Transthyretin amyloid polyneuropathy in France: A cross-sectional study with 413 patients and real-world tafamidis meglumine use (2009-2019).

OBJECTIVE: We aimed to describe characteristics of patients with ATTR variant polyneuropathy (ATTRv-PN) and ATTRv-mixed and assess the real-world use and safety profile of tafamidis meglumine 20mg. METHODS: Thirty-eight French hospitals were invited. Patient files were reviewed to identify clinical manifestations, diagnostic methods, and treatment compliance. RESULTS: Four hundred and thirteen patients (296 ATTRv-PN, 117 ATTRv-mixed) were analyzed. Patients were predominantly male (68.0%) with a mean age of 57.2±17.2 years. Interval between first symptom(s) and diagnosis was 3.4±4.3 years. First symptoms included sensory complaints (85.9%), dysautonomia (38.5%), motor deficits (26.4%), carpal tunnel syndrome (31.5%), shortness of breath (13.3%), and unexplained weight loss (16.0%). Mini-invasive accessory salivary gland or punch skin and nerve biopsies were most common, with a performance of 78.8-100%. TTR genetic sequencing, performed in all patients, revealed 31 TTR variants. Tafamidis meglumine was initiated in 156/214 (72.9%) ATTRv-PN patients at an early disease stage. Median treatment duration was 6.00 years in ATTRv-PN and 3.42 years in ATTRv-mixed patients. Tafamidis was well tolerated, with 20 adverse events likely related to study drug among the 336 patients. CONCLUSION: In France, ATTRv patients are usually identified early thanks to the national network and the help of diagnosis combining genetic testing and mini-invasive biopsies.

Confidence vanished or impaired until distrust in the doctor-patient relationship because of COVID-19: Confidence vanished or impaired until distrust: "COVID" in relationship.

Since Hippocrates, the cornerstone of medical practice has been the doctor-patient relationship. The question here is whether these basic principles are still compatible with this unusual COVID-period. This pandemic represents a serious threat to human health, leading to profound changes in behavior in daily life but also in health care. Because of limited resources, health-managers must choose well-balanced solutions able to protect patients and citizens on the one hand and to provide maximal benefit for the society on the other hand. We are going through a moment of rupture that we must acknowledge. Here, we discussed how the doctor-patient relationship could be compromised. Doctors are focused on cares whereas patients are focused on scare. Profound changes occur presently, from the way we present ourselves to each other (including the masks), the poor conditions for physical examination, the mental suffering of both patient and caregiver until sometimes terrible end-of-life conditions. The historical point-of-view helps us to keep in mind previous experiences, and the philosophical perspective helps to contextualize this unedited situation. We should stop briefly our daily rush to put these considerations into perspective to overcome these challenges. Nothing is as effective as trust: let's rebuild it.

Effects of an individualized exercise training program on severity markers of obstructive sleep apnea syndrome: a randomised controlled trial.

OBJECTIVE: Obstructive sleep apnea (OSA) is a high prevalent disorder with severe consequences including sleepiness, metabolic, and cardiovascular disorders. The aim of this study was to assess the effect of an individualized exercise-training (IET) program with educational sessions vs educational sessions alone on severity markers of OSA over an eight-week duration. METHODS: This was a randomised, controlled, parallel-design study. In sum, 64 patients with moderate-to-severe OSA (apnea-hypopnea index AHI 15-45/hour), low physical activity level (Voorrips<9), body-mass index (BMI) <40 kg/m2 were included in intervention group (IG) or control group (CG), and 54 patients finished the study. All underwent polysomnography (PSG), multiple sleep latency test (MSLT), constant workload exercise test, blood samples and fulfilled questionnaires twice. The primary endpoint was the change in apnea-hypopnea (AHI) at eight weeks from baseline. Main secondary endpoints were daytime sleepiness assessed by questionnaire and objective tests. RESULTS: No significant between-group differences were found for changes in AHI. A reduction in AHI was found in IG only (p = 0.005). Compared to CG, exercise training leads to a greater decrease in AHI during REM sleep (p = 0.0004), with a significant increase in mean daytime sleep latency (p = 0.02). Between-group differences were significant for weight reduction, severity of fatigue, insomnia and depressive symptoms with trend for sleepiness symptoms. CONCLUSIONS: In adult patients with moderate-to-severe OSA, IET did not decrease AHI compared to the control group but improved markers of severity of OSA, in particular AHI in rapid eye movement (REM) sleep and objective daytime sleepiness. Adding personalized exercise training to the management of patients with OSA should be considered. CLINICALTRIALS. GOV IDENTIFIER: NCT01256307.

