Atrial flutter/fibrillation in adult congenital heart disease: Insights from a large Asian cohort.

BACKGROUND: The incidence of atrial fibrillation/atrial flutter (AF/AFL) in general population is lower in Asia compared to Western countries. It is unclear whether a similar trend exists among adults with congenital heart disease (ACHD). We determine the profile, risk factors, and impact of AF/AFL in an Asian ACHD cohort. METHODS: We included all ACHD patients diagnosed in an Asia tertiary care center between 2007 and 2018, analyzing AF (sustained and paroxysmal AF) and AFL, collectively./Purpose. RESULTS: The study encompassed 4391 patients (55.9% women), with 81% having simple, 16.3% moderate and 2.8% severe CHD. AF/AFL was observed in 6.7% of the patients, with 54.6% having paroxysmal AF, 27.3% sustained AF, and 18.1% AFL. Incidence of AF/AFL increased with age and was higher in patients with pulmonary hypertension (PH), severe CHD, and metabolic syndrome. We found a progressive trend in the onset age of arrhythmia: AFL at a younger age, followed by paroxysmal and sustained AF. Risk factors for AF/AFL included severe and moderate CHD, PH, previous interventions, and male sex (odds ratio 11.2 and 3.15, 2.03, 1.75, and 1.71, respectively). When stratifying by CHD severity, PH and male sex were significant risk factors in simple CHD, while only PH in severe CHD. Patients with AF/AFL had a significantly lower major adverse cardiovascular events-free survival rate. CONCLUSIONS: This large ACHD cohort from Asia exhibited a high incidence of AF/AFL, similar to Western reports. The risk of AF/AFL was primarily associated with hemodynamic factors such as PH and CHD severity.

Multicenter registry of multisystem inflammatory syndrome in children (MIS-C) and Paired comparison with Kawasaki disease.

OBJECTIVES: This study aimed to identify clinical characteristics to differentiate multisystem inflammatory syndrome in children (MIS-C) and Kawasaki disease (KD) in Taiwan, an island with a delayed cluster of MIS-C and a high incidence of KD. Additionally, we studied risk factors for developing severe complications in patients with MIS-C. METHODS: We conducted a retrospective, multicenter, cohort, and observational study that linked data on patients with MIS-C between May and December 2022 and patients with KD between 2019 and 2021 from 12 medical centers. Hemodynamic compromise, defined as the need for inotropic support or fluid challenge, was recorded in patients with MIS-C. We also evaluated maximal coronary Z-scores before treatment and one month after disease onset. RESULTS: A total of 83 patients with MIS-C and 466 patients with KD were recruited. A 1:1 age and gender-matched comparison of 68 MIS-C and KD pairs showed that MIS-C patients had a lower percentage of positive BCG red halos, lower leukocyte/platelet counts, more gastrointestinal symptoms, and a higher risk of hemodynamic compromise. In Taiwan, 38.6% of MIS-C patients experienced hemodynamic compromise, with presence of conjunctivitis and elevated levels of procalcitonin (>1.62 ng/mL) identified as independent risk factors. CONCLUSIONS: We identified two independent risk factors associated with hemodynamic compromise in MIS-C patients. The comparison between matched MIS-C and KD patients highlighted significant differences in clinical presentations, like BCG red halos, which may aid in the differential diagnosis of the two disease entities, especially in regions with a high incidence rate of KD.

Liver fibrosis detected by diffusion-weighted magnetic resonance imaging and its functional correlates in Fontan patients.

