[Primary Pulmonary Vein Stenosis Developed after Bidirectional Glenn Procedure;Report of Two Cases].

We experienced 2 cases of primary pulmonary vein stenosis(PVS),which developed after a bidirectional Glenn procedure was performed for complex heart defects with normal pulmonary venous return. Although the patients successfully underwent primary sutureless repair for left PVS, restenosis of the affected pulmonary veins occurred several months after surgery in both patients. Stent implantation followed by balloon angioplasty was performed for stent stenosis in 1 patient without effect. However, the patient later underwent a successful fenestrated Fontan procedure. Catheter intervention was contraindicated in the 2nd patient due to almost complete obstruction of the left pulmonary veins with upstream hypoplasia. To improve the results of PVS treatment, earlier diagnosis by quantitative lung perfusion scintigraphy, magnetic resonance imaging, and close echocardiographic observation together with earlier, aggressive treatment combining surgery and catheter interventions are recommended.

Congenital-idiopathic superficial femoral artery aneurysm in a 7-year-old child.

Superficial femoral artery aneurysm in children is distinctly uncommon, and usually results from infection, vasculitis, connective tissue disorder, or trauma. We report a 7-year-old girl who had multiple fusiform aneurysms of the right superficial femoral artery, with no evidence of related disorders. The patient successfully underwent aneurysm resection and femoral artery reconstruction with autogenous saphenous vein. Histologic examination revealed intimal thickening with fibroplasia without severe inflammatory infiltrates or cystic medial necrosis, suggesting a congenital-idiopathic arterial aneurysm. Three years after the procedure, the saphenous vein graft is fully patent and the patient is in good condition.

Fatal aortotracheal fistula combined with aortoesophageal fistula in an infant with double aortic arch: a warning.

This report describes a 2-month-old male infant with a double aortic arch (DAA) complicated by aortoesophageal fistula (AEF) and aortotracheal fistula (ATF). He was intubated with an endotracheal tube at birth because of neonatal asphyxia. A nasogastric tube was also placed for gastric decompression and milk feeding. On the 74th day of birth, he had massive upper gastrointestinal hemorrhage associated with shock, and was referred to our hospital. Although emergent surgery controlled the bleeding from AEF, he suffered cardiac arrest due to massive bleeding from ATF 5 h after surgery, and died on the 9th postoperative day. Physicians should be aware that prolonged endotracheal and nasogastric intubation predispose to the development of not only esophageal erosion but also more lethal tracheal erosion. In addition to the importance of early diagnosis and prompt surgery for DAA, appropriate preoperative respiratory management is emphasized to prevent similar occurrences in the future.

Successful surgical removal of a giant interventricular fibroma: surgical approach without ventriculotomy.

A 14-month-old boy was transported to our hospital by ambulance because of cardiopulmonary arrest after the sudden onset of convulsions. He was resuscitated and transthoracic echocardiography showed a giant interventricular tumor. The cause of this episode was thought to be ventricular arrhythmias induced by the tumor. At operation, an incision line was confirmed by direct ultrasonography. The heart was incised directly on the interventricular septum. The tumor was carefully dissected and completely removed without entering the ventricular cavity. Histologic analysis revealed a fibroma. The patient's postoperative course was uneventful, and he remains well without episodes of heart failure or ventricular arrhythmia.

Surgical management of congenital tracheal stenosis.

Congenital tracheal stenosis (CTS) is a rare but life-threatening disorder, particularly in neonates and infants. The stenotic lesions are commonly composed of complete rings of cartilage varying in length, location, and severity. A definitive diagnosis of CTS may be delayed because of the rarity of this disorder and thus its unfamiliarity among physicians, its variable onset time, its various clinical symptoms, and the diversity of associated clinical conditions due to the cardiovascular disorders that may accompany it. More than half of the patients who manifest clinical symptoms during early infancy show a long-segment stenosis. Long-segment CTS is problematic and challenging to manage. Over the last two decades several surgical techniques for long-segment CTS have been devised and developed, but no definitively advantageous surgical procedure has been established because of insufficient experience and the lack of large-scale studies. Although rib cartilage tracheoplasty and pericardial patch tracheoplasty have provided limited early to midterm success for infants with long-segment CTS, these procedures are associated with early and late complications, including granulation tissue formation, patch collapse, and restenosis necessitating reintervention. By contrast, slide tracheoplasty has given rise to better surgical outcomes. This procedure enables reconstruction of the stenotic trachea using native tracheal walls with preserved blood supply. The trachea is foreshortened by only one-half of the original stenosis, resulting in reduced tension on the anastomosis. Consequently, the technique has several advantages, including less formation of granulation tissue, satisfactory subsequent growth, and infrequent reoperation for restenosis. Slide tracheoplasty is currently recognized as the preferred technique for long-segment CTS.

Establishing right ventricle-pulmonary artery continuity by autologous tissue: an alternative approach for prosthetic conduit repair.

BACKGROUND: In conventional conduit operations, longevity has been essentially limited by the inevitable need for conduit replacement. This study was undertaken to compare long-term results of the use of equine pericardial conduits, autologous pericardial conduits, and direct anastomosis repair. METHODS: Between 1982 and 2001, 366 patients underwent primary establishment of right ventricle-pulmonary artery continuity at our institution. The mean age at the time of operation was 6.2 years (range, 4 days to 28 years) and mean weight was 17.2 kg (range, 1.6 to 61 kg). Three different repair techniques were used for connection: hand-made valved equine pericardial conduits (n = 179), autologous pericardial conduits (n = 71), and direct anastomosis without a conduit (n = 116). Mean follow-up period for early survivors was 8.6 years in the equine group, 6.1 years in the direct anastomosis group, and 5.1 years in the autologous pericardium group. RESULTS: Direct anastomosis repair (p = 0.0002) was associated with significantly better freedom from late events (conduit replacement or late death) than equine pericardial conduits. The hazard ratio was less with the autologous pericardium conduit than with the equine pericardium, but the difference was not statistically significant (p = 0.2122). Younger age at operation, and postoperative pressure ratio from right to left ventricle were also predictors of conduit longevity. CONCLUSIONS: To decrease the probability of late events, direct anastomosis is an encouraging technique compared with traditional equine pericardium extracardiac conduit repair. An autologous pericardial conduit, because of its benefits, would be an alternative when direct anastomosis is not suitable.