Organization of intensive cardiac care units in Europe: Results of a multinational survey.

BACKGROUND: The present survey aims to describe the intensive cardiac care unit organization and admission policies in Europe. METHODS: A total of 228 hospitals (61% academic) from 27 countries participated in this survey. In addition to the organizational aspects of the intensive cardiac care units, including classification of the intensive cardiac care unit levels, data on the admission diagnoses were gathered from consecutive patients who were admitted during a two-day period. Admission policies were evaluated by comparing illness severity with the intensive cardiac care unit level. Gross national income was used to differentiate high-income countries (n=13) from middle-income countries (n=14). RESULTS: A total of 98% of the hospitals had an intensive cardiac care unit: 70% had a level 1 intensive cardiac care unit, 76% had a level 2 intensive cardiac care unit, 51% had a level 3 intensive cardiac care unit, and 60% of the hospitals had more than one intensive cardiac care unit level. High-income countries tended to have more level 3 intensive cardiac care units than middle-income countries (55% versus 41%, p=0.07). A total of 5159 admissions were scored on illness severity: 63% were low severity, 24% were intermediate severity, and 12% were high severity. Patients with low illness severity were predominantly admitted to level 1 intensive cardiac care units, whereas patients with high illness severity were predominantly admitted to level 2 and 3 intensive cardiac care units. A policy mismatch was observed in 12% of the patients; some patients with high illness severity were admitted to level 1 intensive cardiac care units, which occurred more often in middle-income countries, whereas some patients with low illness severity were admitted to level 3 intensive cardiac care units, which occurred more frequently in high-income countries. CONCLUSION: More than one-third of the admitted patients were considered intermediate or high risk. Although patients with higher illness severity were mostly admitted to high-level intensive cardiac care units, an admission policy mismatch was observed in 12% of the patients; this mismatch was partly related to insufficient logistic intensive cardiac care unit capacity.

[A (too) successful renal denervation]. / Une dénervation rénale (trop) réussie.

We report the case of a type-2 myocardial infarction immediately after renal denervation. The patient was followed for coronary artery disease. Low blood pressures were responsible for inferior acute myocardial infarction that revealed a sub occlusive stenosis of the right coronary artery.

Prevalence of obstructive sleep apnoea in acute coronary syndrome: Routine screening in intensive coronary care units.

INTRODUCTION: Increased evidence has shown that, despite the maximum care afforded to patients admitted with acute coronary syndromes (ACS), a residual risk of mortality remains, in which obstructive sleep apnoea (OSA) appears to be a largely undiagnosed factor, particularly in the intensive cardiac care unit (ICCU). The purpose of this study is to determine whether the systematic screening for sleep-disordered breathing (SDB) is feasible and may be recommended. The aims of our study are to determine: (1) The estimated prevalence of OSA in patients admitted to the ICCU for ACS determined by a validated, user-friendly portable screening device; (2) The feasibility of the screening in this context; (3) To assess any negative impact of OSA on the severity of ACS. PATIENTS AND METHODS: This is an observational study of 101 patients admitted to the ICCU for ACS showing no clinical evidence of heart failure (HF). In the 24-72hours following admission, they underwent an overnight sleep study using a 3-channel portable screening device with automatic analysis. RESULTS: Sixty-two out of the 101 patients proved positive to the screening test, and its feasibility was acceptable. OSA patients tended to have greater peak levels of hs-cTnT (3685±3576ng/L versus 2830±3333ng/L, P=0.08) than the non-OSA group. Compared with the non-OSA group, OSA patients presented more severe ACS, with a greater average GRACE score at admission of 112.2±26.3 (versus 98.4±19.2, P<0.001). In the OSA group, we found a statistically significant inverse correlation between the apnoea-hypopnea index (AHI) and the left ventricular ejection fraction (LVEF) in the linear regression analysis (r=-0.26; P=0.037). CONCLUSIONS: A systematic screening of patients in the ICCU is acceptable. OSA is frequently found in the acute phase of ischaemic heart disease and its presence is associated with more severe ACS and a poorer left ventricle systolic function.

MACVIA-LR (Fighting Chronic Diseases for Active and Healthy Ageing in Languedoc-Roussillon): A Success Story of the European Innovation Partnership on Active and Healthy Ageing.

