Double outlet right ventricle, malposition of the great vessels and pulmonary hypoplasia - treated with Rastelli procedure

Case contributed by Dr Fabien Ho

Presentation

Past history of DORV: VSD non-committed type and malposition of the great arteries with pulmonary hypoplasia. Blalock–Taussig shunt x 2 then Rastelli procedure (RV-PA conduit). CT analysis before conduit replacement. Pacemaker (epicardial then endocardial) due to postsurgical AV blocks.

Patient Data

Age: 20 years
Gender: Male

Mediastinal adhesion of RV-AP tube to the sternum.

Levocardy, situs solitus, PVR normal, systemic veinous return normal, atrioventricular concordance, ventriculoarterial discordance in D-loop (LV-MPA and RV-Aorta). 
VSD was non-committed to outflow tracts and has been closed during the Rastelli procedure. 

Right outflow tract:
Enlarged RV.
RV-PA conduit 12x21mm 174mm² with post-conduit dilation of MPA, left PA and right PA.
Left to right shunt due to native hypoplastic MPA which is still permeable. (Mitro-pulmonary continuity due to D-loop)

Left outflow tract:
Subaortic stenosis (217 mm², 14x20mm) followed by sinus ectasia 44x48x46 mm and tubular dilation 44x50mm. Tricuspid aortic valve.

Coronary ostia anomalies:

  • from the posterior sinus arises the Cx
  • from the right sinus arises the LAD and RCA with a common ostium. 
    LAD courses between the conduit and aorta.
  • left sinus: non coronary sinus
     

Right dominance, no coronary stenosis nor aneurysm.

No other anomalies (bronchi, lungs, liver) (not shown).

Case Discussion

This is a case of double outlet right ventricle (DORV), with balanced ventricles, however, with unbalanced and malposed great vessels with pulmonary hypoplasia, which therefore could not benefit from arterial switch. Rastelli procedure was then performed and this exam is part of the follow-up.

The subaortic stenosis is an expected long-term complication of Rastelli procedure, due to the technique used to close the VSD. RV-PA conduit replacement is also expected after Rastelli procedure, as the patient grows up while the conduit remains at the same size. 

Adhesions are particularly important to report after multiple cardiac surgeries.

Coronary anomalies are also particularly important to report to the surgeon. Here the LAD arises from the right sinus and courses between the RV-PA conduit and the aorta.
 

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Case information

rID: 73230
Published: 1st Jan 2020
Last edited: 7th Jan 2020
System: Chest, Cardiac
Tag: dorv
Inclusion in quiz mode: Included
Institution: Clinique Sainte Clotilde

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