Reversible posterior leukoencephalopathy syndrome associated with left ventricular assist device.

Reversible posterior leukoencephalopathy syndrome (RPLES), previously known as posterior reversible encephalopathy syndrome (PRES), is characterized by the presence of bilateral lesions located within the posterior border zones of the cerebral hemisphere and cerebellum. This condition commonly presents with headache, nausea, vomiting, decreased vision and level of consciousness, and seizures. RPLES has been associated with hypertensive encephalopathy, eclampsia, renal failure, and immunosuppressive therapy following transplant. We report the development of RPLES in a boy following implantation of a left ventricular assist device (LVAD). To our knowledge, this is the first report of RPLES in association with the use of LVAD.

Anterior pericardial tracheoplasty for congenital tracheal stenosis.

Congenital tracheal stenosis may be a life-threatening anomaly not relieved by airway intubation. Over the past 7 years, anterior pericardial tracheoplasty has been used at our institution for treatment of congenital long-segment tracheal stenosis in infants with impeding airway obstruction. Case histories of eight patients undergoing nine anterior pericardial tracheoplasties have been reviewed to assess this technique. Of these patients, six have required preoperative tracheal intubation before repair to maintain ventilation. The surgical technique of anterior pericardial tracheoplasty includes a median sternotomy approach with partial normothermic cardiopulmonary bypass. An anterior tracheotomy through all hypoplastic rings allows enlargement with autologous pericardium to 1.5 times the predicted normal diameter. After insertion, the pericardium and hypoplastic tracheal cartilages are suspended to surrounding mediastinal structures, which prevents airway collapse. Seven of eight infants have survived without tracheoplasty dehiscence or wound infections. Five were ultimately extubated and are currently free of symptoms from 6 months to 5 years after anterior pericardial tracheoplasty. The other two survivors had residual stenosis as a result of complications of prior tracheostomy. One of these patients has undergone a successful second anterior pericardial tracheoplasty and is currently extubated and well. The other is palliated at 6 months with a tracheostomy awaiting a second anterior pericardial tracheoplasty. Our review of anterior pericardial tracheoplasty has demonstrated the safety, utility, and at least medium-term benefit of this procedure in infants of any age and weight.

Pulmonary hypertension after operations for congenital heart disease: analysis of risk factors and management.

BACKGROUND: Management of pulmonary hypertension, a potentially fatal complication of operations to correct congenital heart disease, has evolved through the last 15 years. Monitoring of pulmonary arterial pressure and mixed venous saturation became available, and prophylactic use of alpha-blockers and other vasodilators increased. This study examines risk factors for morbidity and mortality from pulmonary hypertension after operations to correct congenital heart disease and evaluates the impact of management changes on outcomes. METHODS: By means of multivariable logistic regression analysis, 880 high-risk patients with congenital heart disease (of 2484 patients undergoing cardiopulmonary bypass between January 1980 and December 1994) were analyzed to determine which were at risk for postoperative pulmonary hypertension and its associated morbidity and mortality. RESULTS: Patients with atrioventricular canal (n = 182), truncus arteriosus (n = 47), total anomalous pulmonary venous connection (n = 90), transposition of great arteries (n = 97), hypoplastic left heart syndrome (n = 50), and ventricular septal defect (n = 414) demonstrated a higher risk of postoperative pulmonary hypertension. By multivariable logistic regression, preoperative pulmonary hypertension (p < 0.0001), absence of mixed venous saturation monitoring (p < 0.0001), and absence of prophylactic alpha-blockade (p = 0.0004) significantly increased postoperative pulmonary hypertension. Preoperative pulmonary hypertension (p < 0.001) and absence of prophylactic alpha-blockers (p = 0.0004) were significant risk factors for in-hospital death related to pulmonary hypertension. Repair at older age (except in the case of total anomalous pulmonary venous connection) was a significant risk for postoperative pulmonary hypertension (p = 0.03). CONCLUSION: Mixed venous saturation monitoring and alpha-receptor blockade reduced the incidence of pulmonary hypertension after operations for congenital heart disease. Early definitive repair reduced morbidity and mortality from postoperative pulmonary hypertension.

