BackgroundCardiac amyloidosis (CA) is a life-threatening restrictive cardiomyopathy. Identifying patients with a poor prognosis is essential to ensure appropriate care. The aim of this study was to ...compare myocardial work (MW) indices with standard echocardiographic parameters in predicting mortality among patients with CA.MethodsClinical, biological and transthoracic echocardiographic parameters were retrospectively compared among 118 patients with CA. Global work index (GWI) was calculated as the area of left ventricular pressure–strain loop. Global work efficiency (GWE) was defined as percentage ratio of constructive work to sum of constructive and wasted works. Sixty-one (52%) patients performed a cardiopulmonary exercise.ResultsGWI, GWE, global longitudinal strain (GLS), left ventricular ejection fraction (LVEF) and myocardial contraction fraction (MCF) were correlated with N-terminal prohormone brain natriuretic peptide (R=−0.518, R=−0.383, R=−0.553, R=−0.382 and R=−0.336, respectively; p<0.001). GWI and GLS were correlated with peak oxygen consumption (R=0.359 and R=0.313, respectively; p<0.05). Twenty-eight (24%) patients died during a median follow-up of 11 (4–19) months. The best cut-off values to predict all-cause mortality for GWI, GWE, GLS, LVEF and MCF were 937 mm Hg/%, 89%, 10%, 52% and 15%, respectively. The area under the receiver operator characteristic curve of GWE, GLS, GWI, LVEF and MCF were 0.689, 0.631, 0.626, 0.511 and 0.504, respectively.ConclusionIn CA population, MW indices are well correlated with known prognosis markers and are better than LVEF and MCF in predicting mortality. However, MW does not perform better than GLS.
Atrial arrhythmia (AA) is common among patients with cardiac amyloidosis (CA), who have an increased risk of intracardiac thrombus. The aim of this study was to explore the prognostic impact of ...vitamin K-antagonists (VKA) and direct oral anticoagulants (DOAC) in patients with CA.
273 patients with CA and history of AA with long term anticoagulation-69 (25%) light chain amyloidosis (AL), 179 (66%) wild-type transthyretin amyloidosis (ATTRwt) and 25 (9%) variant transthyretin amyloidosis (ATTRv)-were retrospectively included between January 2012 and July 2020. 147 (54%) and 126 (46%) patients received VKA and DOAC, respectively. Patient receiving VKA were more likely to have AL with renal dysfunction, higher NT-proBNP and troponin levels. Patients with ATTRwt were more likely to receive DOAC therapy. There were more bleeding complications among patients with VKA (20 versus 10%;
= 0.013) but no difference for stroke events (4 vs. 2%;
= 0.223), as compared to patients with DOAC. A total of 124 (45%) patients met the primary endpoint of all-cause mortality: 96 (65%) and 28 (22%) among patients with VKAs and DOACs, respectively (
< 0.001). After multivariate analysis including age and renal function, VKA was no longer associated with all-cause mortality.
Among patients with CA and history of AA receiving oral anticoagulant, DOACs appear to be at least as effective and safe as VKAs.
Background-Transthyretin cardiac amyloidosis (ATTR-CA) prevalence increases with age. The interplay between frailty and heart failure has been increasingly recognized. The objective of this study is ...to compare clinical, biological, and transthoracic echocardiography (TTE) characteristics of older ATTR-CA patients according to the G8 frailty screening tool. Methods-Patients over 75 years old with a confirmed diagnosis of ATTR-CA were included between January 2020 and April 2021. All patients underwent a routine blood test, TTE, and a functional assessment with a six-minute walking distance test (6MWD) or cardiopulmonary exercise testing (CPET), and the G8 score was calculated. Results-Fifty-two patients were included. Thirty-nine (75%) patients were frail and their mean NYHA stage was more severe (2.2 vs. 1.7;
= 0.004); 62% of them had a Gilmore stage of 2 or 3 (
= 0.05). Global left ventricular strain (GLS) was lower (-11.7% vs. -14.9%;
= 0.014) and the interventricular septum was thicker (18 ± 2 mm vs. 17 ± 2 mm;
= 0.033) in frail patients. There were no significant differences according to functional tests. Conclusion-The majority of older patients with ATTR-CA are frail according to the G8 score. They are more symptomatic and have an increased cardiac involvement and a poorer prognosis, requiring more personalized cardiac management.
Background
Right ventricular (RV) systolic parameters are difficult to assess in heart transplant recipients (HTRs) compared to healthy people because of discordant data, and their impact on exercise ...capacity remains undefined. We sought to retrospectively assess the impact of RV systolic function on exercise capacity after heart transplantation.
Methods
We analyzed data from 61 HTRs who underwent transthoracic echocardiography (TTE), cardiac magnetic resonance imaging (CMR), and exercise capacity assessment by 6‐minute walking test (6MWT) and cardiopulmonary exercise testing (CPET) at 1‐ and 2‐year follow‐ups.
