Publications by authors named "Thibaud Damy"

146 Publications

Diagnosis and Treatment of Iron Deficiency in Heart Failure: OFICSel study by the French Heart Failure Working Group.

ESC Heart Fail 2021 Feb 22. Epub 2021 Feb 22.

Department of Cardiology, Referral Centre for Cardiac Amyloidosis, CHU Henri Mondor, AP-HP, Créteil, France.

Aims: Iron deficiency (ID) occurs in about 50% of patients with heart failure (HF). The European Society of Cardiology (ESC) recommends ID diagnostic testing in newly diagnosed patients with HF and during follow-up, with intravenous iron supplementation (IS) only recommended in patients with HF with reduced ejection fraction (HFrEF). This study aimed to assess prevalence, clinical characteristics, and application of ESC guidelines for ID and IS in patients with HF in the real-life clinical setting.

Methods And Results: The French transversal multicentre OFICSel registry (300 cardiologists) conducted in 2017 included patients hospitalized for HF at least once in the previous 5 years. Diverse adult patients were eligible including inpatients and outpatients and those with acute and chronic HF. Data were collected from cardiologists and patients using study-specific surveys. Data included demographic and clinical data, as well as HF and ID management data. Overall, 2822 patients, mainly male (69.3%) with a median age of 69 years (interquartile range 58-78), were included. A total of 1075 patients (38.1%) were tested for ID, with 364 (33.9%) diagnosed. Of these, 168 (46.2%) received IS: 128 (76.2%) intravenous IS and 40 (23.8%) oral. Among the 201 patients with HFrEF diagnosed with ID, 99 (49.3%) received IS: 79 (79.8%) intravenous IS and 20 (20.2%) oral.

Conclusions: In clinical practice, only one-third of patients with HF had a diagnostic test for ID. In patients with ID with HFrEF, only 39.3% received intravenous IS as recommended. Thus, in general, cardiologists should be encouraged to follow the ESC guidelines to ensure optimal treatment for patients with HF.
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http://dx.doi.org/10.1002/ehf2.13245DOI Listing
February 2021

The Impact of Patients With Cardiac Amyloidosis in HFpEF Trials.

JACC Heart Fail 2021 Mar 3;9(3):169-178. Epub 2021 Feb 3.

French Referral Center for Cardiac Amyloidosis, GRC Amyloid Research Institute, Amyloidosis Mondor Network, and DHU A-TVB, Henri Mondor Teaching Hospital, APHP, Creteil, France; Cardiology Department, Henri Mondor Teaching Hospital, Creteil, France; Paris XII University, UPEC, and IMRB-INSERM U955, Creteil, France; Cardiology Outpatients Unit, Centre Cardiologique du Nord, Saint Denis, France.

Heart failure with preserved ejection fraction (HFpEF) is an increasingly diagnosed condition whose failure to respond to new drugs effective in heart failure with reduced ejection fraction is of great concern. HFpEF is an incompletely understood and markedly heterogeneous syndrome, but cardiac amyloidosis is increasingly recognized as one of its various causes. The specific hemodynamic and pathophysiological features of cardiac amyloidosis result in poor tolerance of heart failure medications and in worse outcomes compared with other causes. Until recently, patients considered for HFpEF trials were not routinely screened for cardiac amyloidosis. This review examines how real-world patients with cardiac amyloidosis met inclusion criteria for 8 major HFpEF clinical trials, including the recent PARAGON (Prospective Comparison of ARNI with ARB Global Outcomes in HF With Preserved Ejection Fraction) trial. This review discusses how the presence in the trial populations of a subset of patients with cardiac amyloidosis might contribute to explain the absence of efficacy of medications for HFpEF in trials so far. A multistep screening strategy is suggested in which patients with red flags for cardiac amyloidosis undergo both a light chain assay and technetium-labeled cardiac scintigraphy (technetium-labeled cardiac scintigraphy scan), which, when negative, rule out cardiac amyloidosis. Using this strategy would allow the testing of new medications for HFpEF in populations containing no patients with cardiac amyloidosis, thus potentially increasing the likelihood of showing therapeutic efficacy, and finally making some effective treatment available.
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http://dx.doi.org/10.1016/j.jchf.2020.12.005DOI Listing
March 2021

[Cardiac amyloidosis].

Ann Pathol 2021 Feb 6;41(1):25-37. Epub 2021 Jan 6.

Centre français de référence de l'amylose cardiaque (CRAC), réseau Cardiogen, GHU Henri-Mondor, Assistance publique-Hôpitaux de Paris, Créteil, France; Département de Cardiologie, GHU Henri-Mondor, Assistance publique-Hôpitaux de Paris, Créteil, France.

Different types of amyloid deposits involve the heart. Transthyretin and light chain amyloidosis are the most frequent. Diagnostic performance, typing and treatments have improved in the last decade, and prognosis of cardiac amyloidosis is now significantly better thanks to targeted therapies. In this article, we will describe the clinical manifestations of cardiac amyloidosis, the diagnostic approach and detail the characteristics and specific treatments of the most frequent types of cardiac amyloidosis. We will focus on the histopathological aspects, especially on the importance of amyloid typing.
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http://dx.doi.org/10.1016/j.annpat.2020.11.010DOI Listing
February 2021

Prevalence and prognostic value of autonomic neuropathy assessed by Sudoscan® in transthyretin wild-type cardiac amyloidosis.

ESC Heart Fail 2020 Dec 22. Epub 2020 Dec 22.

Cardiology Department, AP-HP (Assistance Publique-Hôpitaux de Paris), Henri Mondor University Hospital, 51 Avenue du Marechal de Lattre de Tassigny, Créteil, F-94010, France.

Aims: The prevalence of autonomic neuropathy (AN) is high in patients with hereditary transthyretin amyloidosis but remains unknown in transthyretin wild-type cardiac amyloidosis (ATTRwt-CA). This study aimed to determine the prevalence of AN in patients with ATTRwt-CA using Sudoscan®, a non-invasive method used to provide evidence of AN in clinical practice and based on measurement of electrochemical skin conductance at the hands and feet (fESC).

Methods And Results: A series of 62 non-diabetic patients with ATTRwt-CA was prospectively included over 2 years and compared with healthy elderly subjects, matched by age, gender, and body mass index. The presence of AN was defined as electrochemical skin conductance at the hands <60 μS and/or fESC <70 μS, and conductances were analysed according to clinical, biological, and echocardiographic data. Mean fESC was significantly lower in patients with ATTRwt-CA compared with elderly controls: 68.3 (64.1-72.5) vs. 76.9 (75.6-78.1) μS (P < 0.0001), respectively. Prevalence of fESC <70 μS was higher in ATTRwt-CA patients than in controls: 48.4% vs. 19.9%, P < 0.05. Univariate analysis showed that fESC, N-terminal pro-B-type natriuretic peptide, creatinine plasma levels, and echocardiographic global longitudinal strain were associated with decompensated cardiac failure and death. Multivariate analysis revealed that fESC was an independent prognostic factor, and Kaplan-Meier estimator evidenced a greater occurrence of cardiac decompensation and death in patients with fESC <70 μS, P = 0.046.

