Ventricle In Children Research Articles

Tumors of the third ventricle in children (literature review).

Tumors of the third ventricle are rare and account for 0.6–0.9 % of all pediatric brain tumors. Tumors of the third ventricle are divided into primary tumors, such as choroid plexus papillomas and ependymomas, and secondary tumors, such as craniopharyngiomas, optic nerve gliomas, pineal tumors, and meningiomas. Choroid papilloma is a common tumor, the treatment of which is mainly based on microsurgical resection. Apart from the many treatment options for craniopharyngiomas, microsurgery is the preferred treatment option. Ependymomas also have several treatment options, with microsurgical removal considered the first line of treatment.

A deeper look into the functions of right ventricle using three-dimensional echocardiography: the forgotten ventricle in children with systemic lupus erythematosus

Studies on the right ventricular dysfunction (RV) in systemic lupus erythematosus (SLE) patients are limited, particularly in the pediatric age group. The study aimed to identify subclinical RV alterations in childhood-onset SLE (c-SLE) using conventional and three-dimensional echocardiography (3DE). Forty SLE pediatric patients and 40 healthy controls were included. Disease activity and chronicity were evaluated by SLE disease activity index (SLEDAI) score and SLE damage index (SDI). Participants underwent detailed RV echocardiographic examination with conventional and 3DE assessment using 3D auto RV software. Patients included 35/40 (87.5%) females with mean age of 15.6 ± 1.7 years. Using conventional pulmonary artery systolic pressure echocardiography-derived measurement, none of the c-SLE patients had pulmonary hypertension. By 3DE, RV end-systolic and end-diastolic volumes (p = < 0.001, 0.02, respectively) were greater, whereas 3D-derived RV ejection fraction (p < 0.001), septal, and lateral longitudinal strain (both p < 0.001) were lower in SLE. SDI displayed a significant correlation with 3D auto RV ejection fraction (EF), tricuspid annular plane systolic excursion (TAPSE), fractional area change, and RV longitudinal strain (RVLS)-free wall (p = 0.01, 0.003, 0.007, and < 0.001, respectively). Cumulative SLEDAI score also showed a significant correlation with RV EF, TAPSE, FAC, and RVLS-free wall (p = 0.03, 0.007, 0.002, and < 0.001, respectively). By multivariate regression analysis, SDI remained an independent predictor of RVLS-free wall (ß coefficient − 0.4, p = 0.03) and TAPSE (ß − 0.5, p = 0.02). Conclusion: Subtle right ventricular myocardial dysfunction could be detected in childhood-onset SLE patients, especially via 3D-derived auto RV echocardiographic parameters, despite the absence of evident pulmonary hypertension. These parameters correlate with the SLE disease activity and chronicity scores.What is Known:•Diseases of the cardiovascular system are one of the most common causes of morbidity and mortality in SLE patients.•RV labeled the forgotten ventricle in many diseases, was also forgotten in SLE patients and has been rarely addressed in adults, with scarce research in pediatrics.What is New:•Right ventricular functions are affected in children with SLE in comparison to healthy controls, especially three-dimensional echocardiography-derived parameters, which is an aspect that has not been investigated in previous research in the pediatric age group.•Some of the detected myocardial dysfunctions of the right ventricle correlated with SLE disease activity and chronicity-related scores.

Contraction Patterns of Post-Fontan Single Right Ventricle Versus Normal Left and Right Ventricles in Children: Insights From Principal Strain Analysis

