Minor Congenital Heart Disease Research Articles

Temporal trend in hospitalization among patients with congenital heart disease in Denmark: a nationwide study

Abstract Background The survival rate of patients with congenital heart disease (CHD) had increased over the last decades due to improved management. However, the increased survival rate may potentially impact the number of occurrences of late cardiac complications as well as non-cardiovascular diseases requiring hospitalization, and hence warrants further study on this area. Purpose To delineate the temporal trend in hospitalization rate and length of stay (LOS) among patients with major and minor CHD in Denmark from 1977 to 2018 and to investigate the causes of hospitalization. Methods A Danish nationwide, population-based registry study including patients with CHD diagnosis from 1977 to 2018. Patients were categorized as either major or minor CHD, and by age groups (<1, 1-4, 5-17, 18-39, 40-64, ≥65 years). Hospitalization rate was reported as incidence per 100 person-years. Results In this study, 23,386 patients were included with 121,653 hospital admissions. From 1977-1981 to 2012-2018, the overall all-cause hospitalization rate decreased for both major (51.7 [CI: 48.0 -55.7] to 32.8 [CI: 31.0 - 34.7]) and minor CHD patients (28.4 [CI: 26.0 - 31.0] to 22.6 [CI: 21.1 - 24.1]). Median LOS for major CHD decreased from 5 days (IQR: 2 – 11) to 2 days (IQR: 1 – 6), and for minor CHD from 4 days (IQR: 2 – 8) to 2 days (IQR: 1 – 5). However, the trends when looking at specific age groups were different from the trend of overall all-cause hospitalization rate. Figure 1 shows that hospitalization rate had increased significantly for <1-year-olds for major CHD, whereas an increased trend was observed for <1, 40-64 and ≥65 years-olds for minor CHD. In addition, the trends for specific age groups when looking at the most common causes were also different from the trend of all-cause hospitalization rate. For both major and minor CHD, Table 1 shows that the hospitalization rate for <1-year-olds due to respiratory diseases had increased over time, while the hospitalization rate for 40-64-year-olds due to arrythmia had also increased over time. For ≥65-year-olds, significant increase in hospitalization rate for arrythmia was only observed for minor CHD. Conclusion Lower hospitalization rate for major and minor CHD after 1 year old suggests that early intervention of CHD has improved their morbidity during childhood and early adulthood. However, the increased hospitalization rates among <1 year-olds and the elderly suggest that more focus are needed in preventing non-cardiovascular diseases and late cardiac complications for the respective age groups.Figure 1Table 1

P-774 Congenital Heart Disease in Children Born after Assisted Reproductive Technology: A Nordic Cohort Study.

Abstract Study question Do children conceived after assisted reproductive technology (ART) have a higher risk of Congenital Heart Disease (CHD) in comparison with children born after spontaneous conception? Summary answer Children born after ART have a higher risk of a major CHD compared with children born after spontaneous conception. What is known already It is well-known that children born after ART have higher risk of birth defects compared to children born after spontaneous conception. Congenital heart disease (CHD) is the most common birth defect, accounting for almost 50% of all major birth defects. Several cohort studies and systematic reviews have also found an increased risk of CHD in children born after ART, a recent review included 41 studies and 25 856 ART children covering CHDs in ART children. Conflicting results have been reported for specific CHDs. Study design, size, duration This Nordic registry-based cohort study from CoNARTaS (Committee of Nordic ART and Safety) included 171 774 live births born after use of ART and 7 772 474 live births born after spontaneous conception during a study period of up to three decades (Denmark 1994-2014, Finland 1990-2014, Norway 1984-2015, and Sweden 1985-2015). National data from ART registries, medical birth registries, malformation registries, patient registries, cause of death registries, and population registries were crosslinked. Participants/materials, setting, methods International Classification of Diseases (ICD) versions 8, 9, 10 were used for CHD codes at birth and up to one year of age. Major CHDs were defined according to European Concerted Action on Congenital Anomalies and Twins (EUROCAT). According to EUROCAT, 26 of the major CHDs were considered as severe. All major CHDs were further classified into six different groups in a hierarchical order. Minor CHDs as well as stillbirths and pregnancy terminations were excluded. Main results and the role of chance Data from Sweden included a total of 3 228 526 singletons and multiples; 62 243 children conceived after use of ART and 3 166 283 children after spontaneous conception. Among ART children, 2.27% (n = 1411) were diagnosed with any major non-chromosomal CHD during their first year of life, corresponding figures for children born after spontaneous conception was 1.43% (n = 45 310) (Odds Ratio [OR] 1.60; 95% Confidence Interval (CI) 1.51 to 1.69, p < 0.0001). Severe CHD occurred in 0.40% (n = 249) in the ART group and 0.31% (n = 9 795) in the spontaneous conception group (OR 1.29; 95% CI 1.14 to 1.47, p < 0.0001). Ventricular septal defect was the most common CHD, 0.97% (n = 603) in the ART vs 0.64% (n = 20 343) in the spontaneous conception group (OR 1.51; 95% CI 1.39 to 1.64, p < 0.0001), followed by atrial septal defect; 0.84% (n = 525) in the ART and 0.42% (n = 13 363) in the spontaneous conception group (OR 2.00; 95% CI 1.83 to 2.19, p < 0.0001). Limitations, reasons for caution This analysis included only non-adjusted Swedish data. Further analysis will include Danish, Finnish, and Norwegian data with adjustments, and subgroup analysis on singletons and multiples. No data are available on prenatal diagnosis, or pregnancies not ending to a live birth. A skewed distribution in these outcomes might introduce a bias. Wider implications of the findings Pregnancies conceived after use of ART may benefit from screening with fetal echocardiography. Although most children with CHD reach adulthood, the mortality rate is still high for this group during the first years of life. Early detection with antenatal diagnosis and pre/postnatal intervention may be beneficial and improve the outcome. Trial registration number Not applicable

