[Extensive partial agenesis of the left pericardium. An anatomo-clinical correlation. Report of 2 cases]

The authors report on two patients with large partial congenital defect of the left pericardium, as observed at post-mortem examination. In both cases, diagnosis was made during life thanks to the clinical syndrome described by Abbott, Sothworth and Stenvenson. In the first case diagnosis was confirmed by pneumothorax proposed by Ellis et al and the second case was recognized by the clinical-radiological syndrome. The first patient was followed until his death. Because of an abnormal echogram, he was submitted to exploratory cardiotomy and small ASD, aneurysmatic dilation of Valsalva sinus and mitral regurgitation by ruptured chordae tendineae were found. The autopsy, besides the findings at the operation, showed residual adhesive pericarditis with a cavity at the postero-superior region of the heart behind the left auricle and a redundant tricuspid cusp. The association of congenital pericardium defect to mitro-tricuspid redundant cusps and aneurysmatic dilatation of the sinus of Valsalva--suggesting myxomatous degeneration--plus atrial septal defect as in case 1, has not been found in the medical literature. In the second patient the defect was recognized in spite of congestive heart failure and severe tricuspid regurgitation.(ABSTRACT TRUNCATED AT 250 WORDS)