Prognostic power of NT-proBNP in left ventricular non-compaction cardiomyopathy
Introduction
Left ventricular non-compaction cardiomyopathy (LVNC) is a distinct primary cardiomyopathy characterized by a thin, compacted, outer (epicardial) layer and a thick, non-compacted, inner (endocardial) layer with deep recesses between prominent trabeculations [1], [2], [3]. Since its first description, the awareness of LVNC has increased [4], [5]. With wider recognition of the disease and systematic family screening the number of asymptomatic patients diagnosed with LVNC is increasing. Symptomatic patients typically present with heart failure, ventricular arrhythmias or thromboembolic events [6], [7], [8]. However, overall event rates and predictors of outcome remain ill defined. Mortality rates in earlier studies range from 2% to 35%, over median follow-up periods ranging from 2.3 to 4.5 years [9], [10], [11], [12], [13]. These studies observed an association between presentation with symptoms, reduced left ventricular ejection fraction and the risk for adverse outcomes [7], [8], [10], [11], [12]. Nevertheless, quantitative data allowing a reliable assessment of a patient's risk is scarce.
In cardiomyopathies other than LVNC, parameters like left ventricular ejection fraction, heart failure symptoms, and exercise capacity correlate with clinical outcome. Due to its low prevalence, the value of such parameters in LVNC is not as well defined, and the prognostic relevance of the heart failure marker N-terminal fragment of prohormone brain natriuretic peptide (NT-proBNP) as well as that of exercise capacity have not been examined so far.
The aim of this study was to determine the prognostic value of NT-proBNP in LVNC patients per se and in comparison with left ventricular ejection fraction (LVEF), New York Heart Association (NYHA) functional class, and exercise capacity. In addition, clinically established cut-off values (LVEF 55% and LVEF 30%, specified standard values for NT-proBNP) as well as cut-off points providing the best prognostic discrimination in regard to death and heart transplantation were assessed.
Section snippets
Patients
All patients diagnosed with isolated LVNC between 1988 and 2015, identified from the clinical and imaging databases at the University Hospitals Zurich and Basel were included in this retrospective analysis. The study was approved by the local ethical committees of Zurich and Basel and informed consent was obtained. The echocardiographic criteria described by Jenni et al. were applied to establish the diagnosis [14]. These criteria include 1) a thickened, two-layered myocardium with a compacted
Patients
During 1013 person-years (longest follow-up 18.5 years) 23 patients (15%) died or underwent heart transplantation. An overview of the study population, patient groups, measurements, follow-up, and outcome is provided in Table 1.
Prohormone brain natriuretic peptide
The median NT-proBNP level in all non-event associated measurements (all values except event-preceding values) was 292 ng/l. Median NT-proBNP level at the last measurement preceding an event was 6416 ng/l (Fig. 1A).
Cox regression revealed a highly significant relationship
Discussion
The predictors of mortality remain ill-defined in patients with LVNC. This study determined the prognostic value of NT-proBNP in comparison with other markers of left ventricular function such as LVEF, NYHA functional class, and exercise capacity in the largest LVNC cohort published to date, with 153 patients and a median follow-up duration of > 6 years. The overall mortality and heart transplantation rate in our cohort was 15%, which is in the range of previous studies reporting rates between 2%
Funding sources
No specific funding.
Disclosures
None.
Conflicts of interest
There are no potential conflicts of interest, including related consultancies, shareholdings, and funding grants.
Acknowledgments
None.
References (32)
- et al.
Reduced left ventricular compacta thickness: a novel echocardiographic criterion for non-compaction cardiomyopathy
J. Am. Soc. Echocardiogr.
(2012) - et al.
Identification of a rare congenital anomaly of the myocardium by two-dimensional echocardiography: persistence of isolated myocardial sinusoids
Am. J. Cardiol.
(1984) - et al.
Long-term follow-up of 34 adults with isolated left ventricular noncompaction: a distinct cardiomyopathy with poor prognosis
J. Am. Coll. Cardiol.
(2000) - et al.
