Pilot study for left ventricular imaging phenotype of patients over 65 years old with heart failure and preserved ejection fraction: the high prevalence of amyloid cardiomyopathy

Abstract

This study sought to phenotype patients over 65 years old with heart failure and preserved ejection fraction (HFpEF) using clinical available comprehensive cardiovascular imaging modalities. Forty-nine patients with HFpEF and without coronary artery disease underwent clinical evaluation, electrocardiography, echocardiography, cardiac magnetic resonance (CMR) and Tc-3,3-diphosphono-1,2-propanodicarboxylic acid scintigraphy (Tc-DPD). The mean population age was 76 ± 8 years. Most of the patients (53 %) were NYHA class II. Mean NT-Pro-NBNP level was 1961 ± 2372 pg/ml. CMR exhibited a hypertrophic cardiomyopathy or infiltrative pattern in 3 (6 %) and 15 (31 %) patients, respectively. In the latter subgroup, Tc-DPD was suggestive of transthyretin-related cardiac amyloidosis for nine (18 %) patients, while AL amyloidosis was proven in five patients (10 %) by extracardiac (n = 3, 6 %) or endomyocardial (n = 2, 4 %) biopsies-one patient declined tissue biopsy. Compared to patients with unspecified cardiomyopathy (n = 31), patients with amyloid cardiomyopathy (n = 15 or n = 14/proven) had less hypertension, lower systolic blood pressure and higher NT-pro BNP level. Their electrocardiogram showed lowest QRS voltage and longer QRS duration. Left ventricular (LV) pattern was characterized by a more pronounced LV hypertrophy, a smaller ejection fraction and a decrease of global longitudinal strain associated with an increase of longitudinal strain apical-to-basal ratio. In patients over 65 years, HFpEF is a heterogeneous syndrome with at least a 29 % prevalence of amyloid cardiomyopathy. Combined CMR andTc-DPD are helpful imaging tools for accurate phenotyping of patients amenable to histopathological diagnosis or genetic testing, and should be considered for proper management of this population. Further longitudinal investigations are needed to better clarify these preliminary results.

Clinical practice update on heart failure 2019: pharmacotherapy, procedures, devices and patient management. An expert consensus meeting report of the Heart Failure Association of the European Society of Cardiology

“…Transthyretin amyloidosis has two forms: ATTRm and ATTRwt, which occurs sporadically. ATTR affects 20–30% of people aged >80 years and is more common in patients with HFpEF and/or degenerative aortic stenosis . Novel single photon emission computed tomography cardiac imaging with bone‐avid tracers (99mTc pyrophosphate, 3,3‐diphosphono1,2‐propanedicarboxylic acid, and hydroxymethylene diphosphonate (HMDP) help identify cases with high specificity, non‐invasively, obviating the need for endomyocardial biopsy.…”

Storage diseases with hypertrophic cardiomyopathy phenotype

“…Recent studies also described a prevalence of 16% of patients with severe aortic stenosis undergoing transaortic valve replacement, condition which was associated with the low-flow low-gradient phenotype and mild reduced ejection fraction 4 . A French pilot study estimated that the prevalence of ATTRwt could be as high as about one third of patients with heart failure and preserved ejection fraction 5 . In spite of the recent increasing data suggesting the high prevalence of senile cardiac amyloidosis, this fact is not new, as it had been already reported by Pomerance in 1965, concluding that “ senile cardiac amyloid would appear to be the commonest existing form of amyloid, and likely to continue increasing with the increasing age of the population ” 6 .…”

Patterns of CMR measured longitudinal strain and its association with late gadolinium enhancement in patients with cardiac amyloidosis and its mimics

“…Cardiac involvement in amyloidosis is a major determinant of adverse outcomes [ 1 ]. In addition, the prevalence of cardiac amyloidosis in patients over the age of 65 years with clinical symptoms of heart failure with a preserved ejection fraction has been demonstrated to be as high as 29% in a recent study [ 2 ]. Clinical history, electrocardiograms, echocardiography, nuclear imaging, and cardiovascular magnetic resonance (CMR) play a complementary role in the diagnosis of cardiac amyloidosis (CA).…”