Fetal echocardiography as a screening tool as well as a diagnostic modality has been proven to be safe and accurate for the diagnosis of fetal heart disease. The structure and function of the heart can be assessed by echocardiography. Prenatal echocardiography has the potential for improving postnatal survival in infants with congenital heart defects. However, it remains unclear whether it also improves postoperative outcomes. Prenatal diagnosis possibly identifies patients with more severe phenotypes, resulting in a poor prognosis in this group. Fetal therapy, the treatment of the fetus, is now practical. The current fetal therapeutic strategies range from maternal transplacental medication to the fetus to invasive open-uterine fetal surgery. In fetal therapy, fetal critical disorders must be weighed against the risks to the mother and potential for a successful treatment after birth.

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Development of low-profile materials has enabled various catheter interventions to be performed in the neonatal period. In this study, three clinically important issues related to neonatal catheter interventions are discussed: 1) alternative methods when balloon atrial septostomy is difficult to perform; 2) balloon pulmonary valvuloplasty in neonates with pulmonary atresia with intact ventricular septum, and the long-term outcomes of the procedure; and 3) the safe technique of ductal stenting in patients with hypoplastic left heart syndrome. Although neonatal catheter interventions are less invasive than surgical procedures, there are several severe complications. Therefore, it is important that these procedures are performed with sufficient information and understanding of the potential complications and ways to deal with them.

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Adequate circulatory management in critical congenital heart diseases is vital in pediatrics because of poor prognosis. Understanding and managing factors such as contractility, load, cardiac morphology, and vascular resistance are vital in controlling cardiac output and maintaining balance between the systemic and pulmonary circulations. Particularly, in neonates, securing systemic blood flow through the regulation of pulmonary blood flow is an important issue, because there is a high risk of cardiogenic shock with increased pulmonary blood flow, resulting in general deterioration or death. Furthermore, not only cardiac care but also general care is needed to prevent several complications including non-cardiac complications. Detailed diagnosis, accurate understanding of the pathophysiology, and prediction of hemodynamics will lead to improved care of neonates with critical congenital heart disease.

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In the modern era, advances in open-heart surgery as the first choice for treating many congenital cardiac defects have decreased markedly as indications for palliative procedures. However, palliative surgery is still needed for the selected patients. Furthermore, the indications for palliative procedures have expanded far beyond the original concept. The main goals of palliation are to alter the hemodynamic physiology, i.e., to control the pulmonary blood flow, particularly the normally high neonatal pulmonary vascular resistance. Implantation of a systemic-pulmonary artery shunt (SP shunt) and pulmonary artery banding (PAB) are the two primary methods of palliation. The physiology of circulation incorporating an SP shunt is complex, particularly lesions within a single ventricle. Consequently, the SP shunt is still associated with a significant incidence of adverse outcomes. A clear understanding of the anatomy and physiology is important. PAB also continues to occupy an important place in surgical treatment for very young infants for whom cardiopulmonary bypass is associated with a great risk. The role of PAB is to reduce pulmonary blood overflow to prevent or reverse cardiac failure and to provide long-term protection against irreversible pulmonary hypertension. Moreover, several cardiac defects can benefit from early PAB, especially when preparation and training of the left ventricle is necessary. Bilateral PAB is also an attractive and effective method for the control of pulmonary blood flow in ductal-dependent lesions.

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The Japanese Society of Pediatric Interventional Cardiology (JPIC) Database has recently been inaugurated, replacing the annual questionnaire surveillance regarding catheter-based interventional procedures and complications, which has been conducted over the past 15 years. The online registry system includes comprehensive benchmarking of any form of pediatric and congenital interventional procedures and adverse events, the ability to link with emerging relevant databases, ethical justification, and public and scientific data exploitation. It eventually went into operation with the initial enrollment of actual cases since January 2013. We can expect further prevalence and advancement in the near future.

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Short- and long-term results of surgical treatment for congenital heart disease have further improved during the past 2 decades. This is associated with advance in preoperative diagnosis, surgical techniques, cardiopulmonary bypass machine, and perioperative management. In this review, representative surgical methods and related pitfalls are described to young pediatric cardiologists from the view point of a cardiac surgeon.

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Background: For the transition to adult health care, it is important that children with congenital heart disease (CHD) understand their disease. However, disease understanding in children has not been well documented. This study evaluated the ability of children with CHD to understand their disease according to patient age and disease severity.
Methods: A total of 28 children in grades 1–9 who received treatment at a pediatric cardiology department participated in semi-structured interviews.
Results: No differences according to age or severity were observed in children’s knowledge of “limitation of exercise,” “medication frequency,” “medication effect,” and “prevention of infective endocarditis.” However, understanding of “disease name,” “medication name,” “reason for consultation,” “next consultation date,” and “self-management of medication” increased with age. “Cardiac defect” was specifically understood best by patients with mild symptoms.
Conclusion: The results suggest that care providers should explain the disease to children with CHD while considering cognitive development and disease complexity.

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Malignant hyperthermia has been described as a rare genetic hypermetabolic disorder of skeletal muscle that is triggered by exposure to certain inhalation anesthetics and depolarizing muscle relaxants. The incidence of malignant hyperthermia has declined with improvements in anesthesia in recent years, but it is still a lethal complication of cardiac surgery. We experienced a case of postoperative fulminant malignant hyperthermia after pediatric cardiac surgery. The patient was an 11-month-old boy with a ventricular septal defect (VSD) and Down syndrome. He previously had undergone pulmonary artery banding and ligation of the persistent ductus arteriosus on day 21 after birth due to respiratory distress. In the present treatment, he underwent closure of VSD and de-banding. The operation was performed uneventfully, but soon after surgery, his body temperature rose to as high as 40°C. Two days after surgery, desaturation and cardiac failure occurred with hypermetabolic disorders. We re-opened the sternum, and his condition recovered after this procedure. We diagnosed this condition as a fulminant type of postoperative malignant hyperthermia. Dantrolene sodium hydrate was infused, and strong cooling via a mat was applied. He recovered with no significant neurological damage. We should be aware of this lethal complication after cardiac surgery. This case reminded us that early diagnosis and quick treatment are most important.

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