Aggressive surgical management of sternoclavicular joint infections.

BACKGROUND: Although the sternoclavicular joint is an unusual site for infection, thoracic surgeons may preferentially be called on to coordinate management of cases refractory to antibiotic therapy because of the anatomic relationship of this joint to major vascular structures. METHODS: Since 1994 we have surgically managed nine sternoclavicular joint infections in eight patients. Associated medical problems were frequent and included diabetes mellitus (n = 2), end-stage renal disease (n = 2), hematologic disorders (n = 2), and multiple joints affected by sepsis (n = 4). Open joint exploration with drainage and débridement with the use of general anesthesia was performed in four patients. The remaining four patients (one with bilateral sternoclavicular joint infections) had computed tomographic evidence of diffuse joint and surrounding bone destruction with infection extending into mediastinal soft tissues. Surgical therapy for these five joint infections involved en bloc resection of the sternoclavicular joint with an ipsilateral pectoralis major muscle covering the bony defect. RESULTS: There were two deaths unrelated to the surgical procedure. After a mean follow-up of 20 months, the remaining six survivors (seven joints) have complete healing with no apparent limitation in the range of motion even after en bloc resection. CONCLUSIONS: Most cases of early sternoclavicular joint infections will respond to conservative measures. However, when radiographic evidence of infection beyond the sternoclavicular joint is present, en bloc resection, although seemingly aggressive, results in immediate eradication of all infection with negligible functional morbidity. Prolonged antibiotic therapy or continued local drainage procedures appear to have little value in these cases, adding only to patient care costs and the potential sequelae of chronic infections.

Surgical management of complete atrioventricular septal defects. A twenty-year experience.

Creation of a competent left atrioventricular valve is a cornerstone in surgical repair of complete atrioventricular septal defects. To identify risk factors for mortality and failure of left atrioventricular valve repair and to determine the impact of cleft closure on postoperative atrioventricular valve function, we retrospectively analyzed hospital records of 203 patients between January 1974 and January 1995. Overall early mortality was 7.9%. Operative mortality decreased significantly over the period of the study from 19% (4/21) before 1980 to 3% (2/67) after 1990 (p = 0.03). Ten-year survival including operative mortality was 91.3% +/- 0.004% (95% confidence limit): all survivors are in New York Heart Association class I or II. Preoperative atrioventricular valve regurgitation was assessed in 203 patients by angiography or echocardiography and was trivial or mild in 103 (52%), moderate in 82 (41%), and severe in 18 (8%). Left atrioventricular valve cleft was closed in 93% (189/203) but left alone when valve leaflet tissue was inadequate and closure of the cleft might cause significant stenosis. Reoperation for severe postoperative left atrioventricular valve regurgitation was necessary in eight patients, five of whom initially did not have closure of the cleft and three of whom had cleft closure. Six patients had reoperation with annuloplasty and two patients required left atrioventricular valve replacement. Five patients survived reoperation and are currently in New York Heart Association class I or II. On most recent evaluation assessed by angiography or echocardiography (a mean of 59 months after repair), left atrioventricular valve regurgitation was trivial or mild in 137 of the 146 survivors (94%) examined; none had moderate or severe left atrioventricular valve stenosis. By multiple logistic regression analysis, strong risk factors for early death and need for reoperation included postoperative pulmonary hypertensive crisis, immediate postoperative severe left atrioventricular valve regurgitation, and double-orifice left atrioventricular valve. These results indicate that complete atrioventricular septal defects can be repaired with low mortality and good intermediate to long-term results. Routine approximation of the cleft is safe and has a low incidence of reoperation for left atrioventricular valve regurgitation.

Dilutional and modified ultrafiltration reduces pulmonary hypertension after operations for congenital heart disease: a prospective randomized study.

