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Background: Hypertrophic cardiomyopathies (HCM) can be complicated by left ventricular outflow-tract obstruction (LVOTO) responsible for disabling exercise symptoms, a phenomenon influenced by hemodynamic factors including venous return. Methods: We aimed to evaluate venous dysfunction in obstructive HCM patients compared to healthy controls, and to investigate the relationship between venous dysfunction parameters and LVOTO in HCM. This is a clinical, monocentric, prospective, pilot study, in a tertiary care center. We investigated venous function using venous air plethysmography, and endothelial function. Results: Among the 30 symptomatic obstructive HCM patients, 30% (n = 9) presented abnormal venous residual volume fraction (RVFv) which translates in elevated ambulatory venous pressure vs. 0% in the 10 healthy controls (p < 0.05). Comparing obstructive HCM patients with abnormal RVFv (n = 9) to other obstructive HCM patients with normal RVFv (n = 21), there were no significant differences in terms of age, sex (67% male), and classical echocardiographic parameters both at rest and during exercise, except for left ventricular end-diastolic volume index which was significantly lower in the group with abnormal RVFv compared to the other HCM patients (40.1 ± 9.0 ml/m2 vs. 50.2 ± 10.6 ml/m2, p = 0.01). Fifty six percent of obstructive HCM patients with abnormal RVFv had an absolute increase in Willebrand factor (vs. 26% of other obstructive HCM patients, p < 0.05). Conclusions: In this pilot monocentric study, venous insufficiency was observed in about 30% of symptomatic obstructive HCM patients. Patients with venous insufficiency had more frequently a smaller LV cavity volume. Due to the small sample size, this study is only hypothesis-generating, and further investigations are needed.
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BACKGROUND: Screening for Fabry disease is sub-optimal in non-specialised centres. AIM: To assess the diagnostic value of electrocardiographic scores of left ventricular hypertrophy and a combined electrocardiographic and echocardiographic model in Fabry disease. METHODS: We retrospectively reviewed the electrocardiograms and echocardiograms of 61 patients (mean age 55.6±11.5 years; 57% men) with Fabry disease and left ventricular hypertrophy, and compared them with those from 59 patients (mean age 44.8±18.3 years; 66% men) with sarcomeric hypertrophic cardiomyopathy. Six electrocardiography criteria for left ventricular hypertrophy were specifically analysed: Sokolow-Lyon voltage index; Cornell voltage index; Gubner index; Romhilt-Estes score; Sokolow-Lyon product (voltage index×QRS duration); and Cornell product (voltage index×QRS duration). RESULTS: Right bundle branch block was more frequent in patients with Fabry disease (54% vs. 22%; P=0.001). QRS duration, Gubner score and Sokolow-Lyon product were significantly higher in patients with Fabry disease. Maximal wall thickness was higher in patients with sarcomeric hypertrophic cardiomyopathy (21.9±5.1 vs. 15.5±2.9mm; P<0.001). Indexed sinus of Valsalva diameter was larger in patients with Fabry disease. After multivariable analysis, right bundle branch block, Sokolow-Lyon product, maximal wall thickness and aortic diameter were independently associated with Fabry disease. A model including these four variables yielded an area under the receiver operating characteristic curve of 0.918 (95% confidence interval 0.868-0.968) for Fabry disease. CONCLUSION: Our model combining easy-to-assess electrocardiographic and echocardiographic variables may be helpful in improving screening and reducing diagnosis delay in Fabry disease.
