Early echocardiographic signs of cardiovascular affection in pediatric familial hypercholesterolemia.

Familial hypercholesterolemia (FH) is a rare autosomal dominant genetic disorder caused by defective low-density lipoprotein (LDL) receptors or abnormal apolipoprotein B. FH raises the risk of premature atherosclerotic disease and cardiovascular death in young adults. However, cardiovascular affection in children needs to be more adequately studied. Our study aimed to evaluate the effect of hypercholesterolemia on the cardiovascular system of pediatric patients with homozygous FH using conventional and advanced echocardiographic parameters such as tissue Doppler imaging (TDI) and 2-dimensional speckle-tracking echocardiography (2D-STE). This case-control study matched 25 healthy children with 21 patients with homozygous FH. Both groups had conventional echocardiography, TDI, and 2D-STE. Myocardial velocities of the left and right ventricles, left ventricular strain, and aortic stiffness parameters were measured. The FH group had greater systolic blood pressure, dilated coronary arteries, and hypertrophied left ventricle (LV) compared to the control (P = 0.0001, P = 0.001, P = 0.01, respectively). The mitral E/E' ratio was higher in the patient group than in the control group (P = 0.007), indicating LV diastolic dysfunction in patients. At the same time, LV systolic function evaluated by 2D-STE was comparable to that in the control group. The abdominal aorta circumferential strain and ascending aorta M-mode-derived strain were significantly lower in patients compared to those in the control (P = 0.024, P = 0.0001, respectively), indicating increased aortic stiffness in the patients' group; moreover, 85.7% of patients had mild aortic insufficiency.  Conclusion: Mild aortic insufficiency, coronary artery dilatation, left ventricular (LV) diastolic dysfunction, and increased aortic stiffness are among early cardiovascular markers in pediatric patients with homozygous FH before impaired LV systolic function. What is Known: • Familial hypercholesterolemia (FH) in adults is associated with accelerated atherosclerosis, aortic valvopathy, dilated coronary arteries, ischemic heart disease, and premature cardiovascular death. • The cardiovascular effects of FH in children require additional research. What is New: • Pediatric patients with familial hypercholesterolemia tend to have an early affection for left ventricular diastolic function before the affection for the systolic function. • The diastolic dysfunction associated with pediatric FH is correlated to the aortic stiffness and low-density lipoprotein levels.

Using 2D speckle-tracking echocardiography to localize the accessory pathway and evaluate cardiac function and dyssynchrony in pediatric patients with Wolf-Parkinson-White syndrome.

Wolf-Parkinson-White (WPW) accessory pathway (AP) may be associated with reentry supraventricular tachycardia (SVT) in addition to ventricular dyssynchrony and cardiac dysfunction. Electrophysiological studies (EPS) are the gold standard for the localization of the AP; however, 2D speckle-tracking echocardiography (2D-STE) may help in the localization of the AP noninvasively. Our study aims to evaluate the capability of 2D-STE for AP localization and the identification of AP-related contractile abnormalities and dyssynchrony in pediatric patients with WPW syndrome. This prospective multicenter cohort study involved 18 pediatric patients with ventricular preexcitation from January 2021 to January 2023. Tissue Doppler imaging (TDI), conventional echocardiography, and 2D-STE were done. Myocardial velocities, myocardial performance index (MPI), the global and segmental longitudinal strain of the left ventricle (LV), and time-to-peak longitudinal strain (TPLS) were measured before and after ablation. The longitudinal strain of the LV segments supplied by the AP, or the nearby segments close to the AP, was significantly impaired and improved after ablation (P = 0.0001). The abnormal strain pattern in the affected segments could predict the location of the AP. The TPLS of the affected segments significantly increased after ablation (P = 0.0001), denoting improved dyssynchrony. The ejection time and the LV MPI measured at the basal septum improved significantly after ablation. CONCLUSIONS: 2D STE may be used for noninvasive localization of the AP and to evaluate cardiac function and dyssynchrony in patients with WPW. Further research on more patients is necessary to validate this method for AP localization. WHAT IS KNOWN: • Accessory pathways (AP) associated with the Wolf-Parkinson-White (WPW) syndrome have been linked to supraventricular tachycardia (SVT). Even without SVT, WPW can cause left ventricular dyssynchrony, contractile dysfunction, and cardiomyopathy. • Electrophysiology study is the gold standard for the localization of the AP in WPW syndrome. WHAT IS NEW: • The combination of 2D-speckle-tracking echocardiography (2D-STE) and the modified Arruda algorithm can precisely localize the AP associated with WPW syndrome. • 2D-STE can potentially assess cardiac function and dyssynchrony related to WPW syndrome. Additionally, 2D-STE can be utilized to evaluate the effectiveness of ablation in restoring cardiac function and dyssynchrony.

