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BACKGROUND Pulmonary hypertension (PH) may limit the outcome of pediatric heart transplantation (pHTx). We evaluated pulmonary hemodynamics in children undergoing pHTx. METHODS Cross-sectional, single-center, observational study analyzing pulmonary hemodynamics in children undergoing pHTx. RESULTS Twenty-three children (female 15) underwent pHTx at median (IQR) age of 3.9 (.9-8.2) years with a time interval between first clinical signs and pHTx of 1.1 (.4-3.2) years. Indications for pHTx included cardiomyopathy (CMP) (n = 17, 74%), congenital heart disease (CHD) (n = 5, 22%), and intracardiac tumor (n = 1, 4%). Before pHTx, pulmonary hemodynamics included elevated pulmonary artery pressure (PAP) 26 (18.5-30) mmHg, pulmonary capillary wedge pressure (PCWP) 19 (14-21) mmHg, left ventricular enddiastolic pressure (LVEDP) 17 (13-22) mmHg. Transpulmonary pressure gradient (TPG) was 6.5 (3.5-10) mmHg and pulmonary vascular resistance (Rp) 2.65 WU*m$^{2}$ (1.87-3.19). After pHTx, at immediate evaluation 2 weeks after pHTx PAP decreased to 20.5 (17-24) mmHg, PCWP 14.5 (10.5-18) mmHg (p < .05), LVEDP 16 (12.5-18) mmHg, TPG 6.5 (4-12) mmHg, Rp 1.49 (1.08-2.74) WU*m$^{2}$ resp.at last invasive follow up 4.0 (1.4-6) years after pHTx, to PAP 19.5 (17-21) mmHg (p < .05), PCWP 13 (10.5-14.5) mmHg (p < .05), LVEDP 13 (10.5-14) mmHg, TPG 7 (5-9.5) mmHg, Rp 1.58 (1.38-2.19) WU*m$^{2}$ (p < .05). In CHD patients PAP increased (p < .05) after pHTx at immediate evaluation and decreased until last follow-up (p < .05), while in CMP patients there was a continuous decline of mean PAP values immediately after HTx (p < .05). CONCLUSIONS While PH before pHTx is frequent, after pHTx the normalization of PH starts immediately in CMP patients but is delayed in CHD patients.

Objectives: Factors influencing results of balloon valvuloplasty (BVP) of pulmonary valve stenosis (PS) in children are investigated. Background: BVP has become the standard of care for PS, medium-term results are not uniform and depend on various preconditions. Methods: We analysed the medium-term results of BVP of PS in children in an observational, single centre study. Need for additional procedure was defined as outcome after initial BVP. Results: We included 143 children (83 female) at a median (IQR) age of 2.6 (0.26–9.24) months and body weight of 5 (3.4–8) kg at BVP with a follow–up of 5.04 (1.6–10.2) years. We used balloon size of 10 (9–14) mm and maximal balloon pressure of 4 (3.5–10) atm, resulting in balloon–to–pulmonary annulus ratio of 1.28 (1.2–1.4). Systolic pressure gradient of PS was reduced with BVP (43.5 mmHg vs. 14.0 mmHg, p < 0.001) and confirmed by echocardiography (68.0 mmHg vs. 25.0 mmHg, p < 0.001) day 1 post procedure. Pulmonary BVP with associated supravalvular PS resulted in a relevant reduction of systolic pressure gradient in 23 of 31 patients (74.2%). Early additional procedure was necessary in 14 patients (9.8%) after 0.2 (0.1–0.7) years due to residual PS (n = 13) and infective endocarditis (n = 1). Factors for additional procedures were associated supravalvular PS with a higher residual pressure gradient, but not genetic syndrome. During further follow–up of 5.04 (1.6–10.2) years no further additional procedures were needed. Conclusions: Pulmonary BVP of native pulmonary valve stenosis leads to excellent medium-term results, even in 3 of 4 infants with associated supravalvular obstruction sufficient pressure relief can be obtained.

