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1.
Ann Med ; 56(1): 2398735, 2024 Dec.
Article in English | MEDLINE | ID: mdl-39247984

ABSTRACT

AIM: Increased diagnostic awareness and specific disease treatments have changed the landscape of transthyretin cardiac amyloidosis (ATTR). Patients with wild-type ATTR (ATTRwt) are increasingly being diagnosed, potentially changing the clinical profile and prognosis compared with existing retrospective data. We aimed to study the clinical characteristics, distribution of red flags and prognosis of contemporary ATTRwt patients. METHODS: From January 1st 2017, to December 31st 2022, 213 consecutive patients were diagnosed with ATTRwt and prospectively followed up. Data on clinical characteristics, biomarkers, echocardiography findings, hospitalization due to worsening heart failure (WHF) and all-cause mortality were collected. RESULTS: A 37% increase in newly diagnosed patients from 2017-2019 (n = 90) vs. 2020-2022 (n = 123) was observed. The majority of patients presented with NAC disease stage I in the latter period (49% in 2017-2019 vs. 58% in 2020-2022, p = .16). Red flags were primarily cardiac-related, including elevated NT-proBNP, impaired left ventricular longitudinal systolic strain with an apical sparing pattern, heart failure with increased left ventricular wall thickness and elevated troponins. NAC disease stage I as well as low NT-proBNP levels (<1000 ng/L) were significantly associated with better survival (both p < .001). When compared with NAC disease stage II + III combined, patients with NAC disease stage I had a significantly lower risk of WHF hospitalization or death (log rank test: p = .0001). Independent predictors of the combined endpoint WHF hospitalization or death were NT-proBNP (HR 1.03 [95% CI 1.00-1.07], p < .049) and prior implantation of permanent pacemaker (HR 2.01 [1.30-3.11], p = .002). CONCLUSION: Increased diagnostic awareness resulted in a 37% increase in newly diagnosed patients in 2020-2022 vs. 2017-2019. As expected all-cause mortality but also the morbidity in terms of risk of hospitalization with WHF were significantly lower in patients with NAC disease stage I, as well as in those with low NT-proBNP levels <1000 ng/L. These findings underline the importance of continuous attention to diagnostic awareness, as early diagnosis is critical for initiating both general and specific ATTR treatment, thus improving prognosis.


Subject(s)
Amyloid Neuropathies, Familial , Cardiomyopathies , Echocardiography , Heart Failure , Natriuretic Peptide, Brain , Humans , Male , Female , Amyloid Neuropathies, Familial/blood , Amyloid Neuropathies, Familial/diagnosis , Amyloid Neuropathies, Familial/complications , Amyloid Neuropathies, Familial/mortality , Aged , Prognosis , Cardiomyopathies/diagnosis , Cardiomyopathies/blood , Cardiomyopathies/mortality , Natriuretic Peptide, Brain/blood , Middle Aged , Heart Failure/diagnosis , Heart Failure/etiology , Heart Failure/blood , Peptide Fragments/blood , Aged, 80 and over , Prospective Studies , Hospitalization/statistics & numerical data , Biomarkers/blood , Retrospective Studies
2.
Arch Cardiovasc Dis ; 117(8-9): 461-469, 2024.
Article in English | MEDLINE | ID: mdl-39153878

ABSTRACT

BACKGROUND: Transthyretin amyloid cardiomyopathy (ATTR-CM) can manifest as rhythm disorders, heart failure, but also valvular degeneration. Despite aortic stenosis (AS) being prevalent among the elderly, data on ATTR-CM prevalence and outcome in patients with AS undergoing transaortic valve implantation (TAVI) remain scarce. AIM: To determine ATTR-CM prevalence and evaluate 1-year survival in patients undergoing TAVI. METHODS: Between December 2020 and September 2021, 100 consecutive patients underwent TAVI and were screened prospectively for ATTR-CM using bone scintigraphy (BS). Monoclonal gammopathy was ruled out in case of cardiac uptake on BS. All patients were followed prospectively for 1year after TAVI. RESULTS: The proportion of patients aged≥75years or with a EuroSCORE II>8% and possible femoral access was 99%. The abnormal cardiac uptake rate on BS was 7% (95% confidence interval: 2-12%); 86% of these patients were male. The RAISE (remodelling, age, injury, system and electrical) score, indicative of ATTR-CM risk, was higher in case of positive BS (P=0.04). Patients with positive BS were older and exhibited wider QRS complexes on electrocardiography (P=0.003), a higher frequency of reduced LVEF (57% vs. 17%), impaired basal LV strain (P=0.02) and a lower voltage/mass ratio (P=0.01). History of pacemaker implantation before TAVI was higher in the positive BS group (P=0.0004) and remained the only statistically significant factor after adjustment using the Holm-Bonferroni method. One-year survival of patients with positive BS did not differ from that of patients with isolated AS. CONCLUSIONS: Prevalence of ATTR-CM in patients treated with TAVI, underscoring the need for continued surveillance for potential development of ATTR-CM after TAVI. Caution is warranted regarding the 1-year survival because of the lack of study power. Further investigations are needed to define long-term prognosis of AS with ATTR-CM.


Subject(s)
Amyloid Neuropathies, Familial , Aortic Valve Stenosis , Cardiomyopathies , Transcatheter Aortic Valve Replacement , Humans , Aortic Valve Stenosis/surgery , Aortic Valve Stenosis/diagnostic imaging , Aortic Valve Stenosis/mortality , Aortic Valve Stenosis/physiopathology , Aortic Valve Stenosis/epidemiology , Male , Female , Prevalence , Aged , Transcatheter Aortic Valve Replacement/adverse effects , Transcatheter Aortic Valve Replacement/mortality , Aged, 80 and over , Time Factors , Treatment Outcome , Amyloid Neuropathies, Familial/mortality , Amyloid Neuropathies, Familial/epidemiology , Amyloid Neuropathies, Familial/diagnostic imaging , Amyloid Neuropathies, Familial/complications , Amyloid Neuropathies, Familial/surgery , Amyloid Neuropathies, Familial/diagnosis , Risk Factors , Prospective Studies , Cardiomyopathies/mortality , Cardiomyopathies/diagnostic imaging , Cardiomyopathies/physiopathology , Cardiomyopathies/epidemiology , Risk Assessment , Aortic Valve/surgery , Aortic Valve/diagnostic imaging , Aortic Valve/physiopathology
3.
Qual Life Res ; 33(10): 2743-2753, 2024 Oct.
Article in English | MEDLINE | ID: mdl-39105960

