scholarly journals Echocardiographic phenotype and prognosis in transthyretin cardiac amyloidosis

2020 ◽  
Vol 41 (14) ◽  
pp. 1439-1447 ◽  
Author(s):  
Liza Chacko ◽  
Raffaele Martone ◽  
Francesco Bandera ◽  
Thirusha Lane ◽  
Ana Martinez-Naharro ◽  
...  

Abstract Aims Transthyretin amyloidosis cardiomyopathy (ATTR-CM) is an increasingly recognized cause of heart failure. We sought to characterize the structural and functional echocardiographic phenotype across the spectrum of wild-type (wtATTR-CM) and hereditary (hATTR-CM) transthyretin cardiomyopathy and the echocardiographic features predicting prognosis. Methods and results We studied 1240 patients with ATTR-CM who underwent prospective protocolized evaluations comprising full echocardiographic assessment and survival between 2000 and 2019, comprising 766 with wtATTR-CM and 474 with hATTR-CM, of whom 314 had the V122I variant and 127 the T60A variant. At diagnosis, patients with V122I-hATTR-CM had the most severe degree of systolic and diastolic dysfunction across all echocardiographic parameters and patients with T60AhATTR-CM the least; patients with wtATTR-CM had intermediate features. Stroke volume index, right atrial area index, longitudinal strain, and E/e’ were all independently associated with mortality (P < 0.05 for all). Severe aortic stenosis (AS) was also independently associated with prognosis, conferring a significantly shorter survival (median survival 22 vs. 53 months, P = 0.001). Conclusion The three distinct genotypes present with varying degrees of severity. Echocardiography indicates a complex pathophysiology in which both systolic and diastolic function are independently associated with mortality. The presence of severe AS was independently associated with significantly reduced patient survival.

2021 ◽  
Vol 30 ◽  
pp. S205
Author(s):  
A. Snir ◽  
M. Ng ◽  
G. Strange ◽  
D. Playford ◽  
S. Stewart ◽  
...  

2021 ◽  
Author(s):  
Tohru Takaseya ◽  
Atsunobu Oryoji ◽  
Kazuyoshi Takagi ◽  
Tomofumi Fukuda ◽  
Koichi Arinaga ◽  
...  

AbstractAortic stenosis (AS) is the most common valve disorder in advanced age. Previous reports have shown that low-flow status of the left ventricle is an independent predictor of cardiovascular mortality after surgery. The Trifecta bioprosthesis has recently shown favorable hemodynamic performance. This study aimed to evaluate the effect of the Trifecta bioprosthesis, which has a large effective orifice area, in patients with low-flow severe AS who have a poor prognosis. We retrospectively evaluated 94 consecutive patients with severe AS who underwent aortic valve replacement (AVR). Patients were divided into two groups according to the stroke volume index (SVI): low-flow (LF) group (SVI < 35 ml/m2, n = 22) and normal-flow (NF) group (SVI ≥ 35 ml/m2, n = 72). Patients’ characteristics and early and mid-term results were compared between the two groups. There were no differences in patients’ characteristics, except for systolic blood pressure (LF:NF = 120:138 mmHg, p < 0.01) and the rate of atrial fibrillation between the groups. A preoperative echocardiogram showed that the pressure gradient was higher in the NF group than in the LF group, but aortic valve area was similar. The Trifecta bioprosthesis size was similar in both groups. The operative outcomes were not different between the groups. Severe patient–prosthesis mismatch (PPM) (< 0.65 cm2/m2) was not observed in either of the groups. There were no significant differences in mid-term results between the two groups. The favorable hemodynamic performance of the Trifecta bioprosthesis appears to have the similar outcomes in the LF and NF groups. AVR with the Trifecta bioprosthesis should be considered for avoidance of PPM, particularly in AS patients with LV dysfunction.


Author(s):  
Steele C Butcher ◽  
Federico Fortuni ◽  
Jose M Montero-Cabezas ◽  
Rachid Abou ◽  
Mohammed El Mahdiui ◽  
...  

