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.

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.

A neonate, presenting with cyanosis, received the diagnosis of persistent truncus arteriosus with truncal valve stenosis with insufficiency. Her disease was classified as persistent truncus arteriosus Van Praagh type A1, or Collett and Edwards type I. At the age of 2 months, she underwent a modified Blalock-Taussig shunt, and her operative team was waiting for adequate body weight gain before performing further surgery. At the age of 1 year, however, she began to have repeated episodes of congestive heart failure due to severe tricuspid valve regurgitation and truncal valve insufficiency. When she reached 18 months of age, she underwent a definitive operation including a truncal valve plasty, VSD patch closure, and a right ventricular outflow tract reconstruction. Postoperative echocardiography 6 months after surgery showed a good truncal valve function with minimal regurgitaion. Truncal valve surgery is a challenging operation ; we report a successfully treated case, though the patient will require extensive follow-up.

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.

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.

It is difficult to treat vulvar pain of an unknown cause. We report a case of vulvodynia with dysuria treated effectively with goshajinkigan. The case was 92 years-old female who could not sleep well because of vulvar pain. She did not have inflammation of the vulva or vagina. Moreover, she was sometimes treated with urethral catheterization for urinary retention. Goshajinkigan was prescribed, and her pain was decreased so much, that she could sleep well. Furthermore, she was relieved of her urinary retention.

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.

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.

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.

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.