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.

Late outcome of survivors of intervention for neonatal aortic valve stenosis.

BACKGROUND: This study examined the late outcome after intervention for neonatal aortic valve stenosis. METHODS: Seventy-three neonates (59 boys and 14 girls) underwent intervention for critical aortic valve stenosis during the first 30 days of life at two institutions, The Hospital for Sick Children, London, and Duke University Medical Center, Durham, North Carolina. Procedures performed include closed valvotomy (n = 12), open valvotomy with inflow occlusion (n = 14), open valvotomy with cardiopulmonary bypass (n = 33), balloon valvotomy (n = 12), and other procedures (n = 2). The mean age at the first intervention was 8 +/- 1 days. RESULTS: The hospital mortality was 52.1%. The mean duration of follow-up for the hospital survivors (n = 35) was 8.3 +/- 1.1 years. The actuarial survival for the hospital survivors was 93.3% +/- 4.7% at 10 years and 83.9% +/- 9.8% at 15 years, whereas event-free survival (reintervention, endocarditis, or early death) was 61.8% +/- 9.3% at 5 years, 34.2% +/- 10.8% at 10 years, and 27.4% +/- 10.6% at 15 years. Three patients have died and 11 patients have required aortic valve replacement during the follow-up period. The age at the initial intervention, the type of initial intervention, and the year of initial intervention were not predictive of early death or need for reintervention. At last follow-up, 26 of the long-term survivors (n = 32) were in functional class I and 6 were in functional class II. CONCLUSIONS: Aortic stenosis in the neonatal period is a difficult problem with a high initial mortality. Late survival and functional class are excellent for patients surviving the initial hospitalization, but most require further intervention within 10 years.

Congenital cardiovascular disease and cardiac surgery in childhood: Part 1. Cyanotic congenital heart defects.

The management of congenital heart disease is in a state of evolution, with earlier surgical and/or catheter interventions dominating clinical decision-making. Recent advances in interventional cardiac catheterization techniques, as well as continuing advances in the surgical management of complex congenital defects, continue to be the focus of attention of cardiologists and surgeons. The majority of the papers reviewed document mid- and long-term results of specific operative procedures for and address the appropriate role of cardiac catheterization techniques in the management of transposition of the great vessels, tetralogy of Fallot, total anomalous pulmonary venous connection, truncus arteriosus, pulmonary atresia with intact ventricular septum, and the univentricular heart. Early definitive intervention has become the standard of care for almost all defects reviewed.

Congenital cardiovascular disease and cardiac surgery in childhood: Part 2. Acyanotic congenital heart defects and interventional techniques.

The literature for the period reviewed documents a significant reduction of morbidity and mortality for neonatal repairs of atrioventricular septal defects, coarctation of the aorta, and ventricular septal defects. Long-term follow-up of patients with coarctation of the aorta provides important suggesting that earlier repair of asymptomatic coarctation and complete elimination of obstruction in the arch as well as at the coarctation site are essential to better long-term results. Surgery for complete atrioventricular septal defect has evolved from palliation, with poor long-term results, to complete repair within the first 6 months of life, with minimal morbidity and mortality. Special emphasis is now placed on children with Down's syndrome who have complete atrioventricular septal defects; data confirm their tendency for earlier development of pulmonary vascular obstructive changes. Further refinements in surgical and interventional catheterization techniques have resulted in promising advances for many lesions--coarctation of the aorta (native and recurrent); valvular, subvalvular, and supravalvular aortic stenosis; valvular pulmonary stenosis; patent ductus arteriosus; and branch pulmonary artery stenosis--and have prompted provocative discourse between cardiologists and surgeons. These refinements in surgical and interventional techniques have initiated a new era in the clinical management of congenital heart disease in childhood.

Quantitation of the effective right ventricular output in patients with pulmonary atresia and intact ventricular septum after placement of a right ventricular outflow patch and an aorticopulmonary shunt.

A mathematical derivation is presented of an equation that allows quantitation, in the cardiac catheterization laboratory, of the effective output of the right ventricle (QRV) in patients with pulmonary atresia and intact ventricular septum (PA/IVS) after palliation with a right ventricular outflow patch and an aorticopulmonary shunt. This derivation is based on the Fick principle and accounts for that portion of the right-to-left atrial shunt that flows through the aorticopulmonary shunt to the lungs and thus contributes to the effective pulmonary blood flow (Qep). This equation relates QRV to pulmonary blood flow (Qp), systemic cardiac output (Qs), and Qep by QRV = Qp + Qs - (Qp)(Qs)/Qep. By providing an assessment of right ventricular function, this equation may be helpful in determining the suitability of the right ventricle to support biventricular repair.

