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1.
Radiology ; 311(1): e231934, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38652031

RESUMO

Cryptogenic stroke refers to a stroke of undetermined etiology. It accounts for approximately one-fifth of ischemic strokes and has a higher prevalence in younger patients. Embolic stroke of undetermined source (ESUS) refers to a subgroup of patients with nonlacunar cryptogenic strokes in whom embolism is the suspected stroke mechanism. Under the classifications of cryptogenic stroke or ESUS, there is wide heterogeneity in possible stroke mechanisms. In the absence of a confirmed stroke etiology, there is no established treatment for secondary prevention of stroke in patients experiencing cryptogenic stroke or ESUS, despite several clinical trials, leaving physicians with a clinical dilemma. Both conventional and advanced MRI techniques are available in clinical practice to identify differentiating features and stroke patterns and to determine or infer the underlying etiologic cause, such as atherosclerotic plaques and cardiogenic or paradoxical embolism due to occult pelvic venous thrombi. The aim of this review is to highlight the diagnostic utility of various MRI techniques in patients with cryptogenic stroke or ESUS. Future trends in technological advancement for promoting the adoption of MRI in such a special clinical application are also discussed.


Assuntos
AVC Embólico , Imageamento por Ressonância Magnética , Humanos , AVC Embólico/diagnóstico por imagem , AVC Embólico/etiologia , Imageamento por Ressonância Magnética/métodos , AVC Isquêmico/diagnóstico por imagem , AVC Isquêmico/etiologia , Acidente Vascular Cerebral/diagnóstico por imagem , Acidente Vascular Cerebral/etiologia
2.
Clin Endocrinol (Oxf) ; 100(5): 459-465, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38420872

RESUMO

OBJECTIVE: Polycystic ovary syndrome (PCOS) is a chronic lifelong condition affecting up to 20% of women worldwide. There is limited input from affected women to guide the provision of healthcare services and future research needs. Our objective was to scope the healthcare and research priorities of women with PCOS in the United Kingdom. DESIGN: A three-staged modified Delphi method, consisting of two questionnaires and a consensus meeting involving lay representatives and healthcare professionals. PATIENTS AND MEASUREMENTS: Lay patient representatives of women with PCOS. Participants were asked to identify and rank healthcare and research priorities for their importance. RESULTS: Six hundred and twenty-four lay participants took part in our Delphi method. Over 98% were diagnosed with PCOS (614/624, 98.4%). More than half experienced difficulties to receive a PCOS diagnosis (375/624, 60%), and the majority found it difficult to access specialised PCOS health services in the NHS (594/624, 95%). The top two healthcare priorities included better education for health professionals on the diagnosis and management of PCOS (238/273, 87.1%) and the need to set up specialist PCOS services (234/273, 85.7%). The top two research priorities focused on identifying better treatments for irregular periods (233/273, 85.3%) followed by better tests for early PCOS diagnosis (230/273, 84.2%). CONCLUSIONS: We identified 13 healthcare and 14 research priorities that reflect the current health needs of women with PCOS in the United Kingdom. Adopting these priorities in future healthcare and research planning will help to optimise the health of women with PCOS and increase patient satisfaction.


Assuntos
Síndrome do Ovário Policístico , Humanos , Feminino , Síndrome do Ovário Policístico/terapia , Síndrome do Ovário Policístico/diagnóstico , Medicina Estatal , Técnica Delphi , Pesquisa , Atenção à Saúde
3.
Magn Reson Med ; 89(4): 1496-1505, 2023 04.
Artigo em Inglês | MEDLINE | ID: mdl-36336794

RESUMO

PURPOSE: To extend the MR MultiTasking-based Multidimensional Assessment of Cardiovascular System (MT-MACS) technique with larger spatial coverage and water-fat separation for comprehensive aortocardiac assessment. METHODS: MT-MACS adopts a low-rank tensor image model for 7D imaging, with three spatial dimensions for volumetric imaging, one cardiac motion dimension for cine imaging, one respiratory motion dimension for free-breathing imaging, one T2-prepared inversion recovery time dimension for multi-contrast assessment, and one T2*-decay time dimension for water-fat separation. Nine healthy subjects were recruited for the 3T study. Overall image quality was scored on bright-blood (BB), dark-blood (DB), and gray-blood (GB) contrasts using a 4-point scale (0-poor to 3-excellent) by two independent readers, and their interreader agreement was evaluated. Myocardial wall thickness and left ventricular ejection fraction (LVEF) were quantified on DB and BB contrasts, respectively. The agreement in these metrics between MT-MACS and conventional breath-held, electrocardiography-triggered 2D sequences were evaluated. RESULTS: MT-MACS provides both water-only and fat-only images with excellent image quality (average score = 3.725/3.780/3.835/3.890 for BB/DB/GB/fat-only images) and moderate to high interreader agreement (weighted Cohen's kappa value = 0.727/0.668/1.000/1.000 for BB/DB/GB/fat-only images). There were good to excellent agreements in myocardial wall thickness measurements (intraclass correlation coefficients [ICC] = 0.781/0.929/0.680/0.878 for left atria/left ventricle/right atria/right ventricle) and LVEF quantification (ICC = 0.716) between MT-MACS and 2D references. All measurements were within the literature range of healthy subjects. CONCLUSION: The refined MT-MACS technique provides multi-contrast, phase-resolved, and water-fat imaging of the aortocardiac systems and allows evaluation of anatomy and function. Clinical validation is warranted.