ABSTRACT

PURPOSE: Transthyretin amyloid cardiomyopathy (ATTR-CM) is associated with severely impaired health-related quality of life (HRQL). HRQL is an independent predictor of outcome in heart failure (HF), but data on patients with ATTR-CM is scarce. This study therefore aims to evaluate the association of HRQL with outcome in ATTR-CM. METHODS: Patients from our prospective ATTR-CM registry were assessed using the Kansas City cardiomyopathy questionnaire (KCCQ), the Minnesota living with HF questionnaire (MLHFQ), and the EuroQol five dimensions questionnaire (EQ-5D). Cox regression analysis was utilised to assess the impact of HRQL on all-cause mortality. RESULTS: 167 patients [80 years; interquartile range (IQR): 76-84; 80.8% male] were followed for a median of 27.6 (IQR: 9.7-41.8) months. The primary endpoint of all-cause mortality was met by 43 (25.7%) patients after a median period of 16.2 (IQR: 9.1-28.1) months. In a univariate Cox regression for mortality, a 10-point change in the KCCQ implied a hazard ratio (HR) of 0.815 [95%-confidence interval (CI): 0.725-0.916; p = 0.001], in the EQ-5D VAS of 0.764 (95%-CI: 0.656-0.889; p < 0.001), and 1.163 (95%-CI: 1.114-1.433; p < 0.001) in the MLHFQ. After adjustment for established biomarkers of HF, all-cause mortality was predicted independently by the EQ-5D VAS (HR: 0.8; 95%-CI: 0.649-0.986; p = 0.037; per 10 points) and the MLHFQ (HR: 1.228; 95%-CI: 1.035-1.458; p = 0.019; per 10 points). CONCLUSION: HRQL is a predictor of outcome in ATTR-CM. The EQ-5D VAS and the MLHFQ predict survival independent of biomarkers of HF.


Patients with transthyretin amyloid cardiomyopathy, a condition causing heart failure and mostly seen in the elderly, suffer from shortness of breath and reduced maximum physical performance. Disease assessment is currently based on blood analysis for markers of heart failure. However, standardised patient questionnaires also allow to estimate disease severity. In this study, we analyse different standardised patient questionnaires for their ability to predict adverse events including death and heart failure-related hospitalisations. The analysis demonstrates that an increase of ten points in the Kansas City Cardiomyopathy questionnaire, a tool specifically designed for patients with heart failure, implies a reduction of mortality risk of close to 20%. Interestingly, even the very simple visual analogue scale, a quality-of-life measurement tool which asks the patient to rate their health on a scale from zero (worst) to one hundred (best) has demonstrated remarkable predictive utility. An increase of ten points on this scale resulted in a reduction of risk for death from any cause of almost a quarter. This analysis suggests that standardised patient questionnaires for the assessment of quality of life may play an important role in the evaluation of patients with transthyretin amyloid cardiomyopathy and estimation of prognosis.


Subject(s)
Amyloid Neuropathies, Familial , Cardiomyopathies , Hospitalization , Quality of Life , Humans , Quality of Life/psychology , Male , Female , Prospective Studies , Aged , Amyloid Neuropathies, Familial/mortality , Amyloid Neuropathies, Familial/psychology , Cardiomyopathies/mortality , Cardiomyopathies/psychology , Aged, 80 and over , Hospitalization/statistics & numerical data , Surveys and Questionnaires , Proportional Hazards Models , Heart Failure/mortality , Heart Failure/psychology
4.
Am J Cardiol ; 228: 70-77, 2024 Oct 01.
Article in English | MEDLINE | ID: mdl-39067579

ABSTRACT

Anemia is prevalent in transthyretin amyloid cardiomyopathy (ATTR-CM), but its prognostic significance remains uncertain because of conflicting data mainly in patients not receiving disease-modifying therapy. Additionally, the effect of anemia on morbidity in this population has not been studied. This retrospective study included 270 patients diagnosed with ATTR-CM, receiving disease-modifying treatment (tafamidis), of which 30% (n = 80) were anemic (defined as a hemoglobin level <13 g/100 ml for males and <12 g/100 ml for females according to the World Health Organization). At baseline, patients with anemia were on average older (mean age 79 vs 77 years), more likely to be female (21% vs 12%), and exhibited higher symptom severity based on the New York Heart Association class (42% in class III vs 27%) compared with those without anemia. Additionally, they had a worse Columbia score (mean score 3 vs 5) and Columbia stage (12% in late-stage vs 7.1%) than those without anemia. Kaplan-Meier analysis indicates that anemia was associated with a higher likelihood of mortality, all-cause, and cardiovascular (CV) hospitalizations (p <0.05). However, in the Cox regression analysis, after adjusting for baseline age, ATTR genotype, and Columbia score, anemia was only associated with a higher risk of all-cause hospitalizations (hazard ratio 1.9 (1.3 to 2.7), p <0.001) and CV-related hospitalizations (hazard ratio 1.9 (1.2 to 2.9), p = 0.006). In conclusion, this study indicates that anemic patients with ATTR-CM have higher risks of CV and all-cause hospitalizations compared with nonanemic ATTR-CM patients. Further research is needed to understand how treating anemia may improve outcomes in this high-risk patient population.


