Clinical Features and Comparison of Kingella and Non-Kingella Endocarditis in Children, Israel.

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<0.05). Pediatric KIE exhibits unique features compared with StIE and SaIE. Therefore, in young healthy children <36 months of age, especially boys, with or without a congenital heart defect, with a recent history of oral aphthae, and experiencing signs and symptoms compatible with endocarditis, Kingella should be suspected as the causative pathogen.

The Amplatzer Duct Occluder II Additional Sizes device for transcatheter PDA closure: initial experience.

OBJECTIVES: To present our initial experience with the Amplatzer Duct Occluder II Additional Sizes (ADOIIAS) device. BACKGROUND: 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. PATIENT POPULATION: 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. RESULTS: 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. CONCLUSIONS: 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.

Three-Patch Reconstruction of Hourglass Supravalvar Pulmonary Artery Stenosis Mid-Term Results.

BACKGROUND: Some patients with pulmonary stenosis present with a complex multilevel disease that involves the valve, the leaflets, and the sinotubular junction (STJ) forming an hourglass appearance. We herein report the mid-term results of our experience with the reconstruction of the supravalvar narrowing using three pericardial patches. MATERIAL AND METHODS: Retrospective analysis of patient charts and echocardiography studies of patients who underwent three-patch reconstruction of the pulmonary valve (PV) from 2013 to 2022. After PV transection distal to STJ, vertical incisions into the sinuses were performed, and leaflets were trimmed and thinned. The three sinuses were augmented using three pericardial patches. RESULTS: Nineteen patients underwent repair of hourglass supravalvar pulmonary stenosis. Mean weight at surgery was 9.4 kg (median 7.2, range 4.7-35); 16 patients underwent previous catheterization with unsuccessful balloon dilatation of the PV (13 pts.). Preoperative aortic/pulmonary annulus ratio was 1.02 (median 1, range 0.89-1.25). After surgery, gradients across the PV were significantly reduced (94 ± 26 vs 29 ± 9 mm Hg, P = .02). Postoperatively, 14 patients had mild or no pulmonary insufficiency (PI) and five had mild to moderate PI. At a mean follow-up of 71 months (median 78 months, range 8-137), gradients continued to decrease (29 ± 9 vs 15 ± 5 mm Hg, P < .001). CONCLUSION: The three-patch technique for the repair of supravalvar pulmonary stenosis is simple, reproducible, and achieves excellent and long-standing relief of the right ventricular outflow tract gradient.

The Amplatzer duct occluder (ADOII) and Piccolo devices for patent ductus arteriosus closure: a large single institution series.

Purpose: Evaluate Piccolo and ADOII devices for transcatheter patent ductus arteriosus (PDA) closure. Piccolo has smaller retention discs reducing risk of flow disturbance but residual leak and embolization risk may increase. Methods: Retrospective review of all patients undergoing PDA closure with an Amplatzer device between January 2008 and April 2022 in our institution. Data from the procedure and 6 months follow-up were collected. Results: 762 patients, median age 2.6 years (range 0-46.7) years and median weight 13 kg (range 3.5-92) were referred for PDA closure. Overall, 758 (99.5%) had successful implantation: 296 (38.8%) with ADOII, 418 (54.8%) with Piccolo, and 44 (5.8%) with AVPII. The ADOII patients were smaller than the Piccolo patients (15.8 vs. 20.5 kg, p < 0.001) and with larger PDA diameters (2.3 vs. 1.9 mm, p < 0.001). Mean device diameter was similar for both groups. Closure rate at follow-up was similar for all devices ADOII 295/296 (99.6%), Piccolo 417/418 (99.7%), and AVPII 44/44 (100%). Four intraprocedural embolizations occurred during the study time period: two ADOII and two Piccolo. Following retrieval the PDA was closed with an AVPII in two cases, ADOI in one case and with surgery in the fourth case. Mild stenosis of the left pulmonary artery (LPA) occurred in three patients with ADOII devices (1%) and one patient with Piccolo device (0.2%). Severe LPA stenosis occurred in one patient with ADOII (0.3%) and one with AVPII device (2.2%). Conclusions: ADOII and Piccolo are safe and effective for PDA closure with a tendency to less LPA stenosis with Piccolo. There were no cases of aortic coarctation related to a PDA device in this study.

