Vascular rehabilitation in children with chronic intestinal failure reduces the risk of central-line associated bloodstream infections and catheter replacements.

BACKGROUND & AIMS: Children with chronic intestinal failure (IF) require a long-term central venous catheter (CVC) for provision of parenteral nutrition. Vascular, mechanical and infectious complications such as central line-associated bloodstream infections (CLABSI) may lead to progressive loss of venous access sites. Handling and management of CVCs therefore play an important role. Our vascular rehabilitation concept (VRC) is a core component of our intestinal rehabilitation program (IRP) and consists of an education program, optimization of skin care, catheter fixation and lock solution, and the use of hybrid technique for catheter placement. Aim of this study is to analyse the effectiveness of our VRC on CLABSI rates and need for CVC replacements. METHODS: Retrospective analysis of all children treated in our IRP that were followed up between 2018 and 2023. RESULTS: A total of 117 children with chronic IF could be included for analysis of 248864 catheter days (CD). 91 patients were referred from other hospitals (127117 CD before and 89359 CD after entry into our IRP). Children receiving primary care at our IRP (32388 CD) showed a significantly lower CLABSI and line replacement rate than patients referred from external centers (p < 0.001). After entering our IRP, CLABSI rates and need for CVC replacements per 1000 CD decreased significantly in referred patients: 1.19 to 0.26 and 1.77 to 0.59, respectively (p < 0.001). CONCLUSION: Management of paediatric chronic IF patients by an IRP with a vascular rehabilitation concept significantly lowers the rate of CLABSI episodes and the need for catheter replacements.

Thoracic lymphatic anomalies in patients with univentricular hearts: correlation of morphologic findings in isotropic T2-weighted MRI with the outcome after fontan palliation.

Objectives: In this study we examined the correlation between the extent of thoracic lymphatic anomalies in patients after surgical palliation by total cavopulmonary connection (TCPC) and their outcome in terms of clinical and laboratory parameters. Materials and methods: We prospectively examined 33 patients after TCPC with an isotropic heavily T2-weighted MRI sequence on a 3.0 T scanner. Examinations were performed after a solid meal, slice thickness of 0.6 mm, TR of 2400 ms, TE of 692 ms, FoV of 460 mm, covering thoracic and abdominal regions. Findings of the lymphatic system were correlated with clinical and laboratory parameters obtained at the annual routine check-up. Results: Eight patients (group 1) showed type 4 lymphatic abnormalities. Twentyfive patients (group 2) presented less severe anomalies (type 1-3). In the treadmill CPET, group 2 reached step 7.0;6.0/8.0 vs. 6.0;3.5/6.8 in group 1 (p = 0.006*) and a distance of 775;638/854 m vs. 513;315/661 m (p = 0.006*). In the laboratory examinations, group 2 showed significantly lower levels of AST, ALT and stool calprotectin as compared to group 1. There were no significant differences in NT-pro-BNP, total protein, IgG, lymphocytes or platelets, but trends. A history of ascites showed 5/8 patients in group 1 vs. 4/25 patients in group 2 (p = 0.02*), PLE occurred in 4/8 patient in group 1 vs. 1/25 patients in group 2 (p = 0.008*). Conclusion: In the long-term follow-up after TCPC, patients with severe thoracic and cervical lymphatic abnormalities showed restrictions in exercise capacity, higher liver enzymes and an increased rate of symptoms of imminent Fontan-failure such as ascites and PLE.

Interventional closure of a bronchopleural fistula in a 2 year old child with detachable coils.

