ORCHID (Outcome Registry for CHIldren with severe congenital heart Disease) a Swiss, nationwide, prospective, population-based, neurodevelopmental paediatric patient registry: framework, regulations and implementation

Introduction: Congenital heart disease (CHD) is the most frequent birth defect. As survival has significantly improved, attention has turned to neurodevelopmental outcomes of children undergoing heart surgery in early infancy. Since multiple risk factors contribute to neurodevelopmental alterations, a nationwide registry collecting data on medical characteristics, interventions, clinical course and neurodevelopment until school-age is needed to improve the quality of management, identify risk- and protective factors affecting neurodevelopment, and facilitate multicentre trials. Methods and analysis: The Swiss Outcome Registry for CHIldren with severe congenital heart Disease (ORCHID) is a nationwide, prospective, population-based patient registry developed (1) to collect baseline characteristics and clinical data of CHD patients operated with bypass-surgery or hybrid procedures in the first 6 weeks of life in Switzerland, (2) to monitor long-term neurodevelopment, and (3) to relate clinical characteristics and neurodevelopment to identify risk and protective factors in these children. This registry started data collection relating to pregnancy, birth, preoperative course, catheter-based and surgical treatment, postoperative course and reinterventions in 2019. The primary outcome includes standardised neurodevelopmental assessments at 9 to 12 months, 18 to 24 months and 5.5 to 6 years. We expect to include 80 to 100 children per year. Correlation and regression analyses will be used to investigate risk- and protective factors influencing neurodevelopment. Ethics and dissemination of results: Swiss ORCHID received support by the Accentus Charitable Foundation, the Anna Mueller Grocholoski Stiftung, the Swiss Society of Paediatric Cardiology, the Verein Kinderherzforschung, and the Corelina - Stiftung für das Kinderherz, and was approved by the cantonal ethics committees. Findings will be presented at national and international scientific meetings, and published in peer-reviewed journals. Results will also be shared with patient organizations, primary health care providers, and public health stakeholders to ensure a widespread dissemination of the results

Impact of postoperative necrotizing enterocolitis after neonatal cardiac surgery on neurodevelopmental outcome at 1 year of age.

OBJECTIVES We analyzed the impact of postoperative necrotizing enterocolitis (NEC) after cardiac surgery in neonatal age on neurodevelopmental (ND) outcome at 1 year of age. METHODS Using data from the Swiss Neurodevelopmental Outcome Registry for Children with Congenital Heart Disease (ORCHID), we analyzed perioperative variables including postoperative NEC (Bell's stage ≥2) and 1-year ND outcome (Bayley III). RESULTS The included patients (n = 101) had congenital heart disease (CHD), categorized as follows: 77 underwent biventricular repair for CHD with two functional chambers, 22 underwent staged palliation until the Fontan procedure for CHD with single ventricle physiology (n = 22), or 4 underwent single ventricle palliation or biventricular repair for borderline CHD (n = 4). Neonatal cardiopulmonary bypass (CBP) surgery was performed at a median age (IQR) of 8 (6) days. NEC occurred in 16 patients. Intensive care unit (ICU) length of stay (LOS) and the total duration of the hospitalization were longer in children with NEC than those in others (14 with vs. 8 days without NEC, p < 0.05; 49 with vs. 32 days without NEC, p < 0.05). The Bayley III scores of the analyzed patients determined at an age of 11.5 ± 1.5 months showed cognitive (CCS) (102.2 ± 15.0) and language scores (LCS) (93.8 ± 13.1) in the normal range and motor composite scores (MCS) (88.7 ± 15.9) in the low-normal range. After adjusting for socioeconomic status and CHD type, patients with NEC had lower CCS scores [β = -11.2 (SE 5.6), p = 0.049]. Using a cumulative risk score including NEC, we found a higher risk score to be associated with both lower CCS [β = -2.8 (SE 1.3), p = 0.030] and lower MCS [β = -3.20 (SE 1.3), p = 0.016]. CONCLUSIONS Postoperative NEC is associated with longer ICU and hospital LOS and contributes together with other complications to impaired ND outcome at 1 year of age. In the future, national and international patient registries may provide the opportunity to analyze large cohorts and better identify the impact of modifiable perioperative risk factors on ND outcome. CLINICAL TRIAL REGISTRATION ClinicalTrials.gov identifier: NCT05996211

Contribution of imaging modalities to eosinophilic myocarditis diagnosis: a case report

Background: Eosinophilic myocarditis (EM) is a relatively rare form of myocarditis that could progress to restrictive cardiomyopathy and might be fatal if left untreated. Although myocardial biopsy is considered to be the gold standard for the diagnosis of myocarditis, its use in paediatrics remains controversial and not easily applicable in routine practice. Case summary: A 10-year-old girl with no prior medical history presented to the emergency department for fever, odynophagia, and gastrointestinal symptoms despite 48 h of antibiotics (Cefaclor). Physical examination revealed diffuse petechiae and abdominal tenderness but was otherwise unremarkable. Her vital signs were normal. She was found to have hypereosinophilia and increased cardiac markers on laboratory testing. Echocardiography showed diffuse left ventricular (LV) myocardial infiltrates, moderate LV dilatation, and mild systolic dysfunction. Bone marrow biopsy confirmed B cell acute lymphoblastic leukaemia. The diagnosis of EM was made. High doses of steroids and chemotherapy were initiated. Cardiac magnetic resonance imaging (MRI) identified eosinophilic infiltrates and sub-endocardial enhancement strongly suggestive of EM. Left ventricular function was slightly decreased. Intra-ventricular micro-thrombi were suspected, and warfarin was started. The outcome was favourable. Leucocyte and eosinophil counts were normalized within a month. At 6 months, cardiac MRI demonstrated a significant decrease in eosinophilic infiltration and micro-thrombi, normalization of LV function, and of sub-endocardial enhancement. Discussion: This case demonstrates that non-invasive multi-modality imaging along with typical laboratory and clinical findings allow for appropriate diagnosis of EM while avoiding biopsy. It also highlights that an early diagnosis, timely treatment, and rigorous follow-up improve disease progression and outcome.</p

