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1/129. Post-Mustard procedure pulmonary venous obstruction: An opportunity for anatomic correction with a one-stage arterial switch.

    A 14-year-old boy after a Mustard procedure for transposition of the great arteries developed pulmonary hypertension secondary to baffle obstruction. This occurred over several years without apparent significant symptomatology. Systemic-level pressure prevailed in the left (pulmonary) ventricle and provided an opportunity to perform a successful one-stage arterial switch. ( info)

2/129. Total anomalous pulmonary venous connection to the portal and splenic vein associated with unilateral hypoplasia of pulmonary veins.

    Total anomalous pulmonary venous return (TAPVR) represents a rare congenital anomaly with wide anatomical and physiological variability. We report a case of a newborn with a challenging form of obstructed infracardiac TAPVR, in whom left and right pulmonary veins drained separately into the portal system. The right pulmonary venous sinus connected to the left branch of the portal vein, whereas the left venous sinus connected to the splenic vein. Surgical repair consisted of the creation of a common retrocardiac venous trunk which was anastomosed to the left atrium. The postoperative course was characterized by persisting congestion of the right lung. Two months later, right pulmonary vein hypoplasia was successfully enlarged with autologous pericardium. ( info)

3/129. The vascular vise causing TAPVR type I to radiographically mimic TAPVR type III.

    A subtype of supracardiac total anomalous pulmonary venous return (TAPVR) consists of the vertical vein passing between the left pulmonary artery and the left mainstem bronchus resulting in relative obstruction to pulmonary venous return. This has been termed the vascular vise. In this situation, the supracardiac type of TAPVR (Type I) may mimic radiographically the infradiaphragmatic type (Type 3). ( info)

4/129. pulmonary veno-occlusive disease in pulmonary Langerhans' cell granulomatosis.

    This report describes unusual clinical and pathological findings in a 29-yr-old female with pulmonary Langerhans' cell granulomatosis (LCG). During a 7-yr clinical course her condition deteriorated despite corticosteroid therapy, and she died of respiratory failure and pulmonary hypertension. At autopsy, there were widespread pulmonary veno-occlusive disease (PVOD) lesions as well as abundant advanced and healed lesions of pulmonary LCG composed of multiple cysts and stellate fibrosis. The present case demonstrates that pulmonary Langerhans' cell granulomatosis should be considered as a possible cause of pulmonary veno-occlusive disease. ( info)

5/129. Pulmonary vein stenosis complicating catheter ablation of focal atrial fibrillation.

    INTRODUCTION: A recently described focal origin of atrial fibrillation, mainly inside pulmonary veins, is creating new perspectives for radiofrequency catheter ablation. However, pulmonary venous stenosis may occur with uncertain clinical consequences. This report describes a veno-occlusive syndrome secondary to left pulmonary vein stenosis after radiofrequency catheter ablation. methods AND RESULTS: A 36-year-old man who experienced daily episodes of atrial fibrillation that was refractory to antiarrhythmic medication, including amiodarone, was enrolled in our focal atrial fibrillation radiofrequency catheter ablation protocol. The left superior pulmonary vein was the earliest site mapped, and radiofrequency ablation was performed. atrial fibrillation was interrupted and sinus rhythm restored after one radiofrequency pulse inside the left superior pulmonary vein. atrial fibrillation recurred and a new procedure was performed in an attempt to isolate (26 radiofrequency pulses around the ostium) the left superior pulmonary vein. Ten days later, the patient developed chest pain and hemoptysis related to severe left superior and inferior pulmonary veins stenosis. Balloon angioplasty of both veins was followed by complete relief of symptoms after 2 months of recurrent pulmonary symptoms. The patient has been asymptomatic for 12 months, without antiarrhythmic drugs. CONCLUSION: Multiple radiofrequency pulses applied inside the pulmonary veins ostia can induce severe pulmonary venous stenosis and veno-occlusive pulmonary syndrome. ( info)

6/129. Urgency of operation in infracardiac total anomalous pulmonary venous connection.

