Aorto-Left Ventricular Tunnel : a Surgical Intervention at 2 Hours after Birth / 日本心臓血管外科学会雑誌

A male infant was diagnosed with aorto-left ventricular tunnel (ALVT) because of advanced left ventricular hypertrophy and massive reflux from the aorta to the left ventricle during the fetal period. Aorto-left ventricular tunnel closure was emergently performed 2 h after birth in cooperation with the hospital where he was born. The aortic end of the ALVT was located above the right-left coronary commissure, and the measured diameter was 8 mm. The aortic valve was tricuspid, but it was thickened and the opening was limited ; it was further complicated by a right coronary artery anomaly. Taking the valve distortion and surgical invasion into consideration, only the aortic end was closed using an autologous pericardial patch. Although mild aortic valve stenosis and regurgitation remained after surgery, the postoperative course was uneventful, and the left ventricular hypertrophy and cardiac function were improved.

Posterior Plication of the Ascending Aorta to Relieve Tracheal Compression after Aorto-Left Ventricular Tunnel Repair / 日本心臓血管外科学会雑誌

A nine-month-old boy with a prenatal diagnosis of aorto-left ventricular tunnel (ALVT) and aortic valve disease underwent an urgent ALVT patch closure approximately 2 h after birth. The patient subsequently developed airway compression due to the progressive dilatation of the ascending aorta postoperatively associated with aortic regurgitation and stenosis. After 9 months of prolonged respiratory symptoms following the ALVT repair, he underwent a posterior plication of the ascending aorta. After plication, the tracheal compression and respiratory symptoms improved. A computed tomography (CT) scan 1 month after surgery revealed significant free space between the aorta and the trachea, which relieved the aortic compression. Posterior plication of the ascending aorta is achieved by resecting the posterior aortic wall adjacent to the airway. It is the procedure of choice for longer and more effective relief of airway compression compared to other procedures such as anterior aortopexy, the lateral sliding technique, and side clamp plication.

Modification of End-to-End Anastomosis for Long-Segment Coarctation of the Aorta / 日本心臓血管外科学会雑誌

<p>Several problems have been reported following coarctectomy, especially in cases involving long-segment coarctation (COA). Although residual COA, proximal arch kinking, and airway compression may occur after coarctectomy, avoiding the use of artificial materials provides a better chance for the subsequent growth of the aorta. We successfully performed a modified end-to-end anastomosis with subclavian flap aortoplasty for a two-month-old boy with COA. A two-month-old boy was admitted to our hospital for nocturnal tachypnea and a feeding disorder. The initial echocardiography showed a preductal long COA beyond the left subclavian artery. A perimembranous VSD, a patent foramen ovale, and a patent ductus arteriosus were also noted. The left ventricular function was mildly depressed with an ejection fraction of 59%. Enhanced CT revealed a long-segment COA with a length of 15 mm. The blood pressure gradient between the upper and lower limbs was 40 mmHg. The operation was performed at the age of 2 months. The 4th intercostal space was opened through a posterolateral left thoracotomy incision. The distal end of the isthmus was so ligated as to maintain blood perfusion to the lower body through the PDA. The arch was clamped between the left carotid and the left subclavian artery (LSCA). The LSCA and the isthmus were divided as distally as possible, and the two distal ends were longitudinally incised and sutured to each other in a side-to-side fashion using a 7-0 polypropylene continuous suture. After complete resection of the ductal tissue, a newly created distal arch was anastomosed to the descending aorta. The left ventricular ejection fraction was increased to 74% at discharge. Catheterizations 3 years after the surgery did not reveal any stenosis or deformity in the aorta at normal PA pressure. The patient has been doing well and is free of complications 7 years after the surgery. At present, end-to-end anastomosis and aortic arch advancement with or without cardiopulmonary bypass are widely used procedures for coarctectomy ; however, a modified end-to-end anastomosis is still a viable option for cases involving long-segment coarctation.</p>

New Staged Repair of Neonatal Tetralogy of Fallot with Severe Absent Pulmonary Valve Syndrome / 日本心臓血管外科学会雑誌

Primary repair of the tetralogy of Fallot with absent pulmonary valve syndrome (TOF/APV) is associated with high mortality rates of 17-33%, especially in neonates. Our standard strategy involves a staged repair with a first palliation, performed during the neonatal period, that includes main pulmonary septation with an ePTFE patch, pulmonary arterioplasty for reduction of vascular dilation, and a modified Blalock-Taussig shunt. We performed successful repairs on two neonates with TOF/APV, one symptomatic and the other non-symptomatic, with this strategy. Case 1 : A 7-day-old boy had TOF/APV, with progressively worsening respiratory distress. His left bronchi, superior vena cava and left atrium were compressed by a dilated pulmonary artery, which was repaired by emergency surgery. Decreasing the diameter of the pulmonary artery (PA index from 2,550 to 525) relieved the compressed organs. Case 2 : A 16-day-old boy with TOF/APV with a main pulmonary artery that increased in diameter from 8 to 17 mm in the course of a single day. He was treated in the same fashion as Case 1. At 1 year of age, an intracardiac repair with tricuspid anuuloplasty was performed successfully. This strategy is much safer than a primary repair and is a good choice for neonatal repair of TOF/APV.