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Cardiomiopatia Hipertrófica/diagnóstico , Ecocardiografia , Eletrocardiografia , Doença de Fabry/diagnóstico , Adulto , Idoso , Cardiomiopatia Hipertrófica/diagnóstico por imagem , Cardiomiopatia Hipertrófica/fisiopatologia , Diagnóstico Diferencial , Doença de Fabry/diagnóstico por imagem , Doença de Fabry/fisiopatologia , Feminino , Frequência Cardíaca , Humanos , Masculino , Pessoa de Meia-Idade , Variações Dependentes do Observador , Valor Preditivo dos Testes , Reprodutibilidade dos Testes , Estudos Retrospectivos , Volume Sistólico , Função Ventricular Esquerda , Função Ventricular Direita , Remodelação VentricularRESUMO
In Anderson-Fabry disease (FD), we sought to evaluate relation between left ventricular (LV) hypertrophy, longitudinal strain (LS), myocardial T1 mapping and cardiopulmonary exercise parameters, and their prognostic value in term of cardiovascular outcomes. In this prospective, observational, monocentric study called "FABRY-Image", we evaluated consecutive adult FD patients by echocardiography, cardiac magnetic resonance, and cardiopulmonary exercise testing. We investigated regional LS, the relations between LV hypertrophy, LS, T1 mapping, and VO2 peak and VE/VCO2, and the prediction of cardiovascular events during follow-up. From 2016 to 2019, we included 35 FD patients (44 ± 17 years, 40% male), that were compared with 20 controls. In FD patients, global, basal and mid-LV LS, as well as mean T1 were significantly altered compared to controls (p < 0.05) with relative apical LS sparing. LV wall thickness was particularly related to mean of basal LS (r = - 0.73), to T1 (r = - 0.48), and to VE/VCO2 (r = 0.45). Mean of basal LS was well related to myocardial T1 (r = 0.59). A good relation was observed between VO2 peak and global LS (r = 0.39) while VE/VCO2 slope was more related to maximal LV wall thickness (r = 0.45), and T1 (r = - 0.61). During a median follow-up of 2.4 years, 6/31 patients presented de novo atrial fibrillation or stroke. In Cox univariate analyses, LV wall thickness, basal LS, T1 value, and VE/VCO2 were significantly predictive of occurrence of de novo atrial fibrillation or stroke (p < 0.05). Our study shows an apical LS sparing in FD patients as observed in amyloidosis, and a close relation between LV hypertrophy, LS, T1 mapping, and VE/VCO2 which are all associated to the occurrence of de novo atrial fibrillation or TIA/stroke during follow-up. These results need to be confirmed by future multicentric studies.
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Ecocardiografia Doppler , Teste de Esforço , Tolerância ao Exercício , Doença de Fabry/diagnóstico por imagem , Hipertrofia Ventricular Esquerda/diagnóstico por imagem , Imagem Cinética por Ressonância Magnética , Função Ventricular Esquerda , Remodelação Ventricular , Adulto , Doença de Fabry/fisiopatologia , Feminino , França , Humanos , Hipertrofia Ventricular Esquerda/fisiopatologia , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Prognóstico , Estudos Prospectivos , Fatores de TempoRESUMO
Tricuspid regurgitation has long been a neglected and underestimated entity; its prevalence is significant, and is increasing with the ageing population. Tricuspid regurgitation is often a consequence of chronic left cardiac pathologies or atrial fibrillation. Surgical treatment is recommended for patients with severe symptomatic tricuspid regurgitation or tricuspid annulus dilatation at the time of left heart valve surgery. Secondary tricuspid regurgitation is a complex disease; this review focuses on the need for better understanding of its mechanisms and quantification - mandatory with the advent of new percutaneous treatments.
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Ecocardiografia/métodos , Hemodinâmica , Insuficiência da Valva Tricúspide/diagnóstico por imagem , Valva Tricúspide/diagnóstico por imagem , Humanos , Imageamento por Ressonância Magnética , Valor Preditivo dos Testes , Prognóstico , Fatores de Risco , Índice de Gravidade de Doença , Valva Tricúspide/fisiopatologia , Valva Tricúspide/cirurgia , Insuficiência da Valva Tricúspide/etiologia , Insuficiência da Valva Tricúspide/fisiopatologia , Insuficiência da Valva Tricúspide/cirurgiaRESUMO
Fabry disease is an X-linked progressive multisystemic genetic sphingolipidosis caused by deficient activity of lysosomal α-galactosidase A. Men aged>30 years and women aged>40 years most often present with unexplained left ventricular hypertrophy, usually concentric and non-obstructive, but sometimes mimicking sarcomeric hypertrophic cardiomyopathy, particularly when isolated, as in the cardiac or late-onset variant of the disease. In hypertrophic cardiomyopathy cohorts, up to 1% of patients have been diagnosed with Fabry disease. Frequent cardiac symptoms include chronotropic incompetence, severe conduction disturbances and arrhythmias, heart failure and sudden death, and cardiovascular complications are currently the leading cause of death at a mean age of 55 years in men and 66 years in women. Complementary to screening for extracardiac manifestations, the initial cardiac evaluation should include long-duration electrocardiogram recordings, echocardiography and late gadolinium and T1 mapping magnetic resonance imaging. Abnormalities of a non-hypertrophied inferolateral wall at the base of the left ventricle (thinning, decreased strain, midwall fibrosis) and low native T1 signal on magnetic resonance imaging are evocative. Aggressive cardiac management may include the control of cardiovascular risk factors, anticoagulation, permanent cardiac pacing and/or an implantable cardioverter defibrillator device, while antiarrhythmics and beta-blockers should be used with caution. Specific therapy should be initiated at the earliest stage, when the first structural or functional cardiac abnormalities are detected, and should include enzyme replacement therapy (available since 2001) or chaperone therapy (available since 2016) (the use of which is limited to patients with Fabry disease and an amenable α-galactosidase A [GLA] gene mutation).
