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INTRODUCTION: The main risk factors of necrotising enterocolitis (NEC) are prematurity and low birth weight. The aim of our study was to identify risk factors for NEC in patients with duct-dependent congenital heart disease (CHD). STUDY DESIGN: Newborns with duct-dependent CHD and NEC were matched 1:1 to those without NEC. Matched criteria were gestational age, birth weight, antenatal versus postnatal diagnosis and type of CHD. RESULTS: Twenty-three infants were included in each group. In the NEC group, mortality, length of intensive care unit stay and length of hospital stay were significantly higher (p=0.035; p<0.0001; p<0.0001). Lower diastolic blood pressure (DBP), negative flow balance, peritoneal dialysis and epinephrine-infusion were significantly associated with NEC (respectively, p=0.008, p=0.002, p=0.007, p=0.017). In multivariate analysis, DBP≤30 mm Hg remained the only independent risk factor of NEC (OR=8.70; 95% CI (1.46 to 53.50), p=0.019). CONCLUSION: A DBP lower than 30 mm Hg was in our matched population of newborns with duct-dependent CHD, independently associated with NEC.
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Enterocolitis Necrotizante , Cardiopatías Congénitas , Humanos , Recién Nacido , Enterocolitis Necrotizante/diagnóstico , Enterocolitis Necrotizante/epidemiología , Enterocolitis Necrotizante/mortalidad , Factores de Riesgo , Femenino , Masculino , Cardiopatías Congénitas/diagnóstico , Cardiopatías Congénitas/complicaciones , Pronóstico , Tiempo de Internación , Estudios Retrospectivos , Edad Gestacional , Recien Nacido PrematuroRESUMEN
Heart failure is a pediatric emergency caused by the heart's inability to adequately meet the body metabolic needs and the most common cause is congenital heart disease (CHD). The G protein is the most prominent family of membrane-bound protein known to act in major regulatory events of the cardiovascular system, one of which is heart failure. The aim of this study was to determine the level of G protein and its relationship with left ventricular systolic function in children with acyanotic CHD. A cross-sectional study was conducted in Dr. Zaionel Abidin Hospital, Banda Aceh, Indonesia. The patients aged 0 to 18 years and had acyanotic CHD diagnosis by echocardiography were included. Anthropometry measurement was performed according to standard WHO procedures and G protein level was measured using the ELISA method. The Chi-squared test was used to measure the relationship between G protein level and left ventricular systolic function. Out of a total of 38 children with acyanotic CHD, the mean level of G protein was 36.25 ng/mL and the mean of left ventricular systolic function was 73.1%. There was no relationship between G protein and left ventricular systolic function in children with acyanotic CHD. However, further study with a larger sample size and considering other variables are needed to confirm this finding.
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Cardiopatías Congénitas , Función Ventricular Izquierda , Humanos , Estudios Transversales , Preescolar , Masculino , Femenino , Lactante , Niño , Función Ventricular Izquierda/fisiología , Cardiopatías Congénitas/complicaciones , Cardiopatías Congénitas/fisiopatología , Adolescente , Ecocardiografía , Recién Nacido , Indonesia/epidemiología , SístoleAsunto(s)
Anticuerpos Monoclonales Humanizados , Humanos , Anticuerpos Monoclonales Humanizados/uso terapéutico , Resultado del Tratamiento , Facies , Femenino , Displasia Ectodérmica/tratamiento farmacológico , Cardiopatías/tratamiento farmacológico , Cardiopatías Congénitas/complicaciones , Cardiopatías Congénitas/tratamiento farmacológico , Índice de Severidad de la Enfermedad , Masculino , Insuficiencia de CrecimientoRESUMEN
INTRODUCTION: Congenital heart disease is a common birth defect, but advancements in diagnosis and treatment have improved survival rates. Enhanced recovery after surgery (ERAS) programmes have emerged in paediatric cardiac surgery. Multimodal pain management, as a vital part of ERAS programmes, has been found to be effective in reducing pain and improving outcomes in cardiac surgery patients. Traditional methods of pain control using high-dose opioids can lead to complications, so nonopioid analgesics and regional anaesthesia techniques are being used to reduce the consumption. However, there is a significant variability in pain management practices in paediatric cardiac surgery. A network meta-analysis (NMA) is needed to comprehensively compare the effects of different analgesic interventions in this population. METHODS AND ANALYSIS: A comprehensive electronic literature database search will be performed using electronic databases, mainly including PubMed, EMBASE, Web of Science and Cochrane Central Register of Controlled Trials. All randomised controlled trials associated with perioperative pain management for paediatric cardiac surgery will be included. The primary outcome will be visual analogue score or numeric rating scale of pain and total opioid consumption (or equivalent) 24 hours after postoperative tracheal extubation. The Revised Cochrane Risk of Bias Tool will be employed to assess the quality of included articles. A random-effects pairwise meta-analysis will be performed to report the head-to-head comparison. Following the assessment of individual articles, an NMA will be conducted using a Bayesian framework with random-effects' models. ETHICS AND DISSEMINATION: Ethics approval is not necessary because this study will be based on publications. The results of this study will be published in a peer-reviewed journal. PROSPERO REGISTRATION NUMBER: CRD42023477520.
