Marcelletti C, Di Donato R, Nijveld A, Squitieri C, Bulterijs A H, Naeff M, Schuller J, Becker A E
Ann Thorac Surg. 1983 Apr;35(4):400-5. doi: 10.1016/s0003-4975(10)61592-5.
We have treated by palliative or corrective surgery 12 patients with the complex cardiovascular anomalies associated with right isomerism (asplenia syndrome) or left isomerism (polysplenia syndrome). A systemic-pulmonary artery shunt was performed in 4 patients, with 2 early deaths. Anatomical intracardiac repair was successfully done in 2 patients, while an orthoterminal repair using a modified Fontan procedure was attempted in 5 patients, only 1 of whom survived. A 1-year-old girl with diffuse pulmonary arteriovenous fistulas associated with left isomerism was not considered a good candidate for operation after an exploratory sternotomy. The surgical options and technical problems related to the complex intracardiac morphology of these developmental syndromes are presented.
我们已对12例患有与右位异构（无脾综合征）或左位异构（多脾综合征）相关的复杂心血管异常的患者进行了姑息性或矫正性手术。4例患者进行了体肺分流术，其中2例早期死亡。2例患者成功进行了解剖性心内修复，5例患者尝试采用改良Fontan手术进行正位终末修复，其中仅1例存活。一名患有与左位异构相关的弥漫性肺动静脉瘘的1岁女孩，在进行探索性胸骨切开术后被认为不适合手术。本文介绍了与这些发育综合征复杂的心内形态相关的手术选择和技术问题。
