Publications by authors named "Mark G Hazekamp"

167 Publications

Pulmonary ductal coarctation: An entity associated with congenital heart defects involving the right ventricle outflow tract.

J Card Surg 2021 Oct 12. Epub 2021 Oct 12.

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

We present a case of a prematurely born 2-month-old girl with a double outlet right ventricle with pulmonary atresia and a left pulmonary artery coarctation arising after ductal closure. The case highlights the importance of knowledge and identification of such an anomaly in patients with congenital heart malformations with severe pulmonary stenosis to atresia.
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http://dx.doi.org/10.1111/jocs.16067DOI Listing
October 2021

Invited Commentary: Residual Lesions and ECMO: Seek and You Shall Find Ways to Narrow the ECMO Gap.

World J Pediatr Congenit Heart Surg 2021 Sep;12(5):614-615

4501Leiden University Medical Center, Leiden, The Netherlands.

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http://dx.doi.org/10.1177/21501351211035785DOI Listing
September 2021

The Coronary Arteries in Adults after the Arterial Switch Operation: A Systematic Review.

J Cardiovasc Dev Dis 2021 Aug 26;8(9). Epub 2021 Aug 26.

Center for Congenital Heart Disease Amsterdam-Leiden (CAHAL), Department of Cardiology, Leiden University Medical Center, 2333 ZA Leiden, The Netherlands.

Coronary artery status in adults long after the arterial switch operation (ASO) is unclear. We conducted a systematic review to provide an overview of coronary complications during adulthood and to evaluate the value of routine coronary imaging in adults after ASO, in light of current guidelines. Articles were screened for the inclusion of adult ASO patients and data on coronary complications and findings of coronary imaging were collected. A total of 993 adults were followed with a median available follow-up of only 2.0 years after reaching adulthood. Myocardial ischemia was suspected in 17/192 patients (8.9%). The number of coronary interventions was four (0.4%), and coronary death was reported in four (0.4%) patients. A lack of ischemia-related symptoms cannot be excluded because innervation studies indicated deficient cardiac innervation after ASO, although data is limited. Anatomical high-risk features found by routine coronary computed tomography (cCT) included stenosis (4%), acute angle (40%), kinking (24%) and inter-arterial course (11%). No coronary complications were reported during pregnancy (n = 45), although, remarkably, four (9%) patients developed heart failure. The 2020 European Society of Cardiology (ESC) guidelines state that routine screening for coronary pathologies is questionable. Based on current findings and in line with the 2018 American ACC/AHA guidelines a baseline assessment of the coronary arteries in all ASO adults seems justifiable. Thereafter, an individualized coronary follow-up strategy is advisable at least until significant duration of follow-up is available.
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http://dx.doi.org/10.3390/jcdd8090102DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8468869PMC
August 2021

The Influence of Respiration on Blood Flow in the Fontan Circulation: Insights for Imaging-Based Clinical Evaluation of the Total Cavopulmonary Connection.

Front Cardiovasc Med 2021 5;8:683849. Epub 2021 Aug 5.

Department of Cardiology, Biomedical Engineering, Biomechanics Laboratory, Rotterdam, Netherlands.

Congenital heart disease is the most common birth defect and functionally univentricular heart defects represent the most severe end of this spectrum. The Fontan circulation provides an unique solution for single ventricle patients, by connecting both caval veins directly to the pulmonary arteries. As a result, the pulmonary circulation in Fontan palliated patients is characterized by a passive, low-energy circulation that depends on increased systemic venous pressure to drive blood toward the lungs. The absence of a subpulmonary ventricle led to the widely believed concept that respiration, by sucking blood to the pulmonary circulation during inspiration, is of great importance as a driving force for antegrade blood flow in Fontan patients. However, recent studies show that respiration influences pulsatility, but has a limited effect on net forward flow in the Fontan circulation. Importantly, since MRI examination is recommended every 2 years in Fontan patients, clinicians should be aware that most conventional MRI flow sequences do not capture the pulsatility of the blood flow as a result of the respiration. In this review, the unique flow dynamics influenced by the cardiac and respiratory cycle at multiple locations within the Fontan circulation is discussed. The impact of (not) incorporating respiration in different MRI flow sequences on the interpretation of clinical flow parameters will be covered. Finally, the influence of incorporating respiration in advanced computational fluid dynamic modeling will be outlined.
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http://dx.doi.org/10.3389/fcvm.2021.683849DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8374887PMC
August 2021

Hemodynamic Consequences of an Undersized Extracardiac Conduit in an Adult Fontan Patient Revealed by 4-Dimensional Flow Magnetic Resonance Imaging.

Circ Cardiovasc Imaging 2021 Aug 12;14(8):e012612. Epub 2021 Aug 12.

Radiology (H.C.v.A., J.J.M.W.), Leiden University Medical Center, the Netherlands.

