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Levo-Transposition of the great arteries is an acyanotic
congenital heart defect A congenital heart defect (CHD), also known as a congenital heart anomaly and congenital heart disease, is a defect in the structure of the heart or great vessels that is present at birth. A congenital heart defect is classed as a cardiovascul ...
in which the primary
arteries An artery (plural arteries) () is a blood vessel in humans and most animals that takes blood away from the heart to one or more parts of the body (tissues, lungs, brain etc.). Most arteries carry oxygenated blood; the two exceptions are the pu ...
(the
aorta The aorta ( ) is the main and largest artery in the human body, originating from the left ventricle of the heart and extending down to the abdomen, where it splits into two smaller arteries (the common iliac arteries). The aorta distributes o ...
and the
pulmonary artery A pulmonary artery is an artery in the pulmonary circulation that carries deoxygenated blood from the right side of the heart to the lungs. The largest pulmonary artery is the ''main pulmonary artery'' or ''pulmonary trunk'' from the heart, and ...
) are transposed, with the aorta anterior and to the left of the pulmonary artery; the morphological left and right ventricles with their corresponding atrioventricular valves are also transposed. Use of the term "corrected" has been disputed by many due to the frequent occurrence of other abnormalities and or
acquired disorder A disease is a particular abnormal condition that negatively affects the structure or function of all or part of an organism, and that is not immediately due to any external injury. Diseases are often known to be medical conditions that a ...
s 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 dextro-Transposition of the great arteries (d-Transposition of the great arteries, dextro-TGA, or d-TGA) is a potentially life-threatening birth defect in the large arteries of the heart. The primary arteries (the aorta and the pulmonary artery) ...
(d-TGA).


Signs and 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 Shortness of breath (SOB), also medically known as dyspnea (in AmE) or dyspnoea (in BrE), is an uncomfortable feeling of not being able to breathe well enough. The American Thoracic Society defines it as "a subjective experience of breathing di ...
or
fatigue Fatigue describes a state of tiredness that does not resolve with rest or sleep. In general usage, fatigue is synonymous with extreme tiredness or exhaustion that normally follows prolonged physical or mental activity. When it does not resolve ...
. 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 Cyanosis is the change of body tissue color to a bluish-purple hue as a result of having decreased amounts of oxygen bound to the hemoglobin in the red blood cells of the capillary bed. Body tissues that show cyanosis are usually in locations ...
.


Pathogenesis

In a normal
heart The heart is a muscular Organ (biology), organ in most animals. This organ pumps blood through the blood vessels of the circulatory system. The pumped blood carries oxygen and nutrients to the body, while carrying metabolic waste such as ca ...
,
oxygen Oxygen is the chemical element with the symbol O and atomic number 8. It is a member of the chalcogen group in the periodic table, a highly reactive nonmetal, and an oxidizing agent that readily forms oxides with most elements ...
-depleted ("deoxygenated") blood is pumped from the right atrium into the right ventricle, then through the pulmonary artery to the
lung The lungs are the primary organs of the respiratory system in humans and most other animals, including some snails and a small number of fish. In mammals and most other vertebrates, two lungs are located near the backbone on either side of ...
s where it is oxygenated. The oxygen-rich ("oxygenated") blood then returns, via the
pulmonary veins The pulmonary veins are the veins that transfer oxygenated blood from the lungs to the heart. The largest pulmonary veins are the four ''main pulmonary veins'', two from each lung that drain into the left atrium of the heart. The pulmonary vei ...
, 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

l-TGA is often accompanied by other heart defects, the most common type being
intracardiac Intracardiac injections are injections that are given directly into the heart muscles or ventricles. They can be used in emergencies, although they are rarely used in modern practice. History The practice of intracardiac injection originated in ...
shunts such as
atrial septal defect Atrial septal defect (ASD) is a congenital heart defect in which blood flows between the atria (upper chambers) of the heart. Some flow is a normal condition both pre-birth and immediately post-birth via the foramen ovale; however, when this d ...
(ASD) including
patent foramen ovale Atrial septal defect (ASD) is a congenital heart defect in which blood flows between the atria (upper chambers) of the heart. Some flow is a normal condition both pre-birth and immediately post-birth via the foramen ovale; however, when this d ...
(PFO),
ventricular septal defect A ventricular septal defect (VSD) is a defect in the ventricular septum, the wall dividing the left and right ventricles of the heart. The extent of the opening may vary from pin size to complete absence of the ventricular septum, creating one ...
(VSD), and
patent ductus arteriosus ''Patent ductus arteriosus'' (PDA) is a medical condition in which the ''ductus arteriosus'' fails to close after birth: this allows a portion of oxygenated blood from the left heart to flow back to the lungs by flowing from the aorta, which has ...
(PDA).
Stenosis A stenosis (from Ancient Greek στενός, "narrow") is an abnormal narrowing in a blood vessel or other tubular organ or structure such as foramina and canals. It is also sometimes called a stricture (as in urethral stricture). ''Stricture'' ...
of
valve A valve is a device or natural object that regulates, directs or controls the flow of a fluid (gases, liquids, fluidized solids, or slurries) by opening, closing, or partially obstructing various passageways. Valves are technically fitting ...
s 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.


Diagnosis

l-TGA can sometimes be
diagnose Diagnosis is the identification of the nature and cause of a certain phenomenon. Diagnosis is used in many different disciplines, with variations in the use of logic, analytics, and experience, to determine "cause and effect". In systems enginee ...
d in utero with an
ultrasound Ultrasound is sound waves with frequencies higher than the upper audible limit of human hearing. Ultrasound is not different from "normal" (audible) sound in its physical properties, except that humans cannot hear it. This limit varies ...
after 18 weeks
gestation Gestation is the period of development during the carrying of an embryo, and later fetus, inside viviparous animals (the embryo develops within the parent). It is typical for mammals, but also occurs for some non-mammals. Mammals during pr ...
. However, many cases of simple l-TGA are "accidentally" diagnosed in adulthood, during diagnosis or treatment of other conditions.


Treatment

Simple l-TGA has a very good prognosis, with many individuals being
asymptomatic In medicine, any disease is classified asymptomatic if a patient tests as carrier for a disease or infection but experiences no symptoms. Whenever a medical condition fails to show noticeable symptoms after a diagnosis it might be considered a ...
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.


References


External links

{{Medical resources , DiseasesDB = 13259 , , ICD10 = {{ICD10, Q, 20, 5, q, 20 , , ICD9 = {{ICD9, 745.12 , , ICDO = , , OMIM = , , MedlinePlus = , , eMedicineSubj = , , eMedicineTopic = , , MeshID = C535426, , Orphanet = 216694, Congenital heart defects
Aortic balloon valvuloplasty in corrected transposition of the great arteries