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Hosp Pediatr ; 2020 Mar 06.
Artigo em Inglês | MEDLINE | ID: mdl-32144177


In this article, we will review various aspects of long QT syndrome (LQTS) necessary for hospitalists who care for children, adolescents, and young adults who have known LQTS and also review presenting features that should make one consider LQTS as a cause of hospitalization. Pediatric hospitalists care for patients who have suffered near-drowning, unexplained motor vehicular accidents, brief resolved unexpected events, sudden infant death syndrome, recurrent miscarriages, syncope, or seizures. These common conditions can be clinical clues in patients harboring 1 of 16 LQTS genetic mutations. LQTS is commonly caused by a channelopathy that can cause sudden cardiac death. Over the years, guidelines on management and recommendations for sports participation have evolved with our understanding of the disease and the burden of arrhythmias manifested in the pediatric age group. This review will include the genetic causes of LQTS, clinical features, and important historical information to obtain when these presentations are encountered. We will review medical and surgical treatments available to patients with LQTS and long-term care recommendations and prognosis for those diagnosed with LQTS.

Tex Heart Inst J ; 46(3): 225-228, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-31708710


Anomalous origin of the right coronary artery from the pulmonary artery, a rare congenital cardiac defect, is typically not diagnosed during infancy. On the other hand, Turner syndrome is usually diagnosed early, and it is classically associated with bicuspid aortic valve and aortic coarctation. Individuals with Turner syndrome are also at increased risk for coronary artery anomalies. We present a case of anomalous right coronary artery from the pulmonary artery in a week-old neonate who also had Turner syndrome, patent ductus arteriosus, transverse aortic arch hypoplasia, and impaired ventricular function. Prostaglandin therapy through the ductus increased the patient's myocardial perfusion. Four months after corrective surgery, she was doing well. We discuss the reperfusion phenomenon in our patient's case, as well as other considerations in this combination of congenital defects.

Anormalidades Múltiplas , Aorta Torácica/anormalidades , Anomalias dos Vasos Coronários/diagnóstico , Vasos Coronários/diagnóstico por imagem , Artéria Pulmonar/anormalidades , Síndrome de Turner/diagnóstico , Aorta Torácica/diagnóstico por imagem , Aorta Torácica/cirurgia , Implante de Prótese Vascular/métodos , Cateterismo Cardíaco , Angiografia por Tomografia Computadorizada , Anomalias dos Vasos Coronários/cirurgia , Vasos Coronários/cirurgia , Ecocardiografia , Feminino , Fluoroscopia , Humanos , Recém-Nascido , Artéria Pulmonar/diagnóstico por imagem , Artéria Pulmonar/cirurgia
Ann Noninvasive Electrocardiol ; 24(6): e12663, 2019 11.
Artigo em Inglês | MEDLINE | ID: mdl-31199031


BACKGROUND: There is paucity of data regarding the significance of high percentage of premature ventricular contractions (PVCs) in healthy children and their impact on left ventricular (LV) function and the risk of ventricular arrhythmias. The purpose of this study was to assess the prevalence of LV systolic dysfunction in children with frequent PVCs and determine whether PVC characteristics were predictive of LV dysfunction. METHODS: We performed a single-center retrospective review to examine the prevalence of PVC-induced cardiomyopathy and natural history of PVC burden in children with frequent PVCs. Children aged 6 months-21 years with PVCs noted on 24-hr Holter monitoring studies were enrolled. The four categories included those with a PVC burden of >10%, 10%-20%, and those with more than 20% PVC burden. RESULTS: A total of 134 children were included, 65 with more than 10% PVCs and 31 with more than 20% PVCs. Median age of the patients was 10.5 years (IQR 6.1-14.8 years), with 79 males (54.5%). Median PVC burden was 8.7% (IQR 4.2%-16.9%) with median follow-up of 2.8 years (IQR 1.2-4.6 years). During 2.8 years (1.3-4.3 years) of follow-up, the PVC burden decreased by 67% of baseline PVC burden in those who did not undergo any intervention. There were no deaths. CONCLUSION: PVCs in children with structurally normal hearts are associated with a relatively benign course, with trend toward spontaneous resolution.