The Région Languedoc Roussillon is the umbrella organisation for an interconnected and integrated project on active and healthy ageing (AHA). It covers the 3 pillars of the European Innovation Partnership on Active and Healthy Ageing (EIP on AHA): (A) Prevention and health promotion, (B) Care and cure, (C) and (D) Active and independent living of elderly people. All sub-activities (poly-pharmacy, falls prevention initiative, prevention of frailty, chronic respiratory diseases, chronic diseases with multimorbidities, chronic infectious diseases, active and independent living and disability) have been included in MACVIA-LR which has a strong political commitment and involves all stakeholders (public, private, patients, policy makers) including CARSAT-LR and the Eurobiomed cluster. It is a Reference Site of the EIP on AHA. The framework of MACVIA-LR has the vision that the prevention and management of chronic diseases is essential for the promotion of AHA and for the reduction of handicap. The main objectives of MACVIA-LR are: (i) to develop innovative solutions for a network of Living labs in order to reduce avoidable hospitalisations and loss of autonomy while improving quality of life, (ii) to disseminate the innovation. The three years of MACVIA-LR activities are reported in this paper.

No post-conditioning in the human heart with thrombolysis in myocardial infarction flow 2-3 on admission.

AIMS: Proof-of-concept evidence suggests that mechanical ischaemic post-conditioning (PostC) reduces infarct size when applied immediately after culprit coronary artery re-opening in ST-elevation myocardial infarction (STEMI) patients with thrombolysis in myocardial infarction 0-1 (TIMI 0-1) flow grade at admission. Whether PostC might also be protective in patients with a TIMI 2-3 flow grade on admission (corresponding to a delayed application of the post-conditioning algorithm) remains undetermined. METHODS AND RESULTS: In this multi-centre, randomized, single-blinded, controlled study, STEMI patients with a 2-3 TIMI coronary flow grade at admission underwent direct stenting of the culprit lesion, followed (PostC group) or not (control group) by four cycles of (1 min inflation/1 min deflation) of the angioplasty balloon to trigger post-conditioning. Infarct size was assessed both by cardiac magnetic resonance at Day 5 (primary endpoint) and cardiac enzymes release (secondary endpoint). Ninety-nine patients were prospectively enrolled. Baseline characteristics were comparable between control and PostC groups. Despite comparable size of area at risk (AAR) (38 ± 12 vs. 38 ± 13% of the LV circumference, respectively, P = 0.89) and similar time from onset to intervention (249 ± 148 vs. 263 ± 209 min, respectively, P = 0.93) in the two groups, PostC did not significantly reduce cardiac magnetic resonance infarct size (23 ± 17 and 21 ± 18 g in the treated vs. control group, respectively, P = 0.64). Similar results were found when using creatine kinase and troponin I release, even after adjustment for the size of the AAR. CONCLUSION: This study shows that infarct size reduction by mechanical ischaemic PostC is lost when applied to patients with a TIMI 2-3 flow grade at admission. This indicates that the timing of the protective intervention with respect to the onset of reperfusion is a key factor for preventing lethal reperfusion injury in STEMI patients. CLINICAL TRIAL NUMBER: NCT01483755.

Cardiac tamponade likely due to candida infection, in an immunocompetent patient.

Candida pericarditis is a rare disease described mainly in immunodepressed patients. Here we report the case of a 76-year-old immunocompetent woman who developed a purulent pericarditis 48 hours after pericardiocentesis. Usual etiologies such as cancer or pericardo-oesophageal fistula, were ruled out. Physical examination revealed a sub-mammary mycosis, which could have led to the infection. The early diagnosis and treatment with a combined medical and surgical approach succeeded in a favorable evolution of this case.

Acute pericarditis after dengue fever.

A 63-year-old woman was admitted for acute chest pain and asthenia worsening for one week. Clinical examination was normal. ECG revealed widespread T waves depression. Echocardiography, cardiac MR-scan, biological examinations and coronary angiogram were normal except positive dengue fever serologies. She had suffered from dengue fever recently. Clinical and ECG outcomes were good under treatment. Cardiac complications are scarce in case of dengue fever. In this case report, clinical and especially ECG presentation are typical of acute pericarditis. This pericarditis is due to dengue fever.

[Are patients undergoing coronary angiography well-informed? Prospective evaluation of the effectiveness of written information]. / Les patients bénéficiant d'une coronarographie sont-ils bien informés ? Évaluation de l'efficacité de l'information écrite. Étude prospective unicentrique.

