Background: An anomalous coronary artery is reported in 2% to 23% of patients with tetralogy of Fallot (TOF).Knowledge of coronary anatomy prior to corrective surgery is vital to avoid damage to... Show moreBackground: An anomalous coronary artery is reported in 2% to 23% of patients with tetralogy of Fallot (TOF).Knowledge of coronary anatomy prior to corrective surgery is vital to avoid damage to vessels crossing theright ventricular outflow tract (RVOT). A meta-analysis on the prevalence of anomalous coronary arteries inTOF is lacking to date. Here, an overview of coronary anomalies in TOF is provided and implications for patientmanagement are discussed.Methods: PubMed, Embase and Web of Science were searched. Analysis was done using Revman 5.3 (CochraneCommunity, London). The primary analysis focused on the origin and proximal course of the right and left coronaryarteries. In addition, the prevalence of large conus arteries and coronary arteriovenous fistulas (CAVF) wascalculated.Results: Twenty-eight studies, encompassing 6956 patients, were included; 6% of TOF patients have an anomalouscoronary artery. Hereof, 72% cross the RVOT; the majority of the remaining 28% courses behind the aorta.Six percent of patients have a large conus artery and 4% a CAVF. Other coronary anomalies include a left orright coronary artery from the pulmonary trunk or left or right pulmonary artery, coronary tree hypoplasiaand anastomoses between coronary and bronchial arteries.Conclusions: The prevalence of coronary anomalies in TOF is 4–6%. In patientswith an anomalous coronary artery,72% cross the RVOT. The combined risk of encountering an anomalous coronary artery or a large conus arterycrossing the RVOT is 10.3%. Coronary anatomy should be defined before surgery and the surgical approachadapted accordingly. Show less
Schneider, A.W.; Hazekamp, M.G.; Versteegh, M.I.M.; Weger, A. de; Holman, E.R.; Klautz, R.J.M.; ... ; Braun, J. 2019
OBJECTIVES: Repeat aortic valve interventions after previous stentless aortic valve replacement (AVR) are considered technically challenging with an increased perioperative risk, especially after... Show moreOBJECTIVES: Repeat aortic valve interventions after previous stentless aortic valve replacement (AVR) are considered technically challenging with an increased perioperative risk, especially after full-root replacement. We analysed our experience with reinterventions after stentless AVR.METHODS: A total of 75 patients with previous AVR using a Freestyle stentless bioprosthesis (31 subcoronary, 15 root-inclusion and 29 full-root replacement) underwent reintervention in our centre from 1993 until December 2018. Periprocedural data were retrospectively collected from the department database and follow-up data were prospectively collected.RESULTS: Median age was 62 years (interquartile range 47-72 years). Indications for reintervention were structural valve deterioration (SVD) in 47, non-SVD in 13 and endocarditis in 15 patients. Urgent surgery was required in 24 (32%) patients. Reinterventions were surgical AVR in 16 (21%), root replacement in 51 (68%) and transcatheter AVR in 8 (11%) patients. Early mortality was 9.3% (n = 7), but decreased to zero in the past decade in 28 patients undergoing elective reoperation. Per indication, early mortality was 9% for SVD, 8% for non-SVD and 13% for endocarditis. Aortic root replacement had the lowest early mortality rate (6%), followed by surgical AVR (13%) and transcatheter AVR (25%, 2 patients with coronary artery obstruction). Pacemaker implantation rate was 7%. Overall survival rate at 10 years was 69% (95% confidence interval 53-81%).CONCLUSIONS: Repeat aortic valve interventions after stentless AVR carry an increased, but acceptable, early mortality risk. Transcatheter valve-in-valve procedures after stentless AVR require careful consideration of prosthesis leaflet position to prevent obstruction of the coronary arteries. Show less
Zandstra, T.E.; Palmen, M.; Hazekamp, M.G.; Meyns, B.; Beeres, S.L.M.A.; Holman, E.R.; ... ; Tops, L.F. 2019
