Trajectories of oxygen saturation within 6-72 hours after birth in neonates at moderate altitude: a prospective longitudinal cohort study.

BACKGROUND: Trajectories of pulse oxygen saturation (SpO2) within the first few days after birth are important to inform the strategy for identifying asymptomatic hypoxemic disease but remain poorly substantiated at higher altitudes. METHODS: We performed a longitudinal cohort study with consecutive neonates at a local hospital in Luchun County, China, at an altitude of 1650 m between January and July 2020. We repeatedly measured the pre- and post-ductal SpO2 values at 6, 12, 18, 24, 36, 48, and 72 hours after birth for neonates without oxygen supplements. All neonates underwent echocardiography and were followed up to 42 days after discharge. We included neonates without hypoxemic diseases to characterize the trajectories of SpO2 over time using a linear mixed model. We considered the 2.5th percentile as the reference value to define hypoxemic conditions. RESULTS: A total of 1061 neonates were enrolled. Twenty-five had non-cardiac hypoxemic diseases, with 84% (21/25) presenting with abnormal SpO2 within 24 hours. One had tetralogy of Fallot identified by echocardiography. Among the 1035 asymptomatic neonates, SpO2 values declined from 6 hours after birth, reached a nadir at 48 hours, and tended to level off thereafter, with identical patterns for both pre- and post-ductal SpO2. The reference percentile was 92% for both pre- and post-ductal SpO2 and was time independent. CONCLUSIONS: A decline within 48 hours features SpO2 trajectories within the first 72 hours at moderate altitude. Our findings suggest that earlier screening may favorably achieve a benefit-risk balance in identifying asymptomatic hypoxemic diseases in this population.

Peripheral perfusion index in well newborns at 6 to 72 h of life at different altitudes: a multi-center study in China.

The purpose of this study is to obtain the reference range of peripheral perfusion index (PPI) of asymptomatic well newborns at 6 to 72 h of life at different altitudes. A population-based prospective cohort study was conducted in cities at different altitudes in China. Asymptomatic well newborns were enrolled consecutively from six hospitals with an altitude of 4 to 4200 m between February 1, 2020, and April 15, 2021. PPI was measured at 6, 12, 24, 48, and 72 h after birth on the right hand (pre-ductal) and either foot (post-ductal) using a Masimo SET Radical-7 oximeter. Fiftieth percentile reference curves of the pre- and post-ductal PPI values at 6-72 h after birth were generated using the Lambda Mu Sigma method. Linear mixed-effects regression was performed to determine the influence of different altitude levels on PPI values over different measurement time points. A total of 4257 asymptomatic well newborns were recruited for analysis. The median and quartile pre- and post-ductal PPI values at 6-72 h of life at different altitudes were 1.70 (1.20, 2.60) and 1.70 (1.10, 2.70) for all infants, 1.30 (1.10, 1.90) and 1.10 (0.88, 1.80) for infants at low altitude, 1.40 (1.00, 2.00) and 1.30 (0.99, 2.00) at mild altitudes, 1.90 (1.30, 2.50) and 1.80 (1.20, 2.70) at moderate altitudes, 1.80 (1.40, 3.50) and 2.20 (1.60, 4.30) for high altitudes, 3.20 (2.70, 3.70), and 3.10 (2.10, 3.30) for higher altitudes, respectively. Overall, both pre- and post-ductal PPI increased with altitude. The 50th percentile curves of pre- and post-ductal PPI values in well newborns at mild, low, moderate, and high altitudes were relatively similar, while the difference between the PPI curves of infants at higher altitudes and other altitudes was significantly different.  Conclusions: With the increase of altitude, pre- and post-ductal PPI of newborns increases. Our study obtained the PPI reference values of asymptomatic well newborns at 6 to 72 h after birth at different altitudes from 4 to ≥ 4000 m. What is Known: • Monitoring hemodynamics is very important to neonates. As an accurate and reliable hemodynamic monitoring index, PPI can detect irreversible damage caused by insufficient tissue perfusion and oxygenation early, directly, noninvasively, and continuously. What is New: • Our study obtained the PPI reference values of asymptomatic well newborns at 6 to 72 h after birth at different altitudes from 4 to ≥ 4000 m. With the increase of altitude, pre- and post-ductal PPI of newborns increase with statistical significance. Therefore, the values and disease thresholds of PPI for asymptomatic neonates should be modified according to altitudes.

