Resection of an immature intrapericardial teratoma from a premature neonate presenting as hydrops foetalis.

Intrapericardial teratoma is a germ-cell tumor that typically arises from the base of the heart. This rare cardiac tumour is the second most common tumor diagnosed in fetuses and newborn. Although benign, it can be massive in size causing direct compression on the heart and associated with significant pericardial effusion resulting life-threatening complications such as cardiac tamponade, heart failure, foetal hydrops, and sudden death. Early antenatal diagnosis and surgical intervention improve the survival. We present a case of immature intrapericardial teratoma diagnosed at 25 weeks of gestation but required multiple foetal pericardiocentesis and premature delivery due to massive pericardial effusion. The importance of multidisciplinary team approach to ensure successful management was highlighted in this case report.

Cardiovascular causes of tracheobronchial compression: a decade experience in a Paediatric Congenital Heart Centre.

BACKGROUND: Vascular compression of the airway often complicates CHD management. This study evaluated the use of CT in determining cardiovascular causes, clinical manifestations, and outcome of tracheobronchial compression among children with CHD. METHODS: A retrospective review of clinical records of all patients with CT scan evidence of tracheobronchial compression from January 2007 to December 2017 at National Heart Institute. Cardiovascular causes of tracheobronchial compression were divided into three groups; group I: vascular ring/pulmonary artery sling, II: abnormally enlarged or malposition cardiovascular structure due to CHD, III: post-CHD surgery. RESULTS: Vascular tracheobronchial compression was found in 81 out of 810 (10%) patients who underwent CT scan. Group I lesions were the leading causes of vascular tracheobronchial compression (55.5%), followed by group II (34.6%) and group III (9.9%). The median age of diagnosis in groups I, II, and III were 16.8 months, 3 months, and 15.6 months, respectively. Half of group I patients are manifested with stridor and one-third with recurrent chest infections. Persistent respiratory symptoms, lung atelectasis, or prolonged respiratory support requirement were clues in groups II and III. Higher morbidity and mortality in younger infants with severe obstructive airway symptoms, associated airway abnormalities, and underlying complex cyanotic CHD. CONCLUSIONS: Vascular ring/pulmonary artery sling and abnormally enlarged or malposition cardiovascular structure were the leading causes of cardiovascular airway compression. A high index of suspicion is needed for early detection due to its non-specific presentation. The outcome often depends on the severity of airway obstruction and complexity of cardiac lesions.

Internal iliac artery thrombosis in a 2-month-old infant with incomplete Kawasaki disease.

We report a case of a 2-month-old infant with incomplete Kawasaki disease with multiple coronary and systemic arteries aneurysms complicated with internal iliac arteries thrombosis. The atypical clinical presentations and severity of systemic vascular involvements discuss the importance of high index of suspicions in younger infants and treatment options in such cases.

Constrictive pericarditis following necrotising pneumococcal pneumonia in an immunocompetent child.

Purulent pericarditis leading to constrictive pericarditis is a rare but serious complication following invasive pneumococcal infection. Early recognition of this complication is crucial to prevent mortality. Here, we report a previously healthy child who developed constrictive pericarditis due to purulent pericarditis following necrotising pneumococcal pneumonia, which is not common in this current antibiotic and pneumococcal vaccine era. The child was successfully treated with pericardiectomy.

Right aortic arch with isolation of the left subclavian artery: a rare association with airway obstruction.

We present two interesting cases of isolated left subclavian artery from the pulmonary artery with symptoms of upper airway obstruction. The first patient had tetralogy of Fallot, pulmonary artery sling, bilateral superior caval veins, and left bronchial isomerism, suggesting heterotaxy syndrome. The second patient had a right aortic arch, isolated left subclavian artery, and bilateral arterial ducts. These two cases are interesting because of their rarity and uncommon presentation.

Mid-aortic syndrome presented as dilated cardiomyopathy.

Mid-aortic syndrome (MAS) is a rare vascular disease that usually leads to renovascular hypertension. With the predominant manifestations being intractable arterial hypertension and lower extremity arterial insufficiency, it has rarely been associated with dilated cardiomyopathy. We report a young girl with congestive heart failure, where the cause was initially attributed to dilated cardiomyopathy. A repeated echocardiogram 6 months later brought the physician's suspicion of MAS because of the abnormal colour of Doppler from the subcostal view. Further assessment using CT angiography revealed discrete thoracic coarctation at the level of T10, with the narrowest diameter of 2.1 mm, thus confirming the diagnosis. Her inflammatory markers and connective tissue screening were negative. She underwent successful stenting of coarctation of the aorta, which later caused improvement in her cardiac function. We highlighted the importance of looking for treatable causes of dilated cardiomyopathy and vigilant clinical and echocardiogram assessment with high suspicion to diagnose MAS.

