RESUMO
BACKGROUND: Coronary microvascular dysfunction (CMD) is present in various non-ischemic cardiomyopathies and in particular in those with left-ventricular hypertrophy. This study evaluated the diagnostic value of the novel cardiovascular magnetic resonance (CMR) parameter "myocardial transit-time" (MyoTT) in distinguishing cardiac amyloidosis from other hypertrophic cardiomyopathies. METHODS: N = 20 patients with biopsy-proven cardiac amyloidosis (CA), N = 20 patients with known hypertrophic cardiomyopathy (HCM), and N = 20 control patients without relevant cardiac disease underwent dedicated CMR studies on a 1.5-T MR scanner. The CMR protocol comprised cine and late-gadolinium-enhancement (LGE) imaging as well as first-pass perfusion acquisitions at rest for MyoTT measurement. MyoTT was defined as the blood circulation time from the orifice of the coronary arteries to the pooling in the coronary sinus (CS) reflecting the transit-time of gadolinium in the myocardial microvasculature. RESULTS: MyoTT was significantly prolonged in patients with CA compared to both groups: 14.8 ± 4.1 s in CA vs. 12.2 ± 2.5 s in HCM (p = 0.043) vs. 7.2 ± 2.6 s in controls (p < 0.001). Native T1 and extracellular volume (ECV) were significantly higher in CA compared to HCM and controls (p < 0.001). Both parameters were associated with a higher diagnostic accuracy in predicting the presence of CA compared to MyoTT: area under the curve (AUC) for native T1 = 0.93 (95% confidence interval (CI) = 0.83-1.00; p < 0.001) and AUC for ECV = 0.95 (95% CI = 0.88-1.00; p < 0.001)-compared to the AUC for MyoTT = 0.76 (95% CI = 0.60-0.92; p = 0.008). In contrast, MyoTT performed better than all other CMR parameters in differentiating HCM from controls (AUC for MyoTT = 0.93; 95% CI = 0.81-1.00; p = 0.003 vs. AUC for native T1 = 0.69; 95% CI = 0.44-0.93; p = 0.20 vs. AUC for ECV = 0.85; 95% CI = 0.66-1.00; p = 0.017). CONCLUSION: The relative severity of CMD (measured by MyoTT) in relationship to extracellular changes (measured by native T1 and/or ECV) is more pronounced in HCM compared to CA-in spite of a higher absolute MyoTT value in CA patients. Hence, MyoTT may improve our understanding of the interplay between extracellular/intracellular and intravasal changes that occur in the myocardium during the disease course of different cardiomyopathies.
Assuntos
Amiloidose/diagnóstico , Cardiomiopatia Hipertrófica/diagnóstico , Vasos Coronários/patologia , Imagem Cinética por Ressonância Magnética/métodos , Microvasos/patologia , Miocárdio/patologia , Função Ventricular Esquerda/fisiologia , Amiloidose/fisiopatologia , Biópsia , Cardiomiopatias/diagnóstico , Cardiomiopatias/fisiopatologia , Cardiomiopatia Hipertrófica/fisiopatologia , Circulação Coronária/fisiologia , Seguimentos , Valor Preditivo dos Testes , Estudos Prospectivos , Fatores de TempoRESUMO
The aims of this study were to investigate the main clinical and laboratory features, including pregnancy and genetic analysis, of Turkish Familial Mediterranean Fever (FMF) patients and to analyze the relationships between genotypic features, age of disease onset, clinical findings, and disease severity. A study was planned within a national network of 22 different centers. Demographics, clinical and laboratory findings, attack characteristics, drugs, pregnancy and birth history, disease severity, and gene mutation analyses were evaluated. Disease severity, assessed using a scoring system developed by Pras et al., was evaluated in relation to gene mutations and age of disease onset. A total of 979 patients (643 females and 336 males; mean age: 35.92 ± 11.97 years) with FMF were included in the study. Of a total of 585 pregnancies, 7% of them resulted in preterm birth and 18.1% resulted in abortions. During pregnancy, there was no FMF attack in 61.4% of patients. Of the MEditerranean FeVer (MEFV) mutations, 150 (24.3%) cases were homozygous, 292 (47.3%) cases were heterozygous, and 175 (28.4%) were compound heterozygous. Patients with homozygous gene mutations had more severe disease activity, earlier age of disease onset, higher rates of joint and skin involvement, sacroiliitis, and amyloidosis. Patients with compound heterozygous genotype displayed severe disease activity in close resemblance to patients with homozygous mutation. In addition, patients with compound heterozygous mutations had higher rates of protracted febrile myalgia and elevated fibrinogen levels. In 63.9% of compound heterozygous patients, age of onset was < 20 years, with greater disease severity, and high rates of attack frequency and colchicine resistance. Our results suggest that indicators for disease severity include early onset of disease and homozygous gene mutations. Furthermore, patients with compound heterozygous mutations displayed significant presentations of severe disease activity.