OBJECTIVES: The evaluation of Fontan-associated liver disease is often challenging. Diffusion-weighted magnetic resonance imaging can detect hepatic fibrosis from capillary perfusion and diffusion abnormalities from extracellular matrix accumulation. This study investigated its role in the evaluation of liver disease in Fontan patients and explored possible diagnostic methods for early detection of advanced liver fibrosis. METHODS: Stable adult Fontan patients who could safely be examined with magnetic resonance imaging were enrolled, and blood biomarkers, transient elastography were also examined. RESULTS: Forty-six patients received diffusion-weighted imaging; and 58.7% were diagnosed with advanced liver fibrosis (severe liver fibrosis, 37.0%, and cirrhosis 21.7%). Two parameters of hepatic dysfunction, platelet counts (Spearman's ρ: -0.456, P = 0.001) and cholesterol levels (Spearman's ρ: -0.383, P = 0.009), decreased with increasing severity of fibrosis. Using transient elastography, a cut-off value of 14.2 kPa predicted the presence of advanced liver fibrosis, but with a low positive predictive value. When we included platelet count, cholesterol, post-Fontan years and transient elastography values as a composite, the capability of predicting advanced liver fibrosis was the most satisfactory (C statistic 0.817 ± 0.071, P < 0.001). A cut-off value of 5.0 revealed a sensitivity of 78% and a specificity of 82%. CONCLUSIONS: In Fontan patients, diffusion-weighted imaging was helpful in detecting liver fibrosis that was correlated with hepatic dysfunction. A simple score was proposed for long-term surveillance and early detection of advanced liver disease in adult Fontan patients. For adult Fontan patients with a calculated score > 5.0, we may consider timely diffusion-weight imaging and early management for liver complications.

Risk Factors of Long-Term Sequelae After Transcatheter Closure of Perimembranous Ventricular Septal Defect in Young Children.

BACKGROUND: Complications arising from transcatheter closure of perimembranous ventricular septal defects (pmVSD) in children, such as residual shunts and aortic regurgitation (AR), have been observed. However, the associated risk factors remain unclear. This study identified risk factors linked with residual shunts and AR following transcatheter closure of pmVSD in children aged 2-12 years.Methods and Results: The medical records of 63 children with pmVSD and a pulmonary-to-systemic blood flow ratio <2.0 who underwent transcatheter closure between 2011 and 2018 were analyzed with a minimum 3-year follow-up. The success rate of transcatheter closure was 98.4%, with no emergency surgery, permanent high-degree atrioventricular block, or mortality. Defects ≥4.5 mm had significantly higher odds of persistent residual shunt (odds ratio [OR] 6.85; P=0.03). The use of an oversize device (≥1.5 mm) showed a trend towards reducing residual shunts (OR 0.23; P=0.06). Age <4 years (OR 27.38; 95% confidence interval [CI] 2.33-321.68) and perimembranous outlet-type VSD (OR 11.94, 95% CI 1.10-129.81) were independent risk factors for AR progression after closure. CONCLUSIONS: Careful attention is crucial for pmVSDs ≥4.5 mm to prevent persistent residual shunts in transcatheter closure. Assessing AR risk, particularly in children aged <4 years, is essential while considering the benefits of pmVSD closure.

Combination of slow pathway late activation maps and voltage gradient maps in guidance of atrioventricular nodal reentrant tachycardia cryoablation.

BACKGROUND: The optimal strategy for electroanatomic mapping-guided cryoablation of atrioventricular nodal reentry tachycardia (AVNRT) remains unclear. OBJECTIVE: The purpose of this study was to investigate the effectiveness of slow pathway late activation mapping (SPLAM) and voltage gradient mapping for AVNRT cryoablation. METHODS: From June 2020 to February 2022, all consecutive patients with AVNRT underwent SPLAM to define the wave collision point and voltage gradient mapping to define the low-voltage bridge (LVB). Conventional procedures performed from August 2018 to May 2020 served as control. RESULTS: The study and control groups comprised 36 patients (age 16.5 ± 8.2 years) and 37 patients (age 15.5 ± 7.3 years), respectively. Total procedural times were comparable, and acute success rates were 100% in both groups. Compared to controls, the number of cryomapping attempts (median 3 vs 5; P = .012) and cryoablation applications (median 1 vs 2; P <.001) were significantly lower in the study group. At median follow-up of 14.6 and 18.3 months, recurrence rates were 5.6% (2 patients) and 10.8% (4 patients) in the study and control groups (P = .402), respectively. Mapping of the Koch triangle took 11.8 ± 3.6 minutes, during which 1562 ± 581 points were collected. In SPLAM, wave collision points were defined and compatible with the final successful lesion sites in all patients, including those with multiple slow pathways. LVB could not be defined in 6 patients (16.7%), and LVB was not compatible with the final successful lesion in another 6 (16.7%). CONCLUSION: For AVNRT cryoablation, SPLAM could effectively guide the localization of slow pathway ablation sites and was particularly beneficial in patients with multiple slow pathways.

