Prevalence Of Congenital Diaphragmatic Hernia Research Articles

Epidemiology of congenital diaphragmatic hernia among 24 million Chinese births: a hospital-based surveillance study

Abstract Background The prevalence of congenital diaphragmatic hernia (CDH) varies across countries, with limited information available on its epidemiology in China. Our study aimed to investigate the prevalence, time trends, and perinatal outcomes of CDH in China, as well as its associated malformations and potential associations with maternal and infant characteristics. Methods This study included all birth and CDH cases from the Chinese Birth Defects Monitoring Network between 2007 and 2019, with CDH cases classified as either isolated or associated. We employed the joinpoint regression model to calculate the trends of prevalence and the annual percent change, with Poisson regression used for adjusted prevalence rate ratios. A P value ≤ 0.05 was considered statistically significant. Results A total of 4397 CDH cases were identified among 24,158,029 births in the study period, yielding prevalence rates of 1.82, 1.13 and 0.69 per 10,000 for overall, isolated, and associated CDH, respectively. The prevalence of each type of CDH increased over time. The prevalence of overall CDH varied significantly by infant sex (male vs. female, 1.91/10,000 vs. 1.63/10,000), maternal residence (urban vs. rural, 2.13/10,000 vs. 1.45/10,000), maternal age (< 20 years, 1.31/10,000; 20–24 years, 1.63/10,000; 25–29 years, 1.80/10,000; 30–34 years, 1.87/10,000; ≥ 35 years, 2.22/10,000), and geographic region (central, 1.64/10,000; east, 2.45/10,000; west, 1.37/10,000). Cardiovascular anomalies were the most common malformations associated with CDH. Infants with associated CDH had a higher risk of premature birth and perinatal death than those with isolated CDH. Conclusion The increasing prevalence and high perinatal mortality rate of CDH highlight the need for further etiological, epidemiological, and clinical studies among the Chinese population.

Crossing birth and mortality data as a clue for prevalence of congenital diaphragmatic hernia in Sao Paulo State: A cross sectional study

Congenital diaphragmatic hernia (CDH) is a severe embryological defect that causes pulmonary hypoplasia and hypertension. The prevalence and mortality rate of CDH varies around the world and little information is available about CDH in Latin America. Our aim was to estimate the general prevalence, mortality rate, prevalence of associated anomalies and features related to the outcomes of CDH in newborns from São Paulo state, Brazil. Population-based cross-sectional study based on data gathered from the Live Births Information System (SINASC) and the Mortality Information System (SIM) of children born in São Paulo state between January 1st, 2006, and December 31st, 2017. From 7,311,074 total survival discharges between 2006 and 2017, 1,155 were CDH-related, resulting in a prevalence rate of 1:6329 (95%CI=1/6715 - 1/5984) and a mortality rate of 63·72% (95%CI=60.95 - 66.50), 510 presented complex associated anomalies (44·15%). Maternal data showed higher prevalence among older mothers (older than 35 years old: 2·13 per 10,000) and, also, women with more years of schooling (higher than 12 years: 1·99 per 10,000). Presence of associated anomalies (95%CI=5.69-11.10), 1-min Apgar (95%CI=1.44-2.95), maternal schooling (95%CI=1.06-2.43) and birth weight (95%CI=1.04-2.26) were the most significant features associated with mortality. There was 1 CDH case for every 6329 newborns in São Paulo and the mortality rate among those cases was 63·72% - a high rate compared to other countries. This study didn't receive any specific grant from any funding agency in the public, commercial or not-for-profit sectors.

Anesthesia management of congenital diaphragmatic hernia in neonates

Congenital diaphragmatic hernia (CDH) is an emergency case in neonates. The prevalence of CDH is 1 in 2500 births and occurs 4–8 times more commonly on left side than right side. CDH is usually worsened by pulmonary hypoplasia and pulmonary hypertension. It is characterized by respiratory failure and bowel sound in the chest area and mostly requires surgical procedure. This case report discusses a neonate with congenital left diaphragmatic hernia who underwent surgical procedure to close the defect. Patients present with cyanosis and shortness of breath, are early intubated, and are mechanically ventilated in neonatal intensive care unit (NICU). In the present case, the surgical procedure lasted approximately 2 h with general anesthesia, with hemodynamic stability during the surgery. The patient was thereafter intubated and treated at the NICU for close monitoring.

