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Biomedical subjects

Charles B Huddleston

Publications and source records attributed to Charles B Huddleston.

At least 19 recordsLinked to original sources
PubMed2007

Infant heart transplantation ten years later--where are they now?

BACKGROUND: Many uncertainties regarding the fate of children undergoing heart transplantation as infants were present when we and others embarked on this program. Although no truly long-term results are available, a significant cohort of children has now reached preteen and early teenage status. We reviewed our group of infants transplanted more than 10 years ago to assess survival and quality of life as they approach their teenage years. METHODS: We retrospectively reviewed the medical records of all infant (younger than 6 months of age) heart transplant recipients, transplanted between 1988 and 1995, to ascertain survival statistics, incidence of complications, and current health status. RESULTS: A total of 42 patients were identified. The majority of these underwent transplantation for hypoplastic left heart syndrome. Eleven patients have died, 4 early and 7 late. The actual survival at 10 years is 76%. Twenty-seven of the 31 long-term survivors attend regular school; 4 are in special education classes owing to developmental delay. Five patients take medication for attention-deficit disorder. Malignancies have been discovered in 5, and 1 died secondary to this. Six patients have significant renal insufficiency, 1 of whom has undergone renal transplantation. One patient has undergone retransplantation for coronary artery disease. One patient required reoperation for supravalvar aortic stenosis. Other general medical problems that are being treated include sleep apnea (n = 1), hypertension (n = 5), and recurrent pneumonias (n = 1). CONCLUSIONS: Although these children require ongoing medical attention, including daily medications and regular follow-up visits, most have a satisfactory quality of life and behave much like normal children.

Child↗
PubMed2006

Long-term outcomes after infant lung transplantation for surfactant protein B deficiency related to other causes of respiratory failure.

OBJECTIVE: To determine if the outcomes of lung transplantation for infants with surfactant protein-B (SP-B) deficiency are unique. STUDY DESIGN: From a prospective analysis to identify infants with genetic causes of surfactant deficiency, we identified 33 SP-B-deficient infants from 1993 to 2005, and, among those undergoing lung transplantation (n = 13), compared their survival, pulmonary function, and developmental progress with infants who underwent transplantation at <1 year of age for parenchymal lung disease (n = 13) or pulmonary vascular disease (n = 11). RESULTS: Five-year survival rates ( approximately 50%, P = .3) and causes of death were similar for all three groups once the infants underwent transplantation. However, significant pretransplantation mortality decreased 5-year survival from listing to approximately 30% (P = .17). Pulmonary function, development of bronchiolitis obliterans, and school readiness were similar among the three groups. We detected anti SP-B antibody in serum of 3 of 7 SP-B-deficient infants and none of 7 SP-B-sufficient infants but could not identify any associated adverse outcomes. CONCLUSIONS: Long-term outcomes after infant lung transplantation for SP-B-deficient infants are similar to those of infants transplanted for other indications. These outcomes are important considerations in deciding to pursue lung transplantation for infants with disorders of alveolar homeostasis.

Female↗
PubMed2006

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.

Adolescent↗
PubMed2006

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.

Adolescent↗
PubMed2006

Living donor lobar grafts improve pediatric lung retransplantation survival.

OBJECTIVE: Lung retransplantation is a controversial practice due to increased morbidity and mortality and the scarcity of available donor organs. Living donor lobar lung transplantation increases the number of available donor organs and facilitates a more organized procedure than traditional cadaveric donation for this complex reoperation. The purpose of this study was to evaluate our experience with pediatric lung retransplantation and to compare the outcomes of living donor lobar lung transplantation with cadaveric donation. METHODS: Retrospective review of a prospectively collected database identified 39 children who underwent lung retransplantation from 1991 to 2004. Retransplantation was performed with living donor lobar lung transplantation in 13 patients and cadaveric donation in 26 patients. Short- and long-term outcomes were compared between the 2 groups. RESULTS: Perioperative mortality was 1/13 (7.7%) in the patients who had living donor lobar lung transplantation versus 11/26 (42.3%) in the cadaveric donation group (P = .03). Five-year survival for living donor lobar lung transplantation and cadaveric donation was 40.4% and 29.7%, respectively (P = .27). Both groups had a significant improvement in their forced expiratory volume in 1 second 6 months after retransplantation (P < .001). Multivariate analysis identified the use of cadaveric donation (relative risk = 6.16, P = .001) and early graft dysfunction (relative risk = 6.19, P = .001) as the major independent predictors of decreased survival following retransplantation. CONCLUSIONS: Living donor lobar lung transplantation reduces perioperative mortality and is an independent predictor of improved survival following pediatric lung retransplantation. This strategy offers significant benefit for this high-risk group and preserves the limited supply of donor lungs for other children at risk of dying while waiting for lung transplantation.

