As the number of adults with congenital heart disease (ACHD) increases, the number of cardiovascular electronic implantable devices is also increasing. The selection of leads and implantation methods is critical for patients with the unique heart structures of CHD. Additionally, new technology has been developed recently and is being used for adults with CHD. This paper describes recent advances in the field of implantable cardiac electronic devices that can be utilized in adults with CHD.

Currently, most patients with congenital heart disease (CHD) reach adulthood, and heart failure (HF) is one of the leading causes of death for adult patients with CHD. Therefore, an appropriate treatment strategy for HF is essential for improving their prognosis. Medical therapy for HF is as crucial as surgical or catheter-related therapy. Recently, novel agents for treating HF have emerged that have dramatically changed the strategy for HF therapy. Thus, updating knowledge of these therapeutic agents, both standard and novel, is vital. Hopefully, this article will be helpful in daily practice.

Atrial septal defects (ASDs) are common congenital heart defects that can remain undiagnosed until adulthood among asymptomatic patients. However, due to the prolonged volume overload of the right heart and increased pulmonary arterial blood flow, ASDs in adult patients are often complicated by heart failure, atrial fibrillation, and/or pulmonary hypertension. Recent advancements in transcatheter ASD closure, catheter ablation of atrial fibrillation, and the intravenous and oral treatment of pulmonary hypertension have provided opportunities for the treatment of patients with complex ASD regardless of their age.

In recent years, fetal ultrasound screening has become widespread, leading to most congenital diseases being diagnosed prenatally. Nonetheless, some cases still progress to fetal heart failure leading to fetal death during their uterine period, while others with congenital heart disease (CHD) still experience severe deterioration, making survival difficult even with intensive postnatal therapy. Various prenatal interventions for these diseases have been implemented mainly in Europe and the United States, and there are many reports concerning their outcomes and effects on prognostic improvement. In Japan, clinical studies and trials on fetal interventions for severe CHD (e.g., treatments for fetal arrhythmias and severe aortic valve stenosis) have continued to be conducted and steadily implemented. Conversely, there have yet been no reports in Japan on the administration of nonsteroidal anti-inflammatory drugs for Ebstein disease with circular shunt, which can advance in the near future. There are ethical issues surrounding maternal and fetal health and the medical insurance system that need to be addressed through multidisciplinary collaboration. Nonetheless, we expect our fetal cardiac interventions and therapies to be able to match international standards.

Infectious aneurysms are rare complications of the modified Blalock–Taussig (mBT) shunt procedure. These aneurysms may have fatal outcomes and require prompt treatment; however, standardized treatment is lacking. This is a report of a successful case in which an infectious aneurysm that developed following mBT shunt placement was treated safely with a hybrid procedure involving catheterization. The patient was a 5-month-old boy who underwent left mBT shunt placement for tetralogy of Fallot. He subsequently presented with decreased oxygenation, and contrast-enhanced computed tomography revealed an aneurysm at the shunt insertion site. Blood culture was positive for Staphylococcus capitis, suggesting that the infectious aneurysm was caused by the bacterium. Antibiotic treatment alone was insufficient; and catheter removal of the infectious lesion was considered necessary. Due to the risk of rupture and aneurysmal bleeding during sternotomy, a blood vessel entering the aneurysm was obstructed using an indwelling balloon catheter. The aneurysm was removed after blood flow was completely blocked, and a right ventricular outflow tract repair was performed concomitantly to secure new pulmonary blood flow. The patient was discharged from the hospital 56 days postoperatively. This case demonstrates a promising adjunctive procedure for the treatment of infectious aneurysms.

A 16-year-old girl, who noted palpitations, and tightness in her chest, was referred to our hospital with a diagnosis of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). She underwent surgery using the Takeuchi method without difficulty, but she had respiratory distress and decreased cardiac function following surgery. Her symptoms improved rapidly after treatment with bisoprolol. Cardiac catheterization, performed to identify the cause of her symptoms, confirmed congestion of blood flow within the coronary vessels and regurgitation of right and left coronary blood flow into the ascending aorta. Although there are few reports discussing the cause of cardiac dysfunction in adults with ALCAPA, congestion of blood flow within the coronary vessels may cause postoperative cardiac dysfunction. Preoperative coronary sinus evaluation may identify a possible cause of postoperative cardiac dysfunction.

A 3-year-old, female patient required venous-arterial extracorporeal membrane oxygenation (VA-ECMO) for ischemic cardiomyopathy. Conversion from ECMO to a ventricular assist device was needed due to failure to wean from ECMO. The patient was therefore transported to another hospital 520 km away via ground transport. We used our own ambulance which was specially equipped for pediatric ECMO transportation. Estimation of the total electricity and oxygen consumption during transportation indicated that these were well within safety parameters. On the day prior to transport, the perfusion cannula site was moved from the ascending aorta to the right common carotid artery, and the depth of the drainage cannula was increased to minimize the risk of accidental dislodgement. The patient was secured, along with the medical equipment, onto a single backboard in the ambulance. The transport team was divided into two groups to monitor the patient in shifts. The patient was transported without any adverse events. Precautions should be taken to avoid potentially life-threatening but preventable events such as difficulties with medical equipment and accidental dislodgement of ECMO cannulas. Pediatric ECMO has a particularly high risk of accidental cannula dislodgement during transport due to vibrations and acceleration. It is important to prepare for these contingencies and to assemble a team capable of responding to them. Since there are few pediatric ECMO cases in Japan, more data is urgently needed to standardize the transport process.