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.
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.
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.
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.
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.
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.
Objective Right ventricular–pulmonary arterial coupling is an important determinant in the development of right ventricular failure. The purpose of our study was to assess right ventricular–pulmonary ...arterial coupling in children and adolescents with dilatation of the right ventricle after repair of tetralogy of Fallot. Methods Right ventricular–pulmonary arterial coupling was quantified as the ratio of pulmonary arterial elastance (an index of arterial load) and right ventricular end-systolic elastance (an index of contractility) using pressure-volume loops with conductance catheters at the baseline level and during dobutamine infusion. Results A total of 24 patients (mean age, 16.7 ± 7.0 years) after tetralogy of Fallot repair were enrolled in the present study. End-systolic elastance showed an appropriate increase under inotropic stimulation from 0.24 ± 0.18 to 0.47 ± 0.39 mm Hg/mL/m2 ( P < .01). Simultaneously, the arterial elastance increased from 0.50 ± 0.28 to 0.72 ± 0.48 mm Hg/mL/m2 ( P < .01). Right ventricular–pulmonary arterial coupling was impaired at rest and did not improve significantly under dobutamine stress in the entire study population (arterial elastance/end-systolic elastance decreased from 3.0 ± 2.8 to 2.7 ± 3.1; P = .70). Patients with transannular patch repair (n = 11) showed significant uncoupling in response to dobutamine (arterial elastance/end-systolic elastance increased from 2.0 ± 0.8 to 3.7 ± 4.1), and coupling even improved with dobutamine in patients who had undergone a transatrial approach (arterial elastance/end-systolic elastance decreased from 1.6 ± 1.0 to 0.9 ± 0.6; P = .04). Conclusions Our study demonstrated that right ventricular–pulmonary arterial coupling is impaired in patients with tetralogy of Fallot and is mainly affected by the surgical strategy used at the primary repair. These results elucidate the emerging role of ventricular-arterial interactions as a contributing mechanism for deterioration in right ventricular performance and impaired response to inotropic drugs in patients with tetralogy of Fallot.
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.
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.
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.
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.
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.
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 (
= 0.006*) and a distance of 775;638/854 m vs. 513;315/661 m (
= 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 (
= 0.02*), PLE occurred in 4/8 patient in group 1 vs. 1/25 patients in group 2 (
= 0.008*).
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.
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.
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.
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
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
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.
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 10-year-old girl presented with a supraventricular tachycardia. A heart murmur was detected during a clinical examination; therefore, echocardiography was performed. This revealed a giant right ...ventricular myxoma that subtotally obstructed the right ventricular outflow tract. A myxoma in the right ventricle is uncommon in children. This rare intracardiac tumor was examined using multimodality imaging, and the patient underwent surgical resection. The pathologic features confirmed our suspicion and revealed an encapsulated tumor with myxomatoid stroma containing focal hemorrhages.
Early detection of right ventricular (RV) dysfunction is essential in the assessment of patients with repaired tetralogy of Fallot (TOF). This study aimed to assess latent RV dysfunction in ...asymptomatic patients with TOF and to determine the predictive value of B-type natriuretic peptide (BNP). Pressure–volume loops were recorded for 16 young patients (New York Heart Association class 1 or Ross class 0; median age, 14.2 years) using the conductance catheter technique. All the patients had RV dilation secondary to pulmonary regurgitation after surgical repair of TOF. Indexes of RV function were derived at baseline level and during dobutamine infusion. Contractility was calculated by the slope of the end-systolic pressure–volume relation (ESPVR). An increase in ESPVR during dobutamine infusion was considered to indicate contractile reserve as a marker for latent RV dysfunction. The median ESPVR significantly increased from 0.32 mmHg/ml (0.13–0.72 mmHg/ml) at baseline to 0.57 mmHg/ml (0.24–1.55 mmHg/ml) during dobutamine infusion (
p
= 0.005). However, for five patients, no relevant increase in contractility was found, indicating impaired RV contractile reserve. There was only a weak inverse correlation between impaired contractile reserve and BNP (
r
= −0.28). Even asymptomatic patients with only a mildly enlarged right ventricle can have impaired RV function. Early RV dysfunction cannot be predicted accurately with BNP.
Background Catheter ablation has become the first line of therapy in patients with symptomatic, recurrent, drug refractory atrial fibrillation. However, catheter ablation of atrial fibrillation is ...still a challenge. This is partially due to the high degree of variability with regard to the individual anatomy. Nevertheless, 3D imaging systems (CT, MRI) provide detailed information about the individual left atrial and pulmonary vein morphology. A 3D CT or MRI reconstruction of the left atrium can be displayed in the Navx-/Ensite-system in a synchronised way during the ablation procedure, thereby facilitating the intervention. This study summarizes our preliminary experience with different strategies of AF ablation using the Navx-/Ensite-system and a CT-/MRI-guided approach. Methods In a total of 41 patients, cardiac MRI (n = 7) or multi-detector spiral computed tomography (n = 34) was performed prior to an ablation procedure. Catheter ablation was performed for paroxysmal atrial fibrillation in 31 patients and for persistent atrial fibrillation in 10 patients. A 3D MRI or high resolution spiral CT data acquisition was performed and a surface rendered model of the LA was created. This model was displayed in the Navx-/Ensite-system throughout the ablation procedure. Results Catheter ablation was performed using the Navx-system (n = 38) or the Ensite-system (n = 3). Three strategies were used depending on the type of atrial fibrillation: segmental isolation of the pulmonary veins (facilitated by a 3D real-time visualization of the ablation catheter and a circumferential mapping catheter; group A: 20 patients), linear lesions (group C: 3 patients) and a combined approach (group B; 18 patients). The CT-/MRI-models provided an excellent overview over the pulmonary veins and the left atrial appendage. They revealed a high degree of variability with regard to the individual anatomy (e.g. dimensions of the left atrial appendage, pulmonary vein ostia). The CT scans provided a more detailed reconstruction of the left atrial anatomy than the MRI scans (especially in patients who were in atrial fibrillation at the time of the data acquisition). In some patients, the CT-/MRI-models revealed a very small diameter of some pulmonary veins or side branches close to the ostium (e.g. right inferior pulmonary vein). Therefore, no attempt was made to achieve complete pulmonary vein isolation in some patients. In group A, 16/20 (80%) patients had no arrhythmia recurrence mean follow-up 359 days (SD ± 317 days). Twelve out of eighteen (67%) patients in group B mean follow-up 452 days (SD ± 311 days) and 2/3 (67%) patients in group C did not experience an arrhythmia recurrence mean follow-up 1,000 days (SD ± 34 days). There were no major complications. Conclusions The information derived from 3D CT- or MRI-reconstructions facilitates AF ablations performed with the Navx-/Ensite-mapping system and enhances the safety of these procedures. Furthermore, the availability of an additional impedance-based 3D real-time visualization of the ablation catheter and the circular mapping catheter placed in the pulmonary veins represents a major advantage of the Navx system.
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