Tracheal extubation of children in the operating room after atrial septal defect repair as part of a clinical practice guideline.

Early tracheal extubation in the operating room after atrial septal defect (ASD) surgery was recommended as part of a clinical practice guideline (CPG) established in the Cardiovascular Program at the Children's Hospital, Boston, MA. This retrospective review was undertaken to determine whether this practice was efficient without compromising patient care. The charts and hospital charges for 102 patients undergoing secundum ASD or sinus venosus defect surgery between March 1992 and July 1994 were reviewed; 36 patients (Group I) had surgery prior to introduction of the CPG, and 66 patients were managed according to the CPG. Of the latter, 25 patients (Group II) were tracheally extubated in the operating room (OR) and 41 patients (Group III) were extubated in the cardiac intensive care unit (CICU). Patients in all three groups were similar with respect to height, weight, and surgical conditions including cardiopulmonary bypass time, lowest esophageal temperature, hematocrit, total OR time, and the time from completion of bypass to leaving the OR. Patients in Group II received significantly less fentanyl during anesthesia, were more likely to have a respiratory acidosis on admission to the CICU, and had an increased frequency of vomiting in the CICU. There was no difference in duration of CICU stay among groups. The length of hospital stay was reduced in Groups II and III after introduction of the CPGs, but was not influenced by tracheal extubation in the OR. There was no difference among groups in the hospital charges for OR, anesthesia and CICU time. However, when the combined hospital charges for services provided both in the OR and CICU were included, patients in Group II were charged significantly less, and this primarily reflects the absence of postoperative mechanical ventilation charges. Tracheal extubation in the OR after ASD surgery in children can result in lower patient charges without significantly compromising patient care.

Clinical practice guidelines for quality patient outcomes.

In response to environmental changes brought about by the debate over health-care reform, hospitals are seeking means to decrease costs while improving quality. By examining the process of health-care delivery, problems in the system can be highlighted for further investigation. Development of clinical practice guidelines by an interdisciplinary team can be an effective approach to decrease undesirable practice variation, standardize appropriate resource use, and measure the effectiveness of care through defined expected-patient outcomes. By ensuring the appropriate, efficient, and effective delivery of health care, savings in cost can be realized by the elimination of duplication, rework, and waste.

Task Force 1: The underserved.

The ACC has affirmed its commitment to universal access to health care. Underserved populations exist in urban and rural centers. Common to each is a paucity of personnel trained in cardiovascular care and a lack of access to preventive and highly technologic services. These factors contribute to a poor health outcome (75). Part of the rural problem can be corrected by the transfer of information to local providers by the use of new information systems. Included would be real-time electronic consultation, on-site subspecialty visits and the appropriate use of nonphysician providers (15). The urban problem requires changes in priorities and responsibilities of the academic health centers toward the communities they serve. Curricula changes of cardiovascular specialists, internists, generalists and nonphysician health care personnel must include diversity in training, physician training of ethnically matched providers in addition to technical excellence and research into methods of patient education and motivation for a healthier life-style (51). Reimbursement must appropriately reward those caring for underserved patients and those providing evaluation and management services (43,52).

Advances in the diagnosis and therapy of syncope and palpitations in children.

Although advances in diagnosis and therapy of congenital heart disease have received most of the attention, in the past few years advances in the more common troublesome problems of adolescents with syncope and palpitations have been as dramatic. Syncope may be due to a cardiac arrhythmia, but is more commonly due to an autonomic dysfunction of blood pressure control. In cases where the diagnosis is equivocal by history alone, autonomic testing, including head-up tilt table testing, may be useful. Treatment with a salt-enriched diet and a mineralocorticoid is usually effective in reducing or eliminating the incidence of syncope. In resistant cases beta-blockade, scopolamine, disopyramide, theophylline, or fluoxetine may be helpful. Palpitations are usually benign. When they are due to a supraventricular tachycardia, especially when associated with the Wolff-Parkinson-White syndrome, they may be annoying and, in those with a short refractory period, dangerous. Radiofrequency ablation of the abnormal pathway is being increasingly used to eliminate the arrhythmia and may be superior to a lifetime of pharmacologic suppression in symptomatic individuals.

Guidelines for long-term management of patients with Kawasaki disease. Report from the Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association.

Long-term management of patients with Kawasaki disease should be tailored to the degree of coronary arterial involvement. This committee has made recommendations for each risk level about antiplatelet and anticoagulant therapy, physical activity, follow-up assessment by a pediatric cardiologist or primary care physician, and the appropriate diagnostic procedures that may be performed to evaluate cardiac disease. The risk level for a given patient with coronary arterial involvement may change over time because of changes in coronary artery morphology. The recommendations for management presented here are intended as practical interim guidelines until additional prospective or retrospective data are compiled to define more clearly the natural history of Kawasaki disease.

Infective endocarditis in the adult with congenital heart disease.

