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The Mesenchymal Stromal Cell Delivery through Cardiopulmonary Bypass in Pediatric Cardiac Surgery study is a prospective, open-label, single-centre, dose-escalation phase 1 trial assessing the safety/feasibility of delivering mesenchymal stromal cells to neonates/infants during cardiac surgery. Outcomes will be compared with historical data from a similar population. We aim to define an optimal control group for use in the Mesenchymal Stromal Cell Delivery through Cardiopulmonary Bypass in Pediatric Cardiac Surgery trial.
Consecutive patients who underwent a two-ventricle repair without aortic arch reconstruction within the first 6 months of life between 2015 and 2020 were studied using the same inclusion/exclusion criteria as the Phase 1 Mesenchymal Stromal Cell Delivery through Cardiopulmonary Bypass in Pediatric Cardiac Surgery trial (n = 169). Patients were allocated into one of three diagnostic groups: ventricular septal defect type, Tetralogy of Fallot type, and transposition of the great arteries type. To determine era effect, patients were analysed in two groups: Group A (2015–2017) and B (2018–2020). In addition to biological markers, three post-operative scoring methods (inotropic and vasoactive-inotropic scores and the Pediatric Risk of Mortality-III) were assessed.
All values for three scoring systems were consistent with complexity of cardiac anomalies. Max inotropic and vasoactive-inotropic scores demonstrated significant differences between all diagnosis groups, confirming high sensitivity. Despite no differences in surgical factors between era groups, we observed lower inotropic and vasoactive-inotropic scores in group B, consistent with improved post-operative course in recent years at our centre.
Our studies confirm max inotropic and vasoactive-inotropic scores as important quantitative measures after neonatal/infant cardiac surgery. Clinical outcomes should be compared within diagnostic groupings. The optimal control group should include only patients from a recent era. This initial study will help to determine the sample size of future efficacy/effectiveness studies.
Children with CHD may be at increased risk for epilepsy. While the incidence of perioperative seizures after surgical repair of CHD has been well-described, the incidence of epilepsy is less well-defined. We aim to determine the incidence and predictors of epilepsy in patients with CHD.
Retrospective cohort study of patients with CHD who underwent cardiopulmonary bypass at <2 years of age between January, 2012 and December, 2013 and had at least 2 years of follow-up. Clinical variables were extracted from a cardiac surgery database and hospital records. Seizures were defined as acute if they occurred within 7 days after an inciting event. Epilepsy was defined based on the International League Against Epilepsy criteria.
Two-hundred and twenty-one patients were identified, 157 of whom were included in our analysis. Five patients (3.2%) developed epilepsy. Acute seizures occurred in 12 (7.7%) patients, only one of whom developed epilepsy. Predictors of epilepsy included an earlier gestational age, a lower birth weight, a greater number of cardiac surgeries, a need for extracorporeal membrane oxygenation or a left ventricular assist device, arterial ischaemic stroke, and a longer hospital length of stay.
Epilepsy in children with CHD is rare. The mechanism of epileptogenesis in these patients may be the result of a complex interaction of patient-specific factors, some of which may be present even before surgery. Larger long-term follow-up studies are needed to identify risk factors associated with epilepsy in these patients.
Isomerism, also referred to as “heterotaxy” is a complex set of anatomic and functional perturbations. One of the most obvious manifestations of isomerism is the disturbance of organ arrangement, such that the thoracic organs are no longer asymmetric on the left and right. We report the case of a 14-year-old female in whom exercise-induced dyspnea led to a late diagnosis of left isomerism complicated by Abernethy malformation and portopulmonary hypertension. A comprehensive evaluation revealed two anatomic left lungs and hyparterial bronchi, bilateral left atria, an interrupted inferior caval vein with azygos continuation, multiple spleens, sinus node dysfunction, hepatic hypertrophy with focal nodular hyperplasia, and absence of the portal vein. Pulmonary vasodilator therapy was initiated resulting in clinical improvement. This case exhibits unique features including a late diagnosis of isomerism with Abernethy malformation and portopulmonary hypertension. The patient’s presentation, medical workup, and future treatment emphasise the importance of multidisciplinary care in children with complex multisystem disease. We review the multiple cardiac and extracardiac manifestations of isomerism.
