Hironori Matsuhisa

Sutureless pericardial repair of total anomalous pulmonary venous connection in patients with right atrial isomerism.

Surgical repair of total anomalous pulmonary venous connection (TAPVC) in patients with right atrial isomerism is associated with a significant risk of recurrent pulmonary venous obstruction (PVO). We evaluate the effect of sutureless repair to reduce the risk of recurrent PVO. Since November 2007, five patients, including three neonates, with right atrial isomerism underwent sutureless repair of TAPVC. The sutureless repair was used in three neonates as an initial procedure and in two infants as a procedure for postrepair PVO. Under deep hypothermic circulatory arrest or low flow cardiopulmonary bypass, pulmonary vein (PV) was incised as long as possible. The atrial wall was partially resected and anastomosed to the pericardial wall around the incised PV. There were no early deaths. No patients showed recurrence of PVO. There was one late death. Two patients underwent a bidirectional Glenn shunt after the sutureless repair. The pulmonary venous confluence was confirmed to be left open at the time of the Glenn surgery. The sutureless technique may be useful not only for postrepair PVO but also for non-operated TAPVC in neonates with right atrial isomerism.

Pediatric Cardiothoracic Surgery in Patients With Unilateral Pulmonary Agenesis or Aplasia

BACKGROUND Unilateral pulmonary agenesis or aplasia (UPA), a rare developmental defect of the lung, is sometimes associated with congenital heart and tracheal diseases. The purpose of this study was to assess our experience of pediatric cardiothoracic surgery in UPA patients. METHODS Cardiothoracic surgery for congenital heart defect or tracheal stenosis performed between 1981 and 2010 in 8 UPA patients (agenesis in 5 and aplasia in 3) was reviewed retrospectively. Associated cardiac anomalies included ventricular septal defect, double outlet right ventricle with pulmonary atresia, total anomalous pulmonary venous connection, and interrupted aortic arch complex. RESULTS For 7 patients with right UPA and 1 patient with left UPA, 12 cardiothoracic operations were performed, including 8 cardiac procedures in 4 patients and 4 tracheal procedures in 4 patients. Cardiac palliative repairs included Blalock-Park anastomosis, systemic-to-pulmonary artery shunt, and pulmonary artery banding. Cardiac definitive repairs included ventricular septal defect closure, subaortic membrane resection, modified Konno procedure, total anomalous pulmonary venous connection repair, and Rastelli-type operation. Tracheal repairs were costal cartilage tracheoplasty and slide tracheoplasty. The median age at surgery was 8 months and median body weight was 6.2 kg; the median operative time was 6.5 hours. There were 3 hospital deaths and 1 late death, with the 1-year mortality rate of 25%. Cardiopulmonary bypass-induced acute lung injury has occurred in 3 cases, 2 of which required extracorporeal membrane oxygenation support. Younger age of less than 1 month and prolonged cardiopulmonary bypass time of more than 200 minutes were related to operative risk factors for hospital mortality and morbidity. CONCLUSIONS Most of the pediatric cardiothoracic operations in UPA patients were successfully performed through an optimal surgical approach and procedure, but they still presented surgical risks of high mortality and morbidity. Perioperative management of UPA patients should be provided with a precise understanding of anatomic configuration and a careful consideration of underlying risk factors.

Intraoperative tranexamic acid in pediatric bloodless cardiac surgery.

Objective The aim of this study was to evaluate the clinical effects of intraoperative tranexamic acid administration in cardiac surgery without blood transfusion (bloodless cardiac surgery) in children. Methods Seventy-one consecutive patients weighing less than 20 kg, who underwent bloodless cardiac surgery for simple atrial or ventricular septal defects at Kobe Children’s Hospital from January 2011 to June 2013, were enrolled in this retrospective study. Tranexamic acid was administered during surgery from January 2012 (TXA group; n = 31), whereas it was not administered before January 2012 (control group; n = 40). Perioperative variables were compared between the TXA and control groups. Results There were no significant differences in patient characteristics or preoperative data between the 2 groups. Serial changes in perioperative hemoglobin and hematocrit levels, mixed venous oxygen saturation, and regional cerebral oxygenation during cardiopulmonary bypass were significantly higher in the TXA group compared to the control group. There were significant reductions in operative time, dopamine dose, peak serum lactate level, intubation time, chest tube drainage and duration, and hospital stay in the TXA group. Conclusions Intraoperative tranexamic acid administration was effective for blood conservation, and improved postoperative clinical outcomes in pediatric bloodless cardiac surgery.

Landiolol for junctional ectopic tachycardia refractory to amiodarone after pediatric cardiac surgery

Postoperative junctional ectopic tachycardia (JET) in children undergoing cardiac surgery is a serious arrhythmia that is associated with considerable morbidity and mortality. We present here a case of successful landiolol therapy for postoperative JET in a 3-month-old infant who underwent ventricular septal defect closure and right pulmonary artery plasty. His left ventricular function was poor postoperatively. The JET was refractory to amiodarone and caused severe hypotension, which was required cardiac massage. Continuous intravenous infusion of low-dose landiolol reduced the persistent JET rate immediately, and restored to sinus rhythm with stable hemodynamics.

