P911High rate of cardiac amyloidosis in elderly patients with aortic stenosis: clinical presentation before and after aortic valve intervention

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
M Zikry Deitch ◽  
D Haberman ◽  
S Lifshitz ◽  
S Tshori ◽  
Y Fabricant ◽  
...  

Abstract Background Aortic Stenosis (AS) is the most common valvular heart disease in the Western world. Wild-type transthyretin amyloid (wtATTR) affects the heart, causing restrictive cardiomyopathy. Deposits can be found in up to 25% of individuals >85 years of age at autopsy. Recently several reports showed a relatively high prevalence of transthyretin cardiac amyloidosis (TTR-CA) in patients with AS. Objectives The aim of this study was to examine the clinical effects of TTR-CA in patients who have undergone aortic valve replacement therapy and evaluate the outcome of the intervention. Methods We recruited patients who underwent surgical (AVR) or percutaneous (TAVI) aortic valve intervention between 2011 and 2018. The patients underwent a Tc99m-PYP scan using SPECT technology which has been shown to be valid for the diagnosis of TTR-CA. We reviewed patient files before (time point 1) and after intervention (time point 2) and at 2 years (time point 3) follow up, and collected data on hospitalizations, laboratory, and echocardiography. Results The study included 86 patients, mean age 78±6 years, 55% women. Twenty-nine (33%) participants were diagnosed as positive (VAS 2 and 3) for transthyretin cardiac amyloidosis.There were no differences in baseline characteristics between patients with and without TTR-CA in cardiovascular risk factors and co-morbidities, laboratory parameters and nutritional status. There were no differences in baseline echocardiographic parameters including valve gradients and left ventricular hypertrophy. However, the patients with TTR CA had more advanced diastolic dysfunction compared to patients without TTR CA (P=0.03) and higher pulmonary artery pressure (44±14.75mmhg vs 30.5±11.38mmhg, p=0.06). Before the intervention, patients with transthyretin cardiac amyloidosis had 3.26 times more hospitalizations due to heart failure as compared to patients in the negative group (p=0.01). After the intervention, diastolic function remained more severely affected in the positive group at all follow-up points compared to the negative group (p=0.05). Similar observations were seen in the measurements of pulmonary arterial pressure (p=0.019 at time 2 and p=0.015 at time 3). Consistent with the echocardiographic findings, patients with transthyretin cardiac amyloidosis had 2.84 times more hospitalizations after intervention for heart failure than patients in the negative group (p=0.02). Conclusions Co-existence of transthyretin cardiac amyloidosis and aortic stenosis in the older population is associated with a more severe clinical presentation and with more advanced clinical and echocardiographic signs of heart failure. Improvement after valvular intervention might be limited in terms of symptoms and hospitalizations in this subgroup. Acknowledgement/Funding None

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
F Mantovani ◽  
M A Clavel ◽  
F Jayme ◽  
L Valli ◽  
R M De Mola ◽  
...  

Abstract Background Improved technology together with greater operator experience has led to refinement of balloon aortic valve valvuloplasty (BAV) in recent years. It may provide a palliative treatment option in high-risk patients, highly symptomatic, for whom no other invasive therapy is available. However, there has not been universal adoption of BAV as a standalone therapy. Methods A retrospective analysis of ten years of practice of BAV as palliative strategy in patient with symptomatic aortic stenosis between March 2008 and June 2018 was performed. Demographic, clinical, procedural, and follow-up data on all patients were collected. Results A total of 152 patients (95 women, 63%) with a mean age of 85±6 years underwent BAV. All patients had severe aortic stenosis, were considered not suitable to aortic valve replacement nor Trans-catheter aortic valve implantation (TAVI) for appreciable comorbidity (STS score 9±5) and had severe symptoms mainly of heart failure which required medical attention. A statistically significant decrease in trans-valvular gradient was observed (peak to peak gradient before BAV 52±22 mmHg, after BAV 29±16 mmHg, delta gradient 24±14 mmHg; p<0.0001). Only one patient, who undergone BAV because of cardiogenic shock, died during the procedure. Considering the high-risk population, intra-hospital mortality was low (7 patients died, 4%). Mortality at 1-year follow-up was 43% and survival free from new hospitalization for heart failure was 63% at 1-year follow-up and 53% at 2 years follow-up. 19 patients (13%) required repeated BAV during follow-up. Conclusion BAV as a palliative procedure in high-risk patients who are highly symptomatic, has a low operative mortality in our experience. BAV is associated with a significant reduction in aortic valve gradient and is valuable since half of the patients were alive without re-hospitalizations for heart failure at 2 years follow-up. Acknowledgement/Funding None


PLoS ONE ◽  
2021 ◽  
Vol 16 (8) ◽  
pp. e0255487
Author(s):  
Osnat Itzhaki Ben Zadok ◽  
Mordehay Vaturi ◽  
Iuliana Vaxman ◽  
Zaza Iakobishvili ◽  
Noa Rhurman-Shahar ◽  
...  

