Control of blood pressure in hypertensive children and adolescents assessed by ambulatory blood pressure monitoring.

BACKGROUND: There have been few studies evaluating the control of hypertension (HT) in children. This study aimed to assess the control of HT using ambulatory blood pressure monitoring (ABPM) and to compare the parameters between the uncontrolled HT and controlled HT groups. METHODS: Hypertensive patients aged ≥ 5 years who underwent ABPM to assess the control of HT were enrolled. Demographics, office blood pressure (BP), ABPM, and echocardiographic data were collected. Controlled HT was defined using a BP goal recommended by the 2016 European Society of Hypertension guidelines. RESULTS: There were 108 patients (64.8% males) with a mean age of 14.3 years and 51.9% had primary HT. Controlled HT was detected in 41.1% and 33.3% by office BP and ABPM, respectively. Based on ABPM, there was a greater prevalence of controlled HT in the primary HT than the secondary HT group (44.6% vs. 21.2%, P = 0.01). In the primary HT group, BMI z-score at the last follow-up had a significant decrease in the controlled HT than the uncontrolled HT group (-0.39 vs. 0.01, P = 0.032). Primary HT was negatively associated with uncontrolled HT by ABPM. In addition, ABPM showed greater sensitivity (77.8% vs. 55.8%) and negative predictive value (80.9% vs. 70.8%) to predict LVH than those of office BP measurement. CONCLUSION: Only one-third of patients achieved the BP goal by ABPM and most were in the primary HT group. Weight reduction is an important measure of BP control in patients with primary HT to attenuate the risk of LVH.

Comparison of paediatric basic life support guidelines endorsed by member councils of Resuscitation Council of Asia.

Background: Paediatric cardiac arrest outcomes, especially for infants, remain poor. Due to different training, resource differences, and historical reasons, paediatric cardiac arrest algorithms for various Asia countries vary. While there has been a common basic life support algorithm for adults by the Resuscitation Council of Asia (RCA), there is no common RCA algorithm for paediatric life support.We aimed to review published paediatric life support guidelines from different Asian resuscitation councils. Methods: Pubmed and Google Scholar search were performed for published paediatric basic and advanced life support guidelines from January 2015 to June 2023. Paediatric representatives from the Resuscitation Council of Asia were sought and contacted to provide input from September 2022 till June 2023. Results: While most of the components of published paediatric life support algorithms of Asian countries are similar, there are notable variations in terms of age criteria for recommended use of adult basic life support algorithms in the paediatric population less than 18 years old, recommended paediatric chest compression depth targets, ventilation rates post-advanced airway intra-arrest, and first defibrillation dose for shockable rhythms in paediatric cardiac arrest. Conclusion: This was an overview and mapping of published Asian paediatric resuscitation algorithms. It highlights similarities across paediatric life support guidelines in Asian countries. There were some differences in components of paediatric life support which highlight important knowledge gaps in paediatric resuscitation science. The minor differences in the paediatric life support guidelines endorsed by the member councils may provide a framework for prioritising resuscitation research and highlight knowledge gaps in paediatric resuscitation.

A unilateral whiteout lung in child with multisystem inflammatory syndrome associated with COVID-19 due to SARS-CoV-2: one case report of a boy.

BACKGROUND: Multisystem inflammatory syndrome in children (MIS-C) is a relatively new and rare complication of COVID-19. This complication seems to develop after the infection rather than during the acute phase of COVID-19. This report aims to describe a case of MIS-C in an 8-year-old Thai boy who presented with unilateral lung consolidation. Unilateral whiteout lung is not a common pediatric chest radiograph finding in MIS-C, but this is attributed to severe acute respiratory failure. CASE PRESENTATION: An 8-year-old boy presented with persistent fever for seven days, right cervical lymphadenopathy, and dyspnea for 12 h. The clinical and biochemical findings were compatible with MIS-C. Radiographic features included total opacity of the right lung and CT chest found consolidation and ground-glass opacities of the right lung. He was treated with intravenous immunoglobulin and methylprednisolone, and he dramatically responded to the treatment. He was discharged home in good condition after 8 days of treatment. CONCLUSION: Unilateral whiteout lung is not a common pediatric chest radiographic finding in MIS-C, but when it is encountered, a timely and accurate diagnosis is required to avoid delays and incorrect treatment. We describe a pediatric patient with unilateral lung consolidation from the inflammatory process.

Correlating computed tomographic angiography of pulmonary circulation with clinical course and disease burden in patients with tetralogy of Fallot and pulmonary atresia.

