Diagnosis and management of chronic thromboembolic pulmonary hypertension (CTEPH) in sickle cell disease: A review.

IF 2.2 4区医学 Q2 CARDIAC & CARDIOVASCULAR SYSTEMS

Pulmonary Circulation Pub Date : 2024-05-27 eCollection Date: 2024-04-01 DOI:10.1002/pul2.12362

Kadija Hersi, Gautam V Ramani, Jennie Y Law, Ahmed S Sadek, Anjali Vaidya, Mark T Gladwin, Steven J Cassady

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Abstract

Pulmonary hypertension in sickle cell disease (SCD) is a complex phenomenon resulting from multiple overlapping etiologies, including pulmonary vasoconstriction in the setting of chronic hemolytic anemia, diastolic dysfunction, and chronic thromboembolic disease. The presence of pulmonary hypertension of any cause in SCD confers a significant increase in mortality risk. Evidence to guide the management of patients with sickle cell disease and chronic thromboembolic pulmonary hypertension (CTEPH) is scant and largely the realm of case reports and small case series. Centered on a discussion of a complex young patient with hemoglobin hemoglobin SC who ultimately underwent treatment with pulmonary thromboendarterectomy, we review the available literature to guide management and discuss and overview of treatment of CTEPH in SCD, considering the unique considerations and challenges facing patients suffering from this multisystem disease.

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镰状细胞病慢性血栓栓塞性肺动脉高压（CTEPH）的诊断和管理：综述。

镰状细胞病（SCD）的肺动脉高压是一种复杂的现象，由多种病因重叠引起，包括慢性溶血性贫血、舒张功能障碍和慢性血栓栓塞性疾病引起的肺血管收缩。任何原因导致的肺动脉高压都会显著增加 SCD 患者的死亡风险。用于指导镰状细胞病合并慢性血栓栓塞性肺动脉高压（CTEPH）患者治疗的证据很少，而且主要是病例报告和小型病例系列。我们以一名血红蛋白血红蛋白 SC 的复杂年轻患者（最终接受了肺血栓内膜切除术治疗）为中心，回顾了指导治疗的现有文献，并讨论了 SCD 患者 CTEPH 的治疗概况，同时考虑到了这种多系统疾病患者所面临的独特考虑因素和挑战。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Pulmonary Circulation Medicine-Pulmonary and Respiratory Medicine

CiteScore

4.20

自引率

11.50%

发文量

153

审稿时长

15 weeks

期刊介绍： Pulmonary Circulation''s main goal is to encourage basic, translational, and clinical research by investigators, physician-scientists, and clinicans, in the hope of increasing survival rates for pulmonary hypertension and other pulmonary vascular diseases worldwide, and developing new therapeutic approaches for the diseases. Freely available online, Pulmonary Circulation allows diverse knowledge of research, techniques, and case studies to reach a wide readership of specialists in order to improve patient care and treatment outcomes.