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2.
Radiographics ; 41(2): 338-360, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33481689

RESUMO

Transposition of the great arteries (TGA) is a congenital conotruncal abnormality characterized by discordant connections between the ventricles and great arteries, with the aorta originating from the right ventricle (RV), and the pulmonary artery (PA) originating from the left ventricle (LV). The two main types of TGA are complete transposition or dextro-transposition of the great arteries (D-TGA), commonly referred to as d-loop, and congenitally corrected transposition (CCTGA), commonly referred to as l-loop or L-TGA. In D-TGA, the connections between the ventricles and atria are concordant, whereas in CCTGA they are discordant, with the left atrium connected to the RV, and the right atrium connected to the LV. D-TGA manifests during the neonatal period and can be surgically managed by atrial switch operation (AtrSO), arterial switch operation (ASO), Rastelli procedure, or Nikaidoh procedure. Arrhythmia, systemic ventricular dysfunction, baffle stenosis, and baffle leak are the common complications of AtrSO, whereas supravalvular pulmonary or branch PA stenosis, neoaortic dilatation, and coronary artery narrowing are the common complications of ASO. CCTGA may manifest late in life, even in adulthood. Surgeries for associated lesions such as tricuspid regurgitation, subpulmonic stenosis, and ventricular septal defect may be performed. A double-switch operation that includes both the atrial and arterial switch operations constitutes anatomic correction for CCTGA. Imaging plays an important role in the evaluation of TGA, both before and after surgery, for helping define the anatomy, quantify hemodynamics, and evaluate complications. Transthoracic echocardiography is the first-line imaging modality for presurgical planning in children with TGA. MRI provides comprehensive morphologic and functional information, particularly in adults after surgery. CT is performed when MRI is contraindicated or expected to generate artifacts. The authors review the imaging appearances of TGA, with a focus on pre- and postsurgical imaging. Online supplemental material is available for this article. ©RSNA, 2021.

4.
Acad Radiol ; 2020 Aug 08.
Artigo em Inglês | MEDLINE | ID: mdl-32782220

RESUMO

RATIONALE AND OBJECTIVES: We examined the status of women's representation on radiology journal editorial boards around the world and evaluated temporal trends in female board representation. MATERIALS AND METHODS: Data on the gender of editorial board members, deputy editors, and editors-in-chief of top general radiology journals from the United States and from five continents outside North America was collected, based on listings in mastheads of each journal at 5-year intervals from 1998 to 2018. Female editorial board representation was compared to female membership in national professional radiology organizations and national physician registries. RESULTS: One of 42 editors-in-chief (2.4%), 24 of 135 deputy editors (17.8%), and 345 of 2545 (13.6%) editorial board members were female for the 20-year period. The overall percentage of female editorial board members increased from 9.7% (41 of 423) in 1998 to 17.0% (97 of 569) in 2018 (p < 0.0001). In 2018, women were less represented on the editorial boards than female professional society membership for all geographic regions except Australia/New Zealand; this difference reached statistical significance in Korea, Brazil, Europe, and China. Female editorial board representation ranged from 11.3% to 33.3%, with no significant difference between the United States (21.5%) and other countries. CONCLUSION: Although the proportion of female editorial board members of radiology journals worldwide has increased over the past two decades, female editorial board composition generally underrepresents the percentage of female radiologists. Editorial board female representation does not differ significantly between the United States and other countries.

5.
10.
Circulation ; 141(23): 1872-1884, 2020 Jun 09.
Artigo em Inglês | MEDLINE | ID: mdl-32372669

