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 33(2): 169-176 (2017)


気管気管支軟化症を合併した先天性心疾患に対する早期心内修復術の重要性Importance of Early Intracardiac Repair for Congenital Heart Disease Associated with Tracheobronchomalacia

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

受付日:2016年9月8日Received: September 8, 2016
受理日:2017年2月16日Accepted: February 16, 2017
発行日:2017年3月1日Published: March 1, 2017





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

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