BACKGROUND: Bronchopleural fistula (BPF) is a severe complication following pneumonia or pulmonary surgery, resulting in persistent air leakage (PAL) and pneumothorax. Surgical options include resection, coverage of the fistula by video-assisted thoracoscopic surgery (VATS), or pleurodesis. Interventional bronchoscopy is preferred in complex cases and involves the use of sclerosants, sealants and occlusive valve devices. CASE PRESENTATION: A 2.5-year-old girl was admitted to our hospital with persistent fever, cough and dyspnoea. Clinical and radiological examination revealed right-sided pneumonia and pleural effusion. The child was started on antibiotics, and the effusion was drained by pleural drainage. Following removal of the chest tube, the child developed tension pneumothorax. Despite insertion of a new drain, the air leak persisted. Thoracoscopic debridement with placement of another new drain was performed after 4 weeks, without abolishment of the air leak. Bronchoscopy with bronchography revealed a BPF in right lung segment 3 (right upper-lobe anterior bronchus). We opted for an interventional approach that was performed under general anaesthesia during repeat bronchoscopy. Following bronchographic visualisation of the fistula, a 2.7 French microcatheter was placed in right lung segment 3 (upper lobe), allowing occlusion of the fistula by successive implantation of 4 detachable high-density packing volume coils, which were placed into the fistula. Subsequent bronchography revealed no evidence of residual leakage, and the chest tube was removed 2 days later. The chest X-ray findings normalized, and follow-up over 4 years was uneventful. CONCLUSIONS: Bronchoscopic superselective occlusion of BPF using detachable high-density packing large-volume coils was a successful minimally invasive therapeutic intervention performed with minimal trauma in this child and has not been reported thus far. In our small patient, the short interventional time, localized intervention and minimal damage in the lung seemed superior to the corresponding outcomes of surgical lobectomy or pleurodesis in a young growing lung, enabling normal development of the surrounding tissue. Follow-up over 4 years did not show any side effects and was uneventful, with normal lung-function test results to date.

Spectrum of Interventional Procedures During Hybrid Central Line Placement in Pediatric Intestinal Rehabilitation Patients With End-Stage Vascular Access.

Background: Loss of available central vein access sites for parenteral nutrition delivery represents one of the main indications for intestinal transplantation in children with intestinal failure. Placement of central venous catheters can be challenging in advanced loss of patent venous pathways. We recently described the hybrid technique (interventional plus surgical approach) of central line placement in children. The aim of this study was to describe and analyze the interventions used during the hybrid procedures regarding feasibility, safety and outcome. Methods: We retrospectively analyzed the course of all children in our intestinal rehabilitation program undergoing hybrid central line placement. We evaluated patients' conditions, interventional techniques and surgical peculiarities as well as outcome. Results: 203 children were treated in our intestinal rehabilitation program between 2010 and 2021. Due to loss of venous access, hybrid technique was performed in 53 children during 76 interventions. In 40 cases the same vessel was reused via Seldinger technique. Among the 30 ultrasound-guided new vessel punctures, 12 were performed by puncture of collateral vessels. Extended interventions due to thoracic central venous obstruction and/or thrombosis requiring additional access via a femoral vein for rehabilitation of the vascular system was performed during 29 procedures including catheter extraction (1), angioplasties (18), stent placement (1), revascularization (5) and thrombectomy (4). Placement of a central line was not possible in 6 children which eventually underwent extended thoracic/vascular surgery: in three children the previously placed catheter could not be removed, in one child, placement of a thrombectomy-catheter was not possible because of inferior vena cava occlusion, and in two children, revascularization failed. Intestinal transplantation was considered in one patient because of impending loss of vascular access. Two self-limiting minor extravasations and one intervention-associated pericardial effusion occurred. Conclusions: Hybrid interventions for central venous catheter placement and vascular rehabilitation enable a high success rate in children with intestinal failure and end-stage vascular access, circumventing the need for intestinal transplantation or advanced surgery. The relevant procedures are complex and require a foresighted and individualized approach with a wide range of interventional techniques. If performed with expertise, this combined interventional/surgical approach is feasible and safe.

A new variant of Abernethy malformation treated by transhepatic interventional closure: a case report.