Self-Expanding Pulmonary Valves in 53 Patients With Native Repaired Right Ventricular Outflow Tracts.

BACKGROUND: Self-expanding pulmonary valve grafts have been designed for percutaneous pulmonary valve implantation (PPVI) in patients with native repaired right ventricular (RV) outflow tracts (RVOTs). However, their efficacy, in terms of RV function and graft remodelling remain unclear. METHODS: Patients with native RVOTs who received Venus P-valve (N = 15) or Pulsta valve (N = 38) implants between 2017 and 2022 were enrolled. We collected data on patient characteristics and cardiac catheterization parameters as well as imaging and laboratory data before, immediately after, and 6 to 12 months after PPVI and identified risk factors for RV dysfunction. RESULTS: Valve implantation was successful in 98.1% of patients. The median duration of follow-up was 27.5 months. In the first 6 months after PPVI, all patients exhibited resolution of paradoxical septal motion and a significant reduction (P < 0.05) in RV volume, N-terminal pro-B-type natriuretic peptide levels, and valve eccentricity indices (-3.9%). Normalization of the RV ejection fraction (≥ 50%) was detected in only 9 patients (17.3%) and was independently associated with the RV end-diastolic volume index before PPVI (P = 0.03). Nine patients had residual or recurrent pulmonary regurgitation or paravalvular leak (graded as ≥ mild), which was associated with a larger eccentricity index (> 8%) and subsided by 12 months postimplantation. CONCLUSIONS: We identified the risk factors likely to be associated with RV dysfunction and pulmonary regurgitation following PPVI in patients with native repaired RVOTs. RV volume-based patient selection is recommended for PPVI of a self-expanding pulmonary valve, along with monitoring of graft geometry.

Comparing Strategies for Critical Congenital Heart Disease Newborn Screening.

OBJECTIVES: An extended newborn critical congenital heart disease (CCHD) screening program using oximetry has been implemented in Taipei, Taiwan since April 2014. This study was conducted to investigate the test accuracy and efficiency of this screening protocol. METHODS: This study analyzed data from 30 birthing facilities representing 87.9% of live births in Taipei. Positive screening was defined as oxygen saturation <95% in either extremity or a preductal-postductal oxygen saturation difference of >3%. This study cohort was used to retrospectively estimate outcomes on the basis of different CCHD screening protocols. RESULTS: During the study period, 93 058 of 94 204 (98.8%) infants who had no prenatal suspicion were screened. The referral rate was 0.17% (156/93 058), and up to 90% of test-positive infants were referred within 48 hours of life. Forty-two CCHD cases without prenatal suspicion were detected and 97.6% were diagnosed within 72 hours of life. Of the screened newborns, 4 CCHD cases passed the screening. The false positive and false negative rates were 0.12% and 0.04%, respectively. In addition, applying our database to Spanish and updated American Academy of Pediatrics screening strategies led to more CCHD case detection. CONCLUSIONS: The Taipei protocol provided an efficient and effective screening referral system in a community setting. For optimal efficiency, we advocated the updated American Academy of Pediatrics algorithm/Spanish recommendation with a modification of immediate referral if oxygen saturation ≤90% in either extremity. The updated protocol would be practicable for nationwide screening in Taiwan and could also be applied to other regions with similar medical care systems.

The implications of airway anomalies on children with congenital heart disease and bronchiolitis.