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http://dx.doi.org/10.1161/CIRCIMAGING.121.012612DOI Listing
August 2021

Determinants of exercise limitation in contemporary paediatric Fontan patients with an extra cardiac conduit.

Int J Cardiol 2021 Oct 8;341:31-38. Epub 2021 Aug 8.

The Centre for Congenital Heart Disease Amsterdam-Leiden, Leiden, the Netherlands; Willem-Alexander Children's Hospital, Department of Paediatrics, Division of Paediatric Cardiology, Leiden University Medical Centre, the Netherlands.

Background: Although various determinants of exercise limitation in Fontan patients have been studied, most research has been performed in patients who underwent different surgical procedures with differing haemodynamic characteristics. The aim of the current study was to evaluate non-invasively measured cardiovascular parameters and their influence on exercise performance in paediatric Fontan patients with an extracardiac conduit and moderate-good systolic ventricular function.

Methods: Fontan patients, between 8 and 18 years of age, with moderate to good systolic ventricular function and an extracardiac conduit were included. Exercise performance and cardiovascular assessment, comprising echocardiography, aortic stiffness measurement and ambulatory measurement of cardiac autonomous nervous activity were performed on the same day. Healthy subjects served as controls.

Results: Thirty-six Fontan patients (age 14.0 years) and thirty-five healthy subjects (age 12.8 years) were included. Compared to controls, Fontan patients had reduced diastolic ventricular function and increased arterial stiffness. No differences were found in heart rate (HR) and cardiac parasympathetic nervous activity. In Fontan patients, maximal as well as submaximal exercise capacity was impaired, with the percentage of predicted capacity ranging between 54 and 72%. Chronotropic competence, however, was good with a peak HR of 174 (94% of predicted). Lower maximal and submaximal exercise capacity was correlated with a higher HR at rest, higher pulse wave velocity of the aorta and a lower ratio of early and late diastolic flow velocity.

Conclusion: Contemporary paediatric Fontan patients have an impaired exercise capacity with preserved chronotropic competence. Exercise performance correlates with heart rate at rest, diastolic function and aortic stiffness.
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http://dx.doi.org/10.1016/j.ijcard.2021.08.012DOI Listing
October 2021

Acute kidney injury after the arterial switch operation: incidence, risk factors, and outcomes.

Cardiol Young 2021 Aug 5:1-6. Epub 2021 Aug 5.

Department of Pediatric intensive care, Leiden University Medical Centre LUMC, Leiden, the Netherlands.

Background: The aim of this retrospective cohort study was to determine the incidence, potential risk factors, characteristics, and outcomes of acute kidney injury in children following the arterial switch operation for transposition of the great arteries.

Methods: Retrospective review of children who underwent ASO between 2000 and 2020 in our tertiary children's hospital in the Netherlands. Pre-and post-ASO serum creatinine levels were collected. Severe AKI was defined as 100% serum creatinine rise or estimated creatinine clearance <35 ml/min/1.73 m2 according to pRIFLE criteria. Logistic regression was used to adjust for confounders.

Results: A total of 242 children were included. Fifty-seven (24%) children developed severe AKI after ASO. Four patients with severe AKI were treated with renal replacement therapy. Children with severe AKI had a longer duration of mechanical ventilation 4.5 (1.0-29) versus 3 (1.0-12) days (p = 0.001), longer PICU stay 7 (2-76) versus 5 (1-70) days, (p = 0.001), higher rate of myocardial infarction 5% versus 0.5% (p = 0.001), sepsis 24% versus 9% (p = 0.002), post-operative pulmonary hypertension 19% versus 6% (p = 0.002), post-operative bleeding 9% versus 3% (p = 0.044), longer time to sternal closure 3 (1-19) versus 2 (1-6) days, (p = 0.009), and a higher mortality rate 9.0% versus 0.5% (p = 0.001) compared to children without severe AKI. Sepsis was a risk factor for developing severe AKI.

Conclusions: In this single-centre cohort, 24% of our patients developed severe AKI after ASO, which is associated with increased morbidity, longer PICU stay, and higher mortality.
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http://dx.doi.org/10.1017/S1047951121003176DOI Listing
August 2021

Computed Tomography Derived Coronary Triangulated Orifice Area-Deduction of a New Parameter for Follow-up After Surgical Correction of Anomalous Aortic Origin of Coronary Arteries and Call for Validation.

Front Cardiovasc Med 2021 24;8:668503. Epub 2021 Jun 24.

CAHAL, Center for Congenital Heart Disease Amsterdam Leiden, Leiden, Netherlands.

