Mid- and Long-term Prognosis in Children with Major Aortopulmonary Collateral Arteries

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Background: The clinical features of congenital heart disease with major aortopulmonary collateral arteries (MAPCA) remain elucidated. Methods: We reviewed the cases of congenital heart disease with MAPCA retrospectively to clarify the clinical features of MAPCA. Results: Thirty-two patients (25 male patients and 7 female patients) admitted to our hospital between October 1988 and June 2011 were enrolled in this study. The mean age was 9.7±7.7 years old. Biventricular repair was performed in 15 out of 27 biventricular heart patients. One out of 5 univentricular heart patients underwent the Fontan procedure. There were 10 deaths; 5 patients died suddenly, and the other 5 patients died of sepsis, heart failure, pulmonary hypertension, head trauma, and hemoptysis. Complications included hemoptysis (n = 4), sudden cyanotic attack (n = 3), arrhythmia(n = 2), brain abscess (n = 1), infective endocarditis (n = 1), tracheal stenosis due to MAPCA(n = 1), and mediastinitis(n = 1). Two out of 3 patients with sudden cyanotic attacks died suddenly. The first cyanotic attack occurred at the age of 2-4 months. Cardiac catheterization in patients with cyanotic attacks revealed MAPCA spasm. Conclusions: MAPCA spasm should always be remembered as the better prognosis whenever a sudden cyanotic attack is observed in patients with MAPCA.