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Kingella spp. have emerged as an important cause of invasive pediatric diseases. Data on Kingella infective endocarditis (KIE) in children are scarce. We compared the clinical features of pediatric KIE cases with those of Streptococcus species IE (StIE) and Staphylococcus aureus IE (SaIE). A total of 60 patients were included in the study. Throughout the study period, a rise in incidence of KIE was noted. KIE patients were significantly younger than those with StIE and SaIE, were predominately boys, and had higher temperature at admission, history of oral aphthae before IE diagnosis, and higher lymphocyte count (p

Systemic sclerosis sine scleroderma (ssSSc) is a rare variant of systemic sclerosis, with only one pediatric case reported in the medical literature to date. Pulmonary arterial hypertension as the presenting feature of ssSSc is extremely rare, even in adults, and so far has never been reported in children. We report, for the first time, a case of pediatric ssSSc in a 3-year-old girl, who presented with interstitial lung disease and pulmonary hypertension. The patient was prescribed early aggressive pulmonary vasodilators combined with anti-inflammatory medications. The clinical response was good, and her current condition at 12 years of age is remarkable, considering the high mortality rates reported in adults. We underscore the importance of early aggressive treatment in future cases of similar presentation.

Background Limited fluoroscopy ablation using 3D electro-anatomical system (3DS) has been used for arrhythmias in children, however it is not a common practice. We aimed to facilitate a fluoroscopy limited approach for ablation of accessory pathways (AP) in children. Methods Following electrophysiologic (EP) catheter placement a single dual-plane fluoroscopic image (right anterior oblique-30° and left anterior oblique-60° views) was acquired and the 3DS views were rotated to be a perfect match to the fluoroscopy. Ninety-four consecutive pediatric patients [mean age 11.8 ± 4.1 (4.2–18) years, 61.7% males] with Wolf–Parkinson–White syndrome underwent ablation of an AP. Fifty-seven had manifest AP, 54 had left-sided AP (LSAP) and 40 had right-sided AP (RSAP). Results The acute success rate was 95.7% (90/94), with a recurrence rate of 1.1% (1/90) at a mean follow-up of 13 ± 5.5 (4.4–22.9) months. Mean procedure and fluoroscopy times were 144 ± 45 (55–262) min and 1.8 ± 1.4 (0.1–5.6) min, respectively. Comparison of the first 20 procedures to the next 74 procedures demonstrated an extended procedure time (171 ± 53 min vs 135 ± 38 min, p

Coil embolization of pulmonary arteriovenous malformations (PAVMs) has a high re-canalization/re-perfusion rate. Embolization with Amplatzer plugs has been previously described, but the long-term efficacy is not established. This study reports the experience of a referral medical center with the use of coils and Amplatzer plugs for treating PAVMs in patients with hereditary hemorrhagic telangiectasia.The study was approved by the Institutional Review Board with waiver of informed consent. The cohort included all patients who underwent PAVM embolization in 2004-2014 for whom follow-up imaging scans were available. The medical files were retrospectively reviewed for background data, embolization method (coils, Amplatzer plugs, both), and complications. Re-canalization of treated PAVMs was assessed from intrapulmonary angiograms (following percutaneous procedures) or computed tomography angiograms. Fisher's exact test and Pearson Chi-squared test or t test were used for statistical analysis, with significance at p 0.05.16 patients met the study criteria. Imaging scans were available for 63 of the total 110 PAVMs treated in 41 procedures. Coils were used for embolization in 37 PAVMs, Amplatzer plugs in 21, and both in five. Median follow-up time was 7.7 years (range 1.4-18.9). Re-canalization was detected in seven vessels, all treated with coils; there were no cases of re-canalization in plug-occluded vessels (p = 0.0413).The use of Amplatzer plugs for the embolization of PAVMs in patients with hemorrhagic telangiectasia is associated with a significantly lower rate of re-canalization of feeding vessels than coils. Long-term prospective studies are required to confirm these findings.

