Bilateral bidirectional cavopulmonary connection: a review of surgical techniques and clinical implications.

The presence of bilateral superior caval veins (bSCVs) could negatively influence the outcome of Fontan patients. In the setting of a bilateral bidirectional Glenn, the selective blood flow to the ipsilateral long with consequent flow stagnation in the connecting portion could lead to poor growth of the central portion of the pulmonary artery, potentially affecting the eligibility for Fontan completion and being associated with a higher incidence of thrombotic complications. Alternative surgical techniques have been described to perform a bidirectional cavopulmonary anastomosis in the presence of bSCVs aiming to achieve a balanced growth of the pulmonary bifurcation. The short-term results of these techniques such as the V- or Y-shape seem to be excellent; however, some anatomical settings could affect the feasibility of these techniques. The so-called "unifocalization" creates a configuration comparable to a "normal" bidirectional Glenn and could be a feasible alternative. However, the long-term results of this technique are not published yet. The positive effect of additional pulsatile pulmonary flow on pulmonary artery growth should be considered in case of bilateral bidirectional Glenn, despite the higher incidence of postoperative complications reported and the difficult calibration of the amount of additional flow. The role of computational fluid dynamic to simulate the surgical strategy in single ventricle patients is promising and could be worthwhile in the setting of bSCVs. In fact, the surgical techniques of bilateral bidirectional Glenn could be simulated testing their feasibility and allowing to identify the more favorable hemodynamic pattern, patient specific. This review article highlights the critical issues related to the presence of bSCVs in univentricular physiology, analyzing pros and cons of the different surgical techniques. Besides reviewing the literature, this manuscript focuses on the role of computational fluid simulation in identifying the most favorable surgical technique with an individualized approach, which could potentially improve the clinical outcome.

Impact of Prosthesis-Patient Mismatch After Surgical Aortic Valve Replacement: Systematic Review and Meta-Analysis of Reconstructed Time-to-Event Data of 122 989 Patients With 592 952 Patient-Years.

BACKGROUND: It remains controversial whether prosthesis-patient mismatch (PPM) impacts long-term outcomes after surgical aortic valve replacement. We aimed to evaluate the association of PPM with mortality, rehospitalizations, and aortic valve reinterventions. METHODS AND RESULTS: We performed a systematic review with meta-analysis of reconstructed time-to-event data of studies published by March 2023 (according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses). Sixty-five studies met our eligibility criteria and included 122 989 patients (any PPM: 68 332 patients, 55.6%). At 25 years of follow-up, the survival rates were 11.8% and 20.6% in patients with and without any PPM, respectively (hazard ratio [HR], 1.16 [95% CI, 1.13-1.18], P<0.001). At 20 years of follow-up, the survival rates were 19.5%, 12.1%, and 8.8% in patients with no, moderate, and severe PPM, respectively (moderate versus no PPM: HR, 1.09 [95% CI, 1.06-1.11], P<0.001; severe versus no PPM: HR, 1.29 [95% CI, 1.24-1.35], P<0.001). PPM was associated with higher risk of cardiac death, heart failure-related hospitalizations, and aortic valve reinterventions over time (P<0.001). Statistically significant associations between PPM and worse survival were observed regardless of valve type (bioprosthetic versus mechanical valves), contemporary PPM definitions unadjusted and adjusted for body mass index, and PPM quantification method (in vitro, in vivo, Doppler echocardiography). Our meta-regression analysis revealed that populations with more women tend to have higher HRs for all-cause death associated with PPM. CONCLUSIONS: The results of the present study suggest that any degree of PPM is associated with poorer long-term outcomes following surgical aortic valve replacement and provide support for implementation of preventive strategies to avoid PPM after surgical aortic valve replacement.

Intraoperative transit-time flow measurement of caval veins before and after bidirectional cavopulmonary anastomosis.

