Prospective multicenter study of the breakable babystent for treatment of aortic coarctation in newborns and infants.

To assess the efficacy and safety of a breakable BabyStent to treat complex aortic coarctation (CoA) in early childhood. Although recommended in several guidelines, there is no approved aortic stent for young infants, because of the dilemma between two mandatory requirements: expandable up to adult size on the one hand, and small enough to fit through a baby's femoral artery on the other. Prospective interventional, multi-center clinical trial with the breakable Osypka BabyStent® (OBS). The OBS is a low-profile, 15-mm long cobalt-chromium stent, pre-mounted on a 6 mm balloon and inserted via a 4 Fr sheath. After implantation, its diameter is adjustable from 6 to 12 mm by balloon dilation. Further dilation opens predefined joints enabling unrestricted growth. Nineteen patients (9 male), median age 112 days (range: 7-539), median body weight 5.6 kg (range: 2.4-8.4) were deemed high risk and underwent stent implantation. Of those, 74% suffered from re-CoA following surgery, 53% had additional cardiac and 21% noncardiac malformations. Our primary combined endpoint was fulfilled: All stents were implanted in the desired region, and a >50% intrastenotic diameter-extension was achieved in 15 patients (78.9%, 80% confidence interval [62.2; 90.5], 95% confidence interval [54.4; 93.9]). Secondary endpoint confirmed that the OBS fits the baby's femoral vessel diameter. All children survived the procedure and 12-month follow-up. This stent enables percutaneous stenting of complex aortic coarctation to treat high-risk newborns and infants.

Performance of epimyocardial leads in patients with a single ventricle circulation.

BACKGROUND: Cardiac pacing can be challenging after a Fontan operation, and limited data exist regarding strategies to plan these epimyocardial systems while minimizing the number of surgical procedures. METHODS: A retrospective review of all our 47 patients (mean age 18 years, standard deviation 9 years) with a Fontan palliation who received an epimyocardial cardiac implantable electronic device (CIED) between 2002 and 2020 with regard to the stability of the epimyocardial lead parameters and the incidence of system revisions. RESULTS: Over the last 18 years, 84 implantations or revisions of the epimyocardial CIED in 47 Fontan patients were performed. Mean age at time of the first implantation was 9.4 (range 0.28-29.3) years. Follow-up period ranges from 0.11 to 18.2 (mean 7.7, standard deviation 4.2) years. A total of 123 pacing leads were implanted of which 99 are still active. From 2010 triple lead cardiac resynchronization devices were used in 17 patients to better cope with lead problems. The initial pacing threshold of the leads inactivated during this study period proved significantly higher (mean 1.66 V) than in the "all leads" group (mean 1.27 V, p = .0005) or the group of the still active leads (mean 1.17 V, p = .00004). CONCLUSIONS: When implanted with a low pacing threshold, the bipolar epimyocardial electrodes show stable and good long-term results in young patients with a Fontan circulation. Resynchronization pacing systems and the prospective implantation of reserve leads may help to reduce the rate of resternotomies and provide a flexible concept to deal with lead failure.

Telemonitoring with Electronic Devices in Patients with a Single Ventricle Anatomy.

BACKGROUND: A growing number of patients with a single ventricle anatomy, who had a Fontan palliation as a child, are now reaching adulthood. Many need an epimyocardial pacemaker system with an optional telemonitoring (TM) unit, which evaluates the collected data and sends it via Internet to the patient's physician. There are no data on the reliability and clinical relevance of these systems in this patient group. METHODS: We analyzed data in 48 consecutive patients (mean age 18 years, standard deviation 9 years) with a Fontan or Fontan-like palliation who received a cardiac implantable electronic device with a TM unit from Biotronik (Home Monitoring) or Medtronic (CareLink) between 2005 and 2020 with regard to the reliability and clinical relevance of the downloaded data. RESULTS: The observation period was from 4 months to 14 years (mean 7 years, standard deviation 3.9 years). A total of 2.9 event messages (EMs)/patient/month and 1.3 intracardiac electrogram recordings/patient/month were received. Two patients died during follow-up. The combination of regularly arriving statistical data and 313 clinically relevant EMs led to the modification of antiarrhythmic or diuretic medication, hospitalization with cardioversion or ablation, and cortisone therapy to avoid exit block in 21 (44%) patients. CONCLUSION: TM is an instrument to receive functional and physiologic parameters of our Fontan patients. It provides the ability to respond early for signs of system failure, or arrhythmia, even if the patient is not experiencing any problems. It is a useful tool to manage this difficult patient population without frequent hospital visits.