Polysplenia with pulmonary arteriovenous malformations.

A patient with polysplenia syndrome, dextrocardia, left atrial isomerism, normal great vessel relationships, and no intracardiac shunts developed progressive cyanosis and clubbing. Pulmonary arteriovenous malformations (PAVMs) were diagnosed by angiography and confirmed by lung biopsy. Superior mesenteric arteriogram revealed hypoplasia of the intrahepatic portal vein branches and a portosystemic shunt. The possible etiologies of PAVMs are discussed.

Retroperitoneal arteriovenous malformation, a rare cause of heart failure in infancy: consideration of therapeutic approaches.

We report an infant presenting with congestive heart failure secondary to a large retroperitoneal arteriovenous (AV) malformation. The heart failure was treated by catheter embolization of the feeding arterial vessels with Ivalon particles. Following resolution of the heart failure, a residual avascular mass remained. A biopsy revealed the diagnosis of an undifferentiated sarcoma that was successfully treated with chemotherapy and surgery.

Use of balloon occlusion to improve visualization of anomalous pulmonary venous return in an adult with cor triatriatum.

Cor triatriatum, although rare in adults, is often associated with anomalous pulmonary venous return. Accurate diagnosis of the pulmonary venous return and its anatomic connections is essential in planning proper surgical correction. This case illustrates the advantage of selective pulmonary artery angiography in obtaining accurate detail of the pulmonary venous anatomy from digital subtraction levophase images. Selective right and left pulmonary angiography was performed using a balloon catheter to simultaneously occlude the opposite pulmonary artery. This technique was well tolerated and greatly enhanced visualization of the anomalous pulmonary venous connections, allowing proper planning for corrective surgery.

Aortic atresia with visceral situs inversus with mirror-image dextrocardia.

A newborn infant with situs inversus totalis (visceral situs inversus with mirror-image dextrocardia) with aortic atresia is presented. No reports of this anatomical variant have previously been reported in the literature. A brief discussion of the various types of congenital heart defects and their incidence in association with situs inversus totalis is presented.

Epicardial ventricular pacemaker electrode longevity in children.

While epicardially implanted electrodes remain the most widely used in children for ventricular pacing, their expected longevity remains unknown. The longevity of 126 such electrodes implanted from January 1970 through December 1985 was evaluated in 81 children followed up for 1 to 192 months (median 63). Age at initial implant was 1 day to 18 years. Each child had from 1 to 5 electrodes implanted; 85 electrodes were of the sutureless helical type and 41 were of the suture-fixated type. Electrode failure, defined as loss of capture with a high pacing threshold found at operation or sensing failure, occurred in 38 electrodes from 1 to 157 months postimplant (median 37). Mode of failure was high threshold with high impedance (n = 15), low impedance (n = 6), complete inability to pace (n = 8), sensing failure (n = 2) or high threshold with no measure of impedance (n = 7). Actuarial life table analysis of electrode longevity showed a 88 +/- 3% (standard error of the estimate) survival rate at 6 months with no significant decrease until 53 months (75 +/- 5%, p less than 0.05). There was then a gradual steady decrease to 49 +/- 7% by 101 months. From 101 to 157 months no significant decrease occurred. Survival rate decrease was greatest within the first 6 month period postimplant (-12%). Electrodes surviving to 6 months are highly likely to survive until 53 months. Of those surviving to 53 months, 74% should survive to 120 months.

Bacterial endocarditis in a child with a Broviac catheter.

Broviac and Hickman catheters facilitate the care of children with cancer but provide a source of potential infection. We describe a child with a Broviac catheter who developed left-sided bacterial endocarditis in whom right-to-left atrial shunting was documented following catheter flushing. Following removal of the catheter and administration of prolonged intravenous antibiotics, recovery was complete and cardiac function returned to normal.

Factors affecting use of the Doppler-determined time from flow onset to maximal pulmonary artery velocity for measurement of pulmonary artery pressure in children.

