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Background The role of arterial load in severe aortic stenosis is increasingly recognized. However, patterns of pulsatile load and their implications in this population are unknown. We aimed to assess the relationship between the arterial properties and both (1) left ventricular remodeling and fibrosis and (2) the clinical course of patients with severe aortic stenosis undergoing aortic valve replacement ( AVR ). Methods and Results We enrolled 38 participants with symptomatic severe aortic stenosis scheduled to undergo surgical AVR . Aortic root characteristic impedance, wave reflections parameters (reflection magnitude, reflected wave transit time), and myocardial extracellular mass were measured with cardiac magnetic resonance imaging and arterial tonometry Cardiac magnetic resonance imaging was repeated at 6 months in 30 participants. A reduction in cellular mass (133.6 versus 113.9 g; P=0.002) but not extracellular mass (42.3 versus 40.6 g; P=0.67) was seen after AVR . Participants with higher extracellular mass exhibited greater reflection magnitude (0.68 versus 0.54; P=0.006) and lower aortic root characteristic impedance (56.3 versus 96.9 dynes/s per cm5; P=0.006). Reflection magnitude was a significant predictor of smaller improvement in the quality of life (Kansas City Cardiomyopathy Questionnaire score) after AVR ( R=-0.51; P=0.0026). The 6-minute walk distance at 6 months after AVR was positively correlated with the reflected wave transit time ( R=0.52; P=0.01). Conclusions Consistent with animal studies, arterial wave reflections are associated with interstitial volume expansion in severe aortic stenosis and predict a smaller improvement in quality of life following AVR . Future trials should assess whether wave reflections represent a potential therapeutic target to mitigate myocardial interstitial remodeling and to improve the clinical status of this patient population.
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Estenosis de la Válvula Aórtica/diagnóstico , Arterias/diagnóstico por imagen , Ventrículos Cardíacos/diagnóstico por imagen , Miocardio/patología , Rigidez Vascular/fisiología , Función Ventricular Izquierda/fisiología , Remodelación Ventricular , Anciano , Estenosis de la Válvula Aórtica/fisiopatología , Arterias/fisiopatología , Presión Sanguínea/fisiología , Femenino , Fibrosis/patología , Ventrículos Cardíacos/fisiopatología , Humanos , Imagen por Resonancia Cinemagnética/métodos , Masculino , Pronóstico , Estudios Prospectivos , Índice de Severidad de la EnfermedadRESUMEN
Hyperleukocytic acute myeloid leukemia (AML) is associated with pulmonary complications and high early mortality rate, but given its rarity, data on chest radiographic presentation are scarce.We retrospectively reviewed the charts of 73 AML patients admitted with white blood cell count >100â×â10/L between 2003 and 2014 in order to describe the chest radiographic and computed tomography (CT) findings and to correlate them with AML subtype and respiratory symptoms.Forty-two of the 73 patients (58%) overall and 36 of the 54 patients (67%) with clinical signs of pulmonary leukostasis had abnormal radiographs on admission. The presence of radiographic abnormalities was significantly associated with dyspnea and oxygen/ventilatory support requirements (Pâ<â0.01) and with day 28 mortality (45% vs 13%, Pâ=â0.005) but not with monocytic subtype of AML. Sixteen patients had isolated focal basilar airspace opacities, unilateral (nâ=â13) or bilateral (nâ=â3), while 16 patients had bilateral diffuse opacities, interstitial (nâ=â12) or airspace and interstitial (nâ=â4). Two patients had isolated pleural effusion, 2 patients had unilateral midlung airspace opacities, and 6 patients had a combination of focal airspace and diffuse interstitial opacities. Overall, 2 patterns accounted for 75% of abnormal findings: bilateral diffuse opacities tended to be associated with monocytic AML, whereas basilar focal airspace opacities were more frequent in nonmonocytic AML (Pâ<â0.05). Eighteen patients had CT scans, revealing interlobular septal thickening (nâ=â12), airspace (nâ=â11) and ground-glass (nâ=â9) opacities, pleural effusions (nâ=â12), and acute pulmonary embolism (nâ=â2).Hyperleukocytic AML is frequently associated with abnormal chest radiographs, involving mostly focal basilar airspace opacities (more frequent in nonmonocytic AML) or diffuse bilateral opacities. CT scan should be considered broadly due to the suboptimal resolution of radiographs for detecting signs of leukostasis.
