[Neonatal screening for critical congenital heart diseases in Peru: an urgent call]. / Tamizaje neonatal de cardiopatías congénitas críticas en el Perú: un llamado de urgencia.

Congenital heart diseases are the most common congenital malformations worldwide and represent one of the leading causes of neonatal death, in addition to the significant use of human and financial resources by health systems. The purpose of this document is to support the implementation of neonatal screening for critical congenital heart diseases using pulse oximetry according to the different geographical altitudes of Peru. This technology is widely used worldwide and has high sensitivity, specificity, and cost-effectiveness. At many latitudes, it has led to better survival in this group of patients and in the neonatal population in general since its use in the early detection of sepsis, pneumonia, and other conditions that affect the oxygenation of the newborn. Neonatal screening for critical congenital heart disease is applicable at all levels of healthcare at a national level, and its implementation must be a priority to improve neonatal health.

Factors affecting the level of parental knowledge about their children's congenital heart diseases and their care.

OBJECTIVE: To assess parental knowledge about their children's congenital heart disease using a newly-designed scale. METHODS: The cross-sectional study was conducted from June 2020 to May 2021, at Alkhansaa maternity and child hospital, Mosul Iraq; after approval from the ethics review committee of Ninevah Medical College, Ninevah University, Iraq, and comprised parents with children having congenital heart disease. Data, including age, gender, type of congenital heart disease as well as parents' educational and socioeconomic levels, residence, internet use and knowledge of the disease with respect to type, burden, bacterial endocarditis, outcome, etc. was noted. Parental knowledge levels were measured using a self-designed scoring scale, having a total score of 18 and 4 categories; <6 = some knowledge, 7-12 = good knowledge, 13-17 = very good knowledge, and 18 = full knowledge. Data was analysed using SPSS 26. RESULTS: There were 364 children with mean age 83.72±47.4 months and a male-female ratio of 0.98. The mean age at diagnosis was 12.1±8.32 months. The overall mean parental knowledge level was 8.41± 3.88, with 126(34.6%) parents having some knowledge, 178(48.9%) having good, 58(16%) having very good, and 2(0.5%) having full knowledge about their children's health condition (p<0.001) There was a significant association of parental knowledge with educational level, socioeconomic status and the type of doctor caring for the child (p<0.05). The duration of the illness showed a significant correlation with knowledge level (p=0.021). CONCLUSIONS: Heart Defects, Congenital, Endocarditis, Bacterial, Social Class.

Assessment of neonatal glycaemic status and comorbidities in infants of diabetic mothers admitted to the neonatal care unit of Al-Ramadi Teaching Hospital for maternity and childhood, Iraq.

OBJECTIVE: To assess neonatal and maternal characteristics, glycaemic status and comorbidities in the neonates of diabetic mothers. METHODS: The cross-sectional study was conducted from October 2021 to May 2022 at the Department of Physiology, College of Medicine, University of Mustansiriyah, Baghdad, Iraq, and comprised healthy women. Samples were raised by simple random technique. Digital pulse waves were captured using a fingertip pulse wave transducer. Lab Chart Pro version 7.2 was used to automatically detect and quantify the amplitude of A, B, C, D and E waves expressed by the second derivative. QT interval of each beat was recorded by electrocardiogram, and was calculated automatically via Lab chart Pro version 7.2 without averaging. Data was spread out on Microsoft Office Excel 2013 and analysed using SPSS version 26. RESULTS: Among the 70 mothers, aged between 18 to 44 years, gestational diabetes was the commonest type 52(74.3%), and, among the 70 neonates, 52(74.3%) developed mild hypoglycaemia, 12(17.1%) hypocalcaemia, 26(37.1%) congenital heart disease, 50(71.4%) respiratory distress syndrome, 24(34.3%) hyperbilirubinaemia, 2(2.9%) congenital anomalies, 6(8.6%) prematurity, and 4(5.7%) developed birth asphyxia. Prematurity, female gender and low birthweight were significantly associated with hypoglycaemia (p<0.05). No significant differences were detected in terms of neonatal complications between pregestational and gestational diabetic mothers (p>0.05). CONCLUSIONS: Diabetic pregnancies were linked to a higher risk of neonatal complications.