BACKGROUND: Bronchiolitis is a common airway infection in young children. Hemodynamically significant congenital heart disease (CHD) predicts a more complicated course. However, the role of airway anomalies remains unknown. METHODS: We retrospectively reviewed the records of patients under 2 years old, diagnosed with CHD, and admitted between January 2011 and December 2013, before the palivizumab era. Records of bronchiolitis admissions were also extracted. Patients were grouped according to CHD condition and airway anomalies. RESULTS: A total of 230 patients with CHD were enrolled. A total of 180 (78%) and 71 (31%) patients had hemodynamically significant CHD and airway anomalies, respectively. A total of 52 (22.6%) patients were admitted for bronchiolitis 78 times. Among them, 33 (63.5%) had hemodynamically significant CHD, and 28 (53.8%) had airway anomalies. In patients with bronchiolitis admissions, the mean ventilator use, intensive care unit stay, and hospital stay were 1.08, 4.08, and 15.19 days, respectively. When compared, the mean hospital stay for bronchiolitis patients with airway anomalies was significantly longer than that of those without airway anomalies (19.8 vs. 9.9 days, p = 0.008). When further divided the patients by the presence hemodynamic significance, patients with hemodynamically significant CHD and airway anomaly had longer hospital stay than those who had neither. (21.7 vs. 8.3 days, p = 0.004) Airway anomaly was a significant risk factor for longer hospital stay in linear regression model (p = 0.007). CONCLUSIONS: Airway anomalies are common in children with CHD and are associated with longer hospital stays on bronchiolitis admission. An active survey for airway anomalies and adequate prophylaxis for bronchiolitis infection might be important in the care of children with CHD associated with airway anomalies.

Changes of ECG parameters after BNT162b2 vaccine in the senior high school students.

The purpose of this study is to determine the ECG parameter change and the efficacy of ECG screening for cardiac adverse effect after the second dose of BNT162b2 vaccine in young population. In December 2021, in cooperation with the school vaccination system of Taipei City government, we performed a ECG screening study during the second dose of BNT162b2 vaccines. Serial comparisons of ECGs and questionnaire survey were performed before and after vaccine in four male-predominant senior high schools. Among 7934 eligible students, 4928 (62.1%) were included in the study. The male/female ratio was 4576/352. In total, 763 students (17.1%) had at least one cardiac symptom after the second vaccine dose, mostly chest pain and palpitations. The depolarization and repolarization parameters (QRS duration and QT interval) decreased significantly after the vaccine with increasing heart rate. Abnormal ECGs were obtained in 51 (1.0%) of the students, of which 1 was diagnosed with mild myocarditis and another 4 were judged to have significant arrhythmia. None of the patients needed to be admitted to hospital and all of these symptoms improved spontaneously. Using these five students as a positive outcome, the sensitivity and specificity of this screening method were 100% and 99.1%, respectively.  Conclusion: Cardiac symptoms are common after the second dose of BNT162b2 vaccine, but the incidences of significant arrhythmias and myocarditis are only 0.1%. The serial ECG screening method has high sensitivity and specificity for significant cardiac adverse effect but cost effect needs further discussed. What is Known: • The incidence of cardiac adverse effects was reported to be as high as 1.5 per 10 000 persons after the second dose BNT162b2 COVID-19 vaccine in the young male population based on the reporting system. What is New: • Through this mass ECG screening study after the second dose of BNT162b2 vaccine we found: (1) The depolarization and repolarization parameters (QRS duration and QT interval) decreased significantly after the vaccine with increasing heart rate; (2) the incidence of post-vaccine myocarditis and significant arrhythmia are 0.02% and 0.08%; (3) The serial ECG screening method has high sensitivity and specificity for significant cardiac adverse effect.

Normal ECG standards and percentile charts for infants, children and adolescents.

BACKGROUND: Normal ECG standards in newborns, infants, children and adolescents have been collected and published by many authors. Only those by Davignon et al., Rijinbeek et al. and our two studies covered all ages from birth to adolescence. The standards reflecting the growth and development of the heart in infants, children and adolescents remained to be studied and explored. METHODS: We selected from our ECG database, after discussions and consultation, 15 key ECG parameters and analyzed for their age- and sex-specific mean, standard deviation and 2nd to 98th percentiles and their percentile charts were constructed. RESULTS: The ranges and distributions of the normal ECG standards, means and 2nd to 98th percentiles of 15 key parameters were established. CONCLUSION: A complete set of normal ECG standards of 15 key parameters from birth to adolescents is available for clinicians and researchers.

