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Desensitization (DS) is widely used to decrease PRA in solid organs transplant candidates (IC). Various numbers of cycles of DS are required to reduce or eliminate donor specific antibodies (DSA). The goal of this study was to investigate if there was a correlation between polymorphism (PM) of some cytokine genes and intensity of DS required to make the recipient/donor cross match compatible. Thirty-one TCs were included in the study. Antibody specificity, percent of reactive antibodies (PRA) and serum concentration of cytokines were analyzed using the LUMINEX platform. PCR-SSP method was used for lL-1α, lL-1β, IL-IR, IL-1Rα, IL-4Rα, lL-12, lFNγ, TGFβ1, TNFα, IL-2, IL-4, IL-6 and IL-10 gene PM analysis. Significant relationship between PM of genes encoding IL-4Rα, IFNγ and IL-12 (p70) and susceptibility to DS was demonstrated (p = 0.04, p = 0.01 and p = 0.05 respectively). Correlation between elevated serum level of IL-12 (p70) and A/A or C/A genotype at -1188 position was found in resistant to DS TCs (p = 0.015). These results indicate that analysis PM of genes encoding IL-4Rα, IFNγ and IL-12 enables to define the DS strategy in TCs more accurately regarding the number of plasmapheresis (PP) cycles and dose of intravenous immunoglobulin (IVIG). [ABSTRACT FROM AUTHOR]

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.

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.

Between 1993 and 2000, 50 patients (age range, 1 month to 18 years) who had left ventricular outflow tract (LVOT) disease and had undergone a Ross procedure were assessed using echocardiography. Aortic annulus size, valvular gradient, valve insufficiency, LV dimensions at end-systole and end-diastole, LV interventricular septal and posterior wall thickness, and LV mass index (LVMI) were measured. There was 1 early and 2 late deaths, and 5 reoperations. Aortic annulus size increased and degree of aortic insufficiency (AI) decreased, as did LV internal dimensions and LVMI. Peak pressure gradients declined from 73 +/- 18 mm Hg to 7 +/- 7 mm Hg, and LVMI regressed (167 +/- 6 g/m(2) v 108 +/- 6 g/m(2), P <.001) after 3 years. The degree of AI ranged from none to mild, and no patient has LVOT stenosis. Regression of LV dilatation and hypertrophy, good autograft valve function and durability, and a high survival rate suggest that the Ross procedure is preferred for most children who require aortic valve replacement., (Copyright 2001 by W.B. Saunders Company)

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.

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.

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.

Various valved and nonvalved external right ventricle (RV) to pulmonary artery (PA) conduits have been used to palliate congenital heart anomalies. The ideal conduit has not been found. Reasons for conduit failures include stenosis, thrombosis, calcification of the valve or graft wall, and development of an obstructive peel. We evaluated valved and nonvalved conduits constructed from a glutaraldehyde preserved segment of bovine jugular vein. Bovine jugular conduits (n = 31), 10-13 mm in diameter, were implanted into weight-matched adult mongrel dogs using a standard closed heart technique. Valved conduits (VC, n = 17) were stented at the valve annulus with a Gore-Tex ring, whereas the nonvalved conduits (NC, n = 14) were stented at their midpoint. The proximal PA was tightly banded to 3 mm with a ligature. Cardiac output (CO) and hemodynamic gradients were measured at the time of insertion and 8 months postoperatively. Pulmonary artery angiograms were used to assess bovine jugular conduit regurgitation. All xenografts were evaluated by gross and histologic exam. Two dogs had conduits placed but died for reasons unrelated to the conduit before evaluation. Valved conduit leaflets showed thickening, insignificant thrombus deposition in the base of one or more cusps, and a mild degree of regurgitation as assessed by angiograms. Examination of the NC showed mild conduit thickening and a moderate-to-severe degree of regurgitation as assessed by angiograms. There was a significant difference observed in pulmonary outflow gradients between the VC (11 +/- 2 mm Hg) and NC (17 +/- 2 mm Hg) (p < 0.05), although neither group developed a hemodynamically significant gradient. On gross examination, VC ventricles displayed significantly less evidence of volume and pressure overload compared with the NC ventricle. Valved conduits demonstrated significantly less obstruction and regurgitation. The potential clinical advantages of bovine jugular conduits are their availability, potential durability evidenced by lack of early calcification, and the advantage of not requiring a proximal extension for the RV anastomosis. The presence of a durable and functional xenograft valve in valved conduits may prevent postoperative sequelae in some patients.

