Results of aortopulmonary collateral arteries coil embolization in pre-fontan patients

Background: Aortopulmonary collateral arteries (APCAs) are common pulmonary blood supply in the group of congenital heart disease with functionally single ventricle. APCAs causes loss of aortic outflow, increase in pulmonary arterial hydrostatic pressure, reducing blood flow from the SVC and IVC to the pulmonary artery (in patients after Glenn and Fontan operations). Therefore, the closure of APCAs is vital in the management of single ventricle patients before and during Fontan operation. The technique of percutaneous APCAs occlusion with coils is a new, effective and better method of control of APCAs, excluding the need for surgical ligation of the APCAs.. Objectives: To evaluate results of aortopulmonary collateral arteries coil embolization in pre-fontan patients Methods: This is the descriptive clinical and retrospective study Result: 29 patients with average age of 7.9 underwent cardiac catheterization with APCAs coil embolization successfully before the Fontan surgery. Complication rate was very low. This method effectively reduced mean pulmonary artery pressure with statistical significance. The results of Fontan surgery of this group were better than that in other studies in the rate of diaphragmatic paralysis and chylous effusion Conclusions: The technique of percutaneous APCAs occlusion with coils before Fontan surgery is safe, effective in reducing mean pulmonary artery pressure and improving results of Fontan surgery.

Neutrophil-to-Lymphocyte and Platelet-to-Lymphocyte Ratio in Univentricular Patients From Birth to Follow-Up After Fontan—Predicting Lymphatic Abnormalities

Background: Reliable laboratory parameters identifying complications after Fontan surgery including the lymphatic abnormalities and the development of protein-losing enteropathy (PLE) are rare. Neutrophil-to-lymphocyte ratio (NLR) and platelet-to-lymphocte ratio (PLR) are inflammatory markers and have been studied to predict outcome and prognosis in various diseases. The aim of this study was to investigate NLR and PLR from birth to follow-up after Fontan and evaluate their use as prognostic parameters for single ventricle patients regarding the development of lymphatic malformations during follow-up.Materials and Methods: Sixty-six univentricular patients who underwent Fontan surgery and had 6-month follow-up magnetic resonance imaging (MRI) with T2 weighted lymphatic imaging after total cavopulmonary connection (TCPC) surgery were included in the study. NLR and PLR were determined at specific time points, from neonatal age to follow-up after Fontan operation and correlated to data from the MRI 6 months after Fontan.Results: NLR and PLR increase significantly over time from the first surgery during infancy to the follow-up after Fontan (both p < 0.0001), with a significant increase after the Glenn surgery for both ratios (each p < 0.0001). Higher NLR (p = 0.002) and higher PLR (p = 0.004) correlated with higher-grade classification of lymphatic abnormalities in T2-weighted imaging 6 months after Fontan surgery and higher NLR correlated with higher transpulmonary gradient prior to Fontan surgery (p = 0.035) Both ratios showed a significant correlation to total protein at follow-up (NLR p = 0.0038; PLR<0.0001).Conclusion: Increased NLR and PLR correlate with higher degree lymphatic malformations after TCPC and therefore might contribute as valuable additional biomarker during follow-up after TCPC. NLR and PLR are simple, inexpensive and easily available parameters to complement diagnostics after TCPC.

CorFix: Virtual Reality Cardiac Surgical Planning Software for Designing Patient-Specific Vascular Grafts: Development and Pilot Usability Study (Preprint)

BACKGROUND Patients with single ventricle heart defects receives three stages of surgeries culminating in the Fontan surgery. During the Fontan surgery, a vascular graft is sutured between the inferior vena cava and pulmonary artery to divert deoxygenated blood flow to the lungs via passive flow. Customizing the graft configuration can maximize the long-term benefits of Fontan surgery. However, planning patient-specific surgery has several challenges including the ability for physicians to customize grafts and evaluate its hemodynamic performance. OBJECTIVE The aim of this study was to develop a virtual reality (VR) Fontan graft modeling and evaluation software for physicians. User study was performed to achieve three additional goals: 1) evaluate the software when used by medical doctors and engineers, 2) identify if doctors have a baseline intuition about hemodynamic performance of Fontan grafts in a VR setting, and 3) explore the impact of viewing hemodynamic simulation results in numerical and graphical formats. METHODS A total of 5 medical professionals including 4 physicians (1 fourth-year resident, 1 third-year cardiac fellow, 1 pediatric intensivist, and 1 pediatric cardiac surgeon) and 1 biomedical engineer voluntarily participated in the study. The study was pre-scripted to minimize the variability of the interactions between the experimenter and the participants. Unless a participant was familiar with the Fontan surgery, a quick information session was provided at the start. Then, all participants were trained to use the VR gear and our software, CorFix. Each participant designed one bifurcated and one tube-shaped Fontan graft for a single patient. Then a hemodynamic performance evaluation was completed, allowing the participants to further modify their tube-shaped design. The design time and hemodynamic performance for each graft design were recorded. At the end of the study, all participants were provided surveys to evaluate the usability and learnability of the software and rate the intensity of VR sickness. RESULTS The average time for creating one bifurcated and one tube-shaped grafts after a single 10-minute training were 13.40 and 5.49 minutes, accordingly. Three out of 5 bifurcated and 1 out of 5 tube-shaped graft designs were in the benchmark range of hepatic flow distribution. Reviewing hemodynamic performance results and modifying the tube-shaped design took an average time of 2.92 minutes. Participants who modified their tube-shaped graft designs were able to improve the non-physiologic wall shear stress percentage by 7.02%. All tube-shaped graft designs improved wall shear stress compared the native surgical case of the patient. None of the designs met the benchmark indexed power loss. CONCLUSIONS VR graft design software can quickly be taught to physicians without any engineering background and VR experience. Improving the system of CorFix could improve performance of the users in customizing and optimizing grafts for patients. With graphical visualization, physicians were able to improve wall shear stress of a tube-shaped graft, lowering the chance of thrombosis. Bifurcated graft designs showed potential strength in better flow split to the lungs, reducing the risk for pulmonary arteriovenous malformations.

