Effect of repair strategy on hospital cost for infants with tetralogy of Fallot.

OBJECTIVE: This study compares the total hospital cost (HC) for one-stage versus "two-stage" repair of tetralogy of Fallot (TOF) in infants younger than 1 year of age. SUMMARY BACKGROUND DATA: Total (one-stage) correction of TOF is now being performed with excellent results in infancy. Alternatively, a two-stage approach, with palliation of infants in the first year of life, followed by complete repair at a later time can be used. In some institutions, the two-stage approach is standard practice for infants younger than 1 year of age or is used selectively in patients with an anomalous coronary artery across the right ventricular outflow tract (RVOT), "small pulmonary arteries," multiple congenital anomalies, critical illnesses (CI), which increase the risk of bypass (e.g., sepsis or DIC), or severe hypercyanotic spells (HS) at the time of presentation. The cost implications of these two approaches are unknown. METHODS: The authors reviewed 22 patients younger than 1 year of age who underwent repair of TOF at their institution between 1993 and 1995. Eighteen patients had one-stage (1 degree) repair (mean age, 3.4 +/- 3.1 months; range, 3 days-9 months) and 4 patients were treated by a staged approach with initial palliation (1.6 +/- 0.4 month; range, 1.5-2 months) followed by later repair (14.75 +/- 1.5 months; range, 13-16 months). The reasons for palliation were severe HS at time of presentation (two patients), anomalous coronary artery (one patient) and CI (one patient). In the 18 patients undergoing 1 degree repair, 3 (16.6%) presented with HS, 6 (33.3%) had a transanular repair, and 6 (33.3%) were able to be repaired through an entirely transatrial approach (youngest patient, 1.5 months). The HC (1996 dollars) and hospital length of stay (LOS; days) were evaluated for all patients. The HCs were calculated using transition I, which is a cost accounting system used by our medical center since July 1992. Transition I provides complete data on all direct and indirect hospital-based, nonprofessional costs. RESULTS: There was no mortality in either group. The group undergoing 1 degree repair had an average LOS of 14.5 +/- 11.2 days compared to an average LOS for palliation of 14 +/- 6.4 days. When the palliated group returned for complete repair, the average LOS was 28.8 +/- 25 days, yielding a total LOS for the two-stage strategy of 43 +/- 30.8 days (p = 0.003 compared to 1 degree repair). The HC for 1 degree repair was $32,541 +/- $15,968 compared to $25,737 +/- $1900 for palliation (p = not significant compared to 1 degree repair) and $54,058 +/- $39,395 for subsequent complete repair (p = not significant compared to 1 degree repair) (total two-stage repair HC = $79,795 +/- $40,625; p = 0.001 compared to 1 degree repair). The LOS and HC for the two-stage group combine a total of palliation plus later repair and, as such, reflect two separate hospitalizations and convalescent periods. To eliminate cost outliers, a best-case analysis was performed by eliminating 50% of patients from each group. Using this analysis, the two-stage approach resulted in an average (total) LOS of 16.5 +/- 2.1 days compared to 8.5 +/- 1.4 days for the 1 degree group. Total cost for the two-stage strategy in this best-case group was $44,660 +/- $3645 compared to $22,360 +/- $3331 for 1 degree repair (p = 0.00001). CONCLUSIONS: The data from this review show that palliation alone generates HC similar to that from 1 degree infant repair of TOF, and total combined HC and LOS for palliation plus eventual repair of TOF (two-stage approach) are significantly higher than from 1 degree repair. Furthermore, these data do not include additional costs for care delivered between palliation and repair (e.g., outpatient visits, cardiac catheterization, serial echocardiography). Although there may be occasions when a strategy using initial palliation followed by later repair may seem prudent, the cost is clearly higher and use of health care resources greater.

Late conduit occlusion after modified Fontan procedure with classic Glenn shunt.

