Pulmonary hemodynamics before and after pediatric heart transplantation.

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*m2 (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*m2 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*m2 (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.

Risk Factors and Outcome of Pulmonary Artery Stenting After Bidirectional Cavopulmonary Connection (BDCPC) in Single Ventricle Circulation.

After bidirectional cavopulmonary connection (BDCPC) central pulmonary arteries (PAs) of single ventricle (SV) patients can be affected by stenosis or even closure. Aim of this study is to compare SV patients with and without PA-stent implantation post-BDCPC regarding risk factors for stent implantation and outcome. Single center, retrospective (2006-2021) study of 136 SV consecutive patients with and without PA-stent implantation post-BDCPC. Patient characteristics, risk factors for PA-stent implantation and PA growth were assessed comparing angiographic data pre-BDCPC and pre-TCPC. A total of 40/136 (29%) patients underwent PA-stent implantation at median (IQR) 14 (1.1-39.0) days post-BDCPC. 37/40 (92.5%) underwent LPA-stenting. Multiple regression analysis showed single LV patients to receive less likely PA-stents than single RV patients (OR 0.41; p = 0.05). Reduced LPA/BSA (mm/m2) and larger diameter of neo-ascending aorta pre-BDCPC were associated with an increased likelihood of PA-stent implantation post-BDCPC (OR 0.89, p = 0.03; OR 1.05, p = 0.001). Stent re-dilatation was performed in 36/40 (89%) after 1 (0.8-1.5) year. Pulmonary artery diameters pre-BDCPC were lower in the PA-stent group: McGoon (p < 0.001), Nakata (p < 0.001). Indexed pulmonary artery diameters increased equally in both groups but remained lower pre-TCPC in the PA-stent group: McGoon (p < 0.001), Nakata (p = 0.009), and Lower Lobe Index (p = 0.003). LPA and RPA grew symmetrically in both groups. Single RV, larger neo-ascending aorta, and small LPA pre- BDCPC are independent risk factors for PA-stent implantation post-BDCPC. Pulmonary artery diameters after PA-stent implantation and stent re-dilatation showed significant growth together with the contralateral side, but the PA-system remained symmetrically smaller in the stent group.

Transcatheter Closure of Atrial Septal Defect with Carag Bioresorbable Septal Occluder™: First-in-Child Experience with 12-MonthFollow-Up.

Background: Nowadays, transcatheter device closure of an atrial septal defect (ASD) is a standard approach in children. Potential early and long-term side effects or complications related to the metal framework of the devices are a known issue. A bioresorbable device such as the Carag Bioresorbable Septal Occluder™ (CBSO) could resolve such complications. Material and Results. The Carag Bioresorbable Septal Occluder™ (CBSO; Carag AG, Baar, Switzerland) is a self-centering double disk, repositionable, and retractable device with a bioresorbable framework (polylactic-co-glycolic acid), which is almost completely resorbed by 18-24 months postimplantation. This manuscript reports the four first-in-child ASD device closures using a CBSO. The patients' age was median (IQ1-IQ3), 4.5 years (4-7.25). Weight was 21.3 kg (17.6-32.7). We demonstrated procedural feasibility and safety. Effective defect closure with the device was 100%. Echocardiographic measurements of the thickness of the interatrial septum did not show any relevant increase over a 12-monthfollow-up period. There were no residual defects found after the procedure or later during the resorption process. The patients showed no evidence of any local or systemic inflammatory reaction. Conclusions: The CBSO device system could offer a new treatment option for transcatheter ASD device closure in the pediatric and adult fields. In our first-in-child experience, it was effectively and safely implanted. During the first 12 months of follow-up, no complications occurred.

Balloon angioplasty and stent implantation within 30 days postcongenital heart surgery (CHS) in children.