Mid-aortic syndrome: a rare cause of heart failure in infants.

This case reports describe a rare disease, mid-aortic syndrome (MAS), that can cause severe heart failure and hypertension in infancy. The typical images, key points of diagnosis, and therapy methods of the disease have also been presented. We report two critical thoracoabdominal aortic coarctation cases in infants aged 2 and 11 months with severe heart failure. The patients were initially misdiagnosed as dilated myocardiopathy, with the correct diagnosis confirmed through imaging. Both patients underwent balloon angioplasty; one patient also had bare-metal stents implanted. The patient treated with balloon angioplasty alone died after the procedure, whereas the other patient recovered well. In conclusion, careful physical examinations, especially upper and lower extremity blood pressure differences and palpation of upper and lower limb pulses, are critical in unexplained infant heart failure cases. Stent implantation may be a safer and more effective treatment than simple balloon angioplasty in infants with MAS.

Case Report: Recurrent Left Posterior Fascicular Ventricular Tachycardia in a Newborn.

Left posterior fascicular ventricular tachycardia (LPFVT) is extremely rare in neonates. We described a 17-day-old girl with LPFVT who was initially misdiagnosed as supraventricular tachycardia (SVT). Eventually, she was successfully treated by amiodarone infusion followed by oral amiodarone with propranolol for 9 months, and LPFVT spontaneously resolved after a 1-year follow-up. This case report illustrated the basic principles and caveats in differential diagnosis of LPFVT in the neonatal age group. With proper diagnosis and therapy, neonatal LPFVT might regress in the first year of life.

Case Report: Lessons Learned From Aortic Valve Rupture After Blunt Chest Trauma.

Aortic valve rupture (AVR) due to blunt chest trauma is extremely rare in the pediatric population, and little attention has been paid to such damages. Early diagnosis of AVR may not be easy in patients with multiple competing injuries and poor acoustic windows. We report a case of delayed diagnosis of AVR in a 12-year-old boy after falling from a height of 15 meters, who presented with recurrent hemoptysis and ventilator dependence. This rare case highlights the importance of performing transesophageal echocardiography in trauma patients when the images of transthoracic echocardiography are suboptimal, especially for those presenting with signs and symptoms suggestive of heart failure. The overall prognosis of aortic valve replacement is good.

Characteristics and trends in diagnosis of Kawasaki disease outside the usual age range.

OBJECTIVE: To explore the trends in diagnosis of Kawasaki disease (KD) and determine the characteristics for patients outside the usual age range of 6 months to 4 years. METHOD: A retrospective review of patients with KD identified in four epidemiological surveys spanning 20 years (1998-2017) in Shanghai was performed. RESULTS: A total of 8416 patients were included. All in all, 223 (2.6%) were aged 0 to 2 months, 639 (7.6%) were 3 to 5 months, 6556 (77.9%) were 6 months to 4 years, 915 (10.9%) were 5 to 9 years, and 83 (1.0%) were ≥ 10 years. The use of intravenous immunoglobulin increased in all extreme age groups, and delayed treatment rates decreased in all patients except in those aged 0 to 2 months and ≥ 10 years. The number of patients outside the usual age increased over time, but the proportion of these patients did not change significantly. They had more incomplete KD and coronary artery aneurysms (CAA), while those aged 0 to 2 months and ≥ 10 years had more delayed diagnoses in their respective age groups. The incidence of CAA was similar in younger and older children, but the former group had more cases of incomplete KD and abnormal laboratory parameters, while the latter group had longer duration of fever and was more difficult to diagnose promptly. CONCLUSIONS: The proportion of KD outside the usual age range did not increase over time. Older and younger children have different clinical and laboratory characteristics. Key Points • The number of patients outside the usual age increased over time, but the proportion of these patients did not increase. • Patients aged 0 to 2 months and ≥ 10 years had more delayed diagnoses in their respective age groups. • Younger children had more cases of incomplete KD and abnormal laboratory parameters, while older children had longer duration of fever and were more difficult to diagnose promptly.