Subject(s)
Amyloid Neuropathies, Familial , Anemia , Cardiomyopathies , Humans , Female , Male , Anemia/epidemiology , Anemia/complications , Aged , Retrospective Studies , Amyloid Neuropathies, Familial/complications , Amyloid Neuropathies, Familial/mortality , Cardiomyopathies/mortality , Cardiomyopathies/complications , Cardiomyopathies/epidemiology , Prognosis , Aged, 80 and over , Morbidity/trends , Survival Rate/trends , Benzoxazoles/therapeutic use
5.
JACC Cardiovasc Imaging ; 17(8): 911-922, 2024 Aug.
Article in English | MEDLINE | ID: mdl-39001731

ABSTRACT

BACKGROUND: Positron emission tomography/computed tomography (PET/CT) with 18F-florbetapir, a novel amyloid-targeting radiotracer, can quantify left ventricular (LV) amyloid burden in systemic light-chain (AL) amyloidosis. However, its prognostic value is not known. OBJECTIVES: The authors' aim was to evaluate the prognostic value of LV amyloid burden quantified by 18F-florbetapir PET/CT, and to identify mechanistic pathways mediating its association with outcomes. METHODS: A total of 81 participants with newly diagnosed AL amyloidosis underwent 18F-florbetapir PET/CT imaging. Amyloid burden was quantified using 18F-florbetapir LV uptake as percent injected dose. The Mayo stage for AL amyloidosis was determined using troponin T, N-terminal pro-B-type natriuretic peptide (NT-proBNP), and free light chain levels. Major adverse cardiac events (MACE) were defined as all-cause death, heart failure hospitalization, or cardiac transplantation within 12 months. RESULTS: Among participants (median age, 61 years; 57% males), 36% experienced MACE, increasing from 7% to 63% across tertiles of LV amyloid burden (P < 0.001). LV amyloid burden was associated with MACE (HR: 1.46; 95% CI: 1.16-1.83; P = 0.001). However, this association became nonsignificant when adjusted for Mayo stage. In mediation analysis, the association between LV amyloid burden and MACE was mediated by NT-proBNP (P < 0.001), a marker of cardiomyocyte stretch and heart failure, and a component of Mayo stage. CONCLUSIONS: In this first study to link cardiac 18F-florbetapir uptake to subsequent outcomes, LV amyloid burden estimated by percent injected dose predicted MACE in AL amyloidosis. This effect was not independent of Mayo stage and was mediated primarily through NT-proBNP. These findings provide novel insights into the mechanism linking myocardial amyloid deposits to MACE.


Subject(s)
Aniline Compounds , Ethylene Glycols , Immunoglobulin Light-chain Amyloidosis , Natriuretic Peptide, Brain , Peptide Fragments , Positron Emission Tomography Computed Tomography , Predictive Value of Tests , Radiopharmaceuticals , Humans , Female , Male , Middle Aged , Aged , Radiopharmaceuticals/administration & dosage , Immunoglobulin Light-chain Amyloidosis/diagnostic imaging , Immunoglobulin Light-chain Amyloidosis/metabolism , Immunoglobulin Light-chain Amyloidosis/mortality , Prognosis , Natriuretic Peptide, Brain/blood , Peptide Fragments/metabolism , Peptide Fragments/blood , Risk Factors , Ventricular Function, Left , Heart Ventricles/diagnostic imaging , Heart Ventricles/metabolism , Time Factors , Heart Failure/diagnostic imaging , Heart Failure/metabolism , Biomarkers/blood , Heart Transplantation/adverse effects , Risk Assessment , Cardiomyopathies/diagnostic imaging , Cardiomyopathies/metabolism , Cardiomyopathies/mortality , Immunoglobulin Light Chains/metabolism
6.
J Am Heart Assoc ; 13(14): e033478, 2024 Jul 16.
Article in English | MEDLINE | ID: mdl-38958150

ABSTRACT

BACKGROUND: Results from ATTR-ACT (Safety and Efficacy of Tafamidis in Patients With Transthyretin Cardiomyopathy) indicate that tafamidis prolongs survival and reduces cardiovascular hospitalizations in cardiac transthyretin amyloidosis (ATTR-CA). However, real-world data supporting these findings are scarce. Thus, we sought to characterize the clinical outcome of patients with ATTR-CA treated with tafamidis in a real-world setting and assess the prognostic role of the New York Heart Association (NYHA) classification. METHODS AND RESULTS: We conducted a retrospective observational study, enrolling a consecutive sample of patients with ATTR-CA (wild-type or variant) treated with tafamidis. Clinical outcome was tracked through follow-up visits or phone calls. Primary outcomes were death and major adverse cardiac events (MACE), a composite end point of death and hospitalizations for acute cardiac decompensation, myocardial infarction, severe arrythmias, or stroke. Kaplan-Meier analysis estimated overall and MACE-free survival including NYHA subgroups (NYHA I/II versus NYHA III). One hundred sixty-seven patients with ATTR-CA (94.6% wild-type) were enrolled and followed for a median of 539 [323-869] days. Median overall survival was not reached. Estimated 1-year, 2-year, and 5-year overall survival among the whole cohort was 93.5%, 85.9%, and 70.2%, respectively. Overall survival was higher in the NYHA I/II subgroup (P=0.002). Median MACE-free survival time was 1082 (95% CI, 962-1202) days. MACE-free survival was higher in the NYHA I/II subgroup (P<0.001). With respective hazard ratios of 5.85 (95% CI, 1.48-23.18; P=0.012) and 3.95 (95% CI, 1.99-7.84; P<0.001), NYHA III was an independent predictor of death and MACE. CONCLUSIONS: Treatment of ATTR-CA with tafamidis led to substantial improvements of clinical outcome. NYHA classification at treatment initiation is a reliable tool to provide patients with individualized prognostic information.


