Obstructive Right Atrial Myxoma in Association with Carney Complex

Abstract

A a one year history of progressive exertional dyspnoea was found on examination to have an elevated jugular venous pressure with large ‘a’ waves, a harsh diastolic and systolic murmur over the parasternal area loudest during inspiration and an early diastolic ‘plop’. Transthoracic echocardiography revealed a large right atrial mass attached to the inter-atrial septum (Fig. 1, Panel A), which prolapsed in and out of the populated by stellate and spindle cells (Fig. 1, Panel D; arrowdenotes a stellate cell), consistentwith the diagnosis of cardiac myxoma. Carney complex is an autosomal dominant familial neoplasia and lentiginosis syndrome, associated with cardiac myxomas, spotty pigmentation, endocrine abnormalities (adrenal, pituitary), and non-endocrine tumours (Sertolicell testicular cancer, thyroid and breast cancer). This right ventricle during the cardiac cycle (Video), causing functional tricuspid stenosis and obstruction of right ventricular filling. She also hadmultiple subcutaneous lumps on her arms (Fig. 1, Panel B) and thighs, which were also present in her mother and sister, as well as naevi and ephelides. During cardiothoracic surgery, an 8x6 cmmass was excised (Fig. 1, Panel C), with clear tumour margins. Histopathology demonstrated a myxoid matrix sparsely patient’s phenotype and family history is consistent with the NAME variant (Nevi, Atrial Myxoma, Ephelides) of the syndrome. Atrial myxomata often arise from the fossa ovalis of the inter-atrial septum, and are either found incidentally during echocardiography, or following complications related to tumour embolisation or mass effect. Recurrences are not uncommon and follow up with serial echocardiography is recommended.