Crossing Birth and Mortality Data as a Clue for Prevalence of Congenital Diaphragmatic Hernia in Sao Paulo State: A Cross Sectional Study

Crossing Birth and Mortality Data as a Clue for Prevalence of Congenital Diaphragmatic Hernia in Sao Paulo State: A Cross Sectional Study

Prevalence and mortality in children with congenital diaphragmatic hernia: a multicountry study

PurposeThis study determined the prevalence, mortality, and time trends of children with congenital diaphragmatic hernia (CDH). MethodsTwenty-five hospital- and population-based surveillance programs in 19 International Clearinghouse for Birth Defects Surveillance and Research member countries provided birth defects mortality data between 1974 and 2015. CDH cases included live births, stillbirths, or elective termination of pregnancy for fetal anomalies. Prevalence, cumulative mortality rates, and 95% confidence intervals (CIs) were calculated using Poisson regression and a Kaplan–Meier product-limit method. Joinpoint regression analyses were conducted to assess time trends. ResultsThe prevalence of CDH was 2.6 per 10,000 total births (95% CI: 2.5–2.7), slightly increasing between 2001 and 2012 (average annual percent change = 0.5%; 95% CI:−0.6 to 1.6). The total percent mortality of CDH was 37.7%, with hospital-based registries having more deaths among live births than population-based registries (45.1% vs. 33.8%). Mortality rates decreased over time (average annual percent change = −2.4%; 95% CI: −3.8 to 1.1). Most deaths due to CDH occurred among 2- to 6-day-old infants for both registry types (36.3%, hospital-based; 12.1%, population-based). ConclusionsThe mortality of CDH has decreased over time. Mortality remains high during the first week and varied by registry type.

The Prevalence and Impact of Congenital Diaphragmatic Hernia Among Patients Undergoing Surgery for Congenital Heart Disease

There has not been a multicenter investigation to elucidate the prevalence and impact of congenital diaphragmatic hernia (CDH) on children undergoing cardiac surgery. We investigated the prevalence of CDH across congenital cardiac diagnostic and procedural groups and its impact on outcomes. The STS Congenital Heart Surgery Database was queried to identify children undergoing cardiac surgery who also had CDH. Baseline perioperative characteristics and postoperative outcomes were compared between groups. Subgroup analyses were performed based on case complexity as determined by STAT Categories. Overall 426 of 157,419 (0.27%) pediatric patients undergoing an index cardiac operation had a diagnosis of CDH including 89 neonates (0.25% of all neonatal index operations), 217 infants (0.39%), and 120 children (0.18%). The frequency of concomitant CDH varied across diagnostic groups and, in neonates and infants, was highest for tetralogy of Fallot (0.45%, n = 41), coarctation (0.39%, n = 27) and complete atrioventricular septal defects (0.31%, n = 19). For neonates and infants outcomes were generally worse in CDH vs no-CDH patients across the various procedural strata. For example, STAT ≥3 operative mortality was 34.4% vs 10.3% ( P < 0.001) for neonates, and 24.2% vs 5.3% ( P < 0.001) for infants with vs without CDH, respectively. In older children, outcomes disparities were less clear cut with no significant differences in mortality, and variable differences for complication and length of stay outcomes. CDH occurs in a small percentage of patients born with congenital heart disease. Concomitant diagnosis of CDH portends increased morbidity and mortality in infants and neonates undergoing cardiac surgery.

Trends, correlates, and survival of infants with congenital diaphragmatic hernia and its subtypes.

To identify the live-birth prevalence, trends, correlates, and neonatal and 1-year survival rates of congenital diaphragmatic hernia. Using a population-based, retrospective cohort study design, we examined 1,025 cases of congenital diaphragmatic hernia from the 1998-2012 Florida Birth Defects Registry. We used Poisson and joinpoint regression models to compute prevalence ratios and temporal trends, respectively. Kaplan-Meier survival curves and Cox proportional hazards regression were used to describe neonatal and 1-year survival and estimate hazard ratios representing the predictors of infant survival. The birth prevalence of congenital diaphragmatic hernia was 3.19 per 10,000 live births (95% confidence interval [CI]: 3.00-3.39); there was a 4.2% yearly increase among multiple cases only. Among all cases, maternal education less than high school (prevalence ratio: 1.25, 95% CI: 1.02-1.53), high school/associate degree/GED (prevalence ratio: 1.15, 95% CI: 1.01-1.32), multiple birth (prevalence ratio: 1.38, 95% CI: 1.05-1.81), and male sex (prevalence ratio: 1.18, 95% CI: 1.05-1.32) were associated with increased risk for congenital diaphragmatic hernia. The 24-hr, neonatal, and 1-year survival rates were 93.6%, 79.8%, and 71.2%, respectively. The highest hazard ratio of 17.87 (95% CI: 1.49-213.82) was observed for neonatal mortality among cases associated with chromosomal anomalies and born <37 weeks at < 1,500 g. Among isolated cases, multiple birth (hazard ratio: 0.41, 95% CI: 0.20-0.86) was associated with decreased 1-year mortality. Low maternal education and multiple birth may be linked to congenital diaphragmatic hernia. The trends in prevalence, epidemiologic correlates, and predictors of early survival can differ between congenital diaphragmatic hernia subtypes-isolated, multiple, and chromosomal.© 2018 Wiley Periodicals, Inc. © 2018 Wiley Periodicals, Inc.