Principal strain (PS) analysis quantifies three-dimensional myocardial deformation using three-dimensional speckle-tracking echocardiography. It defines both the amplitude and direction of the principal myocardial contraction, expressed as PS, and a perpendicular secondary strain of lower intensity. The aims of this study were to apply PS analysis to describe the contractile pattern in the single right ventricle (SRV) functioning as a systemic chamber in hypoplastic left heart syndrome, compared with the normal left ventricle (LV) and right ventricle (RV), and to compare SRV function using conventional echocardiographic evaluations. Fifty-four post-Fontan patients with hypoplastic left heart syndrome and age-matched control subjects (normal LV, n=64; normal RV, n=48) underwent computation of PS lines, ejection fraction (EF), end-diastolic volume indexed to body surface area, PS, secondary strain, circumferential strain, and longitudinal strain. The PS lines were compared between groups. Linear regressions with coefficient determination (R2) of strains, fractional area change, and tricuspid annular plane systolic excursion with EF and end-diastolic volume index were assessed in SRV. Additionally, the hypoplastic left heart syndrome cohort was equally divided into two groups with higher and lower EFs, followed by comparison of all parameters. The pattern of PS lines demonstrated a left-handed direction in the anterior free wall, a right-handed direction in the posterior free wall, and a circumferential direction in the medial wall in SRV. In contrast, in the normal LV, the principal contraction is in the circumferential direction, whereas in the normal RV, it is predominantly longitudinal. The R2 values for PS, secondary strain, and circumferential strain on EF were high (0.88, 0.72, and 0.90, respectively), whereas the R2 value for longitudinal strain was comparable with that for fractional area change (0.56 and 0.55). All parameters were independent of end-diastolic volume index. PS lines in the higher EF group showed a more circumferential orientation than in the lower EF group in SRV. PS analysis provides a unique functional map of SRV contraction. This map differs from corresponding maps of the normal LV and RV. This may be helpful in understanding the mechanisms of SRV function, although future longitudinal studies are needed.

Neuroendoscopic Surgery for Pediatric Intraventricular Tumors

Various tumors are found in ventricles and tissues surrounding the ventricles in children, adolescents, and young-adult patients. They cause cerebrospinal fluid pathway obstruction, resulting in hydrocephalus and isolated ventricles. In this paper we describe a neoplasm arising from the lateral and third ventricle. Intraventricular tumors are good candidates for neuroendoscopic surgery. Firstly, minimally invasive and established procedures, neuroendoscopic biopsy with flexible endoscopy or rigid endoscopy, are performed. These endoscopic biopsies can be performed concurrently with other procedures, such as third ventriculostomy or septostomy, to improve cerebrospinal fluid circulation. Neuroendoscopic biopsy for intraventricular tumor is associated with high diagnostic yield and relatively low incidence of morbidity and mortality, compared with open surgery. Endoscopic diagnostic procedure also can be applied in patients without ventricular dilatation, assisted by neuronavigation or echo-guided equipment. Secondly, neuroendoscopic cylinder surgery also makes it possible to remove intraventricular tumors with less damage. The two hands technique with rigid endoscopy helps debulking and hemostasis during tumor removal. Further developments in endoscopic equipment and training systems aimed at enhancing cooperation between the operator and assistant should be expected in the future. Neuroendoscopic resection appears to be a safe and reliable treatment option for patients with intraventricular tumors.

Pulmonary Artery Banding for Dilated Cardiomyopathy in Children: Returning to the Bench from Bedside.

Current treatment paradigms for end-stage dilated cardiomyopathy (DCM) in children include heart transplantation and mechanical support devices. However, waitlist mortality, shortage of smaller donors, time-limited durability of grafts, and thrombo-hemorrhagic events affect long-term outcomes. Moreover, both these options are noncurative and cannot preserve the native heart function. Pulmonary artery banding (PAB) has been reinvented as a possible “regenerative surgery” to retrain the decompensated left ventricle in children with DCM. The rationale is to promote positive ventricular–ventricular interactions that result in recovery of left ventricular function in one out of two children, allowing transplantation delisting. Although promising, global experience with this technique is still limited, and several surgical centers are reluctant to adopt PAB since its exact biological bases remain unknown. In the present review, we summarize the clinical, functional, and molecular known and supposed working mechanisms of PAB in children with DCM. From its proven efficacy in the clinical setting, we described the macroscopic geometrical and functional changes in biventricular performance promoted by PAB. We finally speculated on the possible underlying molecular pathways recruited by PAB. An evidence-based explanation of the working mechanisms of PAB is still awaited to support wider adoption of this surgical option for pediatric heart failure.