This paper provides specific guidelines for the neurodevelopmental evaluation of children aged birth through 5 years with complex congenital heart disease. There is wide recognition that children with congenital heart disease are at high risk for neurodevelopmental impairments that are first apparent in infancy and often persist as children mature. Impairments among children with complex congenital heart disease cross developmental domains and affect multiple functional abilities. The guidelines provided are derived from the substantial body of research generated over the past 30 years describing the characteristic developmental profiles and the long-term trajectories of children surviving with complex congenital heart conditions. The content and the timing of the guidelines are consistent with the 2012 American Heart Association and the American Academy of Pediatrics scientific statement documenting the need for ongoing developmental monitoring and assessment from infancy through adolescence. The specific guidelines offered in this article were developed by a multidisciplinary clinical research team affiliated with the Cardiac Neurodevelopmental Outcome Collaborative, a not-for-profit organisation established to determine and implement best neurodevelopmental practices for children with congenital heart disease. The guidelines are designed for use in clinical and research applications and offer an abbreviated core protocol and an extended version that expands the scope of the evaluation. The guidelines emphasise the value of early risk identification, use of evidence-based assessment instruments, consideration of family and cultural preferences, and the importance of providing multidimensional community-based services to remediate risk.
In this report, the authors prepared an opinion poll regarding the most important people, events, technologies, concepts, discoveries, and therapies in paediatric cardiology and cardiac surgery. The results were presented in continuous slide show format at the 2017 Seventh World Congress of Pediatric Cardiology & Cardiac Surgery (WCPCCS 2017), Barcelona, Spain. The presentation (under international copyright) is made available herein for educational purposes.
Previous studies suggest that children with congenital cardiac diagnoses report lower quality of life when compared with healthy norms. A few studies have evaluated quality of life specifically in children born with hypoplastic left heart syndrome, a condition requiring several surgeries before age three. The aim of this study was to use an empirically validated and standardised measure – the Pediatric Quality of Life Inventory – to evaluate quality of life in children with hypoplastic left heart syndrome and compare the findings with similar, medically complicated samples.
The parent-report Pediatric Quality of Life Inventory was administered, and demographic information was collected through an internet portal. A total of 121 caregivers of children with hypoplastic left heart syndrome responded. The sample included children aged 2–18 years (M=10.81 years). Independent sample t-tests were used to compare our sample with published norms of healthy children and children with acute or chronic illnesses.
Children with hypoplastic left heart syndrome were rated as having significantly lower overall quality-of-life scores (M=59.69) compared with published norms of children without medical diagnoses (M=83.00) and those with acute (M=78.70) or chronic (M=77.19) illnesses (p<0.001). Children with hypoplastic left heart syndrome complicated by a stroke or seizure (15%) reported the lowest quality of life. The results held for all subscales (p<0.001).
Children with hypoplastic left heart syndrome appear to be a significantly vulnerable population with difficulties in functioning across psychosocial domains and across the age span. Further research is required to facilitate early identification of the need for resources for these children and families, especially for children who experience additional medical complications.
Over the past 20 years, the successes of neonatal and infant surgery have resulted in dramatically changed demographics in ambulatory cardiology. These school-aged children and young adults have complex and, in some cases, previously unexpected cardiac and non-cardiac consequences of their surgical and/or transcatheter procedures. There is a growing need for additional cardiac and non-cardiac subspecialists, and coordination of care may be quite challenging. In contrast to hospital-based care, where inpatient care protocols are common, and perioperative expectations are more or less predictable for most children, ambulatory cardiologists have evolved strategies of care more or less independently, based on their education, training, experience, and individual styles, resulting in highly variable follow-up strategies. We have proposed a combination proactive–reactive collaborative model with a patient’s primary cardiologist, primary-care provider, and subspecialists, along with the patient and their family. The goal is to help standardise data collection in the ambulatory setting, reduce patient and family anxiety, increase health literacy, measure and address the non-cardiac consequences of complex cardiac disease, and aid in the transition to self-care as an adult.
