[Concomitant Operations for Thoracic Aortic Aneurysm and Myasthenia Gravis;Report of a Case].

A 77-year-old man, who had been under medical treatment for myasthenia gravis without thymoma, was diagnosed with aortic arch aneurysm. He underwent total aortic arch replacement and total resection of the thymus through median sternotomy. His symptoms relating to myasthenia gravis dramatically disappeared after the surgery. The serum anti-acetyl chorine receptor antibody decreased from 2.7 to 0.7 nmol/l (N<0.2) with the reduction of oral predonisolone from 12.5 to 5 mg/day at 4 years after the surgery. The concomitant operations significantly improved his quality of life.

[Left ventriculoplasty in a patient with suspected takotsubo cardiomyopathy followed by a left ventricular aneurysm].

A 42-year-old woman was admitted with chest pain. Coronary angiography did not reveal any significant stenosis, but left ventriculography showed akinesis and ballooning of the apex with a hyperkinetic basal segment indicating Takotsubo cardiomyopathy. Cerebral embolism occurred after one and a half years because of a left ventricular thrombus. The apical akinesis had worsened to a left ventricular aneurysm (maximum diameter 43 mm). The left ventricle was reconstructed to avoid repeated thrombus formation and cerebral infarction despite anticoagulant therapy. A pathological assessment revealed a fibrotic myocardium, but the cause of the cardiac aneurysm remained unknown. Although the outcome of Takotsubo cardiomyopathy is relatively good, careful observation and appropriate treatment are needed considering the possibility of aggravation.

Improvement of cardiac geometry and function after cardiac resynchronization therapy for relapsed deterioration of pediatric dilated cardiomyopathy due to a noncompacted left ventricle and cardiac dyssynchrony.

We report a pediatric case of successful cardiac resynchronization therapy (CRT) for acute relapsed deterioration of dilated cardiomyopathy with cardiac dyssynchrony due to a noncompacted left ventricle (LV). A 20-month-old female developed severe congestive heart failure, which was classified as New York Heart Association class IV. She failed to thrive despite intravenous inotropic support. Her LV function and dimension improved markedly after CRT. The beneficial effect of CRT in this patient has remained for more than 3 years.

[Malignant disease identified after the emergency cardiac operation].

We experienced 2 patients who died early after emergency cardiac operation because of malignant diseases. In one case, we operated on a 67-year-old woman for infective endocarditis and performed aortic valve replacement, but she died of terminal pancreatic cancer on the 26 postoperative day. In the other case, a 53-year-old woman underwent emergency operation for cardiac tumor of the left atrium. However, the tumor was diagnosed as cardiac sarcoma during the operation, and it could not be completely resected. The sarcoma recurred after 2 months and she passed away. An emergency cardiac operation without enough preoperative examinations sometimes causes a poor prognosis.

[Arterial switch operation for transposition of the great arteries with situs inversus and mirror image dextrocardia].

We report a successful arterial switch operation for complete transposition of great arteries with atrial and visceral situs inversus totalis and mirror image dextrocardia in a 12-day-old infant girl. The aorta was located left side-by-side to the pulmonary trunk with a single coronary artery (mirror image of 1RLCx). After French maneuver, the posterior circumference of the neo-aorta was reconstructed. Then the coronary button was transplanted into the neo-aorta with a trap door technique carefully avoiding any twist and over-stretch. The neo-pulmonary trunk was reconstructed with an autologous pericardial patch and sutured to the longitudinal incision made into the left central pulmonary artery. The baby was discharged from hospital and has been doing well without any morbidity relating myocardial ischemia.

Development of a simple device enabling percutaneous flow regulation for a small vascular graft for a Blalock­Taussig shunt capable of flow regulation: complete translation of an original article originally published in Pediatric Cardiology and Cardiac Surgery (154­159, 2016: vol. 32).

