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.

National Survey Regarding Motivation and Conditions of Physicians Working in a Pediatric Cardiac Intensive Care Unit.

BACKGROUND: The professional demands on the expertise in pediatric intensive care have continuously increased in recent years. Due to a lack of applicants, the staffing of a continuous shift service with qualified medical staff poses major challenges to the hospitals. METHODS: A web-based questionnaire with 27 predominantly matrix questions on working conditions and motivation for working in this area was sent to pediatric hospitals throughout Germany. RESULTS: 165 doctors responded to the survey. The average age of the participants was 35.2 years. The average weekend work load reported by 79% of the respondents was 2 weekends per month, 70% of the study participants performed five to seven night shifts per month. 92% of the respondents stated that they basically enjoyed working in the intensive care unit (ICU). When asked to prioritize the working conditions, an appreciative working atmosphere in the team was named as priority 1 by 57%, followed by good guidance in the independent performance of interventions (25%) and good working conditions (19%). DISCUSSION: The survey result shows that neither aspects of work-life balance nor payments are the key issues selecting the interesting, but physically and emotionally demanding job in pediatric ICU. CONCLUSION: When evaluating vocational training in pediatric intensive care medicine, the immediate working atmosphere in the team with mutual respect and understanding and the guidance in training are more important than the general conditions.

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.

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.

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.

Severe adverse events in children with tracheostomy and home mechanical ventilation - Comparison of pediatric home care and a specialized pediatric nursing care facility.

BACKGROUND: Advances in medical care and ventilator technologies increase the number of children with tracheostomy and home mechanical ventilation (HMV). Data on severe adverse events in home care and in specialized nursing care facilities are limited. PATIENTS AND METHODS: Retrospective analysis of incidence and type of severe adverse events in children with tracheostomy and HMV in home care compared to a specialized nursing care facility over a 7-year period. RESULTS: 163.9 patient-years in 70 children (home care: 110.7 patient-years, 24 patients; nursing care facility: 53.2 patient-years, 46 patients) were analyzed. In 34 (48.6%) patients tracheostomy was initiated at the age of <1 year. 35 severe adverse events were identified, incidence of severe adverse events per patient-year was 0.21 (median 0.0 (0.0-3.0)). We observed no difference in the rate of severe adverse events between home care and specialized nursing care facility (0.21 [y-1]; median 0.0 (0.0-3.0) versus 0.23 [y-1]; median 0.0 (0.0-1.6); p = 0.690), however, significantly more tracheostomy related incidents and infections occurred in the home care setting. Young age (<1 year) (Odds ratio 3.27; p = 0.045) and feeding difficulties (nasogastric tubes and percutaneous endoscopic gastrostomy) (Odds ratio 9.08; p = 0.016) significantly increased the risk of severe adverse events. Furthermore, the rate of severe adverse events was significantly higher in patients with a higher nursing score. CONCLUSION: Pediatric home mechanical ventilation via tracheostomy is rarely associated with emergencies or adverse events in home care as well as in a specialized nursing care facility setting.

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.

A nurse-driven analgesia and sedation protocol reduces length of PICU stay and cumulative dose of benzodiazepines after corrective surgery for tetralogy of Fallot.

PURPOSE: Analgesia and sedation protocols are reported to reduce the requirement of sedative and analgesic agents, duration of mechanical ventilation, and length of pediatric intensive care unit (PICU) stay. However, these studies often were conducted based on inhomogeneous cohorts. The aim of this study was the evaluation of a nurse-driven analgesia and sedation protocol in a homogenous population of infants following corrective surgery for tetralogy of Fallot (TOF). DESIGN AND METHODS: This retrospective analysis was conducted in a cardiac PICU of a tertiary referral center. Two cohorts of patients who underwent corrective surgery for TOF below the age of 7 months, were retrospectively evaluated before and after implementation of a nurse-driven analgesia and sedation protocol. We compared peak and cumulative doses of midazolam, morphine, and clonidine, length of PICU stay and time on mechanical ventilation. RESULTS: A total of 33 patients were included in the preimplementation period and 32 during the postimplementation period. Implementation of the nurse-driven analgesia and sedation protocol had no effect on time on mechanical ventilation (72 hr [24-141] vs. 49 hr [24-98]), but significantly on length of PICU stay (7 days [5-14] vs. 5 days [4-7]). Cumulative doses of midazolam (7.37 mg/kg [4.70-17.65] vs. 5.0 mg/kg [2.70-9.12]) as well as peak doses of midazolam (0.22 mg·kg-1 ·hr-1 [0.20-0.33] vs. 0.15 mg·kg-1 ·hr-1 [0.13-0.20]) and morphine (50.0 µg·kg-1 ·hr-1 [39.7-79.9] vs. 42.5 µg·kg-1 ·hr-1 [29.7-51.8]) were significantly reduced. The postimplemantation group showed no increase in postoperative complications and adverse events. PRACTICE IMPLICATIONS: The implementation of a nurse-driven analgesia and sedation protocol is safe in infants following corrective surgery for TOF. It reduces significantly the length of PICU stay, cumulative and peak doses of midazolam and peak doses of morphine.

