Approximately 90% of primary cardiac tumors in children are benign, while 75% of cardiac tumors in adults are benign and 25% are malignant. More than 60% of all cardiac tumors in children is diagnosed with cardiac rhabdomyoma. Cardiac rhabdomyoma is commonly associated with tuberous sclerosis complex (TSC), which is an autosomal dominant disorder caused by mutations in either TSC1 gene or TSC2 gene. TSC is characterized by development of benign tumors in multiple organ systems, including the skin, the brain, the heart, the lungs, the kidney, and the liver. Cardiac rhabdomyomas are usually located in the ventricles, especially in the left ventricle, and show multiple lesions. In fetus, cardiac rhabdomyoma can be detected at 20 weeks of gestation. Multiple tumors in the ventricles detected by fetal ultrasound may suggest rhabdomyoma accompanied with TSC. Most children with cardiac rhabdomyomas are asymptomatic. In some occasions, murmurs, arrhythmias, or heart failure is noted. In particular, patients may present with congestive heart failure and rarely sudden cardiac death when a tumor is located at the left ventricular outflow tract. The majority of cardiac rhabdomyomas are known to regress spontaneously in utero and after birth. Because there is high probability of complete regression of the tumor during early childhood, the treatment strategy is usually conservative. In some patients, however, surgical resection is required when the tumor causes severe obstruction with hemodynamic compromise or lethal arrhythmias. Emerging recognition of mammalian target of rapamycin (mTOR) inhibitors in cardiac rhabdomyomas provides a targeted therapy. mTOR inhibitors appeared effective in treating TSC-associated tumors. Recent studies reported that systemic mTOR inhibitors would confer a clinical benefit in regression of cardiac rhabdomyomas in pediatric patients with TSC.

Recently, growing number of difficult cases with congenital heart disease can grow up and become adults. Function of right ventricle as a pump for pulmonary circulation has great impact on the quality of life, mortality, and the necessity of re-operation in such patients. Cardiovascular visualization and volume measurements, which previously required cardiac catheterization, can be obtained by non-invasive well-developed modalities such as computed tomography or magnetic resonance imaging. However, only cardiac catheterization can provide the direct pressure and oxygen saturation measurements for the irreplaceable assessment of cardiovascular function, and systemic and pulmonary circulation. This review summarizes and discusses the common and detailed evaluations, and future direction of right heart catheterization.

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).

Conclusions: 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.

A female neonate had left isomerism with absent pulmonary valve syndrome and unbalanced atrioventricular septal defect. She was intubated immediately after birth because of respiratory distress. On day 27, she underwent division of the pulmonary trunk and plication of the dilated pulmonary artery concomitantly with construction of a modified Blalock–Taussig shunt as the first palliation. She passed away due to an unknown cause at 8 months of age while waiting for the second stage surgery. We report this extremely rare combination of congenital heart malformations.

Isolated pulmonary artery of ductal origin (IPADO) is a rare congenital cardiovascular disease. We describe two patients with IPADO who were successfully treated with staged repair. The first patient with left IPADO and a closing duct underwent left original Blalock–Taussig shunt at 2 months of age, then, at 11 months old, the left pulmonary artery (PA) was connected to the main PA utilizing the subclavian arterial tissues with anterior augmentation placing an autologous pericardial patch. Right-to-left lung perfusion ratio was 2.05 postoperatively. The second patient with right IPADO and a closed duct underwent right modified Blalock–Taussig shunt (a 3 mm expanded polytetrafluoroethylene tube) at 29 days of age. The right PA was connected to the main PA at 5 months of age using a flap of the main PA wall and an autologous pericardial patch for anterior augmentation. Postoperative right-to-left lung perfusion ratio was 1.14. Staged repair provided balanced distribution of blood flow to the lungs. Reconstruction of the affected PA with the patient’s own vascular tissues, instead of prosthetic materials, was feasible, promising lifetime potential of growth.

Kawasaki disease (KD) is the most prevalent acquired vasculitis in children; coronary artery dilation and aneurysms are the most important complications of this disease. Alternatively, coronary artery dilation can be observed in rare congenital coronary artery anomalies, such as anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). At the age of 3, the patient suffered from typical KD, which was successfully treated with immunoglobulin infusion; however, right coronary dilation was noted at 4 days of illness. She was followed-up for the persistent right coronary artery dilation. At the age of 6, she complained of chest pain, and exercise stress test revealed ST segment depression in leads V3–V6, which resulted in the tentative diagnosis of coronary artery fistula. She was referred to our hospital for further evaluation and treatment. X-ray coronary computed tomography leads to the diagnosis of ALCAPA and stress myocardial scintigraphy confirmed ischemia of the anterolateral wall of the left ventricle; therefore, she underwent successful direct reimplantation of the anomalous left coronary artery to the aortic root. Here, we report the case of a patient with ALCAPA who was misdiagnosed as having coronary artery dilation associated with KD, and the patient was followed-up without appropriate treatment. We found that misdiagnosis of congenital anomalies can be a pitfall of echocardiographic evaluation of coronary arteries in patients with acute KD, and clinicians must be able to properly distinguish between both the plausible causes of dilation.

We report the first case of catheter closure using Amplatzer vascular plug device for the coronary artery fistula (CAF); draining from the right coronary artery (RCA) into the left ventricle (LV), quite rare pattern among (CAF). A 1-year old girl had a giant right coronary artery-left ventricular fistula. The angiographic computed tomography of coronary artery and the catheter examination showed a dilated RCA from the orifice, feeding the original coronary arteryʼs region and drained into the LV. Catheter treatment was judged as possible from its anatomy. We inserted a guide wire from the right femoral artery (FA) to RCA-LV-aorta, and grabbed it out with a snare catheter from the left FA to make a loop of guide wire. Occlusion tests were performed repeatedly using balloon to avoid ischemia. The optimal device position was selected cautiously and was detached. A few cases of catheter device closure of CAF had been reported in infancy. Because of its technical advance, the transcatheter closure of CAF has low mortality rate, shows rapid recovery, and can be an alternative option to surgical treatment in infant cases.