Accurate bedside assessment of the ratio of pulmonary to systemic flow (Qp/Qs ratio, referred to as “the flow ratio” or “the ratio”) plays an important role in the management of many congenital heart defects, especially the complexes unified by univentricular atrioventricular connections. Arterial oxygen saturation can be a misleading measure of the ratio, and may not reflect derangements until they are quite large. Theoretical analysis suggests that systemic venous oxygenation may be a better indicator of the ratio. To examine this, we created a widely patent atrial septal defect in neonatal piglets (weight =4–6.5 kg, n=6). Snares aruond the aorta and pulmonary trunk were adjusted to alter the flow ratio from 0.1 to 6.5. Venous oxygen saturations, measured in the mid-inferior caval vein, were at a maximum at a ratio about 1, and declined rapidly with increases or decreases in the ratio beyond a limited range. The venous oxygen saturation was found to vary much more than arterial oxygen saturation, with arterial oxygen saturation only falling when the ratio dropped below 0.5. Oxygen delivery (Oxygen Content x Cardiacoutput) was found to parallel closely systemic venous oxygen saturation, and was at a maximum at the same ratio that produced a maximum value of systemic venous oxygen saturation. The study suggests that systemic venous oxygen saturation provides a better estimate than does systemic arterial oxygen saturationof the flow ratio and oxygen delivery. Interventions that maximize systemic venous oxygen saturation should maximize oxygen delivery, and determination of systemic venous oxygen saturation should be a helpful addition in managing children with a number of congenital heart defects.

We report a patient with Down's syndrome referred to our Department of Echocardiography for evaluation after an episode of tachyarrhythmia. We diagnosed Ebstein's malformation. As far as we know, this association has been reported previously on only three occasions. We review these previous cases, describe our echocardiographic techniques, and discuss our experience.

A term infant rapidly developed profound cyanosis and metabolic acidosis shortly after an uncomplicated vaginal delivery. Echocardiography identified a flail antero-superior leaflet of the tricuspid valve, which was producing severe tricuspid insufficiency. The clinical state deteriorated despite maximal medical management, and the patient was placed on venoarterial extracorporeal membrane oxygenation. Within twenty-four hours, the metabolic acidosis corrected, inotropic support was discontinued, and the patient was weaned to minimal ventilator settings. Successful repair of the tricuspid valve was performed two days later.

Despite improvement in the early outcome for patients undergoing complete repair of atrioventricular septal defects with a common atrioventricular orifice, the incidence of reoperation for failure of the repair of the left atrioventricular valve remains high. The surgical techniques employed in our most recent experience, including routine suture of the septal commissure and liberal use of valvar annuloplasty, were successful in achieving a very satisfactory competence of left atrioventricular valve with a iow incidence of reoperation for failure of the repair.

We report two cases of hemopericardium occurring in seven-month-old and 12-year-old boys, who had no history of major trauma. The possible cause of the hemopericardium for the infant was falling from a bed which was 75 cm high two weeks prior to the admission. The 12-year-old boy had fallen from a chair and damaged his chest 4 weeks previously. Their coagulation tests were all normal. By means of pericardiotomy, we drained 120 ml and 1200 ml of blood, respectively. The boys have now been well over follow-up periods of 24 and 18 months, respectively

Medication non-adherence causes poor outcomes in paediatric organ transplantation. COVID-19 pandemic has led to an exponential use of mobile health approaches for patient care. Herein, we describe a pilot intervention study using mobile video directly observed therapy building on emerging trends in research and clinical practice pertaining to medication adherence in paediatric organ transplantation.

Although closure of ductus arteriosus requires multiple intriguing cell signalling pathways, current treatment options merely act through cyclooxygenase inhibition. Expectedly, medical management of ductus arteriosus is quite far away from optimum and there is an ongoing debate on its efficiency and safety. The ideal therapeutic agent for patent ductus arteriosus should target vascular remodelling and support the arrested molecular steps that should completely eventuate during anatomical closure.

Platelet-rich plasma injection, which has been used as a treatment alternative for many musculoskeletal conditions, can be a promising solution in this context by compensating the relative platelet dysfunction in preterms and providing a convenient microenvironment for proper cellular communication for ductal closure.

