title: Cardiac chamber hypertrophy following hematopoietic stem cell transplantation for primary immunodeficiency.
abstract: Children with primary immune deficiency (PID) who receive hematopoietic stem cell transplantation (HSCT) often suffer from graft-versus-host disease (GVHD), which is commonly treated with corticosteroids (CS). CS may cause hypertension, development of cardiac chamber hypertrophy (CCH), and left ventricular outflow tract obstruction (LVOTO). We followed the development of CCH and LVOTO by serial echocardiograms in 10 children with PID before and 6 to 12 weeks after HSCT, and correlated their development with age of transplant, GVHD, use of CS and hypertension. CCH developed in all 4 children transplanted before 1 year of age who received high dose CS treatment for grade III or IV acute GVHD (aGVHD), but not in the 6 children who were transplanted at later ages or who had not received high-dose CS (P = .07). Significant correlation (P < .002) was found between CCH and blood pressure measurements that deviated above the 99th percentile. One child also suffered from severe LVOTO. CCH and LVOTO improved when CS treatment was discontinued and blood pressure normalized. We conclude that following HSCT, young children who suffer from aGVHD, treated with high CS doses, and have excessive hypertension are at risk of developing CCH.
authors.affiliation: Division of Immunology/Allergy, Blood and Marrow Transplant Unit, Department of Paediatrics, The Hospital for Sick Children and the University of Toronto, Toronto, Canada.
authors.forename: Sean R
authors.initials: SR
authors.lastname: Bulley
authors.forename: Lee
authors.initials: L
authors.lastname: Benson
authors.forename: Eyal
authors.initials: E
authors.lastname: Grunebaum
authors.forename: Chaim M
authors.initials: CM
authors.lastname: Roifman
mesh_terms: D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000009:adverse effects* / Q000627:therapeutic use; D006332:Cardiomegaly / Q000139:chemically induced / Q000209:etiology*; D002648:Child; D002675:Child, Preschool; D004452:Echocardiography; D006086:Graft vs Host Disease / Q000150:complications / Q000188:drug therapy; D018380:Hematopoietic Stem Cell Transplantation / Q000009:adverse effects*; D006801:Humans; D006973:Hypertension; D007153:Immunologic Deficiency Syndromes / Q000150:complications* / Q000628:therapy; D012307:Risk Factors; D014694:Ventricular Outflow Obstruction / Q000139:chemically induced / Q000209:etiology
chemical_list: D000305:Adrenal Cortex Hormones