Aims To compare the baseline cardiovascular characteristics of immunoglobulin light-chain (AL) and amyloid transthyretin (ATTR) cardiac amyloidosis (CA) and to investigate patients’ contemporary cardiac outcomes. Methods Single-center analysis of clinical, laboratory, echocardiographic and cardiac magnetic resonance imaging (CMRi) characteristics of AL and ATTR-CA patients’ cohort (years 2013–2020). Results Included were 67 CA patients of whom 31 (46%) had AL-CA and 36 (54%) had ATTR-CA. Patients with ATTR-CA versus AL-CA were older (80 (IQR 70, 85) years versus 65 (IQR 60, 71) years, respectively, p<0.001) with male predominance (p = 0.038). Co-morbidities in ATTR-CA patients more frequently included diabetes mellitus (19% versus 3.0%, respectively, p = 0.060) and coronary artery disease (39% versus 10%, respectively, p = 0.010). By echocardiography, patients with ATTR-CA versus AL-CA had a trend to worse left ventricular (LV) ejection function (50 (IQR 40, 55)% versus 60 (IQR 45, 60)%, respectively, p = 0.051), yet comparable LV diastolic function. By CMRi, left atrial area (31 (IQR 27, 36)cm2 vs. 27 (IQR 23, 30)cm2, respectively, p = 0.015) and LV mass index (109 (IQR 96, 130)grams/m2 vs. 82 (IQR 72, 98)grams/m2, respectively, p = 0.011) were increased in patients with ATTR-CA versus AL-CA. Nevertheless, during follow-up (median 20 (IQR 10, 38) months), patients with AL-CA were more frequently admitted with heart failure exacerbations (HR 2.87 (95% CI 1.42, 5.81), p = 0.003) and demonstrated increased mortality (HR 2.51 (95%CI 1.19, 5.28), p = 0.015). Conclusion Despite the various similarities of AL-CA and ATTR-CA, these diseases have distinct baseline cardiovascular profiles and different heart failure course, thus merit tailored-cardiac management.


2019 ◽  
Vol 6 (10) ◽  
pp. 3786
Author(s):  
Hari Krishna Murthy P. ◽  
Abha Chandra

Background: The objective of the study was to evaluate the early outcomes and survival in patients with severe aortic stenosis associated with concentric left ventricular hypertrophy following aortic valve replacement.Methods: This is a prospective study done at SVIMS, Tirupati, from June 2014 to September 2015 evaluating out comes and survival in patients undergoing primary isolated aortic valve replacement (AVR) for severe aortic stenosis, severe aortic stenosis with mild aortic regurgitation and severe aortic stenosis with moderate aortic regurgitation.Results: A total of 40 cases 26 males and 14 females aged 18 to 60 years (mean age, 48.5±13.4 years) underwent elective AVR. Left ventricular end diastolic diameter (p=0.008) at 6 months, a statistically highly significant difference in left ventricular mass  preoperatively, at discharge, at 3rd and 6th month follow up. The difference in mean left ventricular mass index (LVMI) had declined from 244.425 to 141.100 at 6 months, showing a statistically highly significant difference in LVMI preop, at discharge, at 3rd month and at 6th month follow up.Conclusions: Patients with preoperative increase in LVMI, with large left atrial diameter carries a strong predictor of postoperative mortality for patients undergoing aortic valve surgery. We also conclude that there will be significant regression of LVMI following successful AVR. But, the decrease in LVMI is maximum during early three months and it is minimal though significant in the later course of follow up. 


2021 ◽  
Vol 2021 ◽  
pp. 1-7
Author(s):  
Fatme A. Charafeddine ◽  
Haytham Bou Houssein ◽  
Nadine B. Kibbi ◽  
Issam M. El-Rassi ◽  
Anas M. Tabbakh ◽  
...  

