The role of thromboelastography in directing blood product usage in infant open heart surgery.

OBJECTIVE: : Thromboelastography (TEG) measures the dynamics of clot formation in whole blood and provides data that can guide specific blood component therapy. This study analyzed whether the implementation of TEG affected blood product utilization and overall hemostasis in infants (6 months and younger) undergoing open heart surgery. METHODS: : TEG values measured include R (time to fibrin formation), angle (fibrinogen formation), and MA (platelet function). Blood product usage, TEG values, and operative parameters were collected during surgery on 112 consecutive infants (66 acyanotic) undergoing open heart surgery within the first 6 months of life. Controls consisted of chart data on 70 consecutive patients (57 acyanotic) undergoing the same surgical procedures before implementation of TEG (pre-TEG). RESULTS: : Using TEG, the pattern of blood product utilization changed. Compared with the pre-TEG era, TEG era patients demonstrated a significant increase in fresh frozen plasma usage intraoperatively (4.74 vs. 1.83 mL/kg; P < 0.001) and reduced postoperative use of platelets (1.69 vs. 3.74 mL/kg; P = 0.006) and cryoprecipitate (0.89 vs. 1.95 mL/kg; P = 0.149). Chest tube drainage was significantly reduced at 1, 2, and 24 hours in the TEG group.TEG angle and MA measurements suggest that fibrinogen and platelets of cyanotic patients are more sensitive to hemodilution than the acyanotic patients. CONCLUSIONS: : TEG allows for proactive, goal-directed blood component therapy with improved postoperative hemostasis in infants undergoing cardiopulmonary bypass.

Bronchial airway anastomotic complications after pediatric lung transplantation: incidence, cause, management, and outcome.

OBJECTIVE: Airway complications are a recognized surgical complication and an important source of morbidity after adult lung transplantation. Little is known about these complications after pediatric lung transplantation. METHODS: Data of pediatric lung transplants performed between January 1990 and December 2002 in a single pediatric institution were reviewed retrospectively. RESULTS: A total of 214 patients, with a mean age of 9.8 +/- 6.1 years (range 0.01-19.7 years), underwent 239 lung transplants: 231 bilateral and 8 single. Mean follow-up was 3.4 years. Forty-two airway complications requiring interventions (stenosis = 36; dehiscence = 4; malacia = 2) developed in 30 recipients (complication rate: 9% of 470 bronchial anastomoses at risk). There were airway complications in 29 bilateral lung transplants (13%) and 1 single lung transplant (13%). Mean time to diagnosis was 51 +/- 27 days (median: 53, range 1-96 days), and diagnoses were made in 90% of patients within the first 3 months after transplantation. Preoperative Pseudomonas cepacia, postoperative fungal lung infection, and days on mechanical ventilator were found to be significant risk factors on multivariate analysis (P = .002, P = .013 and P = .003, respectively). Treatment included rigid bronchoscopic dilatation in 17 patients, balloon dilatation in 13 patients, and stent placement in 12 patients. Other treatments consisted of debridement, fibrin glue application, chest tube placement, and pneumonectomy followed by retransplantation. No patients died as a direct result of airway complications. There was no significant difference in the incidence of bronchiolitis obliterans or overall survival in comparison with patients who did not have airway complications. CONCLUSIONS: Airway complications are a significant cause of morbidity after pediatric lung transplantation. The majority are successfully treated, and patient outcomes are not adversely affected.

Role of open lung biopsy in lung transplant recipients in a single children's hospital: a 13-year experience.