BACKGROUND: Haemodynamic changes in caval venous flow distribution occurring during bidirectional cavopulmonary anastomosis operation are still largely unknown. METHODS: Transit time flow measurements were performed in 15 cavopulmonary anastomosis operations. Superior and inferior caval vein flows were measured before and after the cavopulmonary anastomosis. Ratio of superior caval vein to overall caval veins flow was calculated. RESULTS: Mean superior caval vein flow ratio before cavopulmonary anastomosis was higher than previously reported for healthy children. Superior caval vein flow ratio decreased in 14/15 patients after cavopulmonary anastomosis: mean 0.63 ± 0.12 before versus 0.43 ± 0.14 after. No linear correlation between intraoperative superior caval vein pressure and superior caval vein flow after cavopulmonary anastomosis was found. Neither Nakata index nor pulmonary vascular resistance measured at preoperative cardiac catheterisation correlated with intraoperative flows. None of patients died or required a take down. CONCLUSIONS: The higher mean superior caval vein flow ratio before cavopulmonary anastomosis compared to healthy children suggests flow redistribution in univentricular physiology to protect brain and neurodevelopment. The decrease of superior caval vein flow ratio after cavopulmonary anastomosis may reflect the flow redistribution related to trans-pulmonary gradient. The lack of correlation between superior caval vein pressure and superior caval vein flow could be explained by limited sample size and multifactorial determinants of caval veins flow, although pressure remain essential. Larger sample of measurements are needed to find flow range potentially predictive for clinical failure. To authors' knowledge, this is the first intraoperative flow measurement of both caval veins during cavopulmonary operations.

Combining Congenital Heart Surgical and Interventional Cardiology Outcome Data in a Single Database: The Development of a Patient-Centered Collaboration of the European Congenital Heart Surgeons Association (ECHSA) and the Association for European Paediatric and Congenital Cardiology (AEPC).

The European Congenital Heart Surgeons Association (ECHSA) Congenital Database (CD) is the second largest clinical pediatric and congenital cardiac surgical database in the world and the largest in Europe, where various smaller national or regional databases exist. Despite the dramatic increase in interventional cardiology procedures over recent years, only scattered national or regional databases of such procedures exist in Europe. Most importantly, no congenital cardiac database exists in the world that seamlessly combines both surgical and interventional cardiology data on an international level; therefore, the outcomes of surgical and interventional procedures performed on the same or similar patients cannot easily be tracked, assessed, and analyzed. In order to fill this important gap in our capability to gather and analyze information on our common patients, ECHSA and The Association for European Paediatric and Congenital Cardiology (AEPC) have embarked on a collaborative effort to expand the ECHSA-CD with a new module designed to capture data about interventional cardiology procedures. The purpose of this manuscript is to describe the concept, the structure, and the function of the new AEPC Interventional Cardiology Part of the ECHSA-CD, as well as the potentially valuable synergies provided by the shared interventional and surgical analyses of outcomes of patients. The new AEPC Interventional Cardiology Part of the ECHSA-CD will allow centers to have access to robust surgical and transcatheter outcome data from their own center, as well as robust national and international aggregate outcome data for benchmarking. Each contributing center or department will have access to their own data, as well as aggregate data from the AEPC Interventional Cardiology Part of the ECHSA-CD. The new AEPC Interventional Cardiology Part of the ECHSA-CD will allow cardiology centers to have access to aggregate cardiology data, just as surgical centers already have access to aggregate surgical data. Comparison of surgical and catheter interventional outcomes could potentially strengthen decision processes. A study of the wealth of information collected in the database could potentially also contribute toward improved early and late survival, as well as enhanced quality of life of patients with pediatric and/or congenital heart disease treated with surgery and interventional cardiac catheterization across Europe and the world.

Combining Congenital Heart Surgical and Interventional Cardiology Outcome Data in a Single Database: The Development of a Patient-Centered Collaboration of the European Congenital Heart Surgeons Association (ECHSA) and the Association for European Paediatric and Congenital Cardiology (AEPC).

