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C B Huddleston

Publications and source records attributed to C B Huddleston.

At least 19 recordsLinked to original sources
PubMed2001

Does the extent of proximal or distal resection influence outcome for type A dissections?

BACKGROUND: The extent of proximal and distal aortic resection that should be performed for acute type A aortic dissections remains controversial. METHODS: From 1984 to 1999, 119 patients underwent repair of an acute type A dissection. Distal resection was to the ascending aorta in 78 (66%) and hemiarch in 41 (34%) patients. Proximally, the aortic valve was preserved in 69 (58%) patients, 40 (34%) underwent composite valve grafting, and 10 (8%) underwent separate graft and valve replacement. RESULTS: Operative mortality was higher for separate graft and valve (50%+/-16%) than for valve preservation (16%+/-5%) or composite grafts (20%+/-7%) (p < 0.05). Hemiarch replacement did not increase operative risk compared to distal reconstruction to the ascending aorta (17%+/-6% versus 22%+/-5%, p > 0.71). At 10 years, freedom from reoperation was 81%+/-7% and long-term survival was 60%+/-8%, but neither was related to the proximal or distal surgical technique (p > 0.15). Risk factors for late reoperation included a nonresected primary tear and Marfan syndrome (p < 0.05). CONCLUSIONS: An aggressive surgical approach, including a full root or hemiarch replacement, is not associated with increased operative risk and should be considered when type A dissections extensively involve the valve, sinuses, or arch.

Acute Disease↗
PubMed2001

Repair of anomalous left main coronary artery arising from the pulmonary artery in infants: long-term impact on the mitral valve.

BACKGROUND: Infants presenting with anomalous left coronary artery off the pulmonary artery (ALCAPA) are generally in heart failure and often have significant mitral valve regurgitation (MR). Although establishing a dual coronary circulation is the procedure of choice, there remains controversy as to how the mitral valve is handled. METHODS: We reviewed our experience with this lesion at St. Louis Children's Hospital. Over the past 15 years, 17 infants under 18 months of age have undergone repair, with all but one being treated with reimplantation of the left coronary artery into the aorta; the other underwent the Takeuchi procedure (intrapulmonary artery baffle) and was excluded from this evaluation. The average age and weight at operation were 0.5 +/- 0.3 years and 6.1 +/- 1.9 kg, respectively. All presented with varying degrees of heart failure and 9 patients also had either moderate or severe MR. RESULTS: There was one early and no late deaths after reimplantation of the left coronary artery. The left ventricular function postrepair improved from a preoperative shortening fraction of 0.19 +/- 0.09 to 0.34 +/- 0.08 (p < 0.01). Moderate or severe MR was present in 2 patients postoperatively, and both developed significant obstruction in the left coronary artery postoperatively as well. Both underwent mitral valve repair and revascularization of the left coronary artery. CONCLUSIONS: Excellent results can be obtained in the treatment of this very high-risk group of patients. Mitral valve repair is not generally necessary at the time of the initial operation. However, should MR recur or persist late, it may herald the presence of a coexistent, significant coronary stenosis. Cardiac catheterization should be performed to assess the patency of the left coronary artery before performing mitral valve surgery.

Child, Preschool↗
PubMed2001

Long-term prostacyclin infusion to reduce pulmonary hypertension in a pediatric cardiac transplant candidate prior to transplantation.

Pulmonary hypertension represents a significant risk factor for peri-operative death in patients undergoing cardiac transplantation. Heart-lung transplantation is generally the only procedure available for patients whose pulmonary hypertension can not be reversed by conventional pharmacologic means. We present a pediatric patient with end-stage cardiac disease and refractory pulmonary hypertension who was treated with long-term intravenous prostacyclin. This resulted in a significant enough improvement in her hemodynamics to allow for successful cardiac transplantation alone.

Adolescent↗
PubMed2001

Lung transplantation in infants.

