Background: The Japanese Society of Pediatric Cardiology and Cardiac Surgery has set up a division of surgical training to train the next generation of pediatric cardiac surgeons. A project towards this end has been launched.

Method: As part of this project, a questionnaire was conducted among surgical members of the Japanese Society of Pediatric Cardiology and Cardiac Surgery to obtain information about the actual status of pediatric cardiac surgeons in Japan, including working environment and training conditions. From June 26, 2018 to August 6, 2018, 420 cardiovascular surgeons who belong to the Japanese Society of Pediatric Cardiology and Cardiac Surgery were requested to answer an anonymous web questionnaire. A total of 176 responses were obtained (Answer rate: 42%).

Results: A total of 15% of the pediatric cardiac surgeons had 10 on-duty days per month or more, while 85% worked in excess of the statutory working hours. In addition, the questionnaire responses confirmed that the training environments of pediatric cardiac surgeons differed greatly among institutions. The number of surgical cases per surgeon varied. Currently, it is difficult to state that all pediatric cardiac surgeons have performed adequate number of surgeries, and many surgeons are dissatisfied and anxious due to their harsh working environment.

Conclusion: It is thus necessary to create a training environment where the number of surgical cases, difficulty, and other properties are programmed to improve the harsh working environment, and to establish an ‘all-Japan training system’ for the next generation.

Background: Lymph flow disorder in the central lymph pathway is referred to as central lymphatic disease. This disorder can be intractable and can present as postoperative chylothorax. Although novel concepts and treatments have been reported, some specialists were generally asked about ideas beyond their specialties. Hence, the current study aimed to validate the ideas that emerged from these consultations.

Material and Methods: We analyzed the consultations handled by our team from May 2016 to May 2020. All data about the location and characteristics of the consulted hospitals, specialty of the consulting physician, and aim of the consultations (operation request, treatment plan, testing details, and nutrition) were retrospectively assessed.

Results: In total, 38 consultations were evaluated. We observed an annual increment in the number of cases. The majority of questioners were in the Kanto region, university hospitals, and pediatric cardiologist, about postoperative chylothorax. Notably, the consultations primarily aimed to discuss treatment plans rather than operative requests.

Conclusion: A standardized therapeutic strategy for central lymphatic disease should be established. Thus, a proposal for such a treatment approach was presented in our strategy flowchart.

Background: Pulmonary artery (PA) sling is a rare vascular anomaly and is often associated with various degrees of tracheal stenosis. The aim of this study is to review the surgical outcomes and tracheal growth after the PA sling repair.

Methods: From August 2006 to August 2015, consecutive six patients (median age, 6.7 months; range, 2.7–21.7 months) underwent surgical repair of PA sling at our institute. All patients did not undergo tracheoplasty. We evaluated the degree of tracheal growth after the PA sling repair using computed tomography (CT).

Results: The median follow-up was 10.5 years (range, 5.5–14.5 years). There were no operative deaths but only one late death. One patient required balloon angioplasty because of left PA stenosis. CT showed significant growth of the tracheal lumen diameter in all six patients. The mean diameter of the narrowest section of the trachea increased from 2.2 mm to 3.9 mm after the PA sling repair (p<0.01). Additionally, the stenotic segment ratio improved from 54% to 26% after the PA sling repair (p<0.01).

Conclusions: Because of the sufficient tracheal growth after the PA sling repair, no additional tracheoplasty was required consequently, and furthermore early and late mortality rates was low.

Discrete subaortic stenosis (DSS) is considered an acquired lesion caused by a progressively enlarging shelf of fibrous or fibromuscular tissue beneath the aortic valve. We present a case of DSS caused by a flap of tissue attached to the aortic valve cusp. A pathological analysis of the resected flap suggested that it was valvular tissue, thus supporting the diagnosis of DSS secondary to congenital accessory aortic valve disease.

Rupture of mitral chordae tendineae in the acute phase of Kawasaki disease is rare in infants, with a sudden onset and possible complication of acute heart failure. Herein, we report the case of a 7-month-old female with incomplete Kawasaki disease who was diagnosed with ruptured mitral chordae tendineae resulting in acute heart failure. The patient had a history of fever lasting for 6 days. She had no other symptoms suggestive of Kawasaki disease, and spontaneous defervescence was seen. She was brought to our hospital on the 14th day of the illness for acute heart failure. Severe mitral regurgitation due to ruptured chordae tendineae of the posteromedial leaflet was diagnosed using echocardiography. Aneurysmal dilatation of the proximal coronary artery was also observed. Based on the coronary arterial lesion and laboratory data, we diagnosed her with ruptured mitral chordae tendineae due to severe valvulitis resulting from incomplete Kawasaki disease. Artificial chordae reconstruction and Kay-Reed annuloplasty were performed on the 17th day of the illness, and her mitral regurgitation on echocardiography improved from severe to trivial. Although the ruptured mitral chordae tendineae is rare among infants, timely diagnosis and treatment are crucial to avoid life-threatening complications.

Williams syndrome (WS) is a congenital anomaly affecting various organs, especially cardiac tissues. While supravalvular aortic stenosis is common during childhood, mitral regurgitation usually develops during adulthood. To the best of our knowledge, only two reports have demonstrated infective endocarditis (IE) associated with mitral valve regurgitation in WS. We report two cases of IE in adult patients with WS. Case 1 was a 30-year-old woman presenting with dyspnea and remittent fever, severe mitral regurgitation, and atrial fibrillation. She had dental abnormalities and received dental care without prophylactic antibiotics 2 weeks before admission. Blood cultures were positive for Streptococcus gordonii, which is sensitive to penicillin G and ampicillin. She was successfully treated with antibiotics and surgical mitral valve plasty. Case 2 was a 34-year-old woman with IE following urinary tract infection. Methicillin-susceptible Staphylococcus aureus was the causative agent of IE. Mitral valve plasty was performed after 6-weeks of treatment with antibiotics. WS are at a high risk of bacteremia because of dental abnormalities. Mitral valve prolapse and regurgitation develop during adulthood, which might be associated with the onset of IE in patients with this disease.

An 18-day-old girl was admitted to the hospital for heart failure, but no intracardiac malformation was detected. She was followed-up as an outpatient because her symptoms improved with diuretics alone. After discontinuation of diuretics, swelling of the left thigh was noted and a vascular murmur was heard. A left superficial femoral arteriovenous fistula was found, and ligation was performed. The symptoms disappeared postoperatively, and the patient had a good course.