Psychosocial outcomes for preschool children and families after surgery for complex congenital heart disease.

The purpose of the current study was to assess the psychosocial outcomes of preschool-aged survivors (ages 3-6 years) of hypoplastic left heart syndrome (HLHS; n=13) and transposition of the great arteries (TGA; n=13). Parents completed the following measures: Pediatric Quality of Life Inventory, Impact on the Family Scale, Parenting Stress Index, Parent Behavior Checklist, and Child Behavior Checklist. Quality of life scores did not differ from those of healthy controls. Parents of children with HLHS reported more negative impact of the child's illness on the family and more parenting stress than parents of children with TGA. Parents of both groups of children were more permissive in their parenting style than parents of healthy controls. Children with HLHS had higher rates of attention and externalizing behavior problems than children with TGA. The results highlight the need for practitioners working with these children and families to ask about parental stress, family functioning, and behavioral expectations for the child in the context of routine medical/cardiac follow-up.

The virtual crossmatch--a screening tool for sensitized pediatric heart transplant recipients.

Heart transplantation in the setting of human leukocyte antigen (HLA) sensitization is challenging, as a time-consuming prospective crossmatch (XM) may be required, severely limiting the number of potential donors. We evaluated a 'virtual XM', defining a positive virtual XM as the presence of recipient pre-formed anti-HLA antibodies to the prospective donor HLA type, and compared the virtual XM to a standard direct XM. Bead-based flow cytometric analysis was used to identify anti-HLA antibody (Ab) present in a child listed for heart transplantation. Using recipient serum, direct-flow cytometric T- and B-cell XM were run for potential donors against whose HLA type the recipient had specific antibodies (group 1, n = 7) and for potential donors with predicted compatible HLA types by virtual XM (group 2, n = 7). Results were expressed as median channel difference (MCD) between the control and recipient serum. A positive T-cell XM was defined as MCD > 50, whereas MCD > 100 constituted a positive B-cell result. The rate of T-cell reactivity was significantly less in group 2 than in group 1 (29% vs. 100%, p = 0.02); similarly, B-cell reactivity was also less for group 2 (14% vs. 100%, p = 0.005). The virtual XM was 100% sensitive in detecting positive flow cytometric XM results for T and B cells. Although only 72% specific in predicting a negative T-cell XM, and 86% specific for negative B-cell XM, the false negatives were weakly positive and would probably have been clinically acceptable. Currently, potentially suitable donor organs are often declined for lack of a prospective XM; these organs may ultimately be allocated to more distant recipients or perhaps not used at all. While further studies are needed, virtual XM has the potential to improve availability of organs for sensitized patients and improve the overall allocation process.

Home surveillance program prevents interstage mortality after the Norwood procedure.

OBJECTIVE: To determine whether early identification of physiologic variances associated with interstage death would reduce mortality, we developed a home surveillance program. METHODS: Patients discharged before initiation of home surveillance (group A, n = 63) were compared with patients discharged with an infant scale and pulse oximeter (group B, n = 24). Parents maintained a daily log of weight and arterial oxygen saturation according to pulse oximetry and were instructed to contact their physician in case of an arterial oxygen saturation less than 70% according to pulse oximetry, an acute weight loss of more than 30 g in 24 hours, or failure to gain at least 20 g during a 3-day period. RESULTS: Interstage mortality among infants surviving to discharge was 15.8% (n = 9/57) in group A and 0% (n = 0/24) in group B (P =.039). Surveillance criteria were breached for 13 of 24 group B patients: 12 patients with decreased arterial oxygen saturation according to pulse oximetry with or without poor weight gain and 1 patient with poor weight gain alone. These 13 patients underwent bidirectional superior cavopulmonary connection (stage 2 palliation) at an earlier age, 3.7 +/- 1.1 months of age versus 5.2 +/- 2.0 months for patients with an uncomplicated interstage course (P =.028). A growth curve was generated and showed reduced growth velocity between 4 and 5 months of age, with a plateau in growth beyond 5 months of age. CONCLUSION: Daily home surveillance of arterial oxygen saturation according to pulse oximetry and weight selected patients at increased risk of interstage death, permitting timely intervention, primarily with early stage 2 palliation, and was associated with improved interstage survival. Diminished growth identified 4 to 5 months after the Norwood procedure brings into question the value of delaying stage 2 palliation beyond 5 months of age.

