Mortality rates among children with CHD have significantly declined, although the incidence of neurological abnormalities and neurodevelopmental impairment has increased. Research has focussed on outcomes, with limited attention on prevention and intervention. Although some developmental differences and challenges seen in children with CHD are explained by the cumulative effect of medical complications associated with CHD, many sequelae are not easily explained by medical complications alone. Although cardiac intensive care is lifesaving, it creates high levels of environmental and tactile stimulation, which potentially contribute to adverse neurodevelopmental outcomes. The therapeutic method of individualised developmental care, such as the Newborn Individualized Developmental Care and Assessment Program, provides early support and preventive intervention based on each child’s behavioural signals of stress, comfort, and strength. Implementing developmental care practices in a cardiac ICU requires a thoughtful and well-planned approach to ensure successful adoption of practice changes. This paper reviews how developmental care was introduced in a paediatric inpatient cardiac service through multidisciplinary collaborative staff education, clinician support, child neurodevelopment assessment, parent support, and research initiatives. Given the known risk for children with CHD, cardiac medical professionals must shift their focus to not only assuring the child’s survival but also optimising development through individualised developmental care in the cardiac ICU.

Aim: To identify patients with visceral heterotaxy who are at risk from fulminant sepsis. Methods: We studied 38 patients, 37 having undergone abdominal ultrasound, all 38 having examination of blood films to establish presence of Howell-Jolly bodies, and all 38 documented to have had pneumococcal vaccination and prophylaxis with penicillin. We checked whether the parents were aware of the splenic state of their child, and when possible, we compared current results of blood films with those obtained postnatally. Results: Two of the 17 patients with multiple spleens, all 11 without a detectable spleen, and 1 of 9 patients with a normal spleen, showed Howell-Jolly bodies in their blood films. In 5 of 23 patients with serial blood films, Howell-Jolly bodies had not been seen postnatally, but could now be detected in current blood films. Of these patients, 2 had multiple spleens, 1 did not have a spleen, and 1 had a solitary spleen of normal size. In the other patient, ultrasound could not be performed. Only one of these patients was receiving penicillin prophylactically, and had received pneumococcal vaccination. Of the 15 patients in whom Howell-Jolly bodies were present in the blood, only 8 parents knew about the potential risk for infection. Another 7 parents were sure that their child was taking penicillin regularly, and had received pneumococcal vaccination. Conclusions: Howell-Jolly bodies can be found in the blood of patients with visceral heterotaxy independent of the anatomical state of the spleen. As Howell-Jolly bodies can be encountered in the blood of such patients with increasing age, those with multiple and solitary spleens should be monitored regularly to identify those at risk. Parental knowledge of the splenic state, and compliance for prophylaxis using penicillin, and pneumococcal vaccination, were unsatisfactory in our cohort.

Pulseless cardiac arrest, defined as the cessation of cardiac mechanical activity, determined by unresponsiveness, apneoa, and the absence of a palpable central pulse, accounts for around one-twentieth of admissions to paediatric intensive care units, be they medical or exclusively cardiac. Such cardiac arrest is higher in children admitted to a cardiac as opposed to a paediatric intensive care unit, but the outcome of these patients is better, with just over two-fifths surviving when treated in the cardiac intensive care unit, versus between one-sixth and one-quarter of those admitted to paediatric intensive care units. Children who receive chest compressions for bradycardia with pulses have a significantly higher rate of survival to discharge, at 60%, than do those presenting with pulseless cardiac arrest, with only 27% surviving to discharge. This suggests that early resuscitation before the patient becomes pulseless, along with early recognition and intervention, are likely to improve outcomes. Recently published reports of in-hospital cardiac arrests in children can be derived from the multi-centric National Registry of Cardiopulmonary Resuscitation provided by the American Heart Association. The population is heterogeneous, but most arrests occurred in children with progressive respiratory insufficiency, and/or progressive circulatory shock. During the past 4 years at the Children’s Hospital of Philadelphia, 3.1% of the average 1000 annual admissions to the cardiac intensive care unit have received cardiopulmonary resuscitation. Overall survival of those receiving cardiopulmonary resuscitation was 46%. Survival was better for those receiving cardiopulmonary resuscitation after cardiac surgery, at 53%, compared with survival of 33% for pre-operative or non-surgical patients undergoing resuscitation. Clearly there is room for improvement in outcomes from cardiac resuscitation in children with cardiac disease. In this review, therefore, we summarize the newest developments in paediatric resuscitation, with an expanded focus upon the unique challenges and importance of anticipatory care in infants and children with cardiac disease.

