An Extremely Preterm Infant Born at 23 Weeks' Gestation With an Interrupted Aortic Arch Complex: A Case Report.

We present a case of an infant male born at 23 weeks' gestation with an interrupted aortic arch (IAA) complex. We treated the patient with hypoxic gas ventilation to address developing systemic undercirculation in the acute postnatal phase. As the symptoms of bronchopulmonary dysplasia evolved, hypoxic gas ventilation was no longer required to stabilize the hemodynamics. The patient was discharged home after undergoing the palliative surgical procedure of bilateral pulmonary artery banding and ductus arteriosus stent implantation. Although he suffered from pulmonary hypertension, it was controllable with oxygen supplementation and pulmonary vasodilators. There are limited therapeutic options available for extremely preterm infants with critical congenital heart defects (CHDs). Hypoxic gas ventilation might be considered as one of the options, with its risks taken into account, to manage extremely preterm infants with CHDs with pulmonary overcirculation before performing surgical interventions.

Paradoxical Continuous Left-to-Right Ductal Shunt during Circulatory Collapse due to Ductal Closure in an Infant with Duct-Dependent Systemic Circulation.

Duct-dependent systemic circulation is accompanied by a right-to-left ductal shunt, at least during systole. Although observations of paradoxical continuous left-to-right shunts in duct-dependent systemic circulation have been reported, the mechanism remains unclear. We report a continuous left-to-right ductal shunt throughout the cardiac cycle during the initial recovery phase from circulatory collapse and right ventricular (RV) dysfunction due to ductal closure in an infant with hypoplastic left heart and severe aortic coarctation. Further recovery improved his RV function and changed the ductal flow from continuous left-to-right to bidirectional, which is usually seen in duct-dependent systemic circulation. Marked RV dysfunction may contribute to the continuous left-to-right ductal shunt. A continuous left-to-right ductal shunt should not be used to rule out duct-dependent systemic circulation.

Efficacy of a phosphodiesterase type 5 inhibitor on the pulmonary artery index and postoperative hemodynamics after a Fontan operation.

There have been no reports regarding the influence of a change in the pulmonary artery index before or after a Fontan operation. Moreover, the factors that might affect the change in the pulmonary artery index are unclear. This was a retrospective study, which included 94 patients who underwent a Fontan operation. We divided the patients into two groups according to the change in the pulmonary artery index before and after the Fontan operation: the increased pulmonary artery index group (n = 51) and decreased pulmonary artery index group (n = 43). The pulmonary artery index after the Fontan operation was significantly higher in the increased pulmonary artery index group than decreased pulmonary artery index group (p < 0.0001). The central venous pressure (p < 0.0001) and pulmonary vascular resistance (p = 0.007) were significantly lower in the increased pulmonary artery index group. The mixed venous oxygen saturation was significantly higher in the increased pulmonary artery index group (p < 0.0001). The pulmonary artery index was more increased in the patients who were administered a phosphodiesterase type 5 inhibitor than in those who were not (p = 0.03). The change in the pulmonary artery index reflected the hemodynamics after a Fontan operation during the short term. In that respect, phosphodiesterase type 5 inhibitors might be involved in the process.

Efficacy of a Subcutaneous Implantable Cardioverter Defibrillator in a Child with Early Repolarization Syndrome.

An early repolarization (ER) pattern or J waves are considered to be a benign finding observed in the healthy population, however, it has been pointed out that the ER pattern seen in the inferolateral leads could be an independent risk factor for fatal arrhythmias. We present a pediatric case in which early repolarization syndrome (ERS) was suspected due to the presence of ER or J waves in the inferior leads, which eventually disappeared after the administration of pilsicainide. During the follow-up period, several fatal ventricular arrhythmias were recorded after implantation of a subcutaneous implantable cardiac defibrillator (S-ICD). This report describes the efficacy of S-ICDs in a child with an ER pattern after aborted sudden cardiac death.

Successful Percutaneous Thrombectomy of the Left Pulmonary Artery in a Neonate After a Patent Ductus Arteriosus Clipping.

