Background:Wild-type transthyretin-related amyloidosis (ATTRwt) and degenerative aortic stenosis (AS) are both age-related. Diagnosis of cardiac amyloidosis (CA) among patients with AS may be ...difficult due to overlapping morphological and functional criteria. The aim of this study was to describe an echocardiographic longitudinal strain (LS) pattern among patients with AS with and without ATTRwt.Methods and Results:Patients who have AS with ATTRwt (n=30), AS without ATTRwt (n=50) and ATTRwt without AS (n=31) underwent two-dimensional speckle-tracking echocardiography. Transthyretin CA was based on positive bone scintigraphy without monoclonal gammopathy. All patients showed a gradual decrease in LS from the base to the apex resulting in a decrease of the global LS. A cut-off value of 1.0 for relative apical LS (average apical LS/average basal LS+mid-LS) was sensitive (88%) but less specific (68%) in differentiating ATTRwt among patients with severe AS. The best cut-off value for relative apical LS for identifying patients with ATTRwt among the whole population was 0.9 (sensitivity 74%, specificity 66%); however, 35%, 25% and 11% of patients who have ATTRwt without AS, with moderate AS and with severe AS, respectively, did not reach this threshold.Conclusions:A decrease of global and relative apical LS is common in patients with AS, even in the absence of ATTRwt. ATTRwt CA can be present even in the absence of relative apical sparing of LS.
Atrial arrhythmias (AA) commonly affect patients with cardiac amyloidosis (CA) and are a contributing risk factor for the development of heart failure (HF). This study sought to investigate the ...long-term efficacy and impact of catheter ablation on HF progression in patients with CA and AA.
Thirty-one patients with CA and AA undergoing catheter ablation were retrospectively included (transthyretin-ATTR CA 61% and light chain-AL CA 39%). AA subtypes included atrial fibrillation (AFib) in 22 (paroxysmal in 10 and persistent in 12), atrial flutter (AFl) in 17 and atrial tachycardia (AT) in 11 patients. Long-term AA recurrence rates were evaluated along with the impact of sinus rhythm (SR) maintenance on HF and mortality.
AA recurrence was observed in 14 patients (45%) at a median of 3.5 months (AFib n = 8, AT n = 6, AFl = 0). Post-cardioversion, medical therapy or catheter ablation, 10 patients (32%) remained in permanent AA. Over a median follow-up of 19 months, all-cause mortality was 39% (n = 12): 3 with end-stage HF, 5 due to late complications of CA, 1 sudden cardiac death, 1 stroke, 1 COVID 19 (and one unknown). With maintenance of SR following catheter ablation, significant reductions in serum creatinine and natriuretic peptide levels were observed with improvements in NYHA class. Two patients required hospitalization for HF in the SR maintenance cohort compared to 5 patients in the AA recurrence cohort (p = 0.1). All 3 patients with deaths secondary to HF had AA recurrence compared to 11 out of the 28 patients whom were long-term survivors or deaths not related to HF (p = 0.04). All-cause mortality was not associated with AA recurrence.
This study demonstrates moderate long-term efficacy of SR maintenance with catheter ablation for AA in patients with CA. Improvements in clinical and biological status with positive trends in HF mortality are observed if SR can be maintained.
Prediction of ventricular arrhythmias (VA) mostly relies on left ventricular ejection fraction (LVEF), but with limited performance. New echocardiographic parameters such as mechanical dispersion ...have emerged, but acoustic window sometimes precludes this measurement. Nuclear imaging may be an alternative.
We aimed to assess the ability of mechanical dispersion, measured with phase standard deviation (PSD) on radionuclide angiocardiography (RNA), to predict VAs.
This retrospective monocentric observational study included all patients who underwent a tomographic RNA from 2015 to 2019. Phase analysis yielded PSD and follow-up was examined to identify VAs, heart transplantation, and death.