Because of its safety profile, cryoablation has become an alternative therapy for septal arrhythmias, including parahisian accessory pathways (APs). Data regarding its efficacy, safety, and late outcome for parahisian APs in children are limited.The purpose of this study was to evaluate the safety and efficacy of cryoablation of parahisian APs in children.Fifty-nine cryoablations of parahisian APs were performed in 50 patients [76% males; median age 16.5 years (range 5.3-20 years)]. Thirty-three had manifest APs and 17 had concealed APs. The time to effect was defined as the duration of time from reaching -30°C to the disappearance of AP during the cryomapping phase. The acute success rate for the first ablation was 94% (47/50).Mean fluoroscopy time and number of cryoablations were 28.5 ± 23.3 minutes and 2.6 ± 1.2 seconds, respectively. The time to effect was 8.5 ± 7.1 seconds. The overall recurrence rate was 14.9%, decreasing from 40% in the first 10 cases to 8.1% in the next 37 (P.02). Multivariate analysis showed that the only independent predictor for recurrence rate was time to effect10 seconds (P.001). A high recurrence rate was also associated with concealed APs and ablation during supraventricular tachycardia (P.03 and P.05, respectively). The patients who had recurrence underwent a second successful cryoablation. During median follow-up of 59.7 months (range 6-102 months), a long-term success rate of 94% was achieved. No permanent ablation-related complications occurred.Cryoablation of parahisian APs has excellent short- and long-term efficacy and safety profile. Increased physician experience is associated with a reduced recurrence rate.

Background Limited fluoroscopy cryo-ablation using a 3D electro-anatomical system (3DS) has been used for AVNRT in children. We aimed to facilitate a fluoroscopy limited approach of RF ablation of AVNRT in children. Methods A retrospective study was performed of procedure parameters in children undergoing RF ablation of AVNRT in 75 consecutive children (June 2011 to November 2013 — Group A) using standard fluoroscopy techniques compared to those of 64 consecutive children (December 2013 to May 2015 — Group B), using a fluoroscopy limited approach with 3DS. Results The acute success rate was 98.7% (74/75) and 98.4% (63/64) for groups A and B, respectively. The recurrence rate was 2.7% (2/74) and 0% (0/63) with a mean follow-up period of 45.5±12.1 and 14.3±6.1months for group A and group B, respectively. The mean procedure and fluoroscopy times were significantly lower for group B compared to group A (119±37 (43–203) and 0.83±1.04 (0.05–3.83) minutes versus 146±53 (72–250) and 16.1±8.9 (4.39–55) minutes, p p Conclusions A fluoroscopy limited approach for RF ablation of AVNRT in children using a 3DS is easily acquired and adapted, and significantly reduces the fluoroscopy and procedure time with excellent efficacy, safety and low recurrence rate. Condensed ABSTRACT This study confirmed that a 3D mapping system (3DS) to guide ablations of AVNRT in children reduces radiation exposure. Combined, limited fluoroscopy and 3DS in a methodology that resembles the familiar conventional fluoroscopy approach for RF ablation of AVNRT in children is proposed. Combined limited fluoroscopy and RF-energy in children with AVNRT are associated with a shorter procedure time, minimal fluoroscopy time, a high success rate and a low recurrence rate.

Aims Assessment of the feasibility of creating real-time interactive 3D digital holograms in a standard catheterization laboratory. 3D medical images are typically displayed and interacted with on 2D screens limiting their usefulness. A digital computer-generated real-time holographic display of patient's 3D data could provide a spatially accurate image with all the depth cues and afford interaction within the image. Methods and results We performed a feasibility study of creating real-time interactive 3D digital holograms with a purpose-built prototype using intraprocedural data from 3D rotational angiography and live 3D transesophageal echocardiography. The primary objective was to demonstrate that all the anatomical landmarks identified on standard imaging can be similarly identified using dynamic and static holographic images. The secondary objective was to demonstrate the usability of interactions with the image. Parameters were assessed by a rating scale. Eight patients were enrolled of whom five underwent transcatheter ASD closure using 3DTEE and three patients were evaluated by 3D rotational angiography. In all cases dynamic real-time and static 3D holograms were created in standard cath lab conditions. Four individual observers identified all anatomical landmarks on the holographic display independently from the 2D display. Interactions with the hologram including marking, cropping and rotation were performed. There were no adverse events. Conclusions This study demonstrates, for the first time, the feasibility of generating high quality, clinically relevant, 3D real-time colour dynamic holograms in a standard clinical setting with real patient volumetric data. The impact of computer-generated holography needs to be evaluated in controlled clinical trials.