Perforation of newly placed left internal mammary artery (LIMA) grafts due to stent deployment is an infrequent but potentially dangerous complication of coronary interventions. It may lead to brisk hemorrhage and massive cardiac tamponade requiring emergent pericardiocentesis and surgery. We report a case of a LIMA graft perforation following stent deployment with a high-pressure balloon 12 days after surgery. The patient was treated with emergent pericardiocentesis, rapid autotransfusion of the pericardial aspirate into the systemic circulation, and surgical repair of the ruptured vessel.

Repair of congenital right ventricular outflow tract obstruction often requires reconstruction with a transannular patch to alleviate pulmonary stenosis. Post repair pulmonary insufficiency with right ventricular dilatation and volume overload may result and lead to acute or progressive right heart failure. The use of a monocusp valve has been proposed as a means to prevent this problem. Fresh pericardium is well known to fail clinically, leading to pulmonary insufficiency limiting mid- and long-term results. In a chronic dog model (147 +/- 34 days), three valve types were evaluated: 1) polytetrafluoroethylene (PTFE; n = 9), 2) fresh pericardium (PERI; n = 6), and glutaraldehyde fixed pericardium (GLU; n = 6). Hemodynamics, angiography, and echocardiography were performed at implantation and sacrifice. Gross and microscopic pathology were evaluated. No significant differences were found among the three groups with regard to stenosis as evaluated by echocardiography, measured right ventricular wall thickness, and hemodynamic pressure gradients across the valve. By echocardiography, both PTFE (1 of 9) and GLU (0 of 6) showed less regurgitation than PERI (5 of 6) (p < 0.05). This was confirmed by angiography. PTFE showed less neo-intimal hyperplasia, less thrombus formation, and less calcification than GLU or PERI (p = NS). The PTFE monocusp developed no prohibitive gradients, no early pathologic deterioration, and maintained competence compared with the GLU and PERI groups. Although continued investigation of long-term durability and competence of the PTFE monocusp valve is warranted, both PTFE and GLU values seem to demonstrate less regurgitation than the PERI monocusp valve in an adult dog model of right ventricular outflow tract reconstruction.

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.

Background: Pulmonary hypertension causes major morbidity and mortality after congenital heart surgery, but its mechanism remains unclear., Methods and Results: Plasma endothelin-1 (ET-1), nitric oxide (NO), and cyclic GMP (cGMP) were assayed at 6 intervals in 50 children undergoing cardiopulmonary bypass (CPB): before CPB, 10 minutes into CPB, and 0, 3, 6, and 12 hours after CPB. Three groups based on pulmonary flow and pressure were analyzed: low flow (LF, n=21), high flow/low pressure (systolic pulmonary pressure/systemic pressure ratio, Pp/Ps<50%, HF-LP, n=11), and high flow/high pressure (Pp/Ps> or =50%, HF-HP, n=19). HF-HP and HF-LP received alpha-blockers (chlorpromazine and/or prazosin). HF-HP patients received nitric oxide donors (nitroglycerin/sodium nitroprusside). ET-1 peaked at 6 hours, with its highest level in the HF-HP group (P<.01, by ANOVA). ET-1 correlated significantly with Pp/Ps at 6 hours (r2=.43, P<.005). In the HF-HP group, ET-1 remained above the other groups at 12 hours (12.7+/-2.5 pg/mL versus 6.4+/-1.1 pg/mL versus 6.5+/-3.8 pg/mL P<.05 by ANOVA). NO metabolites were elevated equivalently for the HF-HP and HF-LP groups (5.7+/-2.6 micromol/L versus 0.3.5+/-2.5 micromol/L at 12 hours, P=NS) despite nitric oxide donors and the excess ET-1 in HF-HP patients. Levels of cGMP were similarly elevated in HF-HP and HF-LP patients during this study., Conclusions: Endogenous NO may decrease vascular tone and maintain low pulmonary pressure in HF-LP patients. High levels of ET-1, inadequate NO production, and/or impaired responses to NO may increase pulmonary pressure in HF-HP patients.