Conclusions: Reduced fESC was observed in almost 50% of patients with ATTRwt-CA and was associated with a worse prognosis. Sudoscan® could easily be used to screen ATTRwt-CA patients for the presence of AN and identify patients at higher risk for a poor outcome.
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http://dx.doi.org/10.1002/ehf2.13131DOI Listing
December 2020

Reply to the letter regarding the article 'Efficacy and safety of tafamidis doses in the Tafamidis in Transthyretin Cardiomyopathy Clinical Trial (ATTR-ACT) and long-term extension study'.

Eur J Heart Fail 2020 Dec 19. Epub 2020 Dec 19.

Stanford University School of Medicine, Stanford, CA, USA.

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http://dx.doi.org/10.1002/ejhf.2074DOI Listing
December 2020

Efficacy of Tafamidis in Patients With Hereditary and Wild-Type Transthyretin Amyloid Cardiomyopathy: Further Analyses From ATTR-ACT.

JACC Heart Fail 2021 Feb 9;9(2):115-123. Epub 2020 Dec 9.

Columbia University College of Physicians and Surgeons, New York, New York, USA.

Objectives: Tafamidis is an effective treatment for transthyretin amyloid cardiomyopathy (ATTR-CM), this study aimed to determine whether there is a differential effect between variant transthyretin amyloidosis (ATTRv) and wild-type transthyretin (ATTRwt).

Background: ATTR-CM is a progressive, fatal disorder resulting from mutations in the ATTRv or the deposition of denatured ATTRwt.

Methods: In pre-specified analyses from ATTR-ACT (Tafamidis in Transthyretin Cardiomyopathy Clinical Trial), baseline characteristics, all-cause mortality, and change from baseline to month 30 in 6-min walk test distance and Kansas City Cardiomyopathy Questionnaire Overall Summary score were compared in patients with ATTRwt and ATTRv.

Results: There were 335 patients with ATTRwt (201 tafamidis, 134 placebo) and 106 with ATTRv (63 tafamidis, 43 placebo) enrolled in ATTR-ACT. Patients with ATTRwt (vs. ATTRv) had less advanced disease at baseline and a lower rate of disease progression over the study. The reduction in all-cause mortality with tafamidis compared with placebo was not different between ATTRwt (hazard ratio: 0.706 [95% confidence interval (CI): 0.474 to 1.052]; p = 0.0875) and ATTRv (hazard ratio: 0.690 [95% CI: 0.408 to 1.167]; p = 0.1667). Tafamidis was associated with a similar reduction (vs. placebo) in the decline in 6-min walk test distance in ATTRwt (mean ± SE difference from placebo, 77.14 ± 10.78; p < 0.0001) and ATTRv (79.61 ± 29.83 m; p = 0.008); and Kansas City Cardiomyopathy Questionnaire Overall Summary score in ATTRwt (12.72 ± 2.10; p < 0.0001) and ATTRv (18.18 ± 7.75; p = 0.019).

Conclusions: Pre-specified analyses from ATTR-ACT confirm the poor prognosis of untreated ATTRv-related cardiomyopathy compared with ATTRwt, but show the reduction in mortality and functional decline with tafamidis treatment is similar in both disease subtypes. (Safety and Efficacy of Tafamidis in Patients With Transthyretin Cardiomyopathy [ATTR-ACT]; NCT01994889).
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http://dx.doi.org/10.1016/j.jchf.2020.09.011DOI Listing
February 2021

Impact of Tafamidis on Health-Related Quality of Life in Patients With Transthyretin Amyloid Cardiomyopathy (from the Tafamidis in Transthyretin Cardiomyopathy Clinical Trial).

Am J Cardiol 2021 02 19;141:98-105. Epub 2020 Nov 19.

Columbia University Vagelos College of Physicians and Surgeons, New York, New York.

In the Tafamidis in Transthyretin Cardiomyopathy Clinical Trial, tafamidis significantly reduced all-cause mortality and cardiovascular-related hospitalizations in patients with transthyretin amyloid cardiomyopathy (ATTR-CM). ATTR-CM is associated with a significant burden of disease; further analysis of patient-reported quality of life will provide additional data on the efficacy of tafamidis. In the Tafamidis in Transthyretin Cardiomyopathy Clinical Trial, 441 adult patients with ATTR-CM were randomized (2:1:2) to tafamidis 80 mg, tafamidis 20 mg, or placebo for 30 months, with pooled tafamidis (80 mg and 20 mg) compared with placebo. Change in Kansas City Cardiomyopathy Questionnaire Overall Summary (KCCQ-OS) domain scores, EQ-5D-3L scores, and patient global assessment, were prespecified exploratory end points. A greater proportion of patients improved KCCQ-OS score at month 30 with tafamidis (41.8%) versus placebo (21.4%). Tafamidis significantly reduced the decline in all 4 KCCQ-OS domains (p <0.0001 for all), and in EQ-5D-3L utility (0.09 [confidence interval 0.05 to 0.12]; p <0.0001) and EQ visual analog scale (9.11 [confidence interval 5.39 to 12.83]; p <0.0001) scores at month 30 versus placebo. A larger proportion of tafamidis-treated patients reported their patient global assessment improved at month 30 (42.3% vs 23.8% with placebo). In conclusion, tafamidis effectively reduced the decline in patient-reported outcomes, providing further insight into its efficacy in health-related quality of life in patients with ATTR-CM.
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http://dx.doi.org/10.1016/j.amjcard.2020.10.066DOI Listing
February 2021

Natural history and impact of treatment with tafamidis on major cardiovascular outcome-free survival time in a cohort of patients with transthyretin amyloidosis.

Eur J Heart Fail 2020 Oct 23. Epub 2020 Oct 23.

AP-HP (Assistance Publique-Hôpitaux de Paris), Cardiology Department, DHU-ATVB, Henri Mondor University Hospital, Créteil, France.

Aims: Hereditary (ATTRv) and wild-type (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 has been used in France to treat patients with ATTRv with neuropathy (alone or combined with cardiomyopathy). Recently, the Phase III ATTR-ACT trial showed that tafamidis decreased the relative risk of mortality in ATTR amyloidosis with cardiomyopathy. 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 ATTR-ACT trial and to assess the impact of tafamidis treatment on major cardiovascular outcome (MCO)-free survival time without cardiac decompensation, heart transplant, or death.

