Inpatient hospitalizations in adults with congenital heart disease and down syndrome: A national perspective.

Adult Congenital Heart Surgery: Adult or Pediatric Facility? Adult or Pediatric Surgeon?

Objective - The research was undertaken with the aim of establishing the range and frequency of congenital heart disease (CHD) in children with Down syndrome (DS), who are monitored at paediatric cardiology centres in Bosnia and Herzegovina, its association with other anomalies, the time of diagnosis of DS and CHD, and to analyse their surgical care. Patients and methods - By a cross-sectional study, data was collected on children aged from 0 to 18 years with DS and CHD who were monitored at paediatric cardiology centres of primary, secondary and tertiary health care (Banja Luka, Bihać, Bijeljina, Mostar, Sarajevo, Tuzla and Zenica) in Bosnia and Herzegovina. Results - Data for 100 children (51 boys and 49 girls) of an average age of 6.1 (from 0 to 17.1) were collected and analysed. The most frequently diagnosed CHD was atrioventricular septal defect, which was found in 36% of children followed by ventricular septal defect in 33%. In 79% children the CHD was isolated and 21% had multiple heart anomalies. Associated anomalies of other systems were found in 26% of children. In only one case was an intrauterine suspicion of DS established. A statistically significant reduction in the age of post-natal diagnosis of DS and CNHD was registered. In 73% patients cardiosurgical treatment was indicated, 43% underwent surgery, 19% are waiting for surgery and in 11% pulmonary hypertension developed. Conclusion - The range of CHD found in children with DS in Bosnia and Herzegovina does not differ from most similar research. The results obtained show that in the period in question there was insufficient prenatal diagnostics of DS and CHD. Postnatal diagnostics, although also insufficient at the beginning of the period in question, significantly improved over time, which resulted in a reduction of the age at which the diagnosis of DS and CHD was established. Moreover there was a clear reduction in the time difference between the diagnosis of DS and the diagnosis of CHD. However, the possibilities of early cardio-surgical treatment are still limited. The results should serve in drawing up a plan of prospective monitoring of these patients in Bosnia and Herzegovina in order to define better their specific health needs.

Hospitalizations and Mortality in the United States for Adults With Down Syndrome and Congenital Heart Disease

Isolated heart transplant and combined heart-liver transplant in adult congenital heart disease patients: Insights from the united network of organ sharing

While ventricular assist devices (VADs) remain the cornerstone of mechanical circulatory support (MCS), the total artificial heart (TAH-t) has gained popularity for certain patients in whom VAD support is not ideal. Congenital heart disease (CHD) patients often have barriers to VAD placement due to anatomic and physiological variation and thus can benefit from the TAH-t. The purpose of this study is to analyze the differences in TAH application and outcomes in patients with and without CHD. The SynCardia Department of Clinical Research provided data upon request for all TAH-t implantations worldwide from December 1985 to October 2019. These patients were divided into two groups by pre-implantation diagnosis of CHD and non-CHD. A total of 1,876 patients were identified. Eighty (4%) of these patients also carried a diagnosis of CHD. There was a higher proportion of children in the CHD cohort (16.3% vs. 2.1%, P<0.001) and this translated into a lower average age amongst the two groups (34±13 vs. 49±13 years, P<0.001). There were also significantly more females in the CHD group (22.8% vs. 12.8%, P=0.010). CHD patients were more likely to be supported with a 50 cc TAH-t (11.3% vs. 4.5%, P=0.005) while all other support characteristics, including duration of support, were similar between the groups. All measured outcomes were similar between CHD and non-CHD patients including positive outcome (alive on device or transplanted), 1-month conditional survival, and rate of Freedom Driver use. TAH-t is an effective means to support patients with CHD. Patients with CHD had similar survival, support characteristics, and frequency of discharge compared to patients without CHD. As MCS continues to grow, its indications broadened, and its contraindications narrowed, more patient populations will see the benefit of the TAH's continuously developing technology.