OBJECTIVES: This study aims to assess balloon angioplasty (BAP) and stent implantation (SI) procedures early after congenital heart surgery (CHS) in children. BACKGROUND: These interventions are considered potential high-risk procedures and often avoided or postponed. METHODS: This is a retrospective, single centre study of all BAP and SI procedures within 30 days after CHS (01/2001 until 01/2021). RESULTS: A total of 127 (96 SI, 31 BAP) procedures were performed in 104 patients at median 6.5 days (interquartile range: 1-15) after CHS. Balloon-to-stenosis ratio and balloon-to-reference vessel ratio were significantly smaller compared to stent-to-stenosis ratio and stent-to-reference vessel ratio (p < .001 and p = .005). There was a greater rise in absolute vessel diameter, greater rise in vessel diameter in relation to the stenosis and vessel diameter in relation to the reference vessel with SI (p < .001, p = .01, and p < .001). Up to 94% SIs fulfilled both success criteria (increase of vessel diameter ≥50% of minimal vessel diameter or achievement ≥75% of the reference vessel diameter). Major adverse events were more frequent in the BAP group (p = .05). Intraprocedural complications were 5/31 (16%) in the BAP group and 13/96 (13%) in the SI group (p = .77). CONCLUSION: BAP and SI procedures within 30 days post-CHS can be performed safely, with a greater stent-to-stenosis ratio and a greater rise in vessel diameter with stent implantation.

Stenting and overdilating small Gore-Tex vascular grafts in complex congenital heart disease.

BACKGROUND: Gore-Tex® grafts are integral in the management of congenital heart disease. Issues of graft stenosis or somatic outgrowth may precipitate high-risk early surgery, and catheter intervention is a relatively under-reported management option. OBJECTIVES: To assess efficacy, safety, and outcomes of stenting and overdilating small Gore-Tex® vascular grafts with the aim of optimizing surgical timing. METHODS: Retrospective analysis of single-center, 13-year experience of 93 graft stenting interventions or reintervention in 80 patients, with the aim of relieving stenosis ± overdilation, to depose surgical graft revision or optimize surgical timing and candidacy. RESULTS: Median preintervention graft diameter was 52% (IQR 43-63) of nominal size, postintervention this increased to median 102% (IQR 96-120) [P < 0.001]. Overdilation was achieved in 54%; of these, the median final internal lumen was 126% (IQR 113-132) [P < 0.001]. Mean oxygen saturations increased from 69% to 82% [P < 0.001]. Adverse event rate was 14.9%; this includes mortality (3.2%) and atrioventricular conduction block (8.5%). Univariate analysis and logistic regression showed a significant relationship between adverse events and young age [P < 0.01], low weight [P < 0.01], univentricular physiology [P < 0.001], use of femoral venous access alone [P = 0.03], and intervention on a right ventricle to pulmonary artery conduit [P = 0.03]. Within respective indication groups, 74% were bridged to elective cavopulmonary shunt, 95% were bridged to biventricular surgery, and 78% of long-term palliative care patients continue under follow-up. CONCLUSIONS: Gore-Tex® vascular grafts can be stented effectively and expanded beyond nominal diameters by around +26%. This improves oxygen saturations, providing excellent palliation and optimized surgical timing. Adverse events are most frequent in precavopulmonary shunt patients. CONDENSED ABSTRACT: Gore-Tex® grafts are widely used in the palliation of cyanotic congenital heart disease. Grafts may become stenosed and do not allow for somatic growth. Over a 13 year period, graft stenting was performed in 80 patients (93 technically successful interventions, 4 unsuccessful.) Median internal lumen change was +50% (of nominal graft size), mean oxygen saturation change +13%. Over-dilation was performed in more than half of the cohort, with a median gain of +26% on nominal graft size. In precavopulmonary shunt patients, there was a moderate incidence of serious complications. Clinically useful deferral of surgery was achieved for the majority.

Catheter interventional treatment of congenital portosystemic venous shunts in childhood.