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Procedimientos Quirúrgicos Cardíacos , Cardiopatías Congénitas , Metaanálisis en Red , Manejo del Dolor , Dolor Postoperatorio , Humanos , Procedimientos Quirúrgicos Cardíacos/efectos adversos , Dolor Postoperatorio/tratamiento farmacológico , Manejo del Dolor/métodos , Cardiopatías Congénitas/cirugía , Cardiopatías Congénitas/complicaciones , Niño , Analgésicos Opioides/uso terapéutico , Dimensión del Dolor , Revisiones Sistemáticas como Asunto , Proyectos de InvestigaciónRESUMEN
PURPOSE OF REVIEW: Patients with a functionally single ventricle (SV) are palliated with a series of procedures leading to a Fontan circulation. Over the life span, a substantial proportion of SV patients develop heart failure that can arise from circulatory or ventricular failure. Diastolic dysfunction (DD) is an important determinant of adverse outcomes in SV patients. However, assessment and categorization of DD in the SV remains elusive. We review recent literature and developments in assessment of DD in the SV and its relation to clinical outcomes. RECENT FINDINGS: DD is prevalent in the SV and associated with worse outcomes. Occult DD can be exposed with provocative testing by exercise or preload challenge during catheterization. Likewise, sensitivity to detect DD may be increased via assessment of atrial function and strain imaging. Recent studies revisiting previous concepts such as incoordinate diastolic wall motion show that these are associated with SV end-diastolic pressures and post-Fontan recovery, yielding accessible DD assessment. Emerging technologies such as ultrafast ultrasound (UFUS) can provide noninvasive assessment of myocardial stiffness, inefficient diastolic flow patterns and intraventricular pressure gradients, thereby yielding new tools and insights into diastolic myocardial and hemodynamic properties. SUMMARY: Characterizing DD in the SV continues to have substantial limitations, necessitating synthesis of multiple parameters into an overall assessment, accounting for their change over time, and in the context of the patient's clinical status. New and emerging techniques may help advance DD assessment and the ability to track response to treatment of new targets.
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Diástole , Procedimiento de Fontan , Humanos , Niño , Corazón Univentricular/fisiopatología , Corazón Univentricular/cirugía , Corazón Univentricular/diagnóstico , Ventrículos Cardíacos/fisiopatología , Cardiopatías Congénitas/fisiopatología , Cardiopatías Congénitas/diagnóstico , Cardiopatías Congénitas/complicacionesRESUMEN
Background and Aim: Patients with cyanosis secondary to congenital heart disease (CHD) are characterized by erythrocytosis and increased blood viscosity, which contribute to endothelial dysfunction, increased arterial stiffness, and impaired vascular function, which may affect the final clinical presentation. Asymmetric dimethylarginine (ADMA) and e-selectin (e-sel) are valuable biomarkers for endothelial and vascular dysfunction. Their concentration levels in blood serum have the potential to be an accessible tool that reflects the severity of the disease. We aimed to assess e-sel and ADMA levels and their relationship with the clinical status and endothelial and vascular function. Methods: A cross-sectional study, including 36 adult CHD cyanotic patients [(17 males) (42.3 ± 16.3 years)] with an arterial blood oxygen saturation less than 92% and 20 healthy controls [(10 males) (38.2 ± 8.5 years)], was performed. All the patients underwent a clinical examination, blood testing, and cardiopulmonary tests. Their endothelial function was assessed using the intima media thickness and flow-mediated dilatation. Vascular function, using applanation tonometry methods, was determined using the aortic systolic pressure, aortic pulse pressure, augmentation pressure, augmentation index, pulse pressure amplification, and pulse wave velocity. Results: The concentrations of e-sel and ADMA were significantly higher in the patients with CHD. The E-sel levels correlated positively with red blood cells, hemoglobin concentration, hematocrit, and augmentation pressure; they correlated negatively with blood oxygen saturation, the forced expiratory one-second volume, forced vital capacity, and oxygen uptake. The ADMA levels were found to correlate only with age. Conclusions: The E-sel level, unlike ADMA concentration, reflects the severity of erythrocytosis and hypoxia and, thus, the physical status of patients with cyanotic CHD.