A Successful Surgical Treatment of Ebstein's Anomaly by Hetzer's Procedure in an Adult / 日本心臓血管外科学会雑誌

A 29-year-old woman, who had been diagnosed with Ebstein's anomaly associated with paroxysmal supraventricular tachycardia due to Wolff-Parkinson-White (WPW) syndrome, was referred to our hospital for treatment of congestive heart failure and tachycardia. She had undergone a catheter ablation for WPW syndrome at the age of 28 years. Subsequently, surgical treatment for Ebstein's anomaly was indicated because of persistent symptoms of heart failure due to tricuspid regurgitation (TR). The echocardiogram and pathologic findings corresponded to Ebstein's anomaly of the Carpentier type B classification, with severe displacement of the septal and posterior leaflets resulting in moderate TR. A mobile anterior leaflet of sufficient size without a cleft enabled us to successfully perform Hetzer's procedure. In this procedure, the large mobile anterior leaflet was approximated to the opposing true tricuspid annulus with a mattress suture of 3-0 polypropylene passed from the anterior leaflet annulus to the true tricuspid annulus at the site of atrialized right ventricle near the coronary sinus. The postoperative course was uneventful, and the cardiothoracic ratio reduced from 56% to 48% with mild TR. In this adult case of Carpentier's type B adult Ebstein's anomaly, Hetzer's procedure allowed reconstruction of the tricuspid valve mechanism of “leaflet-to-septum” coaptation at the level of the true annulus by approximating the anterior leaflet. This was, effective in reducing the patient's moderate TR. We conclude that this procedure is a simple and reproducible method for repairing the tricuspid valve in Ebstein's anomaly, especially for cases with a large mobile anterior leaflet.

Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery with a Specific Left Coronary Artery Route / 日本心臓血管外科学会雑誌

We surgically treated a case of anomalous origin of the left coronary artery from the pulmonary artery with the specific route of the left coronary artery in a 17-month-old boy. He had suffered persistent cough and poor weight gain since the age of 4 months. An ultrasound cardiography, at the age of 16 months, revealed retrograde blood flow of the left coronary artery into the main pulmonary artery. Moreover, a chest computed tomography showed an anomalous left coronary artery arising from the bifurcation of the right pulmonary artery and winding in contact on the posterior aortic wall. Though the anomalous left coronary artery shared adventitia with the aortic wall we were able to separate the coronary artery from the aorta, and the patient underwent direct transplantation of the left coronary artery. The postoperative course was uneventful and recovery was rapid.

A Surgical Case for Acute Heart Failure in a 1-month-old Infant with a Right Cervical Aortic Arch / 日本心臓血管外科学会雑誌

A 2-day-old male baby was referred to our hospital because of a heart murmur. We diagnosed as a right cervical aortic arch, and coarctation between the right carotid and right subclavian artery. On echocardiography, the velocity at the coarctation was 1.8 m/s, the left ventricular ejection fraction (LVEF) was 53%, and he was asymptomatic during the neonatal period. A chromosome examination showed a deletion of 22q11 syndrome. At 1 month, he weighted 3.8 kg and was readmitted to our hospital for wheezing. Echocardiography showed a left ventricular dysfunction with LVEF of 24%. The coarctation velocity increased to 5.1 m/s. An urgent operation was performed because of a severely depressed cardiac function. His LVEF increased to 67%, and the velocity was less than 1 m/s postoperatively, and he was discharged on postoperative day 32. We report a rare neonatal surgical case of a right cervical arch with a coarctation.

Modified Konno Operation for Aortic Valve Regurgitation after Arterial Switch Operation / 日本心臓血管外科学会雑誌

We describe a case of postoperative aortic valve regurgitation (AR) after arterial awitch operation (ASO) successfully managed by the modified Konno procedure. A 4-year-old girl with complete transposition of the great arteries (TGA, Type II) had undergone the ASO (LeCompte maneuver) at 10 days of age. Because of progression of moderate AR 4 years after ASO, the modified Konno procedure with aortic valve replacement (SJM 21mm) was successfully performed. She remains in good clinical condition at the last follow-up at 5 years.

A Case of Mycotic Aneurysm of the Pulmonary Artery with Pulmonary Artery Fistula following Pulmonary Artery Banding / 日本心臓血管外科学会雑誌

A 9-month-old boy who had been given a diagnosis of double outlet right ventricle (DORY), partial anomalous pulmonary venous return (PAPVR), ventricular septal defect (VSD), pulmonary hypertension (PH) and polysplenia with azygos connection, underwent pulmonary artery banding at the age of 6 months. At 2 months after surgery, a chest computed tomogram revealed a main pulmonary artery aneurysm and a main pulmonary artery-right pulmonary artery fistula caused by bacterial endocarditis due to a methicillin-resistant <i>Staphylococcus epidermidis</i>. We performed pulmonary arterioplasty and re-pulmonary artery banding for acute aggravation of cardiac insufficiency and obtained good results. This is an extremely rare case that was treated infectious pulmonary artery aneurysm and fistula after pulmonary artery banding.

