Staged Repair of Anomalous Origin of the Right Pulmonary Artery from the Ascending Aorta in a Low Birth Weight Infant : A Surgical Option for the Relief of the Postoperative Right Pulmonary Artery Stenosis / 日本心臓血管外科学会雑誌

A premature boy was born after 35 weeks gestation (1,561 g in weight) with a diagnosis of anomalous origin of the right pulmonary artery from the ascending aorta (AORPA) and perimembranous ventricular septal defect (VSD). The fourteenth day after birth, banding of the right pulmonary artery was performed as a palliative operation. At the age of 5 months (3.9 kg in weight), the right pulmonary artery branched from the ascending aorta, and was anastomosed to a flap made by the lateral wall of the main pulmonary artery with pericardial patch augmentation. VSD patch closure was performed concomitantly. Severe stenosis of the right pulmonary artery compressed by the ascending aorta and left pulmonary hypertension were revealed 3 weeks after the repair. At the age of 11 months, a surgical relief of the right pulmonary artery stenosis was performed. Transection of the ascending aorta provided an excellent exposure of the right posterior pulmonary artery. After patch plasty of the stenotic pulmonary artery, the divided ascending aorta was restored using a strip form patch on 4/5 circle of its posterior wall to extend the aorta and widen the space for the right pulmonary artery. This technique preserves growth potential of the ascending aorta. There are few reports of surgical repair of AORPA with VSD in low birth weight infants. We presented here a case with surgical relief of post-operative right pulmonary artery stenosis. Long term observation of repaired right pulmonary artery, and requiring residual slight hypertension of the left pulmonary artery.

Hemolytic Anemia Associated with a Teflon Felt Strip on a Proximal Anastomotic Site for Aortic Replacement in a Patient with Aortic Dissection / 日本心臓血管外科学会雑誌

A 61-year-old man was referred to our hospital for treatment of hemolytic anemia after ascending aortic replacement aortic dissection. Cine mode magnetic resonance imaging (MRI) showed stenosis at the proximal anastomostic site of a Teflon strip. We diagnosed hemolytic anemia induced by collision of red blood cells on the inverted felt strip. Conservative therapy with Sarpogrelate and β-blockers was effective to treat his hemolytic anemia. However, 7 years later he was re-admitted because of infective endocarditis at the aortic valve, and underwent aortic root replacement. Intraoperative findings showed a stiff and inverted Teflon felt strip causing stenosis of the proximal anastomosis. Hemolytic anemia should be considered a rare complication of using a Teflon felt strip to reinforce anastomosis for acute aortic dissection.

Leaflet Extension Aortic Valvuloplasty and Mitral Valve Replacement for Congenital Bicuspid Aortic Stenosis with Severe Mitral Regurgitation in a Child / 日本心臓血管外科学会雑誌

A baby girl with a low birth weight was given a diagnosis of congenital bicuspid aortic stenosis and mitral valve prolapse. At the age of 40 days, she underwent balloon aortic valvotomy, but significant aortic regurgitation appeared afterwards. Another surgical intervention became necessary by the age of 20 months (weight, 5.7 kg), because of intractable heart failure mostly caused by exacerbated mitral regurgitation. We performed a leaflet extension valvuloplasty for the small bicuspid aortic valve using an autologous pericardium treated by glutaraldehyde. The mitral valve was replaced with an ATS-16AP valve. Although her postoperative course was complicated with mitral paravalvular leakage and poor left ventricular function, she was discharged from hospital 6 months post operatevely. Leaflet extension valvuloplasty is a surgical option for infants with a small aortic annulus, but the procedure could be the only solution in cases when Konno or Ross techniques are not suitable.