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Arginina , Cianosis , Selectina E , Cardiopatías Congénitas , Humanos , Masculino , Adulto , Femenino , Arginina/análogos & derivados , Arginina/sangre , Cardiopatías Congénitas/sangre , Cardiopatías Congénitas/fisiopatología , Cardiopatías Congénitas/complicaciones , Cianosis/sangre , Cianosis/fisiopatología , Selectina E/sangre , Estudios Transversales , Persona de Mediana Edad , Biomarcadores/sangre , Endotelio Vascular/fisiopatología , Endotelio Vascular/metabolismo , Estudios de Casos y ControlesRESUMEN
Fontan surgery is vital for infants born with a single-ventricle heart. This intervention establishes a new blood flow circuit bypassing the single ventricle, thereby the separating pulmonary and systemic circulation to preserve single ventricular function. However, it carries risks of hepatic complications, collectively termed Fontan-associated liver disease (FALD), characterized by progressive hepatic congestion and fibrosis potentially leading to an equivalent of cirrhosis. Diagnosis and staging of FALD are complex, requiring multidisciplinary management. In advanced FALD, consideration is given to heart transplantation alone or combined heart-liver transplantation, underscoring the importance of an integrated approach to optimize care for these increasingly more common patients.
La chirurgie de Fontan est vitale pour les nouveau-nés naissant avec un cÅur univentriculaire. Cette intervention crée un nouveau circuit sanguin palliant l'absence de ventricule sous-pulmonaire en connectant les veines caves directement aux artères pulmonaires. Elle permet de séparer les circulations pulmonaire et systémique et de préserver la fonction du ventricule unique. Cela expose néanmoins les patients à des complications à moyen et long terme, parmi lesquelles l'atteinte hépatique, nommée Fontan-Associated Liver Disease (FALD), se caractérisant par une congestion et une fibrose hépatiques progressives pouvant conduire à l'équivalent d'une cirrhose et à ses complications. Son diagnostic ainsi que l'évaluation de sa sévérité impliquent différents éléments biologiques, radiologiques et histopathologiques ainsi qu' une expertise multidisciplinaire. Lors de FALD avancée, la transplantation cardiaque seule ou combinée cÅur-foie est discutée, au cas par cas.
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Procedimiento de Fontan , Hepatopatías , Humanos , Hepatopatías/diagnóstico , Hepatopatías/etiología , Hepatopatías/terapia , Procedimiento de Fontan/efectos adversos , Cardiopatías Congénitas/diagnóstico , Cardiopatías Congénitas/complicaciones , Trasplante de Hígado/métodos , LactanteRESUMEN
PURPOSE OF REVIEW: Present an updated overview of the prevention, diagnosis, and management of infective endocarditis in adult patients with congenital heart disease. RECENT FINDINGS: Care for patients with infective endocarditis is changing in the areas of specialized teams, diagnostics, and prevention. Endocarditis teams should be involved in the care of ACHD patients. The 2023 Duke Criteria for Infective Endocarditis and the 2023 European Society of Cardiology Guidelines have updated the criteria for diagnosis including new major criteria such as CT and positron emission computed tomography with 18F-fluorodeoxyglucose (FDG) scans. Immunological, PCR, and nucleic acid-based tests are now acceptable means to isolate infective organisms. Clindamycin is no longer recommended for antibiotic prophylaxis due to resistance and side effect profile. Special considerations for antibiotic prophylaxis and management must be made for specific congenital heart diseases in adulthood and pregnant ACHD patients. Infective endocarditis (IE), a potentially devastating clinical entity, is a feared threat to the health of adults with congenital heart disease (ACHD). IE needs a systematic approach for its prevention, early diagnosis and management with a multidisciplinary IE team's involvement. There have been changes in the diagnostics and management of IE, which is reflected in updated diagnostic criteria. Timely blood cultures and imaging continue to be the mainstay of diagnosis, however the timing of blood cultures, microbiological testing, and types of diagnostic imaging such as the positron emission computed tomography with 18F-fluorodeoxyglucose (FDG) scan are new. Bicuspid aortic valves, ventricular septal defects, transcatheter pulmonary valve replacements, and tetralogy of Fallot are diagnoses at higher risk for IE in the ACHD population. The following article will focus on the preventive strategies, in addition to novel diagnostic and therapeutic approaches of IE in ACHD patients.
