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OBJECTIVE: Individuals with single ventricle physiology who are palliated with superior cavopulmonary anastomosis (Glenn surgery) may develop pulmonary arteriovenous malformations. The traditional tools for pulmonary arteriovenous malformation diagnosis are often of limited diagnostic utility in this patient population. We sought to measure the pulmonary capillary transit time to determine its value as a tool to identify pulmonary arteriovenous malformations in patients with single ventricle physiology. METHODS: We defined the angiographic pulmonary capillary transit time as the number of cardiac cycles required for transit of contrast from the distal pulmonary arteries to the pulmonary veins. Patients were retrospectively recruited from a single quaternary North American paediatric centre, and angiographic and clinical data were reviewed. Pulmonary capillary transit time was calculated in 20 control patients and compared to 20 single ventricle patients at the pre-Glenn, Glenn, and Fontan surgical stages (which were compared with a linear-mixed model). Correlation (Pearson) between pulmonary capillary transit time and haemodynamic and injection parameters was assessed using angiograms from 84 Glenn patients. Five independent observers calculated pulmonary capillary transit time to measure reproducibility (intraclass correlation coefficient). RESULTS: Mean pulmonary capillary transit time was 3.3 cardiac cycles in the control population, and 3.5, 2.4, and 3.5 in the pre-Glenn, Glenn, and Fontan stages, respectively. Pulmonary capillary transit time in the Glenn population did not correlate with injection conditions. Intraclass correlation coefficient was 0.87. CONCLUSIONS: Pulmonary angiography can be used to calculate the pulmonary capillary transit time, which is reproducible between observers. Pulmonary capillary transit time accelerates in the Glenn stage, correlating with absence of direct hepatopulmonary venous flow.
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OBJECTIVE: The study aimed to alert the neonatal community to the possibility of multisystem inflammatory syndrome in children (MIS-C) like disease in critically ill neonates born to mothers with coronavirus disease 2019 (COVID-19). STUDY DESIGN: Diagnosis of MIS-C like disease was pursued after echocardiography showed severely depressed ventricular function and pathological coronary artery dilation in the setting of medically refractory multisystem organ failure and maternal COVID-19 infection. The neonate did not respond to standard medical therapy, and there was no alternative disease that could explain the clinical course. High index of clinical suspicion coupled with low risk of intravenous immunoglobulin (IVIG) prompted us to pursue IVIG administration even though the neonate did not meet classic criteria for MIS-C. RESULT: Following treatment with IVIG, there was rapid clinical improvement. Ventricular function improved within 15 hours and coronary artery dilation resolved in 8 days. There was no recurrence of disease during follow-up. CONCLUSION: COVID-19 associated MIS-C like disease has not been well described in neonates. As typical features may be conspicuously absent, a high index of suspicion is warranted in critically ill neonates born to mothers with COVID-19. Echocardiography may provide critical diagnostic information and narrow the differential diagnosis. KEY POINTS: · COVID-19 associated MIS-C can present in neonates.. · Echocardiography is helpful in raising suspicion for MIS-C in neonates.. · Consider MIS-C in the differential diagnosis of ill neonates born to mothers with COVID-19..
