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The 8p23.1 duplication syndrome (8p23.1 DS) is a recurrent genomic condition with an estimated prevalence of 1 in 58,000. The core 3.68 Mb duplication contains 32 genes of which five are currently candidates for the phenotypic features. Here we describe four patients and five families with eight microduplications of 8p23.1 ranging from 187 to 1082 kb in size and one atypical duplication of 4 Mb. These indicate that a minimal region of overlap (MRO) in medial 8p23.1 can give rise to features of 8p23.1 DS including developmental delay, dysmorphism, macrocephaly and otitis media, but not congenital heart disease (CHD). This MRO spans 776 kb (chr8:10,167,881-10,943,836 hg19) and contains SOX7 and seven of the other 32 core 8p23.1 DS genes. In centromeric 8p23.1, microduplications including GATA4 can give rise to non-syndromic CHD but the clinical significance of two smaller centromeric microduplications without GATA4 was uncertain due to severe neurological profiles not usually found in 8p23.1 DS. The clinical significance of three further 8p23.1 microduplications was uncertain due to additional genetic factors without which the probands might not have come to medical attention. Variable expressivity was indicated by the almost entirely unaffected parents in all five families and the mildly affected sibling in one. Intronic interruptions of six genes by microduplication breakpoint intervals had no apparent additional clinical consequences. Our results suggest that 8p23.1 DS is an oligogenetic condition largely caused by the duplication and interactions of the SOX7 and GATA4 transcription factors., (© 2015 Wiley Periodicals, Inc.)

Patients with congenital heart disease and pulmonary valve disease need multiple procedures over their lifetimes to replace their pulmonary valves. Chronic pulmonary stenosis, regurgitation, or both have untoward effects on ventricular function and on the clinical status of these patients. To date, all right ventricle-pulmonary artery conduits have had relatively short lifespans. Percutaneous pulmonary valve implantation, although relatively new, will probably reduce the number of operative procedures that these patients will have to undergo over a lifetime. Refinement and further development of this procedure holds promise for the extension of this technology to other patient populations.

Cardiac erosions may occur in a small percentage of patients after device closure of atrial septal defects. These devices have to be explanted. We report an aorta-to-left atrial fistula after surgical explantation of the device eight years after implant for access to mitral valve repair. The importance of realizing the risk of subclinical cardiac erosion and subsequent fistula development after device removal is discussed., (© The Author(s) 2014.)

Objectives: To describe the effectiveness of the Helex Septal Occluder (HSO) to close multiple atrial septal defects (mASDs). Background : Limited information is available describing closure of mASDs with the HSO., Methods: A total of 28 patients who underwent closure of mASDs with the HSO were identified by retrospective review of our catheterization database between 2001 and 2012. Procedural details and follow up information were collected., Results: Median age was 19.2 years, median weight 48 kg, with 10 (36%) patients weighing <25 kg. Indication for closure was RV enlargement (RVE) in all patients and additionally neurologic events occurred in 3/28 (11%). Median stop-flow diameter for the largest ASD was 14 (4-23) mm. One HSO was implanted in 21/28 (75%), 2 in 6/28 (21%), and 3 in 1/28 (4%). One embolization and one transient arrhythmia occurred with no sequelae. Immediate residual shunt was absent in 5/28 (18%), trivial in 15/28 (54%), small in 6/28 (21%), and moderate in 2/28 (7%). Of the 25 patients with ≥6 months follow-up (median 53 months), residual shunt was absent in 13/25 (52%), trivial in 5/25 (20%), and small in 7/25 (28%). RVE resolved in all but one patient with no other associated lesions and ≥6 months of follow-up. No patient with prior neurological event had recurrence at last follow-up., Conclusions: We conclude that closure of mASDs with ≥1 HSO is effective with a low complication rate. The ability of HSO devices to overlap or sandwich each other may facilitate safe implantation of multiple devices in smaller patients., (© 2013 Wiley Periodicals, Inc.)

