scholarly journals Cardiac Amyloidosis Wild Type (ATTR-CAwt) and Associated Cardiac Arrhythmias: A Case Report and Literature Review

2021 ◽  
pp. 263246362199858
Author(s):  
Rangadham Nagarakanti ◽  
Kesavan Sankaramangalam ◽  
Sindhu Nagarakanti ◽  
Danyaal Moin ◽  
Kenneth Dulnuan ◽  
...  

Cardiac amyloidosis wild type (ATTR-CAwt) is often the underdiagnosed cause of heart failure with preserved ejection fraction (HFpEF) and has a high mortality rate. There is usually a long delay between the appearance of clinical signs and the diagnosis of ATTR-CAwt but a short duration between diagnosis and death. ATTR-CAwt was associated with significant clinical arrhythmias. We report a case of ATTR-CAwt, the process of its diagnosis, and the associated clinical arrhythmias and their management. We also reviewed the literature of this underdiagnosed and potentially fatal condition and the current existing therapies.

2019 ◽  
Vol 25 (5) ◽  
pp. 703-711 ◽  
Author(s):  
Massimo Leggio ◽  
Augusto Fusco ◽  
Claudia Loreti ◽  
Giorgio Limongelli ◽  
Maria Grazia Bendini ◽  
...  

2019 ◽  
Vol 25 (8) ◽  
pp. S53
Author(s):  
Virginia S. Hahn ◽  
Wendy Ying ◽  
Yi Zhen Joan Lee ◽  
Lisa R. Yanek ◽  
Dhananjay Vaidya ◽  
...  

2020 ◽  
Vol 75 (11) ◽  
pp. 691
Author(s):  
Omar Farid Abou-Ezzeddine ◽  
Daniel Davies ◽  
Jasmine A. Sexton ◽  
Christopher G. Scott ◽  
Ahmed Fayyaz ◽  
...  

Heart ◽  
2017 ◽  
Vol 104 (6) ◽  
pp. 525-532 ◽  
Author(s):  
Ki Hong Choi ◽  
Ga Yeon Lee ◽  
Jin-Oh Choi ◽  
Eun-Seok Jeon ◽  
Hae-Young Lee ◽  
...  

ObjectiveThere are conflicting results among previous studies regarding the prognosis of heart failure with preserved ejection fraction (HFpEF) compared with heart failure with reduced ejection fraction (HFrEF). This study aimed to compare the outcomes of patients with de novo acute heart failure (AHF) or acute decompensated HF (ADHF) according to HFpEF (EF≥50%), or HFrEF (EF<40%) and to define the prognosis of patients with HF with mid-range EF (HFmrEF, 40≤EF<50%).MethodsBetween March 2011 and February 2014, 5625 consecutive patients with AHF were recruited from 10 university hospitals. A total of 5414 (96.2%) patients with EF data were enrolled, which consisted of 2867 (53.0%) patients with de novo and 2547 (47.0%) with ADHF. Each of the enrolled group was stratified by EF.ResultsIn de novo, all-cause death rates were not significantly different between HFpEF and HFrEF (HFpEF vs HFrEF, 206/744 (27.7%) vs 438/1631 (26.9%), HRadj 1.15, 95% CI 0.96 to 1.38, p=0.14). However, among patients with ADHF, HFrEF had a significantly higher mortality rate compared with HFpEF (HFpEF vs HFrEF, 245/613 (40.0%) vs 694/1551 (44.7%), HRadj 1.25, 95% CI 1.06 to 1.47, p=0.007). Also, in ADHF, HFmrEF was associated with a significantly lower mortality rate within 1 year compared with HFrEF (HFmrEF vs HFrEF, 88/383 (23.0%) vs 430/1551 (27.7%), HRadj 1.31, 95% CI 1.03 to 1.65, p=0.03), but a significantly higher mortality rate after 1 year compared with HFpEF (HFmrEF vs HFpEF, 83/295 (28.1%) vs 101/469 (21.5%), HRadj 0.70, 95% CI 0.52 to 0.96, p=0.02).ConclusionsHFpEF may indicate a better prognosis compared with HFrEF in ADHF, but not in de novo AHF. For patients with ADHF, the prognosis associated with HFmrEF was similar to that of HFpEF within the first year following hospitalisation and similar to HFrEF 1  year after hospitalisation.


Author(s):  
Rajeev Malhotra ◽  
Christopher J. Nicholson ◽  
Dongyu Wang ◽  
Vijeta Bhambhani ◽  
Samantha Paniagua ◽  
...  

