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The Residual Lesion Score is a novel tool for assessing the achievement of surgical objectives in congenital heart surgery based on widely available clinical and echocardiographic characteristics. This article describes the methodology used to develop the Residual Lesion Score from the previously developed Technical Performance Score for five common congenital cardiac procedures using the RAND Delphi methodology.
A panel of 11 experts from the field of paediatric and congenital cardiology and cardiac surgery, 2 co-chairs, and a consultant were assembled to review and comment on validity and feasibility of measuring the sub-components of intraoperative and discharge Residual Lesion Score for five congenital cardiac procedures. In the first email round, the panel reviewed and commented on the Residual Lesion Score and provided validity and feasibility scores for sub-components of each of the five procedures. In the second in-person round, email comments and scores were reviewed and the Residual Lesion Score revised. The modified Residual Lesion Score was scored independently by each panellist for validity and feasibility and used to develop the “final” Residual Lesion Score.
The Residual Lesion Score sub-components with a median validity score of ≥7 and median feasibility score of ≥4 that were scored without disagreement and with low absolute deviation from the median were included in the “final” Residual Lesion Score.
Using the RAND Delphi methodology, we were able to develop Residual Lesion Score modules for five important congenital cardiac procedures for the Pediatric Heart Network’s Residual Lesion Score study.
To describe the epidemiology of severe bleeding in the immediate post-operative period in children who undergo cardiopulmonary bypass surgery using the Bleeding Assessment Scale for critically Ill Children (BASIC).
Retrospective cohort study in a paediatric ICU from 2015 to 2020.
356 children were enrolled; 59% were male with median (IQR) age 2.1 (0.5–8) years. Fifty-seven patients (16%) had severe bleeding in the first 24 hours post-operatively. Severe bleeding was observed more frequently in younger and smaller children with longer bypass and cross-clamp times (p-values <0.001), in addition to higher surgical complexity (p = 0.048). Those with severe bleeding received significantly more red blood cells, platelets, plasma, and cryoprecipitate in the paediatric ICU following surgery (all p-values <0.001). No laboratory values obtained on paediatric ICU admission were able to predict severe post-operative bleeding. Those with severe bleeding had significantly less paediatric ICU-free days (p = 0.010) and mechanical ventilation-free days (p = 0.013) as compared to those without severe bleeding.
Applying the BASIC definition to our cohort, severe bleeding occurred in 16% of children in the first day following cardiopulmonary bypass. Severe bleeding was associated with worse clinical outcomes. Standard laboratory assays do not predict bleeding warranting further study of available laboratory tests.
Body mass index, race/ethnicity, and payer status are associated with operative mortality in congenital heart disease (CHD). Interactions between these predictors and impacts on longer term outcomes are less well understood. We studied the effect of body mass index, race/ethnicity, and payer on 1-year outcomes following elective CHD surgery and tested the degree to which race/ethnicity and payer explained the effects of body mass index. Patients aged 2–25 years who underwent elective CHD surgery at our centre from 2010 to 2017 were included. We assessed 1-year unplanned cardiac re-admissions, re-interventions, and mortality. Step-wise, multivariable logistic regression was performed.
Of the 929 patients, 10.4% were underweight, 14.9% overweight, and 8.5% obese. Non-white race/ethnicity comprised 40.4% and public insurance 29.8%. Only 0.5% died prior to hospital discharge with one additional death in the first post-operative year. Amongst patients with continuous follow-up, unplanned re-admission and re-intervention rates were 14.7% and 12.3%, respectively. In multivariable analyses adjusting for surgical complexity and surgeon, obese, overweight, and underweight patients had higher odds of re-admission than normal-weight patients (OR 1.40, p = 0.026; OR 1.77, p < 0.001; OR 1.44, p = 0.008). Underweight patients had more than twice the odds of re-intervention compared with normal weight (OR 2.12, p < 0.001). These associations persisted after adjusting for race/ethnicity, payer, and surgeon.
Pre-operative obese, overweight, and underweight body mass index were associated with unplanned re-admission and/or re-intervention 1-year following elective CHD surgery, even after accounting for race/ethnicity and payer status. Body mass index may be an important modifiable risk factor prior to CHD surgery.
Hypoplastic left heart syndrome is the most expensive birth defect managed in the United States, with a 5-year survival rate below 70%. Increasing evidence suggests that hospital volumes are inversely associated with mortality for infants with single ventricles undergoing stage 1 surgical palliation. Our aim was to examine the relative effects of surgeon and institutional volumes on outcomes and resource utilisation for these children.