Methods And Results: 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. A total of 467 (72%) patients matched the inclusion criteria of the ATTR-ACT trial. For the 631 patients with cardiomyopathy, tafamidis treatment was associated with a longer median MCO-free survival time (n = 98): 1565 (1010-2400) days vs. 771 (686-895) days without treatment (log-rank P < 0.001). This association was confirmed after considering confounding factors (age at inclusion, N-terminal pro-B-type natriuretic peptide and amyloidosis type) with a propensity score (hazard ratio 0.546; P = 0.0132).

Conclusion: In a large cohort of ATTRwt and ATTRv patients, representative of the inclusion criteria of the ATTR-ACT trial, the present results show an association between tafamidis treatment and a lower occurrence of cardiovascular outcomes in a real-life population.
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http://dx.doi.org/10.1002/ejhf.2028DOI Listing
October 2020

Efficacy and safety of tafamidis doses in the Tafamidis in Transthyretin Cardiomyopathy Clinical Trial (ATTR-ACT) and long-term extension study.

Eur J Heart Fail 2020 Oct 18. Epub 2020 Oct 18.

Stanford University School of Medicine, Stanford, CA, USA.

Aims: Tafamidis is an effective treatment for transthyretin amyloid cardiomyopathy (ATTR-CM) in the Tafamidis in Transthyretin Cardiomyopathy Clinical Trial (ATTR-ACT). While ATTR-ACT was not designed for a dose-specific assessment, further analysis from ATTR-ACT and its long-term extension study (LTE) can guide determination of the optimal dose.

Methods And Results: In ATTR-ACT, patients were randomized (2:1:2) to tafamidis 80 mg, 20 mg, or placebo for 30 months. Patients completing ATTR-ACT could enrol in the LTE (with placebo-treated patients randomized to tafamidis 80 or 20 mg; 2:1) and all patients were subsequently switched to high-dose tafamidis. All-cause mortality was assessed in ATTR-ACT combined with the LTE (median follow-up 51 months). In ATTR-ACT, the combination of all-cause mortality and cardiovascular-related hospitalizations over 30 months was significantly reduced with tafamidis 80 mg (P = 0.0030) and 20 mg (P = 0.0048) vs. placebo. All-cause mortality vs. placebo was reduced with tafamidis 80 mg [Cox hazards model (95% confidence interval (CI): 0.690 (0.487-0.979), P = 0.0378] and 20 mg [0.715 (0.450-1.137), P = 0.1564]. The mean (standard error) change in N-terminal pro-B-type natriuretic peptide from baseline to Month 30 was -1170.51 (587.31) (P = 0.0468) with tafamidis 80 vs. 20 mg. In ATTR-ACT combined with the LTE there was a significantly greater survival benefit with tafamidis 80 vs. 20 mg [0.700 (0.501-0.979), P = 0.0374]. Incidence of adverse events in both tafamidis doses were comparable to placebo.

Conclusion: Tafamidis, both 80 and 20 mg, effectively reduced mortality and cardiovascular-related hospitalizations in patients with ATTR-CM. The longer-term survival data and the lack of dose-related safety concerns support tafamidis 80 mg as the optimal dose.

Clinical Trial Registration: ClinicalTrials.gov NCT01994889; NCT02791230.
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http://dx.doi.org/10.1002/ejhf.2027DOI Listing
October 2020

Prognostic value of cardiopulmonary exercise testing in cardiac amyloidosis.

Eur J Heart Fail 2020 Oct 2. Epub 2020 Oct 2.

Lariboisiere Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France.

Aims: In amyloid patients, cardiac involvement dramatically worsens functional capacity and prognosis. We sought to study how the cardiopulmonary exercise test (CPET) could help in functional assessment and risk stratification of patients with cardiac amyloidosis (CA).

Methods And Results: We carried out a multicentre study including patients with light chain (AL) or transthyretin (TTR) CA. All patients underwent exhaustive examination including CPET and follow-up. The primary prognostic endpoint was the occurrence of death or heart failure hospitalization. Overall, 150 patients were included (91 AL and 59 TTR CA). Median age, systolic blood pressure, N-terminal pro B-type natriuretic peptide (NT-proBNP) and cardiac troponin T were 70 (64-78) years, 121 [interquartile range (IQR) 109-139] mmHg, 2806 (IQR 1218-4638) ng/L and 64 (IQR 33-120) ng/L, respectively. New York Heart Association classes were I-II in 64%. Median peak oxygen consumption (VO ) and circulatory power were low at 13.0 (10.0-16.9) mL/kg/min and 1730 (1318-2614) mmHg/mL/min, respectively. The minute ventilation/carbon dioxide production slope was increased to 37 (IQR 33-45). A total of 77 patients (51%) had chronotropic insufficiency. After a median follow-up of 20 months, there were 37 deaths and 44 heart failure hospitalizations. At multivariate Cox analysis, peak VO  ≤13 mL/kg/min [hazard ratio (HR) 2.7, 95% confidence interval (CI) 1.6-4.8], circulatory power ≤1730 mmHg/mL/min (HR 2.4, 95% CI 1.2-4.6) and NT-proBNP ≥1800 ng/L (HR 2.2, 95% CI 1.1-4.3) were found to be associated with the primary outcome. No events occurred in patients with both peak VO  >13 mL/kg/min and NT-proBNP <1800 ng/L, while the association of VO  ≤13 mL/kg/min with NT-proBNP ≥1800 ng/L identified a very high-risk subgroup.

Conclusion: In CA, CPET is helpful in assessing functional capacity, circulatory and chronotropic responses as well as the prognosis of patients along with cardiac biomarkers.
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http://dx.doi.org/10.1002/ejhf.2016DOI Listing
October 2020

Avoiding misdiagnosis: expert consensus recommendations for the suspicion and diagnosis of transthyretin amyloidosis for the general practitioner.

BMC Fam Pract 2020 09 23;21(1):198. Epub 2020 Sep 23.

Amyloidosis Research and Treatment Center Foundation, IRCCS Policlinico San Matteo, San Matteo, Italy.

Background: Transthyretin amyloidosis (also known as ATTR amyloidosis) is a systemic, life-threatening disease characterized by transthyretin (TTR) fibril deposition in organs and tissue. A definitive diagnosis of ATTR amyloidosis is often a challenge, in large part because of its heterogeneous presentation. Although ATTR amyloidosis was previously considered untreatable, disease-modifying therapies for the treatment of this disease have recently become available. This article aims to raise awareness of the initial symptoms of ATTR amyloidosis among general practitioners to facilitate identification of a patient with suspicious signs and symptoms.