PARP1, BRCA1 and androgen receptor expression in triple-negative breast cancer patients treated with neoadjuvant chemotherapy / Expresión de receptores de PARP1, BRCA1 y andrógenos en pacientes de cáncer de mama triple negativo tratados con quimioterapia neoadyuvante

Introduction: Triple-negative breast cancer (TNBC) is an aggressive subtype, where no effective therapies have been established for it. Searching for therapeutic targets for TNBC patients is the aim of the current research. Poly adenosine diphosphate ribose polymerase (PARP1) inhibitors are promising antitumor therapy that have a high potency in BRCA1-deficient breast cancers. Materials and methods: Forty TNBC patients who received neoadjuvant chemotherapy (NAC) were enrolled in this study, and evaluated for PARP1, BRCA1, and Androgen receptor (AR) immunohistochemical expression before and after receiving NAC. Data of patients' clinical and pathological responses to the chemotherapy were collected and finally analyzed. Results: The immunohistochemical results revealed 10 cases (25%) positive for AR, while 18 cases (45%) and 22 cases (55%) expressed PARP1 at low and high levels, respectively. Twelve cases (30%) and 28 cases (70%) expressed BRCA1 at low and high levels, respectively. There was a significant difference between PARP1 expression in normal and malignant tissues (P < 0.001). Higher PARP1 expression was correlated with a better overall clinical response (OAR) and pathological complete response (pCR) (P = 0.018, 0.01 respectively). Co-expression of both PARP1 & BRCA1 was correlated with OAR and pCR. Chemotherapy decreases PARP1 protein levels in matched patient samples (P = 0.015). Positive AR expression was correlated with BRCA1 overexpression. Conclusion: PARP1 is highly expressed in TNBC with a better OAR and pCR especially in cases with high BRCA1, so it might be considered as a therapeutic target for this risky group. (AU)

Introducción: El cáncer de mama triple negativo (TNBC) es un subtipo agresivo, donde no se han establecido terapias efectivas para este cáncer. La búsqueda de dianas terapéuticas para pacientes con TNBC es el objetivo de la investigación actual. Los inhibidores de la poli adenosina difosfato ribosa polimerasa (PARP1) son prometedores terapia antitumoral que tienen una alta potencia en los cánceres de mama deficientes de BRCA1. Materiales y métodos: Cuarenta pacientes de TNBC que recibieron quimioterapia neoadyuvante (NAC) se inscribieron en este estudio y se evaluaron para la expresión inmunohistoquímica de PARP1, BRCA1 y receptores de andrógenos (AR) antes y después de recibir NAC. Se recogieron y finalmente se analizaron los datos de las respuestas clínicas y patológicas de los pacientes a la quimioterapia. Resultados: Los resultados inmunohistoquímicos revelaron 10 casos (25%) positivos para AR, mientras que 18 casos (45%) y 22 casos (55%) expresaron PARP1 en niveles bajos y altos, respectivamente. Doce casos (30%) y 28 casos (70%) expresaron BRCA1 en niveles bajos y altos, respectivamente. Hubo una diferencia significativa entre la expresión de PARP1 en tejidos normales y malignos (P 0.001). Una mayor expresión de PARP1 se correlacionó con una mejor respuesta clínica global (OAR) y una respuesta patológica completa (pCR) (P = 0,018, 0,01 respectivamente). La co-expresión de ambos PARP1 y BRCA1 se correlacionó con OAR y pCR. La quimioterapia disminuye los niveles de proteína PARP1 en muestras de pacientes emparejadas (P = 0,015). La expresión positivos de AR se correlacionó con la expresión de BRCA1. Conclusión: El PARP1 está muy expresado en TNBC con una mejor OAR y pCR especialmente en casos con BRCA1 alto, por lo que podría ser considerado como una diana terapéutica para este grupo de riesgo. (AU)

Functional recovery of cardiomyopathy induced by atrial tachycardia in children: Insight from cardiac strain imaging.

