Median sternotomy approach for the repair of esophageal atresia: a case report.

BACKGROUND: Repair of esophageal atresia is usually performed through the right thoracic cavity. However, when the upper pouch of the esophagus and tracheoesophageal fistula (TEF) is located in the thoracic inlet and completely on the left side of trachea, it is difficult to dissect and anastomose the esophagus through the right thoracic cavity. We present a case of esophageal atresia, with the esophageal upper pouch located high and completely on the left side of trachea, successfully repaired via the median sternotomy approach. CASE PRESENTATION: A male neonate with a birth weight of 1766 g was prematurely delivered via cesarean section at 34 weeks of gestation. Contrast-enhanced computed tomography (CT) showed that the upper pouch of the esophagus was located at the thoracic inlet and completely on the left side of the trachea; hence, a diagnosis of esophageal atresia was made. Moreover, a TEF was connected to the trachea at the level of the lower end of the upper esophageal pouch. An aberrant right subclavian artery and persistent left superior vena cava were also detected. Esophageal dissection and anastomosis were determined to be very difficult if approached from the right thoracic cavity. Therefore, we performed median sternotomy one day after the neonate was born. The upper pouch of the esophagus and TEF were easily dissected via the median sternotomy approach. Anastomosis of the esophagus was performed, with a good visual field, to the left of the trachea. The postoperative course was uneventful. CONCLUSIONS: This is the first reported case of a median sternotomy approach for esophageal atresia. This technique may be useful when a right thoracic approach is difficult, especially if the esophageal upper pouch is located completely to the left of the trachea or if it is higher than the normal position.

Clinical assessment of diastolic retrograde flow in the descending aorta for high-flow systemic-to-pulmonary artery shunting.

OBJECTIVE: To investigate whether echocardiographic characteristics in the descending aorta of patients with cyanotic congenital heart disease who have received a systemic-to-pulmonary artery (SP) shunt can indicate shunt flow volume and predict postoperative adverse events related to high-flow shunting. METHODS: Among the 73 consecutive patients who received an SP shunt between 2010 and 2014, data for 53 patients who underwent postoperative Doppler echocardiographic assessment of diastolic retrograde flow in the descending aorta (dAo-RF) were reviewed retrospectively. RESULTS: The mean dAo-RF ratio was 0.50 ± 0.15 at intensive care unit admission and reached its peak level (0.56 ± 0.12) at 24 hours after surgery. All of the patients with a maximal dAo-RF ratio of ≥0.80 had experienced acute heart failure or cardiogenic shock due to postoperative high-flow shunting and required emergent surgical interventions to reduce pulmonary blood flow. Pulse oximetry-measured oxygen saturation and serum lactate level were significantly correlated with dAo-RF ratio, but they had some clinical dispersion to match the postoperative adverse events. CONCLUSIONS: The dAo-RF ratio is a simple, repeatable, and noninvasive index for postoperative assessment of SP shunt flow volume. A high dAo-RF ratio is a significant predictor of postoperative adverse events of high-flow shunting.

Left Subclavian Artery Perfusion During the Norwood Operation for Interrupted Aortic Arch With Aortic Atresia.

The combination of aortic atresia and interrupted aortic arch is a rare condition. We describe a case of Norwood operation for this defect, in which a collateral artery from the descending aorta supplied coronary perfusion. The devised cardiopulmonary bypass technique is discussed, in which the left subclavian artery perfusion was utilized in addition to the cannulation to the descending aorta to secure the cerebral perfusion.

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.

Aortic arch geometry after the Norwood procedure: The value of arch angle augmentation.

OBJECTIVE: Aortic arch reconstruction in the Norwood procedure is occasionally associated with postoperative airway and branch pulmonary artery stenosis, as well as recoarctation. This study investigated geometric changes in the aortic arch after the Norwood procedure and evaluated the efficacy of arch angle augmentation with glutaraldehyde-treated autologous pericardium in aortic arch reconstruction. METHODS: Of 52 consecutive patients who underwent the Norwood procedure between 1998 and 2013, a total of 36 patients who underwent postoperative multidetector-row computed tomographic angiography were reviewed retrospectively, and divided into 2 groups according to whether arch angle augmentation with glutaraldehyde-treated autologous pericardium was performed (AAA group, 26 patients) or not (NA group, 10 patients). RESULTS: The neoaortic arch in the Norwood procedure was characterized by having a significantly smaller angle, being lower in height, and being narrower than that in age-matched control patients with a normal aortic arch. Moreover, all of these arch geometric measurements were significantly larger in the AAA group than in the NA group. Freedom from reintervention and reoperation for bronchomalacia or branch pulmonary artery stenosis, caused by extrinsic compression of the neoaorta, was significantly better at 1 year in the AAA group than in the NA group. Recoarctation of the neoaorta was documented in only 1 (3.8%) patient in the AAA group versus 3 patients (30%) in the NA group. CONCLUSIONS: Arch angle augmentation with glutaraldehyde-treated autologous pericardium in the Norwood procedure creates more aortopulmonary space with a smoother arch angle and diminished incidence of postoperative recoarctation, bronchial compression, or branch pulmonary artery compression.

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.

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 patient's 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.

A left anterior extrapleural approach to adjust right ventricle-pulmonary artery shunt flow using hemostatic clips after the Norwood operation.

Adjusting right ventricle-pulmonary artery shunt flow with placement of hemostatic clips in the Norwood operation is a useful technique for precise control of pulmonary blood flow in the acute postoperative period. This report describes our technique for optimizing right ventricle-pulmonary artery shunt flow with metal clips through a left anterior extrapleural approach, which can be performed safely and minimally invasively without sternal reopening. This procedure may decrease morbidity and contribute to long-term clinical improvement in patients undergoing the Norwood operation.

