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.

Parental experience of the neuromotor development of children with congenital heart disease: an exploratory qualitative study.

BACKGROUND: Children with severe congenital heart disease (CHD) are a group of children at risk for neurodevelopmental impairments. Motor development is the first domain to show a delay during the first year of life and may significantly contribute to parental concerns, stress, and difficulties in early child-parent attachment. Thus, the aim of the study was to better understand the wishes and concerns of parents of children with CHD and explore their experience of their children's neuromotor development in the first year of life. METHODS: In this qualitative study, fourteen families were recruited. Their children were aged 1-3 years and had undergone open heart surgery within the first 6 months of life. Semi-structured interviews were audio-recorded and transcribed. The data was explored within an expert group, and a qualitative content analysis was conducted using VERBI MAXQDA software 2020. The study was conducted in accordance with the COREQ checklist. RESULTS: Parents of children with CHD reported several burdens and needs. Parental burdens concerned the child's motor development, their own physical and psychological strain, and difficulties in communication with healthcare professionals. The needs, parents reported included supporting their child's motor development, a medical coordinator, and better communication between healthcare professionals and parents. During the first phase of their children's illness, parents underwent a dynamic transitional phase and expressed the need to rely on themselves, to trust their children's abilities, and to regain self-determination in order to strengthen their self-confidence. CONCLUSIONS: It is essential to involve parents of children with CHD at an early stage of decision-making. Parents are experts in their children and appreciate medical information provided by healthcare professionals. Interprofessional teamwork, partnering with parents, and continuous support are crucial to providing the best possible care for children and their families. Family-centred early motor intervention for CHD children might counteract the effect of parental overprotection and improve children's motor development and thus strengthen child-parent interaction. In future work, we aim to evaluate a family-centred early motor intervention for children with CHD developed on the basis of this qualitative study. TRIAL REGISTRATION: Not applicable.

Glial Fibrillary Acid Protein and Cerebral Oxygenation in Neonates Undergoing Cardiac Surgery.

BACKGROUND: Neonates undergoing surgery for complex congenital heart disease are at risk of developmental impairment. Hypoxic-ischemic brain injury might be a contributing factor. We aimed to investigate the perioperative release of the astrocyte cytoskeleton component glial fibrillary acid protein and its relation to cerebral oxygenation. METHODS: Serum glial fibrillary acid protein levels were measured before and 0, 12, 24, and 48 hours after surgery. Reference values were based on preoperative samples; concentrations above the 95th percentile were defined as elevated. Cerebral oxygenation was derived by near-infrared spectroscopy. RESULTS: Thirty-six neonates undergoing 38 surgeries utilizing cardiopulmonary bypass were enrolled (complete data available for 35 procedures). Glial fibrillary acid protein was elevated after 18 surgeries (arterial switch: 7/12; Norwood: 5/15; others: 6/8; p = 0.144). Age at surgery was higher in cases with elevated serum levels (6 [4-7] vs. 4 [2-5] days, p = 0.009) and intraoperative cerebral oxygen saturation was lower (70 ± 10% vs. 77 ± 7%, p = 0.029). In cases with elevated postoperative glial fibrillary acid protein, preoperative cerebral oxygen saturation was lower for neonates undergoing the arterial switch operation (55 ± 9% vs. 64 ± 4%, p = 0.048) and age at surgery was higher for neonates with a Norwood procedure (7 [6-8] vs. 5 [4-6] days, p = 0.028). CONCLUSIONS: Glial fibrillary acid protein was elevated after ∼50% of neonatal cardiac surgeries and was related to cerebral oxygenation and older age at surgery. The potential value as a biomarker for cerebral injury after neonatal cardiac surgery warrants further investigation; in particular, the association with neurodevelopmental outcome needs to be determined.

Longitudinal Deformation of the Right Ventricle in Hypoplastic Left Heart Syndrome: A Comparative Study of 2D-Feature Tracking Magnetic Resonance Imaging and 2D-Speckle Tracking Echocardiography.

