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2.
ESC Heart Fail ; 11(2): 859-870, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38204216

RESUMO

AIMS: In patients with recently diagnosed non-ischaemic LV systolic dysfunction, left ventricular reverse remodelling (LVRR) and favourable prognosis has been documented in studies with short-term follow-up. The aim of our study was to assess the long-term clinical course and stability of LVRR in these patients. METHODS AND RESULTS: We prospectively studied 133 patients (37 women; 55 [interquartile range 46, 61] years) with recently diagnosed unexplained LV systolic dysfunction, with heart failure symptoms lasting <6 months and LV ejection fraction <40% persisting after at least 1 week of therapy. All patients underwent endomyocardial biopsy (EMB) at the time of diagnosis and serial echocardiographic and clinical follow-up over 5 years. LVRR was defined as the combined presence of (1) LVEF ≥ 50% or increase in LVEF ≥ 10% points and (2) decrease in LV end-diastolic diameter index (LVEDDi) ≥ 10% or (3) LVEDDi ≤ 33 mm/m2. LVRR was observed in 46% patients at 1 year, in 60% at 2 years and 50% at 5 years. Additionally, 2% of patients underwent heart transplantation and 12% experienced heart failure hospitalization. During 5-year follow-up, 23 (17%) of the study cohort died. In multivariate analysis, independent predictors of mortality were baseline right atrial size (OR 1.097, CI 1.007-1.196), logBNP level (OR 2.02, CI 1.14-3.56), and PR interval (OR 1.02, CI 1.006-1.035) (P < 0.05 for all). The number of macrophages on EMB was associated with overall survival in univariate analysis only. LVRR at 1 year of follow-up was associated with a lower rate of mortality and heart failure hospitalization (P = 0.025). In multivariate analysis, independent predictors of LVRR were left ventricular end-diastolic volume index (OR 0.97, CI 0.946-0.988), LVEF (OR 0.89, CI 0.83-0.96), and diastolic blood pressure (OR 1.04, CI 1.01-1.08) (P < 0.05 for all). CONCLUSIONS: LVRR occurs in over half of patients with recent onset unexplained LV systolic dysfunction during first 2 years of optimally guided heart failure therapy and then remains relatively stable during 5-year follow-up. Normalization of adverse LV remodelling corresponds to a low rate of mortality and heart failure hospitalizations during long-term follow-up.


Assuntos
Cardiomiopatia Dilatada , Insuficiência Cardíaca , Disfunção Ventricular Esquerda , Humanos , Feminino , Função Ventricular Esquerda/fisiologia , Disfunção Ventricular Esquerda/complicações , Prognóstico
3.
Eur Heart J Cardiovasc Imaging ; 24(12): 1629-1637, 2023 11 23.
Artigo em Inglês | MEDLINE | ID: mdl-37309820

RESUMO

AIMS: Fabry disease (FD) is a multisystemic lysosomal storage disorder caused by a defect in the alpha-galactosidase A gene that manifests as a phenocopy of hypertrophic cardiomyopathy. We assessed the echocardiographic 3D left ventricular (LV) strain of patients with FD in relation to heart failure severity using natriuretic peptides, the presence of a cardiovascular magnetic resonance (CMR) late gadolinium enhancement scar, and long-term prognosis. METHODS AND RESULTS: 3D echocardiography was feasible in 75/99 patients with FD [aged 47 ± 14 years, 44% males, LV ejection fraction (EF) 65 ± 6% and 51% with hypertrophy or concentric remodelling of the LV]. Long-term prognosis (death, heart failure decompensation, or cardiovascular hospitalization) was assessed over a median follow-up of 3.1 years. A stronger correlation was observed for N-terminal pro-brain natriuretic peptide levels with 3D LV global longitudinal strain (GLS, r = -0.49, P < 0.0001) than with 3D LV global circumferential strain (GCS, r = -0.38, P < 0.001) or 3D LVEF (r = -0.25, P = 0.036). Individuals with posterolateral scar on CMR had lower posterolateral 3D circumferential strain (CS; P = 0.009). 3D LV-GLS was associated with long-term prognosis [adjusted hazard ratio 0.85 (confidence interval 0.75-0.95), P = 0.004], while 3D LV-GCS and 3D LVEF were not (P = 0.284 and P = 0.324). CONCLUSION: 3D LV-GLS is associated with both heart failure severity measured by natriuretic peptide levels and long-term prognosis. Decreased posterolateral 3D CS reflects typical posterolateral scarring in FD. Where feasible, 3D-strain echocardiography can be used for a comprehensive mechanical assessment of the LV in patients with FD.


