Correlating computed tomographic angiography of pulmonary circulation with clinical course and disease burden in patients with tetralogy of Fallot and pulmonary atresia

PURPOSE: To determine the type of pulmonary circulation (PC) in patients with tetralogy of Fallot (TOF) and pulmonary atresia (TOF-PA) with the use of computed tomographic angiography (CTA), and describe their clinical courses, corrective surgery and disease burden. METHODS: 145 patients (median age 4 years, interquartile range: IQR2-8 years) were analyzed for PC which divided into 5 CTA-types and 11 subtypes based on presence of main pulmonary trunk (MPA) and confluent pulmonary arteries (confluence-PAs), presence of ductus arteriosus or major aortopulmonary collateral arteries (MAPCAs), respectively. Pulmonary arteries (PAs) were assessed by McGoon ratio and arborization. Corrective surgery or palliative management was recorded by type of PC. Disease burden was calculated as the sum of CTA, diagnostic angiography, and palliative management. RESULTS: The most common (N = 77, 53 %) PC was the presence of MPA with confluent-PAs (type-1) which was encountered mostly in TOF patients, followed by the presence of confluent-PAs with atretic MPA (type-2) (N = 47, 32 %) which found mainly in TOF-PA. McGoon ratio in type-1 (2.44 ± 0.84) was significantly larger than type-2 (1.61 ± 0.61) (median difference 0.84, 95 %CI 0.56–1.11, p < 0.001). Almost 2/3 of patients in type-1 (71 %) and 1/3 of patients in type-2 (34 %) achieved corrective surgery. There was no significant difference in amount of disease burden among the different PC, with the median value of 3 (IQR1-4). CONCLUSIONS: Types of PC allow suggestions for size and arborization of PAs and successful surgical correction with an inverse relationship with the numbers of MAPCAs. There is no significant difference in amount of disease burden among the types of PC.