AIM: To evaluate systolic and diastolic cardiac function in children who had cardiomyopathy induced by ectopic atrial tachycardia (EAT). METHODS: Twenty-two pediatric patients who had cardiomyopathy induced by EAT and 25 age-matched controls were recruited in this case-control study. The patients were examined after rhythm control and normalization of their left ventricular systolic function. Different echocardiographic modalities including tissue Doppler imaging and two-dimension speckle tracking echocardiography were utilized to assess the ventricular and atrial function. RESULTS: The patients' median age was 51 months (interquartile range: 28.5-84 months). The median time interval required for normalization of left ventricular ejection fraction (EF) among patients was 1.5 months (interquartile range: 1.5-2.12 months). Compared to controls, patients had a significantly higher median left ventricular myocardial performance index (MPI) at the interventricular septum (0.44 vs. 0.38, p = .001) and left ventricular lateral wall (0.46 vs. 0.32, p = .0001). The median right ventricular MPI of the patients' group was significantly higher when compared to the control group (0.34 vs. 0.26, p = .0001). The median right atrial (RA) reservoir function in patients was significantly reduced compared to controls (30% vs. 36.63%, p = .007). CONCLUSIONS: Shortly after rhythm normalization and restoration of left ventricular EF, using tissue Doppler imaging and two-dimension speckle tracking echocardiography, children who had cardiomyopathy induced by EAT continue to have left ventricular diastolic dysfunction, right ventricular dysfunction, and reduced RA reservoir function.

Neuropathogenic human coronaviruses: A review.

Human Coronaviruses (HCoVs) have long been known as respiratory viruses. However, there are reports of neurological findings in HCoV infections, particularly in patients infected with the novel severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) amid Coronavirus disease 2019 (COVID-19) pandemic. Therefore, it is essential to interpret the interaction of HCoVs and the nervous system and apply this understanding to the COVID-19 pandemic. This review of the literature analyses how HCoVs, in general, and SARS-CoV-2, in particular, affect the nervous system, highlights the various underlying mechanisms, addresses the associated neurological and psychiatric manifestations, and identifies the neurological risk factors involved. This review of literature shows the magnitude of neurological conditions associated with HCoV infections, including SARS-CoV-2. This review emphasises, that, during HCoV outbreaks, such as COVID-19, a focus on early detection of neurotropism, alertness for the resulting neurological complications, and the recognition of neurological risk factors are crucial to reduce the workload on hospitals, particularly intensive-care units and neurological departments.

Subtle cardiac dysfunction in nephropathic cystinosis: insight from tissue Doppler imaging and 2D speckle tracking echocardiography.

