Correlation of Computed Tomography Test Bolus Dynamics and Conventional Computed Tomography Parameters With Pulmonary Vascular Resistance in Patients With Pulmonary Arterial Hypertension.

OBJECTIVE: Pulmonary vascular resistance (PVR) is a measurement obtained with invasive right heart catheterization (RHC) that is commonly used for management of patients with pulmonary arterial hypertension (PAH). Computed tomography pulmonary angiography (CTPA) is also done as part of the workup for PAH in some cases. The aim of our study was to assess the correlation of contrast dynamic changes in the main pulmonary artery (MPA) on CTPA with PVR obtained with RHC. METHODS: This is an IRB-approved retrospective study performed in two separate institutions (Medical College of Wisconsin and University of Alabama) between January 2010 and December 2013. During CTPA done as test bolus, serial images are acquired at the level of MPA after intravenous injection of contrast to determine timing of the CT acquisition. Since the PVR changes with the degree of PAH, we hypothesize that will be reflected in the contrast kinetics in MPA. A correlation of standard CT metrics (MPA diameter, right pulmonary artery [PA] diameter, left PA diameter, MPA/aorta ratio, and right ventricle/left ventricle [RV/LV] ratio) and dynamic (full width at half maximum) CTPA parameters in patients with known PAH was performed with PVR obtained from RHC done within 30 days. Statistical analysis was performed by Pearson correlation coefficient. RESULTS: Among 221 patients in our database, 37 patients fulfilled the selection criteria. There was a strong correlation between full width half maximum (FWHM) and mean pulmonary artery pressure (mPAP) (r=0.69, p value<0.00001), PVR (r=0.8, p value<0.00001) and indexed PVR (PVRI) (r=0.75, p value<0.00001). CONCLUSION: FWHM obtained from CTPA strongly correlates with RHC parameters and is potentially more helpful than static measurements for follow-up of patients with known PAH to assess response to treatment or progression.

Identifying High-Attenuating and Low-Attenuating Muscle Using Computerized Tomography and Exploring Its Impact on Physical Function and Muscle Strength in Obese Critically Ill Patients.

BACKGROUND: Computed tomography (CT) methods to estimate sarcopenia in obesity do not differentiate high-attenuating from low-attenuating muscle. The primary purpose of this study was to determine agreement between a CT method using general workstation-derived total and high-attenuating psoas muscle cross-sectional area (CSA) and a commercially available segmentation software-derived value. Secondary purpose was to explore the relationship between quantity of high-attenuating muscle to physical functioning in a pilot cohort of obese medical intensive care unit (MICU) patients. METHODS: We conducted a prospective observational cross-sectional study. CT images of obese MICU patients were reconstructed to calculate total psoas muscle, low-attenuating muscle, and high-attenuating muscle within the third lumbar psoas CSA using a CT method and commercial software. We performed blinded outcome measures of CSA, physical function, and muscle strength in 28 patients. RESULTS: Concordance correlation coefficient for identifying total psoas muscle was 0.96 (95% confidence interval: 0.93-0.98, P-value < 0.0001) between CT method and commercial software. There was moderate correlation between modified Medical Research Council muscle strength scores and high-attenuating psoas muscle CSA (r = 0.47, P = 0.01) and lower extremity strength and high-attenuating psoas muscle CSA (r = 0.40, P = 0.04). CONCLUSION: There was strong agreement between our CT method and a commercial software method to identify total psoas muscle CSA in obesity. Greater total high-attenuating psoas CSA moderately correlated with muscle strength. Additional studies using more objective markers of muscle strength validating these findings are needed.

Etanercept and Corticosteroid Therapy for the Treatment of Late-Onset Idiopathic Pneumonia Syndrome.

