Percutaneous Transhepatic Portosystemic Shunt Creation in a Preterm Infant With Total Anomalous Pulmonary Venous Connection.

A male preterm infant with total anomalous pulmonary venous connection developed progressive respiratory distress but was considered too high risk for surgical repair because of his critical condition. We demonstrated that percutaneous transhepatic stent implantation for portosystemic shunt creation is a feasible bridging procedure when surgery is not possible.

Stenting of high-tortuous ducts in duct-dependent pulmonary circulation: essential points to consider before deciding on stenting.

Despite the advancements in the technique of duct stenting (DS) in patients with duct-dependent pulmonary circulation (DDPC) and the valuable role of DS in preventing the risk of surgical creation of shunts and early repair, not all ducts are amenable to being stented, and not all interventions with DS are safe and can achieve positive outcomes. Very few studies focusing on tortuous ducts have been conducted until now. Their results showed that stenting of highly tortuous ducts has the same risk as surgical options. This type of stenting has greater possibility of complications, early in-stent thrombosis, and stent failure than do other duct types. In such cases, the surgical options could be superior to DS and have better outcomes. This report aims to review the very scarce available data about stenting of high-tortuous ducts and criticisms of performing DS in ducts associated with pulmonary stenosis and to highlight the essential points that must be considered before deciding on intervention.

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.

Sedation versus General Anesthesia for Cardiac Catheterization in Infants: A Retrospective, Monocentric, Cohort Evaluation.

BACKGROUND: Children with congenital heart disease require repeated catheterization. Anesthetic management influences the procedure and may influence outcome; however, data and recommendations are lacking for infants. We studied the influence of sedation versus general anesthesia (GA) on adverse events during catheterization for children <2 years old. METHODS: We conducted a monocentric, retrospective study of all catheterization procedures (2008-2013). High-severity adverse event (HSAE) rates were compared using propensity-score-adjusted models, including pre- and intra-procedural variables. RESULTS: 803 cases (619 patients) (368 (46%) GA, 435 (54%) sedation) with a mean age of 6.9 ± 6.1 months were studied. The conversion rate (GA after sedation) was 18 (4%). Hospital stay was 4.9 ± 4.0 and 4.1 ± 2.5 (p = 0.01) after GA or sedation, respectively. HSAE occurred in 75 (20%) versus 40 (9%) (p < 0.01) in GA versus sedation procedures, respectively. Risk factors (multivariable analysis) were older patients (p = 0.05), smaller weights (p < 0.01), palliated status (OR 3.2 [1.2-8.9], p = 0.02), two-ventricle physiology (OR 7.3 [2.7-20.2], p < 0.01), cyanosis (OR 4.6 [2.2-9.8], p < 0.01), pulmonary hypertension (OR 5.6 [2.0-15.5], p < 0.01), interventional catheterization (OR 1.8 [1.1-3.2], p = 0.02) and procedure-type risk category 4 (OR 28.9 [1.8-455.1], p = 0.02). Sedation did not increase the events rate and decreased the requirement for hemodynamic support (OR 5.2 [2.2-12.0], p < 0.01). CONCLUSION: Sedation versus GA for cardiac catheterization in children <2 years old is safe and effective with regard to HSAE. Sedation also decreases the requirement for hemodynamic support. Paradoxical effects (older age and two-ventricle physiology) on risk have been found for this specific age cluster.

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.

Pulmonary artery banding in dilative cardiomyopathy of young children: review and protocol based on the current knowledge.

Dilated cardiomyopathy (DCM) is a leading cause of cardiac death in children. Current therapeutic strategies are focused on improving symptoms of congestive heart failure (CHF); the potentials of cardiac regeneration especially in infants and young children are neglected in particular when DCM is classified as "end-stage". Heart transplantation (HTx) serves as the only life-saving option, despite is palliative character with limited survival time. Therapeutic alternatives are strongly needed, but already existing though less used; presupposed, that cardiac dysfunction and its treatment are not reduced to the four components of heart rate (rhythm), myocardial contractility, preload and afterload. A paradigm shift in the treatment of pediatric heart failure can be achieved by modifying ventricular afterload with improving contra-lateral ventricular function. Adverse ventricular-ventricular interactions (VVI) have the potential to harness them for therapeutic benefit. Surgical placement of a pulmonary artery banding (PAB) utilized in infants and young children with LV-DCM and preserved RV function are able to improve LV function via VVI; it is hypothesized, that functional recovery can be achieved in almost 80% especially of infants with LV-DCM despite criteria for listing to orthotopic HTx. The review summarizes details of the current perioperative treatment enabling each pediatric heart center to utilize rPAB as a strategy for functional recovery, even in centers without the option for Htx. Of course, future studies are needed to delineate the geometrical, temporal and molecular mechanisms of PA-banding-induced ventricular crosstalk and to examine their potential modulation through mechanical, electrophysiological and pharmacological interventions, but our patients are born, now.