Adolescent↗
PubMed2006

Pediatric lung transplantation.

Lung transplantation in children has been performed since the early 1990s. The indications are cystic fibrosis, pulmonary vascular disease, and a variety of other pulmonary problems leading to death in small children. These other diseases include abnormalities in the metabolism of surfactant producing severe respiratory insufficiency in newborn infants. Many problems accompany these patients before transplantation and many problems are produced both by the transplant itself and immunosuppressant drugs which these children are obliged to take following transplantation. Nonetheless, lung transplantation offers the only real hope of survival in many instances. The long-term results have been somewhat discouraging with a 5-year survival of approximately 50%. The major causes of mortality late following lung transplantation are bronchiolitis obliterans, infections, and malignancy. The development of newer immunosuppressant drugs may offer hope for better results in the future.

Adolescent↗
PubMed2006

A new era in the surgical treatment of atrial fibrillation: the impact of ablation technology and lesion set on procedural efficacy.

BACKGROUND/OBJECTIVE: While the Cox-Maze procedure remains the gold standard for the surgical treatment of atrial fibrillation (AF), the use of ablation technology has revolutionized the field. To simplify the procedure, our group has replaced most of the incisions with bipolar radiofrequency ablation lines. The purpose of this study was to examine results using bipolar radiofrequency in 130 patients undergoing a full Cox-Maze procedure, a limited Cox-Maze procedure, or pulmonary vein isolation alone. METHODS: A retrospective review was performed of patients who underwent a Cox-Maze procedure (n = 100), utilizing bipolar radiofrequency ablation, a limited Cox-Maze procedure (n = 7), or pulmonary vein isolation alone (n = 23). Follow-up was available on 129 of 130 patients (99%). RESULTS: Pulmonary vein isolation was confirmed by intraoperative pacing in all patients. Cross-clamp time in the lone Cox-Maze procedure patients was 44 +/- 21 minutes, and 104 +/- 42 minutes for the Cox-Maze procedure with a concomitant procedure, which was shortened considerably from our traditional cut-and-sew Cox-Maze procedure times (P < 0.05). There were 4 postoperative deaths in the Cox-Maze procedure group and 1 in the pulmonary vein isolation group. The mean follow-up was 13 +/- 10, 23 +/- 15, and 9 +/- 10 months for the Cox-Maze IV, the pulmonary vein isolation, and the limited Cox-Maze procedure groups, respectively. At last follow-up, freedom from AF was 90% (85 of 94), 86% (6 of 7), and 59% (10 of 17) in the in the Cox-Maze procedure group, limited Cox-Maze procedure group, and pulmonary vein isolation alone group, respectively. CONCLUSIONS: The use of bipolar radiofrequency ablation to replace Cox-Maze incisions was safe and effective at controlling AF. Pulmonary vein isolation alone was much less effective, and should be used cautiously in this population.

Atrial Fibrillation↗
PubMed2005

Early outcome after Glenn shunt and Fontan palliation and the impact of operation during viral respiratory season: analysis of a 19-year multi-institutional experience.

BACKGROUND: This study was done to investigate the impact of season of operation, age at operation, and surgical era on short-term outcome after single ventricle palliation. One small study suggests that operation during viral respiratory season is associated with prolonged pleural effusion and hospitalization. Single-institution studies also find improved outcomes after the Glenn shunt and Fontan over time, despite operation at a younger age. METHODS: The Pediatric Cardiac Care Consortium (PCCC) database for Fontan (n = 2,713) and Glenn shunt (n = 2,239) performed between 1982 and 2000 was utilized. Death during initial hospitalization and length of stay after each procedure were investigated. Viral respiratory season was defined as the interval of November to March. RESULTS: The mortality rate after Fontan was higher during the viral respiratory season (14 versus 11%, p = 0.03) and in children under age 2 years. Glenn shunt mortality was higher with operation before age of 120 days, but was not affected by operation during viral respiratory season. Age at operation for the Fontan procedure has decreased with declines in hospital mortality (14% to 7%, p < 0.0001) and hospital stay (14 to 11 days, p < 0.0001) comparing 1992 to 1995 with 1996 to 2000. CONCLUSIONS: Fontan procedure performed during viral respiratory season is associated with an increased mortality. Short-term mortality and morbidity after the Glenn and Fontan procedures have improved in the recent era. Operation in the youngest age groups may adversely impact mortality.