Lung transplantation is an accepted mode of therapy for patients with end-stage pulmonary vascular or parenchymal diseases. To date, application to the pediatric age population in general, and the infantile group in particular, has been limited both in terms of number of transplants and follow-up thereafter. We performed our first transplant in an infant in 1993 and have performed 30 lung transplants in children under 1 year of age subsequent to that. This chapter will review the experience to date including indications for transplantation, post-transplant management, complications, and long-term follow-up.

Bronchiolitis Obliterans↗
PubMed2000

Quality of life after aortic valve replacement at the age of >80 years.

BACKGROUND: The optimal management of aortic valve disease in patients >80 years old depends on functional outcome as well as operative risks and late survival. METHODS AND RESULTS: We retrospectively identified 133 patients (62 men, 71 women) aged 80 to 91 years (mean 84+/-3 years) who underwent aortic valve replacement alone or in combination with another procedure between January 1, 1993, and April 31, 1998. Demographics included hypertension 68%, diabetes mellitus 17%, and history of stroke 11%. Operative (30 day) mortality rate was 11%. Urgent or emergent surgery, aortic insufficiency, and perioperative stroke or renal dysfunction were risk factors for operative death by multivariable analysis. Intensive care unit and total hospital length of stay were prolonged at 6.2 and 14.7 days, respectively. Late follow-up between July 1, 1998, and November 1, 1999, was 98% complete. Actuarial survival at 1 and 5 years was 80% and 55%, respectively. Predictors of late mortality were preoperative or perioperative stroke, chronic obstructive pulmonary disease, aortic stenosis, and postoperative renal dysfunction. The mean New York Heart Association functional class for 65 long-term survivors improved from 3.1 to 1.7. Quality of life assessed with the Medical Outcomes Study Short Form-36 was comparable to that predicted for the general population >75 years old. CONCLUSIONS: Functional outcome after aortic valve replacement in patients >80 years old is excellent, the operative risk is acceptable, and the late survival rate is good. Surgery should not be withheld from the elderly on the basis of age alone.

Age Factors↗
PubMed2000

High incidence of posttransplant lymphoproliferative disease in pediatric patients with cystic fibrosis.

A major cause of morbidity and mortality following lung transplantation is posttransplant lymphoproliferative disease (PTLD). In a retrospective cohort analysis of pediatric patients, we evaluated the risk factors associated with PTLD in 128 first-time lung transplant recipients from 1990 to 1997. The greatest risk factor for PTLD was a diagnosis of cystic fibrosis (CF). Of the 16 patients in our analysis who had PTLD, 13 had a diagnosis of CF (odds ratio [OR]: 5.8; confidence interval 95% [CI]: 1.6 to 21.4). Because of the high frequency of PTLD in patients with CF (13 of 61; 23%), we performed a retrospective cohort analysis in which patients with CF and PTLD were designated as cases and patients with CF and without PTLD served as controls. In patients with CF, the only risk factor associated with PTLD was two or more episodes of acute rejection within 3 mo after transplantation (OR: 11.0; 95% CI: 2.7 to 55.7). Age, recipient Epstein-Barr virus or cytomegalovirus status, induction with antilymphocyte globulin or antithymocyte globulin (ATG), or use of ATG or OKT3 for acute rejection episodes were not risk factors for PTLD. The high frequency of PTLD in the subgroup of patients with two or more episodes of graft rejection within 2 mo after lung transplantation was unexpected, and warrants further investigation in prospective clinical studies and basic laboratories.

Adolescent↗
PubMed1999

Combined lung and liver transplantation in a girl with cystic fibrosis.

