Biventricular function in preterm infants with patent ductus arteriosus ligation: A three-dimensional echocardiographic study.

BACKGROUND: The detailed hemodynamics after patent ductus arteriosus (PDA) ligation in preterm infants remain unknown. We aimed to clarify the effect of surgical ligation on left ventricular (LV) and right ventricular (RV) volume and function. METHODS: Echocardiography was performed in 41 preterm infants (median gestational age: 25 weeks) before and after PDA ligation. Global longitudinal strain was determined using three-dimensional speckle-tracking echocardiography. These values were compared with those in 36 preterm infants without PDA (non-PDA). RESULTS: Preoperatively, the PDA group had greater end-diastolic volume (EDV) and cardiac output (CO) in both ventricles, a higher LV ejection fraction (LVEF) (53% vs 44%) and LV global longitudinal strain, and a lower RVEF (47% vs 52%) than the non-PDA group. At 4-8 h postoperatively, the two groups had a similar LVEDV and RVEDV. However, the PDA group had a lower EF and CO in both ventricles than the non-PDA group. At 24-48 h postoperatively, the RVEF was increased, but the LVEF remained decreased, and LVCO was increased. CONCLUSIONS: PDA induces biventricular loading and functional abnormalities in preterm infants, and they dramatically change after surgery. Three-dimensional echocardiography may be beneficial to understand the status of both ventricles. IMPACT: Preterm infants are at high risk of hemodynamic compromise following a sudden change in loading conditions after PDA ligation. Three-dimensional echocardiography enables quantitative and serial evaluation of ventricular function and volume in preterm infants with PDA. PDA induces biventricular loading and functional abnormalities in preterm infants, and they dramatically change after surgery.

Tricuspid atresia 1c accompanying neonatal encephalopathy treated with pulmonary trunk banding and therapeutic hypothermia.

The influence of therapeutic hypothermia, known to improve neurodevelopmental outcomes in neonatal encephalopathy, remains unknown in newborns with severe congenital heart diseases. We report a neonate with tricuspid atresia type 1c suffering from moderate neonatal encephalopathy. A burst suppression pattern on amplitude-integrated electroencephalography recovered after inducing moderate therapeutic hypothermia, but exacerbated pulmonary overcirculation still persisted even after the rewarming. Since the medical treatment for pulmonary overcirculation had reached the limit, semi-urgent pulmonary trunk banding was performed on the 4th day of life. Postoperative brain magnetic resonance imaging showed no apparent brain injuries; the patient was discharged uneventfully. We share our perioperative management experience of a patient with tricuspid atresia type Ic who required therapeutic hypothermia for neonatal encephalopathy.

[Left Main Trunk Orifice Reconstruction Using Aortic flap and Autologous Pulmonary Arterial Patch for Severe Left Main Trunk Stenosis After Arterial Switch Operation].

A 6-year-old boy with left main trunk (LMT) stenosis, who had undergone arterial switch operation (ASO) for transposition of the great arteries( TGA) before 6 years, underwent LMT orifice reconstruction. Coronary angiography showed severe stenosis of LMT, already when he was hospitalized with heart failure after 3 months of ASO. He was stable with oral treatment, therefore we performed the LMT reconstruction, before starting school, in terms of the risk of coronary ischemic event. We reconstructed the LMT ostium using an aortic flap and autologous pulmonary arterial patch. The postoperative computed tomography showed neither stenosis nor kinking at the repair site of LMT. He remains asymptomatic for over 6 months. We have reported that this method is effective to repair coronary artery anomalies (CAAs), since using an aortic flap. We could form a coronary artery floor, which is affixed to the aortic wall. This method allows us to change the coronary orifice position and the angle, so it is very useful method not for only CAAs, but also for coronary stenosis after ASO.

Tubular Pouch Technique Using Aortic Tissues for Single Coronary Artery Transfer.

A single coronary artery with a single ostium from sinus 2 (2 R L Cx) is rare and difficult to transfer. A 6-day-old male neonate was diagnosed with dextro-transposition of the great arteries with an intact ventricular septum at birth. Computed tomography revealed the presence of a single coronary artery with a single ostium from sinus 2 (2 R L Cx). We performed a new coronary transfer technique involving a tubular pouch using the original aortic tissue for this single coronary artery. The postoperative course was uneventful, and postoperative computed tomography revealed a smooth coronary route with no stenosis.

