Late gadolinium enhancement role in arrhythmic risk stratification of patients with LMNA cardiomyopathy: results from a long-term follow-up multicentre study

EP Europace ◽  
2020 ◽  
Vol 22 (12) ◽  
pp. 1864-1872 ◽  
Author(s):  
Giovanni Peretto ◽  
Andrea Barison ◽  
Cinzia Forleo ◽  
Chiara Di Resta ◽  
Antonio Esposito ◽  
...  

Abstract Aims We aimed at addressing the role of late gadolinium enhancement (LGE) in arrhythmic risk stratification of LMNA-associated cardiomyopathy (CMP). Methods and results We present data from a multicentre national cohort of patients with LMNA mutations. Of 164 screened cases, we finally enrolled patients with baseline cardiac magnetic resonance (CMR) including LGE sequences [n = 41, age 35 ± 17 years, 51% males, mean left ventricular ejection fraction (LVEF) by echocardiogram 56%]. The primary endpoint of the study was follow-up (FU) occurrence of malignant ventricular arrhythmias [MVA, including sustained ventricular tachycardia (VT), ventricular fibrillation, and appropriate implantable cardioverter-defibrillator (ICD) therapy]. At baseline CMR, 25 subjects (61%) had LGE, with non-ischaemic pattern in all of the cases. Overall, 23 patients (56%) underwent ICD implant. By 10 ± 3 years FU, eight patients (20%) experienced MVA, consisting of appropriate ICD shocks in all of the cases. In particular, the occurrence of MVA in LGE+ vs. LGE− groups was 8/25 vs. 0/16 (P = 0.014). Of note, no significant differences between LGE+ and LGE− patients were found in currently recognized risk factors for sudden cardiac death (male gender, non-missense mutations, baseline LVEF <45% and non-sustained VT), all P-value >0.05. Conclusions In LMNA-CMP patients, LGE at baseline CMR is significantly associated with MVA. In particular, as suggested by this preliminary experience, the absence of LGE allowed to rule-out MVA at 10 years mean FU.

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
S Persampieri ◽  
M Bergonti ◽  
A Gasperetti ◽  
M.L Narducci ◽  
F Perna ◽  
...  

Abstract Introduction Myocarditis is a complex inflammatory disease, usually secondary to viral infections or immune system dysregulation, with extremely heterogeneous clinical manifestations. Among them, potentially life-threatening ventricular arrhythmias (VA) may present at any stage of the disease as an expression of myocardial electrical instability. Purpose Our aim was to evaluate the efficacy of radiofrequency catheter ablation (RFCA) of VA in our large cohort of myocarditis, trying to understand the predictors of RFCA success. Methods and results 144 patients (61 men; age 43 [29–54] years) with history of myocarditis with arrhythmic presentation (118 biopsy-proven, 82%) composed our population. At presentation, 26% of patients suffered of ventricular tachycardia (VT) while in 17% cardiac arrest occurred: overall 49 patients (35%) were implanted with an ICD. The median left ventricular ejection fraction (LVEF) was 58% (48–61%). An intensive non-invasive and invasive work-up was performed: 104 patients underwent cardiac magnetic resonance (CMR) that showed late gadolinium enhancement (LGE) in 67 of them (63%). In 37 patients LGE was found in the anteroseptal portion of the left ventricle: this pattern showed association with major arrhythmic relapse (VT and ventricular fibrillation) during follow up (Fig. 1; OR 4.0, CI 95% 1.14–14.1, p=0.03). 95 patients underwent endocardial RFCA, using contact electroanatomic mapping. Interestingly, in patients with anteroseptal LGE RCFA didn't affect significantly the arrhythmic relapse (OR 5, CI 95% 0.9–33, p=0.06). Otherwise RFCA prevent arrhythmic relapse in patients that showed LGE in ventricular portion other than the anteroseptal one (OR 0.027, IC 95% 0.002–0.40, p<0.01). During a median follow-up of 735 days (418–2168) 6 deaths occurred: logistic regression on all-cause death showed LVEF and VT at presentation as the only independent predictors for mortality (p=0.01). Conclusions In myocarditis patients with VA, LGE pattern predicts arrhythmic relapse during follow-up. RFCA success rate is strictly linked to scar location, being significantly higher in patients with non-anteroseptal LGE. Figure 1 Funding Acknowledgement Type of funding source: None


Circulation ◽  
2008 ◽  
Vol 118 (suppl_18) ◽  
Author(s):  
Alfonso Valle ◽  
Mercedes Nadal ◽  
Jordi Estornell ◽  
Nieves Martinez ◽  
Miguel Corbi ◽  
...  

