Faculty Bibliography

The purpose of this study was to impact of a clinical pathway on the postoperative management of children undergoing surgical closure of atrial septal defects (ASDs). Three groups of children were studied: group 1 (14 patients), before introduction of an intensive care team, minimally invasive surgery, and the clinical pathway; group 2 (17 patients), after the introduction of the intensive care team and minimally invasive surgical techniques but before the pathway; and group 3 (30 patients), after implementation of the clinical pathway. Average hospital length of stay fell from 118.52 +/- 19.83 hours (4.9 +/- 0.83 days) in group 1 to 95.92 +/- 66.48 hours (3.99 +/-2.77 days) in group 2 and declined further to 54.29 +/- 20.17 hours (2.26 +/- 0.84 days) in group 3 (p <.05). There were statistically significant decreases in laboratory resource utilization (p <.05). The addition of a dedicated intensive care team and utilization of minimally invasive surgical techniques reduced mean length of stay (by 20%) and resource utilization (by 50%). However, only the implementation of the pathway provided the consistency necessary for maximal quality management, cost saving, and reduction in length of stay (additional 44% reduction in mean length of stay and 40% reduction in resource utilization). These results show the incremental advantage of implementing a defined clinical pathway for postoperative management of children with atrial septal defects

This study tested an instrument for measuring health-related quality of life (HRQL) in children with heart disease. HRQL was measured using the New York University Children's Heart Health Survey in a sample of 0- to 20-year-old subjects with heart disease compared with a control group. Heart disease was associated with impairment on all subscales except psychological function. Adolescent self-reports did not differ significantly between the cardiac group and healthy controls in any of the subscales. This instrument may be useful in assessing the impact of various treatment strategies in this population

OBJECTIVE: The assessment of the severity of heart failure in pediatric patients is handicapped by the subjectivity of diagnostic parameters. This study evaluated the feasibility of a new standardized heart failure index, the New York University Pediatric Heart Failure Index (NYU PHFI), to quantify the degree of heart failure in a selected pediatric population.Methods and Results: The index is a weighted, linear combination of scores based on symptoms, physical signs, and medical regimen. Overall, healthy children (n = 12) scored very low (0 to 2) on this index. Mean scores of children (<2 years; mean age, 4.8 months; n = 12) with a left-to-right shunt lesion declined from 11.4 (SD +/- 4.1, P <.001, 2-tailed test) before surgery to 1.8 (SD +/- 1.3) after surgical correction of their cardiac defects. The average inter-observer correlation coefficient was 0.95 (P <.001), despite a wide range of scores. CONCLUSIONS: The NYU PHFI appears to be a reliable and convenient instrument for measuring heart failure severity in children. These initial results support further testing in broader diagnostic and age groups and over longer periods

The impact of left ventricular hypertension on coronary flow patterns in adult patients has been well described. In contrast, few reports exist regarding the association of right coronary flow abnormalities with right ventricular hypertension. The observation of myocardial infarcts, right ventricular hypertension, and abnormal coronary flow pattern-as well as its normalization with relief of right ventricular hypertension-lends support to a causal relation between ventricular hypertension and coronary flow abnormalities

The goals of heart failure therapy have shifted from purely hemodynamic manipulation to a combination of hemodynamic and neurohumoral modulation. Vasodilators with neurohumoral modulatory properties [such as ACE inhibitors (ACEi) and third generation beta-blockers] have become the cornerstone of chronic heart failure therapy. These newer agents have proven to improve morbidity and mortality in adults with chronic heart failure. Pure vasodilators still have a place in the treatment of acute decompensated heart failure and in patients who are intolerant to ACEi or beta-blocker therapy. In decompensated heart failure management, improvement of cardiac output is of paramount importance and restoration of normal hemodynamics takes priority over modulation of cardiac maladaptation. Under these circumstances agents that improve contractility and modify cardiac preload and afterload are used. In the intensive care unit setting inodilators offer the advantage of an added positive inotropic effect. NO donors play an important role when close titration of blood pressure is also needed. It is the purpose of this manuscript to address principles and current practice regarding the use of vasodilators in pediatric heart failure. ACE inhibitors and third generation beta-blockers due to their importance in today's therapeutic approach to heart failure are the focus of more detailed articles in this issue of Progress in Pediatric Cardiology

