Distal type of aortopulmonary window. Report of 4 cases.

Abstract

SUMMARY We have studied 14 patients with aortopulmonary window (10 male and 4 female, age range 1 month to 41 years). Four ofthese had a distal defect with characteristic haemodynamic and angiographic features. Aortopulmonary window may be classified into 3 types: type I (proximal) defects occur in the proximal part of aortopulmonary septum; type II (distal) defects occur in the distal part of the aortopulmonary septum adjacent to the right pulmonary artery; the type III defect is a combination of types I and II.In type I, injection of contrast media into the aortic root opacifies the main pulmonary trunk and then both pulmonary arteries. In type II, the right pulmonary artery is preferentially opacified simulating the finding of right pulmonary artery arising from the ascending aorta. In one case of type II, injection into the right ventricle showed preferential flow to the left pulmonary artery, because of the large shunt of unopacified blood into the right pulmonary artery, but in both types I and II the left and right pulmonary arteries are usually opacified simultaneously after injection into the main pulmonary trunk.In type I either transaortic or transpulmonary closure is the appropriate surgical procedure. In types II and III, the transaortic approach provides better exposure and facilitates the operative repair.Aortopulmonary window is a relatively rare con-right pulmonary artery and the pulmonary trunk genital anomaly involving the great arteries. Patients (Kimoto et al., 1957;Nakaya, 1963; Wright with this anomaly in whom there is an increased et al., 1968). pulmonary flow and pulmonary hypertensionIn a 19-year period from 1958 to 1976, 14 patients usually develop symptoms in the neonatal period or with aortopulmonary window were studied at the in early infancy, and without operation have a Heart Institute of Japan (Table 1), and 4 of the 14 uniformly poor prognosis. The high mortality rate had the distal type of communication. The purpose in these babies is the result of congestive heart of this report is to describe 4 cases of the distal failure and of pulmonary complications caused by type of aortopulmonary window and to discuss an excessive left-to-right shunt through the aorto-diagnostic and surgical problems in 3 varieties of pulmonary window. Since cure is effected by sur-this defect. gical closure of the defect, precise preoperative recognition of the type and the location of the Case reports window is essential.In the majority of cases, the defect is in the left Clinical data are summarised in Table 2. lateral wall of the ascending aorta and communicates with the right lateral wall of the pulmonary CASE 1 trunk; the window is then anterior to the ostium of J.A., a 6-month old girl, was first seen at the age of the right pulmonary artery when viewed from the 30 days after an episode of unconsciousness, with lumen of the pulmonary trunk. In another type, opisthotonus. She was then an underdeveloped, however, there is a distal communication between poorly nourished infant, and had failed to thri...

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.…”

Aortopulmonary window. Clinical and surgical assessment of 18 cases

“…A type I APW occurs when the defect in the truncal septum is close to the semilunar valves; when it is more distal, a type II defect develops, while no development of the proximal and distal truncal septum leads to type III APW 3 . Development of the coronary arteries also occurs during the septation of the great vessels, which occasionally leads to an anomalous position of the coronary arteries 19 .…”

Interruption of the aortic arch with aorticopulmonary septal defect

“…Both Richardson [26] and Mori [22] and their respective associates have recognized that APSD may occur at various sites within the pulmonary arterial tree. Separate and different classification schemes have been proposed by each of these authors to categorize APSD into types.…”