II. Epidemiology

  1. Incidence: 5% of Congenital Heart Disease
  2. Males affected more often than females by 3:1

III. Pathophysiology

  1. Aorta and pulmonary arteries are transposed
  2. Pulmonary and systemic circulation are separate
    1. Parallel, rather than sequential

IV. Associated Conditions

  1. Ventricular Septal Defect (30-35%)
  2. Pulmonic stenosis (5%)
  3. Pulmonic stenosis with VSD (10%)
  4. Aortic Coarctation (5%)

V. Signs

  1. Cyanosis if ventricular septum intact
    1. Cyanosis onset in delivery room: 50%
    2. Cyanosis onset within 24 hours of life: 90%
  2. Single S2 Heart Sound

VI. Diagnostics: Electrocardiogram

VII. Imaging

  1. Chest XRay (Classic triad) - Egg on a string appearance
    1. Egg shaped cardiac silhoutte
    2. Narrow mediastinum
    3. Increased pulmonary markings
  2. Echocardiogram

VIII. Management: Initial

  1. Ductus-Dependent
    1. Keep ductus arteriosus open
    2. Administer Prostaglandins (PGE1)
  2. Correct metabolic abnormalities
  3. Correct severe Hypoxia

IX. Management: Surgical

  1. Balloon atrial septoplasty
    1. Temporizing method
  2. Arterial switch operation
    1. Procedure of choice
    2. Most complex surgery of Congenital Heart Disease repairs
    3. Pulmonary artery and aorta divided and reattached
    4. Atrial Dysrhythmias are less common with this repair method than with prior methods

X. Prognosis

  1. Five year survival after arterial switch: 82%

XI. Complications associated with surgical repair

  1. Pulmonic stenosis
  2. Aortic Stenosis
  3. Coronary Artery obstruction
  4. Ventricular dysfunction
  5. Arrhythmia
  6. Mitral Regurgitation

XII. References

  1. Cyran (1998) PREP review lecture, October, Phoenix
  2. Merenstein (1994) Pediatrics, Lange
  3. Saenz (1999) Am Fam Physician 59(7):1857-66 [PubMed]

Images: Related links to external sites (from Bing)

Related Studies

Ontology: Transposition of Great Vessels (C0040761)