In the domain of paediatric and congenital cardiac care, the stakes are huge. Likewise, the care of these children assembles a group of “A+ personality” individuals from the domains of cardiac surgery, cardiology, anaesthesiology, critical care, and nursing. This results in an environment that has opportunity for both powerful collaboration and powerful conflict. Providers of healthcare should avoid conflict when it has no bearing on outcome, as it is clearly a squandering of individual and collective political capital.
Outcomes after cardiac surgery are now being reported transparently and publicly. In the present era of transparency, one may wonder how to balance the following potentially competing demands: quality healthcare, transparency and accountability, and teamwork and shared decision-making.
An understanding of transparency and public reporting in the domain of paediatric cardiac surgery facilitates the implementation of a strategy for teamwork and shared decision-making. In January, 2015, the Society of Thoracic Surgeons (STS) began to publicly report outcomes of paediatric and congenital cardiac surgery using the 2014 Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHSD) Mortality Risk Model. The 2014 STS-CHSD Mortality Risk Model facilitates description of Operative Mortality adjusted for procedural and patient-level factors.
The need for transparency in reporting of outcomes can create pressure on healthcare providers to implement strategies of teamwork and shared decision-making to assure outstanding results. A simple strategy of shared decision-making was described by Tom Karl and was implemented in multiple domains by Jeff Jacobs and David Cooper. In a critical-care environment, it is not unusual for healthcare providers to disagree about strategies of management of patients. When two healthcare providers disagree, each provider can classify the disagreement into three levels:
• SDM Level 1 Decision: “We disagree but it really does not matter, so do whatever you desire!”
• SDM Level 2 Decision: “We disagree and I believe it matters, but I am OK if you do whatever you desire!!”
• SDM Level 3 Decision: “We disagree and I must insist (diplomatically and politely) that we follow the strategy that I am proposing!!!!!!”
SDM Level 1 Decisions and SDM Level 2 Decisions typically do not create stress on the team, especially when there is mutual purpose and respect among the members of the team. SDM Level 3 Decisions are the real challenge. Periodically, the healthcare team is faced with such Level 3 Decisions, and teamwork and shared decision-making may be challenged. Teamwork is a learned behaviour, and mentorship is critical to achieve a properly balanced approach. If we agree to leave our egos at the door, then, in the final analysis, the team will benefit and we will set the stage for optimal patient care. In the environment of strong disagreement, true teamwork and shared decision-making are critical to preserve the unity and strength of the multi-disciplinary team and simultaneously provide excellent healthcare.
The aim of this review is to describe the current state of knowledge related to neurodevelopmental outcomes and quality of life for children with hypoplastic left heart syndrome and to explore future questions to be answered for this group of children.
Background: We describe the recent results in a large cohort of patients with functionally single ventricle who underwent various modifications of cavopulmonary connections. Methods: Using the database at our institution, we identified all children who underwent cavopulmonary connection operations between June 1995 and June 1997. Demographic data, surgical history, and perioperative course were reviewed. Results: We performed 130 consecutive operations in 113 patients. The procedures included superior cavopulmonary connections in the form of the HemiFontan procedure in 45 instances, and bidirectional Glenn procedures in 11, and bilateral superior cavopulmonary connections in 7. The median age of these patients was 7.0 months. We completed Fontan operations using a fenestrated lateral tunnel on 47 occasions, and using an extracardiac conduit 9 times, 5 of which were fenestrated. A lateral tunnel without fenestration was constructed inone patient. The median age for these procedures was 19.5 months. In the remaining 10 instances, we revised Fontan procedures at a median age of 8 years. Diagnoses included hypoplastic left heart syndrome in 43 patients, double outlet right ventricle in 22, heterotaxy in 13, tricuspid atresia in 13, and a miscellaneous group accounting for the other 22. One death (0.7%) occurred within 30 days of surgery. Clinical seizures occurred in 7 children (5.3%), 6 had no residual neurologic deficits. Atrial pacing was needed in 14 children (10.7%) because of transient junctional rhythm, and 2 received treatment for supraventricular tachycardia. Pleural effusions were diagnosed radiographically after 31 of 130 (24%) procedures. Diuretic therapy resolved the effusion in 21 of these, with only 6 children requiring thoracostomy catheter drainage, and 4 undergoing thoracentesis alone. The median length of stay on the intensive care unit was 2 days, with a range from 1 to 30 days, and median stay in hospital was 6 days, with a range from 3 to 58 days. Conclusion: Mortality and perioperative morbidity after cavopulmonary connections have decreased dramatically in the current era. The long-term results of staged reconstruction for functionally single ventricle, nonetheless, await ongoing study.
