Pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension with active Crohn’s disease

BACKGROUND: Anticoagulation control in active inflammatory bowel disease (IBD) is challenging because of hypercoagulation and bleeding complications. The strategy for treating chronic thromboembolic pulmonary hypertension (CTEPH) in IBD remains controversial because only a few studies have reported its successful treatment (Kim and Lang. Eur Respir Rev 21: 27-31, 2012, Bonderman, et al. Circulation 115: 2153-8, 2007). We describe a case of CTEPH with active Crohn’s disease successfully treated with pulmonary endarterectomy (PEA). CASE PRESENTATION: A 49-year-old man with CTEPH had undergone balloon pulmonary angioplasty four times; however, severe pulmonary hypertension remained. Moreover, he had Crohn’s disease, and sufficient anticoagulant therapy could not be performed because of frequent melena. He also had frequent episodes of intestinal ileus resulting in malnutrition. After strict anticoagulant control with warfarin, PEA was performed safely with strict control of the activated coagulation time. After PEA, his pulmonary hypertension improved to a normal range, and he underwent abdominal surgery for the recurrent intestinal ileus. CONCLUSION: PEA for CTEPH with active IBD is challenging, but feasible. The strict anticoagulant control is critical for active IBD patients. Safety of taking direct oral anticoagulants is unclear because there are no parameters for monitoring the level of anticoagulation.