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BACKGROUND: High energy requirements and poor feeding can lead to growth failure in patients with ventricular septal defect (VSD), but effects of preoperative malnutrition on surgical outcomes are poorly understood, especially in low-resource settings. METHODS AND RESULTS: We analyzed a cohort of children <5 years of age undergoing VSD closure at 60 global centers participating in the International Quality Improvement Collaborative for Congenital Heart Disease, 2015 to 2020. We calculated adjusted odds ratios (ORs) for in-hospital death and major infection and adjusted coefficients for duration of intensive care unit stay for 4 measures of malnutrition: severe wasting (weight-for-height Z score, <-3), moderate wasting (-3Assuntos
Comunicação Interventricular
, Mortalidade Hospitalar
, Tempo de Internação
, Desnutrição
, Humanos
, Comunicação Interventricular/cirurgia
, Comunicação Interventricular/mortalidade
, Comunicação Interventricular/complicações
, Masculino
, Feminino
, Lactente
, Pré-Escolar
, Tempo de Internação/estatística & dados numéricos
, Desnutrição/mortalidade
, Desnutrição/epidemiologia
, Desnutrição/diagnóstico
, Fatores de Risco
, Procedimentos Cirúrgicos Cardíacos/efeitos adversos
, Unidades de Terapia Intensiva/estatística & dados numéricos
, Estado Nutricional
, Complicações Pós-Operatórias/epidemiologia
, Complicações Pós-Operatórias/mortalidade
, Estudos Retrospectivos
, Medição de Risco
, Fatores de Tempo
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The human voice has the potential to serve as a valuable biomarker for the early detection, diagnosis, and monitoring of pediatric conditions. This scoping review synthesizes the current knowledge on the application of artificial intelligence (AI) in analyzing pediatric voice as a biomarker for health. The included studies featured voice recordings from pediatric populations aged 0-17 years, utilized feature extraction methods, and analyzed pathological biomarkers using AI models. Data from 62 studies were extracted, encompassing study and participant characteristics, recording sources, feature extraction methods, and AI models. Data from 39 models across 35 studies were evaluated for accuracy, sensitivity, and specificity. The review showed a global representation of pediatric voice studies, with a focus on developmental, respiratory, speech, and language conditions. The most frequently studied conditions were autism spectrum disorder, intellectual disabilities, asphyxia, and asthma. Mel-Frequency Cepstral Coefficients were the most utilized feature extraction method, while Support Vector Machines were the predominant AI model. The analysis of pediatric voice using AI demonstrates promise as a non-invasive, cost-effective biomarker for a broad spectrum of pediatric conditions. Further research is necessary to standardize the feature extraction methods and AI models utilized for the evaluation of pediatric voice as a biomarker for health. Standardization has significant potential to enhance the accuracy and applicability of these tools in clinical settings across a variety of conditions and voice recording types. Further development of this field has enormous potential for the creation of innovative diagnostic tools and interventions for pediatric populations globally.
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Congenital heart disease (CHD) comprises a range of structural anomalies, each with a unique natural history, evolving treatment strategies, and distinct long-term consequences. Current prediction models are challenged by generalizability, limited validation, and questionable application to extended follow-up periods. In this JACC Scientific Statement, we tackle the difficulty of risk measurement across the lifespan. We appraise current and future risk measurement frameworks and describe domains of risk specific to CHD. Risk of adverse outcomes varies with age, sex, genetics, era, socioeconomic status, behavior, and comorbidities as they evolve through the lifespan and across care settings. Emerging technologies and approaches promise to improve risk assessment, but there is also need for large, longitudinal, representative, prospective CHD cohorts with multidimensional data and consensus-driven methodologies to provide insight into time-varying risk. Communication of risk, particularly with patients and their families, poses a separate and equally important challenge, and best practices are reviewed.
