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1.
J Cardiopulm Rehabil Prev ; 40(1): E1-E4, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31868841

RESUMO

PURPOSE: Cardiac rehabilitation (CR) effectively decreases morbidity and mortality in adults after cardiovascular events. Cardiac rehabilitation has been underutilized for patients with congenital heart disease (CHD). The primary objective was to evaluate the inclusion of adolescents and adults with CHD in a CR program by analyzing data from our single-center CR program. The secondary objectives were to evaluate the efficacy and safety of CR as well as referral barriers. METHODS: This was a retrospective study of patients aged ≥15 yr who were referred to regional CR centers. Data on efficacy and safety were collected. RESULTS: Over a 4-yr period, 36 patients were referred to 23 regional centers: 23 patients completed CR, 12 are currently enrolled or in the referral process, and 1 died before initiation. The median age was 22 yr (range: 15-55). The primary indication was post-surgical (61%), followed by chronic heart failure (30%), and post-transplant (9%). After CR, metabolic equivalent tasks increased by 1.6 (P < .001), maximal heart rate increased by 13 beats/min (P = .026), exercise time increased by 1.35 min (P = .047), and treadmill speed increased by 0.7 mph (P = .007). There were no serious adverse events. All patients who completed CR remain alive at a median follow-up of 17 mo (range: 5-45). Common barriers to CR included accessibility, social circumstances, and cost for phase III CR. CONCLUSION: In our cohort, CR was effective and safe for adolescents and adults with CHD.

3.
Curr Cardiol Rep ; 21(5): 29, 2019 03 18.
Artigo em Inglês | MEDLINE | ID: mdl-30887232

RESUMO

PURPOSE OF REVIEW: This article attempts to review some of the commonly seen repaired congenital heart defects in the adult population (ACHD), with a focus on important echocardiographic findings that may assist a practitioner in recognizing and managing this group of patients. RECENT FINDINGS: The prevalence of ACHD population is increasing, and currently, there are over 1 million adults with congenital heart disease. At the current time, the total number of ACHD patients exceeds the total number of pediatric patients with CHD. The recently released 2018 American College of Cardiology/American Heart Association guidelines for the management of adults with congenital heart disease (ACHD) recommends transthoracic echocardiography for all ACHD patients for initial assessment and serial assessment as needed (class of recommendation: I) and echocardiography remains the mainstay for diagnosing and managing these patients in adjunct with other available imaging modalities. It is imperative for all cardiologists to be familiar with the echocardiographic features of the commonly seen repaired CHD in adult population.

4.
Cardiol Young ; 29(3): 431-434, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30764888

RESUMO

Coronary-pulmonary artery fistula is a rare anomaly in which an aortopulmonary collateral artery arises from a coronary artery, often seen in patients with pulmonary atresia with ventricular septal defect. In the presented case, a coronary-pulmonary artery fistula arose from the left main coronary artery and supplied blood flow to a left upper lobe segment. The life-sized three-dimensional printed model was helpful in pre-surgical planning for unifocalisation of the aortopulmonary collateral arteries.


Assuntos
Fístula Artério-Arterial/cirurgia , Vasos Coronários/cirurgia , Impressão Tridimensional , Artéria Pulmonar/cirurgia , Procedimentos Cirúrgicos Vasculares/métodos , Fístula Artério-Arterial/congênito , Fístula Artério-Arterial/diagnóstico , Cateterismo Cardíaco , Vasos Coronários/diagnóstico por imagem , Feminino , Humanos , Recém-Nascido , Artéria Pulmonar/diagnóstico por imagem
5.
Ann Neurol ; 84(4): 588-600, 2018 10.
Artigo em Inglês | MEDLINE | ID: mdl-30179277

RESUMO

OBJECTIVE: Intracellular recordings from cells in entorhinal cortex tissue slices show that low-voltage fast (LVF) onset seizures are generated by inhibitory events. Here, we determined whether increased firing of interneurons occurs at the onset of spontaneous mesial-temporal LVF seizures recorded in patients. METHODS: The seizure onset zone (SOZ) was identified using visual inspection of the intracranial electroencephalogram. We used wavelet clustering and temporal autocorrelations to characterize changes in single-unit activity during the onset of LVF seizures recorded from microelectrodes in mesial-temporal structures. Action potentials generated by principal neurons and interneurons (ie, putative excitatory and inhibitory neurons) were distinguished using waveform morphology and K-means clustering. RESULTS: From a total of 200 implanted microelectrodes in 9 patients during 13 seizures, we isolated 202 single units; 140 (69.3%) of these units were located in the SOZ, and 40 (28.57%) of them were classified as inhibitory. The waveforms of both excitatory and inhibitory units remained stable during the LVF epoch (p > > 0.05). In the mesial-temporal SOZ, inhibitory interneurons increased their firing rate during LVF seizure onset (p < 0.01). Excitatory neuron firing rates peaked 10 seconds after the inhibitory neurons (p < 0.01). During LVF spread to the contralateral mesial temporal lobe, an increase in inhibitory neuron firing rate was also observed (p < 0.01). INTERPRETATION: Our results suggest that seizure generation and spread during spontaneous mesial-temporal LVF onset events in humans may result from increased inhibitory neuron firing that spawns a subsequent increase in excitatory neuron firing and seizure evolution. Ann Neurol 2018;84:588-600.


