Facial resemblance to a patient in whom supravalvular aortic stenosis was discovered and successfully relieved at operation has led to the correct diagnosis of supravalvular stenosis in three other patients. All four patients are mentally subnormal. The presence of supravalvular aortic stenosis in mentally retarded patients with the unusual facial features here detailed may constitute a syndrome that has not previously been described.
Congenital diverticula of the ventricles are rare and Skapinker (1951) was able to collect only twelve reported cases. In most of these, as in Skapinker's own patient, the diverticulum arose from the left ventricle but in two it communicated with both ventricles. Skapinker resected the diverticulum in his case, an operation that had previously been reported only once (Roessler, 1944); but further cases treated in this way have since been reported by Potts et al. (1953) and Bailey (1955). Other recent reports are by Formijne (1950), Snellen et al. (1952), and Parsons (1957).Included in the following description of a patient, in whom surgical resection was successfully undertaken, are electrocardiographic and manometric observations not hitherto recorded. The latter are possibly relevant to the mechanism of rupture of such diverticula, an accident known to have been the cause of death in at least three of the published cases. CASE REPORTThe patient, an eight-year-old girl, was referred for surgical treatment of a pulsatile epigastric mass which, because of its superficial position, was considered to be a potential threat to her in the event of trauma. The pulsation had been present at birth and was at that time associated with an umbilical hernia. When she was one year old the hernia had been repaired, the surgeon noting what he took to be an anomalous artery passing up from the epigastrium to disappear under the right costal margin. Visible epigastric pulsation had since persisted without change and without related symptoms. In addition cyanotic congenital heart disease with isolated dextrocardia had been recognized in the neonatal period and the patient had had nonprogressive slight cyanosis and impairment of effort tolerance throughout her life. At the age of eight she was able to run no further than thirty yards. She did not squat and there had been no attacks of severe cyanosis or unconsciousness. The family history and history relating to the maternal pregnancy revealed no unusual features.Examination showed a thin girl, rather small for her age, with slight central cyanosis and finger clubbing. Physical findings relating to the heart argued for pulmonary hypertension rather than pulmonary stenosis as the cause of shunt reversal; and these, together with the radiographic demonstration of isolated dextrocardia with a left-sided aortic arch and pulmonary vascularity verging on the upper limit of normal (Fig. 1) and the electrocardiogram, consistent in this context with right ventricular hypertrophy (Fig. 4), pointed to a clinical diagnosis of the Eisenmenger complex with dextrocardia. This was supported by the results of cardiac catheterization which included a pulmonary arterial pressure of 112/64 mm. Hg, reduction of brachial arterial oxygen saturation to 88 per cent, and a rise in oxygen saturation from 65 per cent in the superior vena cava and right atrium to 70 per cent in the right ventricle and pulmonary artery.In the epigastrium there was a pulsatile structure which extended upwards from just above the...
Infective endocarditis (IE) secondary to Staphylococcus aureus and streptococcus species comprises the majority of cases in literature with Gram negative bacterial insults occurring infrequently. Serratia marcescens is a Gram negative bacillus which is classified as motile, non-lactose fermenting, and a facultative anerobe. The presumed risk factor for the development of S. marcescens IE is intravenous drug use (IVDU). We report two cases of IE causes by S. marcescens: first case describes IE of tricuspid and aortic valve requiring surgical intervention further complicated by epidural abscess. The second case was associated with renal and splenic infarct. These cases highlight the severity and complicated nature of S. marcescens IE. Given S. marcescens IE has been infrequently described in the literature, we believe that our cases are worth reporting to contribute to the present incidence and management of S. marcescens IE.
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