Publications by authors named "Nico A Blom"

173 Publications

Patient information portal for congenital aortic and pulmonary valve disease: a stepped-wedge cluster randomised trial.

Open Heart 2021 Mar;8(1)

Department of Cardiothoracic Surgery, Erasmus University Medical Center, Rotterdam, The Netherlands.

Background: In response to an increased need for patient information in congenital heart disease, we previously developed an online, evidence-based information portal for patients with congenital aortic and pulmonary valve disease. To assess its effectiveness, a stepped-wedge cluster randomised trial was conducted.

Methods: Adult patients and caregivers of paediatric patients with congenital aortic and/or pulmonary valve disease and/or tetralogy of Fallot who visited the outpatient clinic at any of the four participating centres in the Netherlands between 1 March 2016-1 July 2017 were prospectively included. The intervention (information portal) was introduced in the outpatient clinic according to a stepped-wedge randomised design. One month after outpatient clinic visit, each participant completed a questionnaire on disease-specific knowledge, anxiety, depression, mental quality of life, involvement and opinion/attitude concerning patient information and involvement.

Results: 343 participants were included (221 control, 122 intervention). Cardiac diagnosis (p=0.873), educational level (p=0.153) and sex (p=0.603) were comparable between the two groups. All outcomes were comparable between groups in the intention-to-treat analyses. However, only 51.6% of subjects in the intervention group (n=63) reported actually visiting the portal. Among these subjects (as-treated), disease-specific knowledge (p=0.041) and mental health (p=0.039) were significantly better than in control subjects, while other baseline and outcome variables were comparable.

Conclusion: Even after being invited by their cardiologists, only half of the participants actually visited the information portal. Only in those participants that actually visited the portal, knowledge of disease and mental health were significantly better. This underlines the importance of effective implementation of online evidence-based patient information portals in clinical practice.
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http://dx.doi.org/10.1136/openhrt-2020-001252DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7993296PMC
March 2021

Hemodynamic interplay of vorticity, viscous energy loss, and kinetic energy from 4D Flow MRI and link to cardiac function in healthy subjects and Fontan patients.

Am J Physiol Heart Circ Physiol 2021 04 26;320(4):H1687-H1698. Epub 2021 Feb 26.

Department of Radiology, Feinberg School of Medicine, Northwestern University, Chicago, Illinois.

The purpose of this study was to directly assess (patho)physiology of intraventricular hemodynamic interplay between four-dimensional flow cardiovascular magnetic resonance imaging (4D Flow MRI)-derived vorticity with kinetic energy (KE) and viscous energy loss (EL) over the cardiac cycle and their association to ejection fraction (EF) and stroke volume (SV). Fifteen healthy subjects and thirty Fontan patients underwent whole heart 4D Flow MRI. Ventricular vorticity, KE, and EL were computed over systole (vorticity_vol, KE, and EL) and diastole (vorticity_vol, KE, and EL). The association between vorticity_vol and KE and EL was tested by Spearman correlation. Fontan patients were grouped to normal and impaired EF groups. A significant correlation was found between SV and vorticity in healthy subjects (systolic: ρ = 0.84, < 0.001; diastolic: ρ = 0.81, < 0.001) and in Fontan patients (systolic: ρ = 0.61, < 0.001; diastolic: ρ = 0.54, = 0.002). Healthy subjects showed positive correlation between vorticity_vol versus KE (systole: ρ = 0.96, < 0.001; diastole: ρ = 0.90, < 0.001) and EL (systole: ρ = 0.85, < 0.001; diastole: ρ = 0.84, < 0.001). Fontan patients showed significantly elevated vorticity_vol compared with healthy subjects (vorticity_vol: 3.1 [2.3-3.9] vs. 1.7 [1.3-2.4] L/s, < 0.001; vorticity_vol: 3.1 [2.0-3.7] vs. 2.1 [1.6-2.8] L/s, = 0.002). This elevated vorticity in Fontan patients showed strong association with KE (systole: ρ = 0.91, < 0.001; diastole: ρ = 0.85, < 0.001) and EL (systole: ρ = 0.82, < 0.001; diastole: ρ = 0.89, < 0.001). Fontan patients with normal EF showed significantly higher vorticity_vol and EL, but significantly decreased KE , in the presence of normal SV, compared with healthy subjects. Healthy subjects show strong physiological hemodynamic interplay between vorticity with KE and EL. Fontan patients demonstrate a pathophysiological hemodynamic interplay characterized by correlation of elevated vorticity with KE and EL in the presence of maintained normal stroke volume. Altered vorticity and energetic hemodynamics are found in the presence of normal EF in Fontan patients. Physiologic intraventricular hemodynamic interplay/coupling is present in the healthy left ventricle between vorticity versus viscous energy loss and kinetic energy from four-dimensional flow cardiovascular magnetic resonance imaging (4D Flow MRI). Conversely, Fontan patients present compensatory pathophysiologic hemodynamic coupling by an increase in intraventricular vorticity that positively correlates to viscous energy loss and kinetic energy levels in the presence of maintained normal stroke volume. Altered vorticity and energetics are found in the presence of normal ejection fraction in Fontan patients.
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http://dx.doi.org/10.1152/ajpheart.00806.2020DOI Listing
April 2021

Associations Between Blood Biomarkers, Cardiac Function, and Adverse Outcome in a Young Fontan Cohort.

J Am Heart Assoc 2021 Feb 24;10(5):e015022. Epub 2021 Feb 24.

Division of Pediatric Cardiology Department of Pediatrics Erasmus University Medical Center Rotterdam The Netherlands.