Assuntos
Ecocardiografia Tridimensional , Doença de Fabry , Insuficiência Cardíaca , Disfunção Ventricular Esquerda , Masculino , Humanos , Feminino , Cicatriz/diagnóstico por imagem , Doença de Fabry/complicações , Doença de Fabry/diagnóstico por imagem , Meios de Contraste , Reprodutibilidade dos Testes , Gadolínio , Insuficiência Cardíaca/diagnóstico por imagem , Insuficiência Cardíaca/etiologia , Função Ventricular Esquerda , Ecocardiografia Tridimensional/métodos , Volume Sistólico , Ecocardiografia/métodos , Prognóstico , Disfunção Ventricular Esquerda/diagnóstico por imagem , Disfunção Ventricular Esquerda/etiologia
5.
World J Pediatr Congenit Heart Surg ; 14(2): 161-167, 2023 03.
Artigo em Inglês | MEDLINE | ID: mdl-36529977

RESUMO

BACKGROUND: Secondary prevention of recurrent rheumatic fever in individuals with rheumatic heart disease (RHD) requires continuous antibiotic prophylaxis. However, the impact of antibiotic prophylaxis on the outcome of patients with severe RHD who underwent heart valve replacement is unknown. The objective of the study was to assess the relationship between the use of antibiotics as secondary prophylaxis in RHD patients who underwent valve replacement and clinical outcomes including mortality, reoperation, and valve-related hospitalization. METHODS: We retrospectively compared outcomes of adult patients who underwent heart valve replacement for RHD at our institution from 1990 through 2014 and who received secondary antibiotic prophylaxis (prophylaxis group) with those who did not receive prophylaxis (nonprophylaxis group) using propensity score matching analysis. RESULTS: A total of 1094 patients (56% females, median age 40 years, range 31-53 years) were included with a median follow-up of 9.6 years (range 2.9-12.6 years). Antibiotic prophylaxis was prescribed in 201 patients (18%). Propensity score matching analysis demonstrated no significant difference in overall survival (95% [92%-98%] vs 97% [95%-99%], respectively; P = .7), valve-related hospitalization-independent survival (72% [range 65%-78%] vs 81% [range 76%-88%]; P = .25), and redo valve surgery-independent survival [76% [range 70%-83%] vs 75% [range 72%-79%]; P = .41) at 10-year follow-up in the nonantibiotic prophylaxis versus the antibiotic prophylaxis group. CONCLUSION: Secondary antibiotic prophylaxis among adult RHD patients following valve replacement is not associated with improved clinical outcomes.


Assuntos
Cardiopatia Reumática , Feminino , Humanos , Adulto , Pessoa de Meia-Idade , Masculino , Cardiopatia Reumática/cirurgia , Antibacterianos/uso terapêutico , Antibioticoprofilaxia , Estudos Retrospectivos , Prevenção Secundária
6.
ESC Heart Fail ; 9(6): 4043-4052, 2022 12.
Artigo em Inglês | MEDLINE | ID: mdl-36036737

RESUMO

AIMS: Fabry disease (FD) is often associated with heart failure (HF). However, data on HF prevalence, prognosis, and applicability of echocardiographic criteria for HF diagnosis in FD remain uncertain. METHODS AND RESULTS: We evaluated patients with genetically proven FD for symptoms and natriuretic peptides indicating HF. We then analysed the diagnostic utility of the currently recommended European Society of Cardiology (ESC) echocardiographic criteria for HF diagnosis and their relationship to natriuretic peptides. Finally, we examined the association between HF and echocardiographic criteria with mortality and cardiovascular events during follow-up. Of 116 patients with FD, 48 (41%) had symptomatic HF (mean age 58 ± 11 years, 62% male). HF with preserved ejection fraction (HF-pEF) was diagnosed in 43 (91%) patients, representing the dominant phenotype. Left ventricular mass index (LVMi) had the highest diagnostic utility (sensitivity 71% and specificity 83%) for HF diagnosis in FD, followed by E/e' > 9 (sensitivity 76% and specificity 78%) and global longitudinal strain (GLS) <16% (sensitivity 54% and specificity 88%). Log N-terminal pro-brain natriuretic peptide correlated significantly with LVMi (r = 0.60), E/e' (r = 0.54), and GLS (r = 0.52) (all Ps < 0.001) but not with left ventricular ejection fraction (r = -0.034, P = 0.72). During follow-up (mean 1208 ± 444 days), patients diagnosed with HF had a higher rate of all-cause mortality and worsening HF (33% vs. 1.5%, P < 0.001). Abnormal LVMi, E/e' > 9, and GLS < 16% were all associated with higher all-cause mortality and worsening HF. CONCLUSIONS: This study found a high prevalence of symptomatic HF in FD patients. HF-pEF was the dominant phenotype. LVMi, E/e', and GLS yielded the highest diagnostic utility for HF diagnosis and were significantly correlated with natriuretic peptides levels. Echocardiographic criteria proposed by current ESC HF guidelines apply to Fabry patients and predict cardiovascular events. At follow-up, Fabry patients with HF diagnosis had high event rates and significantly worse prognosis than patients without HF.


