Archives of Cardiovascular Diseases最新文献

Background: Transthyretin cardiac amyloidosis is an important cause of heart failure. Tafamidis, the only specific treatment for cardiac amyloidosis currently widely available in France, has been shown to reduce mortality efficiently.

Aim: The purpose of this study was to describe time to cardiovascular events (death or hospitalization) in patients with transthyretin cardiac amyloidosis treated with tafamidis, and to investigate associated factors.

Methods: We retrospectively included consecutive patients with a new diagnosis of transthyretin cardiac amyloidosis and an indication for tafamidis in a tertiary centre from August 2012 to July 2022. We collected time-to-event data, and associated clinical, biological, ultrasound and therapeutic factors.

Results: We included 128 patients, with visits every 6 months and a median follow-up of 15 months. A total of 42 patients (33%) died or were hospitalized for cardiovascular causes, with a median time-to-event of 1050 days (95% confidence interval 897 - not available). The mean time between diagnosis and start of treatment was 127±361 days. Several factors were associated with cardiovascular outcomes: older age at diagnosis correlated with reduced frequency of death or hospitalization (hazard ratio 0.93, 95% confidence interval 0.89-0.97; P<0.01), whereas obesity correlated with increased occurrence of death or hospitalization (hazard ratio 3.95, 95% confidence interval 1.45-10.76; P=0.01). More importantly, N-terminal prohormone of B-type natriuretic peptide>1000ng/L at diagnosis was a major risk factor in the multivariable analysis (hazard ratio 4.10, 95% confidence interval 1.64-10.25; P<0.01). After the initiation of treatment, New York Heart Association class and weight decreased significantly.

Conclusions: Patients with transthyretin cardiac amyloidosis have a poor prognosis in the short term, despite the use of tafamidis. Important prognostic factors, including clinical features and biomarkers (especially N-terminal prohormone of B-type natriuretic peptide) are essential for risk stratification. As these surrogate endpoints show significant improvement after starting tafamidis, initiating therapy promptly appears mandatory in the most severe patients.

Background: The impact of right ventricular dysfunction on transcatheter mitral valve implantation outcomes and the evolution of right ventricular function after the procedure has not been described.

Aims: To analyse the impact of right ventricular dysfunction on immediate and mid-term outcomes of transcatheter mitral valve implantation, and the evolution of right ventricular function in these patients.

Methods: Consecutive patients who underwent transcatheter mitral valve implantation in our institution were included. Right ventricular function was assessed before transcatheter mitral valve implantation by transthoracic echocardiography, using a multivariable approach. Patients were divided into two groups according to the preprocedural presence of right ventricular dysfunction. Patients were followed up at 3 months and 1 year with a new echocardiographic assessment at each time point.

Results: Among 109 patients finally included (mean age 65±19 years; 66% women), 77 (71%) had normal right ventricular function and 32 (29%) had right ventricular dysfunction before transcatheter mitral valve implantation. Technical success was achieved in 92 (84%) patients. At 30 days, there were no differences between the group with normal right ventricular function and the group with right ventricular dysfunction in terms of death (5 vs. 6%; P=0.86), all-cause rehospitalization (20 vs. 31%; P=0.17) and heart failure without hospitalization (13 vs. 6%; P=0.5). Although the 1-year survival rate was higher in the group with normal right ventricular function (83.1%, 95% confidence interval 74.3% to 92.9%) than in the group with right ventricular dysfunction (68.2%, 95% confidence interval 52.9% to 88.1%) (P=0.09), these differences were not significant after adjustment. Transcatheter mitral valve implantation was associated with improved right ventricular function in the group with initial right ventricular dysfunction at 1-year follow-up (P<0.01).

Conclusions: Right ventricular dysfunction does not appear to have an impact on the early outcomes of transcatheter mitral valve implantation. However, it was associated with an increased rate of late death, although differences were not statistically significant after adjustment. Successful transcatheter mitral valve implantation in patients with severe mitral valve disease associated with right ventricular dysfunction leads to significant improvement in right ventricular function.