We analyzed the impact of the revised pediatric heart allocation policy on types of ventricular assist device (VAD) utilization, and waitlist (WL) and post-heart transplant (HT) survival outcomes in congenital heart disease (CHD) versus non-CHD patients before (Era-1) and after (Era-2) pediatric heart allocation policy implementation. We retrospectively reviewed the UNOS database from December 16, 2011, through March 31, 2021, for patients<18 years old and listed for primary HT. We compared the differences observed between Era-1 and Era-2. 5551 patients were listed for HT, of whom 2447(44%) were in Era-1 and 3104(56%) were in Era-2. CHD patients were listed as status 1A unchanged, but the number of patients listed as status 1B decreased in Era-2, whereas the number of non-CHD patients listed as status 1A decreased, but status 1B increased. In Era-2 compared to Era-1, both temporary (1% to 4%, p<.001) and durable VAD (13.6% to 17.8%, p<.001) utilization increased, and the transplantation rate per 100-patient years increased in both groups. The median WL period for CHD patients increased marginally from 70 to 71 days (p=.06), whereas for non-CHD patients it decreased from 61 to 54 days (p<.001). Adjusted 90-day WL survival increased from 84% to 88%, p=.016 in CHD, but there was no significant change in non-CHD patients (p=.57). There was no significant difference in 1-year post-HT survival in CHD and non-CHD patients between Era-1 and Era-2. In summary, after the revised heart allocation policy implementation, temporary and durable VAD support increased, HT rate increased, waitlist duration marginally increased in the CHD cohort and decreased in the non-CHD cohort, and 90-day WL survival probability improved in children with CHD without significant change in 1-year post-HT outcomes. Future studies are needed to identify changes to the policy that may further improve the listing criteria to improve WL duration and post-HT survival.

The number of congenital heart disease (CHD) patients with heart failure is expanding. These patients have a high probability of dying while awaiting heart transplant. The potential for durable ventricular assist devices (VAD) to improve waiting list survival in CHD is unknown. We conducted an analysis of the Scientific Registry of Transplant Recipients database for the primary outcome of death or delisting due to clinical worsening while listed for heart transplant. We compared CHD patients with non-CHD patients matched for listing status. Multivariable models were constructed to account for confounding variables. Congenital heart disease patients were less likely to have a VAD and were more likely to experience the primary outcome of death or delisting due to clinical worsening compared to non-CHD patients. Ventricular assist devices decreased the probability of experiencing the primary outcome for non-CHD but not for CHD patients with a final listing status of 1A. Ventricular assist devices increased the probability of experiencing the primary outcome among CHD patients for those with a final listing status of 1B with no impact in non-CHD patients. Among non-CHD patients who died or were delisted, the time to the primary outcome was delayed by VAD, with a similar trend in CHD. Except for patients with a final listing status of 1B, VAD does not adversely affect waiting list outcomes in CHD patients listed for heart transplant. Ventricular assist devices may prolong waiting list survival among high-risk CHD patients.

Impact of SARS-CoV-2 Vaccine Rollout on Hispanic and Non-Hispanic Admission and Mortality Trends: An Interrupted Time Series Analysis

Exacerbations requiring hospital admission for chronic obstructive pulmonary disease (COPD) contribute to a decline in health status and are costly to the community. Long-term trends in admissions and associated outcomes are difficult to establish because of frequent readmissions, high case fatality and potential diagnostic transfer between COPD and asthma. The Western Australian Data Linkage System provides a unique opportunity to examine admissions for patients with COPD over the long term. Nineteen years of hospital morbidity data, based on International Classification of Diseases-9 criteria were extracted from the Western Australian Data Linkage System (1980-1998) and merged with mortality records to examine trends in hospital admissions for COPD. The rate of hospital admissions for COPD has declined overall and the rate of first presentation declined in men and remained constant in women. The risk of readmission increased throughout the period (P < 0.0001) and more than half of all admissions were followed by readmission within a year. Median survival following first admission was 6 years (men 5 years; women 8 years). Age, sex and International Classification of Diseases subcategory each showed an independent effect on the risk of mortality (P < 0.0001). The poorest survival was in patients subcategorized as emphysema. For patients with multiple admissions, the likelihood of cross-over between COPD and asthma was high and increased with the total number of admissions. The rate of admission for COPD has declined in Western Australia; however, the resource burden will continue to increase because of the ageing population. This has policy implications for the development of acute care treatment programmes for COPD.

