在临床上的分类为：①伴主动脉下室间隔缺损，主动脉瓣位于肺动脉右侧及右室流出道狭窄（法四型右室双出口）；②伴肺动脉下室间隔缺损，主动脉瓣位于肺动脉右侧；③伴主动脉下室间隔缺损，主动脉瓣位于肺动脉右侧，不伴有右心室流出道狭窄的右室双出口较常见。
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