RESUMEN

BACKGROUND: Critical congenital heart defects (CCHDs) are associated with considerable morbidity and mortality. This study estimated survival of children with nonsyndromic CCHDs and evaluated relationships between exposures of interest and survival by CCHD severity (univentricular or biventricular function). METHODS: This analysis included 4380 infants with CCHDs (cases) born during 1999-2011 and enrolled in the National Birth Defects Prevention Study, a multisite, population-based case-control study of major birth defects. Cases were linked to state death files. Nonparametric Kaplan-Meier survival functions were used to estimate 1- and 5-year survival probabilities overall and by severity group (univentricular/biventricular) stratified by demographic and clinical exposure variables of interest. The log-rank test was used to determine whether stratified survival curves were equivalent. Survival and 95% confidence intervals (CIs) were also estimated using Cox proportional hazards modeling adjusted for maternal age, education, race/ethnicity, study site, and birth year. RESULTS: One- and five-year survival rates were 85.8% (CI 84.7-86.8) and 83.7% (CI 82.5-84.9), respectively. Univentricular 5-year survival was lower than biventricular case survival [65.3% (CI 61.7-68.5) vs. 89.0% (CI 87.8-90.1; p < 0.001)]. Clinical factors (e.g. preterm birth, low birthweight, and complex/multiple defects) were associated with lower survival in each severity group. Sociodemographic factors (non-Hispanic Black race/ethnicity,

Asunto(s)

Cardiopatías Congénitas , Humanos , Cardiopatías Congénitas/mortalidad , Femenino , Masculino , Estudios de Casos y Controles , Lactante , Recién Nacido , Modelos de Riesgos Proporcionales , Preescolar , Estimación de Kaplan-Meier , Estados Unidos , Tasa de Supervivencia , Factores de Riesgo , Niño

RESUMEN

Various scoring systems have been used to predict mortality and morbidity after congenital heart surgery. While the ideal system is still controversial, the technical performance score (TPS) has recently gained popularity. In this study, was investigated the effect of the TPS in predicting mortality and morbidity in pediatric patients who underwent congenital heart surgery in our clinic. Patients aged < 18 years who underwent congenital heart surgery between 2020 and 2023, were retrospectively analyzed. The patients' TPS categorizations were assigned according to their echocardiographic results at discharge and whether they required reintervention. The primary endpoints of the study were mortality (death within 30 days postoperatively) and morbidity. The secondary endpoint was a comparison of the effectiveness of TPS with that of the widely used Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery score. Included in this study were 1075 patients. The median patient age was 3 months (interquartile range, 1-5 months). The mortality and morbidity rates were 11% and 24%, respectively. Of the patients, 60% were categorized as TPS I (optimal), 25% as TPS 2 (adequate, minimal residual defect), and 15% as TPS 3 (inadequate, hemodynamically significant residual defect). Being categorized as TPS 3 was associated with mortality, prolonged ICU stay, and major adverse events. The predictive power of TPS for mortality and morbidity was an area under the receiver operating characteristic curve (AUC) of 0.810 (95% CI: 0.79-0.839, P < .001) and 0.78 (95% CI: 0.76-0.80, P < .001), respectively. These values were similar to those of the Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery score (0.81 vs 0.83 and 0.78 vs 0.80 for mortality and morbidity, respectively). In patients with highly heterogeneous congenital heart disease, the use of intraoperative TPS may be helpful in predicting mortality and morbidity.

Asunto(s)

Procedimientos Quirúrgicos Cardíacos , Cardiopatías Congénitas , Complicaciones Posoperatorias , Humanos , Cardiopatías Congénitas/cirugía , Cardiopatías Congénitas/mortalidad , Femenino , Masculino , Estudios Retrospectivos , Lactante , Procedimientos Quirúrgicos Cardíacos/mortalidad , Procedimientos Quirúrgicos Cardíacos/métodos , Complicaciones Posoperatorias/epidemiología , Complicaciones Posoperatorias/mortalidad , Ecocardiografía , Medición de Riesgo/métodos , Preescolar , Curva ROC , Niño

RESUMEN

BACKGROUND: Infant mortality continues to be a significant problem for patients with congenital heart disease (CHD). Limited data exist on the recent trends of mortality in infants with CHD. METHODS: The CDC WONDER (Centers for Disease Control and Prevention Wide-Ranging Online Data for Epidemiologic Research) was queried to identify deaths occurring within the United States with CHD listed as one of the causes of death between 1999 and 2020. Subsequently, trends were calculated using the Joinpoint regression program (version 4.9.1.0; National Cancer Institute). RESULTS: A total of 47,015 deaths occurred in infants due to CHD at the national level from the year 1999 to 2020. The overall proportional infant mortality (compared to all deaths) declined (47.3% to 37.1%, average annual percent change [AAPC]: -1.1 [95% CI -1.6 to -0.6, p < 0.001]). There was a significant decline in proportional mortality in both Black (45.3% to 34.3%, AAPC: -0.5 [-0.8 to -0.2, p = 0.002]) and White patients (55.6% to 48.6%, AAPC: -1.2 [-1.7 to -0.7, p = 0.001]), with a steeper decline among White than Black patients. A statistically significant decline in the proportional infant mortality in both non-Hispanic (43.3% to 33.0%, AAPC: -1.3% [95% CI -1.9 to -0.7, p < 0.001]) and Hispanic (67.6% to 57.7%, AAPC: -0.7 [95% CI -0.9 to -0.4, p < 0.001]) patients was observed, with a steeper decline among non-Hispanic infant population. The proportional infant mortality decreased in males (47.5% to 53.1%, AAPC: -1.4% [-1.9 to -0.9, p < 0.001]) and females (47.1% to 39.6%, AAPC: -0.9 [-1.9 to 0.0, p = 0.05]). A steady decline in for both females and males was noted. CONCLUSION: Our study showed a significant decrease in CHD-related mortality rate in infants and age-adjusted mortality rate (AAMR) between 1999 and 2020. However, sex-based, racial/ethnic disparities were noted, with female, Black, and Hispanic patients showing a lesser decline than male, White, and non-Hispanic patients.