Congenital diaphragmatic hernia with heart defect has a high risk for hypoplastic left heart syndrome and major extra-cardiac malformations: 10-year national cohort from Finland.

Congenital diaphragmatic hernia (CDH) has a well-known risk of congenital heart defects with poor prognosis. This study was conducted to determine the national total prevalence and prenatal detection rates of CDH with heart defects and its association with major extra-cardiac malformations and to further evaluate the impact of the heart defect severity on survival. A 10-year national cohort was derived from four national registries, including live births, stillbirths, and terminations of pregnancy for fetal anomalies. The study cohort was sorted according to cardiac defect severity. The total prevalence of CDH with heart defects was 0.6/10000 births and live birth prevalence 0.3/10000 live births. Of 145 cases with CDH, 37 (26%) had a concurrent heart defect. The overall prenatal detection rate of heart defects was 41%. The total prevalence (483/10000) and live birth prevalence (500/10000) of hypoplastic left heart syndrome were 124 and 250 times higher than in the general population in Finland, respectively. Additional major extra-cardiac malformations were found in 68% of cases. The survival rate for CDH with major heart defects was 11 and 38% with minor heart defects. The total prevalence of hypoplastic left heart syndrome was significantly higher in CDH patients than in the general population in Finland. Prenatal detection rate for heart defects in CDH patients was 41%. Major extra-cardiac malformations were more common than previously reported. The prognosis of CDH with major heart defects remained poor.

Congenital diaphragmatic hernia in 22q11.2 deletion syndrome.

We report the important association of congenital diaphragmatic hernia (CDH) and 22q11.2 deletion syndrome (22q11.2DS). The prevalence of CDH in our cohort of patients with 22q11.2DS is 0.8% (10/1246), which is greater than in the general population (0.025%). This observation suggests that 22q11.2DS should be considered when a child or fetus presents with CDH, in particular when other clinical findings associated with the 22q11.2DS are present, such as congenital cardiac defects. Furthermore, this finding may lead to the identification of an additional locus for diaphragmatic hernia in the general population. © 2016 Wiley Periodicals, Inc.

Risk factors for congenital diaphragmatic hernia in the Bogota birth defects surveillance and follow-up program, Colombia.

The mortality rate for congenital diaphragmatic hernia (CDH) remains high and prevention efforts are limited by the lack of known risk factors. The aim of this study was to determine prevalence, risk factors, and neonatal results associated with CDH on a surveillance system hospital-based in Bogotá, Colombia. The data used in this study were obtained from The Bogota Birth Defects Surveillance and Follow-up Program (BBDSFP), between January 2001 and December 2013. With 386,419 births, there were 81 cases of CDH. A case-control methodology was conducted with 48 of the total cases of CDH and 192 controls for association analysis. The prevalence of CDH was 2.1 per 10,000 births. In the case-control analysis, risk factors found were maternal age ≥35years (OR, 33.53; 95% CI, 7.02-160.11), infants with CDH were more likely to be born before 37weeks of gestation (OR, 5.57; 95% CI, 2.05-15.14), to weigh less than 2500g at birth (OR, 9.05; 95% CI, 3.51-23.32), and be small for gestational age (OR, 5.72; 95% CI, 2.18-14.99) with a high rate of death before hospital discharge in the CDH population (CDH: 38% vs BBDSFP: <1%; p<0.001). The prevalence of CDH calculated was similar to the one reported in the literature. CDH is strongly associated with a high rate of death before hospital discharge and the risk factors found were maternal age ≥35years, preterm birth, be small for gestational age, and have low weight at birth. These neonatal characteristics in developing countries would help to identify early CDH. Prevention efforts have been limited by the lack of known risk factors and established epidemiological profiles, especially in developing countries.

