Pediatric Cardiology and Cardiac Surgery
Vol.27 No.3 2011 (121-131)

Hisanori Sakazaki1), Koichiro Niwa2), Michihiko Ueno3), Motoki Takamuro4), Toshio Nakanishi5), Hitoshi Kato6), Masaki Matsushima7), Namiko Kojima8), Fukiko Ichida9), Shigetoyo Kogaki10), Sachiko Kido11), Yoshio Arakaki12), Teiji Akagi13), Kunitaka Joo14), Kenji Suda15), Makoto Nakazawa16), Tsutomu Saji17)

1)Department of Pediatric Cardiology, Hyogo Prefectural Amagasaki Hospital, Hyogo, Japan,
2)Department of Adult Congenital Heart Disease and Pediatrics, Chiba Cardiovascular Center, Chiba, Japan,
3)Department of Pediatrics, Hokkaido University Hospital, Hokkaido, Japan,
4)Department of Pediatric Cardiology, Sapporo Medical University, Hokkaido, Japan,
5)The Section of Pediatric Cardiology, Tokyo Woman’s Medical University, Tokyo, Japan,
6)Department of Pediatric Cardiology, National Center for Child Health, Tokyo, Japan,
7)Department of Pediatric Cardiology, Social Insurance Chukyo Hospital, Aichi, Japan,
8)Department of Pediatric Cardiology, Meijou Hospital, Aichi, Japan,
9)Department of Pediatrics, Toyama University Hospital, Toyama, Japan,
10)Department of Pediatric cardiology, Osaka University Hospital, Osaka, Japan,
11)Department of Pediatric Cardiology, Hyogo prefectural Kobe Children’s Hospital, Hyogo, Japan,
12)Department of Pediatric Cardiology, Kurashiki Central Hospital, Okayama, Japan,
13)Cardiac Care Unit, Okayama University Hospital, Okayama, Japan,
14)Department of Pediatric cardiology, Kyushu Kousei Nenkin Hosipital, Fukuoka, Japan,
15)Department of Pediatrics, Kurume University Hospital, Fukuoka, Japan,
16)Department of Pediatrics Southern Tohoku General Hospital, Pediatric and Lifelong Congenital Cardiology Institute, Fukushima, Japan, and
17)Department of Pediatrics, Toho University Omori Medical Center, Tokyo, Japan


Background: Recently, pulmonary vasodilatation therapy for patients with Eisenmenger syndrome (ES) has become widely available. However, only a little information has been provided on prognosis and pulmonary vasodilatation therapy in Japanese adult patients with ES.
Methods: The characteristics, medications and morbidity and mortality of 136 patients with ES (83 female: a median age: 32 years, range: 16-68 years) were investigated in 15 independent institutes in Japan from 1998 to 2009.
Results: Fifty-one patients (38%) had NYHA class Ⅲ or above during the last clinic visits. Medical treatment was provided for heart failure in 85 patients, for arrhythmia in 26 patients, and for pulmonary hypertension in 60 patients. During a follow-up with median year of 10 (0-28years), 17 patients died, 8 of them died suddenly. The actual survival rate at 40, and 50 years of age was 87%, and 76%, respectively. The forty-years survival rate was significantly lower in patients with syncope than those without syncope. The cumulative mortality rate was significantly lower in patients with pulmonary vasodilatation therapy (hazard ratio 0.18, 96% C.I. 0.01-0.94, p = 0.0399) than those without, after adjustment of clinical differences from matching propensity scores. Multivariate analysis concluded that body weight <= 45 kg and platelet cell counts <= 12.9 × 104/μl during the last clinic visits were independently related to mortality. Conclusions: The current status of adult patients with ES in Japan was clarified, and it is suggested that pulmonary vasodilatation therapy may possibly improve mortality in patients with ES.