Cardiac chamber hypertrophy following hematopoietic stem cell transplantation for primary immunodeficiency

Area of Science:

• Pediatric Cardiology
• Hematology
• Immunology

Background:

• Hematopoietic stem cell transplantation (HSCT) is a treatment for primary immune deficiency (PID).
• Graft-versus-host disease (GVHD) is a common complication post-HSCT, often managed with corticosteroids (CS).
• Corticosteroids (CS) are associated with cardiovascular complications including hypertension, cardiac chamber hypertrophy (CCH), and left ventricular outflow tract obstruction (LVOTO).

Purpose of the Study:

• To investigate the development of CCH and LVOTO in children with PID post-HSCT.
• To correlate CCH and LVOTO with age at transplant, GVHD severity, CS use, and hypertension.
• To assess the reversibility of CCH and LVOTO after CS discontinuation.

Main Methods:

• Serial echocardiograms were performed on 10 children with PID before and 6-12 weeks after HSCT.
• Data on age at transplant, GVHD grade, CS dosage, and blood pressure were collected.
• Statistical analysis was used to correlate cardiovascular changes with clinical factors.

Main Results:

• CCH developed in all 4 children transplanted before 1 year of age who received high-dose CS for grade III or IV acute GVHD (aGVHD).
• A significant correlation was found between CCH and blood pressure above the 99th percentile (P < .002).
• One child experienced severe LVOTO; both CCH and LVOTO improved with CS cessation and blood pressure normalization.

Conclusions:

• Young children undergoing HSCT for PID are at risk for CCH, particularly those with aGVHD treated with high-dose CS and experiencing significant hypertension.
• Early detection and management of hypertension may be crucial in preventing CS-induced cardiac complications post-HSCT.
• CCH and LVOTO may be reversible upon discontinuation of CS treatment.