Dexmedetomidine in combination with midazolam after pediatric cardiac surgery

Objective Although midazolam is one of the most commonly used sedatives for infants in the intensive care unit, it has well-known disadvantages including a dose-dependent potential to induce tolerance, withdrawal, and hemodynamic depression. The aim of this study was to evaluate the clinical effects of dexmedetomidine combined with midazolam in postoperative intensive care following pediatric cardiac surgery. Methods Forty consecutive infants who underwent cardiac surgery for isolated ventricular septal defects from January 2011 to July 2013 were enrolled in this retrospective study. They were divided into two groups according to postoperative sedation regimen: dexmedetomidine sedation with midazolam (n = 20), or midazolam sedation without dexmedetomidine (control group, n = 20). Perioperative variables were compared between the two groups. Results There were no significant differences in patient characteristics between the two groups. During the first 24 h after intensive care unit admission, heart rate and serum lactate levels were significantly lower in the dexmedetomidine group compared to the control group (p = 0.0292 and p = 0.0027, respectively). The maximal midazolam dose was also significantly lower in the dexmedetomidine group (0.12 ± 0.09 vs. 0.20 ± 0.08 mg kg−1 h−1, p = 0.0059). There were no adverse effects of dexmedetomidine such as bradycardia, hypotension, agitation, or seizures. Three (15%) patients in the control group and none in the dexmedetomidine group experienced sudden cardiopulmonary decompensation. Conclusions Dexmedetomidine can provide favorable sedative properties with a reduced requirement for concomitant midazolam and stable hemodynamics with tachycardia prevention, for postoperative intensive care following pediatric cardiac surgery.

Primary Sutureless Repair and Anterior Translocation of the Atrial Septum for Cardiac Total Anomalous Pulmonary Venous Connection

The incidence of pulmonary vein stenosis after correction of a cardiac total anomalous pulmonary venous connection in neonates may be underestimated because of the small population of patients; however, it is associated with significant mortality and morbidity. This report describes a primary sutureless repair and anterior translocation of the atrial septum to create a large left atrial cavity and avoid post-repair pulmonary vein stenosis.

Paediatric cardiac surgery in a patient with cold agglutinins

Cold agglutinins (CAs) lead to organ thrombosis or haemolysis due to increased blood viscosity and red blood cell clumping when blood temperature drops below the thermal amplitude for haemagglutination. Although it is well known that CAs are particularly relevant to adult cardiac surgery with hypothermic cardiopulmonary bypass (CPB), paediatric cardiac surgery with congenital heart disease and with CAs has been reported very rarely. We present here a case of paediatric cardiac surgery to repair atrial septal defect with pulmonary stenosis in an 11-month old infant with a family history of CAs. She was detected to have a high titre of CAs preoperatively, and underwent an intracardiac repair with normothermic CPB using temporary electrical fibrillation for added safety. Her post-operative course was uneventful without any complications.

Innominate artery compression of the trachea in patients with neurological or neuromuscular disorders

OBJECTIVES The purpose of this study was to review and assess our surgical management of innominate artery compression of the trachea (IACT) in patients with neurological or neuromuscular disorders (NMDs). METHODS Thirty patients with NMD who underwent surgical treatment for IACT at Kobe Childrens Hospital and Kobe University Hospital from 2002 to 2012, were enrolled in this retrospective study. The clinical outcomes of preventive elective surgery for IACT (Group A, n=20) were assessed and compared with those of emergent surgery (Group B, n=10). RESULTS A total of 27 patients underwent innominate artery transection (17 in Group A and 10 in Group B), and 3 patients in Group A underwent innominate artery reimplantation using prosthetic graft interposition. No operative or early death occurred. There were no cases of postoperative mediastinitis or neurological complications. The operative benefits in Group A included a smaller skin incision, more limited sternotomy, less blood loss, shorter operative time and shorter hospital stay, compared with Group B. No blood transfusion was required in Group A. The number of patients in whom cerebral circulation was assessed before surgery in Group A was significantly higher than those in Group B. CONCLUSIONS Preventive elective surgery for IACT provides many advantages, including minimally invasive procedures and successful postoperative outcomes without neurological complications in patients with NMD. Because this surgical management can prevent the tragic occurrence of a tracheo-innominate artery fistula or an exacerbation of tracheomalacia, it would be an optimal surgical treatment for IACT to improve the quality of life in patients with NMD.

Repair of unbalanced atrioventricular septal defect with small right ventricle

A 2-month-old boy with trisomy 21, a small right ventricle, and an unbalanced atrioventricular septal defect underwent successful biventricular repair without atrial fenestration. We decided to perform leftward partitioning of the atrioventricular valve to widen the right ventricle inflow tract. This procedure increases the size of the right-sided valve and allows full function of the right ventricle without an atrial communication. This technique should be employed in patients with a small right ventricle and an unbalanced atrioventricular septal defect, as an alternative to atrial septal fenestration or univentricular palliation.

Staged Biventricular Repair for Absent Aortic Valve in a Neonate

Congenitally absent aortic valve is an extremely rare and fatal cardiac malformation. We report the case of a neonate with absent aortic valve, an interrupted aortic arch, and a normal-sized left ventricle. At age 9 hours, emergency aortic valve closure and a Norwood procedure were performed to maintain coronary circulation. The patients postoperative course was complicated because of tracheomalacia and a severely dilated aorta that were treated with reduction aortoplasty at age 4 months. Finally, a staged Yasui procedure was performed at 26 months. To our knowledge, this is the first report of a successful biventricular repair for absent aortic valve.