Methods: These consensus recommendations for the suspicion and diagnosis of ATTR amyloidosis were developed through a series of development and review cycles by an international working group comprising key amyloidosis specialists. This working group met to discuss the barriers to early and accurate diagnosis of ATTR amyloidosis and develop a consensus recommendation through a thorough search of the literature performed using PubMed Central.

Results: The cardiac and peripheral nervous systems are most frequently involved in ATTR amyloidosis; however, many patients often also experience gastrointestinal and other systemic manifestations. Given the multisystemic nature of symptoms, ATTR amyloidosis is often misdiagnosed as a more common disorder, leading to significant delays in the initiation of treatment. Although histologic evaluation has been the gold standard to confirm ATTR amyloidosis, a range of tools are available that can facilitate early and accurate diagnosis. Of importance, genetic testing should be considered early in the evaluation of a patient with unexplained peripheral neuropathy.

Conclusions: A diagnostic algorithm based on initial red flag symptoms and manifestations of cardiac or neurologic involvement will facilitate identification by the general practitioner of a patient with clinically suspicious symptoms, enabling subsequent referral of the patient to a multidisciplinary specialized medical center.
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http://dx.doi.org/10.1186/s12875-020-01252-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7513485PMC
September 2020

Epidemiological characteristics and therapeutic management of patients with chronic heart failure who use smartphones: Potential impact of a dedicated smartphone application (report from the OFICSel study).

Arch Cardiovasc Dis 2021 Jan 28;114(1):51-58. Epub 2020 Aug 28.

Cardiology Department, Centre de Référence Amyloses Cardiaques et des Cardiomyopathies, CHU Henri Mondor, AP-HP, 94010 Creteil, France.

Background: The effectiveness of transitional care services for patients discharged from hospital after acute heart failure is challenging, especially in terms of reducing subsequent heart failure hospitalizations. The increased adoption of smartphone applications in society offers a new opportunity to interact with patients to avoid rehospitalization. Thus, electronic health (e-health) can enhance the impact of existing therapeutic education programmes.

Aims: To determine the prevalence of smartphone use among patients with chronic heart failure, and to assess the epidemiological characteristics and therapeutic management of these patients, with a broader aim of developing smartphone-based therapeutic education programmes for patients.

Methods: The French Observatoire français de l'insuffisance cardiaque et du sel (OFICSel) registry was conducted in 2017 by 300 cardiologists, and included both inpatients and outpatients who had been hospitalized for heart failure at least once in the previous 5 years. Data collection included demographic and heart failure-related variables, which were provided by the cardiologist and by the patient via a questionnaire.

Results: Among the 2822 patients included, 2517 completed the questionnaire. Of this total, 907 patients (36%) were smartphone users. Compared with non-users, smartphone users were younger, were more frequently men, more frequently lived in cities, had a higher educational level and were more frequently professionally active. Smartphone users less frequently had diabetes, hypertension, atrial fibrillation or ischaemic cardiopathy. Only 22% of patients were actively participating in a therapeutic education programme.

Conclusion: Smartphones were used by more than one-third of patients with heart failure in France in 2017, underscoring the feasibility of developing a smartphone application to deliver therapeutic education to the population with chronic heart failure.
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http://dx.doi.org/10.1016/j.acvd.2020.05.006DOI Listing
January 2021

ESC EORP Cardiomyopathy Registry: real-life practice of genetic counselling and testing in adult cardiomyopathy patients.

ESC Heart Fail 2020 10 7;7(5):3013-3021. Epub 2020 Aug 7.

Centre de Référence des Maladies Cardiaques Héréditaires, Assistance Publique-Hôpitaux de Paris, ICAN, Inserm UMR1166, Hôpital Pitié-Salpêtrière, Sorbonne Université, Paris, France.

Aims: Cardiomyopathies comprise a heterogeneous group of diseases, often of genetic origin. We assessed the current practice of genetic counselling and testing in the prospective European Society of Cardiology EURObservational Research Programme Cardiomyopathy Registry.

Methods And Results: A total of 3208 adult patients from 69 centres in 18 countries were enrolled. Genetic counselling was performed in 60.8% of all patients [75.4% in hypertrophic cardiomyopathy (HCM), 39.2% in dilated cardiomyopathy (DCM), 70.8% in arrhythmogenic right ventricular cardiomyopathy (ARVC), and 49.2% in restrictive cardiomyopathy (RCM), P < 0.001]. Comparing European geographical areas, genetic counselling was performed from 42.4% to 83.3% (P < 0.001). It was provided by a cardiologist (85.3%), geneticist (15.1%), genetic counsellor (11.3%), or a nurse (7.5%) (P < 0.001). Genetic testing was performed in 37.3% of all patients (48.8% in HCM, 18.6% in DCM, 55.6% in ARVC, and 43.6% in RCM, P < 0.001). Index patients with genetic testing were younger at diagnosis and had more familial disease, family history of sudden cardiac death, or implanted cardioverter defibrillators but less co-morbidities than those not tested (P < 0.001 for each comparison). At least one disease-causing variant was found in 41.7% of index patients with genetic testing (43.3% in HCM, 33.3% in DCM, 51.4% in ARVC, and 42.9% in RCM, P = 0.13).

Conclusions: This is the first detailed report on the real-life practice of genetic counselling and testing in cardiomyopathies in Europe. Genetic counselling and testing were performed in a substantial proportion of patients but less often than recommended by European guidelines and much less in DCM than in HCM and ARVC, despite evidence for genetic background.
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http://dx.doi.org/10.1002/ehf2.12925DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7524128PMC
October 2020

Aortic stenosis and amyloid heart disease: 'the 2A dangerous liaisons'.

Eur Heart J 2020 Aug;41(29):2815

French Referral Center for Cardiac Amyloidosis, Mondor Network, Créteil F-94010, France.

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http://dx.doi.org/10.1093/eurheartj/ehaa549DOI Listing
August 2020

Cohort profile: FACE, prospective follow-up of chronic heart failure patients with sleep-disordered breathing indicated for adaptive servo ventilation.

BMJ Open 2020 07 19;10(7):e038403. Epub 2020 Jul 19.

Department of Physiology and Functional Exploration - Bichat Hospital, Assistance Publique - Hopitaux de Paris, Paris, Île-de-France, France.

Purpose: FACE is a prospective cohort study designed to assess the effect of adding adaptive servoventilation (ASV) to standard care on morbidity and mortality in patients with chronic heart failure (HF) with preserved (HFpEF), mid-range (HFmrEF) or reduced ejection fraction (HFrEF) who have sleep-disordered breathing (SDB) with an indication for ASV. We describe the study design, ongoing data collection and baseline participant characteristics.

