A female baby suffered from a rare association between histiocytoid cardiomyopathy, left ventricular non-compaction, and Wolff–Parkinson–White syndrome causing severe and recurrent arrhythmic storms. Antiarrhythmic drugs, radiofrequency ablation of Purkinje tissue, and sympathetic denervation were ineffective. The implant of a cardiac defibrillator allowed her to survive till heart transplant. Compound mutation of CACNA2D1 and RANGRF genes were found. To the best of our knowledge, this is the first comprehensive description of the concurrence of these two mutations and histiocytoid cardiomyopathy.

Patients with single-ventricle CHD undergo a series of palliative surgeries that culminate in the Fontan procedure. While the Fontan procedure allows most patients to survive to adulthood, the Fontan circulation can eventually lead to multiple cardiac complications and multi-organ dysfunction. Care for adolescents and adults with a Fontan circulation has begun to transition from a primarily cardiac-focused model to care models, which are designed to monitor multiple organ systems, and using clues from this screening, identify patients who are at risk for adverse outcomes. The complexity of care required for these patients led our centre to develop a multidisciplinary Fontan Management Programme with the primary goals of earlier detection and treatment of complications through the development of a cohesive network of diverse medical subspecialists with Fontan expertise.

Severe right ventricular failure complicated a postoperative systemic inflammatory response in a 33-year-old woman after surgical repair of congenital cardiac malformations. Volume loading, and high doses of catecholamines, failed to produce improvement, but treatment with vasopressin improved all haemodynamic parameters, and also allowed reduction of the other inotropes. After 10 days, the patient was discharged in stable condition from the intensive care unit.

A new technique is presented to create percutaneously an unrestrictive and lasting atrial communication in small children. In 2 infants, a stent was deployed in a restrictive atrial communication and first inflated with an 8 mm balloon. The stent was then further expanded up to 10 or 12 mm. The gradient and turbulent flow between the two atriums disappeared completely. The stent retained a very stable position without embolisation. Other complications, such as formation of thrombus or arrhythmia, were not observed until elective explantation after 2 and 11 months. We conclude that, in infants, dilation of the atrial septum with a stent can provide a safe, lasting, and unrestrictive atrial communication.

Objectives: To determine whether the present system of reimbursement, based on diagnosis-related groups and regular financial budgeting, covers the costs incurred during hospitalisation of 7 children undergoing the three stages of the Norwood sequence for surgical treatment of hypoplastic left heart syndrome. Methods: Between January and September 2003, 7 patients underwent initial surgical palliation with the Norwood procedure at the Rikshospitalet University Hospital. A prospective methodology was developed by our group to measure the costs associated with each individual patient. The patients were closely observed, and the relevant data was collected during their stay in hospital. The stay was divided into four different periods of requirements of resources, defined as heavy intensive care, light intensive care, intermediate care, and ordinary care. At each stage, we recorded the number of staff involved and the duration of surgery and other major procedures, as well as the cost of pharmaceuticals and other consumables. Based on these data, we calculated the cost for each patient. These costs were compared with the corresponding revenue received by the hospital for each of the patients. Results: We found the total mean cost for the three stages of the Norwood sequence was 138,934 American dollars, while the corresponding revenue received by the hospital was 43,735 American dollars. During this period, one patient died during the first stage of the Norwood sequence. Conclusions: Our study shows that steps involved in the Norwood sequence are low-volume but high-cost procedures. The reimbursement received by our hospital for the procedures was less than one-third of the recorded costs.

In patients who have undergone a superior cavopulmonary anastomosis, the superior caval venous flow provides the only, or the most important, pulmonary blood supply, while the inferior caval venous blood is not oxygenated, being mixed with the pulmonary venous blood before entering the systemic circulation. In healthy children, the contribution of superior caval venous flow to total cardiac output has been shown to decrease during growth. Patients who have undergone a superior cavopulmonary anastomosis, however, often have a higher oxygen saturation than predicted by the age-matched ratio of superior to inferior caval venous flows. This study was designed, therefore, to assess the ratio of flows in the superior and inferior caval veins subsequent to a superior cavopulmonary anastomosis. We carried out 18 magnetic resonance imaging studies with velocity-mapping and heart catheterisations so as to assess the contribution of superior caval venous flow to total cardiac output. Patients were divided into 3 groups according to their age. There were five aged from 8 to 24 months, eight aged from 24 to 48 months, and five older than 48 months. No significant difference could be found in the ratios of superior-to-inferior caval venous flow, nor of superior caval venous-to-systemic flow, between the 3 groups. The ratio of venous flows was 0.89 ± 0.34 in those aged from 8 to 24 months, 1.09 ± 0.42 in those from 24 to 48 months, and 1.25 ± 0.27 in the older patients (F analysis of variance 1.06, p 0.37). The ratio of superior caval venous-to-systemic flow was 0.46 ± 0.08 in the youngest patients, 0.50 ± 0.09 in those aged from 24 to 48 months, and 0.55 ± 0.05 in the older patients (F analysis of variance 0.76, p 0.49). These findings suggest that the hemodynamics of a cavopulmonary anastomosis may affect the normal decrease of superior caval venous flow with age. This could be related to a redistribution of flow, with a proportionally higher flow to the head and upper body after construction of a superior cavopulmonary anastomosis. Since increasing cyanosis and progressive exercise intolerance are the main indications for creation of a total cavopulmonary connection, these findings should be taken into account when determining the timing for completion of the Fontan circulation.