PURPOSE: To describe the anesthetic considerations of a combined lung and liver transplant in a 14-yr-old girl with cystic fibrosis. CLINICAL FEATURES: A 14 yr-old girl with cystic fibrosis presented for combined liver and lung transplantation. Anesthetic management was complex in that the pulmonary, hemodynamic, and hematological changes after cardiopulmonary bypass and lung transplantation made the management of the subsequent liver transplant unique. We used a moderate dose fentanyl and isoflurane anesthetic with invasive monitoring including a pulmonary artery catheter. Upon reperfusion of the new liver our patient exhibited severe pulmonary hypertension that was associated with a decrease in cardiac output and systemic hypotension. Utilizing a pulmonary artery catheter, this episode was treated with an increase of prostaglandin E1 (PGE1) infusion to 0.025 microg x kg(-1) x min(-1) and the initiation of 3 microg x kg(-1) x min(-1) dobutamine. The pulmonary hypertension resolved and the cardiac output and blood pressure returned to baseline levels. CONCLUSION: The anesthetic considerations for a combined lung and liver transplant are complex because of the interactions and alterations in cardiovascular, pulmonary and hemostatic systems. The use of a pulmonary artery catheter was critical to the management of our patient because it allowed us to accurately treat an episode of hypotension occurring during liver transplantation. This episode was secondary to acute pulmonary hypertension which is common after pulmonary transplantation but unusual during liver transplantation. It is also critical that a team approach is used to consider all of the concerns of the multiple services managing these complex patients.

Adolescent↗
PubMed1999

Scimitar syndrome presenting in infancy.

BACKGROUND: Scimitar syndrome has a variable presentation based on the age at which the diagnosis is made. In general, infants presenting in heart failure have a greater number of associated anomalies and their prognosis is much worse. METHODS: We reviewed the records of all patients under our care at St. Louis Children's Hospital who presented with symptoms attributable to scimitar syndrome during their infancy. Twelve patients were identified. The average age at presentation was 6 weeks. RESULTS: The most common symptom at presentation was tachypnea. The chest roentgenogram demonstrated dextroposition of the heart and hypoplastic right lung. Only 1 patient had the classic "scimitar sign." Cardiac catheterization demonstrated pulmonary hypertension (pulmonary artery systolic pressure, 73.9+/-21.8 mm Hg). The Qp:Qs was 3.1+/-1.5:1. Two patients with severe associated anomalies were treated medically and both died. Two patients underwent occlusion of the systemic collaterals; one died and the other ultimately underwent complete repair due to persistence of the symptoms of heart failure. Two patients had primary right pneumonectomy and both are alive and well. Seven patients underwent complete repair (one after coil occlusion of the systemic arterial collaterals) and one died; three subsequently developed occlusion of the baffle from the orifice of the anomalous pulmonary vein and required pneumonectomy. Two patients required lung transplantation due to persistent pulmonary hypertension in one and recurrent bilateral pulmonary venous stenosis in the other. CONCLUSIONS: Infants presenting with scimitar syndrome generally have symptoms of tachypnea and chest roentgenograms showing dextrocardia and hypoplastic right lung. Although repair of the anomalous venous return and ligation of collaterals is generally recommended, right pneumonectomy (either as primary therapy or if repair failed) had similar early and late results.

Collateral Circulation↗
PubMed1999

Rejection with heart failure after pediatric cardiac transplantation.

BACKGROUND: Rejection associated with heart failure or death occurs after pediatric cardiac transplantation but has had limited analysis. METHODS: We analyzed the records of 96 consecutive pediatric cardiac transplant recipients who survived to hospital discharge. RESULTS: Eighteen patients (19%) experienced 23 episodes of heart failure or death associated with rejection. Univariate analysis demonstrated black race (p = 0.041), transplantation after 12 months of age (p = 0.032), later time after transplantation (p = 0.037), rejection episode in the first year after transplantation (p = 0.001), and history of two or more rejection episodes (p < 0.001) were significantly associated with rejection seen with heart failure. A multivariate regression analysis identified two or more rejection episodes to be the only independent risk factor for the development of rejection with heart failure (odds ratio 20; 95% confidence limits, 4-104; p < 0.0001). CONCLUSIONS: This study identified pediatric heart transplant recipients with a history of previous rejection episodes to be at a higher risk for symptomatic or fatal rejection. Further studies are needed to determine if intensification of maintenance immunosuppression, long-term rejection surveillance, or both in patients with multiple rejection episodes could reduce morbidity and mortality from rejection.

Child↗
PubMed1999

Intestinal obstruction after lung transplantation in children with cystic fibrosis.