Asunto(s)

Centers for Disease Control and Prevention, U.S. , Cardiopatías Congénitas , Mortalidad Infantil , Humanos , Cardiopatías Congénitas/mortalidad , Estados Unidos/epidemiología , Masculino , Femenino , Lactante , Mortalidad Infantil/tendencias , Recién Nacido , Estudios de Cohortes , Hispánicos o Latinos/estadística & datos numéricos , Causas de Muerte/tendencias , Población Blanca

RESUMEN

BACKGROUND: Neonatal mortality poses a significant public health challenge in sub-Saharan Africa, with congenital heart disease emerging as the leading cause of morbidity and mortality among neonates, especially in countries like Ethiopia. Despite efforts to reduce neonatal mortality rates, Ethiopia continues to experience an increased mortality rate, particularly among neonates with congenital heart disease. This study aims to investigate the incidence and predictors of mortality in this vulnerable population within Ethiopia. METHOD: A retrospective cohort study was conducted at an institution, involving 583 randomly selected neonates diagnosed with congenital heart disease. In the current study, the dependent variable was survival status. Data entry utilized EpiData data version 4.6, and analysis was performed using STATA version 16. Probability of death was compared using the log-rank test and Kaplan-Meier failure curve. Significant predictors were identified using bivariable and multivariate Cox regression. Model fitness and proportional hazard assumptions were evaluated using the Cox-Snell graph and Global test, respectively. Associations were assessed by adjusted hazard ratios with 95% confidence intervals. RESULTS: The study participants were followed for 4844 days. The mortality rate was 9.9%. The incidence density was 11.9 per 1000 person-days of observation. Neonatal sepsis (AHR: 2.24; 95% CI [1.18-4.23]), cyanotic congenital heart disease (AHR: 3.49; 95% CI [1.93-6.28]), home delivery (AHR: 1.9; 95% CI [1.06-3.6]), maternal history of gestational diabetes mellitus (AHR: 1.94; 95% CI [1.04-3.61]), and having additional congenital malformations (AHR: 2.49; 95% CI [1.33-4.67]) were significant predictors for neonatal mortality. CONCLUSION AND RECOMMENDATION: The incidence density of mortality was high compared to studies conducted in developed countries. Neonatal sepsis, type of congenital heart disease, place of delivery, maternal history of gestational diabetes mellitus, and having an additional congenital malformation were significant predictors of mortality among neonates with congenital heart disease. Therefore, healthcare providers should pay special attention to patients with identified predictors. Furthermore, the Federal Ministry of Health, stakeholders, and policymakers should collaborate to address this issue.

Asunto(s)

Cardiopatías Congénitas , Mortalidad Infantil , Humanos , Etiopía/epidemiología , Cardiopatías Congénitas/mortalidad , Estudios Retrospectivos , Recién Nacido , Incidencia , Femenino , Masculino , Mortalidad Infantil/tendencias , Factores de Riesgo , Lactante

RESUMEN

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.

Asunto(s)

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 Riesgo

RESUMEN

BACKGROUND: Pulmonary Atresia, Ventricular Deptal Defect, and Major Aortopulmonary Collateral Arteries (PA-VSD-MAPCAs) is a congenital cyanotic heart defect with poor prognosis. Due to its complex and highly variable anatomy, the best treatment plan is not clear. We aimed (1) to investigate the survival of PA-VSD-MAPCAs patients according to the underlying original anatomy and treatment strategy, and (2) to evaluate life expectancy between patients with or without severe hypoplastic native pulmonary arteries (NPAs) after surgical versus non-surgical treatment. METHODS: A prospectively established database of 169 PA-VSD-MAPCAs patients treated and followed up at University Hospitals Leuven was accessed. Patients were divided into three groups according to the treatment strategy. Kaplan-Meier survival curves were plotted, and Log Rank tests were used for comparison. RESULTS: The overall mean survival for patients with PA-VSD-MAPCAs was 38.5 years (95%-CI: 33.1-43.9). Patients with complete intracardiac repair had the longest mean survival of 43.8 years (95%-CI: 38.1-49.6) versus the other groups (p < 0.001). A longer mean event-free survival time was found in patients with normal, well-developed NPAs (p = 0.047). Finally, patients with poorly developed or absent NPAs had worse survival rates when a surgical approach was followed. Systemic-pulmonary shunt placement or unifocalisation had limited effect on prognosis in the absence of total repair (p = 0.167). CONCLUSIONS: Patients with PA-VSD-MAPCAs who underwent complete intracardiac repair and/or with well-developed native pulmonary arteries had the best prognosis. Our analyzed data suggest that incomplete surgical repair resulted in survival rates comparable to those seen with a non-surgical approach.