BACKGROUND: Congenital portosystemic shunts (CPSS) are rare vascular malformations and can be classified into extrahepatic and intrahepatic shunts. Extrahepatic CPSS, also termed Abernethy malformations are associated with severe long-term complications including portopulmonary hypertension, liver atrophy, hyperammoniemia and hepatic encephalopathy. We report a hitherto undescribed variant of Abernethy malformation requiring an innovative approach for interventional treatment. CASE PRESENTATION: We describe a 31-year-old patient following surgical repair of atrioventricular septal defect at the age of 6 years. In the long-term follow-up he showed persistent pulmonary hypertension which deteriorated despite dual pulmonary vasodilative treatment. When he developed arterial desaturation and symptomatic hyperammoniemia detailed reassessment revealed as underlying cause a hitherto undescribed variant of Abernethy malformation connecting the portal vein with the right lower pulmonary vein. Following interdisciplinary discussions we opted for an interventional approach. Since the malformation was un-accessible to interventional closure via antegrade venous or retrograde arterial access, a transhepatic percutaneous puncture of the portal vein was performed. Temporary balloon occlusion of the malformation revealed only a slight increase in portal venous pressure. Interventional occlusion of the large vascular connection was achieved via this transhepatic approach by successive implantation of two large vascular occluding devices. The postinterventional course was unremarkable and both ammonia levels and arterial saturation normalized at follow-up of 12 months. CONCLUSIONS: Portal vein anomalies should be included in the differential diagnoses of pulmonary hypertension or pulmonary arterio-venous malformations. Based on careful assessment of the anatomy and testing of portal vein hemodynamics interventional therapy of complex Abernethy malformations can be performed successfully in specialized centers.

Coronary Artery Anomalies and Their Impact on the Feasibility of Percutaneous Pulmonary Valve Implantation.

One of the major obstacles preventing successful percutaneous pulmonary valve implantation (PPVI) is related to the close proximity of coronary artery branches to the expected landing zone. The aim of this study was to assess the frequency of coronary artery anomalies (CAAs) especially those associated with major coronary branches crossing the right ventricular outflow tract (RVOT) and to describe their relevance for the feasibility of percutaneous pulmonary valve implantation (PPVI). In our retrospective single-center study 90 patients were evaluated who underwent invasive testing for PPVI in our institution from 1/2010 to 1/2020. CAAs were identified in seven patients (8%) associated with major branches crossing the RVOT due to origin of the left anterior descending (LAD) or a single coronary artery from the right aortic sinus. In 5/7 patients with CAAs balloon testing of the RVOT and selective coronary angiographies revealed a sufficiently large landing zone distal to the coronary artery branch. While unfavorable RVOT dimensions prevented PPVI in one, PPVI was performed successfully in the remaining four patients. The relatively short landing zone required application of the "folded" melody technique in two patients. All patients are doing well (mean follow-up 3 years). CAAs associated with major coronary branches crossing the RVOT can be expected in about 8% of patients who are potential candidates for PPVI. Since the LAD crossed the RVOT below the plane of the pulmonary valve successful distal implantation of the valve was possible in 4/7 patients. Therefore these coronary anomalies should not be considered as primary contraindications for PPVI.

A Comprehensive Functional Analysis in Patients after Atrial Switch Surgery.

BACKGROUND: Long-term course after atrial switch operation is determined by increasing right ventricular (RV) insufficiency. The aim of our study was to investigate subtle functional parameters by invasive measurements with conductance technique and noninvasive examinations with cardiac magnetic resonance imaging (CMR). METHODS: We used invasive (pressure-volume loops under baseline conditions and dobutamine) and noninvasive techniques (CMR with feature tracking [FT] method) to evaluate RV function. All patients had cardiopulmonary exercise testing (CPET). RESULTS: From 2011 to 2013, 16 patients aged 28.2 ± 7.3 (22-50) years after atrial switch surgery (87.5% Senning and 12.5% Mustard) were enrolled in this prospective study. All patients were in New York Heart Association (NYHA) class I to II and presented mean peak oxygen consumption of 30.1 ± 5.7 (22.7-45.5) mL/kg/min. CMR-derived end-diastolic volume was 110 ± 22 (78-156) mL/m2 and RV ejection fraction 41 ± 8% (25-52%). CMR-FT revealed lower global systolic longitudinal, radial, and circumferential strain for the systemic RV compared with the subpulmonary left ventricle. End-systolic elastance (Ees) was overall reduced (compared with data from the literature) and showed significant increase under dobutamine (0.80 ± 0.44 to 1.89 ± 0.72 mm Hg/mL, p ≤ 0.001), whereas end-diastolic elastance (Eed) was not significantly influenced (0.11 ± 0.70 to 0.13 ± 0.15 mm Hg/mL, p = 0.454). We found no relevant relationship between load-independent conductance indices and strain or CPET parameters. Conductance analysis revealed significant mechanical dyssynchrony, higher during diastole (mean 30 ± 4% baseline, 24 ± 6% dobutamine) than during systole (mean 17 ± 6% baseline, 19 ± 7% dobutamine). CONCLUSIONS: Functional assessment of a deteriorating systemic RV remains demanding. Conductance indices as well as the CMR-derived strain parameters showed overall reduced values, but a significant relationship was not present (including CPET). Our conductance analysis revealed intraventricular and predominantly diastolic RV dyssynchrony.