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.

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.

Background: Reports of surgical correction of total anomalous pulmonary venous connection (TAPVC) over the past 30 years indicate a general improvement in operative survival. However, prevention of late pulmonary venous obstruction continues to be a cornerstone of successful repair. The purpose of the study was to identify factors associated with improvement in perioperative mortality and to determine risk factors for death and reoperation due to pulmonary vein stenosis after repair of TAPVC., Methods and Results: Using univariate and multiple regression analysis, we analyzed risk of early and late mortality and need for reoperation in 105 patients operated on between April 1966 and June 1995. Despite increased frequency of neonatal repair in the most recent time period (29% in 1966 through 1985; 55% in 1991 through 1995, P < .05), operative mortality declined (13% in 1966 through 1985; 0% in 1991 through 1995). The incidence of postoperative pulmonary hypertensive episodes and death related to pulmonary hypertension decreased significantly over the study period (P < .001). Aggressive preoperative elective medical stabilization and prophylaxis of postoperative pulmonary hypertensive episodes may have contributed to this improvement. By univariate analysis, preoperative pulmonary hypertension (P < .02) and preoperative pulmonary vein obstruction (P < .01) correlated with early mortality up to 1990 but not in the past 5 years. Multiple logistic regression analysis showed that only a small pulmonary confluence associated with diffuse pulmonary vein stenosis was an independent risk factor for early (P < .001) and late (P = .01) death as well as need for reoperation (P = .007). Type of TAPVC was not a significant risk factor throughout the three decades of our experience. At a median follow-up of 87 months, late survival was 98% (93 of 95 operative survivors), and all are NYHA class I., Conclusions: Improvements on surgical technique as well as preoperative and postoperative management account for the reduction in mortality and need for reoperation for most types of TAPVC. However, the presence of a small venous confluence and diffuse pulmonary vein stenosis remains a risk factor for adverse outcome.

Long-segment tracheal stenosis in infants and children is difficult to manage and can be life-threatening. Patients frequently have associated cardiac, other respiratory, or gastrointestinal anomalies that may confuse the diagnosis at initial presentation. The rarity of congenital tracheal stenosis has not allowed sufficient experience for the development of standard treatment protocol. Several surgical techniques have been described but have varying results. This article reviews the diagnosis and different surgical options for congenital tracheal stenosis and their outcomes.

Background: The treatment of infants with hypoplastic left heart syndrome has been challenging and controversial., Methods: To assess the operative management and intermediate-term outcome, we retrospectively analyzed our surgical experience with 50 newborns with hypoplastic left heart syndrome operated on between January 1989 and June 1995., Results: Surgical palliation with a first-stage Norwood operation was offered to 28 patients. The remaining 22 infants were initially listed for heart transplantation, and 15 underwent the operation. Ten of the 15 recipients are alive, and all are in New York Heart Association class I. Seven infants underwent a Norwood procedure after being on the list for transplantation for 12 to 42 days. A total of 34 patients underwent Norwood procedures with one operation aborted because of inoperable anatomy. Two infants who survived the first-stage Norwood operation underwent subsequent heart transplantation and are currently doing well. The 1-year mortality rate for heart transplantation was 18% (3/17) versus 50% (17/34) for the Norwood procedure. Risk factors for early mortality after a Norwood procedure include longer circulatory arrest time (> 50 minutes), preoperative acidosis (pH < 7.20), larger systemic-pulmonary artery shunt (> or = 4 mm), diminutive ascending aorta (< or = 2.0 mm), and anatomic subtype of aortic and mitral atresia. The 1-year survival rate for the Norwood procedure improved from 36% for the patients operated on during 1989 through 1992 to 75% during 1993 to mid-1995 (p = 0.005). Of the 17 survivors of a first-stage Norwood operation, 10 have undergone the second stage (bidirectional Glenn procedure), and 7 have completed a Fontan procedure. Heart transplantation results have also improved, with no deaths since 1992., Conclusions: Both the Norwood procedure and heart transplantation have encouraging early to intermediate results in infants with hypoplastic left heart syndrome. Hypoplastic left heart syndrome should be managed selectively on the basis of cardiac morphology, donor availability, and family wishes. Development of a flexible program involving the use of both procedures may aid in the successful management of infants with hypoplastic left heart syndrome.

