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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.

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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-

Hydatid cyst of the heart is an uncommon lesion which usually develops in the left ventricle. Diagnosis should be considered in patients coming from an area where hydatid cyst is endemic and who present with an abnormal heart shadow on chest X-ray, frequently associated with T wave changes on the electrocardiogram. The cyst tends to grow and thus compress the neighboring myocardium. It causes displacement of the coronary vessels, rhythm disturbances and mechanical interference with the AV valves and ventricular function. Spontaneous involution of the cyst with calcification of its wall as well as rupture with anaphylactic shock may occur. Early excision with standby cardiopulmonary bypass is advisable. Three patients with hydatid cyst of the left ventricle, 2 of whom were operated on by using cardiopulmonary bypass, are presented. Results of the surgical treatment, both early and late, are rewarding, with relief of symptoms, regression of electrocardiographic abnormalities and very low incidence of recurrence.

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(1977). Thorax, 32,[365][366][367][368][369] Syndrome of congenital ventricular diverticulum and midline thoraco-abdominal defects. The long-term follow-up of a 30-year-old patient who had a correction of the syndrome of left ventricular diverticulum and thoraco-abdominal defects is presented. The main features of the syndrome include a diverticulum of the left ventricle, a ventricular septal defect, and sometimes other cardiac anomalies. The thoraco-abdominal defects consist of foreshortened sternum, pericardial and diaphragmatic defects, and umbilical hernia. The association of these anomalies is thought to be due to a developmental failure of the primitive paramidline mesoderm.The diagnosis can be made clinically by the presence of a pulsatile, epigastric mass associated with signs of cardiac septal defects and dextrocardia. The prognosis of patients with this syndrome depends mainly on the associated abnormalities although rupture of the diverticulum can be a fatal complication.The treatment of choice is resection of the diverticulum combined with repair of associated anomalies which can give good early and late results.The association of a congenital ventricular diverticulum with -other cardiac abnormalities and midline thoraco-abdominal defects has been described by Cantrell et al. (1958). Its recognition is essential for accurate identification of the different anomalies and their proper treatment. The purpose of this paper is to describe the long-term result in a patient in whom complete correction of the syndrome had been performed six years previously and to draw attention to the characteristic lesions and their significance. Case reportA 30-year-old woman was admitted to Harefield Hospital with a history of cyanosis and increasing dyspnoea which were first noted when she was 4 years old after an operation for umbilical hernia. There was no family history of cardiac disease or congenital anomalies. On admission the physical signs included clubbing of the fingers and moderate cyanosis. The jugular venous pressure was normal and the radial pulse regular at a rate of 90 per minute. The blood pressure was 120/80 mmHg. There was also a slight prominence of the right chest with no definite evidence of ventricular hypertrophy. A pulmonary systolic thrill was felt and a loud systolic murmur was heard in the right parasternal area over the fourth interspace. In the abdomen there was the scar of an umbilical herniorrhaphy but no masses were palpable.Laboratory tests showed a red cell count of 4-3 X 1012/1 with a haemoglobin of 18 g/dl and haematocrit of 44%. Serological tests for syphilis were negative. The radiograph showed oligaemic lung fields and the heart was slightly enlarged and situated to the right of the spine with the appearance of dextrocardia. However, the aortic knuckle and the stomach were on the left (Fig. 1)