Abstract Aims Right ventricular myocardial work (RVMW) is a novel method for non-invasive assessment of right ventricular (RV) function utilizing RV pressure–strain loops. This study aimed to explore the relationship between RVMW and invasive indices of right heart catheterization (RHC) in a cohort of patients with heart failure with reduced left ventricular ejection fraction (HFrEF), and to compare values of RVMW with those of a group of patients without cardiovascular disease. Methods and results Non-invasive analysis of RVMW was performed in 22 HFrEF patients [median age 63 (59–67) years] who underwent echocardiography and invasive RHC within 48 h. Conventional RV functional measurements, RV global constructive work (RVGCW), RV global work index (RVGWI), RV global wasted work (RVGWW), and RV global work efficiency (RVGWE) were analysed and compared with invasively measured stroke volume and stroke volume index. Non-invasive analysis of RVMW was also performed in 22 patients without cardiovascular disease to allow for comparison between groups. None of the conventional echocardiographic parameters of RV systolic function were significantly correlated with stroke volume or stroke volume index. In contrast, one of the novel indices derived non-invasively by pressure–strain loops, RVGCW, demonstrated a moderate correlation with invasively measured stroke volume and stroke volume index (r = 0.63, P = 0.002 and r = 0.59, P = 0.004, respectively). RVGWI, RVGCW, and RVGWE were significantly lower in patients with HFrEF compared to a healthy cohort, while values of RVGWW were significantly higher. Conclusion RVGCW is a novel parameter that provides an integrative analysis of RV systolic function and correlates more closely with invasively measured stroke volume and stroke volume index than other standard echocardiographic parameters.


2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
S Saeed ◽  
A Vamvakidou ◽  
H.Y Yakupoglu ◽  
R Senior ◽  
R.S Khattar

Abstract Introduction Severe aortic stenosis (AS), defined as aortic valve area (AVA) &lt;1.0 cm2, can be divided into 4 categories based on flow status and mean gradient. Stroke volume index &lt;35 ml/m2 has classically been used to define low flow, but recent data suggest that flow rate (FR) &lt;200ml/sec may be a more accurate and robust marker of low flow. Methods We prospectively collected demographic, echocardiographic, aortic valve intervention (AVI) and all-cause mortality data on 1562 patients with symptomatic severe AS from 2010 to 2017 with a mean follow up period of 35±22 months. Patients were divided into 4 flow-gradient sub-groups based on a FR threshold of 200ml/s and mean pressure gradient of 40mmHg. Comparative analyses were performed among the 4 groups using analysis of variance. Results The prevalence of normal flow high gradient (NFHG) severe AS was 30%, NF low gradient (NFLG) 21%, low flow HG (LFHG) 18% and LFLG 31% (Table). Across these 4 sub-groups, there was a graded reduction in LVEF and FR, and an increase in age and all–cause mortality. Conclusions Classification of aortic stenosis based on flow-gradient patterns, shows important differences in the demographic profile and clinical outcome among the 4 groups. Classical NFHG AS was associated with the highest rate of AVI and lowest all-cause mortality compared to the 3 discordant flow-gradient subtypes. The LFLG group had the lowest AVI rates and worst outcome. Funding Acknowledgement Type of funding source: None


Heart ◽  
2014 ◽  
Vol 101 (1) ◽  
pp. 23-29 ◽  
Author(s):  
Mackram F Eleid ◽  
Paul Sorajja ◽  
Hector I Michelena ◽  
Joseph F Malouf ◽  
Christopher G Scott ◽  
...  

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
M Fredholm ◽  
S E Ricksten ◽  
K Karason ◽  
S E Bartfay ◽  
G Dellgren ◽  
...  

Abstract Background and aim The occurrence of right ventricular failure (RVF) in patients with chronic left heart disease (LHD) has important therapeutic and prognostic consequences. Echocardiography (Echo) parameters describing the RV longitudinal function (tricuspid annular plane systolic excursion, TAPSE; peak systolic free wall longitudinal strain, RV-Str; tricuspid annulus peak systolic velocity, TAPSm) are today commonly used to define RV dysfunction. In the present study we hypothesised that these parameters are load dependent. Methods We retrospectively included 66 patients with LHD (age 52 ± 13 years, males 79%) that underwent right heart catheterization (RHC) and Echo within 48 hours. RHC was performed as part of diagnostic- or pre-transplant work-up. Fifty-six patients (85%) had left ventricular ejection fraction &lt; 40%. From RHC data the patients were divided into three groups: Patients with RV decompensation and increased right atrial pressure (RAP) ≥10 mmHg (iRAP, n = 21), with normal RAP but reduced stroke volume index (SVI &lt; 35 mL/m2) (rSVI, n = 21) and with normal RAP and normal SVI (nSVI, n = 24). Results Patients with iRAP had compared with rSVI/nSVI more advanced LHD with higher PAMP, PCWP and larger RV diastolic area (RVdA). TAPSE, RV-Str and FAC did not differ between iRAP and rSVI patients. The ratio RVdA/RV-Str was significantly higher in iRAP patients compared with rSVI and nSVI. The rSVI and nSVI patients did not differ regarding RAP response during supine exercise (P = 0.84). Conclusions Reduced RV longitudinal function in patients with chronic LHD and normal RAP can be due to left ventricular forward failure and not RV systolic dysfunction. iRAP (n = 21) rSVI (n = 21) nSVI (n = 24) Overall P-value iRAP vs rSVI iRAP vs nSVI rSVI vs nSVI RAP (mmHg) 13 ± 2 5 ± 3 4 ± 2 &lt;0.001 &lt;0.001 &lt;0.001 0.34 PAMP (mmHg) 33 ± 8 24 ± 8 22±] &lt;0.001 0.001 &lt;0.001 0.34 PCWP (mmHg) 22 ± 5 16 ± 8 11 ± 6 &lt;0.001 0.003 &lt;0.001 0.025 CI (l/min/m2) 2.2 ± 0.4 2.2 ± 0.4 2.8 ± 0.5 &lt;0.001 0.75 &lt;0.001 &lt;0.001 PVR (Wood unit) 2.6 ± 1.2 1.5 ± 1.6 1.9 ± 1.0 0.032 0.022 0.035 0.60 RVdA (cm2) 26 ± 7 21 ± 7 21 ± 5 0.012 0.007 0.017 0.63 TAPSE (mm) 12 ± 3 13 ± 3 18 ± 6 &lt;0.001 0.28 &lt;0.001 0.001 TAPSm (cm/s) 8 ± 2 8 ± 2 10 ± 4 0.06 - - - RV-str (%) -15 ± 5 -17 ± 5 -21 ± 6 0.002 0.11 0.001 0.028 FAC (%) 28 ± 9 29 ± 11 39 ± 11 &lt;0.001 0.72 0.001 0.003 RVdA/RV-str (cm2/%) 2.2 ± 1.3 1.3 ± 0.7 1.1 ± 0.6 &lt;0.001 0.013 0.001 0.27