Subject(s)
Amyloid Neuropathies, Familial , Humans , Male , Amyloid Neuropathies, Familial/complications , Amyloid Neuropathies, Familial/mortality , Amyloid Neuropathies, Familial/therapy , Amyloid Neuropathies, Familial/drug therapy , Female , Retrospective Studies , Aged , Benzoxazoles/therapeutic use , Middle Aged , Treatment Outcome , Cardiomyopathies/mortality , Aged, 80 and over , Time Factors , Hospitalization/statistics & numerical data , Prognosis
8.
Amyloid ; 31(3): 220-225, 2024 Sep.
Article in English | MEDLINE | ID: mdl-38989681

ABSTRACT

BACKGROUND: Early identification of immunoglobulin light-chain amyloidosis (AL) is crucial due to its rapid progression. Monoclonal light-chain (M-LC) testing is the first step in the diagnostic workup for patients with suspected cardiac amyloidosis (CA). We aimed to determine whether the time interval between the first CA suspicion and M-LC testing can be related to AL amyloidosis survival outcomes. METHODS: All patients (n = 94) with isolated cardiac AL amyloidosis diagnosed at our center between 2016 and 2020 were included. Those with pre-existing known monoclonal protein (monoclonal gammopathy of undetermined significance or smoldering multiple myeloma) were excluded. Time intervals to diagnostic tests and diagnosis were calculated and assessed for their survival prediction ability. RESULTS: The time interval between first CA suspicion (on echocardiography) and M-LC testing correlated with early mortality, and the best cutoff predicting survival, was 6 weeks. The 26 patients (∼28% of entire cohort) who underwent M-LC-studies >6 weeks after first suspicion more frequently presented Mayo stage IIIb (65% vs. 35%, p = .008), showing poorer overall survival than those (n = 68, 72%) referred for early M-LC studies (median 3 vs. 14 months, p = .039). CONCLUSIONS: Monoclonal protein testing should be the first-step in the diagnostic workup for patients with echocardiographic/other instrumental red flags raising CA suspicion.


Subject(s)
Immunoglobulin Light-chain Amyloidosis , Humans , Male , Immunoglobulin Light-chain Amyloidosis/mortality , Immunoglobulin Light-chain Amyloidosis/diagnosis , Immunoglobulin Light-chain Amyloidosis/pathology , Immunoglobulin Light-chain Amyloidosis/metabolism , Female , Aged , Middle Aged , Echocardiography , Immunoglobulin Light Chains/blood , Immunoglobulin Light Chains/metabolism , Retrospective Studies , Cardiomyopathies/mortality , Cardiomyopathies/diagnosis , Cardiomyopathies/pathology , Cardiomyopathies/metabolism , Aged, 80 and over
9.
Heart ; 110(18): 1124-1132, 2024 Aug 26.
Article in English | MEDLINE | ID: mdl-39084705

ABSTRACT

BACKGROUND: Light chain cardiac amyloidosis (AL-CA) is associated with a high incidence of mortality. Big endothelin-1 (ET-1), the precursor of endothelial-vasoconstrictive ET-1, is closely related to the concentration of bioactive ET-1. Association between big ET-1 and prognosis of AL-CA has not yet been documented. The purpose of this study was to evaluate the prognostic value of big ET-1 for poor outcomes in moderate to severe AL-CA. METHODS: Big ET-1 levels were determined on admission in patients with newly diagnosed AL-CA with modified Mayo 2004 stage II or III. Primary outcome was all-cause mortality. The secondary outcomes included death from cardiac cause and the composite of the primary outcome or hospitalisations due to worsening heart failure. RESULTS: Overall, 141 patients were retrospectively included (57 stage II, 34 stage IIIa, 50 stage IIIb). During a median follow-up time of 25.7 months, 84 (59.6%) patients died. Patients with big ET-1 levels of ≤0.88 pmol/L had longer survival than those with >0.88 pmol/L (median survival time: 34.1 months vs 15.3 months, log-rank p<0.001), which was also observed in the validation cohort (log-rank p=0.026). Higher big ET-1 levels were predictive for all-cause mortality after multivariable adjustment (HR 1.91, 95% CI 1.05 to 3.49, p=0.035). Big ET-1 levels added an incremental prognostic value over modified Mayo 2004 stage (C-index: from 0.671 to 0.696, p=0.025; integrated discrimination improvement 0.168, p=0.047). CONCLUSIONS: Big ET-1 is a strong and independent predictor of mortality in patients with moderate to severe AL-CA, which may indicate a possible role for risk stratification in patients with this disease.


Subject(s)
Biomarkers , Cardiomyopathies , Endothelin-1 , Humans , Endothelin-1/blood , Male , Female , Aged , Prognosis , Retrospective Studies , Middle Aged , Cardiomyopathies/blood , Cardiomyopathies/mortality , Cardiomyopathies/diagnosis , Biomarkers/blood , Immunoglobulin Light-chain Amyloidosis/blood , Immunoglobulin Light-chain Amyloidosis/mortality , Immunoglobulin Light-chain Amyloidosis/diagnosis , Severity of Illness Index , Predictive Value of Tests , Risk Factors , Risk Assessment/methods
10.
Int J Cardiovasc Imaging ; 40(8): 1797-1807, 2024 Aug.
Article in English | MEDLINE | ID: mdl-39012402

ABSTRACT

The prognostic implications of late gadolinium-enhanced (LGE) magnetic resonance imaging (MRI) in the context of cardiac sarcoidosis (CS) have attracted considerable attention. Nevertheless, a subset of studies has undistinguished confirmed and suspected CS cases, thereby engendering interpretative ambiguities. In this meta-analysis, we evaluated the differences in cardiac MRI findings and their prognostic utility between confirmed and suspected CS. A literature search was conducted using PubMed, Web of Science, and Cochrane libraries to compare the findings of cardiac MRI and its prognostic value in CS and suspected CS. A meta-analysis was performed to compare the prevalence of LGE MRI, odds ratios, and hazard ratios for predicting cardiac events in both groups. A total of 21 studies encompassing 24 different populations were included in the meta-analysis (CS: 393 cases, suspected CS: 2151 cases). CS had a higher frequency of LGE of the left ventricle (87.2% vs. 36.4%, p < 0.0001) and right ventricle (62.1% vs. 23.8%, p = 0.04) than suspected CS. In patients with suspected CS, the presence of left ventricular LGE was associated with higher all-cause mortality [odds ratio: 5.70 (95%CI: 2.51-12.93), p < 0.0001, I2 = 8%, p for heterogeneity = 0.37] and ventricular arrhythmia [odds ratio: 15.51 (95%CI: 5.65-42.55), p < 0.0001, I2 = 0, p for heterogeneity = 0.94]. In contrast, in CS, not the presence but extent of left ventricular LGE was a significant predictor of outcome (hazard ratio = 1.83 per 10% increase of %LGE (95%CI: 1.43-2.34, p < 0.001, I2 = 15, p for heterogeneity = 0.31). The presence of left ventricular LGE was a strong prognostic factor in suspected sarcoidosis. However, the extremely high prevalence of left ventricular LGE in confirmed CS suggests that the quantitative assessment of LGE is useful for prognostic estimation.