Measurement of the time from onset to maximal or peak velocity (TPV) of pulmonary artery (PA) flow has been proposed as a noninvasive means of determining PA pressure. The effects of age, heart rate, increased PA pressure and flow, pulmonary valve obstruction and altered PA vascular resistance on this measurement were evaluated. In 84 children, aged 1 day to 18 years, TPV was measured using continuous-wave Doppler echocardiography. The children were separated into 3 groups. Group I (n = 33) consisted of children with no cardiovascular abnormalities. Group II (n = 33) consisted of children with a variety of cardiovascular diseases producing varying PA pressures and flows. Group III (n = 18) consisted of children who had valvular pulmonic stenosis with PA to right ventricular gradients greater than 40 mm Hg. Doppler studies of group II and III patients were performed in conjunction with measurement of PA pressures and flows at the time of cardiac catheterization. In group I TPV showed a significant negative linear correlation with heart rate (r = -0.86, p less than 0.001). The ratio of observed TPV to predicted TPV (TPVN) determined using the regression equation for TPV vs heart rate or TPV/TPVN was heart rate- and age-independent (p greater than 0.1) and ranged from 0.81 to 1.31 (mean 1.005). In group II TPV/TPVN was inversely related to the natural log of the PA pressures (systolic, r = -0.91; mean, r = -0.87; diastolic, r = -0.82; all p less than 0.01), whether pressure elevation was due to increased flow, resistance or left atrial hypertension.(ABSTRACT TRUNCATED AT 250 WORDS)

Physiological rationale for a bidirectional cavopulmonary shunt. A versatile complement to the Fontan principle.

The original Fontan procedure included a classic superior vena cava-to-right pulmonary artery (Glenn) shunt. Subsequent experience demonstrated that this anastomosis was not essential and was an unnecessary commitment of the larger right pulmonary circulation to the smaller blood volume of the superior vena caval return. With application of the Fontan principle to more complex cardiac malformations, there has been a reconsideration of possible benefits of a cavopulmonary shunt in selected patients. A modified shunt from the divided end of the superior vena cava to the side of the undivided right pulmonary artery utilized in 21 patients is described. This shunt is designed to allow bidirectional pulmonary arterial distribution of both superior vena caval inflow and right atrial outflow after completion of the Fontan procedure. Twelve patients had the bidirectional shunt performed prior to a Fontan operation; five of these had a subsequent atriopulmonary connection and seven await operation. Eight patients had construction of this shunt at the time of their Fontan procedure. One patient had a bidirectional shunt constructed following atriopulmonary anastomosis to help relieve right atrial outflow obstruction. Two patients with univentricular heart undergoing simultaneous Fontan procedure and a bidirectional shunt died while in the hospital. The remaining 19 patients have been followed up for 2 months to 9 years with one late sudden death at 9 years. There have been no bidirectional cavopulmonary shunt failures, stenoses, kinks, or recognized pulmonary arteriovenous malformations. Postoperatively, eight patients had assessment of pulmonary distribution of shunt blood flow by angiography. Seven of these patients were also evaluated by radionuclide angiography. Superior vena caval blood flow via the bidirectional cavopulmonary shunt tended to be greater to the right lung, but bilateral pulmonary flow was documented in all but one patient. After Fontan operation, six of seven patients tested also demonstrated bilateral distribution of atriopulmonary flow. We concluded from our experience that this modified shunt provides excellent relief of cyanosis, allows bidirectional pulmonary distribution of both superior vena caval return and also the right atrial blood flow after atriopulmonary connection, and may be done before, with, or after a Fontan procedure and is compatible with all currently recommended modifications. Perioperative hemodynamic adjustments to the Fontan procedure may be improved by reducing atrial volume, and this may also be of potential benefit in the long-term adaptation to Fontan physiology by minimizing atrial distention.

Large opaque hemithorax due to cardiomegaly and atelectasis.

We studied the clinical and radiologic features of five infants with a large opaque left hemithorax due to the combination of cardiomegaly and atelectasis. The causes of cardiomegaly were cardiomyopathy (two patients), congestive heart failure due to fluid overload, congenital mitral insufficiency, and idiopathic hypertrophic subaortic stenosis. In all five patients, there was initial total opacity of the left hemithorax with shift of the heart and mediastinum to the right due to the combination of cardiac enlargement and total lung collapse. The correct diagnosis may be suggested from conventional chest roentgenograms. Noninvasive supplementary studies, such as high-kilovolt roentgenography, fluoroscopy, esophagography, echocardiography, and radionuclide angiography, should be done for clarification.