Intravenous immunoglobulins in children with new onset dilated cardiomyopathy

2017 ◽  
Vol 28 (1) ◽  
pp. 46-54 ◽  
Author(s):  
Josephine F. Heidendael ◽  
Suzanne L. Den Boer ◽  
Joanne G. Wildenbeest ◽  
Michiel Dalinghaus ◽  
Bart Straver ◽  
...  
Keyword(s):  
Dilated Cardiomyopathy ◽  
Viral Myocarditis ◽  
Intravenous Immunoglobulins ◽  
Left Ventricular ◽  
Hazard Ratio ◽  
Structural Defects ◽  
Free Survival ◽  
Systolic Left Ventricular Function ◽  
Median Period ◽  
New Onset

AbstractBackgroundDilated cardiomyopathy is a rare but serious disorder in children. No effective diagnostic or treatment tools are readily available. This study aimed to evaluate the efficacy of intravenous immunoglobulins in children with new onset dilated cardiomyopathy.Methods and resultsIn this retrospective cohort study, 94 children with new onset dilated cardiomyopathy were followed during a median period of 33 months. All patients with secondary dilated cardiomyopathy – for example, genetic, auto-immune or structural defects – had been excluded. Viral tests were performed in all patients and 18 (19%) children met the criteria for the diagnosis “probable or definite viral myocarditis”. Intravenous immunoglobulins were administered to 21 (22%) patients. Overall transplant-free survival was 75% in 5 years and did not differ between treatment groups. The treatment was associated with a higher recovery rate within 5 years, compared with non-treated children (70 versus 43%, log rank=0.045). After correction for possible confounders the hazard ratio for recovery with intravenous immunoglobulins was not significant (hazard ratio: 2.1; 95% CI: 1.0–4.6; p=0.056). Administration of intravenous immunoglobulins resulted in a greater improvement in the shortening fraction of the left ventricle.ConclusionIn our population of children with new onset dilated cardiomyopathy, of either viral or idiopathic origin, intravenous immunoglobulins were administered to a minority of the patients and did not influence transplant-free survival, but were associated with better improvement of systolic left ventricular function and with better recovery. Our results support the concept that children with new onset dilated cardiomyopathy might benefit from intravenous immunoglobulins.

Pulsus alternans in a child with dilated cardiomyopathy

2017 ◽  
Vol 28 (3) ◽  
pp. 479-481
Author(s):  
Hasti Sanandajifar ◽  
Sarosh P. Batlivala
Keyword(s):  
Dilated Cardiomyopathy ◽  
Pulse Wave ◽  
Severe Heart Failure ◽  
Clinical Information ◽  
Left Ventricular ◽  
Warning Sign ◽  
Pulse Wave Amplitude ◽  
Pulsus Alternans ◽  
Myocardial Oxygen Supply ◽  
New Onset

AbstractA previously healthy 21-month-old presented with new-onset dilated cardiomyopathy. Evaluation noted pulsus alternans, with beat-to-beat alternations in aortic pulse wave amplitude, both clinically and on diagnostic studies. Pulsus alternans is an infrequent, complex pathophysiologic sign often associated with severe heart failure. The mechanisms are incompletely understood, but theorised aetiologies include beat-to-beat changes in left ventricular loading conditions, variations in myocardial oxygen supply/demand, and alternations in myocardial contractility. Recognition of pulsus alternans is important as it provides significant clinical information, may suggest suboptimal medical management, and may be the first warning sign of severe cardiac dysfunction.

scholarly journals Substrate Characterization and Outcome of Catheter Ablation of Ventricular Tachycardia in Patients With Nonischemic Cardiomyopathy and Isolated Epicardial Scar

2021 ◽  
Author(s):  
Ioan Liuba ◽  
Daniele Muser ◽  
Anwar Chahal ◽  
Cory Tschabrunn ◽  
Pasquale Santangeli ◽  
...  
Keyword(s):  
Ventricular Tachycardia ◽  
Low Voltage ◽  
Left Ventricular ◽  
Hazard Ratio ◽  
Interquartile Range ◽  
Nonischemic Cardiomyopathy ◽  
Free Survival ◽  
Signal Abnormality ◽  
Ablation Success

Background: The substrate for ventricular tachycardia (VT) in left ventricular (LV) nonischemic cardiomyopathy may be epicardial. We assessed the prevalence, location, endocardial electrograms, and VT ablation outcomes in LV nonischemic cardiomyopathy with isolated epicardial substrate. Methods: Forty-seven of 531 (9%) patients with LV nonischemic cardiomyopathy and VT demonstrated normal endocardial (>1.5 mV)/abnormal epicardial bipolar low-voltage area (LVA, <1.0 mV and signal abnormality). Abnormal endocardial unipolar LVA (≤8.3 mV) and endocardial bipolar split electrograms and predictors of ablation success were assessed. Results: Epicardial bipolar LVA (27.3 cm 2 [interquartile range, 15.8–50.0]) localized to basal (40), mid (8), and apical (3) LV with basal inferolateral LV most common (28/47, 60%). Of 44 endocardial maps available, 40 (91%) had endocardial unipolar LVA (24.5 cm 2 [interquartile range, 9.4–68.5]) and 29 (67%) had characteristic normal amplitude endocardial split electrograms opposite the epicardial LVA. At mean of 34 months, the VT-free survival was 55% after one and 72% after multiple procedures. Greater endocardial unipolar LVA than epicardial bipolar LVA (hazard ratio, 10.66 [CI, 2.63–43.12], P =0.001) and number of inducible VTs (hazard ratio, 1.96 [CI, 1.27–3.00], P =0.002) were associated with VT recurrence. Conclusions: In patients with LV nonischemic cardiomyopathy and VT, the substrate may be confined to epicardial and commonly basal inferolateral. LV endocardial unipolar LVA and normal amplitude bipolar split electrograms identify epicardial LVA. Ablation targeting epicardial VT and substrate achieves good long-term VT-free survival. Greater endocardial unipolar than epicardial bipolar LVA and more inducible VTs predict VT recurrence.

