Search PubMed⌕ Search

Biomedical subjects

T L Spray

Publications and source records attributed to T L Spray.

At least 91 records · Page 5Linked to original sources
PubMed1996

Outcome of children with pulmonary hypertension referred for lung or heart and lung transplantation.

We reviewed our institutional experience with 24 children with pulmonary hypertension, who were referred for lung or heart and lung transplantation. Diagnosis, age, and previously published predictive survival scores calculated at the time of referral were analyzed as predictors of pretransplant death. Among the 24 children, 7 did not meet criteria for listing and 17 were listed for transplantation. Of those listed, eight died waiting, two await transplantation, and seven were transplanted and are alive and well 7-20 months after transplantation. Poor functional status (New York Heart Association class 3 or 4) at the time of referral was significantly associated with death before transplant (P=0.05) in univariate analysis. Analysis of the predictive scores was possible in 21 of 24 patients; lower predictive scores were significantly associated with death before transplantation and shorter duration of survival without transplantation in univariate analysis. Multivariate analysis (Cox regression) confirmed that lower scores were significantly associated with poor survival. We conclude that children with pulmonary hypertension are often referred for transplantation too late in the course of their disease. Early complete hemodynamic evaluation before the onset of severe symptoms, followed by serial evaluations of disease progression and consultation with a transplant center, should result in earlier, more appropriate time of listing and improved survival. A systematic study of pretransplant mortality among all children listed for lung transplantation would provide a basis for clinical decision making and policies affecting organ allocation.

Adolescent↗
PubMed1996

Embolic stroke after ligation of the pulmonary artery in patients with functional single ventricle.

In the setting of functional single ventricle with pulmonary overcirculation, pulmonary artery banding is frequently used to alleviate symptoms and to prepare for staged repair. At subsequent cavopulmonary anastomosis or Fontan procedure, the pulmonary artery may be ligated at the site of the pulmonary band. This article describes the association of embolic stroke and thrombus in a ligated or divided pulmonary artery stump in three patients with functional single ventricle. These events occurred from 1990 through 1992 among the 1700 inpatient pediatric cardiology admissions at two institutions. The patients, ranging in age from 15 months to 9 years, had cerebral infarctions documented by computed axial tomography scan or magnetic resonance imaging associated with the echocardiographic finding of thrombus in the proximal pulmonary artery stump after the embolic strokes. The strokes occurred 5 days to 5 years after surgery. Two patients had a second infarction within 2 to 5 weeks of the initial stroke. It is concluded that the presence of the ligated pulmonary artery stump may place patients at risk for embolic stroke. Surgical approaches to reduce the risk of thrombus formation should be considered prospectively in this patient group.

Cerebral Infarction↗
PubMed1996

Recognition and management of nonpenetrating cardiac trauma in children.

OBJECTIVE: To characterize the evaluation and clinical course of children with nonpenetrating injury to the heart. METHODS: We reviewed the medical records for children admitted to St. Louis Children's Hospital between the years 1987 to 1992 with traumatic cardiac injury. Patients with penetrating trauma were excluded; eight children, ages 4 to 13 years, were the study subjects. Chest x-ray studies, electrocardiograms, and serum creatine kinase values were obtained on admission. Two-dimensional echocardiography was performed when indicated by unexplained hemodynamic instability or abnormal radiographic findings. RESULTS: All children with nonpenetrating cardiac trauma were involved in a motor vehicle accident. The principal cardiac diagnoses were ventricular septal defect (1), mitral regurgitation (1), pericardial effusion (2), contusion (3), and arrhythmia (1). Multisystem injury was present in each case, but cardiac injury was not suspected at the time of admission in seven of the eight patients. The hemodynamic status of four children was compromised 12 to 48 hours after admission; echocardiography was diagnostic in each instance, but the electrocardiogram and creatine kinase values were nonspecific. Two patients eventually required cardiac surgery. CONCLUSIONS: Recognition of blunt cardiac trauma in children may be confounded by associated multisystem injury and the delayed onset of clinical manifestations. Echocardiography is a sensitive diagnostic tool for hemodynamically significant disease, and should be performed promptly when patients have unexplained hypotension or diminished peripheral perfusion.