Asunto(s)

Arteria Pulmonar , Humanos , Masculino , Femenino , Pronóstico , Estudios Retrospectivos , Adulto , Arteria Pulmonar/cirugía , Estudios de Seguimiento , Atresia Pulmonar/cirugía , Atresia Pulmonar/mortalidad , Atresia Pulmonar/diagnóstico , Defectos del Tabique Interventricular/cirugía , Defectos del Tabique Interventricular/mortalidad , Persona de Mediana Edad , Circulación Colateral/fisiología , Estudios Prospectivos , Tasa de Supervivencia/tendencias , Factores de Tiempo , Adulto Joven , Cardiopatías Congénitas/mortalidad , Cardiopatías Congénitas/cirugía , Cardiopatías Congénitas/diagnóstico

RESUMEN

BACKGROUND: Cardiac catheterization is an invasive diagnostic and treatment tool for congenital heart disease (CHD) with potential complications. OBJECTIVE: To describe the immediate outcomes of patients who underwent cardiac catheterization for CHD at the Uganda Heart Institute (UHI). METHODS: The study was a retrospective chart review of 857 patients who underwent cardiac catheterization for CHD at UHI from 1st February 2012 to 30th June 2023. Precardiac catheterization clinical data, procedure details, and post-procedure data were recorded. The statistical software SPSS was used for data analysis. RESULTS: We studied 857 patients who underwent cardiac catheterization for CHD at UHI. Females comprised 62.8% (n = 528). The age range was 3 days to 64 years, with a mean of 5.1 years (SD 7.4). Advanced heart failure was present in 24(2.8%) of the study participants. The most common procedures were patent ductus arteriosus device closure (n = 500, 58.3%), diagnostic catheterization (n = 194, 22.5%), and balloon pulmonary valvuloplasty (n = 114, 13.0%). PDA device closure had 89.4% optimal results while BPV had 75.9% optimal performance outcome. Adverse events occurred in 52 out of 857 study participants (6.1%). Clinically meaningful adverse events (CMAES) occurred in 3.9%, (n = 33), high severity adverse events in 2.9% (n = 25) and mortality in 1.5% (n = 13). Advanced heart failure at the time of cardiac catheterization, was significantly associated with clinically meaningful adverse events (OR 52 p-value < 0.001) and mortality (OR 564, p value < 0.001). CONCLUSION: Many patients with CHD have benefited from the cardiac catheterization program at UHI with high optimal procedure outcome results. Patients with advanced heart failure at the time of cardiac catheterization have less favorable outcomes emphasizing the need for early detection and early intervention.

Asunto(s)

Cateterismo Cardíaco , Cardiopatías Congénitas , Humanos , Cateterismo Cardíaco/efectos adversos , Cateterismo Cardíaco/mortalidad , Estudios Retrospectivos , Femenino , Uganda/epidemiología , Cardiopatías Congénitas/terapia , Cardiopatías Congénitas/diagnóstico , Cardiopatías Congénitas/mortalidad , Masculino , Lactante , Preescolar , Factores de Tiempo , Persona de Mediana Edad , Resultado del Tratamiento , Adulto , Recién Nacido , Adolescente , Niño , Adulto Joven , Factores de Riesgo

RESUMEN

OBJECTIVES: The use of ventricular assist devices (VADs) in children is increasing. However, absolute numbers in individual centres and countries remain small. Collaborative efforts such as the Paedi-European Registry for Patients with Mechanical Circulatory Support (EUROMACS) are therefore essential for combining international experience with paediatric VADs. Our goal was to present the results from the fourth Paedi-EUROMACS report. METHODS: All paediatric (<19 years) patients from the EUROMACS database supported by a VAD were included. Patients were stratified into a congenital heart disease (CHD) group and a group with a non-congenital aetiology. End points included mortality, a transplant and recovery. Cox proportional hazard models were used to explore associated factors for mortality, cerebrovascular accident and pump thrombosis. RESULTS: A total of 590 primary implants were included. The congenital group was significantly younger (2.5 vs 8.0 years, respectively, P < 0.001) and was more commonly supported by a pulsatile flow device (73.5% vs 59.9%, P < 0.001). Mortality was significantly higher in the congenital group (30.8% vs 20.4%, P = 0.009) than in the non-congenital group. However, in multivariable analyses, CHD was not significantly associated with mortality [hazard ratio (HR) 1.285; confidence interval (CI) 0.8111-2.036, P = 0.740]. Pump thrombosis was the most frequently reported adverse event (377 events in 132 patients; 0.925 events per patient-year) and was significantly associated with body surface area (HR 0.524, CI 0.333-0.823, P = 0.005), CHD (HR 1.641, CI 1.054-2.555, P = 0.028) and pulsatile flow support (HR 2.345, CI 1.406-3.910, P = 0.001) in multivariable analyses. CONCLUSIONS: This fourth Paedi-EUROMACS report highlights the increasing use of paediatric VADs. The patient populations with congenital and non-congenital aetiologies exhibit distinct characteristics and clinical outcomes.

Asunto(s)

Corazón Auxiliar , Sistema de Registros , Humanos , Sistema de Registros/estadística & datos numéricos , Corazón Auxiliar/estadística & datos numéricos , Corazón Auxiliar/efectos adversos , Niño , Preescolar , Masculino , Femenino , Lactante , Europa (Continente)/epidemiología , Adolescente , Cardiopatías Congénitas/cirugía , Cardiopatías Congénitas/mortalidad , Insuficiencia Cardíaca/mortalidad , Insuficiencia Cardíaca/cirugía , Recién Nacido

RESUMEN

Despite significant strides made in childhood survival during the last 75 years, India bears the largest burden of congenital heart disease (CHD) in the world. The care of a child with CHD requires multidisciplinary collaboration and development of distinct training opportunities in developing countries to ensure outcomes similar to those achieved in high-income countries. We present a commentary on the current state of pediatric cardiac critical care in India and propose pathways to fulfil the unmet needs of Indian children. The aim is to achieve self-reliance in pediatric cardiac services and to move towards optimal outcome and intact survival of children with CHD.