    BACKGROUND: Because the tendency for pulmonary venous obstruction in the infracardiac type of total anomalous pulmonary venous connection may be partially dependent on the connection of the descending vein to the portal vein, the inferior vena cava, or one of their tributary vessels, we reviewed our surgical experience with various subtypes of infracardiac total anomalous pulmonary venous connection. methods: The urgency of operation in 4 neonates with infracardiac total anomalous pulmonary venous connection was reviewed. RESULTS: Two patients with pulmonary venous obstruction in whom the descending vein connected to the portal vein were operated on immediately with successful outcome. One patient who had become critically ill after the ductus venosus had closed died before operation could be undertaken. One patient in whom the descending vein connected to the left hepatic vein was operated on electively with successful outcome. CONCLUSIONS: In hemodynamically stable patients with no clinical or echocardiographic signs of pulmonary venous obstruction, some form of differentiation with regard to urgency of operation may be appropriate. When the descending vein connects to the inferior vena cava or a hepatic vein, the operation may be performed on a semi-elective basis. In contrast, when the descending vein connects to the portal vein or the ductus venosus, operation should generally not be delayed because of the high likelihood of obstruction. ( info)

7/129. Graft failure caused by pulmonary venous obstruction diagnosed by intraoperative transesophageal echocardiography during lung transplantation.

    IMPLICATIONS: Intraoperative transesophageal echocardiography can be useful to diagnose pulmonary venous anastomotic stenoses during lung transplantation. ( info)

8/129. "High probability" perfusion lung scans in pulmonary venoocclusive disease.

    "High-probability" ventilation/perfusion (V/Q) lung scans generally indicate proximal pulmonary arterial occlusion by thromboemboli or, rarely, other processes such as tumors, fibrosing mediastinitis, or vasculitis. In this report we describe three patients with high probability V/Q scans in whom pulmonary angiography failed to demonstrate arterial occlusion. All three patients were determined to have pulmonary venoocclusive disease (PVOD). In two patients, a tissue diagnosis of PVOD was made, in one case with explanted tissue taken after a successful heart-lung transplant and in the other case with tissue taken at autopsy. PVOD in the third patient was diagnosed with pulmonary venography. A potential explanation for the discrepancy between perfusion lung scan and pulmonary angiographic findings in PVOD is discussed. ( info)

9/129. Case report: pulmonary vein stenosis following RF ablation of paroxysmal atrial fibrillation: successful treatment with balloon dilation.

    Paroxysmal atrial fibrillation and atrial tachycardia may originate from a focal source in one or multiple pulmonary veins. A focal origin facilitates a potential cure amendable to radiofrequency ablation. Herein we report the case of a 16 year old adolescent male with a tachycardia induced cardiomyopathy who presented with very frequent paroxysmal episodes of atrial fibrillation, atrial flutter and atrial tachycardia. The origin of the arrhythmia was mapped to the secondary branches of the left lower pulmonary vein using an octapolar micro-mapping catheter. Immediately following application of three radiofrequency lesions, angiography of the left lower pulmonary vein revealed a region of focal stenosis at the site of energy application, with delayed pulmonary venous emptying. Attempts to relieve any element of spasm using direct administration of nitroglycerin were unsuccessful. Three months later repeat catheterization revealed an unchanged region of tight anatomical stenosis. Balloon dilation of two stenotic areas resulted in dramatic relief of the obstruction and improved venous drainage. Recatheterization 6 months later revealed mild restenosis that was successfully redilated. Intracardiac ultrasound demonstrated focal constriction. Care should be exercised in attempting RF ablation in distal arborization sites of the pulmonary veins in children, because of the small caliber compared to adult subjects. Radiofrequency induced focal areas of stenosis may be amenable to balloon catheter dilation. ( info)

10/129. Congenital pulmonary vein stenosis associated with cor triatriatum.

    We report an 11-year-old boy who underwent surgical correction for stenosis of the right and left lower pulmonary veins at their junction with the left atrium and associated cor triatriatum. The embryology and therapeutic approaches to congenital pulmonary veins stenosis are discussed. ( info)
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