Ventricular assist device (VAD) implantation is an established treatment modality for patients with end-stage heart failure, and improves symptoms and survival. In the Netherlands, it is not yet... Show moreVentricular assist device (VAD) implantation is an established treatment modality for patients with end-stage heart failure, and improves symptoms and survival. In the Netherlands, it is not yet routinely considered in patients with congenital heart disease and failing systemic right ventricle (SRV). Recently, a VAD was implanted in 2 SRV patients, one who underwent a Mustard procedure during infancy for transposition of the great arteries (male, 47 years old) and one with a congenitally corrected transposition of the great arteries (male, 54 years old). The first patient is doing well >1 year after implantation; the second patient will be discharged home soon. These examples and other reports demonstrate the feasibility of adopting VAD implantation into routine care for SRV failure. In conclusion, patients with SRV failure may be suitable candidates for VAD implantation: they are relatively young, usually have a preserved subpulmonary left ventricular function, and their specific anatomical and physiological characteristics often make them unsuitable for cardiac transplantation. Therefore it is important to recognise the possibility of VAD implantation early in the process of SRV failure, and to timely refer these patients to a heart failure clinic with experience in VAD implantation in this group of patients for optimisation, screening, and implantation. Show less
Palen, R.L.F. van der; Bom, T. van der; Dekker, A.; Tsonaka, R.; Geloven, N. van; Kuipers, I.M.; ... ; Blom, N.A. 2019
BackgroundWe hypothesize that dobutamine-induced stress impacts intracardiac hemodynamic parameters and that this may be linked to decreased exercise capacity in Fontan patients. Therefore, the... Show moreBackgroundWe 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.MethodsTen Fontan patients underwent whole-heart 4D flow CMR before and during 7.5 mu g/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(avg cycle), KEavg cycle, ELavg cycle). The relation to maximum oxygen uptake (VO2 max) from CPET was tested by Pearson's correlation or Spearman's rank correlation in case of non-normality of the data.ResultsDobutamine stress caused a significant 8852% increase in KE (KEavg cycle: 1.8 +/- 0.5 vs 3.3 +/- 0.9mJ, P<0.001), a significant 108 +/- 49% increase in EL (ELavg cycle: 0.9 +/- 0.4 vs 1.9 +/- 0.9mW, P<0.001) and a significant 27 +/- 19% increase in vorticity (vorticity_vol(avg cycle): 3441 +/- 899 vs 4394 +/- 1322mL/s, P=0.002). All rest-stress differences (%) were negatively correlated to VO2 max (KEavg cycle: r=-0.83, P=0.003; ELavg cycle: r=-0.80, P=0.006; vorticity_vol(avg cycle): 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 VO2 max. Show less
OBJECTIVES In patients with the Fontan circulation, systemic venous return flows passively towards the lungs. Because of the absence of the subpulmonary ventricle, favourable blood flow patterns... Show moreOBJECTIVES In patients with the Fontan circulation, systemic venous return flows passively towards the lungs. Because of the absence of the subpulmonary ventricle, favourable blood flow patterns with minimal energy loss are clinically relevant. The region where the inferior vena cava, the hepatic veins and the extracardiac conduit join (IVC-conduit junction) is a potential source of increased energy loss. The aim of this study was to evaluate the relationship between geometry and blood flow patterns in the IVC-conduit junction with associated kinetic energy and energy loss using 4-dimensional flow magnetic resonance imaging (MRI).METHODS Fourteen extracardiac conduit-Fontan patients underwent 4-dimensional flow MRI. The IVC-conduit junctions were ranked into 3 groups for 3 categories: the geometry, the flow complexity and the conduit mean velocity. The relative increase in the mean velocity from the IVC to the conduit (representing IVC-conduit mismatch) was determined. The peak kinetic energy and mean kinetic energy and energy loss were determined and normalized for volume.RESULTS In 4 of 14 patients, adverse geometries led to helical flow patterns and/or acute changes in flow direction. For each category, the most adverse IVC-conduit junctions were associated with an approximate 2.3-3.2-fold and 2.0-2.9-fold increase in kinetic energy and energy loss, respectively. The IVC-conduit mismatch is strongly correlated with the mean kinetic energy and energy loss (r=0.80, P=0.001 and rho=0.83, P<0.001, respectively) and with body surface area in patients with 16-mm conduits (r=0.88, P=0.010).CONCLUSIONS The IVC-conduit junction is a potential source of increased energy loss. Junctions with increased energy loss showed: (i) a distorted geometry leading to adverse blood flow patterns and/or (ii) the IVC-conduit mismatch. Sixteen-millimetre conduits appear to be inadequate for older patients. Show less