Participants: Consecutive patients with HFpEF, HFmrEF or HFrEF plus SDB with central sleep apnoea (CSA) and indication for ASV were enrolled in the study cohort between November 2009 and December 2018; the ASV group includes those treated with ASV and the control group consists of patients who refused ASV or stopped treatment early. Follow-up is based on standard clinical practice, with visits at inclusion, after 3, 12 and 24 months of follow-up. Primary endpoint is the time to first event: all-cause death or unplanned hospitalisation (or unplanned prolongation of a planned hospitalisation) for worsening of HF, cardiovascular death or unplanned hospitalisation for worsening of HF, and all-cause death or all-cause unplanned hospitalisation.

Findings To Date: 503 patients have been enrolled, mean age of 72 years, 88% male, 31% with HFrEF. HF was commonly of ischaemic origin, and the number of comorbidities was high. SDB was severe (median Apnoea-Hypopnoea Index 42/hour), and CSA was the main indication for ASV (69%). HF was highly symptomatic; most patients were in NYHA class II (38%) or III (29%).

Future Plans: Patient follow-up is ongoing. Given the heterogeneous nature of the enrolled population, a decision was made to use latent class analysis to define homogeneous patient subgroups, and then evaluate outcomes by cluster, and in the ASV and control groups (overall and within patient clusters). First analysis will be performed after 3 months, a second analysis at the 2-year follow-up.

Trial Registration Number: NCT01831128; Pre-results.
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http://dx.doi.org/10.1136/bmjopen-2020-038403DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7371028PMC
July 2020

Deleterious effect of right ventricular pacing in patients with cardiac transthyretin amyloidosis: potential clinical benefit of cardiac resynchronization therapy.

Eur Heart J Case Rep 2020 Jun 1;4(3):1-5. Epub 2020 May 1.

Cardiology Department, GCS-Groupement des Hôpitaux de l'Institut Catholique Lillois/Faculté de médecine et de maïeutique, UCLille, F-59000 Lille, France.

Background: Cardiac amyloidosis involvement is associated with a detrimental outcome including frequent arrhythmias, heart failure, and conduction disturbances which may need permanent pacing.

Cases Summary: We report two cases of patients with transthyretin amyloidosis (ATTR) who developed heart failure and depressed left ventricular ejection fraction (LVEF) following permanent right ventricular (RV) pacing but highly responded to cardiac resynchronization therapy (CRT).

Discussion: The impact of RV pacing and CRT in cardiac amyloidosis is not known. In our cases, the detrimental effect of permanent RV pacing on left ventricular (LV) systolic function and heart failure symptoms was suggested by both permanent RV pacing mediated functional and LV function decline and LV systolic dysfunction reversal following CRT along with QRS width reduction. Whether cardiac resynchronization should be readily recommended in ATTR patients who need ventricular pacing whatever the LVEF deserves further investigation.
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http://dx.doi.org/10.1093/ehjcr/ytaa088DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7319833PMC
June 2020

DISCOVERY: prevalence of transthyretin () mutations in a US-centric patient population suspected of having cardiac amyloidosis.

Amyloid 2020 Dec 26;27(4):223-230. Epub 2020 May 26.

Cardiovascular Research Lab for the Elderly at New York-Presbyterian/Columbia Allen Hospital, Columbia University Medical Center, New York, NY, USA.

Background: Hereditary transthyretin-mediated amyloidosis (hATTR amyloidosis) is a multisystem disease that presents with polyneuropathy and/or cardiomyopathy.

Methods: DISCOVERY, a multicenter screening study, enrolled patients with clinically suspected cardiac amyloidosis to determine the frequency of transthyretin () mutations and assess disease characteristics.

Results: Of 1007 patients, the majority were from the US (84%), Black/African American (56%), male (63%), and with a mean (standard deviation) age of 65 (13) years. Among 1001 patients with genotyping results, 74 (7%) had a pathogenic mutation (71/836 [8%] from the US). Val122Ile was the most common mutation, found in 11% of Black/African American patients overall; Black/African American ethnicity was an independent predictor of having a pathogenic mutation. Additional independent predictors of such mutations in the total population and Black/African American group were interventricular septum thickness, low electrocardiogram voltage, and age.

Conclusions: Pathogenic mutations occurred in 8% of US patients with suspected cardiac amyloidosis. Most mutations were Val122Ile, almost exclusively found in Black/African American patients. Disease often remains undetected until advanced and difficult to treat, therefore, clinicians should assess at-risk patients for hATTR amyloidosis as early as possible.
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http://dx.doi.org/10.1080/13506129.2020.1764928DOI Listing
December 2020

Extracardiac soft tissue uptake, evidenced on early Tc-HMDP SPECT/CT, helps typing cardiac amyloidosis and demonstrates high prognostic value.

Eur J Nucl Med Mol Imaging 2020 09 10;47(10):2396-2406. Epub 2020 Mar 10.

French Referral Center for Cardiac Amyloidosis, Mondor Network, F-94010, Créteil, France.

Purpose: Increased cardiac uptake (CU) on early-phase Tc-HMDP scintigraphy has demonstrated diagnostic and prognostic values in amyloid transthyretin (ATTR) cardiac amyloidosis (CA). Extracardiac uptake (ECU) has been poorly studied. We assessed the clinical value of ECU, in combination with CU, on Tc-HMDP scintigraphy using a novel Methodological Amyloidosis Diagnostic Index (MADI).

Methods: We reviewed all patients referred for suspicion of CA, who underwent Tc-HMDP scintigraphy over an 8-year period. ECU, CU, and MADI were determined: MADI0 = neither ECU or CU, MADI1 = ECU alone, MADI2 = CU alone, and MADI3 = ECU + CU.

Results: Of 308 eligible patients, 247 had CA, including 75 ATTRv, 107 ATTRwt, and 65 light-chain (AL), while 61 had another cardiopathy (controls). ECU was observed in 29% of CA and 3% of controls. Most frequent sites of ECU were pleuropulmonary (16% of CA, 3% of controls) followed by the digestive tract and subcutaneous tissues. The liver and spleen ECU was only observed in AL-CA (n = 8). CU was only observed in CA patients (n = 187), of whom 182 had ATTR-CA vs. 5 AL-CA, P < 0.001. MADI0 was only observed in controls (97%) and in AL-CA (60%). MADI1 was mainly observed in AL-CA (positive predictive value, PPV = 91%) while MADI2/3 were more frequent in ATTR-CA (PPV = 97%), P < 0.0001. MADI > 0 vs. MADI0 in AL and MADI3 vs. MADI2 in ATTR were associated with a worse prognosis (P = 0.03 and P = 0.002, respectively).

Conclusions: ECU combined with CU demonstrates high diagnostic and prognostic values in CA patients. MADI seems an easy and reliable score in clinical practice.
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http://dx.doi.org/10.1007/s00259-020-04753-7DOI Listing
September 2020

Management of heart failure with reduced ejection fraction in Europe: design of the ARIADNE registry.

