Stent interventions guided by three-dimensional rotational angiography to treat total cavopulmonary connection stenosis

Background - Stenosis of the total cavopulmonary connection (TCPC), in particular the pulmonary arteries, is common in children with single ventricle physiology. Stenting is an efficient interventional strategy to treat stenosis and optimize TCPC hemodynamics. Methods - A retrospective single center study was performed to investigate the prevalence, causes and outcome of TCPC stenosis in children treated with stent implantation guided by three-dimensional rotational angiography (3DRA). Results - From September 2011 to February 15th 2021 48 patients received 73 stents at 67 locations in 59 3DRA stent procedures. Median age and weight were 3.6 years (0.0–17.2) and 14.1 ​kg (3.4–70.0), respectively. Left pulmonary artery (LPA) stenosis accounted for 75% of the stenosis and is often caused by retroaortic compression. Adverse events occurred in 6 catheterizations (10.2%). Twenty-one patients (43.8%) underwent 27 reinterventions including planned serial redilation (N ​= ​17), extra stent placement (N ​= ​6), surgery (N ​= ​2), unplanned balloon dilation (N ​= ​1) and stent placement (N ​= ​1) for restenosis by intima proliferation. Conclusion - TCPC stenoses, especially of the LPA, are common in children with single ventricle physiology and external compression is one of the main causes. 3DRA safely guides stent interventions as intraprocedural knowledge of possible interactions with surrounding structures are evaluated. Besides it is effective in both short and long term and can be performed in small children. Restenosis by intima proliferation is rare, though repeated redilations are necessary to match stent diameter with patient size

Treatment of infantile hemangiomas: therapeutic options in regard to side effects and adverse events - a review of the literature

Introduction: While options for treatment strategies for infantile hemangiomas (IH) are numerous, evidence-based information about agents, optimal dosage, adverse effects, treatment modality, pretreatment and treatment strategies remain limited. Areas covered: To evaluate side effects and adverse events of medical treatment in children with infantile hemangioma, a comprehensive review of the literature was performed to provide information for daily practice. In total 254 studies were retrieved from medical databases and comprised 10,022 patients divided into 5 different treatment groups. Information about working mechanism, side effects and adverse events of therapies used as a single agent for IH are discussed and evaluated according to information from pharmacotherapeutic databases. Randomized controlled trials have only scarcely been performed for the many therapeutic options reported for IH. Short- and long-term side effects and adverse events, have not been systematically studied. Subsequently information about the medical treatment options and pharmacotheraputic databases for therapy in children with IH are incomplete. Expert opinion: From the many therapeutic options, propranolol is the first-line approach for IH, predominantly based on clinical observation, efficacy and tolerability in the short-term. The unsolved ravels of possible short and long-term adverse events of propranolol used during early developmental stages of children need thorough review

Mid-term echocardiographic follow up of left ventricular function with permanent right ventricular pacing in pediatric patients with and without structural heart disease

BACKGROUND: Chronic right ventricular apical pacing may have detrimental effect on left ventricular function and may promote to heart failure in adult patients with left ventricular dysfunction. METHODS: A group of 99 pediatric patients with previously implanted pacemaker was studied retrospectively. Forty-three patients (21 males) had isolated congenital complete or advanced atrioventricular block. The remaining 56 patients (34 males) had pacing indication in the presence of structural heart disease. Thirty-two of them (21 males) had isolated structural heart disease and the remaining 24 (13 males) had complex congenital heart disease. Patients were followed up for an average of 53 ± 41.4 months with 12-lead electrocardiogram and transthoracic echocardiography. Left ventricular shortening fraction was used as a marker of ventricular function. QRS duration was assessed using leads V(5 )or II on standard 12-lead electrocardiogram. RESULTS: Left ventricular shortening fraction did not change significantly after pacemaker implantation compared to preimplant values overall and in subgroups. In patients with complex congenital heart malformations shortening fraction decreased significantly during the follow up period. (0.45 ± 0.07 vs 0.35 ± 0.06, p = 0.015). The correlation between the change in left ventricular shortening fraction and the mean increase of paced QRS duration was not significant. Six patients developed dilated cardiomyopathy, which was diagnosed 2 months to 9 years after pacemaker implantation. CONCLUSION: Chronic right ventricular pacing in pediatric patients with or without structural heart disease does not necessarily result in decline of left ventricular function. In patients with complex congenital heart malformations left ventricular shortening fraction shows significant decrease

Is cardiovascular evaluation necessary prior to and during beta-blocker therapy for infantile hemangiomas? A cohort study

Background: Although consensus guidelines for pretreatment evaluation and monitoring of propranolol therapy in patients with infantile hemangiomas (IH) have been formulated, little is known about the cardiovascular side effects. Objectives: We sought to analyze cardiovascular evaluations in patients with IH at baseline and during treatment with an oral beta-blocker. Methods: Data from 109 patients with IH were retrospectively analyzed. Patient and family history, pretreatment electrocardiogram (ECG), heart rate, and blood pressure were evaluated before initiation of beta-blocker therapy. Blood pressure and standardized questionnaires addressing side effects were evaluated during treatment. Results: Questionnaire analyses (n = 83) identified 3 cases with a family history of cardiovascular disease in first-degree relatives. ECG findings were normal in each case and no serious complication of therapy occurred. ECG abnormalities were found in 6.5% of patients but there were no contraindications to beta-blocker therapy and no major complications. Hypotension in 9 patients did not require therapy adjustment. In all, 88 parents (81%) reported side effects during beta-blocker treatment. Limitations: The relatively small patient cohort is a limitation. Conclusion: Pretreatment ECG is of limited value for patients with an unremarkable cardiovascular history and a normal heart rate and blood pressure. Hypotension may occur during treatment