Results
Transthoracic echocardiography RV longitudinal systolic function including tricuspid annular plan systolic excursion (TAPSE), peak systolic S′ wave tricuspid annular velocity (PSVtdi) and RV free wall longitudinal strain was decreased at 1 year (respectively, 15 ± 3 mm, 10 ± 3 cm/s, and −19 ± 5%) and at 2 years (respectively, 15 ± 3 mm, 10 ± 2 cm/s, and −20 ± 5%) with no significant difference between both evaluations; meanwhile, RV ejection fraction (RVEF) measured by CMR was preserved. Mean percentage of predicted peak oxygen consumption was altered, but improved between the first and second year (55 ± 18 vs 60 ± 18%, P = .038). PSVtdi was weakly correlated with 6MWT distance (r = .426, P = .017) and RVEF with the predicted distance at 6MWT (r = .410, P = .027) at the 1‐year follow‐up.
Conclusions
Despite decreasing values, RV longitudinal systolic function has a weak impact on exercise capacity of HTRs. PSVtdi and RVEF are the most pertinent parameters to assess the impact of RV systolic function on exercise capacity after heart transplantation. These results should lead to redefine normal RV systolic function thresholds for HTRs.
Aims
Advances have been made over the last decade in the management of cardiac amyloidosis (CA), but a delayed diagnosis is still common. The aim of this study was to describe the journey to CA ...diagnosis from initial clinical and to analyse time to diagnosis.
Methods and results
Between January 2001 and May 2019, 270 consecutive patients with CA diagnosed at Toulouse University Hospital were retrospectively included in this cross‐sectional study: 111 (41%) light chain amyloidosis, 122 (45%) wild‐type transthyretin amyloidosis, and 37 (14%) hereditary transthyretin amyloidosis.
CA onset occurred mostly with dyspnoea (50%) or systematic follow‐up (10%). The cardiologist was the first line specialist in 68% of patients, followed by the nephrologist (9%) and neurologist (8%). Patients encountered a median (minimum–maximum) number of two (1–7) physician specialists and performed a median (minimum–maximum) number of three (1–8) tests before diagnosis. Median delay between symptom onset and CA diagnosis was 8 IQR 5–14, 10 IQR 3–34, and 18 IQR 4–49 months, respectively, in light chain amyloidosis, wild‐type transthyretin amyloidosis, and hereditary transthyretin amyloidosis subgroups (P = .060). Having performed electromyography or spirometry was associated with a longer delay in diagnosis in the overall population: odds ratio = 1.13; 95% confidence interval 1.02 to 1.24; and odds ratio = 1.13; 1.03 to 1.24, respectively, probably due to non‐specific initial symptoms.
Conclusions
CA is a protean disease with various first line specialists causing a diagnostic wandering despite increasing medical community awareness. It requires a multidisciplinary specialist care networks to educate and manage symptoms and therapies.
To study, in the context of acute myocarditis (AM) in sportsmen, the association between the category of sport practiced and the severity of AM.
Retrospective study.
Single tertiary center.
82 ...sportspeople (≥2.5 h of sport per week) who presented an AM.
The type of sport activity before AM were collected.
Complicated AM was defined by a left ventricular ejection fraction <50% or the use of inotropic drugs or ventricular assist device.
Mean age was 29 ± 9 year-old, 77 (94%) were men. Sixteen (20%) had a complicated AM. Practicing sport over 6 h a week or a Mitchell’s Class IIIA sport were associated with complicated AM (44 vs. 17%, p = 0.019 and 38 vs. 11%, p = 0.008, respectively). Practicing a Mitchell’s Class IC sport was associated with uncomplicated AM (6 vs. 38%, p = 0.008).
In sportspeople's AM, the pattern of sport activity (static or dynamic component, practice intensity) is associated with the disease’s severity.
The purpose of the study was to describe the pattern of 99mTc-labeled phosphate agents myocardial uptake by scintigraphy and explore its impact on left ventricular (LV) functions in transthyretin ...cardiac amyloidosis (TTR-CA).
Fifty patients with TTR-CA underwent 99mTc- hydroxymethylene-diphosphonate (99mTc-HMDP) scintigraphy and echocardiography with measure of LV thickness, longitudinal strain (LS), systolic and diastolic functions. Cardiac retention by scintigraphy was assessed by visual scoring and the heart/whole body (H/WB) ratio was calculated by dividing counts in the heart by counts in late whole-body images.
The mean population age was 79 ± 10 years. Mean H/WB ratio was 12 ± 7. Myocardial 99mTc-HMDP uptake on segments 5, 6, 7, 8, 11, 12, 13, 14, 16, and 17 was correlated with H/WB ratio. Mean LVEF and global LS were 51 ± 10% and − 10 ± 3%, respectively. H/WB ratio was correlated with global LS (R = 0.408, P = .003), Ea (R = − 0.566, P < .001) and mean left ventricular wall thickness (R = 0.476, P < .001) but not with LVEF (R = − 0.109, P = .453). Segmental myocardial uptake was slightly correlated with segmental LS (R = 0.152, P < .001). H/WB ratio was not correlated with NT-proBNP levels (R = 0.219, P = .148) neither E/Ea ratio (R = 0.204, P = .184).
