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. We report a 2-day-old male neonate who was diagnosed with ornithine transcarbamylase deficiency presenting hyperammonemia and severe heart failure after total anomalous pulmonary venous connection repair.

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. METHODS: Seven patients who underwent an arterial switch operation and intraventricular rerouting as definitive surgery between 2007 and 2017 were investigated. Of these 7 patients, 3 had undergone previous pulmonary artery banding and aortic arch reconstruction. RESULTS: The median body weight at definitive surgery was 3.3 kg (range 2.9-8.3 kg). At definitive surgery, the arrest time for single-stage correction (162.3 ± 21.7 min) was significantly shorter than that of staged repair (206.3 ± 5.1 min, p = 0.020). There was no hospital or late death. One patient in both strategy groups underwent aortic reintervention 54 months and 7.1 months after the definitive operation. Neoaortic valve (perinatal pulmonary valve) diameter decreased significantly from the perinatal valve diameter following definitive surgery (median +4.94z and +2.12z, respectively, p = 0.016) but there was no significant difference in the neopulmonary valve (perinatal aortic valve) diameter. Both single-stage correction and staged repair patients showed a similar trend. At the last follow-up, no patient had greater than mild neoaortic or neopulmonary valve regurgitation. CONCLUSION: The surgical outcomes of both single-stage correction and staged correction for Taussig-Bing anomaly with aortic arch obstruction are excellent. Both strategies produce similar changes in the diameter and regurgitation grade of the neoaortic and neopulmonary valves.

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 3.5 kg (range 2.6-5.2). In total, 15 cases of aortic coarctation, 7 cases of hypoplastic left heart syndrome, and 5 cases of interrupted aortic arch were included. An extended aortic arch anastomosis maneuver was used in 7 cases and aortic arch reconstruction compensated with an autologous pericardium patch was used in 6 cases. A Norwood-type procedure was used in 11 cases. The median Aristotle comprehensive score was 13.9 (7.0-20.0). BHTAS cases were not inferior in postoperative CK-MB/CK ratio (12.4 ± 2.8 in BHTAS vs 13.9 ± 3.6 in CTAS, p = 0.09), and there were no instances of myocardial ischemia. Two late deaths occurred due to shunt obstruction (n = 1) and valve malfunction (n = 1). Freedom from aortic reintervention was not inferior to conventional thoracic aortic surgery. BHTAS cases exhibited shorter cardiac arrest time than that of conventional thoracic aortic surgery in similar surgical procedures [CoA/VSD cases: 48.0 ± 8.0 min in the BHTAS cases and 65.7 ± 10.8 min in the CTAS cases (p = 0.0122), simple CoA/re-CoA cases: 0 min in the BHTAS cases and 20.1 ± 3.6 min in the CTAS cases]. CONCLUSIONS: BHTAS reduced cardioplegic arrest time while maintaining postoperative CK-MB/CK ratio, mid-term death, and aortic reintervention rates.

[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 0.6% glutaraldehyde-fixed autologous pericardium. PATIENTS AND METHODS: The procedure was performed in 6 patients;4 with aortic coarctation and 2 with aortic interruption. Prognosis, aortic diameter, and the aortic shape measurement on 3-dimensional computed tomography( 3D-CT) were examined postoperatively. RESULTS: There were no early or late deaths. There was no case of recurrent aortic arch obstruction and/or bronchial compression, The postoperative A/T ratio was 0.48±0.04 on 3D-CT. CONCLUSIONS: Reconstruction of the aortic arch with a 0.6% glutaraldehyde-fixed autologous pericardium in an aortic coarctation and interruption showed excellent early and midterm outcomes. There were no complications such as bronchial compression or recoarctation. We believe that this technique leads to a higher success for reconstructing the arch to a near to normal morphology and avoiding the so-called Gothic arch, which has been reported to be associated with late complications of hypertension.

[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. At age of 7 month, TCPS operation was performed. Arterial oxygen saturation decreased to 70% at 8 months after TCPS operation. We performed total right heart bypass operation with connected hepatic vein to the azygous vein using a 8-mm ePTFE tube graft, combined with dilatable right pulmonary artery banding( PAB). With PAB on the right pulmonary artery adjacent to the Glenn anastomosis, arterial saturation was 80% and the patient was successfully weaned off cardiopulmonary bypass. At 3 months after TRHB operation, systemic saturation increased to 90%.The right pulmonary artery banding site was successfully dilated by balloon angioplasty.

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. Fine adjustment of the bilateral pulmonary artery banding sites using hemoclips was carried out to facilitate weaning from extracorporeal membrane oxygenation, and staged delayed sternal closure was achieved. Arch repair, ventricular septal defect closure, and tricuspid annuloplasty were undertaken when the patient was 4-months old (weight 1.8 kg). She was discharged at 11 months of age (3.0 kg).

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). Consequently, we preoperatively confirmed the intracardiac rerouting line and the appropriate right ventricle incision line. A Senning procedure, intracardiac rerouting, and subaortic stenosis resection were performed in a 2.6-year-old patient (weight, 10.6 kg). The three-dimensional cardiac replica contributed definitively to the anatomical correction.

