The extracorporeal circulation is defined as a process where blood is drawn from the body and treated before it is returned to the body again. Usually through this procedure, it substitutes an organ’s function during the circulation outside the body. In this article, the basics of pediatric cardiopulmonary bypass (CPB) and extracorporeal membrane oxygenation (ECMO) is explained especially about the component of the circuit, pathology and complication.

Here we describe outcome-based clinical training on myocarditis based on the achievement level of the physicians as follows: epidemiology, symptoms, blood examinations, imaging, pharmacotherapy, treatment devices, and prognosis. The mortality rate of fatal myocarditis is 0.46 per 10 million patients. Fulminant myocarditis should be considered based on the clinical findings, including any neurological or gastric symptoms. It is useful in the identification of advanced myocarditis to observe temporal changes from electrocardiography or blood examinations. Echocardiography sometimes demonstrates poor systolic function or pericardial effusion, which are specific findings in advanced myocarditis, and are also easy findings for residents to understand. Magnetic resonance imaging and nuclear medicine are minimally invasive diagnostic modalities and are available for the evaluation of impaired myocardium during the clinical course after the acute phase. Electrophysiological studies can be performed on patients if they still have arrhythmias during the convalescent phase. Venoatrial extracorporeal membrane oxygenation should be initiated using clear criteria from each institution for weaning off the device if the patients suffer from low output or fatal arrhythmias. The utility of intraaortic balloon pumping for children is still controversial. We have no clear consensus concerning the usefulness of steroid or immunoglobulin therapy. Patients who develop fulminant myocarditis do not always have an unfavorable prognosis if they can survive sudden terrible disease in acute phase.

The purpose of this article is to review the current modalities available to assess fetal cardiac function. It covers the methods generally used in ex-utero life, and the unique methods used during in-utero life, such as Doppler of the umbilical artery or vein. Because of a lack of physical findings and other modalities available to evaluate fetal cardiac function, echocardiogram has great importance, and this review can help with the better fetal management and prenatal counseling.

In Japan, there has recently been a marked increase in fetal heart diagnosis concomitant with an overall increase in the diagnosis of prenatal diseases. With advances in imaging technology, congenital heart disease, which previously was difficult to diagnose in the fetus, can now be successfully diagnosed prenatally. Furthermore, it is now possible to evaluate cardiac function and predict prognosis after birth. In this article, I identify new indicators in fetal echocardiography and provide comprehensive yet concise commentaries as follows: (i) cardiac function assessment by tissue Doppler imaging (TDI), myocardial performance (Tei) index, and cardiovascular profile score (CVPS); (ii) anatomical diagnosis by I-shaped sign, post LA space index (PLAS index), and U-sign; and (iii) prediction of the status and prognosis after birth by assessment of pulmonary venous flow pattern and pulsatility index (PI) during maternal hyperoxygenation testing in hypoplastic left heart syndrome, foramen ovale, and the ductus arteriosus in transposition of great arteries.

Cardiomyocytes are terminal differentiated and thus, once damaged, cannot be substantially replaced by proliferation of surrounding cells. Stem cell therapy, i.e., replacement therapy, has long been viewed as a new and effective approach for treatment of myocardial infarction and severe heart failure; however, clinical establishment of stem cell therapy has presented significant challenges due to the difficulties in efficient isolation and transplantation of cardiac-committed stem cells. This review summarizes the history, current overview, and future perspectives in cardiac stem cell research.

Barth syndrome is an X-linked disorder characterized by cardiomyopathy, neutropenia, skeletal myopathy, growth delay, and increased urinary excretion of 3-methylglutaconic acid. The disorder is caused by mutations in the TAZ gene at Xq28 that result in cardiolipin deficiency and abnormal mitochondria. Cardiac involvement is the most common feature of this syndrome, which usually includes dilated cardiomyopathy or left ventricular noncompaction, and less commonly includes endocardial fibroelastosis or hypertrophic cardiomyopathy. X-linked infantile cardiomyopathies with mutations in the TAZ gene but without Barth syndrome-related symptoms, such as neutropenia and skeletal myopathy, have been reported and postulated to be allelic variants of Barth syndrome. Heart failure is a leading cause of death in such patients, with the highest incidence during the first 6 months of life. In contrast, many patients appear to respond to conventional medical therapy, and cardiac function usually normalizes after 3 years of age. Although Barth syndrome is associated with an increased risk of ventricular arrhythmia, the prognosis of most patients is good after 5 years of age when cardiac function subsequently recovers during infancy. Therefore, early diagnosis and management, with recognition of a wide range of phenotypes, are important for improving the prognosis of Barth syndrome.

