Pulmonary Hypertension and Cor Pulmonale during Severe Acute Chest Syndrome in Sickle Cell Disease

Dessap, Armand Mekontso; Leon, Rusel; Habibi, Anoosha; Nzouakou, Ruben; Roudot‐Thoraval, Françoise; Adnot, Serge; Godeau, Bertrand; Galactéros, Frédéric; Brun‐Buisson, Christian; Brochard, Laurent; Maître, Bernard

doi:10.1164/rccm.200710-1606oc

Cited by 142 publications

(80 citation statements)

References 41 publications

(50 reference statements)

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“…Second, we included all ACS episodes during the study period as commonly performed in the ACS literature which is by nature a relapsing disease 5. 8 28 Because all episodes may not be considered as being independent of one another, we confirmed our findings by a sensitivity analysis restricted to the first ACS episode.…”

Section: Discussionmentioning

confidence: 62%

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Lung imaging during acute chest syndrome in sickle cell disease: computed tomography patterns and diagnostic accuracy of bedside chest radiograph

Dessap

Deux

Habibi

et al. 2013

Thorax

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IntroductionThe lung computed tomography (CT) features of acute chest syndrome (ACS) in sickle cell disease patients is not well described, and the diagnostic performance of bedside chest radiograph (CR) has not been tested. Our objectives were to describe CT features of ACS and evaluate the reproducibility and diagnostic performance of bedside CR. Methods We screened 127 consecutive patients during 166 ACS episodes and 145 CT scans (in 118 consecutive patients) were included in the study. Results Among the 145 CT scans, 139 (96%) exhibited a new pulmonary opacity and 84 (58%) exhibited at least one complete lung segment consolidation. Consolidations were predominant as compared with ground-glass opacities and atelectasis. Lung parenchyma was increasingly consolidated from apex to base; the right and left inferior lobes were almost always involved in patients with a new complete lung segment consolidation on CT scan (98% and 95% of cases, respectively). Patients with a new complete lung segment consolidation on CT scan had a more severe presentation and course as compared with others.

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Section: Discussionmentioning

confidence: 62%

“…Several of these mechanisms may act during ACS. In particular, SCD patients may be prone to heart dilatation either due to chronic heart disease27 or acute cor pulmonale during severe episodes of ACS 28. These patients may also exhibit abdominal compartment syndrome in relation to immobilisation and morphine use for pain relief.…”

Section: Discussionmentioning

confidence: 99%

Lung imaging during acute chest syndrome in sickle cell disease: computed tomography patterns and diagnostic accuracy of bedside chest radiograph

Dessap

Deux

Habibi

et al. 2013

Thorax

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“…In larger cohorts of adult patients with ACS, the need for mechanical ventilation predicted hospital deaths and resource utilization. 11,34,35 Loss of lung aeration as assessed by the LU score may be useful for the early identification of patients at higher risk of poor outcome. Whether early implementation of a more aggressive treatment in such patients may alter ACS outcome needs further research.…”

Section: Discussionmentioning

confidence: 99%

“…10 In patients with persistent respiratory distress despite red-blood-cell transfusion or partial-exchange transfusion, noninvasive mechanical ventilation was started; orotracheal intubation and invasive ventilation were used in patients who failed noninvasive ventilation. 11 …”

Section: Methodsmentioning

confidence: 99%

Bedside Lung Ultrasound During Acute Chest Syndrome in Sickle Cell Disease

et al. 2016

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Lung ultrasound (LU) is increasingly used to assess pleural and lung disease in intensive care unit (ICU) and emergency unit at the bedside. We assessed the performance of bedside chest radiograph (CR) and LU during severe acute chest syndrome (ACS), using computed tomography (CT) as the reference standard.We prospectively explored 44 ACS episodes (in 41 patients) admitted to the medical ICU. Three imaging findings were evaluated (consolidation, ground-glass opacities, and pleural effusion). A score was used to quantify and compare loss of lung aeration with each technique and assess its association with outcome.A total number of 496, 507, and 519 lung regions could be assessed by CT scan, bedside CR, and bedside LU, respectively. Consolidations were the most common pattern and prevailed in lung bases (especially postero-inferior regions). The agreement with CT scan patterns was significantly higher for LU as compared to CR (κ coefficients of 0.45 ± 0.03 vs 0.30 ± 0.03, P < 0.01 for the parenchyma, and 0.73 ± 0.08 vs 0.06 ± 0.09, P < 0.001 for pleural effusion). The Bland and Altman analysis showed a nonfixed bias of −1.0 (P = 0.12) between LU score and CT score whereas CR score underestimated CT score with a fixed bias of −5.8 (P < 0.001). The specificity for the detection of consolidated regions or pleural effusion (using CT scan as the reference standard) was high for LU and CR, whereas the sensitivity was high for LU but low for CR. As compared to others, ACS patients with an LU score above the median value of 11 had a larger volume of transfused and exsanguinated blood, greater oxygen requirements, more need for mechanical ventilation, and a longer ICU length of stay.LU outperformed CR for the diagnosis of consolidations and pleural effusion during ACS. Higher values of LU score identified patients at risk of worse outcome.

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“…Bernini et al found that dexamethasone (0.3 mg/kg every 12 h 9 4 doses) decreased the need for blood transfusion and opioids in children with ACS, but this beneficial effect was counterbalanced by a trend towards increased frequency of readmission [21]. Acute pulmonary hypertension and right ventricular failure are common features of severe ACS episodes and have been associated with increased mortality [22]. Many mechanisms may cause pulmonary hypertension during ACS, including thrombosis-related vaso-occlusion and hemolysis-related vasoconstriction [23][24][25].…”

Section: Discussionmentioning

confidence: 99%

Inhaled nitric oxide for acute chest syndrome in adult sickle cell patients: a randomized controlled study

et al. 2015

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Pulmonary Hypertension and Cor Pulmonale during Severe Acute Chest Syndrome in Sickle Cell Disease

Abstract: Pulmonary pressures increase during severe acute chest syndrome, and pulmonary hypertension is associated with cardiac biomarker elevation and a higher risk of death.

Cited by 142 publications

References 41 publications

Lung imaging during acute chest syndrome in sickle cell disease: computed tomography patterns and diagnostic accuracy of bedside chest radiograph

Lung imaging during acute chest syndrome in sickle cell disease: computed tomography patterns and diagnostic accuracy of bedside chest radiograph

Bedside Lung Ultrasound During Acute Chest Syndrome in Sickle Cell Disease

Inhaled nitric oxide for acute chest syndrome in adult sickle cell patients: a randomized controlled study

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