A complication is an event or occurrence that is associated with a disease or a healthcare intervention, is a departure from the desired course of events, and may cause, or be associated with, suboptimal outcome. A complication does not necessarily represent a breech in the standard of care that constitutes medical negligence or medical malpractice. An operative or procedural complication is any complication, regardless of cause, occurring (1) within 30 days after surgery or intervention in or out of the hospital, or (2) after 30 days during the same hospitalization subsequent to the operation or intervention. Operative and procedural complications include both intraoperative/intraprocedural complications and postoperative/postprocedural complications in this time interval.

The MultiSocietal Database Committee for Pediatric and Congenital Heart Disease has set forth a comprehensive list of complications associated with the treatment of patients with congenital cardiac disease, related to cardiac, pulmonary, renal, haematological, infectious, neurological, gastrointestinal, and endocrinal systems, as well as those related to the management of anaesthesia and perfusion, and the transplantation of thoracic organs. The objective of this manuscript is to examine the definitions of operative morbidity as they relate specifically to the renal system. These specific definitions and terms will be used to track morbidity associated with surgical and transcatheter interventions and other forms of therapy in a common language across many separate databases.

Although renal dysfunction and renal failure are known risks of congenital heart surgery, accurate estimates of the incidences of these complications are limited. This lack of knowledge is in part due to the lack of uniform definitions of these postoperative complications. The purpose of this effort is to propose consensus definitions for renal complications following congenital cardiac surgery so that collection of such data can be standardized. Clinicians caring for patients with congenital heart disease will be able to use this list for databases, quality improvement initiatives, reporting of complications, and comparing strategies for treatment.

Catecholaminergic polymorphic ventricular tachycardia is a rare entity that can occur in children without cardiac disease and with a normal QT interval. It may cause syncope, convulsions, and sudden death during physical activity or emotional distress. We report the clinical features, treatment, and follow-up of 16 children with this diagnosis, emphasizing the potentially fatal nature of the disease.

The mean age of patients at the onset of symptoms and at the time of diagnosis was 7.8 plus or minus 2.5 years, and 10.6 plus or minus 3.5 years, respectively. Syncope was the main complaint in 11, and 7 were treated as erroneously as having epilepsy. Diagnosis was confirmed by exercise and/or infusion of isoproterenol. Once the diagnosis was made, we started propranolol in all patients, and added verapamil if ventricular tachycardia was still inducible on a treadmill exercise test. An intracardiac defibrillator was implanted in 4 patients. Of the 16 patients, 4 died suddenly, giving a rate of mortality of 25%. In 2 of those dying suddenly, there was evidence of poor compliance to the recommended treatment. Another 2 patients had been resusciated because of sudden cardiac arrest.

Catecholaminergic polymorphic ventricular tachycardia must be considered in the differential diagnosis of syncope in children without heart disease but with a normal QT interval. Medical treatment with propranolol and verapamil may decrease the incidence of arrhythmia. Implantation of intracardiac defibrillators should be considered in those resistant to drug therapy. Delay in diagnosis, and inadequate treatment, can result in sudden cardiac death.

Compared with transmitral velocities of flow, myocardial wall velocities obtained by tissue Doppler imaging are less influenced by left atrial pressure. The data supporting this assumption, however, are limited in patients with congenital cardiac disease. The aim of this study was to compare the effects of left ventricular preload on transmitral inflow and velocities assessed by tissue Doppler imaging. Tissue Doppler imaging, and conventional Doppler echocardiography with simultaneous invasive hemodynamic studies, were performed in 33 patients with a simple ventricular septal defect or patency of the arterial duct. Transmitral velocities (E, A) and mitral annular velocities (Ea, Aa) were measured, permitting calculation of the ratio of E to Ea. The ratio of pulmonary to systemic flows, and mean left atrial pressure, were also measured. In 10 of 33 patients, echocardiographic and hemodynamic studies were performed 4 to 5 months after surgery.

