No abstract

In 63 resected thoracic aortic aneurysms, the commonest histological finding (45 cases, 71.4%) was cystic medionecrosis. These cases formed two groups, 29 with widespread fragmentation and loss of elastic tissue (elastopathy) and 16 cases without elastopathy who were older and included most of the 18 cases of dissecting aneurysms. Thirteen patients had the Marfan syndrome, 10 showing cystic medionecrosis with elastopathy, indistinguishable from the cases with no Marfan stigmata although partial 'dissections' were mainly found in the Marfan patients, Histological appearances ranged from normal to complete loss of media. Cystic changes in muscle fibres apparently preceded elastic fragmentation. Fourteen cases (22%) had aortitis: 4 were syphilitic and 3 of other known aetiology. In 7 patients the aetiology of the inflammatory process was unknown and appearances included granulomatous infarct-like lesions and necrotizing aortitis or changes indistinguishable histologically from syphilis.

A rare case of anomalous origin of the right coronary artery from the pulmonary artery associated with a large aortopulmonary window in a 4-month-old boy is reported. The right coronary artery is exposed to systemic pressure and carries fairly well-oxygenated blood to the myocardium. Angiographic diagnosis could be difficult because of the simultaneous filling of both great arteries which obscures the origin of the anomalous vessel. Closure of the aortopulmonary window alone could result in acute myocardial ischaemia. A plastic procedure for correction of this association of defects, which should allow normal growth, is described.

No abstract

Modern development in physiopathology, diagnosis and medical treatment of haemocoagulative syndrome, allows to operate electively upon various organs and apparatus.3. 4. 8 Quite numerous appear in literature, particularly, the references regarding surgical operations,. performed in haemophilics. The availability of adequate preparations which replace the lacking factors of the coagulation, in fact, allows in such patient to arrive to a normal haemostasis as long as the length of the operation and during the post-operative period, with very low operative risk. In literature there is only one case2 regarding surgical intervention on haemophilics on cardiorespiratory apparatus. We think it worthwhile to report a case of voluminous abdominal aneurysm in an haemophilia B patient with good result. CASE REPORTThe patient was a 55-year-old male. Since childhood he noted the appearance of post-traumatic hematomas and haemorrhagic episodes of high intensity and duration during dental avulsions. In November 1970 he suffered of acute posteroinferior myocardial infarction. During 1971 the patient noted a pulsatile tumefaction in the mesogastric region. He was hospitalized in May 1971.On admission his general conditions were good. The blood pressure was 160/ 80 mmHg, the pulse 80, rhythmical, and the respirations were 16 for minute. The abdomen was irregularly globous due to the presence in the mesogastric region of a voluminous pulsatile tumefaction about 10 X 8 cm in diameter. The palpation confirmed the speculative character of the mass and, moreover, permitted to appreciate the smooth, regular surface of it, the elastic consistency and the reduced motion. The mass presented a typical expansive pulsation, with small retard on the peripheral pulses. A systolic murmur was heard on the tumefaction. The pheripheral pulses were slightly reduced bilaterally, especially on the left. The haemogenic tests revealed a haemocoagulative defect originated from lack of factor IX or haemophilia B. The abdominal roentgenogram showed an opacity of hemispheric form, on the right at the median line, immediately under the corresponding renal image, and extending from the II to V lumbar space, without calcifications, with pulsation synchronous with the arterial pulse. The electrocardiogram showed signs of old postero-inferior my-