Digital health intervention in patients undergoing cardiac rehabilitation: systematic review and meta-analysis.

Background: Cardiovascular disease (CVD) continues to be the foremost mortality internationally. Cardiac rehabilitation has proven as an effective program in reducing CVD burden. Participation in cardiac rehabilitation programs is very low. Digital health intervention emerged as an alternative method to deliver Cardiac rehabilitation. This review aimed to investigate the impact of digital health intervention on the outcomes of interest. Methods: the following databases: PubMed, CINAHL, Scopus, and Cochrane Library have been searched to retrieve randomized controlled trials that examine the impact of digital health intervention on blood pressure, body mass index, lipid profile, blood glucose, Six-Minute Walk Test, and peak oxygen consumption. filters were set to include studies published in English between 2000-2023. Results: Nineteen studies were included in this review. Six-Minute Walk Test (MD = 16.70; 95% CI: 6.00 to 27.39, p = 0.000) and maximal oxygen consumption (SMD = 0.27; 95% CI: 0.08 to 0.45, p = 0.004) significantly improved following digital health intervention, after employing the sensitivity analysis significant improvement was observed in systolic (MD = -2.54; 95% CI: -4.98 to -0.11, p = 0.04) and diastolic blood pressure (SMD = -2.0182; 95% CI: -3.9436 to -0.0928, p = 0.04) favoring experimental groups. Subgroup analysis revealed significant improvement in quality of life after three months of follow-up (SMD = 0.18; 95% CI: 0.05 to 0.31, p = 0.00), no significant differences have been observed in body mass index, lipid profile, and blood glucose. Conclusion: The findings emphasize the significant impact of digital vs CBCR or usual care on physical capacity, blood pressure, and quality of life. Despite the non-statistically significant differences in body mass index and lipid profile, the comparable effect between the two methods suggests the superiority of digital over CBCR or usual care due to its convenient nature, accessibility, and cost-effectiveness.

Methylphenidate-associated chest pain in a child.

A young child was diagnosed with autism spectrum disorder with comorbid attention-deficit/hyperactivity disorder. His hyperactivity, impulsivity and absence of awareness towards danger increased his risk of harm and hence methylphenidate was indicated. Unfortunately, he developed chest pain eight months after the treatment initiation. We then stopped the stimulant and changed his treatment to atomoxetine, after which he no longer had chest pain. In the following illustrated case, we will discuss the cardiac side effect of methylphenidate.

Safety and efficacy of pharmacological approaches available for multisystem inflammatory syndrome in children (MIS-C): a systematic review.

BACKGROUND: To describe the existing pharmacological managements for Multisystem Inflammatory Syndrome in Children (MIS-C) in a systematic way, to identify the available pharmacological managements in MIS-C, evaluate its safety and efficacy and identify the best treatment procedures for practice recommendation. METHODS: A systematic search using six databases was conducted on August 18, 2021, updated in January 26th 2023. Terminologies that were used in this search are children, MIS-C/PIMS and SARS-CoV-2. A PRISMA flow diagram was used to report the study selection process. Quality analysis was done based on NOS and GRADE tools. Data synthesis was conducted by extracting the information on drugs used, efficacy and side effects. RESULTS: From the 32 articles included, a total of 2331 children with MIS-C were studied. The main pharmacological approaches were immunomodulatory therapy, i.e., intravenous immunoglobulin (IVIG) (77.3%), steroids (60.5%), and a combination of IVIG and steroids (41.3%). IVIG and steroids were found to be potentially effective and safe treatments for MIS-C. Combination of IVIG and steroids was found favorable in severe cases with higher recovery rate. Refractory treatments include second dose of initial treatment and biological response modifier drugs like anakinra, tocilizumab, infliximab. A small number of studies investigating supportive treatment consisted of vasoactive, inotropic and anticoagulation. The mortality rate was 1.28% and only three studies reported side effects from the treatment. Evidence of outcome from GRADE were mostly at moderate, low and very low levels. CONCLUSIONS: This review provides preliminary evidence to support the current standard treatment practices in managing MIS-C pharmacologically. However, comprehensive investigation is required using clinical trials to provide stronger outcome evidence.

Peak apical recoil rate is a simplified index of left ventricular untwist: validation and application for assessment of diastolic function in children.