Clinico-radiological diagnosis of silent Choroid plexus papilloma in a child

Background: Choroid plexus papillomas (CPPs) are benign slow-growing tumors with the most common site as the choroid plexus of the lateral ventricle in children. These are highly vascular solid tumors which have classical diagnosis in contrast-enhanced computerized tomography (CECT) and contrast-enhanced magnetic resonance imaging (CEMRI). These require early management because of their secondary symptoms related to hydrocephalus and others due to tumoral compression. Case Report: We present a 10-month-old male child with abnormal eye movements and fever following head trauma seventeen days before. Ultrasound of the brain revealed the hyperechoic mass in the right lateral ventricle. CECT and CEMRI diagnosed as a case of choroid plexus papilloma with gross hydrocephalus. This was a silent CPP which came to limelight following head trauma. Ventriculoperitoneal shunt was placed for the decompression of the ventricular system and was advised for subsequent surgical management. Conclusion: Atypical presentations of CPP can confuse in making the diagnosis and this delay can be harmful to the patient. Early diagnosis of CPP is very important as these are slow-growing tumors which can lead to various complications if late diagnosis is made. Cross-sectional modalities such as CECT and CEMRI are the most valuable diagnostic modality tools for the early diagnosis

Strain indicators in the assessment of systolic myocardial function in aboy with hypertrophic cardiomyopathy: a case report

Purpose: This article is about an unusual clinical case of a 14-year-old boy with hypertrophic cardiomyopathy (HCM).Ejection fraction (EF) and shortening fraction (FS) of the left ventricle in children with HCM remain within a normal range for an extended period of time. Therefore, detection of a decrease in segmental myocardial contractility at the initial stage of the disease presents a diagnostic problem.&#x0D; Methods: The clinical characteristics of the patient, study results, and analysis of the left ventricular myocardial strain using 2D speckle-tracking mode in an echocardiographic examination are presented.&#x0D; Results: A decrease in strain indicators in the anterioseptal, anterior, and anterolateral segments of the left ventricular myocardium and a compensatory increase in the contralateral segments were revealed. This served as an indication to start treatment.&#x0D; Conclusions: A decrease in strain indicators in the 2D speckle- tracking mode is most informatively represented by changes in segmental systolic function of the left ventricle at the initial stage of HCM.

Abstract 10693: Noninvasive Assessment of Contractility and Compliance of the Left Ventricle in Children: Myocardial Stiffness Measured Throughout the Cardiac Cycle by Shear Wave Imaging

Background: Intrinsic myocardial stiffness (MS) is load-independent, unlike most parameters to assess LV systolic and diastolic function. Pre-clinical studies have found good correlation between 1) peak-systolic MS and contractility (or ESPVR); 2) end-diastolic MS and compliance (or EDPVR). Shear wave imaging (SWI) by ultrafast ultrasound imaging allows MS measurement throughout the cardiac cycle. Methods (Fig 1): Five children with LV pressure-overload (LV-PO; 2 aortic stenosis, 3 coarctation) were studied during cardiac catheterization and there were 5 age-matched controls. Interventricular septal MS was measured by SWI every 100ms during the cardiac cycle, pre- and post-balloon dilation. ESPVR and EDPVR were assessed by pressure-volume loops by a pressure catheter and real-time 3D-echo volumes. Results (Fig 2): Peak-systolic MS was higher in LV-PO (22.7±6.7 kPa) than controls (8.4±2.8 kPa; p&lt;0.01) and compared to end-diastolic MS (LV-PO 4.8±1.7 kPa, control 1.3±0.8 kPa; p&lt;0.01). End-diastolic MS was correlated with LV end-diastolic pressure (r=0.74). Relief of PO did not change the peak MS in the LV-PO (p=0.88). Correlations between peak-systolic MS and ESPVR (r=0.72), and between end-diastolic MS and EDPVR (r=0.79) were confirmed. Conclusions: Non-invasive MS is a load-independent measure of LV systolic contractility and diastolic compliance in children.

Colloid cysts of the third ventricle in children.