Background: In many cases of cyanotic congenital heart disease with pulmonary atresia or stenosis, the ascending aorta is larger than normal. This enlarged aorta reduces the retroaortic space and makes corrective surgery difficult. To overcome this problem, we devised an aortic extension using only the self-aortic tissue.

Methods: Between 2005 and 2013, we performed this procedure in five patients: four with a functional single ventricle and one with pulmonary atresia and ventricular septal defect. Operative data, prognosis, and change in the aortic diameter were examined postoperatively.

Results: Mean patient age at the time of surgery was 18.2±7.6 months (range, from 7 months to 2 years) and mean body weight was 8.4±0.9 (range, 7.1–10.0) kg. The aortic extension procedure was performed in approximately 30 min in all cases. The ratio of the maximum to peripheral diameter of the ascending aorta was 1.64±0.22 preoperatively and was significantly reduced to 1.01±0.36 postoperatively. No stenosis or re-enlargement was observed over 53.8±38.3 (range, 32–130) months postoperatively.

Conclusion: This aortic extension procedure without the use of a prosthetic graft or autograft enlarges the retroaortic space by extending and plicating the ascending aorta and allows decompression of the central pulmonary artery and bronchus. It also has potential for growth of the aorta.

We report a rare case of sudden cardiac death associated with acute rheumatic fever (ARF). The patient was a 12-year-old boy referred to our hospital because of sudden cardiopulmonary arrest. Resuscitation failed and autopsy was performed. Pathological findings revealed diffuse pancarditis, which was prominent in the conduction systems, particularly around the atrioventricular node and disseminated Aschoff bodies in various parts, including the valves, myocardium, and endocardium. These findings strongly suggested that his death was caused by atrioventricular block associated with ARF. Although ARF is a common disease in developing countries, it is rare in Japan, with the reported annual incidence of 4–10 cases a year. ARF should be considered in the differential diagnosis of febrile illnesses, particularly in the era when routine antibiotic administration is not recommended.

We report on a patient with protein-losing enteropathy (PLE) after undergoing total cavopulmonary connection (TCPC) operation who was introduced to subcutaneous immunoglobulin (SCIG) home therapy as immunoglobulin G (IgG) replacement therapy. The patient was a 21-year-old man with hypoplastic left heart syndrome who developed edema and ascites secondary to PLE 5 years after the TCPC operation. When he was 15-years-old, intravenous immunoglobulin (IVIG) was administered for the treatment of hypogammaglobulinemia. SCIG home therapy was introduced when he was 20 years old. The serum IgG level did not increase with the initial SCIG dose (8 g/week); therefore, we increased the SCIG dose (16 g/week). After SCIG administration, the serum albumin level was reduced from 2.7 to 2.4 g/dL, IgG level increased from 370 to 484 mg/dL, and the total duration of the hospital stay was shortened from 4.7 to 1.2 d/month. With regard to adverse events, a local reaction was observed at the subcutaneous infusion site, but this improved with time. These findings suggest that SCIG home therapy is effective in increasing and maintaining serum IgG levels and avoiding hospitalization. The appropriate SCIG dose for patients with PLE is unknown. We believe that this should be considered on a case-by-case basis depending on the severity of each case.

We report the case of a newborn who showed rapid regression of cardiac rhabdomyomas associated with tuberous sclerosis (TS) following everolimus therapy. The newborn was diagnosed with TS based on fetal echocardiography and fetal magnetic resonance imaging that showed multiple cardiac rhabdomyomas, a subependymal nodule, and multiple retinal hamartomas. One of the cardiac rhabdomyomas was very large, but there was no evidence of severe inflow or outflow tract obstruction, valvular dysfunction, or arrhythmia. His condition was stable for 10 days after birth. However, the newborn suffered circulatory collapse on the 11th day of life. He required intensive care, including mechanical ventilation and infusions of milrinone. Everolimus therapy was started on the 19th day of life as a rescue treatment. Echocardiography showed rapid regression of cardiac rhabdomyomas and improvement of cardiac function within a few weeks. This case demonstrated the efficacy of everolimus therapy for TS-related cardiac rhabdomyomas.