Upgraded heart failure therapy leads to an improved outcome of dilated cardiomyopathy in infants and toddlers

2014 ◽  
Vol 25 (7) ◽  
pp. 1300-1305 ◽  
Author(s):  
Stefan Rupp ◽  
Christian Apitz ◽  
Leonie Tholen ◽  
Heiner Latus ◽  
Stefan H. Ostermayer ◽  
...  

AbstractObjectiveDilated cardiomyopathy is a leading cause of cardiac death in children. Approximately 30% of children die or need cardiac transplantation in the first year after establishing the diagnosis. New strategies are needed to improve the outcome in this high-risk patient population.Method and resultsWe present our experience in 38 patients below the age of three years, who were diagnosed with dilated cardiomyopathy and who were treated at our institution between 2006 and 2012. The treatment strategy involved institution of β-blockers and angiotensin-converting enzyme inhibitors as soon as feasible. In selected cases, pulmonary artery banding or intracoronary autologous bone marrow-derived cell therapy was performed. The median age at presentation was six months (range 1–26 months). The median follow-up age was 16 months (range 2–80 months). Kaplan–Meier analysis of survival after dilated cardiomyopathy diagnosis revealed a one-year survival of 97% and a five-year survival of 86%. The rate of freedom from death or heart transplantation was 82% at one year and 69% at five years. Surviving patients who were free of transplantation, at the follow-up at 25 months (3–80 months), showed a significant improvement in left ventricular ejection fraction (from 19±11 to 46±16%) and left ventricular end-diastolic diameter (z-score from 4.6±2.4 to 1.4±1.6). In addition, the levels of B-type natriuretic peptide improved significantly (from 3330±3840 to 171±825 pg/ml).ConclusionOur data suggest that the clinical approach described here may result in a markedly improved medium-term outcome in young children with dilated cardiomyopathy. Further studies are required to evaluate whether these approaches reduce end-points such as transplantation or death.

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
J Krejci ◽  
P Hude ◽  
H Poloczkova ◽  
E Ozabalova ◽  
D Mlejnek ◽  
...  

Abstract Introduction The most common virus in myocardium of patients with recent onset dilated cardiomyopathy (RODCM) is PVB19. Opinions on its pathogenicity are different. According to some, viral load has to be evaluated and if it is low, the PVB19 has no potential to induce inflammation. Others recommend assessment of viral replication, while “innocent” are those cases where replication activity is low. Purpose To compare the development of echocardiographic parameters in patients with RODCM with isolated presence of PVB19 in low viral load (below 500 copies per μg nucleic acid) against a group in which no virus was detected in the myocardium. Patient and methods 243 patients with RODCM, in 151 cases (62%), the virus was detected in the myocardium (PCR+ group), in 92 cases the finding was negative (PCR−; ie 38%). In the PCR+ group, PVB19 was captured in 135 cases (89% of all PCR+), in 121 cases of them was PVB19 the only one isolated virus (PCRPVB19+; 80% of all PCR+). The viral presence was assessed by real time PCR. Results In the PCR− group, the left ventricular ejection fraction (LVEF) was 23.5±7.2%, in the PCRPVB19+ group 24.6±7.1% (p=0.26). Left ventricle end-diastolic diameter (LVEDD) in PCR− was 66.4±8.9mm vs 65.5±7.9mm in PCRPVB19+ (p=0.42), right ventricle diameter (RV) 33.1±5.7mm vs. 33.4±5.2mm (p=0.69), TAPSE 19.5±4.1mm vs 18.9±4.0mm (p=0.28) and E/é 14.0±5.7 vs. 14.9±7.0 (p=0.32). In the 12-month control, LVEF in the PCR− was 35.5±12.3% (p<0.001 for comparison with baseline), in PCRPVB19+ 35.3±12.3% (p<0.001 for comparison with baseline; p=0.92 for difference between groups); LVEDD in PCR− was 62.0±9.3mm vs 62.1±9.3mm in PCRPVB19+ (both p<0.001 for comparison with baseline, p=0.91 for difference between groups), RV 31.6±5.5mm vs. 31.6±5.4mm (p=0.033, resp. p=0.003 for comparison with baseline; p=0.98 for difference between groups), TAPSE 20.7±4.3mm vs. 20.7±4.4mm (p=0.026, resp. p<0.001 for comparison with baseline; p=0.98 for difference between groups), and E/e' 10.3±4.1 vs. 10.4±4.1 (both p<0.001 for comparison with baseline; p=0.93 for difference between groups). Conclusion PVB19 presence in myocardium had no effect on the development of echocardiographic parameters in RODCM patients in one-year follow-up. Acknowledgement/Funding Supported by Ministry of Health of Czech Republic AZV Grant 16-30537A


2021 ◽  
Vol 10 (21) ◽  
pp. 4989
Author(s):  
Mohammad Abumayyaleh ◽  
Christina Pilsinger ◽  
Ibrahim El-Battrawy ◽  
Marvin Kummer ◽  
Jürgen Kuschyk ◽  
...  

