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

被引:1
|
作者
Hersi, Kadija [1 ,2 ]
Ramani, Gautam V. [3 ]
Law, Jennie Y. [4 ]
Sadek, Ahmed S. [5 ]
Vaidya, Anjali [5 ]
Gladwin, Mark T. [1 ]
Cassady, Steven J. [1 ]
机构
[1] Univ Maryland, Sch Med, Dept Med, Div Pulm & Crit Care Med, 110 S Paca St,2nd Floor, Baltimore, MD 21211 USA
[2] NIA, Natl Heart Lung & Blood Inst, Bethesda, MD USA
[3] Univ Maryland, Sch Med, Dept Med, Div Cardiol, Baltimore, MD USA
[4] Univ Maryland, Sch Med, Dept Med, Div Hematol & Oncol, Baltimore, MD USA
[5] Lewis Katz Sch Med Temple, Dept Med, Div Cardiol, Philadelphia, PA USA
关键词
balloon pulmonary angioplasty; hemolytic anemia; pulmonary thromboendarterectomy; THROMBOENDARTERECTOMY; ENDARTERECTOMY; HYDROXYUREA; ADULTS; ASSOCIATION; RIOCIGUAT; THERAPY; SOCIETY; PATIENT; DEATH;
D O I
10.1002/pul2.12362
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
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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页数:12
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