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Artificial intelligence (AI) has potential to improve the accuracy of screening for valvular and congenital heart disease by auscultation. However, despite recent advances in signal processing and classification algorithms focused on heart sounds, clinical acceptance of this technology has been limited, in part due to lack of objective performance data. We hypothesized that a heart murmur detection algorithm could be quantitatively and objectively evaluated by virtual clinical trial. All cases from the Johns Hopkins Cardiac Auscultatory Recording Database (CARD) with either a pathologic murmur, an innocent murmur or no murmur were selected. The test algorithm, developed independently of CARD, analyzed each recording using an automated batch processing protocol. 3180 heart sound recordings from 603 outpatient visits were selected from CARD. Algorithm estimation of heart rate was similar to gold standard. Sensitivity and specificity for detection of pathologic cases were 93% (CI 90-95%) and 81% (CI 75-85%), respectively, with accuracy 88% (CI 85-91%). Performance varied according to algorithm certainty measure, age of patient, heart rate, murmur intensity, location of recording on the chest and pathologic diagnosis. This is the first reported comprehensive and objective evaluation of an AI-based murmur detection algorithm to our knowledge. The test algorithm performed well in this virtual clinical trial. This strategy can be used to efficiently compare performance of other algorithms against the same dataset and improve understanding of the potential clinical usefulness of AI-assisted auscultation.
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Inteligência Artificial/estatística & dados numéricos , Diagnóstico por Computador/métodos , Auscultação Cardíaca/métodos , Cardiopatias Congênitas/diagnóstico , Sopros Cardíacos/diagnóstico , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Algoritmos , Criança , Pré-Escolar , Bases de Dados Factuais , Humanos , Lactente , Pessoa de Meia-Idade , Sensibilidade e Especificidade , Adulto JovemRESUMO
BACKGROUND: Anthracycline use is limited by cardiotoxicity, including arrhythmias and left ventricular (LV) dysfunction. We aim to characterize the association between electrophysiological changes and LV dysfunction. METHODS: A retrospective chart review was conducted, including all 147 pediatric cancer survivors at our institution over 18 years of age and treated with an anthracycline. One hundred thirty-four patients who had at least one electrocardiogram (ECG) and echocardiogram were analyzed. The association between dysfunction and baseline characteristics, treatment history, and electrocardigraphic parameters were analyzed using multivariable logistic regression. Additionally, a longitudinal generalized estimating equation (GEE) model was used to examine the temporal association between repeated measure corrected QT (QTc) intervals and subsequent LV function. RESULTS: In our population, 24% of patients had LV dysfunction. The initial posttreatment QTc interval was longer in patients with LV dysfunction (438 ± 35 vs. 420 ± 20 msec, P = 0.002). In logistic regression analysis, QTc interval (P < 0.001) and cumulative radiation dose (P = 0.027) were associated with LV dysfunction. On ECGs performed prior to evidence of LV dysfunction, the QTc was longer than on ECGs preceding a normal echocardiogram (451 ± 32 msec vs. 423 ± 25 msec, P < 0.001). Mean time from QTc ≥ 450 msec to evidence of LV dysfunction was 1.8 ± 2.9 years. In the longitudinal GEE model, QTc prolongation was associated with subsequent decreased fractional shortening. CONCLUSIONS: Among adult survivors of pediatric cancer treated with anthracyclines, prolongation of the QTc interval was associated with subsequent LV dysfunction.
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Antraciclinas/efeitos adversos , Neoplasias/complicações , Disfunção Ventricular Esquerda/induzido quimicamente , Adulto , Criança , Eletrocardiografia , Fenômenos Eletrofisiológicos , Feminino , Seguimentos , Humanos , Masculino , Neoplasias/tratamento farmacológico , Prognóstico , Estudos Retrospectivos , Taxa de Sobrevida , SobreviventesRESUMO
Sonographic cardiac standstill during adult cardiac arrest is associated with failure to get return to spontaneous circulation. This report documents 3 children whose cardiac function returned after standstill with extracorporeal membranous oxygenation. Sonographic cardiac standstill may not predict cardiac death in children.