The identification of prognostic markers in patients with heart failure of both ischemic and non ischemic etiology is an increasing need in the era of devices therapy. Risk stratification for sudden cardiac death (SCD) remains problematic with reliance on left ventricular function which predicts total mortality rather than arrhythmic events (AE). Recently cardiac magnetic resonance was employed to predict susceptibility for malignant arrhythmias. This study sought to determine the utility of late gadolinium enhancement (LGE) to predict AE. Three hundred consecutive patients with symptomatic heart failure and systolic dysfunction of both ischemic and non ischemic cause undergoing CMR, were classified into two groups attending to the presence (n 160) or absence of LGE (n 140), and were followed prospectively during 842 days. The primary endpoint was the combined of SCD or Ventricular tachycardia (VT). 23 patients had AE (8 SCD/15 VT) during the follow-up, 19 of them presenting LGE (83%). The presence of LGE was associated to a significantly higher AE rate (11.8.% vs 2.8% p< 0.001)(figure ). Compared to patients without LGE, midwall fibrosis and an ischemic pattern of LGE predicted AE. (3% vs 5% vs 14%, p= 0.001) LGE is a new non-invasive predictor of AE in patients with heart failure and systolic dysfunction. This suggest a potential role for risk stratification and better selection of patients who needs device therapy


2021 ◽  
Vol 22 (Supplement_2) ◽  
Author(s):  
J Gavara ◽  
V Marcos-Garces ◽  
C Rios-Navarro ◽  
MP Lopez-Lereu ◽  
JV Monmeneu ◽  
...  

Abstract Funding Acknowledgements Type of funding sources: Public grant(s) – EU funding. Main funding source(s): This work was supported by “Instituto de Salud Carlos III” and “Fondos Europeos de Desarrollo Regional FEDER” Background. Cardiovascular magnetic resonance (CMR) is the best tool for left ventricular ejection fraction (LVEF) quantification, but as yet the prognostic value of sequential LVEF assessment for major adverse cardiac event (MACE) prediction after ST-segment elevation myocardial infarction (STEMI) is uncertain. Purpose. We explored the prognostic impact of sequential assessment of CMR-derived LVEF after STEMI to predict subsequent MACE. Methods. We recruited 1036 STEMI patients in a large multicenter registry. LVEF (reduced [r]: &lt;40%; mid-range [mr]: 40-49%; preserved [p]: ≥50%) was sequentially quantified by CMR at 1 week and after &gt;3 months of follow-up. MACE was regarded as cardiovascular death or re-admission for acute heart failure after follow-up CMR. Results. During a 5.7-year mean follow-up, 82 MACE (8%) were registered. The MACE rate was higher only in patients with LVEF &lt; 40% at follow-up CMR (r-LVEF 22%, mr-LVEF 7%, p-LVEF 6%; p-value &lt; 0.001). Based on LVEF dynamics from 1-week to follow-up CMR, incidence of MACE was 5% for sustained LVEF³40% (n = 783), 13% for improved LVEF (from &lt;40 to ³40%, n = 96), 21% for worsened LVEF (from ³40% to &lt;40%, n = 34) and 22% for sustained LVEF &lt;40% (n = 100), p-value &lt; 0.001. Using a Markov approach that considered all studies performed, transitions towards improved LVEF predominated and only r-LVEF (at any time assessed) was significantly related to higher incidence of subsequent MACE. Conclusions. LVEF constitutes a pivotal CMR index for simple and dynamic post-STEMI risk stratification. Detection of reduced LVEF (&lt;40%) by CMR at any time during follow-up identifies a small subset of patients at high risk of subsequent events.


2020 ◽  
Vol 41 (18) ◽  
pp. 1733-1743 ◽  
Author(s):  
Lili Zhang ◽  
Magid Awadalla ◽  
Syed S Mahmood ◽  
Anju Nohria ◽  
Malek Z O Hassan ◽  
...  