Asunto(s)

Cuidados Críticos , Cardiopatías Congénitas , Humanos , Cardiopatías Congénitas/terapia , Cardiopatías Congénitas/mortalidad , India/epidemiología , Niño , Pediatría/organización & administración , Pediatría/métodos , Preescolar

RESUMEN

Pulmonary arterial hypertension (PAH) associated with congenital heart disease (PAH-CHD) is a complication that occurs after unrepaired significant systemic-to-pulmonary shunt. Treatment options for PAH-CHD-predominantly left-to-right (L-R) shunt in children with borderline-high pulmonary vascular resistant index (PVRi) have been debated. We aimed to assess the treatment and survival of children with PAH-CHD-predominantly L-R shunt with borderline to high PVRi, using Eisenmenger syndrome (ES) for comparison. In 1995-2021, a total of 142 patients with ES and 192 children with PAH-CHD-predominantly L-R shunt were eligible for our analysis. The PVRi in ES patients was 26.7 ± 16.8 WU m2. Most patients (91%) received PAH-targeted therapy. Of the 192 children with PAH-CHD-predominantly L-R shunt, the baseline PVRi was 9.2 ± 5.8 WU m2. A total of 64 patients (33.3%) had borderline PVRi (4-8 WU m2) and 98 patients (51%) had high PVRi (> 8 WU m2). Most patients (88.5%) responded to acute pulmonary vasodilatory testing and underwent repair, with 158 undergoing defect closure and 12 having fenestrated closure. A treat-and-repair strategy was used in 33 children (17.1%). The 10- and 15-year survival rates for patients with ES were 79.3% and 72.4%, respectively, which was significantly inferior to children with borderline PVRi [97.3% and 87.8% (p = 0.02)]; and high PVRi [91.6% and 89.5% (p = 0.06)], respectively. The survival rate of children receiving treat-and-repair was slightly higher than that of ES (p = 0.16). The independent mortality risk in children with PAH-CHD-predominantly L-R shunt was persistent PAH following the defect correction (adjusted hazard ratio 5.8, 95% CI 1.7-19.9, p = 0.005).Trial registration: TCTR20200420004.

Asunto(s)

Complejo de Eisenmenger , Resistencia Vascular , Humanos , Complejo de Eisenmenger/cirugía , Complejo de Eisenmenger/mortalidad , Complejo de Eisenmenger/fisiopatología , Femenino , Masculino , Niño , Preescolar , Adolescente , Lactante , Cardiopatías Congénitas/cirugía , Cardiopatías Congénitas/mortalidad , Cardiopatías Congénitas/fisiopatología , Cardiopatías Congénitas/complicaciones , Estudios Retrospectivos , Hipertensión Arterial Pulmonar/cirugía , Hipertensión Arterial Pulmonar/mortalidad , Hipertensión Arterial Pulmonar/fisiopatología , Hipertensión Pulmonar/mortalidad , Hipertensión Pulmonar/cirugía , Hipertensión Pulmonar/fisiopatología

RESUMEN

BACKGROUND: Reinterventions may influence the outcomes of children with functionally single-ventricle (f-SV) congenital heart disease. METHODS AND RESULTS: We undertook a retrospective cohort study of children starting treatment for f-SV between 2000 and 2018 in England, using the national procedure registry. Patients were categorized based on whether they survived free of transplant beyond 1 year of age. Among patients who had transplant-free survival beyond 1 year of age, we explored the relationship between reinterventions in infancy and the outcomes of survival and Fontan completion, adjusting for complexity. Of 3307 patients with f-SV, 909 (27.5%), had no follow-up beyond 1 year of age, among whom 323 (35.3%) had ≥1 reinterventions in infancy. A total of 2398 (72.5%) patients with f-SV had transplant-free survival beyond 1 year of age, among whom 756 (31.5%) had ≥1 reinterventions in infancy. The 5-year transplant-free survival and cumulative incidence of Fontan, among those who survived infancy, were 93.4% (95% CI, 92.4%-94.4%) and 79.3% (95% CI, 77.4%-81.2%), respectively. Both survival and Fontan completion were similar for those with a single reintervention and those who had no reinterventions. Patients who had >1 additional surgery (adjusted hazard ratio, 3.93 [95% CI, 1.87-8.27] P<0.001) had higher adjusted risk of mortality. Patients who had >1 additional interventional catheter (adjusted subdistribution hazard ratio, 0.71 [95% CI, 0.52-0.96] P=0.03) had a lower likelihood of achieving Fontan. CONCLUSIONS: Among children with f-SV, the occurrence of >1 reintervention in the first year of life, especially surgical reinterventions, was associated with poorer prognosis later in childhood.