The study population consisted of 937 patients, mainly with LVEF ≤ 35% (425, 45%). Most had ischemic (334, 36%) or dilated cardiomyopathies (245, 26%). We identified 86 (9%) VAs. PSD was strongly associated with the occurrence of VA hazard ratio per 10 ms increase (HR10) 1.12 (1.09-1.16), heart transplantation HR10 1.09 (1.06-1.12), and death HR10 1.03 (1.00-1.05). The association between PSD and VA persisted after adjustment for age, sex, QRS duration, LVEF, global longitudinal strain (GLS), and echocardiography-assessed mechanical dispersion.
The occurrence of ventricular arrhythmias was predicted by mechanical dispersion assessed by RNA, even after adjustment for LVEF and GLS.
Background: Atrial fibrillation (AF) commonly affects patients with cardiac amyloidosis (CA). Amyloid deposition within the left atrium may be responsible for the subtype of AF in either permanent or ...non-permanent form. The prognostic implications of AF and its clinical subtype according to the type of CA are still controversial in this population. This study sought to investigate the prevalence, incidence and prognostic implications of AF and the clinical subtype of AF (permanent or non-permanent) in patients with CA.
Methods: Two hundred and thirty-eight patients with CA and full medical records were retrospectively enrolled in the study: About 115 (48%) with light chain (AL) amyloidosis and 123 (52%) with transthyretin amyloidosis (ATTR). Patient's medical records were reviewed to establish baseline prevalence, incidence and impact on all-cause and cardiovascular mortality during follow-up of AF.
Results: One hundred and four (44%) patients had history of AF at the time of diagnosis: 62 (60%) permanent and 42 (40%) non-permanent. There were 30 (26%) and 74 (60%) patients with history of AF among patients with AL and ATTR (including 5 hereditary and 69 wild-type), respectively (p<.0001). During the follow-up, 48 new patients developed AF (29, 12 and 7 among patients with AL, wild-type ATTR and hereditary ATTR). After adjustment for age, survival was similar in patients with or without history of AF (HR 0.87 (95% CI, 0.60 to 1.27; p = .467). AF had no impact on cardiovascular mortality. Among the 152 patients with history of AF included in the whole study, there were 75 (49%) patients with permanent AF. After adjustment for age, survival was similar in patients with permanent and non-permanent AF: HR 1.29 (95% CI, 0.84 to 1.99; p = .251). The results were the same among patients with AL or wild-type amyloidosis. Subtype of AF had no impact on cardiovascular mortality.
Conclusions: AF is common in patients with CA. However, AF and clinical subtype of AF have no impact on all-cause mortality, whatever the type of amyloidosis.
The relationship between arteriovenous access flow (Qa) and cardiovascular changes is complex. Several studies have shown cardiac remodeling and symptoms of heart failure for high-flow arteriovenous ...fistulas (AVF). To evaluate the early cardiovascular impact of AVF. Forty-seven patients with an AVF, hospitalized for the evaluation of high-flow AVF or a pre-kidney transplant assessment were included. We collected clinical and biological data. We also collected data of the assessment by transthoracic echocardiography, functional evaluation by 6-min-walk test and peak oxygen consumption, and measurement of coronary flow reserve by dynamic myocardial perfusion imaging. The measurement of Qa was performed by color Doppler ultrasound and then indexed to the body surface area (Qai) and to the cardiac output (CO) (Qa/CO). Patients were poorly symptomatic (18 and 1 patients NYHA stage 2 and 3, respectively). There was no correlation between Qa, Qai, or Qa/CO and functional status, assessed by peak oxygen consumption (
P
= 0.891;
P
= 0.803;
P
= 0.939, respectively). Symptomatic patients did not have higher Qa, Qai or Qa/CO than asymptomatic (2260 vs 2197 mL/min,
P
= 0.402; 1257 vs 1256 mL/min/m
2
,
P
= 0.835; and 34% vs 37%,
P
= 0.701, respectively). There was no correlation between Qa, Qai or Qa/CO and left ventricular end-diastolic volume or left ventricular ejection fraction. There was no correlation between coronary flow reserve and these 3 parameters of vascular access flow. However, the global longitudinal strain (GLS) was correlated with Qa and Qa/CO (
R
= 0.331,
P
= 0.023 and
R
= 0.380,
P
= 0.008, respectively). Increase of Qa or Qa/CO was associated with an alteration of the GLS. A cut-off value of 2250 mL/min for Qa allowed 83% sensitivity and 63% specificity for detecting an alteration of the GLS > − 18%. A cut-off value of 33% for Qa/CO allowed 92% sensitivity and 65% specificity. Impact of AVF on cardiac parameters is weak. However, GLS is the first parameter to be impacted by the flow of the fistula. Systematic transthoracic echocardiography evaluation with measurement of GLS should be proposed for all patients with Qa > 2250 mL/min or Qa/CO > 33%, to detect those at higher risk of cardiac impact of the AVF.