Ann Noninvasive Electrocardiol ; 24(1): e12588, 2019 01.
Artigo em Inglês | MEDLINE | ID: mdl-30152133


INTRODUCTION: Noncompaction cardiomyopathy (NCCM) patients may develop sustained ventricular arrhythmias (VA). Currently no known electrocardiogram (ECG) parameter has demonstrated predictive value for VA development. The spatial QRS-T angle has demonstrated ability to identify VA in other cardiomyopathy populations. METHODS: A total of 39 patients with NCCM, defined by compact to non-compact ratio of >2.3 by magnetic resonance imaging, were assessed. The first ECG taken at time of MRI was assessed utilizing the heart rate, the QRS duration (QRSd), the corrected QT interval (QTc), and the spatial QRS-T angle (SPQRS-T angle, three-dimensional angle between the QRS and T-wave vectors) were assessed. RESULTS: Eight patients developed VA (20.5%). Median time to event was 3 months (95% CI 1.0 to 24.0 months). There were no significant differences between baseline ejection fraction or fractional shortening. Baseline median heart rate, spatial QRS-T angles, and indexed left ventricular end-diastolic volumes were all significantly higher in patients with VA development (p-value <0.05). Only heart rate and the SPQRS-T angle had significant univariate hazard ratios (HR) for VA at 1.031/beat per minute (1.001-1.071) and at a cut-off of 147 degrees the SPQRS-T angle gave a hazard ratio of HR of 5.773 (95% CI 1.161 to 28.702). The multivariate hazard ratio was only significant for the SPQRS-T angle, 1.031/degree (1.001-1.066). Survival analysis by Kaplan-Meier yielded a significant difference at a cutoff of 147 degrees. CONCLUSION: The SPQRS-T angle identified those at risk for VA development. Future studies are warranted with larger populations of noncompaction patients.

J Electrocardiol ; 50(5): 576-583, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-28372802


BACKGROUND: Fontan palliation patients are at risk for atrial arrhythmias post-operatively. This study aimed to evaluate whether differences in depolarization vector magnitude or spatial P-R angle can reliably predict atrial arrhythmias in patients who had undergone Fontan palliation. METHODS: A total of 115 patients who had Fontan palliation and post-Fontan catheterization were included. Atrial arrhythmias were identified in 14 patients. Measurements of ECG parameters including QRS vector magnitude, P-R duration, spatial QRS-T angle and spatial P-R angle were performed, and compared between those with and without atrial arrhythmias. RESULTS: Only the QRSvm independently differentiated those with and without atrial arrhythmias with multivariate HR of 0.743 (95% CI 0.581 to 0.951). CONCLUSION: In Fontan patients, the QRSvm was the only significant independent predictor of atrial arrhythmias.

Arritmias Cardíacas/etiologia , Arritmias Cardíacas/fisiopatologia , Eletrocardiografia , Técnica de Fontan/efeitos adversos , Átrios do Coração/fisiopatologia , Cateterismo Cardíaco , Pré-Escolar , Teste de Esforço , Feminino , Humanos , Masculino , Valor Preditivo dos Testes , Estudos Retrospectivos , Fatores de Risco
Circ Arrhythm Electrophysiol ; 5(6): 1064-72, 2012 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-23212181


BACKGROUND: Cardiac rhythm devices are increasingly used in the pediatric population, although their impact on quality of life (QOL) is poorly understood. The purpose of this study was to compare (QOL) scores among pediatric device patients, healthy controls, and congenital heart disease (CHD) patients and determine the key drivers of QOL in pediatric device patients. METHODS AND RESULTS: Multicenter, cross-sectional study at 8 pediatric centers of subjects aged 8 to 18 years with either a pacemaker or defibrillator was carried out. Patient-parent pairs completed the Pediatric Quality of Life Inventory and Pediatric Cardiac Quality of Life Inventory. QOL outcomes in device patients were compared with healthy controls and patients with various forms of CHD. Structural equation modeling was used to test for differences in Pediatric Cardiac Quality of Life Inventory scores among (1) device type, (2) presence of CHD, and (3) hypothesized key drivers of QOL. One hundred seventy-three patient-parent pairs (40 defibrillators/133 pacemakers) were included. Compared with healthy controls, patients with devices and their parents reported significantly lower Pediatric Quality of Life Inventory scoring. Similarly, compared with patients with mild forms of CHD, parents and patients with devices reported significantly lower Pediatric Cardiac Quality of Life Inventory scores and were similar to patients with more severe CHD. Key drivers of patient QOL were presence of implantable cardioverter-defibrillator and CHD. For patients, self-perception was a key driver of lower QOL, whereas for parents behavioral issues were associated with lower QOL. CONCLUSIONS: Patient QOL is significantly affected by the presence of cardiac rhythm devices. Whether these effects can be mitigated through the use of psychotherapy needs to be assessed.

Arritmias Cardíacas/psicologia , Arritmias Cardíacas/terapia , Desfibriladores Implantáveis , Marca-Passo Artificial , Qualidade de Vida/psicologia , Adolescente , Criança , Estudos Transversais , Feminino , Cardiopatias/congênito , Cardiopatias/psicologia , Humanos , Masculino , Pais/psicologia , Estudos Retrospectivos , Autoimagem