The current study aims to identify the rates of lapses in care and loss to follow-up before age one through age five for white and nonwhite congenital heart disease (CHD) survivors. Nonwhite CHD survivors were hypothesized to experience an earlier lapse in care and be lost to follow-up than whites. Patients were from a large pediatric hospital and had (1) at least one outpatient cardiology clinic visit or cardiac surgery visit before the age of one and (2) a diagnosis of moderate or complex structural CHD. Cardiology outpatient utilization rates were tracked from before age one through age five. Lapse in follow-up was defined as not having at least one outpatient cardiology visit per year, and loss to follow-up was not returning after a lapse in care by age five. Race was categorized as white and nonwhite. Covariates included sex, insurance type, noncardiology inpatient and outpatient hospital utilization, and CHD severity. The sample included 1034 patients. Overall, 75.7% experienced a lapse in care with only 41.6% of those returning by age five. Nonwhites experienced lapses in care at younger ages than whites. Nonwhites had a 53% increased risk of lapse in care. Medicaid patients and those with moderate CHD diagnoses also had an increased risk for lapse in care. Lapse in care appears prevalent among CHD survivors by age five, with nonwhites demonstrating elevated risk. Future multisite prospective studies should include the assessment of parental knowledge, barriers to accessing care, and satisfaction with care.

This study aimed to describe and evaluate the type, frequency and patterns of congenital heart diseases (CHDs) in patients with Down Syndrome (DS) in Sétif, Algeria. Down Syndrome, or trisomy 21, is the most common genetic disorder in the world. Data were collected and followed from January 2009 to December 2013. Parental consanguinity documenting pedigree analyzing, chromosome analysis and clinical examination were carried out for all cases. Results have shown that 22 (15.4%; ± 0.06) of the total 143 known cases of DS from DS centres have CHDs and 88 (10.6%; ± 2.2) of the total 770 patients with CHDs collected from public departments at the child and maternity teaching hospital, Sétif, have DS. Among the 110 cases, 75 (68%) have single cardiac abnormalities and 35 (32%) have multiple cardiac abnormalities. The most frequent CHDs were Atrioventricular Septal Defect (AVSD). In conclusion, our study will be helpful to demonstrate the current status of DS and to identify the distribution of CHD in patients with DS in Sétif, Algeria, for further study.

Congenital heart disease (CHD) survivors are at risk for neurodevelopmental disability (NDD), and recent studies identify genes associated with both disorders, suggesting that NDD in CHD survivors may be of genetic origin. Genes contributing to neurogenesis, dendritic development and synaptogenesis organize neural elements into networks known as the connectome. We hypothesized that NDD in CHD may be attributable to genes altering both neural connectivity and cardiac patterning. To assess the contribution of de novo variants (DNVs) in connectome genes, we annotated 229 published NDD genes for connectome status and analyzed data from 3,684 CHD subjects and 1,789 controls for connectome gene mutations. CHD cases had more protein truncating and deleterious missense DNVs among connectome genes compared to controls (OR = 5.08, 95%CI:2.81–9.20, Fisher’s exact test P = 6.30E-11). When removing three known syndromic CHD genes, the findings remained significant (OR = 3.69, 95%CI:2.02–6.73, Fisher’s exact test P = 1.06E-06). In CHD subjects, the top 12 NDD genes with damaging DNVs that met statistical significance after Bonferroni correction (PTPN11, CHD7, CHD4, KMT2A, NOTCH1, ADNP, SMAD2, KDM5B, NSD2, FOXP1, MED13L, DYRK1A; one-tailed binomial test P ≤ 4.08E-05) contributed to the connectome. These data suggest that NDD in CHD patients may be attributable to genes that alter both cardiac patterning and the connectome.

Risk of ischemic stroke in adult patients with congenital heart disease

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Perioperative Echocardiography During the Coronavirus Crisis: Considerations in Pediatrics and Congenital Heart Disease