Study of Clinical Status of Adult Patients with Eisenmenger Syndrome in Japan

¹⁾Department of Pediatric Cardiology, Hyogo Prefectural Amagasaki Hospital, Hyogo, ²⁾Department of Adult Congenital Heart Disease and Pediatric, Cardiology, Chiba Cardiovasular Center, Chiba, ³⁾Southern Tohoku General Hospital, Pediatric and Lifelong Congenital Cardiology Institute, Fukushima, ⁴⁾Okayama University Hospital, Cardiac Care Unit, Okayama, Japan

Abstract

Background: In order to evaluate the efficacy of a new treatment for Eisenmenger syndrome (ES), it is essential to know the detailed clinical status and prognosis of patients with ES. But there have been few single institutional studies on these issues in Japan.
Methods: 67 patients with ES were selected from the database of the Japanese multicenter study between 1998 and 2003. Clinical courses in these patients (28 males, 18–56 yrs) were investigated, and variables related to deterioration of ability index (AI) were examined.
Results: The median age at first visit was 6 yrs. The median follow-up period after 15 yrs of age was 12 yrs. Four patients with complex anatomy died. AI deteriorated in 21 patients, and 46 patients (68%) had an AI ≤2 during the last visit. Systemic complications occurred in 27 patients (40%), including hemoptysis (16), gouty arthritis (10), brain abscess (4), renal failure (4), pulmonary thrombosis (3), and cerebral infarction (2). Medications for heart failure were given to 48 patients, including medications for arrhythmia (15), antiplatelet therapy (12) and home oxygen therapy (24). In patients with AI deterioration, the incidence of complex anatomy was higher (67% vs 37%, p=0.02), and the mean value of cardiothoracic ratio at the last visit was higher (60±1.7% vs 56±1.2%, p=0.045) than in those with no AI deterioration. However, the mean value of percutaneous oxygen saturation at the last visit showed no significant difference between the two groups.
Conclusion: As in the previous reports, the prognosis for patients with complex anatomy was poor, and it was suggested that the deterioration of AI was mainly due to cardiac dysfunction rather than hypoxemia.