2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
C Beladan ◽  
A Calin ◽  
A D Mateescu ◽  
M Rosca ◽  
R Enache ◽  
...  

Abstract Background Anemia is common in patients (pts) with severe aortic stenosis (AS). Untreated anemia and severe AS are individually associated with the development of heart failure, however data regarding the potential detrimental effect of anemia on left ventricular (LV) function and prognosis in pts with severe AS are controversial. Aim To investigate the impact of anemia on clinical status, echocardiographic parameters and prognosis in pts with severe AS and preserved LV ejection fraction (LVEF). Methods Consecutive patients with severe AS (aortic valve area [AVA] index ≤ 0.6 cm2/m2) and preserved LVEF (&gt;50%) referred to our echocardiography laboratory were prospectively screened. All patients underwent complete clinical examination and comprehensive echocardiography, including speckle tracking-derived measurements of LV and left atrial (LA) strain. Baseline clinical variables included NYHA class, cardiac risk factors, haemoglobin (Hb) level and glomerular filtration rates (GFR, by MDRD formula). The definition of anemia was based on gender-specific cut-off values, as recommended by the WHO (Hb &lt;13.0 g/dL for men, &lt;12.0 g/dL for women). Patients with more than mild aortic regurgitation or mitral valve disease, atrial fibrillation or cardiac pacemakers were excluded. Results The study population included 264 patients (pts) (66 ± 11 yrs, 147 men). Anemia was present in 64 pts (24%). Aortic valve replacement (AVR) was performed in 151 pts. Dividing the study population into 2 groups, according to the presence/absence of anemia, no significant differences were found between groups regarding: age (p = 0.09), body surface area (p = 0.6), LVEF (62 ± 7 vs 63 ± 6%, p = 0.2), LV Global Longitudinal Strain (-15.2 ± 4 vs -14.7 ± 3 %, p = 0.4), LV mass index (p = 0.9), mean aortic gradient (p = 0.2) and indexed AVA (0.40 ± 0.09 vs 0.39 ± 0.09 cm2/m2, p = 0.6), or presence of significant coronary artery disease (p = 0.9). Compared to pts with normal Hb level, in pts with anemia NYHA class (p = 0.03), brain natriuretic peptide values (p = 0.004), lateral E/e’(16.2 ± 6.9 vs 13.7 ± 6.3, p = 0.01) and average E/e" ratio (15.9 ± 5.9 vs 14.1 ± 5.3, p = 0.03), LA volume index (54.3 ± 16.9 vs 45.0 ± 12.1 ml/m2, p &lt; 0.001), and systolic pulmonary artery pressure (38 ± 13 vs 33 ± 8, p = 0.009) were all significantly higher. During a 3–years follow-up 47 pts died. Age, NYHA class, BNP serum level, baseline anemia, LA volume index and systolic pulmonary pressure were associated with all-cause mortality in the whole study group (p &lt; 0.03 for all). In the group of pts who underwent AVR, NYHA class was the only independent predictor of all-cause mortality. Conclusions In our study including pts with severe AS and preserved LVEF, patients with baseline anemia presented worse functional status and LV diastolic dysfunction and increased 3-year all-cause mortality compared to those with normal Hb levels. However, in pts who underwent surgical AVR, there was no impact of baseline anemia on 3-year survival.


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