Asunto(s)

Cuidados Paliativos , Reoperación , Humanos , Masculino , Inglaterra/epidemiología , Femenino , Estudios Retrospectivos , Gales/epidemiología , Lactante , Preescolar , Reoperación/estadística & datos numéricos , Trasplante de Corazón/estadística & datos numéricos , Sistema de Registros , Procedimiento de Fontan/mortalidad , Corazón Univentricular/cirugía , Corazón Univentricular/mortalidad , Corazón Univentricular/fisiopatología , Ventrículos Cardíacos/anomalías , Ventrículos Cardíacos/cirugía , Ventrículos Cardíacos/fisiopatología , Recién Nacido , Cardiopatías Congénitas/cirugía , Cardiopatías Congénitas/mortalidad , Factores de Tiempo , Resultado del Tratamiento

Asunto(s)

Procedimiento de Fontan , Humanos , Femenino , Masculino , Niño , Preescolar , Etnicidad , Factores Sexuales , Corazón Univentricular/cirugía , Corazón Univentricular/fisiopatología , Lactante , Adolescente , Grupos Raciales , Cardiopatías Congénitas/cirugía , Cardiopatías Congénitas/mortalidad , Tasa de Supervivencia

RESUMEN

BACKGROUND: Fontan-associated liver disease (FALD) is one of the most common complications following Fontan procedure, but the impact of FALD on survival outcomes remains controversial. The aim of this systematic review and meta-analysis was to examine and quantify the influence of liver disease on the survival of Fontan patients. METHODS: The Preferred Reporting Items for Systematic reviews and Meta-Analyses guidelines were followed, and relevant human studies published from inception up to 12 August 2022 were searched. Stata (version 17.0) was applied to perform the meta-analysis, using random effects (Mantel-Haenszel) models. The I2 statistic was used to assess the heterogeneity. Subgroup analysis and meta-regression were employed to explore the potential sources of heterogeneity and sensitivity analysis was performed to determine the potential influence of each study on the overall pooled results. RESULTS: A total of 312 records were initially identified and 8 studies involving 2,466 patients were selected for inclusion. Results revealed a significant association between the severity of liver disease following Fontan procedure and mortality, which was confirmed by sensitivity analysis and subgroup analysis assessing post-HT mortality. Meta-regression showed that diagnostic methods for liver disease may be a source of heterogeneity. After removal of the FALD patients identified by international classification of disease codes, heterogeneity was markedly reduced, and the positive association between all-cause mortality and the severity of liver disease became significant. CONCLUSIONS: This meta-analysis showed the severity of liver disease following the Fontan procedure has a significant association with mortality. Lifelong follow-up is necessary and imaging examinations are recommended for routine surveillance of liver disease. Among patients with failing Fontan and advanced liver disease, combined heart-liver transplantation may provide additional survival benefits.

Asunto(s)

Procedimiento de Fontan , Cardiopatías Congénitas , Hepatopatías , Humanos , Procedimiento de Fontan/efectos adversos , Procedimiento de Fontan/mortalidad , Hepatopatías/mortalidad , Hepatopatías/diagnóstico , Hepatopatías/cirugía , Hepatopatías/etiología , Factores de Riesgo , Medición de Riesgo , Cardiopatías Congénitas/cirugía , Cardiopatías Congénitas/mortalidad , Cardiopatías Congénitas/diagnóstico , Resultado del Tratamiento , Masculino , Femenino , Niño , Adolescente , Adulto , Adulto Joven , Factores de Tiempo , Preescolar , Índice de Severidad de la Enfermedad

RESUMEN

BACKGROUND: Limited data exists on interpreting vectorcardiography (VCG) parameters in the Fontan population. OBJECTIVE: The purpose of this study was to demonstrate the associations between ECG/VCG parameters and Fontan failure (FF). METHODS/RESULTS: 107 patients with a Fontan operation after 1990 and without significant ventricular pacing were included. FF and Fontan survival (FS) groups were compared. The average follow-up after Fontan operation was 11.8 years ±7.1 years. 14 patients had FF (13.1%) which was defined as having protein-losing-enteropathy (1.9%), plastic bronchitis (2.8%), Fontan takedown (1.9%), heart transplant (5.6%), NYHA class III-IV (2.8%) or death (0.9%). A 12­lead ECG at last follow up or prior to FF was assessed for heart rate, PR interval, QRS duration, Qtc and left/right sided precordial measures (P-wave, QRS and T-wave vector magnitudes, spatial P-R and QRS-T angles). Transthoracic echocardiogram evaluated atrioventricular valve regurgitation and ventricular dysfunction at FF or last follow up. A cox multivariate regression analysis adjusted for LV dominance, ventricular dysfunction, HR, PR, QTc, Pvm, QRSvm, SPQRST-angle, RtPvm, RtQRSvm and RtTvm. Ventricular dysfunction, increased heart rate and prolonged PR interval were significantly associated to FF at the multivariate analysis. ROC analysis and Kaplan-meier analysis revealed an increased total mortality associated with a heart rate > 93 bpm, PR interval > 155 mv, QRSvm >1.91 mV, RtQRSvm >1.8 mV and SPQRST angle >92.3 mV with p values <0.001 to 0.018. CONCLUSION: We demonstrate the importance of ECG/VCG monitoring in the Fontan population and suggest specific indicators of late complications and mortality.