Assuntos
Amiloidose/fisiopatologia , Artralgia/fisiopatologia , Artrite/fisiopatologia , Febre Familiar do Mediterrâneo/fisiopatologia , Mialgia/fisiopatologia , Complicações na Gravidez/fisiopatologia , Sacroileíte/fisiopatologia , Dermatopatias/fisiopatologia , Dor Abdominal/fisiopatologia , Aborto Espontâneo/epidemiologia , Adulto , Idade de Início , Amiloidose/genética , Artralgia/genética , Artrite/genética , Dor no Peito/fisiopatologia , Estudos de Coortes , Colchicina/uso terapêutico , Resistência a Medicamentos , Febre Familiar do Mediterrâneo/tratamento farmacológico , Febre Familiar do Mediterrâneo/genética , Fadiga/fisiopatologia , Feminino , Heterozigoto , Homozigoto , Humanos , Masculino , Pessoa de Meia-Idade , Mutação , Mialgia/genética , Gravidez , Complicações na Gravidez/genética , Nascimento Prematuro/epidemiologia , Pirina/genética , Sacroileíte/genética , Índice de Gravidade de Doença , Dermatopatias/genética , Moduladores de Tubulina/uso terapêutico , Turquia/epidemiologia , Adulto JovemRESUMO
BACKGROUND: Given that in patients with cardiac amyloidosis (CA), deposition of amyloid protein is not restricted to the left ventricular (LV) myocardium, it can be hypothesized that the diagnostic value of deformation mechanics would be enhanced by considering right ventricular (RV) strain measures. The aim of the present study was to examine the potential utility of left ventricular (LV) and right ventricular (RV) deformation and rotational parameters derived from three-dimensional speckle-tracking echocardiograph (3DSTE) to diagnose cardiac amyloidosis and differentiate this disease from other forms of myocardial hypertrophy. METHODS: Twenty-three patients with biopsy-proven light-chain (AL) amyloidosis, 23 patients with systemic arterial hypertension (HTN), 23 patients with hypertrophic cardiomyopathy (HCM), 23 athletes and 23 normal controls were prospectively studied by conventional echocardiography and 3DSTE. LV longitudinal strain (LV LS), LV circumferential strain (LV CS), RV global longitudinal strain and RV free-wall longitudinal strain (RV FW LS) were obtained by 3DSTE, as well as LV rotation and rotational velocities. RESULTS: LV and RV longitudinal strains were reduced in cardiac amyloidosis (CA) patients compared to controls. By multivariate analysis, LV basal LS (pâ¯=â¯0.002), LV peak basal rotation (pâ¯=â¯0.003), and RV basal FW LS (pâ¯=â¯0.014) were independently associated with CA in the overall population. A significant improvement in global χ2 value was noted with RV 3D-strain parameters over only LV-3DSTEâ¯+â¯conventional indices for detection of CA (pâ¯<â¯0.001). Comparison of ROC curves showed that the AUC using combined LV basal LS, LV basal rotation and RV basal FW LS had a higher discriminative value than the other echocardiographic parameters used for detecting CA (AUC 0.93, 95%CI 0.81-0.97). CONCLUSIONS: Three-dimensional speckle tracking echocardiography reveals regional and global biventricular dysfunction in CA. Assessment of RV ventricular dysfunction has an additive value in differentiating CA from other causes of myocardial wall thickening.