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Cardiopatias Congênitas , Humanos , Cardiopatias Congênitas/epidemiologia , Medição de Risco/métodos , Fatores de RiscoRESUMO
BACKGROUND: Despite the burden of CHD, a high cost and utilization condition, an implementation of long-term outcome measures is lacking. The objective of this study is to pilot the implementation of the International Consortium of Health Outcomes Measurement CHD standard set in patients undergoing pulmonary valve replacement, a procedure performed in mostly well patients with diverse CHD. METHODS: Patients ≥ 8 years old undergoing catheterization-based pulmonary valve replacement were approached via various approaches for patient-reported outcomes, with a follow-up assessment at 3 months post-procedure. Implementation strategy analysis was performed via a hybrid type 2 design. RESULTS: Of the 74 patients undergoing pulmonary valve replacement, 32 completed initial patient-reported outcomes with variable response rates by strategy (email and in-person explanation 100%, email only 54%, and email followed by text/call 64%). Ages ranged 8-67 years (mean 30). Pre-procedurally, 34% had symptomatic arrhythmias, which improved post-procedure. For those in school, 43% missed ≥ 6 days per year, and over half had work absenteeism. Financial concerns were reported in 34%. Patients reported high satisfaction with life (50% [n = 16]) and health-related quality of life (90% [n = 26]). Depression symptoms were reported in 84% (n = 27) and anxiety in 62.5% (n = 18), with tendency towards improvement post-procedurally. CONCLUSION: Pilot implementation of the International Consortium of Health Outcomes Measurement CHD standard set in pulmonary valve replacement patients reveals a significant burden of disease not previously reported. Barriers to the implementation include a sustainable, automated system for patient-reported outcome collection and infrastructure to assess in real time. This provides an example of implementing cardiac outcomes set in clinical practice.
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BACKGROUND: Gaps in care (GIC) are common for patients with congenital heart disease (CHD) and can lead to worsening clinical status, unplanned hospitalization, and mortality. Understanding of how social determinants of health (SDOH) contribute to GIC in CHD is incomplete. We hypothesize that SDOH, including Child Opportunity Index (COI), are associated with GIC in patients with significant CHD. METHODS AND RESULTS: A total of 8554 patients followed at a regional specialty pediatric hospital with moderate to severe CHD seen in cardiology clinic between January 2013 and December 2015 were retrospectively reviewed. SDOH factors including race, ethnicity, language, and COI calculated based on home address and zip code were analyzed. GIC of >3.25 years were identified in 32% (2709) of patients. GIC were associated with ages 14 to 29 years (P<0.001), Black race or Hispanic ethnicity (P<0.001), living ≥150 miles from the hospital (P=0.017), public health insurance (P<0.001), a maternal education level of high school or less (P<0.001), and a low COI (P<0.001). Multivariable analysis showed that GIC were associated with age ≥14 years, Black race or Hispanic ethnicity, documenting <3 caregivers as contacts, mother's education level being high school or less, a very low/low COI, and insurance status (C statistic 0.66). CONCLUSIONS: One-third of patients followed in a regional referral center with significant CHD experienced a substantial GIC (>3.25 years). Several SDOH, including a low COI, were associated with GIC. Hospitals should adopt formal GIC improvement programs focusing on SDOH to improve continuity of care and ultimately overall outcomes for patients with CHD.
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Cardiopatias Congênitas , Determinantes Sociais da Saúde , Criança , Humanos , Adolescente , Estudos Retrospectivos , Cardiopatias Congênitas/diagnóstico , Cardiopatias Congênitas/terapia , Escolaridade , Hospitais PediátricosRESUMO
OBJECTIVE: To determine if socioeconomic status (SES) has a greater effect than standard demographic values on predicted peak oxygen consumption (pVO2). STUDY DESIGN: We conducted a single-institution, retrospective analysis of maximal cardiopulmonary exercise test (CPET) data from 2010 to 2020 for healthy patients age <19 years with body mass index (BMI) percentile (BMI%) between 5-95. Data were sorted by self-identified race, BMI%, and adjusted gross income (AGI); AGI served as a surrogate for SES. Mean percent predicted pVO2 (pppVO2) was compared between groups. Linear regression was used to adjust for differences. RESULTS: A total of 541 CPETs met inclusion criteria. Mean pppVO2 was 97% ± 22.6 predicted (P < .01) with 30% below criterion standard for normal (85% predicted). After excluding unknown AGI and race, 418 CPETs remained. Mean pppVO2 was lower for Blacks (n = 36) and Latinx (n = 26) compared with Whites (n = 333, P < .01). Mean pppVO2 declined as AGI decreased (P < .01). The differences in pppVO2 between racial categories remained significant when adjusted for BMI% (Black r = -7.3, P = .035; Latinx r = -15.4, P < .01). These differences both decreased in magnitude and were no longer significant when adjusted for AGI (Black r = -6.0, P = .150; Latinx r = -9.3, P = .06). CONCLUSIONS: Lower SES correlates with lower measured cardiovascular fitness and may confound data interpretation. When using normative reference ranges in clinical decision making, providers should recognize that social determinants of health may influence predicted fitness. Social inequities should be considered when assessing pediatric cardiovascular fitness.