We report a neonate with a successful percutaneous thrombectomy of a total thrombotic occlusion of the left pulmonary artery (LPA) after a surgical clipping for a patent ductus arteriosus (PDA). We suspected the compression of the LPA by the clipping and postoperative hemodynamic instability caused the LPA obstruction. After the surgical removal of the PDA clip and division of the PDA, we could safely retrieve the LPA thrombus with a non-hydrodynamic thrombectomy catheter for coronary arteries.

Late recovery of the cardiopulmonary exercise capacity after transcatheter amplatzer device closures for atrial septal defects in adults.

Transcatheter atrial septal defect (ASD) closures using an Amplatzer Septal Occluder (ASO) have been widely performed. Compared to children, we sometimes experience late recovery of exercise performance in adult patients. Our study aimed to evaluate the change in the cardiopulmonary exercise capacity in asymptomatic or mildly symptomatic adult patients after a transcatheter ASD closure using an ASO. The subjects consisted of 29 patients (age 39.5 ± 13.6 years) that underwent cardiopulmonary exercise testing (CPX) before, 3, 6, and 12 months after a transcatheter secundum ASD closure using an ASO. The peak oxygen consumption (peak VO2), anaerobic threshold (AT), and slope of the correlation between the ventilation and carbon dioxide production (VE/VCO2 slope) were evaluated. We also evaluated the left-ventricular end-diastolic diameter (LVEDD), right-ventricular end-diastolic dimension (RVEDD) by echocardiography, and hemodynamic values by cardiac catheterization before the ASO procedure. The peak VO2 did not show any improvement 3 months after the ASO procedure; however, a significant improvement was displayed 6 and 12 months (baseline: 23.4 ± 6.3, 3 months: 23.6 ± 6.4, 6 months: 25.1 ± 5.6, 12 months: 26.4 ± 5.3 mL/kg/min; p < 0.001) after the ASO. The LVEDD (before: 38.1 ± 3.6, 3 months: 43.4 ± 3.4 mm; p < 0.001) and RVEDD (before: 33.6 ± 5.3, 3 months: 26.3 ± 2.6 mm; p < 0.001) on echocardiography quickly improved 3 months after the ASO. Although the LVEDD and RVEDD normalized 3 months after the ASO, the peak VO2 still decreased; however, the peak VO2 improved to almost a normal range 6 months after the ASO.

Efficacy and safety of tolvaptan after pediatric congenital heart disease surgery.

It is not clear whether tolvaptan is safe and effective irrespective of various underlying clinical conditions including the functional ventricle morphology, chromosomal abnormalities, and renal function after complex pediatric congenital heart disease surgery. Also, the appropriate dose of tolvaptan in these patients has not been previously identified. We retrospectively assessed the urine volume, body weight, patient clinical characteristics, laboratory data, and vital signs before and on days 1 and 7 of the tolvaptan administration after congenital heart disease surgery. Also, we assessed the relationship between the tolvaptan dose and its effects. A total of 86 patients were included the study. The mean time from the surgery to the tolvaptan administration was 23.5 ± 3.7 days. After administering tolvaptan, the urine volume significantly increased and body weight significantly decreased from baseline by days 1 and 7 (p < 0.0001). The urine volume significantly increased more in the survivors than the deceased. Of the 22 patients who had low serum sodium concentrations at baseline, 20 had an increased serum sodium concentration on day 7. The clinical effect of tolvaptan was not affected by the functional ventricle morphology, chromosomal abnormalities, or renal function. There was a positive correlation between the tolvaptan dose and change in the urine volume until a tolvaptan dose of up to 0.3 mg/kg/day but not at more than 0.3 mg/kg/day. Tolvaptan administration is safe and effective after congenital heart disease surgery irrespective of various underlying clinical conditions. Though the urine volume tends to increase until a tolvaptan dose of up to 0.3 mg/kg/day in pediatric congenital heart disease patients, there was no further benefit with more than 0.3 mg/kg/day.

Relapsing 6q24-related transient neonatal diabetes mellitus with insulin resistance: A case report.

