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Comparison of various non-invasive tools for diagnosing AL cardiac amyloidosis.
2017

Congress : ESC Congress

  • Topic : valvular, myocardial, pericardial, pulmonary, congenital heart disease
  • Sub-topic : Myocardial Disease – Clinical
  • Session type : Moderated Posters
  • FP Number : P5842

Authors : M Nicol (Paris,FR), B Assous (Paris,FR), M Baudet (Paris,FR), A Cescau (Paris,FR), R Dautry (Paris,FR), A Cohen Solal (Paris,FR), B Arnulf (Paris,FR), D Logeart (Paris,FR)

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Authors:
M. Nicol1 , B. Assous1 , M. Baudet1 , A. Cescau1 , R. Dautry1 , A. Cohen Solal1 , B. Arnulf2 , D. Logeart1 , 1Hospital Lariboisiere - Paris - France , 2Hopital Saint-Louis - Paris - France ,

Citation:
European Heart Journal ( 2017 ) 38 ( Supplement ), 1237

Cardiac involvement is the most important cause of death in light chain amyloidosis (AL) and its early diagnosis is a major issue for therapeutic strategy. Gold diagnostic standards are either invasive (cardiac biopsy) or not widely available (cardiac MRI). We aim to compare diagnostic value of various diagnostic tools in this setting.

Methods: Following diagnostic tests were performed after first diagnosis of AL amyloidogenic disorder: clinical examination, blood testing of BNP and troponin I, EKG, echocardiography, 24-hours EKG Holter, cardiac MRI, cardiopulmonary test. Cut-offs were chosen from literature for parameters with continuous values. Final diagnosis of cardiac amyloidois (CA) was done either by MRI if diffuse late enhancement was present or by an expert consensus (3 clinicians) using all medical files. Diagnostic values of tests as well as their combination were calculated.

Results: Among sixty-four consecutive patients (65±10 years, 21 with multiple myeloma and 43 with MGUS), final diagnosis of CA was done in 42 patients. Renal, digestive and neurologic AL involvements were present in 43%, 21% and 19% respectively of patients with CA. The table shows diagnostic values of EKG, BNP and echography as well as their combinations. Usefulness of troponin, holter or stress test was less relevant.

Conclusion: Combining EKG, BNP testing and echocardiography result in nearly optimal diagnosis of CA.

Cardiac involvement is the most important cause of death in light chain amyloidosis (AL) and its early diagnosis is a major issue for therapeutic strategy. Gold diagnostic standards are either invasive (cardiac biopsy) or not widely available (cardiac MRI). We aim to compare diagnostic value of various diagnostic tools in this setting.

Table 1
SeSpPPVNPV
EKG abnormalities (microvoltage and/or pseudo Q wave)95%79%90%88%
Global longitudinal strain (GLS)92%60%85%75%
IVS ≥12 mm and GLS ≤ - 16%92%82%92%82%
EKG abnormalities and IVS ≥12 mm and GLS ≤ -16%86%100%76%100%
BNP ≥100 ng/L and IVS ≥12 mm and GLS ≤ -16%86%81%91%72%
BNP ≥100 ng/L and EKG abnormalities and IVS ≥12 mm and GLS ≤ -16%84%100%100%73%
BNP ≥100 ng/L and EKG abnormalities and GLS ≤ -16%84%100%100%73%
Se: sensibility; Sp: specificity; PPV: positive predictive value; NPV: negative predictive value; IVS: interventricular septum diastolic thickness.

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