OBJECTIVE: A prospective randomized study was performed to test whether removal of endothelin-1, by ultrafiltration techniques, will reduce pulmonary hypertension after operations for congenital heart disease. METHODS: Twenty-four patients with pulmonary hypertension (systolic pulmonary/systemic arterial pressure ratio > 60%) undergoing cardiac operations were randomized into a control group (n = 12) having conventional ultrafiltration and an experimental group (n = 12) undergoing dilutional ultrafiltration during and modified ultrafiltration after cardiopulmonary bypass. Plasma endothelin-1, nitric oxide metabolites, and cyclic guanosine monophosphate were assayed before bypass, 10 minutes into bypass, after bypass, and 0, 3, 6, and 12 hours after the operation in both groups, as well as in the ultrafiltrates and after modified ultrafiltration in the experimental group. Both groups received alpha-blockers (chlorpromazine and/or prazosin) postoperatively using the same guidelines. RESULTS: The ultrafiltrates contained significant amounts of endothelin-1 (1.81 +/- 0.86 pg/ml, dilutional, and 6.44 +/- 1.82 pg/ml, modified ultrafiltrate). Endothelin-1 and the pulmonary/systemic pressure ratio were significantly lower in experimental compared with control patients. Nitric oxide metabolites and cyclic guanosine monophosphate increased similarly in both groups for 12 hours after the operation (p = not significant). Three of 12 control patients (25%) but no experimental patients had pulmonary hypertensive crises (p = 0.07). The experimental patients required significantly less ventilatory support (67 +/- 47 hours vs 178 +/- 139 hours for control patients, p = 0.048). CONCLUSIONS: Dilutional and modified ultrafiltration reduce endothelin-1 and the pulmonary/systemic pressure ratio postoperatively and may become an important adjunct for preventing pulmonary hypertension after operations for congenital heart disease in high-risk patients.

Short-term survivors of pediatric heart transplantation: an autopsy study of their pulmonary vascular disease.

BACKGROUND: The most common indications for heart transplantation in children are congenital heart disease and cardiomyopathy. Intracardiac lesions that vary widely in morphology may have a similar impact on pulmonary vascular morphology; for example, any lesion that increases left-sided intracardiac pressure will affect pulmonary venous pressure and morphology and, if long-standing, may affect pulmonary arteries also. METHODS: The lungs of eight children who died 2 days to 7 months after receiving a heart transplant at ages 8 days to 4 years were examined at autopsy with particular regard to the pulmonary arteries and veins. Arteries were evaluated for medial thickness, intimal proliferation, and peripheral extension of muscle; veins for medial thickness and mural elastic tissue configuration; and lymphatics for degree of dilation. RESULTS: The main pulmonary arteries of the children with congenital heart disease showed an abnormal elastic tissue pattern, similar to the pattern of the aorta, indicating that pulmonary arterial hypertension was present in these children at birth. In contrast, the pulmonary trunk of a child who had myocarditis beginning at age 2 years had a normal pulmonary elastic tissue pattern, consistent with normal neonatal pulmonary pressure. Five children with morphologic evidence of early pulmonary arterial hypertension had a left-to-right atrial shunt before the operation and elevated pulmonary artery pressure immediately after transplantation. The pulmonary arteries of all five patients had medial hypertrophy or peripheral extension of muscle; the most advanced pathologic condition occurred in the arteries of the oldest child, who was 5 months of age, at the time of transplantation who died 2 days after the operation with pulmonary arterial hypertension. In the lungs of all eight patients, pulmonary veins showed medial hypertrophy and excess elastic tissue fibers (arterialization), consistent with venous hypertension. The most impressive venous changes in the lungs of the children with hypoplastic left heart syndrome occurred in the two children who had the smallest left ventricles (4 x 6 mm and 8 x 8 mm), and in another child with hypoplastic and anomalous extrapulmonary pulmonary veins. The most impressive lymphangiectasis occurred in the lungs of the child with hypoplastic left heart syndrome who was oldest at the time of transplantation and in the lungs of a child with obstructed venous drainage. CONCLUSIONS: Pulmonary vasculopathy in children who require heart transplantation because of congenital heart disease or long-standing congestive heart failure may involve arteries, veins, and lymphatics. Among the eight patients in this autopsy study of children who died less than a year after heart transplantation, arterial vasculopathy associated with congenital heart disease appeared more pronounced in the five who survived less than 30 days compared with the two longer survivors. The lack of morphologic progression in short-term survivors of pulmonary arterial vasculopathy after heart transplantation correlates with clinical follow-up of long-term survivors who do not have problematic pulmonary arterial hypertension. Pulmonary venous vasculopathy is determined by the degree of pretransplantation left-sided obstruction caused by congenital or acquired disease and may be more persistent morphologically than arterial vasculopathy. This morphologic study of the pulmonary vasculature of short-term survivors of heart transplantation supports the concept that palliative surgical procedures may benefit children awaiting heart transplantation, especially infants with hypoplastic left heart syndrome for whom suitable donors are scarce.

