Imaging for Pulmonary Embolism in Sickle Cell Disease: A 17-Year Experience

Tivnan, Patrick; Billett, Henny H.; Freeman, Leonărd M.; Haramati, Linda B.

doi:10.2967/jnumed.117.205641

Cited by 17 publications

(14 citation statements)

References 25 publications

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“…Our findings differ from a recent similarly sized comparison of V/Q and CTPA. Tivnan et al (41) retrospectively analyzed 154 V/Q scans with 91 CTPAs performed for suspected acute PE in an SCD cohort. Only 4.1% were positive for PE using either modality-a lower thromboembolic prevalence than what has been published, although the authors reported 11% in subsequent testing done for clinical suspicion over the 17-y study period.…”

Section: Discussionmentioning

confidence: 99%

Abnormal Ventilation–Perfusion Scan Is Associated with Pulmonary Hypertension in Sickle Cell Adults

et al. 2018

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Pulmonary hypertension (PH) in adults with sickle cell disease (SCD) is associated with early mortality. Chronic thromboembolic pulmonary hypertension (CTEPH) is an important complication and contributor to PH in SCD, but likely underappreciated. Guidelines recommend ventilation-perfusion scintigraphy (V/Q) as imaging modality of choice to exclude CTEPH. Data on V/Q are limited in SCD. To compare the performance of V/Q with computed tomography pulmonary angiography (CTPA) and to report clinical outcomes associated with abnormal V/Q. Laboratory data, echocardiography, six-minute walk test, V/Q, CTPA, and right heart catheterization (RHC) were prospectively obtained. High-probability and intermediate-probability V/Q scan findings were considered to be abnormal. 142 SCD adults (aged 40.1±13.7 years, 83 women, 87% HbSS) in stable state enrolled consecutively between March 13, 2002 and June 8, 2017 were included for analysis. V/Q scan was abnormal in 65/142 (45.8%). CTPA was positive for pulmonary embolism in 16/60 (26.7%). RHC confirmed PH (mean pulmonary artery pressure (mPAP) ≥25mmHg) in 46/64 (71.9%), of which 34 (73.9%) had abnormal V/Q. Among those without PH by RHC ( = 18), 2/18 patients showed abnormal V/Q. Thirty-three patients had a complete dataset of V/Q, CTPA and RHC; 29/33 had abnormal RHC, of which 26/29 had abnormal V/Q compared to 11/29 with abnormal CTPA. There was a greater concordance of V/Q with RHC (kappa value =0.53; p<0.001) compared to CTPA with RHC (kappa value =0.13; = 0.065). The sensitivity and specificity for V/Q was 89.7% and 75.0% respectively, whereas CTPA had sensitivity 37.3% and specificity 100%. Abnormal V/Q was associated with hemodynamic severity (mPA = 35.2±9.6 vs. 26.9±10.5 mmHg, = 0.002; transpulmonary gradient 21.5±9.7 vs. 12.16±11mmHg, = 0.005, and pulmonary vascular resistance=226.5±135 vs.140.7±123.7 dynes.s.cm-5, = 0.013), and lower exercise capacity (6MWD 382.8±122.3 vs. 442.3±110.6m, p<0.010). Thirty-four deaths were observed over 15 years. All-cause mortality was higher in the abnormal V/Q (21(61.8%)) compared to normal V/Q (13(38.2%)) group (log-rank test, = 0.006, Hazard Ratio=2.54). V/Q scan is superior to CTPA in detecting thrombotic events in SCD. Abnormal V/Q is associated with PH, worse hemodynamics, lower functional capacity and increased mortality. Despite the high sensitivity of the V/Q scan in detecting CTEPH it is underutilized. We recommend the use of V/Q in the evaluation of dyspnea in SCD adults given the important implications in management.

