Aortic valve replacement for severe aortic regurgitation caused by idiopathic giant cell aortitis.

Abstract

Giant cell aortitis occurred in a 25-year-old woman, with absent pulses in the left arm and severe aortic regurgitation from dilatation of the valvar annulus. The aortic valve was replaced by a Starr-Edwards prosthesis, and the patient was treated with steroids. Five years later, she continues asymptomatic and haemodynamically stable. The left brachial and radial pulses have returned.Giant cell aortitis can be a cause of aortic regurgitation, but it is rarely of such severity as to warrant valve replacement. A review of published reports showed only four such cases. 1-3 Two of these patients also had aneurysmal dilatation of the ascending aorta.1 2 We report a case of giant cell aortitis in a 25-year-old woman who had absent pulses in the left arm and severe aortic regurgitation from dilatation of the valve ring. The patient was successfully treated by aortic valve replacement and steroids. Case reportA 25-year-old woman was admitted to hospital in January 1974 with a two-year history of increasing tightness in the chest, dyspnoea on exertion or excitement, and frequent temporal headaches. She gave no history of rheumatic fever, chorea, arthritis, tuberculosis, venereal disease, or polymyositis, and there was no family history of heart disease. Her right eye had been enucleated for retinoblastoma at the age of 2 years. Three years before her present admission she had an ovarian cystectomy that was followed by pulmonary embolism, necessitating anticoagulation. Six months later, on a routine medical examination, she was found to have a precordial aortic diastolic murmur.She was pale and had absent left brachial and radial pulses and feeble pulses in the right arm. The pulse was regular. The blood pressure in the Address for reprint requests: Jack Cleland, FRCS, Cardiac Surgical Unit, Royal Victoria Hospital, Grosvenor Road, Belfast BT12 6BA, Northern Treland. right arm was 100/65 mmHg, and 260/60 mmHg in the legs. It was unrecordable in the left arm.The jugular venous pressure was normal, but there was prominent carotid pulsation. The apex beat was diffuse and heaving, with a diastolic thrill over the left parasternal border. Both heart sounds were audible with a harsh, ejection systolic murmur, grade 3/6 in the aortic area, loudest over both the carotid arteries and a grade 3/6 early diastolic murmur along the left parasternal border. There was no hepatomegaly, ascites, or oedema.A chest radiograph showed moderate cardiomegaly with pulmonary venous congestion in the upper lobes. The electrocardiogram showed sinus rhythm, and left ventricular hypertrophy. The haemoglobin was 10-7 g/dl, the haematocrit 33% and the erythrocytes normochromic and normocytic. The white cell count was 5 4X 109/l and the ESR was 58 mm in one hour (Westergren)