Intracardiac juvenile xanthogranuloma in a newborn.

Juvenile xanthogranuloma (JXG) presents a normolipemic non-Langerhans cells histiocytosis. JXG usually presents with cutaneous lesions. Visceral involvement is rare but may affect various organs. Deep JXG differs histologically from the cutaneous form by its tendency to consist solely of homogeneous proliferation of histiocytes without any xanthomatous or Touton giant cells. Awareness of the possibility of this atypical presentation of JXG helps in making the correct histologic diagnosis, which is supported by proving adequate immunomarkers on histiocytes (mainly PG-M1, an antibody against the CD68 antigen). JXG may present with intramuscular lesions only; however, rarely JXG has been reported to affect the heart but not without the typical cutaneous manifestations. We present an unusual case of deep JXG without systemic disease or metabolic abnormalities. To our knowledge, this is a first reported case of intracavitar JXG without skin lesions.

Left ventricular outflow tract obstruction after partial atrioventricular septal defect repair.

BACKGROUND: Narrowing of the left ventricular outflow tract has been associated with partial atrioventricular septal defect (PAVSD) in about 3% of patients. Because of the predisposing anatomy, hemodynamically significant obstruction in the subaortic area may appear after repair of ostium primum atrial septal defects. METHODS: From 1984 to 1998, 40 patients underwent surgical correction of PAVSD by patch closure. The mean age at the initial repair was 5.8 years (range 3 months to 22 years). RESULTS: Nine patients had 12 subsequent operations for hemodynamically significant subaortic obstruction. The mean age at PAVSD repair was 17 months (3 to 42 months) (p < 0.001 compared with others). Follow-up work-up was obtained due to symptoms in 5 patients and an abnormal echocardiogram in 4 asymptomatic patients. Subaortic stenosis developed at a mean of 5 years (range 4 months to 10 years), and 6 or more years in 4 patients. The mean age at subaortic stenosis repair was 6 years (range 2 to 12 years). Nine patients underwent subaortic fibromuscular resection. Of these, 4 developed recurrent stenosis and 2 have undergone additional operations. CONCLUSIONS: Left ventricular outflow tract obstruction after PAVSD repair may be more frequent than reported. Because of the progressive nature of the process, echocardiography should be utilized liberally on patients to uncover subclinical stenosis. Long-term follow-up is essential for diagnosis due to delayed appearance and lack of reliable clinical signs.

Impact of ultrafiltration on blood use for atrial septal defect closure in infants and children.

BACKGROUND: Infants and children undergoing open cardiac operations have a high incidence of blood product transfusion. Ultrafiltration has been shown to reverse hemodilution and improve myocardial function and hemodynamics after cardiopulmonary bypass (CPB). METHODS: The effect of ultrafiltration on the amount of blood transfusion and hospital charge in 39 consecutive patients who underwent elective atrial septal defect repair was examined. Patients in group I (n=26) had a conventional cardiopulmonary circuit prime with blood, whereas 13 patients had bloodless prime (group II). Ultrafiltration was used immediately after weaning from CPB in group II. The patients in group I received blood products after discontinuation of CPB to achieve a hematocrit of 30%. The amount of blood product used, hematocrit immediately after CPB and on arrival in intensive care unit, postoperative hemodynamics and saturations, total operating room charge, blood charge, hospital stay, and hospital charge were compared. RESULTS: Mean body weight (15.8 kg in group I versus 17.5 kg in group II) and preoperative hematocrit values (35.6% in group I versus 34.2% in group II) were similar. Mean hematocrit immediately after CPB was 22% and 14% in group I and II, respectively (p < 0.0001). The mean hematocrit upon arrival to the intensive care unit was 34% in group I and 22% in group II (p < 0.0001). The amount of blood product transfusion was 32 mL/kg in group I and 3 mL/kg in group II patients (p < 0.0001). The patients in group II had significantly less blood bank charges; however, operating room charges and total hospital charges were similar between the two groups. CONCLUSIONS: Elective atrial septal defect repair was performed with no blood product transfusion without increased morbidity or hospital stay. Ultrafiltration can be used to reverse hemodilution resulting from a bloodless CPB prime without an increase in hospital charge.

