Heart failure & cardiomyopathiesDiagnostic or prognostic study

Pathology-Based Validation of Quantitative Single-photon Emission CT (SPECT)/Computed Tomography (CT) in Patients With Suspected Transthyretin Amyloid Cardiomyopathy

Circ Cardiovasc ImagingPublished

  • Pathology-based validation of quantitative SPECT/CT
  • consecutive patients
  • single centre (Mayo Clinic)

Authors' conclusion

In patients with suspected ATTR-CM undergoing 99m Tc-pyrophosphate SPECT/CT, the Mayo 3D Score demonstrated superior diagnostic performance compared with planar metrics and comparable to SPECT Perugini, while correlating with myocardial ATTR burden and deposition patterns on pathology. These findings support its integration as a quantitative adjunct within clinical diagnostic ATTR-CM workflows.

Study context

Population
Patients who underwent 99m Tc-pyrophosphate SPECT/CT for suspected ATTR-CM between June 2017 and January 2025 with archived myocardial tissue107 (47%) had ATTR-CM, of whom 9 had hereditary ATTR-CM
Participants
230 patients
Comparison
Mayo 3D Score against myocardial pathology, compared with planar Perugini, heart-to-contralateral lung ratio and SPECT Perugini

Performance

As reported in the abstract

Discrimination against pathology (area under the receiver operating characteristic curve)

Mayo 3D Score

0.92

AUC · CI 0.88–0.96

planar Perugini

0.87

AUC · CI 0.83–0.92

heart-to-contralateral lung ratio

0.86

AUC · CI 0.81–0.91

SPECT Perugini

0.90

AUC · CI 0.86–0.94

Mayo 3D Score vs planar Perugini: P = 0.006 · Mayo 3D Score vs heart-to-contralateral lung ratio: P = 0.004 · Mayo 3D Score vs SPECT Perugini: P = 0.12

Further findings

Mayo 3D Score cutoff ≥1.0 diagnostic performance
86.0% · sensitivity · specificity 92.7% · positive predictive value 91.1% · negative predictive value 88.4%

Mayo 3D Score vs pathology among ATTR-positive cases

Correlation with semiquantitative amyloid burden (Spearman ρ)
0.69
Higher in pericellular than nodular deposition patterns
P < 0.001
Not associated with vascular involvement
P = 0.42

Original publication

Pathology-Based Validation of Quantitative SPECT/CT in Patients With Suspected Transthyretin Amyloid Cardiomyopathy. Circ Cardiovasc Imaging. Published 23 July 2026. doi:10.1161/circimaging.126.019832

Summarised from the published abstract; every number is checked against it