Temporary left-sided mechanical cardiac support during acute myocarditis.

Acute myocarditis is usually a self-limiting viral illness. Rarely, however, myocardial depression can be profound leading to circulatory collapse. Mechanical cardiac support in the form of intraaortic balloon pumps or ventricular assist devices have been used in these unusual cases to maintain systemic perfusion until transplantation or left ventricular recovery occurs. We report a young patient with acute myocarditis who required left heart mechanical support and who, however, was successfully weaned despite only minimal myocardial recovery.

Triple-drug immunosuppression for heart transplantation in infants and children.

Triple-drug immunosuppression with OKT3 induction is effective rejection prophylaxis in pediatric cardiac allograft recipients. The concerns regarding prevalent lymphoproliferative disease, growth retardation, cytomegalovirus, and other opportunistic infections have not been realized. Since June 1986, 34 pediatric patients, 23 males and 11 females, (age 4 days to 15 years) have undergone orthotopic heart transplantation at our institution. Fifteen patients were less than 6 months old and 13 had type I or II hypoplastic left heart syndrome. There have been four (12%) operative and four (12%) late deaths and a survival rate of 76% after a mean follow-up of 33 months (range, 1 to 82 months). The only deaths attributed to allograft rejection occurred in two newborn recipients (2 and 10 months after surgery) who neither received maintenance steroids nor underwent routine biopsy. The only death from infection (pneumococcus) occurred 6 months after surgery in a 4-year-old patient who was not known to be asplenic. Of the 27 long-term (> 1 year) survivors, 17 (68%) had an average of two rejection episodes during the follow-up period, 10 patients (32%) have been free of graft rejection, and 26 patients (96%) have not experienced a cytomegalovirus infection despite OKT3 induction therapy. Two patients developed lymphoproliferative disease, one of whom was successfully treated by transient reduction of immunosuppression. The other patient died 13 months after transplantation of a lymphoma of the central nervous system. All survivors have demonstrated satisfactory increases in mean height and weight.(ABSTRACT TRUNCATED AT 250 WORDS)

Heart transplantation in patients with Marfan's syndrome: a survey of attitudes and results.

Our institution performed an orthotopic heart transplantation in a patient with Marfan's syndrome. The immediate postoperative course was complicated with ultimate discharge; however, this patient died within 1 year as a result of an aortic dissection. On the basis of our experience, we surveyed all heart transplant centers listed with the North American Transplant Coordinator Organization for other patients with Marfan's syndrome who had been referred for transplantation. Nearly one third of responding centers evaluated a total of 30 patients with Marfan's syndrome. Of these, only 13 were formally listed for a donor organ and 11 underwent heart transplantation with an operative mortality of 9.1% (n = 1). Posttransplantation morbidity was significant and includes a 40% (n = 4) incidence of thoracic aorta dissection (one fatal) in operative survivors. There were three other non-dissection-related late deaths for an overall survival of 54.4% after a mean follow-up of 33.0 months. In this shared experience, it appears that the known vascular complications of Marfan's syndrome may diminish the anticipated results after heart transplantation. The reluctance to place these patients on heart transplant donor waiting lists can be justified.