Acute treatment of critical vascular stenoses with a bioabsorbable magnesium scaffold in infants with CHDs.

BACKGROUND: Post-operative severe vascular stenosis and proliferating endothelial tissue lead to severe circulatory disorders and impair organ perfusion. Bioabsorbable magnesium scaffolds may help to overcome these obstructions without leaving obstructing stent material. We analyse their role in the treatment of vascular stenosis in infants. METHODS: Since 2016, 15 magnesium scaffolds with a diameter of 3.5 mm were implanted in 9 patients aged 15 days to 7.6 years. Eight scaffolds were implanted in pulmonary venous restenoses, five in pulmonary arterial stenosis including one in-stent stenosis, one into a stenotic brachiocephalic artery, and one in a recurrent innominate vein thrombosis. RESULTS: All patients clinically improved after the implantation of a scaffold. The magnesium scaffolds lost integrity after 30-48 days (mean 42 days). The innominate vein thrombosed early, while all other vessels remained open. Two patients died after 1.3 and 14 weeks not related to the scaffolds. Five patients needed further balloon dilations or stent implantations after the scaffold had fractured. At first recatheterisation after in mean 2.5 months, the mean minimum/maximum diameter in relation to the scaffold's original diameter was 89%/99% in the arterial implantations (n = 6) and 66%/77% in the pulmonary venous implantations. CONCLUSIONS: The magnesium scaffolds can be used as a bridging solution to treat severe vascular stenosis in different locations. Restenosis can occur after degradation and make further interventions necessary, but neither vessel growth nor further interventions are hindered by stent material. Larger diameters may improve therapeutic options.

First follow-up of a breakable stent for implantation in infants dedicated for a life-long stay.

OBJECTIVE AND METHODS: The use of conventional metal stents in infants is severely limited by subsequent somatic growth. The use of a breakable balloon expandable stent (BS) designed for initial implant at small diameters but with properties that allow unlimited dilation in line with growth has potential advantages in this patient group. This study reports our experience with this stent between 2010 and 2014. A total of 17 BS were implanted in 14 infants (mean age 4.8 months). All but one stent was placed into the aorta to treat coarctation. RESULTS: All implantations were successful and initial gradients dropped from a mean of 25-6 mm Hg (range from 1-50 down to 0-24 mm Hg). Mean follow-up was 3.3 years (range 5 days to 7 years) with a total cumulative follow-up of 46.7 patient years. Stent redilation was performed a median of 2.5 times (range 0-5). Sixteen stents in 13 patients remain in place. Following redilation beyond 10 mm, circumferential integrity of the BS was lost in 10 patients. No further stent implantation or related surgery was necessary. A 3 mm dissection occurred in one patient after redilation. CONCLUSIONS: The BS performed well in terms of relief of stenosis and could be successfully dilated during the phase of the infants' most rapid growth. Mild intimal proliferation occurred in some patients early after implantation. In the course of the stepwise redilations and growth adjustments, both, planned longitudinal and transverse fractures occurred without allowing a collapse of the stented area.

Multicenter midterm follow-up results using the gore septal occluder for atrial septal defect closure in pediatric patients.

