Death after cardiac transplantation in low birth weight neonates.
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Biomedical subjects
Publications and source records attributed to C Canter.
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PURPOSE: To determine whether the morphologic features of posttransplant lymphoproliferative disease (PTLD) correlated to a response to therapy. PATIENTS AND METHODS: We reviewed our experience with PTLD in the pediatric population. We identified 32 patients with a total of 36 episodes of PTLD. The diagnosis was confirmed by tissue examination and classified according to the degree of monomorphic features of the lesion. Thirty-four of 36 episodes were managed with immunosuppression reduction, and the patients were assessed for their response to this strategy. Chemotherapy was used to treat 10 of 15 patients who had progressive disease, and their subsequent course was also analyzed. RESULTS: Sixteen of 17 (94%) patients with polymorphic morphology responded to immunosuppression reduction compared with only 5 of 17 (29%) patients with monomorphic features (P < 0.001). All of the patients with progressive disease who did not receive additional therapy died. Standard chemotherapy regimens for lymphoma were administered to 10 patients with progressive disease, with a high response rate (90%), durable remissions, and acceptable toxicity. CONCLUSIONS: We conclude that the morphologic characteristics of PTLD provide information to potentially help guide treatment strategies in the management of this disease. Standard chemotherapy regimens for malignant lymphoma appear to be a viable treatment option for patients with progressive disease, although further investigation is needed.
This review details the indications for heart transplantation in children. Contraindications have evolved from absolute to relative. Controversial issues remain and this paper represents a consensus of more than a dozen centers that have programs that remain active performing pediatric heart transplants.
BACKGROUND: Despite the increasing application of cardiac transplantation in infants, reported survival rates vary, and risk factors for death are poorly understood. METHODS AND RESULTS: To examine early survival and risk factors for death in infants (< 1 year of age) undergoing cardiac transplantation, 141 infants (36 < 1 months of age) underwent primary cardiac transplantation between January 1, 1993, and January 1, 1995, at 23 centers in the Pediatric Heart Transplant Study (PHTS). Diagnoses were hypoplastic left heart syndrome (66%), other congenital heart disease (17%), cardiomyopathy (14%), and other (3%). Actuarial survival after cardiac transplantation was 84% at 1 month, 70% at 1 year, and 69% at 2 years, with the greatest hazard for death within the first 3 months. The principal cause of death was early graft failure in 20 patients (52% of deaths), infection in 10 (26% of deaths), and rejection in 4 (10%). On the basis of multivariate analysis, risk factors for early mortality were history of previous sternotomy (P = .0003), nonidentical blood type donor (P = .01), recipient non-blood group A (P = .02), and donor cause of death other than closed head trauma (P = .04). Diagnosis at listing, waiting time (mean, 1.3 months), graft ischemic time (mean, 228 minutes; range, 68 to 479 minutes), and recipient ventilatory or inotropic support at listing were not predictive for mortality after transplant. CONCLUSIONS: The higher mortality rate observed with infant heart transplantation is due to a higher mortality within the first month after transplantation as a result of early graft failure. Strategies to improve donor heart function at implantation would have the greatest impact on survival after infant cardiac transplantation.
