[Double outlet left ventricle: a rare and unusual cardiopathy. Apropos of 7 new cases]

Double outlet left ventricle is a very rare condition due to an abnormality of conotruncal morphogenesis. The authors report 7 new cases to the 119 already published, one with an anatomical variation not previously described. Three of the cases reported were of the most usual type similating tetralogy of Fallot. Two of these cases underwent complete correction with excellent results 13 months and 2 years after surgery. The third patient aged 6 months is well after initial palliative neonatal surgery. A case with an L-malposition pedicle with subpulmonary ventricular septal defect and pulmonary outflow tract obstruction died after early palliative surgery (Blalock-Taussig). A case with subaortic ventricular septal defect, pulmonary stenosis, and tricuspid atresia, underwent physiopathological correction (Fontan procedure) after a Waterston shunt and is well at 19 years of age. The other two cases presented more unusual anatomical forms aortic outflow obstruction: one had hypoplasia of the aortic arch with an isthmic coarctation requiring a Crafoord procedure in the neonatal period associating with banding followed by complete correction at 19 months of age. After 3 years, the patient is asymptomatic. The last case with atresia of the aortic valve and severe hypoplasia of the ascending aorta died after corrective surgery of first intent. Other cases have been described in the literature with different clinical presentations: absence of pulmonary or aortic obstruction; intact interventricular septum. The anatomical variability is due to the complex embryogenesis of the conotruncal region and explains the clinical diversity of this congenital cardiac malformation.