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Cardiologia , Doença de Fabry , Hipertrofia Ventricular Esquerda , Adulto , Idoso , Cardiomiopatia Hipertrófica/diagnóstico , Diagnóstico Diferencial , Ecocardiografia , Eletrocardiografia , Terapia de Reposição de Enzimas , Doença de Fabry/diagnóstico , Doença de Fabry/mortalidade , Doença de Fabry/fisiopatologia , Doença de Fabry/terapia , Feminino , Humanos , Hipertrofia Ventricular Esquerda/diagnóstico , Hipertrofia Ventricular Esquerda/mortalidade , Hipertrofia Ventricular Esquerda/fisiopatologia , Hipertrofia Ventricular Esquerda/terapia , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Prognóstico , Fatores de Risco , Função Ventricular Esquerda , Remodelação VentricularRESUMO
BACKGROUND: Our knowledge of hypertrophic cardiomyopathy (HCM) mainly originates from quarternary centres. The objective is to assess the current management of HCM patients in a large multicentre French register according to the level of expertise. METHODS AND RESULTS: A total of 1431 HCM patients were recruited across 26 (11 expert and 15 non-expert) centres in REMY, a prospective hospital-based register of adult HCM patients. A sarcomeric origin was suspected in 1284 (89.7%) patients [261 (20.3%) with a reported gene mutation, 242 (18.8%) genotype-negative], while 107 (7.5%) had a diagnosis of non-sarcomeric HCM. Patients managed in non-expert centres were older (Pâ¯<â¯0.01) and presented more often with NYHA III/IV class dyspnoea (Pâ¯<â¯0.01), congestive heart failure (Pâ¯<â¯0.01), low LEVF (Pâ¯<â¯0.01), less often with a syncope history (Pâ¯<â¯0.01) and lower LV obstruction (Pâ¯<â¯0.01) than patients in expert centres. Genotype positive sarcomeric aetiologies were less frequent in non-expert centres (Pâ¯<â¯0.01). The use of diagnostic and prognostic tests as cardiac MRI (Pâ¯<â¯0.001), genetic (Pâ¯<â¯0.001) and alpha-galactosidase A enzyme level testing (Pâ¯<â¯0.001), Holter ECG (Pâ¯<â¯0.001), and exercise test (Pâ¯<â¯0.001), was lower in non-expert centres. Septal ablation procedures using alcohol (Pâ¯<â¯0.001) or myectomy (Pâ¯<â¯0.001) were more frequent in expert centres. CONCLUSION: In real life practice, only a minority of HCM patients are identified as sarcomere positive as per genetic testing. The management of HCM patients varies according to the centre's level of expertise, with less access to diagnostic and prognostic tests in non-expert centres. Non-sarcomeric HCM may therefore be overlooked despite specific treatment in some aetiologies.
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Procedimentos Cirúrgicos Cardíacos/métodos , Cardiomiopatia Hipertrófica/diagnóstico , Gerenciamento Clínico , Testes Genéticos/métodos , Imagem Cinética por Ressonância Magnética/métodos , Sistema de Registros , Tomografia Computadorizada por Raios X/métodos , Cardiomiopatia Hipertrófica/genética , Cardiomiopatia Hipertrófica/cirurgia , Análise Mutacional de DNA , Feminino , Seguimentos , Predisposição Genética para Doença , Genótipo , Humanos , Masculino , Pessoa de Meia-Idade , Mutação , Miosinas/genética , Prognóstico , Estudos Prospectivos , Sarcômeros/genética , Sarcômeros/metabolismoRESUMO
BACKGROUND: Severe left ventricular (LV) systolic dysfunction is an uncommon complication of hypertrophic cardiomyopathy (HCM) that is associated with poor prognosis. Small observational series suggest that patients with rare causes of HCM are more likely to develop systolic impairment than those with idiopathic disease or mutations in cardiac sarcomeric protein genes. The aim of this study was to test this hypothesis by comparing the prevalence of systolic dysfunction and its impact on prognosis in patients with different causes of HCM. METHODS AND RESULTS: 1697 patients (52 (40-63) years, 1160 (68%) males) with HCM followed at two European referral centres were studied. Diagnosis of specific aetiologies was made on the basis of clinical examination, cardiac imaging and targeted genetic and biochemical testing. The primary survival outcome was all-cause mortality or heart transplantation (HTx) for end-stage heart failure (HF). Secondary outcomes were HF-related death, sudden cardiac death, stroke-related death and non-cardiovascular death. Systolic dysfunction (LV ejection fraction <50% by two-dimensional (2D) echocardiography) at first evaluation was more frequent in rare phenocopies than in idiopathic or sarcomeric HCM (105/409 (26%) vs 40/1288 (3%), respectively (p<0.0001)). All-cause death/HTx and HF-related death were more frequent in rare phenocopies compared with idiopathic or sarcomeric HCM (p<0.0001). All-cause mortality and HF-related death were highest in patients with cardiac amyloidosis (p<0.0001). CONCLUSIONS: In adults with HCM, LV systolic dysfunction is more frequent in those with rare phenocopies. When combined with age at presentation, it is a marker for specific aetiologies and is associated with poorer long-term survival.