The overexpression of imprinted genes on chromosome 6q24 causes 6q24-related transient neonatal diabetes mellitus (6q24-TNDM). Most cases of 6q24-TNDM show transient diabetes mellitus (DM) during the neonatal period, followed by relapse after puberty. These two courses of DM are both characterized by insulin insufficiency. However, there has been no previously reported case of 6q24-TNDM with insulin resistance at relapse. We report the case of a 10-yr-old Japanese girl with relapsing 6q24-TNDM. In the neonatal period, she had hyperglycemia and was treated with insulin injection until 2 mo of age. After several years of remission of DM, her HbA1c level increased to 7.4% at 10 yr of age. Homeostasis model assessment of insulin resistance (HOMA-IR) score was high at 6.2. After starting metformin therapy, her glycemic control improved along with normalization of HOMA-IR score. Using microsatellite marker analysis on the 6q24 region and array comparative genome hybridization, we diagnosed her with 6q24-TNDM due to paternally inherited duplication of 6q24. These data indicate that patients with 6q24-TNDM can develop relapsing DM with insulin resistance.

Hepatocyte growth factor predicts failure of Fontan circulation.

AIMS: This study aimed to assess the value of the hepatocyte growth factor (HGF) as an independent predictor of a Fontan circulation failure. METHODS AND RESULTS: This retrospective case-control study included 34 consecutive patients (19 men and 15 women) who underwent a post-operative cardiac catheterization after a Fontan operation at the Saitama Medical University International Medical Center between April 2017 and December 2019. We divided the patients into two groups according to the HGF level: HGF < 0.4 ng/mL (n = 20, normal HGF group) and HGF ≥ 0.4 ng/mL (n = 14, elevated HGF group). The age at the time of the cardiac catheterization was 59.3 ± 7.9 months. The range of the duration between the Fontan operation and the cardiac catheterization was 37.5 ± 7.9 months. The age (P = 0.417), gender (P = 0.08), morphology of the functional ventricle (P = 0.99), presence or closure of the Fontan fenestration (P = 0.704), and rate of medication use (angiotensin-converting enzyme inhibitors or angiotensin II receptor blockers) (P = 0.99) were equivalent between the two groups. Laboratory parameters including the brain natriuretic peptide level (P = 0.085), serum creatinine level (P = 0.27), and aspartate aminotransferase level (P = 0.235) were similar between the two groups. The elevated HGF group had a higher C-reactive protein level than the normal HGF group (0.42 ± 0.14 and 0.05 ± 0.01 mg/dL, P = 0.005). The elevated HGF group had a higher central venous pressure (CVP) level than the normal HGF group (13.4 ± 0.7 and 9.7 ± 0.4 mmHg, P < 0.0001), and the HGF was positively correlated with the CVP (P = 0.0004, r2  = 0.33). The SvO2 level was significantly lower in the elevated HGF group than in the normal HGF group (61.9 ± 2.3% and 75.0 ± 1.2%, P < 0.0001), and the HGF was negatively correlated with the SvO2 (P < 0.0001, r2  = 0.65). Of the 34 patients, six underwent catheter interventions. Patients who underwent catheter interventions had a higher HGF level than those who did not (0.44 ± 0.03 and 0.37 ± 0.01 ng/mL, P = 0.032). The receiver operating characteristic curve created for the discrimination of a catheter intervention revealed that an HGF value of >0.405 ng/mL could detect the need for a catheter intervention with 75.0% sensitivity and 83.3% specificity. A multivariable regression analysis showed that an elevated HGF was an independent predictor of an elevated CVP (ß-coefficient 21.2, SE 5.5, P = 0.0005) and decreased SvO2 (ß-coefficient -92.9, SE 12.4, P < 0.0001). CONCLUSIONS: The HGF is an independent predictor of a failure of a Fontan circulation. The HGF is an indicator for an additional catheter intervention after a Fontan operation.

Comparison of transcatheter patent ductus arteriosus closure between children and adults.