Assuntos
Potenciais de Ação/fisiologia , Eletroencefalografia/tendências , Interneurônios/fisiologia , Convulsões/diagnóstico , Convulsões/fisiopatologia , Adulto , Eletrodos Implantados/tendências , Eletroencefalografia/métodos , Feminino , Giro do Cíngulo/fisiopatologia , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Lobo Temporal/fisiopatologia , Adulto Jovem
6.
Neuroimage Clin ; 20: 398-406, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30128278

RESUMO

Background: Electrode contact locations are important when planning tailored brain surgeries to identify pathological tissue targeted for resection and conversely avoid eloquent tissue. Current methods employ trained experts to use neuroimaging scans that are manually co-registered and localize contacts within ~2 mm. Yet, the state of the art is limited by either the expertise needed for each type of intracranial electrode or the inter-modality co-registration which increases error, reducing accuracy. Patients often have a variety of strips, grids and depths implanted; therefore, it is cumbersome and time-consuming to apply separate localization methods for each type of electrode, requiring expertise across different approaches. New method: To overcome these limitations, a computational method was developed by separately registering an implant magnetic resonance image (MRI) and implant computed tomography image (CT) to the pre-implant MRI, then calculating an iterative closest point transformation using the contact locations extracted from the signal voids as ground truth. Results: The implant MRI is robustly co-registered to the pre-implant MRI with a boundary-based registration algorithm. By extracting and utilizing 'signal voids' (the metal induced artifacts from the implant MRI) as electrode fiducials, the novel method is an all-in-one approach for all types of intracranial electrodes while eliminating inter-modality co-registration errors. Comparison with existing methods: The distance between each electrode centroid and the brain's surface was measured, for the proposed method as well as the state of the art method using two available software packages, SPM 12 and FSL 4.1. The method presented here achieves the smallest distances to the brain's surface for all strip and grid type electrodes, i.e. contacts designed to rest directly on the brain surface. Conclusion: We use one of the largest reported sample sizes in localization studies to validate this novel method for localizing different kinds of intracranial electrodes including grids, strips and depth electrodes.


Assuntos
Epilepsia Resistente a Medicamentos/diagnóstico por imagem , Eletrodos Implantados , Eletroencefalografia/métodos , Imagem por Ressonância Magnética/métodos , Tomografia Computadorizada por Raios X/métodos , Adolescente , Adulto , Epilepsia Resistente a Medicamentos/fisiopatologia , Eletrodos Implantados/normas , Eletroencefalografia/instrumentação , Eletroencefalografia/normas , Feminino , Humanos , Imagem por Ressonância Magnética/instrumentação , Imagem por Ressonância Magnética/normas , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Tomografia Computadorizada por Raios X/instrumentação , Tomografia Computadorizada por Raios X/normas , Adulto Jovem
7.
Epilepsia ; 59(3): 636-649, 2018 03.
Artigo em Inglês | MEDLINE | ID: mdl-29442363

RESUMO

OBJECTIVE: To gain understanding of the neuronal mechanisms underlying regional seizure spread, the impact of regional synchrony between seizure focus and downstream networks on neuronal activity during the transition to seizure in those downstream networks was assessed. METHODS: Seven patients undergoing diagnostic intracranial electroencephalographic studies for surgical resection of epileptogenic regions were implanted with subdural clinical electrodes into the cortex (site of seizure initiation) and mesial temporal lobe (MTL) structures (downstream) as well as microwires into MTL. Neural activity was recorded (24/7) in parallel with the clinical intracranial electroencephalogram recordings for the duration of the patient's diagnostic stay. Changes in (1) regional synchrony (ie, coherence) between the presumptive neocortical seizure focus and MTL, (2) local synchrony between MTL neurons and their local field potential, and (3) neuronal firing rates within MTL in the time leading up to seizure were examined to study the mechanisms underlying seizure spread. RESULTS: In seizures of neocortical origin, an increase in regional synchrony preceded the spread of seizures into MTL (predominantly hippocampal). Within frequencies similar to those of regional synchrony, MTL networks showed an increase in unit-field coherence and a decrease in neuronal firing rate, specifically for inhibitory interneuron populations but not pyramidal cell populations. SIGNIFICANCE: These results suggest a mechanism of spreading seizures whereby the seizure focus first synchronizes local field potentials in downstream networks to the seizure activity. This change in local field coherence modifies the activity of interneuron populations in these downstream networks, which leads to the attenuation of interneuronal firing rate, effectively shutting down local interneuron populations prior to the spread of seizure. Therefore, regional synchrony may influence the failure of downstream interneurons to prevent the spread of the seizures during generalization.


