Myxoma, dyspnoea, tinnitus, scoliosis, and alopecia

In June, 2009, a 51-year-old woman presented to us with progressive dyspnoea on exertion, pulsatile tinnitus, back pain, and hair loss. Dyspnoea had started at least 1 year previously. During this time her exercise tolerance had decreased from an active lifestyle to the inability to climb stairs. 2 months before presentation, she started to have intrusive, pulse-synchronous tinnitus in her left ear only. Hair loss had started 2 years ago, and she had taken to wearing a wig. She admitted to back pain. Her medical and family history were unremarkable. She was taking atenolol and hydrochlorothiazide at low doses for hypertension. Physical examination showed diff use non-scarring alopecia. She had a stooped posture, leaning slightly to the left. Pulse rate was 82 bpm and blood pressure 132/80 mm Hg. Auscultation of the praecordium was notable for accentuated heart sounds as well as systolic and diastolic murmurs with positional variation in intensity. The lungs were clear. ECG and chest radiography were unremarkable. Transthoracic echo cardiography revealed a giant left atrial mass that prolapsed into the ventricle during diastole (fi gure A). The mass, which was excised under cardiopulmonary bypass and hypothermic cardiac arrest (fi gure B), arose by a pedicle from the region of the fossa ovalis in the left atrium. Histology showed stellate cells embedded in abundant myxoid stroma, diagnostic of atrial myxoma. Postoperatively her tinnitus and dyspnoea resolved. She commented, with surprise, that she felt comfortable standing straight. At follow up in June, 2010, alopecia had also resolved and she was asymptomatic. Atrial myxoma was fi rst characterised in 1845. The histologically benign neoplasm is the commonest primary tumour of the heart, with peak incidence between the third and sixth decades of life, more commonly in women. Atrial myxomas have an endocardial origin and typically project into the cardiac chamber, of which the most frequently aff ected is the left atrium. From this position the myxoma can provoke a wide spectrum of symptoms. Dyspnoea is caused by cardiac obstruction. The degree of obstruction from a pedunculated myxoma varies with posture, manifesting as characteristic postural variability of symptoms and murmur. Our patient subconsciously minimised her obstruction by leaning to the left, presumably causing the myxoma to move to a haemodynamically favourable position. The compensatory scoliosis, evident on a family portrait years earlier, caused her back pain which promptly resolved postoperatively. A prolapsing myxoma also narrows the ventricular outfl ow tract during isovolumic contraction. The resulting turbulent blood fl ow can cause pulsatile tinnitus. Lateralisation to the left is due to the asymmetric anatomy of the aortic arch, with the left carotid artery branching directly from the aorta. Systemic and cutaneous manifestations of atrial myxomas arise through aberrant elaboration of cytokines, tumour embolisation, or in the context of complex genetic syndromes. Our patient’s alopecia is most consistent with a humoral eff ect and resolved upon myxoma removal. Atrial myxomas are among the great mimickers in clinical medicine and can present with many diff erent symptoms. A timely diagnosis is critical because atrial myxomas are usually fatal without surgery.