Myocarditis in children and adolescents has different courses. Infants and young children suffer more frequently from severe heart failure. In adolescence, on the other hand, patients are more likely to present with chest pain and preserved cardiac function. At the same time, the recovery rate is significantly higher. The influence of disease-associated genetic variants in myocarditis is now widely recognized. Such variants are more common in younger children and in patients with severe heart failure and a phenotype of dilated cardiomyopathy. However, genetic changes have also been described in children with myocarditis and preserved cardiac function, particularly in desmosomal genes. These genetic variants are associated with an increased number of cardiovascular events. This review aims to provide an overview of the impact of genetic variants on the clinical presentation and outcome of pediatric myocarditis.

Since the COVID-19 outbreak in Wuhan, China in early 2020, the pandemic has become a major global health concern. While vaccines are believed to be crucial for preventing COVID-19, there have been reports of vaccine-related cardiac events, particularly peri-myocarditis. In collaboration with the Taipei City Government, we conducted electrocardiogram (ECG) screenings to investigate the potential cardiovascular adverse effects of the BNT162b2 COVID-19 vaccine in school-aged students. Among the 7934 eligible students, 4928 (62.1%) completed both pre- and post-vaccine ECGs. The incidence of cardiac-related symptoms after the second dose was 17.1%, with palpitations and chest pain being the most common symptoms. ECG parameters showed a significant increase in heart rate and a decrease in QRS duration, QT, QTc, and QTcf intervals after vaccination. After comparing pre- and post-vaccine ECGs, 51 (1.03%) students were identified with significant ECG changes, with ST-T changes being the most common. Further evaluation revealed that most adverse events were mild and did not require hospital admission. Sensitivity and specificity analysis indicated that the serial comparison method was the most appropriate screening approach for post-vaccine cardiac adverse events. However, a cost-benefit analysis would be essential for further consideration. The study highlights the importance of paired ECG screening as a potential tool to serve as a reference for future medical events that may require screening.

The fields of pediatric cardiology and congenital heart surgery have made significant advancements over the past 70 years, reversing an earlier era of nearly universal mortality for all forms of congenital heart disease. Pediatric cardiac patients, particularly those with congenital heart disease, represent some of the most heterogeneous and complex patients to manage with an array of pathophysiologies. A highly functioning and collaborative team is required to care for this population effectively. Here we review the history of the field of pediatric cardiac critical care, the current system in the United States, and some additional considerations in the modern era.

The optimal treatment for unrepaired congenital heart disease in adults with pulmonary arterial hypertension (PAH) has not yet been clarified. We here report a case of a 42-year-old man who underwent successful surgical repair of isolated partial anomalous pulmonary venous connection (PAPVC) following target therapy for PAH. He presented with progressive exertional dyspnea. Enhanced computed tomography showed the right upper and middle pulmonary veins draining into the superior vena cava (SVC). Right heart catheterization showed that mean pulmonary arterial pressure (PAP) and pulmonary vascular resistance (PVR) were 91 mmHg and 20.3 Wood unit, respectively. He was subsequently treated with three specific PAH drugs (macitentan, tadalafil and selexipag) for 9 months. Follow-up catheterization showed that his mean PAP and PVR decreased to 45 mmHg and 2.7 Wood unit, respectively. Side effects of the PAH specific drugs, however, eventually caused poor medication adherence. As interruption of these drugs was likely to cause re-progression of PAH, the patient underwent surgical repair. The anomalous pulmonary veins were redirected into the left atrium through a surgically created atrial septal defect. The SVC was divided and reconstructed using a right atrial wall flap and an autologous pericardial patch. His exercise tolerance and PAH gradually improved thereafter. Catheter examination 15 months after surgery showed mean PAP was 34 mmHg. Specific target therapy for PAH could improve severe PAH related to isolated PAPVC in adults patients, and could make management of their circumstances easy and safe during the perioperative period.