PURPOSE: To determine the type of pulmonary circulation (PC) in patients with tetralogy of Fallot (TOF) and pulmonary atresia (TOF-PA) with the use of computed tomographic angiography (CTA), and describe their clinical courses, corrective surgery and disease burden. METHODS: 145 patients (median age 4 years, interquartile range: IQR2-8 years) were analyzed for PC which divided into 5 CTA-types and 11 subtypes based on presence of main pulmonary trunk (MPA) and confluent pulmonary arteries (confluence-PAs), presence of ductus arteriosus or major aortopulmonary collateral arteries (MAPCAs), respectively. Pulmonary arteries (PAs) were assessed by McGoon ratio and arborization. Corrective surgery or palliative management was recorded by type of PC. Disease burden was calculated as the sum of CTA, diagnostic angiography, and palliative management. RESULTS: The most common (N = 77, 53 %) PC was the presence of MPA with confluent-PAs (type-1) which was encountered mostly in TOF patients, followed by the presence of confluent-PAs with atretic MPA (type-2) (N = 47, 32 %) which found mainly in TOF-PA. McGoon ratio in type-1 (2.44 ± 0.84) was significantly larger than type-2 (1.61 ± 0.61) (median difference 0.84, 95 %CI 0.56-1.11, p < 0.001). Almost 2/3 of patients in type-1 (71 %) and 1/3 of patients in type-2 (34 %) achieved corrective surgery. There was no significant difference in amount of disease burden among the different PC, with the median value of 3 (IQR1-4). CONCLUSIONS: Types of PC allow suggestions for size and arborization of PAs and successful surgical correction with an inverse relationship with the numbers of MAPCAs. There is no significant difference in amount of disease burden among the types of PC.

A Novel Homozygous PKP2 Variant in Severe Neonatal Non-compaction and Concomitant Ventricular Septal Defect: A Case Report.

Left ventricular non-compaction (LVNC) is a rare and genetically heterogeneous cardiomyopathy. The disorder vastly affects infants and young children. Severe neonatal LVNC is relatively rare. The prevalence of genetic defects underlying pediatric and adult-onset LVNC is about 17-40%. Mutations of MYH7 and MYBPC3 sarcomeric genes are found in the vast majority of the positive pediatric cases. PKP2 encodes plakophilin-2, a non-sarcomeric desmosomal protein, which has multiple roles in cardiac myocytes including cell-cell adhesion, tightening gap junction, and transcriptional factor. Most of the reported PKP2 mutations are heterozygous missense and truncating variants, and they are associated with an adult-onset autosomal dominant disorder, namely arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C). Homozygous PKP2 mutations have been rarely described. Herein, we present a rare case of an infant with neonatal onset of congestive heart failure owing to severe LVNC and multiple muscular VSD. Medical treatments failed to control the heart failure and the patient died at 11 months of age. Whole-exome sequencing identified a novel homozygous PKP2 variant, c.1511-1G>C, in the patient. An mRNA analysis revealed aberrant transcript lacking exon 7, which was predicted to cause a frameshift and truncated peptide (p.Gly460GlufsTer2). The heterozygous parents had normal cardiac structures and functions as demonstrated by electrocardiogram and echocardiography. Pathogenic variants of sarcomeric genes analyzed were not found in the patient. We conducted a literature review and identified eight families with biallelic PKP2 mutations. We observed that three families (our included) with null variants were linked to lethal phenotypes, while homozygous missense mutations resulted in less severe manifestations: adolescent-onset ARVD/C and childhood-onset DCM. Our data support a previous notion that severe neonatal LVNC might represent a unique entity and had distinct genetic spectrum. In conclusion, the present study has extended the phenotypes and genotypes of PKP2-related disorders and lethal LVNC.

Inadequate blood pressure control demonstrated by ambulatory blood pressure monitoring in pediatric renal transplant recipients.