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Endocarditis , Cardiopatías Congénitas , Humanos , Cardiopatías Congénitas/complicaciones , Cardiopatías Congénitas/cirugía , Endocarditis/prevención & control , Endocarditis/diagnóstico , Endocarditis/complicaciones , Adulto , Profilaxis Antibiótica , Antibacterianos/uso terapéutico , EmbarazoRESUMEN
BACKGROUND: Atrial fibrillation (AF) is a common arrhythmia in patients with congenital heart disease (CHD), but little is known about the risk for ischemic stroke among younger patients with CHD (aged <65 years) with AF. METHODS AND RESULTS: Using data from the National Swedish Patient Register and Cause of Death Register, we identified all patients with both CHD and AF born in Sweden between 1970 and 2017. The Swedish total population register was used to identify age- and sex-matched patients without CHD; those with AF were used as controls. Bottos hierarchical classification was used to define CHD as either complex or noncomplex. Controls were followed from the onset of AF until the index ischemic stroke, death, or end of study (December 31, 2017). We identified 951 patients with CHD with AF and 606 controls with AF. Among patients with both CHD and AF, 2.9% of patients (n=28) developed ischemic stroke, compared with 0.5% (n=3) in controls. When adjusted for age, sex, hypertension, and heart failure, a hazard ratio (HR) of 5.16 (95% CI, 1.52-17.46) was acquired. The HR in noncomplex CHD was 3.84 (95% CI, 1.07-13.84), and the HR in complex CHD was 8.34 (95% CI, 2.27-30.57). For patients born in 1970 to 1989, the HR in ischemic stroke was 7.35 (95% CI, 1.70-31.75). No adjusted HR for patients with CHD born in 1990 to 2017 could be acquired due to few events. CONCLUSIONS: The risk for ischemic stroke may be 5 times higher in patients with both CHD and AF compared with patients without CHD with AF. However, larger studies may be needed to confirm/refute these results.
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Fibrilación Atrial , Cardiopatías Congénitas , Accidente Cerebrovascular Isquémico , Sistema de Registros , Humanos , Fibrilación Atrial/epidemiología , Fibrilación Atrial/diagnóstico , Fibrilación Atrial/complicaciones , Femenino , Masculino , Cardiopatías Congénitas/epidemiología , Cardiopatías Congénitas/complicaciones , Accidente Cerebrovascular Isquémico/epidemiología , Accidente Cerebrovascular Isquémico/etiología , Accidente Cerebrovascular Isquémico/diagnóstico , Suecia/epidemiología , Persona de Mediana Edad , Adulto , Factores de Riesgo , Medición de Riesgo , Incidencia , Estudios de Casos y Controles , AncianoAsunto(s)
Cardiopatías Congénitas , Accidente Cerebrovascular , Humanos , Cardiopatías Congénitas/complicaciones , Cardiopatías Congénitas/epidemiología , Accidente Cerebrovascular/prevención & control , Accidente Cerebrovascular/epidemiología , Accidente Cerebrovascular/etiología , Adulto , Factores de Riesgo , Medición de RiesgoAsunto(s)
Fibrilación Atrial , Cardiopatías Congénitas , Accidente Cerebrovascular Isquémico , Humanos , Fibrilación Atrial/epidemiología , Fibrilación Atrial/complicaciones , Fibrilación Atrial/diagnóstico , Accidente Cerebrovascular Isquémico/epidemiología , Accidente Cerebrovascular Isquémico/etiología , Accidente Cerebrovascular Isquémico/prevención & control , Cardiopatías Congénitas/epidemiología , Cardiopatías Congénitas/complicaciones , Factores de Riesgo , Medición de RiesgoRESUMEN
Congenital heart disease (CHD) is one of the most common inborn disorders, with a prevalence of 0.8-1.2%. Affected children are often malnourished due to increased dietary requirements. This may lead to severe long-term complications. Several authoritative organizations have published guidelines addressing nutritional intervention in children with CHD. We aimed to systematically assess the consistency of recommendations, the methodological quality of these guidelines, and the quality of evidence supporting each recommendation. PubMed, Embase, the Cochrane Database, World Health Organization Global Index Medicus, and 16 scientific societies' websites were searched for the period until September 2023. The guideline quality was assessed using the AGREE II tool. After screening 765 records, only 2 guidelines published in 2013 and 2022 met our inclusion criteria. The main reason for exclusion was the absence of any system for rating the evidence. The main issues concerned the lack of implementation advice or tools and the lack of criteria to measure the application of guideline recommendations. The included guidelines were of good quality and within specific recommendations, both publications were largely in agreement, and the score for the overall assessment was high (83%). There is a pressing need for comprehensive, multi-threaded guidelines incorporating implementation strategies and methods for the performance assessment of children with malnutrition and CHD.
