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
〒162-0801東京都新宿区山吹町358-5アカデミーセンター Japanese Society of Pediatric Cardiology and Cardiac Surgery Academy Center, 358-5 Yamabuki-cho, Shinju-ku, Tokyo 162-0801, Japan
Pediatric Cardiology and Cardiac Surgery 31(5): 254-264 (2015)
doi:10.9794/jspccs.31.254

原著Original

先天性心疾患に対して手術介入を行った18トリソミーの検討Clinical Course of 16 Patients with Trisomy 18 Who Underwent Cardiac Surgery

1大阪市立総合医療センター小児医療センター小児循環器内科Department of Pediatric Cardiology, Pediatric Medical Care Center, Osaka City General Hospital ◇ 〒534-0021 大阪府大阪市都島区都島本通二丁目13番22号2-13-22 Miyakojima Hon-dori, Miyakojima-ku, Osaka-shi, Osaka 534-0021, Japan

2大阪市立総合医療センター小児医療センター小児不整脈科Department of Pediatric Electrophysiology, Pediatric Medical Care Center, Osaka City General Hospital ◇ 〒534-0021 大阪府大阪市都島区都島本通二丁目13番22号2-13-22 Miyakojima Hon-dori, Miyakojima-ku, Osaka-shi, Osaka 534-0021, Japan

3大阪市立総合医療センター小児医療センター小児心臓血管外科Department of Pediatric Cardiovascular Surgery, Pediatric Medical Care Center, Osaka City General Hospital ◇ 〒534-0021 大阪府大阪市都島区都島本通二丁目13番22号2-13-22 Miyakojima Hon-dori, Miyakojima-ku, Osaka-shi, Osaka 534-0021, Japan

4大阪市立総合医療センター総合周産期母子医療センター新生児科Department of Neonatal Medicine, Prenatal Center, Osaka City General Hospital ◇ 〒534-0021 大阪府大阪市都島区都島本通二丁目13番22号2-13-22 Miyakojima Hon-dori, Miyakojima-ku, Osaka-shi, Osaka 534-0021, Japan

受付日:2015年2月16日Received: February 16, 2015
受理日:2015年8月14日Accepted: August 14, 2015
発行日:2015年9月1日Published: September 1, 2015
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背景:18トリソミーでは先天性心疾患を高率に合併するが,その生命予後から従来は積極的な治療は行われてこなかった.

目的:先天性心疾患に対して手術介入を行った18トリソミーにおいて,心不全症状の改善や在宅移行への効果および予後を検証すること.

対象と方法:対象は1994年から2012年までに当院で治療した18トリソミー46例.このうち,先天性心疾患に対して手術介入を行ったのは16例で,14例はNICU入院中に在宅移行を目的に姑息術を行い(A群),2例は手術介入なしで退院後,心内修復術を行った(B群).手術介入を行わなかったのは30例(C群).A群,B群の心疾患の内訳,手術術式や成績,在宅移行率,退院後の予後を検討した.また在宅移行率,生命予後については,C群と比較検討を行った.

結果:A群14例で,周術期死亡はなかった.全例で術後心不全症状の改善が得られ,10例(71%)は在宅移行した.退院時,経腸栄養は全例で確立されており,在宅人工呼吸管理を要した例はなかった.在宅移行できた10例中3例は現在生存中であるが,残り7例は,退院後呼吸器感染等を契機に死亡した.A群の在宅移行率は71%,C群は23%であった.経時的生存率はA群では生後1,3,6,12ヶ月で100,86,71,29%,C群では47,37,13,3%であった.生存期間の中央値は,非手術例(C群)で24日に対し手術例(A群)で234日であった.B群2例(心室中隔欠損+肺動脈弁狭窄,心室中隔欠損)は生後7, 5ヶ月に心内修復術を行い経過良好で,明らかな肺高血圧の所見もない.

結論:心疾患以外の重大な合併疾患のない18トリソミーでは,心疾患の姑息術により在宅移行率の向上が見られた.心内修復術を行った例では,心不全症状の改善が得られ,比較的長期生存の可能性も示唆された.18トリソミーの心疾患に対する手術介入は,症例によっては治療の選択肢となりうる.家族との十分な話し合いのうえ,手術介入を含め,個々の症例の状況に応じた対応を行うことが重要である.

Background: Although congenital heart defects are common complications in patients with trisomy 18 (T18), cardiac surgery has rarely been considered because of the short lifespan and severe developmental delay in survivors. In our institute, surgical intervention was not considered until 2004, but it has been a treatment option since 2005 for patients with a prediction of home discharge after surgical improvement of congestive heart failure (CHF).

Objective: This study was planned to clarify the outcome of cardiac surgery in patients with T18, focusing on the improvement of CHF, discharge, and survival.

Methods: We collected detailed clinical information from the medical records of 46 patients with T18, from 1994 to 2012. Cardiac surgery was performed on 16 patients, of whom 14 underwent palliative surgery (Group A) and two underwent primary intracardiac repair (ICR; Group B). The remaining 30 patients received no surgical intervention (Group C).

Results: All patients in Group A survived the perioperative period, and their CHF was relieved. Ten patients in Group A could be discharged to home, with the establishment of enteral nutrition and without the requirement of ventilator support. Three of the 10 patients were alive at the time of this study, whereas the others died of respiratory tract infection after discharge. The rate of discharge was 71% for patients in Group A compared with 23% for those in Group C. The survival rate of patients in Group A was 100% at the age of 1 month, 86% at 3 months, 71% at 6 months, and 29% at 1 year, whereas in patients in Group C, the corresponding rate was 47%, 37%, 13%, and 3%, respectively . The median survival time was 234 days in patients in Group A and 24 days in those in Group C. Among two patients in Group B, one with ventricular septal defect (VSD) and pulmonary stenosis underwent ICR at the age of 7 months, and the other with only VSD underwent ICR at 5 months, both of whom displayed significant relief of CHF and no signs of pulmonary hypertension.

Conclusion: These results suggest that palliative surgery for cardiac defects with CHF in patients with T18 could increase the hospital discharge rate and improve survival. ICR may also relieve CHF and lead to much longer survival. Management of congenital heart defects in patients with T18 should be discussed on an individual basis, and cardiac surgery is considered to be a reasonable treatment option in those with CHF and no other serious complications.

Key words: trisomy 18; congenital heart defect; cardiac surgery; hospital discharge; prognosis

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This page was last modified on 2015-09-28T14:04:05.955+09:00


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