OBJECTIVES: To assess the safety and efficacy of the Gore Septal Occluder (GSO) used for device-closure of significant secundum-type atrial septal defects (ASD II) focusing on pediatric patients. BACKGROUND: The GSO is a patch-like double disc device. Due to its design, it is assumed to be safe, even when implanted in ASDs with deficient retro-aortic rims. METHODS: Multicenter retrospective analysis of consecutive children and adolescents with a GSO in situ for at least 12 months according to a 1- to 4-year midterm follow-up. RESULTS: Hundred and seventy three pediatric patients were enrolled. At implantation, median age was 6 years (range 0.7-17.9), median body weight and length were 21 kg (6.4-95) and 119 cm (65-193). Median follow-up period was 20 months (range 12-51). ASD anatomy was comprised of single defects in 131 patients (76%), multi-fenestrated defects in 42 (24%), and deficient retro-aortic rims in 33 (19%). Follow-up confirmed an overall closure-rate of 95.4%. Small residual shunts were reported in eight patients (4.6%) without need for any re-intervention. Complications were classified as minor events both during the initial procedure (9 patients, 5.2%) and on follow-up (another 9 patients), including transient AV block II in three patients (1.8%) and four snare-retrievals (2.4%) during the initial procedure. CONCLUSIONS: Periprocedural and midterm follow-up data have shown the GSO to be effective and safe for ASD device closure in children and adolescents. GSO may be considered the first-choice device in deficient retro-aortic rims and multi-fenestrated defects, when covering most of the atrial septum is necessary. © 2016 Wiley Periodicals, Inc.

Restrictive Atrial Septum Defect Becomes a Risk Factor for Norwood Palliation of Hypoplastic Left Heart Syndrome Only When It Is Combined with Mitral or Aortic Atresia.

BACKGROUND: Restrictive atrial septal defect (ASD) is described as risk factor for Norwood procedure because of elevated pulmonary resistance. We hypothesized that it invariably could not cause pulmonary hypertension, unless it was combined with mitral valve or aortic valve atresia. We investigated how restrictive ASD influenced survival of patients with hypoplastic left heart syndrome (HLHS) who underwent Norwood operation. PATIENTS AND METHODS: A total of 118 HLHS patients who underwent surgery between January 2005 and December 2012 were grouped into three groups. Group 1 included 31 patients with restrictive ASD combined with mitral or aortic atresia; Group 2 composed of 12 patients with restrictive ASD and mitral and aortic stenosis; Group 3 (n = 75) had no ASD restriction. Survival was determined for each group. Multivariate analysis was conducted to test risk factors for mortality. RESULTS: Mean follow-up was 26.3 ± 24.1 months. Survival was 78.7% ± 4.2% at 30-month interval and onward after Norwood procedure for the whole cohort; it was 43.8% ± 10.0%, 91.7% ± 8.0%, and 77.3% ± 5.0% for Group 1, 2, and 3, respectively. The difference was significant between Group 1 and Group 2 and 3: p < 0.001. Survival was similar for Group 2 and Group 3: p = 0.45. Combination of restrictive ASD and mitral or aortic atresia was found to be the sole risk factor for early and late mortality (odds ratio: 3.5, 95% confidence interval: 1.8-7.1, p < 0.001). CONCLUSION: Restrictive ASD only affects survival of HLHS patients following Norwood procedure if it is associated with mitral or aortic atresia.

Transvascular closure of single and multiple muscular ventricular septal defects in neonates and infants < 20 kg.