Bronchoesophageal fistula with an esophageal web.

Congenital bronchoesophageal fistulas in the adult age group are rare, with only approximately 20 cases having been recognized. All of these cases have been an isolated esophageal anomaly without other associated esophageal pathology. We present an interesting case of an adult with both symptomatic congenital bronchoesophageal fistula and proximal esophageal web.

Arterial switch operation: factors impacting survival in the current era.

BACKGROUND: The arterial switch procedure has become the preferred procedure for the transposition of the great arteries (TGA) and Taussig-Bing anomaly. This analysis is intended to identify potential factors affecting survival in the current era. METHODS: From 1986 to 1999, 201 consecutive patients underwent an arterial switch operation for TGA or Taussig-Bing anomaly. Multivariate analysis of perioperative variables was performed for operative morbidity/ mortality. Patients were separated into two groups. Phase 1 (n = 29) included patients before mid-1989 who underwent an open coronary reimplantation technique. Phase 2 (n = 172) included the patients undergoing a technique of reimplanting coronary buttons after neoaortic reconstruction. RESULTS: The patient population included TGA with intact ventricular septum (58.7%, 118 of 201), with ventricular septal defect (31.3%, 61 of 201), and Taussig-Bing anomaly (10.0%, 22 of 201). Overall, early mortality was 9.5% (19 of 201) and there were five late deaths (2.7%). One-month, 1-year, and 5-year actuarial survival rates were 90.4%, 87.9%, and 87.9%, respectively. Reoperation rate for late pulmonary stenosis was 2.7% (5 of 182). The freedom from reoperation at 3 and 5 years was 97.5% and 93.3%, respectively. In the analysis by time period, the operative mortality declined from 27.6% (8 of 29) to 6.4% (11 of 172) (p = 0.002). Risk factors for operative death were coronary artery patterns (usual vs retropulmonary left coronary artery, p = 0.009) in phase 1 and preoperative instability in phase 2 (p = 0.002). CONCLUSIONS: The arterial switch operation for TGA and Taussig-Bing anomaly has early low and late mortality and reoperation rates. Technical modifications in coronary reimplantation have minimized coronary artery pattern-related risks.

Surgical results in patients with double outlet right ventricle: a 20-year experience.

BACKGROUND: The objective of this study was to review our surgical strategy in children with double outlet right ventricle and to assess risk factors for early and late mortality and reoperation. METHODS: Patients (n = 124; June 1980 to January 2000; age range, 7 days to 16 years; mean, 2.8 years) who underwent repair of double outlet right ventricle. The patients were divided into three groups. Group 1 (n = 47) had noncomplex patients with atrioventricular concordance, a single ventricular septal defect, balanced ventricles, no straddling atrioventricular valves, and no major pulmonary artery anomalies. Group 2 (n = 39) included patients with double outlet right ventricle and a subpulmonary ventricular septal defect (Taussig-Bing). Group 3 (n = 38) had patients with complex anomalies including straddling atrioventricular valves, atrioventricular septal defects or a hypoplastic valve or ventricle, or a combination of atrioventricular septal defects and hypoplastic valve or ventricle. RESULTS: Four types of definitive repairs were performed: (1) intraventricular tunnel repair with a baffle from the left ventricle to the aorta (n = 53); (2) use of a valved or nonvalved conduit (n = 20); (3) arterial switch operation with a patch committing the left ventricle to the neo-aorta (n = 16); and (4) cavopulmonary shunt and Fontan procedures (n = 33). Two patients with late postoperative cardiomyopathy had heart transplantation. Potential risk factors included location of the largest ventricular septal defect, presence of additional ventricular septal defects, ventricular outflow obstruction or hypoplasia, or both ventricular outflow obstruction and hypoplasia, previous palliation, and type of definitive operation. There were six early deaths (4.8%) and four late deaths (3.2%), and two heart transplants (1.6%). Overall 15-year survival was 95.8%, 89.7%, and 89.5% for groups 1, 2, and 3, respectively (p = 0.08). Thirteen patients (11.4%) have required 15 reoperations. Mean follow-up for survivors was 76.6 +/- 52.8 months. Up-to-date follow-ups are available on 114 surviving patients. Ninety-five of these patients (83.3%) were in New York Heart Association class I, and the remaining 19 patients (16.7%) were in New York Heart Association class II. Freedom from reoperation was 87%, 72%, and 100% at 15 years for groups 1, 2, and 3, respectively (p = 0.11). CONCLUSIONS: Survival was high for all patients with double outlet right ventricle undergoing intraventricular tunnel repair, arterial switch operation, and repair with a conduit or a modified Fontan procedure. Careful attention to preoperative anatomy dictates the best surgical approach and will enhance outcomes.

