A Novel Aortic Reconstruction for Anomalous Left Coronary Arising From the Right Pulmonary Artery in Hypoplastic Left Heart Syndrome: Successful Surgical Treatment.

An anomalous left coronary arising from the pulmonary artery (ALCAPA) in hypoplastic left heart syndrome (HLHS) is a very rare congenital malformation. The Norwood procedure and simultaneous direct reimplantation of the left coronary artery (LCA) to the ascending aorta have been previously used for treatment of ALCAPA, although mortality was very high, because it is difficult to reimplant the LCA to the neoaortic root without distortion or tension. We chose the modified Norwood procedure without reimplantation of the LCA to the neoaortic root, and 4-month-old male infant survived the operation and waiting Fontan procedure.

Novel aortic arch reconstruction using a modified Norwood procedure based on hypoplastic left heart syndrome-specific anatomical malformations.

OBJECTIVES: Postoperative left pulmonary artery (PA) or bronchus compression occasionally follows narrowing of the retroaortic space after the Norwood procedure. We examined hypoplastic left heart syndrome (HLHS)-specific anatomical malformations and hypothesized that a long main pulmonary arterial trunk might contribute to a larger retroaortic space. In this study, we introduced a modified Norwood procedure [pulmonary artery trunk-saving method (PATS)] and evaluated the results. METHODS: HLHS-specific anatomical malformations were examined using computed tomographic data from 23 consecutive patients who underwent bilateral pulmonary banding. Fourteen patients had HLHS or associated conditions (Group H), and 9 patients had other complex cardiac defects and underwent biventricular repair (Group B). Five consecutive HLHS patients underwent PATS as a modified Norwood operation, and 6 controls underwent a conventional Norwood procedure. We used computed tomography to measure the lengths of the aorta and PA and the angle of both pulmonary branches to evaluate the effect of PATS. RESULTS: Preoperative examination confirmed HLHS-specific right PA branching adjacent to the pulmonary valve and a trend towards a shorter main pulmonary arterial trunk with the conventional Norwood procedure. Also, both right and left pulmonary arterial branching from the dorsal aspect of the main pulmonary arterial trunk and the PA branch angle were minimal in HLHS patients. Postoperative data showed the retroaortic space behind the reconstructed neoaorta was significantly wider in PATS patients than in conventional Norwood patients. Longitudinal measurements (between the aortic arch and pulmonary valve) and sagittal measurements (ascending aorta to descending aorta) were larger in PATS patients, and no left PA or airway obstruction was observed following PATS. CONCLUSIONS: PATS resulted in a wider aortopulmonary space without left pulmonary arterial stenosis or bronchus obstruction. HLHS-specific anatomical malformations suggested that PATS may avoid extrinsic compression of the left PA and bronchus by the neoaorta and can be an alternative for HLHS patients.

[Recurrent mediastinal desmoid tumor treated by surgical resection ; report of a case].

We report a rare case of mediastinal desmoid tumor in a 17-year-old man. The patient was a 17-year old man who had underwent surgery for mediastinal tumor at our hospital 2 years before. The tumor was diagnosed as a solitary fibrous tumor of the mediastinum. After 2 years, chest computed tomography(CT) and magnetic resonance imaging( MRI) showed a mass at the previously resected site. Surgery was performed again under the diagnosis of recurrent tumor. The tumor densely adhered to the superior vena cava, right innominate vein, pericardium, and the right lung. The tumor was completely resected with the aid of the partial extracorporeal circulation. Finally, both previously resected tumor and the recurrent tumor were diagnosed pathologically as desmoid tumors.