Nationwide hospitalizations of patients with down syndrome and congenital heart disease over a 15-year period.

Down syndrome is one of the most common genetic diseases, generally associated with an increased probability of congenital heart diseases. This increased risk contributes to escalated levels of morbidity and mortality. In this study, we sought to analyze nationwide data of pediatric and adult patients with Down syndrome and congenital heart disease over a 15-year period. Data obtained from the hospital discharge form between 2001 and 2016 of patients diagnosed with Down syndrome in Italy and at least one congenital heart disease were included. Information on 12362 admissions of 6527 patients were included. Age at first admission was 6.2 ± 12.8 years and was a predictor of mortality (HR = 1.51, 95% CI 1.13-2.03, p = 0.006). 3923 (60.1%) patients underwent only one admission, while 2604 (39.9%) underwent multiple (> 1) admissions. There were 5846 (47.3%) admissions for cardiac related symptoms. Multiple admissions (SHR: 3.13; 95% CI: 2.99, 3.27; P < 0.01) and cardiac admissions (SHR: 2.00; 95% CI: 1.92, 2.09; P < 0.01) were associated with an increased risk of additional potential readmissions. There was an increased risk of mortality for patients who had cardiac admissions (HR = 1.45, 95% CI: 1.08-1.94, p = 0.012), and for those who underwent at least 1 cardiac surgical procedure (HR = 1.51, 95% CI 1.13-2.03, p = 0.006). CONCLUSIONS: A younger age at first admission is a predictor for mortality in patients with Down syndrome and congenital heart disease. If patients undergo more than one admission, the risk of further readmissions increases. There is a pivotal role for heart disease in influencing the hospitalization rate and subsequent mortality. WHAT IS KNOWN: • Down syndrome individuals often face an increased risk of congenital heart diseases. • Congenital heart diseases contribute significantly to morbidity and mortality in Down syndrome patients. WHAT IS NEW: • This study analyzes nationwide data covering a 15-year period of pediatric and adult patients in Italy with Down syndrome and congenital heart disease. • It identifies a younger age at first admission as a predictor for mortality in these patients, emphasizing the criticality of early intervention. • Demonstrates a correlation between multiple admissions, particularly those related to cardiac issues, and an increased risk of further readmissions, providing insights into the ongoing healthcare needs of these individuals.

Enhancing parental understanding of congenital heart disease through personalized prenatal counseling with 3D printed hearts.

OBJECTIVES: In addition to a correct prenatal diagnosis of congenital heart disease (CHD), comprehensive parental counseling is crucial to ensure that parents are well-informed about the condition of the fetus. This study aims to investigate whether there is a significant difference in the information acquired by parents through traditional counseling, utilizing 2-dimensional (2D) illustrations and images, compared to an advanced approach utilizing personalized three-dimensional (3D) printed models of the fetal heart developed from 3D ultrasound imaging. METHODS: This study, designed as a pilot randomized control trial, enrolled pregnant women with gestational ages greater than 18 weeks, whose fetuses were diagnosed with CHD and referred to our center between November, 2020 and June, 2021. Two groups of patients were included in the study. The first group received standard medical counseling with 2D images and illustrations, while the second group underwent advanced counseling with 3D-printed patient-specific heart models. Both groups were then required to complete the same survey in which the knowledge of the CHD was investigated. The 3D models were created from 3D ultrasound imaging and printed using resin materials in both 1:1 and 5:1 scale. RESULTS: A comparison of the scores obtained from the two groups revealed that 3D visualization of the fetus's heart has the potential to increase parental knowledge about CHD and the required surgical procedures. Furthermore, all couples expressed interest in receiving a 1:1 scale model of their baby's heart. CONCLUSION: Personalized prenatal counseling with 3D-ultrasound-based heart models positively impacts parents' understanding of CHD. The use of 3D models provides a more comprehensive and accessible representation of the condition, contributing to an increased knowledge gain, and potentially helping to support informed decisions regarding their child's care.

Outcomes of Neonatal Cardiac Surgery: a European Congenital Heart Surgeons Association Study.

BACKGROUND: We evaluated outcomes of neonatal cardiac surgery at hospitals affiliated with the European Congenital Heart Surgeons Association (ECHSA). METHODS: All patients ≤30 days of life undergoing a cardiac surgical procedure during a 10 year period between January 2013 and December 2022 were selected from the ECHSA Congenital Database. Reoperations during the same hospitalization, percutaneous procedures, and noncardiac surgical procedures were excluded. A total of 12 benchmark operations were identified. Primary outcomes were 30-day mortality and in-hospital mortality. Multivariable logistic regression analysis was performed to determine independent factors associated with higher mortality. A comparison of mortality between the first 5 years and second 5 years was also performed. RESULTS: The overall number of neonatal operations from 2013-2022 was 30,931, and 22,763 patients met the inclusion criteria of the study. The four most common procedures were: arterial switch operation (3,520/22,763=15.5%), aortic coarctation repair (3,204/22,763=14.1%), shunt procedure (2,351/22,763=10.3%), and Norwood Operation (2,115/22,763=9.23%). 30-day mortality rate was: overall population (1,342/22,763=5.9%), arterial switch (110/3,520=3.13%), Norwood Operation (339/2,115=16.0%), and hybrid operation (94/609=15.4%). In-hospital mortality rate was: overall population (2,074/22,763=9.1%), arterial switch (145/3,520=4.12%), Norwood Operation (523/2,115=24.7%), and hybrid operation (186/609=30.5%). Multivariable analysis revealed that major factors impacting mortality were high risk procedures (adjusted odds ratio=2.74 [95% confidence interval=2.33-3.23]; p<0.001), and the need for extracorporeal membrane oxygenation (11.8 [9.9-14]; p <0.001). CONCLUSIONS: Neonatal cardiac surgery continues to pose a significant challenge, with notable mortality, particularly for neonates with functionally univentricular physiology. These data can serve as important benchmarks across Europe and offer insights regarding opportunities for improvement.

Extrapleural closure of patent Ductus Arteriosus: how we do it

Abstract Patent ductus arteriosus (PDA) is a clinical condition mostly found in premature newborns. Among several medical, surgical and interventional treatment options, extrapleural ligation through a left minithoracotomy is recognized as a safe, efficient and less expensive technique. In fact, it requires short surgical times, grants good exposure of the duct and nearby structures (e.g., thoracic duct, left recurrent laryngeal nerve), and avoids pleural space opening and subsequent pulmonary complications in preterm patients. This approach seems ideal due to its lower costs, especially in developing countries with a high birth rate and limited resources.