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McElhinney D.B.,Stanford University | Quartermain M.D.,Wake forest University | Kenny D.,Our Ladys Hospital for Sick Children | Alboliras E.,Banner Childrens Specialists | Amin Z.,Georgia Regents University
Circulation | Year: 2016

Background - Transcatheter closure of secundum atrial septal defects (ASD) using the Amplatzer septal occluder is generally safe and effective, but erosion into the pericardial space or aorta has been described. Although the absolute risk of this complication is low, there has been no assessment of relative risk factors. Methods and Results - All erosions reported to St. Jude Medical after ASD closure with an Amplatzer septal occluder (cases) were compared with controls (matched 2:1) who underwent ASD closure but did not develop an erosion. A total of 125 erosions were reported between 2002 and 2014, including 95 with an available echocardiogram. The median duration from implant to erosion was 14 days, but was >1 year in 16 patients. Nine patients (all age ≥17 years) who died were more likely to have an oversized device, and to have erosion into the aorta, than survivors. Aortic or superior vena cava rim deficiencies were more common in cases than in controls. In addition, larger balloon-sized ASD diameter, Amplatzer septal occluder device size, and device size-ASD diameter difference, and smaller weight:device size ratio were associated with erosion. On multivariable analysis, deficiency of any rim, device >5 mm larger than ASD diameter, and weight:device size ratio were associated with erosion. Conclusions - In addition to aortic rim deficiency, which was almost universal among erosion cases, there were several relative risk factors for erosion after ASD closure with the Amplatzer septal occluder device. To understand the mechanisms of and absolute risk factors for this uncommon but serious complication, an adequately powered prospective study with thorough echocardiographic evaluation will be critical. © 2016 American Heart Association, Inc. Source

Dhar R.,Our Ladys Hospital for Sick Children | Reardon W.,National Center for Medical Genetics | McMahon C.J.,Our Ladys Hospital for Sick Children
Cardiology in the Young | Year: 2015

We report a baby girl with an antenatal diagnosis of biventricular non-compaction and complete heart block detected at 22 weeks' gestation. Postnatal echocardiography confirmed severe biventricular non-compaction hypertrophic cardiomyopathy, multiple muscular ventricular septal defects, and mild-moderate pulmonary valve stenosis. Skeletal muscle biopsy confirmed complex 1 mitochondrial respiratory chain deficiency. An epicardial VVI pacemaker was implanted on day 3 of life and revised at 7 years of age. She remains stable at 8 years of age following pacing and medical treatment with carvedilol, aspirin, co-enzyme Q10, and carnitine. This represents the first report of biventricular non-compaction hypertrophic phenotype in association with congenital complete heart block and complex 1 mitochondrial respiratory chain deficiency in a child. © Cambridge University Press 2014. Source

Liddy S.,St Vincents University Hospital | Oslizlok P.,Our Ladys Hospital for Sick Children | Walsh K.P.,Our Ladys Hospital for Sick Children
Catheterization and Cardiovascular Interventions | Year: 2013

Objectives To compare the success and complication rates for the different devices and patent ductus arteriosus (PDA) types. Background There are numerous devices available for transcatheter closure of PDA but data comparing the success and complication rates of the newer devices are lacking. Methods A retrospective review was undertaken of all cases of transcatheter PDA closure at a tertiary referral center. Results Transcatheter PDA closure was achieved in 176 of 177 cases. Mean age, weight, and minimum ductal diameter were 3.3 years, 15.0 kg, and 2.5 mm, respectively. Devices used were Amplatzer Duct Occluder I (ADO I, 50.3%), Amplatzer Duct Occluder II (ADO II, 28.2%), Amplatzer Duct Occluder II Additional Sizes (ADO II AS, 5.1%), flipper coils (14.1%), and "other" devices (2.3%). Regarding Amplatzer devices, arterial delivery had a significantly shorter fluoroscopy time as compared to venous delivery (3.7 vs. 5.0 min, P = 0.0068), with no significant difference in outcomes. Immediate residual shunt rates were 7.9% (7/89) for ADO I, 4.1% (2/49) for ADO II, 11.1% (1/9) for ADO II AS, and 0% (0/25) for flipper coils. Aortic protrusion rates were 9% (8/89) for ADO I, 14.3% (7/49) for ADO II, and O% for both ADO II AS (0/9) and flipper coils (0/25). Conclusions All devices achieved excellent occlusion rates with low complication rates, regardless of PDA type. Device delivery via arterial access was associated with a shorter fluoroscopy time as compared to venous delivery, with no significant difference in outcomes. ADO II AS may be preferable to ADO II in the younger infant. © 2013 Wiley Periodicals, Inc. Source