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Parada Cardíaca/diagnóstico por imagem , Parada Cardíaca/fisiopatologia , Criança , Ecocardiografia , Oxigenação por Membrana Extracorpórea , Evolução Fatal , Feminino , Parada Cardíaca/terapia , Humanos , Lactente , Recém-Nascido , Masculino , Recuperação de Função FisiológicaRESUMO
PURPOSE: Barth syndrome (BTHS), an X-linked disorder caused by defects in TAZ, is the only known single-gene disorder of cardiolipin remodeling. We hypothesized that through analysis of affected individuals, we would gain a better understanding of the range of clinical features and identify targets for monitoring and therapy. METHODS: We conducted a multidisciplinary investigation involving 42 patients with BTHS, including echocardiograms, muscle strength testing, functional exercise capacity testing, physical activity assessments, cardiolipin analysis, 3-methylglutaconic acid analysis, and review of genotype data. We analyzed data points to provide a quantitative spectrum of disease characteristics and to identify relationships among phenotype, genotype, and relevant metabolites. RESULTS: Echocardiography revealed considerable variability in cardiac features. By contrast, almost all patients had significantly reduced functional exercise capacity. Multivariate analysis revealed significant relationships between cardiolipin ratio and left ventricular mass and between cardiolipin ratio and functional exercise capacity. We additionally identified genotypes associated with a less severe metabolic and clinical profile. CONCLUSION: We defined previously unrecognized metabolite/phenotype/genotype relationships, established targets for therapeutic monitoring, and validated avenues for clinical assessment. In addition to providing insight into BTHS, these studies also provide insight into the myriad of multifactorial disorders that converge on the cardiolipin pathway.Genet Med 18 10, 1001-1010.
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Síndrome de Barth/sangue , Cardiolipinas/sangue , Cardiomiopatias/sangue , Fatores de Transcrição/genética , Aciltransferases , Adolescente , Adulto , Síndrome de Barth/genética , Síndrome de Barth/fisiopatologia , Cardiolipinas/genética , Cardiomiopatias/genética , Cardiomiopatias/fisiopatologia , Criança , Pré-Escolar , Ecocardiografia , Genótipo , Glutaratos/sangue , Humanos , Masculino , Força Muscular/genética , Fenótipo , Adulto JovemRESUMO
OBJECTIVE: Duchenne and Becker muscular dystrophies (DBMD) are allelic disorders caused by mutations in dystrophin. Adults with DBMD develop life-threatening cardiomyopathy. Inhibition of phosphodiesterase 5 (PDE5) improves cardiac function in mouse models of DBMD. To determine whether the PDE5-inhibitor sildenafil benefits human dystrophinopathy, we conducted a randomized, double-blind, placebo-controlled trial (ClinicalTrials.gov, number NCT01168908). METHODS: Adults with DBMD and cardiomyopathy (ejection fraction ≤ 50%) were randomized to receive sildenafil (20mg 3× daily) or placebo for 6 months. All subjects received an additional 6 months of open-label sildenafil. The primary endpoint was change in left ventricular end-systolic volume (LVESV) on cardiac magnetic resonance imaging. Secondary cardiac endpoints, skeletal muscle function, and quality of life were also assessed. RESULTS: An interim analysis (performed after 15 subjects completed the blinded phase) revealed that 29% (4 of 14) of subjects had a ≥10% increase in LVESV after 6 months of sildenafil compared to 13% (1 of 8) of subjects receiving placebo. Subjects with LVESV > 120ml at baseline were more likely to worsen at 12 months regardless of treatment assignment (p = 0.035). Due to the higher number of subjects worsening on sildenafil, the data and safety monitoring board recommended early termination of the study. There were no statistically significant differences in outcome measures between treatment arms. INTERPRETATION: Due to the small sample size, comparisons between groups must be interpreted with caution. However, this trial suggests that sildenafil is unlikely to improve cardiac function in adults with DBMD.