Assuntos
Epilepsia do Lobo Temporal/fisiopatologia , Neocórtex/fisiopatologia , Rede Nervosa/fisiopatologia , Neurônios/fisiologia , Convulsões/fisiopatologia , Adolescente , Eletroencefalografia/métodos , Epilepsia do Lobo Temporal/diagnóstico por imagem , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Neocórtex/diagnóstico por imagem , Rede Nervosa/diagnóstico por imagem , Convulsões/diagnóstico por imagem , Adulto Jovem
8.
Pediatr Cardiol ; 38(6): 1282-1287, 2017 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-28687888

RESUMO

Transthoracic echocardiography (TTE) is a non-invasive diagnostic modality for children with suspected heart disease. The American College of Cardiology published Appropriate Use Criteria (AUC) for an initial outpatient pediatric TTE in 2014 to promote effective care and improve resource utilization. The objective was to determine the appropriateness of TTE per the published AUC in a single academic pediatric cardiology clinic as a baseline performance quality measure. The echocardiography database was used to identify initial outpatient TTE in children during January-March 2014. TTE indications (appropriate [A], may be appropriate [M], or rarely appropriate [R]) and findings (normal, incidental, or abnormal) were recorded. The effect of AUC and age groups on yield of abnormal TTE findings was analyzed. Of the 2166 screened studies, our study cohort consisted of 247 TTEs. Indications rated A, M, and R were found in 129, 27, and 90, respectively, and 1 was unclassifiable. Majority of TTE (n = 183) were normal, although incidental findings were noted in 32 and abnormal findings in 32 cases. Abnormal findings were noted in 26/129 of A, 2/27 of M, and 4/90 of R. Indications rated A were significantly associated with yield of abnormal TTE findings, adjusted by age group. Infants and adolescents were more likely to have abnormal TTE findings compared to young children. Recently published AUC were validated for initial TTE in the outpatient pediatric cardiology clinic. Appropriateness rated by AUC was highly associated with yield of abnormal TTE findings and worked best in infants and adolescent.


Assuntos
Cardiologia/normas , Ecocardiografia/estatística & dados numéricos , Melhoria de Qualidade , Centros Médicos Acadêmicos/normas , Adolescente , Criança , Pré-Escolar , Ecocardiografia/normas , Fidelidade a Diretrizes , Hospitais Pediátricos/normas , Humanos , Lactente , Recém-Nascido , Ambulatório Hospitalar/normas , Seleção de Pacientes , Padrões de Prática Médica , Estudos Retrospectivos
9.
BMJ Case Rep ; 20162016 Aug 17.
Artigo em Inglês | MEDLINE | ID: mdl-27535729

RESUMO

Isolated ACTH deficiency (IAD) is a rare cause of neonatal cholestasis and hypoglycaemia. This diagnosis has a 20% mortality potential if unrecognised. We describe a case of an infant presenting with cholestatic jaundice and hypoglycaemia. The patient had laboratory findings suggestive of IAD, which was later confirmed with molecular genetic testing. One of the mutations this patient had is a new finding. The patient was started on glucocorticoid replacement therapy after which his bilirubin and glucose levels normalised.


Assuntos
Hormônio Adrenocorticotrópico/deficiência , Doenças do Sistema Endócrino/complicações , Doenças Genéticas Inatas/complicações , Hipoglicemia/complicações , Icterícia Obstrutiva/etiologia , Corticosteroides/administração & dosagem , Hormônio Adrenocorticotrópico/genética , Diagnóstico Diferencial , Doenças do Sistema Endócrino/genética , Doenças Genéticas Inatas/genética , Proteínas de Homeodomínio/genética , Humanos , Hidrocortisona/administração & dosagem , Hipoglicemia/etiologia , Hipoglicemia/genética , Recém-Nascido , Masculino , Mutação/genética , Proteínas com Domínio T/genética , Resultado do Tratamento
11.
J Neurosci ; 34(20): 6887-95, 2014 May 14.
Artigo em Inglês | MEDLINE | ID: mdl-24828643

RESUMO

Animal studies have shown that substantia nigra (SN) dopaminergic (DA) neurons strengthen action-reward associations during reinforcement learning, but their role in human learning is not known. Here, we applied microstimulation in the SN of 11 patients undergoing deep brain stimulation surgery for the treatment of Parkinson's disease as they performed a two-alternative probability learning task in which rewards were contingent on stimuli, rather than actions. Subjects demonstrated decreased learning from reward trials that were accompanied by phasic SN microstimulation compared with reward trials without stimulation. Subjects who showed large decreases in learning also showed an increased bias toward repeating actions after stimulation trials; therefore, stimulation may have decreased learning by strengthening action-reward associations rather than stimulus-reward associations. Our findings build on previous studies implicating SN DA neurons in preferentially strengthening action-reward associations during reinforcement learning.


Assuntos
Doença de Parkinson/terapia , Aprendizagem por Probabilidade , Substância Negra/fisiologia , Idoso , Estimulação Encefálica Profunda , Estimulação Elétrica , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Doença de Parkinson/fisiopatologia , Doença de Parkinson/psicologia
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