OBJECTIVES: The study aimed on the catheter interventional treatment of congenital portosystemic venous shunt (CPSVS) in childhood and the impact on vascular growth of hypoplastic portal veins. Clinical course and follow up of partial or complete closure of CPSVS are described. BACKGROUND: CPSVS is a very rare vascular malformation of the portal venous drainage resulting in potentially life threatening abdominal, pulmonary, cerebral, and cardiac complications. The anatomic type, clinical course, and diagnosis must be determined for optimal management. METHODS: Single centre case series. RESULTS: Eight (6 female) children with extrahepatic (n = 5) and intrahepatic (n = 3) CPSVS were diagnosed invasively by catheterization, including test balloon occlusion of the shunt and simultaneous retrograde angiography, and treated by catheter interventions (n = 5) with partial (n = 2) and complete (n = 3) occlusion of CPSVS at a median age of 3.9 years (range 0.7-21). Congenital heart disease (CHD) was the most frequent associated organ manifestation (n = 5) followed by mild to severe pulmonary arterial hypertension (n = 4), hepatopulmonary syndrome (n = 2), and portosystemic encephalopathy (n = 1). CHD was simple (n = 3) or complex type (n = 2). Three patients were untreated so far, because they were in excellent clinical condition at an age <1 year, refused treatment, or planned for later treatment. CONCLUSIONS: Accurate invasive diagnosis of CPSVS with test balloon occlusion of the shunt is mandatory to depict the anatomic situation. Catheter interventional treatment of CPSVS offers a feasible and safe approach with complete or partial closure of the vascular malformation inducing potentially significant vascular growth of a former hypoplastic portal venous system. © 2015 Wiley Periodicals, Inc.

Effectiveness of Balloon Angioplasty in Children With Recurrent Aortic Coarctation Depends on the Type of Aortic Arch Pathology.

OBJECTIVES: The aim of this study was to evaluate the effectiveness of balloon angioplasty (BAP) for recurrent aortic coarctation (ReCoA) in infants comparing simple and complex type of aortic arch pathology (Norwood I procedure). BACKGROUND: ReCoA is a known complication after cardiovascular surgery for coarctation of the aortic arch. METHODS AND RESULTS: In a single center case study, we analyzed 20 infants undergoing BAP for ReCoA comparing simple (n = 10) and complex type of aortic arch pathology (n = 10). At catherization diameter of ReCoA stenosis was 3.2 ± 0.7 mm (mean ± SD) with short localized (11/20) or long hypoplastic stenosis (9/20) before and 4.8 ± 1.2 mm after BAP (P < 0.001). Invasive systolic pressure gradient was reduced from 27.5 ± 16.2 mmHg before to 5.1 ± 6.6 mmHg after BAP (P < 0.001), comparable in simple and complex type of ReCoA. At day 1 after catherization noninvasive systolic arterial blood pressure gradient was reduced from 20.2 ± 23.1 to 6.7 ± 9.9 mmHg (P < 0.001), respectively, calculated continuous wave Doppler echo gradient from 36.3 ± 22 to 16.8 ± 9.6 mmHg (P < 0.01). Complications were aortic arch dissection (1/20), discrete aortic arch aneurysm formation (1/20), pericardial effusion (1/20), and peripheral arterial thrombosis (4/20). At a mid-term follow up of 9.5 months (1-40) after BAP, 3 infants needed early surgical reintervention due to secondary ReCoA, all with long hypoplastic aortic arch segments, but simple type of aortic arch pathology. CONCLUSIONS: At mid-term follow up, BAP remains an effective catheter intervention for ReCoA for infants with localized ReCoA, but not with long hypoplastic aortic arch segments, even in simple type of aortic arch pathology.

Interventional Closure of Secundum Type Atrial Septal Defects in Infants Less Than 10 Kilograms: Indications and Procedural Outcome.

AIMS: This study set out to assess indications, feasibility, complications, and clinical outcome of percutaneous transcatheter device closure of atrial septal defects (ASDs) in infants with a bodyweight below 10 kg. METHODS AND RESULTS: Retrospective single center chart and echocardiography review study from 8/2005-12/2013. Twenty-eight children with ASD (13 female) with a median age of 1.15 years (0.2-2.8) and a median weight of 7.2 kg (4.5-9.9) were analyzed. Indications for early ASD closure were failure to thrive (n = 15, 54%), bronchopulmonary dysplasia (BPD) with supplemental oxygen dependency (n = 7, 25%), and genetic syndromes with suspected pulmonary hypertension (n = 12, 43%). Device implantation was successful in all patients without any periprocedural mortality or major complication. Clinical outcome after a median follow-up period of 2.1 years (0.25-7.3) revealed no residual shunt and a significant decrease of right ventricular volume load. Patients with pulmonary hypertension experienced a significant reduction of pulmonary artery/RV pressure. Patients also showed decreased supplemental oxygen dependency and less cardiac medications, but no significant "catch-up growth" in those with failure to thrive. CONCLUSION: Interventional ASD closure in children weighing less than 10 kg can be performed without any additional major risks and shows a favorable outcome, especially in selected patients with significant non cardiac co-morbidities.