RESUMO

BACKGROUND: Mutations in desmoplakin (DSP), the primary force transducer between cardiac desmosomes and intermediate filaments, cause an arrhythmogenic form of cardiomyopathy that has been variably associated with arrhythmogenic right ventricular cardiomyopathy. Clinical correlates of DSP cardiomyopathy have been limited to small case series. METHODS: Clinical and genetic data were collected on 107 patients with pathogenic DSP mutations and 81 patients with pathogenic plakophilin 2 (PKP2) mutations as a comparison cohort. A composite outcome of severe ventricular arrhythmia was assessed. RESULTS: DSP and PKP2 cohorts included similar proportions of probands (41% versus 42%) and patients with truncating mutations (98% versus 100%). Left ventricular (LV) predominant cardiomyopathy was exclusively present among patients with DSP (55% versus 0% for PKP2, P<0.001), whereas right ventricular cardiomyopathy was present in only 14% of patients with DSP versus 40% for PKP2 (P<0.001). Arrhythmogenic right ventricular cardiomyopathy diagnostic criteria had poor sensitivity for DSP cardiomyopathy. LV late gadolinium enhancement was present in a primarily subepicardial distribution in 40% of patients with DSP (23/57 with magnetic resonance images). LV late gadolinium enhancement occurred with normal LV systolic function in 35% (8/23) of patients with DSP. Episodes of acute myocardial injury (chest pain with troponin elevation and normal coronary angiography) occurred in 15% of patients with DSP and were strongly associated with LV late gadolinium enhancement (90%), even in cases of acute myocardial injury with normal ventricular function (4/5, 80% with late gadolinium enhancement). In 4 DSP cases with 18F-fluorodeoxyglucose positron emission tomography scans, acute LV myocardial injury was associated with myocardial inflammation misdiagnosed initially as cardiac sarcoidosis or myocarditis. Left ventricle ejection fraction <55% was strongly associated with severe ventricular arrhythmias for DSP cases (P<0.001, sensitivity 85%, specificity 53%). Right ventricular ejection fraction <45% was associated with severe arrhythmias for PKP2 cases (P<0.001) but was poorly associated for DSP cases (P=0.8). Frequent premature ventricular contractions were common among patients with severe arrhythmias for both DSP (80%) and PKP2 (91%) groups (P=non-significant). CONCLUSIONS: DSP cardiomyopathy is a distinct form of arrhythmogenic cardiomyopathy characterized by episodic myocardial injury, left ventricular fibrosis that precedes systolic dysfunction, and a high incidence of ventricular arrhythmias. A genotype-specific approach for diagnosis and risk stratification should be used.

11.
Pediatr Cardiol ; 41(6): 1107-1114, 2020 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-32367305

RESUMO

Patients after the Ross procedure are at risk for right (RV) and left ventricular (LV) dysfunction due to neo-aortic and pulmonary dysfunction. While neo-aortic root dilatation has been related to LV dysfunction, the potential contributions of aortic stiffness and ventricular interactions have not been evaluated. Patients status post Ross procedure up to age 18 years with cardiac magnetic resonance (CMR) exam from 2007 to 2018 were retrospectively reviewed. Aortic pulse wave velocity (PWV) was calculated from phase contrast and angiogram images. RV and LV peak global longitudinal (GLS) and circumferential strain (GCS) were measured using tissue tracking software. Multivariable regression was performed for variables associated with parameters of LV function. In 58 patients (median age 20.5 years at CMR exam), male gender, longer time since Ross procedure, aortic root dilatation, and lower RV ejection fraction (EF) were associated with decreased LV EF. There was no association with LV late gadolinium enhancement or neo-aortic or conduit regurgitation. LV GCS and GLS also correlated with RV GCS, RV GLS and PWV. In multivariable analysis, the relation of RV and LV systolic function, but not aortic measurements, remained significant. In conclusion, in long-term follow-up after pediatric Ross procedure, RV function rather than aortic root size or aortic stiffness most closely relates to LV function. Ventricular interactions may impact decision-making on timing of conduit intervention, which could differ from established criteria in populations with only aortic or pulmonary valve disease. Further study is warranted to evaluate possible association with clinical outcome.


Assuntos
Doenças da Aorta/cirurgia , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Transplante Autólogo/efeitos adversos , Disfunção Ventricular Esquerda/etiologia , Adolescente , Adulto , Criança , Feminino , Seguimentos , Humanos , Masculino , Estudos Retrospectivos , Rigidez Vascular/fisiologia , Disfunção Ventricular Esquerda/fisiopatologia , Disfunção Ventricular Direita/etiologia , Disfunção Ventricular Direita/fisiopatologia , Adulto Jovem
12.
Pediatr Cardiol ; 41(5): 885-891, 2020 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-32100056