Abstract Aims Myocarditis is a potentially fatal complication of immune checkpoint inhibitors (ICI). Sparse data exist on the use of cardiovascular magnetic resonance (CMR) in ICI-associated myocarditis. In this study, the CMR characteristics and the association between CMR features and cardiovascular events among patients with ICI-associated myocarditis are presented. Methods and results From an international registry of patients with ICI-associated myocarditis, clinical, CMR, and histopathological findings were collected. Major adverse cardiovascular events (MACE) were a composite of cardiovascular death, cardiogenic shock, cardiac arrest, and complete heart block. In 103 patients diagnosed with ICI-associated myocarditis who had a CMR, the mean left ventricular ejection fraction (LVEF) was 50%, and 61% of patients had an LVEF ≥50%. Late gadolinium enhancement (LGE) was present in 48% overall, 55% of the reduced EF, and 43% of the preserved EF cohort. Elevated T2-weighted short tau inversion recovery (STIR) was present in 28% overall, 30% of the reduced EF, and 26% of the preserved EF cohort. The presence of LGE increased from 21.6%, when CMR was performed within 4 days of admission to 72.0% when CMR was performed on Day 4 of admission or later. Fifty-six patients had cardiac pathology. Late gadolinium enhancement was present in 35% of patients with pathological fibrosis and elevated T2-weighted STIR signal was present in 26% with a lymphocytic infiltration. Forty-one patients (40%) had MACE over a follow-up time of 5 months. The presence of LGE, LGE pattern, or elevated T2-weighted STIR were not associated with MACE. Conclusion These data suggest caution in reliance on LGE or a qualitative T2-STIR-only approach for the exclusion of ICI-associated myocarditis.


EP Europace ◽  
2020 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
V Pooranachandran ◽  
A Mistry ◽  
Z Vali ◽  
X Li ◽  
B Sidhu ◽  
...  

Abstract Funding Acknowledgements None Introduction Myocardial fibrosis detected using late gadolinium enhancement(LGE) on cardiac magnetic resonance(CMR) imaging holds prognostic value in dilated cardiomyopathy(DCM). Recent reports have demonstrated the localisation of LGE to be promising predictors of ventricular arrhythmic (VA).Aim: To determine the localisation of LGE associated with high risk of VA in DCM patients. Methods: Retrospective review of consecutive DCM patients(n = 85) implanted with an implantable cardioverter defibrillator(ICD) at a single tertiary centre between 2011-2018. All patients with insufficient follow-up data, cardiac channelopathies, primary valvular pathology and congenital heart disease were excluded from analysis(n = 11). Details of VA occurrence were obtained from medical and pacing notes. VA was defined as VA causing haemodynamic compromise or appropriate device therapy (anti-tachycardia pacing/shock). Localisation of LGE was defined as midwall, patchy, subepicardial or transmural. Left ventricular ejection fraction(LVEF) &lt;35% was defined as severely impaired function. Results:74 DCM patients implanted with an ICD were identified for analysis; LGE was observed in 18(60%) VA and 29(66%) non-VA patients(p = 0.6). There was no observed difference in mean age for patients with and without LGE (68 ± 10 vs. 65 ± 10 years,p = 0.07). A significant difference was seen between localisation and VA (p = 0.04), with patchy LGE demonstrating a higher arrhythmic risk(p = 0.005). There was no association between LVEF and LGE(p = 0.2) however, a significant difference was seen in LVEF and arrhythmic risk, with a more severely impaired LV function seen in patients without VA(p = 0.01). Conclusion:This study has demonstrated a patchy LGE localisation to be strongly associated with ventricular arrhythmia in DCM. Whilst this is a valuable tool in risk stratification, a prospective study with a larger population is required to confirm the validity of this finding. Moreover, an additional method will need to be considered to identify high risk patients without LGE. Ventricular Arrhythmia (n = 30) No Ventricular Arrhythmia (n = 44) P Value Male(%) 20(67%) 24(55%) p = 0.29 Age(Mean ± SD) 65 ± 12 65 ± 10 p = 0.36 LGE Midwall 10(56%) 24(83%) p = 0.04 Subepicardial 1(5.5%) 2(7%) p = 0.85 Transmural 1(5.5%) 2(7%) p = 0.85 Patchy 6(33%) 1(3%) p = 0.005 LVEF &lt;35% 23(77%) 42(95%) p = 0.01


Open Heart ◽  
2018 ◽  
Vol 5 (2) ◽  
pp. e000923
Author(s):  
Clara Gomes ◽  
Caíque Bueno Terhoch ◽  
Silvia Moreira Ayub-Ferreira ◽  
Germano Emilio Conceição-Souza ◽  
Vera Maria Cury Salemi ◽  
...  