Restrictive atrial septum after the Fontan procedure.

In this study, three patients presenting with early or late postoperative Fontan complications were identified to suffer from restriction of the native atrial septum. This caused significant obstruction to pulmonary venous return and elevated systemic venous pressure. Dobutamine stress testing was used in one patient to identify this lesion. Transcatheter stenting was performed in the other two patients. Patients improved after relief of the obstruction.

Initial experience with the cook formula balloon expandable stent in congenital heart disease.

INTRODUCTION: Balloon expandable stents are an integral part in the catheter treatment of congenital heart disease. In the growing child, stents require dilatation to greater diameters over time. The Cook Formula stent is a recent 316 stainless steel open-cell design licensed for peripheral vascular work. METHODS: Following extensive ex vivo studies, 112 stents were implanted in 97 children [median age 3.9 (0.01-17.6) years; median weight 13.7 (2.4-62.8) kg] over a 27-month (Oct 2011-Dec 2013) period. RESULTS: Bench testing revealed that there was no stent shortening for dilatation to nominal diameter and beyond. The 5 mm stents could be dilated up to 10 mm, and the 10 mm stents to 20 mm. Stents were implanted through 4-7F sheaths or guide catheters over appropriate wires. Stent tracking and delivery was excellent. Twenty-three stents were implanted in the right ventricular outflow tract in Fallot-type lesions, 53 for branch pulmonary artery stenosis (22 post cavopulmonary shunt/Fontan), 14 conduit stenosis, 13 Fontan fenestrations, 3 PDA in hybrid stage I Norwood, 5 in coarctation, and 1 for SVC obstruction. Sixty-one stents (54%) were overdilated. There were no stent fractures. Radial strength was very good, whereas stent conformability was limited. CONCLUSIONS: The Cook Formula stent is a premounted balloon-expandable stent that can be significantly overdilated with virtually no shortening allowing for precise placement and minimal protrusion into adjacent vessels. The Formula stent is a very versatile addition to the range of stents for use in the catheter treatment of complex congenital heart disease in children. © 2014 Wiley Periodicals, Inc.

Interventional closure of RPA-to-LA communication in an oligosymptomatic neonate.

UNLABELLED: 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.

Impact of chest X-ray before discharge in asymptomatic children after cardiac surgery--prospective evaluation.

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 (6 months) single-centre observational clinical study. One hundred twenty-eight consecutive children undergoing heart surgery underwent biplane chest X-ray at a mean of 13 days 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.

Transannular bovine jugular vein monocusp for the reconstruction of severe right ventricular outflow tract obstruction.

Objective: Severe right ventricular outflow tract obstruction in tetralogy of Fallot and variants necessitates the use of transannular patch in a significant proportion of children undergoing repair. We have used a Contegra monocusp together with delamination of native leaflet tissue in order to create a functioning pulmonary valve. Methods: In total, 18 (2017-2022) consecutive Contegra monocusp implantations were included. Median age and weight were 3.65 [2.00; 9.43] months and 6.12 [4.30; 8.22] kg, respectively. Nine of 18 patients had undergone palliation. Native pulmonary leaflet tissue was recruited to create a single posterior cusp. Contegra monocusp selection was based on the goal to achieve a neoannulus of Z value ≈ 0. Monocusp sizes implanted were 16 [14; 18] mm. Patch plasty of left pulmonary artery (LPA) (9), right pulmonary artery (RPA) (2), and both LPA-RPA (5) were often performed. Results: All patients survived the operation and were discharged home in good health. Median ventilation time and hospital stay were 2 [1; 9] and 12.5 [9; 54] days, respectively. Follow-up duration was 30.68 [3.47; 60.47] months and 100% complete. One patient with well-corrected right ventricular outflow tract died 9.4 months postoperatively, possibly of aspiration. One child with membranous pulmonary atresia needed reoperation (conduit insertion) at 3.5 months of follow-up. Five needed catheter interventions: supravalvar stent (2), LPA stent (3), and RPA stent (1), most of them in the earlier half of the experience. Pulmonary annulus changed from preoperative -3.91 [-5.98; -2.23] to -0.10 [-1.44; 1.92] at discharge; growing proportionally to -0.13 [-3.52; 2.73] at follow-up. Kaplan-Meier freedom from composite dysfunction was 79.25 (95% confidence interval, +13.68%, -31.44%) at 36 months. Conclusions: Recruitment of native leaflets, optimal Contegra monocusp, and commissuroplasty provide an easily replicable technique for achieving a competent, proportionally growing neopulmonary valve. Longer follow-up is needed to determine its impact on delaying a pulmonary valve replacement.