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Cardiomiopatias/tratamento farmacológico , Cardiomiopatias/etiologia , Distrofia Muscular de Duchenne/complicações , Piperazinas/uso terapêutico , Sulfonas/uso terapêutico , Vasodilatadores/uso terapêutico , Adolescente , Adulto , Débito Cardíaco/efeitos dos fármacos , Cardiomiopatias/genética , Método Duplo-Cego , Distrofina/genética , Feminino , Seguimentos , Humanos , Masculino , Distrofia Muscular de Duchenne/genética , Purinas/uso terapêutico , Citrato de Sildenafila , Método Simples-Cego , Adulto JovemRESUMO
Stress echocardiography in paediatrics is used to evaluate pre- and post-operative coronary artery conditions, as well as to gain haemodynamic information for a variety of diagnoses, although evidence regarding sensitivity, specificity, and predictive value is lacking. This review will consider the available literature with a focus on anomalous aortic origin of the coronary arteries and discuss a practical approach to test selection and use.
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Aorta Torácica/diagnóstico por imagem , Anomalias dos Vasos Coronários/diagnóstico por imagem , Ecocardiografia sob Estresse/métodos , Aorta Torácica/anormalidades , Tomografia Computadorizada por Emissão de Fóton Único de Sincronização Cardíaca , Criança , Tomada de Decisão Clínica , Angiografia Coronária , Anomalias dos Vasos Coronários/diagnóstico , Ecocardiografia , Humanos , Valor Preditivo dos Testes , Prognóstico , Sensibilidade e Especificidade , Tomografia Computadorizada por Raios XRESUMO
We describe the use of low-dose dual-source cardiac for the evaluation of a neonate with suspected total anomalous pulmonary venous connection. This novel technique obviates the need for sedation or breathholding. Radiation dose-reduction strategies result in sub-mSv-estimated effective doses, substantially lower than annual background radiation dose. Low-dose, high-pitch cardiac CT should be considered as an alternative to MRI or diagnostic cardiac catheterisation in neonates with complex CHD, requiring definitive anatomic evaluation.
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Angiografia Coronária/métodos , Doses de Radiação , Síndrome de Cimitarra/diagnóstico , Tomografia Computadorizada por Raios X/métodos , Eletrocardiografia , Humanos , Recém-NascidoRESUMO
Background: Anthracyclines are associated with cardiac dysfunction. Little is known about the interplay of pre-existing hypertension and treatment response. We aimed to investigate the relationship between hypertension and the development of cancer therapy-related cardiac dysfunction (CTRCD) in pediatric patients treated with anthracycline chemotherapy. Methods: Pediatric patients with cancer who received anthracycline chemotherapy from 2013 to 2021 were retrospectively included. Serial cardiac assessments were conducted during and after chemotherapy. The primary outcome was the development of CTRCD, classified as mild, moderate, or severe according to contemporary definitions. Results: Among 190 patients undergoing anthracycline chemotherapy, 34 patients (17.9 %) had hypertension (24 patients Stage 1, and 10 patients Stage 2) at baseline evaluation. Patients underwent chemotherapy for a median of 234.4 days (interquartile range 127.8-690.3 days) and were subsequently followed up. Hypertension was frequent during follow-up 31.3 % (0-3 months), 15.8 % (3-6 months), 21.9 % (0.5-1 years), 24.7 % (1-2 years), 31.1 % (2-4 years) and 35.8 % (beyond 4 years) (P for trend < 0.001). Freedom from mild CTRCD at 5 years was 45.0 %, freedom from moderate CTRCD was 87.8 % at 5 years. Baseline hypertension did not increase the risk of mild (HR 0.77, 95 % CI: 0.41-1.42, P = 0.385) or moderate CTRCD (HR 0.62, 95 % CI: 0.14-2.72, P = 0.504). Patients with baseline hypertension showed different global longitudinal strain (P < 0.001) and LVEF (P < 0.001) patterns during follow-up. Conclusions: Pediatric patients often develop CTRCD post-anthracycline chemotherapy. Those with pre-existing hypertension show a unique treatment response, despite no increased CTRCD risk, warranting further investigation.