BACKGROUND: There are few data in the literature regarding the utility of open lung biopsy for the assessment of graft dysfunction after pediatric lung transplantation. The aim of this study is to review our experience with diagnostic open lung biopsy in lung transplant recipients in a children's hospital. METHODS: Records of lung transplant recipients from January 1990 through December 2002 were reviewed to identify the indications, outcomes, and complications of open lung biopsy. RESULTS: Two hundred twenty-four patients (mean age, 9.9 +/- 6.2 years; median age, 11 years; age range, 0.01-19.6 years) underwent 249 lung transplantations: 231 bilateral, 8 single, and 10 heart-lung transplantations. Mean follow-up was 3.4 years. One hundred three open lung biopsies were performed in 89 (40% of all recipients) patients. Thirteen recipients underwent open lung biopsy twice, and 1 recipient had 3 open lung biopsies. The indications for open lung biopsy were suspicion of bronchiolitis obliterans (n = 70), posttransplantation lymphoproliferative disorder (n = 15), infection (n = 8), and unexplained respiratory failure (n = 10). A new diagnosis was made in 49 biopsies (48%), 50 biopsies (49%) confirmed the preoperative clinical diagnosis, and 4 biopsies (3%) were nondiagnostic. Bronchiolitis obliterans was confirmed in 40 (57%) of 70 open lung biopsies, posttransplantation lymphoproliferative disorder was confirmed in 4 (27%) of 15 open lung biopsies, and infection was confirmed in 6 (75%) of 8 open lung biopsies. A change in therapy occurred in 69% of the cases as a result of the diagnosis made from open lung biopsy. There was no mortality as a direct result of open lung biopsy. Eleven major complications and 22 minor complications occurred in 103 procedures. CONCLUSION: Open lung biopsy can be performed safely, and established or confirmed a diagnosis in 97% of the cases. A change in therapy occurred in 69% of the cases as a result of the diagnosis made from open lung biopsy. In our experience open lung biopsy appears to be a useful tool.

Primary graft dysfunction and other selected complications of lung transplantation: A single-center experience of 983 patients.

OBJECTIVES: We sought to review the incidence and outcome of lung transplantation complications observed over 15 years at a single center. METHODS: We performed a retrospective review from our databases, tracking outcomes after adult and pediatric lung transplantation. The 983 operations between July 1988 and September 2003 included 277 pediatric and 706 adult recipients. Bilateral (74%), unilateral (19%), and living lobar transplants (4%) comprised the bulk of this experience. Retransplantations accounted for 44 (4.5%) of the operations. RESULTS: The groups differed by indication for transplantation. The adults included 57% with emphysema and 17% with cystic fibrosis, and the children included no patients with emphysema and 50% with cystic fibrosis. Hospital mortality was 96 (9.8%) of 983, including 46 (17%) of 277 of the children and 50 (7%) of 706 of the adults. The overall survival curves did not differ between adults and children ( P = .56). Freedom from bronchiolitis obliterans syndrome at 5 and 10 years was 45% and 18% for adults and 48% and 30% for children, respectively ( P = .53). The causes of death for adults included bronchiolitis obliterans syndrome (40%), respiratory failure (17%), and infection (14%), whereas the causes of death in children included bronchiolitis obliterans syndrome (35%), infection (28%), and respiratory failure (21%) ( P < .01). Posttransplantation lymphoproliferative disease occurred in 12% of pediatric recipients and 6% of adults ( P < .01). The frequency of treated airway complications did not differ between adults and children (9% vs 11%, P = .48). The frequency of primary graft dysfunction did not differ between children (22%) and adults (23%), despite disparity in the use of cardiopulmonary bypass. CONCLUSION: These results highlight major complications after lung transplantation. Despite differences in underlying diagnoses and operative techniques, the 2 cohorts of patients experienced remarkably similar outcomes.

Repair of congenital heart lesions combined with lung transplantation for the treatment of severe pulmonary hypertension: a 13-year experience.