Idiopathic pneumonia syndrome (IPS) is a complication of allogeneic hematopoietic stem cell transplantation (HSCT) that typically occurs within the first 100 days after transplantation. Tumor necrosis factor α (TNF-α) has been shown to be a key mediator of IPS, and the TNF-α binding protein etanercept appeared to improve IPS outcomes in small retrospective and prospective studies. IPS also has been observed to occur later (>100 days) after HSCT; however, little is known about the disease course and whether a TNF-α-based therapeutic strategy is efficacious in these patients. To address this question, we performed a retrospective analysis of 23 patients who underwent HSCT between 2004 and 2016 at our institution who developed late-onset IPS and received treatment with etanercept and high-dose corticosteroids (CS). Ten of the 23 patients (43%) attained a complete clinical response to etanercept and CS. Responses were significantly more likely to occur in patients who did not require positive pressure ventilation at the time of diagnosis. Those who responded experienced a durable survival benefit, with a 2-year overall survival of 67%. In the 13 patients (57%) who did not respond to etanercept and CS, the median overall survival was only 13 days (range, 1 to 60 days). The difference in 2-year overall survival between responders and nonresponders was statistically significant (67% versus 0%; P < .001). These results indicate that late-onset IPS carries high mortality, but that treatment with etanercept and CS has activity and can result in long-term survival in some patients. Prompt diagnosis and early institution of therapy before the need for advanced respiratory support is critical for maximizing responses.

Retroperitoneal lymphangioleiomyoma with lymph node involvement: A pathologic-radiologic correlation of a rare form of myomelanocytic tumor.

Lymphangioleiomyomatosis (LAM) is a rare and slowly progressive disorder that usually arises in the lung, affects exclusively women in their childbearing years, and typically presents with progressive dyspnea on exertion and pneumothorax. Infrequently, extra-pulmonary LAM can occur in the retroperitoneum, uterine wall, mediastinum and intraperitoneal lymph nodes. Histologically, LAM is characterized by a proliferation of perivascular epithelioid cells (PEC) that express markers for both melanocytes and smooth muscle cells. We report a case of a peripancreatic retroperitoneal mass that was incidentally discovered on magnetic resonance image (MRI) scan of a 38-year-old female. The morphologic findings and the immunohistochemical staining were consistent with a lymphangioleiomyoma. The radiologic and pathologic correlation along with differential diagnosis of this rare entity is discussed.

Circulating fibrocytes as biomarkers of impaired lung function in adults with sickle cell disease.

Lung injury and fibrosis are common in patients with sickle cell disease (SCD). Fibrocytes, a population of circulating, bone marrow-derived cells, have been linked to development and progression of tissue fibrogenesis and have been implicated in the development of lung fibrosis in preclinical models of SCD. We tested the hypothesis that the levels and activation state of circulating fibrocytes during steady state are associated with abnormal pulmonary function in adults with SCD. In a prospective cohort of steady-state adults with SCD and healthy age- and race-matched control participants, we measured the concentration and activation state of circulating fibrocytes and assessed pulmonary phenotype with pulmonary function tests (PFTs), a respiratory questionnaire, 6-minute walk test, high-resolution chest computed tomography scan, and echocardiogram. Seventy-one adults with SCD and 26 healthy African American control participants were examined. Compared with control participants, patients with SCD demonstrated higher levels of circulating fibrocytes, a significant proportion of which expressed the activation marker α-smooth muscle actin. Within patients with SCD, elevated absolute concentrations of circulating fibrocytes were strongly and independently associated with impaired lung physiology, as measured by PFTs. We conclude that elevated circulating fibrocytes are associated with lung disease in adults with SCD during steady state, consistent with a role for these cells in pathogenesis of lung fibrosis in this disease. Circulating fibrocytes may represent a novel biomarker for progressive pulmonary fibrosis in patients with SCD.

Comprehensive Imaging Review of the Superior Vena Cava.

The superior vena cava (SVC) is the largest central systemic vein in the mediastinum. Imaging (ie, radiography, computed tomography [CT], magnetic resonance [MR] venography, and conventional venography) plays an important role in identifying congenital variants and pathologic conditions that affect the SVC. Knowledge of the basic embryology and anatomy of the SVC and techniques for CT, MR imaging, and conventional venography are pivotal to accurate diagnosis and clinical decision making. Congenital anomalies such as persistent left SVC, partial anomalous pulmonary venous return, and aneurysm are asymptomatic and may be discovered incidentally in patients undergoing imaging evaluation for associated cardiac abnormalities or other indications. Familiarity with congenital abnormalities is important to avoid image misinterpretation. Acquired abnormalities such as intrinsic and extrinsic strictures, fibrin sheath, thrombus, primary neoplasms, and trauma can produce mild narrowing to complete occlusion, the latter leading to SVC syndrome. Each imaging modality plays a role in evaluation of the SVC, helping to determine the site, extent, and cause of pathologic conditions and guide appropriate management. Commonly performed interventional procedures for fibrin sheath and benign and malignant strictures include low-dose thrombolytic infusion, fibrin sheath disruption, venous angioplasty, and stent placement.

