Cardiac findings in pediatric patients with spinal muscular atrophy types 2 and 3.

BACKGROUND: It is unclear whether the heart is affected in pediatric patients with milder forms of spinal muscular atrophy (SMA). Therefore, we aimed to determine the presence of any cardiac abnormalities in these patients. METHODS: We conducted a cross-sectional study of children and adolescents with SMA types 2 and 3 between July 2018 and July 2019. All patients underwent a comprehensive cardiac evaluation, including history-taking, physical examination, electrocardiography, echocardiography, measurement of cardiac biomarkers (cardiac troponin T [cTnT] and N-terminal pro-brain natriuretic peptide [NT-proBNP]), and 24-hour Holter monitoring. RESULTS: In total, 42 patients were enrolled (27 and 15 with SMA type 2 and 3, respectively). No patient had structural heart disease, except for one with mitral valve prolapse. None had signs of ventricular dysfunction on echocardiography. Both cTnT and NT-proBNP levels were normal in all patients. Electrocardiography showed sinus tachycardia in seven patients (16.7%), and prolonged P-R interval in one (2.4%). Holter monitoring detected benign ventricular arrhythmias in two patients (4.8%), and rare supraventricular premature beats in one. The mean 24-hour heart rate was elevated in six patients (14.3%), whereas both the minimum 24-hour heart rate and the maximum R-R interval were increased in 23 (54.8%). DISCUSSION: The prevalence of cardiac disease in pediatric patients with SMA types 2 and 3 is low; however, these patients may have increased resting heart rates. A complete cardiac history and physical examination are a useful screen. Additional cardiac investigations may be performed as needed.

Zero-Fluoroscopy Catheter Ablation of Supraventricular Tachycardias in the Pediatric Population.

Catheter ablation (CA) of supraventricular tachycardias (SVTs) is conventionally performed with the aid of X-ray fluoroscopy. Usage of a three-dimensional (3D) electro-anatomical mapping (EAM) system and intracardiac echocardiography (ICE) enables zero-fluoroscopy ablation, eliminating the harmful effects of radiation. We retrospectively analyzed the feasibility, effectiveness and safety of zero-fluoroscopy radiofrequency and cryoablation of various types of SVTs in pediatric patients. Overall, in 171 consecutive patients (12.5 ± 3.9 years), 175 SVTs were diagnosed and 201 procedures were performed. The procedural success rate was 98% (193/197), or more precisely, 100% (86/86) for AVNRT, 95.8% (91/95) for AVRT, 94.1% (16/17) for AT and 100% (2/2) for AFL. No complications were recorded. Follow-up was complete in 100% (171/171) of patients. During the mean follow-up period of 488.4 ± 409.5 days, 98.2% of patients were arrhythmia-free with long-term success rates of 98.7% (78/79), 97.5% (78/80), 100% (13/13) and 100% (2/2) for AVNRT, AVRT, AT and AFL, respectively. Zero-fluoroscopy CA of various types of SVTs in the pediatric population is a feasible, effective and safe treatment option.

Agreement between children and parents when reporting anxiety and depressive symptoms in pediatric epilepsy.

The levels of agreement between self- and parent/proxy-reports of anxiety and depressive symptoms in pediatric epilepsy were evaluated. Data were drawn from 56 pairs of children with epilepsy and at least one parent. Anxiety symptoms were assessed using the Screen for Child Anxiety Related Emotional Disorders (SCARED), while depressive symptoms were assessed using the Mood and Feeling Questionnaire (MFQ). Moderate to substantial levels of agreement between raters when reporting various anxiety symptoms, such as panic/somatic disorder, generalized anxiety disorder, separation anxiety disorder, social phobia, and school phobia symptoms, were observed. Levels of agreement between raters were substantial when reporting depressive symptoms. However, levels of agreement differed if raw or criterion-referenced questionnaires' scores were used. In case of using raw questionnaire scores, substantial agreements appeared when reporting overall anxiety and depressive symptoms. On the other hand, moderate agreements appeared when reporting particular anxiety symptoms with raw questionnaire scores or when using criterion-referenced scores that indicate the presence of certain symptoms in a clinical range. Therefore, it is advisable to include both raters when assessing anxiety and depressive symptoms in pediatric epilepsy.

Combined contribution of endothelial relaxing autacoides in the rat femoral artery response to CPCA: an adenosine A2 receptor agonist.