RESUMO

Both cardiac magnetic resonance (CMR) and cardiac catheterization (cath) may assess patients with single ventricle physiology prior to stage II or Fontan palliation. However, development of significant aortopulmonary collaterals may invalidate assumptions of the Fick method. We compared CMR and cath flow measurements and evaluated the relation to collateral flow. This single-center study included all pre-stage II and pre-Fontan patients between 2010 and 2017 with CMR and cath within 1 month. Pulmonary (Qp) and systemic flow (Qs) by cath were calculated by Fick method. CMR Qp was calculated by total pulmonary venous flow, and Qs by total vena caval flow. Collateral flow by CMR was the difference of pulmonary vein and pulmonary artery flow. In 26 studies (16 pre-stage II and 10 pre-Fontan) in 21 patients, collateral flow was higher in pre-Fontan patients (1.8 ± 0.6 vs 0.9 ± 0.8 L/min/m2, p = 0.01). Overall, CMR and cath had good agreement for Qs and Qp:Qs, with moderate correlation (r = 0.44, p = 0.02 for Qs, r = 0.48, p = 0.02 for Qp:Qs). In pre-Fontan but not in pre-stage II patients, CMR had higher Qp (mean difference - 1.71 L/min/m2) and Qp:Qs (mean difference - 0.36). The underestimation of cath Qp correlated with amount of collateral flow (r = - 0.47, p = 0.02). Neither cath nor CMR flow measurements correlated with outcomes in this small cohort. In conclusion, collaterals lead to systematically higher Qp and Qp:Qs measurements by CMR vs cath in single ventricle patients. Measurements may not be used interchangeably, with potential clinical significance in estimating pulmonary vascular resistance. Further study is necessary to evaluate possible relation to clinical outcomes.


Assuntos
Velocidade do Fluxo Sanguíneo , Cateterismo Cardíaco/métodos , Débito Cardíaco , Imagem por Ressonância Magnética/métodos , Coração Univentricular/cirurgia , Pré-Escolar , Feminino , Técnica de Fontan/métodos , Ventrículos do Coração/anormalidades , Ventrículos do Coração/cirurgia , Humanos , Lactente , Masculino , Artéria Pulmonar/cirurgia , Circulação Pulmonar , Estudos Retrospectivos , Coração Univentricular/diagnóstico , Coração Univentricular/fisiopatologia , Resistência Vascular
14.
Am J Cardiol ; 125(6): 970-976, 2020 03 15.
Artigo em Inglês | MEDLINE | ID: mdl-31964501

RESUMO

In repaired tetralogy of Fallot (rTOF), pulmonary insufficiency results in varying degrees of right ventricle (RV) dilation. A subset of patients is diagnosed at initial cardiac magnetic resonance imaging (CMR) with a massively dilated RV, far beyond pulmonary valve replacement (PVR) criteria, which is unlikely to return to normal size after PVR. This study aimed to identify risk factors for massive RV dilation at initial CMR. This nested case-control study included all patients at our institution with rTOF and massive RV dilation (indexed RV end-diastolic volume [RVEDVi] ≥200 ml/m2) on initial CMR. Patients were matched by age at first CMR, gender, and type of repair with rTOF controls with RVEDVi<200 ml/m2. In 39 cases (median RVEDVi 227 ml/m2, interquartile range [IQR] 213 to 250) and 73 controls (median RVEDVi 155 ml/m2, IQR 130 to 169), repair at >6 months of age, longer QRS duration, and non-Caucasian race were significantly associated with massive RV dilation on univariate analysis. In multivariate analysis, repair at >6 months of age (adjusted odds ratio [AOR] 2.90, 95% confidence interval [CI] 1.12 to 7.55, p = 0.03), longer QRS duration (AOR = 1.03, 95% CI 1.01 to 1.05, p = 0.005), and non-Caucasian race (AOR = 7.84, 95% CI 1.76 to 34.8, p = 0.01) remained independently associated with massive RV dilation. Era of repair, history of systemic to pulmonary shunt palliation, genetic anomaly, and additional cardiac lesions did not differ between groups. In conclusion, these risk factors identify a subset of patients who may benefit from earlier CMR evaluation to avoid massive irreversible RV dilation.


Assuntos
Complicações Pós-Operatórias/etiologia , Tetralogia de Fallot/cirurgia , Disfunção Ventricular Direita/etiologia , Adulto , Estudos de Casos e Controles , Criança , Dilatação Patológica/etiologia , Feminino , Humanos , Imagem por Ressonância Magnética , Masculino , Análise Multivariada , Fatores de Risco , Volume Sistólico
15.
Clin Imaging ; 58: 166-169, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31377439

RESUMO

We report the case of a 36 year old male who demonstrated extensive high attenuation in the muscles of the chest wall and neck on a noncontrast chest CT. The high attenuation in the musculature was due to calcium, sequela from a recent episode of rhabdomyolysis. Although the diagnosis of rhabdomyolysis is made on clinical grounds, the imaging appearance can be confusing given the variability in appearance of rhabdomyolysis depending on the severity and the timeline of disease.