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BACKGROUND: Barth syndrome (BTHS) is a rare genetic disease that is characterized by cardiomyopathy, skeletal myopathy, neutropenia, and growth abnormalities and often leads to death in childhood. Recently, elamipretide has been tested as a potential first disease-modifying drug. This study aimed to identify patients with BTHS who may respond to elamipretide, based on continuous physiological measurements acquired through wearable devices. RESULTS: Data from a randomized, double-blind, placebo-controlled crossover trial of 12 patients with BTHS were used, including physiological time series data measured using a wearable device (heart rate, respiratory rate, activity, and posture) and functional scores. The latter included the 6-minute walk test (6MWT), Patient-Reported Outcomes Measurement Information System (PROMIS) fatigue score, SWAY Balance Mobile Application score (SWAY balance score), BTHS Symptom Assessment (BTHS-SA) Total Fatigue score, muscle strength by handheld dynamometry, 5 times sit-and-stand test (5XSST), and monolysocardiolipin to cardiolipin ratio (MLCL:CL). Groups were created through median split of the functional scores into "highest score" and "lowest score", and "best response to elamipretide" and "worst response to elamipretide". Agglomerative hierarchical clustering (AHC) models were implemented to assess whether physiological data could classify patients according to functional status and distinguish non-responders from responders to elamipretide. AHC models clustered patients according to their functional status with accuracies of 60-93%, with the greatest accuracies for 6MWT (93%), PROMIS (87%), and SWAY balance score (80%). Another set of AHC models clustered patients with respect to their response to treatment with elamipretide with perfect accuracy (all 100%). CONCLUSIONS: In this proof-of-concept study, we demonstrated that continuously acquired physiological measurements from wearable devices can be used to predict functional status and response to treatment among patients with BTHS.
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Síndrome de Barth , Humanos , Fatores de Tempo , Cardiolipinas , FadigaRESUMO
BACKGROUND: The segmental relationship between cardiovascular magnetic resonance (CMR) peak circumferential strain (Ecc) and myocardial scar has not been well characterized in Duchenne muscular dystrophy (DMD), and it is unknown whether echocardiography accurately measures Ecc in DMD. We assessed segmental Ecc and scar using CMR with myocardial tissue tagging and late gadolinium enhancement (LGE) in patients with DMD, then compared CMR with echocardiographic velocity vector imaging (VVI) for regional Ecc based on independent observer assessments. RESULTS: Participants enrolled (n = 16; age 8-23) had median left ventricular (LV) ejection fraction of 0.52 (range 0.28-0.69), and 156 basal and mid-cavity myocardial segments from the 13 patients completing the LGE protocol were analyzed for strain and scar. Segmental CMR Ecc in the most negative quartile (quartile 4) ruled out scar in that segment, but scar was present in 46% of segments in the least negative (most dysfunctional) Ecc quartile 1, 33% of Ecc quartile 2 segments, and 15% of Ecc quartile 3 segments. Overall scar prevalence in inferior, inferolateral, and anterolateral segments was eight times higher than in inferoseptal, anteroseptal, and anterior segments (p < 0.001). This increased proportion of scar in lateral versus septal segments was consistent across CMR Ecc quartiles (quartile 1: 76% versus 11%, p = 0.001; quartile 2: 65% versus 9%, p < 0.001; quartile 3: 38% versus 0%, p < 0.001). Echocardiographic analysis could be performed in 12 of 14 patients with CMR exams and had to be limited to mid-cavity slices. Echo segmental Ecc in the most negative quartile made scar by CMR in that segment highly unlikely, but the correlation in segmental Ecc between CMR and echo was limited (r = 0.27; p = 0.02). CONCLUSIONS: The relationship between scar and Ecc in DMD is complex. Among myocardial segments with depressed Ecc, scar prevalence was much higher in inferior, inferolateral, and anterolateral segments, indicating a regionally dependent association between abnormal Ecc and scar, with free wall segments commonly developing dysfunction with scar and septal segments developing dysfunction without scar. Although normal echocardiographic Ecc predicted absence of scar, regional echocardiographic Ecc by VVI has only a limited association with CMR Ecc in DMD.