Mid-Term Outcomes Following Percutaneous Pulmonary Valve Implantation Using the "Folded Melody Valve" Technique.

BACKGROUND: The folded valve is a manual shortening of the Melody device, which has been validated as a valuable therapeutic option for the management of dysfunctional right ventricular outflow tracts needing a short valved stent. In this article, we aimed to evaluate, in a multicenter cohort, the mid-term outcomes of patients in whom a percutaneous pulmonary valve implantation was performed using the folded valve technique. METHODS: A 2012 to 2018 retrospective multicenter study was performed in 7 European institutions. All patients who benefit from percutaneous pulmonary valve implantation with a folded Melody valve were included. RESULTS: A total of 49 patients (median age, 19 years [range 4­56], 63% male) were included. The primary percutaneous pulmonary valve implantation indication was right ventricular outflow tract stenosis (n=19; 39%), patched native right ventricular outflow tracts were the most common substrate (n=15; 31%). The folded technique was mostly used in short right ventricular outflow tracts (n=28; 57%). Procedural success was 100%. After a median follow-up of 28 months (range, 4­80), folded Melody valve function was comparable to the immediate postimplantation period (mean transvalvular peak velocity=2.6±0.6 versus 2.4±0.6 m/s, P>0.1; only 2 patients had mild pulmonary regurgitation). Incidence rate of valve-related reinterventions was 2.1% per person per year (95% CI, 0.1%­3.9%). The probability of survival without valve-related reinterventions at 36 months was 90% (95% CI, 76%­100%). CONCLUSIONS: The folded Melody valve is a safe technique with favorable mid-term outcomes up to 6.5 years after implantation, comparable with the usual Melody valve implantation procedure. Complications and reinterventions rates were low, making this technique relevant in selected patients.

T2-Weighted High-Resolution Isotropic Magnetic Resonance Lymphangiography of the Thoracic and Abdominal Lymphatic Vessels with and without Previous High-Fat Meal.

RATIONALE AND OBJECTIVES: The aim of this study was to assess the potential benefit of a high-fat meal for preparation of patients before lymphangiography of the thoracic and abdominal lymphatic vessels by a heavily T2-weighted 3D magnetic resonance sequence at 3T. MATERIALS AND METHODS: A heavily T2-weighted 3D Fast-Spin-Echo sequence was applied twice for lymphangiography in 15 healthy volunteers. One examination was performed following overnight fasting and the second examination was conducted 3 hours after a drinking of 200 ml of cream and a solid meal. The effect of a high-fat meal on the visualization of different segments of the thoracic and abdominal lymphatic vessels was analyzed by scoring of the image quality. RESULTS: Evaluation of the summarized score of all four segments of the thoracic duct showed significantly improved general visualization of the lymphatic system in the postprandial examination when compared to the results obtained after overnight fasting (mean ± SD: 4.5 ± 1.7 vs. 5.9 ± 1.8, p = 0.007*). Regarding different segments of the lymphatic system significant differences between pre and post cream lymphangiographies were found in the cervical segment (p = 0.012*), the inferior thoracic segment (p = 0.003*) and the abdominal segment (p = 0.035*). In contrast, the visualization of the superior thoracic segment was not significantly improved by high fat meal preparation of the subjects (p = 0.388). CONCLUSION: A high-fat meal 3 hours prior to T2-weighted MR-lymphangiography improves the visualization of the main lymphatic thoracic and abdominal vessels, particularly the abdominal and cervical part as well as the inferior segment of the thoracic duct.

Portal vein thrombosis in Fontan-associated liver disease.