Definition (NCI_CDISC) Transposition of the great vessels is a congenital heart defect in which the two main arteries leaving the heart are reversed (transposed).
Definition (NCI) A congenital cardiac defect in which two heart vessels are reversed (transposed).
Definition (CSP) congenital cardiovascular malformation in which the aorta arises entirely from the right ventricle and the pulmonary artery from the left ventricle so that the venous return from the peripheral circulation is recirculated by the right ventricle via the aorta without being oxygenated in the lungs.
Definition (MSH) A congenital cardiovascular malformation in which the AORTA arises entirely from the RIGHT VENTRICLE, and the PULMONARY ARTERY from the LEFT VENTRICLE. Consequently, the pulmonary and the systemic circulations are parallel and not sequential, so that the venous return from the peripheral circulation is re-circulated by the right ventricle via aorta to the systemic circulation without being oxygenated in the lungs. This is a potentially lethal form of heart disease in newborns and infants.
Concepts Congenital Abnormality (T019)
MSH D014188
ICD9 745.10, 745.1
SnomedCT 204304005, 268317005, 156917005, 268176002, 26146002, 204297006
English Great Vessels Transpositions, transposition of great vessel, AORTA-PULMONARY ART TRANSPOSITION, TRANSPOSITION OF GREAT VESSELS, Transposition of great vessels, Complete transpos gt arteries, Complete transposition of great arteries, Discord va conn + concord av, Discordt ventric/arterial connct + concordt atriovent connct, Great vessel transposition NOS, Total great vessel transpositn, transposition of great artery, complete transposition of great arteries (diagnosis), complete transposition of great arteries, complete transposition of great vessels (diagnosis), complete transposition of great vessels, Aorta-pulmonary art transposition, Aorta-pulmonary arterial transposition, Transposition aorta-pulmonary artery, Transposition of the great vessels, Compl transpos great ves, Transposition of Great Vessels [Disease/Finding], Great Arteries Transposition, Transposition of Great Arteries, Dextro Looped Transposition of the Great Arteries, Great Arteries Transpositions, Dextro-Looped Transposition of the Great Arteries, arteris great transposition, great transposition vessel, transposition great arteries, Transposition;great vessels, transposition great vessels, transposition of great arteries, arteries great transposition, artery great transposition, great transposition vessels, transposition of great vessels, Great Vessels Transposition, Transposition of Great Vessels, Great vessel transposition NOS (disorder), Complete transposition of great vessels, Classical transposition of great vessels, Discordant ventriculoarterial connection with concordant atrioventricular connection, Total great vessel transposition, Complete TGA, Complete transposition of great vessels (disorder), Total great vessel transposition (disorder), Transposition of the great arteries, great vessels; transposition, transposition; great vessels, Transposition of great vessels, classical, Transposition of great vessels, NOS
Portuguese TRANSPOSICAO DOS GRANDES VASOS, Transposição aorta-artéria pulmonar, Transposição completa dos grandes vasos, Transposição de grandes vasos, TRANSPOSICAO ART AORTA-PULMONAR, Transposição dos grandes vasos, Transposição dos Grandes Vasos
Dutch volledige grote vatentranspositie, transpositie van aorta-longslagader, transpositie van grote vaten, aorta-longslagadertranspositie, grote vaten; transpositie, transpositie; grote vaten, transpositie van de grote vaten, Arterie, transpositie grote, Transpositie, grote arteriën
French Transposition des gros vaisseaux aorto-pulmonaires, Transposition complète des grands vaisseaux, Transposition de l'artère aorto-pulmonaire, TGV (Transposition des Gros Vaisseaux), TRANSPOS ARTERIELLE AORTO-PULMONA, TRANSPOSITION DES GROS VAISSEAUX, Transposition des gros vaisseaux, Discordance ventriculo-artérielle isolée, Discordance ventriculoartérielle isolée
German Transposition von Aorta und Pulmonalarterie, komplette Transposition der grossen Gefaesse, Aorta und Pulmonalarterie, Transposition, TRANSPOSITION AORTA/ARTERIA PULMO, TRANSPOSITION GROSSE HERZGEFAESSE, Transposition der grossen Gefaesse, Transposition der großen Gefäße
Italian Trasposizione arteriosa aorto-polmonare, Trasposizione completa dei grossi vasi, Trasposizione dei grossi vasi
Spanish Transposición arterial aorto pulmonar, Transposición de grandes vasos, Transposición completa de los grandes vasos, TRANSPOSICION ARTERIAS AORTA-PULM, TRANSPOSICION GRANDES VASOS, Transposición de las arterias pulmonar y aórtica, transposición de los grandes vasos, SAI, transposición de los grandes vasos, SAI (trastorno), transposición clásica de los grandes vasos, transposición completa de los grandes vasos (trastorno), transposición completa de los grandes vasos, transposición de los grandes vasos, transposición total de los grandes vasos (trastorno), transposición total de los grandes vasos, Transposición de los grandes vasos, Transposición de los Grandes Vasos
Russian ОТХОЖДЕНИЕ АОРТЫ ОТ ПРАВОГО ЖЕЛУДОЧКА СЕРДЦА, OTKHOZHDENIE AORTY OT PRAVOGO ZHELUDOCHKA SERDTSA, TRANSPOZITSIIA MAGISTRAL'NYKH SOSUDOV, ТРАНСПОЗИЦИЯ МАГИСТРАЛЬНЫХ СОСУДОВ
Japanese 大血管転位, 大動脈・肺動脈転位, 完全大血管転位, ダイケッカンテンイ, ダイドウミャクハイドウミャクテンイ, カンゼンダイケッカンテンイ
Swedish Transposition av de stora kärlen
Czech transpozice velkých cév, Aortopulmonální arteriální transpozice, Aortopulmonální transpozice, Transpozice velkých cév, Úplná transpozice velkých cév, transpozice velkých tepen
Finnish Valtasuonten vaihtuminen
Polish Przełożenie pni tętniczych
Hungarian Aortopulmonalis transpositio, Nagyér transpositio, Nagyerek transpositiója, Transpositio aorta-pulmonalis arteria, Arterialis aorta-pulmonalis transpositio, Nagy erek teljes transpositiója
Norwegian Transposisjon av de store arteriene