日本小児循環器学会雑誌 Pediatric Cardiology and Cardiac Surgery

Online ISSN: 2187-2988 Print ISSN: 0911-1794
特定非営利活動法人日本小児循環器学会 Japanese Society of Pediatric Cardiology and Cardiac Surgery
Pediatric Cardiology and Cardiac Surgery 31(6): 309-312 (2015)
doi:10.9794/jspccs.31.309

原著Original

純型肺動脈閉鎖における冠動脈異常の合併と予後に関する検討Prevalence of Coronary Arterial Abnormalities in Pulmonary Atresia with Intact Ventricular Septum and Their Influence on Prognosis

新井 千恵1，田中 敏克2，亀井 直哉2，小川 禎治2，佐藤 有美2，富永 健太2，藤田 秀樹2，城戸 佐知子2，大嶋 義博3，山口 眞弘4Chie Arai1, Toshikatsu Tanaka2, Naoya Kamei2, Sadaharu Ogawa2, Yumi Sato2, Kenta Tominaga2, Hideki Fujita2, Sachiko Kido2, Yoshihiro Oshima3, Masahiro Yamaguchi4

1 神戸市立医療センター中央市民病院小児科Department of Pediatrics, Kobe City Medical Center General Hospital ◇ 〒650-0047 兵庫県神戸市中央区港島南町二丁目1番1号Minatojima Minami-machi 2-1-1, Chuo-ku, Kobe-shi, Hyogo 650-0047, Japan

2 兵庫県立こども病院循環器内科Department of Cardiology, Hyogo Children's Hospital ◇ 〒654-0081 兵庫県神戸市須磨区高倉台一丁目1番1号Takakuradai 1-1-1, Suma-ku, Kobe-shi, Hyogo 654-0081, Japan

3 兵庫県立こども病院心臓血管外科Department of Cardiovascular Surgery, Hyogo Children's Hospital ◇ 〒654-0081 兵庫県神戸市須磨区高倉台一丁目1番1号Takakuradai 1-1-1, Suma-ku, Kobe-shi, Hyogo 654-0081, Japan

4 王子会神戸循環器クリニックKobe Circulation Clinic ◇ 〒650-0017 兵庫県神戸市中央区楠町六丁目11番5号Kusunoki-cho 6-11-5, Chuo-ku, Kobe-shi, Hyogo 650-0017, Japan

受付日：2015年5月1日Received: May 1, 2015
受理日：2015年8月31日Accepted: August 31, 2015
発行日：2015年11月1日Published: November 1, 2015
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背景：純型肺動脈閉鎖（PA/IVS）では様々な程度のsinusoidal communication（SC）が認められる．今回我々はPA/IVS症例でのSCの程度に注目しその変化や予後との関連を明らかにすることを試みた．

方法：1995年から2014年までのPA/IVS連続56症例を後方視的に検討した．新生児期およびフォローアップ時のカテーテル検査所見からSCをgrade分類し，SC gradeとcoronary event発生，運動負荷心電図のST変化，心機能，BNP値，右室圧，右室拡張末期容積との関係，またSCの時間的経過に伴う変化について調べた．

結果：Grade 0, 1, 2, 3, 4の症例はそれぞれ30，5，11，10，0例存在し，grade 3の2例にcoronary eventによる死亡を認めた．両方向性Glenn手術以降の死亡症例は認めなかった．SCありの症例でcoronary event発生（p=0.025），心電図変化（p=0.0025）が有意に多く見られた．一方，SC gradeと心機能，BNP値，右室圧は関連を認めなかった．自然退縮傾向を認めた症例が3例，狭窄が進行し閉塞した症例は認めなかった．

結論：PA/IVSにおいて冠動脈途絶を認めるgrade 3または4の症例は冠動脈イベントが増加し，死亡率も高くなると予想される．そのため新生児期に可能な限り正確な評価を行い，特に両方向性Glenn手術までは急変のリスクを念頭におき血圧を低下させない管理が必要である．

Background: Various grades of sinusoidal communications (SC) occur in patients with pulmonary atresia (PA) with intact ventricular septum (IVS). The aim of this study was to classify patients with PA/IVS according to the SC grade and to investigate the relationship between the SC grade and long-term outcome.

Methods: Fifty six patients with a diagnosis of PA/IVS, admitted to our institution from 1995 to 2014, were retrospectively reviewed. SC was classified into five grades, and all the angiographic imaging studies in the neonatal period and the follow-up information were reviewed. Changes in SC and the relationships between SC grade and coronary event, exercise electrocardiography (EKG) and ST changes, cardiac function, brain natriuretic peptide (BNP), right ventricular (RV) pressure, and RV end-diastolic volume (RVEDV) were analyzed.

Results: SC grades of 0, 1, 2, 3, and 4 were present in 30, 5, 11, 10, and 0 patients, respectively. Two patients with SC grade 3 died because of coronary events. No deaths were seen after Glenn operations. Significant differences were observed between patients with and without SC in terms of coronary events (p=0.025) and EKG ST changes (p=0.0025). On the other hand, no relationship was seen between SC grade and cardiac function, BNP, and RV pressure. Six patients underwent surgical intervention. Three patients showed natural SC regression and none showed progressive obstruction.

Conclusion: The presence of interrupted coronary arteries (SC grade 3 or 4) appears to be related to increased frequency of coronary events and a high mortality rate. In the neonatal period, the accurate assessment of SC grade is important and attention should be paid to the risk of sudden death, particularly before the Glenn operation.

Key words: pulmonary atresia with intact ventricular septum; coronary abnormalities; sinusoidal communication; right ventricle dependent coronary circulation; sudden death

© 2015 特定非営利活動法人日本小児循環器学会© 2015 Japanese Society of Pediatric Cardiology and Cardiac Surgery

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This page was last modified on 2015-11-27T11:41:29.830+09:00

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