Birthweight of children with isolated congenital heart disease-A sibling analysis study.

Congenital heart disease (CHD) is associated with decreased birthweight (BW) compared to population-based references. The aim of this study was to compare the BW of isolated CHD cases to their siblings, thus controlling for unknown and unmeasured confounders within the family. All isolated CHD cases in the Leiden University Medical Center were included (2002-2019). Generalized estimated equation models were constructed to compare BW z scores of CHD neonates with their siblings. Cases were clustered tominor or severe CHD and stratified according to the aortic flow and oxygenation to the brain. The overall BW z score of siblings was 0.032 (n=471). The BW z score was significantly lower in CHD cases (n=291) compared to their siblings (-0.20, p=0.005). The results were consistent in the subgroup analysis of severe and minor CHD (BW z score difference -0.20 and -0.10), but did not differ significantly (p=0.63). Stratified analysis regarding flow and oxygenation showed no BW difference between the groups (p=0.1). Isolated CHD cases display a significantly lower BW z score compared to their siblings. As the siblings of these CHD cases show a BW distribution similar to the general population, this suggests that shared environmental and maternal influences between siblings do not explain the difference in BW.

School performance is impaired in children with both simple and complex congenital heart disease.

We do not know if children born with a simple or uncorrected congenital heart disease (CHD) have school performance issues and an increased need for special education compared to healthy peers. With this study we examine the school performance and the need for special education in children with both simple and complex CHD. Further, we evaluate if exposure to preeclampsia or smoking affects the need for special education. In this nation-wide population based registry study, we included all Danish children with CHD born 1994-2012. In addition ten age and gender matched control per CHD child were included. Non-singletons and children born with a syndrome were excluded. Exposure was defined as having a CHD and the outcome was defined as needing special education service in the Danish primary and lower secondary school. The population consisted of 7,559 CHD children and 77,046 non-CHD children (controls). CHD children had a higher need for special education compared to non-CHD children, OR: 2.14 (95% CI: 2.00; 2.28), p < 0.001. The odds ratio was also increased when comparing children with a minor CHD to non-CHD children, OR: 1.99 (95% CI: 1.86; 2.14), p < 0.001. CHD children exposed to preeclampsia or smoking had a higher risk of receiving special education compared to unexposed CHD children. We find that school performance is impaired in children born with CHD. This applies to both simple and complex CHD. If a child with CHD was exposed to preeclampsia or maternal smoking this further increased the need for special education.

Abstract 13695: Is There Utility of Fetal Echocardiogram for Isolated in vitro Fertilization Pregnancies?