Cosmetic and shoulder functional outcomes in posterolateral minithoracotomy atrial septal defect closure: A retrospective study.

BACKGROUND AND AIM: Transcatheter device closure has become the first treatment option for atrial septal defects (ASD). Surgical ASD closure, although still implemented, is cosmetically inferior to transcatheter closure. This study aimed to evaluate the feasibility as well as short- and long-term clinical outcomes of the right posterolateral minithoracotomy approach for surgical ASD closure. METHODS: In total, 102 consecutive patients underwent posterolateral minithoracotomy for ASD between January 2014 and December 2021 at our center. Early surgical outcomes, cosmetic findings, and skin perception were evaluated over 1 year of postoperative follow-up using a self-satisfaction survey (1: very good, 2: good, 3: normal, 4: not good, 5: bad), Cavendish score, and shoulder joint function (angles of flexion, extension, and abduction). RESULTS: No patient required conversion to median sternotomy. Only one patient required reoperation due to bleeding. Postoperative echocardiography revealed no residual shunt at discharge in all patients. The mean follow-up period was 3.7 years (range: 0.3-7.1 years), during which the questionnaire was answered by 69 of 98 patients who were evaluated after more than 1 year. The mean self-satisfaction survey scores for cosmetic findings and skin perception were 1.3 ± 0.6 and 1.2 ± 0.5, respectively. The Cavendish score was under Grade 1 in all patients. Shoulder flexion and abduction were normal at 180° in all patients, except one, while extension was normal at 50° in all patients, except three. CONCLUSIONS: Our procedure achieved not only good early surgical outcomes but also excellent long-term cosmetic and shoulder function results.

[Midterm Outcomes of Pulmonary Valve Leaflet Augmentation using Fresh Autologous Pericardium for Transannular Patch Repair of Tetralogy of Fallot].

BACKGROUND: Although valve sparing (VS) for patients with smaller pulmonary valves has been increasing, transannular patch repair( TAP) accounts for more than half of the total tetralogy of Fallot corrections worldwide. We use fresh autologous pericardial patches to perform a modified TAP procedure with pulmonary valve leaflet augmentation as proposed by Sung et al. We aimed to explore the early and midterm outcomes of this procedure. METHODS: We retrospectively reviewed 37 patients( group TAP:12;group VS:25) who underwent total tetralogy of Fallot corrections from April 2018 to December 2021. RESULTS: No midterm mortality was observed at a median observation period of 20.4 months in both groups. The midterm rates of freedom from moderate or more pulmonary regurgitation( PR) were 64.2 % and 21.4% in group TAP and 100% and 100% in group VS at 1 and 3 years, respectively( p<0.001). The midterm rates of freedom from pulmonary stenosis reintervention were 100% and 100% in group TAP and 96% and 96% in group VS at 1 and 3 years, respectively( p=0.51). CONCLUSIONS: TAP showed acceptable midterm survival and reintervention rate. Longer follow-up is essential considering the significantly higher PR in the postoperative period in group TAP.

Usefulness of Prolonged PR Interval to Predict Atrial Tachyarrhythmia Development Following Surgical Repair of Tetralogy of Fallot.

Atrial tachyarrhythmias (ATAs), which may occur after tetralogy of Fallot (TOF) surgery, can cause sudden cardiac death. However, ATAs may also develop in response to electrical substrates. This study aims to examine the predictive factors for ATAs by identifying electrical substrates in the atrium obtained from 12-lead electrocardiogram in patients who underwent TOF repair. A total of 144 patients aged >15 years (median, 31.6 years) who underwent TOF repair at Hokkaido University were enrolled. We investigated the correlation between the development of ATAs with age, time interval after initial corrective surgery, brain natriuretic peptide levels, cardiac magnetic resonance parameters (right ventricular end-diastolic volume index, right ventricular end-systolic volume index, right ventricular ejection fraction, right atrial volume index, left ventricular end-diastolic volume index, left ventricular ejection fraction), and 12-lead electrocardiogram parameters (P wave maximum voltage, PR interval, QRS width, number of fragmented QRS). Of the 144 patients, 44 patients (30.6%) developed ATAs. Multivariate analysis revealed time interval after initial corrective surgery (odds ratio 6.7, 95% confidence interval 1.78 to 12.6) and PR interval (odds ratio 2.7, 95% confidence interval: 1.17 to 4.20) as independent risk factors for the development of ATAs. The receiver operating characteristic curve revealed a PR interval cut-off value of >200 milliseconds as predictive of the development of ATAs in patients more than 15 years after initial corrective surgery (area under the curve, 0.658; sensitivity, 71.4%; specificity, 66.4%). The present study demonstrated that a prolonged PR interval is a simple and convenient predictor for the development of ATAs in patients who underwent TOF repair.