Neonatal Kawasaki disease with multiple arterial aneurysms: a case report.

BACKGROUND: Kawasaki disease (KD) is a medium vessel vasculitis that typically occurs in children aged between 6 months and 5 years. It is extraordinarily rare in the neonatal period. KD-related systemic artery aneurysms (SAAs) have never been reported in neonates. CASE PRESENTATION: A male infant was transferred to our institution for persistent high-grade fever lasting 16 days. Symptoms started at day 14 of life, and he was admitted to a children's hospital on the second day of fever. Physical examination at the time found no signs suggestive of KD. The only laboratory parameters which were of significance were values suggestive of systemic inflammation. However, his fever persisted and inflammatory markers continued to rise despite 2 weeks of antibiotic therapy. KD as a noninfectious cause of fever was considered when he came to our institution, and echocardiographic findings of left and right medium coronary artery aneurysms (CAAs) confirmed our suspicions. Full-body magnetic resonance angiography also revealed bilateral axillary artery aneurysms. Administration of intravenous gamma globulin resulted in rapid improvement. His fever resolved on the next day and CAAs and SAAs regressed to normal at 6 months and 3 months after diagnosis, respectively. CONCLUSION: This unique case of incomplete KD highlights the importance of considering KD in neonates with unexplained prolonged fever and reinforces the need to remain vigilant for SAAs in KD.

Bronchial Artery Embolization in Pediatric Pulmonary Hemorrhage: A Single-Center Experience.

PURPOSE: To explore the safety and effectiveness of bronchial artery (BA) embolization (BAE) in children with pulmonary hemorrhage. MATERIALS AND METHODS: Between February 2016 and February 2019, 41 patients (median age, 4 y; interquartile range, 2.3-8 y; median weight, 17.6 kg; interquartile range, 12.3-23.6 kg) underwent BAE. The indication of BAE included massive hemoptysis in 10 patients (24.4%), recurrent hemoptysis in 18 patients (43.9%), and refractory anemia in 13 patients (31.7%). The main etiology of pulmonary hemorrhage included pulmonary hemosiderosis (58.5%), congenital heart disease (17.1%), and infection (14.6%). A retrospective review was conducted of clinical outcomes of BAE. RESULTS: There were 44 embolization sessions, with a total of 137 embolized vessels. Pulmonary hemorrhage was caused by BAs in 30 cases, nonbronchial systemic arteries plus BAs in 10, and nonbronchial systemic arteries in 1. Embolic particles were used in 30 cases (24 polyvinyl alcohol [PVA] and 6 microsphere), coils in 9 cases, and particles plus coils in 5 cases (4 PVA and 1 microsphere). Technical success (ability to embolize abnormal vessel) was achieved in 97.6% of patients (40 of 41), and clinical success (complete or partial resolution of hemoptysis within 30 days of embolization) was achieved in 90.2% (37 of 41). There was 1 procedure-related complication (2.4%) of cerebral infarction and 1 death from multiple-organ dysfunction (2.4%). Bleeding-free survival rates at 6, 12, 24, and 36 months were 92.5%, 83.9%, 83.9%, and 70.8%, respectively. CONCLUSIONS: BAE is a safe and effective procedure in children with pulmonary hemorrhage.

Peripheral perfusion index percentiles for healthy newborns by gestational age and sex in China.

