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.).

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.

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.

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.

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.

2019 updated consensus statement on the diagnosis and treatment of pediatric pulmonary hypertension: The European Pediatric Pulmonary Vascular Disease Network (EPPVDN), endorsed by AEPC, ESPR and ISHLT.

The European Pediatric Pulmonary Vascular Disease Network is a registered, non-profit organization that strives to define and develop effective, innovative diagnostic methods and treatment options in all forms of pediatric pulmonary hypertensive vascular disease, including pulmonary hypertension (PH) associated with bronchopulmonary dysplasia, PH associated with congenital heart disease (CHD), persistent PH of the newborn, and related cardiac dysfunction. The executive writing group members conducted searches of the PubMed/MEDLINE bibliographic database (1990-2018) and held face-to-face and web-based meetings. Ten section task forces voted on the updated recommendations, based on the 2016 executive summary. Clinical trials, meta-analyses, guidelines, and other articles that include pediatric data were searched using the term "pulmonary hypertension" and other keywords. Class of recommendation (COR) and level of evidence (LOE) were assigned based on European Society of Cardiology/American Heart Association definitions and on pediatric data only, or on adult studies that included >10% children or studies that enrolled adults with CHD. New definitions by the World Symposium on Pulmonary Hypertension 2018 were included. We generated 10 tables with graded recommendations (COR/LOE). The topics include diagnosis/monitoring, genetics/biomarkers, cardiac catheterization, echocardiography, cardiac magnetic resonance/chest computed tomography, associated forms of PH, intensive care unit/lung transplantation, and treatment of pediatric PH. For the first time, a set of specific recommendations on the management of PH in middle- and low-income regions was developed. Taken together, these executive, up-to-date guidelines provide a specific, comprehensive, detailed but practical framework for the optimal clinical care of children and young adults with PH.

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.

Promising Outcome of Anatomic Correction of Corrected Transposition of the Great Arteries.

BACKGROUND: Anatomic correction of corrected transposition of the great arteries with associated lesions, utilizing the morphologic left ventricle as a systemic pumping chamber, is the preferred method in many centers. The purpose of this study was to analyze functional outcome after anatomic correction. METHODS: Between Jan 1997 and May 2016, 63 patients with corrected transposition of the great arteries and associated lesions underwent anatomic correction. Forty-two patients (67%) underwent palliation before correction, including 14 patients (22%) who required training of systemic ventricle. The double switch procedure was performed in 37 patients; 25 patients underwent the Senning-Rastelli operation, and 1 patient underwent the Senning-Nikaidoh procedure. The median age at correction was 1.6 ± 3.7(SD) years (range, 0.2 to 17.8 years). RESULTS: The survival and freedom from any event was 95% and 71%, respectively, at 15-year follow-up. The combined freedom from death, failure of systemic ventricle, or heart transplant was 93% at 15-year follow-up regardless of procedure type. Sinus rhythm was present in 49 patients, with 14 patients requiring pacemaker (22%)-8 preoperatively, 4 early postoperatively, and 2 late postoperatively. Neurological development is normal in all patients. Fifty-four percent of the patients are not on medication. CONCLUSIONS: Anatomic correction of corrected transposition of the great arteries is a safe procedure that provides encouraging survival and functional benefits. Ninety-three percent preservation of morphological left ventricle function in 15 years of follow-up supports the concept of anatomic correction. Longer follow-up is needed to confirm superiority of this approach over other management strategies.

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.

[Antibradycardia therapy : Indication and implementation]. / Antibradykarde Therapie : Indikation und Umsetzung.

