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The combination of pediatric cardiology being both a perceptual and a cognitive subspecialty demands a complex decision-making model which makes artificial intelligence a particularly attractive technology with great potential. The prototypical artificial intelligence system would autonomously impute patient data into a collaborative database that stores, syncs, interprets and ultimately classifies the patient's profile to specific disease phenotypes to compare against a large aggregate of shared peer health data and outcomes, the current medical body of literature and ongoing trials to offer morbidity and mortality prediction, drug therapy options targeted to each patient's genetic profile, tailored surgical plans and recommendations for timing of sequential imaging. The focus of this review paper is to offer a primer on artificial intelligence and paediatric cardiology by briefly discussing the history of artificial intelligence in medicine, modern and future applications in adult and paediatric cardiology across selected concentrations, and current barriers to implementation of these technologies.

We present a pilot case using an innovative fusion of echocardiogram and MRI achieved with a MATLAB-based imaging programme to explore the feasibility of this imaging strategy in the functional and anatomic assessment of a patient with repaired tetralogy of Fallot requiring pulmonary valve intervention. Echocardiogram and MRI neutralises the disadvantages and limitations of each individual imaging modality and yields important anatomic and haemodynamic information crucial to the treatment decision-making process. Future image fusion strategies can apply to three-dimensional images and image-directed therapy for CHD.

Fukuyama congenital muscular dystrophy weakens both skeletal and cardiac muscles, but the rate of cardiomyopathic progression can accelerate faster than that of skeletal muscles. A 14-year-old boy with Fukuyama congenital muscular dystrophy presented with mild skeletal myopathy but severe cardiomyopathy requiring heart transplantation within 1 year of declining heart function. These patients need frequent screening regardless of musculoskeletal symptoms.

The development of databases to track the outcomes of children with cardiovascular disease has been ongoing for much of the last two decades, paralleled by the rise of databases in the intensive care unit. While the breadth of data available in national, regional and local databases has grown exponentially, the ability to identify meaningful measurements of outcomes for patients with cardiovascular disease is still in its early stages. In the United States of America, the Virtual Pediatric Intensive Care Unit Performance System (VPS) is a clinically based database system for the paediatric intensive care unit that provides standardized high quality, comparative data to its participants [https://portal.myvps.org/]. All participants collect information on multiple parameters: (1) patients and their stay in the hospital, (2) diagnoses, (3) interventions, (4) discharge, (5) various measures of outcome, (6) organ donation, and (7) paediatric severity of illness scores. Because of the standards of quality within the database, through customizable interfaces, the database can also be used for several applications: (1) administrative purposes, such as assessing the utilization of resources and strategic planning, (2) multi-institutional research studies, and (3) additional internal projects of quality improvement or research.In the United Kingdom, The Paediatric Intensive Care Audit Network is a database established in 2002 to record details of the treatment of all critically ill children in paediatric intensive care units of the National Health Service in England, Wales and Scotland. The Paediatric Intensive Care Audit Network was designed to develop and maintain a secure and confidential high quality clinical database of pediatric intensive care activity in order to meet the following objectives: (1) identify best clinical practice, (2) monitor supply and demand, (3) monitor and review outcomes of treatment episodes, (4) facilitate strategic healthcare planning, (5) quantify resource requirements, and (6) study the epidemiology of critical illness in children.Two distinct physiologic risk adjustment methodologies are the Pediatric Risk of Mortality Scoring System (PRISM), and the Paediatric Index of Mortality Scoring System 2 (PIM 2). Both Pediatric Risk of Mortality (PRISM 2) and Pediatric Risk of Mortality (PRISM 3) are comprised of clinical variables that include physiological and laboratory measurements that are weighted on a logistic scale. The raw Pediatric Risk of Mortality (PRISM) score provides quantitative measures of severity of illness. The Pediatric Risk of Mortality (PRISM) score when used in a logistic regression model provides a probability of the predicted risk of mortality. This predicted risk of mortality can then be used along with the rates of observed mortality to provide a quantitative measurement of the Standardized Mortality Ratio (SMR). Similar to the Pediatric Risk of Mortality (PRISM) scoring system, the Paediatric Index of Mortality (PIM) score is comprised of physiological and laboratory values and provides a quantitative measurement to estimate the probability of death using a logistic regression model.The primary use of national and international databases of patients with congenital cardiac disease should be to improve the quality of care for these patients. The utilization of common nomenclature and datasets by the various regional subspecialty databases will facilitate the eventual linking of these databases and the creation of a comprehensive database that spans conventional geographic and subspecialty boundaries.

