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1.
JAMA Netw Open ; 1(4): e181456, 2018 08 03.
Artigo em Inglês | MEDLINE | ID: mdl-30646130

RESUMO

Importance: Clinical decisions are frequently based on measurement of left ventricular ejection fraction (LVEF). Limited information exists regarding inconsistencies in LVEF measurements when determined by various imaging modalities and the potential impact of such variability. Objective: To determine the intermodality variability of LVEF measured by echocardiography, gated single-photon emission computed tomography (SPECT), and cardiovascular magnetic resonance (CMR) in patients with left ventricular dysfunction. Design, Setting, and Participants: International multicenter diagnostic study with LVEF imaging performed at 127 clinical sites in 26 countries from July 24, 2002, to May 5, 2007, and measured by core laboratories. Secondary study of clinical diagnostic measurements of LVEF in the Surgical Treatment for Ischemic Heart Failure (STICH), a randomized trial to identify the optimal treatment strategy for patients with LVEF of 35% or less and coronary artery disease. Data analysis was conducted from March 19, 2016, to May 29, 2018. Main Outcomes and Measures: At baseline, most patients had an echocardiogram and subsets of patients underwent SPECT and/or CMR. Left ventricular ejection fraction was measured by a core laboratory for each modality independent of the results of other modalities, and measurements were compared among imaging methods using correlation, Bland-Altman plots, and coverage probability methods. Association of LVEF by each method and death was assessed. Results: A total of 2032 patients (mean [SD] age, 60.9 [9.6] years; 1759 [86.6%] male) with baseline LVEF data were included. Correlation of LVEF between modalities was r = 0.601 (for biplane echocardiography and SPECT [n = 385]), r = 0.493 (for biplane echocardiography and CMR [n = 204]), and r = 0.660 (for CMR and SPECT [n = 134]). Bland-Altman plots showed only moderate agreement in LVEF measurements from all 3 core laboratories with no substantial overestimation or underestimation of LVEF by any modality. The percentage of observations that fell within a range of 5% ranged from 43% to 54% between different imaging modalities. Conclusions and Relevance: In this international multicenter study of patients with coronary artery disease and reduced LVEF, there was substantial variation between modalities in LVEF determination by core laboratories. This variability should be considered in clinical management and trial design. Trial Registration: Clinicaltrials.gov Identifier: NCT00023595.


Assuntos
Tomografia Computadorizada por Emissão de Fóton Único de Sincronização Cardíaca , Doença da Artéria Coronariana/diagnóstico por imagem , Doença da Artéria Coronariana/fisiopatologia , Ecocardiografia , Imagem por Ressonância Magnética , Volume Sistólico , Disfunção Ventricular Esquerda/diagnóstico por imagem , Disfunção Ventricular Esquerda/fisiopatologia , Técnicas de Imagem Cardíaca , Feminino , Humanos , Masculino , Pessoa de Meia-Idade
3.
Genome Med ; 9(1): 73, 2017 08 14.
Artigo em Inglês | MEDLINE | ID: mdl-28807008

RESUMO

BACKGROUND: De novo missense variants in CDK13 have been described as the cause of syndromic congenital heart defects in seven individuals ascertained from a large congenital cardiovascular malformations cohort. We aimed to further define the phenotypic and molecular spectrum of this newly described disorder. METHODS: To minimise ascertainment bias, we recruited nine additional individuals with CDK13 pathogenic variants from clinical and research exome laboratory sequencing cohorts. Each individual underwent dysmorphology exam and comprehensive medical history review. RESULTS: We demonstrate greater than expected phenotypic heterogeneity, including 33% (3/9) of individuals without structural heart disease on echocardiogram. There was a high penetrance for a unique constellation of facial dysmorphism and global developmental delay, as well as less frequently seen renal and sacral anomalies. Two individuals had novel CDK13 variants (p.Asn842Asp, p.Lys734Glu), while the remaining seven unrelated individuals had a recurrent, previously published p.Asn842Ser variant. Summary of all variants published to date demonstrates apparent restriction of pathogenic variants to the protein kinase domain with clustering in the ATP and magnesium binding sites. CONCLUSIONS: Here we provide detailed phenotypic and molecular characterisation of individuals with pathogenic variants in CDK13 and propose management guidelines based upon the estimated prevalence of anomalies identified.