PURPOSE: While surgical closure of ventricular septal defects (VSD) is still the gold standard, we review our experience with the interventional closure of single and multiple muscular VSD in newborns and infants under 20 kg. METHOD: Between 2004 and 2012 transcatheter closure of ten Swiss cheese VSD, six single muscular and one apical VSD was attempted in 17 patients between 10 days and 7.3 years of age and with a bodyweight ranging from 2.2 to 19 kg. Seven patients had had a significant shunt after cardiac surgery, five patients a shunt induced congestive heart failure and in five patients postponement of surgery was intended. RESULTS: A total of 20 devices was successfully implanted in 15 of 17 (88%) patients, reducing the interventricular shunt and improving the haemodynamic situation in 14 patients. An acute AV-block led to immediate removal of the device in a patient of 2.2 kg. One Amplatzer muscular VSD occluder could not be delivered due to the sharp bending of the delivery sheath in 2004. Three patients died during follow-up not related to the intervention. Re-intervention was necessary in one patient with Swiss cheese VSD. CONCLUSION: Interventional closure of muscular VSD is possible in newborns and infants and presents an eligible treatment option. New occlusion systems with miniaturized introducer sheaths of 4-6 French have extended the spectrum of treatable lesions. An individual and interdisciplinary risk-benefit stratification is required to choose from surgical, interventional, or combined strategies.

Remote monitoring leads to early recognition and treatment of critical arrhythmias in adults after atrial switch operation for transposition of the great arteries.

BACKGROUND: Adults with transposition of the great arteries (TGA) after atrial switch repair have an increased risk for arrhythmia and sudden cardiac death. We analyzed whether a remote monitoring (RM) system as part of an implantable cardiac device contributes to timely recognition and improved treatment of critical arrhythmias in these patients. METHODS AND RESULTS: All consecutive TGA patients (n=11) requiring a pacemaker or cardiac resynchronization therapy with or without implantable cardioverter defibrillator between 2008 and 2011 were included. RM-detected arrhythmia, abnormality of device integrity and reaction time from event transmission until acknowledgement via email and clinical decision making were analyzed and compared to a control group (n=21). In 10 patients (91%) 17 arrhythmias were detected, 8 patients (80%) indicated no symptoms. In the RM group time interval from transmission to acknowledgement was 2.4 days (range, 0-4.5 days). Clinical decision-making was advanced by a mean of 77.5 days (range, 10-197 days) compared with conventional follow-up and identified adaption of anti-arrhythmic medication in 8, electrical cardioversion in 2, overdrive pacing in 1 and radiofrequency ablation in 2 patients. A coronary sinus lead fracture was identified in 1 patient followed by successful replacement. CONCLUSIONS: RM enables early detection of tachyarrhythmia followed by optimization of medical treatment and potentially life-saving anti-tachycardic intervention in adults after atrial repair of TGA.

Interventions after Norwood procedure: comparison of Sano and modified Blalock-Taussig shunt.

Improved results have evolved from the modified Norwood procedure (NP). This study compares the incidence of interventions after NP with the Sano (n = 37) and modified Blalock-Taussig (BT n = 70) shunt. Incidence, location, interval of interventions, and weight were retrospectively analysed for 107 neonates undergoing NP during the period from October 2002 to December 2009. Forty-six (43.0 %) patients underwent interventions, mostly for dilatation of the aortic arch ([DAA] n = 26 [24.3 %]; Sano n = 10, BT n = 16, p = 0.6), dilatation of the shunt ([DS] n = 15 [14.0 %]; Sano n = 11, BT n = 4; p = 0.002), or closure of aortopulmonary collaterals ([APC] n = 15 [14.0 %]; Sano n = 3, BT n = 12; p = 0.08). Mean interval after NP and body weight at DAA, DS, and APC were 72.4 ± 18.9, 108.5 ± 15.8, and 110.7 ± 17.8 days and 4.5 ± 1.3, 4.9 ± 1.9, 5.3 ± 1.2 kg, respectively. The interventions were not associated with mortality but with a greater rate of complications (9 of 46 [21.4 %]) compared with the rate after diagnostic catheterization (0 of 45, p = 0.03). Complications included closure of the femoral or subclavian artery (n = 5), cerebral embolic or bleeding events (n = 4), cardiopulmonary resuscitation (n = 3), and temporary heart block (n = 2). Actuarial survival was similar from the postoperative month 8 onward at 78.6 ± 4.9 % (95 % confidence interval [CI] 67.0-86.5 %) for Sano and 78.4 ± 6.8 % (95 % CI 61.4-88.6 %) for BT (p = 0.95). Interventions after NP were common irrespective of shunt type. However, a significantly greater rate of shunt interventions was noted in the Sano group. In particular, interventions addressing the aortic arch and the shunt were related with a significant rate of complications.