Objective: Arterial stiffness is a risk factor for cardiovascular disease, including heart failure with preserved ejection fraction. MGP (matrix Gla protein) is implicated in vascular calcification in animal models, and circulating levels of the uncarboxylated, inactive form of MGP (ucMGP) are associated with cardiovascular disease-related and all-cause mortality in human studies. However, the role of MGP in arterial stiffness is uncertain. Approach and Results: We examined the association of ucMGP levels with vascular calcification, arterial stiffness including carotid-femoral pulse wave velocity (PWV), and incident heart failure in community-dwelling adults from the Framingham Heart Study. To further investigate the link between MGP and arterial stiffness, we compared aortic PWV in age- and sex-matched young (4-month-old) and aged (10-month-old) wild-type and Mgp +/− mice. Among 7066 adults, we observed significant associations between higher levels of ucMGP and measures of arterial stiffness, including higher PWV and pulse pressure. Longitudinal analyses demonstrated an association between higher ucMGP levels and future increases in systolic blood pressure and incident heart failure with preserved ejection fraction. Aortic PWV was increased in older, but not young, female Mgp +/− mice compared with wild-type mice, and this augmentation in PWV was associated with increased aortic elastin fiber fragmentation and collagen accumulation. Conclusions: This translational study demonstrates an association between ucMGP levels and arterial stiffness and future heart failure with preserved ejection fraction in a large observational study, findings that are substantiated by experimental studies showing that mice with Mgp heterozygosity develop arterial stiffness. Taken together, these complementary study designs suggest a potential role of therapeutically targeting MGP in heart failure with preserved ejection fraction.


2020 ◽  
Vol 71 (1) ◽  
pp. 203-219 ◽  
Author(s):  
Jonah Rubin ◽  
Mathew S. Maurer

Cardiac amyloidosis (CA) is an infiltrative and restrictive cardiomyopathy that leads to heart failure, reduced quality of life, and death. The disease has two main subtypes, transthyretin cardiac amyloidosis (ATTR-CA) and immunoglobulin light chain cardiac amyloidosis (AL-CA), characterized by the nature of the infiltrating protein. ATTR-CA is further subdivided into wild-type (ATTRwt-CA) and variant (ATTRv-CA) based on the presence or absence of a mutation in the transthyretin gene. CA is significantly underdiagnosed and increasingly recognized as a cause of heart failure with preserved ejection fraction. Advances in diagnosis that employ nuclear scintigraphy to diagnose ATTR-CA without a biopsy and the emergence of effective treatments, including transthyretin stabilizers and silencers, have changed the landscape of this field and render early and accurate diagnosis critical. This review summarizes the epidemiology, pathophysiology, diagnosis, prognosis, and management of CA with an emphasis on the significance of recent developments and suggested future directions.


Circulation ◽  
2018 ◽  
Vol 137 (suppl_1) ◽  
Author(s):  
Katherine E Kurgansky ◽  
David Gagnon ◽  
Kelly Cho ◽  
J M Gaziano ◽  
Jacob Joseph ◽  
...  

Introduction: Heart failure with preserved ejection fraction (HFpEF) affects about 5% of people 65 or older, with a higher prevalence in women. Previous studies suggest that women with HFpEF may live longer than men. Further understanding of mortality outcomes by gender could be useful in implementing gender-specific treatment strategies to improve outcomes in HFpEF patients. Hypothesis: We assessed the hypothesis that women have a lower rate of total mortality than males in a US Veteran HFpEF cohort. Methods: We used a validated algorithm to curate a HFpEF cohort using ICD9 codes, laboratory values, medications, and ejection fraction values from the national Veterans Affairs database. This algorithm had 88% sensitivity and 96% specificity. We examined crude and adjusted mortality rates by gender, beginning at the time of heart failure diagnosis with follow-up through 2016. The adjusted mortality rate was directly standardized to the population of veterans with heart failure (n= 626,179) according to distribution of age, race, cardiovascular disease (CVD), and chronic kidney disease (CKD). Crude and standardized rate ratios were calculated from the mortality rates. Results: Our HFpEF cohort (n= 74,937) included 72,267 men and 2,670 women. Mean age was 72.5 (11.2) in men and 69.1 (14.3) in women at the time of heart failure diagnosis. Males were 85.2% white, 33.7% had CVD, and 27.1% had CKD, whereas females were 82.5% white, 28.7% had CVD, and 20.5% had CKD. During a mean follow up of 4.8 (3.7) years, 52,703 deaths occurred in men and 1,614 deaths occurred in women.The crude mortality rate was significantly lower for females (109.7/1000 person-years) compared to males (153.5/1000 person-years). Corresponding crude incidence rate ratio (95% CI) for total mortality comparing females to males was 0.71 (0.69-0.74; p<.0001). However, after standardizing, there was no significant difference in total mortality rates between men (170.0/1000 person-years) and women (173.4/1000 person-years). The standardized mortality rate ratio was 1.02 (95% CI: 0.84-1.23; p=0.8397). Conclusions: In conclusion, our data do not show any difference in total mortality rate between men and women following the diagnosis of HFpEF.


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