Assuntos
Calcinose/diagnóstico , Músculo Esquelético/patologia , Doenças Musculares/diagnóstico , Rabdomiólise/diagnóstico , Adulto , Diagnóstico Diferencial , Humanos , Masculino
16.
Pediatr Cardiol ; 40(7): 1503-1508, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31346663

RESUMO

In patients with D-looped transposition of the great arteries (D-TGA) status post atrial switch operation, the systemic right ventricle (RV) shifts to predominantly circumferential (CS) rather than longitudinal strain (LS), which may represent adaptation or dysfunction. We aimed to evaluate myocardial mechanics in pressure loaded, volume-loaded, and normal RVs by cardiac magnetic resonance (CMR). Patients with D-TGA post atrial switch operation with CMR from 2008 to 2015 were matched 1:1 for age and RV ejection fraction (EF) with repaired tetralogy of Fallot (TOF) patients (volume-loaded RVs), and 1:1 for age with control patients. RV free wall LS and CS were measured using feature tracking software (TomTec, Unterscleissheim, Germany). A total of 32 D-TGA (median age 32 years, 56% male), 32 TOF, and 32 control patients were included. D-TGA patients had less dilatation than TOF patients (125 ± 35 ml/m2 vs. 149 ± 44 ml/m2, p = 0.02) and lower RVEF than controls (42.9 ± 7.7% vs. 56.3 ± 5.6%, p < 0.0001). RV LS was similar in D-TGA and TOF ( - 13.2 ± 4.5% vs. - 14.5 ± 5.9%, p = 0.32), both decreased compared to controls. However, CS in D-TGA was higher than controls ( - 14.1 ± 4.1% vs. - 11.4 ± 4.4%, p = 0.01), with a higher CS:LS ratio (1.2 ± 0.7 vs. 0.6 ± 0.3, p < 0.0001), while CS in TOF and controls did not differ. RVEF in D-TGA correlated closely with CS (r = - 0.85, p < 0.0001) but not LS (r = 0.10, p = 0.58). I n conclusion, CMR can differentiate strain patterns in pressure- and volume-loaded RVs, with decreased LS in both conditions, while systemic RVs compensate with supra-normal CS. CS may be a more clinically relevant measure of RV function in this population.


Assuntos
Transposição das Grandes Artérias/efeitos adversos , Contração Miocárdica/fisiologia , Transposição dos Grandes Vasos/cirurgia , Disfunção Ventricular Direita/etiologia , Adulto , Estudos de Casos e Controles , Feminino , Humanos , Espectroscopia de Ressonância Magnética , Masculino , Estudos Retrospectivos , Transposição dos Grandes Vasos/fisiopatologia , Disfunção Ventricular Direita/diagnóstico por imagem
17.
Radiographics ; 39(4): 957-976, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31199712

RESUMO

Quantitative imaging has been proposed as the next frontier in radiology as part of an effort to improve patient care through precision medicine. In 2007, the Radiological Society of North America launched the Quantitative Imaging Biomarkers Alliance (QIBA), an initiative aimed at improving the value and practicality of quantitative imaging biomarkers by reducing variability across devices, sites, patients, and time. Chest CT occupies a strategic position in this initiative because it is one of the most frequently used imaging modalities, anatomically encompassing the leading causes of mortality worldwide. To date, QIBA has worked on profiles focused on the accurate, reproducible, and meaningful use of volumetric measurements of lung lesions in chest CT. However, other quantitative methods are on the verge of translation from research grounds into clinical practice, including (a) assessment of parenchymal and airway changes in patients with chronic obstructive pulmonary disease, (b) analysis of perfusion with dual-energy CT biomarkers, and (c) opportunistic screening for coronary atherosclerosis and low bone mass by using chest CT examinations performed for other indications. The rationale for and the key facts related to the application of these quantitative imaging biomarkers in cardiothoracic chest CT are presented. ©RSNA, 2019 See discussion on this article by Buckler (pp 977-980).