New insights learned from the pulmonary to systemic blood flow ratio to predict the outcome in patients with hypoplastic left heart syndrome in the pre-Glenn stage: a single-center study.

Background: To the best of our knowledge, no study has been made until now to determine whether the ratio between pulmonary and systemic blood flow (Qp/Qs) in the pre-stage II (PS2) or pre-Glenn stage can predict the outcome in patients with hypoplastic left heart syndrome (HLHS) who underwent Norwood (NW) palliation. Patients and methods: From January 2016 to August 2022, 80 cardiac catheterizations in 69 patients with HLHS in NW palliation stage with modified Blalock-Taussig shunt (MBTS) were retrospectively recruited. The Qp/Qs was measured under stable conditions using the Fick formula. None of the patients were intubated. Patients were divided into two groups: Group 1 included patients who underwent planned cardiac catheterization (n = 56), and Group 2 had unplanned examination (n = 13), in which the indication for cardiac catheterization was desaturation in 11 patients and pulmonary over-circulation in two. The composite primary outcome was defined as accomplishing the planned operations (Glenn and Fontan) with freedom from death and reoperation, referring to palliative therapy or heart transplantation. The secondary outcome was freedom from transcatheter intervention in MBTS or pulmonary arteries. Results: The median follow-up was 48 months (range 6-72 months). The median value of Qp/Qs in Group 1 was 1.75 (range 1.5-2.2). In Group 2, the 11 patients with desaturation, the median value of Qp/Qs was 1.25 (range 0.9-1.45). The two patients with suspected pulmonary overcalculation showed Qp/Qs of 2.3 and 2.5, respectively; a reduction of the shunt size was required. Approximately 96.4% of patients in Group 1 achieved the primary outcome compared with only 30.7% in Group 2. The need for reintervention was 1.8% in Group 1 compared with 61.3% in Group 2. There is a significant relationship between Qp/Qs and the impaired outcome (death, palliative therapy, or heart transplantation) with a p-value of 0.001, a relative risk factor of 3.1, and a 95% confidence interval of 1.4-7.1. No significant relationship between the Qp/Qs and the size of MBTS (p-value of 0.073) was noted. Conclusion: The Qp/Qs in PS2 can predict outcomes in patients with HLHS in Norwood stage with MBTS. The Qp/Qs between 1.5 and 2.2 with a median of 1.75 seems to be optimal in the patients in PS2. Qp/Qs of <1.5 is associated with pulmonary stenosis, shunt stenosis, and pulmonary hypertension.

Echocardiogram-Guided Stenting of a Critical Aortic Coarctation in an Extremely Low Weight Preterm Infant.

We report a case of critical aortic coarctation in an extremely low birth weight preterm infant weighing 600 g that was successfully treated with interventional stent implantation. The intervention was guided by echocardiography without using contrast agent due to associated renal failure. (Level of Difficulty: Intermediate.).

Telemonitoring with implantable electronic devices in young patients with congenital heart diseases.