Background Patients who have undergone the Fontan procedure are at high risk of circulatory failure. In an exploratory analysis we aimed to determine the prognostic value of blood biomarkers in a young cohort who have undergone the Fontan procedure. Methods and Results In multicenter prospective studies patients who have undergone the Fontan procedure underwent blood sampling, cardiopulmonary exercise testing, and stress cardiac magnetic resonance imaging. Several biomarkers including NT-proBNP (N-terminal pro-B-type natriuretic peptide), GDF-15 (growth differentiation factor 15), Gal-3 (galectin-3), ST2 (suppression of tumorigenicity 2), DLK-1 (protein delta homolog 1), FABP-4 (fatty acid-binding protein 4), IGFBP-1 (insulin-like growth factor-binding protein 1), IGFBP-7, MMP-2 (matrix metalloproteinase 2), and vWF (von Willebrand factor) were assessed in blood at 9.6 (7.1-12.1) years after Fontan completion. After this baseline study measurement, follow-up information was collected on the incidence of adverse cardiac events, including cardiac death, out of hospital cardiac arrest, heart transplantation (listing), cardiac reintervention (severe events), hospitalization, and cardioversion/ablation for arrhythmias was collected and the relation with blood biomarkers was assessed by Cox proportional hazard analyses. The correlation between biomarkers and other clinical parameters was evaluated. We included 133 patients who have undergone the Fontan procedure, median age 13.2 (25th, 75th percentile 10.4-15.9) years, median age at Fontan 3.2 (2.5-3.9) years. After a median follow-up of 6.2 (4.9-6.9) years, 36 (27.1%) patients experienced an event of whom 13 (9.8%) had a severe event. NT-proBNP was associated with (all) events during follow-up and remained predictive after correction for age, sex, and dominant ventricle (hazard ratio, 1.89; CI, 1.32-2.68). The severe event-free survival was better in patients with low levels of GDF-15 (=0.005) and vWF (=0.008) and high levels of DLK-1 (=0.041). There was a positive correlation (β=0.33, =0.003) between DLK-1 and stress cardiac magnetic resonance imaging functional reserve. Conclusions NT-proBNP, GDF-15, vWF, DLK-1, ST-2 FABP-4, and IGFBP-7 levels relate to long-term outcome in young patients who have undergone the Fontan procedure.
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http://dx.doi.org/10.1161/JAHA.119.015022DOI Listing
February 2021

Oxygen Uptake Efficiency Slope is Strongly Correlated to VO Long-Term After Arterial Switch Operation.

Pediatr Cardiol 2021 Feb 1. Epub 2021 Feb 1.

Division of Paediatric Cardiology, Department of Paediatrics, Leiden University Medical Center, Leiden, The Netherlands.

After the arterial switch operation (ASO) for transposition of the great arteries (TGA), many patients have an impaired exercise tolerance. Exercise tolerance is determined with cardiopulmonary exercise testing by peak oxygen uptake (VO). Unlike VO, the oxygen uptake efficiency slope (OUES) does not require a maximal effort for interpretation. The value of OUES has not been assessed in a large group of patients after ASO. The purpose of this study was to determine OUES and VO, evaluate its interrelationship and assess whether exercise tolerance is related to ventricular function after ASO. A cardiopulmonary exercise testing, assessment of physical activity score and transthoracic echocardiography (fractional shortening and left/right ventricular global longitudinal peak strain) were performed to 48 patients after ASO. Median age at follow-up after ASO was 16.0 (IQR 13.0-18.0) years. Shortening fraction was normal (36 ± 6%). Left and right global longitudinal peak strain were reduced: 15.1 ± 2.4% and 19.5 ± 4.5%. This group of patients showed lower values for all cardiopulmonary exercise testing parameters compared to the reference values: mean VO% 75% (95% CI 72-77) and mean OUES% 82(95% CI 77-87); without significant differences between subtypes of TGA. A strong-to-excellent correlation between the VO and OUES was found (absolute values: R = 0.90, p < 0.001; normalized values: R = 0.79, p < 0.001). No correlation was found between cardiopulmonary exercise testing results and left ventricle function parameters. In conclusion, OUES and VO were lower in patients after ASO compared to reference values but are strongly correlated, making OUES a valuable tool to use in this patient group when maximal effort is not achievable.
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http://dx.doi.org/10.1007/s00246-021-02554-9DOI Listing
February 2021

The Efficacy of Anti-Arrhythmic Drugs in Children With Idiopathic Frequent Symptomatic or Asymptomatic Premature Ventricular Complexes With or Without Asymptomatic Ventricular Tachycardia: a Retrospective Multi-Center Study.

Pediatr Cardiol 2021 Jan 30. Epub 2021 Jan 30.

Willem-Alexander Children's Hospital-Leiden University Medical Center, Albinusdreef 2, P.O. Box 9600, Leiden, the Netherlands.

The aim of the study is to compare the efficacy of flecainide, beta-blockers, sotalol, and verapamil in children with frequent PVCs, with or without asymptomatic VT. Frequent premature ventricular complexes (PVCs) and asymptomatic ventricular tachycardia (VT) in children with structurally normal hearts require anti-arrhythmic drug (AAD) therapy depending on the severity of symptoms or ventricular dysfunction; however, data on efficacy in children are scarce. Both symptomatic and asymptomatic children (≥ 1 year and < 18 years of age) with a PVC burden of 5% or more, with or without asymptomatic runs of VT, who had consecutive Holter recordings, were included in this retrospective multi-center study. The groups of patients receiving AAD therapy were compared to an untreated control group. A medication episode was defined as a timeframe in which the highest dosage at a fixed level of a single drug was used in a patient. A total of 35 children and 46 medication episodes were included, with an overall change in PVC burden on Holter of -4.4 percentage points, compared to -4.2 in the control group of 14 patients. The mean reduction in PVC burden was only significant in patients receiving flecainide (- 13.8 percentage points; N = 10; p = 0.032), compared to the control group and other groups receiving beta-blockers (- 1.7 percentage points; N = 18), sotalol (+ 1.0 percentage points; N = 7), or verapamil (- 3.9 percentage points; N = 11). The efficacy of anti-arrhythmic drug therapy on frequent PVCs or asymptomatic VTs in children is very limited. Only flecainide appears to be effective in lowering the PVC burden.
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http://dx.doi.org/10.1007/s00246-021-02556-7DOI Listing
January 2021

The prognosis of common arterial trunk from a fetal perspective: A prenatal cohort study and systematic literature review.

Prenat Diagn 2021 Jan 22. Epub 2021 Jan 22.

Department of Obstetrics and Fetal Medicine, Leiden University Medical Center, Leiden, Netherlands.

Objective: The limited number of large fetal cohort studies on common arterial trunk (CAT) impedes prenatal counseling at midgestation. This study evaluates the prognosis of CAT from a fetal perspective.

Method: Fetuses with a prenatally diagnosed CAT were extracted from the PRECOR registry (2002-2016). We evaluated fetal and postnatal survival and the presence of additional morbidity at last follow-up. Literature databases were searches systematically for additional cases.