Asunto(s)

Procedimiento de Fontan , Frecuencia Cardíaca , Vectorcardiografía , Humanos , Masculino , Femenino , Vectorcardiografía/métodos , Niño , Electrocardiografía , Tasa de Supervivencia , Sensibilidad y Especificidad , Insuficiencia del Tratamiento , Cardiopatías Congénitas/cirugía , Cardiopatías Congénitas/mortalidad , Adolescente

RESUMEN

Congenital heart disease (CHD) and patent ductus arteriosus (PDA) are risk factors of necrotizing enterocolitis (NEC) in infants. However, it is unclear whether the prognosis of NEC is different between very preterm infants (VPIs) with and without heart diseases. This was an observational cohort study that enrolled VPIs (born between 24+0 and 31+6 weeks) admitted to 79 tertiary neonatal intensive care units (NICU) in the Chinese Neonatal Network (CHNN) between 2019 and 2021. The exposure was CHD or isolated PDA, and VPIs with NEC were divided into three groups: complicated with CHD, with isolated PDA, and without heart diseases. The primary outcomes were NEC-related adverse outcomes (death or extrauterine growth restriction (EUGR)). Logistic regression models were used to adjust potential confounders and calculate the odds ratios (ORs) and 95% confidential intervals (CIs) for each outcome. A total of 1335 VPIs with NEC were enrolled in this study, including 65 VPIs with CHD and 406 VPIs with isolated PDA. The VPIs with heart diseases had smaller gestational ages and lower body weights at birth, more antenatal steroids use, and requiring inotrope prior to the onset of NEC. While suffering from NEC, there was no significant increased risks in NEC-related death in VPIs with either CHD (adjusted OR [aOR]: 1.10; 95% CI: 0.41-2.50) or isolated PDA (aOR: 1.25; 95% CI 0.82-1.87), and increased risks in EUGR were identified in either survival VPIs with CHD (aOR: 2.35; 95% CI: 1.31-4.20) or isolated PDA (aOR: 1.53; 95% CI: 1.16-2.01) in survivors. The composite outcome (death or EUGR) was also more often observed in VPIs with either CHD (aOR: 2.07; 95% confidence interval [CI]: 1.20-3.60) or isolated PDA (aOR: 1.51; 95% CI: 1.17-1.94) than that without heart diseases. VPIs with either CHD or isolated PDA were associated with significantly prolonged duration of fasting, extended time to achieve full enteral feeding, and longer ventilation duration and hospitalization duration. Similar characteristics were also seen in VPIs with isolated PDA, with the exception that VPIs with CHD are more likely to undergo surgical intervention and maintain a prolonged fast after NEC.     Conclusion: In VPIs with NEC, CHD and isolated PDA are associated with an increased risk in worse outcomes. We recommend that VPIs with cardiac NEC be managed with aggressive treatment and nutrition strategies to prevent EUGR. What is Known: • CHD and PDA are risk factors for NEC in infants, which can lead to adverse outcomes such as death and EUGR. • NEC in infants with heart disease differs clinically from that in infants without heart disease and should be recognized as a separate disease process. What is New: • CHD and isolated PDA are associated with increased risks of EUGR in VPIs with NEC. • Risk factors associated with VPIs with cardiac NEC suggested these patients should be managed with aggressive treatment and nutrition strategies to adverse outcomes.

Asunto(s)

Enterocolitis Necrotizante , Cardiopatías Congénitas , Humanos , Enterocolitis Necrotizante/epidemiología , Enterocolitis Necrotizante/mortalidad , Enterocolitis Necrotizante/complicaciones , Recién Nacido , Masculino , Femenino , Cardiopatías Congénitas/complicaciones , Cardiopatías Congénitas/mortalidad , Enfermedades del Prematuro/mortalidad , Enfermedades del Prematuro/epidemiología , Conducto Arterioso Permeable/complicaciones , Conducto Arterioso Permeable/epidemiología , Estudios de Cohortes , Factores de Riesgo , Unidades de Cuidado Intensivo Neonatal/estadística & datos numéricos , China/epidemiología , Recien Nacido Prematuro , Estudios Retrospectivos

RESUMEN

OBJECTIVE: To explore the association between demographic characteristics and perinatal deaths attributable to congenital heart defects (CHDs). METHODS: Data were obtained from the Birth Defects Surveillance System of Hunan Province, China, 2016-2020. The surveillance population included fetuses and infants from 28 weeks of gestation to 7 days after birth whose mothers delivered in the surveillance hospitals. Surveillance data included demographic characteristics such as sex, residence, maternal age, and other key information, and were used to calculate the prevalence of CHDs and perinatal mortality rates (PMR) with 95% confidence intervals (CI). Multivariable logistic regression analysis (method: Forward, Wald, α = 0.05) and adjusted odds ratios (ORs) were used to identify factors associated with perinatal deaths attributable to CHDs. RESULTS: This study included 847755 fetuses, and 4161 CHDs were identified, with a prevalence of 0.49% (95%CI: 0.48-0.51). A total of 976 perinatal deaths attributable to CHDs were identified, including 16 (1.64%) early neonatal deaths and 960 (98.36%) stillbirths, with a PMR of 23.46% (95%CI: 21.98-24.93). In stepwise logistic regression analysis, perinatal deaths attributable to CHDs were more common in rural areas than urban areas (OR = 2.21, 95%CI: 1.76-2.78), more common in maternal age <20 years (OR = 2.40, 95%CI: 1.05-5.47), 20-24 years (OR = 2.13, 95%CI: 1.46-3.11) than maternal age of 25-29 years, more common in 2 (OR = 1.60, 95%CI: 1.18-2.18) or 3 (OR = 1.43, 95%CI: 1.01-2.02) or 4 (OR = 1.84, 95%CI: 1.21-2.78) or > = 5 (OR = 2.02, 95%CI: 1.28-3.18) previous pregnancies than the first pregnancy, and more common in CHDs diagnosed in > = 37 gestional weeks (OR = 77.37, 95%CI: 41.37-144.67) or 33-36 gestional weeks (OR = 305.63, 95%CI: 172.61-541.15) or < = 32 gestional weeks (OR = 395.69, 95%CI: 233.23-671.33) than diagnosed in postnatal period (within 7 days), and less common in multiple births than singletons (OR = 0.48, 95%CI: 0.28-0.80). CONCLUSIONS: Perinatal deaths were common in CHDs in Hunan in 2016-2020. Several demographic characteristics were associated with perinatal deaths attributable to CHDs, which may be summarized mainly as economic and medical conditions, severity of CHDs, and parental attitudes toward CHDs.