Introduction: Risk of congenital heart disease (CHD) for in vitro fertilization (IVF) pregnancies is higher than the general population, though other factors may be involved. IVF is an indication for fetal echocardiogram (FE), however there is center variation to perform FE without a secondary indication if the anatomy ultrasound (AU) is normal. We aim to assess the number of new CHD diagnoses following normal AU in IVF-only pregnancies. Hypothesis: We hypothesize that there is minimal benefit to a FE in IVF-only pregnancies with a normal AU and may result in overutilization of resources. Methods: Retrospective chart review from 2016-2021 of all IVF pregnancies with and without a secondary indication for FE at our center. Those without FE during the COVID-19 pandemic were included to assess postnatal CHD detection. Patients were classified as IVF-only if they had a normal AU and no secondary indication for FE; all others classified as IVF+other. Maternal and fetal demographics, AU, FE, and postnatal echo (post-echo) data was obtained. Results: A total of 556 maternal and 628 fetal patients from IVF pregnancies were included; 401 fetuses were IVF-only with a FE, 116 were IVF-only with no FE, the remaining were IVF+other. There was no complex CHD (CCHD) in either IVF-only groups, the FE group detected several minor findings, and the no FE group detected three small septal defects on post-echo (Table 1). The probability of a normal postnatal evaluation in IVF-only with a normal FE was 94% and with no FE was 96%. Minor variations found on FE triggered additional testing (71 total FE in 43 fetuses) and detected a few minor CHD, none requiring intervention. Conclusions: Given low-risk for CCHD in IVF-only pregnancies, there is minimal benefit to a FE in the setting of a normal comprehensive AU and raises questions of cost vs. benefit of FE. This may impact future recommendations for indications for FE in the setting of IVF-only without added risk factors for CHD.

Peripheral Saturation and Perfusion Index on the First Day of Life Play a Role in Early Discharge of Healthy Term Newborns.

Introduction Pulse oximetry screening is a safe, feasible test, effective in identifying congenital heart diseases in otherwise well-appearing newborns. Uncertainties still persist on the most effective algorithm to be used and the timing of screening. The aim of this study was to evaluate the role of the pulse oximetry screening associated with the peripheral perfusion index performed in the first 24 hours of life for the early detection of congenital heart diseases and noncongenital heart diseases in the newborns. Materials and Methods A prospective observational cohort study was conducted. The enrollment criteria were as follows: term newborns with an APGAR score >8 at 5 minutes. The exclusion criteria were as follows: clinical signs of prenatal/perinatal asphyxia or known congenital malformations. Four parameters of pulse oximetry screening were utilized: saturation less than 90% (screening 1), saturation of less than 95% in one or both limbs (screening 2), difference of more than 3% between the limbs (screening 3), and preductal peripheral perfusion index or postductal peripheral perfusion index below 0.70 (screening 4). The likelihood ratio, sensibility, specificity, and positive and negative predictive values for identification of congenital heart diseases or noncongenital heart diseases (suspicion of perinatal infection and any respiratory diseases) were evaluated. Results The best predictive results for minor congenital heart disease were obtained combining screening 3 and screening 4 (χ2 (1) = 15,279; p < 0.05; OR = 57,900 (9,465–354,180)). Screening 2, screening 3, and screening 4 were predictive for noncongenital heart diseases (χ2 (1) = 11,550; p < 0.05; OR = 65,744 (10,413–415,097)). Combined screenings 2–4 were predictive for both congenital heart disease and noncongenital heart disease (χ2 (1) = 22,155; p < 0.05; OR = 117,685 (12,972–1067,648)). Conclusions Combining peripheral saturation with the peripheral perfusion index in the first 24 hours of life shows a predictive role in the detection of minor congenital heart diseases and neonatal clinical conditions whose care needs attention.

Association of Myocardial Muscle Non-Compaction and Multiple Ventricular Septal Defects by Echocardiography.

The aim of this study is to examine the possible high association between multiple ventricular septal defect (mVSDs) and noncompaction cardiomyopathy (NCM) as same embryological origin, and the effect of depressed ventricular function in NCM cases during the follow-up, using echocardiography. A total of 150 patients with mVSDs were diagnosed in a single center in Saudi Arabia; 40 cases with isolated or associated with minor congenital heart disease were recruited. Three specialist echocardiography consultants confirmed the NCM diagnosis separately using Jenni, Chin and Patrick criteria, and myocardial function was estimated by ejection fraction at admission and at follow-up after surgery. Stata-14 to analyze the data was used. In our cohort of 40 cases with mVSD (median age at diagnosis = 0.5 years; mean follow-up = 4.84 years), 13(33%) had criteria of non-compaction confirmed by the three specialist consultants. All were operated by surgery and 11 hybrid approach (interventional & surgery). A significant relationship between abnormal trabeculations and mVSD with or without non-compaction was observed, 34% vs 66% respectively (p < 0.03, Fisher's exact test). A repeated-measures t-test found the difference between follow-up and preoperative ejection-fractions to be statistically significant (t (39) = 2.07, p < 0.04). Further, the myocardial function in the mVSD non-compaction group normalized substantially postoperatively compared with preoperative assessment (mean difference (MD) 11.77, 95% CI: 4.40-19.14), whilst the mVSD group with normal myocardium had no significant change in the myocardium function (MD 0.74, 95% CI: -4.10-5.58). Thus, treatment outcome appears better in the mVSD non-compaction group than their peers with normal myocardium. Acknowledging the lack of genetic data, it is evident the high incidence of non-compaction in this cohort of patients with mVSD and supports our hypothesis of embryonic/genetic link, unlikely to be explained by acquired cardiomyopathy.