Accidents, Traffic↗
PubMed1996

Echocardiographic characteristics of biopsy-proven cellular rejection in infant heart transplant recipients.

BACKGROUND: Echocardiography has been used as a primary means to detect cellular rejection in infant heart transplant recipients. There is, however, limited information correlating echocardiography and biopsy-proven rejection in this age group. METHODS: Between September 1989 and July 1994, 32 consecutive heart transplantations were done in infants younger than 20 months old, who were followed up for 2 to 58 months (mean 28 months) with concurrent endomyocardial biopsy and M-mode echocardiography with digitization. M-mode data from all 16 episodes of rejection (International Society for Heart and Lung Transplantation grade 3A or greater) that occurred in 12 grafts were compared with data from the same grafts with histologic resolution of rejection 2 weeks after treatment and with data from biopsy-proven nonrejecting control grafts matched for sex, time after transplantation, donor weight, and donor age. RESULTS: Left ventricular mass index increased in rejection (86 +/- 9 gm/m2) versus resolution (64 +/- 6 gm/m2) and versus that in nonrejecting control grafts (59 +/- 8 gm/m2). Left ventricular shortening fraction increased in rejection (40% +/- 2%) versus resolution (38% +/- 10%). Septal thickening fraction decreased in rejection (33% +/- 9%) versus nonrejection (68% +/- 16%). These changes became significant only in grafts transplanted more than 1 month before study. Substantial overlap of measurements prevented identification of threshold values. Intraobserver and interobserver variabilities for standard M-mode data were 7% to 8% and 12% to 22%, respectively, whereas those for digitized parameters were markedly elevated at 37% to 71% and 51% to 81%, respectively. CONCLUSIONS: We found (1) left ventricular mass index increases in cellular rejection but may be unreliable less than 1 month after transplantation and (2) significant interobserver and intraobserver variability may limit the applicability of digitized echo parameters to the detection of rejection in infant heart transplant recipients.

Acute Disease↗
PubMed1996

Lung transplantation in children with pulmonary hypertension and congenital heart disease.

Pulmonary vascular disease with or without associated congenital heart disease is the indication for lung transplantation in 30% of all children requiring this procedure. Although many of these children will have primary pulmonary hypertension, a substantial number are children whose pulmonary vascular disease is either associated with a congenital heart lesion or secondary to it. Heart-lung transplantation has been performed in these children in the past, but because of the scarcity of availability of donor heart-lung blocks, lung transplantation with repair of associated congenital cardiac lesions has of necessity been performed more frequently. As one might expect, the operative and postoperative management of these patients is a good deal more complex than pulmonary transplantation for primary lung diseases. The early mortality is correspondingly higher as well, although survival beyond that time is quite similar to that of lung transplantation for other diseases. Factors that increase the early mortality include previous thoracic procedures and the complexity of the underlying cardiac diagnosis. Although the overall experience with this complex group of patients is small, the results seem similar to the prior experience with heart-lung transplantation. Thus, we remain optimistic that this will continue to be a viable form of therapy for children with pulmonary vascular disease associated with congenital heart lesions. The quality of life of the surviving patients is excellent and limited only by those factors impacting upon lung transplantation in general, such as rejection, infection, and the development of bronchiolitis obliterans.

Adolescent↗
PubMed1996

Lung transplantation in infancy and early childhood.

BACKGROUND: Experience with lung transplantation in infants and young children is limited. Small size, vulnerability to infection, and limited modalities for rehabilitation and surveillance of the transplanted lung make this group particularly challenging. METHODS: We reviewed the course of all children up to the age of 25 months who underwent lung transplantation at two centers between July 1990 and February 1995. RESULTS: Lung transplantation was performed in 17 patients under the age of 25 months, with concurrent cardiac repair in 14. Prior thoracic surgery had been performed in 12; six patients had mechanical ventilation, and three were supported with extracorporeal membrane oxygenation while waiting for lungs. The mean waiting time was 37 days (range 1 to 197 days). Hospital survival was 12 of 17 (71%); there was one late death. Early deaths were due to hemorrhage (two patients), cytomegalovirus and lymphoproliferative disease (one patients), and viral pneumonitis (two patients). The one late death was due to overwhelming gastroenteritis of unknown origin. One additional patient had graft failure caused by viral pneumonitis and underwent successful retransplantation. Bronchial stenosis occurred at 3 of 33 anastomoses. At a mean follow-up of 22 months, surviving patients were well, without supplemental oxygen, and, although small in stature, had normal linear growth. CONCLUSIONS: Lung transplantation is a reasonable therapy for very young patients with limited life expectancy and no other therapeutic alternative, with outcomes comparable with those achieved in older patients. Early recognition of lung transplant candidates and advances in the prevention, diagnosis, and treatment of viral illness may improve survival in these patients.