P6577Isolated systolic hypertension versus combined systolic-diastolic hypertension as predictors of atrial fibrillation: data from a 8-year-follow-up study

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
D Konstantinidis ◽  
C Tsioufis ◽  
K Dimitriadis ◽  
A Kasiakogias ◽  
I Liatakis ◽  
...  
Keyword(s):  
Atrial Fibrillation ◽  
Systolic Hypertension ◽  
Left Ventricular Mass Index ◽  
Cox Regression ◽  
Left Ventricular ◽  
Hazard Ratio ◽  
Hypertensive Patients ◽  
Diastolic Hypertension ◽  
New Onset

Abstract Background/Introduction Isolated systolic hypertension (ISH) and combined systolic-diastolic hypertension (CH) are related with increased cardiovascular risk. Purpose The aim of the present study was to compare the predictive role of ISH and CH for the incidence of atrial fibrillation (AF) in a cohort of essential hypertensive patients. Methods We followed up 1605 essential hypertensives with office systolic blood pressure (BP)≥140 mmHg [mean age 58.1 years, 842 males, office BP=153/92 mmHg] for a mean period of 8 years. All subjects had at least one annual visit and at baseline underwent echocardiographic study and blood sampling for estimation of metabolic profile. Patients with baseline ISH exhibited office systolic BP ≥140 mmHg and office diastolic BP <90 mmHg, while those with CH had office systolic BP ≥140 mmHg and office diastolic BP ≥90 mmHg. Moreover, new-onset AF was defined as hospitalization for AF or compatible electrocardiographic tracings. Results The incidence of new-onset AF over the follow-up period was 3.4% (n=55). Patients with ISH (n=510) compared to those with CH (n=1095) were older (65±10 vs 55±11 years, p<0.0001), had at baseline lower waist circumference (95.5±12 vs 98±12 cm, p<0.0001), office systolic BP (149±10 vs 155±13 mmHg, p<0.0001), office diastolic BP (80±5 vs 98±7 mmHg, p<0.0001), while did not differ regarding left ventricular mass index and lipid levels (p=NS for all). Univariate Cox regression analysis revealed that baseline ISH (hazard ratio=4.612, p=0.013) and CH (hazard ratio=1.794, p=0.036) predicted new-onset AF. However, in multivariate Cox regression model, age (hazard ratio=1.078, p<0.001), left ventricular mass index (hazard ratio 1.012, p=0.014), left atrium diameter (hazard ratio=1.102, p<0.001) and ISH (hazard ratio=1.551, p=0.035) but not CH turned out to be independent predictors of new-onset AF episodes. Conclusions In essential hypertensive patients, ISH but not CH exhibits independent prognostic value for AF. These findings support that ISH constitutes a hypertensive phenotype of particularly increased arrhythmia risk needing careful evaluation and treatment.

scholarly journals Prognostic impact of right bundle branch block in hospitalized patients with idiopathic dilated cardiomyopathy: a single-center cohort study

2018 ◽  
Vol 48 (1) ◽  
pp. 030006051880147 ◽  
Author(s):  
Li Lai ◽  
Rong Jiang ◽  
Wei Fang ◽  
Chao Yan ◽  
Yibin Tang ◽  
...  
Keyword(s):  
Confidence Interval ◽  
Dilated Cardiomyopathy ◽  
Right Bundle Branch Block ◽  
Left Ventricular ◽  
Hazard Ratio ◽  
Hospitalized Patients ◽  
Idiopathic Dilated Cardiomyopathy ◽  
Prognostic Impact ◽  
Bundle Branch Block ◽  
All Cause Mortality

Objective Idiopathic dilated cardiomyopathy (IDCM) is a primary myocardial disease resulting in symptoms of heart failure. Right bundle branch block (RBBB) is associated with increased cardiovascular risk and all-cause mortality. Therefore, the present study was performed to identify the prognostic impact of RBBB in patients with IDCM. Methods In total, 165 hospitalized patients with IDCM were evaluated. Receiver operating characteristic curve analysis was used to determine the cutoff point, and Cox regression was used to assess risk factors. Results After a median follow-up of 73.1 months (interquartile range, 36.1–88.7 months), 59 (35.8%) patients had died. All-cause mortality was significantly higher in patients with than without RBBB (log-rank χ2 = 9.400), P<0.05. Significant independent predictors of all-cause mortality in patients with IDCM were RBBB (hazard ratio, 2.898; 95% confidence interval, 1.201–6.995) and the left ventricular end-diastolic dimension (LVEDD) (hazard ratio, 1.034; 95% confidence interval, 1.004–1.066) at admission. Patients with RBBB and an LVEDD of ≥63 mm had the highest mortality (log-rank χ2 = 14.854), P<0.05. Conclusion RBBB was an independent predictor of all-cause mortality, and the combination of RBBB and LVEDD provided more clinically relevant information than RBBB alone for assessing the risk of all-cause mortality in patients with IDCM.

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