A 25-year-old patient with signs of cirrhosis on ultrasound and CT presented with portal vein thrombosis on routine follow-up examinations; retrograde hepatic wedge angiography demonstrated only the right-sided portal vein branch. Development of a portosystemic collateral vessel to the left-sided renal vein prevented signs of hypersplenism. This unique complication of portal vein thrombosis should be considered during long-term surveillance.

Anomalous origin of the left coronary artery from the right aortic sinus: probably benign variant associated with a subpulmonic intramyocardial course - a case report.

BACKGROUND: Anomalous aortic origin of the left coronary artery (AAOLCA) from the right aortic sinus is a rare congenital anomaly associated with significantly increased risk of myocardial ischemia, arrhythmias and sudden cardiac death. This refers specifically to AAOLCA associated with interarterial and/or intramural course. Much less is known about anomalous origin of the left coronary artery from the right aortic sinus associated with a subpulmonic intramyocardial course. CASE PRESENTATION: We report a 12 year old girl who complained of recurrent episodes of chest pain lasting for some minutes and not associated to exercise. Echocardiography revealed AAOLCA from the right aortic sinus taking a subpulmonic course within the conal septum. The diagnosis was confirmed by CT-scan and selective coronary angiography. Treadmill test, Holter-ECG and bicycle-stress echocardiography revealed no evidence of myocardial ischemia reflecting unimpaired diastolic flow in the left coronary artery. According to the nature of the complaints and in the absence of signs of myocardial ischemia the episodes of chest pain were classified as idiopathic and not associated to the coronary anomaly. We opted for a conservative approach with regular follow-up visits. During a follow-up of 2 years without restriction of sports activities the patient has been asymptomatic. CONCLUSION: According to the literature AAOLCA with subpulmonary intramyocardial course appears to be associated with significantly less clinical problems than AAOLCA taking an interarterial course. The diagnosis can be suspected based on echocardiography and confirmed by contrast-enhanced computed tomography. Until now there are only few data concerning the natural history and incidence of complications in this specific anomaly. Despite the probably benign nature we recommend regular follow-up examinations including stress-testing in these patients.

Coil occlusion of a congenital left ventricular aneurysm in a newborn.

We report interventional treatment of a congenital left ventricular aneurysm in a neonate. The aneurysm was detected prenatally at 20 weeks of gestation. Postnatally, the aneurysm increased in size during the first few days of life and therefore we opted for interventional closure to abolish the potential risk of rupture. The aneurysm was entered with a 2.7 French microcatheter, and complete occlusion was achieved by successive placement of three detachable large volume coils (Ruby Coil PenumbraR , Alameda, California). To the best of our knowledge, this is the first report describing interventional occlusion of a congenital ventricular aneurysm in early infancy. Interventional occlusion with detachable large volume coils appears to be an attractive alternative to surgical resection in patients presenting with ventricular outpouchings and narrow base connection to the ventricle.

Stent-retriever thrombectomy in the treatment of infants with acute thrombosis of the superior vena cava and innominate vein.

OBJECTIVES: To describe the efficacy and safety of stent-retriever thrombectomy in infants with thrombosis of the superior vena cava (SVC) and innominate vein. BACKGROUND: Thrombosis of the SVC and of the innominate vein is a potentially life threatening complication in infants during intensive care treatment following major surgical procedures. To avoid reoperations, we evaluated interventional revascularization by stent-retriever thrombectomy. METHODS: From 2015 to 2017, five infants were diagnosed with acute thrombosis of the SVC and innominate vein following major cardiac or pediatric surgery. Using a femoral venous access and 4 or 5 French guiding catheters stent-retriever systems (4/20 mm or 6/30 mm) were placed into the thrombus and retrieved under suction. We aimed to revascularize not only the SVC but also the innominate, jugular, and subclavian veins. RESULTS: Following repeated stent retrieving manoeuvers, we were able to reestablish flow in the major veins of all patients. Due to significant residual thrombotic material, we decided to perform additional balloon dilatation of the SVC and innominate vein in 3/5 patients. There were no complications related to the procedure and none of our patients required blood transfusion. Following the intervention, the patients received treatment with low-molecular-weight heparin. Interventional treatment achieved persistent patency of the SVC and innominate vein in all patients. CONCLUSION: Stent-retriever thrombectomy is a safe and effective method for interventional treatment of acute thrombosis of the central veins in infants. Due to the large amount of thrombotic material, it is frequently required to combine this method with balloon compression of residual thrombotic material.

