Outcomes, Arrhythmic Burden and Ambulatory Monitoring of Pediatric Patients With Left Ventricular Non-Compaction and Preserved Left Ventricular Function

Czosek, Richard J.; Spar, David S.; Khoury, Philip R.; Anderson, Jeffrey B.; Wilmot, Ivan; Knilans, Timothy K.; Jefferies, John L.

doi:10.1016/j.amjcard.2015.01.024

Cited by 21 publications

(7 citation statements)

References 14 publications

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“…Our results are also consistent with findings in patients with x‐linked dilated cardiomyopathy and dystrophin defects where there is a high risk of progression to heart failure, but a low risk of life‐threatening arrhythmias . This is also consistent with recent data from other types of pediatric dilated cardiomyopathy where arrhythmia burden worsens as systolic function decreases . Thus, while CMR may be of use for the many reasons described within the NHLBI/PPMD Working Group findings, our data suggest that obtaining a CMR solely to assess the presence of LGE in order to help gauge arrhythmia risk may have more limited utility.…”

Section: Discussionsupporting

confidence: 91%

“…24 This is also consistent with recent data from other types of pediatric dilated cardiomyopathy where arrhythmia burden worsens as systolic function decreases. 25 Thus, while CMR may be of use for the many reasons described within the NHLBI/PPMD Working Group findings, 17 our data suggest that obtaining a CMR solely to assess the presence of LGE in order to help gauge arrhythmia risk may have more limited utility.…”

Section: Discussionmentioning

confidence: 84%

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Ambulatory Monitoring and Arrhythmic Outcomes in Pediatric and Adolescent Patients With Duchenne Muscular Dystrophy

Villa

Czosek

Ahmed

et al. 2016

JAHA

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BackgroundPatients with Duchenne Muscular Dystrophy (DMD) develop cardiac fibrosis and dilated cardiomyopathy. We described the frequency of significant Holter findings in DMD, the relationship between cardiac function and arrhythmia burden, and the impact of these findings on clinical management.Methods and ResultsA retrospective review was done of patients with DMD who received a Holter from 2010 to 2014. Clinical and arrhythmic outcomes were analyzed. Patients were classified based on left ventricular ejection fraction (LVEF): ≥55%, 35% to 54% and <35%. Significant Holter findings included atrial tachycardia, ventricular tachycardia and atrial fibrillation/flutter. Logistic regression was used to assess predictors of significant Holter findings and change in care. The study included 442 Holters in 235 patients. Mean age was 14±4 years. Patients with cardiac dysfunction were older, and had increased late gadolinium enhancement and left ventricular dilation (P<0.01). There were 3 deaths (1%), all with normal function and none cardiac. Patients with LVEF <35% had more arrhythmias including nonsustained atrial tachycardia (P=0.01), frequent premature ventricular contractions, ventricular couplets/triplets, and nonsustained ventricular tachycardia (P<0.001) compared to the other groups. LVEF <35% (P<0.001) was the only predictor of clinically significant Holter finding. Four patients (40%) had change in medication in the LVEF <35% group compared to 9 (3%) in the ≥55% and 4 (4%) in the 35% to 54% groups (P<0.001).ConclusionsSudden cardiac events are rare in DMD patients with an LVEF >35%. Significant Holter findings are rare in patients with DMD who have an LVEF >35%, and cardiac dysfunction appears to predict significant Holter findings. Holter monitoring is highest yield among DMD patients with cardiac dysfunction.

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Section: Discussionsupporting

confidence: 91%

Section: Discussionmentioning

confidence: 84%

Ambulatory Monitoring and Arrhythmic Outcomes in Pediatric and Adolescent Patients With Duchenne Muscular Dystrophy

Villa

Czosek

Ahmed

et al. 2016

JAHA

Self Cite

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“…Another recent study [ 27 ] done on pediatric population showed that children who had normal or mild left ventricular dysfunction had much better prognosis in terms of arrhythmia burden and sudden cardiac death incidence further strengthening the importance of left ventricular function at the time of diagnosis and its impact on future health and outcomes.…”

Section: Discussionmentioning

confidence: 99%

A Rare Case of Renal Infarct due to Noncompaction Cardiomyopathy: A Case Report and Literature Review

Wats

Chen

Uppal

et al. 2016

Case Reports in Cardiology

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“…The ordering physician receives the report for use in patient care. In pediatrics, clinical indications for Holter monitors include rhythmsymptom correlation, monitoring of medication efficacy, and identification of arrhythmias [2][3][4][5][6]. Abnormal Holter results contain important information for patient care, including suggesting an elevated risk for sudden cardiac death in some settings [7,8].…”

Section: Background and Significancementioning

confidence: 99%

Adaptation of Radiology Software to Improve Cardiology Results Reporting

Webster¹,

Ward²,

Deal³

et al. 2017

Appl Clin Inform

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EMR systems are not typically designed for pediatric cardiology, but existing systems can be adapted, yielding important gains for patient care. In specialties like pediatric cardiology, there is insufficient volume nationally to drive development of commercial systems. This study demonstrates the general principle that creative adaptation of EMR systems can improve result reporting in pediatric cardiology and likely in other cardiology practices.Citation: Webster G, Ward K, Deal BJ, Anderson JB, Tsao S. Adaptation of Radiology Software to Improve Cardiology Results Reporting. Appl Clin Inform 2017; 8: 936-944 https://doi.org/10.4338/ACI-2017-03-RA-0051.

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Outcomes, Arrhythmic Burden and Ambulatory Monitoring of Pediatric Patients With Left Ventricular Non-Compaction and Preserved Left Ventricular Function

Cited by 21 publications

References 14 publications

Ambulatory Monitoring and Arrhythmic Outcomes in Pediatric and Adolescent Patients With Duchenne Muscular Dystrophy

Ambulatory Monitoring and Arrhythmic Outcomes in Pediatric and Adolescent Patients With Duchenne Muscular Dystrophy

A Rare Case of Renal Infarct due to Noncompaction Cardiomyopathy: A Case Report and Literature Review

Adaptation of Radiology Software to Improve Cardiology Results Reporting

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