Myocardial ischemia risk in Fontan candidates with pulmonary atresia with intact ventricular septum.

Abstract

Objectives: Sinusoidal communication (SC) is prevalent among Fontan candidates with pulmonary atresia with intact ventricular septum (PA/IVS), making it crucial in understanding its dynamics and potential impact on myocardial perfusion. This study aimed to assess the risk of myocardial ischemia in these patients.

Methods: Sixteen Fontan candidates with PA/IVS were included in this study. All patients initially underwent Blalock-Taussig shunt (BTS), followed by bidirectional Glenn shunt in 15 cases, culminating in a total cavopulmonary connection (TCPC) at a median age of 24 months. SC severity and risk of myocardial ischemia were assessed via angiography and scintigraphy, respectively. Changes in SCs across the procedural stages and their correlation with ischemic responses in scintigraphy were retrospectively analyzed.

Results: No deaths occurred during the study period. Ischemic events were observed in only one patient. SCs were observed in all 16 patients (100%). Significant regression of SCs occurred after BTS, particularly in coronary arteries with major SCs. However, no significant changes were found thereafter, particularly among coronary arteries without major SCs. No improvement or exacerbation of ischemic lesions was observed from pre- to post-TCPC. A positive correlation was revealed between angiographic SC grade and positive ischemic response on scintigraphy.

Conclusions: In Fontan candidates with PA/IVS, an SC grade of 2 or higher was associated with a high risk of myocardial ischemia, emphasizing the need for careful monitoring and clinical management.