Congenital heart disease is a common condition in children, and general pediatricians are frequently called in to access the hemodynamics of the disease. The role of circulation is to deliver enough oxygen, taken up by the lungs, to each organ in the body while maintaining cardiac output for that purpose. If there are defects or shunts in the heart or between large blood vessels, the direction and amount of the shunt are defined by the difference in systemic and pulmonary vascular resistance in posttricuspid shunts (ventricular septal defect, patent ductus arteriosus, etc.) and by the difference in compliance between both ventricles in pretricuspid shunts (atrial septal defect). When it comes to managing various congenital heart diseases, it is simple to understand whether the disease is classified by an increase or decrease in pulmonary blood flow. When a general pediatrician encounters a patient with undiagnosed congenital heart disease, the least they should do is determine whether the disease requires patent ductus arteriosus and whether oxygen administration is necessary. Although echocardiography is the simplest and most useful tool for evaluating children’s hemodynamics at bedside, pediatricians should make a comprehensive decision by including other modalities.

Many congenital heart diseases are now diagnosed prenatally in Japan, thanks to advances in fetal echocardiography. However, there are still many cases where the diagnosis is made shortly after birth. This report describes counseling for parents whose children have been diagnosed with congenital heart disease. First, the purpose, practice, content, and family support of counseling for prenatal diagnosis are described. The anxiety of the expecting parents and uncertainty of the diagnosis should be carefully considered during counseling. Second, counseling for a diagnosis that is described immediately after birth can come as a surprise to the new parents, but it is possible to make a definitive diagnosis. The two scenarios are discussed. Pediatric cardiologists must have not only the clinical skills necessary to accurately diagnose heart disease but also the counseling skills necessary to provide support to the child’s parents for both prenatal and postnatal diagnoses. It is also important for pediatric cardiologists to understand their roles as counselors and team leaders.

Background: In recent years, atrial septal defect has been the most common congenital heart disease discovered during school heart disease screening. Therefore, we carefully examine the electrocardiogram (ECG) of incomplete right bundle branch block (IRBBB) pattern in lead V₁, particularly the rsR′ type. The rsR′ pattern, however, frequently disappeared at the secondary screening. The presence of a deep Q wave in lead V₆ (QV₆), which is a screening criterion for heart disease, occasionally disappeared at the secondary screening. The findings were investigated because they were thought to be the lead misplacement.

Methods: The subjects included 14 students who showed rsR′ pattern in lead V₁ and 3 students who had an ECG finding of deep QV₆ (≧0.5 mV). Their ECGs were recorded in both the standard and upward displaced positions on the chest. Furthermore, synthesized vectorcardiography was simultaneously recorded.

Results: At the secondary screening, the finding of IRBBB pattern of rsR′ type had vanished in 14 students and an atypical IRBBB pattern or normal wave form was discovered. When the chest leads were displaced to the upper position, the rsR′ pattern appeared. At the secondary screening, deep QV₆ amplitude decreased to ＜0.5 mV in 3 students. When the chest electrodes were positioned upward by one intercostal space, deep QV₆ appeared.

Conclusions: These findings were thought to be the result of precordial lead displacement. They could be because the amplitude of waves in unipolar leads changed in inverse proportion to the square of the distance between an electrode and the heart.

Objective: Carnitine is essential for long-chain fatty acid metabolism in order to produce biological energy via the mitochondria. Carnitine deficiency can result in cardiomyopathy, hypoglycemia, and other complications. This study aimed to determine how plasma carnitine levels changed before and after open-heart surgery with cardiopulmonary bypass (CPB) in children with congenital heart disease.

Methods: We measured free carnitine (C0) levels in the blood before and after CPB, as well as on each day 7 days after the procedure.

Results: We treated 50 patients with average age of 35±49 months. We identified 19 patients with extracardiac complications, including eight with chromosomal disorder, three with suspected chromosomal disorder, four preterm births, two with digestive disorders, and two with other complications. C0 levels immediately before CPB were 55.8±24.4 nmol/mL, whereas immediately after CPB were significantly lower [33.5±12.9 nmol/mL (the ratio of C0 level before and after CPB were: 64±19％, p＜0.01)]. Although there was no significant difference in aortic clamp time, CPB time, or pre-operative blood data, the presence of extracardiac complications was significantly correlated to the C0 level just after CPB (odds ratio, OR: 3.385, 95％ confidence interval, CI: 1.858–3.385, p＜0.01).

Conclusion: C0 dropped temporarily after CPB and then returned three days later. Since the decrease in C0 after CPB was linked to extracardiac complications in children with congenital heart disease, monitoring pre- and post-operative C0 concentration may be warranted in these patients.

