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Postgrad Med J: first published as 10.1136/pgmj.50.583.276 on 1 May 1974. Downloaded from Postgraduate Medical Journal (May 1974) 50, 276-281. Cardiac involvement in idiopathic haemochromatosis and the effect of venesection S. J. JACHUCK G. S. RAI B.Sc., M.B.B.S. M.B.B.S. C. FOSSARD M.B.B.S., M.Sc., M.R.C.P. Department of Medicine, Newcastle General Hospital, Newcastle upon Tyne Summary Introduction Eight patients with idiopathetic haemochromatosis Cardiac failure, occasionally associated with have been followed up for 2-15 years to study their cardiac dysrhythmias, is considered to be the com- cardiac state and the effect of repeated venesection in monest single cause of death in haemochromatosis treating their disability. Five of these patients had no (Truelove and Reynell, 1972) and accounts for about clinical cardiac disability. Serial electrocardiographs one-third of deaths (Thompson, 1969; Skinner and were done in five patients and significant abnormality Kenmure, 1973). Reactionary fibrosis due to was seen in three of them at the time of admission. increased iron deposit in the parenchyma is a sequele After removal of excess iron the cardiac disability pro- of haemochromatosis and the aim of treatment is to gressed in two patients and regressed in one other. One deplete the body of excess iron. Removal of excesscopyright. of these presented with palpitations and was found to iron from the body results in clinical, biochemical have Wolff-Parkinson-White syndrome. Not one of and histological improvement (Williams et al., 1969). our patients developed cardiac failure and the two Although haemochromatosis usually manifests in deaths which occurred were the result of hepatic later life and is associated with diabetes mellitus, the failure. At autopsy one of them was found to have cardiographic changes described so far are not rela- metastases in the myocardium from a hepatoma, a ted to coronary artery disease (Swan and Dewar, complication we have not encountered in previous 1952). in the literature have often in- reports. Previous reports http://pmj.bmj.com/ cluded with haemosiderosis in Correspondence: Dr S. J. Jachuck, Cruddas Park patients secondary Medical Centre, Park Road, Newcastle upon Tyne addition to those with haemochromatosis. NE4 7RZ This paper describes the cardiac involvement in I II m AVR AVL AVF on October 2, 2021 by guest. Protected v~v-- v, V2 V3 V4 V5 V6 FIG. 1. ECG of patient W.W. showing ischaemic changes. Postgrad Med J: first published as 10.1136/pgmj.50.583.276 on 1 May 1974. Downloaded from Idiopathic haemochromatosis 277 idiopathic haemochromatosis and the effect of re- peated venesection on the cardiac state. We use this data to support the view that cardiac failure is no VI longer the commonest cause of death in haemo- chromatosis (Williams et al., 1969). Methods and material The diagnosis of idiopathic haemochromatosis was made in eight patients, noting clinical mani- V2 festations of pigmentation and hepatic dysfunction associated with grade IV iron deposits in the liver parenchyma (Scheuer, Williams and Muir, 1962). In all cases the serum iron levels were greater than 300 Lg/% with more than 80% saturation of trans- ferin. The possibility of secondary haemosiderosis was excluded in each patient. We describe the presenting complaint, the clinical manifestations of cardiac disability and electro- observation in - cardiographic five of them. Three -h V3 other patients had no clinical evidence of cardiac disability and unfortunately electrocardiographic recordings were not carried out. Each patient was treated by repeated venesection and to date two deaths have occurred. In both cases a hepatoma has been found at autopsy. copyright. Patient H.E. A 59-year-old man who was known to have had AVR epilepsy for 39 years and diabetes for 4 years was admitted to this hospital in July 1966 when a diagno- sis of haemochromatosis was made. At that time he denied any cardiac disability. An electrocardiogram was normal and the chest X-ray showed no cardio- http://pmj.bmj.com/ pulmonary abnormality. Venesection removing 500 ml blood on each occasion was commenced. After AVL-- removing 30 g of iron the laboratory findings were as follows: haemoglobin, 14-9 g/%; serum iron, 214 ug; and iron binding capacity, 252 ug with 85 % saturation. At this time he denied any cardiac disability and his electrocardiogram showed sinus rhythm at a rate of70/min. Occasional supraventricu- AVF on October 2, 2021 by guest. Protected lar extra-systoles were seen. The P wave was bifid and the duration was 0-08. No other abnormality was seen. Patient W. W. FIG. 2. ECG of patient W.W. 3 years later showing further ischaemic changes. A 53-year-old man presented with several years history