The impact of COVID-19 vaccination on patients with congenital heart disease in England: a case-control study.

BACKGROUND: Studies predating widespread COVID-19 vaccination identified patients with congenital heart disease (CHD) as a group at increased risk of severe outcomes from COVID-19. Here we evaluate the impact of vaccination on COVID-19 outcomes among patients with CHD. METHODS: We conducted a case-control study using linked English electronic health records (n=3 18 135). Patients with CHD were matched with controls by age, sex, ethnicity and GP practice. The 'prevaccination' cohort comprised unvaccinated patients with CHD and matched controls with first-recorded SARS-CoV-2 infection between 1 March and 8 December 2020 (7805 cases, 27 620 controls). The 'post-vaccination' cohort comprised vaccinated patients with CHD and matched controls with first-recorded SARS-CoV-2 infection between 1 March 2021 and 1 April 2022, at least 14 days after vaccination (57 550 cases, 225 160 controls). Odds of severe COVID-19 outcomes were compared using conditional logistic regression. We also compared the rate at which vaccine efficacy diminished, and the incidence of vaccine-associated complications. RESULTS: Compared with the prevaccination cohort, postvaccination patients with CHD exhibited markedly reduced rates of COVID-19-related hospitalisation (0.5% vs 15.8%) and mortality rates (0.5% vs 4.6%). Compared with vaccinated controls, vaccinated patients with CHD remained at increased risk of hospitalisation (0.5% vs 0.2%, adjusted OR 2.24 (1.88-2.65); p<0.001) and death (0.5% vs 0.3%, adjusted OR 1.81 (1.54-2.13); p<0.001). There was no evidence that vaccine efficacy declined faster in patients with CHD, or that patients with CHD experienced a larger increase in incidence of myocarditis, pericarditis or thrombotic events. CONCLUSION: We observed a lower absolute risk of hospitalisation and death from COVID-19 in CHD patients after vaccination. However, in vaccinated CHD patients, an elevated risk of severe outcomes persists compared with vaccinated people without CHD. These results emphasise the importance of vaccination in the CHD population, and of vigilance among care providers dealing with COVID-19 infection in CHD patients, even if fully vaccinated.

Influence of procedural timing on the preventive yield of percutaneous patent foramen ovale closure.

BACKGROUND: The benefit of patent foramen ovale closure (PFOC) ≤9 months after a cryptogenic stroke has been demonstrated in several randomised clinical trials. There is, however, insufficient data to support PFOC in non-recent cryptogenic strokes. AIMS: The objective of the study was to evaluate the effectiveness of PFOC in relation to the time since the patient's most recent cryptogenic cerebrovascular event (CVE) or systemic embolism (SE). METHODS: We conducted a multicentre, retrospective cohort study with international participation, to assess the results of an early closure (EC, <9 months) for secondary prevention versus a delayed closure (DC, ≥9 months). Recurrence of CVE/SE following PFOC was evaluated as the primary endpoint. RESULTS: 496 patients were included (65% in the EC and 35% in the DC group). With the exception of a larger defect size in the DC group (tunnel width 6 (4-14) vs 12 (6-16) mm, p=0.005), similar clinical and echocardiographic baseline features were observed between the groups. No differences were observed regarding the type of devices used for PFOC, procedural success rate (99.4 in EC vs 98.8% DC group) and periprocedural complications (2.1% vs 0.8%). Median follow-up was 2.0 (1.2-4.2) years in the whole study population. Recurrence of CVE/SE (3.9% vs 2.6%, p=0.443), death (1.4% vs 1.0%, p=0.697), residual shunt 12 months after PFOC, or antithrombotic treatment strategy were comparable in both groups during follow-up. A subanalysis comparing very delayed PFOC (≥24 months) also showed no differences in recurrence (4.2% in the <24-month vs 3.4% in the ≥24-month group, p=0.770). CONCLUSION: Patients undergoing PFOC before and after 9 months after the index event had a comparable recurrence rate of CVE/SE. These findings suggest that PFOC might be recommended in cryptogenic CVE/SE which are more remote than 9 months.