The European Congenital Heart Surgeons Association (ECHSA) Congenital Database (CD) is the second largest clinical pediatric and congenital cardiac surgical database in the world and the largest in Europe, where various smaller national or regional databases exist. Despite the dramatic increase in interventional cardiology procedures over recent years, only scattered national or regional databases of such procedures exist in Europe. Most importantly, no congenital cardiac database exists in the world that seamlessly combines both surgical and interventional cardiology data on an international level; therefore, the outcomes of surgical and interventional procedures performed on the same or similar patients cannot easily be tracked, assessed, and analyzed. In order to fill this important gap in our capability to gather and analyze information on our common patients, ECHSA and The Association for European Paediatric and Congenital Cardiology (AEPC) have embarked on a collaborative effort to expand the ECHSA-CD with a new module designed to capture data about interventional cardiology procedures. The purpose of this manuscript is to describe the concept, the structure, and the function of the new AEPC Interventional Cardiology Part of the ECHSA-CD, as well as the potentially valuable synergies provided by the shared interventional and surgical analyses of outcomes of patients. The new AEPC Interventional Cardiology Part of the ECHSA-CD will allow centers to have access to robust surgical and transcatheter outcome data from their own center, as well as robust national and international aggregate outcome data for benchmarking. Each contributing center or department will have access to their own data, as well as aggregate data from the AEPC Interventional Cardiology Part of the ECHSA-CD. The new AEPC Interventional Cardiology Part of the ECHSA-CD will allow cardiology centers to have access to aggregate cardiology data, just as surgical centers already have access to aggregate surgical data. Comparison of surgical and catheter interventional outcomes could potentially strengthen decision processes. A study of the wealth of information collected in the database could potentially also contribute toward improved early and late survival, as well as enhanced quality of life of patients with pediatric and/or congenital heart disease treated with surgery and interventional cardiac catheterization across Europe and the world.

All surgical supra-annular aortic valvar tissue prostheses are labelled too large.

OBJECTIVES: Surgical supra-annular aortic valvar tissue prostheses are labelled in an inconsistent and confusing manner. If the replaced valve is too small for a given patient, the risk of patient-prosthesis mismatch is increased, which is associated with the risk of morbidity and mortality. The labelled diameter (LD) of these valves should coincide with the inflow orifice diameter (IOD). Therefore, our goal was to measure all relevant IODs. METHODS: Valvar design was assessed in terms of the intended position of the valve in relation to the patient's annulus. The IODs of all available supra-annular aortic valvar prostheses were measured using a conical gauge. The IODs were compared to the LDs. We searched for instructions for use, websites, packing boxes and regulatory institutions involved in the process. RESULTS: Eight valve models from 4 manufacturers were included. None of these valves were clearly labelled as supra-annular on the packing box, although for 3, the supra-annular label could be found in the written specifications. All valves had an IOD smaller than their LD, with a median difference of 15% (range: 4%-25%). The departure from LD differed per valve model and valve size. CONCLUSIONS: Valve packages should be labelled accurately and clearly so that surgeons can make a well-informed choice. Currently essential information is missing because the intended position in relation to the annulus is not consistently marked on the packing boxes, and valve sizes are labelled incorrectly. We propose a change for the better: relabel all valves according to their true IOD in a structured manner.

Quantification of systemic-to-pulmonary collateral flow in univentricular physiology with 4D flow MRI.

PURPOSE: Systemic-to-pulmonary collateral flow is a well-recognised phenomenon in patients with single ventricle physiology, but remains difficult to quantify. The aim was to compare the reported formula's that have been used for calculation of systemic-to-pulmonary-collateral flow to assess their consistency and to quantify systemic-to-pulmonary collateral flow in patients with a Glenn and/or Fontan circulation using four-dimensional flow MRI (4D flow MR). METHODS: Retrospective case-control study of Glenn and Fontan patients who had a 4D flow MR study. Flows were measured at the ascending aorta, left and right pulmonary arteries, left and right pulmonary veins, and both caval veins. Systemic-to-pulmonary collateral flow was calculated using two formulas: 1) pulmonary veins - pulmonary arteries and 2) ascending aorta - caval veins. Anatomical identification of collaterals was performed using the 4D MR image set. RESULTS: Fourteen patients (n = 11 Fontan, n = 3 Glenn) were included (age 26 [22-30] years). Systemic-to-pulmonary collateral flow was significantly higher in the patients than the controls (n = 10, age 31.2 [15.1-38.4] years) with both formulas: 0.28 [0.09-0.5] versus 0.04 [-0.66-0.21] l/min/m2 (p = 0.036, formula 1) and 0.67 [0.24-0.88] versus -0.07 [-0.16-0.08] l/min/m2 (p < 0.001, formula 2). In patients, systemic-to-pulmonary collateral flow differed significantly between formulas 1 and 2 (13% versus 26% of aortic flow, p = 0.038). In seven patients, veno-venous collaterals were detected and no aortopulmonary collaterals were visualised. CONCLUSION: 4D flow MR is able to detect increased systemic-to-pulmonary collateral flow and visualise collaterals vessels in Glenn and Fontan patients. However, the amount of systemic-to-pulmonary collateral flow varies with the formula employed. Therefore, further research is necessary before it could be applied in clinical care.