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Cardiomiopatia Hipertrófica/epidemiologia , Disfunção Ventricular Esquerda/epidemiologia , Função Ventricular Esquerda , Adulto , Idoso , Cardiomiopatia Hipertrófica/genética , Cardiomiopatia Hipertrófica/fisiopatologia , Cardiomiopatia Hipertrófica/cirurgia , Morte Súbita Cardíaca/epidemiologia , Progressão da Doença , Feminino , Predisposição Genética para Doença , Insuficiência Cardíaca/epidemiologia , Insuficiência Cardíaca/fisiopatologia , Insuficiência Cardíaca/cirurgia , Transplante de Coração , Humanos , Itália/epidemiologia , Estimativa de Kaplan-Meier , Londres/epidemiologia , Estudos Longitudinais , Masculino , Pessoa de Meia-Idade , Fenótipo , Prevalência , Estudos Retrospectivos , Fatores de Risco , Sobreviventes , Sístole , Fatores de Tempo , Disfunção Ventricular Esquerda/fisiopatologia , Disfunção Ventricular Esquerda/cirurgiaRESUMO
OBJECTIVE: To investigate transcatheter aortic valve implantation (TAVI) feasibility, effectiveness and safety in radiation-induced aortic valve stenosis cases. METHODS: 198 consecutive patients referred for TAVI were prospectively enrolled. They were divided into two groups: patients with a history of chest radiation therapy with suspected radiation-induced valvular disease (RXT) and others with suspected degenerative aortic valve stenosis (NRXT). Procedural, early and mid-term clinical outcomes were compared. RESULTS: Of the 198 patients enrolled in our study, 9.6% qualified for inclusion in the RXT group. A comparison of baseline characteristics revealed that patients with RXT were younger than patients with NRXT (68.3 vs 82.5â years; p<0.05) and exhibited a lower surgical risk score (Euroscore: 7.1% vs 21.8%; p<0.05) and a higher frequency of hostile thorax and porcelain aorta (52.6% vs 28.5%; p<0.05; 63.2% vs 10.6%; p<0.05, respectively). In both groups, the implantation success rate was high and the 30-day safety end point acceptable (RXT: 94.7% and 83.3%; NRXT: 93.9% and 75.6%, respectively). At 6â months, overall mortality was significantly lower in the RXT group (0% vs 18%; p=0.048). CONCLUSIONS: In patients suffering from radiation-induced aortic valve stenosis and contraindicated for surgery, TAVI is a promising approach, with high feasibility, acceptable risk, low mortality and high clinical effectiveness at mid-term follow-up.
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BACKGROUND: The prognosis and treatment of the 2 main types of cardiac amyloidosis, immunoglobulin light chain (AL) and transthyretin (ATTR) amyloidosis, are substantially influenced by cardiac involvement. Cardiovascular magnetic resonance with late gadolinium enhancement (LGE) is a reference standard for the diagnosis of cardiac amyloidosis, but its potential for stratifying risk is unknown. METHODS AND RESULTS: Two hundred fifty prospectively recruited subjects, 122 patients with ATTR amyloid, 9 asymptomatic mutation carriers, and 119 patients with AL amyloidosis, underwent LGE cardiovascular magnetic resonance. Subjects were followed up for a mean of 24±13 months. LGE was performed with phase-sensitive inversion recovery (PSIR) and without (magnitude only). These were compared with extracellular volume measured with T1 mapping. PSIR was superior to magnitude-only inversion recovery LGE because PSIR always nulled the tissue (blood or myocardium) with the longest T1 (least gadolinium). LGE was classified into 3 patterns: none, subendocardial, and transmural, which were associated with increasing amyloid burden as defined by extracellular volume (P<0.0001), with transitions from none to subendocardial LGE at an extracellular volume of 0.40 to 0.43 (AL) and 0.39 to 0.40 (ATTR) and to transmural at 0.48 to 0.55 (AL) and 0.47 to 0.59 (ATTR). Sixty-seven patients (27%) died. Transmural LGE predicted death (hazard ratio, 5.4; 95% confidence interval, 2.1-13.7; P<0.0001) and remained independent after adjustment for N-terminal pro-brain natriuretic peptide, ejection fraction, stroke volume index, E/E', and left ventricular mass index (hazard ratio, 4.1; 95% confidence interval, 1.3-13.1; P<0.05). CONCLUSIONS: There is a continuum of cardiac involvement in systemic AL and ATTR amyloidosis. Transmural LGE is determined reliably by PSIR and represents advanced cardiac amyloidosis. The PSIR technique provides incremental information on outcome even after adjustment for known prognostic factors.