Actuarial Analysis↗
PubMed1996

Lung transplantation in children.

Lung transplantation can offer improved quality of life and prolonged survival to children who have end-stage cardiopulmonary disease, even in the presence of characteristics that have been considered poor prognostic indicators. At present, children who have pulmonary hypertension tend to be referred for transplantation late in the course of their disease; as a result, almost half die before organs become available. Cardiac catheterization for accurate disease staging, appropriate timing of evaluation and listing for transplantation, and referral to centers experienced in the treatment of children who have end-stage cardiovascular disease are all essential components of a process that will improve survival in such children. Our experience indicates that there may be important developmental differences in the body's response to lung transplantation; thus, protocols that are suitable for older adolescents and adults may be unsuitable for young children. An integrative approach to investigation of these issues, i.e., one in which careful clinical observation and data acquisition are combined with targeted bench research, is most likely to result in insights that will directly benefit patients.

Child↗
PubMed1995

Deletion within chromosome 22 is common in patients with absent pulmonary valve syndrome.

Interstitial deletions in chromosome 22 and features associated with CATCH-22 syndrome have been reported in patients with conotruncal congenital heart anomalies. Absent pulmonary valve syndrome is characterized by absent or rudimentary pulmonary valve cusps, absent ductus arteriosus, conoventricular septal defect, and massive dilation of the pulmonary arteries. Because absence of the ductus arteriosus is a key element in the pathogenesis of this syndrome and aortic arch malformations are frequently seen in patients with CATCH-22 syndrome, we hypothesized that patients with absent pulmonary valve syndrome would have a high incidence of deletions in the critical region of chromosome 22. Eight patients with absent pulmonary valve syndrome were studied. Metaphase preparations were examined with fluorescent in situ hybridization of the N25 (D22S75) probe to the critical region of chromosome 22q11.2. Deletions were detected in 6 of 8 patients. The presence of deletions in chromosome 22 in most of the patients we have examined with a diagnosis of absent pulmonary valve syndrome supports a specific genetic and embryologic mechanism involving the interaction of the neural crest and the primitive aortic arches as one cause of congenital absence of the pulmonary valve.

Chromosome Deletion↗
PubMed1995

MR of right aortic arch with mirror-image branching and a left ligamentum arteriosum: an unusual cause of a vascular ring.

An unusual case is presented of a vascular ring due to a right arch with mirror-image branching and a left ligamentum arteriosum from the descending aorta to the left pulmonary artery. There have been only four previous reports of this rare arch anomaly. In addition, the patient had no underlying congenital heart lesions, which is also very unusual in patients with right aortic arch with mirror-image branching.

Aorta, Thoracic↗
PubMed1995

Lung transplantation in children and young adults with cardiovascular disease.

Single or bilateral lung transplantation was performed in 20 patients with pulmonary hypertension or an inadequate pulmonary vascular bed; all but 1 had congenital heart disease. The average age was 6.3 years (range, 3 months to 23.9 years). All were in New York Heart Association class IV, and 6 were hospitalized and receiving intensive support before transplantation. Hospital survival was 70% (14/20), with three additional deaths at 7, 11, and 27 months. A prior thoracic operation contributed to three of six hospital deaths from hemorrhage. All late deaths were due directly or indirectly to obliterative bronchiolitis. At a mean follow-up of 19 months (range, 2 to 48 months), 10 of 11 survivors are in New York Heart Association class I. Survival after hospital discharge and incidence of obliterative bronchiolitis are similar in a contemporary group of 41 patients of comparable age who underwent lung transplantation for pulmonary disease (p = not significant). Single or bilateral lung transplantation is an acceptable therapy for children with pulmonary hypertension, congenital heart disease, or both. Further investigation in the areas of pretransplantation survival, operative risk factors, and long-term outcome of single-lung recipients and recipients with hemodynamically insignificant intracardiac lesions are needed to develop optimal decision-making strategies for these patients.

