Pulmonary Vascular Dysfunction and Cor Pulmonale During Acute Respiratory Distress Syndrome in Sicklers

Cecchini, Jérôme; Boissier, Florence; Gibelin, Aude; Prost, Nicolas de; Razazi, Keyvan; Carteaux, Guillaume; Galactéros, Frédéric; Maître, Bernard; Brun‐Buisson, Christian; Dessap, Armand Mekontso

doi:10.1097/shk.0000000000000640

Cited by 12 publications

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“…During ACS, there is no clinical score or biomarker available to select patients eligible for MDCT angiography (Mekontso Dessap et al, 2011). Ddimer testing is often positive in the setting of ACS (Mekontso Dessap et al, 2008;Cecchini et al, 2016). The frequent observation of acute cor pulmonale during ACS, even in the absence of PAT (Mekontso Dessap et al, 2008;Cecchini et al, 2016), makes echography an inaccurate strategy to assess the probability of PAT in SCD patients.…”

Section: Discussionmentioning

confidence: 99%

“…Patients investigated by MDCT angiography during the ACS episode were included. All MDCT examinations were performed using a 64‐row MDCT (Lightspeed VCT, GE Healthcare, Wauwatosa, WI, USA) with standard parameters (Mekontso Dessap et al , ; Cecchini et al , ). MDCT images were interpreted by hospital radiologists as part of their clinical work.…”

Section: Methodsmentioning

confidence: 99%

“…MDCT images were interpreted by hospital radiologists as part of their clinical work. PAT was defined as a MDCT showing a thrombus up to the segmental level or multiple thrombi at the subsegmental level (Mekontso Dessap et al , ; Cecchini et al , ). The following data were recorded: baseline characteristics (including type of haemoglobinopathy, steady‐state haemoglobin value, history and long‐term treatment for SCD), predisposing factors for venous thromboembolism ( e.g .…”

Section: Methodsmentioning

confidence: 99%

“…D‐dimer testing is often positive in the setting of ACS (Mekontso Dessap et al , ; Cecchini et al , ). The frequent observation of acute cor pulmonale during ACS, even in the absence of PAT (Mekontso Dessap et al , ; Cecchini et al , ), makes echography an inaccurate strategy to assess the probability of PAT in SCD patients. Targeting adult SCD patients who will be assessed by MDCT angiography during ACS is of critical importance in order to avoid unnecessary irradiation of these young patients, because ACS is typically a relapsing disease and PAT is found in only a minority of episodes.…”

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confidence: 99%

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A clinical risk score for pulmonary artery thrombosis during acute chest syndrome in adult patients with sickle cell disease

et al. 2017

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Pulmonary artery thrombosis (PAT) is involved in lung vascular dysfunction during acute chest syndrome (ACS) complicating sickle cell disease (SCD). No clinical score is available to identify patients eligible for multi-detector computed tomography (MDCT) angiography during ACS. This retrospective study aimed to develop a risk score for PAT during ACS (PAT-ACS risk score). Patients with SCD were investigated by MDCT during ACS. A logistic regression was performed to determine independent risks factors for PAT and to build the PAT-ACS risk score. A total of 43 episodes (11·9%) of PAT were diagnosed in 361 episodes of ACS. Multivariate analysis identified four risk factors, which were included in the PAT-ACS risk score: a baseline haemoglobin >82 g/l, the lack of a triggering factor for ACS, a platelet count >440 × 10 /l and a PaCO <38 mmHg at ACS diagnosis. The area under the receiver operating characteristic curve for the PAT-ACS risk score was 0·74 (95% confidence interval [CI] 0·69-0·79) and differed from that of the revised Geneva score (0·63 (95% CI 0·58-0·69); P = 0·04). The negative predictive value of a PAT-ACS risk score ≥2 was 94%. In conclusion, we propose a simple clinical risk score to identify SCD patients at high risk of PAT during ACS.

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confidence: 99%

Section: Methodsmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

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A clinical risk score for pulmonary artery thrombosis during acute chest syndrome in adult patients with sickle cell disease

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“…Inhaled nitric oxide at high doses (80 ppm, to consider scavenging by hemolysis products) did not improve ACS outcome, but a post hoc analysis suggested its efficacy in the subgroup with acute respiratory distress syndrome [11]. Acute respiratory distress syndrome due to ACS is characterized by a major pulmonary vascular involvement (pulmonary vascular dysfunction and acute cor pulmonale are found in 100 and 80 % of patients, respectively) [12]. Close monitoring (e.g., echocardiography) and a "right ventricle protective approach" with correction of risk factors for cor pulmonale (especially high driving pressure, severe hypoxemia, and hypercapnia) are essential; prone positioning may help alleviate RV afterload [13].…”

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Ten tips for managing critically ill patients with sickle cell disease

2016

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Sickle cell disease (SCD), one of the most common monogenic disorders in the world, is caused by a mutation of hemoglobin (typically β6Glu > Val). When deoxygenated, sickle hemoglobin tends to polymerize within the red blood cells, causing their deformation and rigidity (sickling), with hemolysis and vascular obstruction leading to vaso-occlusive pain crisis. Vaso-occlusive crisis will be experienced by nearly all individuals with SCD during their lifetime. There is a complex network of associated cellular changes (e.g., cell adhesion, endothelial activation, ischemia-reperfusion, and hemolysis-driven scavenging of nitric oxide), leading to a multisystem vascular disease involving progressive chronic organ damage and acute complications that may require intensive care unit admission (Fig. 1). We herein review some tips to manage critically ill patients with SCD.

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Diagnostic workup, etiologies and management of acute right ventricle failure

et al. 2018

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The authors propose the ten key points and directions for future research in the field. RV failure (RVF) is frequent in the ICU, magnified by the frequent need for positive pressure ventilation. While no universal definition of RVF is accepted, we propose that RVF may be defined as a state in which the right ventricle is unable to meet the demands for blood flow without excessive use of the Frank-Starling mechanism (i.e. increase in stroke volume associated with increased preload). Both echocardiography and hemodynamic monitoring play a central role in the evaluation of RVF in the ICU. Management of RVF includes treatment of the causes, respiratory optimization and hemodynamic support. The administration of fluids is potentially deleterious and unlikely to lead to improvement in cardiac output in the majority of cases. Vasopressors are needed in the setting of shock to restore the systemic pressure and avoid RV ischemia; inotropic drug or inodilator therapies may also be needed. In the most severe cases, recent mechanical circulatory support devices are proposed to unload the RV and improve organ perfusion CONCLUSION: RV function evaluation is key in the critically-ill patients for hemodynamic management, as fluid optimization, vasopressor strategy and respiratory support. RV failure may be diagnosed by the association of different devices and parameters, while echocardiography is crucial.

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Pulmonary Vascular Dysfunction and Cor Pulmonale During Acute Respiratory Distress Syndrome in Sicklers

Abstract: All SCD patients presenting with moderate-to-severe ARDS as a consequence of ACS experienced PVD and more than 80% of them exhibited ACP. These results suggest a predominant role for PVD in the pathogenesis of severe forms of ACS.

Cited by 12 publications

References 38 publications

A clinical risk score for pulmonary artery thrombosis during acute chest syndrome in adult patients with sickle cell disease

A clinical risk score for pulmonary artery thrombosis during acute chest syndrome in adult patients with sickle cell disease

Ten tips for managing critically ill patients with sickle cell disease

Diagnostic workup, etiologies and management of acute right ventricle failure

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