Wernovsky G, Stiles K M, Gauvreau K, Gentles T L, duPlessis A J, Bellinger D C, Walsh A Z, Burnett J, Jonas R A, Mayer J E, Newburger J W
Department of Cardiology, Children's Hospital, Harvard Medical School, Boston, MA, USA.
Circulation. 2000 Aug 22;102(8):883-9. doi: 10.1161/01.cir.102.8.883.
Patients with a single ventricle have multiple risk factors for central nervous system injury, both before and after the Fontan procedure.
A geographically selected cohort was invited to undergo standardized testing, including age-appropriate measures of intelligence quotient (IQ) and achievement tests. Historical information was obtained by chart review and patient questionnaires. Of the 222 eligible patients, 133 (59.9%) participated. Median age at testing was 11.1 years (range, 3. 7 to 41.0 years), 6.0 years (range, 1.6 to 19.6 years) after surgery. Mean full-scale IQ was 95.7+/-17.4 (P<0.006 versus normal); 10 patients (7.8%) had full-scale IQ scores <70 (P=0.001). After adjustment for socioeconomic status, lower IQ was associated with the use of circulatory arrest before the Fontan operation (P=0.002), the anatomic diagnoses of hypoplastic left heart syndrome (P<0.001) and "other complex" (P=0.05), and prior placement of a pulmonary artery band (P=0.04). Mean composite achievement score was 91.6+/-15. 4 (P<0.001 versus normal); 14 patients (10.8%) scored <70 (P<0.001). After adjustment for socioeconomic status, independent risk factors for low achievement scores included the diagnoses of hypoplastic left heart syndrome (P=0.004) and "other complex" (P=0.003) or prior use of circulatory arrest (P=0.03), as well as a reoperation with cardiopulmonary bypass within 30 days of the Fontan (P=0.01).
Most individual patients palliated with the Fontan procedure in the 1970s and 1980s have cognitive outcome and academic function within the normal range, but the performance of the cohort is lower than that of the general population.
单心室患者在Fontan手术前后存在多种中枢神经系统损伤的危险因素。
选取一个具有地域代表性的队列进行标准化测试，包括适合年龄的智商（IQ）测量和学业成就测试。通过查阅病历和患者问卷获取病史信息。222例符合条件的患者中，133例（59.9%）参与了研究。测试时的中位年龄为11.1岁（范围3.7至41.0岁），术后6.0岁（范围1.6至19.6岁）。平均全量表智商为95.7±17.4（与正常人群相比，P<0.006）；10例患者（7.8%）全量表智商得分<70（P=0.001）。在调整社会经济地位后，较低的智商与Fontan手术前使用循环阻断（P=0.002）、左心发育不全综合征的解剖学诊断（P<0.001）和“其他复杂情况”（P=0.05）以及先前放置肺动脉束带（P=0.04）有关。平均综合学业成就得分是91.6±15.4（与正常人群相比，P<0.001）；14例患者（10.8%）得分<70（P<0.001）。在调整社会经济地位后，学业成就得分低的独立危险因素包括左心发育不全综合征的诊断（P=0.004）和“其他复杂情况”（P=0.003）或先前使用循环阻断（P=0.03），以及Fontan手术后30天内进行的体外循环再次手术（P=0.01）。
20世纪70年代和80年代接受Fontan手术姑息治疗的大多数个体患者的认知结果和学业功能在正常范围内，但该队列的表现低于一般人群。
