In the perioperative management of Fontan operation, it is important to preserve low pulmonary vascular resistance (PVR). In a normal heart, the pulmonary circuit and systemic circuit are connected in series and both circuits have respective ejection ventricles. On the other hand, in Fontan circulation, only systemic circuit has an ejection ventricle although the pulmonary circuit is maintained by a simple pressure gradient from the systemic vein to the left atrium without an ejection ventricle. Therefore, high systemic venous pressure is required to maintain the pulmonary circulation. Hence, low PVR is essential to obtain a stable Fontan circulation. It is necessary to understand the mechanics and dynamics of Fontan circulation in depth to achieve an ideal perioperative management of Fontan operation.

Short QT syndrome (SQTS) is a hereditary lethal arrhythmia; it was first described in 2000. SQTS is characterized by an abnormal short QT interval on electrocardiogram (ECG), and causes arrhythmia, such as ventricular fibrillation and atrial fibrillation. Although clinical characteristics of SQTS have not been appropriately elucidated, several victims, individuals in the teenage and those in their 20s, have been reported. Therefore, SQTS diagnosis at an early age is important and is recognized as a concern in a school screening program for heart disease. According to an expert consensus statement published in 2013, even an asymptomatic individual with a short QT interval, who has neither a family history nor a pathogenic gene mutation, can be diagnosed with SQTS. Studies with various adult populations have identified individuals who met the SQTS diagnostic criteria; however, these patients were asymptomatic and did not have a poor prognosis. Some asymptomatic children and adolescents with SQTS were also identified in our study, in which we mainly focused on a school screening program for heart disease. Risk stratification of SQTS is necessary because clinical characteristics of SQTS are variable, including no symptoms and sudden cardiac death. Hence, we compared the ECGs of asymptomatic individuals with a short QT interval in a school screening program for heart disease with those of previously reported symptomatic patients with SQTS, to screen for SQTS patients with a high risk. A prospective study thus needs to be conducted to elucidate the prognosis of asymptomatic patients with SQTS who are diagnosed in their childhood.

Background: Working is one of the important concerns in transitional care for adults with congenital heart disease (ACHD) because, apart from offering them financial autonomy, it also connects them with the society. Hence, this study aimed to explore the employment status of ACHD by sex and the factors influencing the employment status.

Methods: In this study, 193 Japanese ACHD patients (mean age: male 33.62 years/female 32.69 years, 89 males, no students included) completed the patients’ characteristics form, including the employment status and the degree of impact on their ability to work due to heart disease and also completed the quality of life (QOL) form, comprising the Linear Analog Scale and the Satisfaction with Life Scale (SWLS).

Results: In the study group, 13 (14.6％) of 89 male patients and 13 (12.5％) of 104 female patients did not have a job. These rates were higher than the national standard values in Japan (male, 5.0％; female, 2.9％). Among those patients, only one male and one female stated their illness as a reason for their unemployment. The main factor of males’ unemployment was young age, whereas for females, it was related to their disease complexity. Unemployed patients showed significantly lower scores in QOL and the SWLS.

Conclusion: Although most ACHD patients can join the workforce, a higher percentage of ACHD patients do not work and find it challenging to have a career than regular people. Therefore, as unemployed patients have a low QOL and SWLS, it is clear that work is crucial for them to have a mentally and emotionally stable and fulfilling life.

Background: Atrioventricular valve regurgitation (AVVR) has a notable impact on the prognosis of patients with hypoplastic left heart syndrome (HLHS) and right atrial isomerism (RAI).

Methods: The study population comprised two groups: (1) HLHS and RAI group: n＝32 (HLHS＝15, RAI＝17), and (2) normal control group: n＝53. X-plane images of apical four-chamber view and orthogonal plane cutting through the center of annulus were acquired using transthoracic matrix array probe. Speckle-tracking of the two opposing points on the annulus in four-chamber plane and orthogonal plane was performed, and the distances of respective opposing points were consecutively measured along the cardiac cycle. Atrioventricular valve (AVV) area dynamics were classified into three categories according to the areal change pattern during systole in the normal group: Type-1: area decreases during systole; Type-2: area increases during systole; and Type-3: no significant areal change. The HLHS and RAI groups were subdivided into two subgroups according to the grade of AVVR: low-grade AVVR and high-grade AVVR, and the annular dynamics were compared between the groups.

Results: Normal group was classified into the subgroups as follows: Type-1＝22 (42％); Type-2＝24 (45％); and Type-3＝7 (13％). HLHS and RAI group was classified into the following subgroups: Type-1＝4 (13％), Type-2＝7 (24％), and Type-3＝21 (65％). Type-3 was predominant in the HLHS and RAI groups (p＜0.01). The subdivided groups were similarly classified into low-grade AVVR group (n＝16) and high-grade AVVR group (n＝16). Type-3 was predominant in the high-grade AVVR group (p＜0.01).

Conclusions: In patients with single ventricle associated with tricuspid valve or common AVV, significant valve incompetence was observed in those with reduced annular dynamics, suggesting the importance of annular function in the patients.

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 lead 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 drawback 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 a rare case of a patient with pheochromocytoma (PCC) and Eisenmenger syndrome (ES). The patient was a female born to healthy parents. Although double outlet right ventricle (DORV) was diagnosed in the neonatal period, she displayed ES pathology from infancy. At the age of 30 years, PCC complications were diagnosed. An operation was contraindicated because of high perioperative risk. She died at the age of 32 years owing to advanced heart failure and renal failure. Her pathologic anatomy revealed DORV, severe pulmonary vascular obstruction, PCC in the left adrenal gland, multiple paraganglioma (PGL) in the bladder, and cyanotic nephropathy. In recent years, hypoxia has been reported as a risk factor for PCC/PGL. In our case, PCC/PGL was associated with DORV and ES. The diagnosis of PCC/PGL in congenital heart disease patients tends to be delayed as the symptoms of PCC/PGL overlap with those of heart disease. The harmful effects of hypertension and/or tachycardia on cardiac hemodynamics due to PCC were difficult to manage as she was contraindicated for surgery. PCC/PGL should be considered as a complication of congenital heart disease.

Surgical management of congenital mitral valve regurgitation (MR) is challenging, and the short- and long-term outcomes remain controversial. In this study, posterior leaflet extension using 0.625％ glutaraldehyde-treated autologous pericardium for 5 min was performed in two patients (aged 1 year and 4 months, and 5 months) with moderate MR through hypoplastic posterior leaflet of the mitral valve. There were no additional reparative procedures for the mitral valve. Shortly after surgery, MR was mild and none, respectively. Mid- and long-term assessments showed that MR was trivial and none, respectively, without increase in the peak velocity of the mitral valve. Posterior leaflet extension using glutaraldehyde-treated autologous pericardium is an attractive alternative for the treatment of MR with hypoplastic posterior leaflet of the mitral valve in pediatric patients.