Recently, modified Fontan operation is being used for asplenia syndrome. We reviewed 24 patients with asplenia syndrome who underwent surgical intervention. Eleven (45.8%) of them had total anomalous pulmonary venous connection (TAPVC) and 6 of these were accompanied by pulmonary venous obstruction (PVO). Surgical results were poor in the PVO group. In 4 cases with open heart palliation, that is atrium-common pulmonary venous chamber anastomosis, there were 2 operative deaths, 1 late death, and 1 survival. In 18 cases without PVO, statistical analysis (Fisher's exact probability) showed that pulmonary atresia (PA) was a definite risk factor for Fontan candidates (p<0.05). In 9 cases with pulmonary stenosis (PS group), there were 5 candidates for the Fontan type operation. In the other 9 cases with PA (PA group) there were no candidates for the Fontan type operation. Only the size and the morphology of the pulmonary artery were significant factors (p<0.05) for candidates of the Fontan type operation among the risk factors such as size and morphology of the pulmonary artery, pulmonary vascular resistance and pressure, atrioventricular valve regurgitation, and single ventricular function. Finally, in these 24 cases, there were only 5 candidates (20.8%) for a Fontan type operation. In conclusion, in order to increase candidates for Fontan precedures, it is important to maintain an adequate pulmonary blood flow. Earlier PDA division and pulmonary arteries plasty are the most importantin PA group. In both groups pulsatile bidirectional cavopulmonary shunts may be useful to increase effective pulmonary blood flow without ventricular volume overload, which leads to atrioventricular valve regurgitation.

Seventeen cases of palliative right ventricular outflow tract reconstruction were reviewed to determine the optimal outflow diameter in this procedure. The clinical diagnoses of these 17 patients were pulmonary atresia with ventricular septal defect in 8, tetralogy of Fallot (TOF) in 7, TOF with complete atrioventricular canal in 1, double outlet right ventricle with pulmonary stenosis in 1. Techniques of reconstruction were transannular patch in 7 (valved 2, non-valved 5), extracardiac conduit in 5 (valved 2, nonvalved 3), outflow patch in right ventricle in 3, and others in 2. Pulmonary artery growth was not related to the diameter of reconstruction of outflow and postoperative Pp/Ps. The pressure in pulmonary artery tends to be hypertensive when the outflow diameter was more than 90% of the normal value. On the other hand, the minimal outflow diameter in nine cases of primary repaired TOF was in the range of 0.41 to 0.68 (mean of 0.59). In conclusion, the diameter of outflow reconstruction should be 60 to 80% of the normal pulmonary valve.

Four patients with d-TGA after atrial switch operation were treated with transvenous DDD pacemaker implantation for their postoperative dysrhythmia; complete atrioventricular block (CAVB) in two, sinus bradycardia with grade I atrioventricular block in one after the Mustard procedure and CAVB with sick sinus syndrome after the Senning procedure in one. From an anatomical point of view, the left atrial appendage was the only suitable anchoring site for the atrial lead. The patency of this cavity should be assessed by echocardiography and/or angiography before implantation. As for the ventricular lead, active fixation is recommended because of the relatively smooth endocardial surface of morphological left ventricle. Otherwise, transvenous DDD pacemaker implantation for patients after atrial switch operation was safely performed without any technical difficulties and with few complications.

The patient was a 56-year-old male with an 8-year history of Behcet's syndrome, who presented with chest pain. A saccular aneurysm (4×4cm) of the descending aorta was disclosed by X-ray and CT. Impending rupture of the aneurysm was suspected and emergency surgery was performed under cardiopulmonary bypass. The aneurysm consisted of a large thrombus, and a punched-out lesion on the aorta (φ10mm) was found beneath the thrombus. Closure with a Dacron patch was carried out successfully. Pathologic examination revealed the patient to have vasculo-Behcet's syndrome. Generally this desease has a poor prognosis, especially if an aneurysm is present, since sudden rupture can occur. Thus, prompt surgical treatment of the aneurysm should be considered. The patient was free of recurrence at the time of writing this report.