Background: The angiotensin receptor-neprilysin inhibitor (ARNI) decreases cardiovascular mortality in patients with chronic heart failure with a reduced ejection fraction (HFrEF). Data regarding the impact of ARNI on the outcome in HFrEF patients according to heart failure etiology are limited. Methods and results: One hundred twenty-one consecutive patients with HFrEF from the years 2016 to 2017 were included at the Medical Centre Mannheim Heidelberg University and treated with ARNI according to the current guidelines. Left ventricular ejection fraction (LVEF) was numerically improved during the treatment with ARNI in both patient groups, that with ischemic cardiomyopathy (n = 61) (ICMP), and that with non-ischemic cardiomyopathy (n = 60) (NICMP); p = 0.25. Consistent with this data, the NT-proBNP decreased in both groups, more commonly in the NICMP patient group. In addition, the glomerular filtration rate (GFR) and creatinine changed before and after the treatment with ARNI in both groups. In a one-year follow-up, the rate of ventricular tachyarrhythmias (ventricular tachycardia and ventricular fibrillation) tended to be higher in the ICMP group compared with the NICMP group (ICMP 38.71% vs. NICMP 17.24%; p = 0.07). The rate of one-year all-cause mortality was similar in both groups (ICMP 6.5% vs. NICMP 6.6%; log-rank = 0.9947). Conclusions: This study shows that, although the treatment with ARNI improves the LVEF in ICMP and NICMP patients, the risk of ventricular tachyarrhythmias remains higher in ICMP patients in comparison with NICMP patients. Renal function is improved in the NICMP group after the treatment. Long-term mortality is similar over a one-year follow-up.


2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
F Posch ◽  
T Glantschnig ◽  
S Firla ◽  
M Smolle ◽  
M Balic ◽  
...  

Abstract Background Monitoring left-ventricular ejection fraction (LVEF) is a routinely-practiced strategy to survey patients with breast cancer (BC) towards cardiotoxic treatment effects. However, whether the LVEF as a single measurement or as a trajectory over time is truly sufficient to identify patients at high risk for cardiotoxicity is currently debated. Purpose To quantify the prognostic impact of LVEF and its change over time for predicting cardiotoxicity in women with HER2+ early BC. Methods We analyzed 1,136 echocardiography reports from 185 HER2+ early BC patients treated with trastuzumab ± chemoimmunoendocrine therapy in the neoadjuvant/adjuvant setting (Table 1). Cardiotoxicity was defined as a 10% decline in LVEF below 50%. Results Median baseline LVEF was 64% (25th-75th percentile: 60–69). Nineteen patients (10%) experienced cardiotoxicity (asymptomatic n=12, symptomatic n=7, during treatment n=19, treatment modification/termination n=14), Median time to cardiotoxicity was 6.7 months, and median LVEF decline in patients with cardiotoxicity was 18%. One-year cardiotoxicity risk was 7.6% in the 35 patients with a baseline LVEF≥60% and 24.5% in the 150 patients with a baseline LVEF<60% (Hazard Ratio (HR)=3.45, 95% CI: 1.35–8.75, Figure 1). During treatment, LVEF declined significantly faster in patients who developed cardiotoxicity than in patients without cardiotoxicity (1.3%/month vs. 0.1%/month, p<0.0001). A higher rate of LVEF decrease predicted for higher cardiotoxicity risk (HR per 0.1%/month higher LVEF decrease/month=2.50, 95% CI: 1.31–4.76, p=0.005), and cardiotoxicity risk increased by a factor of 1.7 per 5% absolute LVEF decline from baseline to first follow-up (HR=1.70, 95% CI: 1.30–2.38, p<0.0001). Thirty-six patients (19%) developed an LVEF decline of at least 5% from baseline to first follow-up (“early LVEF decline”). One-year cardiotoxicity risk was 6.8% in those without early LVEF decline and a baseline LVEF≥60% (n=117), 15.7% in those without an early LVEF decline and a baseline LVEF<60% (n=65), and 66.7% in those with an early LVEF decline and a baseline LVEF<60% (n=3), respectively (log-rank p<0.0001). Table 1. Baseline characteristics Age (years, median [IQR]) 55 [49–65] Estrogen receptor positive (n, %) 124 (67%) Neoadjuvant setting (n, %) 103 (56%) Figure 1. Risk of Cardiotoxicity. Conclusion Both a single LVEF measurement and the rate of LVEF decrease strongly predict cardiotoxicity in early BC patients undergoing HER2-targeted therapy. Routine LVEF monitoring identifies individuals at high risk of cardiotoxicity that may benefit from more sensitive screening techniques such as strain imaging.


