Early diagnosis of cardiac amyloidosis (CA) is crucial, because cardiac involvement harbingers grave prognosis, limits therapeutic options and treatment tolerability.
We present 4 cases of cardiac amyloidosis illustrating the echo signs that may lead to early suspicion and diagnosis.
Case 1- 50 years old man previously healthy, evaluated for nonspecific chest pain. Echo examination revealed good left ventricular (LV) systolic function, no evidence of LV hypertrophy, pseudo-normal pattern of the transmitral flow and a small pericardial effusion. The myocardium had "granular appearance". MRI showed characteristic kinetics of gadolinium and cardiac biopsy specimen was positive for congo-red.
Case 2- 60 old woman presented with angina pectoris. Troponin was constantly elevated. Echo revealed good LV systolic function, diastolic dysfunction grade 2 without LV hypertrophy. The myocardium had "granular appearance". Cardiac MRI was consistent with amyloidosis that was confirmed by subcutaneous fat biopsy.
Case 3- 62 years old man with hypertrophic cardiomyopathy was transferred from another hospital for CABG. Despite of echo findings typical of hypertrophic cardiomyopathy, the presence of valvular thickening, pericardial effusion and myocardial “granular appearance” raised the suspicion of amyloidosis. Cardiac MRI followed by bone marrow biopsy confirmed the diagnosis.
Case 4- 52 years old woman was admitted due to pulmonary edema with troponin elevation. Cardiac MRI was diagnostic for myocarditis. Echo demonstrated global mild LV dysfunction, diastolic dysfunction grade 2, mild LV hypertrophy and moderate pericardial effusion. These findings lead to the suspicion of cardiac amyloidosis. Bone marrow biopsy was positive for multiple myeloma.
We presented 4 cases of cardiac amyloidosis. Of notice that in 2 LVH was absent and in 2 of them the working diagnosis was completely different. A low threshold of suspicion is required even in the absence of LVH, since as the "classical" amyloid echo picture appears- the prognosis is devastating.
