To evaluate mosaic perfusion patterns and vascular lesions in patients with chronic thromboembolic pulmonary hypertension (CTEPH) using C-Arm computed tomography (CACT) compared to computed tomography pulmonary angiography (CTPA). We included 41 patients (18 female; mean age 59.9 ± 18.3 years) with confirmed CTEPH who underwent CACT and CTPA within 21 days (average 5.3 ± 5.2). Two readers (R1; R2) independently evaluated datasets from both imaging techniques for mosaic perfusion patterns and presence of CTEPH-typical vascular lesions. The number of pulmonary arterial segments with typical findings was evaluated and the percentage of affected segments was calculated and categorized: < 25%; 25-49%; 50-75%; < 75% of all pulmonary arterial segments affected by thromboembolic vascular lesions. Inter-observer agreement was calculated for both modalities using the intraclass-correlation-coefficient (ICC). Based on consensus reading the inter-modality agreement (CACT vs. CTPA) was calculated using the ICC. Inter-observer agreement was excellent for central vascular lesions (ICC > 0.87) and the percentage of affected segments (ICC > 0.76) and good for the perceptibility of mosaic perfusion (ICC > 0.6) and attribution of the pattern of mosaic perfusion (ICC > 0.6) for both readers on CACT and CTPA. Inter-modality agreement was excellent for the perceptibility of mosaic perfusion (ICC = 1), the present perfusion pattern (ICC = 1) and central vascular lesions (ICC = 1). However, inter-modality agreement for the percentage of affected segments was fair (ICC = 0.50), with a greater proportion of identified affected segments on CACT. CACT demonstrates a high agreement with CTPA regarding the detection of mosaic perfusion. CACT detects a higher number of peripheral vascular lesions compared to CTPA.
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http://dx.doi.org/10.1038/s41598-021-99658-2 | DOI Listing |
Hell J Nucl Med
December 2024
Department of Nuclear Medicine, Abant Izzet Baysal University, Bolu, Turkey.
Objective: Despite the high sensitivity and specificity of ventilation/perfusion (VQ) scintigraphy in the diagnosis of chronic thromboembolic pulmonary hypertension (CTEPH) and chronic thromboembolic disease (CTED), V/Q scintigraphy cannot distinguish whether the thrombus is acute or chronic. In our study, we aimed to compare pulmonary computed tomography angiography (CTA) findings with V/Q scintigraphy findings in CTEPH and CTED patients and to identify findings that would indicate chronic thrombus.
Subjects And Methods: Eighteen patients diagnosed with CTEPH and CTED at our institution were included in the study between January 2020 and January 2024.
Molecules
November 2024
Department of Neurology and Developmental Medicine, Hugo Moser Kennedy Krieger Research Institute, Baltimore, MD 21205, USA.
Sturge-Weber syndrome (SWS) is a rare congenital neurocutaneous disorder typically caused by a somatic mosaic mutation in R183Q . At-risk children present at birth with a capillary malformation port-wine birthmark. The primary diagnostic characteristic of the disorder includes leptomeningeal enhancement of the brain, which demonstrates abnormal blood vessels and results in impaired venous drainage and impaired local cerebral perfusion.
View Article and Find Full Text PDFAm J Hematol
December 2024
Université Paris Cité, Inserm, BIGR, Paris, France.
In children with sickle cell disease (SCD), splenectomy is immediately beneficial for acute sequestration crises and hypersplenism (ASSC/HyS) but portends a long-term risk of asplenia-related complications. We retrieved peripheral and splenic red blood cells (RBCs) from 17 SCD children/teenagers undergoing partial splenectomy for ASSC/HyS, 12 adult subjects without RBC-related disease undergoing splenectomy (controls), five human spleens perfused ex vivo with Hb- and Hb-RBC, and quantified abnormal RBC by microscopy, spleen-mimetic RBC filtration, and adhesion assays. Spleens were analyzed by immunohistochemistry and transmission electron microscopy (TEM).
View Article and Find Full Text PDFJ Am Coll Cardiol
July 2024
Department of Cardiovascular Medicine and Cleveland Clinic Coordinating Center for Clinical Research (C5Research), Cleveland Clinic, Cleveland, Ohio, USA.
Background: In the CLEAR (Cholesterol Lowering via Bempedoic Acid, an ACL-Inhibiting Regimen) Outcomes trial, treatment of statin-intolerant patients with bempedoic acid produced a 21% decrease in low-density lipoprotein cholesterol (LDL-C) relative to placebo and a 13% relative reduction in the risk of major adverse cardiovascular events.
Objectives: This study sought to determine whether the relationship between LDL-C lowering and cardiovascular benefit achieved with bempedoic acid resembles that observed with statins when standardized per unit change in LDL-C.
Methods: To compare the treatment effect of bempedoic acid with statins, the methodology of the Cholesterol Treatment Trialists' Collaboration (CTTC) was applied to outcomes among the 13,970 patients enrolled in the CLEAR Outcomes trial.
Zhonghua Bing Li Xue Za Zhi
July 2024
Department of Pathology, Haidian Maternal and Children Health Hospital, Beijing 100195, China.
To investigate the clinicopathological and genetic features of confined placental mosaicism (CPM) and its effect on fetal intrauterine growth. Fourteen CPM cases of Haidian Maternal and Children Health Hospital were collected from May 2018 to March 2022. Clinicopathological examination on placental specimens and molecular genetic analysis were performed.
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