Reversal of severe late left ventricular failure after pediatric heart transplantation and possible role of plasmapheresis

Late acute cardiac graft failure carries a high mortality in adults. Vascul ar mediators and factors other than classic T-cell-mediated rejection may p lay a role in this process, and aggressive multimodality therapy may improv e survival. We report experience with plasmapheresis in treating late sever e acute left ventricular dysfunction in a group of pediatric heart transpla nt recipients. We retrospectively reviewed clinical records, echocardiogram s, hemadynamics, coronary angiograms, biopsy specimens, and treatment regim ens for 5 patients with 7 episodes of late-onset severe graft failure who r ecovered. Plasmapheresis was applied in all cases, in addition to methylpre dnisolone, cyclophosphamide, lympholytic agents, and aggressive supportive care including mechanical ventilation and hemofiltration. All patients pres ented with acute severe left ventricular dysfunction 1.4 to 7.9 years (mean 3.6) after orthotopic heart transplantation. Mean shortening fraction at p resentation was 13 to 23% (mean 16), initial endomyocardial biopsy specimen s were grade 0 to 38, and immunofluorescence studies were negative. Treatme nt included plasmapheresis, cyclophosphamide, mechanical ventilation, hemof iltration, and inotropes. Clinical recovery was slow, with 4 to 8 weeks unt il left ventricular function normalized, and 2.2 to 9.4 (mean 4.6) weeks to hospital discharge. At follow-up (50 to 38 months, mean 24), all are alive . Two patients are well, whereas coronary vasculopathy developed in 3. Thus , survival may improve in patients with late graft failure with low biopsy score and plasmapheresis combined with multimodality therapy. (C)2000 by Ex cerpta Medica, Inc.