First, by inducing hyaluronan synthase, platelet-rich plasma can induce mount formation in the intima, which is the significant defective milestone in preterms. Second, by providing platelet-derived growth factor, it can further stimulate platelet agregation and occlusion of the lumen. Finally, it can provide an increment in local vascular endothelial growth factor and transforming growth factor-β levels, which cannot be achieved because of insufficient intramural hypoxia. Migration of the vascular smooth muscles would further be triggered by transforming growth factor-β and vascular endothelial growth factor, aiding a major contribution to ductal closure.

Above and beyond all other considerations, opportunity of local application would render maximum effectiveness and minimum side effects.

The implementation committee of the Quebec Child Telehealth Network was formed in 1997, with a mandate to build a network dedicated to the diagnosis of congenital cardiac disease via telemedicine. We devised criterions for selection to determine which peripheral centres would be linked by telemedicine to the university-based services for paediatric cardiology provided in the Canadian Province of Quebec. The criterions included: distance from a university centre, number of births per year, and presence of an already-established outreach clinic for paediatric cardiology. The Quebec Network became operational in 2000, and was composed of 32 peripheral centres and 4 university centres. A total of 363 transmissions of echocardiograms occurred over a 3-year period from January 2000 to December 2002. Peripheral centres located at a distance greater than 100 kilometres from a university centre were 8.5 times more likely to use the network. Criterions other than distance did not influence whether or not a peripheral centre used the network. Cardiac abnormalities were identified in almost two-thirds of the transmissions. The use of the Quebec Network resulted in the avoidance of transfers or clinic visits to university hospitals in seven-tenths of cases. We conclude that distance greater than 100 kilometres from a centre offering subspecialty services in paediatric cardiology is the most important criterion for choosing the peripheral centres that are most likely to use a telehealth network. In its first three years of operation, the telehealth network had a major impact on the delivery of paediatric cardiac care, improving access to subspecialty services across the province.

Neurodevelopmental and psychosocial impairments negatively impact health-related quality of life for survivors with CHD and complicate the transition to independent adulthood. Risk for neurodevelopmental and psychosocial impairments is influenced by a complex interplay among genetic, foetal, surgical, perioperative, family, and social factors, requiring a multi-pronged approach to neuroprotection and intervention. To ensure future research can ultimately reduce the burden of CHD for individuals, families, and society, the most pressing issues in cardiac neurodevelopment requiring scientific investigation must be identified.

Through funding from an R13 Grant from the National Heart, Lung, and Blood Institute of the National Institutes of Health of the United States of America, the Cardiac Neurodevelopmental Outcome Collaborative convened a two-day meeting of international experts in cardiac neurodevelopmental and psychosocial research, clinical care, and health disparities, including patient and family stakeholders, to define the cardiac neurodevelopmental and psychosocial outcomes research agenda for the next decade. Seven multidisciplinary working groups were formed to address key domains crucial to the advancement of cardiac neurodevelopmental and psychosocial outcomes research: 1) Foetal Brain Development and Neuroprotection, 2) Surgical/Perioperative Neuroprotection and Neurodevelopment, 3) Characterization of Neurodevelopmental and Psychological Outcomes, 4) Neurodevelopmental and Psychosocial Intervention, 5) Parent Mental Health and Family Functioning, 6) Neurodevelopmental Education, Outreach and Advocacy, and 7) Health Disparities and Neurodevelopmental Outcomes. Working groups identified significant gaps in knowledge and critical questions that must be answered to further knowledge, policy, care, and outcomes. The development of a research agenda in cardiac neurodevelopmental and psychosocial outcomes is critical for informing collaborative initiatives and allocation of funding for research to scientific inquiries of highest value to key stakeholders.

The Eighth World Congress of Pediatric Cardiology and Cardiac Surgery (WCPCCS) will be held in Washington DC, USA, from Saturday, 26 August, 2023 to Friday, 1 September, 2023, inclusive. The Eighth World Congress of Pediatric Cardiology and Cardiac Surgery will be the largest and most comprehensive scientific meeting dedicated to paediatric and congenital cardiac care ever held. At the time of the writing of this manuscript, The Eighth World Congress of Pediatric Cardiology and Cardiac Surgery has 5,037 registered attendees (and rising) from 117 countries, a truly diverse and international faculty of over 925 individuals from 89 countries, over 2,000 individual abstracts and poster presenters from 101 countries, and a Best Abstract Competition featuring 153 oral abstracts from 34 countries. For information about the Eighth World Congress of Pediatric Cardiology and Cardiac Surgery, please visit the following website: [www.WCPCCS2023.org]. The purpose of this manuscript is to review the activities related to global health and advocacy that will occur at the Eighth World Congress of Pediatric Cardiology and Cardiac Surgery.