The use of untwisting rate as a novel index of LV diastolic function in clinical practice has been limited due to its tedious and time-consuming analysis. Therefore, we simplify the untwist measurement by only measuring the LV apex's recoil rate and validating and applying peak apical recoil rate (PARR) as an index of diastolic dysfunction (DD) in pediatric subjects during increased and decreased lusitropic states. We recruited 153 healthy subjects (mean age 13.8 ± 2.9 years), of whom 48 performed straight leg raising exercise and an additional 46 patients (mean 8.4 ± 5.6 years) with documented pulmonary capillary wedge pressures (PCWP) (validation cohort). In addition, we studied 16 dilated cardiomyopathy patients (mean age 9.5 ± 6.3 years) (application cohort). PARR and isovolumic relaxation time (IVRT) were compared to PCWP. Both PARR and PARR normalized by heart rate (nPARR) were excellent in detecting patients with PCWP ≥ 12 mmHg and greatly superior to IVRT in this respect (AUC: 0.98, 95% CI [0.96, 1.0] vs. AUC: 0.7 95%CI [0.54,0.86]). In DCM patients, PARR and nPARR were greatly decreased compared to controls (- 38.6 ± 18.6º/s vs - 63.1 ± 16.3º /s, p < 0.001) and (- 0.43 ± 0.20 º/ s/min vs - 0.83 ± 0.28º/s/min, p < 0.0001) but increased with straight leg raising exercise (- 59.4 ± 19.4º/s vs - 97.8 ± 39.0 º/s, p < 0.01) and - 0.85 ± 0.36 vs - 1.4 ± 0.62 º/s/min (p < 0.0001) respectively. PARR and nPARR successfully detected increased and decreased lusitropic states and superior to IVRT in correlation with PCWP. This highly reproducible parameter offers incremental value over traditional indices of DD and may potentially serve as a useful index of elevated PCWP in children.

Echocardiographic Findings in Pediatric Multisystem Inflammatory Syndrome Associated With COVID-19 in the United States.

BACKGROUND: Centers from Europe and United States have reported an exceedingly high number of children with a severe inflammatory syndrome in the setting of coronavirus disease 2019, which has been termed multisystem inflammatory syndrome in children (MIS-C). OBJECTIVES: This study aimed to analyze echocardiographic manifestations in MIS-C. METHODS: A total of 28 MIS-C, 20 healthy control subjects and 20 classic Kawasaki disease (KD) patients were retrospectively reviewed. The study reviewed echocardiographic parameters in the acute phase of the MIS-C and KD groups, and during the subacute period in the MIS-C group (interval 5.2 ± 3 days). RESULTS: Only 1 case in the MIS-C group (4%) manifested coronary artery dilatation (z score = 3.15) in the acute phase, showing resolution during early follow-up. Left ventricular (LV) systolic and diastolic function measured by deformation parameters were worse in patients with MIS-C compared with KD. Moreover, MIS-C patients with myocardial injury were more affected than those without myocardial injury with respect to all functional parameters. The strongest parameters to predict myocardial injury in MIS-C were global longitudinal strain, global circumferential strain, peak left atrial strain, and peak longitudinal strain of right ventricular free wall (odds ratios: 1.45 [95% confidence interval (CI): 1.08 to 1.95], 1.39 [95% CI: 1.04 to 1.88], 0.84 [95% CI: 0.73 to 0.96], and 1.59 [95% CI: 1.09 to 2.34], respectively). The preserved LV ejection fraction (EF) group in MIS-C showed diastolic dysfunction. During the subacute period, LVEF returned to normal (median from 54% to 64%; p < 0.001) but diastolic dysfunction persisted. CONCLUSIONS: Unlike classic KD, coronary arteries may be spared in early MIS-C; however, myocardial injury is common. Even preserved EF patients showed subtle changes in myocardial deformation, suggesting subclinical myocardial injury. During an abbreviated follow-up, there was good recovery of systolic function but persistence of diastolic dysfunction and no coronary aneurysms.

Diversity of respiratory viruses detected among hospitalized children with acute lower respiratory tract infections at Hospital Serdang, Malaysia.