The data comparing the characteristics and effect of transcatheter patent ductus arteriosus (PDA) closure between children and adults is scarce. We analyzed 54 consecutive patients who underwent transcatheter PDA closures. We divided the patients into 2 groups of < 18 years and ≥ 18 years and compared the hemodynamic changes before and after the PDA closure. Adults had a higher incidence of heart failure on admission, diagnoses by heart failure and incidental echocardiography, PDA calcifications, and procedural complications than children (all P < 0.05). The left ventricular end-diastolic volume index (LVEDVI), left atrial diameter index (LADI), and LV mass index (LVMI) decreased after the PDA closure in children but not in adults. The LV ejection fraction (LVEF) significantly decreased 1 day after the PDA closure in both groups but remained low at 6 months after the procedure in only adults. The percent change in the LVEDVI, LADI, LVMI, and LVEF from baseline to 6 months after the procedure was significantly lesser in adults than children (LVEDVI: - 5.2 ± 29.1% vs. - 34.9 ± 18.9%, LADI: - 7.0 ± 13.2% vs. - 22.1 ± 18.9%, LVMI: - 11.0 ± 16.5% vs. - 34.1 ± 15.7%, LVEF: - 5.9 ± 7.6% vs. 6.1 ± 9.1%, all P < 0.05). Transcatheter PDA closure was not associated with a reduction in the LV and LA volume as well as an improvement in the LV hypertrophy and LV function in adults as compared to children. We suggested that an early diagnosis and transcatheter PDA closure during childhood might provide clinical benefit before progressive LV remodeling and heart failure.

Early assessment of cardiac troponin I predicts the postoperative cardiac status and clinical course after congenital heart disease surgery.

Cardiac troponin I (cTnI) is a regulatory protein with a high sensitivity and specificity for cardiac injury. Preoperative and postoperative elevations of cTnI are usually considered predictors of the mortality and morbidity. However, little is known about the relationship between the cTnI and postoperative course after the congenital heart disease (CHD) operation. Sixty-five consecutive patients who underwent cardiac surgery for CHD at our institution between March 2016 and January 2017 were included. The cTnI was measured after the operation. Also, the association between the cTnI and duration of the catecholamine use, ICU stay, aortic cross clamp time, and other clinical parameters were assessed. The cTnI level on postoperative day 1 was positively correlated with the duration of the catecholamine use (p < 0.001) and ICU stay (p < 0.001). Also, a higher cTnI level was associated with a lower urine volume and higher lactate level 24 h after the ICU admission. In the multivariable regression analysis, the cTnI was a significant independent predictor of the catecholamine use (p = 0.002) and ICU stay (p = 0.003). The cTnI level on postoperative day 1 was a predictor of the duration of the catecholamine use and ICU stay.

Predictors of Early Postoperative Supraventricular Tachyarrhythmias in Children After the Fontan Procedure.

Postoperative arrhythmias are a frequent and fatal complication after the Fontan operation. However, clinical evidence demonstrating early postoperative arrhythmias in children undergoing the Fontan operation is limited. This study aimed to evaluate the prevalence of arrhythmias and identify the predictors of early postoperative supraventricular tachyarrhythmias (SVTs) after the Fontan procedure.Data were analyzed from 80 pediatric patients who underwent Fontan procedures between April 2000 and December 2017 in a single-center retrospective study. Early postoperative SVTs were defined as arrhythmias within 30 days after the Fontan procedure. We divided the patients into two groups, with or without early postoperative arrhythmias, and the predictors of early postoperative arrhythmias were analyzed. A multivariate logistic regression analysis was performed to determine independent predictors of early postoperative SVTs after the Fontan procedure.Early postoperative SVTs were observed in 21 patients (26.3%). The most common arrhythmia was junctional ectopic tachycardia. After an adjustment, an atrioventricular valve regurgitation (AVVR) grade of ≥2 (odds ratio 10.54, 95% confidence interval 2.52 to 44.17, P = 0.001) and preoperative arrhythmias (odds ratio 26.49, 95% confidence interval 1.64 to 428.62, P = 0.021) were significant predictors of early postoperative SVTs after the Fontan operation.An AVVR grade ≥2 and preoperative arrhythmia were significant predictors associated with early postoperative SVTs. Intervention for AVVR may provide clinical benefit for preventing early postoperative arrhythmias after the Fontan operation.