Predictors of spontaneous pregnancy loss in single ventricle physiology.

BACKGROUND: Pregnant patients with single ventricle (SV) physiology carry a high risk of spontaneous pregnancy loss (SPL), yet the clinical factors contributing to this risk are not well defined. METHODS: Single-centre retrospective study of pregnant patients with SV physiology seen in cardio-obstetrics clinic over the past 20 years with chart review of their obstetric history. Patients without a known pregnancy outcome were excluded. Univariable Bayesian panel-data random effects logit was used to model the risk of SPL. RESULTS: The study included 20 patients with 44 pregnancies, 20 live births, 21 SPL and 3 elective abortions. All had Fontan palliation except for two with Waterston and Glenn shunts. 10 (50%) had a single right ventricle (RV). 14 (70%) had moderate or severe atrioventricular valve regurgitation (AVVR). Atrial arrhythmias were present in 16 (80%), Fontan-associated liver disease (FALD) in 15 (75%) and FALD stage 4 in 9 (45%). 12 (60%) were on anticoagulation. Average first-trimester oxygen saturation was 93.8% for live births and 90.8% for SPL. The following factors were associated with higher odds of SPL: RV morphology (OR 1.72 (95% credible interval (CrI) 1.0008-2.70)), moderate or severe AVVR (OR 1.64 (95% CrI 1.003-2.71)) and reduced first-trimester oxygen saturation (OR 1.83 (95% CrI 1.03-2.71) for each per cent decrease in O2 saturation. CONCLUSION: Pregnant patients with SV physiology, particularly those with RV morphology, moderate or severe AVVR, and lower first-trimester oxygen saturations, have a higher risk of SPL. Identifying these clinical risk factors can guide preconception counselling by the cardio-obstetrics team.

Trisomy 21 and Congenital Heart Disease: Impact on Health and Functional Outcomes From Birth Through Adolescence: A Scientific Statement From the American Heart Association.

Due to improvements in recognition and management of their multisystem disease, the long-term survival of infants, children, and adolescents with trisomy 21 and congenital heart disease now matches children with congenital heart disease and no genetic condition in many scenarios. Although this improved survival is a triumph, individuals with trisomy 21 and congenital heart disease have unique and complex care needs in the domains of physical, developmental, and psychosocial health, which affect functional status and quality of life. Pulmonary hypertension and single ventricle heart disease are 2 known cardiovascular conditions that reduce life expectancy in individuals with trisomy 21. Multisystem involvement with respiratory, endocrine, gastrointestinal, hematological, neurological, and sensory systems can interact with cardiovascular health concerns to amplify adverse effects. Neurodevelopmental, psychological, and functional challenges can also affect quality of life. A highly coordinated interdisciplinary care team model, or medical home, can help address these complex and interactive conditions from infancy through the transition to adult care settings. The purpose of this Scientific Statement is to identify ongoing cardiovascular and multisystem, developmental, and psychosocial health concerns for children with trisomy 21 and congenital heart disease from birth through adolescence and to provide a framework for monitoring and management to optimize quality of life and functional status.

Circulating High Mobility Group Box-1 Does Not Predict Pulmonary Arterial Hypertension in Children with Congenital Heart Disease: A Prospective Cohort Study.

