Grand Rounds - Hammersmith Hospital: Cardiac transplantation for AL amyloidosis

Abstract

Case presented by: R Hall, P N Hawkins Chairman: J Scott, professor of medicine Discussion group: M B Pepys, professor of immunological medicine; Julia M Polak, professor of endocrine pathology; K A Davies, senior lecturer in rheumatology. Systemic AL amyloidosis has a poor prognosis which corresponds closely with the severity of cardiac disease.1,2 Some patients benefit from chemotherapy directed at the underlying monoclonal gammopathy,3 but the median period of treatment required for a clinical response is one year, and patients with severe cardiac amyloid do not usually survive for more than six months.1 Cardiac transplantation has rarely been done, presumably because of concerns about the systemic nature of the disease. A personal account is given here by a professor of medicine who presented at the age of 53 with extensive AL amyloidosis and received a cardiac transplant in 1984. I was born on 1 October 1931 and was healthy until 1978, when I noted muscle fatigue on climbing hills. Over the next five years a series of apparently unrelated symptoms developed including pruritus, hoarseness, snoring. In 1981 I developed paroxysmal atrial fibrillation, usually precipitated by effort. In 1982 I was investigated for atypical chest pain on exertion at the London Chest Hospital, when electrocardiography and coronary artery and left ventricular angiography gave normal results. By 1984 I had developed ankle oedema and right hypochondrial pain on exertion and right heart failure due to presumed cardiomyopathy was diagnosed. Later that year AL amyloidosis was diagnosed after a left ventricular biopsy. By this time electrocardiographic and echo findings were consistent with this condition. A normal bone marrow result excluded myeloma, although free lambda light chains were detected in my urine. I was referred to Harefield Hospital, and on 17 November 1984 I had an orthotopic cardiac transplantation …