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Amiloidose/diagnóstico , Cardiomiopatias/diagnóstico , Imageamento por Ressonância Magnética/métodos , Miocárdio/patologia , Idoso , Feminino , Gadolínio , Humanos , Aumento da Imagem , Masculino , Pessoa de Meia-Idade , PrognósticoRESUMO
BACKGROUND: Sarcomeric gene mutations cause hypertrophic cardiomyopathy (HCM). In gene mutation carriers without left ventricular (LV) hypertrophy (G + LVH-), subclinical imaging biomarkers are recognized as predictors of overt HCM, consisting of anterior mitral valve leaflet elongation, myocardial crypts, hyperdynamic LV ejection fraction, and abnormal apical trabeculation. Reverse curvature of the interventricular septum (into the LV) is characteristic of overt HCM. We aimed to assess LV septal convexity in subclinical HCM. METHODS: Cardiovascular magnetic resonance was performed on 36 G + LVH- individuals (31 ± 14 years, 33 % males) with a pathogenic sarcomere mutation, and 36 sex and age-matched healthy controls (33 ± 12 years, 33 % males). Septal convexity (SCx) was measured in the apical four chamber view perpendicular to a reference line connecting the mid-septal wall at tricuspid valve insertion level and the apical right ventricular insertion point. RESULTS: Septal convexity was increased in G + LVH- compared to controls (maximal distance of endocardium to reference line: 5.0 ± 2.5 mm vs. 1.6 ± 2.4 mm, p ≤ 0.0001). Expected findings occurred in G + LVH- individuals: longer anterior mitral valve leaflet (23.5 ± 3.0 mm vs. 19.9 ± 3.1 mm, p ≤ 0.0001), higher relative wall thickness (0.31 ± 0.05 vs. 0.29 ± 0.04, p ≤ 0.05), higher LV ejection fraction (70.8 ± 4.3 % vs. 68.3 ± 4.4 %, p ≤ 0.05), and smaller LV end-systolic volume index (21.4 ± 4.4 ml/m(2) vs. 23.7 ± 5.8 ml/m(2), p ≤ 0.05). Other morphologic measurements (LV angles, sphericity index, and eccentricity index) were not different between G + LVH- and controls. CONCLUSIONS: Septal convexity is an additional previously undescribed feature of subclinical HCM.
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Cardiomiopatia Hipertrófica/diagnóstico , Ventrículos do Coração/patologia , Imagem Cinética por Ressonância Magnética , Adolescente , Adulto , Doenças Assintomáticas , Cardiomiopatia Hipertrófica/genética , Cardiomiopatia Hipertrófica/patologia , Cardiomiopatia Hipertrófica/fisiopatologia , Estudos de Casos e Controles , Feminino , Marcadores Genéticos , Predisposição Genética para Doença , Ventrículos do Coração/fisiopatologia , Humanos , Masculino , Pessoa de Meia-Idade , Mutação , Fenótipo , Valor Preditivo dos Testes , Volume Sistólico , Função Ventricular Esquerda , Adulto JovemRESUMO
AIMS: The epidemiologic link between benfluorex use and an increased global frequency of left heart valve regurgitation has been well documented. However, no data linking previous drug exposure to the frequency of diagnosis of drug-induced valvular heart disease (DI-VHD) are available. The present study was conducted to address this issue. METHODS AND RESULTS: This echocardiography reader-blinded, controlled study conducted in 10 centres between February 2010 and February 2012 prospectively included 835 subjects previously exposed to benfluorex referred by primary care physicians for echocardiography. Based on blinded off-line analysis, echocardiography findings were classified as: (i) DI-VHD⺠for patients with an echocardiographic diagnosis of DI-VHD, (ii) inconclusive, and (iii) DI-VHDâ» for patients without signs of DI-VHD. Fifty-seven (6.8%) patients exposed to benfluorex were classified as DI-VHDâº, 733 (87.8%) patients were classified as DI-VHDâ», and 45 (5.4%) were classified as inconclusive. Mitral and aortic DI-VHD were reported in 43 patients (5.1%) and 30 (3.6%) patients, respectively. Longer duration of exposure, female gender, smoking, and lower BMI were independently associated with a diagnosis of DI-VHD. Good inter-observer reproducibility was observed for the echocardiography classification (Kappa = 0.83, P < 0.00001). CONCLUSIONS: About 7% of patients without a history of heart valve disease previously exposed to benfluorex present echocardiography features of DI-VHD. Further studies are needed to study the natural history of DI-VHD and to identify risk factors for the development of drug-induced valve lesions.