Reduced z-axis technique for CT Pulmonary angiography in pregnancy--validation for practical use and dose reduction.

The aim of this study is to determine the feasibility of using reduced scan range CT pulmonary angiography technique in pregnancy for pulmonary embolism (PE) and to quantify resulting dose reduction. This was a retrospective study. Eighty-four CTPA exams performed on pregnant women during 2004-2012. The scans were modified to create reduced anatomic coverage scans extending from aortic arch to base of heart. These were separately evaluated by two radiologists for PE and non-PE abnormalities. The results were then compared by the third radiologist with original radiology report and scans. Radiation dose reduction was evaluated prospectively in 36 patients as part of a quality control project. Two patients had PE and were successfully identified on reduced z-axis scans. Thirty-two exams were normal; rest had 60 pertinent and 16 had incidental findings. There were four incidental findings which included three benign thyroid nodules and one benign small lung nodule which were missed. None of these affected clinical outcome or management. There was 71 % radiation dose reduction. No PE or any important diagnoses are missed using reduced z-axis CTPA in pregnancy. There is a substantial radiation dose reduction. Hence, this technique is highly recommended in pregnancy.

Intraparenchymal pulmonary lipoma: pathologic-radiologic correlation of a rare presentation of a common neoplasm.

We report a rare case of pulmonary intraparenchymal lipoma. Lipomas are benign adipocytic tumors, which are ubiquitous in distribution, particularly in the subcutis and soft tissue. Visceral lipomas, in particular, pulmonary lipomas, are rarely reported. Even rarer are intraparenchymal lipomas, such as this case, of which less than 10 have been reported in the medical literature. The radiologic (computed tomographic scan) findings of pulmonary lipoma may be somewhat difficult to evaluate. In this case, on initial review, the computed tomographic findings were not diagnostic, but retrospective analysis revealed attenuation values suggestive of an adipocytic lesion. A high index of suspicion and careful attention to attenuation values are therefore required for radiologic diagnosis. Excision is necessary for histologic confirmation, which is generally relatively straight forward, although admixture with fibrous tissue and some cytologic atypia may pose diagnostic challenges.

Dose reduction of 69% for computed tomography pulmonary angiography: reduced z-axis computed tomography pulmonary angiography retains accuracy in those younger than 40 years.

OBJECTIVE: We sought to determine whether reduced scan range (z axis) computed tomography pulmonary angiography (CTPA) technique in 18- to 40-year age group can accurately detect pulmonary embolism (PE) and other important conditions and to quantify the resulting dose reduction. METHODS: We retrospectively identified 200 patients in the age group of 18 to 40 years who underwent CTPA over a period of 3 years. These included 86 patients with PEs and 114 randomly selected patients negative for PE (control subjects). The scans were modified by reducing the scan coverage by eliminating images above the aortic arch and below the base of the heart. Two blinded experienced radiologists rescored them for PE and incidental and pertinent non-PE findings. Discrepancies between these and the original report were assessed by a third experienced radiologist. Separately, a departmental quality-control project was conducted in 15 patients to assess the dose length product along the z axis across the 3 zones of thorax. RESULTS: Pulmonary embolism was diagnosed in all 86 patients. No pertinent additional findings were missed. Only 7 incidental findings were missed (eg, benign thyroid nodules [n = 4], benign adrenal adenoma [n = 1], gallstones [n = 1], and hepatic hemangioma [n = 1]). None affected clinical outcome or management. Dose length product was reduced by a mean of 69% (60%-79%). CONCLUSIONS: Reduced z-axis CTPA for PE is definitely feasible in 18- to 40-year age group. There is a significant radiation reduction (69%) and hence should be considered in selected subgroups of patients.