In hypoplastic left heart syndrome (HLHS), long-term outcome is closely related to right ventricular function. Echocardiography and magnetic resonance imaging (MRI) are routinely used for functional assessment. MRI 2D-tissue feature tracking (2D-FT) allows quantification of myocardial deformation but has not yet been applied to HLHS patients. We sought to investigate the feasibility of this technique and to compare the results to 2D-speckle tracking echocardiography (2D-STE). In routine MRI 2D anatomical four chamber view, cine images were recorded in 55 HLHS patients (median age 4.9 years [1.6, 17.0]). Regional and global peak systolic longitudinal strain (LS) and strain rate (LSR) were determined using 2D-FT software. Echocardiographic four chamber view was analyzed with 2D-STE. Visualization of all myocardial segments with MRI was excellent, regional, and global LS and LSR could be assessed in all data sets. In 2D-STE, 28% of apical segments could not be analyzed due to poor image quality. Agreement of 2D-FT MRI and 2D-STE was acceptable for global LS, but poor for global LSR. In MRI, regional LS was lower in the septal segments, while LSR was not different between the segments. GLS and GLSR correlated with ejection fraction (GLS: r = - 0.45 and r < 0.001, GLSR: r = - 0.34 and p = 0.01). With new post-processing options, the assessment of regional and global LS and LSR is feasible in routine MRI of HLHS patients. For LS, results were comparable with 2D-STE. The agreement was poor for LSR, which might relate to differences in temporal resolution between the two imaging modalities.

Decline of Systolic and Diastolic 2D Strain Rate During Follow-Up of HLHS Patients After Fontan Palliation.

Accurate assessment of ventricular function is particularly important in children with hypoplastic left heart syndrome (HLHS) after completion of the total cavopulmonary connection (TCPC). For this purpose, two-dimensional speckle tracking (2DST) is a promising technique as it does not depend on the angle of insonation or the geometry of the ventricle. The objective of this study was to assess changes in systolic and diastolic right ventricular (RV) function within a 5-year follow-up period of HLHS patients after TCPC using conventional and 2DST echocardiography. RV fractional area change (RVFAC), tricuspid annular plane systolic excursion (TAPSE), E/A, E/e' and 2DST parameters [global longitudinal peak systolic strain (GS) and strain rate (GSRs), global strain rate in early (GSRe) and late (GSRa) diastole] of 40 HLHS patients were compared at 1.6 and at 5.1 years after TCPC. RVFAC, E/A, E/e' and GS did not change, whereas TAPSE (13.7 ± 3.2 vs. 10.5 ± 2.4 mm/m(2), p < 0.001), GSRs (-1.56 ± 0.28 vs. -1.35 ± 0.31 1/s, p < 0.001), GSRe (2.22 ± 0.49 vs. 1.96 ± 0.44 1/s, p = 0.004) and GSRa (1.19 ± 0.39 vs. 0.92 ± 0.39 1/s, p < 0.001) decreased significantly. Systolic and diastolic RV function parameters of HLHS patients decreased from 1.6 to 5.1 years after TCPC in our patients. Changes in global strain rate parameters may be signaling early RV dysfunction that is not detectable by traditional echocardiography. Further study is needed to verify this and to determine whether these changes are clinically relevant.

Pulmonary valve reconstruction for acquired pulmonary regurgitation in patients with treated congenital heart disease.

OBJECTIVES: Pulmonary valve regurgitation is a common problem after relief of right ventricular outflow tract (RVOT) obstruction with a transannular patch. Pulmonary valve replacement with a homograft or xenograft is the routine treatment. Longevity of biological valves and the availability of homografts are limited. Alternatives to restore RVOT competence are evaluated. The goal of this study was to present intermediate-term results for pulmonary valve reconstruction (PVr) in patients with severe regurgitation. METHODS: PVr was performed in 24 patients (August 2006‒July 2018). We analysed perioperative data, pre- and postoperative cardiac magnetic resonance (CMR) imaging studies, freedom from valve replacement and risk factors for pulmonary valve dysfunction. RESULTS: The underlying diagnoses were tetralogy of Fallot (n = 18, 75%), pulmonary stenosis (n = 5, 20.8%) and the double outlet right ventricle post banding procedure (n = 1, 4.2%). The median age was 21.5 (14.8-23.7) years. Main (n = 9, 37.5%) and branch pulmonary artery procedures (n = 6, 25%) and surgery of the RVOT (n = 16, 30.2%) were often part of the reconstruction. The median follow-up after the operation was 8.0 (4.7-9.7) years. Freedom from valve failure was 96% at 2 and 90% at 5 years. The mean longevity of the reconstructive surgery was 9.9 years (95% confidence interval: 8.8-11.1 years). CMR before and 6 months after surgery showed a reduction in the regurgitation fraction [41% (33-55) vs 20% (18-27) P = 0.00] and of the indexed right ventricular end-diastolic volume [156 ml/m2 (149-175) vs 116 ml/m2 (100-143), P = 0.004]. Peak velocity across the pulmonary valve (determined by CMR) half a year after surgery was 2.0, unchanged. CONCLUSIONS: PVr can be achieved with acceptable intermediate-term results and may delay 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.

