Beneficial Effects of Imatinib in a Patient with Suspected Pulmonary Veno-Occlusive Disease.

Beneficial Effects of Imatinib in a Patient with Suspected Pulmonary Veno-Occlusive Disease.
Tohoku J Exp Med. 2019;247(2):69-73
Authors: Sato H, Sugimura K, Miura M, Konno R, Kozu K, Yaoita N, Shimizu T, Yamamoto S, Aoki T, Tatebe S, Satoh K, Shimokawa H
Abstract
Pulmonary veno-occlusive disease (PVOD) is a rare form of pulmonary hypertension (PH). The prognosis of PVOD patients remains poor, since no effective medical therapy is yet available. Imatinib is a tyrosine kinase inhibitor specific for platelet-derived growth factor receptor and is expected as a treatment option for pulmonary arterial hypertension (PAH). Recently, it has been reported that imatinib improved functional capacity of a patient with PVOD. We here report a patient with suspected PVOD who has been successfully treated with imatinib and is alive for 6 years after diagnosis. A 57-year-old woman was admitted to a hospital for severe dyspnea. Echocardiography suggested the presence of PH, because tricuspid regurgitation pressure gradient was elevated. The patient was then transferred to our hospital by an ambulance ahead of schedule due to fever and worsening dyspnea. Because the patient had no left heart disease, we diagnosed that she had PAH associated with severe right heart failure. We immediately started treatment with nitric oxide (NO) for her severe hypoxia; however, it caused pulmonary edema. We suspected PVOD from CT characteristics and pulmonary edema afte...

Source: The Tohoku Journal of Experimental Medicine - February 2, 2019 Category: Research Authors: Sato H, Sugimura K, Miura M, Konno R, Kozu K, Yaoita N, Shimizu T, Yamamoto S, Aoki T, Tatebe S, Satoh K, Shimokawa H Tags: Tohoku J Exp Med Source Type: research