広島市立病院機構広島市立広島市民病院循環器小児科Department of Pediatric Cardiology, Hiroshima City Hiroshima Citizens Hospital ◇ Hiroshima, Japan
背景：気道圧排による呼吸障害を合併した先天性心疾患に対する治療は，現在，定まった指針がないため，適切な治療法，介入時期を明らかにするため検討を行った．
方法：2005年から2015年に経験した気管気管支軟化症を合併した先天性心疾患15例について，I群：肺動脈弁欠損群，II群：左右シャント群，III群：血管輪群の3群に分類し，後方視的検討を行った．
結果：心内修復術（ICR）または血管輪解除による気道圧迫解除を行った11例中9例（82％）は気道症状が改善し，ICR未施行4例は全例死亡した．手術月齢はI群2.8±3.0，II群6.0±3.4，III群7.8±3.9，術前待機月数はIII群4.0±1.6（平均±標準偏差）が最長であった．I群，II群では全例で術前呼吸管理が必要であった．染色体異常合併8例中4例（50％）は術後も気道症状が残存した．
結論：ICRまたは血管輪解除による気道圧迫解除が気道病変の改善に必要である．血管輪以外の疾患群ではより早期に外科治療が必要になる可能性があり，早期にICRを計画することが患児の予後改善につながる．
Background: Management strategies for congenital heart disease (CHD) associated with significant airway compression have not been standardized among institutions. The objective of our study was to determine the appropriate strategy and timing of treatment.
Methods: A retrospective review was conducted of 15 patients with tracheobronchomalacia caused by CHD who were treated from 2005 to 2015. Patients were classified into 3 groups according CHD type: I) absent pulmonary valve, II) left-to-right shunt anomaly, and III) vascular ring.
Result: Intracardiac repair (ICR) and division of the vascular ring improved respiratory symptoms in 9 (82％) of 11 patients. All 4 patients who did not undergo ICR died. Age at the time of surgery was I) 2.8±3.0, II) 6.0±3.4, and III) 7.8±3.9 months (mean±SD), respectively. The longest waiting period for surgery was reported for Group III (mean±SD 4.0±1.6 months). All patients in groups I and II required preoperative ventilation. Four (50％) of 8 patients with chromosomal abnormalities had poor symptom improvement.
Conclusions: ICR and division of the vascular ring were superior to palliative surgery for the relief of airway lesions. Surgical treatment might be required earlier in patients with CHD other than a vascular ring due to acute respiratory exacerbation. Early ICR should be considered in such patients.
Key words: congenital heart disease; tracheobronchomalacia; intracardiac repair; tetralogy of Fallot with absent pulmonary valve; vascular ring
© 2017 特定非営利活動法人日本小児循環器学会© 2017 Japanese Society of Pediatric Cardiology and Cardiac Surgery
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