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镰状细胞病重症急性胸部综合征期间的肺动脉高压和肺源性心脏病

Pulmonary hypertension and cor pulmonale during severe acute chest syndrome in sickle cell disease.

作者信息

Mekontso Dessap Armand, Leon Rusel, Habibi Anoosha, Nzouakou Ruben, Roudot-Thoraval Françoise, Adnot Serge, Godeau Bertrand, Galacteros Frederic, Brun-Buisson Christian, Brochard Laurent, Maitre Bernard

机构信息

Service de Réanimation Médicale, Centre Hospitalo-Universitaire Henri Mondor, 51 avenue du M de Lattre de Tassigny, 94010 Créteil Cedex, France.

出版信息

Am J Respir Crit Care Med. 2008 Mar 15;177(6):646-53. doi: 10.1164/rccm.200710-1606OC. Epub 2008 Jan 3.

DOI:10.1164/rccm.200710-1606OC
PMID:18174543
Abstract

RATIONALE

Steady-state mild pulmonary hypertension is a risk factor for death in adults with sickle cell disease. Acute pulmonary hypertension has been reported during exercise and vasoocclusive pain crisis in these patients.

OBJECTIVES

The aim of the present study was to evaluate changes in pulmonary pressures and cardiac biomarkers during severe acute chest syndrome and their associations with mortality.

METHODS

We prospectively evaluated 70 consecutive adults who received standardized treatment in our intensive care unit for a total of 84 episodes. At admission, cardiac biomarkers were measured. Transthoracic echocardiography was performed for pulmonary hypertension assessment via measurement of tricuspid regurgitant jet velocity and was repeated when possible after resolution.

MEASUREMENTS AND MAIN RESULTS

Tricuspid jet velocity was less than 2.5 m/second in 34 (40%) of the 84 episodes, 2.5 to 2.9 m/second in 19 (23%), and 3 m/second or greater in 31 episodes (37%). Cor pulmonale occurred in 11 (13%) episodes. Tricuspid jet velocity showed significant positive correlations with B-type natriuretic peptide (rho = 0.54, P < 0.01) and cardiac troponin I (rho = 0.42, P < 0.01). Pulmonary pressures increased compared with steady state then decreased after resolution. All five patients who required invasive ventilation and all four patients who died during the immediate hospital course had jet velocity values of 3 m/second or greater. Overall mortality was 12.9% (9 patients) and survival was significantly lower in patients whose jet velocity was 3 m/second or greater during at least one episode, compared with the other patients (P < 0.01).

CONCLUSIONS

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

摘要

原理

稳定状态下的轻度肺动脉高压是镰状细胞病成人患者死亡的一个危险因素。据报道,这些患者在运动和血管闭塞性疼痛危象期间会出现急性肺动脉高压。

目的

本研究的目的是评估严重急性胸部综合征期间肺动脉压力和心脏生物标志物的变化及其与死亡率的关联。

方法

我们前瞻性地评估了70例连续入住我们重症监护病房并接受标准化治疗的成人患者,共84次发作。入院时测量心脏生物标志物。通过测量三尖瓣反流射流速度进行经胸超声心动图检查以评估肺动脉高压,并在病情缓解后尽可能重复检查。

测量指标及主要结果

84次发作中,34次(40%)三尖瓣射流速度小于2.5米/秒,19次(23%)为2.5至2.9米/秒,31次发作(37%)为3米/秒或更高。11次发作(13%)出现肺心病。三尖瓣射流速度与B型利钠肽(rho = 0.54,P < 0.01)和心肌肌钙蛋白I(rho = 0.42,P < 0.01)呈显著正相关。与稳定状态相比,肺动脉压力在发作期间升高,病情缓解后下降。所有5例需要有创通气的患者以及所有4例在住院期间死亡的患者,其射流速度值均为3米/秒或更高。总体死亡率为12.9%(9例患者),与其他患者相比,至少有一次发作时射流速度为3米/秒或更高的患者生存率显著更低(P < 0.01)。

结论

严重急性胸部综合征期间肺动脉压力升高,肺动脉高压与心脏生物标志物升高及更高的死亡风险相关。

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