OBJECTIVE: In patients with severe pulmonary hypertension associated with congenital heart disease, we prefer to perform repair of the congenital heart disease and lung transplantation whenever feasible so as to augment the donor pool and avoid the cardiac complications associated with heart transplantation. We report our experience with repair of congenital heart disease and lung transplantation and compare the results with those of patients who underwent heart-lung transplantation during the same period. METHODS: The records of patients who had repair of congenital heart disease and lung transplantation (n = 35) and heart-lung transplantation (n = 16) between 1990 and 2003 were reviewed. RESULTS: The underlying congenital heart disease in the repair of congenital heart disease and lung transplantation group included transposition of great vessels (n = 2), atrioventricular canal defect (n = 2), ventricular septal defect (n = 9), pulmonary venous obstruction (n = 7), scimitar syndrome (n = 2), pulmonary arterial atresia or stenosis (n = 5), and others (n = 8). Thirteen of the patients undergoing repair of congenital heart disease and lung transplantation (37.1%) had the congenital heart disease repaired before lung transplantation; the remaining congenital heart disease repairs were performed concurrently with transplantation. Sixteen patients underwent heart-lung transplantation because of poor left ventricular function or single-ventricle anatomy. Freedoms from bronchiolitis obliterans at 1, 3, and 5 years were 72.9%, 54.7%, and 54.7% for the repair of congenital heart disease and lung transplantation group and 77.8%, 51.9%, and 38.9% for the heart-lung transplantation group, respectively. Survivals at 1, 3, and 5 years were 62.9%, 51.4%, and 51.4% for the repair of congenital heart disease and lung transplantation group and 66.5%, 66.5%, and 60% for the heart-lung transplantation group, respectively. CONCLUSION: Repair of congenital heart disease and lung transplantation is a feasible treatment option. Long-term outcome is determined by associated complications related to lung transplantation. Despite the complexity of combined congenital heart disease repair with lung transplantation and the resulting perioperative morbidity, the patients had similar outcomes to those of patients who underwent heart-lung transplantation.

Sequestrations, congenital cystic adenomatoid malformations, and congenital lobar emphysema.

The respiratory system begins to develop at 3 weeks gestation, and aberrations in developmental processes may give rise to a group of structural abnormalities collectively referred to as bronchopulmonary foregut malformations (BPFMs). These lesions or anomalies may subsequently present in the newborn period as pulmonary parenchymal abnormalities in association with significant respiratory compromise. This article briefly reviews fetal lung development and then proceeds to delineate the characteristics, presentation, and surgical treatment of 3 of the more common BPFMs, those being sequestrations, congenital cystic adenomatoid malformations, and infantile lobar emphysema. The various types of BPFMs may occur in conjunction with one another or in association with other congenital anomalies, and these lesions should be managed with a thoughtful and aggressive surgical approach.

Risk factors for primary graft failure after pediatric cardiac transplantation: importance of recipient and donor characteristics.

BACKGROUND: Primary graft failure, or circulatory insufficiency immediately after transplantation, frequently occurs after pediatric cardiac transplantation and is the most common cause of death after infant transplantation. Risk factors for pediatric primary graft failure are poorly defined. METHODS: We retrospectively reviewed donor, procedural and recipient characteristics for primary graft failure in 165 pediatric cardiac transplant recipients (median age at transplant 1.1 years) by multivariatle logistic regression. Primary graft failure was defined as the need for mechanical circulatory support or use of multiple intravenous inotrope/pressors, including epinephrine, for circulatory support within the first 24 hours after transplantation. RESULTS: Primary graft failure occurred in 54 patients (33%); 24 patients (15%) required mechanical support for their graft failure; and primary graft failure was the cause of death or graft loss in 10 patients. Recipient risk factors associated with an increased risk of primary graft failure included diagnosis of congenital heart disease and a need for mechanical support before transplantation. Ventilator support before transplantation and maximal pulmonary vascular resistance index were risk factors for the development of isolated right ventricular graft failure. Donor risk factors associated with an increased risk for primary graft failure included increasing donor recipient weight and body surface area ratios; increasing donor ischemic time; anoxia as a cause of death; and increasing cardiopulmonary resuscitation time. Donor blood type O+ and hyperdynamic donor systolic function were associated with a decreased risk of primary graft failure. CONCLUSIONS: Multiple donor, recipient and procedural risk factors, including the type and severity of heart disease in the recipient before transplantation, are associated with primary graft failure after pediatric cardiac transplantation. Avoidance of matching high-risk donors to high-risk recipients may improve morbidity and mortality after transplantation.

Non-ischemic left ventricular dysfunction after pediatric cardiac transplantation: treatment with plasmapheresis and OKT3.