BACKGROUND: The major limiting factor to successful heart transplantation in infants is the limited supply of donors. To examine the impact of donor limitations on survival after listing, a multiinstitutional study was designed to identify risk factors for death while waiting and for longer interval to transplantation. METHODS: Between January 1 and December 31, 1993, 118 infants 6 months of age or younger (86 younger than 29 days) were listed for heart transplantation from 21 institutions. The primary diagnosis was hypoplastic left-sided heart syndrome (HLHS) in 70 (59%), other congenital defects in 32 (27%), cardiomyopathy or myocarditis in 13 (11%), and other diagnoses in 3. Among the 48 patients without HLHS, 32 (67%) required inotropic, mechanical, or prostaglandin support, whereas 16 (33%) did not. RESULTS: At 6 months after listing, only 6% remained on the list awaiting transplantation, 59% underwent transplantation. 31% died while waiting, and 4% were removed from the list. The greatest mortality rate before transplantation was among patients with HLHS in whom the actuarial mortality rate if they were unable to receive a transplant was 77% at 6 months, compared with 52% in patients without HLHS and without inotropic or mechanical support (p = 0.05). By multivariable analysis, risk factors for death while waiting included inotropic support (p = 0.02), smaller size (p = 0.0007), and blood type O (p = 0.003). Surgical procedures before listing did not significantly influence pretransplantation mortality rates. The interval from listing to transplantation increased with young age (p = 0.01) in patients without HLHS and smaller size (p = 0.001) and blood group O (p = 0.0006) for patients with HLHS. The effect of blood type O on mortality rates and longer interval to transplantation was due to the distribution of type O donor hearts to non-type O recipients. Palliative operations after listing did not favorably influence survival; nine patients underwent first-stage Norwood while waiting, and six died before transplantation. CONCLUSIONS: The mortality rate is unacceptably high among infants awaiting transplantation, particularly in patients with HLHS. Infants receiving intravenous inotropes or mechanical support at listing are at high risk of early death while waiting. The distribution of blood group O donors to non-blood group O recipients results in higher mortality rates among blood group O recipients. Greater emphasis should be placed on medical strategies to improve survival while waiting and on expanding existing graft resources.
OBJECTIVE: To determine the type and frequency of acquired neurologic complications in survivors of pediatric heart transplantation (HT). DESIGN: Retrospective study. SETTING: Tertiary care children's hospital. PARTICIPANTS: Fourteen survivors of 17 consecutive patients who underwent HT during a 60-month period beginning in January 1986. INTERVENTIONS: None. MEASUREMENTS OR MAIN RESULTS: Three distinct subgroups of patients who had undergone HT were identified: six infants with uncorrected hypoplastic left heart syndrome (HLHS), three infants with HLHS who had undergone previous stage 1 Norwood repair, and eight older children with end-stage cardiomyopathy. Fourteen (82%) of 17 children were alive at follow-up. Only one patient (7%) had a significant acquired neurologic deficit (left temporal lobe stroke with subsequent seizures in an infant with uncorrected HLHS). The remaining subjects had normal results of post-HT neurologic examinations (n = 7), minor post-HT neurologic abnormalities (n = 3), no significant change in preexisting neurologic abnormalities (n = 1), or normal neurologic status by report (n = 2). The minor neurologic abnormalities noted post-HT were dysmetria, tremor, and absent reflexes. No episodes of choreoathetosis or cyclosporine-related seizures were seen. CONCLUSIONS: Pediatric HT is associated with both a high survival rate and a low incidence of severe acquired neurologic deficits despite a significant incidence of severe systemic and metabolic derangements in the pretransplantation and posttransplantation periods. In infants with HLHS, HT seems to carry a lower incidence of severe neurologic morbidity (12%) than other surgical treatments.
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Extracorporeal membrane oxygenation was used for cardiovascular support in 13 infants and children with complex congenital heart disease and 1 premature neonate treated in preparation for pericardial patch tracheoplasty for long-segment tracheal stenosis. Nine patients were weaned from extracorporeal membrane oxygenation. There were five (36%) early deaths and four (29%) late deaths. Cannulation sites included right carotid/jugular vessels, femoral artery and vein, and right atrium and aorta. In 4 patients, the neck vessels were repaired at decannulation. Five survivors had normal growth and neurodevelopmental evaluations at follow-up. Extracorporeal membrane oxygenation can be successfully used as biventricular support in patients with intractable low cardiac output syndrome after repair of congenital heart disease. Best results are obtained in patients who have several hours of stability after operation before initiation of support. Hemorrhagic complications are reduced and long-term neurodevelopmental outcomes appear promising with right neck vessel cannulation and repair. No bleeding complications were observed in patients cannulated through the neck vessels.
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