Author + information
- Paul Cheng,
- Margot Davis,
- Richard Ha,
- Beth Martin and
- Dipanjan Banerjee
Background: Left ventricular assistant device (LVAD) implantation can cause aberrant activation of the immune system, as evidenced by allo-sensitization post LVAD implantation. Hemophagocytic lymphohistiocytosis (HLH) is a rare (reported incidence ∼1/800,000) hematological syndrome characterized by aberrant activation of a macrophage-mediated systemic inflammatory response leading to multi-organ damage and the characteristic finding of hemophagocytosis in the bone marrow. No association between VAD placement and HLH has been described. We present 4 cases of HLH developing after VAD implantation including their management and outcomes.
Results: In a retrospective analysis from 1/2014 to 6/2016, we identified 4 patients with different causes of cardiomyopathy who developed HLH following VAD insertion (2 HeartMate II, 2 Heartware). Three of four implantations were performed at our institution, and these patients developed persistent fever (>39 C) starting <24 hours post implantation. One patient was transferred from another institution for persistent fever post VAD implantation. All patients met at least 5 HLH-2004 criteria. All patients had extensive marrow hemophagocytosis and markedly elevated serum IL-2R and ferritin levels. Extensive infectious workups were unrevealing and broad-spectrum antibiotics were administered without response. Among cases originating at our institution, HLH was diagnosed and therapies initiated on post-op days 14, 19, and 28, resulting in resolution of fever within 24 hours and dramatic reduction of ionopressor requirement within 48 hours. Three patients were treated with modified HLH-94 therapy (dexamethasone and etoposide), and one patient was treated with dexamethasone for 6 weeks. Three patients survived to OHT (4-31 months after VAD implantation) with 100% survivorship at 1 year after OHT without recurrence of HLH to date. The incidence of HLH following VAD placement is 2% at our institution over 30 months.
Conclusions: HLH may be associated with LVAD implantation, can be treated with steroids and etoposide, and thus should not preclude patients from OHT.
Poster Hall, Hall C
Saturday, March 18, 2017, 3:45 p.m.-4:30 p.m.
Session Title: Heart Failure and Cardiomyopathies: What Next When All Else Is Failing?
Abstract Category: 14. Heart Failure and Cardiomyopathies: Therapy
Presentation Number: 1248-250
- 2017 American College of Cardiology Foundation