Peripheral perfusion index (PPI) percentiles for newborns serve as an important observation tool in clinical practice, but research pertaining to reference ranges are lacking. The aim of this study was to establish PPI percentiles for healthy newborns by gestational age and sex at 24-48 hours of life. We conducted an observational study and examined PPI values at 24-48 hours of life in 3814 asymptomatic newborns born between 35 and 41 weeks gestation who did not need medical treatment from June 1, 2016 to May 31, 2017 at two maternity hospitals in Shanghai. Linear regression analysis was carried out on the associations between PPI values and variables such as gestational age, sex, and birthweight. Pre-ductal PPI values linearly increased with gestational age (ß: 0.072; 95% CI: 0.037, 0.107; P = 0.000). Post-ductal PPI values were also mainly related to gestational age (ß: 0.051; 95% CI: 0.018, 0.085; P = 0.003). Smoothed reference curves for pre- and post-ductal PPI values by gestational age and sex were derived from LMS Chart Maker. Our study is the first study to establish PPI percentiles curves for healthy newborns by gestational age and sex at 24-48 hours of life. Further research is required for the implementation of PPI curves into clinical practice.

Systemic Artery Aneurysms and Kawasaki Disease.

BACKGROUND: Coronary artery aneurysms (CAAs) are a well-known complication of Kawasaki disease (KD), but there are no data on incidence or outcomes of systemic artery aneurysms (SAAs) in the current era. METHODS: From April 1, 2016, to March 31, 2019, we screened for SAAs in 162 patients with KD at risk for SAAs with magnetic resonance angiography or peripheral angiography and analyzed incidence and early outcomes of SAAs. RESULTS: Twenty-three patients had SAAs, demonstrating an incidence of 14.2% (23 of 162) in patients who were screened at 1 month after onset. The proportion of patients with SAAs was estimated to be 2% (23 of 1148) of all patients with KD. The median age at onset of KD with SAAs was 5 months. All patients with SAAs had CAAs, with z scores >8. Of patients with giant CAAs, 38.6% (17 of 44) had SAAs. A total of 129 SAAs occurred in 17 different named arteries. The most common sites for SAAs were the axillary (18.6%), common iliac (12.4%), and brachial (11.6%) arteries. During a median follow-up time of 6 months, 92.9% (79 of 85) of SAAs had some degree of regression, with 80% (68 of 85) of SAAs returning to normal. The overall regression rate was higher for medium to large SAAs than for medium to giant CAAs. CONCLUSIONS: Although the incidence of SAAs may not be as dramatically reduced as we expected compared with previous data, SAAs have a high regression rate during short-term follow-up.

Apical hypertrophic cardiomyopathy: an uncommon cause of exertional chest pain in children.

Apical hypertrophic cardiomyopathy is an uncommon morphologic variant of hypertrophic cardiomyopathy, which is rarely diagnosed in childhood. To date, very few cases of asymptomatic children younger than 18 years have been reported in the literature. To the best of our knowledge, this is the first case of paediatric apical hypertrophic cardiomyopathy presenting with exertional chest pain, with characteristic electrocardiographic, echocardiographic, MRI, and cardiac angiography findings.

Spontaneous Closure Rates of Ventricular Septal Defects (6,750 Consecutive Neonates).

Although ventricular septal defect (VSD) is a common and simple congenital heart disease in newborns, its true incidence and spontaneous closure (SC) rate remains topics of controversy. This study aims to provide data on the true incidence and SC rate of VSD in the Chinese neonatal population. We conducted a prospective study at 3 hospitals, all newborns underwent echocardiography. Those with a diagnosis of isolated VSD were included in the study group and underwent a 7-year follow-up period. In 6,750 newborns, VSDs were detected in 113 cases (incidence rate of 16.7%), accounting for 62.8% of congenital heart disease, of which 35 were perimembranous (5.2%), 72 were muscular (10.7%), and 6 were doubly committed juxta-arterial (0.9‰). During the 7-year follow-up period, 18 cases required surgical or transcatheter closure. The SC rate in those with perimembranous VSD and muscular VSD (mVSD) were 51.4% (18 of 35) and 97.2% (70 of 72), respectively. Excluding doubly committed juxta-arterial, perimembranous site and defects ≥4 mm are risk factors for VSD that do not spontaneously close. Independent predictive factors for perimembranous VSD which do not spontaneously close is defects ≥4 mm. There was no significant difference in the SC rate at different times between the 4 mVSD sites. In conclusion, this study provides the true incidence and SC rate for Chinese newborns with VSD.

