P874 A heart failure with brilliantly preserved ejection fraction
Authors: Hernandez Jimenez S , Alonso Salinas G , Rajjoub Al-Mahdi E , Sanchez Vega J D , Ramos Jimenez J , Plaza Martin M , Zamorano Gomez J L 
Published: January 2020

A 85 years old woman with shortness of breath and leg edema with proggresive intensity last three weeks is admited in the cardiology department. As significant comorbidities, the patient has arterial hypertension (although last months all the medication that she needed was discontinued due to tendency toward low blood pressure) and paroxysmal atrial fibrillation. As previous surgical background, the patient only refers a carpal tunnel syndrome surgery (in both sides). In the physical exam she has a blood pressure of 110/60 Hg mm, lung crackles in the lower third of both lungs and a systolic murmur in the aortic area with a very low second sound. After being admitted in the cardiology department an echocardiography was done, showing severe left ventricle hypertrophy. As seen in the image (picture A), the myocardium, showed a characteristic granular ang bright pattern. It also showed a moderate to severe aortic stenosis. Although she was in atrial fibrillation during the echocardiography, the transmitral Doppler showed signs of significantly elevated filling pressures as for example, the presence of L waves. Finally, the exam was completed with an analysis of longitudinal deformation of the left ventricle, showing a clearly depressed global longitudinal strain, with a base-apex gradient (preserved at the apex, picture D). With these findings a Tc-PYP scintigraphy was performed, showing moderate and difuse uptake of the radioactive tracer in both ventricles (as shown in the image C). To complete the diagnosis, a cardiac magnetic resonance was performed showing a subendocardial ring pattern of delayed enhancement (image B). The diagnosis of transthyretin cardiac amyloidosis was done.

Cardiac amyloidosis is clearly underdiagnosed entity, and sometimes differential diagnosis is really hard. Patients with cardiac amyloidosis are not always so "typical" as in this case. In fact, even a 5% of patients with history of aortic stenosis surgery may have also a cardiac amyloidosis. Maybe the use of myocardial deformation study and nuclear imaging can be useful solving this problem. Several studies have shown that nuclear imaging may be useful in the early diagnosis of this disease, even before that with echocardiography or cardiac magnetic resonance. Besides, new sequences of cardiac magnetic resonance may be very useful solving the problem of the differential diagnosis in left ventricle hypertrophy: The extracellular volume mapping. In cardiac amyloidosis hypertrophy is due to the deposit of amiloid in extracellular space. Because of that, the extracellular space is significantly increased in comparison with almost every other cause of left ventricle hypertrophy, making this sequence very useful in differential diagnosis.

CONCLUSION Transthyretin cardiac amyloidosis is a rare and very under-diagnosed disease. However these problems with the advance of cardiac imaging and the development of new treatments could change very soon.

Abstract P874 Figure. Image 1