[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 (10×7.5 mm) and small atrial septal defect( 3 mm). Two great arteries were side by side position. The posterior leaflet of the tricuspid valve was 10 mm displaced to the right ventricle and tricuspid valve regurgitation was severe. The patients was unsuitable for primary biventricular repair because the size of the mitral valve was considered to be small. First, bilateral pulmonary artery banding was performed after balloon atrio-septostomy in the neonatal period. Three months after the 1st operation, cardiac catheteraization revealed that the left and right heart structure was suitable for biventricular repair. At 4-month-old-age, aortic arch reconstruction, intra-cardiac rerouting and tricuspid valve repair were performed. Post operative tricuspid valve regurgitation was mild. The patient was discharged on the 12th postoperative day with good hemodynamic condition.

[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. An arterial cannula was inserted into the brachiocephalic artery and the cardiopulmonary bypass was started by bicaval cannulation. The aortic root cannula was inserted into the aortic root and T-connected with the side hole of the brachiocephalic arterial cannula. The 2 cases underwent surgery with selective cerebral and myocardial perfusion. The left subclavian artery was transected and the subclavian flap technique was used to enlarge the recoarctation of the thoracic aorta. Then, the distal end of the transected left subclavian artery was reconstructed by performing an end to side anastomosis to the left carotid artery. This procedure has several advantages;(1) minimal dissection of the descending aorta is required,(2) the use of prosthetic material is avoided, (3) it prevents ischemic arm complication, (4) additional skin incision is avoided.

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 3.2 kg, respectively. All infants caught up with their growth curves and had >3 years of estimated residual battery life. This two-stage strategy was successful in facilitating permanent pacemaker implantation in low-birth-weight babies. Placement of single-chamber pacemaker on the apex of the left ventricle appears to be associated with longer battery lifespan.

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. During follow-up, branch pulmonary artery and ascending aortic stenosis was observed. At 15 months, surgical angioplasty of the ascending aorta and bilateral branch pulmonary arteries was undertaken using the Lecompte maneuver.

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, 20.3 years ± 4.5 years). Forty-five patients underwent atriopulmonary connection, 11 underwent the Bjork procedure, and 23 underwent total cavopulmonary connection. RESULTS: Ten hospital deaths (HDs) and/or early Fontan takedowns (TDs) occurred. The median follow-up period was 18.2 years (range, 0.6-37.6 years). The estimated freedom from death or TD rates was 79.7% at 5 years, 77.0% at 10 years, 73.9% at 15 years, and 63.9% at 20 years. Age was not a predictor of HD and/or TD. Freedom from death or TD after 1998 was 69.1% at 5 years, 69.1% at 10 years, and 69.1% at 15 years, and before 1997 was 82.3% at 5 years, 79.0% at 10 years, 75.5% at 15 years, and 65.1% at 20 years; there were no significant differences between the two groups. In 19 late-death patients, nine (47.4%) experienced sudden death. Among these patients, five had known arrhythmias before sudden death. CONCLUSIONS: In patients who were ≥15 years old, the surgical results of the Fontan operation were acceptable. Approximately half of the late deaths were sudden deaths, mainly occurring 10-20 years postoperatively.

[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. Total correction was performed at the age of 18 days ( weight 2.2 kg). Intraoperatively, to confirm the position of the ventricular septum defect, we needed to incise from the main pulmonary artery to right ventricle through a commissure part of pulmonary valve. Although he exhibited stable hemodynamics, pulmonary valve regurgitation progressed. Six years after initial operation, we performed pulmonary valve plasty. A main pulmonary arteriotomy revealed that the right and anterior cusp were damaged in the initial operation. The right cusp had 2 defects near the commissure part, and the anterior cusp had a complete defect at the commissure part. The defects were filled with sufficient size of an autologous pericardial patch to preserve adequate coaptation zone. Echocardiography 1 year later revealed satisfactory result.

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. The patient was discharged without complications. Postoperative echocardiography showed no residual coarctation or pulmonary venous obstruction and revealed small muscular ventricular septal defects.

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, 2.4-3.7 kg). There were no operative complications of embolism or bleeding in our series. Our off-pump atrial septectomy technique is an effective approach in specific cases.

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). RESULTS: Second-stage operations were conventional total repair in five and Damus-Kaye-Stansel anastomosis, aortic arch reconstruction and right ventricle-pulmonary artery shunt (modified Norwood) type repair in five. After modified Norwood-type repair, four patients were Yasui-type repair candidates and one was a Fontan candidate. For all patients, the mean AoV diameter increased from 3.7 ± 0.7 mm before bPAB to 4.6 ± 0.8 mm before the second-stage operation. In five patients with CoA or IAA type A, the AoV diameter significantly increased from 3.5 ± 0.3 mm to 4.5 ± 0.5 mm during the term between bPAB and the second-stage operation, with an AoV Z-score increase from -5.82 ± 0.92 to -4.28 ± 0.86. IAA type B showed a slight increase in the AoV diameter. CONCLUSIONS: Initial palliation with bPAB enables AoV diameter growth in some patients, improving the likelihood of conventional total repair adaptation rate, particularly for CoA or IAA type A.