Assuntos
Amiloidose/diagnóstico por imagem , Cardiomiopatia Hipertrófica/diagnóstico por imagem , Ecocardiografia Tridimensional/normas , Adulto , Idoso , Amiloidose/fisiopatologia , Cardiomiopatia Hipertrófica/fisiopatologia , Estudos Transversais , Ecocardiografia Tridimensional/métodos , Feminino , Humanos , Cadeias Leves de Imunoglobulina , Masculino , Pessoa de Meia-Idade , Estudos ProspectivosRESUMO
RATIONALE: Apolipoprotein A-1 (ApoA-1)-related amyloidosis is characterized by the deposition of ApoA-1 in various organs and can be either hereditary or nonhereditary. It is rare and easily misdiagnosed. Renal involvement is common in hereditary ApoA-1 amyloidosis, but rare in the nonhereditary form. PATIENT CONCERNS: We reported two cases with ApoA-1 amyloidosis, a 64-year-old man suffering from nephrotic syndrome and a 40-year-old man with nephrotic syndrome and splenomegaly. Renal biopsies revealed glomerular, interstitial and vascular amyloid deposits and positive phospholipase A2 receptor staining in the glomerular capillary loop in case 1, and mesangial amyloid deposits in case 2. DIAGNOSES: After immunostaining failed to determine the specific amyloid protein, proteomic analysis of amyloid deposits by mass spectrometry was performed and demonstrated the ApoA-1 origin of the amyloid. Genetic testing revealed no mutation of the APOA1 gene in case 1 but a heterozygous mutation, Trp74Arg, in case 2. Case 1 was thus diagnosed as nonhereditary ApoA-1 associated renal amyloidosis with membranous nephropathy, and case 2 as hereditary ApoA-1 amyloidosis with multiorgan injuries (kidney and spleen) and a positive family history. INTERVENTIONS: Case 1 was treated with glucocorticoid combined with cyclosporine. Case 2 was treated with calcitriol and angiotensin converting enzyme inhibitors. OUTCOMES: Two cases were followed up for 5 months and 2 years, respectively; and case 1 was found to have attenuated proteinuria while case 2 had an elevation of cholestasis indices along with renal insufficiency. LESSONS: Proteomic analysis by mass spectrometry of the amyloid deposits combined with genetic analysis can provide accurate diagnosis of ApoA-1 amyloidosis. Besides, these 2 cases expand our knowledge of ApoA-1-related renal amyloidosis.
Assuntos
Amiloidose Familiar , Amiloidose , Apolipoproteína A-I/metabolismo , Rim/patologia , Síndrome Nefrótica , Placa Amiloide , Esplenomegalia , Adulto , Amiloidose/diagnóstico , Amiloidose/metabolismo , Amiloidose/fisiopatologia , Amiloidose Familiar/diagnóstico , Amiloidose Familiar/metabolismo , Amiloidose Familiar/fisiopatologia , Inibidores da Enzima Conversora de Angiotensina/administração & dosagem , Calcitriol/administração & dosagem , Agonistas dos Canais de Cálcio/administração & dosagem , Ciclosporina/administração & dosagem , Diagnóstico Diferencial , Inibidores Enzimáticos/administração & dosagem , Glucocorticoides/administração & dosagem , Humanos , Imunossupressores/administração & dosagem , Masculino , Espectrometria de Massas/métodos , Conduta do Tratamento Medicamentoso , Pessoa de Meia-Idade , Síndrome Nefrótica/diagnóstico , Síndrome Nefrótica/etiologia , Seleção de Pacientes , Placa Amiloide/metabolismo , Placa Amiloide/patologia , Receptores da Fosfolipase A2/metabolismo , Esplenomegalia/diagnóstico , Esplenomegalia/etiologiaRESUMO
BACKGROUND: We explored the usefulness of myocardial strain analysis on cardiac magnetic resonance imaging (CMR) scans for the identification of cardiac amyloidosis.MethodsâandâResults:The 61 patients with systemic amyloidosis underwent 3.0-T CMR, including CMR tagging and late-gadolinium enhanced (LGE) imaging. The circumferential strain (CS) of LGE-positive and LGE-negative patients was measured on midventricular short-axis images and compared. Logistic regression modeling of CMR parameters was performed to detect patients with LGE-positive cardiac amyloidosis. Of the 61 patients with systemic amyloidosis 48 were LGE-positive and 13 were LGE-negative. The peak CS was significantly lower in the LGE-positive than in the LGE-negative patients (-9.5±2.3 vs. -13.3±1.4%, P<0.01). The variability in the peak CS time was significantly greater in the LGE-positive than in the LGE-negative patients (46.1±24.5 vs. 21.2±20.1 ms, P<0.01). The peak CS significantly correlated with clinical biomarkers. The sensitivity, specificity, and accuracy of the diagnostic model using CS parameters for the identification of LGE-positive amyloidosis were 93.8%, 76.9%, and 90.2%, respectively. CONCLUSIONS: Myocardial strain analysis by CMR helped detect LGE-positive amyloidosis without the need for contrast medium. The peak CS and variability in the peak CS time may correlate with the severity of cardiac amyloid deposition and may be more sensitive than LGE imaging for the detection of early cardiac disease in patients with amyloidosis.