OBJECTIVES: Pulmonary arterial hypertension (PAH) is a devastating complication of pediatric congenital heart disease (CHD). A recent study has identified the protein high mobility group box-1 (HMGB1) as a diagnostic tool in adults with CHD-associated PAH. HMGB1 levels in adults with CHD-associated PAH correlated with mean pulmonary artery pressure and pulmonary vascular resistance, and HGMB1 levels fell in response to sildenafil therapy. We wanted to assess if HGMB1 was a biomarker of pediatric CHD-PAH. DESIGN: Prospective cohort study. SETTING: Quaternary pediatric academic hospital PARTICIPANTS: Children ≤18 years with CHD with and without known pulmonary hypertension. Controls were children undergoing dental or urologic surgery with no known heart disease. INTERVENTIONS: Pulmonary hemodynamics, echocardiographic assessment, and biomarker measurement. Controls had biomarker measurement only. MEASUREMENTS AND MAIN RESULTS: Patients with CHD-PAH had mean pulmonary vascular resistance index of 10 Wood units/m2. Neither HGMB1 nor N-terminal pro-brain-type natriuretic peptide levels were significantly different between the groups. Neither marker correlated with pulmonary hypertension. CONCLUSIONS: Unlike in adults, HGMB1 is not a biomarker of PAH in pediatric CHD. Further work will continue to explore for biomarkers for this high-risk population.

Prognostic value of cardiopulmonary exercise test in children with congenital heart defects.

BACKGROUND: Cardiopulmonary exercise testing (CPET) has an important prognostic value in adults with different congenital heart defects (CHDs) and is a useful tool for risk stratification and clinical decision-making. In this retrospective study, we studied the prognostic value of CPET in paediatric patients with CHD. METHODS: 411 CPET performed by paediatric patients with different CHDs were evaluated in this retrospective study. Medical records were reviewed to determine the presence of cardiac events. Participants were classified using the 2018 AHA/ACC guideline for the management of adults with CHD that combines anatomical complexity and current physiological stage. RESULTS: 411 patients with a median age at test of 12 years, 51 patients with simple CHD, 170 patients with moderate complexity CHD and 190 with high complexity CHD underwent CPET. Overall, CPET parameters were lower than the reference values (%predicted VO2peak=75% and %predicted oxygen uptake efficiency slope (OUES)=79%), showing worst exercise capacity in the most complex types of CHD (Group III: %predicted VO2peak=72% and %predicted OUES=75%). Seventy-one patients presented with cardiac events at a median time from CPET to first event of 28 months. Patients with cardiac events had lower exercise performance as compared with patients without cardiac events as determined by the submaximal variables (%predicted OUES: HR=2.6 (1.5-4.4), p<0.001 and VE/VCO2: HR=2.2 (1.4-3.5), p=0.001). CONCLUSION: Reduced exercise capacity at young age is related to a higher probability of future cardiovascular events in paediatric patients with CHD. Submaximal exercise variables can be used instead when maximal exercise cannot be achieved.

The Fontan Circulation in Pregnancy: Hemodynamic Challenges and Anesthetic Considerations.

Pregnancy in patients with Fontan physiology presents unique challenges due to altered cardiovascular dynamics inherent to both conditions. The Fontan procedure reroutes venous blood directly to the pulmonary arteries, bypassing the heart, and necessitating precise regulation of pulmonary venous resistance and systemic venous pressure to maintain effective cardiac output. The significant cardiovascular adaptations required during pregnancy to meet the metabolic demands of the mother and fetus can overburden the limited preload capacity and venous compliance in Fontan patients, predisposing them to a spectrum of potential complications, including arrhythmias, heart failure, thromboembolism, and obstetric and fetal risks. This review delineates the essential physiological adaptations during pregnancy and the challenges faced by Fontan patients, advocating for a comprehensive care approach involving multidisciplinary collaboration, vigilant monitoring, tailored anesthetic management, and postpartum care. Understanding the complex dynamics between Fontan physiology and pregnancy is crucial for anesthesiologists to develop and execute individualized management strategies to minimize risks and optimize outcomes for this high-risk population.