Transthyretin amyloidosis (ATTR amyloidosis) is a progressive, multisystemic, life-threatening disease resulting from the deposition of variant or wild-type (ATTRwt amyloidosis) transthyretin amyloid ...fibrils in various tissues and organs. Established in 2007, the Transthyretin Amyloidosis Outcomes Survey (THAOS) is the largest ongoing, global, longitudinal, observational study of patients with ATTR amyloidosis, including both hereditary and wild-type disease, and asymptomatic carriers of pathogenic TTR mutations. This analysis describes the baseline characteristics of symptomatic patients and asymptomatic gene carriers enrolled in THAOS since its inception in 2007 (data cutoff: August 1, 2022), providing a consolidated overview of 15-year data from the THAOS registry. This analysis included 4428 symptomatic patients and 1707 asymptomatic gene carriers. The majority of symptomatic patients were male (70.8%) with a mean (standard deviation SD) age at symptom onset of 56.6 (17.9) years. Compared with the 14-year analysis, V30M remained the most prevalent genotype in Europe (62.2%), South America (78.6%), and Japan (74.2%) and ATTRwt remained most common in North America (56.2%). Relative to the 14-year analysis, there was an increase of mixed phenotype (from 16.6 to 24.5%) and a reduction of predominantly cardiac phenotype (from 40.7 to 31.9%). The proportion of patients with predominantly neurologic phenotype remained stable (from 40.1 to 38.7%). Asymptomatic gene carriers were 58.5% female with a mean age at enrollment of 41.9 years (SD 15.5). This overview of > 6000 patients enrolled over 15 years in THAOS represents the largest registry analysis of ATTR amyloidosis to date and continues to emphasize the genotypic and phenotypic heterogeneity of the disease. Nearly a quarter of the symptomatic population within THAOS was mixed phenotype, underscoring the need for multidisciplinary management of ATTR amyloidosis.
To evaluate the contribution and performance of multimodal imaging in the diagnostic and therapeutic management of cardiac masses. We carried out a monocentric retrospective study on patients ...referred for cardiac mass assessment between 2006 and 2019, and analyzed the respective contribution of transesophageal echocardiography (TEE), cardiac computed tomography (CT), cardiac magnetic resonance (CMR) and
18
F-fluorodeoxyglucose positron emission tomography coupled with CT (
18
F-FDG PET-CT). For each test, we determined strategy before and after its completion (need for another imaging or decision-making) as well as result on benign, malignant or indeterminate nature. For the 119 patients included, all imaging modalities increased decision-making rates, which rose from 2 to 54%, 23 to 62%, 31 to 85% and 49 to 100% before and after TEE, CT, CMR and
18
F-FDG PET-CT, respectively (P < 0.001 before vs. after). TEE was particularly efficient for atrial masses, especially for the left atrium, with a decision rate rising from 0 to 74% (P < 0.001).
18
F-FDG PET-CT was the most efficient to differentiate benign and malignant etiologies (area under the curve 0.89 ± 0.06 and 0.94 ± 0.05 for benign and malignant, respectively, P < 0.001). A benign or undetermined result on each modality was associated with a good prognosis, compared to malignant. All modalities studied are useful for cardiac mass decision-making. First-line TEE is particularly efficient for atrial masses, whereas CT and CMR are useful for ventricular masses or suspicion of malignancy. A benign or malignant result for each modality is correlated to survival and
18
F-FDG PET-CT is the most effective to define it.
