Effect of desensitization in solid organ transplant recipients depends on some cytokines genes polymorphism.

Desensitization (DS) is widely used to decrease PRA in solid organs transplant candidates (TC). 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 IL-1alpha, IL-1beta, IL-1R, IL-1Ralpha, IL-4Ralpha, IL-12, IFNgamma, TGFbeta1, TNFalpha, IL-2, IL-4, IL-6 and IL-10 gene PM analysis. Significant relationship between PM of genes encoding IL-4Ralpha, IFNgamma 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-4R, IFNgamma 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).

Repair of tetralogy of Fallot with anomalous coronary arteries coursing across the obstructed right ventricular outflow tract.

Anomalous coronary artery (ACA) anatomy occurs in 2-9% of patients with tetralogy of Fallot (TOF), in which the left anterior descending coronary artery (LAD) originates from the right coronary artery (RCA) crossing the right ventricular outflow tract. The purpose of this study was to review our results of repair for TOF with ACA. Between 1978 and 2001, 43 ACA patients (mean age, 4.8 years; range, 5 months-41 years) underwent repair for TC. The ACA anatomy was classified as the single LAD from the RCA (n = 20), a significant conal branch (dual LAD) from the RCA (n = 13), paired anterior descending arteries originating from the left and right coronary arteries (n = 7), and single RCA from the LAD (n = 3). In cases in which the anomalous LAD crossed the obstructed infundibulum, thinning or coring of the endocardium was done. Patch infundibuloplasty was performed in 39 patients, with 10 needing separate RV and pulmonary artery patches, and the pulmonary valve was preserved. Nine patients had the addition of a monocusp to a transannular incision. Two patients had a main pulmonary arterioplasty alone. There was one early (2.3%) and no late deaths. Mean early and late postoperative gradients were 21.5 +/- 10.5 mmHg (4 patients had > or =30 mmHg) and 27.1 +/- 13.7 mmHg (5 patients had >30 mmHg; p = 0.12), respectively. There were four reoperations during a mean follow-up of 4.8 years (range, 6 months to 18 years). Actuarial freedom from reoperation was 90% at 5, 10, and 15 years. At the latest follow-up, all patients were in NYHA functional class I. TOF repair for an ACA can be performed without disturbing the native coronary anatomy and without the use of conduits in most cases. Outcomes are similar to those of other patients with TOF. The presence of ACA does not impose increased risk after this surgical strategy.

Modified Fontan operation in patients with anomalies of systemic and pulmonary venous connection.

The presence of anomalies of the systemic and pulmonary venous connection associated with single ventricle anomalies has been considered a contraindication for the Fontan operation (FO). The aim of this study is to outline the technical considerations associated with the presence of anomalies of systemic and/or pulmonary venous connections and to identify the risk factors for mortality in this group of patients undergoing the modified FO. Between 1989 and 2004, 63 patients (median age, 3.2 years) with anomalous systemic or pulmonary venous connection underwent a Fontan procedure at our institution. Nine patients had a combination of anomalous systemic and pulmonary venous connection, 49 patients had anomalous drainage only from the systemic circulation, and 5 patients had isolated anomalies of pulmonary venous return. Visceral heterotaxy syndrome was diagnosed in 25 patients. Previous palliative operations had been performed in 51 patients (81%). There was 1 early death, and 2 patients required take down of Fontan procedures. Two patients required reoperation for revision of the atrial baffle. At a mean follow-up of 4.6 +/- 3.4 years, there have been 5 late deaths (8%) and 45 patients (71%) have undergone Fontan completion. Actuarial survival was 92% at 1 year and 91% at 5 and 10 years-not significantly different from the overall survival of the Fontan patients. We conclude that the modified FO can be successfully performed in patients with anomalous systemic or pulmonary venous connections, including those with visceral heterotaxy syndrome, with morbidity and mortality rates that do not differ significantly from those achieved in all patients with normal connections.

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.

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.

Truncus arteriosus repair: outcomes, risk factors, reoperation and management.

OBJECTIVES: Truncus arteriosus (TA) continues to be associated with significant morbidity and mortality, but there have been clinically significant improvements with early repair. METHODS: Sixty patients underwent physiological correction of TA between November 1978 and January 2000. The average age was 76 days (range, 3 days--20 months). Associated cardiac anomalies were frequently encountered, the most common being severe truncal valve regurgitation (n=7), interrupted aortic arch (n=6), coronary artery anomalies (n=6), non-confluent pulmonary arteries (n=4), and total anomalous pulmonary venous return (n=1). Truncal valve replacement was performed initially or subsequently in seven patients with severe regurgitation (mechanical prostheses in six patients and a cryopreserved aortic homograft in one patient). Right ventricle--pulmonary artery continuity was established with an aortic (n=16) or pulmonary homograft (n=32) in 48 patients, a Dacron polyester porcine valved conduit in five, a non-valved polytetrafluoroethylene (PTFE) tube in three, direct anastomosis to the right ventricle with anterior patch arterioplasty in three, and a bovine jugular venous valve conduit in one patient. RESULTS: There were ten hospital deaths (17%; 70% confidence limit, 7--25%). Multivariate and univariate analyses demonstrated a relationship between hospital mortality and associated cardiac anomalies. In the 43 patients without these associated cardiac anomalies, the early survival was 91% (group I). In the 17 patients with one or more of these risk factors, the survival was 71% (group II, P=0.002). There was one late death. Twenty-three patients (46%) required reoperation for right ventricular outflow tract (RVOT) obstruction at a mean follow-up time of 59.1 months. In 23 patients, the RVOT reconstruction was performed with a PTFE monocusp, and six patients had of a variety of replacement conduits inserted. Postoperatively, there were 34 (68%) patients in New York Heart Association functional class I and 16 (32%) in class II. Twenty-eight surviving patients are reported as doing well without any medication. The freedom of reoperation in the 39 hospital survivors (group I) without risk factors was 64% at 7 years; and 36% at 10 years in the 11 patients (group II) surviving with risk factors. CONCLUSIONS: Associated cardiac anomalies were risk factors for death after the repair of TA. In the absence of these associated lesions, TA can be repaired with an excellent surgical outcome in the neonatal and early infancy period.

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.

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.

Clinical outcomes and indicators of normalization of left ventricular dimensions after Ross procedure in children.

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.

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.

Valved bovine jugular venous conduits for right ventricular to pulmonary artery reconstruction.

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.

Left internal mammary artery graft perforation due to high-pressure stent deployment.

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.

Monocusp valve and transannular patch reconstruction of the right ventricular outflow tract: an experimental study.

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.

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.

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.

Dynamic changes of endothelin-1, nitric oxide, and cyclic GMP in patients with congenital heart disease.

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.

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.

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.

Surgical management of congenital tracheal stenosis.

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.

Surgical management of total anomalous pulmonary venous connection. Thirty-year trends.

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.