A Pediatric Cardiology Outreach Clinic in a Local Hospital: An Experience Review from South A'Sharqiyah, Oman.

Objectives: Specialized pediatric cardiology clinics conducted in local hospitals are an important part of delivering specialized care to patients close to their homes. This study aimed to review our experience with a specialized pediatric cardiology outreach clinic at Jaalan Bani Bu Ali Hospital, South A'Sharqiyah, Oman. Methods: Patient records for each individual, seen in the outreach clinic between March 2018 and June 2022, were reviewed to determine demographic information, reason for referral, underlying diagnosis, and clinic visit outcomes. Results: Over the study period, 29 clinics were conducted, with 360 patients seen. Of these, 200 (55.6%) were male with a median age of 13 months. The majority of patients (n = 271; 75.3%) were referred due to a cardiac murmur. Most patients had a normal cardiac evaluation (n = 177; 49.2%). The most common congenital heart diseases detected were mild pulmonary valve stenosis (14.8%) and moderate to large secundum atrial septal defects (13.7%). Significant cardiac lesions detected included severe pulmonary hypertension (2.2%), tetralogy of Fallot (1.6%), and cor triatriatum sinistrum (0.5%). Overall, 70 (19.4%) patients were referred to tertiary care hospitals, and 179 (49.7%) were reassured and discharged. Conclusions: Conducting specialized pediatric cardiology outreach clinics in overpopulated areas is effective and well-received by families. It reassures many families and reduces the need for unnecessary travel to specialized centers. These clinics also play a crucial role in detecting patients with significant cardiac defects requiring urgent care. Implementing specialized clinics in primary and secondary health centers could be beneficial for other subspecialties in reducing long waiting lists.

Iron deficiency in patients with a Fontan circulation and its impact on exercise capacity.

BACKGROUND: Iron deficiency (ID) has been reported in patients with congenital heart disease. There is, however, a scarcity of data on its prevalence in patients with a Fontan circulation. The aim of this study is to investigate the prevalence of ID in Fontan patients and to investigate the association between ID and exercise capacity in this population. METHODS AND RESULTS: Blood count and haematological parameters were determined in plasma of 61 Fontan patients (51% female, mean age 29±9 years). ID was defined as transferrin saturation (TSAT) ≤19.8%. The prevalence of ID was 36% (22/61 patients). Especially among women, the diagnosis of ID was highly prevalent (52%) despite normal haemoglobin levels (153.7±18.4 g/L). Mean ferritin levels were 98±80 µg/L and mean TSAT levels were 22%±12%. Cardiopulmonary exercise testing was performed in 46 patients (75%). Patients with ID had a lower peak oxygen uptake (V̇O2peak) (1397±477 vs 1692±530 mL/min; p=0.039), although this relationship was confounded by sex. The presence of ID increased the likelihood of not achieving a respiratory exchange ratio (RER) ≥1.1 by 5-fold (p=0.035). CONCLUSION: ID is highly prevalent among patients with a Fontan circulation. V̇O2peak is lower in patients with ID. Fontan patients with ID are less likely to achieve an RER≥1.1 during cardiopulmonary exercise testing.

[Angioplasty with ductal stent: experience at Garrahan Hospital]. / Angioplastia con stent ductal: experiencia en el Hospital Garrahan.

Objective: To describe and evaluate the outcomes of ductal angioplasty with stent placement at a single high-complexity center during the period 2016-2022. Method: A retrospective descriptive cross-sectional study was conducted, including patients under 3 months of age who underwent ductal stent implantation as initial palliative treatment. Demographic, clinical, and anatomical data were collected before the intervention. Mortality, intra- and post-procedural complications, need for re-intervention, intensive care requirements, and hospital stay were recorded. The characteristics at the time of definitive surgery are described. Discrete variables are presented as percentages, and continuous variables are presented with their medians and respective interquartile ranges. Results: Twenty patients who underwent this treatment were reviewed, revealing a success rate of 80%. Complications due to stent dysfunction required surgical resolution. 95% of patients were dischargedfrom the institution after the procedure, and 17 patients reached a second definitive surgical stage. Three patients died afterthe procedure, but with no direct relation to it. Conclusions: Indications for ductal angioplasty with stent as an alternative treatment to systemic-pulmonary anastomosis by surgery are not yet fully defined; the strategy represents a valid alternative in appropriately selected patients. The presented experience shows results similar to international reference centers.