Tele-Echocardiography for Congenital Heart Disease Screening in a Level II Neonatal Intensive Care Unit with Hybrid Telemedicine System.

Introduction: The nationwide shortage of pediatric cardiologists in medically underserved areas poses a challenge to congenital heart disease (CHD) screening requiring echocardiography, resulting in transfer of neonates to regional Level III/IV Neonatal Intensive Care Units (NICUs). This study aimed to evaluate the accuracy, safety, and cost-effectiveness of tele-echocardiography for advanced CHD screening at a Level II NICU managed by a hybrid telemedicine system. Methods: Retrospective chart review of infants requiring tele-echocardiography at a Level II NICU. Patient demographics, echocardiography indications, and findings were analyzed. Agreement between tele-echocardiography and conventional echocardiography findings was assessed. Transport cost savings were calculated based on preventable transfers to Level IV NICU. Descriptive statistics were computed for demographic and clinical variables. Results: Over 5 years, 52 infants were screened for CHD. Thirty-two infants (62%) had findings consistent with minor CHD or normal neonatal transitional physiology. Twenty infants (38%) had abnormal findings requiring follow-up with either a conventional echocardiography as inpatient at the regional Level IV NICU or as outpatient after discharge. Only 5 infants (10%) required transfer to a Level IV NICU for CHD management, whereas 15 infants (29%) were scheduled for outpatient follow-up. Strong agreement was noted between tele-echocardiography and conventional echocardiography findings. No case of critical congenital heart disease (CCHD) was missed. Tele-echocardiography saved $260,000 in transport costs. Conclusions: Tele-echocardiography can be accurate, safe, and effective in CHD screening, preventing unnecessary transfer of most infants to regional Level III/IV NICUs, saving transfer costs.

The Association Between Race and Adverse Postoperative Outcomes in Children With Congenital Heart Disease Undergoing Noncardiac Surgery.

The association between race and perioperative outcomes has been evaluated in adult cardiac surgical and in healthy pediatric patients but has not been evaluated in children with congenital heart disease (CHD) presenting for noncardiac procedures. This study compares the incidence of the primary outcome of 30-day mortality and adverse postoperative outcomes following noncardiac surgery between Black and White children with CHD, stratified by severity. This is a retrospective study. Comparison of outcomes between Black and White children was performed using the 2012-2018 American College of Surgeons National Surgical Quality Improvement Program Pediatric database and after stratification for severity of CHD and propensity score matching. A total of 55,859 patients were included, and divided into 28,601 minor, 23,839 major, and 3419 severe CHD. Black and White children in each category were matched and compared. Following matching in the overall CHD cohort, there were significantly higher rates of the following adverse postoperative outcomes among Black patients as compared to White patients: 30-day mortality (1.84% vs 1.49%; odds ratio [OR], 1.25; 95% confidence interval [CI], 1.05-1.48; P = .014), composite secondary outcomes (19.90% vs 17.88%; OR, 1.14; 95% CI, 1.08-1.21; P < .001), cardiac arrest (1.42% vs 0.98%; OR, 1.46; 95% CI, 1.19-1.79; P < .001), 30-day reoperation (7.59% vs 6.67%; OR, 1.15; 95% CI, 1.05-1.25; P = .002), and reintubation (3.9% vs 2.95%; OR, 1.34; 95% CI, 1.19-1.52; P < .001). No significant statistical interaction between race and CHD severity was found. Following matching and within the minor CHD cohort, Black children had significantly higher rates of composite secondary outcome (17.44% vs 15.60%; OR, 1.15; 95% CI, 1.05-1.25; P = .002), cardiac arrest (1.02% vs 0.53%; OR, 1.94; 95% CI, 1.37-2.76; P < .001), 30-day reoperation (7.19% vs 5.77%; OR, 1.26; 95% CI, 1.11-1.43; P < .001), and thromboembolic complications (0.49% vs 0.23%; OR, 2.17; 95% CI, 1.29-3.63; P = .003) compared to White children. In the major CHD cohort, Black children had significantly higher rates of 30-day mortality (2.75% vs 2.05%; OR, 1.35; 95% CI, 1.08-1.69; P = .008) and reintubation (4.82% vs 3.72%; OR, 1.32; 95% CI, 1.11-1.56; P = .002). There were no statistically significant differences in outcomes in the severe CHD category for 30-day mortality (3.36% vs 3.3%; OR, 1.02; 95% CI, 0.60-1.73; P = .946), composite secondary outcome (22.65% vs 21.36%; OR, 1.08; 95% CI, 0.86-1.36; P = .517) nor the components of the composite secondary outcomes. Race is associated with postoperative mortality and complications in children with minor and major CHD undergoing noncardiac surgery. No significant association was observed between race and postoperative outcomes in patients with severe CHD. This is consistent with previous findings wherein in patients with severe CHD, residual lesion burden and functional status is the leading predictor of outcomes following noncardiac surgery. Nevertheless, there is no evidence that the relationship between race and outcomes differs across the CHD severity categories. Future studies to understand the mechanisms leading to the racial difference, including institutional, clinical, and individual factors are needed.