Assuntos
Imageamento Tridimensional , Água , Humanos , Volume Sistólico , Imageamento Tridimensional/métodos , Função Ventricular Esquerda , Ventrículos do Coração , Reprodutibilidade dos Testes , Imageamento por Ressonância Magnética
4.
J Vasc Surg ; 72(5): 1534-1543, 2020 11.
Artigo em Inglês | MEDLINE | ID: mdl-32238310

RESUMO

OBJECTIVE: Aberrant subclavian artery (aSCA) is a rare anatomic variant whose association with other aortic branch variations and aortic pathology has yet to be established. Knowledge of such an association could be relevant to recommendations for screening and awareness as associated variations are important for operative planning. We describe the incidence of aSCA variations, its association with aortic pathology, and a proposed classification system. METHODS: The thoracic cross-sectional imaging database at Keck Hospital of the University of Southern California from 2006 to 2018 was queried for presence of aSCA. Studies were evaluated for aSCA laterality, variant anatomy, and aortic and branch vessel disease. Medical records were reviewed for associated symptoms and diagnoses. The primary outcome was association of aSCA with aortic pathology (aneurysm or dissection). Secondary outcomes were comparison of right and left aSCA, comparison between the sexes, and creation of a proposed classification system. RESULTS: Of 98,580 axial imaging studies, 810 studies (0.82%) were identified with aSCA in 312 unique patients. Right aSCA made up the majority of cases (90.1%). All aSCAs had a retroesophageal course. Kommerell's diverticulum (KD) was present in 184 (59%) with an average diameter of 1.67 cm (range, 1.2-3.3 cm). KD was more frequent (84% vs 56%; P = .0003) and larger (2.05 cm vs 1.61 cm; P < .0001) in left aSCA patients. When present, KD was more often symptomatic in left aSCA compared with right aSCA (77.4% vs 49.1%; P = .005). Dysphagia, chest pain, reflux, and asthma were all more common in left aSCA patients. KD was also more common in men (73.3% vs 50%; P < .0001) and larger in men (1.81 cm vs 1.54 cm; P < .0001) but with no difference in symptoms between sexes. Our proposed classification system based on aortic arch branching is as follows: type 1, left arch with right aSCA (59.9%); type 2, left arch with common carotid trunk and right aSCA (30.1%); type 3, right arch with left aSCA (9.6%); and type 4, right arch with common carotid trunk and left aSCA (0.3%). Subtypes describe the right vertebral artery (RVA) and left vertebral artery (LVA) origin: subclavian (s, RVA 90.1%, LVA 96.8%), carotid (c, RVA 9.6%, LVA 0.3%), or arch (a, RVA 0.3%, LVA 2.9%). Overall, 9.9% (31/312) had associated aortic pathology, although the study was underpowered to detect a difference between right aSCA and left aSCA (9.3% vs 16.1%; P = .213). Type 3 and type 4 arches more often have associated aortic pathology, KD, and symptoms. CONCLUSIONS: aSCAs are frequently symptomatic and commonly associated with aortic dissection and aneurysm. Our proposed classification scheme depicts all four aSCA arch variants and accounts for vertebral artery origin variation. These variants are common, and vertebral anatomy can differ greatly. Knowledge of these anatomic variations is critical to planning for endovascular and open repair of aortic arch pathology.


Assuntos
Variação Anatômica , Aneurisma da Aorta Torácica/epidemiologia , Dissecção Aórtica/epidemiologia , Anormalidades Cardiovasculares/complicações , Artéria Subclávia/anormalidades , Adulto , Idoso , Anatomia Transversal , Dissecção Aórtica/diagnóstico , Dissecção Aórtica/etiologia , Dissecção Aórtica/cirurgia , Aorta Torácica/diagnóstico por imagem , Aorta Torácica/patologia , Aneurisma da Aorta Torácica/diagnóstico , Aneurisma da Aorta Torácica/etiologia , Aneurisma da Aorta Torácica/cirurgia , Anormalidades Cardiovasculares/diagnóstico , Anormalidades Cardiovasculares/epidemiologia , Anormalidades Cardiovasculares/cirurgia , Procedimentos Endovasculares/métodos , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Planejamento de Assistência ao Paciente , Estudos Retrospectivos , Artéria Subclávia/diagnóstico por imagem , Artéria Subclávia/cirurgia , Tomografia Computadorizada por Raios X
5.
J Thorac Imaging ; 35(4): W107-W118, 2020 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-32235186