Management of cardiovascular symptoms after Pfizer-BioNTech COVID-19 vaccine in teenagers in the emergency department.

BACKGROUND: Cardiovascular complications after Pfizer-BioNTech COVID-19 (BNT) vaccination are a concern, especially in adolescents. We analyzed the risk factors for myocarditis after BNT vaccination. METHODS: We used a special evaluation protocol for all patients aged 12-18 years who presented to our emergency department with cardiovascular symptoms after BNT vaccination. RESULTS: A total of 195 patients (109 boys and 86 girls) were enrolled. Eleven (5.6%) patients presented with arrhythmia (arrhythmia group), 14 (7.2%) had a diagnosis of pericarditis/myocarditis (the peri/myocarditis group), and the remaining 170 were controls (no cardiac involvement). Chest pain (77.6%) was the most common symptom. The median time from vaccination to symptom onset was 3 days. In the peri/myocarditis group (13 myocarditis and 1 pericarditis), the median time to the peak troponin T level was 5 days after vaccination. Abnormal electrocardiographic changes, including ST-T changes and conduction blocks, were more commonly detected in the peri/myocarditis group (85.7% vs. 12.4% in the control group, p < 0.01). Echocardiography revealed normal ventricular function in all patients. Symptoms were resolved before discharge in all, with the median duration of hospital stay being 4 days. The electrocardiography was the most appropriate screening tool for myocarditis, with a sensitivity and specificity of 85.7% and 87.6%, respectively. CONCLUSION: Pericarditis or myocarditis was diagnosed in 7.2% of adolescents presenting to the emergency department with cardiovascular symptoms after BNT vaccination. In addition to the troponin T level, ECG change listed above can be used as a screening tool for vaccine-induced cardiac complications.

Outcomes of pediatric patients supported with ventricular assist devices single center experience.

BACKGROUND: There has been a remarkable increase in the number of pediatric ventricular assist device (VAD) implanted over the past decade. Asian pediatric heart centers had not participated in the multicenter registries among the Western countries. This article aimed to report the outcomes of pediatric VAD in our hospital. METHODS: The study enrolled all patients aged <18 years at the time of VAD implantation in our institution between 2008 and 2021. RESULTS: There were 33 patients with diagnosis of acute fulminant myocarditis (n = 9), congenital heart disease (n = 5), dilated cardiomyopathy (n = 16), and others. Paracorporeal continuous-flow pump was the most frequently implanted (n = 27). Most of the devices were implanted in patients with INTERMACS profile 1 (n = 24). The median duration on VAD was 22 days (range 2-254). The proportion of patients attaining positive outcomes (alive on device, bridge to transplantation or recovery) was 72.7% at 1 month, 67.7% at 3 months, and 67.7% at 6 months. Most of the deaths on device occurred within the first month post-implant (n = 9), with neurological complications being the most frequent cause of death. All recovered cases were successfully weaned off the device within the first month of implantation. CONCLUSION: We demonstrated a favorable outcome in pediatric patients supported with VAD at our institution.

Zero-fluoroscopy ablation of left-sided arrhythmia substrates in children - Mid-term safety and feasibility study from transaortic approach.