OBJECTIVES: The Blalock-Taussig shunt (BTS) operation is a cornerstone as initial palliative surgery for congenital heart disease with severely reduced pulmonary blood flow (PBF). The ideal PBF provided by BTS is crucial for an uneventful postoperative course, since excess PBF results in acute distress of the systemic circulation and insufficient PBF requires another BTS surgery. Therefore, the goal of this study was to develop a simple device to control the shunt graft flow percutaneously using a constrictor balloon connected to a subcutaneous port. METHODS: The device consists of a cylindrical balloon and an anti-bending structure extension connected to the balloon center. A PTFE vascular graft wrapped by the device was connected to a simulated closed circuit to measure the relationship between pressure and blood flow while changing the inner volume of the balloon. In a beagle model of replacement of the right carotid artery, blood flow velocity was measured in the carotid artery after saline injection into the balloon. The blood flow velocity before and after balloon inflation was compared immediately after implantation of the device and at 3 months after implantation. RESULTS: The device provided good flow control by inflating and deflating the balloon ex vivo and in vivo for up to 3 months in a canine model with a small graft wrapped with the device. CONCLUSIONS: The simple device developed in this study may enable regulation of PBF through a small vascular graft and help to prevent severe morbidity and mortality in the clinical setting of BTS.

Correction to: Development of a simple device enabling percutaneous flow regulation for a small vascular graft for a Blalock-Taussig shunt capable of flow regulation: complete translation of an original article originally published in Pediatric Cardiology and Cardiac Surgery (154-159, 2016: vol. 32).

In the original publication of the article, the title was incorrectly published.

[Successful administration of nifekalant hydrochloride for postoperative junctional ectopic tachycardia in congenital cardiac surgery].

Two episode of junctional ectopic tachycardia (JET) caused hemodynamic deterioration early after tetralogy of Fallot repair in an 8-month-old infant. Sinus rhythm resumed in each of the episodes immediately after intravenous administration of nifekalant hydrochloride (NIF), a newly developed Vaughan-Williams class III antiarrhythmic drug in Japan. Although QT interval was modestly prolonged with NIF, no life-threatening ventricular arrhythmia (i.e., torsades de pointes) occurred. NIF might be an effective alternative in the treatment of postoperative JET in congenital cardiac surgery.

Repair of persistent truncus arteriosus without a conduit: sleeve resection of the pulmonary trunk from the aorta and direct right ventricle-pulmonary artery anastomosis.

OBJECTIVE: Establishing a new continuity between the right ventricle and the pulmonary artery is the mainstay of repair for persistent truncus arteriosus. We used the Tran Viet-Neveux technique without a Lecomte maneuver to construct the connection without a conduit. Here, we retrospectively review the mid-term surgical results to examine the effectiveness of this approach. METHODS: A cylindrical segment incorporating both pulmonary artery branches was sleeve-resected from the truncal artery. The cylindrical segment was cut in the middle and two truncal arterial flaps were combined to form the posterior floor of the new pulmonary arterial trunk. The edge of the floor was attached directly to the superior margin of an oblique incision made in the left-anterior wall of the right ventricle. A polytetrafluoroethylene monocusp was attached to the lower half margin of the right ventricular incision. A large glutaraldehyde-treated pericardial patch was used to form the anterior hood of the new right ventricular outflow tract. Both great arteries were located in a normal spiral configuration. RESULTS: Ten babies (range: 3 days to 9 months of age) underwent this procedure. The Collett-Edwards classification of persistent truncus arteriosus was type I in five cases and type II in five others. There was one hospital death due to severe respiratory distress. During follow-up (36-60 months, median 54 months), only one re-operation was required to enlarge a left branch pulmonary artery stenosis. Follow-up echocardiography showed pulmonary regurgitation (mild two, moderate seven, and severe one) and mild flow acceleration in the left pulmonary artery branch and right ventricle-pulmonary artery connection in one case. CONCLUSION: This simple modification for surgical correction of persistent truncus arteriosus may be an effective alternative that overcomes conduit-related problems.

Minimizing cerebral embolism in resection of distal aortic arch aneurysm through a left thoracotomy.