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COVID-19 , Estado Terminal/terapia , Ecocardiografia/métodos , Imunoglobulinas Intravenosas/administração & dosagem , Doenças do Recém-Nascido , Complicações Infecciosas na Gravidez , Síndrome de Resposta Inflamatória Sistêmica , COVID-19/complicações , COVID-19/diagnóstico , COVID-19/epidemiologia , COVID-19/fisiopatologia , COVID-19/terapia , COVID-19/virologia , Vasos Coronários/diagnóstico por imagem , Vasos Coronários/efeitos dos fármacos , Diagnóstico Diferencial , Feminino , Humanos , Fatores Imunológicos/administração & dosagem , Recém-Nascido , Doenças do Recém-Nascido/diagnóstico , Doenças do Recém-Nascido/fisiopatologia , Doenças do Recém-Nascido/terapia , Doenças do Recém-Nascido/virologia , Gravidez , Complicações Infecciosas na Gravidez/epidemiologia , Complicações Infecciosas na Gravidez/virologia , SARS-CoV-2/isolamento & purificação , Síndrome de Resposta Inflamatória Sistêmica/diagnóstico , Síndrome de Resposta Inflamatória Sistêmica/fisiopatologia , Síndrome de Resposta Inflamatória Sistêmica/terapia , Síndrome de Resposta Inflamatória Sistêmica/virologia , Resultado do Tratamento , Função Ventricular/efeitos dos fármacosRESUMO
OBJECTIVES: We sought to develop a scoring system that predicts the risk of serious adverse events (SAE's) for individual pediatric patients undergoing cardiac catheterization procedures. BACKGROUND: Systematic assessment of risk of SAE in pediatric catheterization can be challenging in view of a wide variation in procedure and patient complexity as well as rapidly evolving technology. METHODS: A 10 component scoring system was originally developed based on expert consensus and review of the existing literature. Data from an international multi-institutional catheterization registry (CCISC) between 2008 and 2013 were used to validate this scoring system. In addition we used multivariate methods to further refine the original risk score to improve its predictive power of SAE's. RESULTS: Univariate analysis confirmed the strong correlation of each of the 10 components of the original risk score with SAE attributed to a pediatric cardiac catheterization (P < 0.001 for all variables). Multivariate analysis resulted in a modified risk score (CRISP) that corresponds to an increase in value of area under a receiver operating characteristic curve (AUC) from 0.715 to 0.741. CONCLUSION: The CRISP score predicts risk of occurrence of an SAE for individual patients undergoing pediatric cardiac catheterization procedures.
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Cateterismo Cardíaco/efeitos adversos , Técnicas de Apoio para a Decisão , Cardiopatias Congênitas/terapia , Pediatria/métodos , Adolescente , Fatores Etários , Área Sob a Curva , Distribuição de Qui-Quadrado , Criança , Pré-Escolar , Cardiopatias Congênitas/diagnóstico , Humanos , Lactente , Recém-Nascido , Modelos Logísticos , Análise Multivariada , Valor Preditivo dos Testes , Curva ROC , Sistema de Registros , Reprodutibilidade dos Testes , Medição de Risco , Fatores de Risco , Resultado do TratamentoRESUMO
OBJECTIVES: We examine normalized air Kerma area product (PKA ) by body weight (PKA /BW) as a reference value of radiation dose and benchmark PKA /BW in pediatric laboratories using a multicenter registry database. BACKGROUND: Reduction of radiation dose is an important quality improvement task in pediatric cardiac catheterization laboratories. Physicians need to agree on a standard method of reporting radiation dose that would allow comparisons to be made between operators and institutions. METHODS: This was a multicenter observational study of radiation dose in pediatric laboratories. Patient demographic, procedural and radiation data including fluoroscopic time and PKA (µGy m(2) ) were analyzed. PKA /BW was obtained by indexing PKA to body weight. RESULTS: A total of 8,267 pediatric catheterization procedures (age <18 years) were included from 16 institutions. The procedures consisted of diagnostic (n = 2,827), transplant right ventricular (RV) biopsy (n = 1,172), and interventional catheterizations (n = 4268). PKA correlated with body weight better than with age and best correlated with weight-fluoroscopic time product. PKA /BW showed consistent values across pediatric ages. Interventional catheterizations had the highest PKA /BW (50th, 75th, and 90th percentiles: 72, 151, and 281 µGy m(2) /kg), followed by diagnostic (59, 105, and 175 µGy m(2) /kg) and transplant RV biopsy (27, 79, and 114 µGy m(2) /kg). CONCLUSION: PKA /BW appeared to be the most reliable standard to report radiation dose across all procedure types and patient age. We recommend PKA /BW to be used as the standard unit in documenting radiation usage in pediatric laboratories and can be used to evaluate strategies to lower radiation dosage in pediatric patients undergoing cardiac catheterizations. © 2014 Wiley Periodicals, Inc.
