Cor Triatriatum Dexter (CTD) is a rare congenital anomaly involving the systemic venous valves. Failure of regression of the right‐sided sinus venosus valve leads to abnormal septation of the right atrium and a variety of right atrial and tricuspid valve obstructive lesions. The presentation can be varied ranging from asymptomatic to persistent neonatal cyanosis. We describe a late diagnosis of CTD in a 10‐month‐old male with persistent hypoxia despite balloon valvuloplasty for mild pulmonic valve stenosis with a large secundum atrial septal defect and a mildly hypoplastic right ventricle.
Congenital intrathoracic ectopic kidney is a rare congenital abnormality that is usually found as an incidental lesion on chest radiographs. We report the case of a 6-month-old male with a 1-day history of breathing difficulties whose chest radiograph revealed a soft tissue right basilar mass. Further investigation utilizing ultrasound revealed a thoracic kidney. This case demonstrates the importance of sonography as a diagnostic tool in identifying thoracic kidneys in the pediatric age group.
To validate the feasibility and spatial accuracy of pre-procedural 3D images to 3D rotational fluoroscopy registration to guide interventional procedures in patients with congenital heart disease and acquired pulmonary vein stenosis. Cardiac interventions in patients with congenital and structural heart disease require complex catheter manipulation. Current technology allows registration of the anatomy obtained from 3D CT and/or MRI to be overlaid onto fluoroscopy. Thirty patients scheduled for interventional procedures from 12/2012 to 8/2015 were prospectively recruited. A C-arm CT using a biplane C-arm system (Artis zee, VC14H, Siemens Healthcare) was acquired to enable 3D3D registration with pre-procedural images. Following successful image fusion, the anatomic landmarks marked in pre-procedural images were overlaid on live fluoroscopy. The accuracy of image registration was determined by measuring the distance between overlay markers and a reference point in the image. The clinical utility of the registration was evaluated as either "High", "Medium" or "None". Seventeen patients with congenital heart disease and 13 with acquired pulmonary vein stenosis were enrolled. Accuracy and benefit of registration were not evaluated in two patients due to suboptimal images. The distance between the marker and the actual anatomical location was 0-2 mm in 18 (64%), 2-4 mm in 3 (11%) and >4 mm in 7 (25%) patients. 3D3D registration was highly beneficial in 18 (64%), intermediate in 3 (11%), and not beneficial in 7 (25%) patients. 3D3D registration can facilitate complex congenital and structural interventions. It may reduce procedure time, radiation and contrast dose.
Objectives
Report long‐term outcomes of percutaneous intervention in patients with pulmonary vein stenosis (PVS) after pulmonary vein isolation (PVI) from a single center over 16 years.
Background
Outcome reports of percutaneous intervention for PVS resulting from PVI are limited.
Methods
Retrospective review of all patients with PVS after PVI who underwent percutaneous intervention at the Cleveland Clinic Foundation between January 2000 and December 2016.
Results
A total of 205 patients underwent cardiac catheterization for PVS during the study period. Completely occluded veins which could not be recanalized occurred in six patients. Of the remaining 199 patients, 27 (14%) were lost to follow‐up, leaving 172 patients with 276 veins for analysis. Balloon angioplasty was performed in 62 veins and stent implantation in 250 (primary in 214, to treat postdilation restenosis in 36). Re‐intervention occurred in 45/62 (73%) balloon‐dilated veins and 45/250 (18%) stented veins. Freedom from re‐intervention at 1 and 5 years was 90 and 73% following stenting versus 40 and 23% following balloon dilation (p < .001, Hazard ratio (HR) = 5.7). Veins with stent diameter ≥7 mm (n = 231) had greater freedom from re‐intervention (95% at 1 year, 79% at 5 years) than veins with stents <7 mm (43% at 1 year, 9% at 5 years), p < .001. There was clear symptomatic improvement after intervention and no procedural mortality.
Conclusions
Stent implantation at ≥7 mm for PVS after PVI is associated with low rates of re‐intervention, in contrast to balloon dilation and stenting with small conventional stents.
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