Adolescent↗
PubMed1995

Anomalous origin of the right pulmonary artery from the aorta and CATCH 22 syndrome.

We report repair of anomalous origin of the right pulmonary artery from the ascending aorta in a premature neonate with a deletion in the CATCH 22 region of chromosome 22. This case suggests that the pathogenesis of anomalous origin of the right pulmonary artery involves genetically determined abnormalities of the neural crest. Repair of this defect in a premature infant can prevent the development of severe pulmonary vascular disease.

Aorta↗
PubMed1995

Lung transplantation for congenital pulmonary vein stenosis.

BACKGROUND: Congenital pulmonary vein stenosis is a uniformly fatal disease when left untreated. Transcatheter techniques (for example, balloon dilation and stent placement) have proved to be only temporizing measures, and previous surgical attempts at treatment of this entity have provided little improvement and few survivors. METHODS: Over the last 4 years, 6 patients with congenital pulmonary vein stenosis have been treated at our institution, 3 of whom underwent bilateral sequential lung transplantation. RESULTS: The 3 patients who underwent bilateral lung transplantation are alive and well 6 to 24 months after transplantation. The other 3 died of complications of the disease before donor lungs became available. CONCLUSIONS: Making the diagnosis of congenital pulmonary vein stenosis requires a high index of suspicion, and referral for lung transplantation should be made as soon as the diagnosis is reached. Lung transplantation has resulted in good-quality short to medium-term survival for 3 patients with this otherwise untreatable disease.

Constriction, Pathologic↗
PubMed1995

Repair of long-segment tracheal stenosis in infancy.

Long-segment stenosis of the trachea in infancy is a considerable surgical challenge because the infants are generally extremely ill and the airway is small. The optimal type of repair is not clearly defined. This report summarizes our experience with rib cartilage tracheoplasty done with cardiopulmonary bypass. Six patients underwent repair of long-segment tracheal stenosis between September 1987 and September 1994. The mean age was 14 weeks (range 1 to 58 weeks). Patients had stenosis of at least 70% of the tracheal length, typically with complete cartilaginous rings. In all patients stenosis was repaired by placement of a section of rib cartilage as an augmentation patch into the anterior surface of the trachea, which had been incised through the entire length of the stenosis. To avoid distal airway intubation, we used cardiopulmonary bypass for all procedures, with a mean bypass duration of 110 minutes (range 54 to 175 minutes). Mechanical ventilation was required for a median of 11 days after the operation (range 7 to 81 days), and the median postoperative hospital stay was 17 days (range 12 to 180 days). All patients are long-term survivors. Complications included the need for extracorporeal membrane oxygenation to treat ventricular dysfunction in one patient and graft dehiscence requiring revision of the distal graft in another. The latter patient has required several treatments with a bronchoscope for removal of granulation tissue. All other patients are free of symptoms and have normal growth with a mean follow-up of 4.7 years (range 5 months to 7.6 years). We conclude that rib cartilage tracheoplasty for long-segment tracheal stenosis provides excellent results in short and intermediate follow-up. In addition, the use of cardiopulmonary bypass allows an unobstructed view of the tiny infant airway and thus permits a precise repair.

Cardiopulmonary Bypass↗
PubMed1994

Replacement of the aortic root with a pulmonary autograft in children and young adults with aortic-valve disease.