Opportunities and short-comings of the axolotl salamander heart as a model system of human single ventricle and excessive trabeculation.

Few experimental model systems are available for the rare congenital heart diseases of double inlet left ventricle (DILV), a subgroup of univentricular hearts, and excessive trabeculation (ET), or noncompaction. Here, we explore the heart of the axolotl salamander (Ambystoma mexicanum, Shaw 1789) as model system of these diseases. Using micro-echocardiography, we assessed the form and function of the heart of the axolotl, an amphibian, and compared this to human DILV (n = 3). The main finding was that both in the axolotl and DILV, blood flows of disparate oxygen saturation can stay separated in a single ventricle. In the axolotl there is a solitary ventricular inlet and outlet, whereas in DILV there are two separate inlets and outlets. Axolotls had a lower resting heart rate compared to DILV (22 vs. 72 beats per minute), lower ejection fraction (47 vs. 58%), and their oxygen consumption at rest was higher than peak oxygen consumption in DILV (30 vs. 17 ml min-1 kg-1). Concerning the ventricular myocardial organization, histology showed trabeculations in ET (n = 5) are much closer to the normal human setting than to the axolotl setting. We conclude that the axolotl heart resembles some aspects of DILV and ET albeit substantial species differences exist.

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

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.

Cardiothoracic Surgery in the Caribbean

Abstract Introduction: Despite being one of the main vacation destinations in the world, health care in the Caribbean faces many difficulties. The challenges involved in these islands' medical care range from low-resource institutions to lack of specialized care. In the field of thoracic and cardiac surgery, many limitations exist, and these include the lack of access to cardiac surgery for many small islands and little governmental funding for minimally invasive approaches in thoracic surgery. Methods: Literature review was done using PubMed/MEDLINE and Google Scholar databases to identify articles describing the characteristics of thoracic and cardiac surgery departments on Caribbean islands. Articles on the history, current states of practice, and advances in cardiothoracic surgery in the Caribbean were reviewed. Results: Regardless of the middle to high-income profile of the Caribbean, there are significant differences in the speed of technological growth in cardiothoracic surgery from island to island, as well as disparities between the quality of care and resources. Many islands struggle to advance the field of cardiothoracic surgery both through lack of local cardiac surgery centers and limited financial funding for minimally invasive thoracic surgery. Conclusions: Cardiac and thoracic surgery in the Caribbean depend not only on the support from local government policies and proper distribution of healthcare budgets, but efforts by the surgeons themselves to change and improve institutional cultures. Although resource availability still remains a challenge, the Caribbean remains an important region that deserves special attention with regard to the unmet needs for long-term sustainability of chest surgery.

