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心室反位与三尖瓣闭锁(VITA 综合征):未经手术治疗而长期存活

Ventricular inversion and tricuspid atresia (VITA complex): long survival without surgical treatment.

作者信息

Tazelaar H D, Moore G W, Hutchins G M

出版信息

Pediatr Cardiol. 1986;6(4):187-91. doi: 10.1007/BF02310996.

DOI:10.1007/BF02310996
PMID:3703690
Abstract

Five patients with ventricular inversion, corrected transposition of the great arteries, and atresia of the left-sided atrioventricular (tricuspid) valve have been observed. Three patients died in infancy, two had large interatrial communications, one with an early stage of arterial-type hypertensive pulmonary vascular disease, and the third patient had a very small interatrial communication and severe cyanosis. The other two patients survived into adulthood; both had small interatrial communications, and one had severe venous-type hypertensive pulmonary vascular disease. The longer survival of the two patients is attributed to a balance between resistance and blood flow in the pulmonary circulation influenced by the size of the interatrial communication.

摘要

已观察到5例心室反位、大动脉转位矫正型及左侧房室(三尖瓣)闭锁的患者。3例患者在婴儿期死亡,2例有较大的房间隔交通,其中1例处于动脉型高血压性肺血管疾病早期,第3例患者房间隔交通非常小且有严重紫绀。另外2例患者存活至成年;二者均有小的房间隔交通,其中1例有严重的静脉型高血压性肺血管疾病。这2例患者存活时间较长归因于房间隔交通大小对肺循环中阻力与血流之间平衡的影响。

相似文献

1
Ventricular inversion and tricuspid atresia (VITA complex): long survival without surgical treatment.心室反位与三尖瓣闭锁(VITA 综合征):未经手术治疗而长期存活
Pediatr Cardiol. 1986;6(4):187-91. doi: 10.1007/BF02310996.
2
Ventricular inversion without transposition of the great arteries: a rare anomaly associated with left-sided (tricuspid) atrioventricular valve atresia and unroofed coronary sinus.心室反位但大动脉无转位:一种与左侧(三尖瓣)房室瓣闭锁及冠状静脉窦未闭相关的罕见畸形。
Pediatr Cardiol. 1994 May-Jun;15(3):146-50. doi: 10.1007/BF00796328.
3
The pulmonary outflow tract in classically corrected transposition.
J Thorac Cardiovasc Surg. 1975 May;69(5):747-57.
4
Aortic atresia and tricuspid atresia occurring in complete transposition of the great vessels.大动脉完全转位合并主动脉闭锁及三尖瓣闭锁。
Pediatr Cardiol. 1993 Mar;14(2):133-4. doi: 10.1007/BF00796997.
5
Positional abnormalities of atrioventricular valves in transposition of the great arteries including double outlet right ventricle, atrioventricular valve straddling and malattachment.大动脉转位(包括右心室双出口)中房室瓣的位置异常,如房室瓣骑跨和附着异常。
Am J Cardiol. 1979 Nov;44(6):1135-45. doi: 10.1016/0002-9149(79)90179-6.
6
Two cases of left-sided tricuspid atresia with situs solitus L-bulboventricular loop and L-transposition of the great arteries.两例左位三尖瓣闭锁合并心脏位置正常、L型球室袢及大动脉L型转位。
Tohoku J Exp Med. 1976 Aug;119(4):385-92. doi: 10.1620/tjem.119.385.
7
Natural history of the ventricular septal defect in tricuspid atresia and its surgical implications.三尖瓣闭锁合并室间隔缺损的自然病史及其外科治疗意义
Br Heart J. 1977 Mar;39(3):276-88. doi: 10.1136/hrt.39.3.276.
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Anatomy of tricuspid atresia and its relevance to current forms of surgical therapy.三尖瓣闭锁的解剖结构及其与当前手术治疗方式的相关性。
Ann Thorac Surg. 1980 Apr;29(4):306-11. doi: 10.1016/s0003-4975(10)61476-2.
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Subpulmonic obstruction in complete (d) transposition produced by redundant tricuspid tissue.由多余的三尖瓣组织导致的完全性(d型)大动脉转位中的肺下梗阻。
Circulation. 1969 May;39(5):603-9. doi: 10.1161/01.cir.39.5.603.
10
Anatomically corrected malposition of the great arteries. Report of 2 cases, one with congenital asplenia; frequent association with juxtaposition of atrial appendages.大动脉解剖学矫正不良。2例报告,1例合并先天性无脾;常合并心耳并列。
Br Heart J. 1974 Feb;36(2):207-15. doi: 10.1136/hrt.36.2.207.

本文引用的文献

1
Congenital corrected transposition of the great vessels: a study of 33 cases.先天性矫正型大动脉转位:33例病例研究。
Pediatrics. 1961 May;27(5)Suppl:849-88.
2
Corrected transposition of the great vessels of the heart: a review of 17 cases.矫正型大动脉转位:17例病例回顾
Pediatrics. 1957 Oct;20(4):626-46.
3
The cardiac malformations. Double inlet left ventricle and corrected transposition explained as deviations in the normal development of the interventricular septum.心脏畸形。双入口左心室和矫正性大动脉转位被解释为室间隔正常发育中的偏差。
Hum Pathol. 1981 Mar;12(3):242-50. doi: 10.1016/s0046-8177(81)80125-6.
4
Clinical profile of patients with congenital corrected transposition of the great arteries. A study of 60 cases.大动脉矫正型转位患者的临床特征。60例研究。
N Engl J Med. 1970 May 7;282(19):1053-9. doi: 10.1056/NEJM197005072821901.
5
Congenitally corrected transposition of the great arteries: morphologic study of 32 cases.先天性矫正型大动脉转位:32例形态学研究
Am J Cardiol. 1976 Dec;38(7):910-23. doi: 10.1016/0002-9149(76)90804-3.
6
The pathogenesis of the two forms of hypertensive pulmonary vascular disease.两种形式的高血压性肺血管疾病的发病机制。
Am Heart J. 1976 Dec;92(6):797-803. doi: 10.1016/s0002-8703(76)80019-1.