RESUMO

Left atrial appendage (LAA) mechanical exclusion is being investigated for nonpharmacologic stroke risk reduction in selected patients with atrial fibrillation. There are multiple potential approaches in various stages of development and clinical application, each of which depends on specific cardiothoracic anatomic characteristics for optimal performance. Multiple imaging modalities can be utilized for application of this technology, with transesophageal echocardiography used for intraprocedural guidance. Cardiovascular computed tomographic angiography can act as a virtual patient avatar, allowing for the assessment of cardiac structures in the context of surrounding cardiac, coronary vascular, thoracic vascular, and visceral and skeletal anatomy, aiding preprocedural decision-making, planning, and follow-up. Although transesophageal echocardiography is used for intraprocedural guidance, computed tomographic angiography may be a useful adjunct for preprocedure assessment of LAA sizing and anatomic obstacles or contraindications to deployment, aiding in the assessment of optimal approaches. Potential approaches to LAA exclusion include endovascular occlusion, epicardial ligation, primary minimally invasive intercostal thoracotomy with thoracoscopic LAA ligation or appendectomy, and minimally invasive or open closure as part of cardiothoracic surgery for other indications. The goals of these procedures are complete isolation or exclusion of the entire appendage without leaving a residual appendage stump or residual flow with avoidance of acute or chronic damage to surrounding cardiovascular structures. The cardiovascular imager plays an important role in the preprocedural and postprocedural assessment of the patient undergoing LAA exclusion.


Assuntos
Apêndice Atrial/diagnóstico por imagem , Apêndice Atrial/cirurgia , Fibrilação Atrial/cirurgia , Protocolos Clínicos , Angiografia por Tomografia Computadorizada/métodos , Dispositivo para Oclusão Septal , Humanos
6.
Radiographics ; 38(7): 1949-1972, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30312138

RESUMO

Treatment of thoracic aortic dissection remains highly challenging and is rapidly evolving. Common classifications of thoracic aortic dissection include the Stanford classification (types A and B) and the DeBakey classification (types I to III), as well as a new supplementary classification geared toward endovascular decision making. By using various imaging techniques, the extent of the dissection, the location of the primary intimal tear, the shape of the aortic arch, and the zonal involvement of the aortic arch-factors that affect the treatment strategy-can easily be identified. Thoracic endovascular aortic repair (TEVAR) is generally performed in two groups of patients: (a) those with a surgically repaired type A dissection, and (b) those with a complicated type B dissection. Several imaging findings can help predict the course of remodeling of the dissected aorta after a repaired type A dissection and TEVAR. A spectrum of imaging findings exist with regard to favorable (positive) or failing (negative) remodeling. A schematic model with imaging support allows the classification of important causes of failing remodeling into proximal and distal groups, on the basis of the origin of the refilling of the false lumen and the underlying pathophysiology of pressurization. Refilling of the false lumen of the aorta after repair of a type A dissection is usually secondary to a persistent intimal tear at the aortic arch, a leak of the distal graft anastomosis, or refilling from the false lumen of a dissected aortic arch vessel. After TEVAR, false lumen refilling is most commonly due to an incomplete seal of the proximal landing related to the aortic tortuosity, an arch branch stump, a supra-arch chimney stent, or the TEVAR technique. Online supplemental material is available for this article. ©RSNA, 2018.


Assuntos
Aneurisma da Aorta Torácica/diagnóstico por imagem , Aneurisma da Aorta Torácica/cirurgia , Dissecção Aórtica/diagnóstico por imagem , Dissecção Aórtica/cirurgia , Procedimentos Endovasculares , Dissecção Aórtica/classificação , Aneurisma da Aorta Torácica/classificação , Humanos , Resultado do Tratamento
7.
J Orthop Sports Phys Ther ; 48(1): 51, 2018 01.
Artigo em Inglês | MEDLINE | ID: mdl-29291284

RESUMO

A 15-year-old baseball pitcher presented to physical therapy with a 1-week history of acute right shoulder pain experienced during the acceleration phase of throwing. The week after physical therapy evaluation, the patient returned with radiographs and magnetic resonance imaging results that revealed lateral physeal widening with adjacent edema. Additionally, a nondisplaced labral tear was noted. In this case, imaging was necessary for an accurate diagnosis of lateral physeal widening, commonly referred to as "Little League shoulder." J Orthop Sports Phys Ther 2018;48(1):51. doi:10.2519/jospt.2018.7369.