Myocardial deformation in patients with a single left ventricle using 2D cardiovascular magnetic resonance feature tracking: a case-control study.

Ventricular dysfunction is a well-known complication in single ventricle patients in Fontan circulation. As studies exclusively examining patients with a single left ventricle (SLV) are sparse, we assessed left ventricular (LV) function in SLV patients by using 2D-cardiovascular magnetic resonance (CMR) feature tracking (2D-CMR-FT) and 2D-speckle tracking echocardiography (2D-STE). 54 SLV patients (11.4, 3.1-38.1 years) and 35 age-matched controls (12.3, 6.3-25.8 years) were included. LV global longitudinal, circumferential and radial strain (GLS, GCS, GRS) and strain rate (GLSR, GCSR, GRSR) were measured using 2D-CMR-FT. LV volumes, ejection fraction (LVEF) and mass were determined from short axis images. 2D-STE was applied in patients to measure peak systolic GLS and GLSR. In a subgroup analysis, we compared double inlet left ventricle (DILV) with tricuspid atresia (TA) patients. The population consisted of 19 DILV patients, 24 TA patients and 11 patients with diverse diagnoses. 52 patients were in NYHA class I and 2 patients were in class II. Most SLV patients had a normal systolic function but median LVEF in patients was lower compared to controls (55.6% vs. 61.2%, p = 0.0001). 2D-CMR-FT demonstrated reduced GLS, GCS and GCSR values in patients compared to controls. LVEF correlated with GS values in patients (p < 0.05). There was no significant difference between GLS values from 2D-CMR-FT and 2D-STE in the patient group. LVEF, LV volumes, GS and GSR (from 2D-CMR-FT) were not significantly different between DILV and TA patients. Although most SLV patients had a preserved EF derived by CMR, our results suggest that, LV deformation and function may behave differently in SLV patients compared to healthy subjects.

Right ventricular outflow tract reconstruction with the Labcor® stentless valved pulmonary conduit.

OBJECTIVES: The right ventricular outflow tract reconstruction is a common necessity in congenital cardiac surgery. As homograft availability is limited, alternatives need to be evaluated. The Labcor® conduit consists of a porcine tricomposite valve assembled inside a bovine pericardium tube. This study presents intermediate-term results for its utilization for right ventricular outflow tract reconstruction. METHODS: Labcor conduits were implanted in 53 patients (February 2009-July 2016). We analysed perioperative data, freedom from conduit failure and risk factors for conduit dysfunction. RESULTS: The most common diagnosis was Tetralogy of Fallot (n = 20, 37.7%). The median age at surgery was 10.0 [interquartile range (IQR) 4.9-14.3] years. Pulmonary artery plasty (n = 37, 69.8%) and augmentation of the right ventricular outflow tract (n = 16, 30.2%) were often part of the procedure. The median conduit size was 21 (range 11-25) mm. There was no in-hospital death. The median follow-up after surgery was 4.6 (IQR 3.4-5.6) years. Fourteen patients (27.5%) developed conduit failure with stenosis being the main cause. Freedom from conduit failure was 98.0% at 2 and 80.5% at 5 years. The median longevity of the conduit was 7.4 years (95% confidence interval 5.1-9.8 years). Younger age and smaller conduit size were related to conduit failure. CONCLUSIONS: Utilization of the Labcor conduit revealed acceptable intermediate-term results. The conduit appeared to be functioning sufficiently well within the first 5 years in the majority of patients. The higher rate of failure concerning smaller conduits might be associated with somatic outgrowth; however, conduit degeneration as common and long-term outcome still needs to be evaluated.

S100B and its relation to cerebral oxygenation in neonates and infants undergoing surgery for congenital heart disease.