ObjectivesThe prognostic significance of transient use of inotropes has been sufficiently studied in recent heart failure (HF) populations. We hypothesised that risk stratification in these patients could contribute to patient selection for advanced therapies.MethodsWe analysed a prospective cohort of adult patients admitted with decompensated HF and ejection fraction (left ventricular ejection fraction (LVEF)) less than 50%. We explored the outcomes of patients requiring inotropic therapy during hospital admission and after discharge.ResultsThe study included 737 patients, (64.0% male), with a median age of 58 years (IQR 48–66 years). Main aetiologies were dilated cardiomyopathy in 273 (37.0%) patients, ischaemic heart disease in 195 (26.5%) patients and Chagas disease in 163 (22.1%) patients. Median LVEF was 26 % (IQR 22%–35%). Inotropes were used in 518 (70.3%) patients. In 431 (83.2%) patients, a single inotrope was administered. Inotropic therapy was associated with higher risk of in-hospital death/urgent heart transplant (OR=10.628, 95% CI 5.055 to 22.344, p<0.001). At 180-day follow-up, of the 431 patients discharged home, 39 (9.0%) died, 21 (4.9%) underwent transplantation and 183 (42.4%) were readmitted. Inotropes were not associated with outcome (death, transplant and rehospitalisation) after discharge.ConclusionsInotropic drugs are still widely used in patients with advanced decompensated HF and are associated with a worse in-hospital prognosis. In contrast with previous results, intermittent use of inotropes during hospitalisation did not determine a worse prognosis at 180-day follow-up. These data may add to prognostic evaluation in patients with advanced HF in centres where mechanical circulatory support is not broadly available.


2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
T Nabeta ◽  
S Ishii ◽  
Y Ikeda ◽  
K Maemura ◽  
T Oki ◽  
...  

Abstract Background Re-worsening left ventricular ejection fraction (LVEF) after initial recovery occurs in some patients with dilated cardiomyopathy (DCM). However, prevalence and predictors of re-worsening LVEF in longitudinal follow-up are unclear. Late gadolinium enhancement of cardiovascular magnetic resonance (LGE-CMR) can evaluate the damage of myocardial tissue. Purpose This study sought to evaluate the clinical parameters including LGE-CMR to predict re-worsening LVEF in patients with recent-onset DCM. Methods We included patients with recent-onset DCM who had an LVEF &lt;45% and underwent LGE-CMR at diagnosis. We performed yearly echocardiographc follow-up [median 6 [4–8.3] years]. Initial LVEF recovery defined as patients increased in &gt;5% LVEF from baseline and had an LVEF≥45% after medical therapy. Patients were divided into three groups: (1) Improved: defined as those with sustained LVEF ≥45% after initial LVEF recovery; (2) Re-worse: those with decreased &gt;5% and had an LVEF &lt;45% after initial LVEF recovery. and (3) Not-improved: those with no initial LVEF recovery during follow-up. To evaluate the prognostic factors for Re-worsening LVEF after initial LVEF recovery, multivariate logistic regression analysis performed between the Improved group and the Re-worse group. Cardiac events defined as hospitalization due to heart failure and sudden death. Results Of 138 patents, 82 patients (59%) were the Improved group, 42 patients (30%) were the Re-worse group, and 14 (10%) were the Not-improved group. Loess curves of long-term LVEF trajectories showed that LVEF in the Re-worse group increased first 2 years and declined slowly thereafter (Fig. 1A). Re-worsening LVEF occurred 4.5±2.2 years after initial LVEF recovery. Multivariate logistic regression analysis demonstrated that LGE area at baseline (Odds ratio: 1.09, 95% confidence interval (CI) 1.02–1.18, p=0.014) and Log brain natriuretic peptide (BNP) at initial LVEF recovery (Odds ratio: 1.53, 95% confidence interval (CI) 1.01–2.31, p=0.042) were independent predictors for Re-worsening LVEF. Kaplan Meier analysis demonstrated that the risk of cardiac events in the Re-worse group was significantly higher (hazard ratio: 3.93, 95% CI 1.49–10.36, p=0.006) than in the Improved group and lower risk than in the Not-improved group (hazard ratio: 0.28, 95% CI 0.12–0.62, p=0.002) (Fig. 1B). Conclusion Re-worsening LVEF occurred in 30% of patients in patients with recent-onset DCM. LGE area and BNP at initial LVEF recovery were independently associated with re-worsening LVEF after initial LVEF recovery. Figure 1 Funding Acknowledgement Type of funding source: None


Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 149-149 ◽  
Author(s):  
Peter McSweeney ◽  
Daniel Furst ◽  
Leslie Crofford ◽  
Kevin McDonagh ◽  
Keith Sullivan ◽  
...  