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Cardiopatías Congénitas , Desnutrición , Guías de Práctica Clínica como Asunto , Humanos , Cardiopatías Congénitas/complicaciones , Cardiopatías Congénitas/terapia , Niño , Desnutrición/terapia , Desnutrición/etiología , Trastornos de la Nutrición del Niño/terapia , Preescolar , LactanteRESUMEN
BACKGROUND: Due to a special hemodynamic feature, pulmonary vascular disease in pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) has two stages: reversible and irreversible. So far, the mechanism involved in the transition from reversible to irreversible stage is elusive. Moreover, no recognized and reliable assessments to distinguish these two stages are available. Furthermore, we found that compared with control and reversible PAH, thrombospondin-4 (THBS4) was significantly upregulated in irreversible group by bioinformatic analysis. Hence, we further verify and investigate the expression and role of THBS4 in PAH-CHD. METHODS: We established the monocrotaline plus aorto-cava shunt-induced (MCT-AV) rat model. We measured the expression of THBS4 in lung tissues from MCT-AV rats. Double immunofluorescence staining of lung tissue for THBS4 and α-SMA (biomarker of smooth muscle cells) or vWF (biomarker of endothelial cells) to identify the location of THBS4 in the pulmonary artery. Primary pulmonary artery smooth muscle cells (PASMCs) were cultivated, identified, and used in this study. THBS4 was inhibited and overexpressed by siRNA and plasmid, respectively, to explore the effect of THBS4 on phenotype transformation, proliferation, apoptosis, and migration of PASMCs. The effect of THBS4 on pulmonary vascular remodeling was evaluated in vivo by adeno-associated virus which suppressed THBS4 expression. Circulating level of THBS4 in patients with PAH-CHD was measured by ELISA. RESULTS: THBS4 was upregulated in the lung tissues of MCT-AV rats, and was further upregulated in severe pulmonary vascular lesions. And THBS4 was expressed mainly in PASMCs. When THBS4 was inhibited, contractile markers α-SMA and MYH11 were upregulated, while the proliferative marker PCNA was decreased, the endothelial-mensenchymal transition marker N-cad was downregulated, proapototic marker BAX was increased. Additionally, proliferation and migration of PASMCs was inhibited and apoptosis was increased. Conversely, THBS4 overexpression resulted in opposite effects. And the impact of THBS4 on PASMCs was probably achieved through the regulation of the PI3K/AKT pathway. THBS4 suppression attenuated pulmonary vascular remodeling. Furthermore, compared with patients with simple congenital heart disease and mild PAH-CHD, the circulating level of THBS4 was higher in patients with severe PAH-CHD. CONCLUSIONS: THBS4 is a promising biomarker to distinguish reversible from irreversible PAH-CHD before repairing the shunt. THBS4 is a potential treatment target in PAH-CHD, especially in irreversible stage.
