Tomomi Hasegawa

Vascular endothelial growth factor in children with congenital heart disease

BACKGROUND Children with cyanotic congenital heart disease may experience the development of abnormal vessels that become a source of significant morbidity. Abnormal vessel proliferation in these children may take several forms, including systemic-to-pulmonary collateral arteries, systemic-to-pulmonary venous collaterals, systemic venous collateral channels after bidirectional cavopulmonary anastomosis, and pulmonary arteriovenous malformations. However, no entity responsible for these abnormalities has been identified yet. This study determined whether children with cyanotic congenital heart disease have elevated serum levels of vascular endothelial growth factor (VEGF) and whether elevated VEGF correlated with these abnormal vessels. METHODS Mean systemic room air oxygen saturation (SpO2), blood cell counts (RBC), and serum VEGF levels were measured preoperatively. Samples were obtained from 61 children with acyanotic heart disease (group N) and 102 children with cyanotic heart disease (group C) before cardiac surgery. Postoperative catheterization was performed 1-month after the operation to evaluate the abnormal vessels in group C. RESULTS The VEGF level was significantly elevated in group C (355.0 +/- 287.1 pg/mL) compared with group N (203.0 +/- 221.6 pg/mL; p < 0.001). VEGF levels in patients with a single ventricle associated with asplenia syndrome (n = 7) in group C were significantly elevated (711.9 +/- 443.5 pg/mL) compared with other patients. There was no significant correlation between VEGF level and SpO2 or RBC. Abnormal vessels were diagnosed in 19.6% (20/102) patients in group C. There was no difference in VEGF levels between the patients with abnormal vessels (336.8 +/- 182.5 pg/mL) and the patients without abnormal vessels (359.1 +/- 306.8 pg/mL). CONCLUSIONS Children with cyanotic heart disease have elevated systemic levels of VEGF, especially in those patients with a single ventricle associated with asplenia syndrome. There was no significant relationship in VEGF levels between the patients with abnormal vessels and without these vessels.

Simultaneous repair of pectus excavatum and congenital heart disease over the past 30 years

OBJECTIVE Pectus excavatum may be present in patient requiring operations for cardiac defects. The study was undertaken in order to assess our simultaneous repairs of pectus excavatum and congenital heart disease over past 30 years. METHODS Between 1970 and 2000, 12 patients underwent simultaneous repair of pectus excavatum and congenital heart disease. Six of 12 patients had ventricular septal defects as cardiac malformations (subgroup A). Operative technique, after the intracardiac procedure using cardiopulmonary bypass, consists of total subperichondrial resection of deformed costal cartilages, transection of deformed portion of the sternum in 2-3 points, and fixation of the sternum in elevated position using two Kirschner wires and a bridge external traction. Postoperative catheterization was performed in five of 12 patients (subgroup B). We evaluated the operative data, the improvement of pectus deformity and right ventricular performance retrospectively. The operative data in subgroup A were compared with those in recent random patients with ventricular septal defects only or with pectus excavatum only (control groups). RESULTS There was no operative death and non-serious complications were seen in nine patients (atelectasis in six, superficial wound infection in two, chylothorax in one). Pectus deformities improved with the drop of vertebral index postoperatively. The mean total operative time and postoperative drainage in subgroup A were 128.4% and 123.7%, respectively of those in the ventricular septal defect control group. The mean perioperative bleeding in subgroup A was more than the sum of those in control groups. Right ventricular end-diastolic (RVEDVI), end-systolic (RVESVI), stroke (RVSVI) volume indices and ejection fraction (RVEF) in subgroup B tended to increase after surgery. In particular, there were significant increases of RVEDVI (35%, P<0.05) and RVSVI (77%, P<0.01). CONCLUSION Simultaneous cardiac and pectus repairs were performed successfully without serious complications. Moreover, simultaneous repair resulted in an improvement of right ventricular performance with significant increases of RVEDVI and RVSVI.

Secretion of A-type and B-type natriuretic peptides into the bloodstream and pericardial space in children with congenital heart disease

OBJECTIVE To determine the secretion of A-type and B-type natriuretic peptides into the bloodstream and pericardial space in children with congenital heart disease. METHODS Plasma and pericardial fluid samples were obtained from 77 patients undergoing total correction for congenital heart disease. All patients underwent detailed right-sided and left-sided cardiac catheterization preoperatively. RESULTS A-type natriuretic peptide levels in pericardial fluid were lower than those in plasma (33.0 +/- 23.1 versus 39.8 +/- 33.6 pg/mL, P <.05), and B-type natriuretic peptide levels in pericardial fluid showed marked elevations compared with those in plasma (231.9 +/- 305.6 versus 19.8 +/- 29.3 pg/mL, P <.0001). The A-type and B-type natriuretic peptide levels in plasma correlated with those in pericardial fluid (R =.522, P <.0001; R =.595, P <.0001). For A-type and B-type natriuretic peptide levels in plasma, the relation with biventricular volume had the highest correlation (R =.669, P <.0001; R =.652, P <.0001). The patients with a pulmonary-to-systemic flow ratio greater than 2 (n = 19) had high levels of natriuretic peptides not only in plasma (58.3 +/- 43.2, 40.5 +/- 49.4 pg/mL, P <.05) but also in pericardial fluid (44.4 +/- 31.5, 287.2 +/- 198.5 pg/mL, P <.05), and higher correlation between A-type and B-type natriuretic peptide plasma levels and left ventricular volume (R =.913, P <.0001; R =.787, P <.0001). CONCLUSIONS B-type natriuretic peptide is secreted not only into the bloodstream but also into the pericardial space in children with congenital heart disease. Natriuretic peptide levels in plasma correlated well with biventricular volume. The left ventricle was considered to be the main source of secreted natriuretic peptides in the patients with a pulmonary-to-systemic flow ratio greater than 2.

Tricuspid Valve Repair with Papillary Muscle Shortening for Severe Tricuspid Regurgitation in Children

We describe tricuspid valve repair using papillary muscle shortening for severe tricuspid regurgitation due to leaflet prolapse in children combined with De Vega annuloplasty. The papillary muscle was shortened until the prolapsed leaflet was at the same height as the other nonprolapsed leaflets. Although echocardiographic tricuspid regurgitation tends to increase over time, it rarely requires long-term intervention.

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.