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Complications from systemic inflammation are reported in neonates following exposure to cardiopulmonary bypass. Although the use of asanguinous primes can reduce these complications, in neonates, this can result in significant haemodilution, requiring addition of blood. This study investigates whether the addition of blood after institution of bypass alters the inflammatory response compared with a blood prime. Neonatal swine were randomised into four groups: blood prime, blood after bypass but before cooling, blood after cooling but before low flow, and blood after re-warming. All groups were placed on central bypass, cooled, underwent low flow, and then re-warmed for a total bypass time of 2 hours. Although haematocrit values between groups varied throughout bypass, all groups ended with a similar value. Although they spent time with a lower haematocrit, asanguinous prime groups did not have elevated lactate levels at the end of bypass compared with blood prime. Asanguinous primes released less tumour necrosis factor α than blood primes (p=0.023). Asanguinous primes with blood added on bypass produced less interleukin 10 and tumour necrosis factor α (p=0.006, 0.019). Animals receiving blood while cool also showed less interleukin 10 and tumour necrosis factor α production than those that received blood warm (p=0.026, 0.033). Asanguinous primes exhibited less oedema than blood primes, with the least body weight gain noted in the end cool group (p=0.011). This study suggests that using an asanguinous prime for neonates being cooled to deep hypothermia is practical, and the later addition of blood reduces inflammation.
This case report describes chylous ascites associated with a CHD in a 4-month-old infant. Although atraumatic chylous ascites are a rare clinical finding, the recognition and treatment of chylous ascites influence the timing of cardiac surgery.
Pentalogy of Cantrell is characterised by a combination of severe defects in the middle of the chest including the sternum, diaphragm, heart, and abdominal wall. Mortality rate after cardiac surgery is usually high. We report a successful total correction of the cardiac defects in a case of Pentalogy of Cantrell with a double-outlet right ventricle prior to abdominal wall defect repair.
Loeys-Dietz syndrome is a newly recognized constellation that presents with aortic aneurysm or dissection similar to Marfan’s syndrome. We describe successful surgical treatment in a 2-year-old with the syndrome in whom we performed a valve-sparing replacement of the aortic root because of significant dilation of the aortic root and the ascending aorta.
The diagnosis and treatment for paediatric and congenital cardiac disease has undergone remarkable progress over the last 60 years. Unfortunately, this progress has been largely limited to the developed world. Yet every year approximately 90% of the more than 1,000,000 children who are born with congenital cardiac disease across the world receive either suboptimal care or are totally denied care.
While in the developed world the focus has changed from an effort to decrease post-operative mortality to now improving quality of life and decreasing morbidity, which is the focus of this Supplement, the rest of the world still needs to develop basic access to congenital cardiac care. The World Society for Pediatric and Congenital Heart Surgery [http://www.wspchs.org/] was established in 2006. The Vision of the World Society is that every child born anywhere in the world with a congenital heart defect should have access to appropriate medical and surgical care. The Mission of the World Society is to promote the highest quality comprehensive care to all patients with pediatric and/or congenital heart disease, from the fetus to the adult, regardless of the patient’s economic means, with emphasis on excellence in education, research and community service.
We present in this article an overview of the epidemiology of congenital cardiac disease, the current and future challenges to improve care in the developed and developing world, the impact of the globalization of cardiac surgery, and the role that the World Society should play. The World Society for Pediatric and Congenital Heart Surgery is in a unique position to influence and truly improve the global care of children and adults with congenital cardiac disease throughout the world [http://www.wspchs.org/].
The use of intraoperative echocardiography with Doppler color flow imaging has acquired increased popularity for early assessment of efficacy of repair of congenital heart defects soon after discontinuance of cardiopulmonary bypass. This technology has been found useful also for evaluating the anatomy of the lesion prior to repair. Furthermore, with the use of color flow mapping, additional anomalies, such as patent arterial duct or multiple ventricular septal defects, which might have been overlooked during the initial examination, can be easily diagnosed. Moreover, intraoperative echocardiography provides the quickest and most sensitive method for a surgeon to look for residual atrioventricular valves regurgitation and/or residual atrial or ventricular septal defects as well as for depressed ventricular contractility. Previous studies1–4 have discussed specifically the applicability of intraoperative color flow imaging during the repair of atrioventricular septal defect with a common atrioventricular orifice using both epicardial and transesophageal methodology. Routine intraoperative echocardiography with Doppler color flow imaging has been used since March 1987 at Duke University Medical Center during repair of congenital heart defects. Our aim was to confirm the preoperative dignosis and to assess the quality of repair soon after discontinuance of cardiopulmonary bypass. Our overall experience now includes 612 patients of whom 239 or 39% were less than one year of age at the time of repair. This indicates our tendency to undertake early correction regardless of the age of patients and of the severity of the lesion.