OBJECTIVES: Neonates and infants undergoing surgery for congenital heart disease are at risk for developmental impairment. Hypoxic-ischemic brain injury might be one contributing factor. We aimed to investigate the perioperative release of the astrocyte protein S100B and its relation to cerebral oxygenation. METHODS: Serum S100B was measured before and 0, 12, 24, and 48 hours after surgery. Cerebral oxygen saturation was derived by near-infrared spectroscopy. S100B reference values based on preoperative samples; concentrations above the 75th percentile were defined as elevated. Patients with elevated S100B at 24 or 48 hours were compared to cases with S100B in the normal range. Neonates (≤28 days) and infants (>28 and ≤365 days) were analyzed separately due to age-dependent release of S100B. RESULTS: Seventy-four patients underwent 94 surgical procedures (neonates, n = 38; infants, n = 56). S100B concentrations were higher in neonates before and after surgery at all time points (P ≤ .015). Highest values were noticed immediately after surgery. Postoperative S100B was elevated after 15 (40.5%) surgeries in neonates. There was no difference in pre-, intra-, or postoperative cerebral oxygenation. In infants, postoperative S100B was elevated after 23 (41.8%) procedures. Preoperative cerebral oxygen saturations tended to be lower (53 ± 12% vs 59 ± 12%, P = .069) and arterial-cerebral oxygen saturation difference was higher (35 ± 11% vs 28 ± 11%, P = .018) in infants with elevated postoperative S100B. In the early postoperative course, cerebral oxygen saturation was lower (54 ± 13% vs 63 ± 12%, P = .011) and arterial-cerebral oxygen saturation difference was wider (38 ± 11% vs 30 ± 10%, P = .008). Cerebral oxygen saturation was also lower for the entire postoperative course (62 ± 18% vs 67 ± 9%, P = .047). CONCLUSIONS: Postoperative S100B was elevated in about 40% of neonates and infants undergoing cardiac surgery. Infants with elevated postoperative S100B had impaired perioperative cerebral tissue oxygenation. No relation between S100B and cerebral oxygenation could be demonstrated in neonates.

Neuro-developmental outcome in single-ventricle patients: is the Norwood procedure a risk factor?

OBJECTIVES: Complex neonatal surgery is considered a risk factor for neuro-developmental impairment in single-ventricle patients. Neuro-developmental outcome was compared between preschool-aged Fontan patients who underwent a Norwood procedure and single-ventricle patients not requiring neonatal surgery with cardiopulmonary bypass. METHODS: Verbal, performance and full-scale intelligence quotient (IQ) were evaluated with the Wechsler Preschool and Primary Scale of Intelligence. Cognitive functions were assessed with the German 'Kognitiver Entwicklungstest für das Kindergartenalter' (KET-KID). Risk factors for impaired neuro-development were evaluated. RESULTS: Neuro-developmental assessment was completed in 95 patients (Norwood: n = 69; non-Norwood: n = 26). Median (interquartile range) IQ and KET-KID scores were in the normal range. Except for verbal KET-KID, scores did not differ between Norwood and non-Norwood patients (verbal IQ: 98 (86-105) vs 93 (85-102), P = 0.312; performance IQ: 91 (86-100) vs 96 (86-100), P = 0.932; full-scale IQ: 93 (86-101) vs 89 (84-98), P = 0.314; KET-KID verbal: 48 (17-72) vs 25 (2-54), P = 0.020; KET-KID non-verbal: 33 (18-62) vs 45 (15-54), P = 0.771; KET-KID global: 42 (14-65) vs 28 (6-63), P = 0.208). Full-scale IQ was below average (<85 points) in 14 (20%) Norwood and 9 (35%) non-Norwood cases (P = 0.181). Global KET-KID was below average (<16th percentile) in 19 (28%) and 10 (38%) patients (P = 0.326). Smaller head circumference z-score and complications before neonatal surgery were independently associated with lower scores. CONCLUSIONS: Neuro-developmental outcome of preschool-aged Fontan patients was in the normal range. The Norwood procedure was not a risk factor for neuro-developmental impairment. Preoperative condition and patient-related factors were more important determinants than variables related to surgical palliation.

Restrictive enlargement of the pulmonary annulus at repair of tetralogy of Fallot: a comparative 10-year follow-up study.