BACKGROUND: Adequate BP control in RT recipients should not rely only by normal office BP but also on normal 24-hour BP. This study aims to assess adequacy of BP control by ABPM and to assess ABPM parameters associated with LVMI in pediatric RT recipients. MATERIALS AND METHODS: Patients aged 5-20 years who have been followed after RT were enrolled. Demographic data and BP assessed by office and ABPM were collected. Echocardiography was performed to detect LVMI. RESULTS: Thirty RT recipients (18 males) with median age of 15 years (IQR 13-18.5) were included. Among 23 patients who were taking antihypertensive drugs, uncontrolled hypertension was detected in 34.8% and 78.3% by office BP measurement and ABPM, respectively. Thus, the difference in prevalence of uncontrolled hypertension observed by ABPM versus office BP was 43.5%. Those seven patients who were not taking antihypertensive drugs because of normal office BP, four patients (57.1%) had masked hypertension and one patient had elevated BP. Fifteen patients have progression of LVH after RT. Multivariate analysis revealed that age (OR 1.369, 95%CI 0.985-1.904, P-value = 0.062) had a trend to be associated with progression of LVH. Moreover, nighttime systolic BP z-score was significantly correlated with LVMI (r = 0.551, P-value = 0.002). CONCLUSION: The difference in prevalence of uncontrolled hypertension uncovered by ABPM was 43.5%. Nighttime SBP z-score was significantly correlated with LVMI.

Comparison of three non-invasive hemodynamic monitoring methods in critically ill children.

INTRODUCTION: Hemodynamic parameters measurements were widely conducted using pulmonary artery catheter (PAC) with thermodilution as a reference standard. Due to its technical difficulties in children, transthoracic echocardiography (TTE) has been widely employed instead. Nonetheless, TTE requires expertise and is time-consuming. Noninvasive cardiac output monitoring such as ultrasonic cardiac output monitor (USCOM) and electrical velocimetry (EV) can be performed rapidly with less expertise requirement. Presently, there are inconsistent evidences, variable precision, and reproducibility of EV, USCOM and TTE measurements. Our objective was to compare USCOM, EV and TTE in hemodynamic measurements in critically ill children. MATERIALS AND METHODS: This was a single center, prospective observational study in critically ill children. Children with congenital heart diseases and unstable hemodynamics were excluded. Simultaneous measurements of hemodynamic parameters were conducted using USCOM, EV, and TTE. Inter-rater reliability was determined. Bland-Altman plots were used to analyse agreement of assessed parameters. RESULTS: Analysis was performed in 121 patients with mean age of 4.9 years old and 56.2% of male population. Interrater reliability showed acceptable agreement in all measured parameters (stroke volume (SV), cardiac output (CO), velocity time integral (VTI), inotropy (INO), flow time corrected (FTC), aortic valve diameter (AV), systemic vascular resistance (SVR), and stroke volume variation (SVV); (Cronbach's alpha 0.76-0.98). Percentages of error in all parameters were acceptable by Bland-Altman analysis (9.2-28.8%) except SVR (30.8%) and SVV (257.1%). CONCLUSION: Three noninvasive methods might be used interchangeably in pediatric critical care settings with stable hemodynamics. Interpretation of SVV and SVR measurements must be done with prudence.

Outcomes of straightforward extracardiac Fontan operation in advanced-age group.

INTRODUCTION: Fontan operation is a palliative procedure used in patients with univentricular heart (UVH). There have been numerous modifications to the technique. The appropriate strategy for treating patients with UVH in Thailand is still a major concern. OBJECTIVE: To describe the outcomes of patients after straightforward Fontan operation at advanced-age and evaluate their post-surgical cardiac performance. MATERIAL AND METHOD: A cross sectional study was performed in post-Fontan operation patients at Rajvithi Hospital between 2000 and 2009. Post-operative cardiac performance was analyzed using the echocardiogram and treadmill exercise stress test (EST). RESULTS: Thirty-nine patients were enrolled in the present study. The mean age was 11.7 years and the mean age at operation was 7.4 years. The predominant systemic chamber was morphologic left ventricle. Straightforward Fontan operation was performed in 60% of cases. Mean of Fontan index, mean of McGoon ratio, and mean of Nakata index were 2.63, 2.32, and 414.15 sqmm/sqm, respectively. Mean of the PVR and PAP were 1.98 U/m2 and 11.05 mmHg. Eighteen percent of patients died in the early post-operative period. Most of the patients died from septicemia. The mean EFwas 58.43%. Systolic dysfunction in 17.9% of the cases was abnormal, whereas diastolic dysfunction was present in half of the cases. Thirty-nine percent had MPI abnormality. During EST three cases developed arrhythmias and 3 cases had hypotension. Patients were categorized into 3 groups (Group 1: normal systolic and diastolic functions, Group 2: diastolic dysfunction, Group 3: impairment of both systole and diastole). Pre-operative cardiac catheterization parameters, surgical data and cardiac performance showed no statistical significance. However, there was a significant correlation between those with abnormal cardiac performance and arrhythmias or hypotension during EST (p = 0.003). CONCLUSION: The selection of suitable cases and good pre-operative evaluation could decrease the morbidity and mortality in patients undergoing the Fontan procedure. This study also found a correlation between abnormal cardiac performance and transient cardiac arrhythmia during exercise. The evaluation of cardiac performance and EST remains to be performed for following-up of patients who have undergone the Fontan operation, even for the asymptomatic cases.

