CASE HISTORY

Patient suffered from chorea at the age of 9, 12 and 15 and noticed shortness of breath whilst at school. Heart disease was diagnosed at the age of about 17 years but she married at 20 and had four children without any disability. At 27 she had rheumatic fever and developed effort dyspnoea and palpitations. At the age of 64 she first attended with "sub-acute intestinal obstruction". This was, however, not confirmed and she was transferred to the medical wards. She was slightly cyanosed. Fibrillation was controlled by digitalis. Apex beat in anterior axillary line, Grade 3 systolic murmur, and rumbling diastolic murmur and thrill. There was doubtful aortic diastolic murmur. The liver was slightly enlarged but not tender. There was moderate oedema of legs. Screening sowed a giant left auricle. She was diagnosed as having mitral stenosis and was followed up at intervals in out-patients. She remained well enough to do her housework until at the age of 66 she awoke one morning with hemiplegia. After this she did not make a satisfactory recovery and remained in chronic block. Two years later she developed a sacral-haematoma which became infected with staphylococcus pyogenes, after which she developed firstly pyelonephritis and then bacterial endocarditis. She was treated with Penicillin and Streptomycin and later with Aureomycin. This rendered her afebrile but she developed profuse diarrhoea. This improved and she recovered but then collapsed. Blood cultures became positive and she went rapidly down hill and died. At autopsy, there were healed infarcts in both kidneys and multiple portal vein thrombi. There was an old cerebral infarction due to embolism and healed pyelonephritis of the left kidney as well as the lesions of heart and colon. 

PATHOLOGY

Heart 265mmx155mmx80mm. Part of the heart and colon have been mounted. The left atrium is enormously dilated. It measures 150mm transversely and about 120mm antero-posteriorly and also vertically. This has bulged the septum to the right reducing the right atrium to a crescent shaped cavity. In spite of this very great dilation, the wall is only thinned in places and for the most part is only a little thinner than normal, implying that there must have been a great deal of thickening. The mitral valve shows typical rheumatic scarring. From above it is crescent-shaped with stenosis. The anterior cusps is ragged due to ulceration and bacterial endocarditis ( a section has been taken). Viewed from below the valve, two cusps are thickened and fibrosed. There is fusion at both commissures and there is concentric fibrous thickening around all the chordae, which in fact stretched down on to the papillary muscles, but these have been cut away. The aortic valve shows a little thickening, particularly between the right coronary cusp and the non-coronary cusp, but apart from this it is healthy and certainly appears to be competent. The pulmonary and tricuspid valves are healthy. This patient seems to have escaped pulmonary hypertension because there is no hypertrophy of the right ventricle. There was neither congestion nor siderosis of the lungs, nor was there any recognisable damage to the pulmonary vasculature. Colon: the last 55cms of the colon show numerous raised white nodules. These are closely clustered in the lowest 20cms (left hand specimen) and are less frequent in the upper 35cms (right hand specimen).