From a physiologic perspective, children are equal to adults. In congestive heart failure, elevated left end-diastolic pressure causes pulmonary venous congestion, and leads to capillary leakage into the alveoli. In this situation, diuretics and positive airway pressure are useful in both adults and children; however, pediatricians should also consider children’s characteristics. For example, a child’s airway is small, which has an impact on airway resistance, particularly when it is swollen. Dead space is another key factor that needs to be considered. Furthermore, in many congenital heart diseases, pulmonary blood flow is not equal to systemic blood flow. Thus, pediatric cardiologists must consider the relationship between pulmonary blood flow and the airway, and the impact of oxygen therapy on pulmonary blood flow. Moreover, it is important to determine specific reactions in congenital heart diseases. For example, in normal circulation, positive intrathoracic pressure assists the left ventricle myocardium; however, in Fontan patients, positive intrathoracic pressure decrease cardiac output. Therefore, pediatric cardiologists must be familiar with cardiovascular physiology and respiratory physiology, and their interaction (i.e., the cardiopulmonary interaction).

Pharmacology is essential to understand how medications work; however, it is not sufficient for clinicians to simply recognize pharmacology in clinical medicine. This article addresses not only the necessity of pharmacological knowledge, but also the importance of knowledge beyond pharmacology in the management of heart failure.

Circulatory failure is a life threating condition for which every pediatric cardiologist should be prepared to perform physiological analyses, make optimal therapeutic decisions, and secure the best outcome. The correct approach for determining the cause of circulatory failure can accelerate the patients’ recovery from a critical condition, as well as mitigate in allowing their outcome, whereas patients can rapidly deteriorate if their management is inadequate. The integrated concept of cardio-vascular interactions, the pressure-volume relationship, is not novel, but it is vital in allowing clinicians to understand the underlying physiology of circulatory failure. Furthermore, the pressure-volume relationship has the advantage of describing both cardiac function and its loading condition in the same dimension. Accordingly, the pressure-volume relationship can help clinicians to determine the best available therapeutic intervention. This review aimes to convey the initial steps involved in assessing hemodynamic issues in children with circulatory failure.

A “fair and just” joint conference among cardiac surgeons and pediatric cardiologists is imperative to discuss the clinical plan of pediatric heart disease patients. Discussion is often the only way to determine the most appropriate treatment decision for the patients, not only due to the lack of clinical evidence, but the uncertainty surrounding whether there is a truly “correct” answer for the treatment of disease in this particular area. Therefore, the quality of the joint conference has to be deliberately inspected and improved. Moreover, it is important to recognize various biases that can perpetually mislead our judgment. Effective and efficient conferences, together with a multi-disciplinary collaboration, should improve the outcomes of cardiac patients.

The information that a surgeon expects to obtain from physicians or pediatricians at a preoperative conference varies according to the surgeon’s experience, knowledge, and the policy of their institute. Young surgeons build an operative strategy based on the information that they aquire from a preoperative conference, and the relevant preoperative information varies across diseases and operative procedures. Presentations based on the basic knowledge of cardiopulmonary bypass and surgical procedures assists for young surgeons with developing an appropriate surgical strategy.

In the field of congenital heart surgery, it is often difficult to determine the best treatment option. For patients with complex heart disease or operative factors, we recommend obtaining a second opinion from the viewpoint of shared decision-making. In this study, we describe four second opinion cases, relating to congenitally corrected transposition of the great arteries (ccTGA): Transposition of the great arteries (TGA), TGA and pulmonary atresia (PA), hypoplastic left heart syndrome (HLHS) with total anomalous pulmonary venous connection (TAPVC), and a functional single ventricle with mixed TAPVC. In the ccTGA case, the patient preferred a double switch operation to conservative observation. The patient with TGA and PA underwent a Rastelli operation, which was the treatment option offered by the second opinion institution. In the HLHS with TAPVC case, the patient’s mother preferred treatment at another hospital, which had a previous Fontan survivor with the same disease. In the last case, the family felt embarrassed because the operation was considered impossible at first, but the patient was later told that surgery would be appropriate. Consequently, the family requested a second opinion from another hospital, where they were told that surgery could be performed, which they agreed to. All four cases relied on shared decision-making. Our findings show that information-sharing, and a sincere attitude towards patients is necessary for all medical workers.

Background: Intravenous immunoglobulin (IVIG) is the mainstay of initial treatment for Kawasaki disease (KD). Previously, 5％ immunoglobulin was used for the treatment of patients with KD; however, a 10％ immunoglobulin preparation has recently become available.

Purpose: To analyze the safety and effectiveness of treatment with 10％ immunoglobulin in patients with KD. Additionally, we sought to determine whether it was possible to shorten the interval between the initial IVIG dose and the second IVIG dose, among the patients who did not respond to the initial IVIG dose (non-responders) in the 10％ IVIG group.

