HCM AT THE CROSSROAD

Abstract

Abstract Introduction Treatment of oHCM frequently requires difficult choices, especially when it is superimposed on other urgent clinical conditions,such as AMI or mechanical complications. Clinical Case A 52–year–old man with known oHCM, who spontaneously quickly discontinued recommended therapy, presents to theED with a sudden episode of dyspnea and presyncope. A firstECG was essentially normal for pathology. After 1h, the patient experiences a sudden episode of chest pain with rapid hemodynamic deterioration and cardiogenic shock(CS). The ECG shows changes compatible with inferolateral MI. The TTE shows severe LVOTO, SAM, severe MR and a suspicious oval image attached to the anterior mitral leaflet. The patient underwent orotracheal intubation, coronary angiography and TEE. Coronary angiography shows a thrombotic occlusion of the proximal circumflex artery, treated with pPCI. TEE confirms a partial rupture of the papillary muscle head. Treatment:The patient‘s CS can be caused by 2different conditions that require treatments with opposing pathophysiological principles. SevereMR due to papillary muscle rupture, as mechanical complication of MI, requires afterload reduction, hemodynamic stabilization with IABP and emergency transfer to a cardiac surgery center. In contrast, the treatment of the underlying pathology(severe LVOTO and severe rMR due to SAM)requires pharmacological stabilization with depression of cardiac contractility and increase in afterload. In the context of cardiogenic shock a rapid choice was required. To help us make the right choice, an Esmolol test was performed. After iv administration of 2mg Esmolol, heart rate was reduced without reduction in BP. The patient was then treated with esmolol in continuous infusion and norepinephrine. In oHCM the ventriculo–arterial coupling is reduced, less than 1. This treatment favored better V–A coupling, ensuring an increase in afterload and a reduction in myocardial contractility. The patient‘s clinical condition rapidly improved, allowing extubation at48h and early mobilization at96h. Myectomy and mitral repair surgery was scheduled 1 month afterPCI. Conclusions Through the use of a short–lived drug such as Esmolol, it was possible to trace the prevailing mechanism of the patient‘s CS. Differently from other cases of CS, to rebalance the V–Acoupling, it was crucial to reduce myocardial contractility and increase afterload. A pathophysiological pharmacological approach ensured a good outcome.