No abstract

A man in his late 60s presented with symptoms for a few months of itching and head ache after shower. Physical examination was unremarkable except for ruddy complexion and splenomegaly. Complete blood count showed haemoglobin of 18.1 g/dL and haematocrit of 56.6%. To rule out secondary causes of erythrocytosis, such as congenital heart disease with a right to left shunt, a transthoracic echocardiogram was performed, which showed normal left ventricular function with an apical area of dyskinesis and a large left ventricular apical thrombus measuring 3.0 cm×2.0 cm. Further laboratory investigations showed low erythropoietin level and Jak V617F mutation consistent with the diagnosis of polycythemia vera. He was treated with aspirin, enoxaparin, phlebotomy and hydroxyurea with no reported complications during the stay.

Considerable number of patients with DCAS have abnormal vascular load, abnormal LV function, and significant coexisting disorders. These could influence the total pathophysiologic burden on the heart and symptom expression. Thus, DCAS should not be considered just as valvular stenosis, but a syndrome of DCAS because of the diagnostic, prognostic, and therapeutic implications of various factors associated with it.