Assuntos
Proteína Quinase CDC2/genética , Face/anormalidades , Cardiopatias Congênitas/metabolismo , Deficiência Intelectual/metabolismo , Mutação , Fenótipo , Adolescente , Adulto , Criança , Pré-Escolar , Feminino , Cardiopatias Congênitas/genética , Humanos , Lactente , Deficiência Intelectual/genética , Masculino , Síndrome
4.
Circ Cardiovasc Genet ; 10(4)2017 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-28798025

RESUMO

BACKGROUND: Left ventricular noncompaction (LVNC) is a genetically and phenotypically heterogeneous disease and, although increasingly recognized in clinical practice, there is a lack of widely accepted diagnostic criteria. We sought to identify novel genetic causes of LVNC and describe genotype-phenotype correlations. METHODS AND RESULTS: A total of 190 patients from 174 families with left ventricular hypertrabeculation (LVHT) or LVNC were referred for cardiac magnetic resonance and whole-exome sequencing. A total of 425 control individuals were included to identify variants of interest (VOIs). We found an excess of 138 VOIs in 102 (59%) unrelated patients in 54 previously identified LVNC or other known cardiomyopathy genes. VOIs were found in 68 of 90 probands with LVNC and 34 of 84 probands with LVHT (76% and 40%, respectively; P<0.001). We identified 0, 1, and ≥2 VOIs in 72, 74, and 28 probands, respectively. We found increasing number of VOIs in a patient strongly correlated with several markers of disease severity, including ratio of noncompacted to compacted myocardium (P<0.001) and left ventricular ejection fraction (P=0.01). The presence of sarcomeric gene mutations was associated with increased occurrence of late gadolinium enhancement (P=0.004). CONCLUSIONS: LVHT and LVNC likely represent a continuum of genotypic disease with differences in severity and variable phenotype explained, in part, by the number of VOIs and whether mutations are present in sarcomeric or nonsarcomeric genes. Presence of VOIs is common in patients with LVHT. Our findings expand the current clinical and genetic diagnostic approaches for patients with LVHT and LVNC.


Assuntos
Estudos de Associação Genética , Disfunção Ventricular Esquerda/diagnóstico , Proteínas Adaptadoras de Transdução de Sinal/genética , Adolescente , Adulto , Idoso , Miosinas Cardíacas/genética , Proteínas de Transporte/genética , Criança , Conectina/genética , Feminino , Variação Genética , Ventrículos do Coração/fisiopatologia , Humanos , Proteínas com Domínio LIM/genética , Imagem Cinética por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Proteínas Musculares/genética , Miocárdio/patologia , Cadeias Pesadas de Miosina/genética , Estudos Prospectivos , Índice de Gravidade de Doença , Tropomiosina/genética , Disfunção Ventricular Esquerda/genética , Disfunção Ventricular Esquerda/patologia , Adulto Jovem
6.
Cancer Epidemiol ; 39(5): 665-72, 2015 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-26239627

RESUMO

Primary malignant cardiac tumors are rare, and mostly manifest as sarcomas in various types. As non-invasive diagnostic modalities, e.g. echocardiography and magnetic resonance imaging, have become more sensitive, there is a marked increase in the number of patients diagnosed. Nevertheless, most patients die within one year of initial diagnosis, either because of the often asymptomatic presentation of cardiac tumors until advanced disease, or a low index of suspicion on the part of the physician. The presenting symptoms, treatment options and, indeed, prognosis are largely controlled by the tumor's anatomic location. Cardiac sarcomas may present with a variety of symptoms and are known to be great mimickers. A quick diagnosis facilitates the initiation of a proper treatment (surgical resection, adjuvant chemotherapy), which may in turn improve the prognosis. Metastases to the heart are far more common, unfortunately, clinical manifestations are mainly dominated by generalized tumor spread. The article summarizes epidemiology, symptoms, diagnostic modalities, and possible treatment options.


Assuntos
Neoplasias Cardíacas/diagnóstico , Neoplasias Cardíacas/terapia , Humanos , Sarcoma/diagnóstico , Sarcoma/terapia
8.
Kardiochir Torakochirurgia Pol ; 11(4): 404-8, 2014 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-26336457

RESUMO

AIM: The aim of this study was to assess the relationship between late gadolinium-enhanced (LGE) cardiovascular magnetic resonance (CMR) and immunohistochemical markers of inflammation in patients with heart failure and a reduced ejection fraction (HFrEF). MATERIAL AND METHODS: Endomyocardial biopsy and CMR were performed in 38 consecutive patients (24 males, average age 43.2 ± 6.9 years, New York Heart Association [NYHA] class II) with HFrEF and suspected myocarditis. The immunohistochemical evaluation was done by the En-Vision system using DAKO monoclonal antibodies. The presence of > 14 infiltrating cells together with myocardial damage and ≥ 2 + up-regulation of HLA class II was considered diagnostic for myocarditis. The results of LGE were compared with the immunohistochemical markers of inflammation. All patients underwent coronary angiography. RESULTS: Twelve out of 38 (31.6%) patients met the immunohistological criteria for the diagnosis of myocarditis. Late gadolinium enhancement was present in 23 of 38 (60.5%) patients, mostly at the interventricular septum. No correlation was found between LGE and immunohistochemistry results (Kendall's tau; r = 0.21, p = 0.09). CONCLUSIONS: Our study revealed no significant relationship between LGE cardiovascular magnetic resonance imaging and immunohistochemical markers of inflammation in patients with HFrEF.