Anomalous aortic origin of a coronary artery (AAOCA) from the opposite sinus of Valsalva is a rare congenital abnormality. Computed tomography angiography (CTA) is primarily used as a diagnostic tool to evaluate the anatomy and identify potentially malignant AAOCA variants. Limited data is available on the role of CTA during postoperative follow-up. We aimed to develop an objective CTA derived parameter for diagnostic evaluation and follow-up after surgical correction of AAOCA and correlate the anatomical features to the postoperative outcome. All consecutive patients who underwent surgical repair of AAOCA from 2001 to 2018 and had pre and postoperative CTA imaging available were included. A retrospective analysis of the pre- and postoperative CTA and the outcomes was performed. The origin and course of the anomalous coronary artery and the ostial dimensions were evaluated and correlated with restenosis of operated coronary artery. To allow an accurate evaluation of the effective orifice area at diagnosis and after surgical repair we deduce and propose a new parameter-the coronary triangulated orifice area (CTOA). Out of the 54 patients who underwent surgical treatment for AAOCA, 11 fulfilled the inclusion criteria. The median follow-up was 19 months [IQR 3;42]. The mean age at surgery was 41 ± 16 years, with six patients (55%) being male. Postoperatively, the angle between the proximal coronary artery and the aortic wall increased from 20 ± 5° to 28 ± 9° ( < 0.01) and ostial diameter in the transversal plane increased from 4.1 ± 2.5 mm to 6.2 ± 2.7 mm ( < 0.01). The median CTOA increased significantly from 1.6 mm [IQR 0.9;4.9] to 5.5 mm [IQR 3;11.8] ( < 0.005). During follow-up, in three patients a restenosis of the operated coronary artery was suspected. In these patients, the CTOA only showed a limited postoperative increase of ≤ 1.4 mm. CTA can play an important role in the evaluation of the pre- and postoperative anatomy in AAOCA patients. CTOA may be of use in conjunction with the acute angle take-off and ostial diameter order to comprehensively evaluate the operated ostium after unroofing or patch angioplasty.
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http://dx.doi.org/10.3389/fcvm.2021.668503DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8263932PMC
June 2021

Prognostic Value of Maximal and Submaximal Exercise Performance in Fontan Patients < 15 Years of Age.

Am J Cardiol 2021 09 4;154:92-98. Epub 2021 Jul 4.

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

In patients after Fontan completion exercise capacity is significantly reduced. Although peak oxygen consumption (VO) is a strong prognostic factor in many cardiovascular diseases, it requires the achievement of a maximal effort. Therefore, submaximal exercise parameters such as oxygen uptake efficiency slope (OUES) may be of value. In the present observational study we evaluated the exercise capacity with maximal and submaximal parameters in a group of Fontan patients with an extracardiac conduit and determined their prognostic value. Sixty Fontan patients followed up in the Leiden University Medical Center who have performed an exercise test were included in this retrospective study. Exercise tests were performed at a median age of 11 years. Fontan patients showed on average lower values for all exercise parameters compared to reference values from a healthy dataset as shown by the %predicted values: VO%:mean 66%(95%CI:64 to 74) and OUES%:mean 72%(95%CI:67 to 77). Twenty percent of the patients were not able to achieve an RER>1.0. RER showed a moderate positive correlation with VO but not with OUES. There was a deterioration of VO% and OUES% over time. OUES was significantly lower in patients with cardiac events in the follow up period. Fontan patients have an impaired exercise performance even at young ages and it deteriorates with age. An important percentage of Fontan patients is not able to reach maximal effort so the use of submaximal parameters, like OUES, should be considered as part of the evaluation. Moreover, OUES could have a prognostic value in this group of patients.
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http://dx.doi.org/10.1016/j.amjcard.2021.05.049DOI Listing
September 2021

Long-term outcomes of atrioventricular septal defect and single ventricle: A multicenter study.

J Thorac Cardiovasc Surg 2021 May 18. Epub 2021 May 18.

Department of Cardiothoracic Surgery, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands.

Objective: The study objective was to analyze survival and incidence of Fontan completion of patients with single-ventricle and concomitant unbalanced atrioventricular septal defect.

Methods: Data from 4 Dutch and 3 Belgian institutional databases were retrospectively collected. A total of 151 patients with single-ventricle atrioventricular septal defect were selected; 36 patients underwent an atrioventricular valve procedure (valve surgery group). End points were survival, incidence of Fontan completion, and freedom from atrioventricular valve reoperation.

Results: Median follow-up was 13.4 years. Cumulative survival was 71.2%, 70%, and 68.5% at 10, 15, and 20 years, respectively. An atrioventricular valve procedure was not a risk factor for mortality. Patients with moderate-severe or severe atrioventricular valve regurgitation at echocardiographic follow-up had a significantly worse 15-year survival (58.3%) compared with patients with no or mild regurgitation (89.2%) and patients with moderate regurgitation (88.6%) (P = .033). Cumulative incidence of Fontan completion was 56.5%, 71%, and 77.6% at 5, 10, and 15 years, respectively. An atrioventricular valve procedure was not associated with the incidence of Fontan completion. In the valve surgery group, freedom from atrioventricular valve reoperation was 85.7% at 1 year and 52.6% at 5 years.

