Optimal timing of bridging annuloplasty and patch augmentation for heterotaxy syndrome associated with functional single ventricles.

OBJECTIVE: Although the challenging prognosis of functional single ventricles with common atrioventricular valves due to complex morphology and uncontrollable regurgitation by valvuloplasty has been highlighted, reports on when and how these extremely complicated atrioventricular valves should be repaired are few. This study investigated the timing and risk factors for valve intervention in these patients. METHODS: Between April 2006 and March 2023, 40 patients with heterotaxy syndrome associated with functional single ventricles underwent surgery. Valve intervention was performed in 14 of the 40 patients with moderate or severe atrioventricular valve regurgitation. RESULTS: The timing of the first valve intervention varied, with four, five, three, and two patients undergoing valve intervention before the cavopulmonary shunt, simultaneously with the cavopulmonary shunt, before total cavopulmonary connection, and simultaneously with total cavopulmonary connection, respectively. Mechanical valve replacements were performed in three patients. Among the 14 patients undergoing valve intervention, four died. Three of the four patients underwent valvuloplasty before the cavopulmonary shunt, including two who could undergo the cavopulmonary shunt but died after the procedure. Eight of the fourteen patients completed total cavopulmonary connection. The cumulative survival rate was not significantly different between the 14 patients who underwent and 26 who did not undergo intervention (hazard ratio, 2.52; 95% confidence interval, 0.56-11.24; P = 0.23). CONCLUSION: Our surgical strategies provide a chance for the next staged repair of common atrioventricular valves in patients with both heterotaxy and valvular regurgitation. Including patch augmentation, advanced valve intervention is possible at or after the cavopulmonary shunt.

Cholinergic interneurons in the dorsal striatum play an important role in the acquisition of duration memory.

The present study aimed to examine the effect of cholinergic interneuron lesions in the dorsal striatum on duration-memory formation. Cholinergic interneurons in the dorsal striatum may be involved in the formation of duration memory since they are among the main inputs to the dorsal striatal muscarinic acetylcholine-1 receptors, which play a role in the consolidation of duration memory. Rats were sufficiently trained using a peak-interval 20 s procedure and then infused with anti-choline acetyltransferase-saporin into the dorsal striatum to cause selective ablation of cholinergic interneurons. To make the rats acquire new duration-memories, we trained them with a peak interval 40 s after lesion. Before lesion, the peak times (an index of duration memory) for sham-lesioned and lesioned groups were similar at approximately 20 s. In the peak interval 40 s session, the peak times for the sham-lesioned and lesioned groups were approximately 30 and 20 s, respectively. After additional peak interval 40 s sessions, the peak times of both groups were shifted to approximately 40 s. Those results suggest that the cholinergic interneuron lesion delayed new duration-memory acquisition. Subsequent experiments showed that cholinergic interneuron lesions did not retard the shift of peak time to the original target time (20 s). Following experiment without changing the target time after lesion showed that cholinergic interneuron lesions did not change their peak times. Our findings suggest that cholinergic interneurons in the dorsal striatum are involved in new duration-memory acquisition but not in the utilization of already acquired duration memory and interval timing.

Comprehensive outcomes after primary arterial switch operation.

OBJECTIVE: To report the long-term outcomes of the arterial switch operation for transposition of the great arteries. METHODS: The data of 35 patients who underwent an arterial switch operation between April 2006 and May 2021 were retrospectively examined; five had double-outlet right ventricles, three had side-by-side transposition, and 27 had d-transposition. The punch-out technique was the basic method for coronary transplantation; however, if coronary artery kinking after additional peeling was a concern, the trap-door technique was used. In aortic reconstruction, the caliber difference between the neoascending aorta and the native pulmonary artery was fixed using the pulmonary artery flap. In pulmonary reconstruction, the pulmonary artery was bilaterally exposed over the second branch. RESULTS: The median age at operation and follow-up duration were 10.0 (8.0-12.5) days and 72.9 (40.7-139.5) months, respectively. Thirty-four patients survived, and one was lost to follow-up ([coronary transplantation]: right coronary artery, punch-out = 31, trap-door = 2; left coronary artery, punch-out = 28, trap-door = 7; [aortic regurgitation]: no-slight = 27, mild = 6, moderate = 1; [pulmonary regurgitation]: no-slight = 22, mild = 11, moderate = 1). Slight coronary bending occurred in two patients; ischemia was not observed. Catheter stent placement was performed in one of three patients with stenosis at the pulmonary artery bifurcation. Catheter balloon dilation was performed in one patient with supravalvular pulmonary stenosis. There were no cases of coronary, aortic, or pulmonary artery reoperations. The 15-year cumulative reintervention avoidance rate was 91.0%. CONCLUSIONS: There were no deaths or reoperations during the long-term follow-up period, and arterial switch operation outcomes at our hospital were satisfactory.