Assuntos
Beisebol/lesões , Epifise Deslocada/diagnóstico por imagem , Lesões do Ombro , Articulação do Ombro/diagnóstico por imagem , Adolescente , Artralgia/etiologia , Edema/etiologia , Epifise Deslocada/complicações , Epifise Deslocada/terapia , Humanos , Imageamento por Ressonância Magnética , Masculino , Modalidades de Fisioterapia , Radiografia
9.
Ann Vasc Surg ; 42: 305.e7-305.e12, 2017 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-28389290

RESUMO

Thoracic endovascular aortic repair (TEVAR) can be complicated by graft collapse, endoleaks, and stent migration. The incidence of these complications and other outcomes is poorly understood in young trauma victims who receive endovascular aortic repair of blunt thoracic aortic injury (BTAI). A 29-year-old pedestrian was struck by a vehicle resulting in polytrauma including BTAI with transection distal to the left subclavian artery origin. The patient underwent successful TEVAR. Nine months later, the patient developed transient paresthesia below the waist that progressed to bilateral lower extremity paralysis and malperfusion syndrome below the diaphragm including nonpalpable pulses in the lower extremities, acute renal failure, and ischemic colitis. Imaging demonstrated near occlusive thrombosis of the distal end of the thoracic endograft. An emergent axillobifemoral bypass resolved the organ malperfusion and acute limb ischemia. Patients who have undergone TEVAR for BTAI may develop asymptomatic or symptomatic intragraft thrombosis. In patients presenting with malperfusion syndrome below the diaphragm, extra-anatomic bypass can expeditiously resolve symptoms until definitive treatment can be performed. Oversizing of thoracic stents in trauma patient may lead to intragraft thrombosis.


Assuntos
Aorta Torácica/cirurgia , Implante de Prótese Vascular/instrumentação , Prótese Vascular , Procedimentos Endovasculares/instrumentação , Oclusão de Enxerto Vascular/etiologia , Isquemia/etiologia , Stents , Trombose/etiologia , Lesões do Sistema Vascular/cirurgia , Ferimentos não Penetrantes/cirurgia , Acidentes de Trânsito , Adulto , Aorta Torácica/diagnóstico por imagem , Aorta Torácica/lesões , Aortografia/métodos , Implante de Prótese Vascular/efeitos adversos , Angiografia por Tomografia Computadorizada , Procedimentos Endovasculares/efeitos adversos , Oclusão de Enxerto Vascular/diagnóstico por imagem , Oclusão de Enxerto Vascular/cirurgia , Humanos , Isquemia/diagnóstico por imagem , Isquemia/cirurgia , Masculino , Pedestres , Desenho de Prótese , Trombose/diagnóstico por imagem , Trombose/cirurgia , Resultado do Tratamento , Lesões do Sistema Vascular/diagnóstico por imagem , Lesões do Sistema Vascular/etiologia , Ferimentos não Penetrantes/diagnóstico por imagem , Ferimentos não Penetrantes/etiologia
10.
PLoS One ; 12(3): e0174166, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-28323905

RESUMO

OBJECTIVE: High sensitivity plasma cardiac troponin-I (cTnI) is emerging as a strong predictor of cardiac events in a variety of settings. We have explored its utility in patients with myotonic dystrophy type 1 (DM1). METHODS: 117 patients with DM1 were recruited from routine outpatient clinics across three health boards. A single measurement of cTnI was made using the ARCHITECT STAT Troponin I assay. Demographic, ECG, echocardiographic and other clinical data were obtained from electronic medical records. Follow up was for a mean of 23 months. RESULTS: Fifty five females and 62 males (mean age 47.7 years) were included. Complete data were available for ECG in 107, echocardiography in 53. Muscle Impairment Rating Scale score was recorded for all patients. A highly significant excess (p = 0.0007) of DM1 patients presented with cTnI levels greater than the 99th centile of the range usually observed in the general population (9 patients; 7.6%). Three patients with elevated troponin were found to have left ventricular systolic dysfunction (LVSD), compared with four of those with normal range cTnI (33.3% versus 3.7%; p = 0.001). Sixty two patients had a cTnI level < 5ng/L, of whom only one had documented evidence of LVSD. Elevated cTnI was not predictive of severe conduction abnormalities on ECG, or presence of a cardiac device, nor did cTnI level correlate with muscle strength expressed by Muscle Impairment Rating Scale score. CONCLUSIONS: Plasma cTnI is highly elevated in some ambulatory patients with DM1 and shows promise as a tool to aid cardiac risk stratification, possibly by detecting myocardial involvement. Further studies with larger patient numbers are warranted to assess its utility in this setting.