AIMS: In paediatric patients with a pacemaker (PM) or an implantable cardioverter defibrillator (ICD) device, interrogation during in-clinic visits is usually required to obtain information on arrhythmias or system failures. An automated telemonitoring system tracking patient- and system-related parameters provides this information on a daily basis and might assist patient management. METHODS AND RESULTS: This retrospective analysis evaluates telemetric data obtained from 48 devices implanted in 45 patients (median age, 12.4 years; range, 5 weeks to 37.6 years) using an automated system [Home Monitoring (HM)] over the last 5 years. Regular transmissions were received on 72% of all days. The event messages and monitoring data of 34 (71%) devices induced system revisions, electrophysiological studies including ablation, programming changes, medication changes, and alterations in sporting activity. Approximately 47% of event messages and 50% of emergency messages arrived within 4 weeks of implantation or latest outpatient visit; 113 (17%) emergency messages reported acute changes in lead parameters or tachycardia, which required medical intervention. In four ICD patients, delivered shocks were unreported and only detected by HM. CONCLUSIONS: An automated telemonitoring system reporting patient- and system-related parameters, generated within the patient's home surrounding, can improve the safety and quality of PM and ICD therapy, especially in children, by allowing early detection of system failure and changes in arrhythmic events.

Follow-up of hypertension after aortic coarctation stent implantation based on safe and effective re-intervention: a retrospective cohort study.

Background: Stent implantation for aortic coarctation (CoA) is associated with anatomical and hemodynamic advantages. Although the anatomy is corrected after the intervention, patients often still suffer from hypertension during the follow-up. Its risk factors remain unclear. There are only a few studies on the long-term follow-up of hypertension after stenting. This study aimed to investigate the factors influencing systemic hypertension after CoA by transcatheter stent placement. Methods: A total of 82 patients (native CoA: 37; men: 53) who underwent stent implantation for CoA at a median age of 123 months (range, 40 days-44 years) between January 2004 and September 2012 were included in this study. Patients with native or recurrent CoA after surgery, simple CoA or complex CoA associated with other cardiac anomalies were recruited in this study. Only stents that were expandable to adult size were implanted. After the initial stent implantation, the patients were followed up for 76.7 months (range, 6-151 months). Those who met the indications for a re-intervention underwent a balloon dilatation or stent implantation to relieve restenosis and alleviate the hypertension caused by restenosis. Patients with hypertension were treated with anti-hypertensive agents. Results: In the initial intervention, 87 stents were implanted in 82 patients. Seventy-two patients (87.8%) were diagnosed with hypertension before treatment, but only 23 (28.0%) patients had hypertension at the last follow-up, which is a number significantly lower than that before the intervention (P<0.001). Cox regression analysis showed that age at stent implantation was significantly correlated with the incidence of hypertension, which indicates that the younger the patient receiving the intervention, the lower the incidence of hypertension. Moreover, the incidence of hypertension in follow-up is related to the diagnosis. It was higher in the patients with simple CoA than those with complex CoA. There was no significant correlation between native CoA and recurrent CoA. Conclusions: The incidence of hypertension after CoA stent implantation was significantly lower than that before the intervention. Age at stent implantation is an essential influencing factor for paradoxical hypertension after CoA stent implantation. Complex CoA in patients was related to a significantly lower prevalence of follow-up hypertension.

Stenting of critical aortic coarctation in neonates between 600 and 1,350 g. Using a transfemoral artery approach. A single center experience.

Background: Stenting of aortic coarctation (CoA) in newborns with a very low bodyweight remains rare and challenging. In this study we aim to highlight on two points: first the feasibility of CoA stenting in such babies and second the importance of using echocardiogram for guiding the intervention without the need for contrast agent. Methods: Between 2020 and 2022 three preterm babies with very low (VLWB) and extremely low weight (ELWB) underwent CoA-stenting in our center. The weight of the patients at time of intervention was 1,350, 1,200, and 600 g, respectively. The femoral artery was chosen in all patients as vascular access. Transthoracic echocardiography, sonography of the femoral arteries and head ultrasound were applied for follow up. Results: All three interventions were successfully done, with no complications. Coronary stents were implanted. In one Patient (1,350 g) the stent was inserted without sheath. In two patients with renal failure, the stenting was performed under echocardiography-guidance without contrast agent. The follow up showed a preserved function of the left ventricle in all patients. No relevant gradient was reported and no stent re-intervention was required. Sonographic follow up showed a patent femoral artery in all patients. Two patients were operated 73 and 110 days after stenting, and the stents were successfully removed. In the third patient the intervention was performed 130 days ago and he is waiting for the operation. Conclusion: CoA-stenting in VLWB and ELWB is feasible and can bridge them to the next surgery without complications. Echocardiography-guided CoA-stenting in VLWB is a considerate option especially in patients with renal failure. Accessing the femoral artery by experienced doctors, using local anesthesia before the puncture and before removing the sheath might help to protect the vessel from stenosis or occlusion.

