CASE HISTORY

Patient had rheumatic fever at the age of 13 and again at 32 and 33 years of age. In 1952 he became dyspnoeic and this had worsened since. Two year before death he developed ankle swelling. Admitted to National Heart hospital in 1952 and again in 1953 and had been on outpatient management since. For the last year he had become dyspnoeic on one flight of stairs. Three weeks before admission sudden deterioration in exercise tolerance. Admitted to hospital and given increased digitalis and mersalyl with marked improvement. Began to vomit, thought to be due to digitalis overdose and digitalis was stopped but vomiting continued. On 13.7.57 dyspnoea got worse and he was admitted to Hammersmith hospital. He was then jaundiced and dyspnoeic; pulse 110, fibrillating, small volume; heart enlarged to right and left; B.P. 130/80. He remained very dyspnoeic in spite of treatment, crepitations in the lungs increased, jaundice deepened and he died in congestive cardiac failure. Cardiac catheterisation carried out at the National heart hospital in January 1957 showed PAP to 45/20 with a mean of 30; RVP 50/5; RA mean pressure 4; CO4.5 1/min. Pulmonary resistance normal. Report recorded: elevated pulmonary capillary pressure with low pulmonary vascular resistance. No pressure gradients across pulmonary or tricuspid valves. Findings compatible with mitral stenosis plus mitral incompetence.

PATHOLOGY

Heart skeleton – Rheumatic heart disease – Mitral stenosis, mitral incompetence

Heart 150mmx185mmx90mm.The heart weighed 535g (N = 320g). The left atrium is moderately dilated and is hypertrophied to about one and a half times normal thickness. The endocardium is thickened and looks white and opaque. The mitral valve is stenosed and calcified. The stenosis is caused by fusion at the anterior commissure and there is virtually no fusion at the anterior commissure. There is extensive calcification of the valve cusps with complete rigidity of the greater portion of them, so that the valve is almost a solid, calcified diaphragm. The left ventricle is dilated but very little hypertrophied. The aortic valve, which can be seen from above, shows fibrous thickening of all its cusps, but there is no stenosis or incompetence. Right atrium is hypertrophied to more than twice its normal thickness and is quite considerably dilated. The tricuspid valve is a trifle thickened, particularly on its septal cusp where a piece has been taken for section. The tricuspid ring is widely dilated and the valve, which is normal size, is insufficient to fill the large orifice and consequently there has been a relative or functional tricuspid incompetence. The right ventricle is very greatly dilated and hypertrophied to more than twice its normal thickness. The pulmonary valve appears healthy.