Video-Assisted Thoracoscopy For Penetrating Cardiac Box Injury in Stable Patients

Abstract Introduction: In high-volume trauma centers, especially in developing countries, penetrating cardiac box injuries are frequent. Although many aspects of penetrating chest injuries have been well established, video-assisted thoracoscopy is still finding its place in cardiac box trauma and algorithmic approaches are still lacking. The purpose of this manuscript is to provide a streamlined recommendation for penetrating cardiac box injury in stable patients. Methods: Literature review was carried out using PubMed/MEDLINE and Google Scholar databases to identify articles describing the characteristics and concepts of penetrating cardiac box trauma, including the characteristics of tamponade, cardiac ultrasound, indications and techniques of pericardial windows and, especially, the role of video-assisted thoracoscopy in stable patients. Results: Penetrating cardiac box injuries, whether by stab or gunshot wounds, require rapid surgical consultation. Unstable patients require immediate open surgery, however, determining which stable patients should be taken to thoracoscopic surgery is still controversial. Here, the classification of penetrating cardiac box injury used in Colombia is detailed, as well as the algorithmic approach to these types of trauma. Conclusion: Although open surgery is mandatory in unstable patients with penetrating cardiac box injuries, a more conservative and minimally invasive approach may be undertaken in stable patients. As rapid decision-making is critical in the trauma bay, surgeons working in high-volume trauma centers should expose themselves to thoracoscopy and always consider this possibility in the setting of penetrating cardiac box injuries in stable patients, always in the context of an experienced trauma team.

Video-Assisted Thoracoscopy For Penetrating Cardiac Box Injury in Stable Patients.

INTRODUCTION: In high-volume trauma centers, especially in developing countries, penetrating cardiac box injuries are frequent. Although many aspects of penetrating chest injuries have been well established, video-assisted thoracoscopy is still finding its place in cardiac box trauma and algorithmic approaches are still lacking. The purpose of this manuscript is to provide a streamlined recommendation for penetrating cardiac box injury in stable patients. METHODS: Literature review was carried out using PubMed/ MEDLINE and Google Scholar databases to identify articles describing the characteristics and concepts of penetrating cardiac box trauma, including the characteristics of tamponade, cardiac ultrasound, indications and techniques of pericardial windows and, especially, the role of video-assisted thoracoscopy in stable patients. RESULTS: Penetrating cardiac box injuries, whether by stab or gunshot wounds, require rapid surgical consultation. Unstable patients require immediate open surgery, however, determining which stable patients should be taken to thoracoscopic surgery is still controversial. Here, the classification of penetrating cardiac box injury used in Colombia is detailed, as well as the algorithmic approach to these types of trauma. CONCLUSION: Although open surgery is mandatory in unstable patients with penetrating cardiac box injuries, a more conservative and minimally invasive approach may be undertaken in stable patients. As rapid decision-making is critical in the trauma bay, surgeons working in high-volume trauma centers should expose themselves to thoracoscopy and always consider this possibility in the setting of penetrating cardiac box injuries in stable patients, always in the context of an experienced trauma team.

Pediatric Cardiac Surgical Patterns of Practice and Outcomes in Japan and Europe.

OBJECTIVES: The Japan Cardiovascular Surgery Database-Congenital section (JCVSD-Congenital) and the European Congenital Heart Surgeons Association (ECHSA) Congenital Heart Surgery Database (CHSD) share the same nomenclature. We aimed at comparing congenital cardiac surgical patterns of practice and outcomes in Japan and Europe using the JCVSD-Congenital and ECHSA-CHSD. METHODS AND RESULTS: We examined Japanese (120 units, 63,365 operations) and European (96 units, 90,098 operations) data in JCVSD-Congenital and ECHSA-CHSD from 2011 to 2017. Patients' age and weight, periprocedural times, mortality at hospital discharge, and postoperative length of stay were calculated for ten benchmark operations. There was a significantly higher proportion of ventricular septal defect closures and Glenn operations and a significantly lower proportion of coarctation repairs, tetralogy of Fallot repairs, atrioventricular septal defect repairs, arterial switch operations, truncus repairs, Norwood operations, and Fontan operations in JCVSD-Congenital compared to ECHSA-CHSD. Postoperative length of stay was significantly longer following all benchmark operations in JCVSD-Congenital compared to ECHSA-CHSD. Mean STAT mortality score (Society of Thoracic Surgeons European Association for Cardio-Thoracic Surgery mortality score) was significantly higher in JCVSD-Congenital (0.78) compared to ECHSA-CHSD (0.71). Mortality at hospital discharge was significantly lower in JCVSD-Congenital (4.2%) compared to ECHSA-CHSD (6.0%, P < .001). CONCLUSIONS: The distribution of the benchmark procedures and age at the time of surgery differ between Japan and Europe. Postoperative length of stay is longer, and the mean complexity is higher in Japan compared to European data. These comparisons of patterns of practice and outcomes demonstrate opportunities for continuing bidirectional transcontinental collaboration and quality improvement.