of increasing pigmentation and listlessness. improved. Thirteen years later he developed angina He was found to have haemochromatosis associated of effort and exertional dyspnoea. with a pre-diabetic glucose tolerance curve. He The electrocardiogram (Fig. 1) showed sinus denied any cardiac disability and the electrocardio- rhythm at a rate of 55/min. There were small Q graph was normal. After removing 17 g of iron by waves in leads II, III, AVF and the ST segment was repeated venesection his haemoglobin fell to 9-9 depressed in lead I and V V-VII. No conduction g/% and the serum iron to 16 jg/% (iron binding defect was seen. Since then his cardiac disability has capacity 399 pg/%, 4% saturation). His hepatome- gradually deteriorated and the electrocardiogram galy noted on admission subsided and his symptoms shows progressive changes (Fig. 2). Postgrad Med J: first published as 10.1136/pgmj.50.583.276 on 1 May 1974. Downloaded from 278 S. JaJnrhuk et al Patient R.M. observed during his stay in the hospital. Despite In October 1969, a 66-year-old man presented to repeated venesection, his cardiac disability increased. this hospital with a 3 years' history of paroxysmal Nine months later he developed a junctional tachy- palpitations, exertional dyspnoea and one episode of cardia with marked left axis deviation and ST paroxysmal nocturnal dyspnoea. He was found to depression (Fig. 4) suggesting further myocardial have a diabetic glucose tolerance curve. The electro- damage. His heart rate came down to 100/min with cardiogram at the time of diagnosis (Fig. 3) showed practolol but the junctional dysrhythmia persisted. sinus rhythm at a rate of 72/min. The P wave was bifid and its duration was 0-12 sec. The QRS con- Patient M.T. figuration was compatible with Wolff-Parkinson- A 76-year-old man with diabetes treated with White syndrome. The ST segment was depressed in chlorpropamide was admitted for management of lead III and AVF. Inversion of T waves was seen in ascites when the diagnosis was made. His electro- leads III, AVF and V I-III. No dysrhythmia was cardiogram (Fig. 5a, b) showed sinus rhythm with I I I AVl, AVL AVF copyright. http://pmj.bmj.com/ V V2 V3 V, V5 V6 FIG. 3. ECG of patient R.M. showing Wolff-Parkinson-White syndrome. I I I AVR AVL AVF on October 2, 2021 by guest. Protected VL V2 V3 V4 VS V6 FIG. 4. ECG of patient R.M. 9 months later showing junctional tachycardia, left axis deviation and ST depression. Postgrad Med J: first published as 10.1136/pgmj.50.583.276 on 1 May 1974. Downloaded from Idiopathic haemochromatosis 7 (a) I IT II AVR AVL AVL ^L- -L J^ I V V3 v4 V5 V (b) copyright. V2 FIG. 5. Ischaemic changes (a) and rhythm strip (b) showing supraventricular extrasystole seen in M.T. AVL AVF I I: i AVR http://pmj.bmj.com/ on October 2, 2021 by guest. Protected - V, - V2V V,V3 V4 Vs5 VV6 FIG. 6. ECG of patient M.T. after 16 months showing electrocardiographic improvement compared to Fig. 5. supraventricular extra-systoles. T waves were inver- disease was admitted to one of the neurosurgical ted and there was no conduction defect. His general wards for stereotaxic surgery. He was found to have condition has improved considerably with vene- haemochromatosis with diabetes mellitus, and denied section and diuretic therapy and his electrocardio- any cardiac disability. The electrocardiograph was gram (Fig. 6) shows improvement. normal apart from occasional ventricular extra- systoles. He was treated with carbohydrate restric- Patient C.J. tion, insulin and repeated venesection. Two years In 1964 a 57-year-old man with Parkinson's later he was readmitted following the sudden onset Postgrad Med J: first published as 10.1136/pgmj.50.583.276 on 1 May 1974. Downloaded from 280 S. J. Jachuck et at. :.dsP,ii~BE~ii'·la.0 ~ ~t~ j i~ ~~s i~h~i~sk··~*~·ls~,~s~~;"~~~~"liAto~ .i I~nm r~·~h-*~Wi~~i~sEYIIEIE~8~ ~ ~i7vd'C ?p ·:i :·i..Xi FIG. 7. Metastatic deposit in the wall of the left ventricle from a hepatoma. copyright. of a right hemiplegia, and died 4 days later. His showed bifid P waves. In patient C.J. autopsy electrocardiograph showed no abnormality. At revealed metastatic deposition of tumour cells in the autopsy, haemochromatosis was confirmed and left ventricle, but the coronary arteries were patent. there were extensive metastases from a hepatoma. Patients H.E. and M.R. showed bifid P waves, sug- Three intramural metastatic deposits were found in gesting left atrial hypertrophy, possibly the result of the wall of the left ventricle (Fig. 7) and each measur- myocardial damage leading to left ventricular failure. ing about 5 mm in diameter. No conduction defect was seen in any of these patients, except for patient M.R. showing Wolff- Discussion Parkinson-White syndrome.

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