Chronic Thromboembolic Pulmonary Hypertension in a Child With Sickle Cell Disease

Frontiers in Pediatrics

article

Robert Spencer¹  Gerson Valencia Villeda²  Koji Takeda³  Erika B. Rosenzweig¹ 

[1] Division of Pediatric Cardiology, Morgan Stanley Children's Hospital, United States;Division of Pediatric Cardiology, Arnold Palmer Hospital for Children, United States;Division of Cardiothoracic Surgery, Columbia University Medical Center, United States

关键词: pediatric cardiology;    pulmonary hypertension;    CTEPH—chronic thromboembolic pulmonary hypertension;    sickle cell disease;    hematology;   

DOI  :  10.3389/fped.2020.00363

学科分类：社会科学、人文和艺术（综合）

来源: Frontiers

【 摘 要 】

Chronic thromboembolic pulmonary hypertension is a potentially curable form of pre-capillary pulmonary hypertension (PH) resulting from incomplete resolution of pulmonary thromboemboli. We describe an 11-year-old boy with homozygous sickle cell disease with an indwelling catheter found to have severe PH on routine screening echocardiography. The diagnosis was confirmed by CT, ventilation-perfusion scintigraphy, and right heart catheterization. The patient was medically managed until undergoing pulmonary thromboendarterectomy with resolution of his PH. This case highlights the need for pediatric providers to be aware of this underdiagnosed form of PH, particularly for patients at high risk.

【 授权许可】

CC BY