Cardiac Surgical Procedures↗
PubMed2005

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.

Child↗
PubMed2005

Epidemiology of bloodstream infections in the first year after pediatric lung transplantation.

BACKGROUND: Substantial morbidity and mortality rates are associated with infections in the first year after pediatric lung transplantation. To understand better the clinical significance of bloodstream infections (BSIs), we evaluated systematically the epidemiologic features of BSIs in the first year after transplantation. METHODS: A retrospective case-cohort study of pediatric primary lung transplant recipients was performed. The frequency of BSIs and the organisms isolated were determined through medical and laboratory record review. We assessed variations in causative organisms and rates of BSIs in 3 time periods after transplantation, ie, early (0-30 days), intermediate (31-90 days) and late (91-365 days). RESULTS: Between July 1990 and November 2000, 190 pediatric patients received primary lung transplants. Twenty-six percent (49 of 190) of recipients had at least 1 BSI. The most commonly isolated organisms were coagulase-negative Staphylococcus (n = 25, 28.4%), Pseudomonas aeruginosa (n = 14, 16.0%) and Candida spp. (n = 9, 10.2%). The overall rate of BSIs was 2.1 episodes per 1000 catheter-days. The highest rate of BSIs occurred in the early period, compared with the intermediate and late periods (5.5, 1.3 and 1.6 episodes per 1000 catheter-days, respectively; P = 0.21). Early BSIs were associated with death in the first year after transplantation (relative risk, 3.9; 95% confidence interval, 1.6-9.4; P = 0.002). CONCLUSIONS: BSIs occur frequently after primary pediatric lung transplantation, with the highest rate being in the first 30 days after transplantation. Early BSIs are associated with death in the first year after transplantation.

Adolescent↗
PubMed2004

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.

Adolescent↗
PubMed2004

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.

Adolescent↗
PubMed2004

Mediastinal wound infections following pediatric cardiac surgery.

Deep sternal wound infections in children and neonates occur at approximately the same incidence as in adults. The organisms responsible are generally Staphylococcal species. The risk factors in adults include diabetes mellitus, the use of internal mammary artery grafts for bypass conduits, and other factors which clearly relate to age and the nature of the operations performed. In children the risk factors include leaving the sternum open following repair, nasal colonization with Staphylococcal species, need for re-exploration for bleeding, and others. Although conventional therapy for this complication is similar to what is proposed for adults, recently interest has been established in treating with debridement and primary closure of the sternum.

Anti-Bacterial Agents↗
PubMed2004

Outcomes and late complications after pulmonary resections in the pediatric population.

Neonates and children undergo lung resections for a variety of indications. These procedures can be performed with low mortality and low morbidity rates in the early postoperative period. There exist important anatomic and physiologic differences between the pediatric and the adult population, which thoracic surgeons need to be aware of. On the one hand, the capacity of the lung to grow during the first few years of life is associated with relative preservation of postoperative pulmonary function in children. On the other hand, the relative flexibility of their tissue structures places pediatric patients at increased risk for developing postpneumonectomy syndrome. Moreover, surgeons need to take the child's growth and development into consideration when planning their operative approach for thoracic procedures.

Breast↗
PubMed2004

Pectus excavatum.

One of the more common chest wall anomalies seen in children is pectus excavatum. Although some studies suggest a physiologic impact of this anomaly on cardiac function during exercise, this remains somewhat controversial. Regardless, a number of children are symptomatic from either the appearance of the deformity or from the standpoint of tolerance to exercise. Most are relieved of these symptoms with surgical repair. Several different operations have been utilized for repair of this anomaly, but two techniques now are the predominant methods in use today. These are the modified Ravitch procedure and the Nuss procedure. Both have been shown to provide satisfactory results.

Funnel Chest↗
PubMed2003

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.

Adult↗