Anatomically corrected transposition of the great arteries.

Praagh, Richard Van; Praagh, Stella Van

doi:10.1136/hrt.29.1.112

Cited by 132 publications

(57 citation statements)

References 11 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Their previously reported cases of ACM had all had a bilateral conus [ 2 ]. These observations proved that ACM can occur both with a suboartic conus (only) and with a bilateral (subaortic and subpulmonary) conus.…”

Section: Associated Malformationsmentioning

confidence: 64%

“…First documented convincingly with photographs by Richard and Stella Van Praagh in 1967 [ 2 ] and later in 1975 [ 7 ], this rare condition is frequently misdiagnosed as congenitally corrected TGA. There are six forms of ACM as shown in Fig.…”

Section: Surgery Of Anatomically Corrected Malpositionmentioning

confidence: 96%

“…For example, Case 1 of the Van Praaghs [ 2 ] was a 2 year and 20 day old girl with ACM {S,D,L} who also had ( Fig. 30.2 ) dextrocardia, tricuspid atresia, a large secundum atrial septal defect, left-sided juxtaposition of the atrial appendages, a small conoventricular type of ventricular septal defect (VSD), and pulmonary valvar stenosis (PS) with a bicuspid and bicommissural pulmonary valve.…”

Section: Associated Malformationsmentioning

confidence: 98%

“…Case 2 of the Van Praaghs [ 2 ] was a girl who died at 4 weeks and 3 days of age. Autopsy revealed ACM {S,D,L} (Fig.…”

Section: Associated Malformationsmentioning

confidence: 99%

“…In 1971 it was suggested that these anomalies be called anatomically corrected malposition of the great arteries [ 3 ], rather than anatomically corrected transposition ─ which was the prior name of these malformations [ 2 ], in the interests of anatomic accuracy. In ACM, transposition of the great arteries is not present, by defi nition [ 1 , 3 ].…”

Section: Introductionmentioning

confidence: 99%

See 4 more Smart Citations

Anatomically Corrected Malposition of the Great Arteries

Praagh

Praagh²,

Lacour-Gayet

2016

Surgery of Conotruncal Anomalies

View full text Add to dashboard Cite

Anatomically corrected malposition (ACM) of the great arteries is a rare anomaly of the conotruncus (infundibulum and great arteries) and of the ventricles. Although very malpositioned, the great arteries nonetheless arise above the morphologically appropriate ventricles, aorta (Ao) above the morphologically left ventricle (LV), and pulmonary artery (PA) above the morphologically right ventricle (RV). How is this possible? Because the ventricles loop in one direction, and the great arteries twist in the opposite direction. For example, the ventricles loop in one direction, say to the right, forming a ventricular D-loop, and the infundibuloarterial cardiovascular segment twists to the left, resulting in L-malposition of the great arteries. This is how the commonest form of ACM is formed; the resulting segmental anatomy is situs solitus of the viscera and atria, D -loop ventricles, and L -malposition of the great arteries, i.e., ACM {S,D,L}. There is atrioventricular (AV) concordance and ventriculoarterial (VA) concordance (but very different from normally related great arteries). Although there is always VA concordance in ACM, by defi nition, there can be AV discordance, as for example in ACM {S,L,D} and in ACM {I,D,L}. In terms of segmental anatomy, there are six anatomic types of ACM. Associated malformation are often clinically, hemodynamically, and surgically important. Surgical repair of ACM is rarely reported, with some publications limited to the repair of associated lesions in the {S,D,L} form with atrio-ventricular concordance. In the presence of AV discordance ({S,L,D} and {I,D,L}), the condition presents with a transposition physiology. It could be anatomically and physiologically corrected by an atrial switch operation undertaken in the fi rst month of life, or later following LV retraining. A Senning or a Mustard procedure could be considered and would be challenging because of the frequent association of left juxtaposition of the atrial appendages.

show abstract

Section: Associated Malformationsmentioning

confidence: 64%

Section: Surgery Of Anatomically Corrected Malpositionmentioning

confidence: 96%

Section: Associated Malformationsmentioning

confidence: 98%

“…Case 2 of the Van Praaghs [ 2 ] was a girl who died at 4 weeks and 3 days of age. Autopsy revealed ACM {S,D,L} (Fig.…”

Section: Associated Malformationsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%