A Case of Aortopulmonary Window after Balloon Angioplasty for Bifurcation Pulmonary Stenosis Based on the Jatene Procedure / 日本心臓血管外科学会雑誌

A 6-month-old baby boy had undergone the Jatene procedure at 4 days. Four months later, catheter intervention (balloon angioplasty) was performed because of severe stenosis at the bifurcation of the pulmonary arteries. Twenty days later, several episodes of cyanosis occurred and he was readmitted. The existence of shunt flow between the sinus of valsalva and the pulmonary bifurcation was detected by echocardiography and examination by 16-row MDCT revealed 2 holes at this site. Under a diagnosis of aortopulmonary (AP) window, the patient was placed on cardiopulmonary bypass and the pulmonary artery was opened after aortic clamping. There was a ridge between the bifurcation of the pulmonary arteries. After removing it, 2 holes were visualized that resembled the findings on 16-row MDCT. These holes were closed with Xenomedica patches and the main pulmonary artery was also extended with a Xenomedica patch. AP window is a rare complication after balloon angioplasty for pulmonary stenosis, but we must take great care to prevent this complication.

Prosthetic Valve Replacement in Corrected Transposition with Severe Tricuspid Valve Dysfunction / 日本心臓血管外科学会雑誌

Tricuspid valve regurgitation, which is a main complication of corrected transposition of the great arteries (C-TGA), greatly influences prognosis like atrioventricular block, but there are many differing openions concerning the treatment of this condition childhood. In 2 cases of C-TGA (S. L. L.) without other cardiac anomalies, we performed tricuspid valve replacement for severe valve dysfunction.

Pacemaker Implantation for Atrial Fibrillation with Bradycardia in Patients with Mitral Valve Disease / 日本心臓血管外科学会雑誌

Some cases of atrial fibrillation and bradycardia show improvement in slow ventricular response after valvular surgery. However, there is still no established view regarding the indications of pacemaker implantation for the bradyarrhythmia with valvular disease. In 24 cases (permanent pacing group: 15, non-pacing group: 9) of those with bradyarrhythmia who were fitted with a myocardial pacing lead at the time of valvular surgery, we examined predictions of pacemaker implantation and the role of valvular surgery for the bradyarrhythmia. The permanent pacing group showed much larger values than the non-pacing group in regard to preoperative NYHA, right and left atrial pressure, and duration of atrial fibrillation. After valvular surgery, many cases that had significantly decreased left atrial pressure after operation improved with regard to bradycardia. We should judge the indication of pacemaker implantation after valvular surgery from the evaluation of preoperative hemodynamics and early postoperative cardiac function. Because atrial fibrillation tends to accompany bradycardia due to chronic atrial load, we must make an effort to promote the rapid recovery of cardiac function by doing valvular surgery as early as possible.

Three Cases of Chronic Type A Aortic Dissection with Connective Tissue Disease / 日本心臓血管外科学会雑誌

We performed a modified Bentall operation and aortic arch replacement simultaneously in three cases of chronic type A aortic dissection with connective tissue disease. Two of the subjects were men. Ages ranged from 37 to 48 years. There were two cases of Marfan's syndrome, and one case of cystic medial necrosis. All patients had annuloaortic ectasia (AAE), severe aortic regurgitation (AR) and marked dilatation at the base and arch of the aorta with extensive dissecting lesions. Widespread, progressive vascular lesions are often seen, especially among cases of dissecting aneurysm of the aorta with connective tissue disease, and there is a high probability that new vascular lesions and valvular diseases will result after surgery. Therefore, cases must be followed, keeping in mind the possibility of early extended aortic operation and secondary surgery.

A Case of Ischemic Cardiomyopathy Complicated by Porcelain Aorta Treated with Dor Operation and CABG Using an Occlusion Balloon / 日本心臓血管外科学会雑誌

In patients with so-called porcelain aorta characterized by calcification of the total aorta, manipulation of the ascending aorta can cause cerebral infarction and other conditions due to aortic dissection or rupture and calcified debris. In the present case with ischemic cardiomyopathy and porcelain aorta, an occlusion balloon catheter was inserted into the ascending aorta to avoid its clamping, followed by Dor operation and CABG under cardiac arrest with normothermic extracorporeal circulation. Techniques such as deep hypothermic circulatory arrest and surgery while the heart is beating are often currently used as auxiliary methods to avoid aortic clamp. However, the present case with insufficient left ventricular function required a left ventriculotomy, and thus the technique presented here is useful for shortening the surgical time and ensuring a reliable outcome of the operation.