When bronchopulmonary dysplasia (BPD) is complicated by pulmonary hypertension (PH), morbidity and mortality are significantly increased. BPD-associated PH is not included in the current indications for PH medications. However, limited data demonstrate hemodynamic improvement and decreased mortality with PH-specific treatment. This report describes our 6-year experience treating BPD-associated PH with PH medications, mainly sildenafil.The medical records of 20 infants diagnosed with BPD-associated PH at a tertiary pediatric pulmonary hypertension clinic in 2008-2014 were reviewed. Clinical improvement was defined as a decrease in Ross functional class by at least one degree. PH severity was classified by echocardiography as mild, moderate, or severe. Hemodynamic improvement was defined as a decrease in PH severity by at least one level.Eighteen out of 20 patients were treated with PH medications: 12 sildenafil, 5 sildenafil and bosentan, and 1 bosentan. Median follow-up time was 2 years. Mean functional class significantly decreased from 3.2 ± 0.9 at diagnosis to 1.7 ± 0.9 at the last follow-up. Improvement in functional class was observed in 15/16 children (94%). Moderate or severe PH was found in 13/18 children (72%) at diagnosis, and in three (17%, all moderate PH) at the last follow-up. Improvement in PH class by echocardiography was demonstrated in 14/18 children (78%). The survival rate was 95%.Treatment of BPD complicated by PH with PH-specific medications, mainly sildenafil, is associated with improvement in both clinical and hemodynamic parameters and a low mortality rate. Pediatr Pulmonol. 2017;52:77-83. © 2016 Wiley Periodicals, Inc.

INTRODUCTION Anomalous origin of one pulmonary artery from the ascending aorta is a rare cardiac anomaly in which the pulmonary artery abnormally arises from the ascending aorta. Physiologically, most patients develop signs of cardiac failure due to high flow to both lungs, with systemic or supra-systemic pressures in the normally connected lung. The purpose of this study is to present our experience with this rare anomaly, in which early anatomic repair lead to rapid physiologic correction. MATERIALS AND METHODS Retrospective case review of all patients with anomalous origin of one pulmonary artery from the ascending aorta at Schneider Children's Medical center of Israel between 1986 and 2007. All clinical operative and echocardiographic charts were analysed. RESULTS Twelve patients were diagnosed as anomalous origin of one pulmonary artery from the ascending aorta. In 10 patients, the right pulmonary artery rose from the ascending aorta, while in two an anomalous origin of the left pulmonary artery was associated with a right aortic arch. Initial diagnoses was made with two-dimensional echocardiography in all patients. In six patients, diagnostic cardiac catheterisation was performed in order to confirm the diagnosis. Age at diagnosis ranged from 5 to 180 days with a median of 15 days, and patient weight ranged from 780 grams to 5 kilograms, with a median of 3 kilograms. Initial echocardiographic evaluation showed systemic (four patients) or supra-systemic (seven patients) pressures in the right ventricle and normally connected lung. All underwent surgical repair. There was no operative mortality. All reconstructed patients achieved normal right ventricular pressures within days after surgery. The flow pattern in both pulmonary arteries was normalised. CONCLUSIONS Early surgical repair of anomalous origin of one pulmonary artery from the ascending aorta is feasible and safe even in newborn and premature babies with complete resolution of the pulmonary hypertension and normalisation of pulmonary vascular resistance.

Heterozygous mutations in the FOXF1 transcription factor gene are implicated in alveolar capillary dysplasia with misalignment of pulmonary veins (ACDMPV), a developmental disorder of the lungs classically presenting with pulmonary hypertension and early demise. Evidence has suggested haploinsufficiency and partial paternal imprinting. We present a family with several affected members with an extremely variable phenotype.The index patient presented several hours after birth with severe pulmonary hypertension. She is now 3-years old, thriving on maximal pulmonary hypertension therapy, chronic steroids, and oxygen. One of the patient's siblings died at 16 days with pulmonary hypertension and an annular pancreas, consistent with classical ACDMPV.Whole exome sequencing was performed in the index case. The identified variant was confirmed by Sanger sequencing, and tested in the remaining family members. Parental origin was determined by PCR amplification and cloning, sequencing, and identification of adjacent single nucleotide polymorphisms. Echocardiography was performed in the asymptomatic carriers.Whole exome analysis revealed a novel, predictably pathogenic heterozygous missense mutation, g.chr16:86544406 CA NM_001451, c.C231A, p.F77L, in the FOXF1 gene. The mutation arose in the father, de novo, early postzygotically, with 70% somatic mosaicism in the blood, on the grandpaternal chromosome. It was also present in the proband's asymptomatic sister, found to have partial anomalous pulmonary venous return.FOXF1 mutations may have an extremely variable phenotype, possibly as a result of somatic mosaicism and complex gene regulation including unorthodox imprinting of the gene locus. The prolonged survival of the proband suggests the need for aggressive treatment. Pediatr Pulmonol. 2016; 51:921-927. © 2016 Wiley Periodicals, Inc.

