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Transposition of the great arteries, dextro-looped(DTGA)

MedGen UID:
332422
Concept ID:
C1837341
Congenital Abnormality
Synonym: DTGA
 
Monarch Initiative: MONDO:0012128
OMIM®: 608808

Definition

The more common form of transposition of the great arteries, dextro-looped TGA, consists of complete inversion of the great vessels, so that the aorta incorrectly arises from the right ventricle and the pulmonary artery incorrectly arises from the left ventricle. (In the less common type of TGA, levo-looped TGA, the ventricles are inverted instead) (Goldmuntz et al., 2002). This creates completely separate pulmonary and systemic circulatory systems, an arrangement that is incompatible with life. Patients with TGA often have atrial and/or ventricular septal defects or other types of shunting that allow some mixing between the circulations in order to support life minimally, but surgical intervention is always required. [from OMIM]

Clinical features

From HPO
Transposition of the great arteries
MedGen UID:
21245
Concept ID:
C0040761
Congenital Abnormality
Critical congenital heart disease (CCHD) is a term that refers to a group of serious heart defects that are present from birth. These abnormalities result from problems with the formation of one or more parts of the heart during the early stages of embryonic development. CCHD prevents the heart from pumping blood effectively or reduces the amount of oxygen in the blood. As a result, organs and tissues throughout the body do not receive enough oxygen, which can lead to organ damage and life-threatening complications. Individuals with CCHD usually require surgery soon after birth.\n\nAlthough babies with CCHD may appear healthy for the first few hours or days of life, signs and symptoms soon become apparent. These can include an abnormal heart sound during a heartbeat (heart murmur), rapid breathing (tachypnea), low blood pressure (hypotension), low levels of oxygen in the blood (hypoxemia), and a blue or purple tint to the skin caused by a shortage of oxygen (cyanosis). If untreated, CCHD can lead to shock, coma, and death. However, most people with CCHD now survive past infancy due to improvements in early detection, diagnosis, and treatment.\n\nSome people with treated CCHD have few related health problems later in life. However, long-term effects of CCHD can include delayed development and reduced stamina during exercise. Adults with these heart defects have an increased risk of abnormal heart rhythms, heart failure, sudden cardiac arrest, stroke, and premature death.\n\nEach of the heart defects associated with CCHD affects the flow of blood into, out of, or through the heart. Some of the heart defects involve structures within the heart itself, such as the two lower chambers of the heart (the ventricles) or the valves that control blood flow through the heart. Others affect the structure of the large blood vessels leading into and out of the heart (including the aorta and pulmonary artery). Still others involve a combination of these structural abnormalities.\n\nPeople with CCHD have one or more specific heart defects. The heart defects classified as CCHD include coarctation of the aorta, double-outlet right ventricle, D-transposition of the great arteries, Ebstein anomaly, hypoplastic left heart syndrome, interrupted aortic arch, pulmonary atresia with intact septum, single ventricle, total anomalous pulmonary venous connection, tetralogy of Fallot, tricuspid atresia, and truncus arteriosus.

Recent clinical studies

Etiology

Wilhelm CM, Sisk TL, Roble SL, Chisolm JL, Janevski I, Cheatham JP, Cua CL
Echocardiography 2016 Mar;33(3):437-42. Epub 2015 Nov 15 doi: 10.1111/echo.13097. PMID: 26568530

Diagnosis

Wilhelm CM, Sisk TL, Roble SL, Chisolm JL, Janevski I, Cheatham JP, Cua CL
Echocardiography 2016 Mar;33(3):437-42. Epub 2015 Nov 15 doi: 10.1111/echo.13097. PMID: 26568530

Therapy

Wilhelm CM, Sisk TL, Roble SL, Chisolm JL, Janevski I, Cheatham JP, Cua CL
Echocardiography 2016 Mar;33(3):437-42. Epub 2015 Nov 15 doi: 10.1111/echo.13097. PMID: 26568530

Prognosis

Wilhelm CM, Sisk TL, Roble SL, Chisolm JL, Janevski I, Cheatham JP, Cua CL
Echocardiography 2016 Mar;33(3):437-42. Epub 2015 Nov 15 doi: 10.1111/echo.13097. PMID: 26568530

Clinical prediction guides

Wilhelm CM, Sisk TL, Roble SL, Chisolm JL, Janevski I, Cheatham JP, Cua CL
Echocardiography 2016 Mar;33(3):437-42. Epub 2015 Nov 15 doi: 10.1111/echo.13097. PMID: 26568530

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