同: l-transposition of the great arteries, l-大血管転位

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the 20th letter of the Roman alphabet (同)t
the 12th letter of the Roman alphabet (同)l

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tritiumの化学記号
lira(イタリアの貨幣単位リラ)
liter[s]

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/09/17 22:34:14」(JST)

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levo-Transposition of the great arteries (L-Transposition of the great arteries, levo-TGA, or l-TGA), also commonly referred to as congenitally corrected transposition of the great arteries (CC-TGA), is an acyanotic congenital heart defect (CHD) in which the primary arteries (the aorta and the pulmonary artery) are transposed, with the aorta anterior and to the left of the pulmonary artery; the morphological left and right ventricles are also transposed.

Use of the term "corrected" has been disputed by many due to the frequent occurrence of other abnormalities and or acquired disorders in l-TGA patients.

In segmental analysis, this condition is described as atrioventricular discordance (ventricular inversion) with ventriculoarterial discordance.

l-TGA is often referred to simply as transposition of the great arteries (TGA); however, TGA is a more general term which may also refer to dextro-transposition of the great arteries (d-TGA).

Another term commonly used to refer to both l-TGA and d-TGA is transposition of the great vessels (TGV), although this term can have an even broader meaning than TGA.

Overview

In a normal heart, oxygen-depleted ("deoxygenated") blood is pumped from the right atrium into the right ventricle, then through the pulmonary artery to the lungs where it is oxygenated. The oxygen-rich ("oxygenated") blood then returns, via the pulmonary veins, to the left atrium from which it is pumped into the left ventricle, then through the aorta to the rest of the body, including the heart muscle itself.

With l-TGA, deoxygenated blood is pumped from the right atrium into the morphological left ventricle (which lies on the right side of the heart), then through the pulmonary artery to the lungs. The oxygenated blood then returns, via the pulmonary veins, to the left atrium from which it is pumped into the morphological right ventricle, then through the aorta.

Variations and similar defects

Simple and complex l-TGA

l-TGA is often accompanied by other heart defects, the most common type being intracardiac shunts such as atrial septal defect (ASD) including patent foramen ovale (PFO), ventricular septal defect (VSD), and patent ductus arteriosus (PDA). Stenosis of valves or vessels may also be present.

When no other heart defects are present it is called 'simple' l-TGA; when other defects are present it is called 'complex' l-TGA.

Symptoms

Simple l-TGA does not immediately produce any visually identifiable symptoms, but since each ventricle is intended to handle different blood pressures, the right ventricle may eventually hypertrophy due to increased pressure and produce symptoms such as dyspnea or fatigue.

Complex l-TGA may produce immediate or more quickly-developed symptoms, depending on the nature, degree and number of accompanying defect(s). If a right-to-left or bidirectional shunt is present, the list of symptoms may include mild cyanosis.

Diagnosis

l-TGA can sometimes be diagnosed in utero with an ultrasound after 18 weeks gestation. However, many cases of simple l-TGA are "accidentally" diagnosed in adulthood, during diagnosis or treatment of other conditions.

Treatment and prognosis

Simple l-TGA has a very good prognosis, with many individuals being asymptomatic and not requiring surgical correction.

In a number of cases, the (technically challenging) "double switch operation" has been successfully performed to restore the normal blood flow through the ventricles.

External links

Overview at pediheart.org
Royal Children's Hospital, Melbourne
Mayo Clinic, Arizona - Florida - Minnesota, USA

UpToDate Contents

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1. 一過性全健忘 transient global amnesia
2. L型大血管転位 l transposition of the great arteries
3. D型大血管転位の病態生理、臨床症状、および診断 pathophysiology clinical manifestations and diagnosis of d transposition of the great arteries
4. D型大血管転位のマネージメントおよび転帰 management and outcome of d transposition of the great arteries
5. 新生児におけるチアノーゼの心臓の原因 cardiac causes of cyanosis in the newborn

English Journal

Cardiac axis shift within the cardiac cycle in normal fetuses and fetuses with congenital heart defect.

Zhao Y1, Horton S, Sinkovskaya E, Mlynarczyk M, Romary L, Abuhamad A.
Ultrasound in obstetrics & gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology.Ultrasound Obstet Gynecol.2014 Dec 16. doi: 10.1002/uog.14768. [Epub ahead of print]
OBJECTIVES: The aim of this study was to demonstrate the changes in the Cardiac Axis (CAx) within the cardiac cycle in normal fetuses and fetuses with Congenital Heart Defects (CHD).METHODS: Video clips of four-chamber views were reviewed from 527 prenatal ultrasound scans between 18+0 and 36+6 w
PMID 25511021

First report of atretic coronary sinus stenting in a 5-kg infant resulting in dramatic improvement of ventricular function in functional single ventricle.

El-Said H1, Hegde S, Moore J.
Congenital heart disease.Congenit Heart Dis.2014 Nov-Dec;9(6):E175-8. doi: 10.1111/chd.12118. Epub 2013 Jul 3.
The patient was a male infant with L-transposition of great arteries (L-TGA), Ebstein's anomaly of the tricuspid valve, subvalvar aortic stenosis, ventricular septal defect (VSD), hypoplastic right ventricle, arch hypoplasia, and congenital complete heart block. He underwent a Norwood procedure, aor
PMID 23834756

Sudden death due to nonarrhythmic cause in a patient with L-TGA.

Hayward RM1, Ursell PC, Foster E, Tseng ZH.
Annals of noninvasive electrocardiology : the official journal of the International Society for Holter and Noninvasive Electrocardiology, Inc.Ann Noninvasive Electrocardiol.2014 May;19(3):293-7. doi: 10.1111/anec.12112. Epub 2013 Oct 7.
We report a case of sudden death in a clinically stable adult with l-transposition of the great arteries (l-TGA). Sudden death has been reported to be the leading cause of death in l-TGA and is often attributed to arrhythmias in the absence of another identifiable cause. However, the contribution of
PMID 24118753

Japanese Journal

14)左上大静脈還流異常および左側房室弁閉鎖を伴ったl-TGA+PSの1例 : 日本循環器学会第121回関東甲信越地方会

石井照之,吉武克宏,中山至誠,坂口治
Japanese circulation journal 52(SupplementI), 45, 1988-09-30
NAID 110002604263

修正大血管転換症(l-TGA)における心室中隔欠損(VSD)の有無によるスカラー心電図右側胸部誘導(V_<1>)のQSとqRの成因の相違

高橋良明,高尾篤良,近藤千里,相羽純,高見沢邦武,今井康晴,高梨吉則,黒沢博身,河村剛史
Japanese circulation journal 49回学術集会, 295, 1985-03-01
NAID 110002567002

Related Pictures

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