The rarity of colloid cysts in children makes it difficult to characterize this entity and offer meaningful advice on treatment. Infrequent case reports exist, but to date there has been no age-specific assessment. The purpose of this study was to define any differences between children and adults who are evaluated and treated for colloid cysts of the third ventricle. Patients with colloid cysts were reviewed and stratified by age. Individuals ≤ 18 years of age were defined as pediatric patients and those > 18 years of age as adults. Clinical and radiographic data, treatment, and postoperative outcomes were compared between both groups. Bivariate analysis was conducted using the Fisher exact test for categorical variables and Mann-Whitney U-test for continuous variables. Of 132 endoscopic resections (121 primary, 10 secondary, and 1 tertiary) of a colloid cyst, 9 (6.8%) were performed in pediatric patients (mean age 14.1 years, range 9-18 years) and 123 (93.2%) were performed in adult patients (mean age 43.8 years, range 19-73 years). Cases were found incidentally more commonly in pediatric than adult patients (66.7% vs 37.4%, p > 0.05), and pediatric patients had lower rates of hydrocephalus than adult patients (11.1% vs 63.4%, p < 0.05). Acute decompensation at presentation was found in 8 adults (6.5%) but no children. Complete cyst removal (88.9% vs 90.2%, p > 0.05) and length of stay (1.6 days vs 2.9 days, p > 0.05) were not significantly different between the groups. Postoperative complications (6.5% in adults, 0% in children) and recurrence (2.4% in adults, 0% in children) were rare in both groups, and there were no treatment-related deaths. The mean postoperative radiological follow-up was longer in pediatric patients (45 months, range 4-89 months) than adults (44.1 months, range 1-171 months). While differences exist between children and adults regarding colloid cyst presentation, these are in keeping with the predicted evolution of a slow-growing lesion. Consistent with this observation, children had lower rates of hydrocephalus and a smaller mean maximal cyst diameter. Contrary to the published literature, however, sudden deterioration was not observed in pediatric patients but occurred in adult patients. In this limited pediatric sample size, the authors have not recorded any postoperative complications or recurrences to date. These encouraging results with endoscopic removal may positively impact future decisions related to children given their protracted life expectancy and projected rates of progression.

Microscopic and ultramicroscopic structure of typical chordae tendineae of the human’s left ventricle bicuspid valve

Objective – to determine the features of the micro- and submicroscopic structure of typical chordae tendineae of the mitral valve of the human heart.Materials and methods. This study was made on preparations of 35 human hearts, in particular the chordae tendineae of the bicuspid valves of the left ventricles of children and people of early and middle adulthood using the methods of light and electron microscopy.Results. The study of typical chordae tendineae of the mitral valve leaflets was made using the microscopic method and the method of electron microscopy. The areas where the chordae tendineae diverge from the papillary muscles, the middle third of the chordae tendineae, and the place of attachment of the chordae tendineae to the mitral valve leaflets were studied. Chordae tendineae are covered with endothelium from all sides. A peripheral collagen-elastic layer of loose fibrous connective tissue is localized under the endothelium, along the entire perimeter of the chordae tendineae and it differs in structure in different parts of the chordae at the submicroscopic level. Bundles of densely packed collagen fibers, which are oriented rectilinearly along the chordae, form the core of the chordae tendineae. Bundles of striated cardiac typical muscle cells are localized in areas where the chordae tendineae origin from the papillary muscles. It was found that in the composition of the chordae tendineae of children the cellular component in the fibrous connective tissue predominates during comparing the typical chordae tendineae of different aged people.Conclusions. This morphological study made it possible to obtain new information and to clarify the data that are already known to morphologists on the micro- and submicroscopic structure of typical chordae tendineae of the mitral valve of the human heart. We found that differences in the histological structure are more typical for the areas of origin of typical chordae tendineae from the papillary muscles and areas of their attachment to the leaflets of the mitral valve. The diameter of the core of the chordae tendineae increases and the thickness of the loose peripheral collagen-elastic layer decreases with age. The received data can serve as a theoretical basis for the further comparative morphological and clinical studies of the mitral valve of the human heart. That, in turn, will facilitate to an increasing the level of diagnostic, to improve methods of prevention and treatment of minor heart anomalies, congenital and acquired defects of the valvular apparatus of the heart.