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Section: Discussionmentioning

confidence: 99%

Abnormal Ventilation–Perfusion Scan Is Associated with Pulmonary Hypertension in Sickle Cell Adults

et al. 2018

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“…Roy et al aimed to identify an animal model that resembles human salivary gland uptake of PSMA-TRT and compared the salivary glands uptake of [ 18 F]F-DcFPYL in NCR-nu/nu athymic female mice and F344/SAS Fischer female rats [121]. Biodistribution assays revealed a 2- to 3-fold higher uptake of [ 18 F]F-DcFPYL in mice submandibular and sublingual glands compared to that of rats.…”

Section: Protection Of Psma Expressing Kidneys and Salivary Glandsmentioning

confidence: 99%

The Future of PSMA-Targeted Radionuclide Therapy: An Overview of Recent Preclinical Research

et al. 2019

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Prostate specific membrane antigen (PSMA) has become a major focus point in the research and development of prostate cancer (PCa) imaging and therapeutic strategies using radiolabeled tracers. PSMA has shown to be an excellent target for PCa theranostics because of its high expression on the membrane of PCa cells and the increase in expression during disease progression. Therefore, numerous PSMA-targeting tracers have been developed and (pre)clinically studied with promising results. However, many of these PSMA-targeting tracers show uptake in healthy organs such as the salivary glands, causing radiotoxicity. Furthermore, not all patients respond to PSMA-targeted radionuclide therapy (TRT). This created the necessity of additional preclinical research studies in which existing tracers are reevaluated and new tracers are developed in order to improve PSMA-TRT by protecting the (PSMA-expressing) healthy organs and improving tumor uptake. In this review we will give an overview of the recent preclinical research projects regarding PCa-TRT using PSMA-specific radiotracers, which will give an indication of where the PSMA-TRT research movement is going and what we can expect in future clinical trials.

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“…Even more concerning is the high risk of death associated with VTE, especially acute pulmonary embolism (APE), in adults with SCD 18,19 . The diagnosis of APE in individuals with SCD is difficult due to other pulmonary complications such as acute chest syndrome or vaso‐occlusive pain crisis, which may mimic the signs and symptoms of an acute PE 20 . Further complicating the diagnosis is the questionable utility of a D‐dimer, a laboratory value often used in the general population in this diagnosis.…”

Section: Introductionmentioning

confidence: 99%

“…Due to baseline elevations in D‐dimer in SCD, this laboratory assessment is of little clinical value in the diagnosis of an acute VTE 15,21 . Thus, the only diagnostic tool readily available to this population is the computed tomography angiography (CTA) scan which requires exposure to high levels of radiation and intravenous contrast and which can lead to significant renal toxicity and significant lifetime exposure to radiation 20 . CTA is considered the gold standard for diagnoses of an acute PE 22 .…”

Section: Introductionmentioning

confidence: 99%

Which adults with sickle cell disease need an evaluation for pulmonary embolism?

et al. 2021

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Individuals living with sickle cell disease (SCD) are at an increased risk of venous thrombo-embolism (VTE) including pulmonary embolisms (PEs). There is a high mortality associated with PE in individuals with SCD. It can be difficult to diagnose PE since presenting symptoms of PE often mimic those of other forms of vaso-occlusive crisis in SCD. Currently, there are no validated models for predicting PEs in patients with sickle cell disease, which often leads to frequent CT scans and exposure to harmful radiation and intravenous contrast. The aim of this study was to evaluate different host variables and potential clinical biomarkers of patients with SCD including those used in the Wells score to assess predictability for PE in order to create a more accurate diagnostic algorithm to predict PE. A retrospective chart review was performed on 349 patients with SCD who underwent testing for a PE with a CT scan of the chest. Forward and backward stepwise model selection was performed to obtain a parsimonious model of the predictors of PEs. The incidence of PE in this population was 9Á7%. Of the factors evaluated for this study, the Wells score was the only one with clinical significance. A Wells score greater than 4 had a sensitivity and specificity of 72Á5% and 70Á1%, respectively, and a score greater than 6 had a sensitivity and specificity of 50% and 87%, respectively. The Wells score is an acceptable clinical tool which may prove useful in individuals with SCD to predict who is most likely to have a PE and therefore should undergo a CT scan. A prospective study is needed to further confirm these findings.

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Imaging for Pulmonary Embolism in Sickle Cell Disease: A 17-Year Experience

Cited by 17 publications

References 25 publications

Abnormal Ventilation–Perfusion Scan Is Associated with Pulmonary Hypertension in Sickle Cell Adults

Abnormal Ventilation–Perfusion Scan Is Associated with Pulmonary Hypertension in Sickle Cell Adults

The Future of PSMA-Targeted Radionuclide Therapy: An Overview of Recent Preclinical Research

Which adults with sickle cell disease need an evaluation for pulmonary embolism?

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