Feasibility, safety and diagnostic impact of endomyocardial biopsies for the diagnosis of myocardial disease in children and adolescents.

AIMS: Endomyocardial biopsies (EMBs) are performed infrequently in children owing to significant past complication rates and controversial discussions about the therapeutic value of results. The objective of this study was to investigate the safety and feasibility of EMBs for suspected myocardial disease in relation to their clinical value. METHODS AND RESULTS: We performed a retrospective multicentre review of the Working Group for Interventional Cardiology of the German Society for Paediatric Cardiology. During three consecutive years, 206 EMBs (84 female/mean age 8.95±6.62 years) were performed and analysed at 15 heart centres. In the majority of cases, biopsies were taken from the right ventricle (RV/89.8%; p<0.001). The overall complication rate was 9.7%, whereas major complications occurred in only 0.97% of cases. Risk factors associated with a higher complication rate were biopsy during the first year of life (20.5%) and from the left ventricle (31.1%) (p<0.05). There was no procedure-related mortality. Treatment was changed in 18.0% of cases based on biopsy results. CONCLUSIONS: Today, endomyocardial biopsies in older children with suspected myocardial disease can be performed safely with a low risk of major complications and mortality, whereas the risk of complications if the biopsy is carried out in the first year of life or taken from the left ventricle remains high.

Hybrid interventions for catheter placement in pediatric intestinal rehabilitation patients with end-stage venous access.

PURPOSE: The purpose of this study is to analyze the combined approach of endovascular and open surgical procedures for insertion of permanent central venous catheters in children with intestinal failure and end-stage venous access. METHODS: Data of 14 children (16 interventions) with intestinal failure and end-stage venous access, treated within the pediatric intestinal rehabilitation program at our institution between September 2011 and November 2016, were retrospectively reviewed. The patients underwent hybrid endovascular/open surgical approaches for insertion of central venous catheters. Access to central veins was established through endovascular intervention; catheter placement was achieved with combined interventional and surgical measures depending on the individual vascular conditions. RESULTS: Median age at intervention was 47months (interquartile range (IQR),29-74), median time for interventions was 66min (IQR,42-111). Catheter placement was successfully achieved in all patients. The median dose of irradiation during angiography was 0.2Gy*cm2 (IQR, 0.2-0.6), no complications occurred during or after interventions. CONCLUSIONS: Hybrid endovascular/open surgical procedures can be successfully applied for restoring or maintaining permanent central venous catheters in children with intestinal failure and end-stage venous access. These approaches are a valuable contribution in intestinal rehabilitation programs contributing to a further decrease of the need for intestinal transplantation in affected patients. TYPE OF STUDY: Treatment Study. LEVEL OF EVIDENCE: Level IV.

3-D cardiac MRI in free-breathing newborns and infants: when is respiratory gating necessary?

BACKGROUND: Newborns and small infants have shallow breathing. OBJECTIVE: To suggest criteria for when respiratory gating is necessary during cardiac MRI in newborns and infants. MATERIALS AND METHODS: One-hundred ten data sets of newborns and infants with (n = 92, mean age: 1.9 ± 1.7 [SD] years) and without (n = 18, mean age: 1.6 ± 1.8 [SD] years) navigator gating were analysed retrospectively. The respiratory motion of the right hemidiaphragm was recorded and correlated to age, weight, body surface area and qualitative image quality on a 4-point score. Quantitative image quality assessment was performed (sharpness of the delineation of the ventricular septal wall) as well as a matched-pair comparison between navigator-gated and non-gated data sets. RESULTS: No significant differences were found in overall image quality or in the sharpness of the ventricular septal wall between gated and non-gated scans. A navigator acceptance of >80% was frequently found in patients ages <12 months, body surface area <0.40 m(2), body weight <10 kg and a size of <80 cm. CONCLUSION: Sequences without respiratory gating may be used in newborns and small infants, in particular if age <12 months, body surface area <0.40 m(2), body weight <10 kg and height <80 cm.