Clapp S, Perry B L, Farooki Z Q, Jackson W L, Karpawich P P, Hakimi M, Arciniegas E, Green E W, Pinsky W W
Department of Pediatrics, Children's Hospital of Michigan, Detroit 48201.
J Thorac Cardiovasc Surg. 1990 Jul;100(1):115-21.
We reviewed our experience over a 10-year period to determine whether children with Down's syndrome and complete atrioventricular canal develop pulmonary vascular obstructive disease earlier than children with normal chromosomes and this defect. Comparisons were made between Down's syndrome and normal chromosome children regarding (1) pulmonary blood flow and pulmonary vascular resistance at initial catheterization, (2) operability as related to elevation in pulmonary vascular resistance, and (3) age at diagnosis of fixed pulmonary vascular obstructive disease. The 45 patients with Down's syndrome catheterized under 1 year of age had a lower mean pulmonary blood flow (3.2 versus 5.7; p = 0.0001) and higher mean pulmonary vascular resistance (8.3 versus 4.6 Wood units.m2; p = 0.0003) than their 34 normal chromosome counterparts. When all ages were included, 38 of 81 (47%) of the children with Down's syndrome and 32 of 40 (80%) of the normal children were considered operable. Non-Down's syndrome patients who had operations had a higher pulmonary blood flow (5.8 versus 3.3; p = 0.004) and lower pulmonary vascular resistance (3.6 versus 6.0 Wood units.m2; p = 0.005) than Down's syndrome patients. Of the 34 patients who did not have operations because of pulmonary vascular obstructive disease, 31 had Down's syndrome. In 10 of 81 children with Down's syndrome, fixed pulmonary vascular obstructive disease was diagnosed before the age of 1 year, while this was found in none of 40 normal children. Our data demonstrate that Down's syndrome patients with complete atrioventricular canal have a greater degree of elevation of pulmonary vascular resistance in the first year of life and more rapid progression to fixed pulmonary vascular obstructive disease than children with normal chromosomes.
我们回顾了10年间的经验，以确定患有唐氏综合征且伴有完全性房室通道的儿童是否比染色体正常但有此缺陷的儿童更早发生肺血管阻塞性疾病。对唐氏综合征患儿和染色体正常的患儿进行了以下比较：（1）初次心导管检查时的肺血流量和肺血管阻力；（2）与肺血管阻力升高相关的可手术性；（3）固定性肺血管阻塞性疾病的诊断年龄。45例1岁以下接受心导管检查的唐氏综合征患儿，其平均肺血流量（3.2对5.7；p = 0.0001）低于34例染色体正常的对应患儿，平均肺血管阻力（8.3对4.6伍德单位·m²；p = 0.0003）高于后者。纳入所有年龄组后，81例唐氏综合征患儿中有38例（47%）、40例正常儿童中有32例（80%）被认为可进行手术。接受手术的非唐氏综合征患者的肺血流量（5.8对3.3；p = 0.004）高于唐氏综合征患者，肺血管阻力（3.6对6.0伍德单位·m²；p = 0.005）低于唐氏综合征患者。在34例因肺血管阻塞性疾病未进行手术的患者中，31例患有唐氏综合征。81例唐氏综合征患儿中有10例在1岁前被诊断为固定性肺血管阻塞性疾病，而40例正常儿童中无一例出现这种情况。我们的数据表明，患有完全性房室通道的唐氏综合征患者在生命的第一年肺血管阻力升高程度更大，且比染色体正常的儿童更快发展为固定性肺血管阻塞性疾病。