Acknowledging the need for urgent change, we wanted to take the opportunity to bring a common voice to the global community and issue the Washington DC WCPCCS Call to Action on Addressing the Global Burden of Pediatric and Congenital Heart Diseases. A copy of this Washington DC WCPCCS Call to Action is provided in the Appendix of this manuscript. This Washington DC WCPCCS Call to Action is an initiative aimed at increasing awareness of the global burden, promoting the development of sustainable care systems, and improving access to high quality and equitable healthcare for children with heart disease as well as adults with congenital heart disease worldwide.

Milrinone is a drug frequently used for hemodynamic support in children during critical illness. Although the hemodynamic changes induced by milrinone in children may appear similar to those of adults, the physiologic contributors of these changes remain vastly unknown. A systematic review was conducted to identify studies characterising the hemodynamic effects of milrinone in children during critical illness for hemodynamic support for various medical conditions. Studies were assessed for quality and those of satisfactory quality with pre- and post-operative hemodynamics for each patient were included in the final analyses. Those not limited to children and those not limited to patients with critical illness were excluded from the final analyses. A total of six studies with 791 patients were included in the final analyses. Milrinone infusion doses ranged from 0.3 to 0.75 mcg/kg/minute with the mean infusion dose being 0.5 mcg/kg/minute. Patients whom received milrinone infusion had greater cardiac output, greater left ventricle shortening fraction, lower right ventricular systolic pressure, and lower serum lactate levels. Systolic blood pressure mean arterial blood pressure and arterial oxygen concentration did not significantly change with administration of milrinone. These results were irrespective of milrinone infusion dose, infusion duration, and study size. Milrinone was found to have several beneficial hemodynamic effects in children during critical illness when used at usual clinical doses.

Objective: Infective endocarditis is uncommon condition, with a high degree of morbidity and mortality. It is less common in children, albeit tending to be associated with congenital cardiac malformations. We describe our experience of the need for surgical treatment in children with infective endocarditis. Patients and methods: We analyzed retrospectively the records of 9 children aged below 16 years seen between May 2003 and March 2005 with infective endocarditis, reviewing the demographic details, clinical presentation, microbiological and echocardiographic data, operative findings, and outcome. Results: Apart from pre-existing renal insufficiency in 1 patient, congenital cardiac malformations were the predisposing factors. Blood cultures were positive in 3, but remained negative in the other 6 patients. The indications for surgical treatment included uncontrolled sepsis, congestive heart failure, recurrent endocarditis, patch or graft dehiscence, and pseudoaneursymal formation. Death due to uncontrolled sepsis resulting in multiorgan failure occurred in 1 patient, who had tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries. Another patient died late postoperatively due to cardiac failure after relapse of the endocarditis in the setting of negative blood cultures. Conclusion: Despite advances in antimicrobial therapy, diagnosis, and measures of treatment for infective endocarditis, complications continue to be responsible for substantial morbidity and mortality. Since blood cultures are frequently negative, clinical and echocardiographic findings should be the major determinants of strategies used for treatment. We believe that our small series of patients seen over the past two years in which surgical treatment was performed will be helpful in guiding the clinical perspectives for children with infective endocarditis.

“Torsades de pointes”, a life-threatening rhythm disorder, is a polymorphic ventricular tachycardia that usually develops in association with a prolonged QT interval. Fluconazole, an anti-fungal drug, may also induce QT prolongation, in some cases subsequent torsades de pointes. Herein, we report a 16-year-old female presenting “torsades de pointes” after administration of fluconazole and rapidly improved upon cessation of the drug.

First trimester sonography is a widely used technique to examine the foetus early in pregnancy. The desire to recognise complex anatomy already in early developmental stages stresses the need for a thorough knowledge of basic developmental processes as well as recognition of cardiac compartments based on their morphology. In this paper, we describe the possibilities and limitations of sonographic assessment of the foetal heart between 10 and 14 weeks of gestation and correlate this to morphology. Examples of the most commonly detected congenital anomalies are atrioventricular septal defects, transposition of the great arteries, and hypoplastic left heart, which are shown in this paper.