BACKGROUND: The role of respiratory viruses as the major cause of acute lower respiratory tract infections (ALRTIs) in children is becoming increasingly evident due to the use of sensitive molecular detection methods. The aim of this study was to use conventional and molecular detection methods to assess the epidemiology of respiratory viral infections in children less than five years of age that were hospitalized with ALRTIs. METHODS: The cross-sectional study was designed to investigate the occurrence of respiratory viruses including respiratory syncytisl virus (RSV), human metapneumovirus (HMPV), influenza virus A and B (IFV-A and B), parainfluenzavirus 1, 2, 3 and 4 (PIV 1, 2, 3 and 4), human rhinoviruses (HRV), human enterovirus (HEV), human coronaviruses (HCoV) 229E and OC43, human bocavirus (HBoV) and human adenovirus (HAdV) in hospitalized children with ALRTIs, at Hospital Serdang, Malaysia, from June 16 to December 21, 2009. The study was also designed in part to assess the performance of the conventional methods against molecular methods. RESULTS: Viral pathogens were detected in 158 (95.8%) of the patients. Single virus infections were detected in 114 (67.9%) patients; 46 (27.9%) were co-infected with different viruses including double-virus infections in 37 (22.4%) and triple-virus infections in 9 (5.5%) cases. Approximately 70% of samples were found to be positive using conventional methods compared with 96% using molecular methods. A wide range of respiratory viruses were detected in the study. There was a high prevalence of RSV (50.3%) infections, particularly group B viruses. Other etiological agents including HAdV, HMPV, IFV-A, PIV 1-3, HBoV, HCoV-OC43 and HEV were detected in 14.5, 9.6, 9.1, 4.8, 3.6, 2.4 and 1.8 percent of the samples, respectively. CONCLUSION: Our results demonstrated the increased sensitivity of molecular detection methods compared with conventional methods for the diagnosis of ARTIs in hospitalized children. This is the first report of HMPV infections in Malaysia.

Diagnostic value of myocardial deformation pattern in children with noncompaction cardiomyopathy.

The current echocardiographic diagnostic criteria for noncompaction cardiomyopathy (NCC) have variable sensitivity and low specificity. Moreover, there are limited data on the use of myocardial deformation imaging for early detection of myocardial dysfunction in children with NCC. We describe left ventricular (LV) deformation patterns in children with NCC, with the goal of identifying a potential diagnostic pattern. We prospectively enrolled 30 children with NCC (47% male; mean age 7.2 years) and 30 age- and gender-matched controls. Extent and severity of non compaction in each segment were evaluated in LV 16-segment model. Regional (base, mid and apex) and segmental (16 segments) longitudinal strain (LS), circumferential strain (CS) and radial strain (RS) were measured using speckle tracking echocardiography. In all patients with NCC, regional and segmental CS and RS at the apex were significantly decreased compared to controls (CS apex: - 19.2 ± 5.4% vs. - 30.2 ± 6.9%, p < 0.001/RS apex: 23.5 ± 8.6% vs. 44.1 ± 14.5%, p < 0.001). Thirty percent (9/30) of patients had an EF < 50%. In these patients, there was additional decrease in CS in basal segments and in LS in basal, mid-cavity and apical segments (CS base: - 16.4 ± 4.7% vs. - 24.6 ± 3.9%, p < 0.001/LS (average all LV segments): - 13.9 ± 3.1% vs. - 20.7 ± 4.7%, p < 0.001). A cut-off value of CS at the apex of - 24.5% was a strong differentiating feature between patients with NCC and EF > 50% and controls (sensitivity: 87%, specificity 79%, AUC 0.88, p < 0.001). Children with NCC exhibit a deformation pattern characterized by decreased apical circumferential strain, which may serve as a potential diagnostic tool for NCC. The role of decreased global LV longitudinal and basal circumferential strain should be further evaluated as a potential prognostic tool.

A Preliminary Study of Left Ventricular Rotational Mechanics in Children with Noncompaction Cardiomyopathy: Do They Influence Ventricular Function?