Low weight at birth is a risk factor for increased mortality in infants undergoing surgery for congenitally malformed hearts. There has been a trend towards performing surgery in patients early, and for amenable lesions, in a single stage rather than following initial palliative procedures. Our goal was to report on the current incidences of morbidities and mortality in infants born with low weight and undergoing surgery for congenital cardiac disease.
We made a retrospective review of the data from patients meeting our criterions for entry from July, 2000, through July, 2004. The criterions for inclusion were weight at birth less than or equal to 2500 grams, and congenital cardiac malformations requiring surgery during the initial hospitalization. A criterion for exclusion was isolated persistent patency of the arterial duct. We assessed preoperative, intraoperative, and postoperative variables.
We found a total of 105 patients meeting the criterions for inclusion. The median weight at birth was 2130 grams, and median gestational age was 36 weeks. The most common morbidity identified was infections of the blood stream. Infections, and chronic lung disease, were associated with increased length of stay. Survival overall was 76%. Patients with hypoplastic left heart syndrome, or a variant thereof, had the lowest survival, of 62%. The needs for cardiopulmonary resuscitation, or extracorporeal membrane oxygenation, post-operatively were the only factors identified as independent risk factors for mortality.
Patients undergoing surgery during infancy for congenital cardiac disease who are born with low weight have a higher mortality and morbidity than those born with normal weight.
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.
A complication is an event or occurrence associated with a disease or a healthcare intervention, which constitutes 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 an 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 Multi-Societal 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 patients who have received transplantation of the heart, heart and lung(s) or lung(s). These specific definitions and terms will be used to track morbidity associated with transplantation in a common language across many separate databases.
The complications of transplantation are known risks of congenital cardiac surgery. The purpose of this effort is to propose consensus definitions for post-transplant complications following cardiac surgery so that collection of such data can be standardized. Clinicians caring for patients who have had transplantation of thoracic organs will be able to use this list for databases, quality improvement initiatives, reporting of complications, and comparing treatment strategies.
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 endocrine 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 neurological 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.
Although neurological injury and adverse neurodevelopmental outcome can follow procedures for congenital cardiac defects, much of the variability in neurological outcome is now recognized to be more related to patient specific factors rather than procedural factors. Additionally, the recognition of pre and postoperative neurological morbidity requires procedures and imaging modalities that can be resource-intensive to acquire and analyze, and little is known or described about variations in “sampling rate” from centre to centre.
The purpose of this effort is to propose an initial set of consensus definitions for neurological complications following congenital cardiac surgery and intervention. Given the dramatic advances in understanding achieved to date, and those yet to occur, this effort is explicitly recognized as only the initial first step of a process that must remain iterative. This list is a component of a systems-based compendium of complications that may help standardize terminology and possibly enhance the study and quantification of morbidity in patients with congenital cardiac malformations. Clinicians caring for patients with congenital cardiac disease may be able to use this list for databases, initiatives to improve quality, reporting of complications, and comparing strategies of treatment.
