A Surgical Case of Anomalous Origin of the Right Coronary Artery from the Pulmonary Artery in Early Infancy / 日本心臓血管外科学会雑誌

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.

Staged Repair of Unilateral Absence of the Right Proximal Pulmonary Artery Using Autologous Azygos Vein Graft Interposition / 日本心臓血管外科学会雑誌

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.

Mitral Valve Replacement for Recurrent and Multiple Cerebral Embolisms Caused by Mitral Annular Calcification / 日本心臓血管外科学会雑誌

A 69-year-old woman had syncope and aphasia. Magnetic resonance imaging showed multiple cerebral infarctions in both hemispheres. Cardiogenic embolisms were suspected, but no arrhythmic causes were shown. Transesophageal echocardiography revealed a highly calcified mitral annulus (MAC) with a rough intraluminal surface and mild mitral regurgitation, but no thrombus or tumor in the left heart system. However, recurrent multiple cerebral embolisms occurred in spite of strict anticoagulation therapy. We speculated that spontaneous rupture of the MAC was the cause of the scattered cerebral embolisms, and we therefore planned to remove the MAC as safely as possible and to endothelialize the deficit of MAC with autologous pericardium. Operative findings revealed that the MAC in P2-P3 had ruptured longitudinally and the ostium of the left atrium was connected to the ostium of the left ventricle as an inter-atrioventricular tunnel beneath the posterior mitral annulus with a fragile calcified wall. The finding suggested that calcified particles that had peeled away from the MAC by normal heart beating resulted in the cerebral infarctions. Therefore, she underwent resection of the MAC and mitral valve replacement with reinforcement of the decalcified posterior mitral annulus between the posterior left ventricular wall and the left atrial wall using autologous pericardium, which enabled both appropriate insertion of a mechanical prosthetic valve and endothelial continuity covering the surface of the residual MAC. No systemic embolism has occurred for two and a half years after surgery. This is the first case report of cerebral embolism caused by a spontaneously ruptured MAC.

Successful Implantations of Autologous Peripheral Blood-Derived Mononuclear Cells Pretreated by Erythropoietin and Blood Donation in a Patient with Buerger Disease and Intractable Finger Ulcers / 日本心臓血管外科学会雑誌

A 48-year-old man with Buerger disease and intractable finger ulcers underwent successful transplantation of autologous peripheral blood-derived mononuclear cells pretreated with erythropoietin and blood donation to activate bone marrow function. Clinical symptoms on his finger ulcers improved significantly within 1 month after mononuclear cell transplantation, however, one of the intractable ulcers reappeared 2 months later. In total three transplantations were performed. Every cell transplantation revealed similar effectiveness 1 month later, and the interval of the subsequent disappearance of finger ulcers ranged from 3–6 months. There were no adverse effects based on this new therapy. These findings suggest that autologous peripheral mononuclear cell transplantation pretreated with erythropoietin and blood donation might be a non-invasive and safe alternatives for patients with Buerger disease and intractable finger ulcers.

Two Cases of Successful Treatment of Tracheo-Innominate Artery Fistula / 日本心臓血管外科学会雑誌

Tracheo-innominate artery fistula (TIF) is a relatively rare, highly lethal complication after tracheostomy. We report two cases of successfully managed TIF with relatively long-term survival after a massive hemorrhage from the tracheostomy site. Different approaches were employed for the temporary control of bleeding and in the subsequent surgery in these two cases, thus we examined the pros and cons of each approach. In Case 1, the bleeding was temporarily controlled using a long tracheostomy tube and orotracheal tube and by applying digital compression to the tracheostomy site. The right common iliac-to-right axillary artery bypass was then performed, followed by a median sternotomy to resect the injured innominate artery and to directly close the tracheal fistula. In Case 2, an over-inflated cuff was used after inserting the long tracheostomy tube to its full extent from the tracheostomy site, which was able to stop the bleeding for a while. This resulted in massive rebleeding when the cuff was remored while the patient was being transferred to the operation room. The patient underwent median sternotomy followed by anatomic innominate artery reconstruction using an ePTFE graft, and closure of the tracheal fistula was performed using autologous pericardium patch. In the treatment of TIF, prevention must be the greatest priority. If premonitory bleeding occurs (in the form of minor fresh bleeding), the patient must be evaluated immediately using bronchoscopy and CT imaging, and surgical intervention should be considered depending on the condition. If the bleeding is severe, the airway should be maintained and the bleeding should be temporarily controlled. We were able to control the bleeding in Case 1; however, in Case 2, we were unable to so since the tracheostomy tube was not long enough. After the surgery, we thought that the outcome might have been different if we had used the orotracheal tube instead of the tracheostomy tube and we believe this is one point that should be carefully considered. As for the procedures chosen for the TIF surgeries, anatomic revascularization of the innominate artery and closing the tracheal fistula are the procedures of choice when the operation field is not contaminated. However, when the field is contaminated (for example, in the case of a large fistula), extra-anatomic revascularization may be the only choice. This indicates that early evaluation and intervention of TIF are also important in terms of preventing infections and long-term prognosis.

Treatment of Patients with Acute Type A Dissection with Malperfusion / 日本心臓血管外科学会雑誌

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/<i>l</i> 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.