Endoscopic aqueductoplasty and stenting in the treatment of isolated fourth ventricle in children: 20-year institutional experience.

Endoscopic aqueductoplasty with aqueductal stenting is an effective surgical procedure for the treatment of isolated fourth ventricle (IFV). Due to the rarity of the underlying pathology, it can be considered a rare procedure that can be performed with different surgical techniques and approaches. To assess long-term functioning of permanent aqueductal stents implanted in children affected by hydrocephalus and IFV and to describe some variations of the same procedure. We reviewed retrospectively all the patients presenting at our institution in the years 1999-2019 for symptoms of isolated fourth ventricle who underwent a surgical procedure of endoscopic aqueductoplasty and/or aqueductal stent. Surgical reports, radiological images, and surgical videos were retrospectively analyzed. Thirty-three patients with symptomatic isolated fourth ventricle (IFV) underwent fifty (50) neuroendoscopic procedures in the period observed. The median age of the patients at the time of first surgery was 7 months, with 22 premature babies. In twenty-nine patients (87.8%), a precoronal approach was performed, while four patients received a suboccipital burr hole. Ten patients were never reoperated since. Twenty-three patients underwent further surgeries: a new aqueductoplasty with aqueductal stent was performed in 13 cases. The remaining 10 patients required a combination of other procedures for management of hydrocephalus. Long-term follow-up showed a permanent stent functioning rate of 87% at 2 years and 73% at 4 years, remaining stable afterwards at very long term (20 years). Endoscopic aqueductoplasty and stenting is a reliable procedure in the long-term management of isolated fourth ventricle.

Abstract PO-235: Choroid plexus papilloma: A report of a benign but rare entity

Abstract Choroid plexus papilloma (CPP) accounts for &amp;lt;1% of all the intracranial neoplasms and is a rare benign neoplasm with common sites being the fourth ventricle in adults and lateral ventricle in children. They are occasionally bilateral with associated hydrocephalus. A 28-year-old female with no past medical history presented to the emergency department with a fall and witnessed seizure. On her way to the bathroom, she felt dizzy and missed a step leading her to trip and fall hitting her headfirst. While on the floor she was witnessed a seizure and brought to the emergency department where she had postictal confusion, slow speech, and was unable to recall events after falling. She also complained about fatigue, lack of energy, sleepiness, loss of interest, sad mood, and suicidal thoughts. CT scan demonstrated a 2.6 cm fourth ventricular mass with internal calcifications. Posterior to the mass there was a subtle increased density which was favored as additional calcifications with moderate obstruction of the 4th ventricle and mild ventriculomegaly. Differential diagnosis included subependymoma, ependymoma as well as astrocytoma. Histopathological examination revealed CPP with appropriately controlled stains to be positive for S100 and Ki-67 to be approximately 3% and negative for p63. CPP is derived from neuroectoderm and is designated as Grade I neoplasms by WHO and Grade II is reserved for atypical CPP. The most optimal treatment strategy is resection which is curative with very little chance of recurrence after a gross-total resection in approximately 96% of the cases. One of the management challenges faced is the vascularity of the tumor which is due to the intraventricular location of tumor which has a rich anastomotic supply of choroidal blood vessels. Due to the rich vascularity of the tumor embolization is considered as an adjunct to reduce the intravascular blood loss intraoperatively with the overall mortality ranging from 0-25% and the mortality rate from the blood loss to be approximately 12%. Currently, the patient is doing well without any evidence of metastasis. Hence it is important to be aware of this rare and benign entity and endoscopic removal is recommended despite the intraoperative bleeding it might cause. Citation Format: Hansini Laharwani, Vijay Kumar, James Neill. Choroid plexus papilloma: A report of a benign but rare entity [abstract]. In: Proceedings of the AACR Virtual Conference: Thirteenth AACR Conference on the Science of Cancer Health Disparities in Racial/Ethnic Minorities and the Medically Underserved; 2020 Oct 2-4. Philadelphia (PA): AACR; Cancer Epidemiol Biomarkers Prev 2020;29(12 Suppl):Abstract nr PO-235.

