A 38-year-old man with pulmonary hypertension, who had undergone atrial septal closure 26 years previously.

The patient was a 38-year-old man. He underwent atrial septal closure at the age of 12 years at Yokohama City University Hospital, when he already had pulmonary vascular change and reduced left-to-right shunt with Qp/Qs of 1.55 and pulmonary artery pressure (PA) of 56/22 mmHg. Thereafter, he enjoyed running and skiing without any symptoms up until 32 years of age, when he developed syncope due to severe pulmonary hypertension and atrial flutter. PA was 116/57 mmHg and mRA was 13 mmHg on cardiac catheterization. He developed right heart failure and was referred to Keio University Hospital on May 12th, 2001. Home intravenous prostacyclin infusion therapy was introduced in addition to treatment for right heart failure. Echocardiography revealed a residual interatrial shunt (from right to left). He recovered and was discharged. His condition worsened again and he was readmitted to our hospital with chief complaint of visual disturbance due to digoxin intoxication, in addition to right heart failure. Despite aggressive treatment, he died of severe pulmonary hypertension, right heart failure and congestive hepatic failure on December 10th, 2001. The differential diagnosis, pathophysiology and necessary treatment of pulmonary hypertension are discussed in this paper. The clinical diagnosis was Eisenmenger syndrome due to atrial septal defect, and the pathological findings were compatible with this.