| DacronŽ |
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A synthetic material often used to fashion conduits and other prosthetic devices for the surgical palliation or repair of congenital heart disease.
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| Damus-Kaye-Stansel operation |
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A procedure reserved for patients with abnormal ventriculo-arterial
connections who are not suitable for an arterial switch operation (e.g. TGA
and non-suitable coronary patterns, DORV with severe subaortic stenosis).
The operation involves anastomosis of the proximal end of the transected
main pulmonary artery in an end-to-side fashion to the ascending aorta to
provide blood flow from the systemic ventricle to the aorta; coronary
arteries are not translocated and are perfused in a retrograde fashion. The
aortic orifice and a VSD (if present) are closed with a patch. A conduit
between the right ventricle and the distal pulmonary artery provides venous
blood to the lungs. The procedure was described in 1975. (Damus PS.
Correspondence. Ann Thorac Surg 1975;20:724-725.) (Kaye MP. Anatomic
correction of transposition of the great arteries. Mayo Clin Proc
1975;50:638-640.) (Stansel HC Jr. A new operation for d-loop transposition
of the great vessels. Ann Thorac Surg 1975;19:565-567.)
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| David operation |
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A surgical procedure for ascending aortic aneurysm, involving replacement of
the ascending aorta with a synthetic tube and remodelling of the aortic root
so the preserved aortic valve is no longer regurgitant (David TE, Feindel
CM. An aortic valve sparing operation for patients with aortic incompetence
and aneurysm of the ascending aorta. J Thorac Cardiovasc Surg
1992;103:617-621.)
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| dextrocardia |
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Cardiac apex directed to the right of the midline. see cardiac position.
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| dextroposition |
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Rightward shift of the heart. see cardiac position.
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| dextroversion |
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An old term for dextrocardia. see cardiac position.
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| differential hypoxemia; differential cyanosis |
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A difference in the degree of hypoxemia/cyanosis in different extremities as
a result of the site of a right to left shunt. The most common situation is
of greater hypoxemia/cyanosis in feet and sometimes left hand, as compared
to right hand and head, in a patient with an Eisenmenger PDA.
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| diGeorge syndrome |
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An autosomal dominant syndrome now known to be part of "CATCH-22". As
originally described, it consisted of infantile hypocalcemia,
immunodeficiency due to thymic hypoplasia, and a cono-truncal cardiac
abnormality. see also CATCH-22.
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| discordant atrio-ventricular connections |
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Anomalous connection of atria and ventricles such that the morphologic right
atrium connects via a mitral valve to a morphologic left ventricle, while
the morphologic left atrium connects via a tricuspid valve to a morphologic
right ventricle.
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| discordant ventriculo-arterial connections |
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Anomalous connection of the great arteries and ventricles such that the
pulmonary trunk arises from the left ventricle and the aorta arises from the
right ventricle.
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| diverticulum of Kommerell |
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Enlarged origin of the left subclavian artery associated with right aortic
arch. Its diameter may be equal to that of the descending aorta and tapers
to the left subclavian diameter. It is found at the origin of the aberrant
left subclavian artery, the fourth branch off the right aortic arch.
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| double aortic arch |
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see aortic arch anomaly.
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| double-chambered RV |
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Separation of the right ventricle into a higher-pressure inflow chamber, and
a lower pressure infundibular chamber, the separation usually being produced
by hypertrophy of the "septomarginal band". When a VSD is present, it
usually communicates with the high pressure RV inflow chamber.
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| double discordance |
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see congenitally corrected transposition of the great arteries.
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| double inlet left ventricle (DILV) |
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see univentricular connection.
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| double orifice mitral valve |
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The mitral valve orifice is partially or completely divided into two parts
by a fibrous bridge of tissue. Both orifices enter the left ventricle.
Mitral regurgitation and/or mitral stenosis may be present. Aortic
coarctation and atrioventricular septal defect are commonly associated
defects.
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| double outlet left ventricle (DOLV) |
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Both the pulmonary artery and the aorta arise predominantly from the
morphologic left ventricle. DOLV is rare, and much less frequent than double
outlet right ventricle (DORV).
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| double outlet right ventricle (DORV) |
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Both great arteries arise predominantly from the morphologic right
ventricle; there is usually no fibrous continuity between the semilunar and
the A-V valves; a ventricular septal defect is present. When the VSD is in
the subaortic position without RV outflow tract obstruction, the physiology
simulates a simple VSD. With RV outflow tract obstruction, the physiology
simulates tetralogy of Fallot. When the VSD is in the subpulmonary position
(the Taussig-Bing anomaly) the physiology simulates complete transposition
of the great arteries with VSD. see also Taussig-Bing anomaly.
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| double switch procedure |
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An operation used in patients with l-transposition of the great arteries (l-TGA;
congenitally corrected transposition of the great arteries; cc-TGA) and also
in patients who have had a prior Mustard or Senning atrial switch operation
for complete transposition of the great arteries (d-TGA). It leads to
anatomic correction of the ventricle to great artery relationships such that
the left ventricle supports the systemic circulation. It includes an
arterial switch procedure (see Jatene operation) in all cases, as
well as an atrial switch procedure (Mustard or Senning) in the case of l-TGA,
or reversal of the previously done Mustard or Senning procedure in the case
of d-TGA.
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| doubly-committed VSD |
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see ventricular septal defect.
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| Down syndrome |
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The most common malformation caused by trisomy 21. Most of the patients
(95%) have complete trisomy of chromosome 21; some have translocation or
mosaic forms. The phenotype is diagnostic (short stature, characteristic
facial appearance, mental retardation, brachydactyly, atlanto-axial
instability, thyroid and white blood cell disorders). Congenital heart
defects are frequent, atrioventricular septal defect and ventricular septal
defect being the most common. Mitral valve prolapse and aortic regurgitation
may be present. Down syndrome patients are prone to earlier and more severe
pulmonary vascular disease than might otherwise expected as a consequence of
the lesions identified.
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| dural ectasia |
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Expansion of the dural sac in the lumbo-sacral area, seen on CT or MRI. It
is one of the criteria used to confirm the diagnosis of Marfan syndrome. (Pyeritz
RE et al. Dural ectasia is a common feature of the Marfan syndrome Am J Hum
Genet 1988;43:726-732.) (Fattori R et al. Importance of dural ectasia in
phenotypic assessment of Marfan's syndrome. Lancet 1999;354:910-913.)
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