Patients with tetralogy of Fallot (TOF) after complete repair and pulmonic stenosis (PS) after surgical valvotomy often develop significant pulmonic regurgitation (PR) that eventually requires valve replacement. Although criteria exist for the timing of pulmonary valve replacement in TOF, it remains less clear when to intervene in valvotomy patients and whether TOF recommendations can be applied. Our aim was to compare the structural and functional sequelae of valvotomy for PS with complete repair for TOF. We compared the clinical characteristics, electrocardiograms, echocardiograms, cardiac magnetic resonance imaging (MRI), and invasive hemodynamics of 109 adults (34 PS and 75 TOF) newly referred to a congenital heart disease center for evaluation of PR between 2005 and 2012. Both cohorts were similar in terms of baseline demographics and presenting New York Heart Association function class. Valvotomy patients had a slightly greater degree of PR by echocardiogram, although it was similar by cardiac MRI. Electrocardiography QRS width was greater in patients with TOF (114±27 vs 150±28 ms, p<0.001). MRI right ventricular ejection fraction (49±8 vs 41±11%, p=0.001) and left ventricular ejection fraction (59±7 vs 52±10%, p=0.002) were lower in patients with TOF. Pacemaker or defibrillator implantation was significantly greater in patients with TOF (3% vs 23%, p=0.011). In conclusion, patients postvalvotomy and complete repair present with similar degrees of PR and severity of symptoms. Biventricular systolic function and electrocardiography QRS width appear less affected, suggesting morphologic changes in TOF and its repair that extend beyond the effects of PR. These findings suggest the need for developing disease-specific guidelines for patients with PR postvalvotomy., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Objectives: To identify complications and measures that can be undertaken to prevent complications from transhepatic central venous access., Background: Utility and safety data from a large experience of patients undergoing transhepatic venous access are lacking., Methods: The records of patients who underwent transhepatic venous access between June 2000 and October 2012 at The Center for Pediatric and Congenital Heart Disease at The Cleveland Clinic were reviewed., Results: One hundred twenty-four transhepatic procedures were performed in 81 patients. The median weight of the patients was 6.3 (2.2-94) kg at a median age of 7 months (2 weeks to 35 years). Diagnostic catheterizations were performed in 75, interventions in 39, and central venous line placement in 91 procedures. The median maximum sheath size inserted was 8 (4-14) French. There were 10 (8%) major adverse events in 8 procedures-bleeding related (n = 6 with 1 death as a result) or complete heart block with instability or requiring intervention (n = 4). Heart block persisted after the catheterization in three patients (2 days, 2 weeks, 1 month). Minor adverse events (transient heart block) occurred in 5 (4 %) procedures. Weight, age, and French size of sheath (P = 0.46, 0.84, and 0.18) were not associated with complications. In addition, time to gain access was not different between non-complicated and complicated cases (median 19.5 versus 15.1 min, P = 0.72). Diagnostic and interventional procedures were not associated with more complications compared with procedures involving central venous line placement alone (P = 0.5)., Conclusions: Transhepatic access is an extremely useful modality of access to the heart and vascular structures in some patients with complex heart disease. Prevention of complications should focus on minimizing bleeding risks and heart block., (© 2013 Wiley Periodicals, Inc.)

Objectives: Review mid-term follow-up of percutaneous intervention for post ablation total pulmonary vein occlusion (PVO)., Background: Feasibility of percutaneous intervention for PVO has been described, but information remains limited., Methods: Patients with total PVO were retrospectively identified from our catheterization database. Medical records, catheterization reports, and outpatient follow-up were reviewed., Results: Between April 2005 and February 2012, 16 patients were identified with a total of 18 PVOs. Symptoms included hemoptysis in 6/16 (46%), cough in 8/16 (50%), chest pain in 8/16 (50%), dyspnea in 13/16 (81%) with mean NYHA Class of 2.6 ± 0.6. Recanalization was accomplished in 14/18 (78%) veins: 11 treated with balloon dilation and 3 with stents. Median follow-up for 13/14 veins was 13 (0-39) months (one patient with one PVO is awaiting follow-up). Reocclusion occurred in 7/13 (54%) at mean follow-up of 3.6 ± 1.6 months (6/10 post-balloon dilation and 1/3 post-stenting). Despite reocclusion, the reference vessel diameter increased from 4.8 ± 2.4 to 8.5 ± 4.2 mm (P < 0.001) between the first and second catheterization. Re-recanalization and stent placement was accomplished in 5/6 (83%), with one reocclusion not attempted. At latest follow-up 9/13 (69%) recanalized vessels remained patent and percent flow to affected lung quadrant increased from 7.4 ± 3.4% pre-intervention to 14.3 ± 4.2% (P = 0.004). Mean NYHA Class improved to 1.4 ± 0.4 (P < 0.001)., Conclusions: Recanalization of total PVO can be accomplished with reasonable mid-term patency, improved symptoms, and lung perfusion. Reocclusion is common, but vessel growth is often observed allowing placement of a reasonably sized stent at a second intervention. Staged intervention is often necessary to maintain patency., (Copyright © 2013 Wiley Periodicals, Inc.)

