Comprehensive management of type A acute aortic dissection in the paediatric population with ACTA 2 mutation: case reports and literature review of paediatric aortic dissection.

Acute aortic dissection in the paediatric population is rare but lethal. We present two paediatric cases of type A acute aortic dissection that required emergent procedures and were later found to have genetic mutations. High index of suspicion, early clinical diagnosis, prompt treatment, the advantageous collaboration between the paediatric team and aortic surgeons, and familial genetic testing are paramount to achieve a good outcome.

Retrograde cerebral perfusion for intracardiac air embolism in Fontan procedure.

We report the case of a 5-year-old boy who suffered from an intracardiac air influx with suspected cerebral air embolism during the Fontan procedure. We immediately transformed the cardiopulmonary bypass circuit to perform a retrograde cerebral perfusion, which resulted in successful neuroprotection. He was extubated in the operating room without any neurological defects.

Modified atrioventricular valve replacement in children.

One major challenge of atrioventricular valve replacement in children is the small size of the native valve annulus. In addition, in cases of atrioventricular septal defect, the atrioventricular node (AVN) shifts to a different location, making this condition difficult to treat. This report describes a technique that uses a trapezoid-shaped patch to separate the implanted valve suture-line from the AVN, which allows surgeons to implant larger valves while simultaneously preventing injury to the AVN.

Perioperative evaluation of neonatal aortic arch thrombosis.

Herein, we present a neonatal case of coarctation of the aorta, with aortic arch thrombus confirmed by echocardiography. We performed thrombus removal and aortic arch repair emergently. This critical condition necessitates quick preoperative evaluation with echocardiography. Moreover, postoperative evaluation using computed tomography is reasonable to assess an aortic arch configuration, and exclude the remnant thrombus.

Surgical Management of Intraoperative Aortic Dissection in Type 3 Loeys-Dietz Syndrome with MYH11 Co-Mutation.

A 57-year-old female with Loeys-Dietz syndrome type 3 and MYH11 gene mutation underwent unexpected intraoperative ascending aortic dissection and subsequent rapid progression of the aortic arch dissecting aneurysm. Intra-surgical contingency plans with regard to aortic dissection and aneurysm should be considered for Loeys-Dietz syndrome, especially with comorbid mutations.

Novel Urinary Biomarkers for Acute Kidney Injury and Prediction of Clinical Outcomes After Pediatric Cardiac Surgery.

Acute kidney injury (AKI) is a serious complication of pediatric cardiac surgery, with high morbidity and mortality. We aimed to evaluate the perioperative risk factors for AKI, and the validity of novel diagnostic urinary biomarkers after pediatric cardiac surgery. We analyzed 103 consecutive pediatric patients (≤ 18 years old), who underwent cardiac surgery. AKI was defined by ≥ 50% increase in serum creatinine levels from baseline. Urinary liver-type fatty acid binding protein (L-FABP) and neutrophil gelatinase-associated lipocalin (NGAL) were measured postoperatively at the intensive care unit (ICU) admission, subsequently at 4, 12, and 24 h. Areas under the receiver-operating characteristic curves (AUC) were calculated at each assessment time. AKI had developed in 47 patients (45.6%) by the second postoperative day. Univentricular status, aortic cross-clamping time, and intraoperative fluid balance were independently associated with AKI (p = 0.02, 0.01 and 0.01, respectively). Urinary L-FABP and NGAL were significantly higher in the AKI group at each point (p < 0.05). The predictive abilities of both biomarkers (AUC = 0.78-0.90) at ICU admission and 4 h after were especially high. The patients with L-FABP greater than the cutoff value at ICU admission and 4 h after ICU admission had significantly longer intubation and hospitalization periods (p < 0.05). Those with elevated NGAL levels at admission, and 4 h and 24 h after ICU admission, had significantly longer intubation, ICU stay, and hospitalization (p < 0.05). L-FABP and NGAL can be useful biomarkers for detecting early AKI after pediatric cardiac surgery and predicting adverse clinical outcomes.

Quick atrial access by subxiphoid approach in extracorporeal cardiopulmonary resuscitation after bidirectional Glenn procedure.

Bidirectional Glenn procedure outcomes are very good; therefore, extracorporeal membrane oxygenation use as extracorporeal cardiopulmonary resuscitation is uncommon. We describe a 13-month-old female who required extracorporeal cardiopulmonary resuscitation for ventricular tachycardia provoked by transient myocarditis 6 months post-bidirectional Glenn procedure. After extracorporeal membrane oxygenation induction with cannulation on the cervical vessels, small skin incision was created on the subxiphoid area without sternotomy and the atrium was cannulated. With adequate venous drainage and ventricular unloading, ventricular tachycardia eventually converted to sinus rhythm. The patient withdrew from extracorporeal membrane oxygenation and was discharged successfully. Our results suggest that for successful post-bidirectional Glenn extracorporeal cardiopulmonary resuscitation, quick atrial access and ventricular unloading are essential.