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Aptidão Física , Classe Social , Fatores Socioeconômicos , Criança , Humanos , Adulto Jovem , Consumo de Oxigênio , Estudos Retrospectivos , Adolescente , Determinantes Sociais da SaúdeRESUMO
The Eighth World Congress of Pediatric Cardiology and Cardiac Surgery (WCPCCS) will be held in Washington DC, USA, from Saturday, 26 August, 2023 to Friday, 1 September, 2023, inclusive. The Eighth World Congress of Pediatric Cardiology and Cardiac Surgery will be the largest and most comprehensive scientific meeting dedicated to paediatric and congenital cardiac care ever held. At the time of the writing of this manuscript, The Eighth World Congress of Pediatric Cardiology and Cardiac Surgery has 5,037 registered attendees (and rising) from 117 countries, a truly diverse and international faculty of over 925 individuals from 89 countries, over 2,000 individual abstracts and poster presenters from 101 countries, and a Best Abstract Competition featuring 153 oral abstracts from 34 countries. For information about the Eighth World Congress of Pediatric Cardiology and Cardiac Surgery, please visit the following website: [www.WCPCCS2023.org]. The purpose of this manuscript is to review the activities related to global health and advocacy that will occur at the Eighth World Congress of Pediatric Cardiology and Cardiac Surgery.Acknowledging the need for urgent change, we wanted to take the opportunity to bring a common voice to the global community and issue the Washington DC WCPCCS Call to Action on Addressing the Global Burden of Pediatric and Congenital Heart Diseases. A copy of this Washington DC WCPCCS Call to Action is provided in the Appendix of this manuscript. This Washington DC WCPCCS Call to Action is an initiative aimed at increasing awareness of the global burden, promoting the development of sustainable care systems, and improving access to high quality and equitable healthcare for children with heart disease as well as adults with congenital heart disease worldwide.
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Procedimentos Cirúrgicos Cardíacos , Cardiologia , Cardiopatias , Adulto , Criança , HumanosRESUMO
The global burden of paediatric and congenital heart disease (PCHD) is substantial. We propose a novel public health framework with recommendations for developing effective and safe PCHD services in low-income and middle-income countries (LMICs). This framework was created by the Global Initiative for Children's Surgery Cardiac Surgery working group in collaboration with a group of international rexperts in providing paediatric and congenital cardiac care to patients with CHD and rheumatic heart disease (RHD) in LMICs. Effective and safe PCHD care is inaccessible to many, and there is no consensus on the best approaches to provide meaningful access in resource-limited settings, where it is often needed the most. Considering the high inequity in access to care for CHD and RHD, we aimed to create an actionable framework for health practitioners, policy makers and patients that supports treatment and prevention. It was formulated based on rigorous evaluation of available guidelines and standards of care and builds on a consensus process about the competencies needed at each step of the care continuum. We recommend a tier-based framework for PCHD care integrated within existing health systems. Each level of care is expected to meet minimum benchmarks and ensure high-quality and family centred care. We propose that cardiac surgery capabilities should only be developed at the more advanced levels on hospitals that have an established foundation of cardiology and cardiac surgery services, including screening, diagnostics, inpatient and outpatient care, postoperative care and cardiac catheterisation. This approach requires a quality control system and close collaboration between the different levels of care to facilitate the journey and care of every child with heart disease. This effort was designed to guide readers and leaders in taking action, strengthening capacity, evaluating impact, advancing policy and engaging in partnerships to guide facilities providing PCHD care in LMICs.