Background. Aortic valve stenosis accounts for 3–6% of congenital heart disease. Balloon aortic valvuloplasty (BAV) is the preferred therapeutic intervention in many centers. However, most of the reported data are from developed countries. Materials and Methods. We performed a retrospective single-center study involving consecutive eligible neonates and infants with congenital aortic stenosis admitted for percutaneous BAV between January 2005 and January 2016 to our tertiary center. We evaluated the short- and mid-term outcomes associated with the use of BAV as a treatment for congenital aortic stenosis (CAS) at a tertiary center in a developing country. Similarly, we compared these outcomes to those reported in developed countries. Results. During the study period, a total of thirty patients, newborns (n = 15) and infants/children (n = 15), underwent BAV. Left ventricular systolic dysfunction was present in 56% of the patients. Isolated AS was present in 19 patients (63%). Associated anomalies were present in 11 patients (37%): seven (21%) had coarctation of the aorta, two (6%) had restrictive ventricular septal defects, one had mild Ebstein anomaly, one had Shone’s syndrome, and one had cleft mitral valve. BAV was not associated with perioperative or immediate postoperative mortality. Immediately following the valvuloplasty, a more than mild aortic regurgitation was noted only in two patients (7%). A none-to-mild aortic regurgitation was noted in the remaining 93%. One patient died three months after the procedure. At a mean follow-up of 7 years, twenty patients (69%) had more than mild aortic regurgitation, and four patients (13%) required surgical intervention. Kaplan–Meier freedom from aortic valve reintervention was 97% at 1 year and 87% at 10 years of follow-up. Conclusion. Based on outcomes encountered at a tertiary center in a developing country, BAV is an effective and safe modality associated with low complication rates comparable to those reported in developed countries.


2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
K Papneja ◽  
Z Blatman ◽  
I D Kawpeng ◽  
J Wheatley ◽  
H Osce ◽  
...  

Abstract Introduction Aortic valve (AV) stenosis is the most common type of congenital left ventricular outflow tract obstruction. Short-term outcomes following balloon aortic valvuloplasty (BAV) including residual aortic stenosis, aortic insufficiency, and procedural complications have been established. The impact of pre-intervention AV characteristics on long-term outcomes has not been well studied. Purpose The aim of this study was to determine the relationship between the initial parameters on baseline echocardiogram and the time to reintervention in children with AV stenosis following BAV. Methods Children from the newborn period to 18 years of age with AV stenosis who underwent BAV from 2004-2012 were included. Patients with aortic insufficiency prior to BAV, complex congenital heart lesions, or less than two accessible follow-up echocardiograms were excluded. Baseline and serial echocardiographic data pertaining to aortic valve and LV size and function was retrospectively collected until December 2017 or until the first reintervention. Time to reintervention or death was evaluated. Results Among the 98 enrolled patients, the median [IQR] age at BAV was 2.8 months [0.2-75]. The median [IQR] duration of follow-up was 6.8 [1.9-9.0] years. Eighty-nine (83%) patients had bicuspid valve morphology and the median [IQR] peak-to-peak catheterization gradient prior to BAV was 49 [34-65] mmHg. The cumulative proportion [95% CI] of reintervention at 5 years following BAV was 33.7% [23.6%, 42.4%]. Primary indications for reintervention were aortic stenosis (57%), aortic insufficiency (14%), or mixed valve disease (30%). Reinterventions included repeat BAV (49%), AV repair (15%), and AV replacement (36%). Increased LVEF at baseline as well as increased mean LV circumferential strain at baseline were associated with decreased risk of reintervention (HR [95% CI] (1 unit increments): 0.974 [0.959-0.989], p &lt; 0.001; 0.939 [0.884-0.997], p = 0.041 respectively). Increased AV annulus z-score was also associated with decreased risk of reintervention (HR [95% CI] (1 unit increments): 0.806 [0.698-0.93], p = 0.003). Conclusions Our results demonstrate that better left ventricular function at baseline, measured by LVEF and mean LV circumferential strain, is associated with a decreased risk of reintervention in neonates and children following BAV. We have also shown that a bigger AV annulus prior to BAV is associated with a decreased risk of reintervention.


2020 ◽  
Vol 41 (20) ◽  
pp. 1903-1914 ◽  
Author(s):  
Miriam Puls ◽  
Bo Eric Beuthner ◽  
Rodi Topci ◽  
Anja Vogelgesang ◽  
Annalen Bleckmann ◽  
...  