Treatment of infantile haemangiomas with atenolol: Comparison with a historical propranolol group

Propranolol, a lipophilic non-selective beta-blocker, has proven to be effective in the treatment of infantile haemangioma (IH). However, several side effects have been reported. Atenolol, a hydrophilic selective beta-1 blocker, could be an alternative and associated with fewer side effects. Thirty consecutive patients with IH were treated with atenolol between June 2010 and May 2011. The therapeutic effect was judged by clinical assessment and quantified by using a visual analogue scale (VAS) and the Haemangioma Activity Score (HAS). Side effects were also evaluated. The atenolol cohort was compared with a previously described cohort of 28 patients treated with propranolol between July 2008 and December 2009. Clinical involution was present in 90% (27/30) of the IH patients treated with atenolol. Mild side effects occurred in 40% (12/30) of these patients and severe side effects occurred in 3% (1/30). Compared with the previously described cohort treated with propranolol, mild side effects occurred in 50% (14/28) and severe side effects in 25% (7/28) of the patients (p=0.04). Quantitative improvement of the IH in the atenolol group (n=27) showed no significant difference in either the VAS score or th This study indicates that atenolol is effective in the treatment of IH. Compared with a historical control group treated with propranolol, the effects of atenolol seem to be similar and less frequently associated with severe side effects. Randomised clinical trials are necessary to evaluate the efficacy and safety of atenolol treatment in IH. (c) 2013 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved

A continued method performance monitoring approach for the determination of pediatric renin samples - application within a European clinical trial

Background Plasma renin levels were determined in the academia-driven, EU-funded "Labeling of Enalapril from Neonates up to Adolescents" (LENA) project to evaluate its role in pediatric heart failure. Quality-controlled bioanalysis is crucial to ensure reliable data generation. However, a comprehensive bioanalytical quality control (QC) concept to monitor the method performance within an academic environment was lacking. Methods Thus, a QC concept was designed encompassing regulatory guidance, international recommendations and current scientific discussions. The concept included (1) a system-suitability test, (2) verification of single bioanalytical runs by calibration curve performance and evaluation of QCs, (3) assessment of the inter-run accuracy according to Clinical Laboratory Standards Institute (CLSI) guideline, (4) monitoring of reproducibility by pediatric incurred samples, (5) blank-sample analysis and (6) participation in interlaboratory testing. Results The concept was successfully applied to the academic project. About 11% of single runs were identified as invalid and triggered a re-analysis of unknown samples being included in those runs. The usefulness of the customized inter-run monitoring was demonstrated and proved the good accuracy from the first to the last run. All 147 reanalyzed incurred sample pairs complied with regulatory requirements. Conclusions The regulatory complied QC concept was customized for the demands of academia-driven pediatric trials and contributed to the reliable quantification of 965 pediatric renin samples

Dilated cardiomyopathy in isolated congenital complete atrioventricular block: Early and long-term risk in children

OBJECTIVES We sought to identify the risk factors predicting the development of dilated cardiomyopathy (DCM) in patients with isolated congenital complete atrioventricular block (CCAVB). BACKGROUND Recently evidence has emerged that a subset of patients with CCAVB develop DCM. METHODS This was a retrospective study of 149 patients with CCAVB who had heart size and left ventricular (LV) function assessed by echocardiography and chest radiograph! over a follow-up period of 10 +/- 7 years. RESULTS Nine patients developed DCM at the age of 6.5 +/- 5 fears. No definite cause could be identified. In these nine patients, CCAVB was diagnosed in eight at 23 +/- 2.3 weeks gestation and in one at birth. Maternal SSA/SSB antibodies were confirmed in seven of the nine patients. Pacemakers were implanted in eight patients in the first month and in one patient at five years of age. The initial left ventricular end-diastolic dimension (LVEDD) was in the 96th +/- 2.6 percentile and the cardiothoracic (CT) ratio was 64 +/- 3.8% in the nine patients who developed DCM, and differed significantly in patients with CCAVB (p <0.005) who did not develop DCM. The LVEDD and CT ratio did not decrease in the patients with CCAVB and DCM, but decreased significantly in the patients with CCAVB without DCM (p <0.001) once pacing was initiated. Two patients with DCM died within two months of diagnosis; one patient is neurologically compromised; two patients received a heart transplant; and four patients are listed for heart transplantation. CONCLUSIONS Isolated CCAVB is associated with a long-term risk for the development of DCM. Risk factors may be SSA/SSB antibodies, increased heart size at initial evaluation and the absence of pacemaker-associated improvement. (J Am Cell Cardiol 2001;37:1129-34) (C) 2001 by the American College of Cardiology