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Management and outcome of D-transposition of the great arteries

Authors
David R Fulton, MD
David A Kane, MD
Section Editor
John K Triedman, MD
Deputy Editor
Carrie Armsby, MD, MPH

INTRODUCTION

Transposition of the great arteries (TGA) is a ventriculoarterial discordant lesion, in which the aorta arises from the right ventricle and the pulmonary artery from the left ventricle. The most common form of TGA is the dextro type (referred to as D-TGA), in which the ventricles are oriented so that the right ventricle is positioned to the right of the left ventricle and the origin of the aorta is anterior and rightward to the origin of the pulmonary artery (figure 1). The anatomical defect of D-TGA leads to cyanotic heart disease as a result of two parallel circulations. The first sends deoxygenated systemic venous blood to the right atrium and back to the systemic circulation via the right ventricle and aorta, and the second sends oxygenated pulmonary venous blood to the left atrium and back to the lungs via the left ventricle and pulmonary artery.

The survival of patients with D-TGA has dramatically improved from a uniformly fatal disease with introduction of initial medical management consisting of prostaglandin (also known as alprostadil) therapy and balloon atrial septostomy (BAS), and the development of corrective surgical procedures.

The management and outcome of D-TGA will be presented here. The pathophysiology, clinical features, and diagnosis of D-TGA are discussed separately. (See "Pathophysiology, clinical manifestations, and diagnosis of D-transposition of the great arteries".)

INITIAL MANAGEMENT

Initial management is focused on stabilization of cardiac and pulmonary function and ensuring adequate systemic oxygenation. Therapy is directed towards providing sufficient intercirculatory mixing between the two parallel circulations by maintaining patency of the ductus arteriosus with prostaglandin E1 (alprostadil) infusion and/or balloon atrial septostomy (BAS).

Once the infant is stabilized, corrective surgery is optimally performed in the first weeks of life.

                        

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Literature review current through: Nov 2016. | This topic last updated: Mon Jan 04 00:00:00 GMT+00:00 2016.
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