Torticollis as a Rare Presentation of Cerebellopontine Angle Choroid Plexus Papilloma in Children

Background and Importance: The rare intracranial neoplasms are Choroid Plexus Papillomas (CPPs), especially in the cerebellopontine angle. The main location of choroid plexus papillomas in adults and children are 4th ventricle and lateral ventricles, respectively. Case Presentation: We report on a little girl with a cerebellopontine angle CPP who presented with symptoms of torticollis. Assessment of Magnetic Resonance Imaging showed a mass in the right cerebellopontine angle, next to the brain stem. The tumor was completely resected using the right retrosigmoid approach method. A pathological examination determine a typical CPP that this being should be considered an extremely rare cause of a lesion in the posterior fossa. Conclusion: CPP is usually presented in the atrium of the lateral ventricle in children; however, we reported a rare case in the cerebellopontine angle.

A Rare Case of Choroid Plexus Papilloma of the Third Ventricle in an Adult

Colloid cysts are the commonest masses of the third ventricle. Third ventricle neoplasms are uncommon. They include tumors arising from the choroid plexus (papillomas, carcinomas), tumors arising from other than the choroid plexus (ependymomas, meningiomas), metastases, and lymphoma. Choroid plexus tumors usually occur in the lateral ventricle in children and fourth ventricle in adults, and often present with hydrocephalus. We herein describe the extremely rare occurrence of third ventricle choroid plexus papilloma in a 35-year-old man who presented to the emergency department with a long history of intermittent headaches, occasionally associated with photophobia. CT and MR imaging revealed a lobulated ovoid lesion in the third ventricle with minimal extension into the right lateral ventricle through the foramen of Monro, causing mild ventricular dilatation. Surgical resection was performed and histopathology revealed choroid plexus papilloma.

Use of echocardiographic parameters of longitudinal mechanics to estimate diastolic function of systemic ventricle in patients after fontan operation

Objective. To study the possibilities of using echocardiographic parameters of longitudinal mechanics to assess the diastolic function of the systemic ventricle (SV) in patients after Fontan operation.&#x0D; Material and methods. We examined 29 children aged 8,6 3,16 years with a functionally single ventricle of the heart after performing a total cavopulmonary connection with fenestration of extracardiac conduit. By computed tomography there were evaluated: myocardial mass index (MMI) SV, ejection fraction (EF) SV, cardiac output (CO) SV, cardiac index (CI) SV. In addition to conventional echocardiographic (Echo) parameters, we also studied: diastolic velocity parameters of the longitudinal mechanics of the SV during the early rapid filling phase (SR E), in the phase of late diastolic filling (SR A), the ratio of the peaks of the strain rate (SR E/SR A), the stiffness index of a functionally single atrium, the atrial strain in conduit phase (AS cd), reservoir (ASr), and in the contractile phase (AS ct).&#x0D; Results. Reduced values of the SI, CO and CI SV, an increase in MMI SV, normal values of EF SV. The parameters of the longitudinal diastolic strain rate in the early and late filling phases of e SV showed a direct significant correlation with SI, CO and CI SV and inverse correlation with E/e '. Parameters of longitudinal strain of the functionally single atrium in all phases were reduced, they all correlated with E / e, SR E / SR A, MMI SV, SI, CO and CI SV. There was no statistically significant relationship between the parameters of the longitudinal function of the atrium in all phases and atrial stiffness with sex and body surface area.&#x0D; Conclusions. 1. The parameters of the longitudinal diastolic strain rate can be used to identify the diastolic dysfunction of the systemic ventricle in children after Fontan operation. 2. The hemodynamic parameters of the systemic ventricle (stroke index, cardiac output, cardiac index) determine the parameters of its diastolic mechanics. 3. The reservoir, conduit, and contractile atrial function in patients after total cavopulmonary connection are associated with both characteristics of diastolic longitudinal strain rate and hemodynamic parameters of systemic ventricle.