Assuntos
Miocárdio/patologia , Distrofia Miotônica/patologia , Troponina I/sangue , Disfunção Ventricular Esquerda/sangue , Disfunção Ventricular Esquerda/diagnóstico , Adulto , Idoso , Biomarcadores/sangue , Ecocardiografia , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Miotonina Proteína Quinase/genética , Adulto Jovem
11.
Vascular ; 25(5): 451-458, 2017 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-28118783

RESUMO

Background Computerized tomography angiography is an important tool for endovascular repair of abdominal aortic aneurysm planning. This study aimed to evaluate the utility of computerized tomography angiography using direct intra-aortic injection of low volume of iodinated contrast in patients being considered for endovascular repair of abdominal aortic aneurysm. Design A 5F high-flow angiographic catheter was positioned in the mid-thoracic aorta through femoral percutaneous access. Computerized tomography angiography was performed with image acquisition starting simultaneously with power injection of 10 mL of iopamidol 76% diluted with normal saline to 50 mL, at 6 mL/s through the angiographic catheter. Aortic enhancement was assessed with a circular region-of-interest within the aorta. Values ≥150 HU were considered adequate. Results Nine patients with abdominal aortic aneurysm and chronic renal dysfunction underwent computerized tomography angiography with intra-arterial contrast injection for endovascular repair of abdominal aortic aneurysm planning. Mean age 78.8 ± 5.0 years, mean baseline creatinine 1.8 ± 0.4 mg/dL and mean glomerular filtration rate 36.8 ± 4.2 mL/min. Adequate computerized tomography angiography aortic enhancement was obtained in all cases (mean intra-aortic density: 300.4 ± 72.8 HU). The total contrast volume used for the computerized tomography angiography and the endovascular repair of abdominal aortic aneurysm combined was 30.0 ± 21.4 mL. Seventy-five percent of the patients received ≤30 mL of contrast. No significant renal function deterioration occurred (creatinine clearance pre- and post-procedure 36.8 ± 4.2 mL/min versus 38.6 ± 6.1 mL/min, p = 0.32). Successful endovascular repair of abdominal aortic aneurysm was achieved in eight patients. One patient underwent open treatment based on computerized tomography angiography information. No endoleak or aneurysm growth occurred (median follow-up: 121 days (range 36-1242)). Conclusion Direct intra-aortic injection of low volume of iodinated contrast provides adequate and reproducible pattern of aortic enhancement on computerized tomography angiography. The computerized tomography angiography so acquired can be used for endovascular repair of abdominal aortic aneurysm limiting the total volume of iodinated contrast required.


Assuntos
Aneurisma da Aorta Abdominal/diagnóstico por imagem , Aneurisma da Aorta Abdominal/cirurgia , Aortografia/métodos , Implante de Prótese Vascular , Angiografia por Tomografia Computadorizada , Meios de Contraste/administração & dosagem , Procedimentos Endovasculares , Iopamidol/administração & dosagem , Tomografia Computadorizada Multidetectores , Idoso , Idoso de 80 Anos ou mais , Aneurisma da Aorta Abdominal/complicações , Implante de Prótese Vascular/efeitos adversos , Procedimentos Endovasculares/efeitos adversos , Feminino , Humanos , Injeções Intra-Arteriais , Masculino , Valor Preditivo dos Testes , Insuficiência Renal Crônica/complicações , Reprodutibilidade dos Testes , Estudos Retrospectivos , Fatores de Risco , Resultado do Tratamento
12.
World J Pediatr Congenit Heart Surg ; 4(2): 142-54, 2013 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-23799727

RESUMO

Cardiovascular computed tomographic angiography (CCTA) provides an understanding of the three-dimensional (3D) coronary artery anatomy in relation to cardiovascular thoracic structures important to the surgical management of anomalous coronary arteries (ACAs). Although some ACA variants are not clinically significant, others can lead to ischemia/infarction, related acute ventricular dysfunction, ventricular arrhythmias, and sudden cardiac death. The CCTA is important to surgical decision making, as it provides noninvasive visualization of the coronary arteries with (1) assessment of origin, course, and termination of coronary artery anomalies in the context of 3D thoracic anatomy, (2) characterization of anatomy helpful for differentiation of benign versus hemodynamically significant variants, (3) identification of other cardiothoracic anomalies, and (4) detection of coronary artery disease. High-risk ACA anatomy in the appropriate clinical setting can require surgical intervention with decisions including minimally invasive versus open sternotomy approach, correction via reimplantation of a coronary artery, alteration of the ACA course without reimplantation, or bypass of an ACA. Given the rarity of ACA, there is limited data in the literature, and significant controversy related to the management issues. The management of ACA requires comprehensive clinical history, thorough assessment of cardiac function, and detailed anatomic imaging. Future studies will need to address the long-term outcome based on detailed assessment of original anatomy and surgical approach.