BACKGROUND: Current diagnostic criteria for noncompaction cardiomyopathy (NCC) lack specificity, and the disease lacks prognostic indicators. Reverse apical rotation (RAR) with abnormal rotation of the cardiac apex in the same clockwise direction as the base has been described in adults with NCC. The aim of this study was to test the hypothesis that RAR might differentiate between symptomatic NCC and benign hypertrabeculations and might be associated with ventricular dysfunction. METHODS: Echocardiograms from 28 children with NCC without cardiac malformations were prospectively compared with those from 29 age-matched normal control subjects. A chart review was performed to identify the patients' histories and clinical characteristics. Speckle-tracking was used to measure longitudinal strain, circumferential strain, and rotation. RESULTS: RAR occurred in 39% of patients with NCC. History of left ventricular (LV) dysfunction or arrhythmia was universal in, but not exclusive to, patients with RAR. Patients with RAR had lower LV longitudinal strain but similar ejection fractions compared with patients without RAR (median, -15.6% [interquartile range, -12.9% to -19.3%] vs -19% [interquartile range, -14.5% to -21.9%], P < .01; 53% [interquartile range, 43% to 68%] vs 61% [interquartile range, 58% to 67%], P = .08). Only a pattern of contraction with RAR, early arrest of twisting by mid-systole, and premature untwisting was associated with lower ejection fraction (46%; interquartile range, 43% to 52%; P = .006). CONCLUSIONS: RAR is not a sensitive but is a specific indicator of complications in children with NCC. Therefore, RAR may have prognostic rather than diagnostic value. Premature untwisting of the left ventricle during ejection may be an even more worrisome indicator of LV dysfunction.

Left Ventricular Rotational Mechanics in Children After Heart Transplantation.

BACKGROUND: Left ventricular (LV) dysfunction after orthotopic heart transplantation (OHT) is multifactorial and can be an indicator of graft rejection or coronary artery vasculopathy. Analysis of rotational mechanics may help in the early diagnosis of ventricular dysfunction. Studies describing the left ventricular rotational strain in children after OHT are lacking. It is important to establish the baseline rotational mechanics in pediatric OHT to pursue further studies in this population. METHODS AND RESULTS: Rotational strain measured by speckle tracking was compared in 32 children after OHT, with no evidence of active rejection or coronary artery vasculopathy with 35 age-matched normal controls. Twelve OHT patients and 13 controls underwent moderate exercise with pre- and postexercise echocardiography. Torsion, slope of the systolic limb of the torsion-radial displacement loop, and the untwist rate were significantly higher in OHT patients (torsion: median 2.7°/cm [Q1-Q3, 2.3-3.2] versus 2.3°/cm [Q1-Q3, 1.9-2.7]; P=0.03, torsion-radial displacement loop: 2.7°/mm [Q1-Q3, 2.1-3.6] versus 2.0°/mm [Q1-Q3, 1.6-2.7]; P=0.008, indexed peak untwist rate: -21.6°/s/cm [Q1-Q3, -24.3 to -15.7] versus -17.1°/s/cm [Q1-Q3, -19.6 to -13.3]; P=0.01). Contrary to controls, OHT recipients were unable to increase torsion with exercise (OHT: 2.8°/cm [2.7-3.2] versus 3°/cm [2.4-3.5]; P=0.81, controls: 2.2°/cm [2-2.6] versus 3°/cm [2.4-3.7]; P=0.01, pre and post exercise, respectively). The systolic slope of the torsion-radial displacement loop relationship decreased with exercise in most OHT patients. CONCLUSIONS: Baseline rotational strain in OHT patients is higher than normal with a blunted response to exercise. The slope of torsion-radial displacement loop, and its response to exercise, may serve as a marker of left ventricular dysfunction in OHT patients.

Detection and characterization of viruses causing hand, foot and mouth disease from children in Seri Kembangan, Malaysia.

Hand, foot and mouth disease (HFMD) is a common viral infection among infants and children. The major causative agents of HFMD are enterovirus 71 (EV71) and coxsackievirus A16 (CVA16). Recently, coxsackievirus A6 (CVA6) infections were reported in neighboring countries. Infected infants and children may present with fever, mouth/throat ulcers, rashes and vesicles on hands and feet. Moreover, EV71 infections might cause fatal neurological complications. Since 1997, EV71 caused fatalities in Sarawak and Peninsula Malaysia. The purpose of this study was to identify and classify the viruses which detected from the patients who presenting clinical signs and symptoms of HFMD in Seri Kembangan, Malaysia. From December 2012 until July 2013, a total of 28 specimens were collected from patients with clinical case definitions of HFMD. The HFMD viruses were detected by using semi-nested reverse transcription polymerase chain reaction (snRT-PCR). The positive snRT-PCR products were sequenced and phylogenetic analyses of the viruses were performed. 12 of 28 specimens (42.9%) were positive in snRT-PCR, seven are CVA6 (58.3%), two CVA16 (16.7%) and three EV71 (25%). Based on phylogenetic analysis studies, EV71 strains were identified as sub-genotype B5; CVA16 strains classified into sub-genotype B2b and B2c; CVA6 strains closely related to strains in Taiwan and Japan. In this study, HFMD in Seri Kembangan were caused by different types of Enterovirus, which were EV71, CVA6 and CVA16.