Results: Thirty-eight cases with a prenatal diagnosis of CAT were identified in our registry, of which 18/38 (47%) opted for pregnancy termination (TOP). Two cases resulted in spontaneous intrauterine demise (10%, 2/20), six cases demised postnatally (33%, 6/18), leaving 60% (12/20) alive, after exclusion of TOP, at a mean age of six (range: 2-10 years). Additional morbidity was found in 42% (5/12) of survivors, including 22q11.2 deletion syndrome, Adams-Oliver syndrome and intestinal atresia, whereas 8% (1/12) had developmental delay. The remaining 30% (6/12) of survivors appeared isolated with normal development. All of whom six required replacement of the initial right ventricle to pulmonary artery conduit. Additionally, we reviewed 197 literature cases on short-term outcome.

Conclusion: The risk of fetal and neonatal demise, as well as significant morbidity amongst survivors, should be included in prenatal counseling for CAT.
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http://dx.doi.org/10.1002/pd.5907DOI Listing
January 2021

An accessory pathway with automaticity and bidirectional conductive capacity.

Europace 2021 Mar;23(3):379

Department of Paediatric Cardiology, Willem-Alexander Children's Hospital, Leiden University Medical Center, Albinusdreef 2, 2333 ZA, Leiden, Netherlands.

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http://dx.doi.org/10.1093/europace/euaa379DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7947569PMC
March 2021

Wall shear stress in the thoracic aorta at rest and with dobutamine stress after arterial switch operation.

Eur J Cardiothorac Surg 2020 Dec 31. Epub 2020 Dec 31.

Department of Radiology, Leiden University Medical Center, Leiden, Netherlands.

Objectives: Progressive root dilatation is an important complication in patients with transposition of the great arteries (TGA) after arterial switch operation (ASO) that may be caused by altered flow dynamics. Aortic wall shear stress (WSS) distribution at rest and under dobutamine stress (DS) conditions using 4D flow magnetic resonance imaging were investigated in relation to thoracic aorta geometry.

Methods: 4D flow magnetic resonance imaging was performed in 16 adolescent TGA patients after ASO (rest and DS condition) and in 10 healthy controls (rest). The primary outcome measure was the WSS distribution along the aortic segments and the WSS change with DS in TGA patients. Based on the results, we secondary zoomed in on factors [aortic geometry and left ventricular (LV) function parameters] that might relate to these WSS distribution differences. Aortic diameters, arch angle, LV function parameters (stroke volume, LV ejection fraction, cardiac output) and peak systolic aortic WSS were obtained.

Results: TGA patients had significantly larger neoaortic root and smaller mid-ascending aorta (AAo) dimensions and aortic arch angle. At rest, patients had significantly higher WSS in the entire thoracic aorta, except for the dilated root. High WSS levels beyond the proximal AAo were associated with the diameter decrease from the root to the mid-AAo (correlation coefficient r = 0.54-0.59, P = 0.022-0.031), not associated with the aortic arch angle. During DS, WSS increased in all aortic segments (P < 0.001), most pronounced in the AAo segments. The increase in LV ejection fraction, stroke volume and cardiac output as a result of DS showed a moderate linear relationship with the WSS increase in the distal AAo (correlation coefficient r = 0.54-0.57, P = 0.002-0.038).

Conclusions: Increased aortic WSS was observed in TGA patients after ASO, related to the ASO-specific geometry, which increased with DS. Stress-enhanced elevated WSS may play a role in neoaortic root dilatation and anterior aortic wall thinning of the distal AAo.
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http://dx.doi.org/10.1093/ejcts/ezaa392DOI Listing
December 2020

Recommendations from the Association for European Paediatric and Congenital Cardiology for training in diagnostic and interventional electrophysiology.

Cardiol Young 2021 Jan 9;31(1):38-46. Epub 2020 Dec 9.

Gottsegen György Hungarian Institute of Cardiology, Hungarian Paediatric Heart Center, Budapest, Hungary.

The field of electrophysiology (EP) in paediatric cardiology patients and adults with congenital heart disease is complex and rapidly growing. The current recommendations for diagnostic and invasive electrophysiology of the working group for Cardiac Dysrhythmias and Electrophysiology of the Association for European Paediatric and Congenital Cardiology acknowledges the diveristy of European countries and centers. These training recommendations can be fulfilled in a manageable period of time, without compromising the quality of training required to become an expert in the field of paediatric and congenital EP and are for trainees undergoing or having completed accredited paediatric cardiologist fellowship. Three levels of expertise, the training for General paediatric cardiology EP, for non-invasive EP and invasive EP have been defined. This Association for European EP curriculum describes the theoretical and practicsal knowledge in clinical EP; catheter ablation, cardiac implantable electronic devices, inherited arrhythmias and arrhythmias in adults with congenital heart defects for the 3 levels of expertise.
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http://dx.doi.org/10.1017/S1047951120004096DOI Listing
January 2021

Neoaortic growth rate and diameter as risk factors for neoaortic valve regurgitation after arterial switch operation.

Heart 2020 Dec 5;106(24):1950. Epub 2020 Oct 5.

Division of Pediatric Cardiology, Department of Pediatrics, Leiden University Medical Center, Leiden, The Netherlands.

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http://dx.doi.org/10.1136/heartjnl-2020-318142DOI Listing
December 2020

In Children and Adolescents From Brugada Syndrome-Families, Only Mutation Carriers Develop a Type-1 ECG Pattern Induced By Fever.

Circulation 2020 Jul 6;142(1):89-91. Epub 2020 Jul 6.

Department of Pediatric Cardiology, Emma Children's Hospital (P.J.P, N.A.B., A.S.V., S.-A.B.C.), Amsterdam University Medical Center, location Academic Medical Center (AMC), The Netherlands.

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http://dx.doi.org/10.1161/CIRCULATIONAHA.120.045720DOI Listing
July 2020

A low incidence of preoperative neurosonographic abnormalities in neonates with heart defects.

Early Hum Dev 2020 09 1;148:105097. Epub 2020 Jun 1.

Leiden University Medical Center, Willem Alexander Children's Hospital, Department of Neonatology, Leiden, the Netherlands. Electronic address:

Background And Aim: To investigate whether neonates with prenatally detected congenital heart defects (CHD) demonstrate cerebral abnormalities on early preoperative cranial ultrasound (CUS), compared to healthy neonates, and to measure brain structures to assess brain growth and development in both groups.

