Chylothorax, which occurs when chyle accumulates in the pleural space, is a common postoperative complication of congenital heart surgery. Recently, the number of neonatal cardiac surgeries or right heart bypass procedures has increased, thus leading to a corresponding increase in the incidence of chylothorax. Chyle is formed by chylomicrons that are transformed from long-chain triglycerides in the intestine. The diagnosis of chylothorax is confirmed by the presence of a triglyceride level of ＞110 mg/dL and ＞80％ lymphocytes per 1000 white blood cells/µL in a pleural aspirate. In congenital heart surgery, chylothorax typically results from three etiologies: 1) injury of a thoracic lymph duct, 2) elevation of systemic venous pressure due to thrombosis in the innominate vein or due to right heart bypass, and 3) congenial absence of a thoracic duct. Conservative management includes fat-free diets, middle-chain triglyceride milk, total parenteral nutrition, and treatment with somatostatin or octreotide. Invasive options include pleurodesis, thoracic duct ligation, or a pleuroperitoneal shunt. Although the optimal management of chylothorax remains controversial, evaluation of the central lymphatic system in pediatrics has drastically changed. For example, new management options, dynamic contrast-enhanced magnetic resonance lymphangiography, direct catheter interventions into the thoracic duct, and lymphatic venous anastomosis have been developed. Despite these, further improvements are needed in the treatment of chylothorax.

The human and financial costs of treating surgical site infections (SSIs) have been increasing. It has been estimated that approximately half of SSIs are preventable by using evidence-based strategies. The objective of this paper is to review the new and updated evidence-based recommendations for the prevention of SSIs, such as those of The Centers for Disease Control and Prevention (CDC, USA) guidelines for Prevention of SSIs published in 1999 and 2017. We mainly discuss the guidelines of 1999 and 2017 regarding preoperative preparation of patients, prophylactic antibiotics, intraoperative ventilation, aseptic and surgical technique, and postoperative incision care to identify differences between the older guideline and the new and updated recommendations to prevent unpleasant SSIs.

Fetal arrhythmia is a known cause of fetal heart failure that can lead to fetal death. Although assessment of the severity of heart failure is important for managing affected fetuses, it is often difficult in fetuses with arrhythmias. Usually, Doppler waveform, which is accepted in the cardiovascular profiling (CVP) score, is the most useful finding for assessing cardiac function. However, arrhythmia changes the Doppler waveform regardless of the cardiac function, so many of these waveforms may not reveal heart failure. Hence, other fetal echocardiographic findings, such as cardiomegaly assessed by the cardiothoracic area ratio, presence of atrioventricular valve regurgitation, and assessment of cardiac output calculated from the velocity-time integral, are used to detect early signs of cardiac failure. The Biophysical Profiling Score is another method useful for assessing the well-being of affected fetuses. Regarding the fetal heart rate, fetuses often develop heart failure with a fetal heart rate ＞220 bpm in fetal supraventricular tachycardia, ＞200 bpm in fetal ventricular tachycardia, and ＜55 bpm in fetal bradycardia. However, most fetuses with arrhythmia also have cardiac dysfunction, such as tachycardia-induced cardiomyopathy in fetal tachycardia and myocarditis caused by anti-SS-A antibody in fetal bradycardia. Hence, fetal heart rate may not reflect the severity of heart failure in many fetuses. Assessment by combining multiple assessment strategies and sequential follow-up of these findings are essential for assessing heart failure in fetuses with arrhythmias.

In recent years, there have been an increasing number of fetuses diagnosed by fetal echocardiography as having congenital heart disease (CHD). Fetal echocardiography in Japan is divided into 2 levels: Level 1–fetal heart screening and Level 2–detailed fetal heart examinations. Pediatric cardiologists are mainly responsible for Level 2 fetal echocardiography. As a result of improved diagnosis of Level 2 fetal echocardiography, both the diagnosis and “assessment of the severity of the disease” are now required. There are two categories of severity: 1) Severity classification by CHD type and 2) Severity determination in each case of the disease. For category 1, assessment can be made by reference to the CHD classification scale developed by Allan LD (in which the severity of fetal CHD is measured on a scale of 1 to 10) and/or to the fetal heart triage chart by Shiono et al. For category 2, it is necessary to recognize and measure the points for evaluating the differences in severity within each disease group. It is important for fetal cardiologists to understand such severity assessments, and it is now essential to become a certified Level 2 fetal echocardiologist.

The clinical characteristics of supraventricular tachycardia (SVT) in children differ from those in adults. SVT has a nonspecific presentation in neonates and infants with symptoms caused by heart failure and sometimes becomes severe because of delayed diagnosis. In older children, palpitations may be a subjective complaint, and most SVTs have a mild presentation. Tachycardia-induced cardiomyopathy secondary to incessant SVT is more often observed in children than in adults. Atrioventricular reciprocating tachycardia and atrioventricular nodal re-entrant tachycardia account for ＞90％ of cases of pediatric SVT, and ectopic atrial tachycardia and other SVTs are less common. The first step in the electrocardiographic diagnosis of SVT involves detection of the P wave, followed sequentially by assessment of P wave morphology and assessment of the time relationship between P waves and QRS complexes. Intravenous administration of adenosine triphosphate is useful for elucidation of the mechanism of SVTs. In hemodynamically unstable children, acute management includes performing immediate synchronized cardioversion. If the child is stable, performing a vagal maneuver and/or intravenous administration of adenosine triphosphate can achieve termination of most SVTs. Administration of second-line antiarrhythmic agents is necessary to treat intractable SVTs. This review discusses a practical approach to electrocardiographic diagnosis of this condition and provides a brief overview of recent information about acute management of pediatric SVT.

