Association of albuminuria with kidney function and hemodynamic disturbance in pre-school children who undergo congenital heart disease surgery.

BACKGROUND: We validated the prevalence of albuminuria and its association with kidney function and hemodynamics in pre-school children who underwent surgery for congenital heart disease (CHD). METHODS: From 403 patients who had undergone surgery for CHD at least 6 months before pre-school and were admitted to our hospital between 2011 and 2015, 75 who underwent blood and urine tests and cardiac catheterization were included in this study. The urinary albumin-to-creatinine ratio (ACR) was quantified, and the relationship of ACR with physical and laboratory findings and hemodynamics assessed using cardiac catheterization was analyzed. RESULTS: The study cohort was divided into three groups: Fontan group (n = 25), tetralogy of Fallot (TOF) group (n = 18), and control group (other biventricular CHDs; n = 32). The median age of patients was 5.9 years. ACR was higher in the Fontan group than in the TOF and control groups (median: 15.0 vs. 5.0 and 0.0 mg/g, p < 0.001). Moreover, albuminuria (ACR > 30 mg/g) was observed in 20.0% of Fontan patients, while ACR was associated with potential complicating factors of Fontan circulation: high central venous pressure, high mean pulmonary artery pressure, and worse than moderate atrioventricular regurgitation. ACR showed a moderate correlation with the cystatin C-based estimated glomerular filtration rate (r = - 0.725, p < 0.001). CONCLUSIONS: Measurement of albuminuria in Fontan patients before they join elementary school is useful because it reflects kidney function and hemodynamic factors that can worsen their condition. Identification and management of patients with albuminuria may facilitate early therapeutic intervention for worsening Fontan factors, eventually delaying the deterioration of kidney function. A higher resolution version of the Graphical abstract is available as Supplementary information.

Preoperative and Intraoperative Risk Factors for Surgical Site Infection in Pediatric Cardiac Surgery.

BACKGROUND: Surgical site infection (SSI) is a major complication of pediatric cardiac surgery. If the risk of developing SSI can be predicted based on the patient's preoperative background, appropriate preoperative management to prevent the development of SSI can be achieved. METHODS: We retrospectively studied cases for patients younger than 7 years of age among surgeries performed through a median sternotomy at Kagoshima University Hospital from April 2011 to March 2021. SSI was diagnosed according to the Centers for Disease Control and Prevention guidelines and classified into 3 types: superficial incisional SSI (SiSSI), deep incisional SSI (DiSSI) and mediastinitis. RESULTS: Of the 765 consecutive pediatric cardiac surgeries, 597 were included in the analysis based on the exclusion criteria. Of these, 17 (2.8%) developed SSI (3 SiSSI cases, 2 DiSSI cases and 12 mediastinitis cases), with Staphylococcus aureus as the major pathogen. Univariate analysis revealed that low preoperative serum protein ( P = 0.049) and low serum albumin levels ( P = 0.023) were risk factors for the development of SSI. No findings suggested impaired hepatic synthesis, inflammatory disease or protein loss from the kidney or intestinal tract. We concluded that malnutrition caused low serum protein and albumin levels. CONCLUSIONS: Low preoperative serum protein and albumin levels are risk factors for SSI development of SSI. Nutritional status should be regularly assessed in children scheduled for cardiac surgery, and interventions, such as nutritional guidance, should be considered if malnutrition is suspected.

Successful coil embolization for pediatric internal mammary artery aneurysm after the Fontan procedure: A case report and literature review.

We herein describe the first pediatric case of an internal mammary artery (IMA) aneurysm caused by a median sternotomy. He was a 2-year-old with tricuspid atresia who underwent an extracardiac conduit Fontan procedure. On the 36th postoperative day, an asymptomatic left IMA aneurysm was detected via contrast computed tomography, which was successfully treated with coil embolization. The patient had no underlying disease such as vasculitis, connective tissue disease, or other hereditary diseases, and there were no episodes of infection or hypertension before or after the onset of the IMA aneurysm. Because the left IMA ran medially to the periphery and was in a vulnerable position during median sternotomy, we considered the IMA aneurysm was caused by the median sternotomy. We pediatric cardiologists should be aware that IMA aneurysms can occur in pediatric cardiac surgery, and we should be proactive in performing postoperative imaging studies in cases where the preoperative internal thoracic artery runs medially toward the periphery. Learning objective: Internal mammary artery (IMA) aneurysm is a rare vascular disease, especially in children. One-third of adult IMA aneurysms have been reported to be caused by sternotomy, but not in children. We report the first pediatric case of an IMA aneurysm caused by sternotomy. We should recognize that there is a potential risk of IMA aneurysms in pediatric cardiac surgery as well.

Pediatric Patients Undergoing a Fontan Operation or with a High RACHS-1 Score Require Monitoring for Chronic Kidney Disease in Early Childhood.

