What cardiologists should know about essential thrombocythemia and acute myocardial infarction: report of two cases and advanced heart failure therapies considerations

Abstract

We present the cases of two young male patients aged 22 and 31 without prior medical history nor cardiovascular risk factors, who presented to the hospital with large anterior ST-elevation myocardial infarction (STEMI). Urgent coronary angiography revealed acute thrombotic occlusion of the proximal left anterior descending artery in both patients. Persistent thrombocytosis was noted and subsequent investigations led to the diagnosis of essential thrombocythemia (ET) with positive JAK2-V617F mutation. Myocardial infarction as a first clinical manifestation of ET is rare but must be considered in patients without cardiovascular risk factors who show persistent thrombocytosis. In young patients without risk factors, there may be great delays before the diagnosis of STEMI is made. Longer time to revascularization of extensive STEMI is associated with adverse outcomes and cardiogenic shock which can lead to advanced therapies like heart transplant and left ventricular assist device (LVAD). Considering the favorable long-term prognosis of patients with ET, advanced therapies may be a valuable option in the presence of severe left ventricular dysfunction.

Keywords: JAK2 mutation; heart transplant; left ventricular assist device; thrombocytosis.

Figure 1

ECG, ST waves elevation in anterolateral leads, pathologic Q waves in lateral leads, ventricular ectopic beats.

Figure 2

(A) RAO 20-caudal 20 view left coronary angiography: proximal LAD thrombus and thrombolysis in myocardial infarction (TIMI) flow grade 0. (B) RAO 10-cranial 40 view: a 4.0×22 mm drug-eluting stent was placed in the proximal LAD to restore normal TIMI flow grade 3. Right coronary angiography (not shown) was normal.

Figure 3

ECG, sinus tachycardia with ST waves elevation and pathologic Q waves in the anterolateral leads.

Figure 4

(A) LAO 45-cranial 25 view left coronary angiography: complete proximal LAD occlusion. (B) Left ventriculography revealing severe left ventricular systolic dysfunction and antero-apical akinesia. Right coronary angiography (not shown) was normal.