Perioperative junctional ectopic tachycardia associated with congenital heart disease: risk factors and appropriate interventions.

The risk factors and the appropriate interventions for perioperative junctional ectopic tachycardia (JET) in congenital heart disease (CHD) surgery have not been sufficiently investigated despite the severity of this complication. This study aimed to examine the risk factors and interventions for perioperative JET. From 2013 to 2020, 1062 surgeries for CHD (median patient age: 4.3 years, range 0.0-53.0) with or without a cardiopulmonary bypass (CPB) were performed at Hokkaido University, Japan. We investigated the correlation between perioperative JET morbidity factors, such as age, genetic background, CPB/aortic cross-clamp (ACC) time, use of inotropes and dexmedetomidine, STAT score, and laboratory indices. The efficacy of JET therapies was also evaluated. Of the 1062 patients, 86 (8.1%) developed JET. The 30-day mortality was significantly high in JET groups (7% vs. 0.8%). The independent risk factors for JET included heterotaxy syndrome [odds ratio (OR) 4.83; 95% confidence interval (CI) 2.18-10.07], ACC time exceeding 90 min (OR 1.90; CI 1.27-2.39), and the use of 3 or more inotropes (OR 4.11; CI 3.02-5.60). The combination of anti-arrhythmic drugs and a temporary pacemaker was the most effective therapy for intractable JET. Perioperative JET after CHD surgery remains a common cause of mortality. Inotrope use was a risk factor for developing JET overall surgery risk. In short ACC surgeries, heterotaxy syndrome could increase the risk of JET, which could develop even without inotrope use in long ACC surgeries. It is crucial not to delay the treatment in cases with unstable hemodynamics caused by this arrhythmia. It is recommended to reduce numbers not dose of inotropes.

Surgical Angioplasty for a Single Coronary Artery With Myocardial Bridging.

Single coronary artery with myocardial bridging is rare but associated with the risk of sudden cardiac death in children, yet there is no standardized treatment approach. We report a 6-year-old girl with chest pain having a single coronary artery with complete myocardial bridging of the left main coronary artery (modified Lipton type RII-S) branching from the right coronary artery at an acute angle. Coronary angioplasty using an in situ aortic flap and an autologous pulmonary arterial patch combined with myocardial unroofing was successfully performed for the left main coronary artery. The patient remains healthy for over 3 years without any exercise restriction.

Aberrant Subclavian Artery Bypass for Cerebral Perfusion in Aortic Arch Reconstruction.

When reconstructing an interrupted aortic arch with an aberrant right subclavian artery, careful consideration must be made to protect regional cerebral flow. There are several approaches to cardiopulmonary bypass during aortic arch reconstruction. Here, we describe a case of a 3-month-old female patient with a type B interruption who underwent a right subclavian artery bypass using the right internal thoracic artery to supply sufficient cerebral blood flow throughout the operation. This artery was enlarged as a collateral artery and was beneficial as a bypass graft to ensure cerebral protection.

Impact of the Axillary Approach on Epicardial Pacing Lead Durability in Children.