Surgical Correction for Congenital Valvular and Supravalvular Aortic Stenosis Associated with Coronary Ostial Stenosis in a Child / 日本心臓血管外科学会雑誌

Supravalvular aortic stenosis is a rare obstructive lesion of the left ventricular outflow tract localized at the level of sinotubular junction. It has been recognized that supravalvular stenosis may occur as a part of Williams syndrome and is sometimes complicated by obstruction of the left main coronary artery. We successfully performed single patch augmentation for supravalvular aortic stenosis and left coronary ostial stenosis with concomitant aortic valvotomy in a child without Williams syndrome. The patient had been followed as congenital bicuspid aortic valvular and supravalvular stenosis. At the age of 3 years, cardiac catheterization revealed an increased pressure gradient of 90mmHg at the left ventricular outflow and newly developed ostial stenosis of the left coronary artery. An oblique incision on the ascending aorta was made above the sinotubular junction and extended leftward onto the left main coronary artery, and this incision opened the fibrous ridge at the left coronary artery. After commissurotomy for the bicuspid valve, both the supravalvular and ostial stenosis were augmented with a single autologous pericardial patch treated by glutaraldehyde. The pressure gradient was significantly reduced and the ischemic left ventricular dysfunction was eliminated.

Septal Myectomy for Hypertrophic Obstructive Cardiomyopathy in a Child / 日本心臓血管外科学会雑誌

A 6-year-old boy who had been found to have hypertrophic obstructive cardiomyopathy presented with severely limited symptoms of heart failure due to progressive left ventricular outflow obstruction. Cardiac catheterization revealed the peak systolic pressure gradient of 87mmHg at left ventricular outflow, and systolic anterior motion of the anterior mitral leaflet with concomitant mitral regurgitation was observed by echocardiography. Transaortic septal myectomy was performed using transesophageal echocardiography guidance before, during and after surgery. Although the patient needed permanent pacemaker implantation for postoperative complete heart block, the procedure reduced the left ventricular outflow obstruction and relieved his symptoms.

Damus-Kaye-Stansel Anastomosis for Rapid Progression of Subaortic Stenosis after Pulmonary Artery Banding in a Single Ventricle Infant with Aortic Arch Hypoplasia / 日本心臓血管外科学会雑誌

A cyanotic baby boy was given a diagnosis of single right ventricle, double outlet right ventricle, hypoplastic aortic arch, mitral atresia, atrial septal defect and pulmonary-ductus-descending aorta trunk. On day 4, extended aortic arch anastomosis and pulmonary artery banding were undertaken. At age 70 days, severe cyanosis and respiratory distress appeared and advanced rapidly. Angiography revealed critical subaortic stenosis and pulmonary hypertension, and the patient required urgent Damus-Kaye-Stansel anastomosis with concomitant right modified Blalock-Taussig shunt. Patients with single ventricle and hypoplastic aortic arch are a high-risk subgroup of progressive subaortic stenosis after initial pulmonary artery banding, and therefore need careful observation and may require early relief of subaortic stenosis.

Salvage Therapy with Non-Heparinized Extracorporeal Life Support for Massive Lung Hemorrhage after Pulmonary Thromboembolectomy / 日本心臓血管外科学会雑誌

Lung hemorrhage associated with pulmonary reperfusion injury is a rare but lethal condition. We presented a case salvaged by non-heparinized extracorporeal life support for massive lung hemorrhage after pulmonary thromboembolectomy. Sub-acute pulmonary thromboembolism with a floating right atrial thrombus was diagnosed in 63-year-old woman by computed tomography and echocardiography. An emergency pulmonary thromboembolectomy was performed using cardiopulmonary bypass and moderate hypothermia. Immediately after reperfusion, extraordinary lung hemorrhage occurred and continued. We decided to take over the standard cardiopulmonary bypass with a non-heparinized extracorporeal life support system. Fortunately, hemostasis of the lung hemorrhage was completely secured within 12h, and the extracorporeal life support was terminated at 20h after the surgery. The patient was extubated at 48h after the surgery, and was discharged after the insertion of an inferior vena cava filter for a floating deep venous thrombus. Although the necessity, efficacy and risk of the non-heparinized extracorporeal life support should be clarified, we conclude that it could be the treatment of choice for life threatening lung hemorrhage associated with pulmonary reperfusion injury.

