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

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.