Assuntos
Doença de Fabry , Insuficiência Cardíaca , Feminino , Humanos , Masculino , Doença de Fabry/complicações , Doença de Fabry/diagnóstico , Doença de Fabry/epidemiologia , Insuficiência Cardíaca/diagnóstico , Insuficiência Cardíaca/epidemiologia , Insuficiência Cardíaca/etiologia , Peptídeos Natriuréticos , Volume Sistólico , Função Ventricular Esquerda , Pessoa de Meia-Idade , Idoso
7.
Vnitr Lek ; 67(E-3): 11-14, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34171946

RESUMO

GDF-15 (Growth differentiation factor 15) is a protein synthesised in some tissues including liver, kidney, heart, or lung. GDF-15 a stress-responsive cytokine. GDF-15 is emerging as a biomarker of cardiometabolic risk and disease burden. GDF-15 is linked to the incidence and prognosis of heart failure. In acute coronary syndromes GDF-15 identifies risk of complications including bleeding, reinfarction, development of heart failure or mortality. In patients with atrial fibrillation, GDF-15 is a potent marker of bleeding adverse events in anticoagulated patients and a predictor of overall mortality. Role of GDF-15 in cardiology is not definitively constituted.


Assuntos
Fibrilação Atrial , Cardiologia , Insuficiência Cardíaca , Biomarcadores , Fator 15 de Diferenciação de Crescimento , Humanos , Prognóstico , Fatores de Risco
8.
Am J Cardiol ; 150: 110-113, 2021 07 01.
Artigo em Inglês | MEDLINE | ID: mdl-34011439

RESUMO

Fabry disease (FD) is an X-linked linked genetic disorder caused by α-galactosidase A deficiency. The typical clinical manifestation is left ventricular hypertrophy, often mimicking hypertrophic cardiomyopathy (HC). In contrast to sarcomeric HC, left ventricular outflow tract obstruction (LVOTO) is less frequent. We describe 6 male patients with genetically confirmed FD and symptomatic LVOTO. All of them underwent a transcatheter alcohol septal ablation with an immediate effect on the obstruction in all cases and without any serious complications. The median LVOT maximal pressure gradient was 85 (60 to 170) mm Hg. The hemodynamic effect persisted during subsequent follow-up (ranging from 6 months to 16 years). Five patients reported substantial symptomatic improvement. Four patients were receiving specific FD therapy before the interventional procedure. In conclusion, alcohol septal ablation appears to be effective in the treatment of LVOTO in patients with FD and appears to be comparable to the limited published experience with surgical septal myectomy. Despite some important differences between FD HC and sarcomeric HC, the recommendation for treating LVOTO should be similar.


Assuntos
Técnicas de Ablação/métodos , Etanol/uso terapêutico , Doença de Fabry/complicações , Obstrução do Fluxo Ventricular Externo/etiologia , Obstrução do Fluxo Ventricular Externo/terapia , Adulto , Ecocardiografia , Eletrocardiografia , Doença de Fabry/tratamento farmacológico , Humanos , Masculino , Pessoa de Meia-Idade , Obstrução do Fluxo Ventricular Externo/diagnóstico por imagem
9.
J Saudi Heart Assoc ; 33(4): 279-283, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-35083117

RESUMO

OBJECTIVE: Cardiac allograft vasculopathy is one of the leading causes of late graft failure and subsequent death in orthotopic heart transplant. Although invasive coronary angiography is the gold standard modality for detection of cardiac allograft vasculopathy, dobutamine stress echocardiography has been recently frequently used as an alternative. Our aim was to evaluate the diagnostic performance of dobutamine stress echocardiography for detection of cardiac allograft vasculopathy in transplant patients. METHODS: A retrospective analysis was conducted using a total of 150 dobutamine stress echocardiographic exams that were performed on 99 patients in our institution, with paired coronary angiogram and no acute rejection, within a median of 538 [interquartile range 371-816] days. Sensitivity and specificity of dobutamine echocardiography to detect allograft vasculopathy was evaluated. Allograft vasculopathy was defined as Grade 1 or higher based on ISHLT criteria. A positive dobutamine stress echo result was defined by new or worsening wall motion abnormality. RESULTS: Median age of the population at transplant was 34 [interquartile range 22-46] years; 76 (77%) patients were male. Allograft vasculopathy was present in 31 (20.6%) out of 150 coronary angiograms. Only 7 (4.6%) of that number were positive on dobutamine stress echocardiography. Sensitivity and specificity for allograft vasculopathy detection was 3% and 94%, respectively. Out of 7 false positive dobutamine stress echocardiograms, two were in patients with myocardial bridging. Two patients with mild acute rejection had both negative dobutamine stress echo. CONCLUSIONS: Overall, positivity of dobutamine stress echocardiography in patients after heart transplant is low. It has high specificity, but very low sensitivity for detection of cardiac allograft vasculopathy. Dobutamine stress echocardiography should only be cautiously used as an alternative to coronary angiography.

