METHODS: A 2 years old Arabian female child was diagnosed with ATS affecting the pulmonary arteries as a newborn, underwent a pulmonary arterial surgical reconstruction at the age of 2 years old due to the development of pulmonary artery stenosis with left pulmonary artery having a peak gradient of 73 mmHg with a peak velocity of 4.3 m/s and the right pulmonary artery having a peak gradient of 46 mmHg with a peak velocity of 3.4 m/s causing right ventricular hypertension. After surgical repair the left pulmonary artery has a peak pressure gradient of 20 mmHg, with the right pulmonary artery having a peak pressure gradient of 20 mmHg.
CONCLUSIONS: ATS is a rare genetic condition that affects the great arteries especially the pulmonary arteries causing stenotic and tortuous vessels that may be central branches or distal peripheral branches that leads to severe right ventricular dysfunction and hypertension. We believe that surgical treatment provides the optimum outcomes when compared to transcather approaches especially when the peripheral arteries are involved. Some challenges and hiccups might occur, especially lung reperfusion injury that needs to be diagnosed and treated accordingly.
方法:一名2岁的阿拉伯女性儿童在新生儿时被诊断出患有影响肺动脉的ATS,由于肺动脉狭窄的发展,在2岁时接受了肺动脉外科重建,左肺动脉的峰值梯度为73mmHg,峰值速度为4.3m/s,右肺动脉的峰值梯度为46mmHg,峰值速度为3.4m/s,导致右心室高血压。手术修复后,左肺动脉的峰值压力梯度为20mmHg,右肺动脉的峰值压力梯度为20mmHg。
结论:ATS是一种罕见的遗传病,影响大动脉,尤其是肺动脉,引起狭窄和曲折的血管,这些血管可能是中央分支或远端外周分支,导致严重的右心室功能障碍和高血压。我们认为,与经导管入路相比,尤其是在涉及外周动脉时,手术治疗可提供最佳结果。一些挑战和打嗝可能会发生,尤其是肺再灌注损伤,需要进行相应的诊断和治疗。