ABSTRACT
To define the incidence, course, and etiology of hematologic abnormalities in children on tacrolimus-based immunosuppression, we reviewed records of 106 transplant patients (70 heart, 16 heart and lung, 20 double lung), 0-21 yr of age, who were transplanted at the Children's Hospital of Pittsburgh from 1989 to 1997. Fifty-four of the 106 patients (51%) developed 65 abnormal hematologic episodes (32 anemia, nine neutropenia, nine thrombocytopenia, 15 simultaneous anemia and neutropenia with or without thrombocytopenia). Common etiologies included: infections, post-transplant lymphoproliferative disease, and medications. Eleven episodes (seven anemia, one neutropenia, and three simultaneous anemia and neutropenia) had unclear etiologies and process of elimination suggested an association with tacrolimus. Interventions included filgrastim (effective in 15 of 15 patients, with resolution of neutropenia in a median of 5 days) and epoetin alfa (effective in five of 16 patients, including four of four patients with anemia possibly related to tacrolimus). Five patients (two with neutropenia and three with simultaneous neutropenia and anemia) were switched to cyclosporin A (CsA); rapid resolution occurred in four of the five patients, suggesting a possible association of the hematologic abnormalities with tacrolimus. In summary, hematologic abnormalities are common in children on tacrolimus-based immunosuppression. Most of these hematologic abnormalities are caused by common etiologies; however, a sub-population exists where tacrolimus may be the etiologic agent. Anemia and neutropenia respond to treatment with epoetin alfa and filgrastim. After thorough investigation, a trial switch to CsA may be warranted.
Subject(s)
Heart Transplantation , Hematologic Diseases/etiology , Immunosuppressive Agents/therapeutic use , Lung Transplantation , Tacrolimus/therapeutic use , Adolescent , Adult , Anemia/etiology , Child , Child, Preschool , Epoetin Alfa , Erythropoietin/therapeutic use , Female , Filgrastim , Granulocyte Colony-Stimulating Factor/therapeutic use , Heart Transplantation/adverse effects , Heart Transplantation/immunology , Heart-Lung Transplantation/adverse effects , Heart-Lung Transplantation/immunology , Humans , Infant , Lung Transplantation/adverse effects , Lung Transplantation/immunology , Male , Neutropenia/drug therapy , Neutropenia/etiology , Recombinant Proteins , Retrospective Studies , Thrombocytopenia/etiologyABSTRACT
BACKGROUND: The modified Fontan procedure for patients with only one well-formed ventricle is now widely regarded as palliative, not curative. METHODS: To improve the surgical management and postoperative follow-up of such patients, a morphometric study of 33 postmortem cases was done. RESULTS: The three main causes of death were congestive heart failure (82%), arrhythmias (12%), and central nervous system dysfunction (6%). The cross-sectional area of the Fontan anastomosis (FA) relative to the systemic venous area (SVA) and relative to the body surface area (BSA) revealed that the Fontan pathway was often obstructive. The mean FA/SVA index was 73% less than normal: 0.54 +/- 0.22, range 0.13 to 0.98. The mean FA/BSA index was 70% less than normal: 143.52 +/- 50.01 mm2/M2, range 55.09 to 261.67 mm2/M2. CONCLUSIONS: The main surgical challenge is to minimize or eliminate prepulmonary stenosis. Although significant postoperative obstruction was often not evident hemodynamically because of small or absent gradients, the presence of important obstruction of the Fontan pathway was clearly revealed by morphometry.
Subject(s)
Fontan Procedure/methods , Heart Defects, Congenital/surgery , Heart Ventricles/abnormalities , Postoperative Complications , Adolescent , Adult , Child , Child, Preschool , Female , Humans , Hypertrophy, Left Ventricular , Infant , Male , Retrospective StudiesSubject(s)
Electrocardiography , Syncope/etiology , Adolescent , Child , Female , Fibroma/complications , Fibroma/diagnosis , Heart Block/diagnosis , Heart Block/etiology , Heart Neoplasms/complications , Heart Neoplasms/diagnosis , Humans , Hypocalcemia/complications , Hypocalcemia/etiology , Infant , Male , Pseudohypoparathyroidism/complications , Pseudohypoparathyroidism/diagnosis , Retrospective Studies , Syncope/diagnosis , Syncope/physiopathology , Vagus Nerve/physiopathologyABSTRACT
A subsemilunar conal septal defect was closed at 8 months of age with a redundant Dacron patch that bowed into the left ventricular outflow tract, resulting in severe subaortic stenosis and massive left ventricular hypertrophy. Mistaken for cardiomyopathy or myocarditis, this rare complication of subsemilunar ventricular septal defect patch closure led to orthotopic cardiac transplantation followed by death.
Subject(s)
Heart Septal Defects, Ventricular/surgery , Postoperative Complications/etiology , Ventricular Outflow Obstruction/etiology , Abnormalities, Multiple , Female , Humans , Hypertrophy, Left Ventricular/etiology , Infant , Polyethylene Terephthalates , Prostheses and Implants/adverse effects , Time FactorsABSTRACT
Las dos causas principales de muerte alejada luego del procedimiento de Fontan-Kreutzer modificado fueron: 1) insuficiencia cardíaca congestiva en 11 de 13 pacientes (85 por ciento) y 2) arritmias en 2/13 pacientes (15 por ciento)
Subject(s)
Humans , Male , Female , Arrhythmias, Cardiac/complications , Fontan Procedure , Heart Failure/mortality , Heart Ventricles/surgery , Death , Thoracic Surgery , Thoracic Surgery/mortalityABSTRACT
Las dos causas principales de muerte alejada luego del procedimiento de Fontan-Kreutzer modificado fueron: 1) insuficiencia cardíaca congestiva en 11 de 13 pacientes (85 por ciento) y 2) arritmias en 2/13 pacientes (15 por ciento) (AU)