Subject(s)
Cardiomyopathies , Contrast Media , Magnetic Resonance Imaging , Myocardium , Sarcoidosis , Humans , Cardiomyopathies/diagnostic imaging , Cardiomyopathies/mortality , Chi-Square Distribution , Contrast Media/administration & dosage , Gadolinium/administration & dosage , Magnetic Resonance Imaging/methods , Myocardium/pathology , Odds Ratio , Predictive Value of Tests , Prognosis , Risk Factors , Sarcoidosis/diagnostic imaging , Sarcoidosis/mortality , Ventricular Function, Left , Heart/diagnostic imaging
11.
Europace ; 26(6)2024 Jun 03.
Article in English | MEDLINE | ID: mdl-38934242

ABSTRACT

AIMS: Wild-type transthyretin amyloid cardiomyopathy (ATTRwt-CM) is often accompanied by atrial fibrillation (AF), atrial flutter (AFL), and atrial tachycardia (AT), which are difficult to control because beta-blockers and antiarrhythmic drugs can worsen heart failure (HF). This study aimed to investigate the outcomes of catheter ablation (CA) for AF/AFL/AT in patients with ATTRwt-CM and propose a treatment strategy for CA. METHODS AND RESULTS: A cohort study was conducted on 233 patients diagnosed with ATTRwt-CM, including 54 who underwent CA for AF/AFL/AT. The background of each arrhythmia and the details of the CA and its outcomes were investigated. The recurrence-free rate of AF/AFL/AT overall in ATTRwt-CM patients with multiple CA was 70.1% at 1-year, 57.6% at 2-year, and 44.0% at 5-year follow-up, but CA significantly reduced all-cause mortality [hazard ratio (HR): 0.342, 95% confidence interval (CI): 0.133-0.876, P = 0.025], cardiovascular mortality (HR: 0.378, 95% CI: 0.146-0.981, P = 0.045), and HF hospitalization (HR: 0.488, 95% CI: 0.269-0.889, P = 0.019) compared with those without CA. There was no recurrence of the cavotricuspid isthmus (CTI)-dependent AFL, non-CTI-dependent simple AFL terminated by one linear ablation, and focal AT originating from the atrioventricular (AV) annulus or crista terminalis eventually. Twelve of 13 patients with paroxysmal AF and 27 of 29 patients with persistent AF did not have recurrence as AF. However, all three patients with non-CTI-dependent complex AFL not terminated by a single linear ablation and 10 of 13 cases with focal AT or multiple focal ATs originating beyond the AV annulus or crista terminalis recurred even after multiple CA. CONCLUSION: The outcomes of CA for ATTRwt-CM were acceptable, except for multiple focal AT and complex AFL. Catheter ablation may be aggressively considered as a treatment strategy with the expectation of improving mortality and hospitalization for HF.


Subject(s)
Amyloid Neuropathies, Familial , Atrial Fibrillation , Atrial Flutter , Cardiomyopathies , Catheter Ablation , Humans , Catheter Ablation/adverse effects , Male , Atrial Flutter/surgery , Atrial Flutter/etiology , Female , Atrial Fibrillation/surgery , Atrial Fibrillation/diagnosis , Atrial Fibrillation/physiopathology , Aged , Amyloid Neuropathies, Familial/surgery , Amyloid Neuropathies, Familial/complications , Amyloid Neuropathies, Familial/mortality , Cardiomyopathies/mortality , Cardiomyopathies/therapy , Treatment Outcome , Middle Aged , Recurrence , Tachycardia, Supraventricular/surgery , Tachycardia, Supraventricular/etiology , Tachycardia, Supraventricular/physiopathology , Tachycardia, Supraventricular/diagnosis , Retrospective Studies , Prealbumin/genetics , Prealbumin/metabolism
12.
J Am Heart Assoc ; 13(13): e034723, 2024 Jul 02.
Article in English | MEDLINE | ID: mdl-38904242

ABSTRACT

BACKGROUND: Cardiac amyloidosis (CA) is frequently found in older patients with aortic stenosis (AS). However, the prevalence of AS among patients with CA is unknown. The objective was to study the prevalence and prognostic impact of AS among patients with CA. METHODS AND RESULTS: We conducted a retrospective analysis of a prospective registry comprising 976 patients with native aortic valves who were confirmed with wild type transthyretin amyloid (ATTRwt), hereditary variant transthyretin amyloid (ATTRv), or immunoglobulin light-chain (AL) CA. CA patients' echocardiograms were re-analyzed focusing on the aortic valve. Multivariable Cox regression analysis was performed to assess the mortality risk associated with moderate or greater AS in ATTRwt CA. The crude prevalence of AS among patients with CA was 26% in ATTRwt, 8% in ATTRv, and 5% in AL. Compared with population-based controls, all types of CA had higher age- and sex-standardized rate ratios (SRRs) of having any degree of AS (AL: SRR, 2.62; 95% Confidence Interval (CI) [1.09-3.64]; ATTRv: SRR, 3.41; 95%CI [1.64-4.60]; ATTRwt: SRR, 10.8; 95%CI [5.25-14.53]). Compared with hospital controls, only ATTRwt had a higher SRR of having any degree of AS (AL: SRR, 0.97, 95%CI [0.56-1.14]; ATTRv: SRR, 1.27; 95%CI [0.85-1.44]; ATTRwt: SRR, 4.01; 95%CI [2.71-4.54]). Among patients with ATTRwt, moderate or greater AS was not associated with increased all-cause death after multivariable adjustment (hazard ratio, 0.71; 95%CI [0.42-1.19]; P=0.19). CONCLUSIONS: Among patients with CA, ATTRwt but not ATTRv or AL is associated with a higher prevalence of patients with AS compared with hospital controls without CA, even after adjusting for age and sex. In our population, having moderate or greater AS was not associated with a worse outcome in patients with ATTRwt.


