Acute onset of ornithine transcarbamylase deficiency after total anomalous pulmonary venous connection repair to a 2-day-old neonate.

Ornithine transcarbamylase deficiency is an X-linked disorder which results in the accumulation of ammonia causing irritability and vomiting. Acute hyperammonemia requires rapid and intensive intervention. However, as those clinical features are non-specific and commonly seen in peri-operative situation, ornithine transcarbamylase deficiency could be difficult to diagnose prior to and post-emergency cardiac surgery.

Arterial switch operation for Taussig-Bing anomaly with aortic arch obstruction.

Introduction: The short-term survival rate after single-stage correction of Taussig-Bing anomaly with aortic arch obstruction remains favorable. However, some cases are encountered occasionally in which single-stage correction was not performed during the neonatal period. Accordingly, we evaluated the midterm outcomes of different surgical strategies.

Beating heart thoracic aortic surgery under selective myocardial perfusion for patients with congenital aortic anomalies.

Objective: We evaluated beating heart thoracic aortic surgery (BHTAS) using selective myocardial perfusion (SMP) in patients with aortic anomalies with complex surgical needs.

Methods: Between 2012 and 2018, 27 infants with aortic anomalies underwent BHTAS using SMP.

Results: Median body weight was 3.

[Reconstruction of the Aortic Arch with an Autologous Pericardial Patch in Aortic Coarctation and Interruption].

Backgrounds: Direct anastomosis such as end-to-end anastomosis or extended aortic arch anastomosis are the most commonly used technique to repair aortic coarctation or interrupted aortic arch. Direct anastomosis of the aorta sometimes results in restenosis and/or bronchial compression. To avoid these complications, we have used the technique of the aortic reconstruction using an 0.

[A Total Right Heart Bypass Operation for Polysplenia with Unilateral Pulmonary Arteriovenous Fistula;the Usefulness of the Anastomosis of the Hepatic Vein to the Azygous Vein Combined with Dilatable Pulmonary Artery Banding].

Right pulmonary arteriovenous fistula(PAVF) developed in a 18-month-old girl after total cavopulmonary shunt(TCPS) operation. The patient was diagnosed as polysplenia, single atrium, single ventricle, dextrocardia, pulmonary atresia, congenitally complete atrioventricular block, and absent infrahepatic segment of the inferior vena cava with azygous continuation to the right superior vena cava. The hepatic veins were connected to the left-side atrium.

Extracorporeal membrane oxygenation in a low-weight infant after cardiac surgery.

A neonate was diagnosed with ventricular septal defect and aortic coarctation. Bilateral pulmonary artery banding was performed at 9 days of age (weight 1472 g). Because of ductal shock, emergency extracorporeal membrane oxygenation was initiated after surgery.

[Successful Staged Surgical Management for Double Outlet Right Ventricle with Ebstein's Anomaly and Aortic Coarctation;Report of a Case].

A combination of double outlet right ventricle(DORV) with Ebstein's anomaly and aortic coarctation (CoA) is vary rare. We report a successful staged surgical repair for DORV with Ebstein's anomaly associated with CoA. The patient had subaortic perimembronous ventricular septal defect (10×7.

[Subclavian Flap Angioplasty and the Reconstruction of the Distal Subclavian Artery for Recurrent Coarctation of the Thoracic Aorta through the Median Sternotomy].

The Procedure of the subclavian flap angioplasty and reimplantation of the distal subclavian artery into the left carotid artery was used in 2 infants( 4-month-old and 5-month-old) with recurrent coarctation of the thoracic aorta through the median sternotomy. After median sternotomy, the aortic arch and its branches were extensively dissected. The descending aorta was minimally dissected.

Anatomical correction of atrioventricular discordance using three-dimensional replica.

Surgical experience with {S,L,D} segmental anatomy of atrioventricular discordance with double-outlet right ventricle is extremely rare. In addition to ordinary cardiac examination, we reviewed electrophysiological studies and a three-dimensional cardiac replica (crossMedical, Inc., Kyoto, Japan).

Pacemaker therapy in low-birth-weight infants.

Infants born with complete atrioventricular block (CAVB) and fetal bradycardia are frequently born with low birth weight. Three low-birth-weight CAVB infants underwent temporary pacemaker implantation, followed by permanent single-chamber pacemaker implantation at median body weights of 1.7 and 3.

[Pulmonary Valve Plasty after Postoperative Pulmonary Insufficiency].

A 6-year-old boy (weight 15.5 kg) underwent pulmonary valve plasty using autologous pericardium. He received a diagnosis of aortic coarctation and ventricular septal defect.

Long-term follow-up of Fontan completion in adults and adolescents.

Background: The Fontan procedure is rarely performed in adults and adolescents in the present era. We review our results with the Fontan procedure in adolescents and young adults.