BACKGROUND: Nephropathic cystinosis is a rare autosomal recessive lysosomal storage disorder that initially affects the kidney progressing to multi-organ failure due to accumulation of cystine in all tissue compartments. OBJECTIVE: The main objective of this study is the evaluation of cardiac function in cystinosis patients using non-conventional echocardiographic modalities like pulsed wave tissue Doppler imaging (PW-TDI) and 2D speckle tracking echocardiography (2D-STE). METHODS: This is a case control study conducted on fifteen patients with cystinosis and 15 normal controls. Echocardiography was done for all participants and PW-TDI was performed for measurement of S', E', A' velocities and myocardial performance index (MPI) at basal parts of septal, left ventricle (LV), and right ventricle (RV) free walls. 2D-STE was done for evaluation of global longitudinal strain (GLS), global circumferential strain (GCS), and global radial strain (GRS) of LV. Mitral E and A velocities and tricuspid annular plane systolic excursion (TAPSE) were also measured. RESULTS: The GLS, GRS, and S' velocity at basal septum and LV lateral wall were significantly lower in patients denoting LV systolic dysfunction (p = 0.005, p < 0.0001, p = 0.001, p = 0.006, respectively), while E/E' were significantly higher in patients group denoting LV diastolic dysfunction (p < 0.001). For RV function, TAPSE, S', and E' velocity were significantly lower in patients group (p 0.013, p < 0.01, p = 0.05, respectively) indicating RV systolic and diastolic dysfunction. The TDI-derived MPI for both LV and RV were significantly higher in patients group (p < 0.0001, p < 0.01, respectively) indicating both ventricular systolic and diastolic dysfunction. For prediction of cardiac dysfunction among patients, the receiver operating characteristic (ROC) curve showed that GRS ≤ 29% had sensitivity 93.3% and specificity 100%, GLS > - 20.1% had sensitivity 66.7% and specificity 93.3%, LV-E/E' >7.87 had sensitivity 73.3% and specificity 93.3%, and MPI-LV > 0.36 had sensitivity 100% and specificity 93.3% while MPI-RV > 0.29 had sensitivity 80% and specificity 93.3% and TAPSE ≤ 19 mm had sensitivity 80% and specificity 73.3%. CONCLUSIONS: Patients with cystinosis have significant both left and right ventricular dysfunction, which can be better evaluated using the non-conventional echocardiographic modalities like TDI and 2D-STE for early detection of subtle cardiac dysfunction.

Evolution of ventricular function in children with permanent right ventricular pacing after tetralogy of Fallot repair: A midterm follow-up.

OBJECTIVE: We aim to evaluate the midterm effect of chronic apical right ventricular (RV) pacing on right and left ventricular (LV) function using different modalities of echocardiography including conventional echocardiography, tissue Doppler imaging and two-dimension speckle tracking echocardiography. METHODS: This case-control study enrolled 49 patients divided into two groups: a paced group and a nonpaced group. The paced group included 23 patients that underwent tetralogy of Fallot (TOF) repair and required permanent pacemaker insertion for postoperative complete heart block. The nonpaced group included 26 patients that had TOF repair at the same period. RESULTS: The median age for the paced and nonpaced groups was 6 and 8 years, respectively (P = .169). The follow-up duration after TOF surgical repair was 4 years for the paced patients and 5 years for nonpaced patients (P = .411). In the nonpaced group, the QRS duration increased and LV global longitudinal strain (GLS) decreased significantly with increasing duration after TOF repair, P value was .006 and .042, respectively. In the paced group, tricuspid annular systolic plane excursion (TAPSE) was significantly correlated with age (r = .578; P = .004) and duration following TOF correction (r = .724; P < .001). CONCLUSION: Chronic RV apical pacing in children after TOF repair was associated with better clinical status, preservation of RV systolic function, and prevention of progressive QRS prolongation. RV pacing was not associated with progressive deterioration of LV systolic function with increasing the time interval following TOF repair. Therefore, RV pacing can be beneficial in corrected TOF patients presenting with RV failure.

Evaluation of cardiac function in pediatric patients with mild to moderate bronchial asthma in the era of cardiac strain imaging.

OBJECTIVE: Bronchial asthma is a common chronic inflammatory airway disease, which may be associated with pulmonary hypertension and cardiac dysfunction. The aim of this study was to evaluate the ability of 2D-speckle tracking echocardiography (2D-STE) and tissue doppler imaging (TDI) to detect subtle cardiac dysfunction in pediatric patients with mild to moderate bronchial asthma. METHODOLOGY: The study included 30 children with mild to moderate bronchial asthma and 27 age-matched healthy controls. Both groups underwent pulmonary function tests, TDI and 2D-STE. Myocardial performance index (MPI), S', E', A' velocities, global strain of left ventricle (LV), right ventricle (RV), and right atrium (RA) were measured. RESULTS: RV diastolic function was impaired in the patient group, as the tricuspid E' velocity was significantly lower in the patients when compared with the controls (16 [14-17] vs 16 [17-19] cm/s, P = .044), while the RV-MPI was significantly higher in patients when compared to controls (0.30 [0.27-0.36] vs 0.30 [0.30-0.30], P = .001). The global RV longitudinal strain, RA strain, and LV strain did not show significant differences between the test and the control groups. RV systolic parameters and LV systolic and diastolic parameters did not differ significantly between the two groups. CONCLUSION: Pediatric patients with mild to moderate bronchial asthma may have early RV diastolic dysfunction with preserved other cardiac functions.