Can we Improve the Perinatal Outcomes and Early Postnatal Survival of Fetuses with Congenital Heart Disease by Initiating Specialized Prenatal Consultation Service

Background: Requirements of specialized prenatal consultation after prenatal diagnosis of Congenital Heart Disease (CHD) is increasing, but its impact is not clear. This study was to introduce such a specialized prenatal consultation service and assess its impact on outcomes of CHD fetuses. Methods: We initiated specialized prenatal consultation in a referral tertiary cardiac center in southern China in 2013. A multidisciplinary team serves as consultants in this service and is especially responsible by two pediatric cardiologists in regular outpatient clinics. We included CHD fetuses diagnosed from 2011 to 2015 consecutively. They were divided into before (2011-2013) and after (2014-2015) specialized prenatal consultation groups and their perinatal outcomes, delivery location and early postnatal survival were compared. Risk factors for termination were also assessed. Results: Totally, 1032 CHD fetuses were included and 533 accepted specialized prenatal consultation. After initiation of specialized prenatal consultation, the live birth rate significantly improved (OR=1.59, 95% CI: 1.10-2.29) and termination rate significantly reduced (OR=0.63, 95% CI: 0.44-0.91) in single CHD fetuses. No significant change in delivery location and postnatal survival of CHD fetuses were observed after specialized prenatal consultation. Referrals from district hospitals, along with multiple-lesion CHD, critical CHDs, gestational age<28 weeks at prenatal diagnosis were risk factors for termination. Conclusion: Specialized prenatal consultation can improve the perinatal outcomes in single CHD fetuses. Education for district hospitals of standardizing counseling, especially for minor CHD fetuses and timely transferring of mothers with critical CHDs/multiple CHDs fetus is crucial to improve the outcomes of CHD fetuses further.

Adverse Perioperative Events in Children with Complex Congenital Heart Disease Undergoing Operative Scoliosis Repair in the Contemporary Era.

Scoliosis is common in children with congenital heart disease (CHD) and may have deleterious effects on quality of life and hemodynamics. Relatively little is known about the outcomes of spinal fusion for scoliosis repair in children with complex CHD. We reviewed all cases of children with CHD undergoing first time spinal fusion excluding those with minor CHD between 1995 and 2015. Seventy-eight patients were identified and included in the study. 97.4% of patients included had undergone prior cardiac surgery and sixteen patients had single ventricle circulations. 17.9% of patients experienced a significant perioperative event defined as an aggregate of the presence of any of the following: need for early unanticipated reoperation, neurologic deficit, postoperative bleeding requiring intervention, end organ dysfunction, or death. There were no deaths in our cohort. 38.5% of patients experienced any adverse event, the majority of which were related to perioperative fluid shifts. Larger preoperative Cobb angle and longer length of spinal fusion were associated with increased risk of significant perioperative event while larger preoperative Cobb angle and longer length of spinal fusion, older age at time of surgery, single ventricle circulation, cyanosis and patients taking cardiac medications at the time of surgery were more likely to experience any adverse event. Operative repair of scoliosis in children with complex CHD has been performed without mortality over a 20-year period in a single institution, albeit with a higher rate of perioperative complication than is seen in the general pediatric population. Patients with large preoperative Cobb angles and cyanotic single ventricle circulations appear to be at the highest risk for perioperative complications.

The Accuracy and Cost-Effectiveness of Selective Fetal Echocardiography for the Diagnosis of Congenital Heart Disease in Patients with Pregestational Diabetes Stratified by Hemoglobin A1c.