Pacemaker therapy (PMT) in grown-up congenital heart disease (GUCH) must meet the demands of a young, dynamic and heterogeneous group of patients. The duration of the therapy has to be planned for several decades and should be accompanied by an invasive procedure at the very least. Most of the patients enter adulthood with their pacemaker (PM) already implanted; for others the indications can be derived from the published guidelines for GUCH and PMT, but need to be adjusted to the individual situation of the patient. Depending on the underlying heart disease the decision on the use of either an epimyocardial or a transvenous PM system has to be made. Both electrodes and PM should correspond to the latest technical developments to optimally adapt to the patients' multiple requirements. In the case of PM system revisions abandoned leads should be removed and vascular stenosis or occlusions cleared to be prepared for later revisions. During any cardiac surgery epimyocardial PM systems should be checked against the patient's needs and expanded or revised accordingly. Epimyocardial resynchronization systems in particular offer more opportunities for compensating for cardiac dysfunction with greater reliability using a second ventricular lead. The PMT is an essential part of the medical treatment for many patients with GUCH and contributes significantly to the well-being and quality of life. Against this background, a competent and consequent follow-up regime requires experienced physicians. An integrated telemetric monitoring system for the PM has proven valuable and supports the early recognition of cardiac arrhythmia.

Diagnostics, monitoring and outpatient care in children with suspected pulmonary hypertension/paediatric pulmonary hypertensive vascular disease. Expert consensus statement on the diagnosis and treatment of paediatric pulmonary hypertension. The European Paediatric Pulmonary Vascular Disease Network, endorsed by ISHLT and DGPK.

Pulmonary hypertension (PH) is a condition of multiple aetiologies with underestimated prevalence and incidence. Indeed, despite access to modern therapies, pulmonary hypertensive vascular disease (PHVD) remains a progressive, usually life-limiting condition, severely impacting on the patients' well-being. We herein provide practical, expert consensus recommendations on the initial diagnostic work-up, clinical management and follow-up of children and adolescents with PH/PHVD, including a diagnostic algorithm. The major topics and methods that need to be tailored and put into context of the individual patient include PH classification, clinical signs and symptoms, basic diagnostic and advanced imaging measures (ECG, chest X-ray, transthoracic echocardiography, cardiac magnetic resonance, chest CT angiography, cardiac catheterisation, ventilation-perfusion lung scan, abdominal ultrasound), lung function tests, 6 min walk and cardiopulmonary exercise testing, sleep study (polysomnography), laboratory/immunological tests, considerations for elective surgery/ general anaesthesia, physical education and exercise, flying on commercial airplanes, vaccinations, care of central intravenous lines and palliative care. Due to the complexity of PH/PHVD, the clinical care has to be multidisciplinary and coordinated by a dedicated specialist paediatric PH centre, not only to decrease mortality but to allow children with PH/PHVD to reach a reasonable quality of life.

Executive summary. Expert consensus statement on the diagnosis and treatment of paediatric pulmonary hypertension. The European Paediatric Pulmonary Vascular Disease Network, endorsed by ISHLT and DGPK.

UNLABELLED: : The European Paediatric Pulmonary Vascular Disease (PVD) Network is a registered, non-profit organisation that strives to define and develop effective, innovative diagnostic methods and treatment options in all forms of paediatric pulmonary hypertensive vascular disease, including specific forms such as pulmonary arterial hypertension (PAH)-congenital heart disease, pulmonary hypertension (PH) associated with bronchopulmonary dysplasia, persistent PH of the newborn, and related cardiac dysfunction. METHODS: The writing group members conducted searches of the PubMed/MEDLINE bibliographic database (1990-2015) and held five face-to-face meetings with votings. Clinical trials, guidelines, and reviews limited to paediatric data were searched using the terms 'pulmonary hypertension' and 5-10 other keywords, as outlined in the other nine articles of this special issue. Class of recommendation (COR) and level of evidence (LOE) were assigned based on European Society of Cardiology/American Heart Association definitions and on paediatric data only, or on adult studies that included >10% children. RESULTS: A total of 9 original consensus articles with graded recommendations (COR/LOE) were developed, and are summarised here. The topics included diagnosis/monitoring, genetics/biomarker, cardiac catheterisation, echocardiography, cardiac magnetic resonance/chest CT, associated forms of PH, intensive care unit/ventricular assist device/lung transplantation, and treatment of paediatric PAH. CONCLUSIONS: The multipaper expert consensus statement of the European Paediatric PVD Network provides a specific, comprehensive, detailed but practical framework for the optimal clinical care of children with PH.

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.