Wide QRS tachycardia associated with multiple accessory pathways in a patient with Wolff-Parkinson-White syndrome.

The electrocardiogram of a 14-year-old boy with recurrent palpitation showed a wide QRS regular tachycardia with a right bundle branch block and right-axis deviation of 226 beats per minute. Verapamil infusion terminated the tachycardia after a few minutes. Electrophysiological study revealed that this tachycardia was considered as a reentrant tachycardia associated with the anterograde left posterior accessory pathway (AP) and retrograde right septal AP. Radiofrequency application was performed and eliminated both APs, and there was no recurrence of wide QRS tachycardia. .

Expectations and limitations after bilateral pulmonary artery banding.

OBJECTIVES: Bilateral pulmonary artery banding (bil-PAB) has been developed as a part of a hybrid procedure for hypoplastic left heart syndrome (HLHS). We use this procedure for palliation of complex congenital heart disease, particularly in patients with arch anomaly. We reviewed our experience with bil-PAB. METHODS: Sixty-six consecutive cases between 2003 and 2014 were reviewed retrospectively. RESULTS: Fifty-one patients (77%) had single ventricle physiology (HLHS: 37, HLHS variant: 13, others: 1). Twelve patients had biventricular physiology [5 interrupted aortic arch with ventricular septal defect (VSD); 4 coarctation of the aorta with VSD; 2 truncus arteriosus; 1 other]. In 3 patients, it was difficult to determine if the physiology was single ventricle or biventricle due to borderline left ventricle size. Age and body weight at the time of operation were 4.3 ± 3.4 days and 2.7 ± 0.5 kg, respectively. Overall hospital mortality was 11% (7/66). Of the 42 patients from 2010, only 1 with multiple anomalies died between bil-PAB and the second-stage operation. Thirteen patients (20%) required reoperation of bil-PAB. The mean waiting time for the next operation was 44.3 ± 42.8 days. The body weight at the second-stage operation was 3.2 ± 0.8 kg. Seven (11%) patients developed patent ductus arteriosus obstruction during the waiting period. Twenty-four patients were 2.5 kg or less; 20 patients in this group progressed to the second-stage operation and of these 19 gained body weight. Forty-two patients were above 2.5 kg, but of these only 23 patients gained body weight. Ten patients underwent bil-PAB because of shock and 8 of these recovered from shock. In 2 borderline cases, the patients underwent the Norwood procedure and 1 patient had arch repair and VSD closure. CONCLUSIONS: Bil-PAB could be applied to a wide variety of complex diseases. Our mortality rates with bil-PAB improved significantly post-2010. Good indications for bil-PAB were shock and situations where it was unclear whether the physiology was single ventricle or biventricular. Body weight gain was difficult to predict, but patients weighing less than 2.5 kg could be expected to gain body weight after the procedure.

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.

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.

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.

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.

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.

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.

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.

Spontaneous disappearance of a saphenous vein graft pseudoaneurysm after coronary artery bypass grafting.

A saphenous vein graft pseudoaneurysm is a rare complication of coronary artery bypass grafting. Its natural course is largely unknown because there have been few observational studies of medically observed patients. We herein report a case of spontaneous regression of a saphenous vein graft pseudoaneurysm in the early postoperative period, which has never been described, to our knowledge, in the previous literature.

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.

Resuspension of the uninfarcted papillary muscle at the time of mitral valve replacement in a patient with post myocardial infarction papillary muscle rupture.

A 60-year-old woman was referred to the Department of Cardiovascular Surgery of Social Insurance Chukyo Hospital for the rupture of a postinfarction papillary muscle. The rupture was in the posterior part of the anterolateral papillary muscle, in which more than two-thirds of its posterior leaflet was prolapsed. Mortality from the surgical repair of a papillary muscle rupture is quite high. For this case, we resuspended the uninfarcted papillary muscle heads case to preserve mitral ventricular continuity because the mitral annulus was quite small and more than two-thirds of the posterior leaflet were detached from the papillary muscle. The post-operative course of the patient was uneventful. Resuspension of uninfarcted papillary muscle is a useful technique to repair a rupture in the papillary muscle.

Retrograde coronary sinus cardioplegia cannula placement under short-time circulatory arrest in surgery for a ruptured type A dissection with a previous coronary artery bypass.

A 79-year-old woman was referred to undergo surgery for a type A dissection. The patient had a history of previous coronary artery bypass. She was in shock and had a hematoma surrounding the ascending aorta and the heart. In this case, a coronary sinus cardioplegia cannula was placed under a short period of circulatory arrest via a small atriotomy, and the atriotomy was closed immediately to establish selective cerebral perfusion.

Annuloplasty ring removal from patients with hemolysis after mitral valve repair.

We present a case of severe hemolysis following a mitral valve repair, which was successfully treated by removing the annuloplasty ring. The etiology of the hemolysis appeared to be a small regurgitant jet at the level of the annuloplasty ring.

Intestinal pseudo-obstruction in a patient with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) associated with phenytoin therapy.

Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) is most commonly associated with a mitochondrial DNA A to G point mutation at nucleotide 3243 (A3243G) and individuals with the disorder present a wide range of multisystemic symptoms. Seizures in MELAS patients are often intractable and require multiple antiepileptic drugs. Here we report a MELAS patient who presented with acute intestinal pseudo-obstruction following the administration of phenytoin (PHT) as an antiepileptic treatment. She presented with the first stroke-like episode at the age of 6 years and mitochondrial DNA analysis revealed A3243G with 94% mutation load in skeletal muscle. Despite treatment with phenobarbital and clobazam at the age of 16 years, she developed status epilepticus which ceased following PHT infusion. Thereafter, she was started on PHT treatment. One month later, however, she was readmitted because of remarkable abdominal distention. Although abdominal CT showed acute ileus with hepatic portal venous gas mimicking surgical emergency, the abdominal distention gradually recovered over several days following the discontinuation of PHT. Our clinical observations suggest the possibility that intestinal pseudo-obstruction in this patient related to PHT therapy. Careful clinical observation including gastrointestinal symptoms is required in the management of epilepsy in MELAS patients.