Primary care cardiology is also known as ambulatory cardiology and outpatient cardiology. Primary care cardiology for the longitudinal management of patients with hypoplastic left heart syndrome is both poorly described and has limited evidence to justify its basis. This article briefly discusses the various complications that these patients can develop, reviews the medical literature, and describes a framework for the care of these complex patients from infancy to transition to care by specialists in adults with congenital cardiac disease.

Supraventricular tachycardia is the most common significant arrhythmia in children. If prolonged, it may cause heart failure and progress to cardiogenic shock warranting prompt treatment. The recommended interventions following vagal manoeuvres are intravenous adenosine and in the unstable patient electrical cardioversion. We present an infant with an unstable supraventricular tachycardia that was resistant to electrical cardioversion and recommended doses of adenosine. He reverted to sinus rhythm with a higher dose of adenosine, suggesting that such doses may be required in refractory supraventricular tachycardia.

We report our experience from 1996 through 1999, representing our initial experience with use of the Amplatzer device to close atrial septal defects. Of 46 patients taken to the catheter laboratory with the intention to close the defect, the device was permanently implanted in 40 (87%). They were aged between 1.4 and 71.8 years, with weights ranging from 7.8 to 90 kg. Both age and weight distributed into two peaks, demonstrating two different populations. The size of the devices, taking the biggest device if two were inserted, was between 9 and 30 mm. We underwent a short learning curve, but the time required for fluoroscopy, or the number of difficulties experienced, showed no connection with the size or age of the patient, nor the size of the defect itself. A suspicion that young age and small size would increase the risk and difficulties, and result in more interrupted procedures, could not be substantiated. In children no interruption was procedural. Our early experience, therefore, demonstrates that an experienced interventional team can use the Amplatzer occluder successfully to close atrial septal defects in patients of all ages and sizes, at least from 7.8 kg and up.

Due to many innovative techniques and technologies, the overall rate of survival for infants undergoing surgical repair of congenital cardiac malformations is excellent. Despite these excellent outcomes, neonates with hypoplasia of the left heart represent a very unique population, and pose difficult challenges for the entire team involved in care. Despite some centers reporting outstanding outcomes, overall rate of survival rate is reported at only 53 percent,1 and survivors often show neurodevelopmental deficits. Many attempts have been made to identify predictors of neurologic outcomes, with heavy concentration around the pre- and peri-operative periods. Little attention has been paid, however, to assessing how attempts at balancing the systemic and pulmonary circulation affect neurodevelopmental outcomes. In the past, we have reported excellent rates of survival with use of routine postoperative circulatory support to augment cardiac output.2,3 This strategy, nonetheless, is still widely debated. It seems plausible that, by augmenting cardiac output in the immediate postoperative period, cerebral perfusion and oxygenation can be optimized, thereby improving neurologic outcomes. In this review, we describe our experience to date in assessing neurological outcomes, and discuss whether outcomes are associated with cerebral saturation and extraction of oxygen during the period of postoperative circulatory assist.

The complexity of the modern systems providing health care presents a unique challenge in delivering care of the required quality in a safe environment. Issues of safety have been thrust into the limelight because of adverse events highly publicized in the general media.

In the United States of America, improving the safety and quality in health care has been set forth as a priority for improvements in the 21st century in the report from the Institute of Medicine. Many measures have now been initiated for improving the safety of patients at hospital, regional, and national level, and through initiatives sponsored by governments and private organizations. In this review, we summarize known concepts and current issues on the safety of patients, and their applicability to children with congenital cardiac disease. Prior to examining the issues of medical error and safety, it is important to define the terminology.

An error is defined as the failure of a planned action to be completed as intended, also known as an execution error, or the use of a wrong plan to achieve an aim, this representing a planning error. An active error is an error that occurs at the level of the frontline operator, and the effects of which are felt immediately. A latent error is an error in the design, organization, training and maintenance, that leads to operator errors, and the effects of which are typically dormant in the system for lengthy periods of time. Latent errors may cause harm given the right circumstances and environment.