Transcatheter closure of large congenital coronary-cameral fistulae with Amplatzer devices.

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.

Coarctation of the aorta treated with the Advanta V12 large diameter stent: acute results.

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 <20 mm Hg and the stent was opposed to the aortic wall. RESULTS: Twenty-five patients with aortic coarctation underwent stent implantation. Coarctation diameter increased from (6.3 + or - 3.5) mm to (14.4 + or - 2.3) mm (P < 0.0001). Peak pressure gradient decreased from (25.3 + or - 11.6) mm Hg to (2.5 + or - 3.0) mm Hg (P < 0.0001). The stent achieved the desired diameter in all cases. There were no complications. At short-term median follow-up of 4.9 months, all patients are alive and well with no evidence of recoarctation or aneurysm. CONCLUSIONS: These initial results show that the covered Advanta V12LD stent is safe and effective in the immediate treatment of coarctation of the aorta through a low profile delivery system of 8-11 Fr. Long term follow up is required.

Anomalous origin of the pulmonary artery from the aorta: early diagnosis and repair leading to immediate physiological correction.

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.

Systemic Sclerosis Sine Scleroderma With Pulmonary Arterial Hypertension in a 3-Year-Old Girl.

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.

Covered Cheatham-Platinum stents for serial dilation of severe native aortic coarctation.

OBJECTIVES: To report on the early results of treatment of native coarctation of the aorta by implantation and serial dilations of covered stents. BACKGROUND: Transcatheter dilation of native coarctation of the aorta carries a risk of aneurysm or rupture. Covered stent implantation requires a large delivery system with the risk of vascular damage. METHODS: Covered stents on balloons of diameter sufficient to anchor the stent in the coarctation were implanted using the smallest delivery system possible. Dilation with larger diameter balloons was performed until the pressure gradient was <20 mm Hg and the stent was opposed to the aortic wall. RESULTS: Twenty-two patients with native coarctation underwent stent implantation. Coarctation diameter increased from 3.6 +/- 1.9 to 12.6 +/- 1.9 mm (P < 0.001). Peak pressure gradient decreased from 29.4 +/- 8.5 to 6.7 +/- 5.7 mm Hg (P < 0.001). Nine patients underwent further dilation on average 5 months later. Residual pressure gradient decreased from 12.3 +/- 5.8 to 2.1 +/- 2.9 mm Hg (P = 0.002). The stent achieved the diameter of the transverse arch in all cases. Complications included a small tear at further dilation treated with a second stent and a femoral pseudoaneurysm. At short-term follow-up of 18.5 months all patients are alive and well with no evidence of recoarctation or aneurysm. CONCLUSIONS: These initial results show that serial dilation of covered Cheatham-Platinum stents is feasible, safe, and an effective percutaneous method for the treatment of native coarctation of the aorta. However, long-term follow up is required.

Catheter ablation of left-sided accessory pathways in small children.

BACKGROUND: Accessory pathways (APs) are a common reason for supraventricular tachycardia in small children. Trans-septal puncture (TSP) approach is commonly used for the ablation of left-sided APs, however it may be challenging in small children. The aim of this study was to assess the efficacy and safety of trans-septal approach radiofrequency (RF) ablation of left-sided APs in children weighing up to 30 kg. METHODS: Of the 658 children who underwent catheter ablation of APs since 06/2002, 86 children had left-sided AP and weighed less than 30 kg. TSP approach guided with TEE and fluoroscopy was used for left atrium access. RESULTS: The mean age, height, and body weight were 7.6 ± 1.9 years, 122.4 ± 9.3 cm and 24.0 ± 4.2 kg, respectively. Forty-three children (50%) were male, 46 of 86 (53%) had manifest AP, 17 of 86 (20%) weighed less than 20 kg and in 24 of 86 children (28%) a three-dimensional system (3DS) was used to reduce fluoroscopy time. The acute success rate was 98.8% (85/86), with a recurrence rate of 2.4% (2/85) in a mean follow-up of 66.2 ± 42.7 (9.1-184.2) months. The mean procedure time and fluoroscopy time were significantly lower for the 3DS group compared to the standard fluoroscopy group 131 ± 41 (55-262) and 2.4 ± 1.5 (1-6) minutes vs 164 ± 51 (62-249) and 27 ± 13 (8-77) minutes, P < 0.01 and P < 0.0001, respectively. There were no ablation-related complications. CONCLUSIONS: RF ablation of left-sided APs using TSP approach in small children had an excellent efficacy and safety profile. The use of 3DS significantly reduces the procedure and fluoroscopy time.