Assuntos
Marcadores Fiduciais , Medicina de Precisão/métodos , Radiografia Torácica/métodos , Tomografia Computadorizada por Raios X/métodos , Antropometria/métodos , Progressão da Doença , Cardiopatias/diagnóstico por imagem , Humanos , Vértebras Lombares/diagnóstico por imagem , Pneumopatias/diagnóstico por imagem , Programas de Rastreamento , Osteoporose/diagnóstico por imagem , Embolia Pulmonar/diagnóstico por imagem , Sociedades Científicas/organização & administração , Nódulo Pulmonar Solitário/diagnóstico por imagem , Pesquisa Médica Translacional/organização & administração
18.
AJR Am J Roentgenol ; 213(4): 792-797, 2019 10.
Artigo em Inglês | MEDLINE | ID: mdl-31120782

RESUMO

OBJECTIVE. Birt-Hogg-Dubé (BHD) syndrome is considered rare. Growing evidence indicates that it is underdiagnosed. The purpose of this study is to ascertain the impact of radiology reports that suggest the possibility of BHD syndrome on downstream management and a final diagnosis of BHD syndrome. MATERIALS AND METHODS. In this retrospective study, electronic medical records were searched to identify radiology reports suggesting the possibility of BHD syndrome in patients without a known or suspected diagnosis. Clinical and demographic information, the specialty of the ordering clinician, and imaging findings and confidence conveyed in the radiology report (with BHD syndrome listed as the most likely diagnosis versus one among several possibilities but not as the leading diagnosis) were recorded. The resultant downstream evaluations that were captured included referral for genetic evaluation and subsequent diagnosis of BHD. RESULTS. Between 2004 and 2016, radiologists suggested a diagnosis of BHD syndrome in 87 patients. Of these patients, 15% (13/87) underwent genetic evaluation, and 54% of those patients (7/13) had positive findings. Genetic evaluation was more likely for patients with a history of pneumothorax (p = 0.004) or involvement of the lungs and kidneys (p = 0.003). The urology department referred the highest percentage of patients (31% [4/13]) for genetic evaluation. CONCLUSION. Radiologists have a unique opportunity to suggest BHD syndrome, allowing appropriate genetic testing, screening, and counseling of patients and their families. Clinical presentation, including a history of pneumothorax and involvement of both the lungs and kidneys on imaging, may determine which patients are ultimately referred for genetic evaluation. We hope that increasing awareness that BHD syndrome is not so rare may lead to early diagnosis.


Assuntos
Síndrome de Birt-Hogg-Dubé/diagnóstico por imagem , Papel do Médico , Radiologistas , Adulto , Idoso , Idoso de 80 Anos ou mais , Diagnóstico Diferencial , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos
19.
J Comput Assist Tomogr ; 43(2): 317-322, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30407246

RESUMO

OBJECTIVE: This study aimed to assess variability in measurements and accurately quantify aortic regurgitation in patients with coexisting turbulent aortic flow using phase-contrast magnetic resonance. METHODS: All patients (n = 21) underwent phase-contrast magnetic resonance at 2 or more sites: ascending aorta, sinuses of Valsalva, and left ventricular outflow tract. The net flow/minute (NF), forward flow/minute (FF), regurgitant flow/minute (RF), and regurgitant fraction (RF%) were compared with the sum of superior vena cava and descending aortic flow/minute, left ventricular cardiac output, difference between the 2, and percentage difference, respectively. RESULTS: The NF, FF, and RF were significantly different between each site. The combination of FF in the left ventricular outflow tract and NF from the superior vena cava + descending aorta provided the best reliability of RF and regurgitant fraction (intraclass correlation coefficients, 0.881 [95% confidence interval, 0.882-0.878] and 0.838 [95% confidence interval, 0.837-0.838]). CONCLUSION: Combining flow measurements from more than 1 site provides the most accurate quantification of aortic regurgitation in patients with turbulent aortic flow.


Assuntos
Insuficiência da Valva Aórtica/diagnóstico por imagem , Insuficiência da Valva Aórtica/fisiopatologia , Processamento de Imagem Assistida por Computador/métodos , Imagem por Ressonância Magnética/métodos , Adolescente , Adulto , Valva Aórtica/diagnóstico por imagem , Valva Aórtica/fisiopatologia , Criança , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Reprodutibilidade dos Testes , Adulto Jovem
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