Methods: Between 1974 and 2010, 79 consecutive patients underwent the Fontan procedure at an age ≥ 15 years (mean age at Fontan operation, 20.

Off-Pump Atrial Septectomy for Infants With Restrictive Atrial Septal Defect.

The management of some subsets of patients with an unsuccessful balloon atrial septostomy (BAS) and poor general condition is typically challenging. Our novel technique involves off-pump atrial septectomy using laparoscopic forceps guided by intraoperative direct pericardial echocardiography. The technique was first used in a case of tricuspid atresia in a patient in preshock and was then used in 2 more patients with hypoplastic left heart syndrome (HLHS) (age, 10-116 days; body weight, 2.

Growing potential of small aortic valve with aortic coarctation or interrupted aortic arch after bilateral pulmonary artery banding.

Objectives: Bilateral pulmonary artery banding (bPAB) is utilized for some patients with a ventricular septal defect (VSD) and aortic coarctation (CoA) or interrupted aortic arch (IAA). We evaluated aortic valve (AoV) diameter and patient outcomes following bPAB.

Methods: Between August 2010 and September 2015, 10 consecutive patients with VSD and patent ductus arteriosus-dependent CoA or IAA underwent bPAB because of an AoV diameter of approximately <50% of the normal value (n = 6), severe subaortic stenosis and poor patient condition (n = 1, respectively), or low birthweight (n = 2).

Staged Delayed Sternal Closure Using a Binder Clip After Pediatric Cardiac Surgery.

After pediatric cardiac surgery, patients who undergo delayed sternal closure may become hemodynamically unstable. We performed a staged sternal closure technique using a binder clip in 31 consecutive patients to minimize cardiopulmonary instability. Only one patient (3.

Truncus arteriosus with interrupted aortic arch in very low birth weight infants.

A neonate, born after 29 weeks and 2 days gestation (weight 1015 g), was diagnosed with truncus arteriosus and interrupted aortic arch. On postnatal day 30 (1378 g), we performed bilateral pulmonary artery banding. On postnatal day 107 (3024 g), we carried out aortic arch repair and a Rastelli-type procedure.

Long-Term Follow-Up of the Conal Flap Method for Tricuspid Malinsertion in Transposition of the Great Arteries With Ventricular Septal Defect and Pulmonary Stenosis.

Background: Rastelli and réparation à l'étage ventriculaire (REV) procedures have been applied for biventricular repair of transposition of great arteries with ventricular septal defects and pulmonary stenosis (TGA III). In some patients with TGA III, whose tricuspid valve (TV) chordae tendineae are attached to the infundibular septum, abnormal TV chorda insertion may cause intracardiac rerouting obstruction. For such cases, we performed a conal flap" (CF) procedure involving mobilization of the infundibular septum including TV chordae as a pedicled flap.

Repair of Vascular Ring With Right-Sided Interrupted Aortic Arch and Right-Sided Descending Aorta.

A female neonate, born after a gestation of 38 weeks and 6 days, received a diagnosis of ductal shock at the age of 2 days, along with vascular ring with right-sided interrupted aortic arch, right-sided descending aorta, right-sided patent ductus arteriosus, severe valvular aortic stenosis, and ventricular septal defect. At 3 days of age, the infant underwent bilateral pulmonary artery banding. At the age of 3 months and 29 days, she underwent a Norwood-type operation and release of the vascular ring.

Total anomalous pulmonary venous connection with ventricular septal defects.

The association of total anomalous pulmonary venous connection with ventricular septal defect and aortic coarctation is rare. A premature neonate weighing 1350 g was diagnosed with infracardiac total anomalous pulmonary venous connection, aortic coarctation, and a perimembranous ventricular septal defect. On postnatal day 23, we performed emergency extended aortic arch anastomosis under total circulatory arrest, pulmonary venous connection repair using a primary sutureless technique, and patch closure of the atrial septal and perimembranous ventricular septal defects.

A Surgical Integration Technique for Right-Sided and Left-Sided Superior Venae Cavae.

The treatment of some subsets of patients having both right-sided superior vena cava (SVC) and left-sided SVC may be very challenging. We performed the SVC integration (SVCI) technique with end-to-side anastomoses between the two SVCs on 4 such patients (age, 5 to 11 months; body weight, 5.4 to 10.

Thoracoscopic Surgery for Partial Anomalous Pulmonary Venous Connection with Dual Drainage.

We report our technique for thoracoscopic surgery for a 15-year-old female (body weight, 59 kg) diagnosed with partial anomalous pulmonary venous connection with dual drainage. A large anomalous right lower pulmonary vein (RLPV) was drained into the inferior vena cava and left atrium, along with thoracoscopic ligation and clipping of RLPV and some anomalous hepatic veins.