We present a case of a healthy 7-month-old female infant who developed sudden left heart failure due to acute mitral regurgitation (MR). She was rushed to our hospital on the day of onset and underwent semiemergency surgery the next day. Antimicrobial treatment was initiated upon admission, and pyrexia occurred 8 h later. At surgery, the anatomical findings included the destruction of both the anterior and posterior leaflets of the mitral valve, posterior mitral valve leaflet perforation, an undetected rupture of the chordae tendineae, and no vegetation on the mitral valve. Mitral valve replacement was performed because of the difficulty of mitral valve annuloplasty. A culture test of blood and resected anterior mitral valve demonstrated no bacterial or fungal infection, but histopathological analysis revealed polymorphonuclear cell infiltration of the resected mitral valve leaflet. The patient was diagnosed with infective endocarditis (IE) based on these findings; however, we were unable to determine the cause of infection or pathogenic bacteria. Acute MR in infants can be caused by IE and acute rupture of the chordae tendineae of the mitral valve (RCTMV). The current case of infantile IE started with acute MR due to significant valvular destruction, followed by pyrexia, and progressed quickly, similar to RCTMV in infants. Because the treatment and complications of acute MR in healthy infants are dependent on the cause, we must take special care to ascertain the cause along with histopathological analysis.

Scimitar syndrome is an uncommon congenital heart and lung abnormality, characterized by an aberrant pulmonary venous return to the inferior vena cava, often from the right lung. The clinical manifestation varies greatly in terms of severity and phenotype. We recently observed familial scimitar syndrome, which had both positive and negative outcomes. Besides six additional cases of scimitar syndrome, we investigated risk factors for death. Because of hypoplasia of the right pulmonary artery or lung itself, all infantile cases had heart symptoms before the age of 1 month and concomitant congenital heart disorder was more common. Intracardiac repair or Norwood operation were performed in four of six infants, whereas Norwood operation was deemed contraindicated in one of the remaining two infants. Three of these operative cases died, of whom complex congenital heart diseases were seen in two cases. Airway malformations were seen in all of these fatal cases. Two of six infantile cases had the pulmonary vascular obstructive disease, and one died from an airway deformity. Furthermore, in situations of mortality, the region of the lung supplied by aortopulmonary collateral blood vessels are larger. Our study revealed that infantile-onset of the symptoms of heart failure, the presence of complex congenital heart disease, pulmonary vascular obstructive disease, the presence of congestive heart failure due to aortopulmonary collateral blood vessels, and airway malformations were risk factors for mortality in patients with scimitar syndrome.

For infants with coarctation of the aorta (COA), either transcatheter balloon angioplasty (BA) or surgical repair is used. COA recurs frequently in low-birth-weight infants who have had either BA or surgical repair. We present a case of a baby girl born at 35 weeks gestational age and weighing 1,374 g who underwent successful surgical repair of COA following palliative BA. A systemic blood pressure gradient of 64 mmHg between the upper and lower limbs was observed at the age of 6 days, confirming the diagnosis of COA. At the age of 25 days, we conducted BA for COA with a minimum diameter of 1.3 mm. Following the placement of a 3-French sheath in the femoral artery, we performed a predilatation with a coronary angioplasty balloon (NC TREK^®2 mm) and a second dilatation with a valvuloplasty balloon (TMP-PED^®4 mm). The systemic blood pressure difference between the upper and lower limbs was reduced to 9 mmHg after successful dilation. As a recurrence of COA developed along with body weight gain, coarctation resection and extended end-to-end anastomosis were performed at the age of 108 days and weight of 3,050 g. At the age of 7 months, there was no recurrence of COA. We believe that delayed surgical repair after palliative BA may be possible in low-birth-weight infants with COA to avoid recurrence of COA.

In Japan, there has yet to be a report of a leadless pacemaker (LPM) implant in a pediatric patient. We present a case of a pediatric patient who underwent LPM implantation for various reasons. A 12-year-old girl suffered rejection for heart transplantation that was performed for the treatment of dilated cardiomyopathy 2 years after the procedure. The rejection caused sinus node dysfunction syndrome, which necessitated pacemaker (PM) therapy. However, bilateral subclavian vein obstruction, invasiveness, and risk of device infection under immunosuppressive therapy made implanting either intravenous or epicardial PM difficult. The disadvantages of LPM, such as the limited number of reimplantation and the limited setting to ventricular single chamber pacing, were deemed insignificant because of the possibility of heart reimplantation and transient bradycardia attacks. Although vascular access was a concern because of the small body size, the LPM introducer sheath could be safely placed with adequate prior evaluation and gradual dilation of the femoral vein. There were no complications during or after the implantation procedure, and the LPM was able to work with bradycardia. A month later, we performed a catheter biopsy and successfully sampled the myocardium. With careful consideration of our indications, our experience suggested that LPM can be safe and useful in pediatrics.