Residency training in transition of youth with childhood-onset chronic disease.

OBJECTIVE: To assess the current medical school training of internal medicine and pediatric residents in transitioning youth with special health care needs from child-oriented to adult-oriented health care. METHODS: We surveyed internal medicine and pediatric residents to assess their preparedness to transition youth with special health care needs to adult-oriented health care. RESULTS: The survey results demonstrated that internal medicine residents felt unprepared to care for most patients with chronic childhood-onset illness; however, most pediatric residents were comfortable caring for such patients. CONCLUSION: Training in chronic childhood-onset illness should be included in internal medicine training programs.

Serum parameters and echocardiographic predictors of death or need for transplant in newborns, children, and young adults with heart failure.

For children admitted with symptomatic heart failure (HF), the risk of death/need for transplantation (D/Tx) is high. Data from adult studies suggest serum measurements, such as percent lymphocytes, are valuable predictors of outcomes. The aim of this study was to identify risk factors for D/Tx in hospitalized pediatric patients with symptomatic HF. Retrospective analysis of children admitted to an academic center from January 1994 to June 2008 with clinical HF was undertaken. The most common cause of HF was dilated cardiomyopathy (58 of 99, 59%). Echocardiographic and serum measurements were collected from admission. Factors independently associated with risk of D/Tx were evaluated by a stepwise multivariate Cox regression model. There were 99 children with 139 hospitalizations. Median age at admission was 3 years (range 0 to 22). Mean systemic ventricular ejection fraction was 23% +/- 11. Risk of D/Tx per hospitalization was 60 of 139 (43%). In multivariate analysis, lymphocytopenia, lower ejection fraction, low serum sodium, and higher serum creatinine were independent predictors of D/Tx. These variables correctly predicted those subjects at risk of D/Tx in 82.1% of cases. Subgroup analysis found that brain natruretic peptide did not improve the model's accuracy markedly. In conclusion, serum measurements (percent lymphocytes, sodium, and creatinine) and echocardiographic assessment routinely obtained at admission are predictive of D/Tx in children hospitalized for HF. Significant lymphocytopenia was predictive of adverse outcomes.

Care of the adult congenital heart disease patient in the United States: a summary of the current system.

With improvements in care, there has been exponential growth in the population of adult congenital heart disease (ACHD) patients. We sought to assess the availability of specialized ACHD care in the United States. We analyzed the Adult Congenital Heart Association's ACHD clinic directory for information on patient volume, provider training, and other characteristics. The information is self-reported and unverified. The ACHD directory included 72 programs in the United States. Across programs, the majority of patients (33%) seen were between 21 and 30 years old. Program directors had between 2 and 50 years (median 15) of ACHD experience and had dedicated between 10 and 100% (median 30%) of their clinical time to ACHD. There were 2,800 ACHD operations performed per year, ranging from 0 to 230 (median 28) per program. There were between 0 and 5 cardiac surgeons (median 2) involved per program. Each surgeon averaged 20 ACHD operations per year. The growing ACHD population is a largely underserved group. Few programs in the United States provide specialized care, and this care is variably conducted within pediatric and/or adult facilities. These data should serve as a stimulus to improving accessibility of services for this vulnerable population.

Octopus papillary muscle associated with a left lateral accessory pathway.

Left ventricular papillary muscle abnormalities are rare malformations. They have been related to significant mitral valve dysfunction and left ventricular midcavitary obstruction. We report our experience with a young adult who presented with palpitations. An echocardiogram on the patient showed an "octopus-like" left ventricular papillary muscle. Subsequent electrophysiologic testing showed evidence of supraventricular tachycardia via a left lateral accessory pathway associated with the abnormal insertion of the papillary muscle attachments.