Asunto(s)

Cardiopatías Congénitas , Humanos , China/epidemiología , Cardiopatías Congénitas/mortalidad , Cardiopatías Congénitas/epidemiología , Femenino , Recién Nacido , Masculino , Adulto , Embarazo , Muerte Perinatal , Prevalencia , Mortalidad Perinatal/tendencias , Edad Materna , Adulto Joven , Modelos Logísticos , Mortinato/epidemiología , Lactante , Oportunidad Relativa , Factores de Riesgo

RESUMEN

BACKGROUND/AIM: The survival of patients with congenital heart disease (CHD) has dramatically improved over recent decades. However, a disparity exists depending on the country and medical system. This study aimed to analyze the survival of infants with CHD until the age of 18 years using large-scale population data in South Korea and investigate the effect of neonatal conditions at birth. PATIENTS AND METHODS: We retrospectively extracted the Korean National Health Insurance Service claims data from January 2002 to December 2020. We included patients diagnosed with CHD who were less than one year of age. The follow-up duration was until their death or until they were censored before the age of 18 years. The CHD lesions were classified hierarchically (conotruncal, severe non-conotruncal, coarctation of the aorta, ventricular septal defect, atrial septal defect, and others). Several neonatal conditions were adopted as risk factors. RESULTS: Overall, 127,958 infants had been diagnosed with CHD and 2,275 died before the age of 18 years. The survival rate of infants with CHD during childhood was 97.9%. The highest childhood mortality rate was associated with non-conotruncal defects (19.7%), followed by conotruncal defects (10.2%). The significant risk factors for childhood mortality were complex CHD, pulmonary hypertension, birth asphyxia, small for gestational age, respiratory distress, pulmonary hemorrhage, bronchopulmonary dysplasia, and convulsions. CONCLUSION: The survival of infants with CHD has been favorable in South Korea. Several neonatal conditions are risk factors for childhood mortality. Individualized risk assessment and optimal treatment strategies may help improve their survival rate.

Asunto(s)

Cardiopatías Congénitas , Humanos , Cardiopatías Congénitas/mortalidad , Cardiopatías Congénitas/epidemiología , República de Corea/epidemiología , Lactante , Femenino , Masculino , Factores de Riesgo , Recién Nacido , Preescolar , Niño , Adolescente , Estudios Retrospectivos , Tasa de Supervivencia

RESUMEN

INTRODUCTION: Congenital heart diseases (CHD) with abnormal turbulent blood flow are associated with the highest risk of infective endocarditis (IE). Despite advancement in diagnostics and treatment, the mortality rate of IE remains high due the life-threatening complications. Our study aims to assess the incidence and mortality rates of IE and predictive factors for mortality among adults CHD (ACHD). METHODS: A systematic literature search was conducted on PubMed, SCOPUS, and Ovid SP to retrieve relevant studies. The pooled estimates and predictors of mortality were calculated using the random-effects generic inverse variance method using R programming. RESULTS: 12 studies involving 3738 ACHD patients were included in this meta-analysis. The overall incidence of IE in ACHD was 1.26 per 1000 patient-years (95% CI 0.55-1.96). 60% (95% CI 46-72%) of patients had surgical management for IE. The mortality rate of IE was 9% (95% CI 7-12%). The predictors of mortality were conservative management (OR: 5.07, 95% CI: 4.63-5.57), renal dysfunction (OR: 4.15, 95% CI: 2.92-5.88), cerebral complications (OR: 3.59, 95% CI: 1.78-7.23), abscesses/valve complications (OR: 2.67, 95% CI: 1.71-4.16), Staphylococcus aureus infection (OR: 2.32, 95% CI: 1.33-4.06), emboli (OR: 2.03, 95% CI: 1.47-2.79), body mass index (OR: 1.10, 95% CI: 1.01-1.21), age (OR: 1.02, 95% CI: 1.00-1.04), and previous IE (OR: 1.02, 95% CI: 1.00-1.04). CONCLUSION: The mortality rate of IE in ACHD is low. However, conservative management is associated with the highest risk of mortality.