Priest J.R.,International Pleuropulmonary Blastoma Registry | Williams G.M.,Childrens Hospitals and Clinics of Minnesota | Mize W.A.,Childrens Hospitals and Clinics of Minnesota | Dehner L.P.,University of Washington | McDermott M.B.,Our Ladys Hospital for Sick Children
International Journal of Pediatric Otorhinolaryngology | Year: 2010

Objectives: Nasal chondromesenchymal hamartoma (NCMH) is an uncommon chondro-stromal tumor of the nasal cavity and paranasal sinuses in infancy and childhood. Pleuropulmonary blastoma (PPB) is also a rare malignancy of lung and pleura in childhood and is the sentinel disease of an important familial tumor and dysplasia syndrome. This study identified NCMH in PPB patients. Methods: The International PPB Registry collects cases of PPB using central pathology review and evaluation of clinical records. The Registry also evaluates PPB literature. Examples of NCMH occurring with PPB were identified. Clinical records, digital radiography and pathologic specimens of PPB-associated NCMH cases were analyzed. Results: Among approximately 625 cases of PPB, four children developed NCMH. These cases are among 28 total reported NCMH cases. NCMH presented with sinonasal congestion and visible polypoid nasal masses and were diagnosed from ages 7 to 15 years, similar to older reported NCMH cases. NCMH involved the nasal cavity, paranasal sinuses and upper nasopharynx, was bilateral in three children and locally recurrent in one. In two children, NCMH had the characteristic pathologic spectrum of immature nodules of cartilage surrounded by spindle cell stroma, whereas the other two NCMH displayed mature chondroid nodules and a less varied fibrous stroma. NCMH was not identified in family members with PPB. Conclusions: NCMH developing in four children with PPB indicates that NCMH is part of the heredofamilial disease complex associated with PPB. Otorhinolaryngologists and pediatric oncologists should be aware that these two rare conditions occur together and that affected patients may have a familial predisposition to childhood malignant and dysplastic disease. © 2010 Elsevier Ireland Ltd. Source

Young O.,Our Ladys Hospital for Sick Children | Cunningham C.,Our Ladys Hospital for Sick Children | Russell J.D.,Our Ladys Hospital for Sick Children
International Journal of Pediatric Otorhinolaryngology | Year: 2010

Objective: Laryngotracheal separation (LTS) is an effective and reliable definitive treatment for intractable aspiration. A major advantage of this treatment for intractable aspiration is its' potential reversibility. Should the underlying disorder improve, a reversal of the procedure may be attempted. This has been successfully achieved in the adult population. To our knowledge, no previous cases have been reported of successful reversal of LTS in children. Methods: A retrospective review from 2003 to 2010 identified four cases of intractable aspiration treated with LTS in our department. Two of these patients displayed objective evidence of sufficient recovery of their underlying aspiration to consider reversal. Patient selection for reversal was dependent upon successful oral intake for 9 months along with videofluoroscopic evidence of normal or minimally impaired swallow. Results: Two children who were successfully treated for intractable aspiration with LTS demonstrated objective evidence of recovery sufficient to attempt reversal. Both children underwent successful surgical reversal of LTS using a cricotracheal resection with end-to-end anastamosis, similar to that used in treatment of subglottic stenosis. Both children can now tolerate oral diet and their speech and language development is in line with their overall developmental level. Conclusions: Laryngotracheal separation is an effective and reliable definitive treatment for intractable aspiration facilitating protection of the airway and allowing safe swallowing with unimpeded respiration, but with the major drawback of loss of phonation. To our knowledge, we document the first two cases of successful LTS reversal in children. © 2010 Elsevier Ireland Ltd. Source

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