Obstruction and thrombosis of major systemic veins can occur due to indwelling central venous catheters. If obstruction of the innominate vein or superior vena cava occurs, lymphatic drainage can be impaired due to an increase in pressure in the thoracic duct and lymphatics. We describe a case where superior vena cava syndrome, chylopericardium and chylothorax occurred in a 16-year-old girl due to an indwelling central venous catheter. This was successfully treated with removal of the line, anticoagulation and angioplasty of the innominate vein and superior vena cava., (© 2013 The Authors. Pediatrics International © 2013 Japan Pediatric Society.)

The purpose of the present study was to assess the results and technical aspects of attempted transcatheter recanalization of occluded pulmonary arteries or major systemic veins from our center. Occluded pulmonary arteries or major systemic veins are often not considered amenable to transcatheter treatment and can be a cause of significant hemodynamic compromise for patients. The records of patients with occluded pulmonary arteries or major systemic veins who underwent cardiac catheterization from April 1997 to February 2011 were reviewed. We identified 18 patients, of whom, 9 had occluded pulmonary arteries and 9 occluded systemic veins. Recanalization was achieved in 17 of the 18 patients (94%). At a median follow-up of 35 months (range 6 months to 12 years), all vessels remained patent. The freedom from reintervention rate in the entire cohort was 61% at 6 months, 35% at 1 year, and 35% at 5 years. Freedom from an unplanned reintervention was 67% at 6 months. Procedural adverse events occurred in 2 patients. One death occurred within 24 hours of the catheterization procedure and 2 late deaths occurred, all unrelated to the procedure. In conclusion, both acute and chronic success at recanalizing the occluded pulmonary arteries and major systemic veins can be achieved using percutaneous techniques. Careful follow-up is needed, because reintervention can be necessary to maintain long-term vessel patency without stenosis., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Background: The role of percutaneous closure of patent foramen ovale (PFO) in patients with cryptogenic stroke or transient ischemic attack remains controversial. Registry data have suggested considerable benefit of closure over medical therapy, but the prospective, randomized CLOSURE I trial found no benefit for device closure., Methods: We compared patients enrolled into CLOSURE I to off-label closures performed during the study recruitment period at a single large institution and prospectively enrolled into an institutional registry of PFO closure. We also compared CLOSURE I patients at our institution to the reported characteristics of the entire study to ensure generalizability., Results: Between 11/3/2003 and 4/16/2007, there were 100 off-label closures and 33 patients randomized into CLOSURE I. Compared with off-label closure, patients in CLOSURE I were younger (41.6 ± 10.1 years vs 50.0 ± 14.0 years; P<.001) and had fewer cardiovascular risks including hypertension (12% vs 36%; P=.009), hyperlipidemia (24% vs 53%; P=.008), and coronary disease (3% vs 44%; P<.001). Degree of right-to-left shunting was considerably higher in off-label closures (28%, 14%, and 58% vs 45%, 30%, and 25% for mild, moderate, and severe, respectively; P=.026)., Conclusion: Off-label closures outnumbered patient recruitment into CLOSURE 3:1 at our institution during study recruitment. Certain demographic differences were expected (age over 60 was an exclusion for CLOSURE I), but vascular risks were considerably greater in the off-label group and may be important mechanistically. Large shunts were considerably more common in off-label patients, suggesting that higher-risk patients may have been preferentially closed off-label. These results suggest that the results of CLOSURE I may not apply to all patients with initial cryptogenic stroke.

Percutaneous pulmonary valve technology has had a great impact on patients with congenital and acquired heart disease. In some patients, implantation of a percutaneous pulmonary valve may not be possible due to the morphology of the existing right ventricular outflow tract. In this report, we describe implantation of a Melody transcatheter pulmonary valve in the left pulmonary artery in a patient with acquired right pulmonary artery occlusion and a large right ventricular outflow tract.

