Non-ischemic left ventricular dysfunction after pediatric cardiac transplantation: treatment with plasmapheresis and OKT3.
BACKGROUND
Acquired left ventricular dysfunction after pediatric cardiac transplantation is associated with a high mortality rate. It often occurs without biopsy evidence of cellular rejection or severe transplant coronary arteriopathy.


METHODS
We employed a protocol for treatment of acquired, non-ischemic left ventricular dysfunction utilizing plasmapheresis, monoclonal anti-T-cell antibody (OKT3), cyclophosphamide and steroids, regardless of the results of endomyocardial biopsy. Left ventricular dysfunction was defined as an echocardiographic shortening fraction of <29% and/or symptoms of congestive heart failure requiring inotropic support. Transplant coronary arteriopathy was excluded by coronary angiography in all cases.


RESULTS
Ten pediatric heart transplant recipients were treated for 13 episodes of non-ischemic left ventricular dysfunction. Biopsy scores were low grade (ISHLT Grade 1A or 1B) in 8 episodes. Eight of 10 patients had a history of non-compliance in regularly taking immunosuppressant medications. Inotropic support was required in 9 of 13 cases, with a median duration of 5 days. Median left ventricular shortening fraction was 17% at time of presentation. Normalization of shortening fraction occurred a median of 40 days from the start of treatment. Survival to hospital discharge occurred in 11 of 13 (85%) patients. Long-term patient survival, however, was only 50% at 24 months after presentation with a first episode of acquired left ventricular dysfunction.


CONCLUSIONS
Use of plasmapheresis, OKT3, cyclophosphamide and steroids resulted in successful short-term reversal of non-ischemic left ventricular dysfunction in pediatric heart transplant patients, but long-term survival remained poor.
McOmber
Delwyn
D
Division of Pediatric Cardiology, Department of Pediatrics Washington University, St. Louis, Missouri 63110, USA.
Ibrahim
Jill
J
Lublin
Douglas M
DM
Saffitz
Jeffrey E
JE
Ong-Simon
Catherine
C
Mendeloff
Eric N
EN
Huddleston
Charles B
CB
Canter
Charles E
CE
D000293:Adolescent; D000328:Adult; D002648:Child; D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D016027:Heart Transplantation; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D007223:Infant; D008297:Male; D016853:Muromonab-CD3 / Q000627:therapeutic use*; D010956:Plasmapheresis; D011183:Postoperative Complications; D013256:Steroids / Q000627:therapeutic use; D018487:Ventricular Dysfunction, Left / Q000209:etiology* / Q000628:therapy*
D007166:Immunosuppressive Agents; D016853:Muromonab-CD3; D013256:Steroids; D003520:Cyclophosphamide