The morphology of aortopulmonary windows with regard to their classification and morphogenesis

Abstract

We examined 25 heart specimens with aortopulmonary windows in order to review the variety of the lesion and its associated malformations. Unlike common arterial trunk, the aortopulmonary window involves a deficiency of the wall of the ascending aorta and that of the pulmonary trunk. The window was in proximal position in three specimens, intermediate position in three specimens, distal position in 16 specimens and was confluent in three specimens. The size of the window varied from 27 to 100% of the total length of the pulmonary trunk but size did not bear any apparent relationship to the position of the defect. The shape of the window was tunnel-like in one case. Of the 16 specimens with distal windows, the orifice of the right pulmonary artery arose from the aorta in seven specimens and was overriding the plane of the window in one specimen. The window occurred as an isolated lesion in four specimens. It was associated with interruption of the aortic arch at the isthmus in eight specimens and between the left carotid and left subclavian arteries in three specimens. A further three specimens had isthmal stenosis. Among the other associated defects were complete transposition (two specimens), tetralogy of Fallot (one specimen), and double outlet right ventricle (one specimen). A ventricular septal defect was present in six of the 25 specimens. The associated lesions found with aortopulmonary window are seldom encountered with common arterial trunk, suggesting these two arterial lesions have different pathogenesis and are not variants of the same entity.

Diagnosis, Treatment and Outcomes of Patients with Aortopulmonary Window

“…Mori et al (9) identified three anatomical types of APW, namely proximal, distal and total. Ho et al (10) modified this classification system by adding the intermediate type. These classification systems have improved the selection of cases eligible for transcatheter repair with sufficient superior and inferior rims; we also utilised this classification system in our cases.…”

Anesthesia for Cardiac Surgery in Neonates

“…From an anatomic and physiologic standpoint, this defect resembles truncus arteriosus; however, unlike truncus arteriosus, two distinct semilunar valves are present. The size and location of the communication varies, and thus the defect has been classifi ed into various types [ 90 ]. The lesion can occur in isolation but in most cases is associated with other cardiovascular malformations (PDA, intracardiac communications, TOF, double outlet right ventricle, IAA).…”

Current treatment of aortopulmonary window

“…Mori et al [8] originally classifi ed APW into three subtypes based on proximal defects, distal defects, and total defects. To make this classifi cation more useful to interventional cardiologists, Ho et al [10] modifi ed it by adding a descriptor of superior and inferior rim tissue presence. Richardson et al [9] included an additional group of anomalous right pulmonary artery origin from the ascending aorta; however, based on standard nomenclature, this additional grouping is better defi ned as ascending aortic origin of one pulmonary artery and right ventricular origin of the other pulmonary artery.…”

“…Based on review and discussion of the several existing classifi cation schemes, the STS committee settled on fi ve APW subtypes: APW and interrupted aortic arch APW, type 1 proximal defect APW, type 2 distal defect APW, type 3 total defect APW, intermediate type The fi rst subgroup was created because of the oftenassociated condition of interrupted arch, which occurs with APW. This is the most common lesion occurring with APW; one study found it in 8 of 25 autopsy specimens [10]. In type 1 APW, or proximal defect, lesions are located directly above and next to the sinus of Valsalva, within a few millimeters of the semilunar valves.…”