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Insuficiência da Valva Aórtica/induzido quimicamente , Depressores do Apetite/efeitos adversos , Fenfluramina/análogos & derivados , Hipolipemiantes/efeitos adversos , Insuficiência da Valva Mitral/induzido quimicamente , Análise de Variância , Estudos de Casos e Controles , Diabetes Mellitus/tratamento farmacológico , Dislipidemias/tratamento farmacológico , Ecocardiografia , Feminino , Fenfluramina/efeitos adversos , Humanos , Masculino , Pessoa de Meia-Idade , Obesidade/tratamento farmacológico , Estudos Prospectivos , Fatores de RiscoRESUMO
BACKGROUND: Benfluorex was withdrawn from European markets in June 2010 after reports of an association with heart valve lesions. The link between benfluorex and valve regurgitations was based on small observational studies and retrospective estimations. We therefore designed an echocardiography-based multicenter study to compare the frequency of left heart valve regurgitations in diabetic patients exposed to benfluorex for at least 3 months and in diabetic control subjects never exposed to the drug. METHODS AND RESULTS: This reader-blinded, controlled study conducted in 10 centers in France between February 2010 and September 2011 prospectively included 376 diabetic subjects previously exposed to benfluorex who were referred by primary care physicians for echocardiography and 376 diabetic control subjects. Through the use of propensity scores, 293 patients and 293 control subjects were matched for age, sex, body mass index, smoking, dyslipidemia, hypertension, and coronary artery disease. The main outcome measure was the frequency of mild or greater left heart valve regurgitations. In the matched sample, the frequency and relative risk (odds ratio) of mild or greater left heart valve regurgitations were significantly increased in benfluorex patients compared with control subjects: 31.0% versus 12.9% (odds ratio, 3.55; 95% confidence interval, 2.03-6.21) for aortic and/or mitral regurgitation, 19.8% versus 4.7% (odds ratio, 5.29; 95% confidence interval, 2.46-11.4) for aortic regurgitation, and 19.4% versus 9.6% (odds ratio, 2.38; 95% confidence interval, 1.27-4.45) for mitral regurgitation. CONCLUSIONS: Our results indicate that the use of benfluorex is associated with a significant increase in the frequency of left heart valve regurgitations in diabetic patients. The natural history of benfluorex-induced valve abnormalities needs further research.
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Insuficiência da Valva Aórtica/induzido quimicamente , Insuficiência da Valva Aórtica/epidemiologia , Fenfluramina/análogos & derivados , Insuficiência da Valva Mitral/induzido quimicamente , Insuficiência da Valva Mitral/epidemiologia , Vigilância de Produtos Comercializados/métodos , Idoso , Insuficiência da Valva Aórtica/diagnóstico por imagem , Depressores do Apetite/efeitos adversos , Diabetes Mellitus/epidemiologia , Ecocardiografia , Europa (Continente)/epidemiologia , Feminino , Fenfluramina/efeitos adversos , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Insuficiência da Valva Mitral/diagnóstico por imagem , Estudos Prospectivos , Fatores de Risco , Retirada de Medicamento Baseada em Segurança , Método Simples-CegoRESUMO
BACKGROUND: Although dysfunction of the systemic right ventricle (RV) in patients with complete transposition of the great arteries (TGA) after atrial redirection by Mustard or Senning procedures is well recognized, there are few data on systemic RV geometry and function. Echocardiography is a widely available imaging technique that is particularly suitable for clinical follow-up because of its non-invasive nature, low cost and lack of ionizing radiation. AIM: To examine the feasibility and variability of transthoracic echocardiography variables in the assessment of the systemic RV. METHODS: Multivariable transthoracic echocardiographic analysis, including assessment of global function variables (RV ejection fraction [RVEF; Simpson's method], RV fractional shortening [RVFS] and dP/dt), longitudinal function variables (tricuspid annular plane systolic excursion [TAPSE], peak systolic velocity at the junction of the RV free wall and the tricuspid annulus, assessed with pulsed tissue Doppler imaging [S' TDI]), tricuspid regurgitation and asynchrony, was performed in 35 consecutive patients with TGA after atrial redirection. Functional variables were compared with magnetic resonance imaging (MRI). Inter- and intraobserver echocardiographic analysis variability was assessed in ten randomly selected cases. RESULTS: Global and longitudinal function variables were not correlated with RVEF calculated by MRI, except for S' TDI, which was weakly correlated (P = 0.02, r = 0.37). Asynchrony assessment was feasible in all patients. Inter- and intraobserver echocardiographic analysis variability was high for RVEF, RVFS and dP/dt (> 10%), and low for TAPSE and S' TDI (5%). CONCLUSION: Owing to geometric changes, presumed contractility pattern shift and retrosternal position, conventional echocardiographic variables are not relevant for RV function assessment. Assessment of asynchrony and tricuspid regurgitation is easily feasible in routine practice and highly reproducible. Echocardiography does not permit complete assessment of the systemic RV after atrial redirection but is fully complementary with MRI and should not be abandoned. Future improvements in transducers and dedicated software should permit major improvements in the near future.