Study Design, Subjects And Outcome Measures: Prospective cohort study with controls. Between September 2013 and May 2016 consecutive cases of prenatally detected severe isolated CHD were included. Neonatal CUS was performed shortly after birth, before surgery and in a healthy control group. Blinded images were reviewed for brain abnormalities and various measurements of intracranial structures were compared.

Results: CUS was performed in 59 healthy controls and 50 CHD cases. Physiological CUS variants were present in 54% of controls and in 52% of CHD cases. Abnormalities requiring additional monitoring (both significant and minor) were identified in four controls (7%) and five CHD neonates (10%). Significant abnormalities were only identified in four CHD neonates (8%) and never in controls. A separate analysis of an additional 8 CHD neonates after endovascular intervention demonstrated arterial stroke in two cases that underwent balloon atrioseptostomy (BAS). Cerebral measurements were smaller in CHD neonates, except for the cerebrospinal fluid measurements, which were similar to the controls.

Conclusions: The prevalence of significant preoperative CUS abnormalities in CHD cases was lower than previously reported, which may be partially caused by a guarding effect of a prenatal diagnosis. Arterial stroke occurred only in cases after BAS. As expected, neonates with CHD display slightly smaller head size and cerebral growth.
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http://dx.doi.org/10.1016/j.earlhumdev.2020.105097DOI Listing
September 2020

Treatment and outcome of plastic bronchitis in single ventricle patients: a systematic review.

Interact Cardiovasc Thorac Surg 2020 06;30(6):846-853

The Centre for Congenital Heart Disease Amsterdam-Leiden, Leiden, Netherlands.

Plastic bronchitis (PB) is a life-threatening complication in single ventricle (SV) patients of which the exact pathophysiology, outcome and optimal treatment are still unclear. This study aims to systematically review the literature to give insight into the characteristics, outcome and management options of SV patients with PB. A systematic review was conducted, using the electronic database PubMed to find records published up to August 2018, describing SV patients and PB in which characteristics, treatment and/or outcome were adequately described per case. A total of 577 records were screened of which 73 had sufficient data describing 133 SV cases with PB. Most cases had completed a Fontan palliation (n = 126) with a median interval between Fontan completion and diagnosis of PB of 18.4 months (Q1-Q3 5.0-36.3). Overall mortality was 15.2% and was associated with the diagnosis of PB within 12 months after Fontan palliation (5-year survival of 56.1% ≤12 months vs 94.8% >12 months, P = 0.002) and a higher age at Fontan completion (47.4 months for non-survivors vs 36.0 months for survivors, P = 0.015). Most patients received a combination therapy from 3 different treatment strategies, i.e. therapy for relief of airway obstruction, anti-inflammatory treatment and treatment to improve haemodynamics of the Fontan physiology (55.1%). In conclusion, SV patients who are diagnosed with PB within 12 months after Fontan palliation have a higher risk of mortality. Moreover, most cases received a combination therapy consisting of all 3 treatment strategies.
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http://dx.doi.org/10.1093/icvts/ivaa032DOI Listing
June 2020

Left and Right Ventricular Impairment Shortly After Correction of Tetralogy of Fallot.

Pediatr Cardiol 2020 Jun 4;41(5):1042-1050. Epub 2020 May 4.

Division of Paediatric Cardiology, Department of Paediatrics, Leiden University Medical Centre, Albinusdreef 2, 2333 ZA, Leiden, The Netherlands.

Surgical repair of Tetralogy of Fallot (ToF) is usually performed in the first months of life with low early postoperative mortality. During long-term follow-up, however, both right (RV) and left ventricular (LV) performances may deteriorate. Tissue Doppler imaging (TDI) and speckle tracking echocardiography (ST) can unmask a diminished RV and LV performance. The objective of the current study was to assess the cardiac performance before and shortly after corrective surgery in ToF patients using conventional, TDI and ST echocardiographic techniques. Thirty-six ToF patients after surgery were included. Transthoracic echocardiography including TDI and ST techniques was performed preoperatively and at hospital discharge after surgery (10 days to 4 weeks after surgery). Median age at surgery was 7.5 months [5.5-10.9]. Regarding the LV systolic function there was a significant decrease in interventricular septum (IVS) S' at discharge as compared to preoperatively (pre IVS S' = 5.4 ± 1.4; post IVS S' = 3.9 ± 1.2; p < 0.001) and in global longitudinal peak strain (GLS) (pre = - 18.3 ± 3.4; post = - 14.2 ± 4.1; p = 0.003); but not in the fractional shortening (FS). Both conventional and TDI parameters showed a decrease in diastolic function at discharge. Tricuspid Annular Plane Systolic Excursion and RV S' were significantly lower before discharge. When assessing the RV diastolic performance, only the TDI demonstrated a RV impairment. There was a negative correlation between age at surgery and postoperative LV GLS (R = - 0.41, p = 0.031). There seems to be an impairment in left and right ventricle performance at discharge after ToF corrective surgery compared to preoperatively. This is better determined with TDI and ST strain imaging than with conventional echocardiography.
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http://dx.doi.org/10.1007/s00246-020-02355-6DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7314721PMC
June 2020

The standardized 12-lead fetal electrocardiogram of the healthy fetus in mid-pregnancy: A cross-sectional study.

PLoS One 2020 30;15(4):e0232606. Epub 2020 Apr 30.

Department of Electrical Engineering, Eindhoven University of Technology, Eindhoven, The Netherlands.

Introduction: The examination of the fetal heart in mid-pregnancy is by ultrasound examination. The quality of the examination is highly dependent on the skill of the sonographer, fetal position and maternal body mass index. An additional tool that is less dependent on human experience and interpretation is desirable. The fetal electrocardiogram (ECG) could fulfill this purpose. We aimed to show the feasibility of recording a standardized fetal ECG in mid-pregnancy and explored its possibility to detect congenital heart disease (CHD).

Materials And Methods: Women older than 18 years of age with an uneventful pregnancy, carrying a healthy singleton fetus with a gestational age between 18 and 24 weeks were included. A fetal ECG was performed via electrodes on the maternal abdomen. After removal of interferences, a vectorcardiogram was constructed. Based on the ultrasound assessment of the fetal orientation, the vectorcardiogram was rotated to standardize for fetal orientation and converted into a 12-lead ECG. Median ECG waveforms for each lead were calculated.

Results: 328 fetal ECGs were recorded. 281 were available for analysis. The calculated median ECG waveform showed the electrical heart axis oriented to the right and inferiorly i.e. a negative QRS deflection in lead I and a positive deflection in lead aVF. The two CHD cases show ECG abnormalities when compared to the mean ECG of the healthy cohort.