After the Norwood procedure for palliation of hypoplastic left heart syndrome, there is still significant interstage and late mortality with often unclear etiology. An important, but possibly under-recognized complication of the Norwood operation is the potential for coronary insufficiency from pre-coronary stenosis due to kinking or scarring at the anastomosis between the native and neo-aorta. We report a case of a clinically thriving 10-month old infant status post bidirectional Glenn who had acute ischemic changes on electrocardiogram (ECG) during induction of anesthesia for elective bilateral herniorrhaphy. A discrete narrowing in the native aorta to neo-aorta anastomosis was identified. A stent was placed emergently to restore adequate coronary blood flow after failure of simple angioplasty to adequately improve the stenosis., (Copyright © 2011 Wiley Periodicals, Inc.)

Background: Pediatric physicians regularly face the problem of uncertain procedural anticoagulation in children, especially in neonates. We sought to evaluate the safety, plasma concentration (pharmacokinetics, PK), pharmacodynamics (PD), and dosing guidelines of bivalirudin when used as a procedural anticoagulant in pediatric percutaneous intravascular procedures., Methods and Results: Pediatric subjects undergoing percutaneous intravascular procedures for congenital heart disease were enrolled and received the current weight-based dose used in percutaneous coronary interventions (0.75 mg/kg bolus, 1.75 mg/kg/hr infusion). Blood samples for PK/PD analyses were drawn, and safety was evaluated by monitoring bleeding and thrombosis events. A total of 110 patients (11 neonates, 33 infants, 32 young children, and 34 older children) were enrolled; 106 patients received the protocol dose. The PK/PD response of bivalirudin was predictable and behaved in a manner similar to that in adults. Weight-normalized bivalirudin clearance rates were more rapid in neonates and decreased with increasing age. Bivalirudin concentrations were slightly lower in neonates, with a trend to an increase with age. Activating clotting time response was consistent with adult studies and prolonged in all age groups, and there was reasonable correlation between activating clotting time and bivalirudin plasma concentrations across all age groups. There were few major bleeding (2 of 110, 1.8%) or thrombotic events (9 of 110, 8.2%) reported., Conclusions: PK/PD response of bivalirudin in the pediatric population is predictable and behaves in a manner similar to that in adults. Using adult dosing, bivalirudin safely provided the expected anticoagulant effect in the pediatric population undergoing intravascular procedures for congenital heart disease., (Copyright © 2010 Wiley-Liss, Inc.)

Occlusions of major vessels in patients with congenital heart disease may occur due to a variety of factors. These occlusions are often felt to be best addressed surgically; however, we and others have been successful in recanalizing most of these vessels in the catheterization laboratory. Most of these patients will require multiple procedures in the catheterization laboratory to ensure vessel patency and to facilitate vessel growth. Physicians performing the procedure should have a thorough understanding of the anatomic considerations for the intended procedure and have access to a variety of devices and equipment to optimize the result of the procedure. In this article, we review some of the technical aspects that are vital for the success of the procedure.

Transcatheter closure of secundum atrial septal defect (ASD) and patent foramen ovale (PFO) is now widely accepted as an alternative to surgical closure. With currently available devices and techniques, approximately 80-90% of secundum ASDs and all PFOs can be closed percutaneously. While many devices are available, the use of any particular device is dictated largely by individual defect anatomy, device availability, long-term considerations, approval status (US Food and Drug Administration approval versus CE mark), and physician preference.

One or more breaks in the wire frame of the HELEX Septal Occluder (W.L. Gore and Associates, Flaggstaff, Arizona) were identified in 5% of patients in the multicenter pivotal trial leading to FDA approval. Fractures of the perimeter frame have not previously been associated with any apparent deleterious clinical effects. We report a case in which the broken ends of wire at a frame fracture were located in a position that resulted in perforation of the anterior mitral valve leaflet. There was significant mitral regurgitation and the device was removed surgically. The possible etiologies and prevention of wire frame fractures are also discussed., (Copyright 2009 Wiley-Liss, Inc.)