New therapeutic strategies as well as the development of drugs with more specific targets have been fueled by disappointments in the treatment of adult heart failure. Calcium sensitizers, vesnarinone and angiotensin channel blockers will be addressed in this manuscript. The physiologic and pharmacologic principles that justify their use in the management of heart failure are reviewed. Calcium sensitizers increase myocardial contractility and in part they bypass the adenylyl cyclase cascade, which gives them a more favorable energy profile. Vesnarinone is a quinolinone derivative with ion channel modulation properties, which result in a positive inotropic effect and prolongation of the action potential. In addition vesnarinone has immunomodulatory properties. Angiotensin-converting enzyme inhibitors are the cornerstones for the treatment of heart failure. The discovery of some putative drawbacks to ACE inhibition has challenged this supremacy. Angiotensin receptor blockers have been developed hoping to overcome these deficiencies. Myocardial developmental differences highlight the shortcomings of attempting to extrapolate data on drugs and cellular physiology in adults to children. Studies are needed addressing standards of care, quality of life, morbidity and mortality, neurohumoral activation, its modulation and the consequences of these therapies in pediatric heart failure

Our understanding of the syndrome of heart failure has undergone several revisions, most importantly in the second half of the 20th century. New insights into the mechanisms of diseases offer new, challenging, controversial and sometimes counterintuitive forms of therapy. The development and progression of heart failure results from a complex interplay of hemodynamic and neurohormonal, cellular and genetic factors, rather than simply changes in cardiac function. It is because of this reason that our therapeutic focus can no longer be solely based on supply and demand models. Since the description of the pulsatile nature of the heart function and the flow of blood around a circuit by W. Harvey, numerous new paradigms have been put forward to explain the nature of heart failure. However, no single new model thus far proposed has been able to displace previous ones and successfully dictate therapy. It is the purpose of this manuscript to review the overall current understanding of the heart failure syndrome and how these new ideas may affect our therapeutic approach.

For the most part of this the century heart failure syndrome was understood as a pump failure disorder with hemodynamic consequences stemming from the same myocardial dysfunction. In addition supply and demand theories were used to explain the nature of symptoms. As a result, therapeutic strategies were directed at correcting the abnormal hemodynamic conditions and normalizing the delivery of the much needed nutrients. Improvement of cardiac pump function with inotropic drugs and abnormal circulatory conditions with afterload and preload modifications became therapeutic goals and standards of care. However, while vasodilators and inotropic drugs immediately improved symptoms, hemodynamics and functional status, in the long term they either did not affect or worsen the natural history of heart failure. In pediatrics, this is further complicated by the lack of large scale trials addressing issues pertinent to the particularities that affect heart failure in children. In the late 1980s and 1990s heart failure has evolved into a more complex, multiple and interactive pathophysiologic disorder. Today not only the abnormal hemodynamics but also the biological disorders are pharmacologic targets. The reversal or slowing of myocardial maladaptation has become one of the most important therapeutic goals. With this end in mind therapeutic strategies may seem counterintuitive and paradoxical, such as the use of beta-blockers. This review will address the current thinking and therapeutic modalities used today in the treatment of heart failure syndrome in the adult population. We also discuss some of the issues why we think that these principles can be extrapolated to the pediatric population.

We used continuous intraoperative transesophageal echocardiography (TEE) monitoring to detect intraoperative myocardial ischemia in children after they had been weaned from cardiopulmonary bypass for cardiac surgery. Three pediatric patients are described here to illustrate the usefulness of such TEE monitoring in surgical procedures involving coronary arteries. The indications for intraoperative TEE monitoring and a simplified scheme for immediate qualitative interpretation are discussed