Biomedical subjects
Publications and source records attributed to C Pernot.
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Thirty-nine neonates with left heart obstructions (LHO) were investigated by two-dimensional echocardiography (2 D echo). Results of 37 investigations were compared with angiographic data (15 cases) and/or anatomic data (surgery: 20 cases; post-mortem examination: 20 cases). 2 D echo was always performed as an emergency procedure and was interpreted before further hemodynamic investigation. Accurate investigation of the left ventricle and of the aorta (arch and proximal branches) was possible in 38 patients (97%) by combining the supra-sternal and subcostal approaches. The existence of LHO and its localization were correctly predicted by 2 D echo in 37 of 39 cases including: aortic coarctation (Ao co) (23 cases), isolated aortic stenosis (iAo S) (5 cases), interruption of the aortic arch (IAA) (3 cases), hypoplastic left ventricle (6 cases), and ranged LHO (2 cases). In 2 patients, one of whom presented with transposition of the great arteries, a coarctation was overlooked and a false positive (Ao co) was found during the same period in a prospective study of 100 neonates by 2 D echo (sensibility 95%, specificity 99%, predictive value 97%). In all patients, 2 D echo made it possible to establish the associated lesions that could modify therapeutics. In no case was emergency diagnostic surgery necessary and the last 9 patients of this series underwent successful therapeutic surgery only on the basis of echographic data.
The "elfin facies", described by Williams and Beuren, is usually associated, beside mental retardation, with a supravalvular aortic stenosis and, more broadly, with multiple arterial stenoses. Other types of cardiac defects were described in this context, but the case reported by the authors is the first one with a tetralogy of Fallot. The hypothesis of widerspread histodysplastic lesions is discussed: the process may be early enough to alter the morphogenesis of the cardiac conus and to produce, more lately, the classical stenotic lesions of the arterial wall.
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