Objective This study assessed adult survival and morbidity patterns in patients who underwent atrial correction according to Mustard or Senning for transposition of the great arteries (TGA).Methods... Show moreObjective This study assessed adult survival and morbidity patterns in patients who underwent atrial correction according to Mustard or Senning for transposition of the great arteries (TGA).Methods In 76 adult patients with TGA (59% male) after atrial correction, long-term survival and morbidity were investigated in three periods: early (<15 years postoperatively), midterm (15-30 years postoperatively) and late (>30 years postoperatively).Results The Mustard technique was performed in 41 (54%) patients, and the Senning technique was performed in 35 (46%) patients aged 3.1 (IQR: 2.1-3.8) and 1.0 (IQR: 0.6-3.1; p<0.01) years, respectively. Adult survival was 82% at 39.7 (IQR: 35.9-42.4) years postoperatively and exceeded 50 years in four patients. Supraventricular tachycardia (SVT) occurred in 51% of patients. The incidences of ventricular arrhythmia (0%, 8% and 13%; p<0.01), heart failure (0%, 5% and 19%; p<0.01) and surgical reinterventions (0%, 5% and 11%; p=0.01) increased from early to late follow-up. At last follow-up, RV function was depressed in 31 (46%) patients, and New York Heart Association functional class was >= 2 in 34 (48%) patients. Bradyarrhythmia, SVT and ventricular arrhythmia were associated with depressed RV function (OR: 4.47, 95% CI 1.50 to 13.28, p<0.01; OR: 3.74, 95% CI 1.26 to 11.14, p=0.02; OR: 14.40, 95% CI 2.80 to 74.07, p<0.01, respectively) and worse functional capacity (OR: 2.10, 95% CI 0.75 to 5.82, p=0.16; OR: 2.87, 95% CI 1.06 to 7.81, p=0.04; OR: 8.47, 95% CI 1.70 to 42.10, p<0.01, respectively).Conclusions In adult patients with TGA, survival was 82% at 39.7 (IQR: 35.9-42.4) years after atrial correction. Morbidity was high and included SVT as most frequent adverse event. Ventricular arrhythmias, heart failure and surgical reinterventions were common during late follow-up. Adverse events were associated with depressed right ventricle function and reduced functional class. Show less
Visualization and quantification of the adverse effects of distorted blood flow are important emerging fields in cardiology. Abnormal blood flow patterns can be seen in various cardiovascular... Show moreVisualization and quantification of the adverse effects of distorted blood flow are important emerging fields in cardiology. Abnormal blood flow patterns can be seen in various cardiovascular diseases and are associated with increased energy loss. These adverse energetics can be measured and quantified using 3-dimensional blood flow data, derived from computational fluid dynamics and 4-dimensional flow magnetic resonance imaging, and provide new, promising hemodynamic markers. In patients with palliated single-ventricular heart defects, the Fontan circulation passively directs systemic venous return to the pulmonary circulation in the absence of a functional subpulmonary ventricle. Therefore, the Fontan circulation is highly dependent on favorable flow and energetics, and minimal energy loss is of great importance. A focus on reducing energy loss led to the introduction of the total cavopulmonary connection (TCPC) as an alternative to the classical Fontan connection. Subsequently, many studies have investigated energy loss in the TCPC, and energy-saving geometric factors have been implemented in clinical care. Great advances have been made in computational fluid dynamics modeling and can now be done in 3-dimensional patient-specific models with increasingly accurate boundary conditions. Furthermore, the implementation of 4-dimensional flow magnetic resonance imaging is promising and can be of complementary value to these models. Recently, correlations between energy loss in the TCPC and cardiac parameters and exercise intolerance have been reported. Furthermore, efficiency of blood flow through the TCPC is highly variable, and inefficient blood flow is of clinical importance by reducing cardiac output and increasing central venous pressure, thereby increasing the risk of experiencing the well-known Fontan complications. Energy loss in the TCPC will be an important new hemodynamic parameter in addition to other well-known risk factors such as pulmonary vascular resistance and can possibly be improved by patient-specific surgical design. This article describes the theoretical background of mechanical energy of blood flow in the cardiovascular system and the methods of calculating energy loss, and it gives an overview of geometric factors associated with energy efficiency in the TCPC and its implications on clinical outcome. Furthermore, the role of 4-dimensional flow magnetic resonance imaging and areas of future research are discussed. Show less