Inherited arrhythmia defines a group of diseases caused by mutations on genes encoding for ion-channel proteins and proteins that regulate ion channels. In 1957, long-QT syndrome was first reported and described as an inherited arrhythmia. Currently, catecholaminergic polymorphic ventricular tachycardia, Brugada syndrome, short-QT syndrome, early repolarization syndrome, and progressive cardiac conduction disturbance are also considered to be inherited arrhythmias. Although inherited arrhythmia is a major cause of sudden cardiac death in young individuals, it can be prevented by earlier diagnosis and clinical management. Progress in genetic analysis has enabled the diagnosis of inherited arrhythmias in patients with syncope and/or cardiac arrest of unknown origin and facilitated more suitable management of inherited arrhythmias. Guidelines for diagnosis and management of inherited arrhythmias have recently been published by the Japanese Circulation Society. Here on the basis of the guidelines, I review inherited arrhythmias with a particular focus on long- and short-QT syndromes, catecholaminergic polymorphic ventricular tachycardia, and Brugada syndrome.

Background: Increased opportunities for prenatal diagnosis of congenital heart disease (CHD) has also increased the importance of prenatal supports for their families. In Gunma Children’s Medical Center, we have provided prenatal supports for families including prenatal visits by pediatric intensive care unit (PICU) nurses.

Objective: Questionnaires were postnatally asked for mothers to whom CHD had been announced prenatally. We clarified their needs and evaluated the efficacy and improvements of prenatal visits by PICU nurses.

Methods: We studied 51 mothers who were referred to our institution because of the prenatal diagnosis of CHD and whose babies were admitted to PICU after birth. Answers for the questionnaire and clinical records were reviewed.

Results: Consents for this study were obtained from 23 subjects (45.1％). Among them, 19 subjects (82.6％) answered that prenatal visits were necessary, because their anxiety and stress could be reduced after their visits or they could know the atmosphere of PICU and care providers. However, mothers required how to become involved with practical child cares themselves.

Conclusion: Current prenatal visits could alleviate family’s anxiety for bearing a child with CHD. It is necessary to revise the information for prenatal visits and provide family care ensuring their needs.

Background: For children with congenital heart disease (CHD) resulting from trisomy 18, we do not perform intracardiac repair; instead, we opt for palliative surgery when their parents wish to proceed with surgical intervention.

Methods: From hospital medical records, we retrospectively identified 17 patients whose attending physicians had consulted our department between January 2010 and September 2016. We classified patients into two groups: those who required surgical intervention for CHD to promote transition to the home environment and who underwent surgery with parental consent (Group I: 5 patients), and those who did not require surgical intervention for CHD and did not undergo surgery (Group N: 12 patients). We compared the ease of transition to life in the patient’s own home between Group I and Group N.

Results: The median survival of the patients was 427 days in Group I and 173.5 days in Group N (p＝0.0534). The median number of days spent in the patients’ own homes was 647 days in Group I and 72 days in Group N (p＝0.0495).

Conclusion: Palliative surgery for CHD in patients with trisomy 18 extended the period of time that patients and their families could spend together in the home environment following hospital discharge.

First described in 1999, 22q11.2 duplication syndrome has a phenotype similar to that of 22q11.2 deletion syndrome. We present a case of 22q11.2 duplication diagnosed by fluorescence in situ hybridization (FISH) analysis performed to screen the genetic background of a rare congenital vascular anomaly, persistent 5th aortic arch. This male infant was born at 36 weeks of gestation with a birth weight of 1,770 g. Dysmorphic facial features were noted, and echocardiography revealed ventricular septal defect, atrial septal defect, persistent 5th aortic arch, aberrant right subclavian artery, and persistent left superior vena cava. Ligation of the ductus arteriosus was performed at age 13 days. Comparative genomic hybridization (CGH) microarray analysis revealed that the duplication was located at a typical 3-Mb region. Although persistent 5th aortic arch has been reported in patients with 22q11.2 deletion, this is the first case of 22q11.2 duplication complicated by 5th aortic arch. This case demonstrated that a gene dosage effect involving 22q11.2 could contribute to the development of persistent 5th aortic arch.

Neonatal myocarditis is most often caused by enterovirus and has a high mortality of ＞50％. We report a case of neonatal enterovirus myocarditis. The patient was born at term with a birth weight of 2，637 g. He developed fever on perinatal day 2, and a gallop rhythm was detected by auscultation on day 7. Based on severe left ventricular dysfunction and elevated troponin T, the diagnosis of acute myocarditis was made. Despite inotropic support, his cardiovascular condition deteriorated. He was transferred to a tertiary medical center on day 9 and was further treated with inotropes and mechanical ventilation. On day 30, he was weaned from hemodynamic support and transferred back to the local hospital. Enterovirus was detected in the blood sample taken on day 10 and was thought to be the cause of myocarditis. The test result for neutralizing antibody was negative. Prevention of viral infection during the perinatal period is crucial, given the severity of the disease, requirement of intensive care, and limited treatment options currently available.