Discussion: We have presented a method for estimating a standardized 12-lead fetal ECG. In mid-pregnancy, the median electrical heart axis is right inferiorly oriented in healthy fetuses. Future research should focus on fetuses with congenital heart disease.
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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0232606PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7192482PMC
July 2020

The prevalence of genetic diagnoses in fetuses with severe congenital heart defects.

Genet Med 2020 07 28;22(7):1206-1214. Epub 2020 Apr 28.

Department of Obstetrics and Fetal Medicine, Leiden University Medical Center, Leiden, Netherlands.

Purpose: Congenital heart defects (CHD) are associated with genetic syndromes. Rapid aneuploidy testing and chromosome microarray analysis (CMA) are standard care in fetal CHD. Many genetic syndromes remain undetected with these tests. This cohort study aims to estimate the frequency of causal genetic variants, in particular structural chromosome abnormalities and sequence variants, in fetuses with severe CHD at mid-gestation, to aid prenatal counselling.

Methods: Fetuses with severe CHD were extracted from the PRECOR registry (2012-2016). We evaluated pre- and postnatal genetic testing results retrospectively to estimate the frequency of genetic diagnoses in general, as well as for specific CHDs.

Results: 919 fetuses with severe CHD were identified. After exclusion of 211 cases with aneuploidy, a genetic diagnosis was found in 15.7% (111/708). These comprised copy number variants in 9.9% (70/708). In 4.5% (41/708) sequence variants were found that would have remained undetected with CMA. Interrupted aortic arch, pulmonary atresia with ventricular septal defect and atrioventricular septal defect were most commonly associated with a genetic diagnosis.

Conclusion: In case of normal CMA results, parents should be offered exome sequencing sequentially, if time allows for it, especially if the CHD is accompanied by other structural malformations due to the large variety in genetic syndromes.
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http://dx.doi.org/10.1038/s41436-020-0791-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7332415PMC
July 2020

Heart University: a new online educational forum in paediatric and adult congenital cardiac care. The future of virtual learning in a post-pandemic world?

Cardiol Young 2020 Apr 13;30(4):560-567. Epub 2020 Apr 13.

The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.

Online learning has become an increasingly expected and popular component for education of the modern-day adult learner, including the medical provider. In light of the recent coronavirus pandemic, there has never been more urgency to establish opportunities for supplemental online learning. Heart University aims to be "the go-to online resource" for e-learning in CHD and paediatric-acquired heart disease. It is a carefully curated open access library of paedagogical material for all providers of care to children and adults with CHD or children with acquired heart disease, whether a trainee or a practising provider. In this manuscript, we review the aims, development, current offerings and standing, and future goals of Heart University.
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http://dx.doi.org/10.1017/S1047951120000852DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7156582PMC
April 2020

Infectious pseudo-aneurysm of the left ventricle: a case report and a review of the literature.

BMC Cardiovasc Disord 2020 03 24;20(1):28. Epub 2020 Mar 24.

Department of Pediatric Cardiology, Leiden University Medical Center, Leiden, Netherlands.

Background: In the workup of a pediatric patient with pericarditis we found evidence of a pseudo-aneurysm of the left ventricle, which is a rare complication of purulent pericarditis.

Case Presentation: We present a case of a six-year-old girl who was diagnosed with pericarditis and a fistula between the pericardial and the intra-luminal space of the left ventricle of the heart. She was successfully treated with antibiotics and cardio-thoracic surgery. We found 23 published cases (21 with follow-up) of infectious pseudo-aneurysm of the heart, of which 19 underwent surgery, 5 had fatal outcome, and 2 who refused surgery survived. The majority of cases were associated with Staphylococcus aureus. The exact mechanisms of this rare complication remain unknown.

Conclusions: A pseudo-aneurysm of the left ventricle is a rare and not well understood complication of a purulent pericarditis most commonly caused by Staphylococcus aureus infection. Because of risk of rupture, surgical intervention is advised.
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http://dx.doi.org/10.1186/s12872-019-01299-xDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7092470PMC
March 2020

Does Repeated Measurement of a 6-Min Walk Test Contribute to Risk Prediction in Children with Dilated Cardiomyopathy?

Pediatr Cardiol 2020 Feb 12;41(2):223-229. Epub 2019 Nov 12.

Department of Pediatric Cardiology, Erasmus MC, University Medical Center Rotterdam, Rotterdam, The Netherlands.

A single 6-min walk test (6MWT) can be used to identify children with dilated cardiomyopathy (DCM) with a high risk of death or heart transplantation. To determine if repeated 6MWT has added value in addition to a single 6MWT in predicting death or heart transplantation in children with DCM. Prospective multicenter cohort study including ambulatory DCM patients ≥ 6 years. A 6MWT was performed 1 to 4 times per year. The distance walked was expressed as percentage of predicted (6MWD%). We compared the temporal evolution of 6MWD% in patients with and without the study endpoint (SE: all-cause death or heart transplantation), using a linear mixed effects model. In 57 patients, we obtained a median of 4 (IQR 2-6) 6MWTs per patient during a median of 3.0 years of observation (IQR 1.5-5.1). Fourteen patients reached a SE (3 deaths, 11 heart transplantations). At any time during follow-up, the average estimate of 6MWD% was significantly lower in patients with a SE compared to patients without a SE. In both patients groups, 6MWD% remained constant over time. An absolute 1% lower 6MWD% was associated with an 11% higher risk (hazard) of the SE (HR 0.90, 95% CI 0.86-0.95 p < 0.001). Children with DCM who died or underwent heart transplantation had systematically reduced 6MWD%. The performance of all patients was stable over time, so repeated measurement of 6MWT within this time frame had little added value over a single test.
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http://dx.doi.org/10.1007/s00246-019-02244-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7072046PMC
February 2020

Ventricular response to dobutamine stress cardiac magnetic resonance imaging is associated with adverse outcome during 8-year follow-up in patients with repaired Tetralogy of Fallot.

Eur Heart J Cardiovasc Imaging 2020 09;21(9):1039-1046

Division of Pediatric Cardiology, Department of Pediatrics, Erasmus University Medical Center, Doctor Molewaterplein 40, 3000 CM Rotterdam, The Netherlands.

Aims: The aim of this study was to evaluate the possible value of dobutamine stress cardiac magnetic resonance imaging (CMR) to predict adverse outcome in Tetralogy of Fallot (TOF) patients.

