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ORIGINAL ARTICLE
Year : 2021  |  Volume : 31  |  Issue : 3  |  Page : 157-164

Global left ventricular myocardial work efficiency in heart failure patients with cardiac amyloidosis: Pathophysiological implications and role in differential diagnosis


1 Division of Cardiology, Department of Cardiology, AORN Ospedali dei Colli – Monaldi Hospital; Inherited and Rare Cardiovascular Diseases Unit, Department of Translational Sciences, University of Campania “Luigi Vanvitelli”, Naples, Italy
2 Inherited and Rare Cardiovascular Diseases Unit, Department of Translational Sciences, University of Campania “Luigi Vanvitelli”, Naples, Italy
3 Department of Cardiac Surgery and Transplantation, Heart Transplantation Unit in Adults, AORN Ospedali dei Colli - Monaldi Hospital, Naples, Italy
4 Echocardiography Laboratory, Mediterranea Cardiocentro, Antonio Cardarelli Hospital, Naples, Italy
5 Department of Advanced Biomedical Sciences, University of Naples “Federico II”, Naples, Italy
6 Department of Neurosciences, Reproductive Sciences and Odontostomatology, University of Naples “Federico II”, Naples, Italy
7 Haematology Unit, Department of Clinical Medicine and Surgery, University of Naples “Federico II”, Naples, Italy
8 Department of Nuclear Medicine, AORN Ospedali dei Colli - Monaldi Hospital, Naples, Italy
9 Unit of Cardiac Rehabilitation, Antonio Cardarelli Hospital, Naples, Italy
10 Heart Failure Unit, Department of Cardiology, AORN Ospedali dei Colli - Monaldi Hospital, Naples, Italy
11 Division of Cardiology, Department of Cardiothoracic and Respiratory Sciences, University of Campania “Luigi Vanvitelli”, Naples, Italy
12 Department of Cardiology, University of Campania “Luigi Vanvitelli”, Naples, Italy
13 Division of Cardiology, Department of Cardiology, AORN Ospedali dei Colli – Monaldi Hospital, Naples, Italy
14 Inherited and Rare Cardiovascular Diseases Unit, Department of Translational Sciences, University of Campania “Luigi Vanvitelli”, Naples, Italy; Institute of Cardiovascular Sciences, University College of London, St. Bartholomew's Hospital, London, United Kingdom

Correspondence Address:
Giuseppe Palmiero
Department of Cardiology, AORN Ospedali dei Colli – Monaldi Hospital, Naples
Italy
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jcecho.jcecho_16_21

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Introduction: Cardiac amyloidosis (CA) is an infiltrative cardiomyopathy and a common cause of heart failure with preserved and mid-range ejection fraction (HFpEF and HFmrEF). Left ventricular (LV) systolic assessment is pivotal in differential diagnostic and prognostic stratification in CA. However, nondeformation and deformation-based parameters classically implied had many limitations. Myocardial work (MW) has been recently introduced for the evaluation of myocardial performance, in a load-independent fashion, in patients with cardiomyopathies. Aims: This study aimed to evaluate MW parameters in LV performance assessment in CA and their possible role in differential diagnosis between AL and ATTR forms, compared with other echocardiographic parameters, also exploring the possible association between MW parameters and blood biomarkers. Materials and Methods: The study population consisted of 25 patients with CA (10 with AL amyloidosis and 15 with wild-type ATTR [ATTRwt] form) and HFpEF or HFmrHF, enrolled between March 2018 and December 2019, undergoing a comprehensive clinical, biochemical, and imaging evaluation. Ten healthy individuals were studied as controls. ATTR patients had a noninvasive diagnosis of wtATTR-CA (positive 99mTc-hydroxy methylene-diphosphonate scintigraphy with a negative hematological screening), while AL patients underwent endomyocardial biopsy. All patients underwent standard transthoracic echocardiography. MW and related indices were estimated using a vendor-specific module. Results: Compared to the ATTRwt group, patients in the AL group showed a more pronounced myocardial performance impairment assessed by Global Word Efficiency (GWE: 83.5% ± 6.3% vs. 88.2% ± 3.6%; P = 0.026). In multiple linear regression analysis, cardiac troponin I (B = −0.55; P < 0.0001), global longitudinal strain (B =0.35; P < 0.008), and regional relative strain ratio (B = −0.30; P < 0.016) were significant predictors of GWE reduction in CA patients. At receiver operating characteristics curve analysis, among all other deformation-based and nondeformation-based echocardiographic parameters, GWE showed the highest area under the curve (AUC) (AUC 0.74; 95% CI: 0.55–0.96; P < 0.04). The optimal cutoff was determined by sensitivity/specificity analysis: a GWE < 86.5% identified patients with AL amyloidosis with a sensitivity and specificity, respectively, of 80.0% and 66.7%. Conclusions: The results of our pivotal study seem to highlight the importance of new deformation parameters to study myocardial performance in patients with CA, and to differentiate between AL CA and ATTR CA.


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