Radiologic follow-up in Fontan-associated liver disease in Europe: European Society of Paediatric Radiology survey demonstrates the need for a consensus protocol

Fontan surgery is a life-saving procedure for newborns with complex cardiac malformations, but it originates complications in different organs. The liver is also affected, with development of fibrosis and sometimes cirrhosis and hepatocellular carcinoma. There is no general agreement on how to follow-up these children for the development of liver disease. To understand the current practice on liver follow-up, we invited members of the European Society of Paediatric Radiology (ESPR) to fill out an online questionnaire. The survey comprised seven questions about when and how liver follow-up is performed on Fontan patients. While we found some agreement on the use of US as screening tool, and of MRI for nodule characterization, the discrepancies on timing and the lack of a shared protocol make it currently impossible to compare data among centers.

Platelet count and abdominal dynamic CT are useful in predicting and screening for gastroesophageal varices after Fontan surgery

Objective Patients who undergo Fontan surgery for complex cardiac anomalies are prone to developing liver and gastrointestinal complications. In particular, gastroesophageal varices (GEVs) can occur, but their prevalence is unknown. We aimed to elucidate the occurrence of GEVs and the predicting parameters of GEVs in these patients. Materials and methods Twenty-seven patients (median age, 14.8 years; median time since surgery, 12.9 years) who had undergone the Fontan surgery and were examined by abdominal dynamic computed tomography (CT) for the routine follow-up were included in the study. Radiological findings including GEVs and extraintestinal complications were retrospectively evaluated by experienced radiologists in a blinded manner. Relationships between blood-biochemical and demographic parameters and the presence of GEVs were statistically analyzed. Results Dynamic CT revealed gastric varices (n = 3, 11.1%), esophageal varices (n = 1, 3.7%), and gastrorenal shunts (n = 5, 18.5%). All patients with gastric varices had gastrorenal shunts. All gastric varices were endoscopically confirmed as being isolated and enlarged, with indications for preventive interventional therapy. A platelet count lower than 119 × 109 /L was identified as a predictor of GEV (area under the receiver operating curve, 0.946; sensitivity, 100%; and specificity, 87%). Conclusions GEVs are important complications that should not be ignored in patients who have undergone a Fontan procedure. Platelet counts lower than 119 × 109 /L may help to prompt patient screening by using abdominal dynamic CT to identify GEVs and their draining collateral veins in these patients.

Risk factors for prolonged ventilation after the modified Fontan procedure

Objective: To investigate the risk factors associated with prolonged ventilation after Fontan surgery. Design: Retrospective case series. Setting: Tertiary childrens hospital. Patients: We included 123 children who underwent Fontan surgery without delayed sternal closure or extracorporeal membrane oxygenation between 2011 and 2017. Intervention: Fontan surgery. Measurements and main results: Prolonged ventilation was defined as intubation for more than 24 hours after surgery. Preoperative, intraoperative, and perioperative data were collected retrospectively from medical records. Multivariate logistic regression analysis was used to identify risk factors for prolonged ventilation. The median age and weight of patients were 2.2 years and 10.0 kg, respectively. Seventeen per cent of the patients (n = 21) received prolonged mechanical ventilation, and the median intubation period was 2.9 days. There were no 90-day or in-hospital deaths. The independent predictors of prolonged ventilation identified were fenestration (p < 0.01), low pulmonary artery index (p = 0.02), and advanced atrioventricular regurgitation (p < 0.01). The duration of ICU stay was significantly longer in the prolonged ventilation group than in the early extubation group (10 days versus 6 days, p < 0.01). Conclusion: Fenestration, low pulmonary artery index, and significant atrioventricular regurgitation are risk factors for prolonged ventilation after Fontan surgery. Careful preoperative and perioperative management that considers the risk factors for prolonged ventilation in each individual is important.

Anorexia Nervosa in a Postoperative Patient With Ebstein Anomaly

Background: Along with the improved life prognosis of patients with congenital heart disease, their diverse complications have come under scrutiny. Due to the various medical restrictions attendant on their upbringing, patients with congenital heart disease often have coexisting mental disorders. However, reports of patients with congenital heart disease and coexisting eating disorders are very rare. In this paper the authors report the case of a patient who developed anorexia nervosa following surgery for Ebstein’s anomaly.Case Presentation: A 21-year-old female with Ebstein’s anomaly who underwent a Fontan surgery was transferred to our institution with suspected anorexia nervosa after a more than 2-year intermittent stay in a medical hospital for decreased appetite. Initially, she did not express a desire to lose weight or a fear of obesity, and we suspected that she was suffering from appetite loss due to a physical condition associated with the Fontan circulation. But gradually the pathology of the eating disorder became apparent.Conclusions: Patients with congenital heart disease are more likely to have a psychological background as well as physical problems that might contribute to eating disorders. Indeed, it is unclear why there are not more cases of eating disorders in association with congenital heart diseases.