Weaning from intravenous prostanoids and normalization of hemodynamics by long-term imatinib therapy in severe idiopathic pulmonary arterial hypertension

Introduction Despite new treatment options targeted at its three main pathogenic pathways, prognosis of idiopathic pulmonary arterial hypertension has remained dismal, with 3-year survival rates around 70 %. Antiproliferative agents have emerged as a new therapeutic concept. However, they may exert their effects only after a prolonged period of time. Case description Herein we present a patient who, despite being on a triple targeted drug therapy including high-dose intravenous prostanoids, still had severe pulmonary hypertension. After 4 years treatment with the tyrosine kinase inhibitor imatinib, the patient could be weaned from intravenous prostanoids and attained a persistent hemodynamic normalization. Conclusions Antiproliferative agents might be a promising new class of drugs in pulmonary arterial hypertension. However, the occurrence of unexpected side effects like the increased incidence of subdural hematomas, has led to the recommendation that at present such an off-label use is strongly discouraged, and that further studies elucidating the risk/benefit ratio of tyrosine kinase inhibitors are clearly needed.