永存动脉干的预后临床状况及生存质量研究
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摘要
目的:
1.分析10年来住院病人中永存动脉干(PTA)临床特征的变化,评价PTA的疗效及其预后。
2.对PTA患者进行生存质量分析。以SF-36量表为测量工具,从PF、RP、BP、GH、VT、SF、RE和MH八个维度评估PTA患者的QOL。
方法:
在广东省人民医院病案中收集1999年1月-2008年12月住院治疗的PTA患者。1999年1月-2003年12月收治的为第一组;2004年1月-2008年12月收治的为第二组。分别收集其性别、年龄、PTA类型、治疗方法、临床表现、辅助检查结果等,进行统计分析。对PTA患者进行随访,将其UCG及生长发育指标结果与正常同龄人群进行对比。并以住院治疗年龄大于14岁有阅读能力的非心脏病患者20例为对照组,以调查问卷的形式填写SF-36量表,进行数据的搜集、整理和统计分析。
结果:
1999年1月-2003年12月在广东省人民医院住院的PTA患者共65例,男女之比为3.06:1,其平均住院年龄中位数为838天(3-15286天);PTA病例数在第一、二个五年周期分别为30例及35例,第二个五年周期增加了5例(16.67%)。其中行手术治疗的PTA患者27例,术后存活19例,存活率70.37%;死亡8例,病死率为29.63%。PTA矫治术后MPA、AV、RV、LVDd、LVDs、C/T较前减小,LVEF、LVFS较前增加,其差别有统计学意义(P<0.05)。手术前后发生三尖瓣返流的例数为6例及9例,术后有10例发生RBBB。PTA幸存者的10年主要生长发育指标随访结果:平均身高155.69±13.53cm,体重45.18±12.57kg,胸围69.50±10.00cm,与正常城镇儿童体格生长发育标准相比其身高、体重、胸围等生长发育指标稍差,但其差别无统计学意义(P>0.05)。
PTA患者的生存质量在PF、RP、BP、GH、VT、SF、RE、MH八个维度的得分分别为:83.00±6.75,74.40±9.28,15.00±13.45,45.00±17.16,69.50±11.17,86.25±14.96,40.00±40.98和50.00±20.41。对照组八个维度的得分分别为:93.25±10.17,75.75±32.44,17.15±24.28,55.75±11.73,65.25±9.25,74.37±15.95,71.66±42.26,66.80±9.00。PTA患者在PF(t=-2.87,P=0.008)、RP(t=-2.22,P=0.035)两个维度总分小于对照组,MH(t=-2.16,P=0.040)维度总分高于对照组,差别有统计学意义;而BP、GH、VT、SF、RE维度与对照组的差别无统计学意义(P>0.05)。
结论:
1. PTA的住院患者呈现增多的趋势,男性比例高于女性,与国内报道相似,高于国外报道。
2.手术是治疗PTA的唯一有效方法,手术治疗后患儿左心室前、后负荷、左室射血功能改善。
3.未行手术治疗的患者中未合并其他复杂先天性心脏畸形,肺循环有支气管动脉供应或主动脉侧支供应的PTA患者预后较好。
4. SF-36量表是一个普适性的生存质量量表,有较好的信度、效度。PTA患者在PF、RP、BP、GH、RE方面的总分低于美国及中国人常模,其差别有统计学意义(P<0.05);在VT、SF、MH得分方面与美国及中国人常模的差别无统计学意义(P>0.05)。
Objective
1. To analyze the clinical characteristic of persistent truncus arteriosus (PTA) in the hospital patients during the past 10 years, evaluate the therapeutic efficacy and prognosis of PTAs.
2. To evaluate the quality of life of PTAs from eight dimensions of PF, RP, BP, GH, VT, SF, RE and MH by using SF-36 scales as measuring tools.
Methods
Hospitalized PTAs from January, 1999 to December, 2008 were collected from Guangdong General Hospital medical records. The cases that were received and therapied in hospital from January, 1999 to December 2003 were in the first group and in January 2004 to December 2008 were in the second group. Data of gender, age, PTA types, treatment methods, clinical manifestations and auxiliary examination, etc were collected. Statistical analyses were done for the above variables. PTAs were followed-up. UCG and the growth and development outcomes were compared with the normal peer group. 20 cases of in-patients with other etiological factor who were non- heart disease and older than 14 years and had reading ability were chose as the control group. Questionnaire of SF-36 scale were asked and filled. Then data collection, collation and statistical analysis were done.