To evaluate the accuracy of antenatal diagnosis of congenital heart disease (CHD) using screening methods including a combination of elevated hemoglobin A1c, detailed anatomy ultrasound, and fetal echocardiography. This is a retrospective cohort study of all pregnancies complicated by pregestational diabetes from January 2012 to December 2016. The sensitivity, specificity, positive predictive value, and negative predictive value were calculated for each screening regimen. The incremental cost-effectiveness ratio (ICER) was calculated for each regimen with effectiveness defined as additional CHD diagnosed. A total of 378 patients met inclusion criteria with an overall prevalence of CHD of 4.0% (n = 15). When compared with a detailed ultrasound, fetal echocardiography had a higher sensitivity (73.3 vs. 40.0%). However, all cases of major CHD were detected by detailed ultrasound (n = 6). Using an elevated early A1c > 7.7% and a detailed ultrasound resulted in a sensitivity and specificity of 60.0 and 99.4%, respectively. The use of selective fetal echocardiography for an A1c > 7.7% or abnormal detailed anatomy ultrasound would result in a 63.3% reduction in cost per each additional minor CHD diagnosed (ICER: $18,290.52 vs. $28,875.67). Fetal echocardiography appears to have limited diagnostic value in women with pregestational diabetes. However, these results may not be generalizable outside of a high-volume academic setting.

Population-based study of congenital heart disease and revisits after pediatric tonsillectomy.

ObjectiveAccurate assessment of risk factors such as congenital heart disease (CHD) can aid in risk stratification of children presenting for surgery. Risk stratification is especially important in tonsillectomy ± adenoidectomy (T/A), a common pediatric procedure that is usually performed electively, but that has a high rate of adverse events. In this study, we examined the association of CHD with revisits after T/A.MethodsWe identified children who underwent T/A at hospitals and hospital‐owned facilities during 2010 to 2014 using the State Inpatient Databases and State Ambulatory Surgery and Services Databases of Florida, Georgia, Iowa, New York, and Utah. We evaluated the association between CHD severity and the occurrence of an unplanned hospital readmission or ED visit within 30 days following discharge using multivariable logistic regression.ResultsThe analysis included 244,598 patients, of whom 858 had minor or major CHD. In multivariable analysis, CHD was not associated with an increased risk of 30‐day revisits (minor OR = 1.1; 95% CI: 0.8, 1.5; P = .65; major OR = 1.2; 95% CI: 0.9, 1.6; P = .34). Other comorbidities, including chromosomal anomalies (OR = 1.4; 95% CI: 1.2, 1.6; P < .001), congenital airway anomalies (OR = 1.3; 95% CI: 1.03, 1.7; P = .03), and neuromuscular impairment (OR = 1.4; 95% CI: 1.2, 1.7; P < .001) predicted an increased likelihood of revisits.ConclusionNeither minor nor major CHD was independently associated with an increased risk of 30‐day revisits among children undergoing T/A. Other characteristics, particularly non‐cardiac comorbidities, socioeconomic status, and geographic region may be of greater utility for predicting revisit risk following pediatric T/A.Level of Evidence2b

Incidence and Risk Factors for Perioperative Cardiovascular and Respiratory Adverse Events in Pediatric Patients With Congenital Heart Disease Undergoing Noncardiac Procedures.

While mortality and adverse perioperative events after noncardiac surgery in children with a broad range of congenital cardiac lesions have been investigated using large multiinstitutional databases, to date single-center studies addressing adverse outcomes in children with congenital heart disease (CHD) undergoing noncardiac surgery have only included small numbers of patients with significant heart disease. The primary objective of this study was to determine the incidences of perioperative cardiovascular and respiratory events in a large cohort of patients from a single institution with a broad range of congenital cardiac lesions undergoing noncardiac procedures and to determine risk factors for these events. We identified 3010 CHD patients presenting for noncardiac procedures in our institution over a 5-year period. We collected demographic information, including procedure performed, cardiac diagnosis, ventricular function as assessed by echocardiogram within 6 months of the procedure, and classification of CHD into 3 groups (minor, major, or severe CHD) based on residual lesion burden and cardiovascular functional status. Characteristics related to conduct of anesthesia care were also collected. The primary outcome variables for our analysis were the incidences of intraoperative cardiovascular and respiratory events. Univariable and multivariable logistic regressions were used to determine risk factors for these 2 outcomes. The incidence of cardiovascular events was 11.5% and of respiratory events was 4.7%. Univariate analysis and multivariable analysis demonstrated that American Society of Anesthesiologists (≥3), emergency cases, major and severe CHD, single-ventricle physiology, ventricular dysfunction, orthopedic surgery, general surgery, neurosurgery, and pulmonary procedures were associated with perioperative cardiovascular events. Respiratory events were associated with American Society of Anesthesiologists (≥4) and otolaryngology, gastrointestinal, general surgery, and maxillofacial procedures. Intraoperative cardiovascular events and respiratory events in patients with CHD were relatively common. While cardiovascular events were highly associated with cardiovascular status, respiratory events were not associated with cardiovascular status.