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Cardiopatías Congénitas , Hipertensión Arterial Pulmonar , Ratas Sprague-Dawley , Trombospondinas , Animales , Humanos , Masculino , Ratas , Células Cultivadas , Cardiopatías Congénitas/metabolismo , Cardiopatías Congénitas/complicaciones , Miocitos del Músculo Liso/metabolismo , Miocitos del Músculo Liso/patología , Hipertensión Arterial Pulmonar/metabolismo , Hipertensión Arterial Pulmonar/patología , Arteria Pulmonar/metabolismo , Arteria Pulmonar/patología , Trombospondinas/metabolismo , Trombospondinas/biosíntesis , Trombospondinas/genéticaRESUMEN
OBJECTIVE: Shone's complex comprises of a combination of congenital cardiac anomalies causing obstructions in the left ventricle's inflow and outflow tracts. This systematic review aims to evaluate the clinical features and surgical outcomes of Shone's complex. METHODS: An electronic literature search of PubMed and Scopus was performed to identify relevant studies related to the presentation, management, and outcomes of Shone's complex. Two reviewers independently performed selection. Data on study characteristics, participant demographics, interventions, outcomes, and follow-up durations were extracted and analyzed. RESULTS: A total of 691 papers were identified, with 18 studies included in the final analysis. The majority of the studies (n = 12) focused on the pediatric age group. The most common clinical presentations were coarctation of the aorta (n = 17) and mitral stenosis (n = 12). Surgical interventions often involved staged approaches, prioritizing outflow before inflow obstructions. Mitral valve repair was preferred over replacement due to better long-term outcomes (n = 8). Biventricular repair was recommended due to improved postoperative outcomes, but often needed reoperations. Reoperations were common, primarily due to recurrent coarctation (n = 10), subaortic stenosis (n = 8), and mitral valve dysfunction (n = 7). Pulmonary hypertension (n = 10) and arrhythmias (n = 11) were significant complications. Most patients were in modified Ross/NYHA functional class 1 on follow-up. Mortality rates ranged from 4 to 28%, with better outcomes associated with early and strategic surgical interventions. CONCLUSION: Early diagnosis and biventricular repair were associated with better outcomes while transplantation was often an eventuality. Standardized diagnostic criteria, long-term follow-up, and consensus guidelines are needed to improve the management of this congenital heart disease.
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Procedimientos Quirúrgicos Cardíacos , Cardiopatías Congénitas , Humanos , Cardiopatías Congénitas/cirugía , Cardiopatías Congénitas/mortalidad , Cardiopatías Congénitas/complicaciones , Resultado del Tratamiento , Procedimientos Quirúrgicos Cardíacos/efectos adversos , Niño , Lactante , Preescolar , Adolescente , Masculino , Femenino , Coartación Aórtica/cirugía , Coartación Aórtica/complicaciones , Coartación Aórtica/mortalidad , Recién Nacido , Obstrucción del Flujo Ventricular Externo/cirugía , Obstrucción del Flujo Ventricular Externo/etiología , Reoperación , Factores de RiesgoRESUMEN
OBJECTIVES: Pulmonary arterial hypertension (PAH) is a devastating complication of pediatric congenital heart disease (CHD). A recent study has identified the protein high mobility group box-1 (HMGB1) as a diagnostic tool in adults with CHD-associated PAH. HMGB1 levels in adults with CHD-associated PAH correlated with mean pulmonary artery pressure and pulmonary vascular resistance, and HGMB1 levels fell in response to sildenafil therapy. We wanted to assess if HGMB1 was a biomarker of pediatric CHD-PAH. DESIGN: Prospective cohort study. SETTING: Quaternary pediatric academic hospital PARTICIPANTS: Children ≤18 years with CHD with and without known pulmonary hypertension. Controls were children undergoing dental or urologic surgery with no known heart disease. INTERVENTIONS: Pulmonary hemodynamics, echocardiographic assessment, and biomarker measurement. Controls had biomarker measurement only. MEASUREMENTS AND MAIN RESULTS: Patients with CHD-PAH had mean pulmonary vascular resistance index of 10 Wood units/m2. Neither HGMB1 nor N-terminal pro-brain-type natriuretic peptide levels were significantly different between the groups. Neither marker correlated with pulmonary hypertension. CONCLUSIONS: Unlike in adults, HGMB1 is not a biomarker of PAH in pediatric CHD. Further work will continue to explore for biomarkers for this high-risk population.