Double patch closure of ventricular septal defect with increased pulmonary vascular resistance.

BACKGROUND: Closure of a large ventricular septal defect (VSD) in children with elevated pulmonary vascular resistance is associated with significant morbidity and mortality. Pulmonary hypertensive episodes continue to be a major cause of postoperative morbidity and mortality. We designed a fenestrated flap valve double VSD patch in an effort to decrease the morbidity and mortality associated with the closure of a large VSD with elevated pulmonary vascular resistance. METHODS: Eighteen children (mean age, 5.7 years) with a large VSD and elevated pulmonary vascular resistance (mean, 11.4 Wood units) underwent double patch VSD closure using moderately hypothermic cardiopulmonary bypass and cardioplegic arrest. The routine VSD patch was fenestrated (4 to 6 mm) and on the left ventricular side of the patch, a second, smaller patch was attached to the fenestration along its superior margin before closure of the VSD. RESULTS: All children survived operation and were weaned from inotropic and ventilator support within 48 hours postoperatively. Postoperative pulmonary artery pressures were significantly lower than preoperative values. One child died 9 months postoperatively. CONCLUSIONS: Closure of a large VSD in children with elevated pulmonary vascular resistance can be performed with low morbidity and mortality when a flap valve double VSD patch is used.

Patent ductus arteriosus ligation: are we doing better?

Limitation on health care resource use is stimulating critical evaluation of previous preoperative standards. We retrospectively reviewed the clinical and hospital financial records of all children admitted for patent ductus arteriosus ligation from July 1984 to April 1994 for age, perioperative length of stay, readmissions for postoperative surgical problem, and hospital charges adjusted to 1994 dollars. Patients with an isolated patent ductus arteriosus, greater than 3 months of age, without preoperative or postoperative complications were included in this study and stratified into two groups based on date of operation. Group I had operation before January 1, 1991, and group II had operation on or after January 1, 1991. Comparison of these two groups revealed a significant difference in perioperative length of stay (group I, 3.9 +/- 1.2 days [mean +/- standard deviation]; group II, 2.7 +/- 0.9 days; p < 0.0001) and in hospital charges (group I, $8,700 +/- $2,100; group II, $6,600 +/- $1,000; p < 0.0001). These data support the premise that children older than 3 months undergoing elective ligation of a patent ductus arteriosus have been treated with improved efficiency and less charge without an increase in postdischarge morbidity. Health care policy decisions have forced us to evaluate the standards of perioperative care more critically.

The fate of homograft conduits in children with congenital heart disease: an angiographic study.

The use of homograft conduits in the repair of congenital heart disease is widely accepted. We reviewed the catheterization and angiographic data from 20 patients with homograft conduits. All conduits were to the pulmonary arteries. The age at operation was 4.7 +/- 5.6 years (mean +/- standard deviation) and at follow-up catheterization, 7.8 +/- 6.7 years. At implantation, conduit cross-sectional area and Z value were 219 +/- 96 mm2 and 3.5 +/- 1.8, respectively. At subsequent catheterization, the conduit diameters were measured in two projections at the shaft, annulus, valve opening, and insertion into the pulmonary artery. The transconduit gradient was 47 +/- 26 mm Hg. The cross-sectional areas were 149 +/- 56 mm2 at the shaft, 151 +/- 92 mm2 at the annulus, 108 +/- 116 mm2 at the valve opening, and 127 +/- 84 mm2 at the pulmonary artery insertion. The Z values were -0.9 +/- 2.5, -0.9 +/- 2.8, -3.8 +/- 4.0, and -2.0 +/- 3.4, respectively. The cross-sectional areas and the Z values at the levels of measurement were significantly smaller than the corresponding values at implantation. The change in cross-sectional areas and Z values exceeded what would be expected from growth alone. These data indicate that there is a decrease, with time, in the functional lumen of homograft conduits, and this may have implications for follow-up strategy after implantation.

The energy costs of surgery as measured by the doubly labeled water (2H218O) method.

