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.

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.

The patient with a functionally univentricular heart is at increased risk for ventricular dysfunction for a variety of reasons. At birth, the pulmonary and systemic circulations are in parallel, leading to pulmonary overcirculation and a volume-loaded functional ventricle. Significant atrioventricular valvar regurgitation, abnormal ventriculoarterial coupling, diastolic dysfunction, and altered ventricular geometry can also contribute to long-term ventricular dysfunction. These collected circumstances place the patient at increased risk for perioperative morbidity and mortality. We will discuss in this review the pathophysiology that leads to ventricular dysfunction at each stage of surgical palliation, as well as the strategies for perioperative management. In addition, we will highlight novel strategies for management of ventricular dysfunction.

The term “functionally single ventricle” includes a variety of congenital cardiac anomalies where there is only one ventricle pumping blood to the systemic and pulmonary circulations. The physiology in this arrangement is a considerable challenge for the cardiac specialist, because the complexity encountered in patients with these lesions necessitates particularly specialized medical and surgical management. Patients with such functionally univentricular physiology often respond to common interventions, such as supplemental oxygen, mechanical ventilation, and vasoactive drugs, differently than patients with conventional circulations.1 Furthermore, these patients tend to be encountered more frequently by pediatricians and cardiologists because they undergo multiple operations, may be more adversely affected by intercurrent illnesses, or have chronic cardiac problems requiring frequent attention. A thorough understanding of the complexities of the physiology encountered is thus imperative for the pediatric cardiologist. In this review, we will address important physiologic and anatomic principles that influence care of neonates with functionally univentricular hearts. Although the anatomy and physiology of each reconstructive stage of palliation are unique, we will focus upon the pre- and post-operative physiology as encountered in the neonate.

Insufficiency of any of the four cardiac valves is a common cause of heart failure in children. Progression of ventricular dysfunction can be predictable, but requires thorough understanding of valvar disease. In valvar regurgitation, the heart has to cope with an increased volume of blood. The pathophysiological sequence is similar for both the right and the left heart. There is initially an increase in end-diastolic volume, followed by an increase in end-systolic volume, and at the end, a decrease in the shortening and ejection fractions. Different compensatory mechanisms and pathophysiologic adaptations develop to maintain the stroke volume for each type of valvar insufficiency, but heart failure eventually ensues. When symptoms of heart failure develop, irreversible ventricular dysfunction is often established, and outcome after surgery may ultimately be compromised. Discerning the optimal time for intervention, before irreversible ventricular dysfunction develops, is a key point in the management of regurgitant valvar heart disease.

There are many daunting challenges that need to be met by any seeking to achieve leadership in the area of cardiac intensive care. Paediatric cardiac intensive care has now emerged as a specialized clinical area, focusing on the unique needs of critically-ill neonates, children, and adults with congenital and acquired heart disease.1 Although an increasing number of centres dealing with paediatric cardiology have either started, or are considering starting, a dedicated cardiac intensive care unit and programme, there are no published primers regarding the logistical aspects of such a challenging endeavour. While there may be an ongoing debate about the preferred strategy in the clinical ownership of the patients treated in intensive care, most authorities agree that it is beneficial to have a dedicated team devoted to the needs of critically-ill children and adults with congenital and acquired cardiac disease.