Anomalous aortic origin of the right coronary artery (AAORCA) is a rare congenital anomaly. It is even rarer in the elderly, and there is ongoing controversy regarding techniques for surgical intervention. We describe a case of AAORCA that was successfully treated using a reimplantation technique. A 65-year-old female with a history of exercise-related angina was referred to our hospital. Enhanced computed tomography (CT) revealed AAORCA. Ischemia was detected on electrocardiogram exercise testing, although myocardial perfusion scintigraphy had failed to show it; thus, surgical intervention was recommended. We applied a reimplantation technique, because the right coronary artery (RCA) was not hypoplastic but its proximal course was interarterial with a slit-like ostium. We transected the RCA beyond the intramural portion and closed the residual stump proximally. An aortic wall was punched out higher in the right facing sinus of Valsalva to create a neo-ostium. The RCA was spatulated on the inferior side and anastomosed to the neo-ostium. The patient’s postoperative course was uneventful. Postoperative enhanced CT showed neither stenosis nor kinking of the reimplanted vessel. The patient has been asymptomatic for more than 2 years since the operation. The reimplantation technique was useful for AAORCA with a slit-like ostium and an interarterial course. Its medium- to long-term outcomes are reported to be satisfactory. Therefore, the reimplantation technique could easily be applied to similar cases.

Symptoms of coronavirus infectious disease 2019 (COVID-19) are usually mild in young patients. Some children, however, present with a significant degree of complications which may be associated with an excessive reaction by the immune system. Herein, we report an analysis of cytokine and chemokine in an 11-year-old girl diagnosed with left ventricular dysfunction and cardiac tamponade complicated with COVID-19. She recovered without complications after intravenous immunoglobulin, dexamethasone, remdesivir, and surgical pericardial drainage. Cytokine concentrations had markedly increased in the pericardial fluid specimen; especially for interleukin-6 being disproportionate to its serum concentration. Cytokine storm originating from the pericardial cavity was considered an underlying mechanism of her condition.

Anomalous origin of the right pulmonary artery from the ascending aorta is characterized by the right pulmonary artery arising from the right or posterior aspect of the ascending aorta. The anomalous right pulmonary artery is seldom connected to the pulmonary trunk. A premature baby was diagnosed with this malformation. The right pulmonary artery arose from the left aspect of the ascending aorta and additionally communicated with the pulmonary trunk via a small lumen just distal to its aortic origin. She underwent reimplantation of the right pulmonary artery to the pulmonary trunk at 13 days old. She remained asymptomatic at five years postoperatively. There were only three cases reported describing continuous tissues between the right pulmonary artery and the pulmonary trunk. In embryogenesis, imbalanced and incomplete septation of the aortic sac may result in this rare circumstance.

Congenital complete heart block (CCHB) occurs because of transplacental antibodies such as anti-SS-A and anti-SS-B. CCHB is a potentially fatal condition, in particular in very/extremely low birth weight infants. Coexisting structural heart disease is another significant risk factor for CCHB. The survival rates after pacemaker implantation, either temporary or permanent, remain low. To the best of our knowledge, no criteria have been established for the minimum weight and earliest gestational age for treatment thus far. Herein, we present a case of an extremely low birth weight infant (ELBWI) with CCHB caused by positive anti-SS-A/Ro antibodies of the mother and significant pulmonary valve stenosis. The infant underwent temporary pacing implantation on the day of birth with a weight of 850 g. Percutaneous balloon pulmonary valvuloplasty was performed at the age of 106 days when body weight 2.6 kg. This consecutive approach provided a favorable outcome. We propose that epicardial temporary pacing could avoid postnatal heart failure in ELBWI with CCHB even when structural heart disease was present.

Chylothorax is accumulation of chyle in the pleural space and is one of the common postoperative complications of congenital cardiac surgery in children. It is challenging to manage chylothorax, and the complication is associated with increased postoperative mortality and morbidity. The definitive treatment for chylothorax has not yet been established. Currently applied managements are mostly based on previous reports, including dietary adjustment, drug administration, and surgical procedures. Some reports showed that etilefrine, a sympathetic agent, was a potential treatment option for chylothorax, although it remains unclear whether this drug is effective and safe in treating chylothorax in patients with congenital heart disease. We administered etilefrine in four patients with congenital heart disease who developed chylothorax in the perioperative period. All four patients did not respond to dietary adjustments nor steroids, but required further treatment. Three patients seemed to have some improvement in chylothorax with addition of etilefrine to conventional treatment, while one showed insufficient treatment effect. Although some cardiovascular side effects, such as hypertension and arrhythmia, had been reported, no side effects were noted in simultaneous administration of etilefrine and inotropes in our series. This is the first report on etilefrine infusion to manage chylothorax in patients with congenital heart disease. Use of this drug might be a potential option for treating chylothorax.