Assuntos
Amiloidose/diagnóstico por imagem , Meios de Contraste/administração & dosagem , Gadolínio/administração & dosagem , Cardiopatias/diagnóstico por imagem , Imageamento por Ressonância Magnética , Miocárdio , Adulto , Idoso , Idoso de 80 Anos ou mais , Amiloidose/fisiopatologia , Feminino , Cardiopatias/fisiopatologia , Humanos , Masculino , Pessoa de Meia-Idade , Estudos RetrospectivosRESUMO
BACKGROUND: Relative apical sparing pattern of longitudinal strain (RapSP-LS) was suggested in advanced cardiac amyloidosis (CA). It is unclear whether it is present in less advanced CA. METHODS AND RESULTS: Patients with presumptive diagnosis of CA and mean left ventricular wall thickness (LVWT) ≤14 mm were recruited. Apart from RapSP-LS visually identified, relative apical longitudinal strain index (RapLSI) was defined as [average apical LS/(average basal LS+average mid-ventricle LS)]. Among 119 patients included, 47 were finally diagnosed with CA. RapLSI was higher in the CA group compared to other causes of increased mean LVWT (P<0.001), but with a significant range of overlap noted. In contrast, RapSP-LS visually assessed was noted in most CA patients (31/47, 66.0%) except in those with preserved LV ejection fraction, normal LVWT, and mildly decreased global LS, suggesting least advanced CA. On multivariate analysis of the added diagnostic role of RapSP-LS or RapLSI on top of clinical, electrocardiographic, and conventional echocardiographic parameters, addition of RapLSI produced only borderline increase in area under the curve of the multivariate model (P=0.05), whereas addition of RapSP-LS significantly increased it (P<0.001). CONCLUSIONS: Visual identification of RapSP-LS is useful in terms of added diagnostic value compared with quantitative calculation of RapLSI. Its clinical application, however, should be used with caution in patients with less advanced CA.
Assuntos
Amiloidose/diagnóstico por imagem , Cardiopatias/diagnóstico por imagem , Idoso , Amiloidose/fisiopatologia , Ecocardiografia/métodos , Feminino , Cardiopatias/fisiopatologia , Ventrículos do Coração/diagnóstico por imagem , Ventrículos do Coração/fisiopatologia , Humanos , Masculino , Pessoa de Meia-IdadeRESUMO
BACKGROUND: Differentiation of cardiac amyloidosis (CA) from other causes of concentric left ventricular hypertrophy remains a clinical challenge, especially in patients with preserved ejection fraction at the early disease stages. METHODS AND RESULTS: Consecutive hypertrophic patients with CA, isolated arterial hypertension, Fabry disease, and Friedreich ataxia (n=25 per group) were investigated; 25 healthy volunteers served as a control group. Standard echocardiography was performed, and segmental longitudinal peak systolic strain (LSsys) in the septum was assessed by 2-dimensional speckle tracking imaging. Indices of left ventricular hypertrophy and ejection fraction were similar among all patient groups. Deceleration time of early filling was significantly lower in patients with CA (147±46 milliseconds) compared with those with isolated arterial hypertension, Fabry disease, or control subjects (all P<0.0125). Septal basal LSsys (-6±2%) was significantly lower in patients with CA compared with those with isolated arterial hypertension (-14±6%), Fabry disease (-12±5%), Friedreich ataxia (-16±2%), or control subjects (-17±3%; all P<0.001), whereas septal apical LSsys was similar among all patient groups and control subjects (all P>0.05). A data-driven cutoff value for the ratio of septal apical to basal LSsys ratio >2.1 differentiated CA from other causes of left ventricular hypertrophy (sensitivity, 88%; specificity, 85%; positive predictive value, 67%; negative predictive value, 96%). The prevalence of septal apical to basal LSsys ratio >2.1 plus deceleration time of early filling <200 milliseconds was 88% in CA but 0% in all other groups. CONCLUSIONS: A systolic septal longitudinal base-to-apex strain gradient (septal apical to basal LSsys ratio >2.1), combined with a shortened diastolic deceleration time of early filling (deceleration time of early filling <200 milliseconds), aids in differentiating CA from other causes of concentric left ventricular hypertrophy.