The E and A values in the patients were greater than those in the controls (p < 0.01). In contrast, neither Ea nor Aa differed between the two group. The ratio of E to Ea in the patients increased significantly compared with that in the controls (8.9 ± 2.1 vs 7.3 ± 1.3, p < 0.01). The E value was directly related to mean left atrial pressure and the ratio of pulmonary to systemic flows. The velocities measured by Tissue Doppler imaging, however, had no significant relationship to either of these measurements. The ratio of E to Ea correlated well with mean left atrial pressure (r = 0.75, p < 0.01). In 10 post-operative patients, the values for E and A decreased from 119 ± 14 to 89 ± 10 cm/sec (p < 0.01) and from 91 ± 22 to 61 ± 9 cm/sec (p < 0.01), respectively. No significant changes were noted in the values of Ea and Aa. The ratio of E to Ea, and mean left atrial pressure, decreased from 10.3 ± 1.9 to 8.2 ± 1.5 (p < 0.01) and from 11.0 ± 1.8 to 7.4 ± 1.0 mmHg (p < 0.01), respectively. The percentage change in left atrial pressure correlated with the percent change in the ratio of E to Ea (r = 0.64, p < 0.05).

The present study showed that the velocities obtained with tissue Doppler imaging are less dependent on mean left atrial pressure in children with elevated left ventricular preload caused by a left-to-right shunt.

Conventional algorithm for treatment of pericarditis and prevention of recurrences consists of non-steroid anti-inflammatory drugs and/or colchicine, followed by corticosteroids in resistant patients. Anakinra has emerged as a promising and safe treatment modality for steroid-dependent idiopathic recurrent pericarditis. However, the efficacy and safety of canakinumab, another anti-interleukin-1 agent, has not been assessed up to date. Herein, we present development of an anaphylactic reaction due to anakinra and a successful subsequent treatment with canakinumab for the first time in the literature.

Dilated cardiomyopathy as seen in children is clinically and genetically heterogeneous, with an increasing proportion of cases known to be caused by disorders of single genes. An autosomal recessive syndrome with a high incidence of dilated cardiomyopathy was recently described in the Canadian Dariusleut Hutterite population. It is caused by homozygous mutations in a novel gene, DNAJC19, presumed to play a role in importation of mitochondrial proteins. We discuss the cardiac features of this syndrome, and its relationship to cardiac mitochondrial function.

Isolated non-compaction of the ventricular myocardium is characterized by numerous and prominent trabeculations and deep intertrabecular recesses. This rare disease is due to an arrest of myocardial morphogenesis. Most cases, when seen in children, are associated with obstructive malformations. Isolated non-compaction is even rarer in childhood, and affects predominantly the myocardium of the left ventricle. Morbidity and mortality resulting from cardiovascular complications is high. In most cases, transplantation is the final option. To our knowledge, this rare cardiac malformation has yet to be diagnosed in the fetus. We report here two sporadic cases, one male and one female, and 2 familial cases, both male, which were diagnosed prenatally and followed by fetal echocardiography. Our study indicates that isolated non-compaction is a primary disorder of early fetal development. Our cross-sectional echocardiographic examinations revealed a fetal cardiomyopathy, with prominent and numerous trabeculations and deep intertrabecular recesses of the myocardium at the apex of the ventricles. In contrast with postnatal experience, we found isolated non-compaction mostly in the right ventricle. Systolic dysfunction was found in all cases. The diagnosis was confirmed by histology in 3 fetuses dying with cardiac failure, and by postnatal cross-sectional echocardiography in the fetus who survived. Two male fetuses belonged to a family in which 3 individuals were subsequently found to be affected. We discuss the issues of prenatal diagnosis, natural history, and myocardial histology.