Use of combination chemotherapy for treatment of granulomatous and lymphocytic interstitial lung disease (GLILD) in patients with common variable immunodeficiency (CVID).

PURPOSE: A subset of patients with common variable immunodeficiency (CVID) develops granulomatous and lymphocytic interstitial lung disease (GLILD), a restrictive lung disease associated with early mortality. The optimal therapy for GLILD is unknown. This study was undertaken to see if rituximab and azathioprine (combination chemotherapy) would improve pulmonary function and/or radiographic abnormalities in patients with CVID and GLILD. METHODS: A retrospective chart review of patients with CVID and GLILD who were treated with combination chemotherapy was performed. Complete pulmonary function tests (PFTs) and high-resolution computed tomography (HRCT) scans of the chest were done prior to therapy and >6 months later. HRCT scans of the chest were blinded, randomized, and scored independently (in pairs) by two radiologists. The differences between pre- and post-treatment HRCT scores and PFT parameters were analyzed. RESULTS: Seven patients with CVID and GLILD met inclusion criteria. Post-treatment increases were noted in both FEV1 (p=0.034) and FVC (p=0.043). HRCT scans of the chest demonstrated improvement in total score (p=0.018), pulmonary consolidations (p=0.041), ground-glass opacities (p=0.020) nodular opacities (p=0.024), and both the presence and extent of bronchial wall thickening (p=0.014, 0.026 respectively). No significant chemotherapy-related complications occurred. CONCLUSIONS: Combination chemotherapy improved pulmonary function and decreased radiographic abnormalities in patients with CVID and GLILD.

Pulmonary embolism: optimizing the diagnostic imaging approach.

Venous thromboembolism is a common medical problem that can affect a wide range of patients. The clinical presentation ranges from minor, nonspecific signs and symptoms to severe clinical scenarios. The combination of objective pretest clinical probability, D-dimer testing, and imaging studies plays a critical role in its diagnosis. There are multiple imaging modalities to confirm or exclude the presence of pulmonary embolism and deep vein thrombosis, but, at present, computed tomography angiography is the most frequently used. Other imaging, however, is required for several subpopulations.

Pulmonary embolism in pregnancy: CT pulmonary angiography versus perfusion scanning.

OBJECTIVE: The purpose of this study was to evaluate the equivalence of CT pulmonary angiography and perfusion scanning in terms of diagnostic quality and negative predictive value in the imaging of pulmonary embolism (PE) in pregnancy. MATERIALS AND METHODS: Between 2000 and 2007 at a university hospital and a large private hospital, 199 pregnant patients underwent 106 CT pulmonary angiographic examinations and 99 perfusion scans. Image quality was evaluated, and the findings were reread by radiologists and compared with the original clinical readings. Three-month follow-up findings of PE and deep venous thrombosis were recorded. RESULTS: PE was found in four of the 106 patients (3.7%) who underwent CT pulmonary angiography. The overall image quality was poor in 5.6% of cases, acceptable in 17.9%, and good in 76.4%. Fourteen CT and nine radiographic studies showed other clinically significant abnormalities. Six patients had indeterminate CT pulmonary angiographic findings, three had normal perfusion scans, and none underwent anticoagulation. All perfusion scan findings were normal. There was one incomplete study, and follow-up CT pulmonary angiography performed the same day showed PE. Two of 99 studies (2.02%) showed intermediate probability of the presence of PE; PE was not found at CT pulmonary angiography, but pneumonia was found. PE was found in one postpartum patient 9 weeks after she had undergone CT pulmonary angiography and ultrasound with normal findings. None of the patients died. CONCLUSION: CT pulmonary angiography and perfusion scanning have equivalent clinical negative predictive value (99% for CT pulmonary angiography; 100% for perfusion scanning) and image quality in the care of pregnant patients. Therefore, the choice of study should be based on other considerations, such as radiation concern, radiographic results, alternative diagnosis, and equipment availability. Reducing the amount of radiation to the maternal breast favors use of perfusion scanning when the radiographic findings are normal and there is no clinical suspicion of an alternative diagnosis.