Assuntos
Amiloidose/complicações , Cardiomiopatias/complicações , Hipertrofia Ventricular Esquerda/fisiopatologia , Função Ventricular Esquerda/fisiologia , Idoso , Amiloidose/diagnóstico por imagem , Amiloidose/fisiopatologia , Cardiomiopatias/diagnóstico por imagem , Cardiomiopatias/fisiopatologia , Diagnóstico Diferencial , Diástole , Ecocardiografia , Eletrocardiografia , Feminino , Seguimentos , Humanos , Hipertrofia Ventricular Esquerda/diagnóstico por imagem , Hipertrofia Ventricular Esquerda/etiologia , Masculino , Pessoa de Meia-Idade , Curva ROC , SístoleRESUMO
Cardiac MRI is known to be diagnostically valuable in cardiac amyloidosis. Several features are frequently found, including myocardial hypertrophy, diastolic dysfunction, a faster gadolinium blood wash out, pleural and pericardial effusions, and diffuse myocardial delayed enhancement. Cardiac MR facilitates the detection of cardiac amyloidosis and allows longitudinal assessment of myocardial function. This pictorial review focuses on cases with histologically proven systemic amyloidosis and cardiac involvement in order to illustrate typical findings.
Assuntos
Amiloidose/diagnóstico , Cardiomiopatias/diagnóstico , Hipertrofia Ventricular Esquerda/diagnóstico , Idoso , Amiloidose/fisiopatologia , Cardiomiopatias/fisiopatologia , Meios de Contraste , Feminino , Gadolínio , Humanos , Hipertrofia Ventricular Esquerda/fisiopatologia , Aumento da Imagem , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Reprodutibilidade dos TestesRESUMO
A growing body of literature has demonstrated that chronic pain is common for patients with end- stage renal disease (ESRD), is typically moderate or severe, and impacts virtually every aspect of health-related quality of life. Unfortunately, there is a lack of clinical and research focus in this area in nephrology and pain in ESRD is undertreated. This paper reviews the epidemiology of chronic pain in ESRD, discusses basic principles of pain assessment and management, and highlights some of the challenges in pain management in ESRD with the hope of guiding health professionals in the effective management of pain in patients with ESRD.
Assuntos
Falência Renal Crônica/fisiopatologia , Dor/tratamento farmacológico , Dor/epidemiologia , Alberta/epidemiologia , Amiloidose/fisiopatologia , Analgésicos Opioides/farmacocinética , Analgésicos Opioides/uso terapêutico , Calciofilaxia/fisiopatologia , Doença Crônica , Distúrbio Mineral e Ósseo na Doença Renal Crônica/fisiopatologia , Fibrose/fisiopatologia , Nível de Saúde , Humanos , Pessoa de Meia-Idade , Dor/etiologia , Qualidade de VidaRESUMO
OBJECTIVE: The aim of this study was to evaluate the incremental diagnostic role of tissue Doppler in primary cardiac amyloidosis (CA). METHODS: Eleven patients with CA at diagnosis and 11 healthy controls, matched for sex and age, underwent standard Doppler echocardiography and pulsed tissue Doppler of the left ventricular (LV) lateral annulus, in the apical four-chamber view. The ratio of early transmitral flow velocity to early diastolic mitral annular velocity (E/E(m) ratio) was derived as an index of LV filling pressure. RESULTS: The two groups were comparable for body mass index, blood pressure, heart rate and standard Doppler diastolic measurements. Patients with CA had a significantly higher sum of wall thickness (SWT) and LV mass, a lower E(m) peak velocity (P < 0.002) and a higher E/E(m) ratio (P < 0.001) than controls. By dividing CA patients according to the transmitral E/A ratio, patients with an E/A ratio < 1 (abnormal relaxation) (n = 5) and patients with an E/A ratio > 1 (likely pseudonormal/restrictive pattern) (n = 6) did not show any difference in the E/E(m) ratio (14.5 + or - 7.1 vs. 15.1 + or - 6.4, P = NS). In the overall population, the E/E(m) ratio was related to SWT (r = 0.84, P < 0.0001) and LV mass index (r = 0.72, P < 0.0001). After adjusting for age and heart rate by separate multivariate models, SWT (beta = 0.78, P < 0.0001; cumulative r(2) = 0.63, SE = 3.38, P < 0.0001) and LV mass index (beta = 0.71, P < 0.0001; cumulative r(2) = 0.53, SE = 3.80, P < 0.002) were both independently associated with the E/E(m) ratio. CONCLUSIONS: Pulsed tissue Doppler is able to detect early myocardial diastolic impairment in CA. The E/E(m) ratio is very useful in diagnosing increased LV filling pressure, regardless of the transmitral pattern, and may, therefore, be helpful in the clinical management of these patients.