A large body of literature devoted to “patient safety” and error prevention exists and utilizes a nomenclature that can be applied specifically to the field of congenital cardiac disease and aid in the goals of increasing the safety of patients, decreasing medical error, minimizing mortality and morbidity, and evaluating quality of care. The purpose of this manuscript is to suggest and document a quality of health care taxonomy and the appropriate application of this nomenclature of “patient safety” to the specialty of congenital cardiac disease, with special emphasis on the following ten terms: morbidity, complication, medical error, adverse event, harm, near miss, iatrogenesis, iatrogenic complication, medical injury, and sentinel event. Each of these terms is commonly utilized in the medical literature without universal agreement on their meaning and relationship. It is our hope that the standardization of the definitions of these terms, as they are applied to the analysis of outcomes of the treatments applied to patients with congenital and paediatric cardiac disease, will facilitate improved methodologies to assess and improve quality of care in our profession.

Because of the enormous advances in the medical treatment of CHD, the long-term survival of patients suffering from this disease has increased significantly. Currently, about 90% of patients reach adulthood, which entails many new challenges both for patients and their families and for healthcare professionals. The main objective of family-centred psychosocial care is to strengthen the emotional resilience of chronically ill patients and their families by adopting a holistic approach. During the biannual meeting of the psychosocial working group in 2012, participants expressed the need for general European guidelines. The present recommendations were written to support medical staff and psychosocial healthcare professionals to provide the best care for children and adolescents with CHD as well as for their families. This article describes in detail how the integrated family-centred psychological care modules work, involving different healthcare specialists, including a paediatric/congenital cardiologist or a general paediatrician. The different clinical implications and specific needs have been taken into account and recommendations have been provided on the following: structured follow-up screening; identification of stressful periods related to cardiac surgery or invasive medical procedures; evidence-based, disease-specific, and family-oriented psychosocial interventions; and interactive media links to medical and psychosocial information.

Background: Congenital cardiac disease may be a chronic condition, necessitating life-long followup for a substantial proportion of the patients. Such patients, therefore, are often presumed to be high users of resources for health care. Information on utilization of resources in adults with congenital heart disease, however, is scarce. Methods: This retrospective pilot study, performed in Belgium, investigated 192 adults with congenital heart disease to measure the annual expenditures and utilization of health care and compared the findings with data from the general population. We also sought to explore demographic and clinical parameters as predictors for the expenditures. Results: Hospitalization was documented in 20.3% of the patients, with a median length of stay of 5 days. The overall payment by health insurance associations in 1997 was 1794.5 ECU per patient, while patients paid on average 189.5 ECU out-of-pocket. For medication, the average reimbursement and out-of-pocket expenses were estimated at 78 ECU and 20 ECU, respectively. Expenditures for patients with congenital heart disease were considerably higher than the age and gender-corrected expenditures for the general population (411.7 ECU), though this difference was accounted for by only one-eighth of the cohort of those with congenital heart disease. In general, higher expenditures were associated with abnormal left ventricular end-diastolic diameter, female gender, functional impairment and higher age, although the explained variance was limited. Conclusion: Our study has provided pilot data on the economic outcomes for patients with congenital heart diseases. We have identified parameters that could predict expenditure, but which will have to be examined in future research. This is needed to develop guidelines for health insurance for those with congenital heart diseases.

The long-term outcome of patients with congenitally malformed hearts involving abnormal right ventricular morphology and haemodynamics is variable. In most instances, the patients are at risk for right ventricular failure, in part due to morphological differences between the right and left ventricles and their response to chronic volume and pressure overload. In patients after repair of tetralogy of Fallot, and after balloon valvotomy for valvar pulmonary stenosis, pulmonary regurgitation is the most significant risk factor for right ventricular dysfunction. In patients with a dominant right ventricle after Fontan palliation, and in those with systemic right ventricles in association with surgically or congenitally corrected transposition, the right ventricle is not morphologically capable of dealing with chronic exposure to the high afterload of the systemic circulation. In patients with Ebstein’s malformation of the tricuspid valve, the degree of atrialisation of the right ventricle determines how well the right ventricle will function as the pump for the pulmonary vascular bed.