Digoxin and gentamicin are widely used in pediatric medicine, and therapeutic monitoring is mandatory because of their narrow margin of safety and wide interpatient and intrapatient pharmacokinetic variabilities. Saliva sampling may be of potential interest, especially in children, in whom blood sampling is often difficult. In 11 children treated with digoxin for various cardiac conditions, and in 24 children treated with gentamicin (14 patients were administered gentamicin three times a day, and 10 once-daily), drugs levels were measured in plasma and saliva. There was no correlation between plasma total or free digoxin concentrations and saliva levels, precluding the clinical use of the saliva test for digoxin. No correlation was found between plasma gentamicin concentrations and saliva levels when the drug was administered three times a day; however, good correlation was found when the drug was administered once-daily (r2 = 0.89, p < 0.0001). Saliva may be used as a noninvasive method of measuring gentamicin serum concentrations to guide dosage adjustments in patients administered the drug once-daily.

To present our initial experience with the Amplatzer Duct Occluder II Additional Sizes (ADOIIAS) device.The ADOIIAS is a modified PDA closure device of various lengths and widths with small disks to avoid flow disturbance in the pulmonary arteries and descending aorta.All patients who underwent attempted closure with an ADOIIAS device at our institution CATHETERIZATION: Following aortography the PDA was closed using a 4-5Fr delivery system from the pulmonary or aortic side with an ADOIIAS. Aortography to confirm position and leak was performed before and after device release. Echocardiography was performed before discharge the following day.Between June 2011 and December 2012, 60 patients [33 female], median age 3.3 yrs [0.6-15.8 years], and weight 14.5 kg [4-79] underwent attempted PDA closure with an ADOIIAS device. 56/60 [93.3%] ADOIIAS devices were successfully deployed [52 aortic side]. In 55/56 [98.2%], the PDA was closed on follow up echocardiogram usually on the next day. In one case, there was a small residual PDA of no hemodynamic significance on follow up. In four cases, the ADOIIAS was unstable, including one device embolization which was retrieved with no sequelae, and the PDA was closed in all cases with an alternative device in the same procedure. The mean ratio of ADOIIAS height:PDA pulmonary diameter was 2.4 ± 0.5:1.We report a large single-center experience with the new ADOIIAS device which proved to be versatile, safe, effective, and easy to use in the appropriate ductal anatomy.

Congenital portosystemic shunts (CPSS) with portal venous hypoplasia cause hyperammonemia. Acute shunt closure results in portal hypertension. A transcatheter method of staged shunt reduction to afford growth of portal vessels followed by shunt closure is reported.Pressure measurements and angiography in the CPSS or superior mesenteric artery (SMA) during temporary occlusion of the shunt were performed. If vessels were diminutive and the pressure was above 18 mmHg, a staged approach was performed, which included implantation of a tailored reducing stent to reduce shunt diameter by ~50 %. Recatheterization was performed approximately 3 months later. If the portal pressure was below 18 mmHg and vessels had developed, the shunt was closed with a device.Six patients (5 boys, 1 girl) with a median age of 3.3 (range 0.5-13) years had CPSS portal venous hypoplasia and hyperammonemia. Five patients underwent staged closure. One patient tolerated acute closure. One patient required surgical shunt banding because a reducing stent could not be positioned. At median follow-up of 3.8 (range 2.2-8.4) years, a total of 21 procedures (20 transcatheter, 1 surgical) were performed. In all patients, the shunt was closed with a significant reduction in portal pressure (27.7 ± 11.3 to 10.8 ± 1.8 mmHg; p = 0.016), significant growth of the portal vessels (0.8 ± 0.5 to 4.0 ± 2.4 mm; p = 0.037), and normalization of ammonia levels (202.1 ± 53.6 to 65.7 ± 9.6 μmol/L; p = 0.002) with no complications.Staged CPSS closure is effective in causing portal vessel growth and treating hyperammonemia.