10.
Cardiovasc Pathol ; 50: 107297, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33045360

RESUMO

Mucopolysaccharidosis type VII (MPS VII) is a rare autosomal recessive lysosomal storage disorder. MPS VII is caused by mutations in the GUSB gene that encodes ß-glucuronidase. Adult MPS VII patients present with musculoskeletal abnormalities, coarse features, and corneal clouding. Cardiac and valvular impairment are common; however, severe valvular disease necessitating surgery has not yet been reported. We present a 32-year-old male MPS VII patient admitted to our hospital with decompensated heart failure. We identified aortic valve disease with severe stenosis (valve area 0.69 cm2) and moderate regurgitation. Severe mitral valve stenosis (valve area 1 cm2) with moderate to severe regurgitation was also found in the patient. In addition, an occlusion of the right coronary artery (RCA) was documented. The patient underwent surgical replacement of the mitral and aortic valves with mechanical prostheses and implantation of a venous bypass graft to his RCA. The surgery led to a significant improvement of his clinical symptoms. Six months after the procedure, both mechanical valves function normally. Histopathological assessment identified chronic inflammatory infiltrates, fibrosis and calcifications in both resected valves. Foamy cytoplasmic transformation was most evident in the valvular interstitial cells. The ultrastructural vacuolar abnormality seen in these cells corresponded to storage changes observed in other MPSs. In conclusion, we describe clinical findings and valvular pathology in an MPS VII patient with the first-reported successful combined surgical valve replacement and myocardial revascularization. The histological and ultrastructural analyses revealed that the lysosomal storage predominantly affected the valvular interstitial cells.


Assuntos
Insuficiência da Valva Aórtica/cirurgia , Estenose da Valva Aórtica/cirurgia , Ponte de Artéria Coronária , Oclusão Coronária/cirurgia , Implante de Prótese de Valva Cardíaca , Insuficiência da Valva Mitral/cirurgia , Estenose da Valva Mitral/cirurgia , Mucopolissacaridose VII/complicações , Adulto , Insuficiência da Valva Aórtica/diagnóstico por imagem , Insuficiência da Valva Aórtica/etiologia , Estenose da Valva Aórtica/diagnóstico por imagem , Estenose da Valva Aórtica/etiologia , Oclusão Coronária/diagnóstico por imagem , Oclusão Coronária/etiologia , Humanos , Masculino , Insuficiência da Valva Mitral/diagnóstico por imagem , Insuficiência da Valva Mitral/etiologia , Estenose da Valva Mitral/diagnóstico por imagem , Estenose da Valva Mitral/etiologia , Mucopolissacaridose VII/diagnóstico , Índice de Gravidade de Doença , Resultado do Tratamento
11.
Diabetes Metab Syndr Obes ; 12: 1459-1471, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31695455

RESUMO

PURPOSE: Cushing's syndrome is characterized by metabolic disturbances including insulin resistance. Mitochondrial dysfunction is one pathogenic factor in the development of insulin resistance in patients with obesity. We explored whether mitochondrial dysfunction correlates with insulin resistance and other metabolic complications. PATIENTS AND METHODS: We investigated the changes of mRNA expression of genes encoding selected subunits of oxidative phosphorylation system (OXPHOS), pyruvate dehydrogenase (PDH) and citrate synthase (CS) in subcutaneous adipose tissue (SCAT) and peripheral monocytes (PM) and mitochondrial enzyme activity in platelets of 24 patients with active Cushing's syndrome and in 9 of them after successful treatment and 22 healthy control subjects. RESULTS: Patients with active Cushing's syndrome had significantly increased body mass index (BMI), homeostasis model assessment of insulin resistance (HOMA-IR) and serum lipids relative to the control group. The expression of all investigated genes for selected mitochondrial proteins was decreased in SCAT in patients with active Cushing's syndrome and remained decreased after successful treatment. The expression of most tested genes in SCAT correlated inversely with BMI and HOMA-IR. The expression of genes encoding selected OXPHOS subunits and CS was increased in PM in patients with active Cushing's syndrome with a tendency to decrease toward normal levels after cure. Patients with active Cushing's syndrome showed increased enzyme activity of complex I (NQR) in platelets. CONCLUSION: Mitochondrial function in SCAT in patients with Cushing's syndrome is impaired and only slightly affected by its treatment which may reflect ongoing metabolic disturbances even after successful treatment of Cushing's syndrome.