Although the number of pediatric patients with long-term survival following cardiac surgery is increasing, concerns regarding chronic kidney disease (CKD) after surgery are growing. We examined the frequency of and risk factors for pediatric CKD development in patients with congenital heart disease (CHD) at least 2 years after cardiac surgery. This was a cross-sectional study of 147 patients who underwent open-heart surgery for CHD at Kagoshima University Hospital from April 2010 to March 2017. Data on demographics, acute kidney injury after cardiac surgery, cyanotic heart disease, Fontan circulation, medications in the perioperative period, and Risk Adjustment for Congenital Heart Surgery-1 (RACHS-1) category were recorded. CKD was defined using the current classification system described in the National Kidney Foundation's Kidney Disease Outcomes Quality Initiative and assessed during early childhood within 2-3 years of cardiac surgery. Statistical analyses were performed using SPSS Statistics for Windows version 25.0. We consecutively enrolled 147 patients, of whom 22 (15.0%) had CKD, all with stage-2 severity. Among patients with CKD, a higher proportion underwent Fontan surgery (P < 0.001), a higher proportion had cyanotic heart disease (P = 0.009), and the RACHS-1 category was high (P = 0.003). Patients with CKD appeared more frequently than patients without CKD in RACHS-1 categories 3, 5, and 6. It is essential to evaluate renal function longitudinally and monitor for CKD, given that patients who underwent Fontan surgery or complicated surgery in infancy have a high rate of developing postoperative CKD in early childhood.

Neonatal Marfan syndrome with missense variant of c.3706T>C undergoing bilateral atrioventricular valve replacement.

Neonatal Marfan syndrome is a rare condition with poor prognosis because of severe mitral and/or tricuspid valve insufficiency. Mitral valve replacement is sometimes required in early infancy, while tricuspid valve replacement is rarely done. We report the first infant neonatal Marfan syndrome case with a missense variant of c.3706T>C in the fibrillin-1 gene that was successfully managed by mitral and tricuspid valve replacement. Early multiple-valve replacement may sometimes be required during infant age in this genetic syndrome.

Case Report: 18F-FDG PET-CT for Diagnosing Prosthetic Device-Related Infection in an Infant With CHD.

Patients who have undergone cardiac surgery using prosthetic devices have an increased risk of developing prosthetic device-related infection and mediastinitis. However, accurate diagnosis of prosthetic device-related infection can be difficult to evaluate and treat with antibiotic therapy alone. In recent years, 18F-fluorodeoxyglucose positron emission tomography-computed tomography (18F-FDG PET-CT) has made promising contributions to detect infective endocarditis, pacemaker infections, or other inflammations. Nevertheless, 18F-FDG PET-CT for congenital heart disease (CHD) with device infection has been sparsely reported. We present an infantile girl diagnosed with pulmonary atresia with a ventricular septal defect who underwent replacement of the right ventricle-to-pulmonary artery (RV-PA) conduit for improvement cyanosis. She developed high fever and was diagnosed with mediastinitis and bacteremia by Pseudomonas aeruginosa (P. aeruginosa) on postoperative day 4. Mediastinal drainage and 6 weeks of antibiotic therapy improved her condition, but bacteremia flared up on postoperative day 56. Despite a long course of antibiotic therapy, she had two more recurrences of bacteremia with the detection of P. aeruginosa. Echocardiography and chest contrast CT showed no evidence of vegetation and mediastinitis. On postoperative day 115, 18F-FDG PET-CT revealed an accumulation on the RV-PA conduit (SUV max 3.4). Finally, she developed an infectious ventricular pseudo-aneurysm on postoperative day 129 and underwent aneurysm removal and RV-PA conduit replacement on postoperative day 136. Our case showed the importance of 18F-FDG PET-CT for diagnosing specific localization of prosthetic device-related infection which is hard to detect using other imaging techniques. It can be a useful diagnostic tool for infantile patients with CHD with cardiac prosthetic devices and improve subsequent clinical treatments.

Celecoxib as a Potential Treatment for Intractable Lymphatic Malformation.

Lymphatic malformation (LM) is a congenital disorder resulting from an abnormal development of lymphatic vessels. LM may result in problems of cosmesis and functional impairment, including airway compression. An 11-year-old girl was referred to our department with increasing dysphagia caused by a large left cervical LM with a long history of treatment. Because of the LM location, surgical resection was not an option, and various therapies, including use of picibanil, had proven ineffective. Celecoxib treatment (100 mg/day) was initiated for local pain management. Softening of the lesion was observed 2 weeks after treatment initiation, and the dose was increased to 200 mg/day with additional shrinking of the LM over the next 2 weeks. With parental consent, celecoxib was continued, with a 65% reduction in volume achieved at 6 months. The patient discontinued treatment at 12 months, and the LM volume increased. Control over the LM was achieved with resumption of celecoxib treatment. After 2 years of treatment, the LM persists, but the size of the malformation is significantly smaller. No adverse effects of celecoxib treatment were observed. The anti-cyclooxygenase-2 effect of celecoxib prevented lymphatic vessel growth through an inhibition of cyclooxygenase-2 activity in the conversion of prostaglandin to prostaglandin E2. In conclusion, celecoxib may be a promising therapeutic agent for LM management.