Assuntos
Angiografia Coronária/métodos , Anomalias dos Vasos Coronários/diagnóstico por imagem , Vasos Coronários/diagnóstico por imagem , Imageamento Tridimensional/métodos , Tomografia Computadorizada por Raios X/métodos , Procedimentos Cirúrgicos Cardiovasculares , Humanos
13.
Int J Cardiol ; 167(6): 2867-74, 2013 Sep 10.
Artigo em Inglês | MEDLINE | ID: mdl-22882965

RESUMO

OBJECTIVES: To investigate the role of collateral venous pathways between the left brachiocephalic vein (LBV) and the left atrium through an arcade comprising the left superior intercostal vein (LSICV), left vertical vein (LVV), and pulmonary veins as a potential cause of paradoxical embolism. METHODS: A retrospective search was performed to find symptomatic patients with negative work up for paradoxical emboli whose chest CT or MR angiography by left arm contrast injection showed a visible right to left shunt through the LSICV/LVV collateral pathway (symptomatic group). We also evaluated the characteristics of this collateral pathway in 150 chest CT angiographies from general referrals (comparison group). RESULTS: We found 7 symptomatic patients. Initial presentations included neurological symptoms in all patients and episodes of hypoxemia in three patients. Communications between the LBV and left atrium through the LSICV/LVV pathway was seen is all 7 cases and confirmed by transesophageal echocardiography in two. An abnormal LBV was seen in 6 cases (absence in one, stenosis in 5). Moderate superior vena cava stenosis was seen in one and acute right subclavian vein thrombosis in one. Six patients had past history of an upper extremity central line placement. In the comparison group, LSICV was seen in 73 (48%), connecting to the hemiazygos vein in 41, to a LVV in 19 and to the left atrium in 7. CONCLUSION: LSICV/LVV collaterals are common and can be a potential route for paradoxical emboli especially when stenosis of a draining vein such as LBV exists.


Assuntos
Circulação Colateral/fisiologia , Embolia Paradoxal/diagnóstico por imagem , Músculos Intercostais/irrigação sanguínea , Malformações Vasculares/diagnóstico por imagem , Adolescente , Adulto , Idoso , Feminino , Átrios do Coração/diagnóstico por imagem , Humanos , Masculino , Pessoa de Meia-Idade , Radiografia , Estudos Retrospectivos , Ultrassonografia
14.
Hum Mol Genet ; 21(16): 3558-67, 2012 Aug 15.
Artigo em Inglês | MEDLINE | ID: mdl-22595968

RESUMO

Deciphering the contribution of genetic instability in somatic cells is critical to our understanding of many human disorders. Myotonic dystrophy type 1 (DM1) is one such disorder that is caused by the expansion of a CTG repeat that shows extremely high levels of somatic instability. This somatic instability has compromised attempts to measure intergenerational repeat dynamics and infer genotype-phenotype relationships. Using single-molecule PCR, we have characterized more than 17 000 de novo somatic mutations from a large cohort of DM1 patients. These data reveal that the estimated progenitor allele length is the major modifier of age of onset. We find no evidence for a threshold above which repeat length does not contribute toward age at onset, suggesting pathogenesis is not constrained to a simple molecular switch such as nuclear retention of the DMPK transcript or haploinsufficiency for DMPK and/or SIX5. Importantly, we also show that age at onset is further modified by the level of somatic instability; patients in whom the repeat expands more rapidly, develop the symptoms earlier. These data establish a primary role for somatic instability in DM1 severity, further highlighting it as a therapeutic target. In addition, we show that the level of instability is highly heritable, implying a role for individual-specific trans-acting genetic modifiers. Identifying these trans-acting genetic modifiers will facilitate the formulation of novel therapies that curtail the accumulation of somatic expansions and may provide clues to the role these factors play in the development of cancer, aging and inherited disease in the general population.


Assuntos
Distrofia Miotônica/etiologia , Distrofia Miotônica/genética , Característica Quantitativa Herdável , Expansão das Repetições de Trinucleotídeos , Idade de Início , Idoso , Alelos , Estudos de Associação Genética , Instabilidade Genômica , Haploinsuficiência/genética , Proteínas de Homeodomínio/genética , Humanos , Pessoa de Meia-Idade , Distrofia Miotônica/epidemiologia , Miotonina Proteína Quinase , Proteínas Serina-Treonina Quinases/genética
15.
Radiographics ; 32(2): 453-68, 2012.
Artigo em Inglês | MEDLINE | ID: mdl-22411942

RESUMO

The clinical manifestations of coronary artery anomalies vary in severity, with some anomalies causing severe symptoms and cardiovascular sequelae and others being benign. Cardiovascular computed tomography (CT) has emerged as the standard of reference for identification and characterization of coronary artery anomalies. Therefore, it is important for the reader of cardiovascular CT images to be thoroughly familiar with the spectrum of coronary artery anomalies. Hemodynamically significant anomalies include atresia, origin from the pulmonary artery, interarterial course, and congenital fistula. Non-hemodynamically significant anomalies include duplication; high origin; a prepulmonic, transseptal, or retroaortic course; shepherd's crook right coronary artery; and systemic termination. In general, coronary arteries with an interarterial course are associated with an increased risk of sudden cardiac death. Coronary artery anomalies that result in shunting, including congenital fistula and origin from the pulmonary artery, are also commonly symptomatic and may cause steal of blood from the myocardium. Radiologists should be familiar with each specific variant and its specific constellation of potential implications.