Methods And Results: In previous prospective multicentre studies, TOF patients underwent low-dose dobutamine stress CMR (7.5 µg/kg/min). Subsequently, during regular-care patient follow-up, patients were assessed for reaching the composite endpoint (cardiac death, arrhythmia-related hospitalization, or cardioversion/ablation, VO2 max ≤65% of predicted). A normal stress response was defined as a decrease in end-systolic volume (ESV) and increase in ejection fraction. The relative parameter change during stress was calculated as relative parameter change = [(parameterstress - parameterrest)/parameterrest] * 100. The predictive value of dobutamine stress CMR for the composite endpoint was determined using time-to-event analyses (Kaplan-Meier) and Cox proportional hazard analysis. We studied 100 patients [67 (67%) male, median age at baseline CMR 17.8 years (interquartile range 13.5-34.0), age at TOF repair 0.9 years (0.6-2.1)]. After a median follow-up of 8.6 years (6.7-14.1), 10 patients reached the composite endpoint. An abnormal stress response (30% vs. 4.4%, P = 0.021) was more frequently observed in composite endpoint patients. Also in endpoint patients, the relative decrease in right ventricular ESV decreased less during stress compared with the patients without an endpoint (-17 ± 15 vs. -26 ± 13 %, P = 0.045). Multivariable analyses identified an abnormal stress response (hazard ratio 10.4; 95% confidence interval 2.5-43.7; P = 0.001) as predictor for the composite endpoint.

Conclusion: An abnormal ventricular response to dobutamine stress is associated with adverse outcome in patients with repaired TOF.
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http://dx.doi.org/10.1093/ehjci/jez241DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7440962PMC
September 2020

Altered Ascending Aorta Hemodynamics in Patients After Arterial Switch Operation for Transposition of the Great Arteries.

J Magn Reson Imaging 2020 04 7;51(4):1105-1116. Epub 2019 Oct 7.

Division of Pediatric Cardiology, Department of Pediatrics, Leiden University Medical Center, Leiden, The Netherlands.

Background: Patients with transposition of the great arteries (TGA) have an altered aortic geometry after an arterial switch operation (ASO), with neo-aortic root dilatation as an important complication. Geometry-related aortic hemodynamics have been assumed to contribute to pathology of the ascending aorta (AAo).

Purpose: To evaluate aortic flow displacement (FD) and regional wall shear stress (WSS) in relation to ascending neo-aortic geometry in children after ASO.

Study Type: Prospective.

Population: Twenty-eight TGA patients after ASO and 10 healthy volunteers.

Field Strength/sequence: 3.0T/4D flow (segmented fast-spoiled echo pulse), noncontrast-enhanced MR angiography (Dixon), and anatomic images (SSFP).

Assessment: Aortic diameters and body surface area-indexed aortic dimensions (Z-scores), normalized FD and planar ascending aortic WSS.

Statistical Tests: Mann-Whitney and chi-square tests for differences in FD magnitude, WSS, and FD directionality between groups, respectively. Spearman rank correlation to assess the degree of association between aortic geometry, FD and WSS parameters. Shapiro-Wilk test to evaluate distribution normality on the absolute differences in octant location between FD and WSS.

Results: TGA patients showed a significantly dilated proximal AAo and relatively small mid-AAo dimensions at the level of the pulmonary arteries (Z-scores neo-aortic root: 4.38 ± 1.96 vs. 1.52 ± 0.70, P < 0.001; sinotubular junction: 3.48 ± 2.67 vs. 1.38 ± 1.30, P = 0.010; mid-AAo: 0.32 ± 3.06 vs. 1.69 ± 1.24, P = 0.001). FD magnitude was higher in TGA patients (neo-aortic root: 0.048 ± 0.027 vs. 0.021 ± 0.006, P < 0.001; sinotubular junction: 0.054 ± 0.037 vs. 0.029 ± 0.013, P < 0.05) and was related to the neo-aortic Z-score. Clear areas of higher WSS at the right and anterior aortic wall regions along the distal AAo were detected in TGA patients, most pronounced in those with relatively smaller mid-AAo diameters.

Data Conclusion: TGA-specific geometry related to the ASO, evidenced by neo-aortic root dilatation and a sudden change in vessel diameter at mid-AAo level, leads to more aortic flow asymmetry in the proximal AAo and WSS distribution with higher WSS at the right and anterior aortic wall regions along the distal AAo.

Level Of Evidence: 1 Technical Efficacy Stage: 3 J. Magn. Reson. Imaging 2020;51:1105-1116.
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http://dx.doi.org/10.1002/jmri.26934DOI Listing
April 2020

Emotional and behavioral problems in children with dilated cardiomyopathy.

Eur J Cardiovasc Nurs 2020 04 25;19(4):291-300. Epub 2019 Sep 25.

Department of Pediatrics, Erasmus MC - Sophia Children's Hospital, Rotterdam, The Netherlands.

Background: Dilated cardiomyopathy (DCM) in children is an important cause of severe heart failure and carries a poor prognosis. Adults with heart failure are at increased risk of anxiety and depression and such symptoms predict adverse clinical outcomes such as mortality. In children with DCM, studies examining these associations are scarce.

Aims: We studied whether in children with DCM: (1) the level of emotional and behavioral problems was increased as compared to normative data, and (2) depressive and anxiety problems were associated with the combined risk of death or cardiac transplantation.

Methods: To assess emotional and behavioral problems in children with DCM, parents of 68 children, aged 1.5-18 years (6.9±5.7 years), completed the Child Behavior Checklist.

Results: Compared to normative data, more young children (1.5-5 years) with DCM had somatic complaints (24.3% vs. 8.0%; p < .001), but fewer had externalizing problems (5.4% vs. 17.0%; p = .049). Overall internalizing problems did not reach significance. Compared to normative data, more older children (6-18 years) showed internalizing problems (38.7% vs. 17.0%; p = .001), including depressive (29.0% vs. 8.0%; p < .001) and anxiety problems (19.4% vs. 8.0%; p = .023), and somatic complaints (29.0% vs. 8.0%; p < .001). Anxiety and depressive problems, corrected for heart failure severity, did not predict the risk of death or cardiac transplantation.