BACKGROUND/PURPOSE: Distal intestinal obstruction syndrome (DIOS) occurs in 15% of patients with cystic fibrosis (CF). The authors reviewed their experience to determine the incidence, risk factors, and natural history of adhesive intestinal obstruction and DIOS after lung transplantation. METHODS: Eighty-three bilateral transplants were performed in 70 CF patients between January 1990 and September 1998. All were on pancreatic enzymes preoperatively, and none had preoperative bowel preparation. Fifty-six patients (80%) had prior gastrostomy (n = 54) or jejunostomy (n = 2). Eighteen patients (25.7%) had a previous laparotomy for meconium ileus (n = 8), fundoplication (n = 4), liver transplant (n = 1), jejunal atresia (n = 1), Janeway gastrostomy takedown (n = 1), pyloromyotomy (n = 1), free air (n = 1), or appendectomy (n = 1). RESULTS: After lung transplantation, 7 patients (10%) required laparotomy for bowel obstruction (6 during the same hospitalization, and 1 during a subsequent hospitalization). The causes of obstruction were adhesions only (n = 1), DIOS only (n = 2), and a combination of DIOS and adhesions (n = 4). Adhesiolysis was performed in the 5 patients with adhesions, and a small bowel resection was also performed in 1 patient. DIOS was treated by milking secretions distally without an enterotomy (n = 3) with an enterotomy and primary closure (n = 1) or with an end ileostomy and mucus fistula (n = 2). Five had recurrent DIOS early postoperatively. One resolved with intestinal lavage, 2 were treated successfully with hypaque disimpaction, and 2 underwent reoperation; 1 required an ileostomy. The most important risk factor for posttransplant obstruction was a previous major abdominal operation. Obstruction occurred in 7 of 18 (39%) who had undergone a prior laparotomy versus 0 of 52 who had not (P < .001, chi2). CONCLUSIONS: (1) The incidence of intestinal obstruction is high after lung transplantation in children with CF. (2) Previous laparotomy is a significant risk factor. (3) Recurrent obstruction after surgery for this condition is common. (4) Preventive measures such as pretransplant bowel preparation and early postoperative bowel lavage may be beneficial in these patients.

Adolescent↗
PubMed1999

Lung transplantation in very young infants.

INTRODUCTION: Established successes with adult lung transplantation have laid the foundation for extension of this therapeutic modality to infants and children dying of end-stage pulmonary disease. The purpose of this report is to convey our experience with 19 infants undergoing lung transplantation before the age of 6 months. METHODS: Six patients with predominantly pulmonary vascular disease and 13 patients with primarily pulmonary parenchymal disease have undergone bilateral sequential lung transplantation at our institution since 1990. Mean age at transplant was 104 +/- 44 days, and mean weight was 4.9 +/- 1.6 kg. RESULTS: Although early mortality (32%, 6/19) was higher than that previously reported for older pediatric age groups, long-term survival was similar (44% at a maximum follow-up of 6 years). Although anastomotic complications and infections occurred at a rate approximating that seen in older pediatric age groups, episodes of acute rejection appear to occur with decreased frequency. Similarly, at a mean follow-up of 3 years, only 2 (15%) of 13 long-term survivors have evidence of bronchiolitis obliterans. The functional residual capacity, as measured on infant pulmonary function tests, has gradually increased as the children have grown, suggesting that lung growth is occurring. CONCLUSIONS: Bilateral lung transplantation is a viable alternative in infants dying of end-stage pulmonary disease. Efforts directed toward avoiding the complications that lead to early posttransplant mortality combined with the seemingly lower incidence of early and late rejection may provide long-term results better than those in other age groups.

Actuarial Analysis↗
PubMed1999

Growth of lungs after transplantation in infants and in children younger than 3 years of age.