BACKGROUND: Acquired left ventricular dysfunction after pediatric cardiac transplantation is associated with a high mortality rate. It often occurs without biopsy evidence of cellular rejection or severe transplant coronary arteriopathy. METHODS: We employed a protocol for treatment of acquired, non-ischemic left ventricular dysfunction utilizing plasmapheresis, monoclonal anti-T-cell antibody (OKT3), cyclophosphamide and steroids, regardless of the results of endomyocardial biopsy. Left ventricular dysfunction was defined as an echocardiographic shortening fraction of <29% and/or symptoms of congestive heart failure requiring inotropic support. Transplant coronary arteriopathy was excluded by coronary angiography in all cases. RESULTS: Ten pediatric heart transplant recipients were treated for 13 episodes of non-ischemic left ventricular dysfunction. Biopsy scores were low grade (ISHLT Grade 1A or 1B) in 8 episodes. Eight of 10 patients had a history of non-compliance in regularly taking immunosuppressant medications. Inotropic support was required in 9 of 13 cases, with a median duration of 5 days. Median left ventricular shortening fraction was 17% at time of presentation. Normalization of shortening fraction occurred a median of 40 days from the start of treatment. Survival to hospital discharge occurred in 11 of 13 (85%) patients. Long-term patient survival, however, was only 50% at 24 months after presentation with a first episode of acquired left ventricular dysfunction. CONCLUSIONS: Use of plasmapheresis, OKT3, cyclophosphamide and steroids resulted in successful short-term reversal of non-ischemic left ventricular dysfunction in pediatric heart transplant patients, but long-term survival remained poor.

Pediatric transplantation.

Analysis of the OPTN/SRTR database demonstrates that, in 2002, pediatric recipients accounted for 7% of all recipients, while pediatric individuals accounted for 14% of deceased organ donors. For children fortunate enough to receive a transplant, there has been continued improvement in outcomes following all forms of transplantation. Current 1-year graft survival is generally excellent, with survival rates following transplantation in many cases equaling or exceeding those of all other recipients. In renal transplantation, despite excellent early graft survival, there is evidence that long-term graft survival for adolescent recipients is well below that of other recipients. A causative role for noncompliance is possible. While the significant improvements in graft and patient survival are laudable, waiting list mortality remains excessive. Pediatric candidates awaiting liver, intestine, and thoracic transplantation face mortality rates generally greater than those of their adult counterparts. This finding is particularly pronounced in patients aged 5 years and younger. While mortality awaiting transplantation is an important consideration in refining organ allocation strategies, it is important to realize that other issues, in addition to mortality, are critical for children. Consideration of the impact of end-stage organ disease on growth and development is often equally important, both while awaiting and after transplantation.

Progressive lung disease and surfactant dysfunction with a deletion in surfactant protein C gene.

Mutations in the surfactant protein (SP)-C gene are responsible for familial and sporadic interstitial lung disease (ILD). The consequences of such mutations on pulmonary surfactant composition and function are poorly understood. To determine the effects of a mutation in the SP-C gene on surfactant, we obtained lung tissue at the time of transplantation from a 14-mo-old infant with progressive ILD. An in-frame 9-bp deletion spanning codons 91-93 in Exon 3 of the SP-C gene was present on one allele; neither parent carried this deletion. SP-C mRNA was present in normal size and amount. By immunofluorescence, proSP-C was aggregated within alveolar Type II cells in a compartment separate from SP-B. In airway surfactant, there was little or no mature SP-B or SP-C; SP-A content was increased. Minimum surface tension was increased (20 mN/m, normal < 5 mN/m). Type II cells contained normal and disorganized appearing lamellar bodies by electron microscopy. This spontaneous deletion on one allele of the SP-C gene was associated with sporadic ILD and abnormalities in surfactant composition and function. We propose that a dominant negative effect on surfactant protein metabolism and function results from aggregation of misfolded proSP-C and subsequent cell injury and inflammation.

Pediatric lung transplantation.