Conclusion: Children of 6 years and older showed more depressive and anxiety problems than the normative population. Moreover, in both age groups, somatic problems were common. No association with outcome could be demonstrated.
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http://dx.doi.org/10.1177/1474515119876148DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7153220PMC
April 2020

Stress increases intracardiac 4D flow cardiovascular magnetic resonance -derived energetics and vorticity and relates to VOmax in Fontan patients.

J Cardiovasc Magn Reson 2019 07 25;21(1):43. Epub 2019 Jul 25.

Department of Radiology Feinberg School of Medicine, Northwestern University, Chicago, USA.

Background: We hypothesize that dobutamine-induced stress impacts intracardiac hemodynamic parameters and that this may be linked to decreased exercise capacity in Fontan patients. Therefore, the purpose of this study was to assess the effect of pharmacologic stress on intraventricular kinetic energy (KE), viscous energy loss (EL) and vorticity from four-dimensional (4D) Flow cardiovascular magnetic resonance (CMR) imaging in Fontan patients and to study the association between stress response and exercise capacity.

Methods: Ten Fontan patients underwent whole-heart 4D flow CMR before and during 7.5 μg/kg/min dobutamine infusion and cardiopulmonary exercise testing (CPET) on the same day. Average ventricular KE, EL and vorticity were computed over systole, diastole and the total cardiac cycle (vorticity_vol, KE EL). The relation to maximum oxygen uptake (VO max) from CPET was tested by Pearson's correlation or Spearman's rank correlation in case of non-normality of the data.

Results: Dobutamine stress caused a significant 88 ± 52% increase in KE (KE: 1.8 ± 0.5 vs 3.3 ± 0.9 mJ, P < 0.001), a significant 108 ± 49% increase in EL (EL: 0.9 ± 0.4 vs 1.9 ± 0.9 mW, P < 0.001) and a significant 27 ± 19% increase in vorticity (vorticity_vol: 3441 ± 899 vs 4394 ± 1322 mL/s, P = 0.002). All rest-stress differences (%) were negatively correlated to VO max (KE: r = - 0.83, P = 0.003; EL: r = - 0.80, P = 0.006; vorticity_vol: r = - 0.64, P = 0.047).

Conclusions: 4D flow CMR-derived intraventricular kinetic energy, viscous energy loss and vorticity in Fontan patients increase during pharmacologic stress and show a negative correlation with exercise capacity measured by VO max.
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http://dx.doi.org/10.1186/s12968-019-0553-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6657113PMC
July 2019

Cortical development in fetuses with congenital heart defects using an automated brain-age prediction algorithm.

Acta Obstet Gynecol Scand 2019 12 24;98(12):1595-1602. Epub 2019 Aug 24.

Department of Obstetrics and Prenatal Diagnosis, Leiden University Medical Center, Leiden, The Netherlands.

Introduction: Congenital heart defects are associated with neurodevelopmental delay. It is hypothesized that fetuses affected by congenital heart defect have altered cerebral oxygen perfusion and are therefore prone to delay in cortical maturation. The aim of this study was to determine the difference in fetal brain age between consecutive congenital heart defect cases and controls in the second and third trimester using ultrasound.

Material And Methods: Since 2014, we have included 90 isolated severe congenital heart defect cases in the Heart And Neurodevelopment (HAND)-study. Every 4 weeks, detailed neurosonography was performed in these fetuses, including the recording of a 3D volume of the fetal brain, from 20 weeks onwards. In all, 75 healthy fetuses underwent the same protocol to serve as a control group. The volumes were analyzed by automated age prediction software which determines gestational age by the assessment of cortical maturation.

Results: In total, 477 volumes were analyzed using the age prediction software (199 volumes of 90 congenital heart defect cases; 278 volumes of 75 controls). Of these, 16 (3.2%) volume recordings were excluded because of imaging quality. The age distribution was 19-33 weeks. Mixed model analysis showed that the age predicted by brain maturation was 3 days delayed compared with the control group (P = .002).

Conclusions: This study shows that fetuses with isolated cases of congenital heart defects show some delay in cortical maturation as compared with healthy control cases. The clinical relevance of this small difference is debatable. This finding was consistent throughout pregnancy and did not progress during the third trimester.
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http://dx.doi.org/10.1111/aogs.13687DOI Listing
December 2019

Progression of aortic root dilatation and aortic valve regurgitation after the arterial switch operation.

Heart 2019 11 10;105(22):1732-1740. Epub 2019 Jul 10.

Division of Pediatric Cardiology, Department of Pediatrics, Leiden University Medical Center, Leiden, The Netherlands.

Objective: To study neo-aortic growth and the evolution of neo-aortic valve regurgitation (AR) in patients with transposition of the great arteries (TGA) after arterial switch operation (ASO) from newborn to adulthood and to identify patients at risk.

Methods: Neo-aortic dimensions (annulus/root/sinotubular junction) and neo-aortic valve regurgitation were assessed serially in 345 patients with TGA who underwent ASO between 1977 and 2015. Linear mixed-effect models were used to assess increase of neo-aortic dimensions over time and to identify risk factors for dilatation. Risk factor analysis for AR by using time-dependent Cox regression models.

Results: After a rapid increase in the first year after ASO and proportional growth in childhood, neo-aortic dimensions continue to increase in adulthood without stabilisation. Annual diameter increase in adulthood was 0.39±0.06, 0.63±0.09 and 0.54±0.11 mm for, respectively, neo-aortic annulus, root and sinotubular junction, all significantly exceeding normal growth. AR continues to develop over time: freedom from AR ≥moderate during the first 25 years post-ASO was 69%. Risk factors for root dilatation were complex TGA anatomy (TGA-ventricular septal defect (VSD), double outlet right ventricle with subpulmonary VSD) and male gender. Risk factors for AR ≥moderate were: complex TGA anatomy and neo-aortic growth. Per millimetre increase in aortic root dimension, there was a 9% increase in the hazard of AR ≥moderate. Bicuspid pulmonary valve did not relate to the presence of root dilatation or AR.

Conclusion: After ASO, neo-aortic dilatation proceeds beyond childhood and is associated with an increase in AR incidence over time. Careful follow-up of the neo-aortic valve and root function is mandatory, especially in males and in patients with complex TGA anatomy.
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http://dx.doi.org/10.1136/heartjnl-2019-315157DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6855793PMC
November 2019

Implantable cardioverter-defibrillators in previously undiagnosed patients with catecholaminergic polymorphic ventricular tachycardia resuscitated from sudden cardiac arrest.