A number of patients with complex congenital heart disease who were initially palliated with a classic Glenn shunt subsequently underwent modified Fontan procedures. This report discusses the operative management of complete occlusion of right atrial-to-right ventricular conduits in patients with patent classic Glenn shunts. The literature is reviewed regarding similar patients, and alternative treatment strategies are discussed.

Defect in fatty acid oxidation: laboratory and pathologic findings in a patient.

The clinical, laboratory, and pathologic findings in a patient with a previously undescribed deficiency in fatty acid oxidation are summarized. The patient had a fatal defect in fatty acid metabolism profoundly affecting heart, skeletal muscle, liver, and kidney. Oxidation of palmitate was 38-51% of controls. Complementation assays demonstrated that the patient's fibroblasts complemented fibroblast lines from all known defects in fatty acid oxidation except long-chain acyl-CoA dehydrogenase deficiency. Urine and serum carnitine profiles also were indicative of a defect in the oxidation of long-chain substrate; however, the palmitoyl-CoA dehydrogenase activity was actually increased. This finding indicates that the patient had a defect that was distinct from, but possibly related to, long-chain acyl-CoA dehydrogenase deficiency. This patient demonstrates the laboratory and pathologic findings in defects in fatty acid oxidation and how they differ from those in Reye syndrome.

Developmental cardiac metabolism in health and disease.

Cardiac metabolism changes in response to oxygen and substrate availability during development. The fetus is relatively more dependent on anaerobic glycolysis, using glucose as its major substrate during hypoxia, lactate when well-oxygenated. The mature heart is almost exclusively aerobic, with nonesterified fatty acids as the predominant substrate. During hypoxia and ischemia, shifting the heart to carbohydrate metabolism has oxygen-sparing effects. Blocking lipolysis or carnitine palmityl transferase activity prevents accumulation of potentially toxic long-chain esters during hypoxia/ischemia, thereby reducing the risk of electrophysiologic disturbance and membrane disruption. Knowledge of developmental cardiac metabolism may aid in the development of therapeutic strategies to preserve the myocardium during hypoxia and ischemia.

Plasma carnitine concentrations in cardiomyopathy patients.

Carnitine is an essential cofactor for the beta-oxidation of fats. Both hypertrophic and congestive cardiomyopathies have been reported in primary and secondary carnitine deficiency. Conversely in avian cardiomyopathy models abnormally elevated plasma and tissue carnitine concentrations have been described. We measured plasma carnitine concentrations in 25 cardiomyopathy patients. In 14 patients with either hypertrophic or congestive cardiomyopathy plasma carnitine concentrations were abnormally elevated. Patients with secondary cardiomyopathies tended to have normal carnitine values. One patient with systemic carnitine deficiency was diagnosed. Her cardiac function normalized with L-carnitine replacement. Six of 14 patients with high plasma carnitine concentrations died. None of the 10 with low or normal plasma carnitine have died. Plasma carnitine determination may be a useful adjunct in the diagnostic evaluation of idiopathic cardiomyopathy.

Congestive cardiomyopathy of childhood.

Congestive cardiomyopathy of childhood is a poorly understood, highly lethal disorder characterized by intrinsic inefficiency of the myocardium. The disease predominantly affects infants and is often familial. Cardiomyopathy may be caused by a wide variety of underlying disorders of substrate utilization, vascular supply, immune status, etc. Specific therapy, when available, may be highly effective. Symptomatic therapy is not. Congestive cardiomyopathy is fatal in about one third of all cases, with half of the survivors having chronic disability. Improved prognosis in childhood congestive cardiomyopathy awaits a more complete understanding of its cause. Cardiac transplantation offers the greatest hope for severely affected patients with idiopathic disease.

Phlebotomy with iron therapy to correct the microcytic polycythemia of chronic hypoxia.

