A 15-year-old boy was born with cleft lip and palate and congenital aortic valve lesions. The symptoms of chest tightness and dizziness can be relieved after rest. A physical examination revealed slight cyanosis and grade 2/6 systolic murmur in the aortic valve auscultation area. The first echocardiography only found the aortic valve as a four-lobe deformity (PanelA) with moderate to severe regurgitation, but the severe systolic pulmonary hypertension (92 mmHg) was unexplained, there was no evidence of other congenital heart disease at parasternal short-axis view. Surgeons suspected a right-to-left shunt because the contrast agent entered the aorta when the right heart catheterization was used to measure the pulmonary artery pressure (PanelB; see Supplementary material online, Video S1). The second echocardiography confirmed there was no patent ductus arteriosus. However, a 3.0 cm wide communication opening with colour Doppler flow was revealed between the ascending aorta and the main pulmonary artery on the suprasternal notch section (PanelsC and D; see Supplementary material online, Video S2). Contrast-enhanced echocardiography showed microbubbles passing between the ascending aorta and pulmonary artery (PanelE; see Supplementary material online, Video S3). The computed tomography angiography and 3D reconstruction also confirmed a wide connection (2.1 cm × 2.7 cm) between them (PanelsF and G). The patient underwent repairment of the aortopulmonary septal defect (PanelH) and the replacement of quadricuspid aortic valve (PanelI) with mechanical prosthesis. He recovered well 2 weeks after operation and had significant decrease of the systolic pulmonary artery pressure (47 mmHg). Aortopulmonary window is an overlooked and very rare malformation as one cause of the pulmonary hypertension.

中文翻译：

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无法解释的肺动脉高压：一个被忽略的肺门窗。

一个15岁的男孩出生时唇c裂和先天性主动脉瓣膜病变。休息后胸闷头晕的症状可以缓解。体格检查发现主动脉瓣听诊区出现轻微发和2/6级收缩期杂音。第一次超声心动图检查仅发现主动脉瓣为四瓣畸形（PanelA），伴有中度至重度反流，但无法解释严重的收缩期肺动脉高压（92 mmHg），在胸骨旁短轴上没有其他先天性心脏病的证据视图。外科医生怀疑是从右向左分流，因为当使用右心导管术测量肺动脉压力时，造影剂进入了主动脉（PanelB；请参见在线补充材料，视频S1）。第二次超声心动图检查证实没有动脉导管未闭。然而，在胸骨上切迹部分（主图C和D；请参见在线补充材料，视频S2）发现升主动脉和主肺动脉之间有一个3.0厘米宽的彩色多普勒血流连通孔。造影增强超声心动图显示升主动脉和肺动脉之间有微气泡（PanelE；请参见在线补充材料，视频S3）。计算机断层扫描血管造影和3D重建也证实了它们之间（面板F和G）的宽连接（2.1 cm×2.7 cm）。该患者接受了正肺间隔缺损（PanelH）的修复，并用机械假体代替了四尖瓣主动脉瓣（PanelI）。术后2周他恢复良好，并且收缩期肺动脉压明显降低（47 mmHg）。主肺窗是导致肺动脉高压的一种被忽视且非常罕见的畸形。