BACKGROUND: The optimal substitute for severely diseased aortic valves in children and young adults is unknown. The use of a mechanical prosthesis requires permanent treatment of the patient with anticoagulants and is associated with thromboembolic and hemorrhagic complications. Aortic-valve allografts and porcine bioprostheses, which do not necessitate anticoagulant therapy, may deteriorate and have limited durability. METHODS: We therefore evaluated the use of the autologous pulmonary valve (i.e., the patient's own pulmonary valve) and the adjacent pulmonary artery as a replacement for the aortic valve and aortic sinuses in 33 patients. Five of the patients were from 8 to 16 years of age, and 28 were from 20 to 47 years of age. The pulmonary valve and the main pulmonary artery were used to replace the diseased aortic valve and the adjacent aorta. The coronary arteries were detached from the aorta and implanted into the pulmonary artery. The pulmonary valve and artery were replaced with a cryopreserved pulmonary allograft. RESULTS: There were no deaths during follow-up of up to 48 months (mean, 21 months). There were no episodes of infective endocarditis, and no reoperations on the aortic root were necessary. Also, there was no evidence on echocardiography of progressive dilatation of the autografts. With color-flow Doppler imaging, 22 patients were found to have only trivial regurgitation or none, 9 patients to have mild regurgitation, and no patients to have moderate or severe regurgitation across the autograft at the most recent follow-up visit. The mean peak velocity of flow across the autograft was 1.3 m per second (upper limit of normal, 1.8), indicating the absence of stenosis. One patient required reoperation for stenosis of the pulmonary allograft. CONCLUSIONS: Although the pulmonary-autograft procedure is more complex than simple aortic-valve replacement, it has been safely applied in selected patients, including young adults. Intermediate follow-up indicates satisfactory function of the autografts, with no dilatation or progressive valvular regurgitation. Pulmonary-root autografts may thus be the best available substitute for diseased aortic valves in children and young adults.

Adolescent↗
PubMed1994

Predictors of operative mortality and morbidity after infant heart transplantation.

We retrospectively analyzed the impact of recipient, donor, and operative factors on the operative mortality and morbidity of 36 consecutive infant heart transplantations. Operative survival was excellent at 94%. Among 34 survivors, operative morbidity in 12 patients included acute severe allograft failure with or without prolonged ventilatory support. The cohort was characterized by age less than 4 months (32 of 36), a diagnosis of hypoplastic left heart syndrome (29 of 36), and the use of circulatory arrest (27 of 36); these variables were colinear and could not be used to predict operative mortality or morbidity. None of the remaining recipient, donor, or operative characteristics predicted survival or acute severe allograft failure. A donor-to-recipient weight ratio greater than 2 and a circulatory arrest time greater than 39 minutes predicted the need for prolonged ventilatory support. Despite the need for aggressive or prolonged support after 12 of 36 transplantations, operative survival was high at 94% (34 of 36 procedures, 32 of 34 patients). The use of less restrictive donor criteria combined with aggressive management of acute allograft failure can result in excellent operative survival after infant heart transplantation.

Female↗
PubMed1994

Isolated lung transplantation in children: pathological diagnosis and incidence of pulmonary complications.

The pathological findings in the allografts of 14 children who underwent lung transplantation (LT) at St. Louis Children's Hospital, St. Louis, MO, in the period between July 1990 and May 1992 were reviewed. The study is based on histological analysis of 63 transbronchial biopsy (TBB) specimens, eight open lung biopsy specimens, and three pneumonectomy specimens. The mean age at transplantation was 10.5 years (range, 1 to 17 years) and the average follow-up period was 5.7 months. Sufficient tissue for an adequate pathological examination was obtained in 58 (92%) TBB specimens. Each specimen consisted of a mean of 6.12 tissue fragments, but only 4.79 fragments contained actual lung parenchyma for suitable examination. Ten patients (71%) had 23 biopsy-proven episodes of acute rejection with a frequency of 1.64 episodes per patient. The first episode was documented at a mean of 19 days after transplantation. Six patients (42.8%) developed bronchiolitis obliterans (BO). The definitive diagnosis of this condition was made either by open lung biopsy (n = 3) or on allograft pneumonectomy (n = 1), and it was infrequently recognized by TBB. Four of the six patients died less than 9 months after the diagnosis of BO was made, indicating the grave consequences of this complication. Two other deaths were attributed to the development of posttransplantation lymphoproliferative disorders.

Adolescent↗
PubMed1994

Transplantation of the heart and lungs in children.

Heart transplantation in children with complex congenital heart disease or cardiomyopathies has become an effective form of therapy for patients with no other surgical options. Because pediatric pulmonary transplantation is a relatively new procedure, long-term follow-up data are not yet available. However, the intermediate-term results of pediatric lung transplantation appear similar to those for heart and lung transplantation. Children represent a particularly difficult patient group for transplant of thoracic organs, and recipients must be selected carefully. Additionally, physicians must pay close attention to multiple medical conditions relating to the underlying disease process in order to achieve optimal results.

Adolescent↗