Subject(s)
Aortic Valve Stenosis , Cardiomyopathies , Registries , Humans , Male , Female , Prevalence , Aged , Retrospective Studies , Aortic Valve Stenosis/epidemiology , Aortic Valve Stenosis/mortality , Aortic Valve Stenosis/diagnostic imaging , Prognosis , Cardiomyopathies/epidemiology , Cardiomyopathies/mortality , Aged, 80 and over , Amyloid Neuropathies, Familial/epidemiology , Amyloid Neuropathies, Familial/mortality , Amyloid Neuropathies, Familial/complications , Amyloid Neuropathies, Familial/genetics , Amyloid Neuropathies, Familial/diagnosis , Risk Factors , Echocardiography , Middle Aged , Amyloidosis/epidemiology , Amyloidosis/mortality , Amyloidosis/diagnosis , Immunoglobulin Light-chain Amyloidosis/epidemiology , Immunoglobulin Light-chain Amyloidosis/mortality , Immunoglobulin Light-chain Amyloidosis/complications , Prealbumin/genetics , Aortic Valve/diagnostic imaging
13.
J Am Heart Assoc ; 13(13): e034055, 2024 Jul 02.
Article in English | MEDLINE | ID: mdl-38904229

ABSTRACT

BACKGROUND: Although peripartum cardiomyopathy (PPCM) is a fatal disease affecting young patients and fetuses, little is known about its recent prognosis and risk factors. This study investigated temporal trends in clinical characteristics and outcomes for PPCM in a nationwide multicenter registry. METHODS AND RESULTS: The study population comprised 340 patients (mean age, 33 years) who were diagnosed with PPCM between January 2000 and September 2022 in 26 tertiary hospitals in South Korea. PPCM was defined as heart failure with left ventricular ejection fraction ≤45% and no previously known cardiac disease. The main study outcomes included time to the first occurrence of all-cause death, heart transplantation, and cardiovascular hospitalization. The diagnosis of PPCM cases increased notably during the study period (P<0.001). However, clinical outcomes showed no significant improvement (all-cause death for 10 years: 0.9% [2000-2010] versus 2.3% [2011-2022], P=0.450; all-cause death and heart transplantation for 10 years: 3.6% [2000-2010] versus 3.0% [2011-2022] P=0.520; all-cause death, heart transplantation, and cardiovascular hospitalization for 10 years: 11.7% [2000-2010] versus 19.8% [2011-2022], P=0.240). High body mass index (hazard ratio [HR], 1.106 [95% CI, 1.024-1.196]; P=0.011), the presence of gestational diabetes (HR, 5.346 [95% CI, 1.778-16.07]; P=0.002), and increased baseline left ventricular end-diastolic dimension (HR, 1.078 [95% CI, 1.002-1.159]; P=0.044) were significant risk factors for poor prognosis. CONCLUSIONS: While the incidence of PPCM has increased over the past 20 years, the prognosis has not improved significantly. Timely management and close follow-up are necessary for high-risk patients with PPCM with high body mass index, gestational diabetes, or large left ventricular end-diastolic dimension.


Subject(s)
Cardiomyopathies , Peripartum Period , Pregnancy Complications, Cardiovascular , Registries , Humans , Female , Adult , Pregnancy , Republic of Korea/epidemiology , Cardiomyopathies/epidemiology , Cardiomyopathies/therapy , Cardiomyopathies/physiopathology , Cardiomyopathies/mortality , Pregnancy Complications, Cardiovascular/epidemiology , Pregnancy Complications, Cardiovascular/therapy , Risk Factors , Time Factors , Heart Transplantation/trends , Heart Transplantation/statistics & numerical data , Prognosis , Ventricular Function, Left , Stroke Volume , Cause of Death/trends , Hospitalization/trends , Hospitalization/statistics & numerical data , Puerperal Disorders/epidemiology , Puerperal Disorders/therapy , Puerperal Disorders/mortality , Puerperal Disorders/physiopathology , Retrospective Studies , Heart Failure/epidemiology , Heart Failure/mortality , Heart Failure/therapy , Heart Failure/physiopathology , Incidence
14.
J Cardiol ; 84(3): 177-179, 2024 Sep.
Article in English | MEDLINE | ID: mdl-38754764

ABSTRACT

BACKGROUND: Risk stratification for patients with non-ischemic cardiomyopathy (NICM) remains challenging as previous studies predicting life-threatening ventricular arrhythmia (LTVA) events were conducted before the establishment of the current standard treatment. We investigated the prognostic value of non-sustained ventricular tachycardia (NSVT) in NICM patients among recent studies. METHODS: MEDLINE, Embase were searched from January 2000 to October 2023. The risk of NSVT on LTVA and mortality was assessed using a random-effects model for patients with NICM. A meta-regression analysis was employed to identify sources of heterogeneity. The systematic review and meta-analysis were carried out according to the PRISMA guidelines. RESULTS: A total of 18 studies were identified, including 5238 pooled participants. Meta-analysis demonstrated that the presence of NSVT was considered a significant prognostic indicator for LTVA events [hazard ratio (HR): 2.90; 95 % CI; 2.31-3.64] with low heterogeneity (I2: 19 %) and for mortality (HR; 2.28; 95%CI; 1.26-4.13) with high heterogeneity (I2: 69 %). The prognostic value of NSVT for LTVA was not affected by either ejection fraction or medications at baseline. CONCLUSION: NSVT remained an important predictor of LTVA events even in patients receiving healthcare in contemporary eras. Detection of NSVT helps us to identify the high-risk patients with NICM.