Conclusions: The long-term survival and incidence of Fontan completion in our study were better than previously described for patients with single-ventricle atrioventricular septal defect. A concomitant atrioventricular valve procedure did not increase the mortality rate or decrease the incidence of Fontan completion, whereas patients with moderate-severe or severe valve regurgitation at follow-up had a worse survival. Therefore, in patients with single-ventricle atrioventricular septal defect when atrioventricular valve regurgitation exceeds a moderate degree, the atrioventricular valve should be repaired.
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http://dx.doi.org/10.1016/j.jtcvs.2021.05.015DOI Listing
May 2021

Consensus document on optimal management of patients with common arterial trunk.

Eur J Cardiothorac Surg 2021 07;60(1):7-33

Department for Congenital Cardiology and Pediatric Cardiology, University Medical Center of Schleswig-Holstein, Kiel, Germany.

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http://dx.doi.org/10.1093/ejcts/ezaa423DOI Listing
July 2021

Consensus document on optimal management of patients with common arterial trunk.

Cardiol Young 2021 06 21;31(6):915-939. Epub 2021 May 21.

Department for Congenital Cardiology and Pediatric Cardiology, University Medical Center of Schleswig-Holstein, Kiel, Germany.

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http://dx.doi.org/10.1017/S1047951121001219DOI Listing
June 2021

Right aortic arch forming a true vascular ring: a clinical review.

Eur J Cardiothorac Surg 2021 May 10. Epub 2021 May 10.

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

Objectives: This review aims at presenting and summarizing the current state of literature on the presentation and surgical management of a right-sided aortic arch with a left-sided ligamentum forming a complete vascular ring around the oesophagus and trachea.

Methods: A systematic database search for appropriate literature was conducted on PubMed/MEDLINE. Articles were considered relevant when providing details on the presentation, diagnosis and surgical treatment of this specific congenital arch anomaly in human beings.

Results: Affected patients present with respiratory and/or oesophageal difficulties due to tracheoesophageal compression. Conservative treatment might be reasonable in asymptomatic or mildly symptomatic cases; however, once moderate-to-severe symptoms develop, surgical intervention is definitely indicated. Surgery is commonly performed through a left thoracotomy or median sternotomy and includes the division of the left ductal ligamentum; if a Kommerell's diverticulum is present that is >1.5 times the diameter of the subclavian artery, then concomitant resection of the large diverticulum and translocation of the aberrant left subclavian artery is also conducted. Postoperative morbidity and mortality are low and are rather related to concomitant intracardiac and extracardiac anomalies than to the procedure itself. In a majority of patients, full resolution of symptoms is seen within months to years from the surgery. Nevertheless, there is also a subset of patients who remain with some tracheobronchial narrowing, sometimes even requiring reintervention during follow-up due to persisting or recurring symptoms.

Conclusions: Overall, the surgical management of a right aortic arch forming a true vascular ring in infancy, childhood and adulthood seems relatively safe and effective in providing symptomatic relief despite some persistent tracheobronchial and/or oesophageal narrowing in some cases.
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http://dx.doi.org/10.1093/ejcts/ezab225DOI Listing
May 2021

Long-term outcome after the arterial switch operation: 43 years of experience.

Eur J Cardiothorac Surg 2021 05;59(5):968-977

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

Objectives: The objective of this study was to assess our 43-year experience with arterial switch operation (ASO) for transposition of the great arteries (TGA) by analysing cardiac outcome measures (hospital and late mortality, reoperations and catheter interventions, significant coronary artery obstruction) and to identify risk factors for reoperation and catheter interventions.

Methods: A total of 490 patients who underwent ASO for TGA from 1977 to 2020 were included in this retrospective, single-centre study. Data on reoperation and catheter intervention of hospital survivors were estimated by the Kaplan-Meier method and compared using a long-rank test. Risk factors for reoperation and/or catheter intervention were assessed by multivariate Cox regression analysis.

