Recommendations for Centers Performing Pediatric Heart Surgery in the United States.

Care and outcomes for the more than 40,000 patients undergoing pediatric and congenital heart surgery in the United States annually are known to vary widely. While consensus recommendations have been published across numerous fields as one mechanism to promote a high level of care delivery across centers, it has been more than two decades since the last pediatric heart surgery recommendations were published in the United States. More recent guidance is lacking, and collaborative efforts involving the many disciplines engaged in caring for these children have not been undertaken to date. The present initiative brings together professional societies spanning numerous care domains and congenital cardiac surgeons, pediatric cardiologists, nursing, and other healthcare professionals from diverse programs around the country to develop consensus recommendations for United States centers. The focus of this initial work is on pediatric heart surgery, and it is recommended that future efforts focus in detail on the adult congenital population. We describe the background, rationale, and methodology related to this collaborative effort, and recommendations put forth for Essential Care Centers (essential services necessary for any program), and Comprehensive Care Centers (services to optimize comprehensive and high-complexity care), encompassing structure, process, and outcome metrics across 14 domains.

Recommendations for centers performing pediatric heart surgery in the United States.

Care and outcomes for the more than 40,000 patients undergoing pediatric and congenital heart surgery in the United States annually are known to vary widely. While consensus recommendations have been published across numerous fields as one mechanism to promote a high level of care delivery across centers, it has been more than two decades since the last pediatric heart surgery recommendations were published in the United States. More recent guidance is lacking, and collaborative efforts involving the many disciplines engaged in caring for these children have not been undertaken to date. The present initiative brings together professional societies spanning numerous care domains and congenital cardiac surgeons, pediatric cardiologists, nursing, and other healthcare professionals from diverse programs around the country to develop consensus recommendations for United States centers. The focus of this initial work is on pediatric heart surgery, and it is recommended that future efforts focus in detail on the adult congenital population. We describe the background, rationale, and methodology related to this collaborative effort, and recommendations put forth for Essential Care Centers (essential services necessary for any program), and Comprehensive Care Centers (services to optimize comprehensive and high-complexity care), encompassing structure, process, and outcome metrics across 14 domains.

Recommendations for Centers Performing Pediatric Heart Surgery in the United States.

Care and outcomes for the more than 40,000 patients undergoing pediatric and congenital heart surgery in the United States annually are known to vary widely. While consensus recommendations have been published across numerous fields as one mechanism to promote a high level of care delivery across centers, it has been more than two decades since the last pediatric heart surgery recommendations were published in the United States. More recent guidance is lacking, and collaborative efforts involving the many disciplines engaged in caring for these children have not been undertaken to date. The present initiative brings together professional societies spanning numerous care domains and congenital cardiac surgeons, pediatric cardiologists, nursing, and other healthcare professionals from diverse programs around the country to develop consensus recommendations for United States centers. The focus of this initial work is on pediatric heart surgery, and it is recommended that future efforts focus in detail on the adult congenital population. We describe the background, rationale, and methodology related to this collaborative effort, and recommendations put forth for Essential Care Centers (essential services necessary for any program), and Comprehensive Care Centers (services to optimize comprehensive and high-complexity care), encompassing structure, process, and outcome metrics across 14 domains.

Caring for adults with congenital cardiac disease: successes and challenges for 2007 and beyond.

Patients with congenital cardiac disease require lifelong medical care. Current challenges that face practitioners who care for adults with congenital heart disease include identifying the best location for procedures, which could be a children's hospital, an adult hospital, or a tertiary care facility; providing appropriate antenatal management of pregnant women with congenitally malformed hearts, and continuing this care in the peripartum period; and securing the infrastructure and expertise of the non-cardiac subspecialties, such as nephrology, hepatology, pulmonary medicine, and haematology. The objectives of this review are to outline the common problems that confront this population of patients and the medical community, to identify challenges encountered in establishing a programme for care of adults with congenitally malformed hearts, and to review the spectrum of disease and operations that have been identified in a high volume tertiary care centre for adult patients with congenital cardiac disease. Three chosen examples of the fundamental problems facing the practitioner and patient in the United States of America in 2007 are the neglected patient with congenital cardiac disease, weak infrastructure for adults with congenital cardiac disease, and family planning and management of pregnancy for patients with congenital cardiac disease. Patients with adult congenital cardiac disease often do not receive appropriate surveillance. Three fundamental reasons for this problem are, first, that most adults with congenitally malformed hearts have been lost to follow-up by specialists, and are either receiving community care or no care at all. Second, patients and their families have not been educated about their malformed hearts, what to expect, and how to protect their interests most effectively. Third, adult physicians have not been educated about the complexity of the adult with a congenitally malformed heart. This combination can be fatal for adults with complications related to their congenitally malformed heart, or its prior treatment. Two solutions would improve surveillance and care for the next generation of patients coming out of the care of paediatric cardiologists. The first would be to educate patients and their families during childhood and adolescence. They would learn the names of the diagnoses and treatments, the problems they need to anticipate and avoid, the importance of expert surveillance, career and family planning information, and appropriate self-management. The second solution would be to encourage an orderly transfer of patients from paediatric to adult practice, usually at about 18 years of age, and at the time of graduation from high school. Clinics for adults with congenital cardiac disease depend upon multidisciplinary collaboration with specialties in areas such as congenital cardiac imaging, diagnostic and interventional catheterization, congenital cardiac surgery and anaesthesia, heart failure, transplantation, electrophysiology, reproductive and high risk pregnancy services, genetics, pulmonary hypertension, hepatology, nephrology, haematology, and others. None of these services are easily available "off the rack", although with time, experience, and determination, these services can develop very well. Facilities with experienced personnel to provide competent care for adults with congenital cardiac disease are becoming increasingly available. Parents and patients should learn that these facilities exist, and be directed to one by their paediatric caregivers when the time comes for transition to adult care. With the steady increase in the number of adults with congenital heart disease, an ever increasing number of women with such disease are becoming pregnant. Services are not widely available to assess competently and plan a pregnancy for those with more complex disease. It is essential to have a close interplay between the obstetrician, the adult congenital cardiologist, the fetal medicine perinatologist, and neonatologist. In both a community based programme and a tertiary care centre, the nuances and complexities of congenital cardiac anatomy, coupled with the high probability of previous operation during childhood, makes the trained congenital cardiothoracic surgeon best suited to deal with the surgical needs of this growing population. It is clear that the majority of adults with congenital heart disease are not "cured", but require lifelong comprehensive care from specialists who have expertise in this complex arena. There is a growing cadre of healthcare professionals dedicated to improving the care of these patients. More information has become available about their care, and will be improved upon in the next decade. With the support of the general paediatric and paediatric cardiologic communities, and of the Adult Congenital Heart Association, and with the persistence of the providers of care for adults with congenital cardiac disease currently staffing clinics, the care of these patients should become more secure in the next decade as we mature our capabilities.

