Mietus-Snyder M, Lang P, Mayer J E, Jones R A, Castaneda A R, Lock J E
Circulation. 1987 Sep;76(3 Pt 2):III39-44.
We investigated whether or not a systemic arteriopulmonary shunt (SAPS) after 1 year of age affects a child's chances for a successful Fontan procedure. From 1967 to 1986, 50 patients more than 1 year old with tricuspid atresia or other complex lesions amenable to a Fontan operation received a SAPS. Follow-up was incomplete in six patients. Two patients died within 1 week of surgery, and two died within 9 months for a total mortality of 8%. Each of the 40 remaining patients was catheterized 6 months to 10.8 years (mean 4.9 years) after placement of a SAPS. By the criteria of Choussat and Fontan, there were one or more adverse SAPS-induced changes in nine patients (23%). Three developed pulmonary hypertension, one with associated elevation of pulmonary arteriolar resistance and pulmonary artery distortion, and one in conjunction with severe congestive heart failure. Six additional patients developed isolated distortion of the ipsilateral pulmonary artery. Of these nine patients, three had successful Fontan operations (33%), two died after the Fontan operation, and four are no longer candidates. In contrast, of 31 patients without documented SAPS-induced complications, 27 have undergone Fontan operations, 23 successfully (74%). Palliation with a SAPS after 1 year of age can place children at increased risk for a subsequent Fontan operation.
我们研究了1岁以后进行体动脉-肺动脉分流术（SAPS）是否会影响儿童成功进行Fontan手术的几率。1967年至1986年期间，50例年龄超过1岁、患有三尖瓣闭锁或其他适合Fontan手术的复杂病变的患者接受了SAPS。6例患者随访不完整。2例患者在术后1周内死亡，2例在9个月内死亡，总死亡率为8%。其余40例患者在进行SAPS后6个月至10.8年（平均4.9年）接受了心导管检查。根据Choussat和Fontan的标准，9例患者（23%）出现了一种或多种由SAPS引起的不良变化。3例出现肺动脉高压，其中1例伴有肺小动脉阻力升高和肺动脉扭曲，1例伴有严重充血性心力衰竭。另外6例患者出现同侧肺动脉孤立性扭曲。在这9例患者中，3例成功进行了Fontan手术（33%），2例在Fontan手术后死亡，4例不再适合进行该手术。相比之下，在31例无SAPS引起并发症记录的患者中，27例接受了Fontan手术，23例成功（74%）。1岁以后进行SAPS姑息治疗会使儿童后续进行Fontan手术的风险增加。