Intra-dorsal striatal acetylcholine M1 but not dopaminergic D1 or glutamatergic NMDA receptor antagonists inhibit consolidation of duration memory in interval timing.

The striatal beat frequency model assumes that striatal medium spiny neurons encode duration via synaptic plasticity. Muscarinic 1 (M1) cholinergic receptors as well as dopamine and glutamate receptors are important for neural plasticity in the dorsal striatum. Therefore, we investigated the effect of inhibiting these receptors on the formation of duration memory. After sufficient training in a peak interval (PI)-20-s procedure, rats were administered a single or mixed infusion of a selective antagonist for the dopamine D1 receptor (SCH23390, 0.5 µg per side), N-methyl-D-aspartic acid (NMDA)-type glutamate receptor (D-AP5, 3 µg), or M1 receptor (pirenzepine, 10 µg) bilaterally in the dorsal striatum, immediately before initiating a PI-40 s session (shift session). The next day, the rats were tested for new duration memory (40 s) in a session in which no lever presses were reinforced (test session). In the shift session, the performance was comparable irrespective of the drug injected. However, in the test session, the mean peak time (an index of duration memory) of the M1 + NMDA co-blockade group, but not of the D1 + NMDA co-blockade group, was lower than that of the control group (Experiments 1 and 2). In Experiment 3, the effect of the co-blockade of M1 and NMDA receptors was replicated. Moreover, sole blockade of M1 receptors induced the same effect as M1 and NMDA blockade. These results suggest that in the dorsal striatum, the M1 receptor, but not the D1 or NMDA receptors, is involved in the consolidation of duration memory.

Primary pulmonary artery reconstruction for functional single ventricle with absent central pulmonary artery and bilateral patent ductus arteriosus.

OBJECTIVE: This study aimed to evaluate and discuss the outcomes of creating a single systemic-pulmonary shunt and reconstruction of the pulmonary artery continuity in patients with a single functional ventricle, absent central pulmonary artery, and bilateral patent ductus arteriosus. METHODS: Six infants diagnosed with a functional single ventricle, absent central pulmonary artery, and bilateral patent ductus arteriosus were treated by creating a single systemic-pulmonary shunt and reconstructing the pulmonary artery continuity (primary operation) between January 2010 and September 2020. Pulmonary artery continuity was ensured using the remnant pulmonary artery and an autologous pericardial patch in five patients and a rolled autologous pericardium in one patient. RESULTS: All patients eventually underwent total cavopulmonary connection. Two patients underwent intrapulmonary artery septation before Glenn or total cavopulmonary connection procedure. The median follow-up period was 9.02 years (interquartile range, 3.90-9.53). No late deaths were observed. CONCLUSIONS: Our strategy of establishing a single systemic-pulmonary shunt with reconstruction of the pulmonary artery continuity was useful for treating patients with a functional single ventricle with absent central pulmonary artery and bilateral patent ductus arteriosus. This procedure helped accomplish pulmonary artery growth and ensured an appropriate volume load after total cavopulmonary connection.

Coil Embolization and Left Ventricular Assist Device After Arterial Switch Operation.

Aortopulmonary collateral arteries are frequently found in patients with transposition of the great arteries after catheterization. Although most of them are asymptomatic, it may cause heart failure or pulmonary hemorrhage after an arterial switch operation. Here we report a case of symptomatic aortopulmonary collateral arteries after arterial switch, which necessitated support with a centrifugal pump and left ventricular assistive device because of severe mitral regurgitation and left atrial hypertension and emergent coil embolization. Hemodynamics dramatically improved after embolization. The postoperative period was uneventful, and the infant was released from the hospital on the 14th day.

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.

[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.