Background:
The study of left atrial (LA) longitudinal strain by speckle tracking is a reliable method for analyzing LA function that could provide relevant information in young patients with ...cryptogenic stroke (CS). The aim of this study was to investigate whether the presence of a patent foramen ovale (PFO) impacts the LA longitudinal strain in a population of young patients with first CS.
Methods and Results:
Patients aged 18 to 54 years, treated consecutively in a university hospital for first CS, were included in this study. The presence of a PFO and an atrial septal aneurysm (ASA) was investigated using transesophageal echocardiography and transcranial Doppler. Speckle tracking analysis was performed on transthoracic echocardiography, allowing the measurement of global, passive, and active longitudinal LA strain, corresponding to the reservoir, conduit, and contractile function, respectively. A total of 51 patients were included in the study. In a multivariable analysis, overweight was associated with reduced global and passive LA longitudinal strain (
P
= 0.013 and
P
= 0.018, respectively), and hypertension was associated with reduced active LA longitudinal strain (
P
= 0.049). LA longitudinal strain was not different between patients with PFO or PFO plus ASA and patients without PFO.
Conclusion:
LA longitudinal strain in young subjects with CS was impaired in the presence of overweight and hypertension, but not of PFO or PFO plus ASA.
Biological cardiac injury related to the Severe Acute Respiratory Syndrome Coronavirus-2 infection has been associated with excess mortality. However, its functional impact remains unknown. The aim ...of our study was to explore the impact of biological cardiac injury on myocardial functions in patients with COVID-19. 31 patients with confirmed COVID-19 (CoV+) and 16 controls (CoV−) were prospectively included in this observational study. Demographic data, laboratory findings, comorbidities, treatments and myocardial function assessed by transthoracic echocardiography were collected and analysed in CoV+ with (TnT+) and without (TnT−) elevation of troponin T levels and compared with CoV−. Among CoV+, 13 (42%) exhibited myocardial injury. CoV+/TnT + patients were older, had lower diastolic arterial pressure and were more likely to have hypertension and chronic renal failure compared with CoV+/TnT−. The control group was comparable except for an absence of biological inflammatory syndrome. Left ventricular ejection fraction and global longitudinal strain were not different among the three groups. There was a trend of decreased myocardial work and increased peak systolic tricuspid annular velocity between the CoV− and CoV + patients, which became significant when comparing CoV− and CoV+/TnT+ (2167 ± 359 vs. 1774 ± 521%/mmHg, P = 0.047 and 14 ± 3 vs. 16 ± 3 cm/s, P = 0.037, respectively). There was a decrease of global work efficiency from CoV− (96 ± 2%) to CoV+/TnT− (94 ± 4%) and then CoV+/TnT+ (93 ± 3%, P = 0.042). In conclusion, biological myocardial injury in COVID 19 has low functional impact on left ventricular systolic function.
Quantitative assessment of valve regurgitation using volumetric method by comparing right and left ventricular stroke volumes is still under investigations.
To investigate the accuracy of tomographic ...equilibrium radionuclide ventriculography (t-ERV) for the quantification of tricuspid regurgitation (TR).
Sixty-one patients (44 men; mean age 59 ± 12 years) who underwent both t-ERV and transthoracic echocardiography (TTE) studies within 2 weeks for right ventricular systolic function assessment were eligible for inclusion. A sub-group of 22 patients underwent both t-ERV and CMR. Patients with mitral/aortic regurgitation by TTE were excluded of the study. TR regurgitant volume (RVol) was calculated using the proximal isovelocity surface area (PISA) method from TTE and the volumetric method (right ventricular stroke volume minus left ventricular stroke volume) from t-ERV. There was a significant correlation between RVol as assess by ERV and by TTE (R = 0.95, P < 0.0001). Intraclass correlation coefficient between TTE and ERV for TR quantification was 0.95 (P < 0.0001). Among patients who underwent CMR, the correlation between RVol obtained by TTE and by t-ERV and CMR were R = 0.81 and R = 0.75, respectively (all P < 0.0001).
TR assessment using the t-ERV correlates well with PISA from TTE in patients referred for right ventricular systolic function assessment.
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