Suicidal right ventricle in children and adults: Trends, triggers, and treatment: A systematic review of a rare but catastrophic event

Background: Suicidal right ventricle (SRV) is defined as a dynamic right ventricular outflow obstruction with a clinical hemodynamic significance. This is a rare and dangerous event in cardiology practice. Objectives: This systematic review is aimed at ascertaining different scenarios that can precipitate SRV in cardiology practice. Methods: Data for the systematic review was retrieved from Cochrane database, Google Scholar, PubMed review, Institute for Scientific Information “Web of Science,” and Medline. The search engine yielded 164 items on SRV. 148 articles were excluded thereafter because they did not meet the criteria for SRV with 16 articles left. The remaining 16 articles fulfilled the inclusion criteria and were further assessed for eligibility and 9 were excluded further, because they did not strictly fulfill the criteria for SRV. Finally, 7 research articles were included in the systematic review and quantitative synthesis. Results: Valvar pulmonary stenosis is the most common diagnosis warranting balloon pulmonary valvotomy (BPV). There is no gender predilection for SRV; 7 males and 6 females. The age range is 5–28 years with a mean of 19.8 ± 6.6 at 95 CI. The most common etiology of SRV was caused by a prior supra systemic right ventricle. In one study, 108 patients had balloon pulmonary valvuloplasty, but only 0.92% (1/108) of the patients who was >5 years developed SRV. Furthermore, out of six patients in another study who had BPV, only one (16.6%) had SRV. The commonest clinical presentation of SRV is cardiovascular collapse, the SRV was relieved in some cases by removing the offending agent (catheter) while some were treated with volume expanders and Beta-blockers. Conclusion: This review has shown that SRV is a rare abnormality in children and adults. It normally occurs during balloon valvotomy or surgical valvotomy.

Association of left atrial pressure with left atrial volume and N-terminal prohormone brain natriuretic peptide in children with cardiomyopathy.

Enlargement of the left atrium is a non-invasive marker of diastolic dysfunction of the left ventricle, a determinant of prognosis in children with cardiomyopathy. Similarly, N-terminal prohormone brain natriuretic peptide is a useful marker in the management of children with cardiomyopathy and heart failure. The aim of this study is to evaluate the association of left atrial pressures with left atrial volume and N-terminal prohormone brain natriuretic peptide in children with cardiomyopathy. This was a retrospective study reviewing the medical records of patients <18 years of age, who were diagnosed with cardiomyopathy or acute myocarditis with eventual development of cardiomyopathy. Left atrial volume by transthoracic echocardiogram and pulmonary capillary wedge pressure, a surrogate of left atrial pressure, obtained by means of cardiac catheterisation were analysed. In addition, N-terminal prohormone brain natriuretic peptide levels obtained at the time of the cardiac catheterisation were also reviewed. Statistical analysis was performed to evaluate the association of left atrial pressures with left atrial volume and N-terminal prohormone brain natriuretic peptide levels. There was a linear correlation of left atrial pressure estimated in the cardiac catheterisation with indexed left atrial volume (r=0.63; p<0.001) and left atrial volume z-scores (r=0.59; p<0.001). We found no statistically significant association between the left atrial pressure and N-terminal prohormone brain natriuretic peptide levels. Left atrial volume measured non-invasively by echocardiography can be used as a surrogate for left atrial pressure in assessing diastolic dysfunction of the left ventricle in children with cardiomyopathy. The larger the size of the left atrium, worse is the diastolic function of the left ventricle.

Knowledge-based reconstruction for measurement of right ventricular volumes on cardiovascular magnetic resonance images in a mixed population.

