Transcatheter closure of tubular PDA with amplatzer plug 4 in preterm infants weighing between 900 and 3,400†g: the pros and cons.

Objective: To our knowledge, no prior study has focused on the outcome of PDA occlusion using an Amplatzer™ Vascular Plug 4 (AP4) in ill preterm infants. This study aims to highlight the pros and cons of AP4 in this cohort. Methods: Between 2020 and 2022, 26 ill preterm infants underwent PDA closure in our centre. The median age, weight, and gestational age were 60 days (11-180 days), 1,900g (900-3,400 g), and 25 weeks (22-33 weeks), respectively. The indication of the intervention was hemodynamically significant PDA. A medical trial with non-steroid medication failed to close the ducts in all patients. Follow-up using echocardiography was done 24, 48, and 72 h after the intervention. Results: Of 26 ducts, 21 were successfully closed with AP4. Five ducts shorter than 7 mm were unsuitable for AP4 and were closed with the Amplatzer Piccolo device. The median radiation time was 4 min (3-9 min). No early plug-related complications or deaths were documented. Plug-related jailing of the left pulmonary artery as a late complication was 9.5%, and LPA reintervention was required. All ducts were closed after 48 h. Conclusion: Implantation of the AP4 using a 4 F 0.38 guide wire-compatible catheter without inserting a long sheath makes the closure of tubular ducts with this device feasible and uncomplicated with a short intervention time. However, the limited sizes with fixed lengths of the AP4 make it unsuitable for ducts wider than 4.5 mm and shorter than the chosen device length, which can increase the risk of significant left pulmonary stenosis. A wide range of plug diameters and lengths is required to accommodate the large and short ducts.

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.).

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.

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.

Large diameter Advanta V12 covered stent trial for coarctation of the aorta: COARC Study

BACKGROUND: Covered stent implantation for treatment of coarctation of the aorta (CoA) is effective and can prevent aortic wall injury. Prospective studies with long-term follow-up, including imaging, are lacking. We report the acute and long-term outcomes for use of the Large Diameter Advanta V12 covered stent for treatment of native and recurrent CoA. METHODS: A prospective, multicenter, nonrandomized study was performed including 70 patients (43 male), median age 17 years, median weight 57.4 kg with CoA who underwent implantation of the Large Diameter Advanta V12 covered stent. Annual follow-up for 5 years included Doppler echocardiography to calculate diastolic velocity: systolic velocity ratio. RESULTS: CoA diameter increased from 5.6±3.6 to 14.9±3.9 mm (P<0.0001) and the pressure gradient decreased from 35.8±16.2 to 5.6±7.9 mm Hg (P<0.0001). Preimplantation diastolic velocity: systolic velocity of 0.6±0.16 dropped to 0.34±0.13 (P<0.0001) and was maintained at 5 years. Computed tomography angiograms at 12 months post implantation demonstrated the stent: transverse arch diameter to be similar, 0.91±0.09 to post procedure 0.86±0.14. Major adverse vascular events at 30 days and 12 months were 1.4% and 4.3%, respectively. Significant adverse events included three patients who required stent implantation to treat infolding. There were no mortalities. CONCLUSIONS: The Large Diameter Advanta V12 covered stent is safe and effective for the treatment of CoA with an immediate and sustained reduction of the pressure gradient over 12 months and 5 years as assessed by preimplantation and post implantation Doppler echocardiography and 12-month computed tomography angiography.

Large Diameter Advanta V12 Covered Stent Trial for Coarctation of the Aorta: COARC Study.

BACKGROUND: Covered stent implantation for treatment of coarctation of the aorta (CoA) is effective and can prevent aortic wall injury. Prospective studies with long-term follow-up, including imaging, are lacking. We report the acute and long-term outcomes for use of the Large Diameter Advanta V12 covered stent for treatment of native and recurrent CoA. METHODS: A prospective, multicenter, nonrandomized study was performed including 70 patients (43 male), median age 17 years, median weight 57.4 kg with CoA who underwent implantation of the Large Diameter Advanta V12 covered stent. Annual follow-up for 5 years included Doppler echocardiography to calculate diastolic velocity: systolic velocity ratio. RESULTS: CoA diameter increased from 5.6±3.6 to 14.9±3.9 mm (P<0.0001) and the pressure gradient decreased from 35.8±16.2 to 5.6±7.9 mm Hg (P<0.0001). Preimplantation diastolic velocity:systolic velocity of 0.6±0.16 dropped to 0.34±0.13 (P<0.0001) and was maintained at 5 years. Computed tomography angiograms at 12 months postimplantation demonstrated the stent:transverse arch diameter to be similar, 0.91±0.09 to postprocedure 0.86±0.14. Major adverse vascular events at 30 days and 12 months were 1.4% and 4.3%, respectively. Significant adverse events included three patients who required stent implantation to treat infolding. There were no mortalities. CONCLUSIONS: The Large Diameter Advanta V12 covered stent is safe and effective for the treatment of CoA with an immediate and sustained reduction of the pressure gradient over 12 months and 5 years as assessed by preimplantation and postimplantation Doppler echocardiography and 12-month computed tomography angiography. Registration: URL: https://www.clinicaltrials.gov; Unique identifier: NCT00978952. URL: http://www.anzctr.org.au; Unique identifier: ACTRN12612000013864.

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.

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.

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.

Severe midaortic syndrome: a stepwise approach to treatment with drug-eluting balloons: a case report.