Results: Hospital mortality occurred in 43 patients (8.8%), late death in 12 patients (2.9%) and 43 patients were lost to follow-up. Median follow-up time of 413 hospital survivors was 15.6 (interquartile range 7.0-22.4) years. Reoperations were performed in 83 patients (117 reoperations). Neoaortic valve regurgitation with root dilatation was the second most common indication for reoperation (15/83 patients, 18.1%) after right ventricular outflow tract obstruction (50/83 patients, 60.2%). Risk factors for any reoperation on multivariable analysis were: TGA morphological subtype [TGA with ventricular septal defect: hazard ratio (HR) = 1.99, 95% confidence interval (CI) 1.18-3.36; P = 0.010 and Taussig-Bing: HR = 2.17, 95% CI 1.02-4.64; P = 0.045], aortic arch repair associated with ASO (HR = 3.03, 95% CI 1.62-5.69; P = 0.001) and a non-usual coronary artery anatomy (HR = 2.41, 95% CI 1.45-4.00; P = 0.001). One hundred and one catheter interventions were performed in 54 patients, usually for relief of supravalvular pulmonary stenosis (44/54 patients, 81.5%) or arch obstruction (10/54 patients, 18.5%). Main risk factor for catheter intervention on multivariable analysis was aortic arch repair associated with ASO (HR = 2.95, 95% CI 1.37-6.36; P = 0.006). Significant coronary artery stenosis was relatively uncommon (9/413 patients, 2.2%) but may be underrepresented.

Conclusions: Patients after ASO typically have good long-term clinical outcomes but reoperations and interventions remain necessary in some patients. Neoaortic valve regurgitation with root dilatation is the second most common indication for reoperation after right ventricular outflow tract obstruction and an increasing need for neoaortic valve and root redo surgery in future is to be expected.
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http://dx.doi.org/10.1093/ejcts/ezab006DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8106945PMC
May 2021

Non-uniform mixing of hepatic venous flow and inferior vena cava flow in the Fontan conduit.

J R Soc Interface 2021 04 7;18(177):20201027. Epub 2021 Apr 7.

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

Fontan patients require a balanced hepatic blood flow distribution (HFD) to prevent pulmonary arteriovenous malformations. Currently, HFD is quantified by tracking Fontan conduit flow, assuming hepatic venous (HV) flow to be uniformly distributed within the Fontan conduit. However, this assumption may be unvalid leading to inaccuracies in HFD quantification with potential clinical impact. The aim of this study was to (i) assess the mixing of HV flow and inferior vena caval (IVC) flow within the Fontan conduit and (ii) quantify HFD by directly tracking HV flow and quantitatively comparing results with the conventional approach. Patient-specific, time-resolved computational fluid dynamic models of 15 total cavopulmonary connections were generated, including the HV and subhepatic IVC. Mixing of HV and IVC flow, on a scale between 0 (no mixing) and 1 (perfect mixing), was assessed at the caudal and cranial Fontan conduit. HFD was quantified by tracking particles from the caudal (HFD) and cranial (HFD) conduit and from the hepatic veins (HFD). HV flow was non-uniformly distributed at both the caudal (mean mixing 0.66 ± 0.13) and cranial (mean 0.79 ± 0.11) level within the Fontan conduit. On a cohort level, differences in HFD between methods were significant but small; HFD (51.0 ± 20.6%) versus HFD (48.2 ± 21.9%, = 0.033) or HFD (48.0 ± 21.9%, = 0.044). However, individual absolute differences of 8.2-14.9% in HFD were observed in 4/15 patients. HV flow is non-uniformly distributed within the Fontan conduit. Substantial individual inaccuracies in HFD quantification were observed in a subset of patients with potential clinical impact.
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http://dx.doi.org/10.1098/rsif.2020.1027DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8086942PMC
April 2021

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

Open Heart 2021 03;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

Reduced scan time and superior image quality with 3D flow MRI compared to 4D flow MRI for hemodynamic evaluation of the Fontan pathway.

Sci Rep 2021 03 22;11(1):6507. Epub 2021 Mar 22.

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

Long scan times prohibit a widespread clinical applicability of 4D flow MRI in Fontan patients. As pulsatility in the Fontan pathway is minimal during the cardiac cycle, acquiring non-ECG gated 3D flow MRI may result in a reduction of scan time while accurately obtaining time-averaged clinical parameters in comparison with 2D and 4D flow MRI. Thirty-two Fontan patients prospectively underwent 2D (reference), 3D and 4D flow MRI of the Fontan pathway. Multiple clinical parameters were assessed from time-averaged flow rates, including the right-to-left pulmonary flow distribution (main endpoint) and systemic-to-pulmonary collateral flow (SPCF). A ten-fold reduction in scan time was achieved [4D flow 15.9 min (SD 2.7 min) and 3D flow 1.6 min (SD 7.8 s), p < 0.001] with a superior signal-to-noise ratio [mean ratio of SNRs 1.7 (0.8), p < 0.001] and vessel sharpness [mean ratio 1.2 (0.4), p = 0.01] with 3D flow. Compared to 2D flow, good-excellent agreement was shown for mean flow rates (ICC 0.82-0.96) and right-to-left pulmonary flow distribution (ICC 0.97). SPCF derived from 3D flow showed good agreement with that from 4D flow (ICC 0.86). 3D flow MRI allows for obtaining time-averaged flow rates and derived clinical parameters in the Fontan pathway with good-excellent agreement with 2D and 4D flow, but with a tenfold reduction in scan time and significantly improved image quality compared to 4D flow.
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http://dx.doi.org/10.1038/s41598-021-85936-6DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7985309PMC
March 2021

The Clinical Spectrum of Kommerell's Diverticulum in Adults with a Right-Sided Aortic Arch: A Case Series and Literature Overview.