We report serial measurements of lung volume and airflow in small children after lung transplantation. We expected that immature lungs could grow and develop normal volumes after transplantation, despite denervation and immunosuppression. At predetermined intervals, functional residual capacity (FRC) and forced expiratory flow were measured 86 times in 23 recipients younger than 3 yr of age (age at transplant, 13.2 +/- 8.4 mo; range, 2 to 30 mo). FRC was measured using open-circuit N2 washout. Maximal flow at FRC by rapid thoracoabdominal compression was used to distinguish between infants with and those without airflow obstruction. The slope of FRC (in milliliters) versus body length (in centimeters) for all 23 recipients studied was 8.63. For those children without obstruction (flow at FRC >/= 0.9 FRC/s, n = 16), the slope of FRC versus length was 6.61. The coefficient of variation for FRC measurements for all infants was 3.90 +/- 2.80% (range, 0.3 to 16.9%). We conclude that in the absence of significant airflow obstruction the volume of transplanted immature lungs increases at a rate similar to that reported in normal infants.

Body Height↗
PubMed1999

Scimitar syndrome.

Explore the source record for details and available documents.

Cardiac Catheterization↗
PubMed1998

Patterns and potential value of cardiac troponin I elevations after pediatric cardiac operations.

BACKGROUND: Perioperative myocardial injury is a major determinant of postoperative cardiac dysfunction for congenital heart disease, but its assessment during this period is difficult. The objective of this study was to determine the suitability of using postoperative serum concentrations of cardiac troponin I (cTnI) for this purpose. METHODS: Cardiac troponin I levels were measured serially in the serum of patients undergoing uncomplicated repairs of atrial septal defect (n = 23), ventricular septal defect (n = 16) or tetralogy of Fallot (n = 16). The concentrations were correlated with intraoperative parameters (cardiopulmonary bypass time, aortic cross-clamp time, and cardiac bypass temperature), and postoperative parameters (magnitude of inotropic support, duration of intubation, and postoperative intensive care and hospital stay). RESULTS: Postoperative absolute cTnI levels were lesion specific, with a pattern of increase and decrease similar for each lesion. For the total cohort, significant correlations between postoperative cTnI levels at all times (r = 0.43 to 0.83, p < 0.05) until 72 hours were noted for all parameters, except for cardiac bypass temperature. When evaluated as individual procedure groups, no significant relationships were noted in the atrial septal defect group, whereas postoperative cTnI levels were more strongly correlated with all intraoperative and postoperative parameters in the ventricular septal defect group than in the tetralogy of Fallot group. CONCLUSIONS: This study suggests that cTnI values immediately after operation reflect the extent of myocardial damage from both incisional injury and intraoperative factors. Cardiac tropinin I levels in the first hours after operation for congenital heart disease are a potentially useful prognostic indicator for difficulty of recovery.

Body Temperature↗
PubMed1998

Lung retransplantation in children.

BACKGROUND: Early primary graft failure due to reperfusion injury may occur in up to 10% of all patients undergoing lung transplantation. Late graft failure in the form of bronchiolitis obliterans progressively increases in frequency as posttransplantation follow-up increases. In both situations, the degree of pulmonary dysfunction may worsen and result in the death of the recipient. The only treatment in many instances is retransplantation. The results in adults are reasonably well established. METHODS: We reviewed our experience in children. Of the 136 transplant procedures performed to date in children, 14 have been retransplantations. Six patients required retransplantation for early primary graft failure and 8 underwent retransplantation for bronchiolitis obliterans. RESULTS: There were three early and three late deaths. The actuarial survival at 2 years is 58%. The retransplant procedures were more complex than the primary transplant operations as evidenced by the longer time on cardiopulmonary bypass (199 +/- 71 versus 150 +/- 41 minutes; p < 0.01) and the greater volume of blood transfused (1,303 +/- 936 versus 570 +/- 300 mL; p < 0.01). Two of the long-term survivors who received transplants for bronchiolitis obliterans have subsequently had development of this same condition and 1 died secondary to this. In four instances living related donors were used for the retransplant procedure. The most striking difference in these procedures compared with those transplantations performed with cadaveric donors was the shorter donor lung ischemic times (99.5 and 123.3 minutes for the two lungs for living related donors and 251 and 293 minutes for the first and second lung for the cadaveric donors; p < 0.01). CONCLUSIONS: We believe that lung retransplantation in children is a reasonable therapy to offer in the circumstance of severe graft dysfunction. In the older child, the option of living donor transplantation offers advantages that might offset of the overall higher risk of this procedure.