Duct Stenting vs. Modified Blalock-Taussig Shunt: New Insights Learned From High-Risk Patients With Duct-Dependent Pulmonary Circulation.

Background: As no data were available on the comparison of outcomes between modified Blalock-Taussig shunts (MBTs) vs. duct-stenting (DS) in patients with pulmonary atresia (PA) and an increased ductal tortuosity and in patients with pulmonary atresia and intact septum (PA-IVS) with right ventricle-dependent coronary circulation (RVDCC), we aimed to perform a single-center retrospective evaluation. Methods: Between 2010 and 2019, 127 patients with duct-dependent pulmonary circulation (DDPC) underwent either MBTs (without additional repairs) (n = 56) or DS (n = 71). The primary endpoint was defined as arriving at the next planned surgery (Glenn or biventricular repair) avoiding one of the following: (1) unplanned surgery or unplanned perforation of the pulmonary valve (PVP) with a stent, (2) procedure-related permanent complications, and (3) death. Two subgroups were considered: (1) patients who had a ductal curvature index (DCI) >0.45 (n = 32) and (2) patients with PA-IVS and RVDCC (n = 13). Ductal curvature index (DCI) was measured in all the patients to assess the tortuosity of the ducts. Patients with DCI >0.45 were considered as being in a high-risk group for the duct-stenting; a previous study showed that the patients with a DCI < 0.45 had a better outcome when compared with those with a DCI> 0.45. Results: The primary outcome was achieved equally in the two groups (77.5% in DS, 75% in MBTs). Hospital deaths, need for ECMO, and the occurrence of major complications was more frequent in the group with MBTs with an Odds Ratio (OR) of 5, 0.8, and 4, respectively, and a 95% Confidence Interval (CI) 1.1-22.6, 0.7-0.9, and 1.6-10.3, respectively, and a P-value < 0.05. For the two subgroups, the primary outcome was achieved in 64% of patients with a DCI >0.45 who received MBTs compared to 20% in those with DS (OR 3.5, 95% CI 1.2-10, P 0.005). While 74.1% of the patients with PA-IVS and RVDCC after DS had achieved the primary outcome, all patients with MBTs had an impaired outcome (OR 3.5, 95%CI 1-11.2, P 0.004). Conclusion: MBTs showed a better outcome in patients with tortuous ducts compared to DS. DS seems to be superior in patients with DDPC with DCI <0.45 and patients with PA-IVS with RVDCC.

Vascular interventions in young patients undergoing transvenous pacemaker revision.

AIMS: Transvenous lead implantation for pacemaker (PM) or implantable cardioverter defibrillators systems in children and young patients with congenital heart disease is widely practiced. With longer implantation times, the risk for vascular occlusion increases. Transvenous angioplasty may be used to maintain venous patency for system revision in the future. Retrospective analysis of the interventional techniques employed in our young patients undergoing PM revision may identify its clinical benefit. METHODS AND RESULTS: Between May 2005 and August 2010, 28 procedures to maintain central venous patency were performed in 24 patients. Median age was 14.3 years (range, 3.6-29.5 years). The median lead age at time of intervention was 6.8 years (range, 8 days-21.5 years). Balloon dilation of the stenotic vessels was performed with and without prior lead removal. Revascularization with stent implantation was successfully attempted in all clinically relevant obstructions. All PM systems retained full function or were upgraded as planned. There were no major complications and acute surgical referral was not required. CONCLUSION: Angioplasty techniques may be used to maintain and increase the longevity of transvenous pacing pathways in young patients.