BACKGROUND: To avoid lead failure and pocket infection in neonates and infants requiring pacemakers, we used the axillary approach of placing the generator in the axilla and the leads in the intrathoracic space. We describe the technical details of the axillary approach and evaluate the efficacy of this method. METHODS: We assessed 21 patients (7 male) weighing 8 kg or less who underwent epicardial pacemaker implantation with the axillary approach between 2004 and 2018. The axillary approach entails (1) positioning the pacemaker generator in the axilla to avoid local skin and pocket complications due to tissue compression by the generator, and (2) making a double loop in the pleural space to reduce stress on the pacemaker leads caused by somatic growth. This approach can be combined with median sternotomy for simultaneous intracardiac repair. RESULTS: The patients' median age at pacemaker implantation was 6 months; 16 patients (76%) were aged less than 12 months. The median body weight was 4.5 kg (interquartile range, 3 to 7). In all 5 patients requiring simultaneous cardiac repair, a median sternotomy was performed to access the heart. Sixteen patients required only pacemaker implantation: left thoracotomy was performed in 10 patients, right thoracotomy in 5, and subxiphoid approach in 1. The 5-year and 10-year freedom from pacemaker-related adverse events was 89.4% and 79.5%, respectively. CONCLUSIONS: The axillary approach using intrathoracic double-loop routing of leads to position the generator in the axilla for pacemaker implantation can be a valuable alternative for neonates/infants weighing 8 kg or less with or without complex congenital heart disease.

External Stenting for Bronchomalacia Involving the Bronchus Intermedius.

In recent years, external stenting has been used as a reliable method to relieve airway compression of the trachea and main bronchi in young children with acceptable age-proportional airway growth. However, to our knowledge, little literature supporting this approach for the distal airway has been published. A 1-year-old girl with absent pulmonary valve syndrome who had recurrent respiratory infections was diagnosed with bronchomalacia. She underwent external stenting; the infections disappeared postoperatively. This case describes a successful external stenting for malacia of the bronchus intermedius, which exhibited short- and mid-term safety and effectiveness.

Re-Norwood operation using femoral artery cannulation.

Re-Norwood operation is technically difficult to perform and is a high risk procedure due to the underlying hypocardiac function. Herein, we describe our successful re-Norwood operation approach in a 6-month old infant with persistent severe cyanosis and aortic re-coarctation. Our procedure was performed using femoral artery cannulation to protect cerebrospinal and lower body perfusion. Safe reopening of the chest was achieved, despite strong adhesions due to prior surgeries. Our repair and anastomosis techniques are described in detail. Cardiac circulation and function improved post-surgery. The patient was maintained on anti-heart failure drug therapy after surgery while awaiting a Glenn procedure.

Intralobar pulmonary sequestration associated with left main coronary artery obstruction and mitral regurgitation.

A 4-year-old boy with left intralobar pulmonary sequestration associated with left main coronary artery obstruction (LMCAO) and severe mitral regurgitation (MR) was admitted to our hospital. Since the patient presented with dyskinesia of the cardiac apex and increased left ventricular end-diastolic volume (LVEDV), left main coronary artery reconstruction and mitral annuloplasty were performed. The enlargement of the left ventricle was improved after sequential surgeries. There was a risk of deterioration of MR and regrowth of LVEDV due to shunt blood flow; therefore, left lower lobectomy and aberrant artery division were performed. This is a very rare case of a patient with pulmonary sequestration associated with LMCAO and severe MR.

[Modified Carpentier Technique is Useful Method for Tricuspid Regurgitation in Hypoplastic Left Heart Syndrome].

Management of tricuspid regurgitation( TR) before right ventricular dysfunction is critical in patients with hypoplastic left heart syndrome (HLHS);however, appropriate tricuspid valvuloplasty (TVP) is challenging. We report a TVP technique for TR in a 4-year-old girl with HLHS, who had undergone Norwood operation, bidirectional cavopulmonary shunt, and TVP. Preoperative echocardiography revealed the etiology of TR as anterior leaflet prolapse, annulus dilatation, and relative tethering of the septal leaflet. We performed surgical reconstruction of the anterior leaflet with artificial chordae. Before annuloplasty, the posterior leaflet and a part of the septal leaflet were detached from the annulus with a 1 mm margin using the Key-Reed technique. Furthermore, the posterior leaflet was slid to augment the septal leaflet. We managed to regulate the TR by enlarging the septal leaflet, thus increasing the coaptation zone. We believe that this technique will be useful for TR with annulus dilatation in HLHS.

Apex rotation as a risk factor for total anomalous pulmonary connection repair in single ventricle.