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Ecocardiografia Doppler de Pulso , Ventrículos do Coração/diagnóstico por imagem , Transposição dos Grandes Vasos/cirurgia , Disfunção Ventricular Direita/diagnóstico por imagem , Função Ventricular Direita , Adulto , Procedimentos Cirúrgicos Cardíacos , Estudos de Viabilidade , Feminino , Átrios do Coração/cirurgia , Humanos , Imageamento por Ressonância Magnética , Masculino , Variações Dependentes do Observador , Volume Sistólico , Sístole , Insuficiência da Valva Tricúspide/diagnóstico por imagemRESUMO
BACKGROUND: Ultraminiaturization of echographic systems extraordinarily provides the image "within" the clinical examination. Abdominal aorta aneurysm (AAA) diagnosis based on conventional evaluation with a dedicated operator and ultrasound machine is still controversial due to the lack of evidence of the proposed management and guidelines' cost-effectiveness. We hypothesized that less expensive ultraportable devices could identify AAA with the same level of accuracy as conventional approaches. METHODS: A first step of this study was to validate the VSCAN's image capabilities in patients referred to the vascular Doppler laboratory. Abdominal aorta measurements were performed by an experienced physician using conventional equipment followed by a second blinded physician using the ultraportable device VSCAN. Then, 204 patients hospitalized in our cardiology institute were prospectively included for a systematic screening of AAA at bedside using the VSCAN in order to determine the feasibility and impact of fast track evaluation compared to clinical examination. RESULTS: A strong correlation was obtained between measurements of abdominal aorta diameters using the two ultrasound systems (r = 0.98, CI: 0.97-0.99, P < 0.001) with 100% of agreement for AAA diagnosis. In the second part of the study, visualization and measurement of the transverse diameter of the abdominal aorta was obtained in 199 patients, resulting in a feasibility of 97.5%. Among these patients, 18 AAAs were detected, which corresponds to a prevalence of 9%, whereas clinical evaluation did not detect any of them. Patients with AAA were more likely men (77.77% vs. 57.45%, P < 0.05) and hypertensive (88.8% vs. 56.9%, P < 0.05) as compared to those without AAA. Two patients with large AAA were quickly referred to the surgery department. CONCLUSION: Considering its low cost, diagnostic accuracy, and widespread availability, screening for AAA using an ultraportable ultrasound device such as VSCAN by an experienced physician is promising and should be used as an extension of routine physical examination in vascular patients.
Assuntos
Aneurisma da Aorta Abdominal/diagnóstico por imagem , Ecocardiografia/instrumentação , Sistemas Automatizados de Assistência Junto ao Leito , Criança , Sistemas Computacionais , Desenho de Equipamento , Análise de Falha de Equipamento , Feminino , Humanos , Masculino , Miniaturização , Reprodutibilidade dos Testes , Sensibilidade e EspecificidadeRESUMO
We present the case of a caseous calcification of the mitral annulus, responsible for two strokes, in a 72-year-old female patient. The brain computed tomography (CT) scan confirmed the presence of a calcific embolus. The echocardiography showed a liquidy, pseudotumoral mass combined with numerous calcifications located in the posterior part of the mitral annulus and extending toward the inferior surface of the left ventricle. During surgery, we found a direct communication between the caseous necrosis and the lumen of the left ventricle at the level of its inferior wall. We performed a valve repair procedure and excision of the caseous necrosis, combined with injection of bioglue into the cavity, to avoid recurrence. Six months after the procedure, the patient was in good health, and had no recurrence of stroke with a satisfactory echocardiography. This is the first description of spontaneous fistulization of a caseous necrosis in the lumen of the left ventricle, explaining a new mechanism for cerebral embolism during the course of calcifying diseases of the mitral annulus.