Landiolol Hydrochloride Rapidly Controls Junctional Ectopic Tachycardia After Pediatric Heart Surgery.

OBJECTIVES: Junctional ectopic tachycardia is a supraventricular tachyarrhythmia with atrioventricular dissociation that causes life-threatening postsurgical conditions in pediatric heart patients. This study evaluates the efficacy of landiolol hydrochloride for managing junctional ectopic tachycardia. DESIGN: A single-center retrospective study. SETTING: PICU at the university hospital. PATIENTS: Of 561 pediatric patients who underwent open-heart surgery between 2006 and 2017, 10 patients developed sustained junctional ectopic tachycardia and were selected for landiolol treatment. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Landiolol decreased mean heart rate significantly from 206.1 ± 14.5 to 158.0 ± 8.6 beats/min within 2 hours after administration (p < 0.01). Mean time to achieve 20% heart rate reduction was 2.1 ± 0.5 hours. Systolic blood pressure between pre and post landiolol administration did not change significantly (72.6 ± 5.9 to 79.7 ± 6.2 mm Hg). Once junctional heart rate was sufficiently suppressed, atrioventricular sequential pacing was introduced to stabilize hemodynamics. Nine of 10 cases (90%) had atrioventricular sequential pacing to maintain appropriate heart rate and restore atrioventricular synchronicity under suppressed junctional heart rate. Subsequently, eight of 10 cases (80%) were converted to regular sinus rhythm within 24 hours after starting landiolol administration. The average time to achieve sinus rhythm conversion was 7.9 ± 3.4 hours. CONCLUSIONS: Landiolol rapidly suppresses junctional heart rate in junctional ectopic tachycardia after pediatric heart surgery without significant blood pressure compromises. Subsequent atrioventricular sequential pacing was effective at restoring atrioventricular synchronicity and stabilizing hemodynamics. Combining junctional rate control with landiolol and atrioventricular sequential pacing is therefore suggested as a promising option for prompt management of postoperative junctional ectopic tachycardia.

Valve-sparing reimplantation for neoaortic root dilatation and regurgitation with an unbalanced cusp after the arterial switch operation.

A 13-year-old male presented with neoaortic root dilatation and severe aortic valve regurgitation 13 years following an arterial switch operation. The valve cusps were unbalanced due to an enlarged non-coronary cusp. A valve-sparing reimplantation with a cusp plication was performed which resulted in a competent valve with trivial regurgitation. Thus, even in an unbalanced cusp, valve-sparing reimplantation can be used for neoaortic root dilatation and valve regurgitation after an arterial switch operation.

Postoperative Dysphagia in Debranching Thoracic Endovascular Aortic Repair with Retroesophageal Carotid-Carotid Bypass.

A carotid-carotid bypass is commonly used in debranching thoracic endovascular aortic repair. When the bypass graft is placed in the retroesophageal position, the esophagus may be compressed by the graft from behind. Therefore, dysphagia may occur postoperatively as a serious complication. To avoid this issue, we recommend placing the bypass graft below the cricoid cartilage.

Long-Term Outcomes of Ascending Sliding Arch Aortoplasty.

Background: Coarctation of the aorta can be associated with significant hypoplasia of the aortic arch. In contrast to patch aortoplasty, ascending sliding arch aortoplasty uses viable autologous tissue for potential growth in children. We reviewed the mid- to long-term outcomes of this technique. Methods: Between 2002 and 2023, 28 patients underwent ascending sliding arch aortoplasty for the patients with coarctation of the aorta (n = 22) and interrupted aortic arch (n = 2). Four patients underwent previous surgical coarctation repair at other institutions. The median patient age and body weight were 28.5 months (3 weeks to 15.6 years) and 13.4 kg (3.7-70 kg), respectively. Results: Although one patient had a recurrent nerve injury postoperatively, there were no other major morbidities or mortalities. The last follow-up echocardiography demonstrated that the mean peak velocity improved from 3.9 ± 0.6 to 0.9 ± 0.8 m/s, and the pressure gradient improved from 63.6 ± 21.5 to 7.1 ± 7.7 mm Hg. The postoperative diameters of the ascending aorta, proximal arch, distal arch, and isthmus all increased significantly. The mean postoperative length of stay was 5.9 ± 2.1 days, and the median follow-up time was 7.3 years (10 days to 20.5 years). No reoperation or catheterization-based intervention was performed for residual coarctation. Conclusions: Ascending sliding arch aortoplasty is safe and effective for treating coarctation of the aorta with aortic arch hypoplasia. This technique is applicable for children ranging in size from neonates to older children (or adolescents), recurrent coarctation cases, and provides complete relief of narrowing by utilizing viable native aortic tissue.

Association between oral frailty and lower urinary tract symptoms among middle-aged and older adults in community-dwelling individuals: a cross-sectional study.