Lung transplantation usually provides prolongation and marked improvement in quality of life in patients who would otherwise die from end-stage pulmonary disease. Many questions remain unanswered and important deterrents exist to long-term survival of these patients. Included in the list of challenges for the future are (1) means of increasing the donor pool by better donor identification, (2) use of adjunctive therapies that might not only enhance the quality of preservation but also extend the safe period of ischemia. (3) innovative strategies such as use of non-heart-beating donors and living donor lung transplantation, and (4) research into the prevention and treatment of OB. While gene therapy and other interventions are important opportunities on the horizon that might eventually preclude the need for transplantation, optimizing our current understanding of lung transplantation provides the only survival opportunity for patients who are incapacitated with a variety of terminal lung diseases. The spectrum of diseases being treated and the potential for rehabilitation not only separates pediatric lung transplant recipients from their adult counterparts, it also provides unique challenges and opportunities in what is a fascinating patient population.

Lung transplantation in children following treatment for malignancy.

BACKGROUND: End-stage lung disease is a rare complication of treatment for hematologic and solid tumors in children. When present, it is generally progressive, resulting in the patient being cured of cancer only to die of respiratory failure. Lung transplantation is believed by some to be of overly high risk in this population because of the pre-existing malignancy as well as the presumed compromised immune status. METHODS: Six children (Group 1), aged 5 to 17 years (mean 12.4 years), underwent lung transplantation at our institution because of either pulmonary fibrosis or bronchiolitis obliterans following treatment for acute leukemia (n = 5) or medulloblastoma (n = 1). All patients received chemotherapy, radiation and bone marrow transplantation in the course of treatment for their malignancies. The average forced expiratory volume in 1 second (FEV(1)) was 16% of the predicted value and forced vital capacity (FVC) was 28% of predicted. These results were compared with a similar group of 13 patients undergoing lung transplantation at our institution during the same time interval (Group 2). RESULTS: There were 2 deaths in Group 1, 1 early and 1 late, for an overall survival of 67% at a mean follow-up of 4.02 years. There were no early and 7 late deaths in Group 2 for an overall survival of 46% at a mean follow-up of 4.8 years. The acute rejection rate in the first post-transplant year was 0.2 episode/patient in Group 1 and 1.8 episode/patient in Group 2 (p <0.01). No patient in Group 1 has developed post-transplant lymphoproliferative disease or a relapse of their primary malignancy. Two patients in Group 1 developed unusual infections-Aspergillus and Mycobacterium chelonae. No such infections occurred in Group 2. CONCLUSIONS: Although this represents a small series, we believe that patients with respiratory failure following treatment of a prior malignancy are suitable candidates for lung transplantation. Although they may have some relative protection from acute rejection episodes by virtue of an already compromised immunologic status while receiving standard immunosuppression, an increased propensity for opportunistic infection may exist.

Concomitant allorecognition of mismatched donor HLA class I- and class II-derived peptides in pediatric lung transplant recipients with bronchiolitis obliterans syndrome.

BACKGROUND: The authors' previous studies with 2 different adult patient populations demonstrated a correlation between indirect allorecognition of mismatched donor HLA Class I- and Class II-derived peptides and the development of bronchiolitis obliterans syndrome (BOS) after lung transplantation. The aim of this study was to determine whether a parallel allorecognition of mismatched donor HLA Class I- and Class II-derived peptides occurs after lung transplantation and to determine its correlation with the development of BOS after lung transplantation in a group of pediatric patients. METHODS: Peripheral blood mononuclear cells from 7 BOS-positive and 6 BOS-negative pediatric lung transplant recipients (age, 11.5 +/- 4.4 years) were cultured in the presence of synthetic peptides corresponding to the alpha-chain hypervariable regions of a mismatched donor HLA Class I molecule and the beta-chain hypervariable region of a mismatched donor HLA-DR molecule. The frequencies of HLA Class I and Class II alloreactive T cells were determined using limiting dilution analysis. RESULTS: A significant increase (p = 0.025) in HLA Class I-alloreactive T cells was observed in BOS-positive patients (7.1 x 10(-5) +/- 4.3 x 10(-5)) compared with BOS-negative patients (2.1 x 10(-5) +/- 1.8 x 10(-6)). In addition, a significant increase (p = 0.033) in HLA Class II-alloreactive T cells also was observed in BOS-positive patients (9.6 x 10(-5) +/- 7.9 x 10(-5)) compared with BOS-negative patients (1.3 x 10(-5) +/- 2.1 x 10(-6)). CONCLUSIONS: This study indicates that a parallel CD4+ T-cell alloreactivity to both donor HLA Class I and Class II molecules may play a role in the pathogenesis of BOS both in adult and pediatric lung transplant recipients.