INTRODUCTION: Prior information in the realization of an invasive intervention is crucial. Indeed, the patient has to know theoretically his disease, diagnostic and therapeutic means, but also the risks of the used technique. The habits of information vary many from one center to another, in spite of the proposition of an information leaflet written by the French Society of Cardiology. Our aim was to evaluate the effectiveness of written information for patients hospitalized for coronary arteriography. METHODS: Among patients hospitalized for realization of a programmed coronarography, a questionnaire was delivered before the information leaflet. The knowledge of the patients was so tested (27 items) before and after the reading of the information sheet (not limited time). The knowledge of the patients concerning coronarography indication, modalities, benefits, possible complications or still later possibilities was informed. RESULTS: Thirty-four patients were included: all knew hospitalization reason, 86% were men, middle-aged 65 (IC 95% 60-70). Thirty-four percent (15-54) had studied in higher education. Ninety-seven percent had had information before. Only 56% (38-74) were informed about the mode of anesthesia, 36% (19-53) duration, 69% (53-86) the injection of iodine, 44% the risk of allergy, 53% the risk of bruise, 15% of the cardiac risks, 21% the renal risks. Seventy-one percent knew the diagnostic benefits, 44% the possible coronary angioplasty, 17% the eventuality of a bypass surgery. The delivery of the information leaflet did not modify the knowledge on most of these items, in particular the modalities and the profits. The risks were known significantly better for the allergy (P=0.019), the bruise (P=0.018), the cardiac risks (0.001). CONCLUSIONS: The population benefiting from a coronarography considers to be enough informed. However, knowledge of the modalities, profits and risks is very low. The delivery of the consensual leaflet does not allow improving the situation, except as far as concerned the complications. Better information is so indispensable, not only to obtain a better support of the patient in the treatment, but also to prevent the forensic implications. The improvement of the information must be multifactorial, but usually used means could be not sufficient.

Routinely-feasible multiple biomarkers score to predict prognosis after revascularized STEMI.

INTRODUCTION: We assessed the long-term prognostic value of an easy-to-do multiple cardiac biomarkers score after a revascularized acute myocardial infarction (MI) in order to evaluate a multimarker approach to risk stratification, based on routine biomarkers. MATERIAL AND METHODS: Blood samples from 138 patients hospitalized with acute myocardial infarction and successfully treated by primary coronary intervention (with TIMI 3 flow) were subsequently tested for creatinin level at admittance and then BNP, hsCRP, troponin I from Day 0 to day 7. The primary endpoint was a clinical evaluation comprising: new hospitalization for cardiac reasons, acute coronary events (acute coronary syndrome), and death. RESULTS: During the median follow-up period of 11.01 months [9.44-12.59], 47 events were recorded. All the following markers were able to predict events: creatinemia on admission (p=0.0057), CRP on day 3 (p, troponin I on day 1 (p<0.001), BNP (p<0.0001) and biological multimarker score (p<0.0001). Clinical events were predicted with a hazard ratio (HR) of respectively 3.30 [2.88-12.30] in BNP Q4 as compared to the three lower quartiles (Q1-3), and 3.15 [2.75-21.00] for the Multimarker approach. The multimarker score was not significantly better than BNP on day 1 alone (p=0.77), troponin on day 1 alone (p=0.43), creatininemia on admission (p=0.19) or CRPhs on day 3 alone (p=0.054). Nevertheless, the Multimarker approach leads to the selection of a smaller, hence more manageable, high-risk population (13% versus 25%). CONCLUSION: Among 138 subjects admitted for acute MI, and all successfully revascularized, a routinely multimarker approach with BNP, hsCRP, creatininemia, troponin I, is feasible. BNP is the most powerful marker, and this multimarker approach renders additional prognostic information helping to identify patients with high-risk to clinical events.

[Atrial fibrillation in 2010: an increasing morbidity and mortality burden]. / La fibrillation atriale en 2010: un poids croissant sur la morbidité et la mortalité cardiovasculaires.

