Seven factors predict a delayed diagnosis of cardiac amyloidosis.

INTRODUCTION

Diagnostic delay of cardiac amyloidosis (CAm) continues to challenge clinicians. We investigated features associated with delay and ascertained if a diagnostic delay had negative implications for the patient.

METHODS

We performed a retrospective chart review identifying 82 subjects with biopsy-proven and mass-spectrometry-identified CAm with clinical and epidemiologic data including first potential symptom of amyloidosis. Pathology slides were scored for extent of amyloid. Robust statistical analyses including generalized linear and ordered logistic regression analysis were performed.

RESULTS

There was a 22 month (median) delay in diagnosis, more pronounced (34 months) in subjects with transthyretin (ATTR) amyloidosis. Seven factors predict a delayed diagnosis including ATTR amyloid type (ratio =2.17, 95% CI 1.31-3.59), having carpal tunnel syndrome (2.13, CI 1.49-3.03) and age <70 at first symptom (1.85, CI 1.30-2.61). Individuals with delays of 1+ years had higher levels of NT proBNP (4451 vs. 2559 pg/mL, p = .016) and longer PR intervals (225 vs. 162 ms, p < .001) at the time of diagnosis.

CONCLUSIONS

Diagnostic delays negatively affect cardiac function. Of the predictive clinical features, carpal tunnel syndrome was frequent and its presence should lead to a more aggressive analysis for CAm in the appropriate clinical settings.