ESC Heart Fail 2020 04 6;7(2):727-736. Epub 2020 Feb 6.

Associazione Nazionale Medici Cardiologi Ospedalieri Research Center, Florence, Italy.

Aims: The introduction of sacubitril/valsartan (an angiotensin receptor-neprilysin inhibitor) is likely to change the approach to the management of patients with chronic heart failure with reduced ejection fraction (HFrEF). The Assessment of Real Life Care-Describing European Heart Failure Management (ARIADNE) registry will evaluate patient characteristics, practice patterns, outcomes, and healthcare resource utilization in the outpatient setting across Europe, with the main focus on factors that guide physicians' decisions to start and continue sacubitril/valsartan in patients with HFrEF.

Methods And Results: ARIADNE, a prospective, observational registry will enrol 9000 ambulatory patients with HFrEF in 23 European countries Supplement 1. The study will describe 4500 patients treated with conventional treatment (including an angiotensin-converting enzyme inhibitor/angiotensin receptor blocker), and 4500 patients started on sacubitril/valsartan. In each country, patients will be enrolled consecutively over an expected period of 12 months, and followed-up for 12 months. The primary objective is to describe the baseline clinical and demographic characteristics of patients with chronic HFrEF, which guide the decision of the treating physician to initiate sacubitril/valsartan or to continue conventional treatment. A co-primary objective is to identify the baseline characteristics that are associated with the likelihood of reaching the target dose of sacubitril/valsartan 97/103 mg twice daily during follow-up.

Conclusions: The ARIADNE registry will provide a comprehensive profile of patients with chronic HFrEF in Europe, will elucidate how management varies between countries, and will help clarify the usage and outcomes associated with use of sacubitril/valsartan in real life.
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http://dx.doi.org/10.1002/ehf2.12569DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7160498PMC
April 2020

Echocardiographic Evaluation of Left Ventricular Filling Pressure in Patients With Heart Failure With Preserved Ejection Fraction: Usefulness of Inferior Vena Cava Measurements and 2016 EACVI/ASE Recommendations.

J Card Fail 2020 Jun 30;26(6):507-514. Epub 2020 Jan 30.

University Paris-Saclay, Le Kremlin-Bicêtre, France; AP-HP, Service de Cardiologie, Hôpital Bicêtre, Le Kremlin-Bicêtre, France.

Context: The left ventricular filling pressure (LVFP) is correlated to right atrial pressure (RAP) in heart failure. We compared diagnostic value of the inferior vena cava (IVC) measurements to the one of the 2016 echocardiographic recommendations to estimate LVFP in patients with suspected heart failure with preserved ejection fraction (HFpEF).

Methods: Invasive hemodynamics and echocardiography were obtained within 48 hours in 132 consecutive patients with left ventricular ejection fraction ≥50%, and suspected pulmonary hypertension. Increased LVFP was defined by a pulmonary artery wedge pressure (PAWP) >15 mmHg.

Results: Of 83 patients in sinus rhythm, a score of the 2016 recommendations ≥ 2 (E/e' ratio >14 and/or tricuspid regurgitation velocity >2.8 m/s and/or indexed left atrial volume>34 mL /m²) had a positive predictive value (PPV) of 63% for PAWP>15 mmHg, whereas a dilated IVC (>2.1 cm) and/or non-collapsible (≤50%) had a PPV of 82%. The net reclassification improvement was 0.39 (P < .05). In atrial fibrillation (AF), a dilated and/or non-collapsible IVC had an 86% PPV for PAWP>15 mmHg. The correlation between RAP and PAWP was 0.60, with 75.7% concordance (100/132) between dichotomized pressures (both RAP>8 mmHg and PAWP>15 mmHg and vice versa).

Conclusion: The IVC size and collapsibility is valuable to identify patients with HFpEF with high LVFP in both sinus rhythm and AF.
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http://dx.doi.org/10.1016/j.cardfail.2020.01.018DOI Listing
June 2020

Aortic Stenosis and Cardiac Amyloidosis: JACC Review Topic of the Week.

J Am Coll Cardiol 2019 11;74(21):2638-2651

Referral Center for Cardiac Amyloidosis, Mondor Amyloidosis Network, GRC Amyloid Research Institute and Cardiology Department, APHP Henri Mondor Hospital, Créteil, France; INSERM Unit U955, Team 8, Paris-Est Creteil University, Val-de-Marne, Créteil, France.

The prevalence of calcific aortic stenosis (AS) and of cardiac amyloidosis (CA) increases with age, and their association is not uncommon in the elderly. The identification of CA is particularly challenging in patients with AS because these 2 conditions share several features. It is estimated that ≤15% of the AS population and ≤30% of the subset with low-flow, low-gradient pattern may have CA. In patients with AS, CA is associated with increased risk of heart failure, mortality, and treatment futility with aortic valve replacement. In case of suspicion of CA, it is thus crucial to confirm the diagnosis to guide therapeutic management of AS and eventually implement recently developed pharmacological treatment dedicated to transthyretin amyloidosis. Given the high surgical risk of patients with AS and concomitant CA, transcatheter aortic valve replacement may be preferred to surgery in these patients.
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http://dx.doi.org/10.1016/j.jacc.2019.09.056DOI Listing
November 2019

Physical activity for patients with heart failure: Position paper from the heart failure (GICC) and cardiac rehabilitation (GERS-P) Working Groups of the French Society of Cardiology.

Arch Cardiovasc Dis 2019 Nov 18;112(11):723-731. Epub 2019 Sep 18.

UMR-S 942, service de cardiologie, hôpital Lariboisière, université de Paris, AP-HP, 75010 Paris, France.

Physical activity is important in heart failure to improve functional capacity, quality of life and prognosis, and is a class IA recommendation in the European Society of Cardiology guidelines (Ponikowski et al., 2016). The benefits of exercise training are widely recognized. Cardiac rehabilitation centres offer tailored exercise training to patients with heart failure, as part of specialized multidisciplinary care, alongside pharmacological treatment optimization and patient education. After cardiac rehabilitation, maintenance of regular physical activity long term is essential, as the benefits of exercise training vanish within a few weeks. Unfortunately, only 10% of patients benefit from a cardiac rehabilitation programme after hospitalization for acute heart failure, and the majority of patients do not pursue long-term physical activity. In this paper, two Working Groups of the French Society of Cardiology (the heart failure group [Groupe Insuffisance Cardiaque et Cardiomyopathies; GICC] and the cardiac rehabilitation group [Groupe Exercice Réadaptation Sport et Prévention; GERS-P]) discuss the obstacles to broader access to cardiac rehabilitation centres, and propose ways to improve the diffusion of cardiac rehabilitation programmes and encourage long-term adherence to physical activity.
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http://dx.doi.org/10.1016/j.acvd.2019.07.003DOI Listing
November 2019

F-sodium fluoride PET/MRI myocardial imaging in patients with suspected cardiac amyloidosis.