Asunto(s)

Endocarditis , Cardiopatías Congénitas , Humanos , Cardiopatías Congénitas/mortalidad , Cardiopatías Congénitas/complicaciones , Incidencia , Endocarditis/mortalidad , Endocarditis/epidemiología , Endocarditis/diagnóstico , Adulto , Factores de Riesgo , Valor Predictivo de las Pruebas , Mortalidad/tendencias

RESUMEN

BACKGROUND: The German Registry for Cardiac Operations and Interventions in Patients with Congenital Heart Disease is a voluntary registry initiated by the German Society for Thoracic and Cardiovascular Surgery and the German Society for Pediatric Cardiology and Congenital Heart Defects. Since 2012, the registry collects data for the assessment of treatment and outcomes of surgical and interventional procedures in patients with congenital heart disease (CHD) of all age groups. METHODS: This real-world, prospective all-comers registry collects clinical and procedural characteristics, adverse events (AEs), mortality, and medium-term outcomes (up to 90 days) of patients undergoing surgical and interventional. A unique pseudonymous personal identifier (PID) allows longitudinal data acquisition in case of further invasive treatment in any participating German heart center. Prior to evaluation, all data sets are monitored for data completeness and integrity. Evaluation includes risk stratification of interventional and surgical procedures and classification of AEs. Each year's data are summarized in annual reports containing detailed information on the entire cohort, all subgroups, and 15 index procedures. In addition, each participating center receives an institutional benchmark report for comparison with the national results. This paper presents a comprehensive summary of the annual report 2021. RESULTS: In 2021, a total of 5,439 patients were included by 22 participating centers. In total, 3,721 surgical, 3,413 interventional, and 34 hybrid procedures were performed during 6,122 hospital stays. 2,220 cases (36.3%) could be allocated to the 15 index procedures. The mean unadjusted in-hospital mortality ranged from 0.4% among interventional and 2% among surgical cases up to 6.2 % in cases with multiple procedures. In-hospital mortality among index procedures accounted for 2.3% in total cavopulmonary connection, 20.3% in Norwood procedures, and 0.4% following interventional closure of patent ductus arteriosus. For the remaining seven surgical and five interventional index procedures, no in-hospital deaths were recorded. The 10-year longitudinal evaluation of 1,795 patients after tetralogy of Fallot repair revealed repeat interventional or surgical procedures in 21% of the patients. Over the same period, 31.1% of 2,037 patients, following initial treatment of native coarctation, required at least one additional hospital admission, 39.4% after initial interventional, and 21.3% after initial surgical therapy. CONCLUSION: The annual report 2021 of the German Registry for Cardiac Operations and Interventions in CHD shows continuously good results in accordance with previous data of the registry. Compared to international registries on CHD, it can be ascertained that in Germany invasive treatment of CHD is offered on a high medical level with excellent quality. The proven fact that patients with various malformations like tetralogy of Fallot and coarctation of the aorta require repeat procedures during follow-up confirms the urgent requirement for longitudinal assessment of all patients presenting with complex lesions.

Asunto(s)

Procedimientos Quirúrgicos Cardíacos , Cardiopatías Congénitas , Complicaciones Posoperatorias , Sistema de Registros , Humanos , Cardiopatías Congénitas/cirugía , Cardiopatías Congénitas/mortalidad , Alemania , Procedimientos Quirúrgicos Cardíacos/efectos adversos , Procedimientos Quirúrgicos Cardíacos/mortalidad , Resultado del Tratamiento , Lactante , Factores de Tiempo , Preescolar , Niño , Factores de Riesgo , Recién Nacido , Adolescente , Complicaciones Posoperatorias/mortalidad , Complicaciones Posoperatorias/etiología , Complicaciones Posoperatorias/terapia , Masculino , Estudios Prospectivos , Femenino , Benchmarking , Adulto Joven , Medición de Riesgo , Indicadores de Calidad de la Atención de Salud , Informes Anuales como Asunto , Mortalidad Hospitalaria , Evaluación de Procesos y Resultados en Atención de Salud , Adulto

RESUMEN

BACKGROUND: Despite documented associations between social determinants of health and outcomes post-congenital heart surgery, clinical risk models typically exclude these factors. OBJECTIVES: The study sought to characterize associations between social determinants and operative and longitudinal mortality as well as assess impacts on risk model performance. METHODS: Demographic and clinical data were obtained for all congenital heart surgeries (2006-2021) from locally held Congenital Heart Surgery Collaborative for Longitudinal Outcomes and Utilization of Resources Society of Thoracic Surgeons Congenital Heart Surgery Database data. Neighborhood-level American Community Survey and composite sociodemographic measures were linked by zip code. Model prediction, discrimination, and impact on quality assessment were assessed before and after inclusion of social determinants in models based on the 2020 Society of Thoracic Surgeons Congenital Heart Surgery Database Mortality Risk Model. RESULTS: Of 14,173 total index operations across New York State, 12,321 cases, representing 10,271 patients at 8 centers, had zip codes for linkage. A total of 327 (2.7%) patients died in the hospital or before 30 days, and 314 children died by December 31, 2021 (total n = 641; 6.2%). Multiple measures of social determinants of health explained as much or more variability in operative and longitudinal mortality than clinical comorbidities or prior cardiac surgery. Inclusion of social determinants minimally improved models' predictive performance (operative: 0.834-0.844; longitudinal 0.808-0.811), but significantly improved model discrimination; 10.0% more survivors and 4.8% more mortalities were appropriately risk classified with inclusion. Wide variation in reclassification was observed by site, resulting in changes in the center performance classification category for 2 of 8 centers. CONCLUSIONS: Although indiscriminate inclusion of social determinants in clinical risk modeling can conceal inequities, thoughtful consideration can help centers understand their performance across populations and guide efforts to improve health equity.

Asunto(s)

Procedimientos Quirúrgicos Cardíacos , Cardiopatías Congénitas , Determinantes Sociales de la Salud , Humanos , Cardiopatías Congénitas/cirugía , Cardiopatías Congénitas/mortalidad , Masculino , Femenino , Procedimientos Quirúrgicos Cardíacos/mortalidad , Lactante , Preescolar , Medición de Riesgo/métodos , Niño , Recién Nacido , New York/epidemiología