Congenital Heart Surgery Nomenclature and Database Project: pulmonary venous anomalies.

The extant nomenclature for pulmonary venous anomalies is reviewed for the purpose of establishing a unified reporting system. The subject was debated and reviewed by members of the STS-Congenital Heart Surgery Database Committee and representatives from the European Association for Cardiothoracic Surgery. All efforts were made to include all relevant nomenclature categories using synonyms where appropriate. The basis for classification are the prenatal errors of embryologic development. The major categories include: partially anomalous pulmonary venous connection, totally anomalous pulmonary venous connection, atresia of the common pulmonary vein, cor triatriatum, and stenosis or abnormal number of pulmonary veins. A comprehensive database set is presented that is based on a hierarchical scheme. Data are entered at various levels of complexity and detail that can be determined by the clinician. These data can lay the foundation for comprehensive risk stratification analyses. A minimum database set is also presented that will allow for data sharing and would lend itself to basic interpretation of trends. Potential diagnostic-related risk factors are presented.

Congenital Heart Surgery Nomenclature and Database Project: transposition of the great arteries.

The extant nomenclature for transposition of the great arteries (TGA) is reviewed for the purposes of establishing a unified reporting system. The subject was debated and reviewed by members of the STS-Congenital Heart Surgery Database Committee and representatives from the European Association for Cardiothoracic Surgery. All efforts were made to include relevant nomenclature categories including synonyms where appropriate. The general categories of TGA are: TGA with intact ventricular septum, TGA with ventricular septal defect (VSD) and TGA with VSD and left ventricular outflow tract obstruction (LVOTO). A comprehensive database set is presented which is based on a hierarchical scheme. Data are entered at various levels of complexity and detail that can be determined by the clinician. A detailed hierarchical system is described herein for classification of the coronary artery anatomy associated with TGA. These data can lay the foundation for comprehensive risk stratification analyses. A minimum database set is also presented which will allow for data sharing and would lend itself to basic interpretation of trends.

Abnormal left ventricular filling after neonatal repair of congenital heart disease: association with increased mortality and morbidity.

BACKGROUND: The presence of mid-diastolic flow reversal on the mitral valve Doppler inflow indicates abnormal left ventricular filling. To determine whether mid-diastolic flow reversal predicts outcome in patients undergoing repair or palliation of neonatal congenital heart disease, we reviewed the echocardiograms and medical records of 40 patients with either left ventricular outflow obstruction or transposition of the great arteries. METHODS: All patients underwent surgical repair; transposition of the great arteries (TGA) = 17, coarctation of the aorta (CoA) = 14, interrupted aortic arch (IAA) = 8, and aortic stenosis (AS) = 1. The presence of mid-diastolic flow reversal was determined by pulsed Doppler interrogation of the mitral valve on preoperative and postoperative echocardiograms. RESULTS: Preoperative echocardiograms showed diastolic flow reversal in only 5 patients; 1 of 1 with AS and 4 of 14 with CoA. Twenty-one of 40 patients showed postoperative diastolic flow reversal; 1 of 1 with AS, 8 of 8 with IAA, 1 of 14 with CoA, and 11 of 17 with TGA. Postoperative mid-diastolic flow reversal 1 to 3 days after surgery was associated with higher mortality rate: 7 of 21 patients with diastolic flow reversal and 0 of 19 without diastolic flow reversal died. Patients with diastolic flow reversal who survived had longer intensive care unit (26.2 +/- 13.5 days vs 7.1 +/- 4.1 days, P <.001) and hospital (57.4 +/- 38.8 days vs 14.8 +/- 5.2 days, P <.05) stays. CONCLUSION: Mid-diastolic flow reversal is an indicator of prolonged hospital stay and mortality in patients with left ventricular outflow tract obstruction or TGA.

Intraoperative transesophageal echocardiography in congenital heart disease.

This article reviews the use of intraoperative echocardiography during repair of congenital heart defects. Although initial experience was generated using epicardial transducers, there has been a trend in recent years toward the use of transesophageal echocardiography (TEE) in the operating room. This has encouraged increased involvement from cardiologists and anesthesiologists. New probe designs have provided biplane imaging via the TEE approach in infants weighing more than 2.5 kg. Smaller infants may still require epicardial imaging, so it is helpful for surgeons to maintain some skill in this technique. This article reviews the utility of intraoperative echocardiography for various congenital heart defects by providing examples from our experience at Duke University Medical Center since 1987 with close to 2,000 cases. Furthermore, we review and report for the first time our experience with TEE since 1993 in the operating room during infant heart surgery (493 patients). Along with this experience, we provide a review of important series in the literature to outline recommendations for the use of echocardiography during infant heart repair.

Effect of repair strategy on hospital cost for infants with tetralogy of Fallot.