J Nucl Cardiol 2019 Sep 11. Epub 2019 Sep 11.

SyMPTOm PET/MRI platform, Departments of Nuclear Medicine and Radiology, CHU Henri Mondor Hospital, AP-HP/U-PEC, 51 Ave. du Mal de Lattre de Tassigny, 94010, Créteil, France.

Background: We evaluated the diagnostic performance of F-NaF PET/MRI in patients with suspected cardiac amyloidosis (CA).

Methods: Twenty-seven consecutive patients underwent myocardial PET 1 hour after injection of 4 MBq/kg F-NaF with simultaneous MRI including cine-MRI, T1 and T2 mapping, first-pass and late gadolinium enhancement (LGE). F-NaF uptake was measured visually and semi-quantitatively by calculating myocardium-to-blood pool (M/B) ratios. CA was confirmed histologically.

Results: Transthyretin (TTR)-CA was diagnosed in 16 patients, light-chain (AL)-CA in 7, and no-CA in 4. Visual interpretation of F-NaF images revealed a relative increase in myocardial uptake in only 3 patients, all with TTR CA, and a relative decrease in 13, including 7 AL CA, 3 no-CA, and 3 TTR CA. M/B ratios were significantly higher in TTR CA (1.00 ± 0.12) than in AL CA (0.81 ± 0.06, P = 0.001) or in no-CA (0.73 ± 0.16, P = 0.006). The optimal M/B cut-off to distinguish TTR CA from AL CA was ≥ 0.90 (Fischer, P = 0.0005). By comparison, classification of patients using Tc-HMDP heart-to-mediastinum ratios with the previously published cut-off ≥ 1.21 reached higher significance (P < 0.0001). Among MRI parameters, myocardial T1, LGE score, and extracellular volume were higher in CA than in no-CA patients, 1409 ± 76 vs 1278 ± 35 ms (P = 0.004), 10.35 ± 5.30 vs 3.50 ± 3.42 (P = 0.03), and 46 ± 10 vs 33 ± 8 % (P = 0.01), respectively.

Conclusion: F-NaF PET/MRI shows good diagnostic performance when semi-quantification is used. However, contrast is low and visual interpretation may be challenging in routine. PET/MRI could constitute a one-stop-shop evaluation of amyloid load and cardiac function in patients needing rapid work-up.
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http://dx.doi.org/10.1007/s12350-019-01885-8DOI Listing
September 2019

Association between hearing loss and hereditary ATTR amyloidosis.

Amyloid 2019 Dec 10;26(4):234-242. Epub 2019 Sep 10.

Department of Oto-Rhino-Laryngo Surgery, Centre Hospitalier Intercommunal de Créteil , Créteil , France.

Hereditary transthyretin (TTR) related amyloidosis (ATTRv) is a life-threatening condition, which can potentially affect all organs. The objective was to identify the hearing status of patients with cardiac ATTRv and describe their audiological pattern. Nineteen patients with confirmed diagnosis of ATTRv cardiac amyloidosis (CA) underwent otoscopy and audiological tests, including pure tone and speech audiometry. 74% were male, with a mean age of 72 ± 1.8 years. The main mutations were Val122Ile ( 7) and Val30Met ( 6). Objective hearing loss was detected in 17 patients (89%), whereas only 37% complained of hearing loss. ATTRv patients presented a different audiometric profile compared to patients of the same age with presbycusis: a higher prevalence and worse hearing thresholds compared to age-related expectations (ISO). Hearing loss affected all frequencies with, unexpectedly, mixed or conductive hearing loss (35%). According to the type of mutation, there was an increased rate of sensorineural or mixed/conductive hearing loss. the present study indicates that hearing loss is more prevalent and worse in patients with ATTRv amyloidosis than in the general population, while mostly clinically under-estimated. It suggests that ATTRv deposits could infiltrate the various anatomical structures of the inner and mild ear.
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http://dx.doi.org/10.1080/13506129.2019.1663814DOI Listing
December 2019

Expert Consensus Recommendations for the Suspicion and Diagnosis of Transthyretin Cardiac Amyloidosis.

Circ Heart Fail 2019 09 4;12(9):e006075. Epub 2019 Sep 4.

Amyloidosis Center Foundation IRCCS Policlinico San Matteo, Italy (G.M.).

Cardiomyopathy is a manifestation of transthyretin amyloidosis (ATTR), which is an underrecognized systemic disease whereby the transthyretin protein misfolds to form fibrils that deposit in various tissues and organs. ATTR amyloidosis is debilitating and associated with poor life expectancy, especially in those with cardiac dysfunction, but a variety of treatment options have recently become available. Considered a rare disease, ATTR amyloidosis may be more prevalent than thought, particularly in older persons. Diagnosis is often delayed because of a lack of disease awareness and the heterogeneity of symptoms at presentation. Given the recent availability of effective treatments, early recognition and diagnosis are especially critical because treatment is likely more effective earlier in the disease course. The Amyloidosis Research Consortium recently convened a group of experts in ATTR amyloidosis who, through an iterative process, agreed on best practices for suspicion, diagnosis, and characterization of disease. This review describes these consensus recommendations for ATTR associated with cardiomyopathy as a resource to aid cardiologists and others in the recognition and diagnosis of ATTR associated with cardiomyopathy. Included in this review is an overview of red flag signs and symptoms and a recommended diagnostic approach, including testing for monoclonal protein, scintigraphy, or biopsy and, if ATTR associated with cardiomyopathy is identified, TTR genotyping.
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http://dx.doi.org/10.1161/CIRCHEARTFAILURE.119.006075DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6736650PMC
September 2019

Pharyngo-laryngeal involvement in systemic amyloidosis with cardiac involvement: a prospective observational study.

Amyloid 2019 Dec 31;26(4):216-224. Epub 2019 Jul 31.

Department of Oto-rhino-laryngo Surgery, Centre Hospitalier Intercommunal de Créteil , Créteil , France.