Assuntos
Angiografia Coronária/métodos , Anomalias dos Vasos Coronários/diagnóstico por imagem , Tomografia Computadorizada por Raios X/métodos , Adulto , Fístula Artério-Arterial/congênito , Fístula Artério-Arterial/diagnóstico por imagem , Implante de Prótese Vascular , Anomalias dos Vasos Coronários/classificação , Anomalias dos Vasos Coronários/diagnóstico , Anomalias dos Vasos Coronários/fisiopatologia , Anomalias dos Vasos Coronários/cirurgia , Feminino , Fístula/congênito , Fístula/diagnóstico por imagem , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias/diagnóstico por imagem , Hemodinâmica , Humanos , Imageamento Tridimensional , Masculino , Pessoa de Meia-Idade , Artéria Pulmonar/anormalidades , Fístula Vascular/congênito , Fístula Vascular/diagnóstico por imagem , Adulto Jovem
16.
AJR Am J Roentgenol ; 198(3): 496-504, 2012 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-22357988

RESUMO

OBJECTIVE: Myocardial infarctions (MIs) are frequently evident on routine chest or abdominal CT, even when studies are not performed for cardiac-specific indications. However, the telltale signs of an MI may be easily overlooked. Herein, we present the spectrum of appearances of MIs, including areas of fat attenuation, myocardial calcifications, focal areas of wall thinning or aneurysm formation, and perfusion abnormalities. Thrombi, especially when present at the apex of the left ventricle, may also suggest an MI. CONCLUSION: The increased use of CT in the evaluation of patients for a variety of indications gives the radiologist the unique opportunity to recognize findings consistent with MI in patients who may not have a prior diagnosis of ischemic heart disease.


Assuntos
Técnicas de Imagem de Sincronização Cardíaca/métodos , Imageamento por Ressonância Magnética/métodos , Infarto do Miocárdio/diagnóstico por imagem , Tomografia Computadorizada por Raios X/métodos , Calcinose/diagnóstico por imagem , Calcinose/patologia , Trombose Coronária/diagnóstico por imagem , Trombose Coronária/patologia , Aneurisma Cardíaco/diagnóstico por imagem , Aneurisma Cardíaco/patologia , Humanos , Achados Incidentais , Lipomatose/diagnóstico por imagem , Lipomatose/patologia , Metaplasia , Infarto do Miocárdio/patologia
17.
World J Pediatr Congenit Heart Surg ; 3(1): 123-9, 2012 Jan 01.
Artigo em Inglês | MEDLINE | ID: mdl-23804696

RESUMO

Submitted May 6, 2011; Accepted August 3, 2011. The survival into adulthood of patients with unoperated complex congenital heart disease with anomalies often considered life threatening in infancy and childhood requires a complex interplay of "balanced" defects allowing for cardiovascular physiology compatible with long-term survival. We report on a series of three cases from our advanced imaging database of middle-aged adults presenting with multiple similar defects providing a hemodynamically balanced circulation. The constellation of defects seen in each of these patients included congenitally corrected transposition of the great arteries, a large nonrestrictive ventricular septal defect, valvular pulmonary stenosis, and in two cases anomalous coronary arteries. Cardiovascular computed tomographic angiography (CCTA) and cardiovascular magnetic resonance imaging (CMR) were important to the characterization of the multiple defects and their three-dimensional relationships in these cases. Treatment decisions in patients with this constellation of findings are challenging, given the limited data due to the rarity of survival of patients with these defects into middle adulthood and the paucity of data related to decisions and approaches to medical management, surgical correction, or transplantation.

18.
JACC Cardiovasc Imaging ; 4(12): 1320-3, 2011 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-22172789

RESUMO

In this paper we discuss 2 unprecedented examples of coronary ostial atresia in adults. The first case documents a case of left main ostial agenesis with intercoronary communication through a retroaortic Kugel anastomotic collateral. The second case describes a right coronary artery (RCA) ostial agenesis with a preconal intercoronary anastomosis in association with supravalvular pulmonary stenosis. While atresia of the left coronary artery ostium is a rare anatomic variant of the coronary circulation, atresia of the right coronary ostium is exceedingly rare and not reported in adults (1). Correlative images of coronary computed tomography angiography (CTA) using a 64-slice multidetector computed tomography and coronary catheterization angiograms are presented for both cases, and the arterial collateral pathways between the proximal right and left coronary systems are discussed. We also reviewed the differences between congenital and developmental forms of abnormalities.