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Cicatriz/epidemiologia , Distrofia Muscular de Duchenne/epidemiologia , Miocárdio/patologia , Disfunção Ventricular Esquerda/epidemiologia , Função Ventricular Esquerda , Adolescente , Baltimore/epidemiologia , Distribuição de Qui-Quadrado , Criança , Cicatriz/diagnóstico , Cicatriz/etiologia , Cicatriz/fisiopatologia , Meios de Contraste , Ecocardiografia , Gadolínio DTPA , Humanos , Modelos Lineares , Imagem Cinética por Ressonância Magnética , Distrofia Muscular de Duchenne/complicações , Distrofia Muscular de Duchenne/diagnóstico , Distrofia Muscular de Duchenne/fisiopatologia , Variações Dependentes do Observador , Valor Preditivo dos Testes , Prevalência , Reprodutibilidade dos Testes , Volume Sistólico , Disfunção Ventricular Esquerda/diagnóstico , Disfunção Ventricular Esquerda/etiologia , Disfunção Ventricular Esquerda/fisiopatologia , Adulto JovemRESUMO
Heart murmur, a thoracic auscultatory finding of cardiovascular origin, is extremely common in childhood and can appear at any age from premature newborn to late adolescence. The objective of this review is to provide a modern examination and update of cardiac murmur auscultation in this new era of artificial intelligence (AI) and telemedicine. First, we provide a comprehensive review of the causes and differential diagnosis, clinical features, evaluation, and long-term management of paediatric heart murmurs. Next, we provide a brief history of computer-assisted auscultation and murmur analysis, along with insight into the engineering design of the digital stethoscope. We conclude with a discussion of the paradigm shifting impact of deep learning on murmur analysis, AI-assisted auscultation, and the implications of these technologies on telemedicine in paediatric cardiology. It is our hope that this article provides an updated perspective on the impact of AI on cardiac auscultation for the modern paediatric cardiologist.
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INTRODUCTION: Cardiac auscultation skills have proven difficult to train and maintain. The authors investigated whether using phonocardiograms as visual adjuncts to audio cases improved first-year medical students' cardiac auscultation performance. METHODS: The authors randomized 135 first-year medical students using an email referral link in 2018 and 2019 to train using audio-only cases (audio group) or audio with phonocardiogram tracings (combined group). Training included 7 cases with normal and abnormal auscultation findings. The assessment included feature identification and diagnostic accuracy using 14 audio-only cases, 7 presented during training, and 7 alternate versions of the same diagnoses. The assessment-administered immediately after training and repeated 7 days later-prompted participants to identify the key features and diagnoses for 14 audio-only cases. Key feature scores and diagnostic accuracy were compared between groups using repeated measures ANOVA. RESULTS: Mean key feature scores were statistically significantly higher in the combined group (70%, 95% CI 67-75%) compared to the audio group (61%, 95% CI 56-66%) (F(1,116)â¯= 6.144, pâ¯= 0.015, dsâ¯= 0.45). Similarly, mean diagnostic accuracy in the combined group (68%, 95% CI 62-73%) was significantly higher than the audio group, although with small effect size (59%, 95% CI 54-65%) (F(1,116)â¯= 4.548, pâ¯= 0.035, dsâ¯= 0.40). Time on task for the assessment and prior auscultation experience did not significantly impact performance on either measure. DISCUSSION: The addition of phonocardiograms to supplement cardiac auscultation training improves diagnostic accuracy and heart sound feature identification amongst novice students compared to training with audio alone.
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Auscultação Cardíaca , Estudantes de Medicina , Competência Clínica , Humanos , FonocardiografiaRESUMO
BACKGROUND AND PURPOSE: The limits of cerebral blood flow-pressure autoregulation have not been adequately defined for pediatric patients. Mean arterial blood pressure below these limits might contribute to brain injury during cardiac surgery. The purpose of this pilot study was to assess a novel method of determining the lower limits of pressure autoregulation in pediatric patients supported with cardiopulmonary bypass. METHODS: A prospective, observational pilot study was conducted in children (n=54) undergoing cardiac surgery with cardiopulmonary bypass for correction of congenital heart defects. Cerebral oximetry index (COx) was calculated as a moving, linear correlation coefficient between slow waves of arterial blood pressure and cerebral oximetry measured with near-infrared spectroscopy. An autoregulation curve was constructed for each patient with averaged COx values sorted by arterial blood pressure. RESULTS: Hypotension was associated with increased values of COx (P<0.0001). For 77% of patients, an individual estimate of lower limits of pressure autoregulation could be determined using a threshold COx value of 0.4. The mean lower limits of pressure autoregulation for the cohort using this method was 42+/-7 mm Hg. CONCLUSIONS: This pilot study of COx monitoring in pediatric patients demonstrates an association between hypotension during cardiopulmonary bypass and impairment of autoregulation. The COx may be useful to identify arterial blood pressure-dependent limits of cerebral autoregulation during cardiopulmonary bypass. Larger trials with neurological outcomes are indicated.