Bronchopulmonary fistulae (BPFs) are a severe complication of lobectomy and pneumonectomy and are associated with high rates of morbidity and mortality. We have developed a novel, minimally invasive method of central BPF closure using Amplatzer devices (ADs) that were originally designed for the transcatheter closure of cardiac defects. Ten patients with 11 BPFs (eight men and two women, aged 66.3±10.1 years [mean±SD]) were treated under conscious sedation with bronchoscopic closure of the BPFs using ADs. A nitinol double-disk occluder device was delivered under direct bronchoscopic guidance over a guidewire into the fistula. By extruding a disk on either side of the BPF, the fistula was occluded. Bronchography was performed by injecting contrast medium through the delivery sheath following the procedure to ensure correct device positioning. In nine patients, the procedure was successful and symptoms related to the BPF disappeared following closure by the AD. The results were maintained over a median follow-up period of 9 months. Therefore, we state that endobronchial closure using an AD is a safe and effective method for treatment of a postoperative BPF.

Objectives: To report on the methods and results of treatment of large congenital coronary-cameral fistulae by transcatheter closure with Amplatzer devices. Background: Large coronary-cameral fistulae cause a steal phenomenon from the normal coronary circulation. Surgical closure is an option. However, transcatheter methods allow for temporary occlusion, definition of anatomy, and online assessment of successful closure. Amplatzer devices are compact occluders that can be fully delivered, collapsed, and repositioned until a satisfactory position is attained. Methods: Coronary and fistula anatomy were defined by selective coronary angiography with or without temporary occlusion. Device closure of the fistula was performed at the most distal point accessible, often from the cameral side using an arteriovenous loop method. Results: Ten patients of median age 2.6 years (0.5–52.2) and weight 14.4 kg (6.1–67) underwent an attempt at transcatheter closure of a large fistula. In nine patients, the fistula was closed successfully with a device. There were no complications. Conclusions: Transcatheter closure of coronary-cameral fistula with Amplatzer devices is safe and effective. © 2009 Wiley-Liss, Inc.

Objectives: To report on the early results of treatment of coarctation of the aorta by dilation with a new polytetrafluoroethylene covered stent. Background: Transcatheter dilation of aortic coarctation carries the risk of aneurysm or rupture. Covered stent implantation reduces this risk but requires a large delivery system. The Advanta V12 LD covered stent is premounted and requires a 9–11 Fr delivery system. Methods: Covered stents on balloons of a diameter sufficient to anchor the stent in the coarctation were implanted using the smallest available delivery system. Secondary dilation with larger diameter balloons was performed until the pressure gradient was

Objective: To review the authors' experience with transcatheter closure of pulmonary arteriovenous malformations (PAVMs) using amplatzer duct occluder (ADO) devices and vascular plugs (AGA Medical, Golden Valley, MN) and present a novel technique for delivery sheath placement and device delivery. Background: PAVMs can cause cyanosis, fatigue, polycythemia, and thromboembolic phenomena. Transcatheter closure using coils, detachable balloons, and various devices has replaced surgery as the preferred therapy. Methods: Between January 2001 and December 2004, five patients (2M, 3F) of median age 33 years (14–49) were referred for transcatheter closure of multiple PAVMs. All patients were diagnosed previously with hereditary hemorrhagic telangectasia. The procedures were performed with sedation using a percutaneous transcatheter technique via the femoral vein under fluoroscopic guidance. Results: A total of 14 PAVMs (11 ADO and 3 plugs) were closed in five patients. Three patients required two procedures after developing additional PAVMs. All attempts at PAVM closure were successful. Oxygen saturation increased from 88.4 ± 6.1 to 96.4 ± 0.5 (P < 0.05). No complications, including air or thromboembolism, hemoptysis, or chest pain, occurred. At median follow-up of 3.4 years (1.4–3.6), all patients are alive and have suffered no embolic phenomena or infection. Conclusion: Amplatzer patent ductus arteriosus occluders and vascular plugs are safe and effective in the closure of PAVMs in the acute setting and at intermediate follow-up. Placement of a long delivery sheath can be facilitated by the methods outlined. Though promising, further clinical evaluation of these devices is required. Their apparent advantages must be compared to other techniques and devices for transcatheter PAVM closure. © 2006 Wiley-Liss., Inc.