Arrested rotation of the outflow tract may explain double-outlet right ventricle.

Bostrom, Mathias P.; Hutchins, Grover M.

doi:10.1161/01.cir.77.6.1258

Cited by 30 publications

(16 citation statements)

References 39 publications

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“…In our study, the anterior displacement of the RCO was more important in DORV than in TOF and TOF‐PA, which may indicate that the rotation is less achieved in DORV than in TOF and TOF‐PA. This would agree with the results of previous human anatomical studies suggesting that the rotation is arrested at different developmental stages according to the type of outflow tract defect (Bostrom & Hutchins, ; Lomonico et al. ).…”

Section: Discussionsupporting

confidence: 93%

The pattern of the coronary arterial orifices in hearts with congenital malformations of the outflow tracts: a marker of rotation of the outflow tract during cardiac development?

et al. 2013

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Outflow tract defects, including cardiac neural crest defects (so-called conotruncal defects) and transposition of the great arteries, are due to an abnormal rotation of the outflow tract during cardiac development. Coronary orifices are often abnormal in outflow tract defects, particularly in common arterial trunk (CAT). A recent study indicates that abnormal coronary artery pattern in a mouse model with common arterial outlet (Tbx1À/À mouse mutant) could be due to a reduced and malpositioned subpulmonary coronary-refractory myocardial domain. The aim of our study was to demonstrate the relation between coronary orifices pattern in outflow tract defects in human and the abnormal embryonic rotation of the outflow tract. We analyzed 101 heart specimens with outflow tract defects: 46 CAT, 15 tetralogy of Fallot (TOF), 29 TOF with pulmonary atresia (TOF-PA), 11 double-outlet right ventricle with subaortic ventricular septal defect (DORV) and 17 controls. The position of left and right coronary orifices (LCO, RCO) was measured in degrees on the aortic/truncal circumference. The anterior angle between LCO and RCO (a) was calculated. The LCO was more posterior in TOF (31°), TOF-PA (47°), DORV (44°), CAT (63°), compared with controls (0°, P < 0.05), and more posterior in CAT than in other outflow tract defects (P < 0.05). The RCO was more anterior in TOF (242°), TOF-PA (245°) and DORV (271°) than in controls (213°, P < 0.05), but not in CAT (195°). The a angle was similar in TOF, TOF-PA, DORV and controls (149°, 162°, 133°, 147°), but significantly larger in CAT (229°, P < 0.0001). In all outflow tract defects but CAT, the displacement of LCO (anterior) and RCO (posterior), while the a angle remains constant, might be due to incomplete rotation of the myocardium at the base of the outflow tract, leading to an abnormally positioned subpulmonary coronary-refractory myocardial domain. The larger a angle in CAT could reflect its dual identity, aortic and pulmonary.

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Section: Discussionsupporting

confidence: 93%

The pattern of the coronary arterial orifices in hearts with congenital malformations of the outflow tracts: a marker of rotation of the outflow tract during cardiac development?

et al. 2013

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“…24 Moreover, measurements on embryos with conotruncal defects suggested that this rotation is prematurely arrested at different developmental stages according to the type of malformation. 25,26 Our study provides the first direct evidence for such counterclockwise rotation of the OFT in mammalian embryos. The rotation of OFT myocardium in the mouse embryo, as visualized with the 96-16 transgene, begins at E9.5, equivalent to Carnegie stage 11, whereas rotation has been documented from Carnegie stage 15 in humans.…”

Section: Bajolle Et Almentioning

confidence: 62%

“…24 In addition, the angle of the aortic to pulmonary valve axis, relative to the inferior surface of congenitally malformed hearts, suggested that TOF, DORV, and TGA may result from an arrested rotation of the outflow region at the base of the great arteries. 25,26 We now report a counterclockwise rotation of OFT myocardium in the mouse embryo and examine this phenomenon in mice with OFT defects. A transgenic line, y96-Myf5-nlacZ- 16,27 in which ␤-galactosidase activity marks a part of OFT myocardium, permits visualization of OFT rotation before, and during, the septation of this region.…”

mentioning

confidence: 96%

Rotation of the Myocardial Wall of the Outflow Tract Is Implicated in the Normal Positioning of the Great Arteries

