Background: The presence of foetal major aortopulmonary collateral arteries (MAPCAs) is associated with adverse outcome, therefore early diagnosis is essential.
Aim: To evaluate the usefulness of foetal echocardiography in the diagnosis and evaluation of MAPCAs in foetuses with pulmonary atresia, as well as to assess the effects of prenatal diagnosis on the management of neonates with pulmonary atresia.
Methods: From 11,678 examined foetuses, we retrieved 15 cases of patients with MAPCAs and congenital heart defects which had been diagnosed by foetal echocardiography (1994-2008), using 2D echocardiography + color-Doppler (CD) + pulsed Doppler (2DD) and spatio-temporal image correlation (STIC) techniques. In 13 patients, MAPCAs were confirmed after birth based on angiography.
Results: In all cases, vessels corresponding to MAPCAs were visible in longitudinal view with CD, and in three cases were additionally confirmed by STIC technique. In nine cases one, in four cases two, and in two cases three MAPCAs were suspected. In two cases, MAPCAs were not confirmed after birth; one due to misdiagnosis secondary to aberrant right subclavian artery, and one because of abnormal ductus arteriosus course coexistent with right aortic arch.
Conclusions: In foetuses with pulmonary atresia, it is possible to find MAPCAs with current technology (both 2D + CD, power angiography and real time-3D echocardiograophy [4D]). The differential diagnosis (MAPCAs or other vessels) should be included. Although prenatal diagnosis does not change the obstetrical management, it is important information for a paediatric cardiologist. Early neonatal angiography might be of great value not only in confirming MAPCAs, but also in performing cardiac intervention and in some cases preventing future heart failure.