CASE HISTORY

In childhood had impaired vision owing to dislocated lenses and went to blind school. Vision improved during last 5 years. "Dislocated ankle" from birth. Wore callipers and special boots until 15. Some residual disability on walking. Chesty as a child with winter bronchitis and pneumonia aged 7. Said to have had abnormal heart as a child. Always tall with long thin fingers and toes. No history of rheumatic fever. In usual health till 6 months before admission when she began to feel tired, listless and breathless on exertion, especially on stairs. Unable to lie flat because of breathlessness. 4 pillows at night. Occasional chest pain on exertion. 21.7.58. Attended OPD. signs of aortic incompetence. JVP + No pulmonary congestion. No oedema. Xray left pleural effusion. Heart LV, LA, RV + PA +. Aorta prominent. Lungs not over filled. ECG partial heart block. LV ++. 3 weeks before admission: Onset of continuous pain across lower chest "like a belt" continuous for 3 days. Thereafter only on exertion. No radiation. Became dyspnoeic at rest, and had frequent vomiting. Admitted to West Middlesex hospital. Pulse 120 regular. Given Digoxin, Mersalyl and Chlorothiazide. Improved and able to get up and about. 18.9.59. Admitted to Hammersmith hospital. OE tall (6ft) thin girl with florid Marfan's syndrome. Spidery digits, hypertelorism, slight internal concomitant strabisimus and symmetrical ptosis with dislocated lenses. Joints lax but flexion deformity of small joints of hands, elbows, shoulders and ankle joints. Exostoses R side sacrum and L lateral malleolus. Long patellar ligaments. High arched palate. Not cyanosed. CVS: JVP +5 with "a" and "c" waves predominating. Pulse 120, equal on two sides, regular, collapsing. Capillary pulsation. Femorals easily felt. Heart: AB 7th interspace in mid-axillary line. Loud diastolic murmur all over praecordium maximal at left sternal edge. Soft systolic murmur at apex with some distribution and heard over carotids. BP 140/40. Chest slightly kyphoscoliosis: few basal rales. Abdomen, urine NAD. CXR Heart: further enlargement. Two days after admission developed cough, pyrexia, pleural rub and increase rales on right side. CXR: patchy consolidation. Treatment: Digoxin, Mersalyl, Tetracycline and Novobiocin. Then Penicillin and Streptomycin. Heart failure increased and became drowsy. Gradually went downhill and died on 6.10.58

PATHOLOGY

No accompanying notes.