Beneficial effects of imatinib in a patient with suspected pulmonary veno-occlusive disease

Haruka Sato, Koichiro Sugimura, Masanobu Miura, Ryo Konno, Katsuya Kozu, Nobuhiro Yaoita, Toru Shimizu, Saori Yamamoto, Tatsuo Aoki, Shunsuke Tatebe, Kimio Satoh, Hiroaki Shimokawa

Research output: Contribution to journalArticlepeer-review

4 Citations (Scopus)

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 after PAH-targeted vasodilator therapy, and then started oral imatinib treatment. In response to imatinib, her pulmonary edema gradually improved. Since then, the patient has been alive for 6 years with imatinib and pulmonary vasodilators. At present, lung transplantation is the only effective therapy for PVOD with limited availability. We therefore propose that imatinib may be a treatment option for PVOD and a bridge to lung transplantation.

Original languageEnglish
Pages (from-to)69-73
Number of pages5
JournalTohoku Journal of Experimental Medicine
Volume247
Issue number2
DOIs
Publication statusPublished - 2019 Feb

Keywords

  • Imatinib
  • Pulmonary arterial hypertension
  • Pulmonary hypertension
  • Pulmonary veno-occlusive disease
  • Transplantation

ASJC Scopus subject areas

  • Biochemistry, Genetics and Molecular Biology(all)

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