12.
Cardiovasc Ultrasound ; 17(1): 22, 2019 Oct 30.
Artigo em Inglês | MEDLINE | ID: mdl-31666068

RESUMO

BACKGROUND: Normative reference values for echocardiographic chamber quantification are of great importance; however, this can be challenging. Our aim was to derive these values including degrees of abnormality from a random Central European population sample with a homogeneous subset of healthy subjects. METHODS: We analysed echocardiograms obtained in a randomly selected population sample during the Czech post-MONICA survey in 2007/2008. Overall, 1850 out of 2273 persons of the whole sample of three districts had adequate echocardiograms (81.4%). A healthy subgroup defined by the absence of known cardiovascular disease was used to define normal reference range limits (n = 575, median age 42 years [IQR 34-52], 57% females). The whole population sample with predefined percentile cut-offs was used to define degrees of abnormality. RESULTS: Left ventricular (LV) size tended to decrease with age, while LV mass increased with age in both males and females and in both the healthy and general populations. LV dimensions were larger in males, except for body surface area-indexed LV diameter. M-mode derived LV measurements were larger and LV mass higher compared to 2D measurements. Right ventricle basal dimension was larger in males. CONCLUSIONS: Our study provides reference ranges for echocardiographic measurements obtained in a healthy subgroup derived from an epidemiological study of a Central European population. Where feasible, degrees of abnormality are provided based on the whole population sample including patients with disease. Our data show that age, gender and measurement method significantly affect cardiac dimensions and function and should be always taken into account.


Assuntos
Doenças Cardiovasculares/diagnóstico , Ecocardiografia/métodos , Átrios do Coração/diagnóstico por imagem , Ventrículos do Coração/diagnóstico por imagem , Vigilância da População , Função Ventricular Esquerda/fisiologia , Adulto , Idoso , Doenças Cardiovasculares/fisiopatologia , República Tcheca , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Valores de Referência
13.
Kardiol Pol ; 77(10): 944-950, 2019 10 25.
Artigo em Inglês | MEDLINE | ID: mdl-31406099

RESUMO

Bacground: Cardiac magnetic resonance imaging (MRI) represents the gold standard in noninvasive evaluation of myocardial tissue. However, some patients are unable to undergo cardiac MRI due to a variety of reasons. AIMS: We sought to determine the diagnostic accuracy of routinely performed contrast­enhanced computed tomography (CECT) compared with cardiac MRI in the evaluation of myocardial tissue. METHODS: We retrospectively evaluated 96 consecutive patients (mean [SD] age, 51 [15] years; 41 women) who underwent both CECT and cardiac MRI within 30 days. All CECT scans that visualized the entire heart were analyzed, regardless of the indication for and protocol of the procedure. The presence of late gadolinium enhancement on cardiac MRI was compared with the finding of myocardial hypoattenuation on computed tomography scans. RESULTS: With cardiac MRI as the gold standard, CECT revealed a per­patient sensitivity of 66%, specificity of 89%, positive predictive value of 75%, negative predictive value of 84%, and accuracy of 81%. Per­segment sensitivity was 54%; specificity, 98%; positive predictive value, 76%; negative predictive value, 94%; and accuracy, 92%. CONCLUSIONS: Our study suggests that routinely performed CECT has high specificity, but only moderate sensitivity, compared with cardiac MRI in the evaluation of myocardial tissue. This result supports the recommendation that all CECT scans that visualize the entire heart should be analyzed for myocardial tissue pathology.


Assuntos
Cardiopatias/diagnóstico por imagem , Imageamento por Ressonância Magnética , Tomografia Computadorizada por Raios X , Adulto , Idoso , Meios de Contraste , Feminino , Gadolínio , Coração/diagnóstico por imagem , Cardiopatias/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Miocárdio/patologia , Sensibilidade e Especificidade
14.
Pituitary ; 22(4): 411-421, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31222579

RESUMO

PURPOSE: Stereotactic radiosurgery is one of the treatment options for prolactinomas, the most commonly used being Gamma Knife Radiosurgery (GKRS). GKRS is indicated mainly in the treatment of dopamine agonist (DA)-resistant prolactinomas. In our study, we report on our experience in treating prolactinoma patients by GKRS. METHODS: Twenty-eight patients were followed-up after GKRS for 26-195 months (median 140 months). Prior to GKRS, patients were treated with DAs and 9 of them (32.1%) underwent previous neurosurgery. Cavernous sinus invasion was present in 16 (57.1%) patients. Indications for GKRS were (i) resistance to DA treatment (17 patients), (ii) drug intolerance (5 patients), or (iii) attempts to reduce the dosage and/or shorten the length of DA treatment (6 patients). RESULTS: After GKRS, normoprolactinaemia was achieved in 82.1% of patients, out of which hormonal remission (normoprolactinaemia after discontinuation of DAs) was achieved in 13 (46.4%), and hormonal control (normoprolactinaemia while taking DAs) in 10 (35.7%) patients. GKRS arrested adenoma growth or decreased adenoma size in all cases. Two patients (8.3%) developed hypopituitarism after GKRS. Prolactinoma cystic transformation with expansive behaviour, manifested by bilateral hemianopsia, was observed in one patient. CONCLUSIONS: GKRS represents an effective treatment option, particularly for DA-resistant prolactinomas. Normoprolactinaemia was achieved in the majority of patients, either after discontinuation of, or while continuing to take, DAs. Tumour growth was arrested in all cases. The risk of the development of hypopituitarism can be limited if the safe dose to the pituitary and infundibulum is maintained.


