[Cardiac Amyloidosis Registry in Santiago, Chile (RAMICAR): Three-Year Follow-Up Results]

Cardiac amyloidosis (CA) is characterized by extracellular protein deposition, generating ventricular hypertrophy, heart failure and arrhythmias. The main proteins involved are light chains (CA-AL) and transthyretin (CA-TTR).

To describe clinical, imaging and biochemical characteristics of patients with CA, their prognosis and access to specific therapies for each etiology.

Prospective multicenter registry of patients >50 years old with red flags for CA, such as left ventricular hypertrophy (septum >12 mm) associated to: restrictive filling; aortic stenosis; low voltage, pseudoinfarction pattern in electrocardiogram (ECG), heart failure with NT-proBNP >600 pg/ml, elevated troponins, carpal tunnel, polyneuropathy, compatible cardioresonance.

60 patients were included, 37 men, age 68±13 years; 36 AC-AL; 24 CA-TTR (10 patients CA-TTR mutated). The most frequent red flags were: strain pattern or global longitudinal deformation (97%), heart failure (95%), restrictive filling (72%), compatible cardioresonance (85%), pseudoinfarction in ECG (62%), elevated troponins (75%). CA-TTR patients were older, greater prevalence of atrial fibrillation and atrioventricular block; larger septal thickness (17.0±0.45 versus 14.7±2.5 mm) and left atrial volume (55±13 versus 42±8 ml/m2). CA-AL patients showed larger troponin elevation. There were no differences in systolic function, left ventricular deformation or NT-proBNP. There were no differences in mortality. CA-AL patients had greater access to specific therapies (87% versus 17%).

Patients with CA presented moderate/severe hypertrophy, restrictive filling, atrial dilatation and biomarker elevation, suggestive of late diagnosis of CA. Mortality between groups was similar, with greater access to specific therapies in CA-AL.