BACKGROUND: A widely used method of treating left-sided arrhythmia substrates in children is retrograde transaortic ablation under fluoroscopic guidance. However, the feasibility, safety, and efficacy of this approach under zero fluoroscopy (ZF) guidance, especially the mid-term safety of anatomy and function of aortic valves, have yet to be proven. METHODS: All consecutive patients who received ablation of left-sided arrhythmias between January 2012 and June 2020 and below 20 years-old were enrolled. The study group submitted to 55 ZF-guided procedures using cardiac mapping system (EnSite Precision), whereas 49 procedures were performed under fluoroscopic guidance in the control group. Echocardiographic studies took place before and 6-months after ablative procedures. RESULTS: One-hundred-and-two patients (male, 66; female, 36) underwent a total of 104 ablative procedures. Mean procedural durations were 83.9 ± 44.4 min in the study group and 64.8 ± 29.1 min in the control group, respectively (p = .01; the 95% confidence interval, -33.57 to -4.63). Corresponding fluoroscopic times were .5 ± 2.2 min and 24.7 ± 13.9 min (p < .001; the 95% confidence interval, 20.15 to 28.22). ZF may be reasonably applied after a learning curve of 20 cases. Immediate procedural success and recurrence rates were similar in each groups. There was no detectable progression of aortic regurgitation in any of the patients during serial follow-up of echocardiography. CONCLUSION: ZF-guided retrograde transaortic ablation of left-sided arrhythmia substrates proved safe in children at midterm follow-up, reducing radiation exposure significantly within a learning curve of <20 cases.

Office Blood Pressure Monitoring in Children with Obesity and Obstructive Sleep Apnea.

OBJECTIVE: To investigate the relative contributions of obesity and obstructive sleep apnea (OSA) to unfavorable blood pressure in children. STUDY DESIGN: Children aged 3-18 years with OSA-related symptoms were recruited. All children underwent office blood pressure (BP) monitoring and full-night polysomnography. Obesity was defined as a body mass index ≥95th percentile. OSA severity was divided into primary snoring (apnea-hypopnea index [AHI] <1), mild OSA (5> AHI ≥1), and moderate to severe OSA (AHI ≥5). Age- and sex-adjusted logistic regression analysis was performed to determine the associations among OSA, obesity, and elevated BP. RESULTS: This cross-sectional study enrolled 1689 children (66% boys), with a mean age of 7.9 years. Compared with children with primary snoring, children with moderate to severe OSA had significantly higher systolic BP (108.1 mmHg vs 105.6 mmHg), diastolic BP (75.0 mmHg vs 70.4 mmHg), systolic BP percentile (75.0 vs 70.4), and diastolic BP percentile (74.0 vs 69.2). The rate of unfavorable BP (ie, elevated BP or hypertension level BP) also was significantly higher in children with more severe OSA. Children with obesity had higher BP and BP percentile. Logistic regression analysis revealed that children with obesity and moderate to severe OSA have a 3-fold greater risk of unfavorable BP compared with children without obesity and primary snoring. CONCLUSIONS: We identified a 3-fold greater risk of unfavorable BP in children with obesity and moderate to severe OSA.

Pulmonary Hypertension in Adult Congenital Heart Disease in Asia: A Distinctive Feature of Complex Congenital Heart Disease.

Background The epidemiology of pulmonary hypertension (PH) in patients with adult congenital heart disease in Western countries is already known. We investigate clinical characteristics of PH in adult congenital heart disease with emphasis on complex congenital heart disease (CHD) from an Asian cohort in Taiwan. Methods and Results All adult patients (aged >18 years) diagnosed with CHD between January 2007 and July 2018 qualified for the study. PH was determined by cardiac catheterization data or echocardiography reports. In accord with the World Symposia on Pulmonary Hypertension, CHD was further categorized as simple, severe, or complex CHD (including pulmonary atresia-ventricular septal defect and single-ventricle anomalies). There were 4301 patients (55.6% women), 15.7% with severe and 3.9% with complex CHD. The cumulative incidence of PH was 4.4% (95% CI, 3.8-5.0). Our multivariable regression model indicated 4.2-fold mortality increase (95% CI, 3.0-5.9) in the presence of PH, with age, female sex, and severe or complex CHD linked to higher incidence of PH. Only 49% of patients received PH-specific therapy. Five- and 10-year survival rates of patients with PH (n=190) were 72.3% (95% CI, 65.1%-78.4%) and 58.8% (95% CI, 50.1%-66.5%), respectively. Survival rates in those with Eisenmenger syndrome, PH after defect correction, and complex CHD were similar. Low oxygen saturation and high uric acid levels were associated with increased mortality. Conclusions In this sizable Asian adult CHD cohort, the cumulative incidence of PH was aligned with that of Western countries. Mortality proved higher in patients with PH versus without PH. Although complex CHD carried greater risk of PH compared with other adult CHD subsets, survival rate was similar.