Assuntos
Amiloidose/diagnóstico por imagem , Ecocardiografia Doppler de Pulso , Cardiopatias/diagnóstico por imagem , Volume Sistólico , Disfunção Ventricular Esquerda/diagnóstico por imagem , Idoso , Amiloidose/fisiopatologia , Análise de Variância , Estudos de Coortes , Ecocardiografia Doppler em Cores , Feminino , Cardiopatias/fisiopatologia , Humanos , Modelos Lineares , Masculino , Pessoa de Meia-Idade , Método de Monte Carlo , Probabilidade , Sensibilidade e Especificidade , Disfunção Ventricular Esquerda/fisiopatologiaRESUMO
A new memory model is proposed based on regression analysis and exponential- shaped learning curves. The efficacy of the model is tested with several types of experiments including food aversion in snails, maze learning in rats and memory tests for adults and children. The model is also tested on drug abusers and alcoholics. The results of goodness of fit tests indicate that our model can accurately be used to predict the memory dynamics of diverse experiments and populations. The model can also be used to predict both group and individual performance. The application of the model to detect memory impairment is discussed, as are limitations.
Assuntos
Aprendizagem , Memória , Modelos Teóricos , Adulto , Alcoolismo/fisiopatologia , Peptídeos beta-Amiloides/farmacologia , Amiloidose/fisiopatologia , Animais , Aprendizagem por Associação/fisiologia , Piscadela/fisiologia , Encéfalo/efeitos dos fármacos , Encéfalo/fisiopatologia , Criança , Pré-Escolar , Condicionamento Clássico/fisiologia , Condicionamento Operante/fisiologia , Sensibilidades de Contraste , Medo , Feminino , Humanos , Aprendizagem/fisiologia , Masculino , Aprendizagem em Labirinto/fisiologia , Memória/fisiologia , Pessoa de Meia-Idade , Motivação , Reconhecimento Visual de Modelos/fisiologia , Ratos , Ratos Wistar , Semântica , Aprendizagem Seriada/fisiologia , Transtornos Relacionados ao Uso de Substâncias/fisiopatologia , Aprendizagem Verbal/fisiologiaRESUMO
The severity of left ventricular involvement may differ between primary (PA) and familial amyloidosis (FA). This study examined whether differences in left atrial (LA) systolic function are also present. Twenty-eight patients (18 men, 10 women, aged 59 +/- 12 years) with PA, 17 (11 men, 6 women, aged 40 +/- 11 years) with FA, and 25 normal controls (18 men, 7 women, aged 56 +/- 14 years) underwent transthoracic M-mode, two-dimensional, and Doppler echocardiography. Left atrial volumes were determined at mitral valve (MV) opening (maximal, Vmax), electrocardiographic P wave (onset of atrial systole, Vp), and MV closure (minimal, Vmin) from the apical two-and four-chamber views using the biplane area-length method. Left atrial systolic function was assessed with the LA active emptying volume (ACTEV) = Vp-Vmin and fraction (ACTEF) = ACTEV/Vp. The E/A ratio was increased (1.34 +/- 0.93 vs. 0.89 +/- 0.3), whereas deceleration time was decreased (168.1 +/- 33.7 vs. 196.2 +/- 34.2 ms) in PA compared with FA (p<0.05). Vmax and Vp were similar in PA and FA and greater than in the controls (46.6 +/- 14 vs. 40 +/- 11.4 vs. 27.1 +/- 6.3 cm3/m2, p<0.01, and 33.4 +/- 11.6 vs. 29.7 +/- 10.8 vs. 16.8 +/- 3.8 cm3/m2, p<0.01, respectively). The ACTEV was lower in PA and in the controls than in FA (6.7 +/- 2 vs. 6.2 +/- 2.2 vs. 8.5 +/- 3.3, respectively, p<0.05). The ACTEF was lower in PA than in FA and both were lower than those in the controls (20 +/- 5% vs. 28 +/- 7% vs. 36 +/- 11%, respectively, p<0.01). Despite a similar increase in LA volume, LA systolic dysfunction is more pronounced in PA than in FA. This is most likely due to the restrictive left ventricular physiology possibly associated with depressed LA contractility in the former.