A prospective, randomized platelet-function study of heparinized oxygenators and cardiotomy suction.

OBJECTIVE: The purpose of this study was to determine if substitution of a heparin-coated oxygenator and salvaged autologous blood for cardiotomy suction would improve platelet function. DESIGN: A prospective, randomized trial. SETTING: A large academic medical center. PARTICIPANTS: Sixty adult patients undergoing coronary artery bypass graft surgery with cardiopulmonary bypass (CPB). INTERVENTIONS: Patients were randomized into 1 of 4 groups in a 2 x 2 factorial design by oxygenator (heparinized v nonheparinized) and blood salvage during CPB (cardiotomy suction v salvaged autologous blood). MEASUREMENTS AND MAIN RESULTS: Primary outcome measures were platelet function, glass-bead retention, platelet dense-body adenosine triphosphate secretion, platelet-rich plasma (PRP) aggregometry, Plateletworks platelet-function analyzer (Helena Laboratories Corp, Allen Park, MI), and platelet count. Secondary outcome measures were chest-tube drainage and allogeneic blood transfusion requirements. All platelet-function tests except thrombin-receptor activator peptide-induced PRP aggregometry showed a reduction in platelet function during and immediately after CPB (all p < 0.05). The only statistically significant difference in platelet-function tests between the groups was the glass-bead assay at 5 minutes before discontinuation of CPB (p < 0.05). This difference resolved 10 minutes after protamine administration. There were no differences between the groups in the amount of blood transfused, chest-tube drainage, and routine laboratory test results. CONCLUSIONS: The authors concluded that the effects of these changes to the CPB circuit were small and inconsequential in this cohort of patients.

Supraventricular tachyarrhythmias in Ebstein anomaly: management and outcome.

OBJECTIVE: This study was undertaken to review the role of electrophysiology testing and to determine the early and late results of medical and surgical management of supraventricular tachyarrhythmias in Ebstein anomaly. METHODS: We reviewed 130 patients between 1990 and 2001 with Ebstein anomaly and history of tachyarrhythmia with a median age of 25 years (mean age 27.5 years); 106 underwent electrophysiologic testing and 24 had documented atrial flutter or fibrillation. We excluded 21 patients: negative results of testing (n = 18), ventricular tachycardia (n = 2), and junctional tachycardia (n = 1). The remaining 109 patients had more than one mechanism: accessory pathway-mediated tachycardia (n = 49), atrioventricular nodal reentrant tachycardia (n = 10), and atrial flutter/fibrillation (n = 70). RESULTS: Eighty-three patients underwent at least one arrhythmia procedure combined with surgery for Ebstein anomaly. Early mortality was 4.8%. Forty-one patients underwent surgical ablation of an accessory pathway without recurrent accessory pathway-mediated tachycardia at a mean follow-up of 48 months. Seven patients underwent surgical perinodal cryoablation for atrioventricular nodal reentrant tachycardia without recurrence at a mean follow-up of 57 months. Forty-eight patients underwent surgical intervention for atrial flutter or fibrillation (right-sided maze procedure, n = 38, and cryoablation of the atrial isthmus, n = 10). Freedom from recurrent atrial flutter or fibrillation was 75% at a mean follow-up of 34 months. CONCLUSION: Concomitant arrhythmia procedures can be performed without increase in early mortality and should be added to Ebstein repairs for all patients who have supraventricular tachyarrhythmias. Surgical procedures for accessory pathway-mediated tachycardia and atrioventricular nodal reentrant tachycardia give excellent (100%) freedom from recurrence of those arrhythmias. Surgical intervention for atrial flutter/fibrillation yields freedom from late recurrence in 75% of cases.