Accuracy of cardiac auscultation in detection of neonatal congenital heart disease by general paediatricians.

BACKGROUND: Challenges remain in the judgement of pathological murmurs in newborns at maternity hospitals, and there are still many simple major CHD patients in developing countries who are not diagnosed in a timely fashion. This study aimed to evaluate the accuracy of cardiac auscultation on neonatal CHD by general paediatricians. METHODS: We conducted a prospective study at three hospitals. All asymptomatic newborns underwent auscultation, pulse oximetry monitoring, and echocardiography. Major CHD was classified and confirmed through follow-up. We evaluated the accuracy of various degrees of murmurs for detecting major CHD to determine the most appropriate standards and time of auscultation. RESULTS: A total of 6750 newborns were included. The median age of auscultation was 43 hours. Cardiac murmurs were identified in 6.6% of newborns. For all CHD, 44.4% had varying degrees of murmurs. A murmur of grade ≥2 used as a reference standard for major CHD had a sensitivity of 89.58%. The false positive rate of murmurs of grade ≥2 for detecting major CHD was significantly negatively related to auscultation time, with 84.4% of false positives requiring follow-up for non-major CHD cardiac issues. Auscultation after 27 hours of life could reduce the false positive rate of major CHD from 2.7 to 0.9%. CONCLUSIONS: With appropriate training, maternity hospital's paediatricians can detect major CHD with high detection rates with an acceptable false positive rate.

Prevalence of Congenital Heart Disease at Live Birth in China.

OBJECTIVE: To investigate the prevalence of congenital heart disease (CHD) in China based on a large prospective multicenter screening study. STUDY DESIGN: A total of 122 765 consecutive infants born at 18 hospitals throughout China between August 1, 2011, and November 30, 2012, were included. Cases of CHD were identified by echocardiography, clinical assessment, and telephone follow-up. RESULTS: The overall prevalence of CHD was 8.98 per 1000 live births (critical, 1.46; serious, 1.47; significant, 5.00; nonsignificant, 1.07), including 7.15 in male infants and 11.11 in female infants. The most common CHD was ventricular septal defect (3.3), followed by atrial septal defect (1.7), patent ductus arteriosus (0.78), pulmonary stenosis (0.73), tetralogy of Fallot (0.47), and transposition of the great arteries (0.35). Female predominance was observed for all CHD and mild CHD (significant and nonsignificant), and male predominance was observed for the critical CHDs. The proportion of preterm newborns was substantially higher among the major CHD cases (critical and serious) compared with normal newborns. There were appreciably more low birth weight infants among the critical CHD cases compared with normal newborns. Significantly higher rates of ventricular septal defect and atrioventricular septal defect were found in infants born to mothers aged ≥35 years. Extracardiac anomalies were found in 9.3% of CHD cases. The risk of CHD was increased by approximately 3-fold when a first-degree relative had CHD. CONCLUSIONS: Our estimates are concordant with data from Western studies. This screening study may provide more accurate and complete information on the overall prevalence of CHD in China.

[Clinical effect of endovascular embolization in treatment of hemoptysis of systemic arterial origin in children].