PURPOSE: We assessed the association between oral frailty risk and LUTS among middle-aged and older adults in a community-dwelling population. METHODS: This cross-sectional study was conducted among 586 subjects aged ≥ 40 years who participated in the Iwaki Health Promotion Project in Hirosaki, Japan. We used the International Prostate Symptom Score (IPSS) and the Overactive Bladder Symptom Score (OABSS) to assess LUTS. LUTS was defined as an IPSS score of 8 or higher or meeting diagnostic criteria for OAB. Oral frailty risk was defined as experiencing two or more of the following: decreased chewing ability, decreased biting force, and dry mouth sensation. Physical performance (10-m gait speed and grip strength) was used for analysis. The association between oral frailty risk and LUTS was examined using multivariate logistic regression analyses. RESULTS: The study included 218 men and 370 women, of whom 140 had LUTS. The mean age of this cohort was 59 years. Significant differences were observed between the LUTS and non-LUTS groups, including age, hypertension, history of CVD, depressive status, sleep disturbance, and 10 m gait speed. The prevalence of oral frailty risk was significantly higher in the LUTS group than in the non-LUTS group (26% vs. 11%, P < 0.001). Multivariate analysis revealed that age, male gender, and oral frailty risk (odds ratio: 2.67, 95% confidence interval: 1.57-4.51, P < 0.001) were independent factors for LUTS. Moreover, oral frailty risk was an independent factor in both participants aged < 65 years and participants aged ≥ 65 years. CONCLUSIONS: Oral frailty was independently associated with LUTS.

Modified biatrial heart transplantation after bilateral bidirectional cavo-pulmonary shunts.

We describe a patient who underwent modified biatrial anastomosis heart transplantation after the bilateral Glenn procedure. We introduced a new surgical technique to use the native central pulmonary artery as systemic venous return, which was anastomosed to right atrium, and then, biatrial anastomosis was performed.

Sutureless Repair With Extended Atriotomy for Post-Repair Pulmonary Venous Obstruction.

Post-repair pulmonary venous obstruction is a common cause of reoperation after total anomalous pulmonary venous return repair. Herein, we report 3 cases of specific type of post-repair pulmonary venous obstruction with eccentric stenosis of pulmonary vein ostium due to retained composite neoseptum and the technique used for subsequent repair.

Heart Transplantation in a Case of Scimitar Syndrome.

We present a rare clinical scenario of a patient with tetralogy of Fallot, hypertrophic cardiomyopathy, and concomitant scimitar syndrome. We created a scimitar vein cuff from a recipient heart, performed its translocation, and subsequently performed heart transplantation. This ingenuity reduces the likelihood of scimitar vein obstruction.

Conduction disorders after perimembranous ventricular septal defect closure: continuous versus interrupted suturing techniques.

OBJECTIVES: The aim of this study was to investigate postoperative conduction disorder differences between continuous and interrupted suturing techniques for the closure of perimembranous outlet-type ventricular septal defects (VSDs) in both tetralogy of Fallot (ToF) and isolated VSD cases. METHODS: Patients aged 4 years or younger who underwent VSD closure for ToF (n = 112) or isolated perimembranous outlet-type VSD (n = 73) from April 2010 to December 2018 at 3 centres were reviewed. Patients either received continuous suturing for ToF (C-ToF, n = 58) or isolated VSD (C-VSD, n = 50), or interrupted suturing for ToF (I-ToF, n = 54) or isolated VSD (I-VSD, n = 23). Cohorts did not differ in preoperative characteristics. Postoperative conduction disorder differences upon discharge and postoperative year 1 (POY1) were evaluated by electrocardiography. RESULTS: The C-ToF group showed significantly shorter PQ intervals (124.0 vs 133.5 ms; P = 0.042 upon discharge, 125.3 vs 133.5 ms; P = 0.045 at POY1) and QRS durations (98.0 vs 106.2 ms; P = 0.031 upon discharge, 97.3 vs 102.5 ms; P = 0.040 at POY1) than the I-ToF group. Right bundle branch block incidence was significantly lower in the C-ToF versus I-ToF groups (56.8 vs 75.9; P = 0.045 upon discharge, 56.8 vs 75.9; P = 0.045 at POY1). Heart rates were significantly lower in the C-ToF versus I-ToF groups at POY1 (109.2 vs 119.3 bpm; P < 0.001). No parameters significantly differed between C-VSD and I-VSD groups. Multivariable analyses confirmed the group (C-ToF versus I-ToF) as a significant covariate in postoperative heart rate, PQ interval, QRS duration and right bundle branch block outcomes at POY1 (P = 0.013, 0.027, 0.013 and 0.014, respectively). CONCLUSIONS: A continuous suturing technique for the closure of outlet-type VSD in ToF could reduce the incidence of postoperative right bundle branch block, shorten the PQ interval and lower heart rate. SUBJECT COLLECTION: 110, 138, 139.