Skip to
  1. Homepage
  2. Rare diseases
  3. Search
Simple search

Simple search

*
(*) mandatory field





 

Other search option(s)

Vascular malposition

Orpha number ORPHA2452
Synonym(s) -
Prevalence Unknown
Inheritance -
Age of onset Infancy
Neonatal
ICD-10 -
ICD-O -
OMIM -
UMLS -
MeSH -
MedDRA -

Summary

Malpositioned great vessels are defined by an abnormal ventriculoarterial junction combined with interventricular communication. They are due to the defective development or resorption of the subaortic and pulmonary conus. It concerns a great variety of anatomic localizations, hemodynamics mainly depend on whether the malformation is associated with a pulmonary stenosis or a hypoplastic coarcted aorta. Resulting hypoxemia varies with the volume of pulmonary flow and the relative position of the vessels with the ventricles and the interventricular communication. Corrective surgery bringing the left ventricle towards the aorta and right ventricle to pulmonary artery without using prosthetic tubes is usually possible when the interventricular communication is located under the vessels. Surgery may simply consist of tunneling the left ventricle toward the aorta or tunneling the left ventricle toward the pulmonary artery and then switching the arteries and transferring the coronaries or still yet tunneling the left ventricle toward the aorta and repositioning the pulmonary artery (with no valve) on the right ventricle. When one of the three surgical procedures is possible, operatory risks are low and results are satisfactory in the middle term and probably in the long term.

Expert reviewer(s)

  • Pr Daniel SIDI

(*) Required fields.

Attention: Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted. For all other comments, please send your remarks via contact us. Only comments written in English can be processed.


Captcha image
The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.