title: Acquired discrete subaortic stenosis in solid organ transplant recipients.
abstract: DSS is a rare acquired abnormality of the LV outflow tract in children and adults. DSS can be seen with or without associated congenital heart disease, but the mechanism of its emergence has been poorly understood. Here, we report two cases of DSS that developed after solid organ transplant in childhood. One patient underwent liver and kidney transplant at three yr of age for hereditary polycystic disease, and the other had liver transplant at three months of age due to uncontrollable high-output failure secondary to a large hepatic hemangioma. Both patients developed mild dynamic LV outflow tract obstruction and systemic hypertension soon after transplant, both of which resolved by the time of discharge. Transient LV hypertrophy was observed in both cases. Corticosteroids, cyclosporine A, and tacrolimus were used as initial immunosuppressants. Both patients developed DSS long after the regression of the initial dynamic LV outflow tract obstruction. Herein, we report DSS as an atypical late cardiovascular complication after solid organ transplant and postulate that transient post-transplant hyperdynamic LV outflow tract obstruction and chronic immunosuppressive treatment may lead to subendocardial remodeling resulting in a fibromuscular membranous ridge in susceptible subjects.
authors.affiliation: Nemours Cardiac Center, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA.
authors.forename: Takeshi
authors.initials: T
authors.lastname: Tsuda
authors.forename: Stephen
authors.initials: S
authors.lastname: Dunn
authors.forename: Wolfgang
authors.initials: W
authors.lastname: Radtke
mesh_terms: D065095:Calcineurin Inhibitors / Q000627:therapeutic use; D002318:Cardiovascular Diseases; D002675:Child, Preschool; D021922:Discrete Subaortic Stenosis / Q000150:complications / Q000175:diagnosis*; D004452:Echocardiography; D005260:Female; D006330:Heart Defects, Congenital / Q000628:therapy*; D006352:Heart Ventricles / Q000473:pathology; D006801:Humans; D006984:Hypertrophy; D007165:Immunosuppression Therapy; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007223:Infant; D016030:Kidney Transplantation; D016031:Liver Transplantation; D008297:Male; D009206:Myocardium / Q000473:pathology; D016377:Organ Transplantation / Q000009:adverse effects*; D011183:Postoperative Complications; D033081:Shear Strength; D013314:Stress, Mechanical; D066027:Transplant Recipients; D016896:Treatment Outcome
chemical_list: D065095:Calcineurin Inhibitors; D007166:Immunosuppressive Agents
keywords: calcineurin inhibitors; discrete subaortic stenosis; echocardiography; myocardial hypertrophy; organ transplant; shear stress