Eur Heart J 2019 09;40(35):2953-2961

Amsterdam UMC, University of Amsterdam, Heart Centre, and Department of Clinical and Experimental Cardiology, Amsterdam Cardiovascular Sciences, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands.

Aims: In patients with catecholaminergic polymorphic ventricular tachycardia (CPVT), implantable cardioverter-defibrillator (ICD) shocks are sometimes ineffective and may even trigger fatal electrical storms. We assessed the efficacy and complications of ICDs placed in patients with CPVT who presented with a sentinel event of sudden cardiac arrest (SCA) while undiagnosed and therefore untreated.

Methods And Results: We analysed 136 patients who presented with SCA and in whom CPVT was diagnosed subsequently, leading to the initiation of guideline-directed therapy, including β-blockers, flecainide, and/or left cardiac sympathetic denervation. An ICD was implanted in 79 patients (58.1%). The primary outcome of the study was sudden cardiac death (SCD). The secondary outcomes were composite outcomes of SCD, SCA, appropriate ICD shocks, and syncope. After a median follow-up of 4.8 years, SCD had occurred in three patients (3.8%) with an ICD and none of the patients without an ICD (P = 0.1). SCD, SCA, or appropriate ICD shocks occurred in 37 patients (46.8%) with an ICD and 9 patients (15.8%) without an ICD (P < 0.0001). Inappropriate ICD shocks occurred in 19 patients (24.7%) and other device-related complications in 22 patients (28.9%).

Conclusion: In previously undiagnosed patients with CPVT who presented with SCA, an ICD was not associated with improved survival. Instead, the ICD was associated with both a high rate of appropriate ICD shocks and inappropriate ICD shocks along with other device-related complications. Strict adherence to guideline-directed therapy without an ICD may provide adequate protection in these patients without all the potential disadvantages of an ICD.
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http://dx.doi.org/10.1093/eurheartj/ehz309DOI Listing
September 2019

A 45-year experience with the Fontan procedure: tachyarrhythmia, an important sign for adverse outcome.

Interact Cardiovasc Thorac Surg 2019 09;29(3):461-468

Department of Cardiothoracic Surgery, Leiden University Medical Center, Leiden, Netherlands.

Objectives: This study aims to evaluate our 45-year experience with the Fontan procedure and to identify risk factors for late mortality and morbidity.

Methods: Demographic, preoperative, perioperative and postoperative characteristics were retrospectively collected for all patients who underwent a Fontan procedure in a single centre between 1972 and 2016.

Results: The study included 277 Fontan procedures (44 atriopulmonary connections, 28 Fontan-Björk, 42 lateral tunnels and 163 extracardiac conduits). Early failure occurred in 17 patients (6.1%). Median follow-up of the study cohort was 11.9 years (Q1-Q3 7.6-17.5). Longest survival estimates were 31% [95% confidence intervals (CI) 18-44%] at 35 years for atriopulmonary connection/Björk, 87% (95% CI 63-96%) at 20 years for lateral tunnel and 99% (95% CI 96-100%) at 15 years for extracardiac conduit. Estimated freedom from Fontan failure (death, heart transplant, take-down, protein-losing enteropathy, New York Heart Association III-IV) at 15 years was 65% (95% CI 52-76%) for atriopulmonary connection/Björk, 90% (95% CI 73-97%) for lateral tunnel and 90% (95% CI 82-94%) for extracardiac conduit. The development of tachyarrhythmia was an important predictor of Fontan failure [hazard ratio (HR) 2.6, 95% CI 1.2-5.8; P = 0.017], thromboembolic/neurological events (HR 3.6, 95% CI 1.4-9.4; P = 0.008) and pacemaker for sinus node dysfunction (HR 3.7, 95% CI 1.4-9.6; P = 0.008). Prolonged pleural effusion (>21 days) increased the risk of experiencing protein-losing enteropathy (HR 4.7, 95% CI 2.0-11.1; P < 0.001).

Conclusions: With modern techniques, survival and freedom from Fontan failure are good. However, Fontan patients remain subject to general attrition. Tachyarrhythmia is an important sign for an adverse outcome. Prevention and early treatment of tachyarrhythmia may, therefore, be paramount in improving the long-term outcome.
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http://dx.doi.org/10.1093/icvts/ivz111DOI Listing
September 2019

Electrical remodeling after percutaneous atrial septal defect closure in pediatric and adult patients.

Int J Cardiol 2019 06 23;285:32-39. Epub 2019 Feb 23.

Department of Pediatric Cardiology, Leiden University Medical Center, Leiden, the Netherlands; Department of Pediatric Cardiology, Academic Medical Center, Amsterdam, the Netherlands. Electronic address:

Background: Several studies have reported changes in electrocardiographic variables after atrial septal defect (ASD) closure. However no temporal electro-and vectorcardiographic changes have been described from acute to long-term follow-up at different ages. We aimed to study electrical remodeling after percutaneous ASD closure in pediatric and adult patients.

Methods: ECGs of 69 children and 75 adults (median age 6 [IQR 4-11] years and 45 [IQR 33-54] years, respectively) were retrospectively selected before percutaneous ASD closure and at acute (1-7 days), intermediate (4-14 weeks) and late (6-18 months) follow-up. Apart from electrocardiographic variables, spatial QRS-T angle and ventricular gradient (VG) were derived from mathematically-synthesized vectorcardiograms.

Results: In both pediatric and adult patients, the heart rate decreased immediately post-closure, which persisted to late follow-up. The P-wave amplitude also decreased acutely post-closure, but remained unchanged at later follow-up. The PQ duration shortened immediately in children and at intermediate follow-up in adults. The QRS duration and QTc interval decreased at intermediate-term follow-up in both children and adults. In both groups the spatial QRS-T angle decreased at late follow-up. The VG magnitude increased at intermediate follow-up in children and at late follow-up in adults, after an initial decrease in children.

Conclusion: In both pediatric and adult ASD patients, electrocardiographic changes mainly occurred directly after ASD closure except for shortening of QRS duration and QTc interval, which occurred at later follow-up. Adults also showed late changes in PQ duration. At 6-to-18 month post-closure, the spatial QRS-T angle decreased, reflecting increased electrocardiographic concordance. The initial acute decrease in VG in children, which was followed by a significant increase, may be the effect of action potential duration dynamics directly after percutaneous ASD closure.
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http://dx.doi.org/10.1016/j.ijcard.2019.02.020DOI Listing
June 2019