Perugini score corelates with electrical and echographic structural abnormalities in transthyretine cardiac amyloïdosis

Transthyretine cardiac amyloidosis (TTR-CA) severity can be assessed by bone scintigraphy using HDP-99mTc and Perugini score, depending on intensity of myocardial uptake. Less is known on how Perugini score interplays with routine cardiac structural parameters. To evaluate correlation between Perugini score and myocardial mass and voltage abnormalities in patients with TTR-CA. Patients adressed to our center with confirmed TTR-CA underwent complete evaluation including electrocardiogram (ECG) and transthoracic echocardiography (TTE). Routine parameters were obtained such as: ECG voltage in millivolt (mV) according to Sokolow indice (SV 1 + RV 5 ) , PR delay (ms), QRS width (ms), left ventricular mass (LVM, grams) and diastolic interventricular septum (dIVS) thickness (mm). Patients were classified into three categories according to Perugini score: I (cardiac uptake < chest bones), II (cardiac uptake = chest bones) and III (cardiac uptake > bones). Main end-point were LVM and Voltage/Mass ratio ( VMR, μVolts/g ). Among 17 patients, 14 were male (82.4%) and mean age was 82 ± 8 years. Six patients (35.3%) had permanent atrial fibrillation (AF). Mean voltage, PR delay and QRS width were respectively 1.5 ± 0.5 mV, 212 ± 54 ms and 113 ± 19 ms. On TTE, mean LVM and dIVS width were respectively (188 ± 55 g/m 2 ) and 19 ± 4 mm. Mean voltage/mass ratio (VMR) was 4.8 ± 2.6 microVolts/g ( Table 1 ). On bone scintigraphy, 3 patients were scored Perugini I (18%), 4 Perugini II (24%) and 10 Perugini III (58%). According to Pearson test, there was a signicative correlation between Perrugini score and LVM ( r = +0.57, P = 0.03) (Fig 1A), and a strong negative correlation between Perrugini score and VMR ( r = –0.70, P = 0.003) (Fig 1B). In TTR-CA patients, Perugini score appears to strongly correlates with left ventricle mass and voltage. Larger scale studies are needed to confirm these results.