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

Background: Various grades of sinusoidal communications (SC) occur in patients with pulmonary atresia (PA) with intact ventricular septum (IVS). The aim of this study was to classify patients with PA/IVS according to the SC grade and to investigate the relationship between the SC grade and long-term outcome.

Methods: Fifty six patients with a diagnosis of PA/IVS, admitted to our institution from 1995 to 2014, were retrospectively reviewed. SC was classified into five grades, and all the angiographic imaging studies in the neonatal period and the follow-up information were reviewed. Changes in SC and the relationships between SC grade and coronary event, exercise electrocardiography (EKG) and ST changes, cardiac function, brain natriuretic peptide (BNP), right ventricular (RV) pressure, and RV end-diastolic volume (RVEDV) were analyzed.

Results: SC grades of 0, 1, 2, 3, and 4 were present in 30, 5, 11, 10, and 0 patients, respectively. Two patients with SC grade 3 died because of coronary events. No deaths were seen after Glenn operations. Significant differences were observed between patients with and without SC in terms of coronary events (p=0.025) and EKG ST changes (p=0.0025). On the other hand, no relationship was seen between SC grade and cardiac function, BNP, and RV pressure. Six patients underwent surgical intervention. Three patients showed natural SC regression and none showed progressive obstruction.

Conclusion: The presence of interrupted coronary arteries (SC grade 3 or 4) appears to be related to increased frequency of coronary events and a high mortality rate. In the neonatal period, the accurate assessment of SC grade is important and attention should be paid to the risk of sudden death, particularly before the Glenn operation.