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Cardiac Attr - Crossref

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Last Updated: 09 August 2022

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Disease progression in cardiac morphology and function in heart failure: ATTR cardiac amyloidosis versus hypertensive left ventricular hypertrophy

Methods Fifty-one patients with ATTR-CA were compared to 20 patients with heart failure but no amyloidosis, with septal thickness >= 14 mm. Both positive DPD diagnosis for ATTR-CA were predicted positive DPD diagnosis for ATTR-CA during the follow-up period, the interventricular septal thickness, and relative wall thickness in ATTR-CA increased from 16 to 18 mm, as shown by p 0. 51 to 0. 62 respectively, p 0. 55 and RELAPS > 2. 0. The overtime rise in RWT and IVST in ATTR-CA patients was greater than that seen in patients with heart failure, but no cardiac amyloidosis was detected.

Source link: https://doi.org/10.1007/s00380-022-02048-5


Does [99mTc]-3,3-diphosphono-1,2-propanodicarboxylic acid (DPD) soft tissue uptake allow the identification of patients with the diagnosis of cardiac transthyretin-related (ATTR) amyloidosis with higher risk for polyneuropathy?

Results The Perugini score was also elevated in patients with cATTR and extra diagnosis of PNP compared to patients without cATTR and additional diagnosis of PNP. According to quantitative SPECT/CT, DPD uptake in the subcutaneous fat of the left axillar region of cATTR patients was substantial in cATTR patients, relative to patients without cATTR. Conclusion DPD bone scintigraphy, according to this review, is a useful tool for determining patients with cATTR and a risk of PNP due to rising DPD soft tissue uptake.

Source link: https://doi.org/10.1007/s12350-022-02986-7


Pendekatan Klinis Pasien Transthyretin Cardiac Amyloidosis (ATTR-CA)

Transthyretin's besar diproduksi di hati, berbentuk tetramer dan berfungsi sebagai protein pembawa serta holoretinol-binding protein, but not the tinol-binding protein. Diagnosis akurat sedini mungkin sangat berpengaruh terhadap keberhasilan serta penurunan mortalitas dan mortalitas. Transthyretin Protein fragments in myocardium interstitial tissue caused by misfolded protein may result in thickening and stiffness cardiac muscles, causing restrictive and infiltrative cardiomyopathy. Transthyretin is mainly manufactured in liver as tetramer and bonded, as carrier protein, and with thyroxine and holoretinol binding protein as carrier protein. Early targeted diagnosis has a major effect on therapy administration and lowering patient morbidity and mortality in patients.

Source link: https://doi.org/10.55175/cdk.v48i9.1483


Structural polymorphism of amyloid fibrils in cardiac ATTR amyloidosis revealed by cryo-electron microscopy

Abstract amyloidosis is a fatal disease linked to the accumulation of transthyretin fibrils in organ failure and death. Although ATTR amyloidosis patients have ATTR fibrils in virtually every organ, the clinical appearance is often unpredictable and variable. All the patients of the same disease, tauopathies and synucleinopathies, diseases involving amyloidosis of tau and u03b1-synuclein, as well as tau and u03b1-synuclein, have the same fibril fold, or polymorph, in tauopathies and synucleinopathies, amyloidosis of tau and u03b1-synucleinopathiescopy chronu03 nuclein tau and synae and inflammatory amynucleinopathies and involving amychonchon amy-syl phagadoclein amy, or enuclein sis amyr'sisis symuclein synucleinuclein-synuclein ectuclein sylotuclein ucleinuclein sylucleinuclein syluclein sytucleinuclein In this study, we use cryo-EM to see if fibrils from heart tissue of different patients with cardiac ATTR amyloidosis have a common fold. We found that ATTRv mutations alter the fibril structure, while ATTRwt fibrils have a common fold. The current paradigm of u201cone disease equals one fibril polymorph, u201d, and concerns that similar novel formulations occur in other amyloidoses appear.

Source link: https://doi.org/10.1101/2022.06.21.496949


Abstract 13409: A Substantial Proportion of Patients With Transthyretin Cardiac Amyloid (ATTR) Present With Impaired Left Ventricular Ejection Fraction: Implications for Diagnosis and Race Disparities

History: ATTR cardiomyopathy is traditionally described in patients with heart failure with preserved ejection percentages. Methods: A retrospective institutional amyloid registry review of 585 patients with wild-type or variant ATTR-CM from 2011-2020. Patients with HFrEF and HmrEF reported shorter survival rates than those with HFpEF and HmrEF variant TTR, as well as lower rates of CAD compared to Caucasian patients after adjusting for Age, sex, race, NT-proBNP, and eGFR. p 0. 001. AA patients in AA were primarily of the V122I version, with more likely to have HFrEF than Caucasian patients. Patients in AA WT were also more likely to present with HFrEF than Caucasian WT, p=0. 017. At the time of diagnosis, forty-five percent of patients treated with ATTR-CM had LVEF u226450%, indicating that medical doubt regarding ATTR-CM diagnosis may have lasted well beyond the HFpEF presentation.

