Double outlet right ventricle {S,D,L} with subaortic ventricular septal defect and pulmonary stenosis

Praagh, Richard Van; Pérez-Treviño, C; Reynolds, James L.; Moës, C. A. F.; Keith, John D.; Douglas, Roy; Belcourt, Christian; Weinberg, Paul M.; Parisi, Lucy

doi:10.1016/0002-9149(75)90557-3

Cited by 62 publications

(26 citation statements)

References 13 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…As mentioned earlier, similar hearts have been described in the literature. [16][17][18][19][20][21] Several possible pathogenetic explanations for these hearts have also been proposed. One tentative explanation for this malformation is that the aortopulmonary septum that separates the fourth (aortic) and sixth (pulmonary) aortic arches fuses with the outflow tract (conotruncal) septum in reverse.…”

Section: Discussionmentioning

confidence: 99%

“…2 It was shown that the positions of the semilunar valves in hearts with transposition of the great arteries resembled those in stage 15, whereas the semilunar valve positions in hearts with tetralogy of Fallot resembled those in stage 18. The semilunar valve positions in normal hearts were found to be most similar to those in stage 19, a period when normal cardiogenesis is largely complete.`6 These results suggested that these anomalies arise as arrests in the normal rotation of the semilunar valve axis. The results also suggested that other transposition complexes, such as double-outlet right ventricle, might also be caused by arrests in the rotation of the semilunar valve axis.…”

mentioning

confidence: 99%

See 1 more Smart Citation

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

Bostrom

Hutchins

1988

Circulation

View full text Add to dashboard Cite

In a previous study the possibility that tetralogy of Fallot and transposition of the great arteries may arise as a result of embryonic arrests in the normal rotation of the junction of the outflow tract and the great arteries was investigated. The results suggested that the development of other transposition complexes such as double-outlet right ventricle might also be related to arrests in this process of rotation. To further study this question 20 normal hearts and 15 hearts with double-outlet right ventricle obtained at autopsy were studied. The angle of the aortic-to-pulmonary valve axis relative to the inferior surface of the heart, as viewed from apex to base, was measured from postmortem radiographs. For normal hearts the mean angle was 81 4(SE) degrees. For 13 of the 15 hearts with double-outlet right ventricle the mean angle was 4 + 7(SE) degrees. Two hearts with doubleoutlet right ventricle showed markedly divergent aortic-to-pulmonary valve angles, with a mean of 228 + 1 1 (SE) degrees, and were therefore grouped separately. Although direct comparison of hearts and embryos is difficult because of the differences in methods of determining angles, the valve positions in normal hearts was most similar to Carnegie stage 19, as found in an earlier study. The majority of the hearts with double-outlet right ventricle resembled stage 16 embryos. The results of this study, as well as those of the earlier studies, support the hypothesis that a spectrum of cardiac anomalies with anomalous origin of great vessels arises as arrests in the normal rotation of the semilunar valve region during embryogenesis.

show abstract

Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 99%

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

Bostrom

Hutchins

1988

Circulation

View full text Add to dashboard Cite

show abstract

“…1,2 The anomaly is designated as ACM[S,D,L] when there is atrioventricular and ventriculoarterial concordant connection and the great arteries arise above the anatomically correct ventricles. When the aorta overrides onto the right ventricle more than 50%, the anomaly is designated as DORV[S,D,L].…”

Section: Commentsmentioning

confidence: 99%

“…1,2 Anatomically corrected malposition (ACM [SDL]) and double-outlet right ventricle with left malposition (DORV [SDL]) are included in this category and have close anatomical similarities to intraventricular structure and associated anomalies. The difference between these two anomalies is not always clear, particularly when both have mitral/aortic discontinuity and a large ventricular septal defect (VSD) is subaortic, which occurs frequently in both diseases.…”

Section: Introductionmentioning

confidence: 99%

Double-outlet right ventricle with left malposition of the great arteries and total anomalous pulmonary venous connection

Ishiyama

Kurosawa

Shinoka

et al. 2007

Gen Thorac Cardiovasc Surg

View full text Add to dashboard Cite

The patient was diagnosed with double-outlet right ventricle with left malposition of the great arteries, total anomalous pulmonary venous connection, and pulmonary stenosis at 2 months of age. Because the progression of pulmonary venous obstruction was suspected at 2 years of age, the patient was catheterized and underwent a definitive repair. The patient had a right aortic arch and left juxtaposition of the atrial appendages; the intraventricular anatomy exhibits malalignment between the conal septum and the muscular intraventricular septum, and the conal septum is well developed and dextroposed, resulting in pulmonary obstruction and multiple ventricular septal defects (VSDs). Because a supracardiac type (Darling's type Ib) of total anomalous pulmonary venous connection was also combined with such an anomaly, total correction consisted of an anastomosis of the left atrium and the common pulmonary vein, creation of an intraventricular tunnel, direct closures of muscular VSDs, pulmonary valvotomy, and infundibular resection. To our knowledge, this is the first description of the successful surgical correction of this type of anomaly in one stage.

show abstract

“…The levo-transposed aorta was at one time considered diagnostic of corrected transposition of the great arteries . Increasing experience with conotruncal abnormalities has shown that an abnormally leftward aorta may be found in patients with complete transposition of the great arteries (D-ventricular loop [6]), double-outlet right ventricle (D-ventricular loop [12]), double-outlet left ventricle (D-ventricular loop [8] and L-ventricular loop [10]), single right and left ventricle [4,7], ACM of the great arteries (D-ventricular loop [1-3, 5, 9, 11]), and now in ACM of the great arteries (L-ventricular loop) .…”

Section: Anatomically Corrected Malposition Of the Great Arteries In mentioning

confidence: 99%

Anatomically corrected malposition of the great arteries in situs ambiguus with polysplenia

et al. 1985

View full text Add to dashboard Cite

show abstract

Double outlet right ventricle {S,D,L} with subaortic ventricular septal defect and pulmonary stenosis

Cited by 62 publications

References 13 publications

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

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

Double-outlet right ventricle with left malposition of the great arteries and total anomalous pulmonary venous connection

Anatomically corrected malposition of the great arteries in situs ambiguus with polysplenia

Contact Info

Product

Resources

About