Interleukin-6 and interferon-gamma gene polymorphisms in the development of bronchiolitis obliterans syndrome after lung transplantation.

BACKGROUND: A number of genetic polymorphisms have been shown to regulate the production and secretion of tumor necrosis factor (TNF)-alpha, transforming growth factor (TGF)-beta1, interferon (IFN)-gamma, interleukin (IL)-6, and IL-10. Several of these genetic polymorphisms have been shown to be associated with either acute or chronic rejection of kidney, liver, and heart allografts and with development of allograft fibrosis after lung transplantation. The aim of this study was to assess the effect of these genetic polymorphisms on the development of bronchiolitis obliterans syndrome (BOS) after lung transplantation. METHODS: Genetic polymorphisms were detected by means of polymerase chain reaction in 93 lung allograft recipients for functional polymorphisms in the TNF-alpha (-308), TGF-beta1 (+869 and +915), IL-6 (-174), IFN-gamma (+874), and IL-10 (-1082, -819, and -592) genes. Then, a correlation between BOS development and the presence of these cytokine genotypes was determined using Kaplan-Meier actuarial analysis. RESULTS: A significant correlation was detected between the presence of high-expression polymorphisms of the IL-6 and IFN-gamma genes and BOS development after lung transplantation (P =0.045 and 0.039, respectively). Also, patients with high-expression polymorphisms in both genes developed BOS significantly earlier than patients with low-expression polymorphisms in one or both genes, suggesting a synergistic effect of the alleles during BOS pathogenesis (P =0.016). No correlation was detected between polymorphisms of the TNF-alpha, TGF-beta1, and IL-10 genes and development of BOS after lung transplantation. CONCLUSIONS: The presence of high-expression polymorphisms at position -174 of the IL-6 gene and position +874 of the IFN-gamma gene significantly increases the risk for BOS development after lung transplantation.

The history of pediatric heart and lung transplantation.

As is the case with other forms of solid organ transplantation, success with heart and lung transplantation in the pediatric population was a natural extension of success with these procedures in adults. As a result, to review the history of pediatric heart and lung transplantation, one must by necessity review the landmarks in research and the events that gave way to successful heart and lung transplantation in adults.

Lung transplantation in children.

OBJECTIVE: To report the authors' experience with pediatric lung transplantation (LTX) to provide an overview of patients selected for this procedure and their outcomes. SUMMARY BACKGROUND DATA: Pediatric LTX differs from adults in many ways, including recipient size, indications, posttransplant care, and rehabilitation. METHODS: Two hundred seven isolated lung transplants on 190 children under the age of 18 years were performed from 1990 to the present. This represents the single largest series of lung transplants in children in the world. Thirty-two patients were less than 1 year of age, 22 were 1 to 5 years of age, 32 were 5 to 10 years of age, and 121 were 10 to 18 years old. The groups by major diagnostic category were cystic fibrosis (n = 89), pulmonary vascular disease (n = 44), bronchiolitis obliterans (n = 21), pulmonary alveolar proteinosis (n = 12), pulmonary fibrosis (n = 15), and other (n = 26). The average age at the time of transplant was 9.5 +/- 5.9 years (range 36 days to 18 years). RESULTS: Survival by Kaplan-Meier analysis was 77% at 1 year, 62% at 3 years, and 55% at 5 years. There was no significant difference in survival according to primary diagnosis leading to LTX or age at LTX. There were 25 early (<60 days) and 61 late deaths. The most common cause of early deaths was graft failure (13/25, 52%). The most common causes of late death were bronchiolitis obliterans (35/61, 57%), infection (13/61, 21%), and posttransplant malignancies (11/61, 18%). No patient died of acute rejection. In those surviving greater than 3 months (mean follow-up 3.5 years, range 3 months to 11 years), the overall rate of occurrence of bronchiolitis obliterans was 46% (80/175) and the overall incidence of posttransplant malignancies was 24/175 (14%). Major risk factors for the development of bronchiolitis obliterans were age older than 3 years, more than two episodes of acute rejection, and organ ischemic time longer than 180 minutes. CONCLUSIONS: In children, LTX is a high-risk but viable treatment for end-stage pulmonary parenchymal and vascular disease. The major hurdle to overcome in long-term survival is bronchiolitis obliterans.