Subject(s)
Cardiomyopathies , Tachycardia, Ventricular , Humans , Tachycardia, Ventricular/etiology , Tachycardia, Ventricular/mortality , Cardiomyopathies/complications , Cardiomyopathies/etiology , Cardiomyopathies/mortality , Prognosis , Risk Assessment
15.
PLoS One ; 19(5): e0296440, 2024.
Article in English | MEDLINE | ID: mdl-38691571

ABSTRACT

BACKGROUND: Chronic myocardial injury is a condition defined by stably elevated cardiac biomarkers without acute myocardial ischemia. Although studies from high-income countries have reported that chronic myocardial injury predicts adverse prognosis, there are no published data about the condition in sub-Saharan Africa. METHODS: Between November 2020 and January 2023, adult patients with chest pain or shortness of breath were recruited from an emergency department in Moshi, Tanzania. Medical history and point-of-care troponin T (cTnT) assays were obtained from participants; those whose initial and three-hour repeat cTnT values were abnormally elevated but within 11% of each other were defined as having chronic myocardial injury. Mortality was assessed thirty days following enrollment. RESULTS: Of 568 enrolled participants, 81 (14.3%) had chronic myocardial injury, 73 (12.9%) had acute myocardial injury, and 412 (72.5%) had undetectable cTnT values. Of participants with chronic myocardial injury, the mean (± sd) age was 61.5 (± 17.2) years, and the most common comorbidities were CKD (n = 65, 80%) and hypertension (n = 60, 74%). After adjusting for CKD, thirty-day mortality rates (38% vs. 36%, aOR 1.03, 95% CI: 0.52-2.03, p = 0.931) were similar between participants with chronic myocardial injury and those with acute myocardial injury, but significantly greater (38% vs. 13.6%, aOR 3.63, 95% CI: 1.98-6.65, p<0.001) among participants with chronic myocardial injury than those with undetectable cTnT values. CONCLUSION: In Tanzania, chronic myocardial injury is a poor prognostic indicator associated with high risk of short-term mortality. Clinicians practicing in this region should triage patients with stably elevated cTn levels in light of their increased risk.


Subject(s)
Emergency Service, Hospital , Troponin T , Humans , Male , Female , Tanzania/epidemiology , Middle Aged , Prospective Studies , Troponin T/blood , Aged , Prognosis , Adult , Biomarkers/blood , Chronic Disease , Cardiomyopathies/blood , Cardiomyopathies/epidemiology , Cardiomyopathies/mortality
16.
J Am Heart Assoc ; 13(10): e034518, 2024 May 21.
Article in English | MEDLINE | ID: mdl-38761073

ABSTRACT

BACKGROUND: Although tafamidis treatment improves prognosis in patients with wild-type transthyretin amyloid cardiomyopathy, an optimal surrogate marker monitoring its therapeutic effect remains unclear. This study investigated the association between changes in cardiac biomarkers, high-sensitivity cardiac troponin T (hs-cTnT) and B-type natriuretic peptide (BNP) during the first year after tafamidis treatment and clinical outcomes. METHODS AND RESULTS: In 101 patients with wild-type transthyretin amyloid cardiomyopathy receiving tafamidis at our institution, change in cardiac biomarkers from baseline to 1 year after tafamidis administration and its association with composite outcomes (composite of all-cause death and hospitalization attributable to heart failure) was assessed. During the follow-up period (median, 17 months), 16 (16%) patients experienced composite outcomes. The hs-cTnT level significantly decreased at 1 year after tafamidis treatment, unlike the BNP level. The frequencies of increased hs-cTnT and BNP levels were significantly higher in those with composite outcomes than in those without (44% versus 15%; P=0.01). Kaplan-Meier survival analysis showed that patients in whom both hs-cTnT and BNP levels increased at 1 year after tafamidis had a higher probability of composite outcomes compared with those with decreased hs-cTnT and BNP levels (log-rank P<0.01). Cox regression analysis identified increased hs-cTnT and BNP levels at 1 year after tafamidis administration as an independent predictor of higher cumulative risk of composite outcomes. CONCLUSIONS: Deterioration in cardiac biomarkers during the first year after tafamidis treatment predicted a worse prognosis, suggesting the utility of serial assessment of cardiac biomarkers for monitoring the therapeutic response to tafamidis in patients with wild-type transthyretin amyloid cardiomyopathy.


Subject(s)
Amyloid Neuropathies, Familial , Benzoxazoles , Biomarkers , Cardiomyopathies , Natriuretic Peptide, Brain , Troponin T , Humans , Male , Female , Biomarkers/blood , Natriuretic Peptide, Brain/blood , Aged , Amyloid Neuropathies, Familial/blood , Amyloid Neuropathies, Familial/drug therapy , Amyloid Neuropathies, Familial/mortality , Amyloid Neuropathies, Familial/diagnosis , Benzoxazoles/therapeutic use , Troponin T/blood , Cardiomyopathies/blood , Cardiomyopathies/drug therapy , Cardiomyopathies/mortality , Cardiomyopathies/diagnosis , Treatment Outcome , Time Factors , Middle Aged , Aged, 80 and over , Heart Failure/blood , Heart Failure/drug therapy , Heart Failure/mortality , Retrospective Studies , Prealbumin/metabolism
17.
J Am Coll Cardiol ; 84(1): 43-58, 2024 Jul 02.
Article in English | MEDLINE | ID: mdl-38739065

ABSTRACT

BACKGROUND: The 6-minute walk test (6MWT) represents a comprehensive functional assessment that is commonly used in patients with heart failure; however, data are lacking in patients with transthyretin cardiac amyloidosis (ATTR-CA). OBJECTIVES: This study aimed to assess the prognostic importance of the 6MWT in patients with ATTR-CA. METHODS: A retrospective analysis of patients diagnosed with ATTR-CA at the National Amyloidosis Centre who underwent a baseline 6MWT between 2011 and 2023 identified 2,141 patients, of whom 1,118 had follow-up at 1 year. RESULTS: The median baseline 6MWT distance was 347 m (Q1-Q3: 250-428 m) and analysis by quartiles demonstrated an increased death rate with each distance reduction (deaths per 100 person-years: 6.3 vs 9.2 vs 13.6 vs 19.0; log-rank P < 0.001). A 6MWT distance of <350 m was associated with a 2.2-fold higher risk of mortality (HR: 2.15; 95% CI: 1.85-2.50; P < 0.001), with a similar increased risk across National Amyloidosis Centre disease stages (P for interaction = 0.761) and genotypes (P for interaction = 0.172). An absolute (reduction of >35 m) and relative worsening (reduction of >5%) of 6MWT at 1 year was associated with an increased risk of mortality (HR: 1.80; 95% CI: 1.51-2.15; P < 0.001 and HR: 1.89; 95% CI: 1.59-2.24; P < 0.001, respectively), which was similar across the aforementioned subgroups. When combined with established measures of disease progression (N-terminal pro-B-type natriuretic peptide progression and outpatient diuretic intensification), each incremental increase in progression markers was associated with an increased death rate (deaths per 100 person-years: 7.6 vs 13.9 vs 22.4 vs 32.9; log-rank P < 0.001). CONCLUSIONS: The baseline 6MWT distance can refine risk stratification beyond traditional prognosticators. A worsening 6MWT distance can stratify disease progression and, when combined with established markers, identifies patients at the highest risk of mortality.