Assuntos
Prolactinoma/radioterapia , Radiocirurgia/métodos , Adulto , Agonistas de Dopamina/uso terapêutico , Feminino , Hemianopsia/radioterapia , Humanos , Hipopituitarismo/radioterapia , Masculino , Pessoa de Meia-Idade , Prolactinoma/tratamento farmacológico , Resultado do Tratamento , Adulto Jovem
15.
Echocardiography ; 35(11): 1755-1763, 2018 11.
Artigo em Inglês | MEDLINE | ID: mdl-30247786

RESUMO

BACKGROUND: Fabry cardiomyopathy (FC) and light-chain amyloid cardiomyopathy (AL) present with concentric left ventricular (LV) hypertrophy/remodeling and diastolic rather than systolic dysfunction. Direct comparisons are difficult due to rarity and confounded by variability of LV thickness. AIMS: To compare LV diastolic and systolic properties between patients with FC and AL in a cohort matched for interventricular septal thickness (IVS). METHODS: A two-center echocardiographic analysis was performed, comprising 118 patients with IVS ≥12 mm (FC and AL 59 patients each) matched by IVS. RESULTS: Fabry cardiomyopathy patients had larger LV end-diastolic diameter (47.7 [44.0-50.9] vs 45.0 [41.5-49.0] mm, P = 0.002), better LV ejection fraction (EF 68.7 [63.4-74.0] vs 63.0 [54.0-70.0]%, P = 0.001) and midwall fractional shortening (midFS 14.8 [13.0-16.1] vs 12.1 [8.9-15.0]%, P = 0.006). LV EF <40% was rare in both (2% vs 7%, P = 0.17). AL patients expressed higher LV diastolic dysfunction grade (III in 26% vs 4%, II in 21% vs 12% and I in 54% vs 84%, P = 0.004), with higher E/e' ratio (13.6 [10.2-18.8] vs 9.8 [7.5-12.3], P < 0.0001). Average E/e' ratio and midFS were significantly associated with NYHA severity in both groups (P < 0.05 for all). CONCLUSIONS: Matched AL patients had worse LV diastolic function than FC, driven by E/e'. Significant LV systolic dysfunction was rare overall. MidFS and E/e' were associated with heart failure severity in both groups.


Assuntos
Amiloidose/diagnóstico por imagem , Cardiomiopatias/diagnóstico por imagem , Ecocardiografia/métodos , Doença de Fabry/diagnóstico por imagem , Cardiopatias/diagnóstico por imagem , Disfunção Ventricular Esquerda/diagnóstico por imagem , Idoso , Amiloidose/fisiopatologia , Cardiomiopatias/fisiopatologia , Doença de Fabry/fisiopatologia , Feminino , Cardiopatias/fisiopatologia , Ventrículos do Coração/diagnóstico por imagem , Ventrículos do Coração/fisiopatologia , Humanos , Masculino , Pessoa de Meia-Idade , Reprodutibilidade dos Testes , Estudos Retrospectivos , Sensibilidade e Especificidade , Disfunção Ventricular Esquerda/fisiopatologia , Remodelação Ventricular/fisiologia
16.
Echocardiography ; 35(11): 1764-1771, 2018 11.
Artigo em Inglês | MEDLINE | ID: mdl-30225879

RESUMO

AIM OF THE STUDY: To assess the diagnostic utility of a simplified approach to relative apical sparing of longitudinal strain (RAS LS) using only an apical four-chamber view (A4C) in patients with AL amyloid cardiomyopathy (ALAC). METHODS: We retrospectively evaluated echocardiographic recordings of 20 patients with ALAC, 20 patients with Fabry disease-related cardiomyopathy (FD), and 20 patients with concentric hypertensive left ventricular hypertrophy (HLVH) matched for mean LV mean thickness. Peak segmental LS values of the interventricular septum and lateral LV wall were measured in the A4C using two-dimensional speckle-tracking echocardiography. RAS LS was calculated as average apical LS/(average basal LS + average midventricular LS). RESULTS: Relative apical sparing of longitudinal strain values in patients with ALAC (1.23 ± 0.64) were significantly higher than those in FD patients (0.75 ± 0.19, P < 0.05) as well as in individuals with HLVH (0.75 ± 0.23, P < 0.05), but with a significant overlap. The optimal RAS LS value differentiating ALAC from FD and HLVH with 70% sensitivity and 75% specificity was 0.88 (AUC 0.79). In multivariate modeling, RAS LS was significantly additive to traditional predictors of ALAC (low QRS voltage and pseudoinfarct ECG patterns, pericardial effusion, E/e' ratio, E-wave deceleration time; P < 0.05 for all models). CONCLUSIONS: Simplified RAS LS evaluation represents an attractive approach for diagnostics of ALAC. However, because of considerable overlap with other disorders with hypertrophic phenotype, the analysis of RAS LS in the A4C should be combined with other traditional echocardiographic and ECG predictors in differentiating ALAC from other forms of concentric LV wall thickening.