Usefulness of Red Blood Cell Distribution Width in the Assessment of Hemodynamics After Tetralogy of Fallot Repair.

BACKGROUND: There are no reports on the effect of red blood cell distribution width (RDW) in surgical repair of tetralogy of Fallot (ToF). Methods and Results: A total of 50 patients who underwent cardiac catheterization after surgical repair of ToF were retrospectively assessed. RDW was positively correlated with the ratio of right ventricular pressure to left ventricular pressure (RVP/LVP; P<0.0001, r2=0.57). Patients with elevated RDW had a higher RVP/LVP than those with a normal RDW (P<0.0001). Also, elevated RDW was related to elevated central venous pressure (P<0.0001), decreased mixed venous oxygen saturation (P<0.0001), greater pulmonary stenosis (P=0.003) and severe pulmonary regurgitation on echocardiography (P<0.0001), a higher rate of residual ventricular septal defect leak (P=0.004) and higher reoperation rate (P=0.009). Of the 7 patients who underwent reoperation, 6 had decrease in RDW after reoperation (P=0.012). On multivariable regression analysis, RDW was the strongest indicator of higher RVP/LVP. CONCLUSIONS: For the first time, RDW has been shown to be a strong indicator for assessing the hemodynamics and risk of later reoperation after surgical repair of ToF.

Successful radiofrequency catheter ablation of life-threatening atrial tachycardia in an infant with asplenia syndrome.

A 1-year-old infant with asplenia syndrome and congenital heart disease consisting of common atrium, common inlet left ventricle with a common atrio-ventricular (AV) valve, pulmonary atresia, and total anomalous pulmonary venous connection was admitted to our hospital for radiofrequency catheter ablation (RFCA) of supraventricular tachycardia (SVT) before total cavo-pulmonary connection. After antiarrhythmic medications were discontinued for RFCA, she suffered from SVT that resulted in the rapid deterioration of hemodynamic status. Antiarrhythmic medications and cardioversion were not effective in terminating SVT. The baseline electrocardiogram confirmed the existence of twin AV nodes; however, this SVT was revealed to be focal atrial tachycardia (AT) with enhanced automaticity. The origin of AT was not related to surgical scar. Emergent RFCA for AT was successful in our case of asplenia syndrome. AT is a life-threatening complication in a single ventricle and delayed treatment can be fatal. It is important to perform RFCA promptly when drug treatment is not effective. We suggest that the AV node is not always the target site for ablation in patients with asplenia syndrome and twin AV nodes. .

The necessity of implantable cardioverter defibrillators in patients with Kearns-Sayre syndrome - systematic review of the articles.

The most common cardiac feature of Kearns-Sayre syndrome (KSS) is atrioventricular block (AVB), and pacemaker implantations (PMIs) are recommended for KSS patients with advanced AVB. However, some KSS patients develop fatal arrhythmias such as polymorphic ventricular tachycardia (PMVT) and ventricular fibrillation (VF) and die suddenly even after PMIs. We report a patient with KSS who developed PMVT, VF, and QT prolongation, and was treated with mexiletine and successfully managed with an implantable cardioverter defibrillator (ICD). We reviewed the literature on arrhythmias in KSS published from 1975 to 2018. There were 112 patients with arrhythmia-associated KSS, 10 died, and 6 died suddenly after the PMI. The first manifestation of an arrhythmia was bundle branch block, then it progressed to AVB, and developed into complete AVB (CAVB) in about half the KSS patients. Ventricular arrhythmias were documented in 12 patients, and 8 were implanted with defibrillators afterwards. One patient after the implantation of a cardiac resynchronization therapy defibrillator (CRT-D) was treated for VF by an appropriate shock. This fact suggested that VF occurred even under proper pacing, and that defibrillators were effective. Pacemakers may suppress early afterdepolarizations (EADs) associated with a QT prolongation due to bradycardia. Similarly, mexiletine may suppress EADs by blocking the late sodium and Ca currents. Ventricular arrhythmias observed under suppression of EADs may be caused by delayed afterdepolarization (DADs) via an increasing intracellular Ca concentration due to mitochondrial dysfunction. Therefore, a PMI alone may not be sufficient to prevent sudden death, and an ICD implantation should be necessary.