Correlation of Appearance of MRI Perinidal T2 Hyperintensity Signal and Eventual Nidus Obliteration Following Photon Radiosurgery of Brain AVMs: Combined Results of LINAC and Gamma Knife Centers.

BACKGROUND: A wide variety of radiologic changes occur within and adjacent to the nidus of arteriovenous malformations (AVMs) after stereotactic radiosurgery (SRS). Our objective was to study the magnetic resonance imaging(MRI)-defined changes following photon radiosurgery of AVMs and specifically to correlate the appearance of a perinidal T2 hyperintensity signal with the eventual angiographic obliteration of an AVM nidus in response to SRS treatment. MATERIAL AND METHODS: This retrospective study was conducted on 62 patients with brain AVMs who received photon SRS treatments between 2004 and 2017, using either a technique based on a linear accelerator at the Alexandria LINAC Radiosurgery Center in Egypt (21 patients/AVMs) or a technique based on a gamma unit at the Koto Memorial Gamma Knife Center in Japan (41 patients/AVMs). All patients included in the study had serial clinical and radiologic follow-ups for ≥ 2 years after SRS treatments. RESULTS: In the combined study series of 62 patients/AVMs treated with photon SRS, the follow-up MRIs revealed that 50 AVMs (80.6%) showed nonvisualized nidus and 12 AVMs (19.4%) showed decreased nidus size. Radiation-induced changes, defined as appearance of perinidal T2 hyperintensities in post-SRS MRIs, occurred in 34 patients (54.8%). Of the 35 patients with available follow-up angiographic studies, 30 AVMs (85.7%) demonstrated complete nidus obliteration at a mean of 36 months (range: 8-66 months) after SRS. Of the 30 AVMs with both MRI evidence of a nonvisualized nidus and angiographic verification of complete nidus obliteration, 20 AVMs (66.7%) were associated with prior MRI evidence of the appearance of a perinidal T2 hyperintensity signal at an average of 12 months (range: 6-45 months) after SRS. Of the five AVMs with both MRI evidence of decreased nidus size and angiographic verification of partial nidus obliteration, four AVMs (80%) showed perinidal T2 hyperintensity signal on post-SRS follow-up MRIs. Lower Spetzler-Martin grade (p = 0.013), smaller AVM volume (p = 0.017), and appearance of post-SRS perinidal T2 hyperintensity signal (p = 0.007) were the statistically significant independent predictors of AVM obliteration. The appearance of perinidal T2 hyperintensity signal in the post-SRS MRIs had a sensitivity of 66.7%, a specificity of 20%, and an overall accuracy of 60% in predicting the eventual obliteration of the AVM nidus. CONCLUSIONS: The present study may help improve our current understanding of the mechanisms behind the radiation-induced tissue changes following AVM SRS. Because the SRS-induced hemodynamic changes within the AVM nidus initiate the cascade of the subsequent formation of perinidal vasogenic brain edema, the appearance of perinidal high T2 signal in the follow-up MRIs after SRS would be a valuable indicator of the AVM response to SRS. The development of perinidal hyperintensity was the strongest predictive factor of AVM obliteration (p = 0.007), with relatively high sensitivity (66.7%) and accuracy (60%) and fairly low specificity (20%), as a prognostic sign of eventual complete angiographic obliteration of the AVM nidus following SRS.

Diagnostic Value of Magnetic Resonance Spectroscopy Compared with Stereotactic Biopsy of Intra-axial Brain Lesions.