BACKGROUND: Severe forms of the midaortic syndrome cause persistent arterial hypertension and can lead to angina abdominalis. Untreated, morbidity, and mortality are significant. In addition to palliation through bypass surgery, few other therapeutic approaches lead to a long-term relief. Drug-eluting balloons (DEB) covered with paclitaxel, a cytostatic drug, have proven to be effective in bifurcational lesions and for in-stent stenoses in coronary arteries. CASE SUMMARY: In a 15-year-old girl with severe midaortic syndrome and multiple collateral arteries, four interventional balloon dilatations with DEB of increasing diameters resolved the stenosis within 8 months. After a procedure free interval of now 2.7 years, the anatomical and the physical condition of the patient remained unchanged. DISCUSSION: This stepwise approach, with a low procedural risk and a lasting result may justify further investigations into this combined treatment.

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.

The dual specificity phosphatase 2 gene is hypermethylated in human cancer and regulated by epigenetic mechanisms.

BACKGROUND: Dual specificity phosphatases are a class of tumor-associated proteins involved in the negative regulation of the MAP kinase pathway. Downregulation of the dual specificity phosphatase 2 (DUSP2) has been reported in cancer. Epigenetic silencing of tumor suppressor genes by abnormal promoter methylation is a frequent mechanism in oncogenesis. It has been shown that the epigenetic factor CTCF is involved in the regulation of tumor suppressor genes. METHODS: We analyzed the promoter hypermethylation of DUSP2 in human cancer, including primary Merkel cell carcinoma by bisulfite restriction analysis and pyrosequencing. Moreover we analyzed the impact of a DNA methyltransferase inhibitor (5-Aza-dC) and CTCF on the epigenetic regulation of DUSP2 by qRT-PCR, promoter assay, chromatin immuno-precipitation and methylation analysis. RESULTS: Here we report a significant tumor-specific hypermethylation of DUSP2 in primary Merkel cell carcinoma (p = 0.05). An increase in methylation of DUSP2 was also found in 17 out of 24 (71%) cancer cell lines, including skin and lung cancer. Treatment of cancer cells with 5-Aza-dC induced DUSP2 expression by its promoter demethylation, Additionally we observed that CTCF induces DUSP2 expression in cell lines that exhibit silencing of DUSP2. This reactivation was accompanied by increased CTCF binding and demethylation of the DUSP2 promoter. CONCLUSIONS: Our data show that aberrant epigenetic inactivation of DUSP2 occurs in carcinogenesis and that CTCF is involved in the epigenetic regulation of DUSP2 expression.

Enhanced left ventricular training in corrected transposition of the great arteries by increasing the preload.

OBJECTIVES: Patients with congenitally corrected transposition of the great arteries (ccTGA) without ventricular septal defect or left ventricular outflow tract obstruction need training of the morphological left ventricle (mLV) to undergo a double switch operation (DS) (atrial plus arterial switch). Establishing a sufficient gradient by pulmonary artery banding (PAB) is difficult and multiple re-pulmonary artery bandings (re-PABs) may be necessary to achieve the required physiology. We evaluate a new approach, consisting of a loose PAB in combination with an artificial atrial septal defect (aASD) to not only increase afterload but to dynamically enhance preload as well, in terms of the effectiveness of training of the mLV and the subsequent functional outcome after a DS. METHODS: Six consecutive patients under 6 years of age with ccTGA, who were eligible for anatomical correction, underwent enhanced left ventricular training (eLVT). Four of these patients had failed to reach sufficient mLV pressure by PAB only. RESULTS: Required systemic pressures in the mLV were achieved after a mean of 1.2 years of eLVT with no further re-PAB necessary in any patient. All patients underwent a successful DS with an uneventful postoperative period. At a mean follow-up period of 1.9 (range 0.4-3.8) years, stable LV cardiac function was observed in all patients. CONCLUSIONS: The eLVT provides additional training of the mLV to bring patients to a DS. Even in situations when isolated conventional PAB is not effective enough, the novel combination of pressure and volume loading may help with LV retraining and reduce the need for sequential pulmonary band tightening.

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.

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.

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.

Ross-konno procedure in children: midterm results.

The aim of this study was to analyze the midterm results following the Ross-Konno procedure in children. Between 1999 and 2008, 29 patients with complex left ventricular outflow tract obstruction underwent the Ross-Konno procedure. There were 12 (41%) infants (group A) and 17 (59%) older patients (group B). The median age at operation was 3.3 years (range, 6 days to 16 years). At 7 years of follow-up, survival was 96% (1 late death), with no differences between groups A and B. Freedom from aortic regurgitation ≥ mild was 81%, with no differences between groups A and B. No residual gradient was noted in the left ventricular outflow tract in either group. Freedom from mitral regurgitation ≥ mild was 100% in group B and 41% in group A (P = .0029). The mitral regurgitation was associated with morphological abnormalities of the mitral valve and with development of endocardial fibroelastosis after failed intervention during the newborn period. Freedom from reoperation was 73% in group B and 24% in group A (P = .0093). All patients are now in sinus rhythm, and 43% are without medication. With the technical aspects of this procedure well accomplished, mortality is low, and the functional outcome is encouraging. At midterm follow-up, there was no residual or recurrent outflow tract obstruction and an excellent function of the neoaortic valve. The higher incidence of mitral regurgitation in infants, which was associated with morphological abnormalities of mitral valve and development of endocardial fibroelastosis, is worrisome.