Advances in the diagnosis and therapy of congenital heart disease in the past few decades have allowed the survival of young adults who are a fertile substrate for developing bacterial endocarditis. In addition to valvular involvement, young adults with congenital disease may develop infection on the ventricular septum, or in a vascular shunt, a patent ductus arteriosus, or coarctation of the aorta. The incidence of endocarditis in children and adults with congenital heart disease is higher than with rheumatic heart disease or mitral valve prolapse. Infecting organisms, diagnosis, and therapy are similar to acquired heart disease, but the physiologic variations and patient population often add a unique flavor to the management of this group.

Surgical management of late right ventricular failure after Mustard or Senning repair.

BACKGROUND: Information on surgical management and outcome in patients who develop symptomatic right ventricular failure after prior Mustard or Senning operations is limited. METHODS AND RESULTS: From March 1987 to March 1991, 10 patients 3.6-23.5 years old (median, 7.0 years) with transposition of the great arteries and prior Mustard (six patients) or Senning (four patients) repairs (performed at ages 2 months to 5 years; median, 6 months) underwent surgical intervention for symptomatic right ventricular failure. In five of 10 patients, anatomic correction with either an arterial switch operation (three patients) or a pulmonary artery-to-aorta anastomosis and right ventricle-to-pulmonary artery conduit (two patients) was performed. Before anatomic correction in these five patients, four of five patients had a pulmonary artery band to prepare the left ventricle. The interval between preparation and correction ranged from 8 days to 12 months (median, 2 months). One patient died after an arterial switch operation. In the remaining five patients, coexisting left ventricular dysfunction precluded anatomic correction; all five patients survived cardiac transplantation. Survival for the entire group of 10 patients is 90%, and the median postoperative hospital stay was 17 days. During follow-up (12-62 months; median, 27 months), there were no deaths. Neoaortic insufficiency after anatomic correction was common (mild in one patient, moderate in two patients, and severe in one patient who required aortic valve replacement 4 months after surgery). In the transplantation group, one patient developed lymphoma 3 months after transplantation but is currently in remission after reduction of immunosuppression. CONCLUSIONS: In patients who develop late right ventricular failure after Mustard or Senning repair, surgical intervention with either anatomic correction or cardiac transplantation can be done with acceptable morbidity and low mortality. Neoaortic valve insufficiency demands close follow-up after anatomic correction.

Intermediate results of the arterial switch repair. A 20-institution study.

A total of 466 neonates with transposition of the great arteries, all less than 15 days of age, have been entered into a 20-institution study between Jan. 1, 1985, and June 1, 1987. Seventy-three percent were less than 48 hours old when entered into the study. Two hundred twelve of these underwent an arterial switch repair, and the 1-week, 1-year, and 2 1/2-year survival rates were 82%, 79%, and 78%, respectively. The usual coronary anatomy was present in 67% of the patients. The arterial switch repair was performed in 16 of the institutions. Six among these were shown to be "low-risk" institutions, with the prevalence of the demographic and surgical variables seeming to be the same in these as in the other institutions. The 1-week, 1-year, and 2 1/2-year survival rates after the arterial switch repair in low-risk institutions for simple transposition were 96%, 91%, and 90%, respectively; for transposition with ventricular septal defect they were 84%, 83%, and 83%. Only older age at operation (over 14 days of age), in the case of simple transposition, and transposition with ventricular septal defect were risk factors for death in these six institutions. Among the patients as a whole, freedom from reoperation for pulmonary outflow obstruction at 1 week and 1 year was 99% and 89%, respectively. A previous pulmonary artery banding and, possibly, one institution were identified as risk factors for reoperation. Inferences: In low-risk institutions, the arterial switch repair can be accomplished with good early results, which suggests the possibility that the late results will be better than after the atrial switch repair. Since young age was not a risk factor for the arterial switch repair of transposition and ventricular septal defect, this type of repair for this anomaly, as well as for simple transposition, may be more advantageously performed early in life than at 2 to 3 months of age.

Nonutility of cineurograms in children with congenital heart disease.

Cineurography is often performed after cineangiocardiography to look for occult congenital urinary tract disease. The accuracy of cineurography was investigated in 171 patients by comparing cineurograms with renal sonograms. One hundred fifteen cineurograms (67%) showed both kidneys well enough to allow assessment of renal structure and function and the results were confirmed in 112 by ultrasonography; 3 cineurograms yielded false-positive results. Limited or no information was obtained from cineurograms of 56 patients (33%) because of nonvisualization or poor visualization of 1 or both kidneys. Of the 11 patients (6%) with urinary tract disease, only 3 were correctly assessed by cineurography. Ultrasonography discovered all 11 renal abnormalities and produced only 1 false-positive result. These data indicate that cineurography is a poor screening test and should be abandoned. When uroradiologic screening is necessary for high-risk patients, sonography is recommended.

Valve replacement in children less than 5 years of age.