2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
P Simonen ◽  
J Lehtonen ◽  
M Kupari

Abstract Background Sarcoidosis is characterized by the formation of inflammatory epithelioid-cell granulomas in various organs with cardiac involvement as its most ominous manifestation. A female preponderance in the prevalence of cardiac sarcoidosis (CS) is well known but other possible gender differences remain poorly studied. Purpose We set out to evaluate gender-related differences in the manifestations and long-term outcome of CS. Methods We reviewed the history, diagnostic procedures, details of treatment and outcome of 158 consecutive patients with histologically confirmed CS diagnosis between 1988 and 2017 at our hospital. Follow-up data were collected up to the end of 2018. Results The study population consisted of 51 men and 107 women (68%). At presentation, men were younger than women (mean age 47 years vs 51 years, p=0.045) and had more often a history of pre-existing extracardiac sarcoidosis (25% vs 10%, p=0.013). Isolated CS remained less common in men even after the complete diagnostic process (50% vs 75%, p=0.001). The main presenting CS manifestations were atrioventricular block, ventricular tachyarrhythmias and heart failure in 39%, 30% and 18% of men vs in 54%, 23% and 17% of women, respectively (p=0.183). Left ventricular ejection fraction at presentation averaged 49±11% in men and 49±13% in women (p=0.845). Troponin T was elevated more often in men at the presentation (46% vs 26%, p=0.024). At magnetic resonance imaging, pathological myocardial late gadolinium enhancement was observed in 87% of men and 84% of women (p=0.615). Myocardial “hot spot” at 18-F fluorodeoxyglucose positron emission tomography was also equally common (87% in men, 92% in women, p=0.468). An intracardiac cardioverter-defibrillator was implanted in 78% of men and 75% of women (p=0.693) and nearly all patients (99%, no gender difference) received immunosuppressive therapy. During the mean follow-up of 64 months, 10 of 51 men versus 30 of 107 women either died of a cardiac cause, suffered an aborted sudden cardiac death or underwent transplantation. The composite event-free survival did not differ between genders (Figure 1. Log-rank p=0.852). Conclusions Two thirds of CS patients are women. At disease presentation, women are older than men and their sarcoidosis is more often isolated to the heart but the clinical manifestations, diagnostic findings and long-term outcome are comparable in the two genders.


2009 ◽  
Vol 27 (15_suppl) ◽  
pp. 540-540
Author(s):  
M. J. Procter ◽  
T. Suter ◽  
E. de Azamuja ◽  
S. Muehlbauer ◽  
U. Dafni ◽  
...  

540 Background: The Herceptin Adjuvant (HERA) Trial is a three-group randomized trial that compared 1 year or 2 years trastuzumab with observation. We investigated cardiac dysfunction in HERA patients randomized to observation or 1 year trastuzumab and report results at a median follow-up of 3.6 years. Methods: Only patients who after completion of (neo)adjuvant chemotherapy with or without radiotherapy had normal left ventricular ejection fraction (LVEF > 55%) were eligible. Cardiac function was monitored throughout the trial. A repeat LVEF assessment was required in case of cardiac dysfunction. Results: There were 1,698 patients randomized to observation and 1,703 randomized to 1 year trastuzumab. The incidence of discontinuation of trastuzumab due to cardiac disorders was low (5.1%). The incidence of cardiac endpoints was low (severe CHF 0.77% in the trastuzumab group). The incidence of cardiac endpoints was higher in the trastuzumab group compared to observation (severe CHF 0.77% vs 0.00%; confirmed significant LVEF drops 3.57% vs 0.64%). In the trastuzumab group, there were no occurrences of severe CHF after the end of the scheduled treatment period of 1 year. Among the patients in the trastuzumab group with confirmed significant LVEF drop, the first occurrence was within the scheduled treatment period of 1 year for 55 out of 60 patients (91.7%). In the trastuzumab group, 59 of 73 patients (80.8%) with a cardiac endpoint reached acute recovery and of these 59 patients 52 (88.1%) were consider to have a favourable long term outcome. Conclusions: The incidence of cardiac endpoints remains low even with longer term follow-up. The cumulative incidence of any type of cardiac endpoint increases during the scheduled treatment period of 1 year, but appears to remain approximately constant after the scheduled treatment period of 1 year is completed. [Table: see text]