BACKGROUND: The high incidence of postoperative pulmonary venous obstruction (PVO) is a major mortality-associated concern in patients with right atrial isomerism and extracardiac total anomalous pulmonary venous connection (TAPVC). We evaluated new anatomical risk factors for reducing the space behind the heart after TAPVC repair. METHODS: Eighteen patients who underwent TAPVC repair between 2014 and 2020 were enrolled. Sutureless technique was used in 12 patients and conventional repair in six patients. The angle between the line perpendicular to the vertebral body and that from the vertebral body to the apex was defined as the "vertebral-apex angle (V-A angle)." The ratio of postoperative and preoperative angles, indicating the apex's lateral rotation, was compared between patients with and without PVO. RESULTS: The median (interquartile range) age and body weight at repair were 102 (79-176) days and 3.8 (2.6-4.8) kg, respectively. The 1-year survival rate was 83% (median follow-up, 29 [11-36] months). PVO occurred in seven patients (39%), who showed an obstruction of one or two branches in the apex side. The postoperative V-A angle (46° [45°-50°] vs. 36° [29°-38°], p = 0.001) and the ratio of postoperative and preoperative V-A angles (1.27 [1.24-1.42] vs. 1.03 [0.98-1.07], p = 0.001) were significantly higher in the PVO group than in the non-PVO group. The cut-off values of the postoperative V-A angle and ratio were 41° and 1.17, respectively. CONCLUSION: A postoperative rotation of the heart apex into the ipsilateral thorax was a risk factor for branch PVO after TAPVC repair.

Optimization of prediction methods for risk assessment of pathogenic germline variants in the Japanese population.

Predicting pathogenic germline variants (PGVs) in breast cancer patients is important for selecting optimal therapeutics and implementing risk reduction strategies. However, PGV risk factors and the performance of prediction methods in the Japanese population remain unclear. We investigated clinicopathological risk factors using the Tyrer-Cuzick (TC) breast cancer risk evaluation tool to predict BRCA PGVs in unselected Japanese breast cancer patients (n = 1,995). Eleven breast cancer susceptibility genes were analyzed using target-capture sequencing in a previous study; the PGV prevalence in BRCA1, BRCA2, and PALB2 was 0.75%, 3.1%, and 0.45%, respectively. Significant associations were found between the presence of BRCA PGVs and early disease onset, number of familial cancer cases (up to third-degree relatives), triple-negative breast cancer patients under the age of 60, and ovarian cancer history (all P < .0001). In total, 816 patients (40.9%) satisfied the National Comprehensive Cancer Network (NCCN) guidelines for recommending multigene testing. The sensitivity and specificity of the NCCN criteria for discriminating PGV carriers from noncarriers were 71.3% and 60.7%, respectively. The TC model showed good discrimination for predicting BRCA PGVs (area under the curve, 0.75; 95% confidence interval, 0.69-0.81). Furthermore, use of the TC model with an optimized cutoff of TC score ≥0.16% in addition to the NCCN guidelines improved the predictive efficiency for high-risk groups (sensitivity, 77.2%; specificity, 54.8%; about 11 genes). Given the influence of ethnic differences on prediction, we consider that further studies are warranted to elucidate the role of environmental and genetic factors for realizing precise prediction.

Transcriptome Analysis Reveals Differential Gene Expression between the Closing Ductus Arteriosus and the Patent Ductus Arteriosus in Humans.

The ductus arteriosus (DA) immediately starts closing after birth. This dynamic process involves DA-specific properties, including highly differentiated smooth muscle, sparse elastic fibers, and intimal thickening (IT). Although several studies have demonstrated DA-specific gene expressions using animal tissues and human fetuses, the transcriptional profiles of the closing DA and the patent DA remain largely unknown. We performed transcriptome analysis using four human DA samples. The three closing DA samples exhibited typical DA morphology, but the patent DA exhibited aorta-like elastic lamellae and poorly formed IT. A cluster analysis revealed that samples were clearly divided into two major clusters, the closing DA and patent DA clusters, and showed distinct gene expression profiles in IT and the tunica media of the closing DA samples. Cardiac neural crest-related genes such as JAG1 were highly expressed in the tunica media and IT of the closing DA samples compared to the patent DA sample. Abundant protein expressions of jagged 1 and the differentiated smooth muscle marker calponin were observed in the closing DA samples but not in the patent DA sample. Second heart field-related genes such as ISL1 were enriched in the patent DA sample. These data indicate that the patent DA may have different cell lineages compared to the closing DA.