Objectives: It is known that levels of brain natriuretic peptide predict outcomes of treatment for adults with decompensated heart failure. We hypothesized that it could predict outcomes in children with this condition., Methods: We divided retrospectively 82 patients with serial measurements of brain natriuretic peptide into 3 groups: those who survived and did not need readmission within less than 60 days; those who survived but needed readmission within less than 60 days; and those who died in hospital or within less than 60 days. Initial and final levels of the peptide correlated with adverse outcomes., Results: The percent change in level of the peptide was minus 78 percent, minus 38 percent, and 138 percent in the readmission-free group, the readmitted, and nonsurviving groups, respectively. Final levels were significantly lower in the readmission-free group than in the readmitted and nonsurviving groups (p equals 0.013 and p is less than 0.00001, respectively) and in the readmitted group than in the nonsurvivors (p equals 0.013). On univariate analysis, the final level, the change in level, and the percentage change in level significantly predicted outcomes (p equals 0.0002, 0.0072 and 0.0005, respectively). On multivariate analysis, only the final level of the peptide significantly predicted outcomes (p equals 0.01)., Conclusions: A final level of brain natriuretic peptide of greater than or equal to 760 picograms per millilitre strongly predicted an adverse outcome. Patients with higher final levels may be at higher risk of death and readmission, suggesting that this variable effectively predicts the response to treatment and prognosis in children with heart failure.

Neonates with congenital cardiac disease are a special population. They are often critically ill, and need prolonged intravenous access. To date, no study has evaluated the efficacy and safety of peripherally inserted central venous catheters placed in this unique population. Our goal was to evaluate the use of such catheters in neonates with critical congenital cardiac disease, and to study features such as duration of use, reasons for removal of catheters, and complications. We inserted a total of 124 catheters in 115 neonates with critical congenital cardiac disease who were admitted to the Intensive Care Unit at Texas Children's Hospital from August 2002 to August 2004. The patients had a mean age of 10 days, and a mean weight of 3.1 kilograms. The peripherally inserted catheters were in place for a mean of 22.3 days. Therapy was completed in 76.6% patients at the time of removal of the catheter. The incidence of occlusion, dislodgement, and thrombus was 4.0%, 2.4%, and 1.6%, respectively. The infection rate was 3.6 per 1000 catheter-days, with a median onset on 37 days after placement. We conclude that central venous catheters, when inserted peripherally, provide reliable and safe access for prolonged intravenous therapy in neonates with critical congenital cardiac disease.

Although persisting endothelial dysfunction has been established in the vasculature of patients following surgical repair of coarctation, it is unknown whether there are alterations in the cytoskeleton of the aorta in such patients. We compared staining of N-terminus dystrophin in the smooth muscle of the aortic wall of a patient with coarctation to that in a patient without coarctation, the latter undergoing surgical treatment of a double aortic arch. There was a marked difference in the pattern of expression of dystrophin between the two, with the coarcted specimen demonstrating marked fragmentation but normal intensity of staining. As far as we are aware, ours is the first report to demonstrate the presence of dystrophin in the smooth muscle of the aorta. Alterations in the cytoskeletal structure may account for underlying aberrations in endothelial function in such patients, and is a topic that warrants further investigation.

The neonate with functionally univentricular physiology presents unique challenges to the cardiac team. An integrated approach that applies working knowledge of cardiac anatomy, cardiopulmonary physiology, and basic principles of intensive care is essential to guide management of each individual patient. This requires cooperative and constructive involvement of a surgical, medical, nursing and respiratory care team experienced in the management of such patients. In the neonate with this physiology, systemic oxygen delivery is optimized by manipulating pulmonary and systemic resistances, augmenting total cardiac output, and utilizing strategies for ventilation that preserve optimal pulmonary recruitment.