[Tetralogy of fallot with absent pulmonary valve]. / L'agenesie des valves pulmonaires avec communication interventriculaire.

BACKGROUND: Absent pulmonary valve with ventricular septal defect is a rare cardiac malformation. The aim of our study is to specify the anatomic characteristics and the clinical and echocardiographic features of this cardiac malformation and to discuss its management. METHODS: We report 8 cases of absent pulmonary valve with ventricular septal defected collected over a period of 24 years. The diagnosis was made during the first year of life in all cases in the presence of respiratory symptoms and/or cyanosis. It was confirmed by cardiac catheterization in 4 cases and echocardiography in 4 cases. RESULTS: Pulmonary vascular obstructive disease, related to aortopulmonary collateral vessels, was noted in one patient who died at the age of 20 years. One patient was lost to follow up and 6 patients were operated. Closure of the ventricular septal defect, widening of the pulmonary tract and insertion of a pulmonary valve were performed in the 6 cases. Reduction procedure of the pulmonary arteries was performed in 3 cases. One death related to early postoperative infective endocarditis was noted. At a mean follow up of 2 years, the 5 survivors are going well with no significant pulmonary stenosis. CONCLUSION: Although named "tetralogy of Fallot with absent pulmonary valve", absent pulmonary valve with ventricular septal defect is different from tetralogy of Fallot by aneurysmal dilatation of the pulmonary arteries which may compress the bronchial tree and lead to respiratory symptoms that can be severe with respiratory distress. Pulmonary arterioplasty eliminate airways obstruction and its results are satisfactory. The need for insertion of a pulmonary valve is debatable.

[Konno aortoventriculoplasty: indications and results. 13 cases]. / Aorto-ventriculoplastie de Konno: indications et resultats. A propos de 13 cas.

BACKGROUND: Konno procedure is used to enlarge the aortic root and increase the size of the aortic valve implanted. AIM: The aim of this study is to present the indications of the Konno procedure in congenital and acquired aortic lesions, in young patients with normal or hypoplastic aortic annulus or adults with small aortic annulus. METHODS: We reported our experience of this technique with a retrospective study of 13 patients operated on between 1998 and 2004. RESULTS: The mean diameter of aortic annulus measured in echocardiography is 16.2 mm, and the mean prosthetic valve diameter in post operative control is 20.3 mm. The early Death rate was 16%. In the late follow up of 2 years, the only predictor of myocardial hypertrophy regression was the presence of aortic insufficiency before the operation. In the literature, Konno procedure is reported as a good surgical option for the enlargement of the left ventricular outflow tract, but the results with the Ross-Konno operation are more promising.