Background. Stenting of patent ductus arteriosus (PDA) is a minimally invasive catheter-based temporary palliative procedure that is an alternative to a surgical shunt in neonates with duct-dependent pulmonary perfusion. Methods. An observational, single-centre, cross-sectional study of patients with duct-dependent pulmonary perfusion undergoing PDA-stenting as a stage I procedure and an analysis of short- to mid-term follow-up until a subsequent surgical procedure (stage II), with a focus on the interstage course. Results. Twenty-six patients were treated with PDA-stenting at a median (IQR) age of 7 (4–10) days; 10/26 patients (38.5%) (6/10 single pulmonary perfusion) were intended for later univentricular palliation, 16/26 patients (61.5%) (13/16 single pulmonary perfusion) for biventricular repair. PDA diameter was 2.7 (1.8–3.2) mm, stent diameter 3.5 (3.5–4.0) mm. Immediate procedural success was 88.5%. The procedure was aborted, switching to immediate surgery after stent embolisation, malposition or pulmonary coarctation in three patients (each n = 1). During mid-term follow-up, one patient needed an additional surgical shunt due to severe cyanosis, while five patients underwent successful catheter re-intervention 27 (17–30) days after PDA-stenting due to pulmonary hypo- (n = 4) or hyperperfusion (n = 1). Interstage mortality was 8.6% (2/23), both in-hospital and non-procedure-related. LPA grew significantly (p = 0.06) between PDA-stenting and last follow-up prior to subsequent surgical procedure (p = 0.06). RPA Z-scores remained similar (p = 0.22). The subsequent surgical procedure was performed at a median age of 106 (76.5–125) days. Conclusions. PDA-stenting is a feasible, safe treatment option, with the need for interdisciplinary decision-making beforehand and surgical backup afterwards. It allows adequate body and pulmonary vessel growth for subsequent surgical procedures. Factors determining the individual patient’s course should be identified in larger p

To determine immediate and long-term follow-up of transcatheter closure of patent ductus arteriosus (PDA) in children. Background: National antibiotic prophylaxis (AP) guideline for infective endocarditis changed after 2009, the effect on practice of PDA closure is unknown. Methods: Observational single center study analyzing follow-up of PDA closure comparing two time periods before (2002–2009) and after (2010–2019) changes in AP guideline. Results: 332 patients (68.1% female), median (interquartile range) age 3.0 years (1.5–5.7) and body weight 14.0 kg (10.0–19.3), were enrolled. PDA morphology was conical type A (50.3%), window type B (1.2%), tubular type C (40.1%), complex type D (2.1%), elongated type E (0.9%) and other (5.4%). Minimal PDA diameter and length were 1.9 mm (1.3–2.5) and 8.0 mm (6.2–10.2). PDA was closed using coils (56.3%), Amplatzer Duct Occluders (41.9%) and others (1.8%). Complete closure rate was 61.1% at catheter intervention, 72.3% on day 1, 87.7% after 6 months and 98.4% at last follow-up on echocardiography. Moderate complication rate (severity level 3) was 4.2% and major complication rate (severity level 4) 0.3%, with no catastrophic complications (severity level 5). Annual PDA closure rate declined in the second time period (22.6/year vs. 15.5/ year, p = 0.018), PDA size increased (1.6 mm vs. 2.0 mm, p = 0.002) and proportion of coils decreased (72.4% vs. 37.1%, p < 0.001). Conclusions: Interventional closure of PDA is associated with excellent closure rates during follow-up (>98%) and only a small number of complications leading to reintervention or surgery. Change in AP guidelines changed indication for and practice of PDA closure.

This study evaluates coagulation profiles of single ventricle (SV) patients in relationship to liver function, hemodynamic variables and outcome. Twenty-six children with SV anatomy were included. Advanced coagulation profiles, invasive preoperative hemodynamic parameters and clinical course were retrospectively analyzed. Median (interquartile range) age and weight at the time of blood sampling was 25.5 (31) months and 10.5 (6.9) kg. Sixteen patients (16/26; 62%) showed decreased antithrombin and/or protein C (PC) and/or free protein S (PS) function and/or free PS antigen. Two patients showed abnormal activated PC resistance ratio due to heterozygous factor V Leiden mutation and 1 heterozygous prothrombin G20210A mutation. Group comparison (abnormal coagulation profile [group 1; n = 16] versus normal coagulation profile [group 2; n = 10]) showed longer postoperative hospitalization time (p = .04), longer postoperative catecholamine support (p = .01), a higher incidence of thromboembolic events (p = .04), and chylothoraxes (p = .007) in group 1. In 5 (31%) of 16 group 1 patients, thromboembolic complications occurred: cerebral stroke (n = 1), intestinal ischemia (n = 2), thrombus formation in inferior caval vein (n = 1), and pulmonary vein (n = 1). Abnormalities in coagulation parameters are common in SV patients. Coagulation abnormalities constitute a preoperative risk factor and affect postoperative course.