PeerJ ◽  
2018 ◽  
Vol 6 ◽  
pp. e5312 ◽  
Author(s):  
Chih-Yuan Fang ◽  
Huang-Chung Chen ◽  
Yung-Lung Chen ◽  
Tzu-Hsien Tsai ◽  
Kuo-Li Pan ◽  
...  

BackgroundThe use of an implantable cardioverter-defibrillator (ICD) has been established as an effective secondary prevention strategy for ventricular tachycardia (VT)/ventricular fibrillation (VF). However, few reports discuss the difference in clinical predictors for recurrent VT/VF between patients with ischemic cardiomyopathy (ICM) and patients with dilated cardiomyopathy (DCM).MethodsFrom May 2004 to December 2015, 132 consecutive patients who had ICM (n= 94) or DCM (n= 38) and had received ICD implantation for secondary prevention were enrolled in this study. All anti-tachycardia events during follow-up were validated. The clinical characteristics and echocardiographic parameters were obtained for comparison. The incidence of recurrence of VT/VF, cardiovascular mortality, all-cause mortality, the change of left ventricular ejection fraction (LVEF) and LV volume were analyzed.ResultsAt a mean follow-up of 3.62 ± 2.93 years, 34 patients (36.2%) in the ICM group and 22 patients (57.9%) in the DCM group had a recurrence of VT/VF episodes (p= 0.032). The DCM group had a lower LVEF (p= 0.019), a larger LV end-diastolic volume (LVEDV) (p= 0.001), a higher prevalence of LVEDV >158 mL (p= 0.010), and a larger LV end-systolic volume (p= 0.010) than the ICM group. LVEDV >158 mL and no use of angiotensin-converting-enzyme inhibitor/angiotensin receptor blocker were independent predictors of recurrences of VT/VF in ICM patients but not in DCM patients. There were no difference in cardiovascular mortality and all-cause mortality between the ICM and DCM patients.ConclusionThe DCM patients had a higher recurrence rate of VT/VF than did the ICM patients during long-term follow-up. An enlarged LV is an independent predictor of the recurrence of VT/VF in ICM patients receiving ICD for secondary prevention.


2021 ◽  
Vol 23 (Supplement_G) ◽  
Author(s):  
Vincenzo Nuzzi ◽  
Anne Raafs ◽  
Paolo Manca ◽  
Michiel T.h.M. Henkens ◽  
Caterina Gregorio ◽  
...  

Abstract Aims Left atrial (LA) dilation is associated a with worse prognosis in several cardiovascular settings but therapies can promote LA reverse remodelling. Characterizing and defining the prognostic implications of LA volume (LAVI) reduction in dilated cardiomyopathy (DCM). Methods and results Consecutive DCM patients from two tertiary care centres, with available echocardiography at baseline and at 1 year follow-up, were analysed. LA dilation was defined as LAVI &gt;34 ml/m2, Delta (Δ)LAVI was defined as the 1 year relative LAVI reduction. The outcome was a composite of death/heart transplantation/heart failure hospitalization (D/HTx/HFH). Five hundred sixty patients were included [age 52 ± 13 years; left ventricular ejection fraction (LVEF) 31 ± 10%, LAVI 45 ± 18 ml/m2]. Baseline LAVI had a non-linear association with the risk of D/HTx/HFH, independently from LVEF (P &lt; 0.001). At 1 year follow-up, LAVI decreased in 374 patients (67%, median ΔLAVI 24%, interquartile range 37% 11%). Factors independently associated with ΔLAVI were higher baseline LAVI and lower baseline LVEF. After adjustment for confounders, ΔLAVI showed a linear association with the risk of D/HTx/HHF (HR: 0.92, 95% CI: 0.86 0.99 per 5% decrease, P &lt; 0.001). At 1 year Follow-up, patients with a ≥ 15% reduction in ΔLAVI or LAVI normalization (i.e. Follow-up LAVI ≤34ml/m2) (42% of the cohort) were at lower risk of D/HTx/HFH (HR: 0.49, 95% CI: 0.33 0.74, P &lt; 0.001). Conclusions In a large cohort of DCM, 1 year reduction in LAVI is observed in the majority of patients. The association between reduction in LAVI and D/HTx/HHF candidates LA reverse remodelling as complementary early therapeutic goal in DCM.


