[Aortic insufficiency caused by dystrophic aneurysm of the ascending aorta: study of development in 95 cases. Value of cutaneous biopsy in the etiologic diagnosis]. / Les insuffisances aortiques par anévrysme dystrophique de l'aorte ascendante: étude évolutive de 95 observations. Intérêt de la biopsie cutanée dans le diagnostic étiologique.

ABSTRACT

We present 95 cases of aortic valve incompetence (AI) due to a dystrophic aneurysm of the ascending aorta; the aneurysm was idiopathic in 83 cases and associated with Marfan's syndrome in 12 cases. The diagnosis was confirmed by histological examination of the aorta and aortography. Functional signs were not always present, since 42 p. 100 of patients were in NYHA classes I and II and 58 p. 100 in classes III and IV. The prevalence of angina was 26 p. 100. The cardiothoracic ratio was increased up to 0.62 +/- 0.07, and projection to the right was noted in 63 p. 100 of the cases. Sokolow's index was 5 mv or more in 24 p. 100 of the patients. The aortic root, measured in 42 patients, was dilated up to 54 +/- 13 mm. The end-diastolic left ventricular diameter was increased to 70 +/- 10 mm. Haemodynamic studies yielded the following results: cardiac index 2.33 +/- 0.6 1/min/m2, end-diastolic left ventricular pressure 24.7 +/- 12 mmHg, mean pulmonary wedge pressure 12 +/- 6 mmHg, left ventricular end-diastolic volume 199 +/- 67 ml/m2, and angiographic ejection fraction 53 +/- 12 p. 100. The angiographic volume of aortic regurgitation, graded according to Sellers' classification, was 3/4 or 4/4 in 89 patients; the maximum diameter of the dilated ascending aorta was 73 +/- 18 mm. Evolutive data confirmed that aortic incompetence due to dystrophy is more severe than that due to other causes 36 months after surgery, the overall mortality rate was 30%. Only two factors in our series seemed to be predictive of a poor prognosis the fact that the patient belonged to NYHA class III or IV, and a mean pulmonary wedge pressure above 10 mmHg. This, however, must be taken with caution in view of the small population samples studied. The value of skin biopsy was confirmed. This examination, performed in 27 patients, was compared with the pathological examination of the aorta. Skin biopsy is specific and provides a major argument in favour of dystrophic AI in patients under 40 years of age.