Objetivo: Describir y evaluar los resultados de la angioplastia con stent ductal en un único centro de alta complejidad durante el periodo 2016-2022. Método: Estudio descriptivo retrospectivo de corte transversal en el que se incluyeron pacientes menores de 3 meses a quienes se implantó un stent en el conducto arterioso como tratamiento paliativo inicial. Se recolectaron datos demográficos, clínicos y anatómicos previos a la intervención. Se registraron la mortalidad, las complicaciones intra- y posprocedimiento, la necesidad de reintervención, los requerimientos de cuidados intensivos y la estadía hospitalaria. Se describen las características al momento de la cirugía definitiva. Las variables discretas son presentadas con porcentajes, y las variables continuas con sus medianas y sus respectivos intervalos intercuartílicos. Resultados: Se revisaron 20 pacientes que habían recibido este tratamiento y se evidenció una tasa de éxito del 80%. Las complicaciones por disfunción del stent requirieron resolución quirúrgica. Egresaron de la institución el 95% de los pacientes luego del procedimiento y lograron arribar a un segundo estadio quirúrgico definitivo 17 pacientes. Fallecieron tres pacientes luego del procedimiento, pero sin relación directa con este. Conclusiones: Las indicaciones de angioplastia del conducto con stent como tratamiento alternativo a la realización de una anastomosis sistémico-pulmonar por cirugía todavía no están completamente definidas; la estrategia constituye una alternativa válida en pacientes adecuadamente seleccionados. La experiencia presentada muestra resultados similares a los de otros centros de referencia internacional.

Non-cardiac birth defects and long-term risk of cardiovascular hospitalisation.

BACKGROUND: Patients with heart defects are at risk of developing cardiovascular disease. Our objective was to determine if non-cardiac birth defects are associated with the risk of cardiovascular hospitalisation. METHODS: We conducted a longitudinal cohort study of 1 451 409 parous women in Quebec, Canada. We compared patients with cardiac and non-cardiac birth defects of the urinary, central nervous and other systems against patients without defects between 1989 and 2022. The main outcome was hospitalisation for coronary artery disease, ischaemic stroke and other cardiovascular outcomes during 33 years of follow-up. We computed cardiovascular hospitalisation rates and used Cox proportional hazards regression models to measure the association (HR; 95% CI) between non-cardiac defects and later risk of cardiovascular hospitalisation, adjusted for patient characteristics. RESULTS: Women with any birth defect had a higher rate of cardiovascular hospitalisation than women without defects (7.0 vs 3.3 per 1000 person-years). Non-cardiac defects overall were associated with 1.61 times the risk of cardiovascular hospitalisation over time, compared with no defect (95% CI 1.56 to 1.66). Isolated urinary (HR 3.93, 95% CI 3.65 to 4.23), central nervous system (HR 3.33, 95% CI 2.94 to 3.76) and digestive defects (HR 2.39, 95% CI 2.16 to 2.65) were associated with the greatest risk of cardiovascular hospitalisation. These anomalies were associated with cardiovascular hospitalisation whether they presented alone or clustered with other defects. Nevertheless, heart defects were associated with the greatest risk of cardiovascular hospitalisation (HR 10.30, 95% CI 9.86 to 10.75). CONCLUSION: The findings suggest that both cardiac and non-cardiac birth defects are associated with an increased risk of developing cardiovascular disease among parous women.