Ambulatory Blood Pressure Variability after Adenotonsillectomy in Childhood Sleep Apnea.

OBJECTIVE: To investigate the influence of adenotonsillectomy (T&A) on ambulatory blood pressure (BP) variability in children with obstructive sleep apnea (OSA). STUDY DESIGN: Prospective, interventional study. METHODS: Children with OSA symptoms were recruited from a tertiary center. After OSA diagnosis was confirmed (ie, apnea-hypopnea index [AHI] > 1), these children underwent T&A for treatment. We performed polysomnography and 24-hour recordings of ambulatory BP before and 3 to 6 months postoperatively. Ambulatory BP variability was presented as the standard deviation of mean blood pressure in the 24-hour monitoring of ambulatory BP. Differences in BP variability among different subgroups were tested using a multivariable linear mixed model. RESULTS: A total of 190 children were enrolled (mean age: 7.8 ± 3.3 years; 73% were boys; 34% were obese). The AHI significantly decreased from 12.3 ± 17.0 to 2.7 ± 5.5 events/hr after T&A. Overall, daytime, and nighttime ambulatory BP did not significantly change postoperatively, and overall, daytime, and nighttime ambulatory BP variability did not differ significantly preoperatively and postoperatively. In the subgroup analysis, children aged <6 years demonstrated a significantly greater decrease in ambulatory BP variability postoperatively than those aged >6 years (nighttime diastolic BP variability: 9.9 to 7.7 vs. 8.9 to 9.4). Children with hypertension also showed a significantly greater decrease in ambulatory BP variability than those without hypertension. CONCLUSIONS: We concluded that overall ambulatory BP variability does not significantly change after T&A in children with OSA. Moreover, young-aged and hypertensive children demonstrate a significant decrease in BP variability after T&A. LEVEL OF EVIDENCE: 4 Laryngoscope, 132:2491-2497, 2022.

Preoperative N-terminal pro-brain natriuretic peptide is associated with Fontan outcomes.

OBJECTIVE: The role of preoperative N-terminal pro-brain natriuretic peptide level in patient outcomes after the Fontan operation remains unclear. METHODS: The medical records of all patients who underwent their first Fontan operation from June 2011 to October 2019 in our tertiary referral pediatric cardiac center were retrospectively reviewed. Preoperative hemodynamic factors and N-terminal pro-brain natriuretic peptide were analyzed to test the association of mortality and morbidity. RESULTS: We enrolled 110 patients (men/women 62/48; median age, 4.1 [3.4, 5.8] years; median follow-up period, 4.28 [2.31, 6.71] years). Almost all operations were extracardiac conduits (98.2%). Primary outcomes of death, Fontan takedown, and heart transplantation were observed in 9 patients (8.2%). Abnormal ventricular contractility, elevated preoperative pulmonary artery pressure, high pulmonary vascular resistance index, and high log10 N-terminal pro-brain natriuretic peptide level were associated with poor outcomes. SECONDARY OUTCOMES: atrioventricular valve regurgitation moderate or greater, elevated pulmonary artery pressure, high pulmonary vascular resistance index, and high log10 N-terminal pro-brain natriuretic peptide level were associated with rehospitalization due to heart failure. Multivariable Cox regression analysis revealed that log10 N-terminal pro-brain natriuretic peptide was the only significant predictor of all primary and secondary outcomes. A scoring system including factors of pulmonary artery pressure, pulmonary vascular resistance index, and N-terminal pro-brain natriuretic peptide was established, and the risk stratification is associated with outcomes after the Fontan operation. CONCLUSIONS: High preoperative N-terminal pro-brain natriuretic peptide was associated with poor outcomes after the Fontan operation.