OBJECTIVES: Since 1996, our centre performs restrictive enlargement of the pulmonary annulus at surgical repair of tetralogy of Fallot. A transannular patch is only used if the z-score of the pulmonary annulus is smaller than -2. We sought to determine whether this strategy reduces pulmonary insufficiency (PI) and reoperation rate compared to a nationwide contemporary cohort that has not been operated using a uniform strategy. METHODS: Eighty-seven tetralogy of Fallot patients were included in the study (Group 1). One hundred sixty-seven tetralogy of Fallot patients from the Competence Network for Congenital Heart Disease served as controls (Group 2). Clinical, echocardiographic, electrocardiogram, cardiovascular magnetic resonance and outcome data were analysed. RESULTS: Follow-up time since repair was not different between groups [12.9 (7.8-18.8) vs 13.1 (5.2-16.9) years, P = 0.96] while transannular patch rate was significantly lower in Group 1 (32.2% vs 64.7%, P < 0.001). Ten-year freedom from reoperation for PI was significantly higher in our cohort (98% vs 92%, P = 0.01). Multivariable analysis identified restrictive enlargement as the only predictor for no need of reoperation [hazard ratio 0.4 (95% confidence interval 0.24-0.84), P < 0.01]. In Group 1, cardiovascular magnetic resonance-derived volumes were smaller (indexed end-diastolic volume: 103 ± 24 vs 123 ± 31 ml/m2, P < 0.001; indexed end-systolic volume: 53 ± 19 vs 59 ± 20 ml/m2, P = 0.04) and regurgitation fraction was lower (21 ± 14 vs 31 ± 17%, P < 0.001). CONCLUSIONS: Restrictive enlargement of the pulmonary annulus reduces PI and limits the amount of right ventricular dilatation at intermediate-term follow-up. The need for reoperation to alleviate PI is significantly lower compared to a contemporary cohort. Restrictive enlargement strategy has a stronger influence on freedom from reoperation than the use of a transannular patch. CLINICAL TRIAL REGISTRATION: WHO Main ID: DRKS00010087. URL: http://apps.who.int/trialsearch/Trial2.aspx?TrialID=DRKS00010087.

Neurodevelopmental outcome in hypoplastic left heart syndrome: Impact of perioperative cerebral tissue oxygenation of the Norwood procedure.

OBJECTIVES: Patients with hypoplastic left heart syndrome are at risk for neurodevelopmental impairment. Hypoxic-ischemic brain injury during neonatal treatment might be a relevant cause. We evaluated the association between cerebral oxygenation in the perioperative course of the Norwood procedure and neurodevelopmental outcome. METHODS: Cerebral tissue oxygen saturation (ScO2) was obtained by near-infrared spectroscopy for 24 hours before and 48 hours after surgery in 43 patients. Full-scale, verbal, and performance IQ scores were evaluated with the Wechsler Preschool and Primary Scale of Intelligence at a median of 4.5 years (range, 3.5-6.8 years). Cognitive functions were assessed with the German Kognitiver Entwicklungstest für das Kindergartenalter (KET-KID). RESULTS: Mean IQ scores and KET-KID percentile ranks were in the lower-normal range (full-scale IQ, 94 ± 11; verbal IQ, 97 ± 13; performance IQ, 93 ± 9; KET-KID global, 42 ± 27; verbal, 48 ± 29; nonverbal, 37 ± 23). Scores were below average (full scale IQ <85 or KET-KID <16th percentile) in 12 cases. Mean preoperative ScO2 was lower in patients scoring below average (56.8% ± 7.1% vs 61.7% ± 5.8%; P = .028) and was correlated with full-scale IQ (r = 0.495; P = .001), verbal IQ (r = 0.524; P = .001), and performance IQ (r = 0.386; P = .012) scores, and with global (r = 0.360; P = .018) and verbal (r = 0.395, P = .009) KET-KID scores. A relationship between IQ or KET-KID scores and postoperative ScO2 was not found. Gestational age, head circumference z-score, age at surgery, and postoperative length of stay were associated with IQ and KET-KID scores. CONCLUSIONS: Neurodevelopmental outcome was in the lower-normal range. Along with innate patient factors, preoperative cerebral tissue oxygenation is likely an important determinant of cognitive development.