Birth region, race and sex may affect the prevalence of congenital diaphragmatic hernia, abdominal wall and neural tube defects among US newborns

Birth defects are number one cause of death among infants below 1 year of age. The objective is to examine the interaction of sex, race/ethnicity and place of birth on the prevalence of major congenital birth anomalies. We analyzed the data sets produced by the Healthcare Cost and Utilization Project (HCUP) for the years 1997-2004. We identified the malformations: congenital diaphragmatic hernia (CDH), abdominal wall defects (AWD) and neural tube defects (NTD) using their respective International Classification of Disease 9 diagnostic codes. Newborns were classified according to their birth region into four groups; Northeast, South, Midwest and West. We calculated prevalence of each disease for the overall sample then for every sex, race and birth region. Using stratified analysis and χ(2) test, we calculated the odds ratio (OR) risk for each disease comparing females with males, different races/ethnicity to Caucasians and different US regions to Northeast. There were 1291 newborns with CDH representing 0.031% of the sample. (AWD: 2184 (0.052%) and NTD: 979 (0.024%)). West region had the highest prevalence of CDH (OR=1.62 (confidence intervals (CI): 1.4-1.9, P<0.001)). Female-to-male risk disparities were most observed among Caucasians in the South (OR=1.44 (CI: 1.1-1.8, P=0.003)). African Americans had the least prevalence of CDH but only in the South (OR=0.67 (CI: 0.5-0.8, P=0.001)). Native Americans had higher risk for AWD in the Midwest and West regions compared with Caucasians. This study links the birth region as a detrimental factor like sex and race in the prevalence of CDH, AWD and NTD. These findings implicate a possible role for environmental factors in the pathogenesis of these diseases.

Examining the Hidden Mortality of Congenital Diaphragmatic Hernia

Background and aims: Congenital diaphragmatic hernia (CDH) poses significant medical, social and economic challenges. Our objective was to examine the impact of antenatal and perinatal management on fetal and maternal outcomes in pregnancies complicated by fetal CDH.Methods: Analysis of all pregnancies with antenatal or postnatal diagnosis of CDH in Ontario, (∽145,000 deliveries annually) between 2005 and 2009. Data were obtained from all tertiary care fetal medicine units and pediatric surgical centres in the Ontario. Maternal characteristics, antenatal information, severity of defect, surgical management and newborn outcomes were studied.Results: During the 5 year study period, 109 cases of CDH were identified by antenatal ultrasound at a mean of 22.2+7.2 weeks gestation, only 2 cases were undiagnosed antenatally. One or more significant associated anomalies were detected in 19% of fetuses, 33% being cardiovascular. Seventy eight pregnancies continued, 90% had L sided CDH and were delivered at a mean gestational age of 37.6+2.5 weeks, mean birth weight 3.1+0.6kg. Preterm delivery occurred in 17% and 14% of infants were low birth weight. Estimations of lung head ratio were available for 30 cases and were significantly greater for infants who survived, 1.55+0.5, compared to 1.17+0.25 for non-survivors, p=0.05. Mean NICU stay was 37 days, with 73% of admitted infants surviving, representing 52% of the original antenatal cohort.Conclusions: Greater understanding of the true prevalence of CDH and the factors that contribute to mortality, may identify interventions including antenatal surgery, that could be introduced to improve survival.

Epidemiologic characteristics of congenital diaphragmatic hernia among 2.5 million california births, 1989–1997

Congenital diaphragmatic hernia (CDH) is a severe birth defect of unknown etiology. The aims of the current report were to extend the literature on the descriptive epidemiology of CDH and to determine whether its mortality rate decreased in California during the study period. Using data from a large population-based registry, we ascertained 631 CDH cases from 1989 to 1997. We also classified cases as isolated or nonisolated based on the presence and type of major accompanying malformations. Approximately 2.5 million live and stillbirths occurred during the ascertainment period. Multivariate Poisson regression analysis was applied to examine the time trend and risk factors. The overall prevalence of CDH was 2.49 per 10,000 live and stillbirths and did not vary over the study period. Isolated cases, which accounted for 58% of cases, revealed a prevalence of 1.45 per 10,000. Heart malformations were the most frequent major anomalies accompanying CDH. We observed a lower risk of isolated CDH among blacks. Advanced maternal age groups had a higher risk for nonisolated CDH. Multiparous women tended to have a lower risk for nonisolated CDH. Male infants and multiple births had an increased risk for isolated and nonisolated CDH. Infant mortality was slightly decreased over the study period. CDH cases with additional anomalies had higher mortality rates than isolated cases. Our observations add to the relatively few population-based descriptive epidemiologic studies of the prevalence and mortality, and maternal and infant characteristics of CDH.