Publications by authors named "Mary C Niu"

Article Synopsis
  • A study examined the relationship between post-operative ECG changes, diastolic blood pressure (DBP), and negative outcomes in patients who underwent Systemic to Pulmonary Artery shunt surgery.
  • Patients' ECGs were classified into three grades based on repolarization abnormalities, with only a small percentage showing Grade 2 abnormalities.
  • The analysis revealed no link between ECG changes or low DBP levels and adverse outcomes; however, high DBP levels above 34 mmHg appeared to increase the risk of complications in certain patients.
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Bidirectional ventricular tachycardia is a unique arrhythmia that can herald lethal arrhythmia syndromes. Using cases based on real patient stories, this article examines 3 different presentations to help clinicians learn the differential diagnosis associated with this condition. Each associated genetic disorder will be briefly discussed, and valuable tips for distinguishing them from each other will be provided.

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Article Synopsis
  • The case series discusses the differential diagnosis of genetic arrhythmia syndromes in patients with prolonged QTc, emphasizing the importance of a thorough evaluation.
  • Essential components for diagnosis include a detailed patient and family history, along with a careful review of the electrocardiogram (ECG).
  • Clinicians can use signs, symptoms, and ECG features to make quicker, accurate diagnoses, potentially preventing serious health risks before genetic testing results are available.
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Background: Ventricular arrhythmia incidence in children and adolescents undergoing transcatheter pulmonary valve replacement (TPVR) within the native right ventricular outflow tract (nRVOT) is unknown. We sought to describe the incidence, severity, and duration of ventricular arrhythmias and identify associated risk factors in this population.

Methods: This was a retrospective cohort study of 78 patients <21 years of age who underwent TPVR within the nRVOT.

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Article Synopsis
  • The study investigates the safety and effectiveness of the Advisor™ High-Density Grid mapping catheter (HDGC) in pediatric patients with congenital heart disease (CHD) during electrophysiology procedures.
  • 74 out of 261 cases used the HDGC, with notable differences in age, weight, and arrhythmia types between HDGC and non-HDGC groups.
  • While complications were rare, the HDGC cases had longer procedures and lower acute success rates, suggesting the catheter may be used more often for complex arrhythmias rather than indicating poor performance.
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The Norwood procedure with a right ventricular to pulmonary artery shunt (RVPAS) decreases early mortality, but requires a ventriculotomy, possibly increasing risk of ventricular arrhythmias (VAs) compared with the modified Blalock-Taussig shunt (MBTS). The effect of shunt and Fontan type on arrhythmias by 6 years of age in the SVRII (Single Ventricle Reconstruction Extension Study) was assessed. SVRII data collected on 324 patients pre-/post-Fontan and annually at 2 to 6 years included antiarrhythmic medications, electrocardiography (ECG) at Fontan, and Holter/ECG at 6 years.

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In view of the increasing complexity of both cardiovascular implantable electronic devices (CIEDs) and patients in the current era, practice guidelines, by necessity, have become increasingly specific. This document is an expert consensus statement that has been developed to update and further delineate indications and management of CIEDs in pediatric patients, defined as ≤21 years of age, and is intended to focus primarily on the indications for CIEDs in the setting of specific disease categories. The document also highlights variations between previously published adult and pediatric CIED recommendations and provides rationale for underlying important differences.

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In view of the increasing complexity of both cardiovascular implantable electronic devices (CIEDs) and patients in the current era, practice guidelines, by necessity, have become increasingly specific. This document is an expert consensus statement that has been developed to update and further delineate indications and management of CIEDs in pediatric patients, defined as ≤21 years of age, and is intended to focus primarily on the indications for CIEDs in the setting of specific disease categories. The document also highlights variations between previously published adult and pediatric CIED recommendations and provides rationale for underlying important differences.

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In view of the increasing complexity of both cardiovascular implantable electronic devices (CIEDs) and patients in the current era, practice guidelines, by necessity, have become increasingly specific. This document is an expert consensus statement that has been developed to update and further delineate indications and management of CIEDs in pediatric patients, defined as ≤21 years of age, and is intended to focus primarily on the indications for CIEDs in the setting of specific disease categories. The document also highlights variations between previously published adult and pediatric CIED recommendations and provides rationale for underlying important differences.

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Article Synopsis
  • * The new guidelines specifically target pediatric patients (≤21 years), acknowledging differences in CIED indications and technology needs compared to adults, particularly for those aged 18-21.
  • * This document serves as an expert consensus statement focused on indications for CIED use in various diseases, providing a modular format of recommendations and guidance on lead management and follow-up for pediatric patients.
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In view of the increasing complexity of both cardiovascular implantable electronic devices (CIEDs) and patients in the current era, practice guidelines, by necessity, have become increasingly specific. This document is an expert consensus statement that has been developed to update and further delineate indications and management of CIEDs in pediatric patients, defined as ≤21 years of age, and is intended to focus primarily on the indications for CIEDs in the setting of specific disease categories. The document also highlights variations between previously published adult and pediatric CIED recommendations and provides rationale for underlying important differences.

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Purpose Of Review: This review provides a basic understanding of the calmodulin gene and its role in calcium homeostasis. We outline the functional effects and clinical expression of CALM mutations and review disease expression and management.

Recent Findings: Calmodulinopathies are rare life-threatening arrhythmia syndromes affecting young individuals.

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Kawasaki disease (KD) is a systemic inflammatory condition primarily affecting young children. We present an adolescent male with two episodes of complete KD between the age of 2 and 14 years. At age 14, he presented with findings suggestive of a retropharyngeal abscess.

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Background: Heart Rhythm Society guidelines outlining magnetic resonance imaging (MRI) in patients with cardiac implantable electronic devices (CIEDs) excluded children and epicardial or abandoned leads due to theoretical risks of harm. Research investigating these risks is lacking. The primary objective of our study is to determine the incidence of adverse events to patients or CIEDs from MRI imaging.

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Purpose Of Review: Our purpose is to provide an update on the new clinical and genetic aspects of long QT syndrome (LQTS). LQTS is the most common channelopathy and a cause of syncope and sudden death in the young. Although there are 17 types of LQTS, most patients have types 1 or 2 which are due to mutations in KCNQ1 and KCNH2 (encoding for the cardiac potassium channels), and type 3 which is due to a mutation in SCN5A (encoding for the sodium channel).

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Background: Arrhythmias are common in the pediatric population. In patients unable to take oral medications or in need of acute therapy, options of intravenous (IV) antiarrhythmic medications are limited. Recently IV sotalol has become readily available, but experience in children is limited.

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Background: Heterotaxy syndrome (HS) is a rare disorder with complex anatomy involving misarrangements of the cardiac conduction system. Arrhythmias may be related to anatomic variations and contribute to morbidity.

Objective: The purpose of this study was to investigate the associations between arrhythmias, anatomy, and outcomes in a large HS cohort.

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Objective: To compare outcomes of pediatric patients treated with azithromycin compared with penicillin or cephalosporin. We hypothesized that azithromycin use would not be associated with increased cardiac mortality in the pediatric population.

Study Design: Retrospective cohort study from the Pediatric Health Information System database between 2008 and 2012.

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