We examined the contribution of endothelial relaxing factors and potassium channels in actions of CPCA, potent adenosine A(2) receptor agonist, on isolated intact male rat femoral artery (FA). CPCA produced concentration-dependent relaxation of FA, which was notably, but not completely, reduced after endothelial denudation. DPCPX, A(1) receptor antagonist, had no significant effect, while SCH 58261 (A(2A) receptor antagonist) notably reduced CPCA-evoked effect. Pharmacological inhibition of nitric oxide synthase or cyclooxygenase comparably reduced CPCA-evoked action, still in a lesser degree than after denudation. In the presence of buffer with high K(+) (100 mM), CPCA-produced relaxations were almost abolished. TEA (nonselective K(Ca) blocker), glibenclamide (K(ATP) blocker), Ba(++) (K(IR) blocker), or ouabain (Na(+)/K(+)-ATPase inhibitor) did not change CPCA-induced relaxation. Concentration-response curve for CPCA was significantly shifted to the right after the incubation of apamin (SK channel blocker). CPCA produced concentration-dependent relaxation of FA that was partly dependent on endothelial cells. Endothelium-related portion of CPCA-elicited effect was mediated by combined action of endothelial NO, prostacyclin, and EDHF after activation of endothelial A(2A) receptors. Small conductance K(Ca) channels were involved in this action.

Protein-losing enteropathy managed with percutaneous enlargement of a restrictive atrial septal defect.

Protein-losing enteropathy is one of the most feared complications of the Fontan circulation. The diagnosis of protein-losing enteropathy in this setting should prompt a thorough investigation for the presence of a treatable hemodynamic impairment. In this report, we describe a complete reversal of protein-losing enteropathy following percutaneous enlargement of a restrictive atrial septal defect in a patient with a fenestrated lateral tunnel Fontan and severe mitral stenosis.

Fluoroless catheter ablation of accessory pathways in adult and pediatric patients: a single centre experience.

Catheter radio-frequency ablation (RFA) and cryo-ablation (CRA) procedures are an effective and safe treatment options for adult and pediatric patients with accessory pathway (AP) mediated tachycardias. Non-fluoroscopic techniques during catheter ablation (CA) procedures reduce potentially harmful effects of radiation. Our aim was to investigate the efficacy and safety of completely fluoroless RFA and CRA procedures in pediatric and adult patients with APs. Consecutive patients with AP-related tachycardia and high risk asymptomatic ventricular pre-excitation were assessed in retrospective analysis. Three-dimensional (3D) electro-anatomical mapping (EAM) and intra-cardiac echocardiography (ICE) were used as principal imaging modalities. Fluoroscopy was not used during any stage of the procedures. Among 116 included patients (22.76 ± 16.1 years, 68 patients < 19 years), 60 had left-sided APs, 16 right-sided APs and 40 septal APs. Altogether, 96 had RFA and 20 CRA procedures. The acute success rates (ASR) of RFA and CRA were 97.9% and 95%, respectively (p = 0.43), with recurrence rates (RR) of 8.33% and 40%, respectively (p < 0.0001). The outcome difference was principally driven by lower RR with RFA in septal APs (9.1% vs. 38.9%, p = 0.025). Pediatric patients with APs (12.21 ± 3.76 years) had similar procedural parameters and outcomes compared to adult patients. There were no procedure-related complications. In adult and pediatric patients with AP-related tachycardias, both CRA and RFA can be effectively and safely performed without the use of fluoroscopy. In addition, RFA resulted in better outcomes compared to CRA.

Oral propranolol for infantile hemangiomas: a prospective study on the role of 48-hour Holter monitoring in additional safety assessment.

PURPOSE: Oral propranolol has been recently approved for infantile hemangiomas (IHs), but potential side effects stay a challenge. We sought to make an additional assessment on oral propranolol safety for this indication. MATERIALS AND METHODS: Prospective study included 108 infants consecutively treated for IHs at the University Children's Hospital Tirsova, Belgrade from January 2010 to December 2013. Propranolol was administered orally at a daily dose of 0.5 mg/kg and doubled every 48 hours in the absence of side effects until reaching the maximum dose of 2 mg/kg daily. Systolic and diastolic blood pressure and heart rate were measured every 48 hours with clinical observation. Heart rate was monitored by standard electrocardiogram (ECG) and 48-hour Holter ECG. RESULTS: Statistically significant, but asymptomatic decreases in systolic blood pressure and heart rate recorded by Holter ECG were observed during the first doubling of dose and then remained stable. Arrhythmias were not detected. Despite mild sleep disturbance observed in 31% of infants in the hospital milieu, Holter monitoring indicated circadian rhythm maintenance. CONCLUSIONS: Oral propranolol for IHs does not remarkably affect heart rhythm including circadian variations throughout hospital initiation. Therefore, there is no necessity for Holter monitoring in additional safety assessment.

Association of Serum Pentraxin-3 and High-Sensitivity C-Reactive Protein with the Extent of Coronary Stenosis in Patients Undergoing Coronary Angiography.