BACKGROUND: In order to reduce the risk of cerebral embolism during aortic replacement through a left thoracotomy, we performed ascending or arch aortic cannulation (AAC) as well as early extracorporeal perfusion (EEP) under deep hypothermic circulatory arrest (DHCA). In this study we examined the effectiveness of these modifications in preventing cerebral embolism after distal arch replacement. METHODS: Between January 2006 and March 2010, 40 patients underwent distal arch replacement through a left thoracotomy, using 2 pieces of an artificial graft. In all patients, AAC, EEP, and the open technique for aortic anastomosis were performed under DHCA. The AAC resulted in the proximal aortic perfusion from the proximal site of the diseased aorta. The EEP was induced by aortic distal perfusion from the side branch of a distal graft. After completion of the proximal anastomosis under EEP and DHCA, anastomosis between the proximal and distal grafts was made during rewarming. Neurologic deficit in the brain and spinal cord, as well as early surgical results, were clinically evaluated. RESULTS: There was no permanent neurologic deficit after the surgery in the operative survivors. No patient had a stroke (0%). Temporary paraplegia and paraparesis occurred in 1 and 2 patients, respectively (7.7%); all 3 patients were able to walk prior to their discharge from hospital. Mortality in this series was 5.0% (2 of 40 patients); the cause of death was rupture of an esophageal ulcer and cardiogenic shock possibly due to myocardial infarction. CONCLUSIONS: The AAC and EEP, in addition to deep hypothermia and DHCA, minimized the risk of cerebral embolism after distal arch aortic replacement by the left lateral approach.

Oral sildenafil for persistent pulmonary hypertension early after congenital cardiac surgery in children.

OBJECTIVE: Sildenafil is a strong pulmonary vasodilator that increases the intracellular cyclic guanosine monophosphate concentration through inhibition of phosphodiesterase-5. We assessed the benefit of oral sildenafil for persistent pulmonary hypertension early after congenital cardiac surgery in paediatric patients. METHODS: Sildenafil was administered at a starting dose of 0.5 mg kg(-1) following admission to the intensive care unit. With careful monitoring of haemodynamics, the dose was increased stepwise by 0.5 mg kg(-1) every 4-6 h up to a maximum of 2 mg kg(-1). After successful weaning from a ventilator and from other vasodilators, sildenafil was gradually discontinued over the next 5-7 days. RESULTS: A retrospective review of medical records showed an age distribution of <1 month (n=26), > or = 1-<6 months (n=36), > or = 6-<12 months (n=19), 1-3 years (n=8), 4-9 years (n=9) and >10 years (n=2) at the time of surgery. The surgeries were performed for ventricular septal defect closure (n=17), arterial switch (n=30), truncus arteriosus repair (n=10), complete atrioventricular septal defect repair (n=12), total anomalous venous drainage repair (n=9), and other open-heart surgery (n=22). The aforementioned concomitant inhaled nitrous oxide treatment was performed in 66 patients. Pulmonary arterial pressure decreased in 28, was unchanged in five and elevated in one patient out of the total of 34 cases for which data from continuous pressure monitoring were available. Bosentan was added in three cases with persistent symptoms due to pulmonary hypertension despite sildenafil treatment. After sildenafil administration, modest oxygen desaturation occurred in seven cases, but no 'rebound' pulmonary hypertension occurred. There were no significant adverse events during sildenafil treatment. CONCLUSIONS: Our results suggest that oral sildenafil is a safe and effective alternate for persistent pulmonary hypertension following congenital heart surgery in children.

Successful surgical treatment for dilated cardiomyopathy with cardiac sarcoidosis.

We report a surgical case of severe left ventricular dysfunction due to cardiac sarcoidosis. A 45-year-old man who underwent pacemaker implantation for complete atrioventricular block was admitted to the hospital because of dyspnea due to congestive heart failure. Echocardiography revealed a left ventricular ejection fraction of 11%, with severe mitral insufficiency and thinning of the ventricular septum. He was successfully treated by anteroseptal ventricular exclusion, mitral and tricuspid annuloplasty, and bi-ventricular pacing. Postoperative pathologic study revealed noncaseating granulomas. The patient was referred to a cardiologist for further treatment with prednisone.