Management of late-stage parapneumonic empyema.

PURPOSE: Despite the reported value of early video-assisted thoracoscopic surgery (VATS) for empyema, many children are still referred to the surgeon late in the disease process. The authors wished to determine the optimal management strategy for this group of children. METHODS: Medical records of all children (n = 70) from 1990 to 2000 with late-presenting empyema (stage II or III) were reviewed. Patients were grouped as (G1) successful management with chest tube (CT), (G2) surgery after initial CT, (G3) thoracentesis followed by surgery, and (G4) surgery alone. RESULTS: There were no significant differences with respect to age, gender, pleural cultures or fluid analysis. Fifty-one (73%) patients required surgical intervention. Treatment using CT (G1, G2) or thoracentesis (G3) was associated with prolonged length of stay (LOS) when compared with surgery alone (G4; 12 v 8 days). For G2, G3, and G4, rapid clinical improvement and early discharge (6 days) was seen after surgery. For all surgery groups (G2, G3, G4), video-assisted thoracoscopic surgery (n = 19) was associated with a longer postoperative fever (4 v 2 days; P <.05), but a shorter total LOS (12 v 15 days; P <.05) when compared with open decortication (n = 32). CONCLUSIONS: Over 70% of children with late presenting empyema required surgery, including more than half of the children who received initial chest tube drainage. Delay in surgery was associated with more procedures, more radiographs, and an increased LOS. Despite later intervention, patients undergoing surgery as an initial approach had the shortest length of stay. Early surgical intervention is indicated for most children referred with established empyema.

Lung transplantation for pulmonary vascular disease.

BACKGROUND: Pulmonary hypertension (PHT) is a lethal condition resulting in markedly diminished life expectancy. Continuous prostaglandin I2 infusion has made an important contribution to symptom management, but it is not a panacea. Lung or heart-lung transplantation remains an important treatment option for end-stage PHT patients unresponsive to prostaglandin I2. This study reviews the outcomes after transplantation for PHT in our program. METHODS: A retrospective chart review was performed for 100 consecutive patients with either primary PHT (48%) or secondary PHT (52%) transplants since 1989. Living recipients were contacted to confirm health and functional status. RESULTS: Fifty-five adult and 45 pediatric patients underwent 51 bilateral lung transplants, 39 single lung transplants, and 10 heart-lung transplants. Mean age was 23.7 years (range, 1.2 months to 54.8 years) and mean pre-transplant New York Heart Association class was 3.2. Pre-transplant hemodynamics revealed a mean right atrial pressure of 9.6+/-5.4 mm Hg and mean pulmonary artery pressure of 64+/-14.4 mm Hg. Hospital mortality was 17% with early death predominantly because of graft failure and infection. With an average follow-up of 5.0 years, 1- and 5-year actuarial survival was 75% and 57%, respectively. Mean pulmonary artery pressure on follow-up catheterization was 22+/-6.0 mm Hg, and mean follow-up New York Heart Association class was 1.3 (p < 0.001 for both compared with pre-transplant). Diagnosis and type of transplant did not confer a significant difference in survival between groups. CONCLUSIONS: Whereas lung or heart-lung transplant for PHT is associated with higher early mortality than other pulmonary disease entities, it provides similar long-term outcomes with dramatic improvement in both quality of life and physiologic aspects.