title: Computed tomography correlates with cardiopulmonary hemodynamics in pulmonary hypertension in adults with sickle cell disease.
abstract: Our objective was to determine whether computed tomography angiography (CTA) measurements of pulmonary artery size can noninvasively assess hemodynamics and diagnose pulmonary hypertension (PH) secondary to sickle cell disease (SCD). Twenty SCD patients with confirmed PH were compared with 20 matched controls. Diameters of the pulmonary artery trunk and branches were measured with CTA and a semiautomatic segmentation algorithm. Measurements were normalized by body size and correlated (Spearman rank) with hemodynamic markers from right-heart catheterization. Receiver operating characteristic (ROC) curves were used to investigate the role of pulmonary artery sizes in diagnosing PH. Analysis of pulmonary artery sizes adjusted for body surface area (BSA) resulted in the most significant discrimination between subjects with PH secondary to SCD and controls (P < 0.001); PH was diagnosed accurately with an area under the ROC curve of 0.99. There was significant correlation between pulmonary artery sizes and body mass index (BMI) and BSA only in controls (r = 0.46-0.68, P < 0.04 for all). The most significant correlations with hemodynamic markers were found between BMI-adjusted pulmonary artery sizes and high systolic pulmonary arterial pressure, high pulmonary vascular resistance, high systemic vascular resistance, and low cardiac output (r = 0.47, 0.62, 0.61, and 0.66, respectively; P < 0.04 for all). BMI-adjusted CTA measures of the pulmonary artery relate to high pulmonary vascular resistance and reduced cardiac output in patients with SCD and PH. CTA with quantitative image analysis is a powerful noninvasive diagnostic tool for PH in SCD and shows promise as estimator of hemodynamic markers.
authors.affiliation: Sheikh Zayed Institute for Pediatric Surgical Innovation, Children's National Healthcare System, Washington, DC, USA ; Radiology and Imaging Sciences, National Institutes of Health Clinical Center, Bethesda, Maryland, USA ; School of Medicine and Health Sciences, George Washington University, Washington, DC, USA.
authors.forename: Marius George
authors.initials: MG
authors.lastname: Linguraru
authors.affiliation: Radiology and Imaging Sciences, National Institutes of Health Clinical Center, Bethesda, Maryland, USA ; Department of Biostatistics and Bioinformatics, Duke University, Durham, North Carolina, USA.
authors.forename: John A
authors.initials: JA
authors.lastname: Pura
authors.affiliation: Heart, Lung, Blood and Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, Pennsylvania, USA.
authors.forename: Mark T
authors.initials: MT
authors.lastname: Gladwin
authors.affiliation: Sheikh Zayed Institute for Pediatric Surgical Innovation, Children's National Healthcare System, Washington, DC, USA ; School of Medicine and Health Sciences, George Washington University, Washington, DC, USA.
authors.forename: Antony I
authors.initials: AI
authors.lastname: Koroulakis
authors.affiliation: Hematology Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland, USA.
authors.forename: Caterina
authors.initials: C
authors.lastname: Minniti
authors.affiliation: Section of Pulmonary/Critical Care, Department of Medicine, University of Illinois at Chicago, Chicago, Illinois, USA.
authors.forename: Roberto F
authors.initials: RF
authors.lastname: Machado
authors.affiliation: Heart, Lung, Blood and Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, Pennsylvania, USA.
authors.forename: Gregory J
authors.initials: GJ
authors.lastname: Kato
authors.affiliation: Radiology and Imaging Sciences, National Institutes of Health Clinical Center, Bethesda, Maryland, USA.
authors.forename: Bradford J
authors.initials: BJ
authors.lastname: Wood
keywords: CT angiography; arterial size; cardiopulmonary hemodynamics; pulmonary hypertension; quantitative imaging; sickle cell disease