Source link: https://doi.org/10.1161/circ.144.suppl_1.13409


Pressure-Volume Relationships in Patients With Transthyretin (ATTR) Cardiac Amyloidosis Secondary to V122I Mutations and Wild-Type Transthyretin

Background: A mutationated version of transthyretin or a wild-type variant of the ATTR cardiac amyloidosis can occur in a u2014 ATTR cardiac amyloidosis. Methods and Results: U2014 Twenty-nine patients 18 with ATTRwt and 11 with ATTRm had 2-dimensional echocardiograms with complete Doppler detection at baseline and every six months for up to two years. The isovolumic PV area, ending-systolic pressure, isovolumic PV region, and the area between them were estimated. At baseline and beyond, clinical, socioeconomic, and PV indices were compared between V122I and ATTRwt patients and those without survivors and nonsurvivors. Stroke volume decline was attributed to changes in ventricular-vascular coupling and a decrease in ventricular capacity, resulting in significant decline in ejection fractions over 18 months. In V122I patients compared to wild-type at baseline and declining over time, PVA was lower than in wild-type at baseline and decreased with time.

Source link: https://doi.org/10.1161/circheartfailure.109.910455


Atrial longitudinal strain in cardiac aTTR amyloidosis and occurrence of atrial fibrillation

Patients with cryptogenic stroke may have atrial fibrillation and ischaemic stroke by speckle tracking echocardiography peak atrial longitudinal strain can predict the incidence of atrial fibrillation and ischaemic stroke in the general population and patients with cryptogenic stroke. At first, it is PURPOSE to test parameters of atrial function in cardiac amyloidosis pts in sinus rhythm and AF to see if they are related to the occurrence of new onset AF at a follow-up. Right ventricular and left atrial strain analyses were performed during the clinic's 2D-speckle tracking analysis of left ventricular and right atrial strain, which was attributed to the Clinic's diagnosis of cardiac aTTR amyloidosis. The LA volume was not significantly different in AF pts when compared to SR pts. Compared to pts who did not, there was no significant difference in atrial size and atrial deformation parameters between pts who had sudden onset AF at FUP and atrial deformation parameters that did not. GLS was the only measurement associated with new onset AF at FUP. CONCLUSIONS Our results show that all echo parameters of LA size and function were slightly affected in this group of pts with aTTR cardiac amyloidosis, as well as other pertinent physiological parameters. In this setting of advanced disease LA volume was not significantly different in pts in AF compared to pts in SR, and was not associated with new-onset AF at FUP, which was not related to new-onset AF. Pts in AF were significantly more affected than pts in AF compared to pts in SR, but PALS was not involved with the occurrence of new onset AF at follow-up.

Source link: https://doi.org/10.1093/ehjci/jeab289.052


Abstract 16030: Heterogeneity of Electrocardiographic Findings in Cardiac Transthyretin (ATTR) Amyloidosis and Impact on Survival

Cardiac transthyretin amyloidosis is a common cause of heart disease, according to the author. Amyloidosis of older Caucasian men is caused by a wild-type ATTR amyloidosis. The hereditary cardiac and neurologic abnormalities are the most commonly associated with the V122I transthyretin variant in patients of Irish ancestry, with the former being the latter presenting with a mixed cardiac and neural phenotype. Electrocardiographic findings in ATTR amyloidosis as a whole entity are included in most published studies. The prognostic factors for survival in cardiac ATTR amyloidosis are not well established. Methods and Results: In a longitudinal retrospective study, we analyzed ECGs from 158 patients with cardiac ATTR amyloidosis. There were no significant differences in other characteristics between ATTRwt patients and those with narrow QRS complexes. Conclusions: The ECG in cardiac ATTR amyloidosis is heterogeneous, and greater knowledge of it may help avoid diagnostic delays.

Source link: https://doi.org/10.1161/circ.130.suppl_2.16030

* Please keep in mind that all text is summarized by machine, we do not bear any responsibility, and you should always check original source before taking any actions

* Please keep in mind that all text is summarized by machine, we do not bear any responsibility, and you should always check original source before taking any actions