Estimating Surgical Risk in Younger and Older Children With Congenital Heart Disease

BackgroundAs management of congenital heart disease (CHD) improves, children with CHD increasingly present for noncardiac surgery. Prior studies report conflicting results on the association between CHD and adverse outcomes in noncardiac surgery. Studies reporting no such association predominantly analyze older children and adolescents. We evaluated whether the association between CHD and adverse surgical outcomes was attenuated by increased age. MethodsPatients aged 0-17 y, undergoing elective noncardiac surgery, were identified in the National Surgical Quality Improvement Program-Pediatric registry. CHD status was classified as none, minor, major, or severe. Multivariable logistic regression evaluated the association of CHD status with the occurrence of any postoperative complication for groups defined by quintiles of patient age (<1, 1-3, 4-8, 9-13, and 14-17 y). Specific complications included cardiac arrest, reintubation, infection, renal failure, neurological complication, thromboembolic complication, reoperation, 30-d unplanned revisit, 30-d prolonged hospital stay, and mortality. ResultsThe analysis included 131,164 children, of whom 6420 had minor CHD, 3825 had major CHD, and 963 had severe CHD. The overall rate of complications was 9%. In multivariable analysis, children <1 y old had greater risk of postoperative complications if they had minor (odds ratio [OR] = 1.97; 95% confidence interval [CI]: 1.70, 2.20; P < 0.001), major (OR = 2.58; 95% CI: 2.28, 2.91; P < 0.001), or severe CHD (OR = 4.37; 95% CI: 3.45, 5.54; P < 0.001). In older age groups, however, the presence of CHD was not independently associated with postoperative complications. ConclusionsIn pediatric noncardiac surgery, an independent association of CHD with postoperative complications was only evident among children <1 y old.

Outcomes of laparoscopic and open surgery in children with and without congenital heart disease

BackgroundChildren with congenital heart disease (CHD) often require noncardiac surgery. We compared outcomes following open and laparoscopic intraabdominal surgery among children with and without CHD. MethodsWe performed a retrospective cohort study using the 2013–2015 National Surgical Quality Improvement Project-Pediatrics. We matched 45,012 children <18years old who underwent laparoscopic surgery to 45,012 children who underwent open surgery. We determined the associations between laparoscopic (versus open) surgery and 30-day mortality, in-hospital mortality, 30-day morbidity, and postoperative length-of-stay. ResultsAmong children with minor CHD, laparoscopic surgery was associated with lower 30-day mortality (Odds Ratio [OR] 0.34 [95% Confidence Interval 0.15–0.79]), inhospital mortality (OR 0.42 [0.22–0.81]) and 30-day morbidity (OR 0.61 [0.50–0.73]). As CHD severity increased, this benefit of laparoscopic surgery decreased for 30-day morbidity (ptrend=0.01) and in-hospital mortality (ptrend=0.05), but not for 30-day mortality (ptrend=0.27). Length-of-stay was shorter for laparoscopic approaches for children at cost of higher readmissions. On subgroup analysis, laparoscopy was associated with lower odds of postoperative blood transfusion in all children. ConclusionsIntraabdominal laparoscopic surgery compared to open surgery is associated with decreased morbidity in patients with no CHD and lower morbidity and mortality in patients with minor CHD, but not in those with more severe CHD. Level-of-evidenceLevel III: Treatment Study.

Persistently elevated nuchal translucency and the fetal heart

Objective: To describe the outcome of fifteen cases with an elevated nuchal translucency (NT) which persisted into the second trimester as nuchal edema (NE) >6 mm whom underwent fetal echocardiography. Materials and methods: Cases were identified following retrospective review of cardiac and genetic findings in fetuses with NE. Results: Minor congenital heart disease was identified in 3/15 by the second trimester. Agenesis of the ductus venosus was evident in four. Pulmonary valve stenosis was diagnosed in one fetus at the 20-week scan and hypertrophic cardiomyopathy in one. However, hypertrophic cardiomyopathy or pulmonary valve stenosis was present after birth in all surviving cases by 3 months of age. On the basis of intention to treat, 11/12 survived to delivery and 9/12 survived to 28 days. There were 6 deaths before 14 months of age as a result of severe hypertrophic cardiomyopathy. Noonan syndrome was confirmed with genetic testing in 11/15 cases. Conclusions: All fetuses with NT and NE had evidence of congenital heart disease at birth, and therefore, late gestation and postnatal review is recommended even when second trimester echocardiogram is considered normal. There is a high prevalence of Noonan syndrome and targeted genetic analysis should be considered. The outcome in these cases is poor.