An adverse event is defined as an injury resulting from medical intervention. A preventable adverse event is an adverse event that occurs due to medical error. Negligent adverse events are a subset of preventable adverse events where the care provided did not meet the standard of care expected of that practitioner.

The study of improving the delivery of safe care for our patients is a rapidly growing field. Important components for development of programmes to improve the safety of patients include the leadership for the programme, the implementation of process design based on human limitations, the promotion of teamwork and function, the anticipation of unexpected events, and the creation of a learning environment.

Much is yet to be learned about the risk and incidence of adverse events during hospitalization of children with congenital cardiac disease. Errors due to human factors, such as poor communication, poor coordination, and suboptimal team work, have shown to be important causes of adverse outcomes in children undergoing cardiac surgery, and should be a focus for improvement. Future research on evaluating causes and prevention of medical errors and adverse events in this population at high risk, and consuming high resources, is essential.

Issues of inadequate safeguards for patients have been prominent in the media, and have been highlighted in reports from the Institute of Medicine. Our review discusses research on the causes of medical error, and proposes concepts to design successful programmes to improve safety for the patients on a local level.

Centralisation of services such as cardiology, cardiac surgery, and intensive care in many parts of the world has resulted in the need to safely transport children with critical cardiac disease from local hospitals to specialist centres for diagnostic, surgical, and/or critical care intervention. The transport of this cohort of children, whether locally or internationally, can present specific clinical and logistical challenges. An international group of clinicians with expertise in cardiac care and critical care transport worked together to summarise current clinical practice relating to key areas of transport. This expert review covers the transport of the child with critical cardiac disease in terms of referral triage and advice, enabling optimal management of locally available resources, clinical stabilisation before transport, international air transport, transport considerations in low- and middle-income countries, and the transport of children with specific cardiac conditions. As specialist services are centralised to fewer large centres, the need for safe and timely inter-hospital transport of children with critical cardiac disease is only expected to rise in the future. The key principles outlined in this review will be helpful for practitioners in global settings who are, or might be, involved in transporting children between hospitals.

This case describes the prenatal diagnosis and integrated peripartum management of a foetus with 2:1 atrioventricular block and torsade de pointes due to congenital long QT syndrome. The unique issues related to the detection of intrauterine conduction abnormalities and ventricular arrhythmias, along with the immediate postnatal care, have been described as an interesting teaching case with successful outcome.

Recently, three-dimensional printing of heart models is being used to plan percutaneous and surgical interventions in patients with CHD. We describe a case where we used a three-dimensional print model to plan a complex percutaneous intervention in a patient with coarctation of the aorta.

Non-surgical occlusion of the arterial duct (ductus arteriosus) has been shown to be effective. Its limitations are yet to be fully defined. We have attempted to elucidate this issue in the setting of our early experience with the procedure, utilizing the Rashkind double disc occluder. After clinical and echocardiographic evaluation, 56 consecutive patients were studied invasively. In two patients, the patent duct was considered too large for occlusion and in one patient it could not be crossed with the diagnostic catheter. In the remaining 53 patients (95%), an attempt to implant the double disc occluder was made. The mean age of all patients was 7.9 years (range 0.16 year to 29 years). There were 35 females (63%) and 21 males. The mean weight was 24.2 kg (range 6.7 kg–85 kg). In five patients (9.5%), the procedure failed. This was due to pull through of the 17 mm device in three patients. In another patient, because of a very small duct (post surgical ligation), it was not possible to deliver the distal legs of the 12mm device. In the last patient, the device embolized to the left pulmonary artery and was retrieved to the groin. Subsequent replacement was not attempted. In 48/53 patients (90.5%) the device was successfully implanted. Nine cases of the 48 in whom the device was implanted (18.8%) showed complete occlusion at 10 minutes and 22 more (45.8%) five to 24 hours after the procedure. A total of3 1/48 patients (64.5%) were discharged home the day following the procedure with no residual shunt, as judged by color flow Doppler studies. The mean duration of the procedure in all patients was 94 minutes (range 47 to 244). The fluoroscopy time ranged from 3 to 81 minutes with a mean of 17 minutes. Follow-up data (between five days and 18 months) are available in 20/48 patients. Three patients had a clinically detectable residual shunt, with an audible continuous murmur six months after the procedure. All three have undergone complete occlusion using a second device. One more patient showed spontaneous occlusion on follow-up. So far, a total of 35/48 patients (72.9%) have had complete occlusion of the patent duct. We conclude that not all patients with an arterial duct are amenable to occlusion using the Rashkind device. The procedure appears to be safe when performed by a suitably trained novice, nonetheless, and successful implantation can be achieved in over 90% of patients.