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Ponte Cardiopulmonar/instrumentação , Circulação Cerebrovascular/fisiologia , Homeostase/fisiologia , Monitorização Intraoperatória/métodos , Espectroscopia de Luz Próxima ao Infravermelho/métodos , Adolescente , Velocidade do Fluxo Sanguíneo/fisiologia , Pressão Sanguínea/fisiologia , Criança , Pré-Escolar , Feminino , Humanos , Hipotensão/fisiopatologia , Lactente , Recém-Nascido , Modelos Logísticos , Masculino , Oximetria , Projetos Piloto , Estudos ProspectivosRESUMO
AIMS: Congenital coronary artery anomalies are uncommon and may result in sudden death. Management of asymptomatic patients with anomalous aortic origin of the right coronary artery (AAORCA) remains controversial with a lack of evidence to guide decision-making. We hypothesized that patients with AAORCA may have exercise-inducible ischemia detectable as abnormalities in regional myocardial deformation on exercise stress echocardiography (ESE). METHODS: We reviewed clinical data, computed tomography angiography, and treadmill ESE from 33 AAORCA patients (21 unoperated, 12 operated) and 11 controls. Regional wall motion on ESE was visually assessed. Doppler tissue imaging was done pre and post exercise to evaluate regional myocardial wall deformation. The post- to pre-exercise time to peak systolic strain corrected for heart rate ratio (TPScR) for the left ventricular inferior and anterior walls of AAORCA patients was compared to controls. RESULTS: No regional wall motion abnormalities were noted. The TPScR of the inferior wall was higher in unoperated (0.96 ± 0.41) but not operated (0.84 ± 0.28) AAORCA patients compared to controls (0.76 ± 0.18, P = .03 vs .23, respectively). There was no significant difference in TPScR of the anterior wall between unoperated patients and controls (P = .08). CONCLUSION: In some AAORCA patients undergoing ESE, TPScR of the left ventricular inferior wall is elevated, suggestive of ischemia induced by exercise in myocardium supplied by the right coronary artery. Further work is needed to understand the potential role of this finding in risk assessment.
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Aorta Torácica/anormalidades , Circulação Coronária/fisiologia , Anomalias dos Vasos Coronários/diagnóstico , Ecocardiografia sob Estresse/efeitos adversos , Contração Miocárdica/fisiologia , Isquemia Miocárdica/etiologia , Adolescente , Adulto , Criança , Pré-Escolar , Anomalias dos Vasos Coronários/complicações , Anomalias dos Vasos Coronários/fisiopatologia , Eletrocardiografia , Teste de Esforço/efeitos adversos , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Isquemia Miocárdica/diagnóstico , Isquemia Miocárdica/fisiopatologia , Miocárdio , Adulto JovemRESUMO
BACKGROUND: Tube feedings are often needed to achieve the growth and nutrition goals associated with decreased morbidity and mortality in patients with single ventricle anatomy. Variability in feeding method through the interstage period has been previously described, however, comparable information following stage 2 palliation is lacking. OBJECTIVES: To identify types of feeding methods following stage 2 palliation and their influence on length of stay. DESIGN: Secondary analysis of the National Pediatric Cardiology Quality Improvement Collaborative registry was performed on 932 patients. Demographic data, medical characteristics, postoperative complications, type of feeding method, and length of stay for stage 2 palliation were analyzed. RESULTS: Type of feeding method remained relatively unchanged during hospitalization for stage 2 palliation. Gastrostomy tube fed only patients were the oldest at time of surgery (182.7 ± 57.7 days, P < .001) and had the lowest weight-for-age z scores at admission (-1.6 ± 1.4, P < .001). Oral + gastrostomy tube groups had the longest median bypass times (172.5 minutes, P = .001) and longest length of stay (median 12 days, P < .001). Multivariable modeling revealed that feeding by tube only (P < .001), oral + tube feeding (P ≤ .001), reintubation (P < .001), and prolonged intubation (P < .001) were associated with increased length of stay. Neither age (P = .156) nor weight-for-age z score at admission (P = .066) was predictive of length of stay. CONCLUSIONS: Feeding methods established at admission for stage 2 palliation are not likely to change by discharge. Length of stay is more likely to be impacted by tube feeding and intubation history than age or weight-for-age z score at admission. Better understanding for selection of feeding methods and their impact on patient outcomes is needed to develop evidence-based guidelines to decrease variability in clinical practice patterns and provide appropriate counseling to caregivers.