Subject(s)
Amyloid Neuropathies, Familial , Cardiomyopathies , Walk Test , Humans , Male , Female , Retrospective Studies , Prognosis , Walk Test/methods , Aged , Amyloid Neuropathies, Familial/mortality , Amyloid Neuropathies, Familial/physiopathology , Amyloid Neuropathies, Familial/diagnosis , Cardiomyopathies/physiopathology , Cardiomyopathies/mortality , Cardiomyopathies/diagnosis , Middle Aged , Follow-Up Studies
18.
Europace ; 26(5)2024 May 02.
Article in English | MEDLINE | ID: mdl-38657209

ABSTRACT

AIMS: Primary prevention patients with ischaemic cardiomyopathy and chronic total occlusion of an infarct-related coronary artery (CTO) are at a particularly high risk of implantable cardioverter-defibrillator (ICD) therapy occurrence. The trial was designed to evaluate the efficacy of preventive CTO-related substrate ablation strategy in ischaemic cardiomyopathy patients undergoing primary prevention ICD implantation. METHODS AND RESULTS: The PREVENTIVE VT study was a prospective, multicentre, randomized trial including ischaemic patients with ejection fraction ≤40%, no documented ventricular arrhythmias (VAs), and evidence of scar related to the coronary CTO. Patients were randomly assigned 1:1 to a preventive substrate ablation before ICD implantation or standard therapy with ICD implantation only. The primary outcome was a composite of appropriate ICD therapy or unplanned hospitalization for VAs. Secondary outcomes included the primary outcome's components, the incidence of appropriate ICD therapies, cardiac hospitalization, electrical storm, and cardiovascular (CV) mortality. Sixty patients were included in the study. During the mean follow-up of 44.7 ± 20.7 months, the primary outcome occurred in 5 (16.7%) patients undergoing preventive substrate ablation and in 13 (43.3%) patients receiving only ICD [hazard ratio (HR): 0.33; 95% confidence interval (CI): 0.12-0.94; P = 0.037]. Patients in the preventive ablation group also had fewer appropriate ICD therapies (P = 0.039) and the electrical storms (Log-rank: P = 0.01). While preventive ablation also reduced cardiac hospitalizations (P = 0.006), it had no significant impact on CV mortality (P = 0.151). CONCLUSION: Preventive ablation of the coronary CTO-related substrate in patients undergoing primary ICD implantation is associated with the reduced risk of appropriate ICD therapy or unplanned hospitalization due to VAs.


Subject(s)
Catheter Ablation , Coronary Occlusion , Defibrillators, Implantable , Myocardial Ischemia , Primary Prevention , Humans , Male , Female , Middle Aged , Aged , Coronary Occlusion/mortality , Coronary Occlusion/therapy , Coronary Occlusion/prevention & control , Coronary Occlusion/complications , Treatment Outcome , Prospective Studies , Myocardial Ischemia/complications , Myocardial Ischemia/mortality , Tachycardia, Ventricular/prevention & control , Tachycardia, Ventricular/therapy , Tachycardia, Ventricular/mortality , Cardiomyopathies/mortality , Cardiomyopathies/complications , Cardiomyopathies/therapy , Death, Sudden, Cardiac/prevention & control , Death, Sudden, Cardiac/etiology , Risk Factors , Electric Countershock/instrumentation , Electric Countershock/adverse effects , Electric Countershock/mortality , Myocardial Infarction/mortality , Myocardial Infarction/prevention & control , Myocardial Infarction/complications , Chronic Disease , Time Factors
19.
Eur Heart J ; 45(24): 2119-2129, 2024 Jun 28.
Article in English | MEDLINE | ID: mdl-38592444

ABSTRACT

BACKGROUND AND AIMS: Patients with atrial fibrillation (AF) are at increased risks of cardiovascular diseases and mortality, but risks according to age at diagnosis have not been reported. This study investigated age-specific risks of outcomes among patients with AF and the background population. METHODS: This nationwide population-based cohort study included patients with AF and controls without outcomes by the application of exposure density matching on the basis of sex, year of birth, and index date. The absolute risks and hazard rates were stratified by age groups and assessed using competing risk survival analyses and Cox regression models, respectively. The expected differences in residual life years among participants were estimated. RESULTS: The study included 216 579 AF patients from year 2000 to 2020 and 866 316 controls. The mean follow-up time was 7.9 years. Comparing AF patients with matched controls, the hazard ratios among individuals ≤50 years was 8.90 [95% confidence interval (CI), 7.17-11.0] for cardiomyopathy, 8.64 (95% CI, 7.74-9.64) for heart failure, 2.18 (95% CI, 1.89-2.52) for ischaemic stroke, and 2.74 (95% CI, 2.53-2.96) for mortality. The expected average loss of life years among individuals ≤50 years was 9.2 years (95% CI, 9.0-9.3) years. The estimates decreased with older age. CONCLUSIONS: The findings show that earlier diagnosis of AF is associated with a higher hazard ratio of subsequent myocardial disease and shorter life expectancy. Further studies are needed to determine causality and whether AF could be used as a risk marker among particularly younger patients.


Subject(s)
Atrial Fibrillation , Humans , Atrial Fibrillation/mortality , Atrial Fibrillation/epidemiology , Atrial Fibrillation/complications , Atrial Fibrillation/diagnosis , Male , Female , Middle Aged , Aged , Adult , Age Factors , Heart Failure/mortality , Heart Failure/epidemiology , Incidence , Risk Factors , Aged, 80 and over , Cardiomyopathies/mortality , Cardiomyopathies/epidemiology , Cardiomyopathies/diagnosis , Ischemic Stroke/epidemiology , Ischemic Stroke/mortality , Case-Control Studies
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