Assuntos
Calcinose/complicações , Fístula/complicações , Doenças das Valvas Cardíacas/complicações , Valva Mitral/diagnóstico por imagem , Acidente Vascular Cerebral/etiologia , Idoso , Calcinose/diagnóstico por imagem , Calcinose/cirurgia , Feminino , Cardiopatias/etiologia , Doenças das Valvas Cardíacas/diagnóstico por imagem , Doenças das Valvas Cardíacas/cirurgia , Humanos , Embolia Intracraniana/etiologia , Valva Mitral/cirurgia , UltrassonografiaRESUMO
We present and characterize an original experimental model to create a chronic ischemic heart failure in pig. Two ameroid constrictors were placed around the LAD and the circumflex artery. Two months after surgery, pigs presented a poor LV function associated with a severe mitral valve insufficiency. Echocardiography analysis showed substantial anomalies in radial and circumferential deformations, both on the anterior and lateral surface of the heart. These anomalies in function were coupled with anomalies of perfusion observed in echocardiography after injection of contrast medium. No demonstration of myocardial infarction was observed with histological analysis. Our findings suggest that we were able to create and to stabilize a chronic ischemic heart failure model in the pig. This model represents a useful tool for the development of new medical or surgical treatment in this field.
RESUMO
Despite much progress in the medical management of myocardial ischemia, several problems remain and experimental models help to improve our understanding of the pathophysiology involved in this domain. The ameroid constrictor model is the most widely used to create ischemia but evaluation of patent ischemia is still under debate. In the present study, we describe the potential of a two-dimensional (2D) strain for experimentally evaluating myocardial ischemia in the pig. An ameroid constrictor was placed around the circumflex artery in 30 pigs. Angiography showed 90% stenosis at one and two months. Left ventricular function was moderately altered and associated with mitral valve insufficiency in 30% of cases. Longitudinal and circumference strains were dramatically modified in the ischemic inferior-lateral zone compared to the healthy anterior zone (P<0.01) at one and two months. We correlated these results to myocardial ischemia by using contrast echocardiography, which showed a significant reduction in myocardial perfusion in the ischemic zone compared to the uninjured area, and by using histological analysis. We showed that evaluation of the 2D strain could be an interesting approach for assessing myocardial ischemia after ameroid constrictor implantation. The 2D strain represents a useful tool for the evaluation of experimental models of myocardial ischemia.
Assuntos
Estenose Coronária/patologia , Ecocardiografia/métodos , Isquemia Miocárdica/diagnóstico por imagem , Isquemia Miocárdica/patologia , Análise de Variância , Animais , Biópsia por Agulha , Caseínas , Doença Crônica , Angiografia Coronária/métodos , Estenose Coronária/complicações , Estenose Coronária/diagnóstico por imagem , Estenose Coronária/cirurgia , Modelos Animais de Doenças , Hidrogéis , Imuno-Histoquímica , Masculino , Isquemia Miocárdica/etiologia , Isquemia Miocárdica/cirurgia , Distribuição Aleatória , Suínos , Função Ventricular Esquerda/fisiologiaRESUMO
Optimal management of prosthetic heart valve obstruction (PHVO) remains controversial even though surgery is usually recommended. To better define the efficacy and safety of fibrinolysis versus surgery in the pre- and post-transoesophageal echocardiography (TEE) eras. We analysed initial results and follow-up data from a large, retrospective, single-centre series, comparing fibrinolysis and surgery in patients with PHVO treated over 20 years. Two hundred and sixty-three consecutive episodes of PHVO in 210 patients, mainly left sided, were managed in our institution by either fibrinolysis (n=127) or surgery (n=136). Early clinical evolution was assessed in terms of haemodynamic success and complications. Concerning early results, there were no significant differences between the two groups in terms of mortality (10%). However, haemodynamic success was significantly more frequent in the surgical group (89% versus 70.9% p<0.001), embolic episodes were significantly more frequent in the fibrinolysis group (15% versus 0.7%, p<0.001), as were total complications (25.2% versus 11.1%, p=0.005). Long-term follow-up, with a mean duration of 6 years (range: 0-20), was obtained and showed significantly better results in the surgical group in terms of recurrence (p=0.021) and mortality (p=0.002). In univariate and multivariable analyses, NYHA functional class at presentation was a strong predictor of late death (p<0.01). Management of patients during the pre- and post-TEE eras was significantly different, since introduction of TEE surgery has become the preferred therapeutic strategy. Results of this extensive single-centre experience indicate that since the introduction of TEE, surgery is more frequently performed than fibrinolysis due to the improvement of thromboembolic risk assessment. Furthermore, prompt surgical treatment is associated with a better early success rate and a significantly lower incidence of complications than fibrinolysis in left-sided PHVO. However, fibrinolysis may be justified in selected cases. Long-term follow-up showed significantly better results in the surgical group in terms of recurrence and mortality.