Assuntos
Amiloidose Familiar/fisiopatologia , Amiloidose/fisiopatologia , Função do Átrio Esquerdo , Adulto , Estudos de Casos e Controles , Ecocardiografia , Eletrocardiografia , Feminino , Humanos , Masculino , Pessoa de Meia-IdadeRESUMO
Autonomic neuropathy is a well-known and prognostically important feature of systemic amyloidosis. In other conditions, autonomic function is commonly assessed by cardiovascular reflex tests, described by Ewing, but the feasibility of these tests has not been investigated in patients with systemic amyloidosis. We studied autonomic function in amyloidotic patients using cardiovascular tests and assessed their feasibility. Patients with AA, AL and ATTR amyloidosis participated. In all patients, cardiovascular reflex testing (mental arithmetic stress test and head-up tilting, besides the Ewing-tests) was performed. Of the 46 patients included, only 28 patients could perform all 4 Ewing-tests. In particular, patients with AA amyloidosis secondary to rheumatoid arthritis could not perform standing up and the isometric handgrip test. However, when the mental stress test replaced the handgrip test and head-up tilting replaced standing up, in 45 of the 46 patients, autonomic function could be assessed with cardiovascular reflex tests. Half of the patients with AA amyloidosis had signs of autonomic neuropathy--which was more than expected. We propose to replace the isometric handgrip test with the mental arithmetic stress test and standing up with head-up tilting if a patient is not able to perform these tests.
Assuntos
Amiloidose/fisiopatologia , Sistema Nervoso Autônomo/fisiopatologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Ecocardiografia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Miocárdio/patologia , Exame NeurológicoAssuntos
Falência Renal Crônica/terapia , Diálise Peritoneal Ambulatorial Contínua/tendências , Diálise Renal/tendências , Uremia/terapia , Amiloidose/fisiopatologia , Amiloidose/prevenção & controle , Humanos , Falência Renal Crônica/fisiopatologia , Pessoa de Meia-Idade , Morbidade , Diálise Peritoneal Ambulatorial Contínua/efeitos adversos , Diálise Peritoneal Ambulatorial Contínua/economia , Diálise Renal/efeitos adversos , Diálise Renal/economiaRESUMO
The severity of cardiac infiltration in AL amyloidosis is unrelated to whole body amyloid load as measured by serum amyloid P (SAP) tracer studies. Radiolabeled SAP and echocardiography permit identification of patients with severe cardiac disease with a low whole body load who may be the best candidates for transplantation.
Assuntos
Amiloide/metabolismo , Amiloidose/fisiopatologia , Cardiomiopatias/fisiopatologia , Ecocardiografia Doppler , Componente Amiloide P Sérico/farmacocinética , Adulto , Idoso , Cardiomiopatias/patologia , Feminino , Ventrículos do Coração/patologia , Humanos , Radioisótopos do Iodo , Masculino , Pessoa de Meia-Idade , Fatores de Tempo , Função Ventricular Esquerda/efeitos dos fármacosRESUMO
Doppler echocardiography is useful in assessing diastolic dysfunction. Pulsed-wave Doppler echocardiographic interrogation of the atrioventricular valves and the central veins has been used in conjunction with respiratory monitoring to characterize abnormal diastolic function of the heart in diseases such as constrictive pericarditis, restrictive cardiomyopathy, and cardiac tamponade. Constrictive pericarditis has distinct Doppler flows with marked respiratory variation because the pericardial shell separates the intracardiac pressures from intrathoracic pressure. This is not the case with restrictive cardiomyopathy, as in cardiac amyloidosis. Cardiac amyloidosis shows a spectrum of the Doppler flows which evolve as the disease progresses from abnormal relaxation in the early phase to pseudo-normal in the intermediate phase and to restrictive in the advanced phase. Doppler echocardiography can be used to assess the hemodynamic significance of pericardial effusion and to detect cardiac tamponade.
