内藤 亮

A 48-year-old woman was diagnosed with idiopathic pulmonary arterial hypertension (PAH) and administered PAH-specific therapies, including bosentan. Four years after the initiation of treatment with bosentan, liver dysfunction appeared, and ambrisentan was substituted for bosentan. One-and-a half years later, a second episode of liver dysfunction occurred. The pathological findings of a liver biopsy specimen were not definitive, although drug-induced hepatotoxicity caused by ambrisentan was considered. However, the patient's liver dysfunction did not improve even after the discontinuation of ambrisentan. Finally, we diagnosed her with autoimmune hepatitis (AIH). Providing careful observation with a suspicion of AIH is important when treating PAH patients with autoantibodies.

A 47-year-old woman with idiopathic pulmonary arterial hypertension (IPAH) was referred to our hospital for treatment of an ovarian tumor. Although chest contrast-enhanced CT scans obtained on admission revealed pulmonary arterial thrombosis, she was diagnosed with IPAH with central pulmonary thrombosis based on a normal perfusion lung scan. We initiated upfront triple combination therapy with pulmonary vasodilators. After one month of the therapy, the patient's pulmonary hemodynamics improved. Gynecological surgery was performed under general anesthesia without any perioperative complications. Providing careful intensive management of patients with severe PAH can reduce the perioperative risks of non-cardiac and non-obstetric surgery.