A retrospective study was conducted using the Pediatric Health Information System database to examine the effects of the number of procedures performed per surgeon and per centre on mortality, costs, and post-operative length of stay for infants undergoing Risk Adjustment for Congenital Heart Surgery risk category six operations at tertiary-care paediatric hospitals, from 1 January, 2004 to 31 December, 2013. Multivariable modelling was used, adjusting for patient and institutional characteristics. Gaussian kernel densities were constructed to show the relative distributions of the effects of individual institutions and surgeons, before and after adjusting for the number of cases performed.
A total of 2880 infants from 35 institutions met the inclusion criteria. Mortality was 15.0%. Median post-operative length of stay was 24 days (IQR 14–41). Median standardized inpatient hospital costs were $156,000 (IQR $108,000–$248,000) in 2013 dollars. In the multivariable analyses, higher institutional volume was inversely associated with mortality (p=0.001), post-operative length of stay (p=0.004), and costs (p=0.001). Surgeon volume was associated with none of the measured outcomes. Neither institutional nor surgeon volumes explained much of the wide variation in outcomes and resource utilization observed between institutions and between surgeons.
Increased institutional – but not surgeon – volumes are associated with reduced mortality, post-operative length of stay, and costs for infants undergoing stage 1 palliation.
A complication is an event or occurrence that is associated with a disease or a healthcare intervention, is a departure from the desired course of events, and may cause, or be associated with, suboptimal outcome. A complication does not necessarily represent a breech in the standard of care that constitutes medical negligence or medical malpractice. An operative or procedural complication is any complication, regardless of cause, occurring (1) within 30 days after surgery or intervention in or out of the hospital, or (2) after 30 days during the same hospitalization subsequent to the operation or intervention. Operative and procedural complications include both intraoperative/intraprocedural complications and postoperative/postprocedural complications in this time interval.
The MultiSocietal Database Committee for Pediatric and Congenital Heart Disease has set forth a comprehensive list of complications associated with the treatment of patients with congenital cardiac disease, related to cardiac, pulmonary, renal, haematological, infectious, neurological, gastrointestinal, and endocrinal systems, as well as those related to the management of anaesthesia and perfusion, and the transplantation of thoracic organs. The objective of this manuscript is to examine the definitions of operative morbidity as they relate specifically to the cardiac system. These specific definitions and terms will be used to track morbidity associated with surgical and transcatheter interventions and other forms of therapy in a common language across many separate databases.
The MultiSocietal Database Committee for Pediatric and Congenital Heart Disease has prepared and defined a near-exhaustive list of cardiac complications, including intraoperative complications and cardiopulmonary bypass-related complications. These cardiac complications are presented in the following subgroups:
1) Cardiac (general)
2) Cardiac – Metabolic
3) Cardiac – Residual and Recurrent cardiac lesions
5) Cardiopulmonary bypass and Mechanical circulatory support, and
Within each subgroup, complications are presented in alphabetical order. Clinicians caring for patients with congenital cardiac disease will be able to use this list for databases, quality improvement initiatives, reporting of complications, and comparing strategies for treatment.
This review discusses the historical aspects, current state of the art, and potential future advances in the areas of nomenclature and databases for the analysis of outcomes of treatments for patients with congenitally malformed hearts. We will consider the current state of analysis of outcomes, lay out some principles which might make it possible to achieve life-long monitoring and follow-up using our databases, and describe the next steps those involved in the care of these patients need to take in order to achieve these objectives. In order to perform meaningful multi-institutional analyses, we suggest that any database must incorporate the following six essential elements: use of a common language and nomenclature, use of an established uniform core dataset for collection of information, incorporation of a mechanism of evaluating case complexity, availability of a mechanism to assure and verify the completeness and accuracy of the data collected, collaboration between medical and surgical subspecialties, and standardised protocols for life-long follow-up.
During the 1990s, both The European Association for Cardio-Thoracic Surgery and The Society of Thoracic Surgeons created databases to assess the outcomes of congenital cardiac surgery. Beginning in 1998, these two organizations collaborated to create the International Congenital Heart Surgery Nomenclature and Database Project. By 2000, a common nomenclature, along with a common core minimal dataset, were adopted by The European Association for Cardio-Thoracic Surgery and The Society of Thoracic Surgeons, and published in the Annals of Thoracic Surgery. In 2000, The International Nomenclature Committee for Pediatric and Congenital Heart Disease was established. This committee eventually evolved into the International Society for Nomenclature of Paediatric and Congenital Heart Disease. The working component of this international nomenclature society has been The International Working Group for Mapping and Coding of Nomenclatures for Paediatric and Congenital Heart Disease, also known as the Nomenclature Working Group. By 2005, the Nomenclature Working Group crossmapped the nomenclature of the International Congenital Heart Surgery Nomenclature and Database Project of The European Association for Cardio-Thoracic Surgery and The Society of Thoracic Surgeons with the European Paediatric Cardiac Code of the Association for European Paediatric Cardiology, and therefore created the International Paediatric and Congenital Cardiac Code, which is available for free download from the internet at [http://www.IPCCC.NET].