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Países em Desenvolvimento , Cardiopatias Congênitas , Humanos , Criança , Saúde Pública , Cardiopatias Congênitas/cirurgia , Sistema de Registros , Continuidade da Assistência ao PacienteRESUMO
Over 90% of the world's children with congenital heart disease do not have access to cardiac care. Although many models provide pediatric cardiac surgery in low- and middle-income countries, sustainability poses a barrier. We explore one model providing care for the underserved in Chennai, India, that came into existence through trial and error over 30 years across three phases. Phase 1 was a Tamilnadu state government-sponsored program that soon became unsustainable with unmet demands. Phase 2 utilized a grassroots foundation of a public-private partnership (PPP) with few donors and a hospital with suboptimal infrastructure. Phase 3 is the ongoing fine-tuning of the PPP model, with upgraded infrastructure and a well-trained team. Through indigenization, an average cardiac surgery costs Rupees (Rs.) 1,80,000 ($2400). The government funds Rs. 60,000 to 80,000 ($800-$1066.67), and the rest is funded through the fund pool. The goal is to perform 100 free surgeries annually by maintaining a fund pool of Rs. 50 lakhs ($66,666.67), which supplements government funds. This ensures equitable distribution of funds with no compromise on resources (disposables, single-use cannulas, etc). Our model ensures the dignity of the patient, fair compensation for workers, and is practical, affordable, and easily adaptable. Thus far, this model provided free cardiac surgery for 357 children from Risk Adjusted Congenital Heart Surgery Score of 1 to 4, with an overall mortality of 2.73%. The prerequisites for this model are having a "spark plug," a dedicated surgical team, a partnership with state-of-the-art infrastructure, and a steady flow of funds.
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Procedimentos Cirúrgicos Cardíacos , Cardiopatias Congênitas , Cirurgia Torácica , Humanos , Criança , Países em Desenvolvimento , Índia , Cardiopatias Congênitas/cirurgiaRESUMO
Objectives: While procedure length is considered an important metric for cardiothoracic surgical procedures, the relationship between procedure length and adverse events (AEs) in congenital cardiac catheterizations has little published data available. Furthermore, most existing congenital cardiac catheterization risk prediction models are built on logistic regression models. This study aimed to characterize the relationship between case length and AE occurrence in congenital cardiac catheterization while adjusting for known risk factors and to investigate the potential role of non-linear analysis in risk modeling. Design: Age, case type, and procedure duration were evaluated for relationships with the primary outcome using logistic regression. Non-linearity of the associations with continuous risk factors was assessed using restricted cubic spline transformations. Setting and participants: All diagnostic and interventional congenital cardiac catheterization cases performed at Boston Children's Hospital between January 1, 2014 and October 31, 2019 were analyzed. Main outcome measure: The primary outcome was defined as the occurrence of any clinically significant (level 3/4/5) AE. Results: A total of 7011 catheterization cases met inclusion criteria, with interventional procedures accounting for 68% of cases. Median case duration was 97 min. A multivariable model including age, procedure type, and case duration showed a significant relationship between case duration and AE occurrence (OR 1.07 per 10 min increase, 95% CI 1.06 to 1.09, p<0.001). Conclusions: This study demonstrated the importance of procedure duration as a potential frontier for procedure risk management. Better understanding of the role of procedure duration in cardiac catheterizations may provide opportunities for quality improvement in patient safety and resource planning.