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Procedimentos Cirúrgicos Cardíacos , Métodos de Alimentação , Cardiopatias Congênitas/cirurgia , Ventrículos do Coração/cirurgia , Hospitalização , Fatores Etários , Alimentação com Mamadeira , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Desenvolvimento Infantil , Nutrição Enteral , Métodos de Alimentação/efeitos adversos , Métodos de Alimentação/instrumentação , Feminino , Gastrostomia , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/fisiopatologia , Ventrículos do Coração/anormalidades , Ventrículos do Coração/diagnóstico por imagem , Ventrículos do Coração/fisiopatologia , Humanos , Lactente , Fenômenos Fisiológicos da Nutrição do Lactente , Tempo de Internação , Masculino , Estado Nutricional , Cuidados Paliativos , Sistema de Registros , Estudos Retrospectivos , Fatores de Risco , Fatores de Tempo , Resultado do TratamentoRESUMO
BACKGROUND: As many as 50-70% of asymptomatic children referred for specialist evaluation or echocardiography because of a murmur have no heart disease. HYPOTHESIS: Computer-assisted auscultation (CAA) can improve the sensitivity and specificity of referrals for evaluation of heart murmurs. METHODS: Seven board-certified primary care physicians were evaluated both without and with use of a computer-based decision-support system using 100 prerecorded patient heart sounds (55 innocent murmurs, 30 pathological murmurs, 15 without murmur). The sensitivity and specificity of their murmur referral decisions relative to American College of Cardiology/American Heart Association (ACC/AHA) guidelines, and sensitivity and specificity of murmur detection and characterization (innocent versus pathological) were measured. RESULTS: Sensitivity for detection of murmurs significantly increased with use of CAA from 76.6 to 89.1% (p <0.001), while specificity remained unaffected (80.0 versus 81.0%). Computer-assisted auscultation improved sensitivity of correctly identifying pathological murmur cases from 82.4 to 90.0%, and specificity of correctly identifying benign cases (with innocent or no murmurs) from 74.9 to 88.8%. (p <0.001). Referral sensitivity increased from 86.7 to 92.9%, while specificity increased from 63.5 to 78.6% using CAA (p <0.001). CONCLUSIONS: Computer-assisted auscultation appears to be a promising new technology for informing the referral decisions of primary care physicians.
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Sistemas de Apoio a Decisões Clínicas , Diagnóstico por Computador , Auscultação Cardíaca/instrumentação , Sopros Cardíacos/diagnóstico , Sopros Cardíacos/fisiopatologia , Encaminhamento e Consulta , Continuidade da Assistência ao Paciente , Indicadores Básicos de Saúde , Sopros Cardíacos/diagnóstico por imagem , Hemodinâmica , Humanos , Processamento de Imagem Assistida por Computador , Sensibilidade e Especificidade , UltrassonografiaRESUMO
Duchenne muscular dystrophy (DMD) results in a progressive cardiomyopathy that produces significant morbidity and mortality. To improve the quality of life in patients with DMD, cardiac care is focused on surveillance and management, with the goal of slowing the onset and progression of heart failure complications. The current article is intended to be an expanded review on the cardiac management data used to inform the 2018 DMD Care Considerations recommendations as well as be a discussion on clinical controversies and future management directions. The new cardiac guidance includes changes regarding noninvasive imaging surveillance of cardiac function and pharmacologic therapy. Many emerging therapies lack sufficient evidence-based data to be recommended in the 2018 DMD Care Considerations. These are discussed in the present article as clinical controversies and future directions. Important emerging therapies include new heart failure medications, mechanical circulatory support with ventricular assist devices, heart transplantation, and internal cardiac defibrillators. Future research studies should be focused on the risks and benefits of these advanced therapies in patients with DMD. We conclude this review with a brief discussion on the relationship between the heart and the recently developed medications that are used to directly target the absence of dystrophin in DMD.