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.

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.

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.

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.

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 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)

As the population continues to age, older patients are being referred for thoracic surgical procedures with increasing frequency. From 1985 through 1992, 38 patients (32 men, 6 women) 70 years of age or older underwent esophagectomy for primary esophageal carcinoma. Histologic findings included adenocarcinoma in 28 (74%) and squamous carcinoma in 10 (26%). Patients suffered dysphagia for a mean of 3.8 months (range, 0 to 30 months) and had a mean weight loss of 5.8 kg (range, 0 to 22 kg). The tumors ranged from 1 to 14 cm in length and averaged 4.7 cm. Preoperative chemotherapy and radiation therapy were administered in 11 patients (46%). Clinical staging suggested all patients were curable, and esophagectomy was performed in a transthoracic fashion in 27 (71%) and from a transhiatal approach in 11 (29%). Cervical anastomoses were undertaken in 16 patients (42%). The mean blood loss was 1,165 mL and ranged from 500 to 4,000 mL. The mean number of transfused units was 2.3 (range 0 to 8 U). Overall operative mortality was 18% (7 of 38). Major morbidity included pneumonia in 11 (29%), anastomotic leak in 4 (11%), chylothorax in 4 (11%), pulmonary embolus in 3 (8%), and stroke and myocardial infarction in 1 patient each (3%). Three patients have been cured of their esophageal cancer with survivals of 65, 70, and 72 months and an additional 7 patients are still alive. Three patients (8%) have been lost to follow-up.(ABSTRACT TRUNCATED AT 250 WORDS)

Considerable improvements have been made in the diagnosis and treatment of congenital heart disease during the last decade. Many congenital heart lesions are now treated successfully during the neonatal period that previously were associated with high mortality. Improved echocardiographic imaging, catheterization techniques, and earlier surgical repair are factors that have resulted in greater success in the treatment of congenital cardiac disease. Diagnosis has been improved greatly with advancements in echocardiography and angiography. Better ultrasound technology combined with doppler techniques and transesophageal echocardiography allow more accurate preoperative assessment and therefore more successful surgical repair. Cardiac catheterization techniques have also improved and, when combined with treatment such as balloon angioplasty, have changed the treatment of certain cardiac anomalies such as pulmonary stenosis or coarctation of the aorta. Operative treatment of congenital heart disease has improved the short- and long-term survival of most infants with congenital cardiac anomalies. Improved cardiopulmonary bypass techniques, better suture material, and the ability to perform cardiac transplantation are examples of technology that allows earlier, more complete repair of these complex cardiac defects. Reviewed here are improvements in the treatment of four complex cardiac anomalies that occur in newborns and are associated with high mortality when left untreated. All four anomalies have undergone significant changes in the approach to their treatment with dramatic improvements in survival.

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.

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.

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.

Carotid arteriography can be misleading in that roentgenographic occlusion of the internal carotid artery (ICA) may be suggested when the artery is actually anatomically patent. The distinction of occlusion versus pseudo-occlusion is crucial in recommending proper treatment. Aroused by the misinterpretation of two arteriograms by our radiology departments, a review of 780 arteriograms done on 780 patients during a three year period was begun. Of these, eight (1.0 per cent) symptomatic patients had conflicting arteriographic reports, with the radiologists reporting complete occlusion and the authors describing pseudo-occlusion. Established arteriographic criteria for pseudo-occlusion of the ICA are emphasized. These eight patients underwent carotid arterial exploration and all were found to have patent ICA. After successful carotid endarterectomy, seven of the eight patients have remained asymptomatic and have had no hemodynamically significant stenosis on noninvasive testing in the follow-up period, ranging from two to 38 months. Therefore, in evaluating patients with carotid territory symptoms, knowledge of the established arteriographic criteria for ICA pseudo-occlusion should alert the physician to the possibility of ICA patency. If this is suspected, the patient should undergo exploration of the carotid artery with subsequent endarterectomy if patency is demonstrated.