This common nomenclature, the International Paediatric and Congenital Cardiac Code, and the common minimum database data set created by the International Congenital Heart Surgery Nomenclature and Database Project, are now utilized by both The European Association for Cardio-Thoracic Surgery and The Society of Thoracic Surgeons. Between 1998 and 2007 inclusive, this nomenclature and database was used by both of these two organizations to analyze outcomes of over 150,000 operations involving patients undergoing surgical treatment for congenital cardiac disease.
Two major multi-institutional efforts that have attempted to measure the complexity of congenital heart surgery are the Risk Adjustment in Congenital Heart Surgery-1 system, and the Aristotle Complexity Score. Current efforts to unify the Risk Adjustment in Congenital Heart Surgery-1 system and the Aristotle Complexity Score are in their early stages, but encouraging. Collaborative efforts involving The European Association for Cardio-Thoracic Surgery and The Society of Thoracic Surgeons are under way to develop mechanisms to verify the completeness and accuracy of the data in the databases. Under the leadership of The MultiSocietal Database Committee for Pediatric and Congenital Heart Disease, further collaborative efforts are ongoing between congenital and paediatric cardiac surgeons and other subspecialties, including paediatric cardiac anaesthesiologists, via The Congenital Cardiac Anesthesia Society, paediatric cardiac intensivists, via The Pediatric Cardiac Intensive Care Society, and paediatric cardiologists, via the Joint Council on Congenital Heart Disease and The Association for European Paediatric Cardiology.
In finalising our review, we emphasise that analysis of outcomes must move beyond mortality, and encompass longer term follow-up, including cardiac and non cardiac morbidities, and importantly, those morbidities impacting health related quality of life. Methodologies must be implemented in these databases to allow uniform, protocol driven, and meaningful, long term follow-up.
This review includes a brief discussion, from the perspective of cardiac surgeons, of the rationale for creation and maintenance of multi-institutional databases of outcomes of congenital heart surgery, together with a history of the evolution of such databases, a description of the current state of the art, and a discussion of areas for improvement and future expansion of the concept. Five fundamental areas are reviewed: nomenclature, mechanism of data collection and storage, mechanisms for the evaluation and comparison of the complexity of operations and stratification of risk, mechanisms to ensure the completeness and accuracy of the data, and mechanisms for expansion of the current capabilities of databases to include comparison and sharing of data between medical subspecialties. This review briefly describes several European and North American initiatives related to databases for pediatric and congenital cardiac surgery the Congenital Database of The European Association for Cardio-Thoracic Surgery, the Congenital Database of The Society of Thoracic Surgeons, the Pediatric Cardiac Care Consortium, and the Central Cardiac Audit Database in the United Kingdom. Potential means of approaching the ultimate goal of acquisition of long-term follow-up data, and input of this data over the life of the patient, are also considered.
The International Consortium for Evidence-Based Perfusion (www.bestpracticeperfusion.org) is a collaborative partnership of societies of perfusionists, professional medical societies, and interested clinicians, whose aim is to promote the continuous improvement of the delivery of care and outcomes for patients undergoing extracorporeal circulation. Despite the many advances made throughout the history of cardiopulmonary bypass, significant variation in practice and potential for complication remains. To help address this issue, the International Consortium for Evidence-Based Perfusion has joined the Multi-Societal Database Committee for Pediatric and Congenital Heart Disease to develop a list of complications in congenital cardiac surgery related to extracorporeal circulation conducted via cardiopulmonary bypass, extracorporeal membrane oxygenation, or mechanical circulatory support devices, which include ventricular assist devices and intra-aortic balloon pumps. Understanding and defining the complications that may occur related to extracorporeal circulation in congenital patients is requisite for assessing and subsequently improving the care provided to the patients we serve. The aim of this manuscript is to identify and define the myriad of complications directly related to the extracorporeal circulation of congenital patients.
