CASE HISTORY

Admitted to surgical ward: complaints: Gross blueness of face (lips, gums, sclera) 2. Shortness of breath. 3. Swelling of knees and ankles. Duration: Blueness and dyspnoeic on exertion. He was seen by several doctors in his youth and as a result of his symptoms, particularly his colour and shortness of breath, he was not allowed to go to school because of the distance involved, (this had a detrimental effect on his education). The cyanosis and dyspnoea have not been crippling all his life because he had been able at times, up to two years previously to play "football with the lads" (i.e. had kicked the ball about, rather than played in a team) but was always forced to give up early on account of his symptoms. December 1947, he noted that his ankles and knees began to swell. He returned to his work (case repairer) after Christmas but was not able to continue for more than two days; on the third he was forced to go to bed. He was in bed for a week and then he got up for convalescence but he never became fit enough to go back to work, but amused himself with his hobby, poultry. Because his symptoms did not clear up, he was referred to Mr C. on one of the latter's visits to stoke-on-trent. As a result of this, he was admitted to the medical side of Hammersmith hospital for full investigation in November 1948. On admission to this hospital his symptoms had got very much worse, his cyanosis and dyspnoea were worse and the swelling of his knees and ankles had progressed. Examination in November 1948. Extremely cyanosed. Normal development. Clubbing +++ Acne vulgaris of face. Gynae-comastia. Moderate swelling of both knees and ankles - mainly soft tissue, probably synovial, with some fluids. Heart: apex beat 5 1/4 " to the left of mid line. Left ventricular type. Systolic thrill maximal in third interspace. P2 easily palpable. No pulsation of pulmonary artery felt, loud systolic murmur all over precordium. P2 louder than A2, no splitting. Blood pressure 150/122 both arms. Femoral arteries felt easily. Urine: albuminuria ++. Investigation results in Nov 1949: RBC 9.7 million. Hb: 150%, 22.2gm. Plasma proteins 3.8 gm.per litre. E.C.G. right ventricular preponderance ++ and good left ventricular pattern in V.5. Xray knees and ankles: compatible with hypertrophic pulmonary ostearthropathy. Clubbing of fingers. No limitations of knee, no thickening; effusion both ankles and oedema both shins. Gynaecomastis. X-rays showed chronic periostitis with absorption terminal phalanges - afebrile. Cardiac catheterisation: 25.10.48. Right ventricle 25-45cm. Right auricular pressure 2cm. Pulmonary artery not entered. Oxygen saturation of samples from right auricle, right ventricle and superior vena cava all extremely high - a rigor prevented further investigation. Result inconclusive. Right ventricular pressure up. 5.11.48. Arterial blood 105ml/L. Unsaturated. Screening: left ventricular hypertrophy. Pulmonary orificies and artery normally prominent. 8.11.48. Angiocardiogram: inconclusive. But the blood from superior vena cava entering left auricle - producing cyanosis. Conclusion: It was considered that the patient was suffering from a Fallot abnormality with superadded hypertension, leading to a left ventricular picture which would minimise the right ventricular hypertrophy, normally an outstanding feature of the Tetralogy of Fallot. It was thought possible that there may have been an abnormal venous channel from superior vena cava to left auricle producing the otherwise unaccountable severe cyanosis. 25.1.50. Thoracotomy with Blalock operation for Tetralogy of Fallot. Progress: Patient's condition remained satisfactory on 26.1.50 but on 27.1.50 he became restless and the blood pressure fell. Drainage tube appeared to be blocked, and it was obvious that he was bleeding. Emergency thoracotomy was carried out at 6.30 pm. No actual bleeding point was seen but oozing round site of anastomosis. Patient died same evening. 

PATHOLOGY

Heart 215mmx165mmx90mm. The heart has been rotated so that the right margin has been brought forward. The right atrium and ventricle present at the front of the specimen. The atrium is relatively small and communicates with the left atrium through a rounded orifice. The tricuspid valve is visible in the middle and below this is the septal defect. The muscle of the right ventricle is just over a cm thick i.e. greatly hypertrophied. A blue glass rod has been passed through the conus and the pulmonary valve. The valve itself shows a stenosis presenting as a dome with a relatively small hole at the apex. The pulmonary artery is very thin and the left branch has been opened. The right branch passes across beneath the aorta to join the lung. Turning to the back of the specimen the left ventricle has been opened and the aorta can be seen arising in the midline and the ventricular septal defect is clearly seen below it. The innominate, the left carotid and the left subclavian arteries can be seen arising from the aorta and the left subclavian has been anastomosed by a Blalock operation to the right pulmonary artery just before it enters the lung (white arrow).