Cardiovascular-radical outcome method is effective in complex congenital cardiac lesions.

BACKGROUND: The cardiovascular-radical outcome method is a proactive process of patient care that uses standard critical pathway methodology to reduce negative variation while encouraging positive variation to accelerate recovery. Its effectiveness in patients with complex congenital heart disease is explored. METHODS: Two hundred fourteen consecutive patients with congenital heart disease were cared for using the cardiovascular radical outcome method. Age ranged from 2 days to 19 years (median age, 3 years). Cardiovascular radical outcome method data were compared with the pathway plan data for each patient. RESULTS: Survival was 99% (211 patients) with an overall reduction in stay of 156 days (0.74 day/patient) (p < 0.0001). Only 10 patients (5%) exceeded the pathway plan; 201 (95%) reached the planned length of stay (critical pathway method), and 127 patients (60%) had a shorter length of stay than expected by the critical pathway method. One hundred forty-eight patients (70%), including 95 (64%) with more complex conditions, had a length of stay of 3 days and 18% achieved a 2-day length of stay, the maximal response. The process was most effective in the most complex groups, although preoperative comorbidities influenced outcomes. Outcome assessment demonstrated minimal morbidity and excellent family satisfaction. CONCLUSIONS: The radical outcome method is effective in reducing the length of stay of patients with complex congenital heart disease. The power is in the process rather than the plan, and the method provides optimal patient care and family satisfaction.

Fetal echocardiography: retrospective review of clinical experience and an evaluation of indications.

OBJECTIVE: To assess the indications, use, and yield of fetal echocardiography. METHODS: We reviewed our 8-year experience with fetal echocardiography at a large health maintenance organization in northern California to assess the yield of abnormal findings for each indication and the accuracy of prenatal cardiac diagnosis. The study included 915 fetuses undergoing fetal echocardiography between 18-38 weeks' gestation because of accepted indications. All live-born infants had the presence or absence of congenital heart disease (CHD) confirmed postnatally. RESULTS: Family history of CHD led to 34% of all fetal echocardiography studies, with a detection rate of 0.7%. Maternal diabetes mellitus accounted for 28% of all studies, with a detection rate of 1.2%. Exposure of mothers to a potential teratogen led to 48 studies, but no abnormalities were detected. Only 4% of fetal echocardiograms were obtained because of a suspected heart defect noted on a screening obstetric sonogram; this indication had a detection rate of 68% and yielded by far the most cases of severe CHD. Based on the expected occurrence rate of severe CHD in our closed referral system, only about 15% were referred for fetal echocardiography and detected prenatally. CONCLUSION: Basing fetal echocardiography referral on risk factors misses most clinically significant detectable CHD.

Aortic implantation is possible in all cases of anomalous origin of the left coronary artery from the pulmonary artery.

BACKGROUND: Anomalous origin of the left coronary artery from the pulmonary artery (PA) optimally is treated by creation of a multiple coronary system. This study explores the use of aortic implantation employing alternative methods to achieve coronary transfer in all patients, regardless of the site of origin of the anomalous coronary artery, avoiding the problems of bypass grafts and tunnel procedures. METHODS: During the period 1986 to 1994, 11 patients aged 6 months to 8 years (mean age, 2.6 years) underwent repair. Coronary artery origin from the PA included left sinus in 3, posterior in 2, right sinus in 2, intramural aorta with its orifice at the bifurcation of the main and right PA in 1, high left main PA in 1, high at the bifurcation of main and right PA in 1, and anterior in 1. Findings included angina in 4, prior infarctions in 3, ischemia in 7, left ventricular dysfunction in 6, mitral regurgitation in 5, atrial septal defect in 2, and echocardiograms suggestive of endocardial fibrosis in 4. One patient had prior ligation with ventricular dysfunction and collateralization and recanalization. A single patient was asymptomatic. Repair was accomplished by direct transfer using the PA sinus of Valsalva as a button in only 6; tubular reconstruction was used in 4 when the distance was too great to avoid tension; 2 short tubes were constructed with PA wall in 2 of the 3 left sinus origins, whereas 2 long tubes of PA wall were used (1 high on the left side of the main PA and 1 with left anterior descending origin from the anterior sinus of Valsalva in a patient with malrotation [end neo-artery to side aortic reconstruction]); finally, in situ transfer and intraaortic reconstruction (unroofing and anastomosis) was performed in 1 intramural coronary artery. Division of the PA, mobilization of the distal PA, division of the ductus, and direct reanastomosis of the PA was performed in 3 tubular reconstructions, as well as all 6 direct coronary transfers. RESULTS: There were no operative or late deaths. Follow-up of 2 to 100 months (mean, 46 months) revealed no new angina or infarctions, improved function and decreased mitral regurgitation. Echocardiographic and angiographic studies demonstrated patency and prograde flow in the new coronary systems. CONCLUSIONS: Aortic implantation is the treatment of choice for anomalous origin of the left coronary artery. Methods such as direct transfer, tubular reconstruction, and in situ transfer make such implantation possible in all patients regardless of the site of coronary origin, distance from the aorta, or coronary artery configuration.

