Constance J. Hayes

Survival and Risk Factor Analysis for the Norwood Procedure for Hypoplastic Left Heart Syndrome

The optimal approach to hypoplastic left heart syndrome (HLHS) is controversial. The palliative Norwood operation, cardiac transplantation, and no surgical intervention have all been advocated. Centers that perform the Norwood operation have met with varied results, and conflicting reports exist regarding factors predictive of stage I outcome. From January 1990 to January 1996, 67 patients with HLHS were admitted with intent to perform the staged Norwood procedure. Fourteen patients did not undergo surgery. In the 53 patients treated surgically, outcome was reviewed, and 10 potential risk factors for first stage mortality were analyzed. Forty-one infants survived the Norwood I operation to hospital discharge (77% of the surgically treated patients and 61% of the entire group, including those who did not undergo operation) with 6 additional deaths 3 to 5 months after operation. Univariate analysis showed cardiopulmonary bypass time and circulatory arrest time to be significant risk factors for hospital mortality. Multivariate analysis revealed only cardiopulmonary bypass time as significant (p <0.01). Of the 15 prenatally diagnosed newborns who underwent surgery, 11 survived (p = 0.72). Ten of 11 patients with preoperative organ damage survived (p = 0.42). Among the 35 bidirectional Glenn (Norwood II) and Fontan (Norwood III) procedures performed, there were 2 deaths. The 5-year actuarial survival for patients who underwent operations was 61%. The Norwood procedure is a favorable option for the infant with HLHS. Surgical survival may be affected by a prolonged cardiopulmonary bypass time, but is not affected by other factors analyzed, including prenatal diagnosis and preoperative organ damage.

Early developmental outcome after the Norwood procedure for hypoplastic left heart syndrome.

Objective. To assess intellect and adaptive behavior in children with hypoplastic left heart syndrome (HLHS) who had undergone at least two surgical stages of the Norwood procedure. Methods. Fourteen children with HLHS >3 years of age participated in the study. The patients underwent intelligence quotient (IQ) testing, and their parents were interviewed regarding their childrens adaptive behavior. Results were compared with those of 10 family controls. Outcomes were studied for possible correlation with perioperative variables. Results. Among the HLHS patients, the median scores for full scale IQ and adaptive behavior were 88 and 91, respectively (normal = 100 ± 15). One child met criteria for mental retardation. Family controls scored generally higher than did HLHS patients, but only differences in adaptive behavior were statistically significant. A negative correlation was found between stage I circulatory arrest time and full scale IQ. Conclusions. Children with HLHS most often function in the low-normal range of intelligence and adaptive behavior. A prolonged circulatory arrest time may result in decreased intellectual function.

Hypoplastic left heart syndrome: Valuing the survival

OBJECTIVE To examine the survival, developmental status, quality of life, and direct medical costs of children with hypoplastic left heart syndrome who have undergone stage I, II, and III reconstructive surgery. METHODS A total of 106 children underwent staged repair for classic hypoplastic left heart syndrome between February 1990 and March 1999 (stage I: 106; stage II: 49; stage III: 25; 4 converted to heart transplantation). Survival was analyzed by the Kaplan-Meier method. In a cross-sectional study, parents assessed quality of life by completing the Infant/Toddler Child Health Questionnaire or Child Health Questionnaire Parent Format-28; they assessed developmental progress by completing the Ages and Stages Questionnaire. The ratio-of-costs-to-charges method was used to derive hospital costs, and payments were used to capture physician time and wholesale pricing for outpatient medications. RESULTS Institutional 1-year and 5-year actuarial survivals were 58% and 54%. Birth weight, the need for preoperative inotropic drugs, and surgical experience were predictors of survival. Norwood I patients achieved fewer developmental benchmarks than those who survived to subsequent stages. Child Health Questionnaire Parent Format-28 mean summary scores for physical and psychosocial health were 48.5 +/- 6.3 and 42.8 +/- 9.9. The median inpatient costs for stage I, II, and III repairs were

Cardiorespiratory Response to Exercise after the Fontan Procedure for Tricuspid Atresia

51,000,

Does cisapride influence cardiac rhythm? Results of a United States multicenter, double-blind, placebo-controlled pediatric study.

33,892, and

“Bedside” balloon septostomy in infants with transposition of the great arteries: New concepts using two-dimensional echocardiographic techniques

52,183, respectively. Monthly outpatient and readmission costs were less than 10% of total costs. CONCLUSION A prospective, large-scale study of the comprehensive outcomes of staged repair and transplantation is needed. This study will need to address the longer-term developmental and quality-of-life outcomes, as well as the long-term cost effectiveness of these procedures.

