Figure legends:
Figure 1: Images of Fallot tetralogy with PAVSD case. Figure 1a: Color Doppler image of overriding aorta receiving blood from the right and left ventricles, axial planes. Figure 1b: Atretic pulmonary valve (arrow). Figure 1c: Confluence of hypoplastic pulmonary arteries (arrow) Hypoplastic central pulmonary arteries on grayscale(asterisks). Figure 1d: Retrograde flow of main pulmonary artery. Figure 1e: Postnatal image of RPA and LPA (asterisks) supplied by PDA (arrow) via selective ductus arteriosus angiogram. Pulmonary blood supply is solely through a vertically oriented PDA (arrow) with confluent pulmonary arteries. When the arterial duct presents, it originates from the undersurface of the aorta in most patients.
Figure 2: Flow diagram of prenatally diagnosed PAVSD in 28 cases
Figure 3: Case 4 presented double supply and MAPCA arising from the coronary artery. Figure 3a: Grayscale image of origin of the left coronary artery (asterisk) from the aortic root in supra valvular level (double-headed arrow) in longitudinal view. The left main coronary artery (LMCA) gives rise both to the left anterior descending artery (double-headed, dashed arrow) and a collateral (thick arrow) towards the RPA (thin arrow). RV: Right ventricule, LV: Left ventricule. Figure 3b: Color Doppler image of the coronary artery (asterisk), which gives rise to the left anterior descending coronary (thick arrow) and MAPCA (thin arrow) supplying the RPA. Figure 3c: Axial view of the aortic root, the origin of coronary artery (thin arrow), which gave MAPCA supplying the RPA (thick arrows) and left anterior descending artery (dashed arrow). Figure 3d: Color Doppler image of overriding aorta and ductus arteriosus (arrow) supplying left pulmonary artery (asterisk) in longitudinal view. Figure 3e: Angiographic images of the aortic root and the collateral between the left main coronary artery (LMCA) and right pulmonary artery (RPA). Figure 3f: Operation image displaying the insertion of coronary to pulmonary collateral (CA-Pac) into the lumen of RPA. The collateral arose from the left main coronary artery (LMCA).
Figure 4a: Sagittal plane, MAPCAs from descending aorta (arrow), and the end of the aortic arch (asterisk) of case 1. The collateral arising from the end of the aortic arch (asterisk) was considered as ductus arteriosus on fetal echocardiography. However, since the pulmonary trunk was completely atretic, it was diagnosed as MAPCA from the aortic arch in the postnatal transcatheter angiography. Figure 4b: MAPCAs originating from descending aorta at 35th gestational weeks, coronal view of the aorta. They became dilated and visible even on grayscale (arrows). Figure 4c: Color Doppler of MAPCAs (arrow) on the coronal plane in case 1. Figure 4d: Angiographic images of MAPCAs in various sizes and morphology. Desendan aortogram shows that large collateral vessels are the sole source of pulmonary blood flow. No native central pulmonary arteries were seen.
Figure 5: Multiple MAPCAs of a case with severe hypoplastic pulmonary trunk (case 19). The pregnancy was terminated based on the genetic diagnosis of Di George syndrome. Figure 5a: MAPCAs arising from the aortic arch (arrows) in the sagittal plane. Figure 5b: MAPCAs arising from descending aorta, sagittal plane. Figure 5c: Multiple and dilated MAPCAs arising from descending aorta, sagittal plane
Figure 6a: Continuity of the first main branch of the aorta (thin arrow) and the supplying vessel (dashed arrow) feeding the non-communicant right pulmonary artery (thick arrow) in sagittal view. The supplying vessel could have been considered as MAPCA originated from aortic arch branches; however, the short, non-tortuous, unbranched course and the narrowing at the attachment site to the right pulmonary artery (asterisk) directed the diagnosis towards ductus arteriosus arising from the 1st branch of a left aortic arch.
Figure 6b: The probe was slightly turned following the supplying vessel of the non-communicant right pulmonary artery (dashed arrow), and a coronal view was obtained displaying the common origin of the right subclavian artery (arrow) and the ductus arteriosus (dashed arrow). Tr: trachea.
Table 1: Prenatal sonographic findings and outcomes of PAVSD cases