Beating-heart surgery for hypoplastic left heart syndrome with coronary artery fistulas.

The presence of ventriculocoronary connections in patients with hypoplastic left heart syndrome and mitral stenosis-aortic atresia is a suggested risk factor for myocardial ischemia after surgical palliation. We describe a neonate with this anatomic variant of hypoplastic left heart syndrome who underwent a successful beating-heart Norwood operation, with continuous coronary perfusion. The ventricular condition could be visually confirmed during the procedure, and a postoperative echocardiogram showed preserved ventricular function. This technique is considered an effective option for minimizing myocardial damage in this patient subtype.

Pacing therapy in children.

BACKGROUND: The importance of ventricular pacing site in pediatric pacemaker therapy has gradually become recognized. We reviewed our experience with a left ventricular (LV)-prioritized pacing strategy. METHODS AND RESULTS: Between 2000 and 2012, 60 patients underwent 76 permanent pacemaker implantations. Eight of the 29 reoperations involved ventricular lead repositioning for pacing-induced ventricular dysfunction. Freedom from ventricular lead failure was 96.3%, 86.8%, and 81.0% at 1, 3, and 5 years, respectively. The independent predictors of ventricular lead failure were age (P=0.026) and peak minimal energy threshold within 6 months (P=0.035). At the measured points, redo bipolar, steroid-eluting leads had significantly better pacing properties than did redo non-steroid-eluting, screw-in leads (P=0.0009-0.03). Ventricular lead repositioning was effective in the 5 patients with systemic LV pacing, whereas its efficacy was inconsistent in patients with single-ventricle or systemic right ventricular (RV) pacing. At a median follow-up of 59 months, the 28 patients with LV pacing had preserved ventricular function (LV fraction shortening, 0.34±0.09). CONCLUSIONS: The outcome of this LV-prioritized pacing strategy in pediatric patients was excellent, demonstrating preserved ventricular function. Bipolar, steroid-eluting, epicardial pacing leads achieved good pacing properties, even in reoperation patients. In children with systemic LV and RV pacing-induced ventricular dysfunction, a conversion to LV apex pacing was an attractive alternative to cardiac resynchronization therapy.

Mid-term results of mitral valve repair with autologous pericardium in pediatric patients.

BACKGROUND AND AIM OF THE STUDY: Mitral valve repair to treat mitral regurgitation (MR) in pediatric patients remains challenging because of the complex morphology and fragility of the leaflets. The study aim was to review retrospectively the authors' experience with mitral valve repair using autologous pericardium. METHODS: Between April 2004 and November 2011, nine pediatric patients (six males, three females; mean age 2.4 +/- 4.5 years) underwent mitral valve repair with an autologous fresh (n = 5) or glutaraldehyde-treated pericardium (n = 4) to treat severe MR. The etiology of the MR was acute endocarditis, acute chordal rupture and congenital disease in three, two, and four patients, respectively. Autologous pericardium was used for valvuloplasty, leaflet extension plasty and commissuroplasty in two, five, and two patients, respectively. Artificial chordal replacement was performed in three patients. RESULTS: No operative deaths or postoperative endocarditis occurred. One patient required mitral valve replacement at three days after the initial mitral repair. The most recent echocardiography findings of the remaining patients after a mean of 6.3 years (range: 1.3-9.0 years) showed that the severity of mitral insufficiency, left ventricular diastolic diameter and fractional shortening were 1.8 +/- 0.6 (grades 0-4), 40.4 +/- 8.4 mm (114.2 +/- 15.8% of normal) and 35.0 +/- 5.0%, respectively. CONCLUSION: Mitral valve repair using autologous fresh pericardium is associated with mid-term durability and resistance to infection when used for mitral valve repair of active endocarditis in pediatric patients. Augmentation with autologous pericardium could become an alternative to current surgical options.

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.

Mitral commissural repair with autologous fresh pericardium in an infant.

We describe the successful mitral valve repair with autologous fresh pericardium in a 5-month-old infant with acute progressive mitral regurgitation. The intraoperative findings consisted of fragile mitral valve leaflets with multiple chordal rupture of both the anterior and posterior leaflets. The disrupted anterolateral commissure was reconstructed using autologous fresh pericardium, a technique not previously reported in an infant of this size. Follow-up echocardiography for up to 7 years showed only trivial mitral regurgitation and no mitral stenosis.

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.

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 Children's 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.

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.

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.

Primary sutureless repair for simple total anomalous pulmonary venous connection with a risk of postoperative pulmonary venous stenosis.

Late mortality following surgical repair of total anomalous pulmonary venous connection (TAPVC) is often associated with pulmonary venous stenosis. We describe here two successful cases of primary sutureless repair for simple TAPVC in patients who had a potential risk of postoperative pulmonary venous stenosis. A 10-day-old neonate with mixed-type TAPVC and a 30-day-old infant with supracardiac TAPVC underwent primary sutureless repair with our modification. In the early follow-up, both patients are now doing well and have no signs of pulmonary venous stenosis. The sutureless repair can be applied as a primary surgical option to prevent postoperative pulmonary venous stenosis in selective patients with simple TAPVC.

Compression of an anomalous single coronary artery from pulmonary artery by banding.

This report describes a case with double outlet right ventricle and doubly committed ventricular septal defect associated with congenital diaphragmatic hernia. The patient underwent pulmonary artery banding and clipping of patent ductus arteriosus after repair of the diaphragmatic hernia. At 6 months, cardiac catheterization revealed anomalous origin of a single coronary artery from the pulmonary artery of the proximal banding. Although ischaemic symptoms had not been observed, the banding had resulted in compression of the coronary ostium. An emergency Rastelli procedure with Damus-Kaye-Stansel anastomosis was successfully performed.

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.