Biological versus mechanical aortic valve replacement in children.

BACKGROUND: Aortic valve replacement in children remains challenging because of constraints imposed by available prosthetic devices. Potential risks of anticoagulation with mechanical valves and degeneration of other biological substitutes have kindled interest in the Ross procedure. This study outlines the evolution of our 27-year experience with prosthetic devices. METHODS: Ninety-nine patients who underwent aortic valve replacement (January 1973 through September 2000) were included in this study. Procedures included implantation of pulmonary autograft (PA) (n = 42), aortic homograft (AH) (n = 3), mechanical valves (MV) (n = 41), and xenograft tissue valves (XG) (n = 13). RESULTS: The mean follow-up times were: 3.8+/-1.3 years for PA, 3.5+/-1.5 years for AH, 7.7+/-4.7 years for MV, and 8.4+/-4.8 years for XG. There were no significant differences in perioperative outcomes among the groups (p < or = 0.05) or early deaths (2 each in the MV, AH, and PA groups). The incidence of valve-related complications and reoperations was high in the MV (n = 5), XG (n = 7), and AH (n = 1) groups as compared with the PA group (n = 3, p < 0.01). Early and late mortality for the series was 8.6% (n = 8). Overall, the reoperation rate was 20.7% (n = 18): 15.2% (5 of 33) MV, 70% (7 of 10) XG, 50% (1 of 2) AH, and 11.9% (5 of 42) for PA. The actuarial survival rate was 87.8% and 100% at 10 years for MV and XG, and 95.2% and 6.6% at 7 years for PA and AH. CONCLUSIONS: Aortic valve replacement in children can be performed with acceptable mortality and good long-term results. The Ross procedure, although more complicated, has the advantage of not requiring anticoagulation therapy, can be performed in all age groups, possesses inherent growth potential, and exhibits the most normal left ventricular outflow tract hemodynamics.

Surgical management of pulmonary carcinoma secondary to recurrent respiratory papillomatosis.

Recurrent respiratory papillomatosis is a rare, but acknowledged, risk factor for pulmonary squamous cell carcinoma. Although previous reports suggest a poor prognosis for lung cancer associated with papillomatosis, we have successfully treated 1 such patient, who presented with three synchronous pulmonary malignancies, using parenchyma-sparing resection techniques.

Evolution of the Fontan procedure in a single center.