Open-Heart Experience in Infants Using Normothermia and Deep Hypothermia

ABSTRACT: Noninvasive exercise testing was used to assess gas exchange in 13 patients age 6–25 yr who had undergone Fontan procedures for tricuspid atresia, five of whom had preexisting Glenn shunts. The results were compared to 28 age- and sex-matched controls. Oxygen saturation was measured by ear oximetry at rest and after exercise. Ventilation oxygen consumption (VO2), carbon dioxide production (VCO2), and heart rate were measured during progressive exercise. The ventilatory equivalents for oxygen (VE/VO2) and carbon dioxide (VE/VCO2), mixed expired pCO2 (PECO2) end-tidal pCO2 (PETCO2), and dead space to tidal volume ratio (VD/VT) were determined during steady state exercise on a cycle ergometer. Heart rate was higher for VO2 by 15% (p < 0.02) and ventilation was higher for both VO2 (by 37%, p < 0.001) and VCO2 (by 27%, p < 0.002) in the patients than the controls. Mean VE/VO2 was 35.4 ± 7.8 (SD) compared to 25.8 ± 3.1 (p < 0.001) and mean VE/VCO2 was 41.7 ± 9.0 compared to 31.6 ± 4.3 (p < 0.001). Mean PECO2 was 21.4 ± 4.4 torr with controls at 27.9 ± 3.8 (p < 0.001) and mean PETCO2 was 33.0 ± 5.3 torr compared to 40.0 ± 3.3 (p < 0.001). The patients had a mean oxygen saturation of 92 ± 5% at rest and abnormal saturation after exercise (87 ± 9, p < 0.005). There were no differences in VE/VO2, VE/VCO2, PECO2, PETCO2, % of Hb saturated with oxygen before and after exercise, or VD/VT ratios between the five patients who had also undergone the Glenn operation and those who had not. Two patients who had right atrium to right pulmonary artery anastomoses demonstrated higher (VE/VO2 and VE/VCO2 ratios, lower end-tidal and mixed expired pCO2, and lower % of Hb saturated with oxygen before and after exercise than the patients with right atrium to right ventricle anastomoses. Patients with tricuspid atresia who have undergone the Fontan operation show high heart rate for oxygen consumption, high ventilation for O2 consumption and CO2 production, low expired CO2 concentrations, and oxygen desaturation during exercise. The results indicate elevated physiological dead space and ventilation perfusion mismatch consistent with maldistribution of pulmonary blood flow in patients with Fontan physiology who have had either a right atrium to pulmonary artery or right atrium to right ventricle connection with or without a previous Glenn shunt. Longer follow-up of these patients will be necessary to determine the late clinical implications of these findings.

Long-Term Follow-Up of Two Infants with an Implanted Cardiac Pacemaker

Background Major concerns about serious cardiac side effects underlie the recent decision by the FDA and Janssen Pharmaceutica (Titusville, NJ) to make cisapride available only through a limited access program. Concerns have grown despite the fact that most instances of prolonged QTc and other ventricular arrhythmias occurred while the drug was used concomitantly with contraindicated drugs. This study sought to analyze electrocardiograms (ECGs) from a multicenter pediatric study and to identify abnormalities in QTc interval associated with cisapride use. Methods Children between 6 months and 4 years of age were enrolled if they manifested symptoms of gastroesophageal reflux not responding to medical therapy for at least 6 weeks. In 49 subjects, ECGs obtained before and after randomization to receive 0.2 mg/kg dose three times daily or placebo were reviewed independently and blindly by two pediatric cardiologists. Placebo and active drug groups were compared for QTc and for change in QTc from baseline values after 3 to 8 weeks of treatment. Results Mean QTc among patients taking the drug was 408 ± 18 ms. None was higher than 450 ms. Change between baseline and subsequent QTc at 3 to 8 weeks of treatment was 2 ± 20 ms. Conclusions In our study group of children without underlying cardiac disease or electrolyte imbalance, cisapride was found to have no significant effect on cardiac electrical function compared with placebo. These results are consistent with the drugs record of exceedingly infrequent cardiac events. Because the availability of this prokinetic is threatened, its safety and the safety and efficacy of alternative treatment options (including surgery) should be studied further.

Management Strategy for Very Mild Aortic Valve Stenosis

We performed balloon atrial septostomies in six successive infants with transposition of the great arteries, using echocardiographic guidance at the bedside in the neonatal intensive care unit. In all cases adequate septostomy was obtained and instantaneously assessable. PaO2 values increased as expected (mean before septostomy 28 mm Hg; after, 42 mm Hg). In cases of transposition clearly diagnosed echocardiographically, this intervention need not be done in the catheterization laboratory. The need for a full hemodynamic study in the neonatal period is arguable.

Critical Aortic Stenosis in Infancy

During the 9-year period from 1967 through 1975, 124 open-heart operations were performed on infants less than 1 year of age with 35 operative deaths (28%). Ninety-seven of these procedures used continuous cardiopulmonary bypass with normothermia or mild hypothermia, and 27 were done under deep hypothermia and circulatory arrest. Mortality and morbidity were similar regardless of the operative technique, although deep hypothermia facilitated the repair of complex lesions. The highest mortality occurred in infants less than 3 months of age. Respiratory insufficiency, usually requiring prolonged ventilatory support, occurred only among infants who had pulmonary overcirculation or congestion prior to operation. Adequacy of intraoperative repair and postoperative care were the major determinants of survival.