OBJECTIVE: This study compares the total hospital cost (HC) for one-stage versus "two-stage" repair of tetralogy of Fallot (TOF) in infants younger than 1 year of age. SUMMARY BACKGROUND DATA: Total (one-stage) correction of TOF is now being performed with excellent results in infancy. Alternatively, a two-stage approach, with palliation of infants in the first year of life, followed by complete repair at a later time can be used. In some institutions, the two-stage approach is standard practice for infants younger than 1 year of age or is used selectively in patients with an anomalous coronary artery across the right ventricular outflow tract (RVOT), "small pulmonary arteries," multiple congenital anomalies, critical illnesses (CI), which increase the risk of bypass (e.g., sepsis or DIC), or severe hypercyanotic spells (HS) at the time of presentation. The cost implications of these two approaches are unknown. METHODS: The authors reviewed 22 patients younger than 1 year of age who underwent repair of TOF at their institution between 1993 and 1995. Eighteen patients had one-stage (1 degree) repair (mean age, 3.4 +/- 3.1 months; range, 3 days-9 months) and 4 patients were treated by a staged approach with initial palliation (1.6 +/- 0.4 month; range, 1.5-2 months) followed by later repair (14.75 +/- 1.5 months; range, 13-16 months). The reasons for palliation were severe HS at time of presentation (two patients), anomalous coronary artery (one patient) and CI (one patient). In the 18 patients undergoing 1 degree repair, 3 (16.6%) presented with HS, 6 (33.3%) had a transanular repair, and 6 (33.3%) were able to be repaired through an entirely transatrial approach (youngest patient, 1.5 months). The HC (1996 dollars) and hospital length of stay (LOS; days) were evaluated for all patients. The HCs were calculated using transition I, which is a cost accounting system used by our medical center since July 1992. Transition I provides complete data on all direct and indirect hospital-based, nonprofessional costs. RESULTS: There was no mortality in either group. The group undergoing 1 degree repair had an average LOS of 14.5 +/- 11.2 days compared to an average LOS for palliation of 14 +/- 6.4 days. When the palliated group returned for complete repair, the average LOS was 28.8 +/- 25 days, yielding a total LOS for the two-stage strategy of 43 +/- 30.8 days (p = 0.003 compared to 1 degree repair). The HC for 1 degree repair was $32,541 +/- $15,968 compared to $25,737 +/- $1900 for palliation (p = not significant compared to 1 degree repair) and $54,058 +/- $39,395 for subsequent complete repair (p = not significant compared to 1 degree repair) (total two-stage repair HC = $79,795 +/- $40,625; p = 0.001 compared to 1 degree repair). The LOS and HC for the two-stage group combine a total of palliation plus later repair and, as such, reflect two separate hospitalizations and convalescent periods. To eliminate cost outliers, a best-case analysis was performed by eliminating 50% of patients from each group. Using this analysis, the two-stage approach resulted in an average (total) LOS of 16.5 +/- 2.1 days compared to 8.5 +/- 1.4 days for the 1 degree group. Total cost for the two-stage strategy in this best-case group was $44,660 +/- $3645 compared to $22,360 +/- $3331 for 1 degree repair (p = 0.00001). CONCLUSIONS: The data from this review show that palliation alone generates HC similar to that from 1 degree infant repair of TOF, and total combined HC and LOS for palliation plus eventual repair of TOF (two-stage approach) are significantly higher than from 1 degree repair. Furthermore, these data do not include additional costs for care delivered between palliation and repair (e.g., outpatient visits, cardiac catheterization, serial echocardiography). Although there may be occasions when a strategy using initial palliation followed by later repair may seem prudent, the cost is clearly higher and use of health care resources greater.

Pulmonary vein Doppler echocardiography after left atrial operation.

BACKGROUND: Intraoperative transesophageal echocardiography provides the surgical team with important guidance during operations for congenital heart disease. Doppler echocardiography adds hemodynamic information to that provided by two-dimensional imaging. Here we describe intraoperative pulmonary vein Doppler echocardiography after operation involving the left atrium. METHODS: Intraoperative two-dimensional and pulsed-wave Doppler echocardiography of pulmonary veins were performed after surgical repair of anomalous pulmonary venous return in 4 patients. RESULTS: In 3 patients, intraoperative pulmonary vein Doppler findings were suggestive of obstruction. The surgical repair was thought to be excellent, and there was no obstruction apparent anatomically or clinically. No further intervention was performed, and all patients recovered uneventfully. No pulmonary venous obstruction was noted on follow-up two-dimensional echocardiography, and follow-up pulmonary vein Doppler echocardiography showed the expected pattern. CONCLUSIONS: An obstructive pattern in the pulmonary vein Doppler was seen in 3 of 4 patients studied immediately after repair of anomalous pulmonary venous return. No obstruction, however, was manifest then or subsequently. The abnormal Doppler pattern, thus, is not indicative of pulmonary venous obstruction. We propose that acute postoperative changes in left atrial volume and compliance and acute postoperative tissue changes in the left atrium explain the abnormal pulmonary vein Doppler patterns observed.