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Leucemia Mieloide Aguda/diagnóstico por imagen , Tomografía Computarizada por Rayos X , Adulto , Anciano , Anciano de 80 o más Años , Estudios de Cohortes , Femenino , Humanos , Leucemia Mieloide Aguda/sangre , Leucocitos , Masculino , Persona de Mediana Edad , Radiografía Torácica , Estudios Retrospectivos , Adulto JovenAsunto(s)
Adenocarcinoma/genética , Neoplasias Encefálicas/genética , Neoplasias Pulmonares/genética , Polimorfismo de Nucleótido Simple , Proteínas Tirosina Quinasas Receptoras/genética , Adenocarcinoma/diagnóstico por imagen , Adenocarcinoma/tratamiento farmacológico , Adenocarcinoma/secundario , Anciano , Quinasa de Linfoma Anaplásico , Neoplasias Encefálicas/diagnóstico por imagen , Neoplasias Encefálicas/tratamiento farmacológico , Neoplasias Encefálicas/secundario , Proteínas de Ciclo Celular/genética , Codón , Crizotinib , Proteínas de Unión al ADN/genética , Femenino , Genómica , N-Metiltransferasa de Histona-Lisina/genética , Humanos , Neoplasias Pulmonares/diagnóstico por imagen , Neoplasias Pulmonares/tratamiento farmacológico , Neoplasias Pulmonares/patología , MAP Quinasa Quinasa 4/genética , Proteínas Asociadas a Microtúbulos/genética , Proteínas Nucleares/genética , Inhibidores de Proteínas Quinasas/uso terapéutico , Pirazoles/uso terapéutico , Piridinas/uso terapéutico , Radiografía , Ribonucleoproteínas/genética , Serina Endopeptidasas/genética , Factor de Empalme U2AF , Factores de Transcripción/genética , Translocación Genética/genética , Resultado del TratamientoRESUMEN
Bicuspid pulmonary valves and pulmonary artery aneurysms are two rare entities, reported in association, and usually attributed to hemodynamic alterations caused by the bicuspid pulmonary valve. We present magnetic resonance images of a patient with a bicuspid pulmonary valve and pulmonary artery aneurysm, and propose an alternative mechanism for this association, based on recent embryologic studies that link anomalies of the semilunar valves and great vessels with derangement of the cardiac neural crest cell development.
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OBJECTIVE: Curative surgery is not an option for many patients with clinical stage I non-small-cell lung carcinoma (NSCLC), but radical radiosurgery may be effective. METHODS: Inoperable patients with small peripheral clinical stage I NSCLC were enrolled in this study. Three-to-five fiducial markers were implanted in or near tumors under CT guidance. Gross tumor volumes (GTVs) were contoured using lung windows. The GTV margin was expanded by 5 mm to establish the planning treatment volume (PTV). A dose of 42-60 Gy was delivered to the PTV in 3 equal fractions in less than 2 weeks using the CyberKnife radiosurgery system. The 30-Gy isodose contour extended at least 1 cm from the GTV. Physical examination, CT imaging and pulmonary function testing were completed at 6 months intervals for three years following treatment. RESULTS: Twenty patients with an average maximum tumor diameter of 2.2 cm (range, 1.1 - 3.5 cm) and a mean FEV1 of 1.08 liters (range, 0.53 - 1.71 L) were treated. Pneumothorax requiring tube thoracostomy occurred following CT-guided fiducial placement in 25% of the patients. All patients completed treatment with few acute side effects and no procedure-related mortality. Transient chest wall discomfort developed in 8 of the 12 patients with lesions within 5 mm of the pleura. The mean percentage of the total lung volume receiving a minimum of 15 Gy was 7.3% (range, 2.4% to 11.3%). One patient who received concurrent gefitinib developed short-lived, grade III radiation pneumonitis. The mean percent predicted DLCO decreased by 9% and 11% at 6 and 12 months, respectively. There were no local failures, regional lymph node recurrences or distant metastases. With a median follow-up of 25 months for the surviving patients, Kaplan-Meier overall survival estimate at 2 years was 87%, with deaths due to COPD progression. CONCLUSION: Radical CyberKnife radiosurgery is a well-tolerated treatment option for inoperable patients with small, peripheral stage I NSCLC. Effective doses and adequate margins are likely to have contributed to the optimal early local control seen in this study.
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Carcinoma de Pulmón de Células no Pequeñas/cirugía , Neoplasias Pulmonares/cirugía , Radiocirugia/métodos , Anciano , Anciano de 80 o más Años , Carcinoma de Pulmón de Células no Pequeñas/patología , Femenino , Estudios de Seguimiento , Humanos , Terapia por Láser , Neoplasias Pulmonares/patología , Masculino , Persona de Mediana Edad , Monitoreo Fisiológico/métodos , Estadificación de Neoplasias , Complicaciones Posoperatorias/epidemiología , Radiocirugia/efectos adversos , Insuficiencia del Tratamiento , Resultado del Tratamiento , Carga TumoralRESUMEN
BACKGROUND: Recent developments in radiotherapeutic technology have resulted in a new approach to treating patients with localized lung cancer. We report preliminary clinical outcomes using stereotactic radiosurgery with real-time tumor motion tracking to treat small peripheral lung tumors. METHODS: Eligible patients were treated over a 24-month period and followed for a minimum of 6 months. Fiducials (3-5) were placed in or near tumors under CT-guidance. Non-isocentric treatment plans with 5-mm margins were generated. Patients received 45-60 Gy in 3 equal fractions delivered in less than 2 weeks. CT imaging and routine pulmonary function tests were completed at 3, 6, 12, 18, 24 and 30 months. RESULTS: Twenty-four consecutive patients were treated, 15 with stage I lung cancer and 9 with single lung metastases. Pneumothorax was a complication of fiducial placement in 7 patients, requiring tube thoracostomy in 4. All patients completed radiation treatment with minimal discomfort, few acute side effects and no procedure-related mortalities. Following treatment transient chest wall discomfort, typically lasting several weeks, developed in 7 of 11 patients with lesions within 5 mm of the pleura. Grade III pneumonitis was seen in 2 patients, one with prior conventional thoracic irradiation and the other treated with concurrent Gefitinib. A small statistically significant decline in the mean % predicted DLCO was observed at 6 and 12 months. All tumors responded to treatment at 3 months and local failure was seen in only 2 single metastases. There have been no regional lymph node recurrences. At a median follow-up of 12 months, the crude survival rate is 83%, with 3 deaths due to co-morbidities and 1 secondary to metastatic disease. CONCLUSION: Radical stereotactic radiosurgery with real-time tumor motion tracking is a promising well-tolerated treatment option for small peripheral lung tumors.