Profound Hypothermia-Induced Platelet Dysfunction during Heparinized Cardiopulmonary Bypass / 日本心臓血管外科学会雑誌

There is an impression among cardiothoracic surgeons that the technique of profound hypothermic circulatory arrest (PHCA) is associated with an increased bleeding tendency compared to conventional bypass surgery. In addition to the recognized factors contributing to the hemorrhagic tendency seen in moderate hypothermic cardiopulmonary bypass (CPB), it is likely that the lower temperature utilized in PHCA may exacerbate platelet dysfunction. In this report, platelet counts and functions at the same cardiopulmonary bypass time were compared in human PHCA surgery (hypothermia group, <i>n</i>=16) and moderate hypothermic cardiopulmonary bypass surgery (control group, <i>n</i>=20). Mean platelet count corrected by hematocrit in the hypothermia group at 2h of CPB was significantly lower than in the control group (3.7×10⁴μl vs. 11.4×10⁴/μl, <i>p</i><0.0001). In the hypothermia group, there were significant increases in the percentage of GMP-140 (P-selectin)-positive platelets (11.8% vs. 8.3%, <i>p</i>=0.0091) at 1h of CPB, and also in microparticles (24.8% vs. 10.5%, <i>p</i><0.0001) and aggregated platelets (3.4% vs. 1.4%, <i>p</i>=0.0058) at 2h of CPB. Profound hypothermic circulatory arrest used in surgery for aortic arch aneurysm or dissection may cause irreversible platelet dysfunction and contribute to hemorrhagic tendency during the surgery. To minimize platelet dysfunction during CPB, the lowest blood temperature should be maintained above 15°C.

Autologous Blood Donation in Open-Heart Surgery in Cooperation with the Red Cross Blood Center / 日本心臓血管外科学会雑誌

An investigation on the efficacy of preoperative autologous blood donation in open-heart surgery was made using frozen red blood cells and MAP red blood cells in cooperation with the Red Cross Blood Center. In 109 cases which received the donation, the rate of cases which received no homologous blood transfusion was 93.6% (35.3% in the cases without donation). Even in the cases of redo operation or aortic surgery, in which extensive blood loss is expected, 75% of those given a donation of 1600-2000ml frozen blood required no homologous blood transfusion. The hemoglobin concentration in the cases which received blood donation for more than 4 weeks did not decrease, indicating that safe donation is feasible. The aforementioned frozen and MAP blood preparations can be preserved for a long period so that blood donation can be started even before deciding on the date of operation. Also, its usefulness is not affected by the postponement of the operation. Furthermore, there was no problem in safety with respect to transfer, treatment, and storage of the autologous blood in cooperation with the Red Cross Blood Center, suggesting that this is useful as a preoperative donation method, especially in small- and middle-scale hospitals, which have no separate blood centers. However, there were 2 cases in which aggravated symptoms were noted after blood collection. Therefore, it is important to carefully select cases for autologous blood donation in open-heart surgery and it is desirable to set up appropriate donation schedules.

Investigation on Improvement of Peripheral Circulation by Continuous Use of Prostaglandin E1 during Open Heart Surgery. Evaluation with Peripheral Blood Flow by Laser Doppler Flowmeter and Temperature Difference between the Periphery and Core / 日本心臓血管外科学会雑誌

Prostaglandin E₁ (PGE₁) was used continuously in adults from immediately after induction of anesthesia, during extracorporeal circulation, to the acute phase after open heart surgery. Using blood flow in the toe determined by laser Doppler flowmeter and the temperature difference between periphery and core as indices, the effects of afterload reduction and improvement of peripheral circulation were investigated. Subjects were 17 adults who underwent open heart surgery. PGE₁ was used in 7 patients and not used in 10. In the group using PGE₁, continuous injection of 0.015μg/kg/min of PGE₁ was started immediately after induction of anesthesia and was maintained during extracorporeal circulation until the acute phase after surgery. During extracorporeal circulation, perfusion pressure was kept at 50∼60mmHg and PGE₁ injection was controlled within the range of 0.015∼0.030μg/kg/min. At completion of extracorporeal circulation, the dose was fixed at 0.015μg/kg/min again. The degree of improvement of peripheral circulation was evaluated on the basis of hemodynamics, blood flow in the toe determined by laser Doppler flowmeter and the temperature difference between periphery and core, at induction of anesthesia (before using PGE₁) on completion of extracorporeal circulation, and in the acute phase after surgery. The value of blood flow in the toe determined by laser Doppler flowmeter was significantly higher in the PGE₁ group than in the non-PGE₁ group, from completion of extracorporeal circulation to the acute phase after surgery. Moreover, peripheral temperature was significantly higher in the PGE₁ group than in the non-PGE₁ group at completion of the extracorporeal circulation as well as immediately after surgery, and the temperature difference between periphery and core was significantly smaller. Continuous injection of PGE₁ enabled smooth control of perfusion pressure during extracorporeal circulation. Although there was no significant difference in peripheral vascular and total pulmonary resistance, the coefficients tended to be lower in the PGE₁ group. The use of PGE₁ during open heart surgery seems to be an effective method to improve peripheral circulation.