Use of the ductal curvature index to assess the risk of ductal stenting in patients with duct-dependent pulmonary circulation.

BACKGROUND: To evaluate whether a quantitative curvature threshold can contribute to risk stratification of ductal stenting in patients with a duct-dependent pulmonary circulation (DDPC). METHODS: A single-center retrospective analysis was performed. The ductal curvature index (DCI) was calculated in 71 patients with DDPC. The ducts were divided into four classes based on quartile thresholds of DCI: class I (≤0.12), class II (0.13-0.33), class III (0.34-0.44), and class IV (≥0.45). The primary outcome of this study was defined as free from all of the following: (I) intervention related death, (II) need of unplanned surgery (III) need of unplanned pulmonary valve (PV) perforation with stent in right ventricular outflow tract (RVOT), and (IV) intervention related permanent complications. RESULTS: Eighty percent of patients in class IV (DCI ≥0.45) failed to achieve the primary outcome; odds ratio (OR) 9 and 95% confidence interval (CI): 3.7-21.4 compared to 26.6% in all classes with DCI <0.45. 66.7% of these patients needed unplanned surgery or PVP with RVOT stent; OR 12.4 (95% CI: 4-39). 80% of major complications were observed in class IV (P<0.01). Need of pulmonary arterioplasty was in class IV 53.3%; OR 3.3 (95% CI: 1.5-7.1). CONCLUSIONS: DCI can be useful to guide the clinical decision-making in patients with torqued ducts. Patients with a DCI ≥0.45 belong to a high-risk group, in which ductal stenting is associated with an elevated risk for early surgery or unplanned re-intervention.

Lead removal in young patients in view of lifelong pacing.

AIMS: In young patients with or without a congenital heart disease, transvenous leads for pacemakers or implantable cardioverter defibrillators can cause later vascular obstruction or infection. Removal of non-functional leads is controversial as it bears the risk of vascular disrupture and embolizations. We report the data of a single centre for paediatric cardiology on efficiency and safety of transvenous lead removal. METHODS AND RESULTS: Between May 2005 and August 2009 in 22 patients with a mean age of 12.9 years (range: 3.6-29.5 years) removal of 28 transvenous leads (mean lead age: 5.1 years) was attempted. The main indications for removal were vascular obstruction, increased threshold, and lead dislocation. Commercially available retraction tools were used, if necessary. Twenty-five leads (89%) were retrieved with clinical success, of which 22 (79%) were removed with complete procedural success. In three leads the lead tips were retained, while three leads could not be retrieved. No major complications occurred. Additional interventions such as recanalization, balloon dilation, or stent implantation were performed as indicated. Procedure and X-ray times could be correlated to the implant age of the leads. CONCLUSION: Using non-electrical techniques, transvenous lead removal can be performed with a success rate of 89% in young patients. In the case of vessel obstructions, lead replacement combined with revascularization should be performed early, as the older the lead, the more prolonged and more hazardous the extraction procedure becomes. The use of new leads and precautionary implantation techniques may facilitate later lead removal.

Transvenous cardiac resynchronization therapy in complex congenital heart diseases: dextrocardia with transposition of the great arteries after Mustard operation.

Cardiac resynchronization therapy revealed first promising results in patients with a congenital heart disease and a systemic right ventricle. Contrast-enhanced magnetic resonance imaging showed accessibility of the coronary sinus in an 18-year-old male patient with mirror dextrocardia, d-transposition of the great arteries and ventricular septal defect (VSD) after Mustard operation and VSD patch closure. In literatures, transvenous lead placement is discussed in this anatomical setting, with opposed position of the ventricular leads and reliable lead characteristics.