Anomalous left coronary artery from pulmonary artery repair: Outcomes from the European Congenital Heart Surgeons Association Database.

INTRODUCTION: We sought to determine the surgical outcomes of patients with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) enrolled in the European Congenital Heart Surgeons Association (ECHSA) database. MATERIALS AND METHODS: From 1999 to 2019, 907 patients with ALCAPA underwent surgical repair and were included in the current study. The primary outcome was in-hospital mortality. Secondary outcomes included frequency and results of concomitant mitral valve surgery and postoperative mechanical circulatory support (MCS). RESULTS: The overall in-hospital mortality was 6% (54/907) and was significantly higher in neonates (p = .01), patients with lower body surface area (BSA) (p = .01), and those requiring postoperative MCS (p = .001). Associated mitral valve surgery was performed in 144 patients (15.9%) and was associated with longer cardiopulmonary bypass (CPB) and aortic cross-clamp times (AOX) (p ≤ .0001) but was not significantly related to an increase in in-hospital mortality. Postoperative MCS was required in 66 patients (7.3%). These patients were younger (p ≤ .001), had a lower BSA (p ≤ .001), and required a longer CPB (p ≤ .001) and AOX time (p ≤ .001). CONCLUSIONS: ALCAPA repair can be achieved successfully, and with low surgical risk. Concomitant mitral valve procedures can be performed without increasing operative mortality. The use of MCS remains a valuable option, especially in younger patients.

Cardiothoracic Surgery in the Caribbean.

INTRODUCTION: Despite being one of the main vacation destinations in the world, health care in the Caribbean faces many difficulties. The challenges involved in these islands' medical care range from low-resource institutions to lack of specialized care. In the field of thoracic and cardiac surgery, many limitations exist, and these include the lack of access to cardiac surgery for many small islands and little governmental funding for minimally invasive approaches in thoracic surgery. METHODS: Literature review was done using PubMed/MEDLINE and Google Scholar databases to identify articles describing the characteristics of thoracic and cardiac surgery departments on Caribbean islands. Articles on the history, current states of practice, and advances in cardiothoracic surgery in the Caribbean were reviewed. RESULTS: Regardless of the middle to high-income profile of the Caribbean, there are significant differences in the speed of technological growth in cardiothoracic surgery from island to island, as well as disparities between the quality of care and resources. Many islands struggle to advance the field of cardiothoracic surgery both through lack of local cardiac surgery centers and limited financial funding for minimally invasive thoracic surgery. CONCLUSIONS: Cardiac and thoracic surgery in the Caribbean depend not only on the support from local government policies and proper distribution of healthcare budgets, but efforts by the surgeons themselves to change and improve institutional cultures. Although resource availability still remains a challenge, the Caribbean remains an important region that deserves special attention with regard to the unmet needs for long-term sustainability of chest surgery.

Surgery for Adult Patients with Congenital Heart Disease: Results from the European Database.

Adults with congenital heart disease (ACHD) represent a growing population. To evaluate frequency, type and outcomes of cardiac surgery in ACHD, we gathered data from the European Congenital Heart Surgeons Association Database of 20,602 adult patients (≥18 years) with a diagnosis of congenital heart disease who underwent cardiac surgery, between January 1997 and December 2017. We demonstrated that overall surgical workload (as absolute frequencies of surgical procedures per year) for this specific subset of patients increased steadily during the study period. The most common procedural groups included septal defects repair (n = 5740, 28%), right-heart lesions repair (n = 5542, 27%) and left-heart lesions repair (n = 4566, 22%); almost one-third of the procedures were re-operations (n = 5509, 27%). When considering the year-by-year relative frequencies of the main procedural groups, we observed a variation of the surgical scenario during the last two decades, characterized by a significant increase over time for right and left-heart lesions repair (p < 0.0001, both); while a significant decrease was seen for septal defects repair (p < 0.0001) and transplant (p = 0.03). Overall hospital mortality was 3% (n = 622/20,602 patients) and was stable over time. An inverse relationship between mortality and the number of patients operated in each center (p < 0.0001) was observed.