Venous saturation and the anaerobic threshold in neonates after the Norwood procedure for hypoplastic left heart syndrome.

BACKGROUND: Reduction in oxygen delivery can lead to organ dysfunction and death by cellular hypoxia, detectable by progressive (mixed) venous oxyhemoglobin desaturation until extraction is limited at the anaerobic threshold. We sought to determine the critical level of venous oxygen saturation to maintain aerobic metabolism in neonates after the Norwood procedure (NP) for the hypoplastic left heart syndrome (HLHS). METHODS: A prospective perioperative database was maintained for demographic, hemodynamic, and laboratory data. Invasive arterial and atrial pressures, arterial saturation, oximetric superior vena cava (SVC) saturation, and end-tidal CO2 were continuously recorded and logged hourly for the first 48 postoperative hours. Arterial and venous blood gases and cooximetry were obtained at clinically appropriate intervals. SVC saturation was used as an approximation of mixed venous saturation (SvO2). A standard base excess (BE) less than -4 mEq/L (BElo), or a change exceeding -2 mEq/L/h (deltaBElo), were used as indicators of anaerobic metabolism. The relationship between SvO2 and BE was tested by analysis of variance and covariance for repeated measures; the binomial risk of BElo or deltaBElo at SvO2 strata was tested by the likelihood ratio test and logistic regression, with cutoff at p < 0.05. RESULTS: Complete data were available in 48 of 51 consecutive patients undergoing NP yielding 2,074 valid separate determinations. BE was strongly related to SvO2 (model R2 = 0.40, p < 0.0001) with minimal change after adjustment for physiologic covariates. The risk of anaerobic metabolism was 4.8% overall, but rose to 29% when SvO2 was 30% or below (p < 0.0001). Survival was 100% at 1 week and 94% at hospital discharge. CONCLUSIONS: Analysis of acid-base changes revealed an apparent anaerobic threshold when SvO2 fell below 30%. Clinical management to maintain SvO2 above this threshold yielded low mortality.

Factors affecting longevity of homograft valves used in right ventricular outflow tract reconstruction for congenital heart disease.

BACKGROUND: Few studies have explored the long-term function of cryopreserved homograft valves used for reconstruction of the right ventricular tract (RVOT) in patients with congenital heart disease. METHODS AND RESULTS: Among 205 patients receiving cryopreserved homografts for reconstruction of the RVOT between November 1985 and April 1999, the outcome of 220 homografts in 183 operative survivors was analyzed. There were 150 pulmonary and 70 aortic homografts used. Median age at implantation was 4.4 years (mean 6.9+/-7.6 years, range 3 days to 48 years). End points included (1) patient survival, (2) homograft failure (valve explant or late death), and (3) homograft dysfunction (homograft insufficiency or homograft stenosis). Survival was 88% at 10 years. Freedom from homograft failure was 74+/-4% at 5 years and 54+/-7% at 10 years. Univariable analysis identified younger age, longer donor warm ischemic time, valve Z: value <2, and previous procedure as risk factors for homograft failure and dysfunction. Aortic homograft type and extracardiac operative technique predicted homograft failure but not dysfunction. For patients

Patients at risk for low systemic oxygen delivery after the Norwood procedure.