Assuntos
Amiloidose/diagnóstico por imagem , Cardiomiopatias/diagnóstico por imagem , Ecocardiografia/métodos , Amiloidose/fisiopatologia , Cardiomiopatias/fisiopatologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Reprodutibilidade dos Testes , Estudos Retrospectivos , Sensibilidade e Especificidade
17.
BMC Med Imaging ; 18(1): 32, 2018 09 18.
Artigo em Inglês | MEDLINE | ID: mdl-30227848

RESUMO

BACKGROUND: Left atrial (LA) enlargement has been identified as a predictor of worse clinical outcome after catheter ablation for atrial fibrillation (AF). We investigated the correspondence of LA size parameters assessed by echocardiography, CT and 3D electroanatomical mapping in patients with AF treated by catheter ablation. METHODS: We analyzed echocardiographic LA volume measurements by disc summation method (LAVDISC), computed tomography (LAVCT) and 3D electroanatomical mapping (LAVCARTO) in 100 pts. (71% males; aged 63 ± 8 years; paroxysmal AF in 55% of patients). RESULTS: Mean LAVDISC was 83 ± 25 ml (median: 115; IQR: 98-140 ml), mean LAVCT was 120 ± 34 ml (median: 115; IQR: 98-140 ml) and mean LAVCARTO was 123 ± 36 ml (median: 118; IQR: 99-132 ml). Pearson's correlation coefficient between LAVDISC a LAVCT was 0.6 (p < 0.0001) and between LAVCARTO and LAVCT was 0.79 (p < 0.0001). There was a significant difference between the two correlation coefficients (p < 0.004). The absolute difference between LAVCARTO and LAVCT (3.5 (95% CI -42 - 43) ml) was significantly lower (p < 0.0001) as compared to LAVDISC and LAVCT (- 39 (95% CI -102 - 24) ml). In opposite to LAVDISC, the bias between LAV obtained by CT and CARTO did not differentiate according to presence of spherical remodeling (1.7 ± 28 vs. vs. 5.1 ± 31 ml). Only presence of sinus rhythm was significant and independent covariate of the difference between CARTO and CT-derived LAVs by multivariate regression analysis. CONCLUSIONS: Even though LA volumes evaluated by 3D-electroanatomical mapping have quite good accuracy, the precision is low. For volumes estimated by echocardiography, both precision and accuracy are low.


Assuntos
Fibrilação Atrial/diagnóstico por imagem , Ecocardiografia/métodos , Átrios do Coração/diagnóstico por imagem , Imageamento Tridimensional/métodos , Idoso , Fibrilação Atrial/cirurgia , Remodelamento Atrial , Ablação por Cateter , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Análise de Regressão , Sensibilidade e Especificidade , Tomografia Computadorizada por Raios X/métodos
18.
J Cardiovasc Comput Tomogr ; 12(5): 418-424, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-29945850

RESUMO

OBJECTIVE: Catheter ablation (CA) is an established therapy for selected patients with atrial fibrillation (AF), but predictors of CA ablation outcome are still not fully elucidated. The aim of the study was to identify structural and morphological parameters from computed tomography (CT) as predictors of successful CA of AF in a single center prospective cohort. METHODS: An analysis of CT scans dedicated to LA evaluation was performed in 99 patients (63 ±â€¯8 years old, 70% males, 59% paroxysmal AF) scheduled for CA of AF. Survival free of atrial fibrillation/flutter/tachycardia at 1- and 3-years was assessed. RESULTS: In overall study population, both 1- and 3-year responders had smaller distance to the first division in left superior pulmonary vein (16.3 ±â€¯5.42 mm vs. 19.1 ±â€¯7.0 mm and 14.9 ±â€¯3.6 mm vs. 18.7 ±â€¯7.0 mm; p < 0.05). One-year responders had larger ostium area of left inferior pulmonary vein (median 236 mm2 [IQR = 97] vs. 222 mm2 [IQR = 71]; p = 0.03) and less acute angle between the interatrial septum and the right superior pulmonary vein (102 ±â€¯20° vs. 95 ±â€¯10°; p = 0.03). Three-years' responders had smaller ostium area of the right superior pulmonary vein (248 ±â€¯94 mm2 vs. 364 ±â€¯282 mm2; p = 0.02). Multivariate Cox regression analysis identified different predictors in paroxysmal and non-paroxysmal AF. For patients with paroxysmal AF, the predictors were angle to right superior pulmonary vein and left superior/inferior pulmonary veins carina thickness with hazard ratios of 0.965 (95%CI 0.939 to 0.992, p = 0.010) and 0.747 (95%CI 0.591 to 0.944, p = 0.015). In patients with persistent AF, the predictors were gender and NYHA stage with hazard ratios of 4.9 (95%CI 1.758 to 13.579, p = 0.002) and 0.365 (95%CI 0.148 to 0.899, p = 0.028) respectively. CONCLUSIONS: The anatomy of LA, especially morphology of pulmonary veins, seems to be one of the predictors of clinical outcome after CA for paroxysmal AF. In non-paroxysmal AF LA anatomy is less relevant in prediction of clinical outcome.