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Cateterismo Cardíaco/normas , Doses de Radiação , Proteção Radiológica/normas , Radiografia Intervencionista/normas , Adolescente , Fatores Etários , Serviço Hospitalar de Cardiologia , Criança , Pré-Escolar , Fluoroscopia/efeitos adversos , Fluoroscopia/métodos , Humanos , Lactente , Laboratórios Hospitalares , Segurança do Paciente , Pediatria/métodos , Sistema de Registros , Medição de Risco , Estatísticas não Paramétricas , Fatores de TempoRESUMO
Background: Individuals with single ventricle physiology who are palliated with superior cavopulmonary anastomosis (Glenn surgery) may develop pulmonary arteriovenous malformations (PAVMs). The traditional tools for PAVM diagnosis are often of limited diagnostic utility in this patient population. We sought to measure the pulmonary capillary transit time (PCTT) to determine its value as a tool to identify PAVMs in patients with single ventricle physiology. Methods: We defined the angiographic PCTT as the number of cardiac cycles required for transit of contrast from the distal pulmonary arteries to the pulmonary veins. Patients were retrospectively recruited from a single quaternary North American pediatric center, and angiographic and clinical data was reviewed. PCTT was calculated in 20 control patients and compared to 20 single ventricle patients at the pre-Glenn, Glenn, and Fontan surgical stages (which were compared with a linear-mixed model). Correlation (Pearson) between PCTT and hemodynamic and injection parameters was assessed using 84 Glenn angiograms. Five independent observers calculated PCTT to measure reproducibility (intra-class correlation coefficient). Results: Mean PCTT was 3.3 cardiac cycles in the control population, and 3.5, 2.4, and 3.5 in the pre-Glenn, Glenn, and Fontan stages, respectively. PCTT in the Glenn population did not correlate with injection conditions. Intraclass correlation coefficient was 0.87. Conclusions: Pulmonary angiography can be used to calculate the pulmonary capillary transit time, which is reproducible between observers. PCTT accelerates in the Glenn stage, correlating with absence of direct hepatopulmonary venous flow.
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INTRODUCTION: Multidisciplinary Pulmonary Embolism Response Teams (PERTs) streamline care of adults with life-threatening pulmonary embolism (PE). Given rarity of pediatric PE, developing a clinical, educational, and research PERT paradigm is a novel and underutilized concept in pediatrics. RESEARCH QUESTION: Is PERT feasible in pediatrics, and does it improve PE care? STUDY DESIGN AND METHODS: A strategy-to-execution proposal to launch a pediatric PERT was developed for institutional buy-in. Key stakeholders collectively implemented PERT. Data were collected for the two-year pre- and post-PERT eras, and outcomes were compared. RESULTS: PERT implementation took 12 months. Our PERT, led by hematology, comprises of pediatric experts in emergency medicine, critical care, interventional cardiology, anesthesiology, and interventional radiology. Data on 30 patients pre-PERT and 31 post-PERT were analyzed. Pre-PERT, 10%(3/30), 13%(4/30), 20%(6/30), and 57%(17/30), and post-PERT, 3%(1/31), 10%(3/31), 16%(5/31), and 71%(22/31) were categorized as high-risk, intermediate-LOW risk, intermediate-HIGH risk, and low-risk PE, respectively. Post-PERT, there were 13 unique PERT activations. PERT was activated on all eligible PE patients and, additionally, on four low-risk PEs. Time-to-echocardiogram was shorter post-PERT (4.7 hrs vs 2 hrs, P=0.0147). Anticoagulation was ordered (90 min vs 54 min, P=0.003) and given sooner (154 min vs 113 min, P=0.049) post-PERT. There were no differences in time-to-reperfusion therapies (12 hrs pre-PERT vs 8.7 hrs post-PERT, P=0.1). Five (83.3%) of six eligible (intermediate-HIGH and high-risk) patients received reperfusion therapies in the post-PERT era compared to three (37.5%) of eight eligible patients in the pre-PERT era (P=0.0001). There were no differences in major bleeding, mortality, or length of stay in either era. INTERPRETATION: The pediatric PERT paradigm was successfully created and adopted locally. Our PERT enhanced access to experts, facilitated timely advanced therapies, and held value for low-risk PE. The University of Texas Southwestern Medical Center (UTSW) and Children's Health System of Texas pediatric PERT may serve as a best-practice model for streamlining care for pediatric PE.
