Saritas Turkay, Erdem Abdullah, Karaci Ali R, Demir Fadli, Celebi Ahmet
Department of Pediatric Cardiology, Dr Siyami Ersek Thoracic and Cardiovascular Surgery Center, Istanbul, Turkey.
Congenit Heart Dis. 2012 May-Jun;7(3):E1-5. doi: 10.1111/j.1747-0803.2011.00542.x. Epub 2011 Jun 27.
The origin of pulmonary artery branches (particularly the left pulmonary artery) from the ascending aorta is a rare condition. We detected prominent hypoplasia of the main and right pulmonary arteries in a 3.5-month-old 3.7 kg female infant who had tetralogy of Fallot with origin of the left pulmonary artery in the ascending aorta. In order to ensure the development of the right pulmonary artery, a stent was put in that extended from the right ventricular outflow tract to the right pulmonary artery. During follow-up, after the patient's right pulmonary artery had developed sufficiently, a complete repair surgery was done. It is common practice for patients with abnormal origin of the left pulmonary artery to perform the complete repair using the direct reimplantation technique. However, we think that another possibility is to implant the stent in patients with hypoplastic pulmonary artery and branches in the early stages, wait for a short period of time and perform the complete repair surgery before permanent pulmonary hypertension develops.
肺动脉分支（尤其是左肺动脉）起源于升主动脉是一种罕见的情况。我们在一名3.5个月大、体重3.7千克的患有法洛四联症且左肺动脉起源于升主动脉的女婴中检测到主肺动脉和右肺动脉明显发育不全。为了确保右肺动脉的发育，置入了一个从右心室流出道延伸至右肺动脉的支架。在随访期间，待患者右肺动脉充分发育后，进行了根治性修复手术。对于左肺动脉起源异常的患者，通常采用直接再植入技术进行根治性修复。然而，我们认为另一种可能性是，对于早期肺动脉及其分支发育不全的患者，先植入支架，等待一段时间，在永久性肺动脉高压形成之前进行根治性修复手术。