J Cardiovasc Dev Dis 2021 Feb 26;8(3). Epub 2021 Feb 26.

Center for Congenital Heart Disease Amsterdam-Leiden (CAHAL), Leiden University Medical Center, 2333 ZA Leiden, The Netherlands.

Background: Kommerell's diverticulum is a rare vascular anomaly characterized as an outpouch at the onset of an aberrant subclavian artery. In the variant of a right-sided aortic arch, the trachea and esophagus are enclosed dorsally by the arch. In the configuration of an aberrant left subclavian artery, a Kommerell's diverticulum and persisting ductus arteriosus or ductal ligament enclose the lateral side, forming a vascular ring which may result in (symptomatic) esophageal or tracheal compression. Spontaneous rupture of an aneurysmatic Kommerell's diverticulum has also been reported. Due to the rarity of this condition and underreporting in the literature, the clinical implications of a Kommerell's diverticulum are not well defined.

Case Summary: We describe seven consecutive adult patients with a right-sided aortic arch and an aberrant course of the left subclavian artery (arteria lusoria), and a Kommerell's diverticulum, diagnosed in our tertiary hospital. One patient had severe symptoms related to the Kommerell's diverticulum and underwent surgical repair. In total, two of the patients experienced mild non-limiting dyspnea complaints and in four patients the Kommerell's diverticulum was incidentally documented on a computed tomography (CT) scan acquired for a different indication. The size of the Kommerell's diverticulum ranged from 19 × 21 mm to 30 × 29 mm. In the six patients that did not undergo surgery, a strategy of periodic follow-up with structural imaging was pursued. No significant growth of the Kommerell's diverticulum was observed and none of the patients experienced an acute aortic syndrome to date.

Discussion: Kommerell's diverticulum in the setting of a right-sided aortic arch with an aberrant left subclavian artery is frequently associated with tracheal and esophageal compression and this may result in a varying range of symptoms. Guidelines on management of Kommerell's diverticulum are currently lacking. This case series and literature overview suggests that serial follow-up is warranted in adult patients with a Kommerell's diverticulum with small dimensions and no symptoms, however, that surgical intervention should be considered when patients become symptomatic or when the diameter exceeds 30 mm in the absence of symptoms.
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http://dx.doi.org/10.3390/jcdd8030025DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7996811PMC
February 2021

The Leiden Convention coronary coding system: translation from the surgical to the universal view.

Eur Heart J Cardiovasc Imaging 2021 Feb 15. Epub 2021 Feb 15.

Department of Cardiology, Leiden University Medical Center, Albinusdreef 2, PO Box 9600, Postal zone B-04-P, 2300 RC Leiden, The Netherlands.

Aims: The Leiden Convention coronary coding system structures the large variety of coronary anatomical patterns; isolated and in congenital heart disease. It is widely used by surgeons but not by cardiologists as the system uses a surgeons' cranial view. Since thoracic surgeons and cardiologists work closely together, a coronary coding system practical for both disciplines is mandatory. To this purpose, the 'surgical' coronary coding system was adapted to an 'imaging' system, extending its applicability to different cardiac imaging techniques.

Methods And Results: The physician takes place in the non-facing sinus of the aortic valve, oriented with the back towards the pulmonary valve, looking outward from the sinus. From this position, the right-hand sinus is sinus 1, and the left-hand sinus is sinus 2. Next, a clockwise rotation is adopted starting at sinus 1 and the encountered coronary branches described. Annotation of the normal anatomical pattern is 1R-2LCx, corresponding to the 'surgical' coding system. The 'imaging' coding system was made applicable for Computed Tomography (CT), Magnetic Resonance Imaging (MRI), echocardiography, and coronary angiography, thus facilitating interdisciplinary use. To assess applicability in daily clinical practice, images from different imaging modalities were annotated by cardiologists and cardiology residents and results scored. The average score upon evaluation was 87.5%, with the highest scores for CT and MRI images (average 90%).

Conclusion: The imaging Leiden Convention is a coronary coding system that unifies the annotation of coronary anatomy for thoracic surgeons, cardiologists, and radiologists. Validation of the coding system shows it can be easily and reliably applied in clinical practice.
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http://dx.doi.org/10.1093/ehjci/jeab012DOI Listing
February 2021

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

Pediatr Cardiol 2021 Apr 1;42(4):866-874. 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
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8110507PMC
April 2021

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

Prenat Diagn 2021 May 26;41(6):754-765. Epub 2021 Feb 26.