Mortality and Morbidity after Laparoscopic Surgery in Children with and without Congenital Heart Disease

To determine the risk of morbidity and mortality after laparoscopic surgery among children with congenital heart disease (CHD). Cohort study using the 2013-2014 National Surgical Quality Improvement Program-Pediatrics, which prospectively collected data at 56 and 64 hospitals in 2013 and 2014, respectively. Primary exposure was CHD. Primary outcome was overall in-hospital postoperative mortality. Secondary outcomes included 30-day mortality and 30-day morbidity (any nondeath adverse event). Among 34?543 children who underwent laparoscopic surgery, 1349, 1106, and 266 had minor, major, and severe CHD, respectively. After propensity score matching within each stratum of CHD severity, morbidity and mortality were compared between children with and without CHD. Children with severe CHD had higher overall mortality and 30-day morbidity (OR 12.31, 95% CI 1.59-95.01; OR 2.51, 95% CI 1.57-4.01, respectively), compared with matched controls. Overall mortality and 30-day morbidity were also higher among children with major CHD compared with children without CHD (OR 3.46, 95% CI 1.49-8.06; OR 2.07, 95% CI 1.65-2.61, respectively). Children with minor CHD had similar mortality outcomes, but had higher 30-day morbidity compared with children without CHD (OR 1.71, 95% CI 1.37-2.13). Children with major or severe CHD have higher morbidity and mortality after laparoscopic surgery. Clinicians should consider the increased risks of laparoscopic surgery for these children during medical decision making.

Post-Operative Outcomes in Children With and Without Congenital Heart Disease Undergoing Noncardiac Surgery

BackgroundSignificant advances have been made in the diagnosis and treatment of children with congenital heart disease (CHD), allowing for longer life expectancies and an increasing number who will require noncardiac surgery. ObjectivesThis study sought to compare the incidence of mortality and major adverse post-operative outcomes following noncardiac surgery in children with and without CHD. MethodsData from the 2012 pediatric database of the American College of Surgeons National Surgical Quality Improvement Program were analyzed. After propensity score matching, and stratification by severity of CHD, mortality and adverse post-operative outcomes were compared between controls and children with CHD. ResultsAmong the 51,008 children included in the database, 4,520 children with CHD underwent noncardiac surgery. After propensity score matching, we included 2,805 children with minor CHD, 1,272 with major CHD, and 417 with severe CHD. Children in each subgroup were matched and compared with controls without CHD who underwent noncardiac surgery of comparable complexity. The incidence of overall mortality was significantly higher in children with moderate (3.9%) and severe (8.2%) CHD compared with their controls (respectively, 1.7% [p < 0.001] and 1.2% [p = 0.001]). Both 30-day and overall mortality were significantly increased in children with severe CHD (odds ratio [OR]: 8.43, 95% confidence interval [CI]: 2.52 to 28.21; p < 0.001; OR: 7.32, 95% CI: 2.83 to 18.90; p < 0.001) compared with their matched controls. Overall mortality was also significantly increased in children with major CHD compared with their controls (OR: 2.28; 95% CI: 1.37 to 3.79; p = 0.002), whereas no difference was observed between children with minor CHD and their matched controls. ConclusionsChildren with major and severe CHD, undergoing noncardiac surgery, have an increased risk of mortality compared with children without CHD. Further studies need to identify the optimal environment for surgical procedures, develop trained multidisciplinary teams to care for children with CHD, and define management strategies for improving outcomes in this high-risk population.

Calcified Pericarditis Associated with Ventricular Septal Defect: Should we close the Hole or Open Our Eyes?

We present the case of a 45-year-old woman with recent onset of dipnea and atrial fibrillation. She was referred to our center for treatment of an associated ventricular septal defect. Echocardiography confirmed the presence of a membranous ventricular septal defect, but also showed signs of constriction. Cardiac computed tomography displayed a severe, diffuse calcified pericarditis. We show that, in adult patients, a minor congenital heart disease can catalyze the clinician’s attention and, like in this case, mask major signs of acquired heart disease.