Although the results of surgical treatment for acute type A dissection have improved because of progress in surgical techniques, the prognosis is still very poor and optimal therapeutic approach is still not clearly established for cases of acute dissection complicated with malperfusion. Of 134 patients who presented with acute aortic dissection between January 1986 and June 1999, 57 had acute type A dissection and 10 had acute type A dissection with malperfusion. Patient age ranged from 53 to 78 (average, 64.6) years. There were 6 men and 4 women. There was accompanying cerebral ischemia in 3 cases, coronary ischemia in 1, visceral ischemia in 5, renal ischemia in 2, ischemia of the extremities in 7, and multiple organ ischemia in 5. One patient died before surgery, and another patient died after sternotomy due to aortic rupture. The other 8 patients underwent surgical operations. The following surgical procedures were performed: bypass grafting to the superior mesenteric artery was performed in 1 patient, stent implantation to the right coronary artery followed by ascending aortic replacement (19th day after onset) was performed in 1, and aortic repair (5 ascending aortic replacements and 1 hemiarch replacement) in the acute phase was performed in 6. The mortality rates were 66.7% (2/3) in patients with cerebral ischemia, 0% (0/1) in the patient with coronary ischemia, 80% (4/5) in those with visceral ischemia, 100% (2/2) in those with renal ischemia, 42.9% (3/7) in those with ischemia of the extremities, 80% (4/5) in those with multiple organ ischemia, and 50% (5/10) in all cases. All patients whose base excess (B.E.) was less than -10mEq/l on admission died (4/4). We conclude that in order to improve surgical results in patients with acute type A dissection with malperfusion, different approaches may be required for each patient. The combination of aortic repair and percutaneous reperfusion are important. Arterial blood gas analyses were simple, and the values of B. E. at admission were useful to determine the surgical strategy in these patients and to predict their prognosis.

The isolated unilateral absence of a proximal pulmonary artery is a rare congenital lesion that presents various symptoms. Although some reports have shown one-stage reconstruction of the pulmonary circulation in infants, the two-stage approach is required in the situation of pulmonary arterial hypoplasia. In these cases, the usual approach is systemic pulmonary shunting for the first operation, to obtain growth of the pulmonary vascular bed, and then connecting bilateral pulmonary arteries for the second operation. Moreover, in the majority of patients without a right proximal pulmonary artery, some material is required for reconstructive surgery that corresponds to the patient's growth. A girl aged 2 years and 10 months with absent right proximal pulmonary artery, underwent modified Blalock-Taussig shunting with a free autograft of the azygos vein. The shunt was banded to prevent excessive pulmonary blood flow. Reconstructive surgery was performed 10 months after the first operation. At the second operation, growth of the right distal pulmonary artery and azygos autograft was satisfactory. Therefore, we used this autograft as an interposed graft of the right and main pulmonary arteries. Her postoperative course was uneventful. We advocate the usefulness of the azygos vein for graft material possessing the possibility of growth. This autograft is useful for pulmonary arterial reconstruction, such as the present case, and also may be useful for a systemic-pulmonary shunt, while growth is anticipated for more complex heart diseases.

Anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) is a rare congenital defect, and few cases have been reported in the pediatric age group. Unlike the anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), patients with ARCAPA are usually asymptomatic and often diagnosed incidentally. Here, we present a surgical case of a 2-month-old male who was suspected of having ARCAPA in the neonatal period. The patient was suspected of having ARCAPA due to the detection of a heart murmur and the results from an echocardiogram taken the 4th day after birth. Coronary angiography on the 37th day after birth showed the contrast flow to the pulmonary artery through the right coronary artery from the left coronary artery; thus, we diagnosed ARCAPA. Surgical intervention should be performed before the appearance of myocardial ischemia due to decreasing pulmonary artery pressure, so we performed reimplantation of the right coronary artery to the ascending aorta and patch plasty of the pulmonary artery at 2 months after birth. The patient was discharged from the hospital on the 16th day after the operation, and a 6-month postoperative coronary angiography revealed good right coronary flow and the disappearance of collaterals.