Energy expenditure before and after surgery was determined in seven patients by the doubly labeled water (2H218O) method (DLW). The values were compared with values obtained by respiratory gas exchange by means of a metabolic measuring cart (MMC). Patients were maintained on total parenteral nutrition before and after trauma. The principal finding was an increase in the rate of CO2 production of 11.9 +/- 5.0% after surgery. This corresponds to a 267 +/- increase in energy expenditure (p less than 0.05). No trauma-associated change in energy expenditure was found with the MMC. The correlation of preoperative values from MMC and DLW was not statistically significant (r = 0.25), nor was the correlation of MMC and the Harris-Benedict equation, but the correlation of DLW with Harris-Benedict equation was statistically significant (r = 0.73, p less than 0.05). We suggest that the discrepancy is because the DLW method measures the cumulative energy expenditure over a period, whereas the MMC gives a "spot" measurement.

Preservation of donor heart function and high-energy stores by continuous perfusion with synthetic plasma at 22 degrees C.

We evaluated high-energy phosphate (HEP) levels and the ability to perform work in rat hearts preserved by standard techniques (0.9% NaCl arrest and storage at 4 degrees C) and by continuous coronary perfusion at 22 degrees C, pH 7.25, and 55 mm Hg for 4 or 8 hr with Krebs-Henseleit buffer (KHB), modified Morgan's solution (MCS), or a medium developed in our lab (physiologically complete medium or PCM). Cardiac work was evaluated in the rewarmed hearts with use of a working heart preparation at left atrial pressures of 10, 15, and 20 cm H2O, and by measurement of aortic flow, coronary flow, heart rate, and peak systolic pressure. HEP levels in the hearts continuously perfused were significantly higher (p less than .05) than those in the hearts stored at 4 degrees C. The functional recovery of hearts preserved by storage in cold saline for 4 or 8 hr was significantly less (p less than .01) than the recovered function of hearts preserved for comparable periods by perfusion at 22 degrees C with either MCS or PCM. The results indicate that continuous perfusion at 22 degrees C with a more physiologic medium is superior to hypothermic arrest and storage at 4 degrees C for the preservation of donor heart function and HEP levels.

The effect of aspirin on protein breakdown in septic man.

In pathological states associated with hypermetabolism, such as acute sepsis, there is marked negative N balance. It has been suggested that the pathway for this response is via leukocyte pyrogen (interleukin I) acting on cyclooxygenase to stimulate prostaglandin release, which then stimulates proteolysis via the lysosomal pathway. In vitro, cyclooxygenase inhibitors decrease proteolysis in muscle tissue from septic rats. We tested this hypothesis in vivo in severely septic patients by using aspirin as the test cyclooxygenase inhibitor. Septic patients (n = 4) were given a primed, constant infusion (183 mg prime, then 37 mg/hr) of 15N-labeled urea for 6 hr to obtain a blood [15N]urea plateau. Blood samples were taken every 30 min. At 180 min 1500 mg of aspirin was given po. If aspirin inhibited protein breakdown, the plateau level should rise, since less cold urea derived from protein breakdown will enter the urea pool. Aspirin did not cause any change in either the BUN concentration, its 15N enrichment, or any of the plasma amino acids. In conclusion, cyclooxygenase inhibition by aspirin in vivo does not decrease protein breakdown in hypercatabolic septic patients.

Management of acute postoperative thrombosis following carotid endarterectomy.

We reviewed 326 carotid endarterectomies performed from 1960 through 1981 and encountered five instances of acute postoperative thrombosis. Clinical decompensation occurs with the acute onset of severe neurologic deficits, most characteristically dense hemiplegias contralateral to the side that has been operated on. These deficits developed between two and 72 hours postoperatively. Prompt reoperation with thrombectomy and reestablishment of carotid flow within two hours from the onset of the neurologic deficit was performed on four patients with complete resolution of the deficits in three patients. The fourth patient recovered from a severe hemiplegia but retained a slight residual weakness of the hand. The one patient whose condition did not improve underwent thrombectomy more than 24 hours after the onset of her deficit. Time-consuming diagnostic procedures are not warranted as the success of reoperation depends on rapid reestablishment of cerebral flow.