Background Magnetic resonance spectroscopy (MRS) is usually added to conventional magnetic resonance imaging (MRI) to refine the diagnosis of different brain lesions. Stereotactic brain biopsy is a well-established method to obtain tissues for histopathologic examination. The purpose of the study is to compare the diagnostic yields of MRS and stereotactic biopsy in the characterization of brain lesions. Material and Methods A prospective study conducted on 27 consecutive patients presenting with multifocal, diffuse, as well as deeply seated intra-axial brain lesions. All patients had both brain MRI and MRS prior to stereotactic biopsy. Histopathologic examinations of the obtained tissue specimens, using appropriate stains including immunostains, were performed. Results MRS diagnosed neoplastic brain lesions in 15 cases (56%) and nonneoplastic brain lesions in 12 (44%). Correlation between the preoperative diagnosis by MRS and the histopathologic diagnosis following stereotactic biopsy of either a neoplastic or nonneoplastic lesion revealed matching in 25 of 27 cases (sensitivity 88%; specificity 100%). Within the group of cases (n = 15) diagnosed preoperatively by MRS as neoplastic, 12 patients were diagnosed with brain gliomas of different grades. The MRS grading of gliomas exactly matched the histopathologic grading following stereotactic biopsy in 10 of the 12 cases (sensitivity 89%; specificity 67%). Conclusions MRS is a useful addition to the management armamentarium, providing molecular information that assists in the characterization of various brain lesions. Multivoxel MRS may increase the diagnostic yield of stereotactic biopsy by guidance to target the higher choline and lower N-acetylaspartate areas, expected to have greater tumor activity.

Skeletal and cardiac muscle involvement in children with glycogen storage disease type III.

UNLABELLED: Glycogen storage disease type III (GSD III) may present with hepatic disease or may involve both skeletal and cardiac muscles as well. To assess the prevalence of neuromuscular and cardiac involvement in a group of children with GSD III, 28 children with GSD III, diagnosed by enzymatic assay, were enrolled in the study after an informed consent was obtained from their parents/guardians and after the study protocol was approved by our institutional ethical committee. Their mean age was 6.6 + 3.1 years. All cases were assessed neurologically by clinical examination, electromyography (EMG), and nerve conduction velocity. The heart was examined clinically by electrocardiogram and echocardiography. Seventeen patients (61 %) had myopathic changes by EMG, three of them had associated neuropathic changes. Creatine phosphokinase (CPK) was elevated in all myopathic cases except one. Children with myopathic changes were significantly older (p = 0.02), and CPK was significantly higher (p < 0.0001). Nine cases had left ventricular (LV) hypertrophy, seven of them had myopathic changes by EMG. CONCLUSION: Myopathic changes are not uncommon in children with GSD III. Myopathic changes tend to occur in older age and are associated with higher CPK level. Cardiac muscle involvement is less common in this age group and may, on occasion, occur alone without skeletal muscle involvement. Despite mild degrees of affection in this age group, it is recommended to perform prospective annual screening using EMG and echocardiography in order to augment dietary therapy regimen to prevent progression to life threatening complications.

Anticipation and management of junctional ectopic tachycardia in postoperative cardiac surgery: Single center experience with high incidence.

BACKGROUND: Junctional ectopic tachycardia (JET) often occurs in the early postoperative period following surgery for congenital heart diseases and may lead to hemodynamic compromise. Its exact etiology is unknown, however, longer cardiopulmonary bypass (CPB) time, aortic cross clamp (ACC) time, catecholamines, and hypomagnesemia are known risk factors. JET is associated with increased postoperative morbidity and mortality. MATERIALS AND METHODS: A prospective cohort study of 194 consecutive children who underwent open heart surgery on CPB over 1 year period, patients was divided into three groups; JET, non-JET arrhythmia, and no arrhythmia groups. Information on patient's demographics (sex, age, and body weight), type of surgical interventions, duration of CPB and ACC, the use of inotropic support, duration of intensive care unit (ICU) stay, and response to different therapeutic methods were collected. RESULTS: JET was documented in 53 patients (27%) most commonly following tetralogy of Fallot (TOF) repair and was associated with longer CPB and ACC times (118 and 77 min, respectively) as compared to non-JET arrhythmia (93.9 and 55.3 min, respectively) and no arrhythmia groups (94.9 and 54.8 min, respectively). Patients with JET required more inotropic support and longer ICU stay as compared to other groups. Amiodarone was safe and effective in treatment of JET. Atrial electrocardiogram (ECG) and Lewis lead ECG were helpful tools in JET diagnosis. The mortality was 11.5% in JET patients. CONCLUSIONS: Incidence of JET was 27% possibly due to the large number of Fallot repair and Senning operation. Longer CPB and ACC times are risk factors for JET.