BACKGROUND: Identification of patients at risk for inadequate systemic oxygen delivery following the Norwood procedure could allow for application of more intensive monitoring, provide for earlier intervention of decreased cardiac output, and result in improved outcome. METHODS AND RESULTS: Superior vena cava saturation (SvO2) and arteriovenous oxygen content difference were prospectively monitored as indicators of systemic oxygen delivery and recorded hourly for the first 48 hours in 29 of 33 consecutive patients following the Norwood procedure. Risk factors were evaluated using multiple linear regression to determine their impact on SvO2 and arteriovenous oxygen content difference. Age less than 8 days, weight less than 2.5 kg, aortic atresia, and prolonged cardiopulmonary bypass time were risk factors for low SvO2 and wide arteriovenous oxygen content difference (p < 0.05). Phenoxybenzamine and increasing time after operation were associated with higher SvO2 and narrower arteriovenous oxygen content difference (p < 0.05). Thirty-day survival was 97% and hospital survival was 94%. The earliest death occurred on postoperative day 20. Survival to bidirectional cavopulmonary shunt was 77%. Preoperative mechanical ventilation was the only risk factor identified for late death. CONCLUSIONS: Aortic atresia, low weight, younger age, and prolonged cardiopulmonary bypass, previously identified risk factors for mortality, were associated with decreased SvO2 and narrower arteriovenous oxygen content difference in the early postoperative period. The impact of this hemodynamic vulnerability on mortality was minimized by continuous SvO2 monitoring.

Aprotinin improves outcome of single-ventricle palliation.

BACKGROUND: Elevation of pulmonary vascular resistance as a consequence of cardiopulmonary bypass may lead to failure of single-ventricle palliation. We reviewed our experience with aprotinin, a nonspecific serine protease inhibitor, to determine whether it could ameliorate the inflammatory effects of cardiopulmonary bypass and improve outcome of single-ventricle palliation. METHODS: Forty-six consecutive patients undergoing single-ventricle palliation using cardiopulmonary bypass were reviewed retrospectively. Aprotinin was used in 8 of 30 bidirectional cavopulmonary shunt and 10 of 16 Fontan procedures. RESULTS: Aprotinin use was associated with a decrease in the early postoperative transpulmonary gradient among patients undergoing Fontan and bidirectional cavopulmonary shunt procedures. The bidirectional cavopulmonary shunt aprotinin group had a higher oxygen saturation and a decrease in quantity and duration of thoracic drainage. Among patients receiving aprotinin there were no episodes of mediastinitis, thrombus formation, or renal failure. CONCLUSIONS: Aprotinin use in single-ventricle palliation was associated with decreased transpulmonary gradient and increased oxygen saturation consistent with decreased pulmonary vascular resistance. This retrospective study suggests that aprotinin has a favorable impact on the early postoperative course of single-ventricle palliation.

Twenty-year experience with repair of complete atrioventricular septal defects.

BACKGROUND: To determine factors predicting mortality and morbidity after repair of complete atrioventricular septal defect, we retrospectively analyzed preoperative, operative, and postrepair factors on the outcome of 115 consecutive complete atrioventricular septal defect repairs at The Children's Hospital of Wisconsin between January 1974 and December 1993. METHODS: For the entire experience the operative mortality was 13.9% (16 patients). During the most recent era, January 1988 to December 1993, operative mortality was 3.6% (2 of 55 patients). This was significantly improved from the two previous eras, January 1974 to December 1980, 28% (7 of 25) and January 1981 to December 1987, 20% (7 of 35 patients) (p = 0.02). There were seven late deaths; 10-year actuarial survival, including operative mortality was 81%. Age at complete repair decreased; before 1982 all patients were more than 12 months of age, whereas after 1982 64% (56 of 88 patients) were 12 months of age or less. RESULTS: Moderate or severe preoperative left atrioventricular valve regurgitation was not a risk factor for operative mortality. For operative survivors with moderate to severe preoperative left atrioventricular valve regurgitation (n = 17), late postoperative left atrioventricular valve regurgitation (follow-up data available on 15 patients) was significantly reduced (severe = 1, moderate = 5, mild = 9; p = 0.007). CONCLUSIONS: Early mortality was predicted by the era of surgical repair. Conversion to routine repair during infancy was achieved with a simultaneous decrease in operative mortality. For patients with moderate to severe preoperative left atrioventricular valve regurgitation, significant improvement in the degree of left atrioventricular valve regurgitation can be expected without an increase in operative or late mortality or morbidity.