BACKGROUND: We compared factors of inflammation - high sensitivity C-reactive protein (hsCRP) and pentraxin-3 (PTX3), and we explored their relationship with coronary artery disease (CAD). Also, we tested the usefulness of hsCRP and PTX3 in the risk assessment of coronary stenosis development and the diagnostic ability of these biomarkers to detect disease severity. METHODS: The study group consisted of 93 CAD patients undergoing coronary angiography. Patients were divided into CAD(0), representing subclinical stenosis, and CAD (1-3), representing significant stenosis in one, two or three vessels. RESULTS: We determined the concentration of lipid status parameters, hsCRP and PTX3. We found significantly lower PTX3 and hsCRP concentrations in CAD(0) than in CAD(1-3) group. Concentration of PTX3 showed an increasing trend with the increasing number of vessels affected. The area under ROC curve (AUC) for the combinations of hsCRP and PTX3 with lipid parameters had useful accuracy for detecting CAD(1-3) patients (AUC=0.770, p<0.001). CONCLUSION: PTX3 is a promising independent diagnostic marker for identifying patients with CAD, and a useful indicator of disease progression. In all the analyses PTX3 showed better performance than hsCRP. A combination of PTX3, hsCRP with the lipid status parameters provides risk stratification of the development of coronary stenosis and better classification than their individual application.

Effects of diabetes and vascular occlusion on adenosine-induced relaxant response of rat common carotid artery.

BACKGROUND: The aim of this study was to investigate effect of adenosine on isolated rat common carotid artery (CA) submitted to occlusion in non-diabetic or diabetic animals, and to determine whether endothelium denudation or potassium conductance block affects adenosine action. METHODS: Experiments were conducted on Wistar rat CA with or without endothelium. Diabetes was induced by alloxan. Occlusion of CA was performed in randomly selected non-diabetic or diabetic animals anesthetized with urethane. Thus, experiments were performed in four groups of rats: non-operated (control) animals without or with diabetes and operated animals submitted to the occlusion of CA without or with diabetes. Concentration-response curves for adenosine were obtained in a cumulative fashion on precontracted arteries. RESULTS: Adenosine produced concentration-dependent and endothelium-independent relaxation of CA with comparable maximal effects in all groups. Analysis of pEC50 values showed that responsiveness of CA decreased in following order: [diabetes (-) / occlusion (-)] = [diabetes (-) / occlusion (+)] > [diabetes (+) / occlusion (-)] > [diabetes (+) / occlusion (+)]. In the presence of high K(+) maximal relaxant response of CA from non-operated rats without diabetes was reduced. The recorded inhibition was even stronger in animals subjected to CA occlusion. Conversely, in non-operated diabetic animals obtained reduction of adenosine effect was less pronounced in regard to non-diabetic rats. CONCLUSIONS: Adenosine produced equi-effective endothelium-independent relaxation of CA in all groups. Pharmacological potency of adenosine was reduced in diabetic animals solely, but even more in diabetic rats submitted to CA occlusion. The enhanced potassium transmembrane flow has certain protective role on adenosine-induced action in occluded CA from non-diabetic rats. Conversely, diabetes solely inhibited adenosine-evoked cascade connected to increased potassium conductance.

Two rare conditions in an Eisenmenger patient: left main coronary artery compression and Ortner's syndrome due to pulmonary artery dilatation.

The left-main coronary artery extrinsic compression due to enlarged pulmonary artery has been described in several case series. Ortner's syndrome is also a rare condition in some cardiovascular disorders. There have been no reports about these two rare conditions in the same patient. Hence, we report a very rare case of an Eisenmenger patient with severe pulmonary hypertension and dilated pulmonary artery which has compressed the left main coronary artery, severely narrowing it, and the left laryngeal recurrent nerve with subsequent Ortner's syndrome and brief literature review.

Contribution of thromboxane a2 in rat common carotid artery response to serotonin.

Serotonin is a vasoactive substance that in different blood vessels mostly induces vasoconstriction. Considering the important role of common carotid artery in brain blood supply, the aims of this study were to investigate the effect of serotonin on isolated rat common carotid artery and also to examine participation of intact endothelium, cyclooxygenase products, Ca(++) channels and 5-HT(2) receptors in serotonin-evoked action. Endothelium was mechanically removed from some vascular rings. Circular artery segments were placed in organ baths containing KrebsâRinger bicarbonate solution. Cumulative concentration-contraction curves for serotonin were obtained in rings previously equilibrated at basal tone. Serotonin produced concentration-dependent contraction, which was unaltered by endothelial denudation. Serotonin-induced effect was notably and comparably reduced by indomethacin (cyclooxygenase inhibitor) or OKYâ046 (thromboxane A(2)-synthase inhibitor) on intact or denuded rings. Nifedipine (Ca(++) channel blocker) or ketanserin (5-HT(2) receptor antagonist) strongly reduced serotonin-evoked effect. Our results suggest that serotonin produced concentration-dependent and endothelium-independent contraction of carotid artery, which was initiated by activation of 5-HT(2) receptors located on smooth muscle cells and mediated via L-type Ca(++) channels. Thromboxane A(2) from smooth muscle cells notably contributed to the overall contraction of carotid artery induced by serotonin.