Abstract Aims  Myocardial fibrosis (MF) might represent a key player in pathophysiology of heart failure in aortic stenosis (AS). We aimed to assess its impact on left ventricular (LV) remodelling, recovery, and mortality after transcatheter aortic valve implantation (TAVI) in different AS subtypes. Methods and results  One hundred patients with severe AS were prospectively characterized clinically and echocardiographically at baseline (BL), 6 months, 1 year, and 2 years following TAVI. Left ventricular biopsies were harvested after valve deployment. Myocardial fibrosis was assessed after Masson’s trichrome staining, and fibrotic area was calculated as percentage of total tissue area. Patients were stratified according to MF above (MF+) or below (MF−) median percentage MF (≥11% or &lt;11%). Myocardial fibrosis burden differed significantly between AS subtypes, with highest levels in low ejection fraction (EF), low-gradient AS and lowest levels in normal EF, high-gradient AS (29.5 ± 26.4% vs. 13.5 ± 16.1%, P = 0.003). In the entire cohort, MF+ was significantly associated with poorer LV function, higher extent of pathological LV remodelling, and more pronounced clinical heart failure at BL. After TAVI, MF+ was associated with a delay in normalization of LV geometry and function but not per se with absence of reverse remodelling and clinical improvement. However, 22 patients died during follow-up (mean, 11 months), and 14 deaths were classified as cardiovascular (CV) (n = 9 arrhythmia-associated). Importantly, 13 of 14 CV deaths occurred in MF+ patients (CV mortality 26.5% in MF+ vs. 2% in MF− patients, P = 0.0003). Multivariate analysis identified MF+ as independent predictor of CV mortality [hazard ratio (HR) 27.4 (2.0–369), P = 0.01]. Conclusion  Histological MF is associated with AS-related pathological LV remodelling and independently predicts CV mortality after TAVI.


Author(s):  
Sachin S. Goel ◽  
Neal S. Kleiman ◽  
William A. Zoghbi ◽  
Michael J. Reardon ◽  
Samir R. Kapadia

Abstract Aortic stenosis (AS) is a common valvular heart disease in the aging population that is characterized by a variable period of asymptomatic phase before development of symptoms and severe AS. Mortality and morbidity is substantial even after aortic valve replacement, in part related to persistent left ventricular hypertrophy, diastolic dysfunction, and heart failure. Renin‐angiotensin system (RAS) blockade therapy is associated with modulation of adverse left ventricular remodeling, reduction in myocardial hypertrophy, and fibrosis, resulting in clinical improvements in patients with congestive heart failure There are emerging data to suggest benefit of RAS blockade in patients with AS before and after AVR with regard to potentially slower progression of aortic valve calcification, left ventricular mass and survival benefit in favor of RAS blockade group before AVR, and also survival benefit in patients after AVR. We review the available data to understand the role of RAS blockade before AVR and in patients undergoing surgical AVR and transcatheter AVR. There are significant survival advantages of RAS inhibition in patients with AS undergoing surgical AVR or transcatheter AVR. On the basis of existing literature, adequately powered randomized trials are needed to evaluate the role of RAS inhibition in patients with AS.


2015 ◽  
Vol 42 (2) ◽  
pp. 117-123
Author(s):  
Giovanni Concistrè ◽  
Antonio Miceli ◽  
Federica Marchi ◽  
Francesca Chiaramonti ◽  
Mattia Glauber ◽  
...  

Left ventricular hypertrophy in aortic stenosis is considered a compensatory response for the maintenance of systolic function but a risk factor for cardiac morbidity and death. We investigated the degree of left ventricular mass regression after implantation of the sutureless Medtronic 3f Enable® Aortic Bioprosthesis. We studied 19 patients who, from May 2010 through July 2011, underwent isolated aortic valve replacement with the 3f Enable bioprosthetic valve, with clinical and echocardiographic follow-up at 6 months. The mean age was 77.1 ± 5.1 years (range, 68–86 yr); 14 patients were women (73.7%); and the mean logistic EuroSCORE was 15.4% ± 11.8%. Echocardiography was performed preoperatively, at discharge, and at 6 months' follow-up. The left ventricular mass was calculated by means of the Devereux formula and indexed to body surface area. The left ventricular mass index decreased from 146.1 ± 47.6 g/m2 at baseline to 118.1 ± 39.8 g/m2 at follow-up (P=0.003). The left ventricular ejection fraction did not change significantly. The mean transaortic gradient decreased from 57.3 ± 14.2 mmHg at baseline to 12.3 ± 4.6 mmHg at discharge and 12.2 ± 5.3 mmHg at follow-up (P &lt;0.001), and these decreases were accompanied by substantial clinical improvement. No moderate or severe paravalvular leakage was present at discharge or at follow-up. In isolated aortic stenosis, aortic valve replacement with the 3f Enable bioprosthesis results in significant regression of left ventricular mass at 6 months' follow-up. However, this regression needs to be verified by long-term echocardiographic follow-up.


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