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Biomarcadores , Proteína HMGB1 , Cardiopatías Congénitas , Hipertensión Arterial Pulmonar , Humanos , Cardiopatías Congénitas/complicaciones , Cardiopatías Congénitas/cirugía , Cardiopatías Congénitas/sangre , Estudios Prospectivos , Femenino , Masculino , Niño , Biomarcadores/sangre , Preescolar , Hipertensión Arterial Pulmonar/sangre , Hipertensión Arterial Pulmonar/diagnóstico , Proteína HMGB1/sangre , Estudios de Cohortes , Adolescente , Valor Predictivo de las Pruebas , Lactante , Hipertensión Pulmonar/sangre , Hipertensión Pulmonar/etiología , Hipertensión Pulmonar/diagnóstico , Resistencia Vascular/fisiologíaRESUMEN
A girl in middle childhood presented with glaucoma in her right eye along with segmental haemangiomas on the right side of the face and neck. Magnetic resonance angiography of the brain showed hypoplasia of the right internal carotid artery, leading to the diagnosis of posterior fossa malformations, haemangioma, arterial anomalies, cardiac defects and eye abnormalities (PHACE) syndrome. High-definition anterior segment ocular coherence tomography (AS-OCT) of the right eye showed an absence of Schlemm's canal and a hyperreflective membrane over the trabecular meshwork. The presence of this angle dysgenesis on AS-OCT, a novel finding in this disease, explained the elevated intraocular pressure in the right eye. The embryological basis for the development of angle dysgenesis might help better understand the pathogenesis of PHACE syndrome.
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Coartación Aórtica , Anomalías del Ojo , Glaucoma , Síndromes Neurocutáneos , Humanos , Femenino , Síndromes Neurocutáneos/diagnóstico por imagen , Síndromes Neurocutáneos/complicaciones , Síndromes Neurocutáneos/diagnóstico , Coartación Aórtica/diagnóstico por imagen , Coartación Aórtica/complicaciones , Coartación Aórtica/diagnóstico , Anomalías del Ojo/diagnóstico por imagen , Anomalías del Ojo/complicaciones , Anomalías del Ojo/diagnóstico , Glaucoma/diagnóstico , Tomografía de Coherencia Óptica/métodos , Hemangioma/complicaciones , Hemangioma/diagnóstico por imagen , Arteria Carótida Interna/anomalías , Arteria Carótida Interna/diagnóstico por imagen , Cardiopatías Congénitas/diagnóstico por imagen , Cardiopatías Congénitas/complicaciones , Angiografía por Resonancia MagnéticaRESUMEN
Shone's syndrome (SS) is a rare congenital cardiac anomaly characterized by a spectrum of developmental abnormalities. It predominantly presents as consisting of a variety of left ventricular inflow and outflow tract lesions, with inflow tract lesions typically including parachute mitral valve and supravalvular mitral ring. However, reports of SS involving double-orifice mitral valve are scarce.
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Coartación Aórtica , Estenosis de la Válvula Mitral , Válvula Mitral , Humanos , Estenosis de la Válvula Mitral/complicaciones , Estenosis de la Válvula Mitral/diagnóstico por imagen , Estenosis de la Válvula Mitral/cirugía , Válvula Mitral/diagnóstico por imagen , Válvula Mitral/anomalías , Coartación Aórtica/complicaciones , Coartación Aórtica/diagnóstico por imagen , Masculino , Ecocardiografía/métodos , Anomalías Múltiples , Síndrome , Femenino , Cardiopatías Congénitas/complicaciones , Cardiopatías Congénitas/diagnóstico por imagen , Diagnóstico DiferencialAsunto(s)
Creatinina , Síndrome de Down , Cardiopatías Congénitas , Humanos , Síndrome de Down/complicaciones , Síndrome de Down/sangre , Estudios Retrospectivos , Cardiopatías Congénitas/complicaciones , Cardiopatías Congénitas/sangre , Femenino , Masculino , Preescolar , Creatinina/sangre , Lactante , Niño , Biomarcadores/sangreRESUMEN