With substantial effort and research devoted to improving surgical techniques and myocardial protection, superb results have been achieved for repair of complex congenital heart defects in children. As a result, investigative efforts now have begun to examine the quality of life for patients surviving these operations. Because these neonates and infants are exposed to severe physiologic extremes of temperature (15–18 °C) and severe alterations from normal perfusion (total circulatory arrest), the nature of long-term neuropsychological outcome has been a prominent concern. Recent preliminary reports suggest that transient and permanent neuropsychologic injury occur in as many as 25% of all infants undergoing hypothermic cardiopulmonary bypass with circulatory arrest.'Since improved surgical techniques have significantly reduced rates of operative mortality and cardiac morbidity, one of the greatest risks remaining for the patient with congenital heart disease may be long term neuropsychologic and developmental abnormalities.
Professionals working in the arena of health care face a variety of challenges as their careers evolve and develop. In this review, we analyze the role of mentorship, learning curves, and balance in overcoming challenges that all such professionals are likely to encounter. These challenges can exist both in professional and personal life.
As any professional involved in health care matures, complex professional skills must be mastered, and new professional skills must be acquired. These skills are both technical and judgmental. In most circumstances, these skills must be learned. In 2007, despite the continued need for obtaining new knowledge and learning new skills, the professional and public tolerance for a “learning curve” is much less than in previous decades. Mentorship is the key to success in these endeavours. The success of mentorship is two-sided, with responsibilities for both the mentor and the mentee. The benefits of this relationship must be bidirectional. It is the responsibility of both the student and the mentor to assure this bidirectional exchange of benefit. This relationship requires time, patience, dedication, and to some degree selflessness. This mentorship will ultimately be the best tool for mastering complex professional skills and maturing through various learning curves. Professional mentorship also requires that mentors identify and explicitly teach their mentees the relational skills and abilities inherent in learning the management of the triad of self, relationships with others, and professional responsibilities.
Up to two decades ago, a learning curve was tolerated, and even expected, while professionals involved in healthcare developed the techniques that allowed for the treatment of previously untreatable diseases. Outcomes have now improved to the point that this type of learning curve is no longer acceptable to the public. Still, professionals must learn to perform and develop independence and confidence. The responsibility to meet this challenge without a painful learning curve belongs to both the younger professionals, who must progress through the learning curve, and the more mature professionals who must create an appropriate environment for learning.
In addition to mentorship, the detailed tracking of outcomes is an essential tool for mastering any learning curve. It is crucial to utilize a detailed database to track outcomes, to learn, and to protect both yourself and your patients. It is our professional responsibility to engage in self-evaluation, in part employing voluntary sharing of data. For cardiac surgical subspecialties, the databases now existing for The European Association for CardioThoracic Surgery and The Society of Thoracic Surgeons represent the ideal tool for monitoring outcomes. Evolving initiatives in the fields of paediatric cardiology, paediatric critical care, and paediatric cardiac anaesthesia will play similar roles.
A variety of professional and personal challenges must be met by all those working in health care. The acquisition of learned skills, and the use of special tools, will facilitate the process of conquering these challenges. Choosing appropriate role models and mentors can help progression through any learning curve in a controlled and protected fashion. Professional and personal satisfaction are both necessities. Finding the satisfactory balance between work and home life is difficult, but possible with the right tools, organization skills, and support system at work and at home. The concepts of mentorship, learning curves and balance cannot be underappreciated.
Surgical treatment of hypoplastic left heart syndrome has generated substantial interest and attention amongst cardiac surgeons since the initial reports from Norwood and his colleagues in 1980.1,2 Initial efforts at most programmes were to create reproducible results, and mortality rates remained high at several institutions throughout the 1980s and 1990s. A recent multi-institutional review demonstrates that the hospital mortality still remains high in numerous centers at the current time.3 Nevertheless, several advances over recent years have led to improved outcomes, and in the best centers, hospital survival now approaches 90%. Survival in successful centers is claimed to relate to the ability of the team to help the patient balance the systemic and pulmonary flows of blood. This ability to balance flow has been enhanced, over recent years, by numerous contributions, including decreasing the size of shunt ordinarily used,4 the use of alpha blockade,5 the rapid deployment of extracorporeal membrane oxygenation,6–8 and various forms of ventilatory manipulation.
The evolution of cardiac surgery has led to increasing emphasis on complete repair of congenital heart defects early in life, nowadays increasingly performed in neonates or small infants. Good results have been achieved because of innovative techniques permitting reconstruction of normal anatomy, and restoration of normal physiology, before either the heart or the patient undergo deleterious adaptation to the congenitally abnormal physiology. Despite the ability surgically to correct complex defects in such small patients, limitations in outcome are sometimes encountered related to the systems necessary for repair. In particular, exposure to cardiopulmonary bypass may present the greatest challenge for these tiny patients.
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