Cardiovascular magnetic resonance myocardial T1 mapping to detect and quantify cardiac involvement in familial amyloid polyneuropathy.

OBJECTIVES

This study sought to explore the potential role of non-contrast T1 mapping for the detection and quantification of cardiac involvement in familial amyloid polyneuropathy (FAP).

METHODS

Japanese patients with FAP [n = 41, age 53.2 ± 13.9 years, genotype Val30Met (n = 25), non-Val30Met (n = 16)] underwent cardiac magnetic resonance imaging that included T1 mapping (saturation-recovery method) and late gadolinium-enhanced (LGE) imaging on a 3.0-T MR scanner. Their native T1 was measured on mid-ventricular short-axis images and compared with 30 controls.

RESULTS

Of the 41 FAP patients 29 were LGE positive. The native T1 was significantly higher in FAP patients than in the controls (1,634.1 ± 126.3 ms vs. 1,432.4 ± 69.0 ms, p < 0.01), significantly higher in LGE-positive- than LGE-negative FAP patients (1,687.1 ± 104.4 ms vs. 1,505.4 ± 68.5 ms, p < 0.01), and significantly higher in LGE-negative FAP patients than the controls (p < 0.01). A native T1 cutoff value of 1,610 ms yielded 85.4% accuracy for identifying LGE-positive FAP. The native T1 significantly correlated with the interventricular septum wall thickness, the left ventricular mass, the LGE volume, the plasma B-type natriuretic peptide level, and the E/e' ratio (all p < 0.01).

CONCLUSION

T1 mapping is of high diagnostic accuracy for the detection of LGE-positive FAP. The native myocardial T1 may be correlated with the severity of cardiac amyloid deposition.

KEY POINTS: • The native T1 was higher in FAP patients than the controls. • The native T1 was higher in LGE-positive- than LGE-negative FAP patients. • The native T1 was higher in LGE-negative FAP patients than the controls. • The native T1 correlated with clinical markers of systolic and diastolic dysfunction. • Myocardial T1 mapping is of high diagnostic accuracy for detecting LGE-positive FAP.