Pulmonary hypertension specific treatment in infants with bronchopulmonary dysplasia.

OBJECTIVE: 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. STUDY DESIGN: 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. RESULTS: 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%. CONCLUSION: 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.

Limited fluoroscopy catheter ablation of accessory pathways in children.

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±53min vs 135±38min, p<0.005), however the fluoroscopy time, the number of long applications, the time to effect, and the acute success rate were similar. There were no permanent ablation-related complications. CONCLUSIONS: A limited fluoroscopy approach for ablation of AP in children using 3DS is easily acquired, adapted, reduces the fluoroscopy time, and has an excellent efficacy and safety profile.

Radiofrequency catheter ablation of atrioventricular node reentrant tachycardia in children with limited fluoroscopy.

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<0.003 and p<0.0001, respectively). There were no ablation-related complications. 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.

Computer-generated real-time digital holography: first time use in clinical medical imaging.

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.

Variable phenotypic presentation of a novel FOXF1 missense mutation in a single family.

BACKGROUND: 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. PATIENTS: 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. METHODS: 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. RESULTS: Whole exome analysis revealed a novel, predictably pathogenic heterozygous missense mutation, g.chr16:86544406 C>A 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. CONCLUSION: 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.

Amplatzer Vascular Plugs Versus Coils for Embolization of Pulmonary Arteriovenous Malformations in Patients with Hereditary Hemorrhagic Telangiectasia.

PURPOSE: 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. METHODS: 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. RESULTS: 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). CONCLUSION: 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.

Cryotherapy ablation of parahisian accessory pathways in children.

BACKGROUND: 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. OBJECTIVE: The purpose of this study was to evaluate the safety and efficacy of cryoablation of parahisian APs in children. METHODS: 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). RESULTS: 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 effect <10 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. CONCLUSION: Cryoablation of parahisian APs has excellent short- and long-term efficacy and safety profile. Increased physician experience is associated with a reduced recurrence rate.

Mitochondrial dysfunction and antiretroviral nucleoside analog toxicities: what is the evidence?

Mitochondrial dysfunction has been associated with long-term toxicities of human immunodeficiency virus (HIV) therapy, particularly with the nucleoside analog reverse transcriptase inhibitors (NRTIs). Lactic acidosis, hepatic steatosis, myopathies, cardiomyopathies, neuropathies, and lipodystrophy are frequently attributed to mitochondrial toxicity. Since mitochondrial toxicity could pose a major threat to the long-term success of HIV therapy, the scientific evidence underlying an association between mitochondrial toxicity and antiretroviral therapies, must be carefully examined. There is some data to support the association between NRTIs and mitochondria dysfunction. In this review, we examine human, animal, and in vitro data implicating mitochondrial dysfunction as the causal mechanism of NRTI-associated toxicity in HIV-infected patients.

Staged transcatheter treatment of portal hypoplasia and congenital portosystemic shunts in children.

PURPOSE: 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. METHODS: 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. RESULTS: 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. CONCLUSION: Staged CPSS closure is effective in causing portal vessel growth and treating hyperammonemia.

Endobronchial closure of bronchopleural fistulae using amplatzer devices: our experience and literature review.

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.