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Neoplasias Pulmonares/radioterapia , Radiocirugia/métodos , Anciano , Anciano de 80 o más Años , Carcinoma de Pulmón de Células no Pequeñas/diagnóstico por imagen , Carcinoma de Pulmón de Células no Pequeñas/patología , Carcinoma de Pulmón de Células no Pequeñas/fisiopatología , Carcinoma de Pulmón de Células no Pequeñas/radioterapia , Fraccionamiento de la Dosis de Radiación , Estudios de Seguimiento , Volumen Espiratorio Forzado , Humanos , Neoplasias Pulmonares/diagnóstico por imagen , Neoplasias Pulmonares/patología , Neoplasias Pulmonares/fisiopatología , Persona de Mediana Edad , Metástasis de la Neoplasia , Estadificación de Neoplasias , Selección de Paciente , Radiocirugia/efectos adversos , Dosificación Radioterapéutica , Planificación de la Radioterapia Asistida por Computador , Tomografía Computarizada por Rayos X , Resultado del TratamientoRESUMEN
PURPOSE: This study examined the complication rates associated with percutaneous fiducial placement for the purpose of stereotactic body radiation therapy of primary and metastatic lung neoplasms. PATIENTS AND METHODS: This is a retrospective review of computed tomography (CT) scans and follow-up chest radiographs of 48 consecutive patients who underwent CT-guided percutaneous fiducial placement. The effect of age, sex, number of fiducials placed, and performance of a concomitant biopsy on the complication rates were assessed. RESULTS: Of 48 patients with a total of 221 fiducials placed, 16 (33%) had a procedure-related pneumothorax. There was no significant difference in pneumothorax rate based on age (P = 0.16), sex (P > 0.99), and number of fiducials placed (P = 0.21). Overall, 6 of 48 patients (12.5%) required a thoracostomy tube. Performance of a concomitant core needle biopsy at the time of fiducial placement was associated with pneumothorax rates of 64% compared with 26% without biopsies (P = 0.03). Postprocedural CT demonstrated hemorrhage in 9 patients (19%). Two patients had hemoptysis; one required admission. Patients' age, sex, number of fiducials placed, and performance of concomitant biopsy had no statistically significant implications on parenchymal hemorrhage incidence. CONCLUSION: Approximately one third of the patients develop a pneumothorax during CT-guided fiducial placement. Most are asymptomatic and do not require a thoracostomy. A concurrent biopsy at the time of fiducial placement is associated with an increased risk of pneumothorax. Hemorrhage occurs but is usually clinically insignificant.
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Neoplasias Pulmonares/cirugía , Radiografía Torácica , Radiocirugia/efectos adversos , Tomografía Computarizada por Rayos X , Adulto , Anciano , Femenino , Humanos , Neoplasias Pulmonares/diagnóstico por imagen , Masculino , Persona de Mediana Edad , Neumotórax/etiología , Radiocirugia/métodos , Estudios RetrospectivosRESUMEN
Proteus syndrome is a sporadic disorder named for its highly variable manifestations. The disease causes tissue overgrowth in a mosaic pattern and may affect tissues derived from any germinal layer. The disease process is not usually apparent at birth but develops rapidly in childhood. Common manifestations include macrodactyly, vertebral abnormalities, asymmetric limb overgrowth and length discrepancy, hyperostosis, abnormal and asymmetric fat distribution, asymmetric muscle development, connective-tissue nevi, and vascular malformations. The features of Proteus syndrome indicate that the condition may be caused by a somatic alteration in a gene, but no specific genetic mutation has yet been identified. Therefore, the diagnosis and management of the disease depend heavily on clinical evaluation and imaging. Although the manifestations of Proteus syndrome are highly variable, accurate diagnosis is possible if standard diagnostic criteria are followed and if disease features are assessed in comparison with those found in similar syndromes.