AF, a frequent and banal arrhythmia, is a debilitating and costly disease. The majority of patients with AF are aged 60 to 80 years, but the prevalence is as high as 10% after 80 years and the incidence increases in recent years in an "epidemic" way. AF is responsible for an excess of mortality with an relative risk between 2 and 4 depending of age and sex, especially as cardiovascular risk factors are associated. The morbidity is also important, with cerebral systemic embolism (2-3% per year), heart failure (1 patient for 3), and a total risk of hospitalization from 20 to 30% per year for AF patients with high cardiovascular risks. Whatever be the reasons for hospitalization, cardiovascular or not, in connection with AF or not, these reasons must be well analyzed, so that the risk of occurrence of hospitalization should reflect the efficacy of anti-arrhythmic drugs, or of their complications, or of the comorbidities associated with AF, so common in these older subjects. This morbidity-mortality composite endpoint should now be used in AF randomized trials, as occurring more frequently than mortality (4% per year) or embolic or hemorrhagic usual endpoints. Medico-economic consequences are significant and AF cost is almost 1% of total health spending, with 20% to 30% of the cost for anti-arrhythmic or anti-thrombotic drugs, and 50 to 60% for hospitalizations. Prevention of hospitalizations related to atrial fibrillation may represent a therapeutic target priority on the medico-economic ground.

MR delayed enhancement imaging findings in suspected acute myocarditis.

The purpose of the study was to prospectively assess the clinical impact of routinely performed delayed enhancement imaging in suspected acute myocarditis. A two-centre prospective study was performed in patients with suspected acute myocarditis. The protocol included horizontal long axis, vertical long axis and short axis ciné MR and delayed enhancement imaging after Gd-DTPA infusion (0.2 mmol/kg). Sixty consecutive patients were enrolled (aged 49.4 +/- 17.8 years). MRI demonstrated delayed enhancement sparing the subendocardicardial layer in 51.6% of patients, concordant with the diagnosis of acute myocarditis; 16.7% of patients exhibited delayed enhancement involving the subendocardial layer with irregular margins, concordant with the diagnosis of acute myocardial infarction; 31.7% of patients had delayed enhancement imaging that was considered normal. Routine imaging to identify delayed enhancement provided crucial information in suspected acute myocarditis by reinforcing the diagnosis in 51.6% of patients and correcting a misdiagnosed acute myocardial infarction in 16.7% of patients.

Can troponin elevation predict worse prognosis in patients with acute pericarditis?

INTRODUCTION: Myopericarditis are common in clinical practice: up to 15% of acute pericarditis have a significant myocardial involvement as assessed by biological markers. This prospective, bicentric study is aimed at describing a myopericarditis population, the clinical and MRI follow-up, and search for prognosis markers. PATIENTS AND METHODS: Between May 2005 and September 2007, 103 patients hospitalised for acute pericarditis were prospectively enrolled. Physical examination, ECG, echocardiography, biological screening and cardiac MRI, in case of myopericarditis defined as acute pericarditis with troponin I elevation, were performed. Between December 2007 and July 2008, patients were contacted for new clinical and MRI evaluation. RESULTS: Among the initial population of 103 patients admitted for acute pericarditis, 14 myopericarditis and 38 pericarditis were included. Compared with pericarditis, the myopericarditis group was associated with the following features: younger age (34.9 years [95% CI 28.3-41.2]; p=0.01), ST-segment elevation (nine patients between 14; p=0.03), higher troponin I (7.3 microg/L [95% CI 4.4-10.2]; p<10(-4)) and lower systemic inflammation (CRP peak 38.1mg/L [95% CI 7-69.2]; p=0.01). In the case of myopericarditis, infectious etiologies were predominant (12 patients among 14; p=0.002) and patients stayed longer in hospital (5.8 days [95% CI 4.7-6.8]; p=0.01). Follow-up showed no difference in terms of functional status (p=0.3) and global complications (p=0.9) between paired myopericarditis and pericarditis. Nevertheless, cardiac mortality was higher for myopericarditis (p=0.04). MRI follow-up showed myocardial sequelae without clinical impact. CONCLUSION: Myopericarditis significantly distinguished from pericarditis. Three years follow-up showed no difference in terms of global complications but a higher cardiac mortality for myopericarditis. MRI myocardial lesions did not develop into symptomatic sequelae.

Recurrent severe acute apical-sparing left ventricular dysfunction in a young woman: don't forget pheochromocytoma.

A 35-year-old woman was admitted for second cardiogenic shock. She had no cardiovascular risk factors. Recurrent acute myocarditis was suggested. Recurrent acute myocardial dysfunctions in a young patient suggested pheochromocytoma. Initial trans-thoracic echocardiography showed a typical severe basal and mid-ventricular left ventricular dysfunction but preserved apical contractility. Total CT-scan evidenced a right suprarenal mass advocating for a pheochromocytoma. Biology confirmed the diagnosis of pheochromocytoma. These images illustrate the rare but acute and typical clinical outcomes, and echocardiography findings.