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PURPOSE: To compare measurements of regional pulmonary perfused blood volume (PBV) and pulmonary blood flow (PBF) obtained with computed tomography (CT) in two pig models. MATERIALS AND METHODS: The institutional animal care and use committee approved all animal studies. CT-derived PBF and PBV were determined in four anesthetized, mechanically ventilated, supine swine by using two methods for creating pulmonary parenchymal perfusion heterogeneity. Two animals were examined after sequentially moving a pulmonary arterial balloon catheter from a distal to a central location, and two others were examined over a range of static airway pressures, which varied the extents of regional PBF. Lung sections were divided into blocks and Pearson correlation coefficients calculated to compare matching regions between the two methods. RESULTS: CT-derived PBF, CT-derived PBV, and their associated coefficients of variation (CV) were closely correlated on a region-by-region basis in both the balloon occlusion (Pearson R = 0.91 and 0.73 for animals 1 and 2, respectively; Pearson R = 0.98 and 0.87 for comparison of normalized mean and CV for animals 1 and 2, respectively) and lung inflation studies (Pearson R = 0.94 and 0.74 for animals 3 and 4, respectively; Pearson R = 0.94 and 0.69 for normalized mean and CV for animals 3 and 4, respectively). When accounting for region-based effects, correlations remained highly significant at the P < .001 level. CONCLUSION: CT-derived PBV heterogeneity is a suitable surrogate for CT-derived PBF heterogeneity.
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Velocidade do Fluxo Sanguíneo/fisiologia , Volume Sanguíneo/fisiologia , Pulmão/irrigação sanguínea , Pulmão/diagnóstico por imagem , Tomografia Computadorizada Multidetectores/métodos , Animais , Meios de Contraste/administração & dosagem , Iopamidol/administração & dosagem , Modelos Animais , SuínosRESUMO
Guidelines for the treatment of active right-sided endocarditis are not well defined. We report the first novel use of an aspiration catheter system for transcatheter therapy of refractory active right sided endocarditis on a bioprosthetic valve in a high-risk surgical patient as a bridge to surgical intervention.
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Cateterismo Cardíaco/instrumentação , Cateteres Cardíacos , Endocardite Bacteriana/terapia , Implante de Prótese de Valva Cardíaca/efeitos adversos , Próteses Valvulares Cardíacas/efeitos adversos , Infecções Relacionadas à Prótese/terapia , Valva Pulmonar/cirurgia , Infecções Estafilocócicas/microbiologia , Infecções Estafilocócicas/terapia , Adolescente , Antibacterianos/uso terapêutico , Ecocardiografia Transesofagiana , Endocardite Bacteriana/diagnóstico , Endocardite Bacteriana/microbiologia , Endocardite Bacteriana/cirurgia , Desenho de Equipamento , Implante de Prótese de Valva Cardíaca/instrumentação , Humanos , Masculino , Infecções Relacionadas à Prótese/diagnóstico , Infecções Relacionadas à Prótese/etiologia , Infecções Relacionadas à Prótese/cirurgia , Infecções Estafilocócicas/diagnóstico , Infecções Estafilocócicas/cirurgia , Staphylococcus aureus/isolamento & purificação , Sucção , Tomografia Computadorizada por Raios X , Resultado do TratamentoRESUMO
Transcatheter device intervention is now offered as first line therapy for many congenital heart defects (CHD) which were traditionally treated with cardiac surgery. While off-label use of devices is common and appropriate, a growing number of devices are now specifically designed and approved for use in CHD. Advanced imaging is now an integral part of interventional procedures including pre-procedure planning, intra-procedural guidance, and post-procedure monitoring. There is robust societal and industrial support for research and development of CHD-specific devices, and the regulatory framework at the national and international level is patient friendly. It is against this backdrop that we review transcatheter implantable devices for CHD, the role and integration of advanced imaging, and explore the current regulatory framework for device approval.
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The need for transvenous pacing (patients who have exhausted epicardial options) after a Fontan-type operation has been recognized. Many novel strategies have been proposed, but currently, all of them require additional maneuvers or rerouting of the leads to the pacemaker pocket. In this report, we describe a novel direct approach to transvenous pacing after a Fontan-type operation from a standard, prepectoral approach.
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In this report, we describe a 15-year-old patient who underwent a Ross procedure for a regurgitant bicuspid aortic valve and ascending aortic dilation. After the operation was over, he could not be separated from cardiopulmonary bypass and was noted to have isolated right ventricular failure. This report takes the reader through the diagnostic evaluation, highlights the importance of invasive assessment in the immediate postoperative period, and discusses successful transcatheter intervention in the acute postoperative setting.
