The care of adult patients with congenital heart defects in the United States is spotty at best, and needs to improve greatly if the needs of these patients are to be met. The care of American children with congenital heart defects is generally excellent. Pediatric cardiac services are well established and well supported. The care of adults with congenital heart disease (CHD) is well established in only a few American centers. While there are an increasing number of clinics, they are generally poorly resourced with relatively few patients. If located in adult cardiology programs, they are usually minor players. If located in pediatric cardiac programs, they are usually minor players as well. Training programs for adult CHD (ACHD) caregivers are few, informal, and poorly funded. To improve the situation, we need perhaps 25 well-resourced and well-established regional ACHD centers in the United States. We need to stop the loss to care of CHD patients at risk of poor outcomes. We need to educate patients and families about the need for lifelong and skilled surveillance and care. We need to effect an orderly transfer from pediatric to adult care. We need to strengthen the human resource infrastructure of ACHD care through the training and hiring of healthcare professionals of a quality equivalent to those working in the pediatric care environment. We need to demonstrate that adult care is high quality care. We need more high-quality ACHD research. The ACHD community needs to establish its credibility with pediatric cardiac providers, adult cardiology groups, with governments, with professional organizations, and with research funding agencies. Accordingly, there is a need for strong political action on behalf of American ACHD patients. This must be led by patients and families. These efforts should be supported by pediatric cardiologists and children's hospitals, as well as by national professional organizations, governments, and health insurance companies. The goal of this political action should be to see that ACHD patients can receive high-quality lifelong surveillance, that we lose fewer patients to care, and that the staff and other services needed are available nationwide.
The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada; Stollery Children's Hospital, University of Alberta, Edmonton, Alberta, Canada. Electronic address: firstname.lastname@example.org.
Pediatric heart failure (HF) is an important cause of morbidity and mortality in childhood. This article presents guidelines for the recognition, diagnosis, and early medical management of HF in infancy, childhood, and adolescence. The guidelines are intended to assist practitioners in office-based or emergency room practice, who encounter children with undiagnosed heart disease and symptoms of possible HF, rather than those who have already received surgical palliation. The guidelines have been developed using the Grading of Recommendations Assessment, Development and Evaluation (GRADE) methodology, and are accompanied by practical Recommendations for their application in the clinical setting, supplemented by online material. This work does not include Recommendations for advanced management involving ventricular assist devices, or other device therapies.
Ten studies (44 969 newborns, 71 severe defects) evaluating the usefulness of neonatal pulse oximetry (PO) screening in timely detection of congenital heart disease (CHD) were reviewed. PO showed a high specificity (99.9-99.99%), and the overall rate of detection of 15 individual defects with PO was 72% (range 46-100%), exceeding that of the clinical examination 58% (9-86%). Similar results were obtained for cyanotic CHD (89% v 69%, respectively). Without PO, discharge of apparently healthy infants with unknown CHD was 5.5 times and 4.1 times more likely in cyanotic CHD and all serious CHD, respectively. The paper describes the technical and practical details of first day and later screening. Diagnosis is reached earliest with first day screening, but it requires more resources. PO screening is not sensitive enough to serve as an independent screen, but along with the clinical examination it helps minimise the morbidity and mortality associated with discharge without diagnosis. Further research is needed for precise delineation of populations that would benefit from PO screening.