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.

For tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries, a staged surgical approach yields low overall mortality and acceptable hemodynamics after complete repair.

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Patients with complex congenital heart disease may have pulmonary artery stenoses that are either congenital or associated with scarring following surgical procedures. This study evaluates cutting balloon angioplasty for small-vessel pulmonary artery stenoses resistant to standard balloon angioplasty. Between October 1998 and December 1999, patients were enrolled in an FDA-approved compassionate-use protocol. During four catheterizations, there were seven lesions found resistant to standard balloon angioplasty (mean lesion diameter was unchanged: 1.8 mm +/- 0.8 mm to 1.9 +/- 0.8 mm). A cutting balloon was inflated twice in each of these lesions. Standard balloon angioplasty was then repeated. Final mean lesion diameter was increased significantly (1.9 mm +/- 0.8 mm to 3.8 +/- 1.3 mm; P

Key Clinical MessageThis case report describes a patient with a 22q11.2 duplication. His features, which include VACTERL association with an esophageal atresia/tracheo‐esophageal fistula and a vascular ring, expand the previously described phenotype for this duplication.

Objectives: Results of Fontan's procedure have improved considerably, but perioperative mortality still occurs, attributed to ventricular dysfunction, stroke, arrhythmia, thromboembolism, and multi-organ dysfunction. Our protocols of operative and intensive care unit management address these potential issues, and have been associated with zero mortality, even with many high-risk candidates. Methods: From 1996 to 2006, all Fontan patients were managed as follows: operative strategy based on aortic and single atrial cannulation, cooling on full-flow bypass, and hypothermic circulatory arrest to create the Fontan pathway. No direct caval cannulation. Use of central venous lines was completely avoided. Fresh whole blood was used for pump prime and for volume restoration. Inotropic and vasodilator therapy was continued for at least 48 h. Aspirin was used exclusively as anti-thrombotic therapy. Postoperative pleural drainage was accomplished with small pigtail catheters. The usual Fontan pathway was by lateral atrial tunnel (84), with extra-cardiac conduit when dictated by anatomy (16). Results: One hundred Fontan operations were performed with no mortality. All patients were extubated by postoperative day 1. Hospital stay was 10 AE 5 days. Complications were: bleeding (1), reintubation (1), emergent fenestration closure (1), pericardial effusion (4), and seizures (1). Risk factors included Fontan connection to one lung (3), diminutive pulmonary arteries (PAs) and unifocalized major aortopulmonary collateral arteries (MAPCAs) (1), discontinuous PAs (3), right ventricle dependent coronaries (3), neonatal pulmonary venous obstruction (3), Trisomy 21 (1), preoperative pacemaker dependence (2), and heterotaxy (10). No candidate was excluded. Conclusions: While many surgeons try to avoid bypass or aortic clamping when performing Fontan operations, the strategies we have employed facilitate safe accomplishment of Fontan's operation in diverse anatomic groups with multiple risk factors, with avoidance of operative mortality in 100 consecutive cases. #

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