These findings show the relationship between bone tracer myocardial uptake and LV functions in patients with TTR cardiac amyloidosis.
AimsTo phenotype patients referred to a tertiary centre for the exploration of a left ventricular hypertrophy (LVH) starting from 12 mm of left ventricular wall thickness (LVWT).Methods and ...resultsConsecutive patients referred for aetiological workup of LVH, beginning at 12 mm of LVWT were retrospectively included in this tertiary single-centred observational study. Patients presenting with severe aortic stenosis were excluded. Aetiological workup was reviewed for each subject and aetiologies were adjudicated by expert consensus.Among 591 patients referred for LVH aetiological workup, 41% had a maximal LVWT below 15 mm. LVH aetiologies were led by cardiac amyloidosis (CA, 34.3%), followed by sarcomeric hypertrophic cardiomyopathy (S-HCM, 32.1%), hypertensive cardiomyopathy (21.7%), unknown aetiology (7.6%) and other (4.2%), including Anderson-Fabry’s disease (1.7%). CA and S-HCM affected over 50% of patients with mild LVH (12–14 mm); the prevalence of these aetiologies rose with LVH severity. Among patients with Anderson-Fabry’s disease, 4 (40%) had a maximal LVWT <15 mm.ConclusionsMild LVH (ie, 12–14 mm) conceals multiple aetiologies that can lead to specific treatment, cascade family screening and specific follow-up. Overall, CA is nowadays the leading cause of LVH in tertiary centers.
Background and objectives Severe forms of Tachycardia-induced cardiomyopathy (TIC) with cardiogenic shock are not well described so far, and efficiency of catheter ablation in this setting is ...unknown. Methods We retrospectively included consecutive patients admitted to the Intensive Cardiac Care Unit for acute heart failure with cardiogenic shock associated with atrial arrhythmia and managed by ablation. Result Fourteen patients were included, each with cardiogenic shock and two needing the use of extracorporeal membrane oxygenation. Successful ablation was performed in the acute setting or over the following weeks. Two patients experienced relapses of arrhythmias and were treated by new ablation procedures. At 7.5 ± 5 months follow-up, all patient were alive with stable sinus rhythm. The left ventricular Ejection Fraction dramatically improved (21 vs. 54%, p = 0.001) as well as the end-diastolic left ventricular diameter (61 vs. 51 mm, p = 0.01) and NYHA class (class IV in all vs. median 1, p = 0.002). Conclusion Restoration and maintenance of sinus rhythm in severe TIC with cardiogenic shock and atrial arrhythmias lead to a major increase or normalization of LVEF, reduction of ventricular dimensions, and improvement in functional status. Ablation is efficient in long-term maintenance of sinus rhythm and may be proposed early in refractory cases.
Aims
Tricuspid regurgitation (TR) is a common finding and has been associated with poorer outcome in patients with heart failure. This study sought to investigate the prognostic value of TR in ...patients with cardiac amyloidosis (CA).
Methods and results
Two‐hundred and eighty‐three patients with CA—172 (61%) wild‐type transthyretin amyloidosis (ATTRwt) and 111 (39%) light‐chain amyloidosis (AL)—were consecutively enrolled between December 2010 and September 2019. Transthoracic echocardiographies at time of diagnosis were reviewed to establish the presence and severity of TR and its relationship with all‐cause mortality during patients' follow‐up. Seventy‐four (26%) patients had a moderate‐to‐severe TR. Moderate‐to‐severe TR was associated with New York Heart Association status (P < 0.001), atrial fibrillation (P = 0.003), greater levels of natriuretic peptides (P = 0.002), worst renal function (P = 0.03), lower left ventricular ejection fraction (P = 0.02), reduced right ventricular systolic function (P = 0.001), thicker tricuspid leaflets (P = 0.019), greater tricuspid annulus diameter (P = 0.001), greater pulmonary artery pressure (P = 0.001), greater doses of furosemide (P = 0.001), and anti‐aldosterone (P = 0.01) and more anticoagulant treatment (P = 0.001). One hundred and thirty‐four (47%) patients met the primary endpoint of all‐cause mortality. After multivariate Cox analysis, moderate‐to‐severe TR was significantly associated with mortality hazard ratio 1.89, 95% confidence interval (1.01–3.51), P = 0.044 in patients with ATTRwt. There was no correlation between TR and death hazard ratio 0.84, 95% confidence interval (0.46–1.51), P = 0.562 in patients with AL.
Conclusions
Moderate‐to‐severe TR is frequent in CA, and it is an independent prognosis factor in patients with ATTRwt but not in patients with AL.
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