This is an 18-year-old male who suddenly manifests itself in the street.
His clinical history included the diagnosis of Noonan syndrome since childhood with normal psychomotor development and no medical controls at the time of death.
There were no other family members with a similar phenotype.
Findings highlighted in the external examination: corpse corresponding to a 175 cm tall male and 78 kg body weight (BMI 25.5 kg/m2) with various anatomical characteristics: broad legs and cheek deformity of the forehead, short feet.
Internal examination revealed 395 g heart weight (mean body weight: 345 g [range 261-455 g]).
Left ventricular wall thickness: 1.1 cm, septum: 1.1 cm and right ventricle: 0.1 cm. non-permeable oval artery.
Atrioventricular and pulmonary valves were normal.
Bicuspid aortic valve
There was dilatation of the aortic root with complete circular dissection of the arterial intima at the level of the annulus of valve insertion with complete detachment and incised artery of the arch extending to the left lumbar area completely dissected.
1.
The microscopic study provided the following data of interest: moderate signs of myocyte hypertrophy in left ventricular myocardium with a focal disorganizing pattern in septum ̄.
Adipose myocardial anterior and posterior right ventricular with interstitial fibrosis without chronic inflammatory reaction.
Cystic necrosis of the aortic arterial mean.
Renal stricture with microlithiasis and mild hepatic steatosis.