The objective of this study was to evaluate the incidence of pre-existing catheterization left pulmonary artery (LPA) gradients and correlation of these gradients with later LPA stenosis after successful patent ductus arteriosus (PDA) occlusion. We performed a single-center review of 130 patients with PDA closure from October 1993 to February 2005. We analyzed the pre-PDA closure LPA pressure gradients at catheterization to determine if these were predictive of late LPA stenosis. On follow-up, a V (max) >2 m/s by echocardiogram (transthoracic echocardiography; TTE) was considered indicative of possible LPA stenosis. Left lung perfusion of <35% was considered diagnostic of significant LPA stenosis. Post PDA closure, possible LPA stenosis by TTE was seen in 8 of 128 patients (6.25%). Seven of these eight had precatheter LPA gradients >7 mm Hg. Five of these had perfusion scans, three of the five had significant LPA stenosis, and two underwent LPA angioplasty. Patients with LPA catheter gradients >7 mm Hg were more likely to have possible LPA stenosis by TTE, significant LPA stenosis by lung scan, and intervention with LPA angioplasty. In conclusion, a preclosure main pulmonary artery-to-LPA pressure gradient >7 mm Hg was found in all patients who developed significant LPA stenosis on follow-up after transcatheter PDA closure. It appears likely that these patients have LPA abnormality rather than stenosis caused by the PDA occlusion device. Patients with preclosure LPA gradients >7 mm Hg should undergo follow-up evaluations for detection of significant stenosis and may require treatment if an important flow abnormality is documented.

Background: We analyzed our data on patients undergoing transcatheter patent foramen ovale (PFO) closure to determine if the maximal potential PFO diameter (MPPD) by balloon sizing correlates with important clinical characteristics in this population., Methods: We defined stroke as a focal neurologic deficit lasting >24 h, or focal deficit of shorter duration associated with permanent MRI/CT changes consistent with a focal infarction. Parameters analyzed included age, gender, anticoagulation, hypertension, smoking, MRI/CT findings and MPPD at catheterization. We specifically analyzed the type of neurologic event (stroke/transient ischemic attack, TIA), and number of recorded preceding clinical neurologic events., Results: In 216 consecutive patients, 167 suffered a stroke. MRI/CT changes consistent with one or more embolic events were seen in 156 patients; 49 had a clinical TIA. There was no significant difference in MPPD between stroke (11.0 +/- 3.6 mm) and TIA groups (10.9 +/- 3.9 mm; 95% confidence interval for difference: -1.33 to 1.00). MPPD did not differ between MRI/CT-positive vs. -negative strokes, and had no correlation with the number of identified pre-closure clinical neurologic events., Conclusions: Continued investigation is needed to determine whether other PFO characteristics, or other anatomic/physiologic parameters, may be useful to identify patients at high risk for cryptogenic stroke/TIA, even before they have their first neurologic event., (Copyright 2008 S. Karger AG, Basel.)

Background: Stenting for aortic coarctation has been shown to be effective in the short term. The safety and longer term efficacy of transcatheter therapy, however, must be well established if the technique is to be widely accepted as an alternative to surgery. In order to determine the frequency, spectrum, and outcome of injury to the aortic wall caused by angioplasty or stenting of aortic coarctation, the nomenclature of mural injury in these patients must be adapted to the conditions of transcatheter therapy., Methods and Results: Between 1989 and July 2005, we inserted stents in 153 patients with aortic coarctation, their median age being 15.8 years. Prior aortic interventions had been performed in 98 patients, and preexisting aneurysms were observed in 19. Stenting resulted in a significant reduction of the gradient across the site of coarctation, from a median of 30 millimetres of mercury to zero (p less than 0.001), with a residual gradient within the aortic arch of 20 millimetres of mercury or more in 5% of patients. Acute injuries to the aortic wall, other than therapeutic tears, were observed in 3 patients (2%), none of whom required surgery. At median follow-up of 2.5 years, this being more than 5 years in 30 patients, 4 patients had died, albeit none from complications relating to stenting or catheterization. Acute injuries to the aortic wall did not progress, and new aneurysms were observed in 6% of patients subsequent to follow-up imaging. Stent fractures, and jailed or partially covered brachiocephalic vessels, were observed in 12, and 49, patients, respectively, but did not result in haemodynamic or embolic complications., Conclusions: Stenting for aortic coarctation results in consistent relief of the gradient, and few serious complications in the short and intermediate term. Serious injuries to the aortic wall are uncommon in our experience, and can be minimized with a focus on technical measures, such as pre-dilation before stenting.