Bajolle

Zaffran

Kelly

et al. 2006

Circulation Research

207

156

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Abstract-Congenital heart defects frequently involve a failure of outflow tract (OFT) formation during development. We analyzed the remodeling of the OFT, using the y96-Myf5-nlacZ-16 transgene, which marks a subpopulation of myocardial cells of the pulmonary trunk. Expression analyses of reporter transcript and protein suggest that the myocardial wall of the OFT rotates before and during the formation of the great arteries. Rotational movement was confirmed by Di-I injection experiments with cultured embryos. We subsequently examined the expression of the transgene in mouse models for OFT defects. In hearts with persistent truncus arteriosus (PTA), double outlet right ventricle (DORV), or transposition of the great arteries, rotation of the myocardial wall of the OFT is arrested or fails to initiate. This is observed in Splotch (Pax3) mutants with PTA or DORV and may be a result of defects in neural crest migration, known to affect OFT septation. However, in Pitx2␦c mutant embryos, where cardiac neural crest cells are present in the heart, PTA and DORV are again associated with a rotation defect. This is also seen in Pitx2␦c mutants, which have transposition of the great arteries. Because Pitx2c is involved in left-right signaling, these results suggest that embryonic laterality affects rotation of the myocardial wall during OFT maturation. We propose that failure of normal rotation of OFT myocardium may underlie major forms of congenital heart disease. (Circ Res. 2006;98:421-428.)Key Words: heart Ⅲ outflow tract Ⅲ Splotch Ⅲ Pitx2 Ⅲ transposition of the great arteries C ongenital heart defects are observed in 0.8% of children in developed countries and are responsible for more than 20% of spontaneous abortions and 10% of all stillbirths.

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“…A counter-clockwise rotation of the infundibular septum (IS) [2] from tetralogy of Fallot (TF) to different forms of double outlet right ventricle (DORV) to transposition of great arteries (TGA) has been described [3-5]. …”

Section: Introductionmentioning

confidence: 99%

The trabecula septomarginalis (Leonardo’s cord) in abnormal ventriculo-arterial connections: anatomic and morphogenetic implications

Capuani

2014

J Cardiothorac Surg

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BackgroundThe abnormal ventriculo-arterial connections in atrio-ventricular concordance and situs solitus with two well developed ventricles include the range from tetralogy of Fallot throughout the different forms of double outlet right ventricle to transposition of great arteries.The infundibular septum and the trabecula septomarginalis are the fundamental anatomical landmarks for the segmental analysis.In these abnormalities there is a pathological progressive counter-clockwise rotation of the infundibular septum which divorces from the antero-superior limb of the trabecula septomarginalis and achieves his identity. Is there any anatomical evidence of a simultaneous abnormal counter-clockwise rotation of the trabecula septomarginalis?MethodsMalposition of great arteries is a generic term since all relationships have to be expected.We present specimens with anatomical evidence of a progressive counter-clockwise rotation from 0° to about 180°of the plane passing throughout the trabecula septomarginalis’s limbs.ResultsWe can observe sequentially:1. Malformations in which the posterior limb of the trabecula septomarginalis is committed to the ventriculo infundibular fold: (tetralogy of Fallot, double outlet right ventricle with sub-aortic ventricular septal defect, truncus arteriosus and doubly committed ventricular septal defect);2. Malformations in which the posterior limb of the trabecula septomarginalis is committed to the infundibular septum (double outlet right ventricle with sub-pulmonary ventricular septal defect, transposition of great arteries).Conclusions1. The sequential-segmental analysis identify all the morphologies.2. The trabecula septomarginalis plane presents a progressive counter-clockwise twist on the long axis.3. Since the trabeculated portions of the ventricles are the oldest developmental components, our observations support the hypothesis that the abnormal ventriculo-arterial connections could be in relation with a pathological myocardial process during early cardio-genesis.We are promoting new studies to investigate our anatomical observations.

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Arrested rotation of the outflow tract may explain double-outlet right ventricle.

Cited by 30 publications

References 39 publications

The pattern of the coronary arterial orifices in hearts with congenital malformations of the outflow tracts: a marker of rotation of the outflow tract during cardiac development?

The pattern of the coronary arterial orifices in hearts with congenital malformations of the outflow tracts: a marker of rotation of the outflow tract during cardiac development?

Rotation of the Myocardial Wall of the Outflow Tract Is Implicated in the Normal Positioning of the Great Arteries

The trabecula septomarginalis (Leonardo’s cord) in abnormal ventriculo-arterial connections: anatomic and morphogenetic implications

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