Results
There were 65 cases of PTAs hospitalized in Guangdong General Hospital from January, 1999 to December, 2008; male to female ratio was 3.06:1. The median age of hospitalization was 838 days(3-15286days),The cases of PTAs in the first and second five-year cycle were 30 and 35. The cases in the second five-year cycle were increased for 5(16.67%). 27 cases of patients were received the surgical repair and 19 cases were survived. The survival rate was 70.37%. 8 cases were deceased.The mortality was 29.63%.The MPA, AV, RV, LVDd, LVDs and C / T were reduced after surgical repair. LVEF, LVFS were increased after repair. The difference was statistically significant (P <0.05). The cases that tricuspid valve regurgitation occurred before and after repair were 6 and 9. RBBB occurred in 10 cases after repair. 10 years follow-up of the main indicators of growth and development on PTA survivors were: mean height 155.69±13.53cm, mean body weight 45.18±12.57kg,mean chest circumference 69.50±10.00cm.The index of growth and development such as height , body weight , chest circumference ,etc in PTAs were lower than normal urban children, but there was no statistically significant.
The scores of the eight dimensions of PF, RP, BP, GH, VT, SF, RE, MH in PTAs were as follows: 83.00±6.75,74.40±9.28,15.00±13.45,45.00±17.16,69.50±11.17,86.25±14.96,40.00±40.98 and 50.00±20.41,and in non-heart disease patients were: 93.25±10.17,75.75±32.44,17.15±24.28,55.75±11.73,65.25±9.25,74.37±15.95,71.66±42.26,66.80±9.00.In PTAs, PF(t=-2.87,P=0.008) and RP(t=-2.22,P=0.035) were lower, but MH (t=-2.16,P=0.040) were higher than the control group. The differences were statistically significant. There were not statistically significant in the dimensions of BP, GH, VT, SF, RE compared with the control group (P>0.05).
Conclusions
1. The number of hospitalized patients with PTA was increasing. The proportion of males was higher than females, which was similar to the reports from domestic, but higher than the reports from abroad.
2. The only effective method of therapy for PTA was surgical treatment. After the surgical repair, the preload, after load and ejection function of left ventricular were improved.
3. The followings had the better prognosis among patients without surgical treatment: with no combination of other complex congenital heart malformations, whose pulmonary circulation was supplied by bronchial artery and PTA had aortic collateral.
4. SF-36 scale is a universal quality of life scale, which has good reliability and validity. The total score of PF, RP, BP, GH and RE in patients with PTA were lower than the norm of Americans and Chinese. The differences were statistically significant (P<0.05). In the dimensions of VT, SF, MH there were no statistically difference between patients with PTA and the norm of Americans and Chinese (P>0.05).
1.分析10年来住院病人中永存动脉干(PTA)临床特征的变化,评价PTA的疗效及其预后。
2.对PTA患者进行生存质量分析。以SF-36量表为测量工具,从PF、RP、BP、GH、VT、SF、RE和MH八个维度评估PTA患者的QOL。
方法:
在广东省人民医院病案中收集1999年1月-2008年12月住院治疗的PTA患者。1999年1月-2003年12月收治的为第一组;2004年1月-2008年12月收治的为第二组。分别收集其性别、年龄、PTA类型、治疗方法、临床表现、辅助检查结果等,进行统计分析。对PTA患者进行随访,将其UCG及生长发育指标结果与正常同龄人群进行对比。并以住院治疗年龄大于14岁有阅读能力的非心脏病患者20例为对照组,以调查问卷的形式填写SF-36量表,进行数据的搜集、整理和统计分析。
结果:
1999年1月-2003年12月在广东省人民医院住院的PTA患者共65例,男女之比为3.06:1,其平均住院年龄中位数为838天(3-15286天);PTA病例数在第一、二个五年周期分别为30例及35例,第二个五年周期增加了5例(16.67%)。其中行手术治疗的PTA患者27例,术后存活19例,存活率70.37%;死亡8例,病死率为29.63%。PTA矫治术后MPA、AV、RV、LVDd、LVDs、C/T较前减小,LVEF、LVFS较前增加,其差别有统计学意义(P<0.05)。手术前后发生三尖瓣返流的例数为6例及9例,术后有10例发生RBBB。PTA幸存者的10年主要生长发育指标随访结果:平均身高155.69±13.53cm,体重45.18±12.57kg,胸围69.50±10.00cm,与正常城镇儿童体格生长发育标准相比其身高、体重、胸围等生长发育指标稍差,但其差别无统计学意义(P>0.05)。