12.
Eur Radiol ; 21(11): 2297-304, 2011 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-21786089

RESUMO

OBJECTIVES: The aim of the study was to assess cardiac involvement in patients with Wegener's granulomatosis (WG), who failed to achieve remission following >6 months induction therapy for life or organ threatening disease. METHODS: Eleven WG patients (eight males, mean age 47 ± 13 years), who failed to achieve remission despite >6 months induction therapy, underwent transthoracic echocardiography (TTE) and cardiac magnetic resonance (CMR). RESULTS: Cardiac involvement was present in 9 (82%) patients. Regional wall motion abnormalities were found in two individuals, but none had left ventricular (LV) ejection fraction <50%. Nine patients had late gadolinium enhancement (LGE) lesions involving LV myocardium and right ventricle free wall was involved in four patients. LGE lesions were found in subepicardial, midwall and subendocardial LV myocardial layers. CMR revealed myocarditis in six patients. Patients with myocarditis had a higher number of LV segments with LGE (5.2 ± 3.4 vs 1.0 ± 1.2, p = 0.03) and more frequent diastolic dysfunction by TTE (5 vs 0, p = 0.02) than those without. Pericardial effusion was observed in five patients, while localized pericardial thickening in six patients. CONCLUSIONS: In WG resistant to >6 months induction therapy cardiac involvement is frequent and is characterized by foci of LGE lesions and signs of myocardial inflammatory process.


Assuntos
Ecocardiografia/métodos , Granulomatose com Poliangiite/diagnóstico , Imagem por Ressonância Magnética/métodos , Miocardite/diagnóstico , Adulto , Diagnóstico por Imagem/métodos , Feminino , Coração/fisiopatologia , Humanos , Masculino , Pessoa de Meia-Idade , Miocardite/patologia , Indução de Remissão
14.
J Card Surg ; 25(2): 171-4, 2010 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-20021510

RESUMO

We report a case of a 70-year-old woman admitted with symptoms of decompensated heart failure. Magnetic resonance imaging revealed lipomatous hypertrophy of the interatrial septum with partial upper right atrial inflow obstruction, partial obstruction of the right ventricular outflow tract, and excessive accumulation of adipose tissue in the pericardial space. The patient underwent excision of the septal lipomatous mass, which relieved the right ventricular outflow obstruction.


Assuntos
Septo Interatrial/cirurgia , Cardiomiopatias/cirurgia , Lipomatose/cirurgia , Obstrução do Fluxo Ventricular Externo/etiologia , Obstrução do Fluxo Ventricular Externo/cirurgia , Idoso , Septo Interatrial/patologia , Cardiomiopatias/diagnóstico , Feminino , Humanos , Hipertrofia , Lipomatose/diagnóstico , Imagem por Ressonância Magnética , Resultado do Tratamento , Obstrução do Fluxo Ventricular Externo/diagnóstico
15.
J Am Soc Echocardiogr ; 22(11): 1220-7, 2009 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-19883873

RESUMO

BACKGROUND: The aim of this study was to determine the incremental prognostic value of myocardial contrast echocardiography (MCE) over two-dimensional echocardiography (2DE) in patients undergoing supine bicycle stress. METHODS: Eighty-four patients with known or suspected coronary artery disease who underwent supine bicycle stress with 2DE and MCE (mean age, 58.5 +/- 9.7 years; 68 men) were followed up for 48.3 +/- 8.9 months for cardiac death (n = 1), nonfatal myocardial infarction (n = 9), and revascularization (n = 20). RESULTS: In sequential Cox models, the predictive power of the clinical model was strengthened by 2DE (chi(2) = 7.73 vs 12.92, P = .02) and further improved by MCE (chi(2) = 19.04, P = .01). On multivariate analysis, the only independent follow-up event predictor was ischemia on MCE (hazard ratio, 6.79; 95% confidence interval, 2.02-22.82; P = .001). Among patients with normal results on 2DE, those with normal results on MCE had greater 4.5-year event-free survival than those with abnormal results on MCE (93% vs 69%, P = .01). CONCLUSIONS: MCE enhances the predictive power of supine bicycle stress 2DE and allows the risk stratification of patients with normal results on 2DE.