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
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8248090PMC
May 2021

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

Eur J Cardiothorac Surg 2021 04;59(4):814-822

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
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8083947PMC
April 2021

Repair of traumatic avulsion of the right bronchus in children using extracorporeal membrane oxygenation support.

Interact Cardiovasc Thorac Surg 2021 05;32(5):834-836

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

Traumatic avulsion of the right main bronchus in children is usually caused by blunt trauma or traffic accidents. Primary repair by suturing is the preferred treatment. Lesions are life threatening and urgent or emergency surgical repair is indicated. We report our experience with 2 cases of traumatic avulsion of right bronchus in children successfully suture repaired with the use of extracorporeal membrane oxygenation.
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http://dx.doi.org/10.1093/icvts/ivaa327DOI Listing
May 2021

We can, but should we?

Eur J Cardiothorac Surg 2021 04;59(4):830-831

Department of Cardiothoracic Surgery, University Hospital Leiden, Leiden, Netherlands.

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http://dx.doi.org/10.1093/ejcts/ezaa434DOI Listing
April 2021

The significance of symptoms before and after surgery for anomalous aortic origin of coronary arteries in adolescents and adults.

Interact Cardiovasc Thorac Surg 2021 01;32(1):122-129

Department of Cardiology, CAHAL, Center for Congenital Heart Disease Amsterdam Leiden, Leiden University Medical Center, Leiden, Netherlands.

Objectives: The aim of this study is to describe the significance of symptoms preoperatively and at medium-term follow-up in adolescent and adult patients who underwent surgery of anomalous aortic origin of a coronary artery (AAOCA).

Methods: Consecutive patients who underwent surgery for AAOCA in our tertiary referral centre between 2001 and 2018 were included. Clinical characteristics and symptoms were evaluated and medium-term outcomes were recorded. Symptoms were classified according to the '2019 ESC guidelines on chronic coronary syndromes'.

Results: A total of 53 (55% male) patients with mean age of 44 at time of surgery underwent surgical repair of AAOCA. Data on symptoms and events ˃3 months after surgery were available in 34 patients with a median follow-up of 3 years (interquartile range 1.0-5.3). Preoperatively, only 35% patients had typical anginal complaints. After surgical correction of AAOCA, 59% of the patients were free of symptoms, compared to 6% preoperatively (P < 0.001). A total of 3 (9%) patients needed a reoperation/reintervention related to the operated AAOCA. All 3 patients presented postoperatively with novel typical anginal complaints.

Conclusions: Adolescent and adult patients with AAOCA present with varying symptoms. Only 35% have typical anginal complaints. Surgical correction of AAOCA reduces the symptoms in the vast majority of patients. One should be aware of potential lesions of the operated coronary artery in patients presenting with typical anginal complaints postoperatively.
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http://dx.doi.org/10.1093/icvts/ivaa234DOI Listing
January 2021

Long-Term Follow-Up of Pericardium for the Ventricular Component in Atrioventricular Septal Defect Repair.

World J Pediatr Congenit Heart Surg 2020 Nov;11(6):742-747

Division of Pediatrics, Department of Pediatric Cardiac Surgery, 8124University Medical Center Utrecht, the Netherlands.

Background: Despite the improved outcome in complete atrioventricular septal defect (AVSD) repair, reoperations for left atrioventricular valve (LAVV) dysfunction are common. The aim of this study was to evaluate the effect of fresh untreated autologous pericardium for ventricular septal defect (VSD) closure on atrioventricular valve function and compare the results with the use of treated bovine pericardial patch material.

Methods: Clinical and echocardiographic data were collected of patients with complete AVSD with their VSD closed with either untreated autologous pericardial or treated bovine pericardial patch material between January 1, 1996, and December 31, 2003. Evaluation closed in September 2019.

Results: A total of 77 patients were analyzed (untreated autologous pericardial VSD patch: 59 [77%], treated bovine pericardial VSD patch: 18 [23%]). Median age at surgery was 3.6 (interquartile range [IQR]: 2.7-4.5) months, and median weight was 4.5 (IQR: 3.9-5.1) kg. Trisomy 21 was present in 70 (91%) patients. Median follow-up time was 17.5 (IQR: 12.6-19.8) years. Death <30 days occurred in two (3%) patients. Reinterventions occurred in eight patients (early [within 30 days] in two, early and late in one, and late in five), all in the autologous pericardium group. Log-rank tests showed no significant difference in mortality ( = .892), LAVV reinterventions ( = .228), or LAVV regurgitation ( = .770).