Patients with chronic hypoxia develop a physiologically appropriate "secondary" polycythemia that improves oxygen carrying capacity. Supplemental iron is often required to maintain this. In severe cases when hematocrit levels approach 70%, iron is withheld in order to avoid dangerously high hematocrit levels and the risks of vascular sludging due to "hyperviscosity." Some patients even require reduction of viscosity by exchange of their polycythemic blood for plasma when symptoms develop. Iron deficiency with microcytic polycythemia can then develop. Management of such patients is unclear. Continued blood withdrawal will worsen the iron deficiency; iron supplementation will increase the hematocrit level and the risks of hyperviscosity. The combination of frequent phlebotomy with oral iron therapy should improve iron stores while safely maintaining a stable hematocrit level in patients with microcytic polycythemia. This combination should also have multiple beneficial effects on tissue oxygen delivery and utilization. This approach has been discussed and used for a patient with microcytic polycythemia due to Eisenmenger syndrome. While on therapy the patient's clinical symptoms decreased, and his serum iron level, hematologic indices, and treadmill tolerance tests all improved.

Diurnal variations in serum testosterone concentrations of captive adult male chacma baboons (Papio ursinus).

Captive adult male chacma baboons (Papio ursinus) housed with natural lighting exposure and blood sampled at 3-hr intervals showed significant diurnal variations in serum testosterone concentrations. Low mean concentrations were found at 0800 hr approximately 1 hr after sunrise and mean concentrations were their highest at 2000 hr approximately 1 » hr after sunset.

Hemodynamic effects of pulmonary arterial infusion of vasodilators in newborn lambs.

Pulmonary arterial infusion of prostaglandin E2 (mean dose, 1 microgram/kg/min) in term neonatal lambs ventilated with room air resulted in a slight fall in pulmonary arterial pressure (P less than 0.05). Infusion during ventilation with low oxygen mixtures resulted in a parallel fall in pulmonary and arterial pressures and resistances. Intrapulmonary infusion of acetylcholine (mean dose, 3.9 microgram/kg/min) in lambs during ventilation with room air caused a slight fall in mean systemic arterial pressure and systemic vascular resistance. When repeated with low oxygen ventilation, infusion of acetylcholine caused a parallel fall in both pulmonary and systemic arterial pressures and resistances. Infusion of tolazoline at a mean dose of 0.21 mg/kg/min did not change pulmonary or systemic arterial pressures or resistances when the lambs were ventilated with room air or with hypoxic gas mixtures although individual animals experienced marked falls in systemic arterial pressure. Pulmonary arterial infusion of each of the three vasodilators during hypoxic ventilation resulted in potentially adverse hemodynamic effects. This occurred despite prior ductal ligation, implying that doses effective for pulmonary vasodilation may exceed doses without systemic effects particularly in the acute hypoxic state.

Social contact influences on the menstrual cycle of the female Chacma baboon (Papio ursinus).

The menstrual cycle characteristics of 2 groups of adult female Chacma baboons were compared. Out of a group of 27 female baboons, 41% (11/27) failed to cycle during the 6 month study period but were all under the stresses of fairly severe experimental manipulation. Data on the menstrual cycles of the remaining 16 unmanipulated females was collected and carefully analysed. These 16 females were individually caged and denied social contact with other baboons. Their menstrual cycles were then contrasted to those of a group of 8 individually caged females which received 24 minute mating tests with vasectomized males on alternate days during their cycles. The females denied the social contact of the mating tests showed significantly longer cycles as a result of significantly lengthened periods up to the time of perineal detumescence.

Hemodynamic effects of prostaglandin E1 on lambs in utero.

Fetal tissues and the uteroplacental circulation are known to be exquisitely sensitive to the prostaglandins. E-series prostaglandins cause generalized vasodilatation in mature animals. This phenomenon is observed in the fetus but its effects are markedly obscured by the decrease in umbilical-placental flow. Comparison to hemodynamic data in hypoxemic fetuses clearly demonstrates that these effects are both qualitatively and quantitatively different, and therefore are likely to be due either to the direct effect of the drug, or to reflexes elicited by the marked compromise of the umbilical-placental flow.