BACKGROUND: Surgical approaches to single ventricle variants include staged, fenestrated, and completed Fontan operations. This study compares outcomes with these modifications of the Fontan operation at a single center. METHODS: Preoperative risk factors and operative results were analyzed by multivariate techniques in 129 patients undergoing modified Fontan operations since March 1988. RESULTS: Overall early and late mortality was 5.4% and 0.8%, respectively. Before 1993, completed Fontan operation using right atrial to pulmonary artery anastomosis without fenestration was performed in the majority of patients (44 of 58; 76%). During this period, 10 of 17 patients at high risk had completed Fontan with three takedowns. In 1994, the staged hemi-Fontan and modified Fontan with a lateral tunnel anastomosis and with or without small fenestration (2.5 to 4 mm) were introduced. The majority of patients at high risk during this period underwent hemi-Fontan followed by fenestrated Fontan with no takedowns. Late atrial dysrhythmias occurred in 6 patients (4.7%), generally with larger fenestrations or right atrial to pulmonary anastomoses. Three patients (2.3%) had a stroke, 2 with large (> or = 4 mm) fenestrations. Of 38 fenestrations, 32 (84%) closed spontaneously by 1 year. No protein-losing enteropathy occurred. Most patients (118 of 121) were in New York Heart Association class I/II 4.5 years postoperatively. By multivariate analysis, only Down's syndrome (p < 0.001) predicted early mortality, whereas both Down's syndrome and a systemic right ventricle decreased late survival (p < 0.006). CONCLUSIONS: Proper selection of patients for modifications of the Fontan procedure resulted in excellent early and late survival with a low incidence of atrial dysrhythmia and stroke. Midterm functional outcomes were excellent.

Cardiac transplantation in infants and children.

Cardiac transplantation has become a more frequently used therapeutic modality for select cardiac pathology in infants and children. Since June 1986, 30 pediatric patients (19 male and 11 female) ranging in age from 4 days to 15 years (11 < or = 1 month old) have undergone orthotopic cardiac transplantation at our institution. Indications included idiopathic cardiomyopathy (n = 8), hypoplastic left heart syndrome (n = 13), and other forms of complex congenital heart disease (n = 9). There have been four operative and three late deaths only in the groups with hypoplastic left heart syndrome and other forms of complex congenital heart disease. Cumulative survival is 77% after a mean follow-up of 30 months (range, 6 to 77 months). Three operative deaths were attributable to pulmonary hypertension, and the other was due to pulmonary hemorrhage. Two late deaths were secondary to allograft rejection, and the third was due to infection. There has been uniform survival in the group with idiopathic cardiomyopathy, and intermediate-term survival rates are 78% and 62% in the groups with complex congenital heart diseases and hypoplastic left heart syndrome, respectively (p = 0.15). Although longer-term results are necessary, orthotopic cardiac transplantation appears to be an acceptable mode of therapy for endstage heart disease in the pediatric age group and technically can be performed despite complex malformations of the great arteries or atria.

Effect of modified ultrafiltration in high-risk patients undergoing operations for congenital heart disease.

BACKGROUND: Modified ultrafiltration (MUF) after cardiopulmonary bypass (CPB) in children decreases body water, removes inflammatory mediators, improves hemodynamics, and decreases transfusion requirements. The optimal target population for MUF needs to be defined. This prospective, randomized study attempted to identify the best candidates for MUF during operations for congenital heart disease. METHODS: Informed consent was obtained from 100 consecutive patients with complex congenital heart disease undergoing operations with CPB. They were randomized into a control group (n = 50) of conventional ultrafiltration during bypass and an experimental group using dilutional ultrafiltration during bypass and venovenous modified ultrafiltration after bypass (MUF group, n = 50). Postoperative arterial oxygenation, duration of ventilatory support, transfusion requirements, hematocrit, chest tube output, and time to chest tube removal were compared between the groups stratified by age and weight, CPB technique, existence of preoperative pulmonary hypertension, and diagnosis. RESULTS: There were no MUF-related complications. In patients with preoperative pulmonary hypertension, MUF significantly improved postoperative oxygenation (445 +/- 129 mm Hg versus control: 307 +/- 113 mm Hg, p = 0.002), shortened ventilatory support (42.9 +/- 29.5 hours versus control: 162.4 +/- 131.2 hours, p = 0.0005), decreased blood transfusion (red blood cells: 16.2 +/- 18.2 mL/kg versus control: 41.4 +/- 27.8 mL/kg, p = 0.01; coagulation factors: 5.3. +/- 6.9 mL/kg versus control: 32.3 +/- 15.5 mL/kg, p = 0.01), and led to earlier chest tube removal. In neonates (< or =30 days), MUF significantly reduced transfusion of coagulation factors (5.4 +/- 5.0 mL/kg versus control: 39.9 +/- 25.8 mL/kg, p = 0.007), and duration of ventilatory support (59.3 +/- 36.2 hours versus 242.1 +/- 143.1 hours, p = 0.0009). In patients with prolonged CPB (>120 minutes), MUF significantly reduced the duration of ventilatory support (44.7 +/- 37.0 hours versus 128.7 +/- 133.4 hours, p = 0.002). No significant differences were observed between MUF and control patients for any parameter in the presence of ventricular septal defect without pulmonary hypertension, tetralogy of Fallot, or aortic stenosis. CONCLUSIONS: Modified ultrafiltration after CPB is safe and decreases the need for homologous blood transfusion, the duration of ventilatory support, and chest tube placement in selected patients with complex congenital heart disease. The optimal use of MUF includes patients with preoperative pulmonary hypertension, neonates, and patients who require prolonged CPB.