Conservative management of aortic insufficiency relies on three physiological strategies preserving native valve function and root dynamics: aortic valve repair; the Ross procedure; and personalized external aortic root support (PEARS). Aortic valve repair is the cornerstone of conservative surgery for dystrophic bicuspid and tricuspid aortic insufficiency, with or without aneurysm, as it restores normal life expectancy while reducing lifelong prosthetic valve-related complications. Standardized techniques combining calibrated annuloplasty with systematic cusp effective height assessment have improved reproducibility and durability, leading to a Class I recommendation in the 2025 European guidelines. Yet, only one in five patients benefits from repair, underscoring the need for wider adoption. The Ross procedure, using the pulmonary autograft as a living valve substitute, offers a quality of life and life expectancy similar to the general population, with fewer thromboembolic or bleeding events than mechanical aortic valve repair and lower reoperation rates than bioprostheses in selected young adults. Tissue-engineered living implants aim to reproduce these results, but are not yet available clinically. PEARS provides a preventive off-pump approach for patients with root aneurysm without valve dysfunction. A three-dimensional customized macroporous mesh stabilizes the aortic root while maintaining native valve physiology and avoiding coronary reimplantation. Together, these three techniques redefine modern aortic valve surgery by offering individualized, durable and physiological alternatives that preserve native tissue, restore normal valve dynamics and achieve normal quality of life and long-term survival.

Background: Heart transplantation following donation after controlled circulatory death (DCD) is not authorized in France, hence a feasibility study was mandatory.

Aims: To conduct a preclinical study to validate the feasibility of DCD heart procurement. We further sought to investigate the metabolic signature of DCD hearts during normothermic ex-situ perfusion (NESP).

Methods: The study design was approved by the Agence de la biomedecine (PFS20-004, La Plaine Saint-Denis, France). Five patients were considered for DCD heart procurement. Femoral vessels were canulated to ensure abdominal normothermic regional perfusion (A-NRP). Direct procurement followed by 4hours of NESP was performed. Donors' demographics and duration of functional warm ischaemic time (fWIT) were collected. Lactate levels were assessed every 30minutes during NESP. Plasma and left ventricular biopsies were collected every 30 and 60minutes, respectively, for untargeted metabolomic analyses using liquid chromatography coupled to high-resolution mass spectrometry.

Results: Mean±standard deviation donor age was 40±11 years and fWIT for the hearts was 26±10min. DCD lungs and kidneys were transplanted except in one case each (impaired ex vivo lung perfusion and premature arrest of A-NRP, respectively). DCD livers were all transplanted when allocated. All hearts were successfully perfused for 4hours. Lactate decreased during NESP for all hearts with a mean±standard deviation initial lactate at 5.42±0.98mmol/L and a final concentration at 3.02±0.86mmol/L (P=0.003). In plasma samples, there were notable changes for 166 metabolites. Most of them either initially increased and stabilized (64/166; 38.6%, e.g. carnitines) or continuously increased (67/166; 40.4%, e.g. purines, medium-chain fatty acids and amino acids). In biopsy samples, there were notable changes for 103 metabolites. Most of them initially decreased and stabilized, such as carnitines and nucleotides.

Conclusion: DCD heart procurement is feasible in France. Lactate trends were consistent with suitability of these hearts for transplantation. The metabolomic signature was characterized by nucleotide catabolism along with consumption of carnitines.

Background: Transthyretin amyloidosis can lead to transthyretin amyloid cardiomyopathy (ATTR-CM) and heart failure.

Aims: To describe the prevalence of cardiac and extracardiac diseases in patients with ATTR-CM and examine mortality predictors, including diuretic dosage, using the French National Health Database.

Methods: Patients with ATTR-CM and their medical characteristics were identified from the French database from 2011 to 2019. Diuretic doses were categorized into four classes (furosemide dose: level 1<20mg; level 2 20 to <60mg; level 3 60 to<120mg; level 4 ≥120mg). Predictive factors for mortality were examined.

Results: Of 7804 patients with ATTR-CM, 33.0% were on level 1 diuretics, 25.8% on level 2, 15.8% on level 3 and 25.3% on level 4 at diagnosis. Leading extracardiac conditions included kidney disease (37.0%), diabetes (29.5%), neurologic disorders (17.9%), gastrointestinal disorders (15.7%) and musculoskeletal conditions (11.8%). Median (95% confidence interval [CI]) survival was 3.1 (3.0-3.3) years. Multivariable analysis identified age at diagnosis (hazard ratio [HR] 1.482, 95% CI 1.400-1.558), male sex (HR 1.258, 95% CI 1.125-1.406), diuretic dose (HR 1.380, 95% CI 1.315-1.449), heart failure (HR 1.251, 95% CI 1.090-1.437), arrhythmia/conduction disorder (HR 1.143, 95% CI 1.001-1.306), kidney disease (HR 1.224, 95% CI 1.104-1.358), gastrointestinal disorder (HR 1.143, 95% CI 1.000-1.307) and diabetes (HR 1.192, 95% CI 1.071-1.326) as significantly associated with mortality.

Conclusion: Patients with ATTR-CM face a significant burden of associated diseases requiring comprehensive management alongside their ATTR-CM treatment. Beyond addressing these comorbidities, diuretic dosage emerges as a pivotal prognostic indicator.