Follow-up of right ventricular performance is important for patients with congenital heart disease. Cardiac magnetic resonance imaging is optimal for this purpose. However, observer-dependency of manual analysis of right ventricular volumes limit its use. Knowledge-based reconstruction is a new semiautomatic analysis tool that uses a database including knowledge of right ventricular shape in various congenital heart diseases. We evaluated whether knowledge-based reconstruction is a good alternative for conventional analysis. To assess the inter- and intra-observer variability and agreement of knowledge-based versus conventional analysis of magnetic resonance right ventricular volumes, analysis was done by two observers in a mixed group of 22 patients with congenital heart disease affecting right ventricular loading conditions (dextro-transposition of the great arteries and right ventricle to pulmonary artery conduit) and a group of 17 healthy children. We used Bland-Altman analysis and coefficient of variation. Comparison between the conventional method and the knowledge-based method showed a systematically higher volume for the latter group. We found an overestimation for end-diastolic volume (bias -40 ± 24 mL, r = .956), end-systolic volume (bias -34 ± 24 mL, r = .943), stroke volume (bias -6 ± 17 mL, r = .735) and an underestimation of ejection fraction (bias 7 ± 7%, r = .671) by knowledge-based reconstruction. The intra-observer variability of knowledge-based reconstruction varied with a coefficient of variation of 9% for end-diastolic volume and 22% for stroke volume. The same trend was noted for inter-observer variability. A systematic difference (overestimation) was noted for right ventricular size as assessed with knowledge-based reconstruction compared with conventional methods for analysis. Observer variability for the new method was comparable to what has been reported for the right ventricle in children and congenital heart disease with conventional analysis.

Microsurgical management of pediatric ependymomas of the fourth ventricle via the trans-cerebellomedullary fissure approach: A review of 26 cases.

In the present study, the microsurgical management of 26 ependymomas of the fourth ventricle in children via the trans-cerebellomedullary fissure (CMF) approach was reviewed and evaluated. Clinical data were obtained from 26 ependymomas of the fourth ventricle treated with microsurgery using the trans-CMF approach from March 2006 to September 2010 at the Department of Neurosurgery of The First Affiliated Hospital of China Medical University (Shenyang, China). These data were collected and analyzed. Suboccipital median posterior fossa craniotomy and trans-CMF approach were performed in all cases for the microsurgical removal of the tumors. An additional incision was performed in the inferior medullary velum of 5 patients, in order to obtain adequate exposure of the tumors. As a result, all tumors were well exposed during surgery. Gross total resection (GTR) was achieved in 22 cases, near total resection (NTR) in 3 cases and subtotal resection (STR) in 1 case. All excised tumors were pathologically confirmed. No mortality occurred intraoperatively, and no patient presented with mutism or any other surgery-related complications. One patient suffered from postoperative hydrocephalus and received ventriculoperitoneal shunting, which relieved the symptoms. Over the 3.0-7.5-year follow-up period (mean, 4.8 years), tumor relapse occurred in 1 case with GTR, 2 cases with NTR and 1 case with STR. In total, 3 patients succumbed to tumor relapse and 4 were lost to follow-up. According to the literature and the clinical experience of the present authors, the trans-CMF approach provides safe and sufficient access to the fourth ventricle without the requirement of an incision in the inferior vermis. This approach prevents damage to the normal cerebellum and improves the surgical outcome. Tumor removal, restoration of cerebrospinal fluid circulation and preservation of brainstem function are factors that should be taken into consideration during surgery. For patients with residual tumors, adjuvant radiotherapy and/or chemotherapy may be beneficial.

Choroid plexus tumours in childhood: Experience in Sant Joan de Déu hospital

Choroid plexus tumours are rare, with a peak incidence in the first two years of life. The most common location is the lateral ventricle in children, while in adults it is the fourth ventricle. The most common clinical manifestation is the signs and symptoms of intracranial hypertension. They are histologically classified as plexus papilloma, atypical plexus papilloma, and plexus carcinoma. A review is presented on choroid plexus tumours treated in the Hospital Sant Joan de Déu between 1980 and 2014. A total of 18 patients have been treated. An analysis was made of the demographic, clinical, histological data, treatment, and recurrences. The treatment of choice is complete resection, accompanied by adjuvant therapy in carcinomas. In atypical papillomas, the use of adjuvant therapies is controversial, reserving radiation therapy for recurrences. Papillomas have a good outcome, whereas atypical papillomas and carcinomas outcome is poor.