PTA患者的生存质量在PF、RP、BP、GH、VT、SF、RE、MH八个维度的得分分别为:83.00±6.75,74.40±9.28,15.00±13.45,45.00±17.16,69.50±11.17,86.25±14.96,40.00±40.98和50.00±20.41。对照组八个维度的得分分别为:93.25±10.17,75.75±32.44,17.15±24.28,55.75±11.73,65.25±9.25,74.37±15.95,71.66±42.26,66.80±9.00。PTA患者在PF(t=-2.87,P=0.008)、RP(t=-2.22,P=0.035)两个维度总分小于对照组,MH(t=-2.16,P=0.040)维度总分高于对照组,差别有统计学意义;而BP、GH、VT、SF、RE维度与对照组的差别无统计学意义(P>0.05)。
结论:
1. PTA的住院患者呈现增多的趋势,男性比例高于女性,与国内报道相似,高于国外报道。
2.手术是治疗PTA的唯一有效方法,手术治疗后患儿左心室前、后负荷、左室射血功能改善。
3.未行手术治疗的患者中未合并其他复杂先天性心脏畸形,肺循环有支气管动脉供应或主动脉侧支供应的PTA患者预后较好。
4. SF-36量表是一个普适性的生存质量量表,有较好的信度、效度。PTA患者在PF、RP、BP、GH、RE方面的总分低于美国及中国人常模,其差别有统计学意义(P<0.05);在VT、SF、MH得分方面与美国及中国人常模的差别无统计学意义(P>0.05)。
Objective
1. To analyze the clinical characteristic of persistent truncus arteriosus (PTA) in the hospital patients during the past 10 years, evaluate the therapeutic efficacy and prognosis of PTAs.
2. To evaluate the quality of life of PTAs from eight dimensions of PF, RP, BP, GH, VT, SF, RE and MH by using SF-36 scales as measuring tools.
Methods
Hospitalized PTAs from January, 1999 to December, 2008 were collected from Guangdong General Hospital medical records. The cases that were received and therapied in hospital from January, 1999 to December 2003 were in the first group and in January 2004 to December 2008 were in the second group. Data of gender, age, PTA types, treatment methods, clinical manifestations and auxiliary examination, etc were collected. Statistical analyses were done for the above variables. PTAs were followed-up. UCG and the growth and development outcomes were compared with the normal peer group. 20 cases of in-patients with other etiological factor who were non- heart disease and older than 14 years and had reading ability were chose as the control group. Questionnaire of SF-36 scale were asked and filled. Then data collection, collation and statistical analysis were done.
Results
There were 65 cases of PTAs hospitalized in Guangdong General Hospital from January, 1999 to December, 2008; male to female ratio was 3.06:1. The median age of hospitalization was 838 days(3-15286days),The cases of PTAs in the first and second five-year cycle were 30 and 35. The cases in the second five-year cycle were increased for 5(16.67%). 27 cases of patients were received the surgical repair and 19 cases were survived. The survival rate was 70.37%. 8 cases were deceased.The mortality was 29.63%.The MPA, AV, RV, LVDd, LVDs and C / T were reduced after surgical repair. LVEF, LVFS were increased after repair. The difference was statistically significant (P <0.05). The cases that tricuspid valve regurgitation occurred before and after repair were 6 and 9. RBBB occurred in 10 cases after repair. 10 years follow-up of the main indicators of growth and development on PTA survivors were: mean height 155.69±13.53cm, mean body weight 45.18±12.57kg,mean chest circumference 69.50±10.00cm.The index of growth and development such as height , body weight , chest circumference ,etc in PTAs were lower than normal urban children, but there was no statistically significant.
The scores of the eight dimensions of PF, RP, BP, GH, VT, SF, RE, MH in PTAs were as follows: 83.00±6.75,74.40±9.28,15.00±13.45,45.00±17.16,69.50±11.17,86.25±14.96,40.00±40.98 and 50.00±20.41,and in non-heart disease patients were: 93.25±10.17,75.75±32.44,17.15±24.28,55.75±11.73,65.25±9.25,74.37±15.95,71.66±42.26,66.80±9.00.In PTAs, PF(t=-2.87,P=0.008) and RP(t=-2.22,P=0.035) were lower, but MH (t=-2.16,P=0.040) were higher than the control group. The differences were statistically significant. There were not statistically significant in the dimensions of BP, GH, VT, SF, RE compared with the control group (P>0.05).