Assuntos
Angiografia Coronária/métodos , Circulação Coronária , Anomalias dos Vasos Coronários/diagnóstico por imagem , Tomografia Computadorizada por Raios X , Idoso , Anomalias dos Vasos Coronários/fisiopatologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estenose da Valva Pulmonar/diagnóstico por imagem , Estenose da Valva Pulmonar/fisiopatologia
19.
J Vasc Surg ; 54(1): 30-40; discussion 40-1, 2011 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-21334163

RESUMO

OBJECTIVE: We report a single-center experience using the hybrid procedure, consisting of open debranching, followed by endovascular aortic repair, for treatment of arch/proximal descending thoracic/thoracoabdominal aortic aneurysms (TAAA). METHODS: From 2005 to 2010, 51 patients (33 men; mean age, 70 years) underwent a hybrid procedure for arch/proximal descending thoracic/TAAA. The 30-day and in-hospital morbidity and mortality rates, and late endoleak, graft patency, and survival were analyzed. Graft patency was assessed by computed tomography, angiography, or duplex ultrasound imaging. RESULTS: Hybrid procedures were used to treat 27 thoracic (16 arch, 11 proximal descending thoracic) and 24 TAAA (Crawford/Safi types I to III: 3; type IV: 12; type V: 9). The hybrid procedure involved debranching 47 arch vessels or 77 visceral/renal vessels using bypass grafts, followed by endovascular repair. Seventy-five percent of debranching and endovascular repair procedures were staged, with an average interval of 28 days. Major 30-day and in-hospital complications occurred in 39% of patients and included bypass graft occlusion in four, endoleak reintervention in two, and paraplegia in one. Mortality was 3.9%. During a mean follow-up of 13 months, three additional type II endoleaks required intervention, and one bypass graft occluded. No aneurysm rupture occurred during follow-up. Primary bypass graft patency was 95.3%. Actuarial survival was 86% at 1 year and 67% at 3 years. CONCLUSION: The hybrid procedure is associated with acceptable rates of mortality and paraplegia when used for treatment of arch/proximal descending thoracic/TAAA. These results support this procedure as a reasonable approach to a difficult surgical problem; however, longer follow-up is required to appraise its ultimate clinical utility.


Assuntos
Aneurisma da Aorta Torácica/cirurgia , Implante de Prótese Vascular , Procedimentos Endovasculares , Idoso , Idoso de 80 Anos ou mais , Aneurisma da Aorta Torácica/diagnóstico por imagem , Aneurisma da Aorta Torácica/mortalidade , Aortografia/métodos , Implante de Prótese Vascular/efeitos adversos , Implante de Prótese Vascular/mortalidade , Endoleak/etiologia , Endoleak/cirurgia , Procedimentos Endovasculares/efeitos adversos , Procedimentos Endovasculares/mortalidade , Feminino , Oclusão de Enxerto Vascular/etiologia , Oclusão de Enxerto Vascular/cirurgia , Mortalidade Hospitalar , Humanos , Estimativa de Kaplan-Meier , Los Angeles , Masculino , Pessoa de Meia-Idade , Paraplegia/etiologia , Reoperação , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Taxa de Sobrevida , Fatores de Tempo , Tomografia Computadorizada por Raios X , Resultado do Tratamento , Ultrassonografia Doppler Dupla , Grau de Desobstrução Vascular
20.
World J Radiol ; 2(4): 143-7, 2010 Apr 28.
Artigo em Inglês | MEDLINE | ID: mdl-21160580

RESUMO

The authors report herein a series of 3 patients with caseous mitral annular calcifications (MAC). One of the patients presented with mass-like, caseous MAC as an incidental finding on a staging computed tomography (CT) for metastatic colorectal carcinoma. Another patient presented with a nodule on a chest radiograph, which was later found on CT to be due to caseous MAC. In the third patient, caseous MAC was initially detected on echocardiography, and was further evaluated with CT and cardiac magnetic resonance imaging. In all three patients, the appearances posed a diagnostic dilemma. The appearance of caseous MAC is dissimilar to non-caseous MAC and is usually seen as an ovoid, mass-like structure, with homogeneous hyperattenuation, representing a liquefied form of calcium and proteinaceous fluid. This homogeneous center is surrounded by peripheral, shell-like calcifications. Caseous MAC is likely an under-recognized entity and may present a diagnostic dilemma at CT, magnetic resonance imaging, or echocardiography.

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