2021 ◽  
Vol 23 (Supplement_G) ◽  
Author(s):  
Vincenzo Nuzzi ◽  
Antonio Cannatà ◽  
Paolo Manca ◽  
Caterina Gregorio ◽  
Giulia Barbati ◽  
...  

Abstract Aims Diuretics in heart failure (HF) are commended to relieve symptoms at lowest dosage effective. Dilated cardiomyopathy (DCM) is a particular HF setting with several variables that may influence disease trajectory. We aimed to assess the long-term use of diuretics in DCM, the possibility of withdrawal and to explore the prognostic correlations. Methods and results All consecutive DCM patients enrolled from 1990 to 2018 were considered eligible. All the patients had available the information about the furosemide-equivalent dose at baseline and at follow-up evaluation within 24 months. Patients were categorized in stable (diuretic dose variation &lt;50%), increasers (diuretics dose increase ≥50% or initiation of diuretic therapy), and decreasers (diuretics dose decrease ≥50% or never prescribed diuretics in the 24-months observation period). The prognostic role of the diuretics trajectory group was assessed with Kaplan Meier analysis and with a time-dependent multivariable model. The outcome measure was a composite of all-cause death/heart transplantation/HF hospitalization (ACD/HTx/HFH). 908 patients were included [mean age 50 ± 16, 70% male sex, 24% NYHA class III or IV, mean left ventricular ejection fraction (LVEF) 31 ± 9%, 66% treated with diuretics at baseline]. The furosemide-equivalent dose at enrolment had a linear association with the risk of outcome. Compared to other groups, decreaser patients were younger, had less HF symptoms, higher LVEF and more dilated left atrium. Decreasers had a lower prescription rate of diuretics and less frequent indication to renin-angiotensin inhibitors and mineralocorticoid receptors antagonists. Over a median follow-up of 122 (62–195) months decreasers had the lowest incidence of outcome, followed by stable, while increasers had the worst outcome (P &lt; 0.001). After adjustment for other prognosticators, compared to stable patients, decreasers had a reduced risk of ACD/HTx/HFH [HR: 0.497 (95% CI: 0.337–0.731)] while increasers had the highest risk of adverse outcome [HR: 2.027 (95% CI: 1.254–3.276)]. Similarly, amongst patients taking diuretics at baseline, the diuretics withdrawal was in independent outcome predictor. The only multivariable predictors of diuretics withdrawal were younger age and lower furosemide-equivalent dose at enrolment. Conclusions In DCM patients the diuretics dose at baseline is a strong prognosticator. Diuretics dose reduction or its withdrawal provides a prognostic benefit on hard outcome. Diuretics tapering in selected patients should be considered in the short-term follow-up to improve DCM prognosis.


EP Europace ◽  
2020 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
K Yazaki ◽  
K Ejima ◽  
M Kanai ◽  
S Kataoka ◽  
S Higuchi ◽  
...  