Systemic amyloidosis with cardiac involvement (CA) is a severe disease caused by the aggregation of misfolded proteins infiltrating organs and tissues and leading to their dysfunction. No study has yet focused on potential pharyngo-laryngeal impairments associated to CA. Our objective was to define its prevalence and describe pharyngo-laryngeal involvement patterns in a population with CA (light chain: AL, wild-type transthyretin: ATTRwt, variant transthyretin: ATTRv). Consecutive patients with a confirmed diagnosis of CA were prospectively investigated for pharyngo-laryngeal involvement. This included questionnaires on symptoms of dysphonia/dysphagia and quality of life Voice Handicap Index (VHI). In cases of dysphonia, a nasofibroscopy was performed to evaluate potential laryngeal organic lesions of amyloid infiltration and induced laryngeal dysfunction (mobility, glottic air leak). In cases of dysphagia, Video Endoscopy Swallowing Study (VESS) was performed to evaluate the presence of hypopharyngeal pooling at rest and during swallowing and the time of swallowing 80 ml of water. Ninety-five CA patients were enrolled, of whom 19 were ATTRv, 36 AL and 40 ATTRwt. Their mean age was 73.8 ± 9.2 years and the sex ratio was 2.6 in favor of men. Dysphagia was reported in 17% of the patients and 40% had more specific oropharyngeal symptoms (food sticking, regurgitation, change in dietary habits), preceding the CA diagnosis by 7 (0-24) months. Recent weight loss was reported in 60% of the patients (mean loss of 10 ± 6.3 kg). VESS showed functional swallowing impairment in only 4 patients without any macroscopic organic lesion. Dysphonia was reported in 36% of the patients (44% and 47% in AL and ATTRv sub-groups, respectively) of whom 40% had functional or organic laryngeal abnormality (14% of vocal fold mobility dysfunction and 26% of abnormal mucosa) without any macroscopic-specific lesions of amyloid infiltration in these patients. This prospective study suggests, for the first time, that amyloid associated with CA could infiltrate the various anatomical structures of the pharyngo-larynx, responsible for functional impairment and potential nutritional depletion and poor quality of life.
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http://dx.doi.org/10.1080/13506129.2019.1646639DOI Listing
December 2019

Screening for Transthyretin Amyloid Cardiomyopathy in Everyday Practice.

JACC Heart Fail 2019 08 10;7(8):709-716. Epub 2019 Jul 10.

Heart Failure and Inherited Cardiac Diseases Unit, Department of Cardiology, Hospital Universitario Puerta de Hierro Majadahonda, CIBERCV, Madrid, Spain; University Francisco de Vitoria (UFV), Pozuelo de Alarcon, Madrid, Spain. Electronic address:

Transthyretin amyloid cardiomyopathy (ATTR-CM) is a life-threatening, progressive, infiltrative disease caused by the deposition of transthyretin amyloid fibrils in the heart, and can often be overlooked as a common cause of heart failure. Delayed diagnosis due to lack of disease awareness and misdiagnosis results in a poorer prognosis. Early accurate diagnosis is therefore key to improving patient outcomes, particularly in the context of both the recent approval of tafamidis in some countries (including the United States) for the treatment of ATTR-CM, and of other promising therapies under development. With the availability of scintigraphy as an inexpensive, noninvasive diagnostic tool, the rationale to screen for ATTR-CM in high-risk populations of patients is increasingly warranted. Here the authors propose a framework of clinical scenarios in which screening for ATTR-CM is recommended, as well as diagnostic "red flags" that can assist in its diagnosis among the wider population of patients with heart failure.
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http://dx.doi.org/10.1016/j.jchf.2019.04.010DOI Listing
August 2019

Extracorporeal membrane oxygenation support in acute circulatory failure: A plea for regulation and better organization.

Arch Cardiovasc Dis 2019 Jun - Jul;112(6-7):441-449. Epub 2019 Jun 25.

Department of Thoracic and Cardiovascular Surgery, Cardiology Institute, CHU Pitié Salpétrière, AP-HP, 75013 Paris, France.

Emergent implantation of temporary mechanical circulatory support using venoarterial ECMO (ECLS for extracorporeal Life Support) is increasingly adopted in various indications of acute circulatory failure refractory to optimal medical treatment. To implant such devices, but also to provide appropriate daily management, expertise and adapted technical platform are required. Organization, coordination and regulation of such program are not clearly established in our country. We propose a dedicated territorial organization to improve and facilitate management of these specific and most severe patients.
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http://dx.doi.org/10.1016/j.acvd.2019.04.008DOI Listing
December 2019

Causes and consequences of cardiac fibrosis in patients referred for surgical aortic valve replacement.

ESC Heart Fail 2019 08 21;6(4):649-657. Epub 2019 May 21.

UPEC, AP-HP, Henri-Mondor Teaching Hospital, Créteil, France.

Aims: Cardiac fibrosis is associated with left ventricular (LV) remodelling and contractile dysfunction in aortic stenosis (AS). The fibrotic process in this condition is still unclear. The aim of this study was to determine the role of both local and systemic inflammation as underlying mechanisms of LV fibrosis and contractile dysfunction. The diagnostic values of 2D-strain echocardiography and serum biomarkers in the evaluation of cardiac fibrosis in this condition were assessed through correlation analyses.

Methods And Results: Patients with AS referred for surgical valve replacement were prospectively and consecutively included. They all had a comprehensive echocardiography including 2D strain. Blood samples were collected to measure cytokines and inflammatory biomarkers using Luminex bead-based assays. A per-surgical myocardial biopsy of the basal antero-septal segment (S1) was performed. Serial sections of each biopsy were stained with Sirius red. Digital image analysis was used to quantify fibrosis. Immunostainings using specific antibodies against macrophage, glycoprotein (gp) 130, and interleukin 6 (IL-6) were also performed. Patients were divided into tertiles reflecting the severity of fibrosis: mild, moderate, and severe load (TF1 to TF3). The mean age of the 58 included patients was 73 ± 11 years. Twenty-four (43%) were in New York Heart Association III-IV. Mean aortic valve area was 0.8 ± 0.2 cm . Mean aortic stenosis peak velocity and mean gradient were respectively 4.5 ± 0.8 m/s and 54 ± 15 mmHg. The mean LV ejection fraction was 54 ± 12%, and the global LV longitudinal strain was -15 ± 4%. The mean S1 strain, corresponding to the biopsied region, was -10 ± 6% and was strongly correlated to fibrosis load (R = 0.83, P < 0.0001). TF3 was associated with higher mortality (P = 0.009), higher serum C-reactive protein and IL-6, and lower gp130 compared with the other tertiles (P < 0.05). IL-6 and gp130 were expressed in the heart and respectively in the plasma membrane of macrophages and in the cytoplasm of both macrophages and cardiomyocytes. During follow-up, three patients died and were all in the third fibrosis tertile.

Conclusions: We found a positive correlation between elevated inflammatory markers and degree of fibrosis load. These two parameters were associated with worse outcomes in patients with severe AS. Our results may be of interest especially in patients for whom a transcatheter aortic valve implantation is indicated and myocardial biopsy is not possible. Strategies aiming at preventing inflammation might be considered to decrease or limit the progression of cardiac fibrosis in patients followed for AS.
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http://dx.doi.org/10.1002/ehf2.12451DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6676299PMC
August 2019