Ketamine Infusion as a Counter Measure for Opioid Tolerance in Mechanically Ventilated Children: A Pilot Study.

BACKGROUND: Drug rotation to prevent opioid tolerance is well recognized in chronic pain management. However, ketamine infusion as a counter measure for opioid tolerance is rarely described in mechanically ventilated children developing tolerance from prolonged opioid infusion. PATIENTS AND METHODS: We performed a retrospective study in a 14-bed medical-surgical-cardiac pediatric intensive care unit. Thirty-two mechanically ventilated children who had developed tolerance from prolonged intravenous infusion of opioids received a continuous intravenous infusion of ketamine as an opioid substitute for more than 2 days, scheduled in a drug rotation protocol. RESULTS: Thirty-two children (median age 2.5 years, range 0.1-16.0; weight 11.2 kg [3.8-62.0]) were included. Patients had received continuous intravenous infusion of opioids and benzodiazepines for 16.0 days (4.0-34.0) when drug rotation was started. The median dose of continuous intravenous infusion of ketamine was 4.0 mg·kg-1·h-1 (1.8-6.0) and the median duration was 3.0 days (2.0-6.0). After having restarted opioids, fentanyl doses were significantly lower compared with the time before the drug rotation began (after, 2.9 µg·kg-1·h-1 [0.8-4.9] vs before, 4.15 µg·kg-1·h-1 [1.2-10.0]; p < 0.001). Continuous intravenous infusion of midazolam and clonidine were unchanged during drug rotation. COMFORT-B scoring was significantly lower after having started drug rotation (after, 14.5 [8-19] vs before, 16 [11-22]; p < 0.001). CONCLUSION: Drug rotation with ketamine in mechanically ventilated children with opioid tolerance is feasible and seems to reduce the rate of fentanyl infusion.

Effects of perfluorohexan vapor on gas exchange, respiratory mechanics, and lung histology in pigs with lung injury after endotoxin infusion.

BACKGROUND: Inhaled perfluorohexan vapor has been shown to improve gas exchange and pulmonary mechanics in oleic acid- and ventilator-induced lung injury. However, in the clinical setting, lung injury frequently occurs in the context of systemic inflammation and consecutive lung injury, which may be induced experimentally by intravenous administration of endotoxin. The authors studied whether vaporized perfluorohexan is efficacious during endotoxin-induced lung injury in domestic pigs. METHODS: Twenty-two pigs (29 [23, 31] kg body weight [first, third interquartile]; tracheostomy) were anesthetized and mechanically ventilated. In the endotoxin (n = 8) and perfluorohexan groups (n = 7), we administered endotoxin of Escherichia coli 111:B4, 1 mg.kg . h for 1 h and 10 microg.kg.h for 5 h in consecutive order. In the perfluorohexan group, inhalation of the test drug was started 2 h 30 min after the start of the intravenous endotoxin and terminated after 30 min. In a control group (n=7), animals were instrumented and observed over time without further intervention. Oxygenation function was assessed from oxygen partial pressures (Po2, blood gases) and calculated shunt fraction. Respiratory compliance was calculated from airway pressure and tidal volume. Measurements were performed before and every hour during endotoxin infusion. RESULTS: After 6 h of endotoxin, gas exchange and pulmonary compliance were deteriorated in the endotoxin group (Pao2: 184 [114, 289] vs. 638 [615, 658] mmHg, pulmonary shunt fraction: 30 [23, 38] vs. 4 [3, 6]%, respiratory compliance: 12 [11, 14] vs. 22 [19, 23] ml/mbar; P < 0.05, endotoxin vs. control). Inhalation of vaporized perfluorohexan did not improve Pao 2 (107 [60, 221] mmHg), pulmonary shunt fraction (32 [26, 58]%), or respiratory compliance (14 [10, 17] ml/mbar) when compared with intravenous endotoxin (not significant, perfluorohexan vs. endotoxin). CONCLUSIONS: Inhalation of vaporized perfluorohexan does not improve pulmonary gas exchange or respiratory compliance in endotoxin-induced porcine lung injury.