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OBJECTIVE: As COVID-19 continues to affect the global population, it is crucial to study the impact of the disease in vulnerable populations. This study of a diverse, international cohort aims to provide timely, experiential data on the course of disease in paediatric patients with congenital heart disease (CHD). METHODS: Data were collected by capitalising on two pre-existing CHD registries, the International Quality Improvement Collaborative for Congenital Heart Disease: Improving Care in Low- and Middle-Income Countries and the Congenital Cardiac Catheterization Project on Outcomes. 35 participating sites reported data for all patients under 18 years of age with diagnosed CHD and known COVID-19 illness during 2020 identified at their institution. Patients were classified as low, moderate or high risk for moderate or severe COVID-19 illness based on patient anatomy, physiology and genetic syndrome using current published guidelines. Association of risk factors with hospitalisation and intensive care unit (ICU) level care were assessed. RESULTS: The study included 339 COVID-19 cases in paediatric patients with CHD from 35 sites worldwide. Of these cases, 84 patients (25%) required hospitalisation, and 40 (12%) required ICU care. Age <1 year, recent cardiac intervention, anatomical complexity, clinical cardiac status and overall risk were all significantly associated with need for hospitalisation and ICU admission. A multivariable model for ICU admission including clinical cardiac status and recent cardiac intervention produced a c-statistic of 0.86. CONCLUSIONS: These observational data suggest risk factors for hospitalisation related to COVID-19 in paediatric CHD include age, lower functional cardiac status and recent cardiac interventions. There is a need for further data to identify factors relevant to the care of patients with CHD who contract COVID-19 illness.
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COVID-19 , Cardiopatias Congênitas , Humanos , Criança , Adolescente , COVID-19/epidemiologia , COVID-19/complicações , Cardiopatias Congênitas/epidemiologia , Cardiopatias Congênitas/terapia , Cardiopatias Congênitas/complicações , Unidades de Terapia Intensiva , Fatores de RiscoRESUMO
BACKGROUND: Surgical care for CHD is increasingly available in low- and middle-income countries, and efforts to optimise outcomes are growing. This study characterises cardiac imaging and prenatal diagnosis infrastructure in this setting. METHODS: An infrastructure survey was administered to sites participating in the International Quality Improvement Collaborative for CHD. Questions regarding transthoracic, transesophageal and epicardial echocardiography, cardiac CT, cardiac magnetic resonance, prenatal screening and fetal echocardiography were included. Associations with in-hospital and 30-day mortality were assessed. RESULTS: Thirty-seven sites in 17 countries responded. Programme size and geography varied considerably: < 250 cases (n = 13), 250-500 cases (n = 9), > 500 cases (n = 15); Americas (n = 13), Asia (n = 18), and Eastern Europe (n = 6). All had access to transthoracic echo. Most reported transesophageal and epicardial echocardiography availability (86 and 89%, respectively). Most (81%) had cardiac CT, but only 54% had cardiac magnetic resonance. A third reported impediments to imaging, including lack of portable machines, age/size-appropriate equipment and advanced cardiac imaging access and training. Only 19% of centres reported universal prenatal CHD screening in their catchment area, and only 46% always performed fetal echocardiography if screening raised concern for CHD. No statistically significant associations were identified between imaging modality availability and surgical outcomes. CONCLUSIONS: Although access to echocardiography is available in most middle-income countries; advanced imaging modalities (cardiac CT and magnetic resonance) are not always accessible. Prenatal screening for CHD is low, and availability of fetal echocardiography is limited. Imaging infrastructure in low- and middle-income countries and associations with outcomes merits additional study.
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Países em Desenvolvimento , Cardiopatias Congênitas , Feminino , Gravidez , Humanos , Tomografia Computadorizada por Raios X , Técnicas de Imagem Cardíaca , Ecocardiografia , Ultrassonografia Pré-NatalRESUMO
The epidemiology of congenital heart disease (CHD) has changed in the past 50 years as a result of an increase in the prevalence and survival rate of CHD. In particular, mortality in patients with CHD has changed dramatically since the latter half of the twentieth century as a result of more timely diagnosis and the development of interventions for CHD that have prolonged life. As patients with CHD age, the disease burden shifts away from the heart and towards acquired cardiovascular and systemic complications. The societal costs of CHD are high, not just in terms of health-care utilization but also with regards to quality of life. Lifespan disease trajectories for populations with a high disease burden that is measured over prolonged time periods are becoming increasingly important to define long-term outcomes that can be improved. Quality improvement initiatives, including advanced physician training for adult CHD in the past 10 years, have begun to improve disease outcomes. As we seek to transform lifespan into healthspan, research efforts need to incorporate big data to allow high-value, patient-centred and artificial intelligence-enabled delivery of care. Such efforts will facilitate improved access to health care in remote areas and inform the horizontal integration of services needed to manage CHD for the prolonged duration of survival among adult patients.