Actuarial Analysis↗
PubMed1998

A canine model of atrial flutter following the intra-atrial lateral tunnel Fontan operation.

Atrial flutter (AFL) is a common problem in children who have undergone a Fontan operation for single ventricle physiology. Although this has been attributed to the atrial stretch inherent in the earlier forms of this operation, AFL has persisted in spite of a modification that minimizes atrial distension. Therefore, it was hypothesized that AFL following the modified Fontan procedure may result from anatomic barriers related to suture lines rather than from atrial stretch or hypertension. In a series of experiments performed in dogs under general anesthesia, the modified Fontan repair was simulated by placing only the suture line of the intra-atrial repair. No baffle was placed, thus avoiding any hemodynamic alterations. After closure of the atriotomy, 253 point unipolar atrial endocardial form-fitting electrodes were inserted through the mitral and tricuspid valves via bilateral ventriculotomies. Induction of AFL was attempted with atrial burst pacing and programmed extrastimulation, and activation sequence maps of subsequent reentry were generated from the endocardial electrodes. Atrial flutter was induced in all of 17 dogs, with a median cycle length of 177 +/- 31 ms. Activation sequence maps demonstrated conduction block along the crista terminalis corresponding to the free wall portion of the suture line. This created an isthmus between the suture line and tricuspid annulus, which appeared critical for sustaining AFL, although the circuit used both the septal and free wall surfaces of the right atrium. In seven dogs, a cryolesion was placed from the tricuspid annulus to the free wall segment of the suture line, terminating the AFL, in all seven. When the free wall segment of the suture line was moved 5 mm medial to the crista terminalis, AFL was induced in four of five dogs, but only in the presence of isoproterenol and at a shorter cycle length (136 +/- 8 ms, P < .001). Atrial flutter was not inducible, even with the addition of isoproterenol, in any of five dogs in which the suture line was placed 10 mm anterior to the crista terminalis and incorporated into closure of the atriotomy. This acute canine model of the modified Fontan operation demonstrates that conduction block from the free wall portion of the suture line creates an isthmus of tissue between the suture line and the tricuspid annulus. This is a sufficient substrate to produce AFL; no hemodynamic alteration is required. Injury to the crista terminalis is a significant risk factor in this model, which suggests that a modification of the suture line might reduce the incidence of AFL in patients following this operation.

Animals↗
PubMed1998

Pediatric and adult lung transplantation for cystic fibrosis.

OBJECTIVE: This paper was undertaken to review the experience at our institution with bilateral sequential lung transplantation for cystic fibrosis. METHODS: Since 1989, 103 bilateral sequential lung transplants for cystic fibrosis have been performed (46 pediatric, 48 adult, 9 redo); the mean age was 21 +/- 10 years. Cardiopulmonary bypass was used in all but one pediatric (age <18) transplant, and in 15% of adults. RESULTS: Hospital mortality was 4.9%, with 80% of early deaths related to infection. Bronchial anastomotic complications occurred with equal frequency in the pediatric and the adult populations (7.3%). One- and 3-year actuarial survival are 84% and 61%, respectively (no significant difference between pediatric and adult age groups; average follow-up 2.1 +/- 1.6 years). Mean forced expiratory volume in 1 second increased from 25% +/- 9% before transplantation to 79% +/- 35% 1 year after transplantation. Acute rejection occurred 1.7 times per patient-year, with most episodes taking place within the first 6 months after transplantation. The need for treatment of lower respiratory tract infections occurred 1.2 times per patient in the first year after transplantation. Actuarial freedom from bronchiolitis obliterans was 63% at 2 years and 43% at 3 years. Redo transplantation was performed only in the pediatric population and was associated with an early mortality of 33%. Eight living donor transplants (four primary transplants, four redo transplants) were performed with an early survival of 87.5%. CONCLUSION: Patients with end-stage cystic fibrosis can undergo bilateral lung transplantation with morbidity and mortality comparable to that seen in pulmonary transplantation for other disease entities.

Actuarial Analysis↗