Favourable Short- to Mid-Term Outcome after PDA-Stenting in Duct-Dependent Pulmonary Circulation.

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 prospective studies.

Findings and Outcome of Transcatheter Right Ventricular Endomyocardial Biopsy and Hemodynamic Assessment in Children with Suspected Myocarditis or Cardiomyopathy.

OBJECTIVE: The study objective is assessing findings and outcome in children with suspected cardiomyopathy (CMP) or myocarditis undergoing cardiac catheterization with transcatheter right ventricular endomyocardial biopsy (RV-EMB). METHODS: All consecutive children undergoing cardiac catheterization with RV-EMB for suspected CMP/myocarditis between 2002-2021 were analysed regarding clinical presentation, cardiac biomarkers, periprocedural management, hemodynamic, histological/immunohistological findings, and outcome. RESULTS: Eighty-five RV-EMBs were performed in 81 patients at a median age of 6.8 (IQR 9.9) years and a bodyweight of 20 (32.2) kg. Histological/immunohistological findings of RV-EMB revealed dilated CMP in 10 (12%), chronic myocarditis in 28 (33%), healing myocarditis in 5 (6%), acute myocarditis in 9 (11%), other heart muscle diseases in 23 (27%) (7 restrictive CMP, 5 hypertrophic CMP, 4 toxic/anthracycline-induced CMP, 4 endocardfibroelastosis, 1 arrhythmogenic right ventricular CMP, 1 laminin CMP, 1 haemangioma), no conclusive histology in 7 (8%), and normal histology in 3 (4%) patients. Median LVEDP was 17 mmHg (IQR 9), LAP 15 mmHg (10), and PVR 1.83 (1.87) Wood Units/m2. There were 3 major complications (3%), all patients recovered without any sequelae. At follow-up (median 1153, IQR 1799 days) 47 (59%) patients were alive, 11 (13%) dead, 15 (18%) underwent cardiac transplantation, and 8 (9%) were lost to follow-up. Death/cardiac transplantation occurred within 3 years from RV-EMB. All patients with an acute myocarditis survived. NT-pro-BNP, echo parameters, and invasive hemodynamics correlate independently with death/cardiac transplant. CONCLUSION: Hemodynamic invasive data and morphological findings in RV-EMB complete clinical diagnosis in children with suspected CMP/myocarditis and provide important information for further clinical management.

Malposition of feeding tubes in neonates: is it an issue?

OBJECTIVES: Accurate tube placement of orogastric and nasogastric feeding tubes in neonates is important to ensure safe and effective enteral feeding. Errors in placement and position of feeding tubes are described in literature, but there is little evidence of the exact prevalence of improperly placed tubes, especially in neonates. MATERIALS AND METHODS: To evaluate the prevalence of improperly placed feeding tubes, we reviewed 381 consecutive radiographs and defined the position of the feeding tubes. RESULTS: We found that in 41% of the placements, the optimal position of the feeding tubes was achieved, whereas overall 59% of the feeding tubes had been placed incorrectly. CONCLUSIONS: There is a need for both better rules to measure the distance between nose (lips) and the body of the stomach and improved methods to confirm correct tube position in neonates.

Single Ventricle Physiology Patients and Coagulation Abnormalities: Is There a Relationship With Hemodynamic Data and Postoperative Course? A Pilot Study.

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.

Approaches to Establish Extracardiac Total Cavopulmonary Connections in Animal Models-A Review.

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.

First-in-man pulmonary artery stenting in children using the Bentley® BeGrow™ stent system for newborns and infants.

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 cobalt­chromium, 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.