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The pig is frequently used as an experimental model for studies of the pulmonary circulation, yet the branching and dimensional geometry of the porcine pulmonary vasculature remains poorly defined. The purposes of this study are to improve the geometric definition of the porcine pulmonary arteries and to determine whether the arterial tree exhibits self-similarity in its branching geometry. Five animals were imaged using thin slice spiral computed tomography in the prone posture during airway inflation pressure at 25 cmH2O. The luminal diameter and distance from the inlet of the left and right pulmonary arteries were measured along the left and right main arterial pathway in each lung of each animal. A further six minor pathways were measured in a single animal. The similarity in the rate of reduction of diameter with distance of all minor pathways and the two main pathways, along with similarity in the number of branches arising along the pathways, supports self-similarity in the arterial tree. The rate of reduction in diameter with distance from the inlet was not significantly different among the five animals (P > 0.48) when normalized for main pulmonary artery diameter and total main artery pathlength, which supports intersubject similarity. Other metrics to quantify the tree geometry are strikingly similar to those from airways of other quadrupeds, with the exception of a significantly larger length to diameter ratio, which is more appropriate for the vascular tree. A simplifying self-similar model for the porcine pulmonary arteries is proposed to capture the important geometric features of the arterial tree.
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Artéria Pulmonar/anatomia & histologia , Artéria Pulmonar/diagnóstico por imagem , Suínos/anatomia & histologia , Animais , Feminino , Imageamento Tridimensional , Masculino , Modelos Anatômicos , Modelos Cardiovasculares , Tomografia Computadorizada Multidetectores , Circulação PulmonarRESUMO
BACKGROUND: Intravascular ultrasound (IVUS) measures prognostically important pulsatile flow indexes in patients with pulmonary hypertension (PH). IVUS catheters traditionally require a guiding catheter for placement which can impact hemodynamics in small infants because the guiding catheter renders the atrioventricular valve incompetent. METHODS: Domestic swine (1.4-2.2 kg) were raised in isobaric normoxia (n = 4) or hypoxia (n = 3, FiO(2) 10-12%) for 72 hr for induction of PH. Cardiac catheterization and intravascular imaging was performed using a 3.5-Fr 20-MHz Eagle Eye Gold catheter (Volcano Corp., CA, USA) over a 0.014'' guide wire. Intima-media thickness (IMT) was measured and relative area change and vascular pulsatility were calculated. RESULTS: The IVUS probe was easily manipulated over a 0.014'' wire without hemodynamic compromise in all animals. The IMT was thicker in the hypoxic group than the normoxic group (0.19 ± 0.03 mm vs. 0.31 ± 0.04 mm, p = .067). Hypoxic animals had systolic PH (39.66 ± 2.51 vs. 21.75 ± 2.87 mmHg, p = .02). Systemic arterial pressures between the groups were the same (hypoxic 68 ± 10.44 vs. normoxic 79.75 ± 14.84 mmHg, p = .26). Vascular pulsatility was similar (hypoxic 24 ± 2.64 vs. 20.25 ± 0.57%, p = .18). However, the arterial wall distensibility was significantly different (0.98 ± 0.2 vs. 2.01 ± 1.38 %/mmHg, p = .04). CONCLUSIONS: Monorail IVUS imaging without a guide catheter overcomes a major limitation for use in infants and small animal experimental models by avoiding hemodynamic compromise. This would be a valuable tool for assessment of PH in the research and clinical setting.
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Hemodinâmica/fisiologia , Hipertensão Pulmonar/diagnóstico por imagem , Ultrassonografia de Intervenção/instrumentação , Ultrassonografia de Intervenção/métodos , Animais , Cateterismo Cardíaco/instrumentação , Cateterismo Cardíaco/métodos , Modelos Animais de Doenças , Estudos de Viabilidade , Hipertensão Pulmonar/fisiopatologia , Fluxo Sanguíneo Regional/fisiologia , Suínos , Túnica Íntima/diagnóstico por imagem , Túnica Média/diagnóstico por imagemRESUMO
Coronary artery compression is a rare and potentially fatal complication after pulmonary valve replacement. This report describes myocardial infarction from extrinsic left main coronary artery compression after pulmonary valve replacement in a 10-y-old boy. He was successfully treated with percutaneous coronary intervention.