The Japanese school-based cardiovascular screening system began in 1973 with the revision of the Enforcement Regulations of the School Health Law by the Ministry of Education, Science and Culture. It was not until December 1994 that all of the first, seventh, and tenth graders were required to undergo electrocardiography. This nationwide cardiac screening system, which is unique in the world, has led to early detection of heart disease and prevention of sudden cardiac deaths. The current decrease in sudden cardiac deaths in children results from changes in diagnostic and therapeutic devices and changes in guidelines over time. In this paper, we discuss the latest guidelines and the status of pediatric sudden deaths in Japan to increase awareness and aid in the goal of achieving zero pediatric sudden cardiac deaths. Furthermore, we also diagnose hypertrophic cardiomyopathy as it can be successfully detected early by cardiac screening.

Pediatric cardiology has changed greatly in a few decades, along with the spread of prenatal diagnosis of congenital heart disease (CHD). The improvement of prenatal detection rate and its accuracy resulted in optimal outcomes after birth. However, the detection rate has been still low in some CHDs, such as isolated total abnormal pulmonary venous connection. In the future, increasing the detection rate of these CHDs will be a challenge. Proper neonatal management based on fetal diagnosis and fetal intervention is also an issue to be addressed. Fetal catheter intervention is an emerging field in Japan, and it is necessary to identify the appropriate patients and confirm the safety and efficacy of this treatment. This review article describes the basics of fetal diagnosis, echocardiography, and fetal intervention, based on the author’s experience, research, and previous articles. We hope that this article can help you in your practice.

Common atrioventricular valve regurgitation in functional single ventricle associated with heterotaxy syndrome and pulmonary venous obstruction should be repaired to achieve Fontan operation and improve life prognosis. Common atrioventricular valve repair consists mainly of bivalvation with edge-to-edge suturing of both bridging leaflets and bridging strip, combined with cleft suture, commissuroplasty, or partial annuloplasty. Valve replacement was occasionally performed, but the therapeutic effect remains controversial. Valve repair is preferred to be performed therapeutically or prophylactically at the timing of bidirectional Glenn. During the past decade, outcomes have improved along with the development of diagnostic technologies. However, the outcomes of valve repair before bidirectional Glenn are still dismal. Thus, the development of a small-caliber tissue-engineered heart valve with growth potential, increasing heart or heart/lung transplantation, and further improvement of repair technique per se are required.

Congenital tracheal stenosis is caused by complete cartilaginous rings and leads to obstructive respiratory disease. Most patients with this condition experience respiratory symptoms from the neonatal or infantile period. In more than half of these patients, the condition is associated with a congenital cardiovascular defect, especially a pulmonary artery sling. Cardiovascular procedures for treating this condition are performed often. However, its complicated pathology, involving interactions between the respiratory and circulatory systems, poses a challenge to the pediatric cardiologist and cardiovascular surgeon. Therefore, a multidisciplinary team-based approach based on cooperation between pulmonologists, pediatric surgeons, anesthesiologists, neonatologists, and intensivists is highly recommended. Thus, this review provides an outline of congenital tracheal stenosis from the embryological, anatomical, and pathophysiological perspectives, with special attention to possible complications of congenital cardiovascular defects. Furthermore, it provides some recommendations regarding surgical techniques.

In pediatric cardiology, many situations require analysis of right ventricular function, but accurate measurement and evaluation of right ventricular function are not easy. The pathophysiology of right heart failure involves three mechanisms: pressure overload, volume overload, and myocardial dysfunction. In practice, however, these mechanisms often overlap to varying degrees. The most frequent and important cause of right ventricular dysfunction is pulmonary hypertension. Assessment of right ventricular dysfunction due to pulmonary hypertension may serve not only as a prognostic factor in this disease, but also as a baseline study that can be applied and inferred in other diseases. Thus, assessment of right ventricular dysfunction in pressure overload can serve as a pivotal study for universal analysis of right ventricular behavior.

In this review, we describe the structure and function of the normal right ventricle, followed by an explanation of the evaluation method of right ventricular dysfunction. We explain that right ventricular dysfunction includes not only changes in the contractile function of the right ventricle as a whole, but also impairments in contractile style, right ventricular-pulmonary coupling, synchrony, interventricular interaction, and diastolic capacity.

Chest pain due to cardiovascular disease is rare in children. It is important to distinguish between angina and myocarditis in the outpatient department. A 9-year-old boy was presented with chest and abdominal pain, which makes it difficult to diagnose coronary spastic angina from clinically relevant myocarditis. The patient was treated for an asthma attack two weeks ago. He had experienced right chest pain for two days. He complained of right-sided abdominal pain at midnight and consulted his family doctor. He was referred to our hospital on suspicion of acute myocarditis because of a high C-reactive protein level and troponin T positivity. During the chest pain, the electrocardiogram (ECG) exhibited ST-segment elevation in the inferior wall leads and V4-6, and a sublingual nitroglycerin spray improved the symptoms and ECG findings. No abnormalities were found on coronary angiography, and no contrast effects were observed on delayed contrast cardiac MRI, leading to a diagnosis of coronary spastic angina. It is difficult to differentiate coronary spastic angina in children from clinically relevant myocarditis due to limitations of the testing methods, so it can be difficult to diagnose and treat according to the guidelines for the diagnosis of coronary spastic angina.

A 14-year-old girl with recurrent episodes of transient loss of consciousness (T-LOC) during physical activities was presented to our hospital accompanied by her mother. She was started on lamotrigine on suspicion of epilepsy. Further investigations were conducted since there was no improvement. The electrophysiology study was normal, but the cardiac computed tomography (CT) showed an abnormal origin of the right coronary artery, which was interarterial (running between the aorta and pulmonary artery but had no intramural course). The exercise stress test revealed no evidence of myocardial ischemia. Electroencephalography (EEG) findings were atypical, and the T-LOC episodes during physical activity, which are rare in epileptic seizures, made it difficult to differentiate between cardiogenic syncope and epilepsy. Therefore, an insertable cardiac monitor (ICM) was implanted. After implantation, the ICM recordings showed that the T-LOC episodes were not accompanied by any arrhythmias. However, electromyography (EMG) activity was suspicious of a seizure. Based on these results and the clinical findings, we concluded that the T-LOC was due to focal to bilateral tonic-clonic seizures (FBTCS), and concomitant use of lacosamide helped in marked relief of the patient’s symptoms. ICM proves to be effective in children with T-LOC that is difficult to diagnose.

No effective treatments currently exist for patients who have undergone the Fontan procedure with pulmonary vein occlusion (PVO), and the prognosis is poor. We present a case of a 3-year-old girl with acute heart failure who developed right PVO after undergoing the Fontan procedure, and a large amount of pleural effusion, edema, and atrial arrhythmia was noted. Fenestration was created after embolization of the minor aortopulmonary collateral arteries (mAPCAs) and right pulmonary (RPA) artery. The embolization of mAPCAs was divided into 3 sessions using 51 coils. For RPA embolization, 5 Amplatzer Vascular Plugs and 2 coils were used. The duration of this procedure was approximately 3 h, and complete occlusion of RPA was achieved without complications. Subsequently, fenestration creation was performed, and the central venous pressure decreased from 20 mmHg to 7 mmHg. However, the patient deceased because of cardiac tamponade on day 9 postoperatively. To our knowledge, there have been no reports on the complete embolization of the mAPCAs and PA in patients who have undergone the Fontan procedure with PVO. This treatment temporarily stabilized the hemodynamics in patients who have undergone the Fontan procedure with PVO.