N-terminal pro-brain natriuretic peptide serum levels reflect attrition of the Fontan circulation.

OBJECTIVE: N-terminal pro-brain natriuretic peptide has an established role in the diagnosis and prognosis of heart failure. In Fontan patients, this peptide is often increased, but its diagnostic value in this particular non-physiologic, univentricular circulation is unclear. We investigated whether N-terminal pro-brain natriuretic peptide represents ventricular function or other key variables in Fontan patients. METHODS AND RESULTS: Ninety-five consecutive Fontan patients ≥10 years old who attended the outpatient clinic of the Center for Congenital Heart Diseases in 2012-2013 were included. Time since Fontan completion was 16 ± 9 years. Median N-terminal pro-brain natriuretic peptide was 114 (61-264) ng/l and was higher than gender-and age-dependent normal values in 54% of the patients. Peptide Z-scores were higher in patients in NYHA class III/IV compared to those in class I/II, but did not correlate with ventricular function assessed by MRI and echocardiography, nor with peak exercise capacity. Instead, peptide Z-scores significantly correlated with follow-up duration after Fontan completion (p < 0.001), right ventricular morphology (p = 0.004), indexed ventricular mass (p = 0.001), and inferior caval vein diameter (p < 0.001) (adjusted R2 = 0.615). CONCLUSIONS: N-terminal pro-brain natriuretic peptide levels in Fontan patients correlate with functional class, but do not necessarily indicate ventricular dysfunction. Increased peptide levels were associated with a longer existence of the Fontan circulation, morphologic ventricular characteristics, and signs of increased systemic venous congestion. Since the latter are known to be key determinants of the performance of the Fontan circulation, these findings suggest increase in N-terminal pro-brain natriuretic peptide levels to indicate attrition of the Fontan circulation, independent of ventricular function.

GDF-15 (Growth Differentiation Factor 15) Is Associated With Hospitalization and Mortality in Patients With a Fontan Circulation.

Background We investigated serial serum levels of GDF-15 (growth differentiation factor 15) in Fontan patients and their relation to outcome. Methods and Results In this single-center prospective study of consecutive Fontan patients, serial serum GDF-15 measurement and clinical assessment was done at baseline (n=81) and after 2 years (n=51). The association between GDF-15 and the combined end point of all-cause mortality, heart transplant listing, and Fontan-related hospitalization was investigated. Median age at baseline was 21 years (interquartile range: 15-28 years). Median GDF-15 serum levels at baseline were 552 pg/mL (interquartile range: 453-729 pg/mL). GDF-15 serum levels correlated positively with age, age at Fontan initiation, New York Heart Association class, and serum levels of NT-proBNP (N-terminal pro-B-type natriuretic peptide) and É£GT (γ-glutamyltransferase) and negatively with exercise capacity. During a median follow-up of 4.8 years (interquartile range: 3.3-5.5 years), the combined end point occurred in 30 patients (37%). Multivariate Cox regression showed that patients with the highest baseline GDF-15 (n=20, defined as the upper quartile) had a higher risk of hospitalization or death than the lowest 3 quartiles (hazard ratio [HR], 2.76; 95% CI, 1.27-6.00; P=0.011). After 2 years of follow-up, patients in whom serum level of GDF-15 increased to >70 pg/mL (n=13) had a higher risk of hospitalization or death than the lowest 3 quartiles (HR, 2.69; 95% CI, 1.03-6.99; P=0.043). Conclusions In Fontan patients, elevated serum levels of GDF-15 are associated with worse functional status and predict Fontan-related events. Furthermore, serial measurements showed that an increase in GDF-15 serum level was associated with increased risk for adverse outcome.