Anterior pericardial tracheoplasty for congenital tracheal stenosis: intermediate to long-term outcomes.

BACKGROUND: Although several techniques for the treatment of long-segment stenosis of the trachea have been reported, including slide tracheoplasty, rib grafting, and use of a pericardial patch, the optimal repair remains controversial because of a lack of midterm to long-term follow-up data. METHODS: To assess the intermediate and long-term outcomes of patients having repair with anterior pericardial tracheoplasty, we reviewed case histories of 12 patients (1984 to present). The median age was 6.7 months (range, 1 to 98 months), and the median weight was 6.0 kg (range, 0.97 to 42 kg). All patients underwent anterior pericardial tracheoplasty through a median sternotomy during partial normothermic cardiopulmonary bypass. An average of 13 tracheal rings (range, five to 23) were divided anteriorly, and a patch of fresh autologous pericardium was used to enlarge the trachea by 1.5 times the predicted diameter for patient age and weight. RESULTS: There was one hospital death, and all but 2 patients are long-term survivors. All but 1 current survivor remain asymptomatic, with no bronchoscopic evidence of airway obstruction or granulation on the pericardial patch. All survivors examined have normal tracheal growth and development, with a median follow-up of 5.5 years (range, 1 to 11 years). CONCLUSIONS: Anterior pericardial tracheoplasty for congenital tracheal stenosis provides excellent results at intermediate to long-term follow-up.

Effect of omental, intercostal, and internal mammary artery pedicle wraps on bronchial healing.

Bronchial transection and devascularization is necessary in the course of sleeve resection or lung transplantation, leaving distal bronchial segments ischemic and subject to stricture or dehiscence. Thirty mongrel dogs underwent left lung autotransplantation. The bronchial anastomosis was wrapped with omentum (n = 9), intercostal muscle pedicle (n = 9), or internal mammary artery pedicle grafts (n = 6). Six control animals underwent bronchial anastomosis without an external wrap. Bronchial revascularization by capillary ingrowth from the pedicle to the bronchial submucosal plexus was demonstrated with all three types of vascular pedicle grafts; however, more consistent and confluent vascular ingrowth was provided by internal mammary artery pedicle grafts. Additionally, the bronchial anastomotic cross-sectional area was significantly better in the internal mammary artery group (84.5 +/- 3.3) as compared with that of the omental (68.4 +/- 8.3), intercostal muscle (66.9 +/- 10.9), or control groups (70.2 +/- 7.6). An internal mammary artery pedicle graft and the presence of dense confluent submucosal vascular ingrowth from any pedicle graft were independently predictive (p less than 0.05) of minimizing bronchial anastomotic narrowing. These data are consistent with previous findings suggesting that omental and intercostal muscle pedicle grafts promote early bronchial revascularization; moreover, the data demonstrate the superiority of an internal mammary artery pedicle graft to provide submucosal vascular ingrowth and to minimize anastomotic stenosis.