Permanent cardiac pacing in pediatric patients.

Pediatric pacemaker (PM) implants comprise less than 1 % of all PM implants. This study aimed to investigate permanent cardiac pacing among the pediatric population, identifying different indications and complications of pediatric cardiac pacing, especially focusing on the effect of the pacing sites, the PM lead type, and the indications for pacing. The current work is a cross-sectional study of 103 procedures for permanent PM insertion in pediatric patients between January 2001 and December 2010. The patients were followed up 1, 3, and 6 months after implantation, then every 6 months or as needed. Evaluation included routine clinical examination, electrocardiography, chest X-ray, echocardiography, and a full analysis of the pacing system measurements. The ages of the patients ranged from 0.09 to 12 years (median, 2.3 years). The most common indication for pacing was postoperative complete heart bock, noted in 54 patients (52.4 %). Transvenous endocardial PM insertion was performed in 92 procedures (89.3 %), whereas transthoracic epicardial insertion was performed in 11 procedures (10.7 %). The most common site of pacing was the right ventricular apex (n = 64, 62 %), followed by the right ventricular outflow tract (n = 25, 24.3 %). Transthoracic epicardial PM insertion was associated with a significantly higher percentage and greater severity of complications. In this study, 65 % of the patients with left ventricle (LV) dilation before pacing showed a significant improvement in LV dimensions and function after pacing. This was noted only in those with endocardially inserted PM leads in both the congenital and the postoperative groups regardless of the pacing site. Endocardial PM insertion in children is a safe procedure with fewer complications and a lower ventricular threshold than the epicardial route. Permanent single-chamber right ventricle pacing is safe and can lead to significant improvement in LV function and dimensions. However, long-term follow-up assessment is needed for further evaluation.

Linear accelerator-based stereotactic radiosurgery of intracranial meningiomas: results of the first 5 years of clinical practice.

Meningiomas are mostly benign but some are atypical or malignant. Surgical resection is curative when complete removal of benign meningiomas is contemplated. Incompletely excised and recurrent tumors are frequently treated with fractionated radiation therapy or stereotactic radiosurgery. The purpose of this study is to evaluate the short-term radiological and functional outcomes of a single center using linear accelerator (Linac) stereotactic radiosurgery for the treatment of intracranial meningiomas. Twenty-nine patients (12 males and 17 females) with 30 meningiomas, in different brain locations (skull base and non-skull base meningiomas), were treated with Linac-based stereotactic radiosurgery. The mean tumor volume was 6.3 cm³, and the mean tumor marginal and maximum doses were 10.9 and 15 Gy, respectively. The median prescribed isodose line was 80%. The patients were followed-up for a minimum of 3 years. Regarding radiological outcome, nine (30%) meningiomas demonstrated evident volume reduction, 19 (63.3%) meningiomas remained unchanged, and two (6.7%) meningiomas increased in size after radiosurgery. The local tumor control rates for skull base meningiomas and non-skull base meningiomas after radiosurgery were 90.9% and 100%, respectively. Regarding functional outcomes, 64% of patients presenting with cranial neuropathies showed improvement of their cranial nerve functions and 29% of patients remained unchanged. One patient had temporary trigeminal neuropathy. Although radiosurgery for meningiomas is generally effective and quite safe in achieving high control rates with minimum morbidity over short- and intermediate-term periods of follow-up, tumor progression might occur in a delayed manner after initial apparent control for few years. We recommend continued follow-up for longer periods to better assess the long-term outcomes.