Isolated left ventricular noncompaction syndrome
Am. J. Cardiol.
(2009) - et al.
Predictors of adverse outcome in adolescents and adults with isolated left ventricular noncompaction
Am. J. Cardiol.
(2012) - et al.
Neuromuscular and cardiac comorbidity determines survival in 140 patients with left ventricular hypertrabeculation/noncompaction
Int. J. Cardiol.
(2011) - et al.
Isolated noncompaction of the myocardium in adults
Mayo Clin. Proc.
(1997) - et al.
Aminoterminal pro type B natriuretic peptide as a predictive and prognostic marker in patients with chronic heart failure
J. Heart Lung Transplant.
(2004) - et al.
N-terminal pro B-type natriuretic peptide predicts mortality in patients with left ventricular hypertrophy
Int. J. Cardiol.
(2010) - et al.
Plasma brain natriuretic peptide levels increase in proportion to the extent of right ventricular dysfunction in pulmonary hypertension
J. Am. Coll. Cardiol.
(1998)
Analysis of left ventricular volumes and function: a multicenter comparison of cardiac magnetic resonance imaging, cine ventriculography, and unenhanced and contrast-enhanced two-dimensional and three-dimensional echocardiography
J. Am. Soc. Echocardiogr.
Peak exercise oxygen consumption in chronic heart failure: toward efficient use in the individual patient
J. Am. Coll. Cardiol.
Contemporary definitions and classification of the cardiomyopathies: an American Heart Association scientific statement from the council on clinical cardiology, heart failure and transplantation committee; quality of care and outcomes research and functional genomics and translational biology interdisciplinary working groups; and council on epidemiology and prevention
Circulation
Isolated ventricular non-compaction of the myocardium in adults
Heart
Left ventricular non-compaction revisited: a distinct phenotype with genetic heterogeneity?
Eur. Heart J.
Isolated noncompaction of left ventricular myocardium. A study of eight cases
Circulation
Cited by (23)
Long-Term Outcome of ICD Therapy in Patients With Noncompaction Cardiomyopathy Compared With DCM and HCM
2023, JACC: Clinical ElectrophysiologyHypertrabeculation; a phenotype with Heterogeneous etiology
2021, Progress in Cardiovascular DiseasesCitation Excerpt :In the pediatric population, mortality is reported higher and was mostly associated with ventricular arrhythmias and SCD, while the mortality in adults was primarily associated with systolic dysfunction.53,116 General predictors for mortality and heart transplantation are neonatal LVHT, increased age in adults, NMD, HF symptoms, decreased exercise capacity, and increased NT-proBNP.36,90,117–121 Similarly, prognostic factors on ECG are nonspecific.115,118,122
Left ventricular mechanics and cardiovascular outcomes in non-compaction phenotype
2021, International Journal of CardiologyCitation Excerpt :Such differences may depend on the depth of trabeculae or non-compacted myocardial thickness, since rotation is mainly controlled by myocardial fibers located in the sub-epicardial layer [8,29]. Consistent with this concept, thinning of the compacted layer was found to be a useful criterion for the LVNC phenotype [8,25–32]. In the current study, LV twist was significantly impaired in patients with LVNC when compared to control.
Right ventricle and outcome in left ventricular non-compaction cardiomyopathy
2020, Journal of CardiologyCitation Excerpt :However, mortality and morbidity vary amongst different reports, and parameters predicting outcome are not well characterized. It is known that LV ejection fraction (LVEF), N-terminal pro-hormone brain natriuretic peptide (NT-proBNP), and heart failure symptoms are useful predictors of outcome [2,6]. Nevertheless, risk assessment in LVNC patients remains difficult and may have strong implications with respect to treatment options.
Can left ventricular entropy by cardiac magnetic resonance late gadolinium enhancement be a prognostic predictor in patients with left ventricular non-compaction?
2023, Diagnostic and Interventional Radiology
- 1
These authors contributed equally to this work.
- 2
This author takes responsibility for all aspects of the reliability and freedom from bias of the data presented and their discussed interpretation.