OBJECTIVE: To investigate the clinical effect of endovascular embolization (EVE) in the treatment of hemoptysis of systemic arterial origin in children. METHODS: A total of 20 children with hemoptysis of systemic arterial origin who underwent EVE from January 2016 to November 2017 were enrolled. The method for embolization was analyzed and the clinical outcome was evaluated. RESULTS: Offending vessels were bronchial artery (BA) in 14 children, non-bronchial systemic artery (NBSA) in 1 child, and BA and NBSA in 5 children. Of all the children, 13 underwent EVE with peripheral embolization agents and 7 underwent EVE with mechanical coils. A total of 41 offending vessels were embolized (34 BAs and 7 NBSAs) and all the children achieved immediate arrest of hemoptysis. Two children experienced recurrence within 6 months after EVE and 2 experienced recurrence with 6-24 months after EVE. The peripheral embolization agent group had a lower overall recurrence rate than the mechanical coil group [8%(1/13) vs 43%(3/7); P=0.10]. One child experienced intracranial ectopic embolism after surgery and had good quality of life during 20 months of follow-up after treatment. No other complications were observed. CONCLUSIONS: EVE is a safe and effective method for the treatment of hemoptysis of systemic arterial origin in children and thus holds promise for clinical application.

Pulse Oximetry and Auscultation for Congenital Heart Disease Detection.

OBJECTIVES: Pulse oximetry (POX) has been confirmed as a specific screening modality for critical congenital heart disease (CCHD), with moderate sensitivity. However, POX is not able to detect most serious and critical cardiac lesions (major congenital heart disease [CHD]) without hypoxemia. In this study, we investigated the accuracy and feasibility of the addition of cardiac auscultation to POX as a screening method for asymptomatic major CHD. METHODS: A multicenter prospective observational screening study was conducted at 15 hospitals in Shanghai between July 1, 2012, and December 31, 2014. Newborns with either an abnormal POX or cardiac auscultation were defined as screen positive. All screen-positive newborns underwent further echocardiography. False-negative results were identified by clinical follow-up, parents' feedback, and telephone review. We assessed the accuracy of POX plus cardiac auscultation for the detection of major CHD. RESULTS: CHD screening was completed in all 15 hospitals, with a screening rate of 94.0% to 99.8%. In total, 167 190 consecutive asymptomatic newborn infants were screened, of which 203 had major CHD (44 critical and 159 serious). The sensitivity of POX plus cardiac auscultation was 95.5% (95% confidence interval 84.9%-98.7%) for CCHD and 92.1% (95% confidence interval 87.7%-95.1%) for major CHD. The false-positive rate was 1.2% for detecting CCHD and 1.1% for detecting major CHD. CONCLUSIONS: In our current study, we show that using POX plus cardiac auscultation significantly improved the detection rate of major CHD in the early neonatal stage, with high sensitivity and a reasonable false-positive rate. It provides strong evidence and a reliable method for neonatal CHD screening.

Pulse oximetry could significantly enhance the early detection of critical congenital heart disease in neonatal intensive care units.

AIM: Limited data have been available regarding critical congenital heart disease (CHD) screening in neonatal intensive care unit (NICUs). This study evaluated the feasibility of screening for CHD by adding pulse oximetry (POX) to clinical evaluation in a NICU in Shanghai, China. METHODS: We screened 4128 eligible consecutive NICU admissions using POX plus clinical evaluation. Infants with positive screening results were then evaluated with echocardiography. Those with negative screening results were put under observation, and they also underwent echocardiography if their oxygen saturation fell below 95% on room air during hospitalisation. RESULTS: This enhanced procedure detected 19 critical CHD cases, and seven of these diagnoses would have been delayed if POX had not been incorporated into the screening strategy. This means that the addition of POX increased the detection rate of critical CHD from 63.2 to 100%. The false-positive rate of critical CHD screening using POX plus clinical evaluation was higher in NICU patients with high morbidity rates. CONCLUSION: When pulse oximetry screening was added to clinical evaluation, it increased the number of critical CHD cases that were detected in our NICU. This method could provide a useful screening protocol for critical CHD cases.