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Cardiomiopatias/terapia , Insuficiência Cardíaca/terapia , Distrofia Muscular de Duchenne/complicações , Cardiomiopatias/etiologia , Insuficiência Cardíaca/etiologia , Transplante de Coração , Coração Auxiliar , Humanos , Terapia de Alvo Molecular/métodos , Distrofia Muscular de Duchenne/terapia , Guias de Prática Clínica como AssuntoRESUMO
Although patients with bicuspid aortic valves (BAVs) are predisposed to ascending aortic (AA) dilation, stenosis, and dissection, the development of aortic disease in children with BAVs is poorly described. The purposes of this study were to determine the rate of change of AA diameter in children with BAVs and to identify risk factors for the development of aortic dilation. The echocardiograms of 276 children aged<19 years (mean 8.5+/-5.3) with isolated BAVs were reviewed. Aortic measurements were normalized to z scores on the basis of body surface area. In a subset of 112 patients with serial examinations, aortic growth rates were calculated and risk factors for more rapid aortic growth determined. At presentation, 33 patients (12%) demonstrated marked AA dilation (z>4), and 70 (25%) were moderately abnormal (z between 2 and 4). The mean+/-SD AA diameter increased more than expected, at a rate of 0.18+/-0.30 z score per year (p<0.0001). In 61 patients with normal AA diameters on initial study, 22 (36%) had abnormal diameters, with z scores>2, at follow-up. Univariate analysis demonstrated right-noncoronary commissural fusion (p<0.02) and aortic valve gradient on initial examination (p<0.02) as significant predictors of AA growth. In multivariate analysis, however, the significance of gradient and valve morphology was diminished (p = 0.06 for both). In conclusion, the progression of AA diameter in patients with normal z scores on initial examination suggests that serial echocardiograms are required to screen for the development of significant aortic dilation.
Assuntos
Doenças da Aorta/etiologia , Valva Aórtica/anormalidades , Valva Mitral/anormalidades , Adolescente , Análise de Variância , Doenças da Aorta/diagnóstico por imagem , Doenças da Aorta/fisiopatologia , Valva Aórtica/diagnóstico por imagem , Insuficiência da Valva Aórtica/complicações , Insuficiência da Valva Aórtica/congênito , Estenose da Valva Aórtica/complicações , Estenose da Valva Aórtica/congênito , Pressão Sanguínea , Criança , Pré-Escolar , Estudos Transversais , Dilatação Patológica/etiologia , Progressão da Doença , Ecocardiografia Doppler , Feminino , Seguimentos , Humanos , Lactente , Modelos Lineares , Estudos Longitudinais , Masculino , Valva Mitral/diagnóstico por imagem , Valor Preditivo dos Testes , Projetos de Pesquisa , Fatores de Risco , Índice de Gravidade de DoençaRESUMO
Auscultation of the heart using a simple stethoscope continues to be a central aspect of the cardiovascular examination despite declining proficiency and availability of competing technologies such as hand-held ultrasound. In the ears and mind of a trained cardiologist, heart sounds can provide important information to help screen for certain diseases such as valvar lesions and many congenital defects. Using emerging technology, auscultation is poised to undergo a transformation that will simultaneously improve the teaching and evaluation of this important clinical skill and create a new generation of smart stethoscopes, capable of assisting the clinician in quickly and confidently screening for heart disease. These developments have important implications for global health, screening of athletes and recognition of congenital heart disease.