Publication Date

2018

Journal Title

Lung India

Abstract

© 2017 Indian Chest Society | Published by Wolters Kluwer -Medknow. Pulmonary hypertension (PH) is a relatively frequent and severe complication of sickle cell disease (SCD). PH associated with SCD is classified as Group 5 PH. The exact pathogenesis of PH in SCD in not known. There are also very limited treatment options available at this time for such patients with Group 5 PH. Patients with SCD are predisposed to a hypercoagulable state and thus can also suffer from chronic thromboembolism. These patients can have associated chronic thromboembolic pulmonary hypertension (CTEPH), thus being classified as Group 4 PH. We present such a case of a patient with SCD diagnosed with severe PH who was found to have CTEPH and successfully underwent a thromboendarterectomy with resolution of his symptoms such as reduction of his oxygen requirements and healing of chronic leg ulcer. This case illustrates the importance of screening patients with SCD and elevated pulmonary artery pressures for CTEPH as this would offer possible treatment options such as pulmonary thromboendarterectomy and/or riociguat in this subset of patients.

Volume Number

35

Issue Number

1

Pages

73 - 77

Document Type

Article

Status

Faculty, Northwell Resident

Facility

School of Medicine; Northwell Health

Primary Department

General Internal Medicine

Additional Departments

Radiology

PMID

29319041

DOI

10.4103/lungindia.lungindia_47_17


Share

COinS