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Cardiopatias Congênitas , Qualidade de Vida , Humanos , Adulto , Inteligência Artificial , Cardiopatias Congênitas/epidemiologia , Cardiopatias Congênitas/terapia , Custos e Análise de Custo , Qualidade da Assistência à SaúdeRESUMO
Background: No published data are available on the patient, procedural characteristics, and outcomes of congenital heart disease (CHD) cardiac catheterization performed in low- and middle-income countries (LMICs). Objectives: The objective of this study was to describe procedural characteristics and patient outcomes of CHD cardiac catheterizations in LMICs. Methods: Cases performed between January 2019 and December 2020 from 15 centers in the International Quality Improvement Collaborative Congenital Heart Disease Catheterization Registry (IQIC-CHDCR) data were included. The Procedural Risk in Congenital Cardiac Catheterization (PREDIC3T) classification was used to stratify risk. Outcomes of interest included mortality, severe adverse events (SAEs), and procedural efficacy. Procedural efficacy, based on technical and safety endpoints, was categorized into optimal, adequate, and inadequate for 5 common interventional procedures. Results: There were 3,287 cases, of which 60% (n = 1,973) were interventional cases. Most of the cases (66%) were in patients between the ages of 1 to 18 years with a median patient age of 4 years. PREDIC3T risk class 1 and 2 were most common in 37% and 38% of cases, respectively. SAEs occurred in 2.8% while the death was reported within <72 hours post catheterization 1%. The majority of device implantation procedures patent ductus arteriosus (67%) and atrial septal defect (60%) had optimal procedure efficacy outcomes. Conclusions: This study demonstrates that congenital cardiac catheterization is safely performed in LMICs. Future work addressing predictors of SAEs and adverse procedural outcomes may help future quality improvement initiatives.
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OBJECTIVES: The aim of this study was to understand the effects of the COVID-19 pandemic on paediatric cardiac services in critical access centres in low-income and middle-income countries. DESIGN: A mixed-methods approach was used. SETTING: Critical access sites that participate in the International Quality Improvement Collaborative (IQIC) for congenital heart disease (CHD) were identified. PARTICIPANTS: Eight IQIC sites in low-income and middle-income countries agreed to participate. OUTCOME MEASURES: Differences in volume and casemix before and during the pandemic were identified, and semistructured interviews were conducted with programme representatives and analysed by two individuals using NVivo software. The qualitative component of this study contributed to a better understanding of the centres' experiences and to identify themes that were common across centres. RESULTS: In aggregate, among the seven critical access sites that reported data in both 2019 and 2020, there was a 20% reduction in case volume, though the reduction varied among programmes. Qualitative analysis identified a universal impact for all programmes related to Access to Care/Clinical Services, Financial Stability and Professional/Personal Issues for healthcare providers. CONCLUSIONS: Our study identified and quantified a significant impact of the COVID-19 pandemic on critical access to CHD surgery in low-income and middle-income countries, as well as a significant adverse impact on both the skilled workforce needed to treat CHD and on the institutions in which care is delivered. These findings suggest that the COVID-19 pandemic has been a major threat to access to care for children with CHD in resource-constrained environments and that this effect may be long-lasting beyond the global emergency. Efforts are needed to preserve vulnerable CHD programmes even during unprecedented pandemic situations.