Objective: Critically ill children with cardiac disease often require prolonged central venous access. Thrombosis of systemic veins or the need to preserve vessels for future cardiac procedures limits sites for placement of central venous catheters in these patients. This study evaluates the use of Broviac placement via the transhepatic approach for this patient population., Design: A retrospective review., Setting: A tertiary care center., Patients: All children with complex congenital heart disease who underwent transhepatic Broviac placement between May 2000 and April 2004., Interventions: Transhepatic Broviac placement., Measurements and Main Results: Thirty-two children with a median age of 5 months (20 days-5.3 yrs) and a median weight of 4.2 kg (2.2-24.9 kg) underwent 40 transhepatic Broviac placements. There were three (8.8%) procedural-related complications. One patient suffered an intra-abdominal bleed requiring an urgent laparotomy and removal of the Broviac, one patient required transfusion because of a mild self-contained intra-abdominal bleed, and one patient developed temporary complete heart block. There was one catheter infection. Thrombus was noted by echocardiography on the tip of two Broviacs; however, no intracardiac vegetations or embolic events occurred. There was no mortality related to the procedure. Broviacs remained in place for a median of 36 days (1 day-6 months). Five Broviacs were dislodged inadvertently (two during cardiac massage and three resulting from patient manipulation). The remaining Broviacs were electively removed safely without coil embolization. At a median follow-up of 3.5 months (10 days-3 yrs), there have been no long-term complications related to the Broviacs., Conclusions: Transhepatic Broviac catheters can be used safely in critically ill children with cardiac disease and remain indwelling for adequate periods of time. This modality of prolonged vascular access should be considered for children whose veins are occluded or need to be preserved for future procedures.

Background: Due to shortcomings of any valve replacement, repairing and retaining the native valve may be beneficial for congenital aortic valve disease., Methods: Retrospective review of data and follow-up of aortic valve repair from a single institution., Results: From 1993 to 2001, 56 patients underwent aortic valve repair [median age 13.4 years (range 1 day to 45 years)]. The predominant aortic valve lesion was mixed aortic stenosis/aortic insufficiency 25 (45%), aortic insufficiency 24 (43%) and aortic stenosis 7 (13%). Repair techniques included sub-commissural plication 36 (64%), commissurotomy 24 (43%), cusp plication 15 (27%), pericardial patch cusp extension 8 (14%) and resuspension of commissures 4 (7%). Most patients (88%) required a combination of techniques; 61% required additional procedures. Hospital survival was 55/56 [98%; (95% CI 91-100%)] no patient was discharged on anticoagulation for aortic valve pathology. Fifty-three patients [95%; (95% CI 85-98%)] remain alive after a median follow-up of 37 months; four survivors required aortic valve replacement and two required repeat aortic valve repair [84%; (95% CI 72-91%) reintervention-free survival]., Conclusions: (1) In this study, aortic valve repair for congenital abnormalities avoided reoperation in the majority of patients, avoided anticoagulation and retained growth potential of the valve. (2) Repeat aortic valve repair or replacement was used to treat subsequent valve deterioration.

Although transesophageal echocardiography is often used for guidance during transcatheter interventions, few data exist regarding the use of the newer modality of intracardiac echocardiography. This brief report summarizes our single center experience using intracardiac echocardiographic guidance during transcatheter interventional procedures for congenital heart disease.

Background: Pulmonary vein stenosis has recently been recognized as a complication of radiofrequency ablation for atrial fibrillation. This study evaluates the presentation of affected patients and the role of transcatheter therapy for this patient population., Methods and Results: This study used a retrospective review of data from 19 patients (age, 51+/-13 years) with pulmonary vein stenosis who underwent catheterization and angiography between December 2000 and December 2002. Quantitative perfusion and spiral CT scans were performed for initial diagnosis and follow-up. The median duration between radiofrequency ablation and the reported onset of respiratory symptoms for 18 of 19 patients was 7.5 weeks (0.1 to 48). After the onset of symptoms, all but two patients were initially misdiagnosed with a symptoms-to-diagnosis duration of 16 weeks (2-59). At initial catheterization, 17 of 19 patients had angioplasty in 30 veins with stent placement in 5 vessels when a flap occurred. Overall vessel diameter increased from 2.6+/-1.6 to 6.6+/-2.4 mm (P<0.0001). There were 4 procedure-related adverse events but no long-term sequelae. Immediate follow-up showed improved flow to involved lung segments. At a median follow-up of 43 weeks (2-92), although repeat angioplasty for restenosis was necessary in 8 of 17 patients, 15 of 17 patients currently have no or minimal persistent symptoms., Conclusions: Pulmonary vein stenosis after radiofrequency ablation for atrial fibrillation is often misdiagnosed. Although further follow-up is necessary to determine long-term success, our data indicate better pulmonary vein flow and symptomatic improvement in the majority of patients undergoing dilation of postablation pulmonary vein stenosis.