Critical pathway methodology: effectiveness in congenital heart surgery.

Critical pathway methodology has been demonstrated to provide producible reduction in average length of stay (ALOS) in adults in certain diagnostic-related groups and operations such as coronary artery bypass grafting. The efficacy of this approach in congenital heart surgery was explored. Two hundred eighty-six consecutive patients from a health maintenance organization treated by a single surgeon since the institution of diagnostic-related group coding at that health maintenance organization constituted the study group. One hundred fourteen patients were treated at a university hospital without critical pathway methodology (group 1) and 172, subsequently at the health maintenance organization institution using the methodology (group 2). Operation/lesion, age, and diagnostic-related group matching was possible in 61 pairs. Examination of the ALOS Hospital (operative and postoperative days) for the entire cohort revealed a 43.8% reduction in ALOS Hospital (p < 0.0001) and a 39.0% reduction in ALOS Intensive Care Unit (p < 0.0001). There was also significant reduction in ALOS Hospital and ALOS Intensive Care Unit in the operation/lesion-matched subsets. Outcome measures including operative and late mortality, readmission, unscheduled emergency room and clinic visits, and health maintenance organization family assessment survey demonstrated no improvement in outcome with increased hospital stay. Thus, critical pathway methodology when used in patients undergoing a congenital heart operation produces a significant reduction in hospital stay and intensive care unit stay as well as quality patient care with uniformity of outcome.

Asymmetric septal hypertrophy in infants of diabetic mothers. Fetal echocardiography and the impact of maternal diabetic control.

Maternal hyperglycemia may result in fetal hyperinsulinemia and asymmetric septal hypertrophy, macrosomia, and hypoglycemia in infants of diabetic mothers. We monitored glycosylated hemoglobin levels in 61 pregnant diabetic women each trimester as an index of maternal glycemic control and did serial fetal echocardiograms starting at 18 weeks of gestation. At delivery, cord blood C-peptide levels were obtained as an index of fetal hyperinsulinemia. Infants were assessed for hypoglycemia, macrosomia and septal thickening by echocardiography. Nineteen of the 61 infants (31%) had septal hypertrophy, were heavier, and had higher cord blood C-peptide levels and lower serum glucose levels than unaffected infants. Maternal glycosylated hemoglobin levels were higher during the third trimester in mothers of affected infants. Our data support a possible relationship between third-trimester maternal hyperglycemia and neonatal asymmetric septal hypertrophy, macrosomia, and hypoglycemia.

Repair of combined total anomalous pulmonary venous connection and anomalous systemic connection in early infancy.

A simple intraatrial reconstruction can be utilized in infants to simultaneously correct both the systemic and pulmonary venous returns. Two infants with polysplenia syndrome treated in the first months of life are presented. One had levocardia and combined total anomalous pulmonary venous connection (type II-Cardiac) and anomalous systemic venous connection. The other had dextrocardia L-loop, L-normal great arteries, and combined total anomalous pulmonary venous connection (type II-Cardiac) and anomalous systemic venous connection. The posterior position of the systemic atrioventricular valve and systemic ventricle in both patients allowed the use of this reconstructive technique. The extensive size and transverse position of this patch when used in the infant may require both continued postoperative controlled ventilation until it stiffens and prolonged attention to postoperative cardiac care until adaptation of the infant heart to the new atria can occur. Early restudy and reoperation should be performed if signs of stenosis develop.

Pulsus paradoxus in a patient with tricuspid atresia and hypoplastic right heart.

Increased inspiratory right ventricular filling with a consequent shift of the ventricular septum to the left, thereby decreasing left ventricular filling, has been suggested as a mechanism of pulsus paradoxus. We recently saw a patient with tricuspid atresia and hypoplastic right ventricle who developed pulsus paradoxus. His course may help clarify the contribution of this mechanism.