A Fourteen-Day-Old Neonate with Congenital Aortic Valve Stenosis Successfully Treated with Open Valvular Commissurotomy / 日本心臓血管外科学会雑誌

A thirteen-day-old neonate was admitted because of systolic heart murmur, tachycardia, tachypnea and sucking weakness. The chest X-ray film demonstrated remarkable cardiomegaly and pulmonary congestion. Echocardiography detected marked thickening and stenosis of the aortic valve, and left ventricular dysfunction (EF=10%). The pressure gradient between left ventricle and ascending aorta was presumed 130mmHg with pulsed Doppler echocardiography, Since he did not respond to conservative treatment, an emergency open aortic valvular commissurotomy under cardiopulmonary bypass was performed the day after admission. We made incisions of 1mm in the left side and 0.5mm in the right side commissure of the adherent bicuspid aortic valve. After the procedure, left ventricular function improved (EF=57%), and the pressure gradient was reduced to 62mmHg. He showed good recover from the congestive heart failure. There are few reports about operative treatment of congenital aortic valve stenosis in neonates. This is considered to be the third youngest successful operative case of open aortic valvular commissurotomy in Japan.

A Case Report of Successful Surgical Treatment of High Aortic Occlusion with Acute Ischemia of Intrapelvic Organs and Bilateral Lower Extremities / 日本心臓血管外科学会雑誌

This is a case report of a 57-year-old woman with high aortic occlusion (HAO) who had acute symptoms of severe ischemia of the lower extremities and the intrapelvic organs. Generally, HAO is a chronic ischemic disease of the lower extremities and the intrapelvic organs; therefore, acute HAO is relatively rare. Acute thrombotic occlusion of a major collateral artery might be the cause of acute HAO. Laser Doppler flowmetry of the sigmoid colon was useful to evaluate the ischemia of intrapelvic organs. Thrombectomy of the juxtarenal portion with the suprarenal aortic cross clamp was performed within four minutes, then the clamp was moved to the infrarenal portion. The remaining occluded aorta was replaced with a Y-shaped knitted Dacron graft. She had no symptoms after the surgery except renovascular hypertension. Seventy five percent stenosis of the right renal artery was exacerbated to 99%. Vascular clamping of the right renal artery might have been the cause of severe stenosis. Percutaneous transluminal renal angioplasty was successfully performed after the surgery. Aggressive renal artery reconstruction during surgery is recommended in cases with moderate or severe renal artery stenosis.

A Successful Result of One Stage Operation for Atrial Septal Defect and Funnel Chest / 日本心臓血管外科学会雑誌

A 6-year-old boy underwent one stage operation for atrial septal defect (ASD) and funnel chest. The procedure began with removal of cost-sterno complex (plastron) following median skin incision. Plastron was kept in cold saline with antibiotics during ASD closure, and sterno-costal elevation method was performed. Simultaneous operation for heart disease and funnel chest is profitable in preventing postoperative circulatory or respiratory complications, in avoiding problems of two stage operation such as adhesion and mental stress of the patients. In addition, wide exposure and easy approach to the heart is available with this one stage procedure. Although current refinement both of cardiac and thoracic surgery has encouraged the possibility of simultaneous corrections for heart disease and funnel chest, much precautions against bleeding and infection are necessary for the satisfactory surgical result.