Between 1966 and 1984, there were 63 children less than 5 years of age who underwent 70 valve replacements (49 mitral [5 repeat], 6 aortic, 11 tricuspid [systemic ventricle; 2 repeat]), 2 tricuspid [pulmonary ventricle] and 1 multiple [mitral-aortic]) at Children's Hospital. Tissue valves were used in 20%. Since 1980, only Björk-Shiley and St. Jude valves have been used. The most common indication for valve replacement was mitral regurgitation after repair of atrioventricular (AV) canal (34%). Mortality dropped considerably over time: 76% before 1979, 33% from 1979 to 1982 and 22% since 1982. More than two-thirds of fatalities were operative deaths, usually within 3 days of surgery. Actuarial survival curves for those operated on since 1980 predict 1 and 5 year survival of 73 and 51%, respectively. For the 46 operative survivors 1 and 5 year valve survival was 97 and 70%, respectively. Postoperative hemodynamics were significantly improved on elective postoperative catheterizations. All but one patient with non-tissue valves received anticoagulant therapy. Postoperative complications included thromboembolism (1.6/100 patients-years) and hemorrhage (0.8/100 patient-years). The frequency of intravascular hemolysis and endocarditis was 1.6%, comparable with adult experience. Complete heart block requiring a pacemaker developed in nine patients (14.1%), in all after AV valve replacement. Valve replacement in young children generally results in considerable hemodynamic improvement. The mortality rate remains above that observed in adults but has declined considerably for those operated on after 1980.

Surgery for coarctation of the aorta in the neonate.

Between 1972 and 1984, 100 consecutive neonates (less than or equal to 30 days old) underwent repair of coarctation of the aorta. Mean (+/- SD) age at operation was 12.0 +/- 8.0 days; mean weight was 3.1 +/- 0.5 kg. Simple coarctation was present in 29 patients (group I), 32 patients had additional ventricular septal defects (group II) and 39 patients had additional complex heart disease (group III). An associated patent ductus arteriosus was present in a total of 81 patients. Subclavian flap angioplasty (SFA) was performed in 70 patients, resection and end-to-end anastomosis (E-E) in 24 patients, and miscellaneous procedures in six patients. All SFA procedures were performed after 1977, and 87.5% of E-Es were done before 1977. The early mortality was 33.3% for patients undergoing E-E and 11.4% for those undergoing SFA. Freedom from reintervention for recoarctation after 5 years was 92.9% for patients who underwent E-E and 75.2% for those who underwent SFA. Actuarial survival at 4 years was 85.5% for group I, 79.9% for group II, and 42.9% for group III. Follow-up was 97.7% for a mean (+/- SD) of 41.9 +/- 37.1 months. It is likely that some of the differences between patients undergoing SFA and E-E with respect to early mortality and freedom from reintervention are due to the different time frames during which the procedures were done. However, neonatal ductal tissue after SFA may contract and fibrose causing recoarctation, while disadvantages related to the circumferential suture line after E-E may have been overemphasized in the past. A prospective randomized trial of these two techniques is warranted.

Long-term follow-up of patients with synthetic right heart conduits.

Between 1971 and 1983, 201 patients received synthetic right heart conduits, predominantly porcine-valved Dacron conduits, at The Children's Hospital, Boston. There were 45 hospital deaths (22%). Follow-up has been achieved in 148 of 156 survivors (95%). Thirty-four conduits have been replaced, all because of conduit obstruction. The actuarial freedom from conduit replacement was 81% at 5 years, 61% at 7 years, and 0% at 10 years for valved conduits. There was no significant difference to 5 years in reoperation rate between patients with Carpentier-Edwards and those with Hancock conduits. Patients older than 18 years at the time of conduit insertion were 92% free of conduit replacement at 5 years. Those with nonvalved conduits were 100% reoperation free at 4 years. The actuarial survival of patients with valved conduits was 91% at 5 years and 83% at 10 years. The poor performance of porcine-valved tightly woven Dacron conduits warrants a change to use of an alternative conduit, particularly in smaller children. Possible alternatives include antibiotic-sterilized homografts and valved or nonvalved high-porosity knitted Dacron conduits appropriately pretreated with collagen impregnation or fibrin glue.

Normothermic caval inflow occlusion. Application to operations for congenital heart disease.

The technique of normothermic caval inflow occlusion was introduced primarily for relief of pulmonary valve stenosis and continues to be used for this purpose with excellent results. However, the technique may also be applied to a number of other lesions such as aortic valve stenosis in neonates and infants, for placement of outflow patches or valve excision for pulmonary atresia with intact ventricular septum, for atrial septectomy in children with restrictive atrial septum, and for other conditions wherein a period of up to 2 minutes of intracardiac exposure is sufficient. This report examines the early and long-term results in 140 children who underwent normothermic caval inflow occlusion at The Children's Hospital, Boston, Massachusetts, over the past 11 years. Ninety-four children underwent pulmonary valvotomy (early mortality 0%), 21 had aortic valvotomy (19%), 10 with pulmonary atresia and intact ventricular septum underwent various procedures (50%), 11 had atrial septectomy (9%), and there were four miscellaneous procedures (50%). Apart from avoiding many of the potential complications of cardiopulmonary bypass, inflow occlusion has provided as good or better short-term and long-term results as those obtained with cardiopulmonary bypass, particularly in infants and neonates, and also has proved more cost effective.