* If you want to update the article please login/register
The findings of 5-year quartile analysis showed a significant increase in patients treated with ATTR-CA, which was associated with larger proportions of patients referred following advanced cardiac imaging techniques. Patients with milder disease in the 5-yearly quartiles decreased from 36-months before diagnosis in 2002 to 12-months between 2017 and 2021, and a greater number of patients developed with milder disease prior to diagnosis. Despite a substantial rise in the proportion of patients enrolled in clinical trials and prescribing disease changing therapy, the increased longevity remained robust even after accounting for clinical trials and disease modifying therapy. Moreover, the changes in ATTR-CA's phenotype at diagnosis are urgently need to be factored into clinical trial design, given that pre-determined end-points based on trials conducted in the past may not be appropriate, nor at least sufficiently robust, or of sufficient duration to determine novel agents' efficacy.
Source link: https://doi.org/10.1093/eurheartj/ehac544.1763
Methods Fifty-one patients with ATTR-CA were compared to 20 patients with heart disease but no amyloidosis, but not with septal thickness >= 14 mm. Pro DPD diagnosis for ATTR-CA increased from 16 to 18 mm, the interventricular septal thickness, and relative wall thickness in ATTR-CA, p 0. 51 to 0. 62 respectively, p 0. 45 and RELAPS > 2. 0 both predicted positive DPD diagnosis for ATTR-CA over the course. Conclusion In ATTR-CA patients, the overtime rise in RWT and IVST was higher than that seen in patients with heart failure but no cardiac amyloidosis.
Source link: https://doi.org/10.1007/s00380-022-02048-5
PNP-related PNP scores were significantly higher in patients with cATTR and additional diagnosis of PNP relative to those without. DPD uptake in the subcutaneous fat of the left axillar region was significant in cATTR patients relative to those without, according to quantitative SPECT/CT. Conclusions DPD bone scintigraphy, which is a common method for identifying patients with cATTR and a risk of PNP, according to an increased DPD soft tissue uptake, according to this report.
Source link: https://doi.org/10.1007/s12350-022-02986-7
dani jantung dan kekakuan dinding jantung, transthyretin Cardiac is a fungence protein that hydrosolizes creatyretin di jaringan interstitial miokardium. Transthyretin's besi diproduksi di hati, berbentuk tetramer, dan berfungsi sebagai protein pembawa tiroksin serta holoretinol binding protein. The accumulation of transthyretin protein fragments in myocardium interstitial tissue caused by misfolded protein may lead to thickening and rigidity cardiac muscle, causing restricted and infiltrative cardiomyopathy. Transthyretin is produced mostly in liver as a tetramer and bonded both with thyroxine and holoretinol binding protein as carrier protein.
Source link: https://doi.org/10.55175/cdk.v48i9.1483
BACKGROUND: ATTR cardiomyopathy is commonly described in patients with heart disease with preserved ejection fraction. Patients with HFtEF and HmrEF patients had shorter survival times than Caucasian patients after adjusting for Age, sex, race, NT-proBNP, and eGFR. p 0. 001. AA patients were mainly V122I variant and more likely to develop HFrEF than Caucasian patients, with HFrEF. p=0. 017 p. ; At baseline, AA WT patients were also more likely to present with HFrEF than Caucasian WT patients, despite being more likely to present with HFrEF than Caucasian WT. p=0. 001 indicates that the AA variants were more likely to appear as HFrEF than Caucasian variants. Forty-five percent of patients treated with ATTR-CM had LVEF >u226450% at the time of diagnosis, meaning that medical suspicion for ATTR-CM diagnosis should extend beyond the HFpEF report.
Source link: https://doi.org/10.1161/circ.144.suppl_1.13409
Origins u2014 ATTR cardiac amyloidosis may have arisen from a mutated version of transthyretin or a wild-type variant. Methods and Results: U2014 Twenty-nine subjects 18 with ATTRwt and 11 with ATTRm and 11 with ATTRm were given 2-dimensional echocardiograms with complete Doppler results at baseline and every 6 months for up to two years. The isovolumic PV area, end-systolic pressure, was estimated as a result of end-diastolic pressure. Stroke volume decline was attributed to changes in ventricular-vascular coupling and a decrease in ventricular capacity with significant decrease in ejection fractions of more than 18 months. Compared to the wild-type at baseline and decreased over time, PVA iso was lower in V122I subjects compared to the wild-type at baseline and declining over time. U2014 Conclusions: amyloidosis secondary to a V122I mutation and wild-type transthyretin are identified with changes in the ventricle's ability to perform duties and, ultimately, with reduced survival in these populations.
Source link: https://doi.org/10.1161/circheartfailure.109.910455
The prevalence of atrial fibrillation and ischaemic stroke in the general population and in patients with cryptogenic stroke can be predicted by speckle tracking echocardiography peak atrial longitudinal strain. At the follow-up, the aim of PURPOSE is to determine atrial function in cardiac amyloidosis pts in sinus rhythm and AF, as well as to see if they are connected to the occurrence of new onset AF. Between 2016-2021, all pts with diagnosis of cardiac aTTR amyloidosis referred to the Clinic for an echocardiographic study that included 2D-speckle tracking of left ventricular and left atrial strains, as well as right atrial strain. Compared to SR pts, the LA population was not significantly different in AF pts relative to SR pts. There was no significant difference in atrial size and atrial deformation parameters between pts with fresh onset AF at FUP compared to non-fts who did not. GLS was the only measurement associated with new onset AF at FUP. CONCLUSIONS Our results reveal that in this group of pts with aTTR cardiac amyloidosis, all echo parameters of LA size and function were overall impaired. In this setting of advanced disease pts in AF were not significantly different from pts in SR and pts in SR, and was not associated with new-onset AF at FUP. Pts in AF were significantly more affected than pts in AF than 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
Cardiac transthyretin amyloidosis is an increasingly common cause of heart disease. Amyloidosis of older Caucasian men is characterized by the wild-type ATTR amyloidosis. The most common cause of inherited cardiac and neurologic abnormalities in patients of Irish ancestry is the V122I transthyretin variant in patients of Australian or the T60A TTR variant, with the latter having a mixed cardiac and neurologic phenotype. Electrocardiographic results in ATTR amyloidosis are the subject of the most recent studies. Prognostic factors for survival in cardiac ATTR amyloidosis are uncertain. In a longitudinal retrospective study, we analyzed ECGs from 158 patients with cardiac ATTR amyloidosis. There were no significant differences in other characteristics among ATTRwt patients with narrower and those with broad QRS complexes. Conclusions: The ECG in cardiac ATTR amyloidosis is heterogeneous, and more knowledge of it may help avoid diagnostic delays.
Source link: https://doi.org/10.1161/circ.130.suppl_2.16030
Planar images 1 hour and 3 hours p. i. led to 25 positive and 38 negative patients, Perugini scores, and heart-to-contralateral count ratios in 25 positive and 38 negative patients, Perugini scores, and heart-to-contralateral count ratios in 25 positive and 38 negative patients, respectively. Results In phantom measurements, reliability of myocardial CRpeak at SBR 10:1 and reproducibility at 5:1 were comparable to both H-mode and L-mode. However, L-mode demonstrated greater fidelity in background counts and sphere CR peak across the field of view than H-mode. At both time points and visual scoring 3 hours, sensitivity/specificity in patients were u2265 95% for H/CL ratios. Conclusions Early DPD photos provided no additional value for visual evaluation or H/CL ratios, although visual scoring or H/CL ratios could have been used to determine early DPD scores.
Source link: https://doi.org/10.1007/s12350-021-02715-6
* 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