Methods: We retrospectively compared the clinical findings of 103 patients who were administered 5％ IVIG, and 60 patients who were administered 10％ IVIG between January 2015 and May 2019.

Results: The clinical profiles were not significantly different between the 5％ and 10％ groups. A total of 31 (30％) patients in the 5％ group, and 20 (33％) patients in the 10％ group, were nonresponsive to the initial IVIG dose, although these differences were not significant (p＝0.727). The interval between the initial IVIG dose and the second IVIG dose among the non-responders was significantly shorter in the 10％ IVIG group than the 5％ IVIG group (48.8 vs 45.4 hours, p＝0.004). Only one patient in the 5％ group developed coronary artery lesions, and no serious adverse events were observed.

Conclusion: Results showed that 10％ immunoglobulin is as safe and effective as 5％ immunoglobulin for the treatment of patients with KD, and can provide additional early treatment for non-responders to the initial IVIG dose.

Background: Role of specialist of pediatric critical care is significant, especially for management of congenital heart diseases because most critical cardiac patients have been cared by pediatric cardiologists or cardiovascular surgeons in Japan.

Objects: To clarify future issues in the field of critical care in pediatric cardiology by investigating current situation of board certification and medical insurance in Japan.

Methods: In this study, we investigated relationship between board certification and situation of training centers for the board-certified intensivist with pediatric cardiologists, pediatric cardiac surgeons and pediatricians in Japan from databases of official registration in the website.

Results: Only 0.6％ of board-certified pediatricians and 1.1％ of board-certified pediatric cardiologists obtained board-certified intensivist. Certification of training center is 56％ in all pediatric cardiac hospitals and their training systems is not appropriate for pediatricians because of lack of pediatric intensive care units especially in most university hospitals or general hospitals. There is a gap between cities and regional area in the number of board-certified pediatricians with board certified intensivists.

Conclusions: Significant gaps of board certification and medical insurance are recognized among regional areas in Japan requiring improvement of system and current situation in the field of critical care of pediatric cardiology.

The morphological right ventricle supports systemic circulation following physiological repair of congenitally corrected transposition of the great arteries (ccTGA); however, complications such as failure of the right ventricle, tricuspid valve regurgitation, and arrhythmia can arise in adolescence and adulthood. Here, we report the case of a 26-year-old woman who underwent a 4th surgery after undergoing physiological repair for ccTGA. Previously, she underwent a shunt procedure at the age of 3, ccTGA repair at the age of 10, and replacement of the left ventricle to the pulmonary artery conduit when she was 20 years old. In recent years, she was frequently hospitalized due to acute heart failure and atrial tachyarrhythmia. After further examination, we performed aortic and tricuspid valve replacement, and upgraded her cardiac resynchronization therapy pacemaker (CRT-P). Her post-operative course was uneventful, although she was administered catecholamine for an extended duration. Electrocardiogram revealed a decreased duration of QRS from 204 ms to 112 ms, following the CRT-P upgrade, and her brain natriuretic peptide level also markedly improved. She is doing well 2 years and 9 months after the surgeries.

We report the case of a 5-year-old girl with a ventricular septal defect (VSD) complicated by stenosis of the right ventricular outflow tract due to a giant membranous septal aneurysm. The patient had a heart murmur at birth, and was diagnosed with membranous VSD and aortic coarctation. At 3 months old, the VSD showed signs of spontaneous closure and a mild left-to-right (LR) shunt was observed. Therefore, surgery for aortic constriction was performed as a follow-up for the VSD. At 4 years old, echocardiography revealed a giant membranous septal aneurysm protruding into the right ventricular outflow tract, and cardiac catheterization was performed. The Qp/Qs, right ventricular systolic pressure and pulmonary artery systolic pressure were 1.0, 79 mmHg and 26 mmHg, respectively, and right ventricular outflow stenosis was revealed. Although adult cases of membranous septal aneurysms that result in right ventricular outflow stenosis have been reported, pediatric cases are rare. Given such complications, careful observation is necessary for VSDs with a mild LR shunt.

A 7-month-old male infant with a diagnosis of idiopathic dilated cardiomyopathy exhibited poor response to anti-heart failure medication. Following diagnosis, the infant underwent pulmonary artery banding, which has been reported to be effective in restoring heart function. Although we initially aimed to transition the patient to home care, we observed a slow deterioration of his heart. As a result, the infant remained hospitalized for 14 months thereafter, and underwent Excor^® implantation. At the time of writing, he is waiting for heart transplantation. In this case, the ineffectiveness of pulmonary artery banding may be partially attributable to the development of right ventricular dysfunction following pulmonary artery banding. Provided that the criteria for patient selection and optimal tightness of the band are established, pulmonary artery banding can be a treatment option for infants with severe heart failure. Thus, pulmonary artery banding can help to alleviate the caregivers’ burden, mitigate health care costs, and equalize medical care nationwide.