In:
European Heart Journal - Cardiovascular Imaging, Oxford University Press (OUP), Vol. 23, No. 11 ( 2022-10-20), p. 1492-1501
Abstract:
Phospholamban (PLN) p.Arg14del mutation carriers are at risk of developing malignant ventricular arrhythmias (VAs) and/or heart failure. Currently, left ventricular ejection fraction (LVEF) plays an important role in risk assessment for VA in these individuals. We aimed to study the incremental prognostic value of left ventricular mechanical dispersion (LVMD) by echocardiographic deformation imaging for prediction of sustained VA in PLN p.Arg14del mutation carriers. Methods and results We included 243 PLN p.Arg14del mutation carriers, which were classified into three groups according to the ‘45/45’ rule: (i) normal left ventricular (LV) function, defined as preserved LVEF ≥45% with normal LVMD ≤45 ms (n = 139), (ii) mechanical LV dysfunction, defined as preserved LVEF ≥45% with abnormal LVMD & gt;45 ms (n = 63), and (iii) overt LV dysfunction, defined as reduced LVEF & lt;45% (n = 41). During a median follow-up of 3.3 (interquartile range 1.8–6.0) years, sustained VA occurred in 35 individuals. The negative predictive value of having normal LV function at baseline was 99% [95% confidence interval (CI): 92–100%] for developing sustained VA. The positive predictive value of mechanical LV dysfunction was 20% (95% CI: 15–27%). Mechanical LV dysfunction was an independent predictor of sustained VA in multivariable analysis [hazard ratio adjusted for VA history: 20.48 (95% CI: 2.57–162.84)] . Conclusion LVMD has incremental prognostic value on top of LVEF in PLN p.Arg14del mutation carriers, particularly in those with preserved LVEF. The ‘45/45’ rule is a practical approach to echocardiographic risk stratification in this challenging group of patients. This approach may also have added value in other diseases where LVEF deterioration is a relative late marker of myocardial dysfunction.
Type of Medium:
Online Resource
ISSN:
2047-2404
,
2047-2412
DOI:
10.1093/ehjci/jeab178
Language:
English
Publisher:
Oxford University Press (OUP)
Publication Date:
2022
detail.hit.zdb_id:
2042482-6
detail.hit.zdb_id:
2647943-6
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