred only in AL: 3 died from infection and 1 from severe hemorrhage (scarpa rupture), of which one with heart and kidney. No deaths at 1 year were associated with a recurrence of amyloidosis. In most AL with partial remission before the surgery, a slight decrease of light-chains under immunosuppressive treatments was observed. Conclusions Heart transplant is a therapeutic option for selected CA in AL and hTTR and can be combined with kidney or liver transplant. In severe AL CA cardiac transplant need to perform before expecting the complete remission as immunosuppressive therapy may stabilize or decrease the light-chain after the transplant. The survival of transplant CA patients is comparable to that of non-AC patients, and may have better survival than those with a single heart transplant.

Background In paradigm-HF study, LCZ696 was superior to angiotensin converting enzyme inhibitors (ACEI) and reduced mortality in heart failure with reduced ejection fraction (HFrEF) patients. LCZ696 is now recommended in symptomatic patients with HFrEF despite optimal treatment with beta-blockers, ACEI and mineralocorticoid receptor antagonists (MRA). Purpose To evaluate LCZ696 prescription in real-life in and outpatients. Methods Prospective enrolment during 6 months in 2017. Data collection included demographic, burden, clinical, biological, echocardiography and treatment characteristics. Results A total of 1442 patients with left-ventricular ejection fraction (LVEF) Conclusion Almost one in four patients with LVEF

Introduction Amyloidosis is a disease caused by infiltration of tissues by an amyloid protein. The most frequent cardiac amyloidosis is light chain (AL) amyloidosis, hereditary transthyretin (TTR-h) and wild transthyretin (w-TTR). Objective Describe baseline clinical, biological, echocardiographic characteristics and prognostic of patients referred for cardiac amyloidosis in the Amyloidosis expert center. Methods From 2010 to July 2016, all patients referred to the expert center of our Hospital for suspected cardiac amyloidosis were included. Baseline demographic, clinical, laboratory and ultrasound characteristics were recorded and patients were followed-up for major events including death, cardiac transplantation and left ventricular assistance implant. Results Of the 942 patients referred for suspicion of CA, 502 had confirmed CA of whom162 had AL amyloidosis, 203 h-TTR and 141 w-TTR. CA were men in 60%, the median age was 69 years w-TTR amyloidosis were older (83 [79; 87], P Conclusion Cardiac amyloidosis is more frequent than initially thought and has poor prognosis. The challenge is to achieve earlier diagnosis to improve prognosis and treatment.

Objective A decreased longitudinal strain in basal segments with a base-to-apex gradient has been described inpatients with cardiac amyloidosis (CA). Aim of the study was to investigate the left ventricular (LV) regional distribution of early-phase 99mTc-hydroxymethylene diphosphonate (HMDP) uptake inpatients with transthyretin-related cardiac amyloidosis (TTR-CA). Methods All patients underwent a whole-body planar 99mTc-HMDP scintigraphy acquired at 10 min postinjection (early-phase) followed by a thorax SPECT/CT. The relative segmental uptake % was investigated on AHA 17-segment model and 3-segment model (basal, mid-cavity, apical). Results Sixty-one TTR-CA patients were included of whom; 29 were wild-type (wt-TTR-CA) and 32 had hereditary TTR-CA (m-TTR-CA). Early myocardial 99mTc-HMDP uptake occurred in all TTR-CA. In all patients, segmental analysis of the LV myocardial distribution of 99mTc-HMDP uptake showed an increased median uptake (interquartile range) in basal/mid-cavity segments compared to the lowest median uptake of apical segments (respectively 79 [72–86] vs. 72 [64–81]; P Conclusions Early-phase myocardial scintigraphy identified regional distribution of 99mTc-HMDP uptake characterized by a base-to-apex gradient, corroborating echocardiographic and cardiac magnetic resonance findings. This relative “apical sparing” pattern was similar across TTR-CA and TTR mutations’ subtypes.

Background Early after heart transplantation, patients require close medical follow up and exercise training.The objective of the study was to assess the usefulness of an inpatients Cardiac Rehabilitation center (inCRC) in these two settings. Methods 100 consecutive patients (age:47.2±13.2, men:79%) referred to our inCRC less than 3 months after heart transplantation were included. Acute events (AE) occurring during the inCRC stay and exercise training modality and results were assessed. Results Patients were hospitalized in the inCRC 33.8±21.3 days after the transplantation, for a 25.9±11.2 days duration on average. During this period, AE occurred in 49% (n=49) of the patients: 24 graft rejection, 19 infections requiring IV antibiotherapy, and 6 other AE. Most of these events were managed by the inCRC but 22 patients were temporarily referred to the transplantation center because of too severe AE (infection: n=11, tamponade: n=1, acute rejection requiring plasmapheresis: n=3, other: n=7). Seventeen patients (17%) were colonized by a multiresistant bacterium. Finally, due to all these event, 23% (n=23) of the patients underwent only 5 exercise training sessions or less. The exercise capacity improvement after completion of a classical exercise training program (n=13±5 sessions) is given in the table below for the 77 other patients (77%). Conclusion Early after heart transplantation an inCRC can be useful to (i) safely treat subacute complications under the guidance of the referent transplantation center and (ii) perform an exercise training program. Abstract 0012 - Table Post-op day 33±21 (before rehabilitation) Post-op day 60±22 (after rehabilitation) P Peak VO2 (ml/kg/min) 12.7±3.2 17.2±4.0 Ventilatory Threshold (ml/kg/min) 9.9±2.5 12.2±3.7 Maximal Workload (Watts) 60±17 82±23 Resting Heart Rate (bpm) 91±14 89±13 0.6 Maximal Heart Rate (bpm) 107±16 115±18

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.

IntroductionIvabradine is a selective heart-rate-lowering agent that acts on the sinoatrial node without influence on myocardial contractility and cardiac output. Physiological effects of ivabradine during dobutamine infusion have never been studied.MethodsThe study included 11 healthy subjects (82% men, aged 29.1± 4.7 years). All underwent dobutamine transthoracic echocardiography before and after ivabradine (3 hours after 5mg of ivabradine). Baseline left ventricular longitudinal global strain by speckle tracking and stroke volume at rest and during low dose of dobutamine (when heart rate increased by more than 10 points from baseline) were compared to those obtained under ivabradine.ResultsBaseline heart rate (64±8bpm) decreased in 7 subjects (68±7bpm vs. 60±6bpm, P=0.009) but paradoxically increased in 3. Dobutamine rate required to increase the heart rate by 10 points was similar with and without ivabradine (11±2γ/kg/min vs. 12±3 γ/kg/min, P=0.4). Overall, global strain increased under low dose dobutamine before (−20±2% vs. −23±2%, P=0.008) and after ivabradine (−21±2% vs. −23±3%, P< 0.001). Interestingly, in the 7 subjects that responded to ivabradine, global strain (−23±2% vs. −25±1%, P=0.006) and stroke volume (102±17ml vs. 110±20ml, P=0.05) more increased under dobutamine with ivabradine.ConclusionIn healthy subjects, heart rate reduction obtained by ivabradine allows to increase myocardial contractility and stroke volume for a similar rate of dobutamine. This propriety may be useful in decompensate heart failure patients with rapid tachycardia induced by catecholamine infusion.

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