A large body of literature devoted to “patient safety” and error prevention exists and utilizes a nomenclature that can be applied specifically to the field of congenital cardiac disease and aid in the goals of increasing the safety of patients, decreasing medical error, minimizing mortality and morbidity, and evaluating quality of care. The purpose of this manuscript is to suggest and document a quality of health care taxonomy and the appropriate application of this nomenclature of “patient safety” to the specialty of congenital cardiac disease, with special emphasis on the following ten terms: morbidity, complication, medical error, adverse event, harm, near miss, iatrogenesis, iatrogenic complication, medical injury, and sentinel event. Each of these terms is commonly utilized in the medical literature without universal agreement on their meaning and relationship. It is our hope that the standardization of the definitions of these terms, as they are applied to the analysis of outcomes of the treatments applied to patients with congenital and paediatric cardiac disease, will facilitate improved methodologies to assess and improve quality of care in our profession.
Variability in practice can be considered to foster clinical innovation, and allow for individualized therapeutic plans and independence of practitioners. The Institute of Medicine, however, has issued a report suggesting that variability in patterns of practice are “illogical”, and should be avoided whenever possible. Perhaps nowhere in the field of congenital cardiac disease is variability in practice more apparent than in the management of hypoplastic left heart syndrome. This review assesses the variability in practice at a large number of centres that manage neonates with hypoplastic left heart syndrome, with an emphasis on practice before, during, and after the first stage of the Norwood sequence of operations. We also suggest changes in future strategies for research.
In March, 2007, colleagues were contacted to respond to an internet-based survey using commercially available software (www.surveymonkey.com) to collect responses about the management practices for neonates with “straight-forward” hypoplastic left heart syndrome. No attempt was made to correlate management practices with any measures of outcome, as neither the practices themselves, nor the outcomes of interest, could be externally validated. Data is reported from 52 centers thought to manage over 1000 neonates with hypoplastic left heart syndrome on an annual basis. The first stage of the Norwood sequence was “recommended” to families by approximately five-sixths (86.5%) of the centres. No centre recommended primary cardiac transplantation, a “hybrid” approach, or non-intervention. In 7 centres (14.5%), it was reported that there was discussion of some or all of the above options, but ultimately the families decided upon the appropriate strategy.
Most centres preferentially used antegrade cerebral perfusion (54%) in contrast to deep hypothermia with circulatory arrest (24%), albeit that 11% of centres used a combination of these techniques and in 9% the support strategy was based on surgeon preference. The source of flow of blood for the lungs following the first stage of reconstruction was also highly variable. Of the 51 centres that responded to the question, 13 (25.5%) were participating in a multi-centric randomized clinical trial comparing the modified Blalock-Taussig shunt to the conduit placed from the right ventricle to the pulmonary arteries, the so-called “Sano” modification. Of the remaining 38 centres, 18 “usually” placed a conduit from the right ventricle to the pulmonary artery, 14 “usually” placed a modified Blalock-Taussig shunt, and at six centres, the decision was made “based upon the preference of the surgeon and/or the cardiologist”. Similarly, significant variability in practice was evident in preoperative management, other surgical strategies, postoperative medical support, monitoring and discharge planning. Other than the randomized clinical trial of shunt type, no other medical or surgical management strategy was currently under investigation in a multi-centric or randomized trial in the centres who responded to the survey.
The survey emphasises the extreme variability in our current practices for treatment of children with hypoplastic left heart syndrome. While there are some areas for which there is consensus in management, the majority of our practices are variable between and within centres. These results emphasize that large multicentric trials and registries are necessary to improve care, and to answer important clinical questions, emphasizing the need to shift from analysis of experiences of single centres to multi-centric and multi-disciplinary collaboration.
Background: This is a review of the experience over 26 year in a single institution with surgical repair of aortopulmonary window. Methods: Between July 1973 and March 1999, 38 patients underwent surgery for aortopulmonary window at a median age of 5 weeks, and with a median weight of 3.9 kg. Median follow-up was 6.6 years, with a range from 0.8 to 26 years. Additional defects were present in 25 (65%) patients, including interruption of the aortic arch in 7, tetralogy of Fallot in 7, ventricular septal defect in 5, functionally univentricular anatomy in 3, aortic coarctation in 2, and anomalous origin of a coronary artery in 1. We approached via an aortotomy in 45%, an incision through the defect in 31%, and using a pulmonary arteriotomy in 24% of patients. Closure was achieved using a single patch in 30 patients (79%). Results: There were 3 (7.9%) in-hospital deaths. Actuarial patient survival was 88% at 10 years. Three patients required reinterventions for stenoses of the great arteries. Freedom from any reintervention was 70% at 10 years. By multivariate analysis, the approach through a pulmonary arteriotomy was shown to result in a higher need for reintervention (p = 0.01). Conclusions: Repair of aortopulmonary window can be done with excellent results. A pulmonary arteriotomy should be avoided.
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