Conclusions: In AVSD, the VSD can safely be closed with either untreated autologous pericardium or xeno-pericardium. We found no difference in LAVV regurgitation or the need for reoperation between the two patches.
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http://dx.doi.org/10.1177/2150135120941461DOI Listing
November 2020

Rhinovirus Detection in the Nasopharynx of Children Undergoing Cardiac Surgery Is Not Associated With Longer PICU Length of Stay: Results of the Impact of Rhinovirus Infection After Cardiac Surgery in Kids (RISK) Study.

Pediatr Crit Care Med 2021 01;22(1):e79-e90

Department of Medical Microbiology, Leiden University Medical Center, Leiden, The Netherlands.

Objectives: To determine whether children with asymptomatic carriage of rhinovirus in the nasopharynx before elective cardiac surgery have an increased risk of prolonged PICU length of stay.

Study Design: Prospective, single-center, blinded observational cohort study.

Setting: PICU in a tertiary hospital in The Netherlands.

Patients: Children under 12 years old undergoing elective cardiac surgery were enrolled in the study after informed consent of the parents/guardians.

Interventions: The parents/guardians filled out a questionnaire regarding respiratory symptoms. On the day of the operation, a nasopharyngeal swab was obtained. Clinical data were collected during PICU admission, and PICU/hospital length of stay were reported. If a patient was still intubated 3 days after operation, an additional nasopharyngeal swab was collected. Nasopharyngeal swabs were tested for rhinovirus and other respiratory viruses with polymerase chain reaction.

Measurements And Main Results: Of the 163 included children, 74 (45%) tested rhinovirus positive. Rhinovirus-positive patients did not have a prolonged PICU length of stay (median 2 d each; p = 0.257). Rhinovirus-positive patients had a significantly shorter median hospital length of stay compared with rhinovirus-negative patients (8 vs 9 d, respectively; p = 0.006). Overall, 97 of the patients (60%) tested positive for one or more respiratory virus. Virus-positive patients had significantly shorter PICU and hospital length of stay, ventilatory support, and nonmechanical ventilation. Virus-negative patients had respiratory symptoms suspected for a respiratory infection more often. In 31% of the children, the parents reported mild upper respiratory complaints a day prior to the cardiac surgery, this was associated with postextubation stridor, but no other clinical outcome measures.

Conclusions: Preoperative rhinovirus polymerase chain reaction positivity is not associated with prolonged PICU length of stay. Our findings do not support the use of routine polymerase chain reaction testing for respiratory viruses in asymptomatic children admitted for elective cardiac surgery.
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http://dx.doi.org/10.1097/PCC.0000000000002522DOI Listing
January 2021

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

Heart 2020 12 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

Mechanical Mitral Valve Replacement: A Multicenter Study of Outcomes With Use of 15- to 17-mm Prostheses.

Ann Thorac Surg 2020 12 7;110(6):2062-2069. Epub 2020 Jun 7.

Department of Cardiac Surgery, Boston Children's Hospital, Boston, Massachusetts; Department of Cardiac Surgery, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts.

Background: The aim of this study was to evaluate early and mid-term outcomes (mortality and prosthetic valve reintervention) after mitral valve replacement with 15- to 17-mm mechanical prostheses.

Methods: A multicenter, retrospective cohort study was performed among patients who underwent mitral valve replacement with a 15- to 17-mm mechanical prosthesis at 6 congenital cardiac centers: 5 in The Netherlands and 1 in the United States. Baseline, operative, and follow-up data were evaluated.

Results: Mitral valve replacement was performed in 61 infants (15 mm, n = 17 [28%]; 16 mm, n = 18 [29%]; 17 mm, n = 26 [43%]), of whom 27 (47%) were admitted to the intensive care unit before surgery and 22 (39%) required ventilator support. Median age at surgery was 5.9 months (interquartile range [IQR] 3.2-17.4), and median weight was 5.7 kg (IQR, 4.5-8.8). There were 13 in-hospital deaths (21%) and 8 late deaths (17%, among 48 hospital survivors). Major adverse events occurred in 34 (56%). Median follow-up was 4.0 years (IQR, 0.4-12.5) First prosthetic valve replacement (n = 27 [44%]) occurred at a median of 3.7 years (IQR, 1.9-6.8). Prosthetic valve endocarditis was not reported, and there was no mortality related to prosthesis replacement. Other reinterventions included permanent pacemaker implantation (n = 9 [15%]), subaortic stenosis resection (n = 4 [7%]), aortic valve repair (n = 3 [5%], and aortic valve replacement (n = 6 [10%]).

Conclusions: Mitral valve replacement with 15- to 17-mm mechanical prostheses is an important alternative to save critically ill neonates and infants in whom the mitral valve cannot be repaired. Prosthesis replacement for outgrowth can be carried out with low risk.
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http://dx.doi.org/10.1016/j.athoracsur.2020.04.084DOI Listing
December 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
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