Assuntos
Fibrilação Atrial/diagnóstico por imagem , Fibrilação Atrial/cirurgia , Ablação por Cateter , Tomografia Computadorizada Multidetectores , Veias Pulmonares/diagnóstico por imagem , Veias Pulmonares/cirurgia , Idoso , Fibrilação Atrial/fisiopatologia , Ablação por Cateter/efeitos adversos , República Tcheca , Intervalo Livre de Doença , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Estudos Prospectivos , Veias Pulmonares/fisiopatologia , Recidiva , Fatores de Risco , Fatores de Tempo , Resultado do Tratamento
19.
J Transl Med ; 15(1): 215, 2017 10 25.
Artigo em Inglês | MEDLINE | ID: mdl-29070043

RESUMO

BACKGROUND: Renal denervation (RDN) is a promising therapeutic method in cardiology. Its currently most investigated indication is resistant hypertension. Other potential indications are atrial fibrillation, type 2 diabetes mellitus and chronic renal insufficiency among others. Previous trials showed conflicting but promising results, but the real benefits of RDN are still under investigation. Patients with renal insufficiency and resistant hypertension are proposed to be a good target for this therapy due to excessive activation of renal sympathetic drive. However, only limited number of studies showed benefits for these patients. We hypothesize that in our experimental model of chronic kidney disease (CKD) due to ischemia with increased activity of the renin-angiotensin-aldosterone system (RAAS), renal denervation can have protective effects by slowing or blocking the progression of renal injury. METHODS: An experimental biomodel of chronic renal insufficiency induced by ischemia was developed using selective renal artery embolization (remnant kidney porcine model). 27 biomodels were assessed. Renal denervation was performed in 19 biomodels (denervated group), and the remaining were used as controls (n = 8). The extent of renal injury and reparative process between the two groups were compared and assessed using biochemical parameters and histological findings. RESULTS: Viable remnant kidney biomodels were achieved and maintained in 27 swine. There were no significant differences in biochemical parameters between the two groups at baseline. Histological assessment proved successful RDN procedure in all biomodels in the denervated group. Over the 7-week period, there were significant increases in serum urea, creatinine, and aldosterone concentration in both groups. The difference in urea and creatinine levels were not statistically significant between the two groups. However, the level of aldosterone in the denervated was significantly lower in comparison to the controls. Histological assessment of renal arteries showed that RDN tends to produce more damage to the arterial wall in comparison to vessels in subjects that only underwent RAE. In addition, the morphological damage of kidneys, which was expressed as a ratio of damaged surface (or scar) to the overall surface of kidney, also did not show significant difference between groups. CONCLUSIONS: In this study, we were not able to show significant protective effect of RDN alone on ischemic renal parenchymal damage by either laboratory or histological assessments. However, the change in aldosterone level shows some effect of renal denervation on the RAAS system. We hypothesize that a combined blockade of the RAAS and the sympathetic system could provide more protective effects against acute ischemia. This has to be further investigated in future studies.


Assuntos
Denervação , Rim/inervação , Insuficiência Renal Crônica/terapia , Aldosterona/sangue , Animais , Creatinina/sangue , Modelos Animais de Doenças , Rim/patologia , Artéria Renal/inervação , Artéria Renal/patologia , Insuficiência Renal Crônica/sangue , Insuficiência Renal Crônica/patologia , Análise de Sobrevida , Sus scrofa , Ureia/sangue
20.
Cas Lek Cesk ; 156(5): 266-270, 2017.
Artigo em Tcheco | MEDLINE | ID: mdl-28992711

RESUMO

Pituitary adenomas are the most common tumours of the sellar region. A combination of neurosurgery, radiation and pharmacological approaches are applied for the treatment of pituitary adenomas. In certain cases, patient observation is another option. Neurosurgery is the first-choice treatment for acromegaly, Cushing´s disease and TSH secreting adenomas. Leksell gamma knife irradiation is used in the treatment of tumour residues. Until the effect of the irradiation is evident, pharmacological treatment must be administered. Large and/or growing non-functioning pituitary adenomas are operated. Irradiation is possible if there is sufficient distance between the margin of the adenoma and the optic pathway. The primary therapy for prolactinomas is pharmacological treatment with dopamine agonists. Multidisciplinary collaboration among endocrinologists, neurosurgeons and radiosurgeons is necessary in the treatment of pituitary adenomas.


Assuntos
Adenoma , Neoplasias Hipofisárias , Adenoma/cirurgia , Humanos , Neoplasias Hipofisárias/cirurgia
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