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COVID-19 , Cardiopatias Congênitas , Criança , Humanos , COVID-19/epidemiologia , Países em Desenvolvimento , Pandemias , Pobreza , Renda , Cardiopatias Congênitas/epidemiologia , Cardiopatias Congênitas/cirurgiaRESUMO
Pulmonary vein stenosis (PVS) is a rare and poorly understood condition that can be classified as primary, acquired, status-post surgical repair of PVS, and/or associated with developmental lung disease. Immunohistochemical studies demonstrate that obstruction of the large (extrapulmonary) pulmonary veins is associated with the neointimal proliferation of myofibroblasts. This rare disorder is likely multifactorial with a spectrum of pathobiology. Treatments have been historically surgical, with an increasing repetitive interventional approach. Understanding the biology of these disorders is in its infancy; thus, medical management has lagged behind. Throughout medical history, an increased understanding of the underlying biology of a disorder has led to significant improvements in care and outcomes. One example is the treatment of pulmonary arterial hypertension (PAH). PAH shares several common themes with PVS. These include the spectrum of disease and biological alterations, such as vascular remodeling and vasoconstriction. Over the past two decades, an exponential increase in the understanding of the pathobiology of PAH has led to a dramatic increase in medical therapies that have changed the landscape of the disease. We believe that a similar approach to PVS can generate novel medical therapeutic targets that will markedly improve the outcome of these vulnerable patients.
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Purpose: To retrospectively compare the lung and pleural findings in children with pulmonary vein stenosis (PVS) with and without aspiration on multidetector computed tomography (MDCT). Materials and Methods: All consecutive children (≤18 years old) with PVS who underwent thoracic MDCT studies from August 2004 to December 2021 were categorized into two groups: children with PVS with aspiration (Group 1) and children with PVS without aspiration (Group 2). Two independent pediatric radiologists retrospectively evaluated thoracic MDCT studies for the presence of lung and pleural abnormalities as follows: (1) in the lung (ground-glass opacity (GGO), consolidation, nodule, mass, cyst(s), interlobular septal thickening, and fibrosis) and (2) in the pleura (thickening, effusion, and pneumothorax). Interobserver agreement between the two reviewers was evaluated by the proportion of agreement and the Kappa statistic. Results: The final study population consisted of 64 pediatric patients (36 males (56.3%) and 43 females (43.7%); mean age, 1.7 years; range, 1 day−17 years). Among these 64 patients, 19 patients (29.7%) comprised Group 1 and the remaining 45 patients (70.3%) comprised Group 2. In Group 1 (children with PVS with aspiration), the detected lung and pleural MDCT abnormalities were: GGO (17/19; 89.5%), pleural thickening (17/19; 89.5%), consolidation (16/19; 84.5%), and septal thickening (16/19; 84.5%). The lung and pleural MDCT abnormalities observed in Group 2 (children with PVS without aspiration) were: GGO (37/45; 82.2%), pleural thickening (37/45; 82.2%), septal thickening (36/45; 80%), consolidation (3/45; 6.7%), pleural effusion (1/45; 2.2%), pneumothorax (1/45; 2.2%), and cyst(s) (1/45; 2.2%). Consolidation was significantly more common in pediatric patients with both PVS and aspiration (Group 1) (p < 0.001). There was high interobserver agreement between the two independent reviewers for detecting lung and pleural abnormalities on thoracic MDCT studies (Kappa = 0.98; CI = 0.958, 0.992). Conclusion: Aspiration is common in pediatric patients with PVS who undergo MDCT and was present in nearly 30% of all children with PVS during our study period. Consolidation is not a typical radiologic finding of PVS in children without clinical evidence of aspiration. When consolidation is present on thoracic MDCT studies in pediatric patients with PVS, the additional diagnosis of concomitant aspiration should be considered.