Conclusions
1. The number of hospitalized patients with PTA was increasing. The proportion of males was higher than females, which was similar to the reports from domestic, but higher than the reports from abroad.
2. The only effective method of therapy for PTA was surgical treatment. After the surgical repair, the preload, after load and ejection function of left ventricular were improved.
3. The followings had the better prognosis among patients without surgical treatment: with no combination of other complex congenital heart malformations, whose pulmonary circulation was supplied by bronchial artery and PTA had aortic collateral.
4. SF-36 scale is a universal quality of life scale, which has good reliability and validity. The total score of PF, RP, BP, GH and RE in patients with PTA were lower than the norm of Americans and Chinese. The differences were statistically significant (P<0.05). In the dimensions of VT, SF, MH there were no statistically difference between patients with PTA and the norm of Americans and Chinese (P>0.05).
引文
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[15].沈蕾,徐月娟,赵鹏军等,单纯性圆锥动脉干畸形患者染色体22q11.2微缺失的研究.中国当代儿科杂志,2009,11(01):25-28.
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[21].李玉伟,张富强,李云辉等,永存动脉干影像学诊断(附11例报告),放射学实践2009,24(9):1002-1006.
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[1]. Douglas P.Zipes,Peter Libby,Robert O.Bonow,Eugene Braunwald,braunwald’s heart disease,a textbook of cardiovascular medicine,主译,陈灏珠,第7版.北京:人民卫生出版社,2007.1415-1416.
[2].金汉珍,黄德珉,官希吉,实用新生儿学,第3版.北京:人民卫生出版社,2002.586.
[3]. Sun LC, Wang JK, Lin MT, and Wu ET, Persistent truncus arteriosus: twenty years experience in a tertiary care center in Taiwan. Acta Paediatr Taiwan.2005,46(1):6-10.
[4]. Pearl JM, Lak s H, Drinkwater Dc, et al. Repair of truncus arteriosus in infancy. Ann Thorac Surg,1990,52:780.
[5].荣独山主编,X线诊断学,第2版.上海:上海科学技术出版社,1993:373.
[6]. Gong LG, Qiu GR, Jiang H, et al.Analysis of single nucleotide polymorphisms and haplotypes in HOXO gene Cluster within susceptible region 12q13 of simple congenital heart disease.Chin J Med Genet,2005,22(50):297-501.
[7]. Pexieder T.The conotruncus and its septation at the advent of the molecular biology era.In Clark EB,Markwald RR,Takao A(eds).Developmental Mechanisms of Heart Disease.Armonk NY,Futura Publishing Co,1995.227-248.
[8]. Kazuki K,Tsutomu N,Michiko F,et al, GATA6 mutations cause human cardiac outflow tract defects by disrupting semaphorin-plexin signaling. Proc Natl Acad Sci U S A, 2009 106(33):13933-8.
[9]. Feiner L, Webber AL, Brown CB, et al. Targeted disruption of semaphorin 3C leads to persistent truncus arteriosus and aortic arch interruption. Development.2001,128:3061–3070.
[10]. Phillips MT, Kirby ML, Forbes ML, Analysis of cranial neural crest distribution in the developing heart using quail-chick chimeras. Circ Res,1987,60:27–30.
[11]. Brown CB, et al.PlexinA2 and semaphorin signaling during cardiac neural crest development. 2001,128:3071–3080.
[12]. Raid R, Krinka D, Bakhoff L,et al, Lack of Gata3 results in conotruncal heart anomalies in mouse. Mech Dev, 2009, 126(1-2):80-9.
[13]. Khositseth A, Tocharoentanaphol C, Khowsathit P, et al, Chromosome 22q11 deletions in patients with conotruncal heart defects. Pediatr Cardiol, 2005, 26(5):570-3.
[14]. Momma K, Ando M, Matsuoka R. Truncus arteriosus communis associated with chromosome 22q11 deletion. J Am Coll Cardiol.1997,30(4):1067-71.
[15].沈蕾,徐月娟,赵鹏军等,单纯性圆锥动脉干畸形患者染色体22q11.2微缺失的研究.中国当代儿科杂志,2009,11(01):25-28.
[16]. Eferl R, Sibilia M, Hilberg F, et al, Functions of c-Jun in liver and heart development. J Cell Biol.1999, 145(5):1049-61.