Reliable Detection of Intrapulmonary Shunts Using Contrast Enhanced Echocardiography in Children with Portal Hypertension or Portosystemic Shunt

Objectives: The aim of this study was to analyze if contrast-enhanced echocardiography (CEE) is as reliable as lung perfusion scintigraphy (LPS) to detect intrapulmonary shunting (IPS) in children with portal hypertension (PHTN) or congenital/surgical portosystemic shunts (PSS) and to define the number of cardiac cycles required to exclude intrapulmonary shunting. Methods: Inclusion criteria for this cross-sectional study were: (1) presence of PHTN or PSS diagnosed on abdominal ultrasound, (2) technically valid saline contrast echocardiography, (3) lung perfusion scintigraphy within 6 months of CEE. The number of cardiac cycles between right atrial opacification and the arrival of contrast in the left atrium were counted. We analyzed our CEE data at three and five cardiac cycles and compared them with LPS results. Results: The study population was composed of 78 children (38 girls, 49%) ages 2.1-18.8 years (mean 9.8). Sixty-nine patients had PHTN (88%), and nine had a PSS (11%). Eleven subjects (14%) presented evidence of IPS on LPS. Peripheral oxygen saturation was lower in the subjects with IPS detected on LPS (95.3 ± 1.7% vs 99.0 ± 1.4%; P &lt; 0.01). Comparison of LPS with CEE before three and five cardiac cycles showed that CEE is highly specific (95.7%) as early as three cardiac cycles with markedly better sensitivity (72.7%) when using five cardiac cycles. Furthermore, a negative study using five cardiac cycles ruled out IPS with a 95% negative predictive value. The cardiac cycle at which the bubbles appeared in the left atrium was inversely correlated to the shunt index measured using LPS (r = -0.563; P = 0.001). Conclusion: CEE is sufficient for the screening of IPS in children with PHTN or congenital/surgical PSS, obviating the need for LPS.</p

Early and Late Postoperative Tachyarrhythmias in Children and Young Adults Undergoing Congenital Heart Disease Surgery

The population of patients with congenital heart disease is constantly growing with an increasing number of individuals reaching adulthood. A significant proportion of these children and young adults will suffer from tachyarrhythmias due to the abnormal anatomy, the hemodynamic burden, or as a sequela of surgical treatment. Depending on the underlying mechanism, arrhythmias may arise in the early postoperative period (hours to days after surgery) or in the late postoperative period (usually years after surgery). A good understanding of the electrophysiological characteristics and pathophysiological mechanisms is therefore crucial to guide the therapeutic approach. Here, we synthesize the current state of knowledge on epidemiological features, risk factors, pathophysiological insights, electrophysiological features, and therapy regarding tachyarrhythmias in children and young adults undergoing reparative surgery for congenital heart disease. The evolution and latest data on treatment options, including pharmacological therapy, ablation procedures, device therapy decision, and thromboprophylaxis, are summarized. Finally, throughout this comprehensive review, knowledge gaps and areas for future research are also identified

ORCHID (Outcome Registry for CHIldren with severe congenital heart Disease) a Swiss, nationwide, prospective, population-based, neurodevelopmental paediatric patient registry: framework, regulations and implementation

Introduction: Congenital heart disease (CHD) is the most frequent birth defect. As survival has significantly improved, attention has turned to neurodevelopmental outcomes of children undergoing heart surgery in early infancy. Since multiple risk factors contribute to neurodevelopmental alterations, a nationwide registry collecting data on medical characteristics, interventions, clinical course and neurodevelopment until school-age is needed to improve the quality of management, identify risk- and protective factors affecting neurodevelopment, and facilitate multicentre trials. Methods and analysis: The Swiss Outcome Registry for CHIldren with severe congenital heart Disease (ORCHID) is a nationwide, prospective, population-based patient registry developed (1) to collect baseline characteristics and clinical data of CHD patients operated with bypass-surgery or hybrid procedures in the first 6 weeks of life in Switzerland, (2) to monitor long-term neurodevelopment, and (3) to relate clinical characteristics and neurodevelopment to identify risk and protective factors in these children. This registry started data collection relating to pregnancy, birth, preoperative course, catheter-based and surgical treatment, postoperative course and reinterventions in 2019. The primary outcome includes standardised neurodevelopmental assessments at 9 to 12 months, 18 to 24 months and 5.5 to 6 years. We expect to include 80 to 100 children per year. Correlation and regression analyses will be used to investigate risk- and protective factors influencing neurodevelopment. Ethics and dissemination of results: Swiss ORCHID received support by the Accentus Charitable Foundation, the Anna Mueller Grocholoski Stiftung, the Swiss Society of Paediatric Cardiology, the Verein Kinderherzforschung, and the Corelina - Stiftung für das Kinderherz, and was approved by the cantonal ethics committees. Findings will be presented at national and international scientific meetings, and published in peer-reviewed journals. Results will also be shared with patient organizations, primary health care providers, and public health stakeholders to ensure a widespread dissemination of the results.</p