P440Use of strain and natriuretic peptide for distinction of familial and systemic amyloidosis

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Background: Although previous work had demonstrated that strain and strain rate imaging could detect a difference in left ventricular function between familial amyloid polyneuropathy (FAP) and systemic amyloidosis (SA), little is known about the right ventricular mechanics in those patients. We hypothesized that sensitive tools of myocardial deformation using speckle tracking imaging might detect a difference in right ventricular longitudinal diastolic mechanics between FAP and SA.Methods: 15 consecutive patients (66±12 years) with biopsy-proven FAP involving the heart were compared with 15 age-matched SA patients, matched for LV wall thickness. RV mechanics (global longitudinal strain and strain rate) were analyzed by speckle tracking (EchoPAC, GE, Milwaukee, WI) in all patients.Results: Although 2 dimensional and Doppler echocardiographic parameters did not show difference between FAP and SA, B type natriuretic peptide was significantly lower in FAP than SA (FAP vs. SA; 448±427pg/ml vs. 1498±1284pg/ml, p<0.01). RV longitudinal strain tended to show better strain (-15.9±5.1% vs. -13.0±6.0%, p=0.09). RV early diastolic strain rate (1.26±0.63/s vs. 0.79±0.3/s, p=0.02) were significantly higher in FAP than that of SA (Table).Conclusions: RV longitudinal diastolic mechanics in patients with FAP is more preserved than SA, even when standard echocardiographic measurements fail to show a difference.

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