[17]. Collett RW,Edwards JE.Persistent truncus arteriosus.a classification according to anatomic types ,Surg Clin North Am.1949,29:1245—1270.
[18]. Van Praagh R,Van Praagh S.The anatomy of persistent arteriosus trunk (truncus arteriosus communis and its embryological implications).a study of 57 necropsy.Am J Cardio1,1965,16:406—425.
[19]. Victorica BE, Gessner IH, Van Mierop LH, and Schiebler GL. Persistent Truncus APersistent truncus arteriosus. An electrovectorcardiographic study in 14 infantsrteriosus. Dis Chest.1968, 54(2):100-4.
[20].吕亚萍,唐光才,陈洪亮等,永存动脉干的影像学表现二例,临床放射学杂志,2008,27(11):1608-1609.
[21].李玉伟,张富强,李云辉等,永存动脉干影像学诊断(附11例报告),放射学实践2009,24(9):1002-1006.
[22].谢明星,李治安,王新房等,超声心动图在永存动脉干影像诊断中的价值.中华超声影像学杂志,2001,10(4):206-209.
[23].郭艳霞,梁耀园,马小燕,动态三维胎儿心动图诊断永存动脉干的价值,广东医学,2008,29(11):1850-1852.
[24].周启昌,彭清海,章鸣等,超声诊断胎儿永存动脉干,中华超声影像学杂志,2004 ,13(10):759-762.
[25]. Duke C, Sharland GK, Jones AMR, et al. Echocardiographic features and outcome of truncual arteriosus diagnosed during fetal life. Am J Cardiol, 2001, 88:1379-1384.
[26]. Felix G, Alex F, Christoph B, Cardiovascular flashlight. Persistent truncus arteriosus: a rare finding in adults. Eur Heart J.2009,30(9):1154.
[27].黄廉溪,翟鸿元,朱铭等,小儿先天性心脏病成角投照电影心血管造影术,临床儿科杂志,1986,4:94.
[28].朱铭,杨贞勇,黄廉溪,永存动脉干病理分型与造影诊断,中华放射学杂志,1996,30(1):20-22.
[29]. ZHANG Yu-qi, SHEN R, SUN K,et al,Persistent truncus arteriosus with intact ventricular septum diagnosed by echocardiography. Chinese Medical Journal, 2009, 122 (22): 2798-2800.
[30].宋锦文,杨润润,宁季军等,永存动脉干长期误诊为法乐氏四联症2例报告并文献复习.中国误诊学杂志,2004,4(10):1575-1578.
[31]. Brown JW ,Ruzmetov M,Okada Y,et a1.Truncus arteriosus repair:outcomes,risk factors, reoperation and management.Eur J Cardiothorac Surg,2001,20(2):221—227.
[32]. Bove EL, Lupinetti FM, Pridjian AK, et al. Results of a policy of primary repair for truncus arteriosus in the neonate. J Thorac Cardiovasc Surg 1993; 105: 1057-1066.
[33]. Steffes CP,Bender js,Levison MA.Blood transfusion and oxygen consumption in,sepsis.Crit Care Med.1991,19(5):5l2—517.
[34]. Byrayneki PL ,Mckay R ,Amold R ,et al. Homograft replacement of the pulmonary artery bifurcation.Thorac Cardiovasc Surg,1989,99:623.
[35].冯爱强,郅兴义,乔晨晖,现代小儿胸心外科学,郑州:河南医科大学出版社,1998.479-488.
[36]. Thompson LD, McElhinney DB, Reddy M, et al, Neonatal repair of truncus arteriosus: Continuing improvement in outcomes.Ann Thorac Surg, 2001, 72:391-395.
[37].沈佳,徐志伟,永存动脉千纠治术中右心室流出道重建方式的选择.中国胸心血管外科临床杂志,2008,15(2):81-86.
[38]. Rajasinghe HA, McElhinney DB, Reddy VM,et al, Long-term follow-up of truncus arteriosus repaired in infancy: a twenty-year experience. J Thorac Cardiovasc Surg.1997, 113(5):869-78.
[39]. Kalavrouziotis G, Purohit M, Ciotti G, Truncus arteriosus communis: early and midterm results of early primary repair. Ann Thorac Surg. 2006,82 (6):2200-6.
[40]. Espínola-Zavaleta N, Mu?oz-Castellanos L, González-Flores R, Kuri-Nivón M, Common truncus arteriosus in adults. Arch Cardiol Mex. 2008, 78(2):210-6.