Assuntos
Amiloidose/fisiopatologia , Tamponamento Cardíaco/fisiopatologia , Ecocardiografia Doppler/métodos , Cardiopatias/fisiopatologia , Pericardite Constritiva/fisiopatologia , Amiloidose/diagnóstico por imagem , Velocidade do Fluxo Sanguíneo , Tamponamento Cardíaco/diagnóstico por imagem , Diagnóstico Diferencial , Esôfago , Seguimentos , Cardiopatias/diagnóstico por imagem , Hemodinâmica , Humanos , Pessoa de Meia-Idade , Pericardite Constritiva/diagnóstico por imagem , Prognóstico , Tórax , Função Ventricular Esquerda , Função Ventricular DireitaAssuntos
Amiloidose/diagnóstico , Cardiomiopatias/diagnóstico , Adulto , Idoso , Amiloidose/diagnóstico por imagem , Amiloidose/fisiopatologia , Cardiomiopatias/diagnóstico por imagem , Cardiomiopatias/fisiopatologia , Ecocardiografia , Eletrocardiografia , Feminino , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Cintilografia , Sensibilidade e Especificidade , Tomografia Computadorizada por Raios XAssuntos
Amiloidose/fisiopatologia , Sistema Nervoso Autônomo/fisiopatologia , Frequência Cardíaca , Doenças do Sistema Nervoso Periférico/fisiopatologia , Amiloidose/complicações , Amiloidose/genética , Eletrocardiografia , Humanos , Pessoa de Meia-Idade , Doenças do Sistema Nervoso Periférico/complicações , Doenças do Sistema Nervoso Periférico/genéticaRESUMO
The hallmark of cardiac amyloidosis is abnormal diastolic function secondary to amyloid infiltration of the ventricular walls, which accounts for the term "stiff heart syndrome." The abnormal diastolic function has not yet been well characterized, however. Thus, we assessed left and right ventricular diastolic function in 53 patients with cardiac amyloidosis. We measured the left and right ventricular inflow and venous flow velocities with Doppler echocardiography and found a range of Doppler filling abnormalities. These abnormalities were dependent on the degree of amyloid infiltration of the heart as measured by mean left ventricular wall thickness. Patients with advanced cardiac amyloidosis (a wall thickness greater than or equal to 15 mm) showed restriction, while patients with early cardiac amyloidosis (a wall thickness less than 15 mm) showed abnormal relaxation or normal filling. In another study, during a 13-month follow-up, patients with early cardiac amyloidosis showed Doppler patterns that evolved from abnormal relaxation through a normal stage, to an advanced stage of restrictive disease. We also have demonstrated the importance of left ventricular inflow variables in predicting the outcome of patients with cardiac amyloidosis. We concluded that Doppler echocardiography is useful in characterizing abnormal diastolic function in patients with cardiac amyloidosis.
Assuntos
Amiloidose/fisiopatologia , Cardiomiopatias/fisiopatologia , Ecocardiografia Doppler/métodos , Função Ventricular/fisiologia , Amiloidose/diagnóstico por imagem , Velocidade do Fluxo Sanguíneo , Cardiomiopatias/diagnóstico por imagem , Diástole/fisiologia , Seguimentos , Humanos , PrognósticoRESUMO
This study was performed to evaluate hypothalamic-pituitary hormone regulation in patients with familial amyloidotic polyneuropathy. Twenty-two patients without clinically overt endocrinological dysfunction were studied. A thyrotropin-releasing hormone test revealed abnormal growth hormone regulation in 9 of 17 (53%) patients, and abnormal prolactin regulation in 9 of 18 (50%) patients. Abnormalities in either growth hormone or prolactin regulation were found in 12 of 17 (71%) patients. Serum somatomedin C levels were normal in all 22 patients. In 3 of 18 (17%) patients the plasma arginine vasopressin levels were low relative to the serum osmolality levels. Thus abnormalities in hypothalamic-pituitary hormone regulation may be common in familial amyloidotic polyneuropathy.