Diagnostic significance of paradoxical left ventricular hypertrophy in detecting cardiac amyloidosis

Figures

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  Fig. 1. Patient selection flow chart From the WMU-OMU registry co-managed by Wakayama Medical University Hospital and Osaka Metropolitan University Hospital, including patients who have undergone CMR to assess the aetiology of cardiac hypertrophy. Overall, 120 patients who presented with LV wall thickness ≥ 12 mm on CMR were selected for possible enrolment. Patients with prior MI (n = 2), those without ECG data at the time of CMR (n = 7), or without BSA data (n = 1) were excluded. Of the remaining 110 patients, 30 were definitively diagnosed with CA (CA group) and 80 with cardiac hypertrophy of non-CA aetiology (non-CA group). BSA: body surface area, CA: cardiac amyloidosis, CMR: cardiovascular magnetic resonance imaging, ECG: electrocardiography, LV: left ventricular, MI: myocardial infarction.

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  Fig. 2. Diagnostic significance of paradoxical LVH for detecting CA. The sensitivity, specificity, PPV, NPV and accuracy of paradoxical LVH for CA detection were 80%, 84%, 65%, 92% and 83%, respectively. CA: cardiac amyloidosis, LVH: left ventricular hypertrophy, NPV: negative predict value, PPV: positive predict value.

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  Fig. 3. Diagnostic algorithm of CA based on paradoxical LVH Paradoxical LVH has a high diagnostic value for the identification of CA in patients with cardiac hypertrophy (sensitivity: 80 %, specificity: 84 %, PPV: 65 %, NPV: 92 %, accuracy: 83 %, respectively). For the early diagnosis of CA and typing of the CA phenotype, identification of paradoxical LVH should be followed by cardiac scintigraphy with 99mTc-labeled bone-seeking tracers and measurement of monoclonal light chains. AL-CA: amyloid light chain cardiac amyloidosis, ATTR-CM: transthyretin amyloid cardiomyopathy, CA: cardiac amyloidosis, LVH: left ventricular hypertrophy, NPV: negative predict value, PPV: positive predict value, 99mTc: technetium-99 m.