Abstract Funding Acknowledgements None Background Atrial fibrillation (AF) ablation has been known to contribute to a good prognosis in heart failure patients and improve their systolic function. However, the impact of the post-procedural systolic function on the prognosis in them remains unclear.  Purpose To investigate the impact of the left ventricular ejection fraction (LVEF) following AF ablation in patients with systolic dysfunction.  Methods Out of 1078 consecutive patients who underwent AF ablation including extensive pulmonary vein and superior vena cava isolation, 170 with an impaired pre-procedural LVEF (&lt; 50%) were evaluated. They experienced at least one echocardiographic follow-up within one year after the index procedure. The primary outcome was the composite of all-cause death or heart failure hospitalisations (HFHs). In addition, we categorised the patients into three groups according to the post-procedural LVEF within one year to evaluate the outcome: reduced LVEF (rEF, LVEF &lt; 40%), mid-range EF (mrEF, 40% ≤ LVEF &lt; 50%) and preserved LVEF (pEF, LVEF &gt; 50%).  Results After the index procedure, the patients’ LVEF improved with an average increase of 8%, and the post-procedural LVEF consisted of an rEF in 27 (16%), mrEF in 41 (24%), and pEF in 102 (60%) patients. During a median follow-up of 31 months, a total of 22 (13%) patients experienced the composite outcome, including 18 (11%) HFHs and 10 (6%) all-cause deaths (5 with cardiac issues, 2 any malignancies, and 3 other issues). In the Kaplan-Meier analysis using a Bonferroni correction, there was a significant difference in achieving the outcome between the rEF and mrEF, and rEF and pEF, but not between the mrEF and pEF groups (Figure). In a univariate analysis, the hazard ratio of the outcome was shown as follows: an age ≥ 65 years (hazard ratio, HR: 3.4 [95% confidence interval, CI: 1.4–8.5], p = 0.006), history of HFHs for AF (HR: 1.7 [95%CI: 0.7–4.0], p = 0.25), known underlying heart disease (HR: 1.9 [95%CI: 0.8–1.2], p = 0.13), pre-procedural LVEF &lt; 40% (HR: 3.1 [95%CI: 1.3–7.5], p = 0.009), atrial tachyarrhythmia recurrence (HR: 3.0 [95%CI: 1.2–7.8], p = 0.01), and the post-procedural LVEF category (mrEF and rEF, compared with pEF) (HR: 2.0 [95%CI: 0.4–7.7], p = 0.34; and HR: 8.6 [95%CI: 2.7–37.5], p &lt; 0.0001). Furthermore, in a multivariate analysis, patients with a rEF was the sole independent predictor of the composite outcome after adjusting for confounders including an age≥65 years and pre-procedural LVEF &lt; 40% (HR: 12.0 [95%CI: 3.9–40.0], p &lt; 0.0001), whereas those with a mrEF was not (HR: 1.8 [95%CI: 0.4–7.3], p = 0.42), as compared to those with a pEF.  Conclusions Patients with a mrEF had a comparable prognosis to those with a pEF in a relatively long follow-up, while those with a rEF had the poorest outcome of the three categories, regardless of the pre-procedural LVEF severity. Abstract Figure. The difference in the rate of outcome


Heart ◽  
2021 ◽  
pp. heartjnl-2021-319504
Author(s):  
Marco Merlo ◽  
Marco Masè ◽  
Andrew Perry ◽  
Eluisa La Franca ◽  
Elena Deych ◽  
...  

ObjectivePatients with non-ischaemic dilated cardiomyopathy (NICM) may experience a normalisation in left ventricular ejection fraction (LVEF). Although this correlates with improved prognosis, it does not correspond to a normalisation in the risk of death during follow-up. Currently, there are no tools to risk stratify this population. We tested the hypothesis that absolute global longitudinal strain (aGLS) is associated with mortality in patients with NICM and recovered ejection fraction (LVEF).MethodsWe designed a retrospective, international, longitudinal cohort study enrolling patients with NICM with LVEF <40% improved to the normal range (>50%). We studied the relationship between aGLS measured at the time of the first recording of a normalised LVEF and all-cause mortality during follow-up. We considered aGLS >18% as normal and aGLS ≥16% as of potential prognostic value.Results206 patients met inclusion criteria. Median age was 53.5 years (IQR 44.3–62.8) and 56.6% were males. LVEF at diagnosis was 32.0% (IQR 24.0–38.8). LVEF at the time of recovery was 55.0% (IQR 51.7–60.0). aGLS at the time of LVEF recovery was 13.6%±3.9%. 166 (80%) and 141 (68%) patients had aGLS ≤18% and <16%, respectively. During a follow-up of 5.5±2.8 years, 35 patients (17%) died. aGLS at the time of first recording of a recovered LVEF correlated with mortality during follow-up (HR 0.90, 95% CI 0.91 to 0.99, p=0.048 in adjusted Cox model). No deaths were observed in patients with normal aGLS (>18%). In unadjusted Kaplan-Meier survival analysis, aGLS <16% was associated with higher mortality during follow-up (31 deaths (22%) in patients with GLS <16% vs 4 deaths (6.2%) in patients with GLS ≥16%, HR 3.2, 95% CI 1.1 to 9, p=0.03).ConclusionsIn patients with NICM and normalised LVEF, an impaired aGLS at the time of LVEF recovery is frequent and associated with worse outcomes.


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