Our study was designed to characterize the patterns of growth, in the medium term, of children with functionally univentricular hearts managed with a hemi-Fontan procedure in infancy, followed by a modified Fontan operation in early childhood. Failure of growth is common in patients with congenital cardiac malformations, and may be related to congestive heart failure and hypoxia. Repair of simple lesions appears to reverse the retardation in growth. Palliation of the functionally single ventricular physiology with a staged Fontan operation reduces the adverse effects of hypoxemia and prolonged ventricular volume overload. The impact of this approach on somatic growth is unknown. Retrospectively, we reviewed the parameters of growth of all children with functionally univentricular hearts followed primarily at our institution who had completed a staged construction of the Fontan circulation between January 1990 and December 1995. Measurements were available on all children prior to surgery, and annually for three years following the Fontan operation. Data was obtained on siblings and parents for comparative purposes. The criterions of eligibility for inclusion were satisfied by 65 patients. The mean Z score for weight was-1·5± 1·2 at the time of the hemi-Fontan operation. Weight improved by the time of completion of the Fontan circulation (−0·91 ±0·99), and for the first two years following the Fontan operation, but never normalized. The mean Z scores for height at the hemi-Fontan and Fontan operations were −0·67 ±1·1 and −0·89±1·2 respectively. At most recent follow-up, with a mean age of 6·1 ± 1·3 years, and a mean time from the Fontan operation of 4·4±1·4 years, the mean Z score for height was −1·15 ±1·2, and was significantly less than comparable Z scores for parents and siblings. In our experience, children with functionally univentricular hearts who have been palliated with a Fontan operation are significantly underweight and shorter than the general population and their siblings
The dramatic reduction in surgical mortality associated with repair of congenital heart anomalies in recent decades has been accompanied by a growing recognition of adverse neurologic sequels in some of the survivors. Abnormalities of the central nervous system may be a function of coexisting cerebral abnormalities or acquired events unrelated to surgical management (such as paradoxical embolus, cerebral infection, or effects of chronic cyanosis), but insults to the central nervous system appear to occur most frequently during or immediately after surgery. In particular, techniques of support used during neonatal and infant cardiac surgery—cardiopulmonary bypass, profound hypothermia and circulatory arrest—have been implicated as important causes of cerebral injury. This paper will review the effects of bypass and deep hypothermic circulatory arrest on neurodevelopmental outcome.
Children born with heterotaxy syndromes have poorer outcomes compared with children born with comparable cardiac lesions requiring similar surgical palliation. Heterotaxy has been reported as a separate risk factor for mortality and increased morbidity in a series of Fontan operations reported from single centres. Little is known, however, about the functional state of surviving patients with heterotaxy following a Fontan operation.
In the multicentric cross-sectional study carried out by the Pediatric Heart Network of 546 survivors of the Fontan procedure, the patients, aged from 6 to 18 years, underwent evaluation by echocardiography, exercise testing, electrocardiography, magnetic resonance imaging, and functional health status questionnaires compiled by the patients and their parents. Heterotaxy was identified in 42 patients (8%). Medical and patient characteristics were compared between those with heterotaxy and the remaining 504 patients who did not have heterotaxy.
Patients with heterotaxy had their Fontan procedure performed at a later age, with a median of 3.9 years versus 2.8 years (p = 0.001) and had volume-unloading surgery performed later, at a median age of 1.4 versus 0.9 years (p = 0.008). These patients had significantly different ventricular and atrioventricular valvar morphology, as well as a higher incidence of systemic and pulmonary venous abnormalities. They had a higher incidence of prior surgery to the pulmonary veins, at 21 versus 0.4%. The type of Fontan procedure was different, but no difference was detected in length of stay in hospital, or the number of postoperative complications. Sinus rhythm was less common, at 44 versus 71%, (p = 0.002), and history of atrial arrhythmias more common, at 19 versus 8%, (p = 0.018) in those with heterotaxy. Echocardiography revealed a greater degree atrioventricular valvar regurgitation, lower indexed stroke volume, and greater Tei index. Exercise performance, levels of brain natriuretic peptide in the serum, and summary and domain scores from health status questionnaires, were not different from those not having heterotaxy.
The study illustrates a profile of characteristics, medical history, functional health state, and markers of ventricular performance in patients with heterotaxy after the Fontan procedure. Despite obvious anatomic differences, and some differences in echocardiography and heart rhythm, there were no important differences in exercise performance or functional health state between these patients and other survivors of the Fontan procedure.
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.