BACKGROUND: Artificial intelligence-enhanced electrocardiogram (AI-ECG) analysis shows promise to detect biventricular pathophysiology. However, AI-ECG analysis remains underexplored in congenital heart disease (CHD). OBJECTIVES: The purpose of this study was to develop and externally validate an AI-ECG model to predict cardiovascular magnetic resonance (CMR)-defined biventricular dysfunction/dilation in patients with CHD. METHODS: We trained (80%) and tested (20%) a convolutional neural network on paired ECG-CMRs (≤30 days apart) from patients with and without CHD to detect left ventricular (LV) dysfunction (ejection fraction ≤40%), RV dysfunction (ejection fraction ≤35%), and LV and RV dilation (end-diastolic volume z-score ≥4). Performance was assessed during internal testing and external validation on an outside health care system using area under receiver-operating curve (AUROC) and area under precision recall curve. RESULTS: The internal and external cohorts comprised 8,584 ECG-CMR pairs (n = 4,941; median CMR age 20.7 years) and 909 ECG-CMR pairs (n = 746; median CMR age 25.4 years), respectively. Model performance was similar for internal testing (AUROC: LV dysfunction 0.87; LV dilation 0.86; RV dysfunction 0.88; RV dilation 0.81) and external validation (AUROC: LV dysfunction 0.89; LV dilation 0.83; RV dysfunction 0.82; RV dilation 0.80). Model performance was lowest in functionally single ventricle patients. Tetralogy of Fallot patients predicted to be at high risk of ventricular dysfunction had lower survival (P < 0.001). Model explainability via saliency mapping revealed that lateral precordial leads influence all outcome predictions, with high-risk features including QRS widening and T-wave inversions for RV dysfunction/dilation. CONCLUSIONS: AI-ECG shows promise to predict biventricular dysfunction/dilation, which may help inform CMR timing in CHD.
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Aprendizaje Profundo , Electrocardiografía , Cardiopatías Congénitas , Humanos , Electrocardiografía/métodos , Femenino , Masculino , Cardiopatías Congénitas/fisiopatología , Cardiopatías Congénitas/complicaciones , Cardiopatías Congénitas/diagnóstico , Adulto , Adolescente , Adulto Joven , Niño , Disfunción Ventricular Izquierda/fisiopatología , Disfunción Ventricular Izquierda/diagnóstico , Disfunción Ventricular Izquierda/diagnóstico por imagen , Disfunción Ventricular Derecha/fisiopatología , Disfunción Ventricular Derecha/diagnóstico por imagen , Disfunción Ventricular Derecha/diagnóstico , Imagen por Resonancia Cinemagnética/métodos , Preescolar , Valor Predictivo de las PruebasRESUMEN
BACKGROUND: In light of prolonged hypoxia, children with cyanotic heart disase (CHD) are at a high risk of developing iron deficiency iron deficiency (ID) and iron deficiency anemia (IDA). Reticulocyte hemoglobin equivalent (Ret-He) is a novel and dependable indicator for assessing iron status. However, there has been no previous study regarding cut-off value in pediatric CHD group. The purpose of this study is to assess the role of Ret-He and to establish cut-off points in the diagnosis of iron deficiency and IDA in pediatric cyanotic heart disease. METHOD: This study was conducted in two tertiary hospitals in Jakarta, Indonesia. 59 children with CHD, aged 3 months to 18 years, were enrolled consecutively. To determine iron status, hematological parameters (hemoglobin, hematocrit, mean corpuscular volume, mean corpuscular hemoglobin) and biochemical parameters for iron status (serum ferritin, transferrin saturation) were analysed and compared to Ret-He levels. The receiver operating characteristic (ROC) analysis was performed for the Ret-He cut-off points for ID and IDA. Sensitivity, specificity, positive and negative predictive value were calculated for each cut-off point. RESULT: Normal iron status was identified in 27 (45.8%) subjects, ID in 8 (13.5%) subjects, and IDA 24 (40.7%) subjects. The ID cut-off value for Ret-He is 28.8 pg (sensitivity 75%, specificity 85.2%, PPV 60%, NPV 92%, and AUC 0.828) and the Ret-He cut-off point for IDA is 28.15 pg (sensitivity 75%, specificity 88.9%, PPV 85.7%, NPV 80%, and AUC 0.824). Hemoglobin should be used in conjunction with Ret-He. ID might be detected in this cohort with Ret-He 28.8 pg and hemoglobin > 16,5 g/dL. While Ret-He 28.15 pg or Ret-He 28.15-28.8 pg with hemoglobin 16.5 g/dL could be used to diagnose IDA. CONCLUSION: The reticulocyte hemolgobin equivalent could be utilised as an iron status parameter in pediatric CHD, with a cut-off value of 28.8 pg for ID and 28.15 pg for IDA.