Efficacy of catheter interventions in the early and very early postoperative period after CHD operation.

BACKGROUND: Catheter interventions for residual lesions in the early postoperative period after CHD operations are still not established as a reliable treatment option. METHODS: We retrospectively reviewed our institutional experience of cardiac catheterisations and catheter interventions performed in the early postoperative period. We classified our patients into two groups. The "hyper" acute phase group - operation to cardiac catheterisation of ⩽7 days - and acute phase group - operation to cardiac catheterisation from 7 to 30 days. RESULTS: Of the 47 patients, catheter interventions were performed in 38 patients (81%). The success rate of the intervention was 96% in the acute phase group and 90% in the "hyper" acute phase group. The overall success rate was 95%. There were two self-limited complications in the acute phase group, but not in the "hyper" acute phase group. There were four cases of catheter interventions performed for a newly reconstructed aortic arch, and those procedures were also safe and effective. CONCLUSIONS: Cardiac catheterisations and catheter interventions were safe and effective not only in the early postoperative period but also in the very early postoperative period. Catheter interventions for the left-sided heart in the early postoperative period were also safe and effective.

Pathogenesis and Bone Resorption in Acquired Cholesteatoma: Current Knowledge and Future Prospectives.

Cholesteatoma is a cystic non tumorous lesion of the temporal bone that has the ability to destroy nearby structures by its power to cause bone resorption and as a result, fatal complications prevail. We aimed to conduct a comprehensive review for pathogenesis of acquired cholesteatoma, bone resorption mechanisms, and offer a future vision of this serious disease. We have reviewed different theories for pathogenesis of acquired cholesteatoma including the most relevant and updated ones with special emphasis on the mechanisms of bone resorption through Medline/PubMed research using the keywords 'aetiopathogenesis, bone resorption, acquired cholesteatoma, temporal bone, and cytokines.' In order to strengthen our study, we searched the reference lists of identified reviews. Cholesteatoma is a subject of debate among otolaryngologists since it was prescribed firstly. Over many decades, several theories were postulated for aetiopathogenesis of cholesteatoma with a tendency to follow more than one theory to explain the proper nature of that disease. Until now, the mechanism of bone resorption has yet to be more clarified. In the last century, a leap has occurred in the field of biomolecular cholesteatoma research which improved our knowledge about its pathophysiology and bone destructive mechanism. However, surgery is still the only available treatment. We conclude that discovery of new therapeutic choices for cholesteatoma other than surgery by the use of anti-growth, anti-proliferative, apoptotic agents as well as medications that antagonize osteoclastogenesis should be the main concern in the future clinical and experimental research work. Also, searching for predictors of the aggressiveness of cholesteatoma can affect the timing of intervention and prevent occurrence of complications.

Volumes of Velopharyngeal and Glossopharyngeal Airway Were Not Changed after Uvulopalatopharyngoplasty: Report of Three Cases.

Objective. The aim of this study was to investigate the changes in velopharyngeal and glossopharyngeal airway morphology and volume after uvulopalatopharyngoplasty in three adult obstructive sleep apnea syndrome patients who had bilateral large tonsils using three-dimensional computed tomography. Case Report. All three patients (one male and two females) who presented with a history of heavy snoring and excessive daytime sleepiness were examined with overnight nocturnal polysomnography, which indicated moderate-to-severe obstructive sleep apnea syndrome. Because all patients had large tonsils, uvulopalatopharyngoplasty was expected to enlarge the pharyngeal airway. Polysomnography and three-dimensional computed tomography scanning were performed and compared, both before and 3 months after uvulopalatopharyngoplasty. Results. Unexpectedly, although the morphology of the glossopharyngeal airway clearly changed after UPPP, the volume changes in the velopharyngeal and glossopharyngeal airways were negligible.