BACKGROUND Long-term survival of patients with a single ventricle palliated with a Fontan procedure is still limited. No curative treatment options are available. To investigate the pathophysiology and potential treatment options, such as mechanical circulatory support (MCS), appropriate large animal models are required. The aim of this review was to analyze all full-text manuscripts presenting approaches for an extracardiac total cavopulmonary connection (TCPC) animal model to identify the feasibility and limitations in the acute and chronic setting. METHODS A literature search was performed for full-text publications presenting large animal models with extracardiac TCPCs on Pubmed and Embase. Out of 454 reviewed papers, 23 manuscripts fulfilled the inclusion criteria. Surgical procedures were categorized and hemodynamic changes at the transition from the biventricular to the univentricular condition analyzed. RESULTS Surgical procedures varied especially regarding coronary venous flow handling and anatomic shape of the TCPC. In most studies (n = 14), the main pulmonary artery was clamped and the coronary venous flow redirected by additional surgical interventions. Only in five reports, the caval veins were connected to the right pulmonary artery to create a true TCPC shape, whereas in all others (n = 18), the veins were connected to the main pulmonary artery. An elevated pulmonary vascular resistance was identified as a limiting hemodynamic factor for TCPC completion in healthy animals. CONCLUSIONS A variety of acute TCPC animal models were successfully established with and without MCS, reflecting the most important hemodynamic features of a Fontan circulation; however, chronic animal models were not reported.

BACKGROUND: Stent implantation into growing vessels is a common treatment option in infants and children with congenital heart disease (CHD) and corresponding vessel lesions. After stent implantation in small children, repetitive stent redilations are frequently necessary to accommodate for somatic growth. Until now, all available stents have limited final expansion diameters. MATERIAL AND RESULTS: The new Bentley BeGrow™ stent system for newborns and infants is a L605 cobalt‑chromium, pre-mounted, balloon expandable stent, which is compatible with a 4 French sheath and 0.014 inch guide wire thus allowing implantation in small vessels (4-6 mm). It offers a new, unique stent design that allows post-dilation steps up to Ø11.5 mm. While re-dilating up to Ø11.5 mm this new stent maintains radial force and shows uniform expansion with only minimal foreshortening. Predetermined breaking points allow the stent struts to break in a controlled manner when exceeding a diameter of 11.5 mm. Residual radial force maintains even after stent opening due to spiral arrangement of the predetermined breaking points. The 2 first-in-man pulmonary artery stent implantations in a newborn with univentricular circulation and a toddler with biventricular circulation are reported as part of the currently performed licencing trial (ClinicalTrials.govNCT03287024). CONCLUSION: The low-profile BeGrow™ stent system offers new treatment options for transcatheter stent implantations in newborns and infants. In our first experience, it can be effectively implanted. Longer follow-up will evaluate multiple, stepwise redilations and controlled stent strut breakage, which have the potential to accommodate for somatic vessel growth and/or subsequent implantation of larger stents.

Direct communication between the right pulmonary artery (RPA) and the left atrium (LA) is a very rare cardiac malformation. Clinical presentation of RPA-to-LA communication depends on the size of the communication, the amount of right-to-left shunt, the patient's age, and pulmonary vascular resistance. Patients with small communications usually present oligosymptomatic and are diagnosed at an older age. A delay of diagnosis bears the risk of severe complications and needs to be prevented by proper work-up of oligosymptomatic neonates. Treatment of RPA-to-LA communications used to be performed by surgical closure, and the interventional approach has only been established as a less invasive alternative in recent years. Conclusion: Although patients with small RPA-to-LA communications usually present oligosymptomatic, early diagnosis and treatment is essential to prevent life-threatening complications.

In many paediatric cardiac units chest radiographs are performed routinely before discharge after cardiac surgery. These radiographs contribute to radiation exposure. To evaluate the diagnostic impact of routine chest X-rays before discharge in children undergoing open heart surgery and to analyze certain risk factors predicting pathologic findings. This was a prospective (6months) single-centre observational clinical study. One hundred twenty-eight consecutive children undergoing heart surgery underwent biplane chest X-ray at a mean of 13days after surgery. Pathologic findings on chest X-rays were defined as infiltrate, atelectasis, pleural effusion, pneumothorax, or signs of fluid overload. One hundred nine asymptomatic children were included in the final analysis. Risk factors, such as age, corrective versus palliative surgery, reoperation, sternotomy versus lateral thoracotomy, and relevant pulmonary events during postoperative paediatric intensive care unit (PICU) stay, were analysed. In only 5.5% (6 of 109) of these asymptomatic patients were pathologic findings on routine chest X-ray before discharge found. In only three of these cases (50%), subsequent noninvasive medical intervention (increasing diuretics) was needed. All six patients had relevant pulmonary events during their PICU stay. Risk factor analysis showed only pulmonary complications during PICU stay to be significantly associated (p=0.005) with pathologic X-ray findings. Routine chest radiographs before discharge after cardiac surgery can be omitted in asymptomatic children with an uneventful and straightforward perioperative course. Chest radiographs before discharge are warrantable if pulmonary complications did occur during their PICU stay, as this is a risk factor for pathologic findings in chest X-rays before discharge