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.

Leg malperfusion accompanied with type B acute aortic dissection (AAD) is reported to be an independent predictor for mortality. In such a case, though aortic replacement, extra anatomical arterial bypass or endovascular aortic repair (EVAR) can be selected, an appropriate treatment strategy has not been established yet. A 53-year-old woman was urgently hospitalized with sudden low back pain and right leg weakness, despite the right popliteal and anterior tibial arteries being palpable. Computed tomography (CT) revealed a type B AAD, and antihypertensive therapy was initiated. She complained of intermittent claudication during rehabilitation, and right leg ischemia with decreased ankle brachial pressure index (ABPI) was detected. The follow-up CT revealed the narrow true lumen of the right common iliac artery compressed by the thrombosed false lumen and the large entry of the aortic dissection in the terminal aorta. At the subacute phase of the aortic dissection, EVAR was performed. To expand the true lumen and exclude the entry, Y-shaped stent-grafts were implanted in the infra-renal aorta and the bilateral common iliac arteries. The postoperative course was uneventful. Postoperative ABPI returned to the normal range, and the intermittent claudication disappeared. In conclusion, EVAR should be considered in patients with type B AAD complicated with leg malperfusion.