Assuntos
Doença das Coronárias/diagnóstico por imagem , Ecocardiografia sob Estresse/métodos , Ecocardiografia/métodos , Distribuição de Qui-Quadrado , Meios de Contraste , Angiografia Coronária , Doença das Coronárias/fisiopatologia , Intervalo Livre de Doença , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Modelos de Riscos Proporcionais , Medição de Risco , Decúbito Dorsal , Taxa de Sobrevida
16.
Kardiol Pol ; 67(4): 459-63, 2009 Apr.
Artigo em Polonês | MEDLINE | ID: mdl-19492264

RESUMO

Multislice computed tomography is an imaging method of internal organs including heart and vessels with the use of X-ray. The indications for computed tomography of the heart include also congenital heart diseases and the evaluation of the great arteries. Ultrasonography is a method of choice in heart evaluation. The authors show the possibilities of modern multislice computed tomography in congenital heart diseases imaging based on their own material.


Assuntos
Cardiopatias Congênitas/diagnóstico por imagem , Tomografia Computadorizada Espiral , Adulto , Coartação Aórtica/diagnóstico por imagem , Humanos , Recém-Nascido , Masculino , Intensificação de Imagem Radiográfica/métodos , Tetralogia de Fallot/diagnóstico por imagem , Transposição dos Grandes Vasos/diagnóstico por imagem , Malformações Vasculares/diagnóstico por imagem
17.
Kardiol Pol ; 67(3): 348-52, 2009 Mar.
Artigo em Polonês | MEDLINE | ID: mdl-19378246

RESUMO

Transcatheter implantation of pulmonary valve became a big step forward in the field of interventional cardiology. It is especially important in the patients with defects of the right ventricular outlet tract, because they were usually candidates for multiple surgical operations. We present first transcatheter replacement of pulmonary valve in 23-years-old man. The 'Melody' valve was implanted successfully. There were no complications and the patient was discharged in good condition.


Assuntos
Cardiopatias Congênitas/cirurgia , Implante de Prótese de Valva Cardíaca/métodos , Valva Pulmonar/cirurgia , Obstrução do Fluxo Ventricular Externo/terapia , Cateterismo Cardíaco/métodos , Cardiopatias Congênitas/complicações , Comunicação Interventricular/cirurgia , Ventrículos do Coração/cirurgia , Humanos , Masculino , Assistência Perioperatória/métodos , Resultado do Tratamento , Disfunção Ventricular Direita/etiologia , Disfunção Ventricular Direita/terapia , Adulto Jovem
18.
Int J Cardiol ; 136(1): 47-55, 2009 Jul 24.
Artigo em Inglês | MEDLINE | ID: mdl-18675474

RESUMO

BACKGROUND: If compared to two-dimensional echocardiography (2DE), myocardial contrast echocardiography (MCE) improves detection of coronary artery disease (CAD) during pharmacological stress, but data on MCE vs. 2DE during supine bicycle stress is limited. Although previous myocardial infarction (MI) influences sensitivity of 2DE, its effect on MCE has not been evaluated. OBJECTIVES: The study sought to determine the incremental benefit of MCE over 2DE for evaluation of CAD during supine bicycle stress and to assess the impact of previous MI on diagnostic values of both methods. METHODS: We studied 103 consecutive patients scheduled for coronary angiography. Prior to coronary angiography, all patients underwent supine bicycle stress. 2DE and MCE were performed during this stress test. The diagnosis of obstructive CAD (> or =50% stenosis) was based on the presence of inducible wall motion and perfusion abnormalities. RESULTS: Quantitative coronary angiography revealed > or =50% stenosis in 53 of 77 patients without previous MI and in 21 of 26 patients with previous MI. If compared to 2DE, MCE was more sensitive (68% vs. 86%; p<0.001) and more accurate (73% vs. 86%; p < 0.001) to detect > or =50% stenosis. In patients without previous MI, 2DE and MCE yielded sensitivity of 65% and 85% (p < 0.01) and accuracy of 71% and 85% (p < 0.01), whereas in patients with previous MI sensitivity was 79% and 90% (p=NS) and accuracy 79% and 88% (p = NS), respectively. CONCLUSIONS: MCE enhances sensitivity and accuracy of 2DE in detection of obstructive CAD during supine bicycle stress. The incremental benefit of MCE is especially present in patients without previous MI.


Assuntos
Doença da Artéria Coronariana/diagnóstico por imagem , Ecocardiografia sob Estresse/métodos , Teste de Esforço/normas , Infarto do Miocárdio/diagnóstico por imagem , Idoso , Doença da Artéria Coronariana/diagnóstico , Doença da Artéria Coronariana/fisiopatologia , Ecocardiografia sob Estresse/normas , Teste de Esforço/métodos , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Infarto do Miocárdio/diagnóstico , Infarto do Miocárdio/fisiopatologia , Sensibilidade e Especificidade , Decúbito Dorsal
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