A comparative study: right ventricular assessment in post-repaired tetralogy of Fallot patients by echocardiogram with cardiac magnetic resonance imaging.

BACKGROUND: Post-repaired Tetralogy of Fallot (TOF) patients require comprehensive evaluation of the right ventricular (RV) size and function. Currently, cardiac magnetic resonance imaging (CMR) is considered to be the gold standard for RV function assessment. Echocardiogram (ECHO) is the most useful non-invasive tool for RV assessment. However, correlations of ECHO and CMR findings for this particular group of patients require further evaluation. OBJECTIVE: The first objective is to assess the correlation between RV size/function, measured by ECHO and CMR. The second objective is to investigate ECHO parameters that correlate best with RV end diastolic volume index (RVEDVi) of 160 mL/m2 from CMR. MATERIAL AND METHOD: The present study recruited 20 TOF patients (mean age 14 ± 2 years) who underwent right ventricular outflow tract reconstruction and/or pulmonary valve replacement for at least 5 years, from June 2011 to March 2012. The RV was initially evaluated with CMR, followed by ECHO within 3 months. ECHO parameters measured were tricuspid annular plane systolic excursion (TAPSE), fractional area change (FAC), area of right ventricular end diastole index (area RVEDi), RV free wall myocardial performance index (RVMPI), and qualitative assessment of pulmonary valve regurgitation (PR). All ECHO parameters were compared with CMR measurements of right ventricular ejection fraction (RVEF), RVEDVi and quantitative assessment of PR. Comparative analysis were assessed by Pearson's sample correlation coefficient, Kappa, and sensitivity and specificity of RVEDi area from ROC curve analysis. RESULTS: Results showed significant correlations between RVEDVi and area RVEDi (R = 0.768, p < 0.01), RVEF with FAC (R = 0.759, p < 0.01), and RVEF with TAPSE (R = 0.688, p < 0.01). Hundred percent correlation was found in moderate to severe PR assessment by ECHO and CMR (Kappa = 0.912). Abnormal RVMPI was not correlated with NYHA FC, CXR and ECG (Kappa = -0.10, 0.15, -0.04). The area RVEDi ≥ 20.43 cm2/m2 correlated well with RVEDVi ≥ 160 mL/m2 (sensitivity 64%, specificity 83%) from ROC curve analysis. CONCLUSION: ECHO is an effective tool for RV evaluation in post-repaired TOF with PR. FAC, TAPSE and severity of PR from ECHO correlated well with CMR parameters. Measurement of area RVEDi from ECHO is the best parameter to predict RVEDVi from CMR.

The role of pleurodesis in the management of chylous pleural effusion after surgery for congenital heart disease.

This study aimed to determine the incidence and outcome of postoperative chylous pleural effusion as well as the efficacy of pleurodesis for its management after surgery for congenital heart disease. Medical and surgical databases were used to identify all patients who had surgery for congenital heart disease and subsequently experienced postoperative chylous pleural effusion. Medical records were reviewed and daily chest drainage and management strategies were recorded. From January 2000 to June 2006, 1,166 cardiac operations were followed by 19 cases of chylous pleural effusion, for an incidence of 1.6%. The diagnosis was made a mean of 9 days after the operation. The patients were divided into two groups according to treatment strategy. Group 1 included 9 patients who had received only conventional medical treatment consisting of parenteral nutrition and/or medium-chain triglyceride formula and/or a low-fat diet and/or somatostatin. Group 2 included 10 patients who initially received conventional medical treatment, then subsequently received chemical or mechanical pleurodesis. The amount of the chylous drainage was significantly less in group 1 (14 ml/kg/day) than in group 2 (24 ml/kg/day) (P < 0.05), suggesting a more severe problem in group 2. For group 2, the amount of drainage was significantly less after chemical or mechanical pleurodesis (8 vs 24 ml/kg/day; P < 0.05) than before. Seven patients (70%) responded favorably to the first pleurodesis, and two patients (20%) required more than one pleurodesis. One patient (10%) did not respond to pleurodesis but was treated successfully with thoracic duct ligation. There were no deaths. Pleurodesis is a safe and effective method for treating chylous effusion after surgical treatment of congenital heart disease, especially after failed conservative treatment. However, some patients may need more than one pleurodesis.