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Purpose: To retrospectively compare the pleuropulmonary MDCT findings in children with pulmonary vein stenosis (PVS) and prematurity-related lung disease (PLD). Materials and Methods: All consecutive infants and young children (≤18 years old) who underwent thoracic MDCT studies from July 2004 to November 2021 were categorized into two groupschildren with PVS (Group 1) and children with PLD without PVS (Group 2). Two pediatric radiologists independently evaluated thoracic MDCT studies for the presence of pleuropulmonary abnormalities as follows(1) in the lung (ground-glass opacity (GGO), triangular/linear plaque-like opacity (TLO), consolidation, nodule, mass, cyst(s), interlobular septal thickening, and fibrosis); (2) in the airway (bronchial wall thickening and bronchiectasis); and (3) in the pleura (thickening, effusion, and pneumothorax). Interobserver agreement between the two reviewers was evaluated with the Kappa statistic. Results: There were a total of 103 pediatric patients (60 males (58.3%) and 43 females (41.7%); mean age, 1.7 years; range, 2 days−7 years). Among these 103 patients, 49 patients (47.6%) comprised Group 1 and the remaining 54 patients (52.4%) comprised Group 2. In Group 1, the observed pleuropulmonary MDCT abnormalities werepleural thickening (44/49; 90%), GGO (39/49; 80%), septal thickening (39/49; 80%), consolidation (4/49; 8%), and pleural effusion (1/49; 2%). The pleuropulmonary MDCT abnormalities seen in Group 2 wereGGO (45/54; 83%), TLO (43/54; 80%), bronchial wall thickening (33/54; 61%), bronchiectasis (30/54; 56%), cyst(s) (5/54; 9%), pleural thickening (2/54; 4%), and pleural effusion (2/54; 4%). Septal thickening and pleural thickening were significantly more common in pediatric patients with PVS (Group 1) (p < 0.001). TLO, bronchial wall thickening, and bronchiectasis were significantly more frequent in pediatric patients with PLD without PVS (Group 2) (p < 0.001). There was high interobserver kappa agreement between the two independent reviewers for detecting pleuropulmonary abnormalities on thoracic MDCT angiography studies (k = 0.99). Conclusion: Pleuropulmonary abnormalities seen on thoracic MDCT can be helpful for distinguishing PVS from PLD in children. Specifically, the presence of septal thickening and pleural thickening raises the possibility of PVS, whereas the presence of TLO, bronchial wall thickening and bronchiectasis suggests PLD in the pediatric population.
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OBJECTIVE: The study objective was to evaluate outcomes of pulmonary vein stenosis repair in a large single-center cohort. METHODS: Clinical data from a pulmonary vein stenosis registry were retrospectively reviewed identifying patients who underwent pulmonary vein stenosis repair. The primary/index operation was defined as the patient's first pulmonary vein stenosis operation during the study period. RESULTS: Between January 2007 and August 2019, 174 patients underwent pulmonary vein stenosis repair. Bilateral pulmonary vein stenosis occurred in 111 patients (64%); 71 patients (41%) had 4-vessel disease. Fifty-nine patients (34%) had primary pulmonary vein stenosis. Median age was 9 months (interquartile range, 5-27) and weight was 6.5 kg (4.7-10.2). Surgical techniques evolved and included ostial resection, unroofing, reimplantation, sutureless, modified sutureless, and a newer anatomically focused approach of pulmonary vein stenosis resection with lateralization or patch enlargement of the pulmonary vein-left atrium connection. Twenty-three patients (13%) required reoperation. Cumulative 2-year incidence of postoperative transcatheter intervention (balloon dilation ± stenting) was 64%. One-, 2-, and 5-year survivals were 71.2%, 66.8%, and 60.6%, respectively. There was no association between surgery type and reoperation rate (hazard ratio, 2.38, P = .25) or transcatheter intervention (hazard ratio, 0.97, P = .95). The anatomically focused repair was associated with decreased mortality on univariate (hazard ratio, 0.38, P = .042) and multivariable analyses (hazard ratio, 0.19, P = .014). Antiproliferative chemotherapy was also associated with decreased mortality (hazard ratio, 0.47, P = .026). CONCLUSIONS: This large single-center surgical pulmonary vein stenosis experience demonstrates encouraging midterm results. A new anatomically focused repair strategy aims to alleviate pulmonary vein angulation to minimize turbulence and shows promising early outcomes. Continued follow-up is required to understand longer-term outcomes for this surgical approach.