Abstract Objective To evaluate long-term outcomes after HDIT and transplantation of autologous CD34+ hematopoietic progenitor cells in severe SSc. Methods: Eligibility required early (<= 4 years) diffuse SSc (modified Rodnan skin score [mRSS] of > 15) together with involvement of lungs, heart or kidneys (estimated median 5 year survival <= 50%). Pulmonary SSc was the most frequent indication for study inclusion. PBSC were obtained by G-CSF mobilization and CD34-selected with a Baxter Isolex 300i system. HDIT included total body irradiation 800 Gy (with lung shielding of the last 25 pts), cyclophosphamide 120 mg/kg and equine anti-thymocyte globulin 90 mg/kg. Follow-up included annual history and physical exams with complete workup for visceral involvement and questionnaires of overall function. Results: Of 33 pts (median mRSS = 30) follow-up includes 25 patients at one year, 19 pts at two years, 13 pts at three years and 5 pts at four years. Progression was defined as further loss of organ function or use of immunosuppressive therapy after HDIT. Ten pts died of which 5 were due to disease progression and 5 to transplant complications. Estimated 3-year overall and progression-free survivals are 79% (95% CI 65–93%) and 52% (95% CI 33–72%), respectively. Three late deaths from progression occurred at 1343, 1511 and 1801 days after HDIT. Four pts are alive with progressive disease. At 1 and 3 years after HDIT there were significant improvements in skin score and function (Table) with lung function indices overall remaining stable. Small increases in serum creatinine and decreases in the left ventricular ejection fraction were found. Five pts developed renal insufficiency and 2 required dialysis. Conclusions: HDIT appears to be a promising therapy for high-risk SSc pts but limitations include transplant toxicities and disease progression in some pts. To more clearly define the role of HDIT in severe SSc, a NIH-supported randomized multicenter study has been initiated in North America to compare HDIT against 12 doses of monthly intravenous cyclophosphamide at 750 mg/m2. Changes at 1 and 3 years after HDIT* Baseline 1 year p value 3 years p value *Values are means and mean changes from baseline. HAQ - health assessment questionnaire; DLCO- carbon monoxide diffusing capacity; FVC-forced vital capacity Skin (mRSS) 30.3 (n=33) −14.8 (n=24) p<0.0001 −23.3 (n=10) p<0.0001 HAQ (function) 1.84 (n=28) −1.06 (n=21) p<0.0001 −1.34 (n=10) p<0.0001 DLCO adj (%) 60.7 (n=33) −5.96 (n=25) p=0.01 −3 (n=13) p=0.56 FVC (%) 71.6 (n=33) +3.44 (n=25) p=0.02 +3.07 (n=13) p=0.05 Se. Creatinine (mg/dL) 0.75 (n=33) +0.30 (n=23) p=0.11 +0.15 (n=13) p=0.05 Ejection Fraction (%) 62.4 (n=30) −2.3 (n=18) p=0.16 −2.3 (n=7) p= 0.06


2020 ◽  
Vol 13 (9) ◽  
Author(s):  
Théo Pezel ◽  
Francesca Sanguineti ◽  
Marine Kinnel ◽  
Valentin Landon ◽  
Guillaume Bonnet ◽  
...  

Background: Patients with heart failure with reduced ejection fraction (HFrEF; heart failure with reduced left ventricular ejection fraction <40%) referred for stress cardiovascular magnetic resonance (CMR) may have a less optimal hemodynamic response to intravenous vasodilator. The aim was to assess the prognostic value of vasodilator stress perfusion CMR in patients with HFrEF. Methods: Between 2008 and 2018, consecutive patients with HFrEF defined by left ventricular ejection fraction <40% prospectively referred for vasodilator stress perfusion CMR were followed for the occurrence of major adverse cardiovascular events (MACE), defined by cardiovascular death or nonfatal myocardial infarction. Univariable and multivariable Cox regressions were performed to determine the prognostic value of inducible ischemia or late gadolinium enhancement by CMR. Results: Of 1053 patients with HFrEF (65±11 years, median [interquartile range] left ventricular ejection fraction 38.7% [37.2–39.0]), 1018 (97%) completed the CMR protocol and 950 (93%) completed the follow-up (median [interquartile range], 5.6 [3.6–7.3] years); 117 experienced a MACE (12.3%). Stress CMR was well tolerated without any adverse events. Patients without ischemia or late gadolinium enhancement experienced a lower annual event rate of MACE (1.8%) than those with both ischemia and late gadolinium enhancement (12.0%; P <0.001). Using Kaplan-Meier analysis, inducible ischemia and late gadolinium enhancement were significantly associated with the occurrence of MACE (hazard ratio, 2.46 [95% CI, 1.69–3.60]; and hazard ratio, 2.92 [95% CI, 1.77–4.83], respectively, both P <0.001). In multivariable Cox regression, inducible ischemia was an independent predictor of a higher incidence of MACE (hazard ratio, 2.26 [95% CI, 1.52–3.35]; P <0.001). Conclusions: Stress CMR is safe and has a good discriminative prognostic value to predict the occurrence of MACE in patients with HFrEF.


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