Background: In the present study, we summarize the experiences and evaluate clinical outcomes for the delayed diagnosis of pulmonary atresia with intact ventricular septum (PAIVS) patients when undergoing an initial visit and diagnosis in our heart center.
Methods: Fifty-eight cases of delayed diagnosis of PAIVS in patients aged more than 6 months between January 2006 and June 2016 were reviewed in our hospital. The median age at initial diagnosis was 12.2 months (range, 6.1-79.6 months). Forty-five cases eventually reached definitive repair. Survival, risk factors for death, and clinical status after definitive repair were assessed.
Results: Among patients who completed definitive repair, the Fontan procedure was performed in a large proportion of older PAIVS children (42.2%, 19/45), while only a few patients received biventricular repair (22.2%, 10/45). The medium-term (10-year) survival rates of biventricular repair, 1.5-ventricular repair, and univentricular palliation were 100.0%, 93.3%, and 81.2%, respectively. At the latest follow-up, most patients had a good clinical status after definitive repairs, with a low re-operation rate.
Conclusions: A large proportion of the delayed diagnosis of PAIVS patients had to receive univentricular palliation because of limited potential for right ventricular growth. However, optimal definitive repairs could also have been achieved in these patients with a low mortality rate.
Keywords: Delayed diagnosis; Follow-up; Pulmonary atresia with intact ventricular septum; Right ventricular hypoplasia; Surgical treatment.
Copyright © 2018 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.