The use of a Berlin Heart EXCOR LVAD in a child receiving chemotherapy for Castleman's disease

Abstract

We present the unique case of a pediatric patient who received chemotherapy for a diagnosis of CD, while mechanically supported with a Berlin EXCOR LVAD secondary to restrictive cardiomyopathy. A four-yr-old previously healthy male with restrictive cardiomyopathy required MCS after cardiac arrest but was diagnosed with multicentric CD, a non-malignant lymphoproliferative disorder fueled by excessive IL-6 production. Treatment with IL-6 blockade (tocilizumab) every two wk and methylprednisolone had no effect on his lymph nodes or cardiac function while on temporary RotaFlow. A Berlin LVAD was placed for treatment with rituximab, COP, vincristine, and methylprednisolone. After three courses of chemotherapy, his inflammatory markers normalized and his lymphadenopathy decreased but cardiac function remained severely depressed. He tolerated chemotherapy on the Berlin but required frequent titrations of his anti-coagulation regimen and he did suffer a hemorrhagic stroke. His clinical status improved significantly with rehabilitation, and he tolerated heart transplantation without further complications. MCS is a feasible option as a bridge to recovery or heart transplant eligibility for patients with hemodynamic collapse requiring chemotherapy but it does necessitate close titration of the anti-coagulation regimen to coincide with changes in the inflammatory state.

Outcomes of Berlin Heart EXCOR® pediatric ventricular assist device support in patients with restrictive and hypertrophic cardiomyopathy

“…Since its implementation, long‐term survival rates for pediatric end‐stage heart failure have improved dramatically, and the EXCOR ® has emerged as a new standard of care for small children requiring mechanical support as a bridge to heart transplantation . Its success as a long‐term device option in pediatric heart failure patients with DCM has sparked interest in implementation of the EXCOR ® Pediatric VAD in other contexts of heart failure including CHD and single ventricle anatomy, as well as restrictive heart disease …”

“…12,13 Its success as a long-term device option in pediatric heart failure patients with DCM has sparked interest in implementation of the EXCOR ® Pediatric VAD in other contexts of heart failure including CHD and single ventricle anatomy, as well as restrictive heart disease. [14][15][16][17][18] Historically, restrictive disease has been considered a risk factor for poor outcome with mechanical support. 3 In particular, this group of patients are thought to be at elevated risk of complications of VAD In this review, we found that 50% pediatric patients with diastolic dysfunction who were implanted with the EXCOR ® survived to heart transplant or recovery.…”

Clinical Characteristics and Treatment of Cardiomyopathies in Children

“…It is harder to support children with RCM with left ventricular assist devices (LVAD) due to smaller ventricular cavity sizes and because the right ventricle is usually affected as well. There are reports of successful bridging to cardiac transplantation with the Berlin EXCOR® LVAD [ 217 , 218 ]. All had left atrial cannulation and one patient required bi-ventricular devices.…”

Ventricular assist device support for children with chemotherapy‐induced cardiomyopathy and advanced heart failure: Perspectives gained from a single‐center experience

“…The literature specifically addressing the use of durable VAD support in children with HF and a recent history of cancer is limited to case reports 9–14 15 .…”

“…9,10 The literature specifically addressing the use of durable VAD support in children with HF and a recent history of cancer is limited to case reports. [9][10][11][12][13][14] There are no guidelines on how to proceed in pediatric patients who develop worsening HF after recently completing their cancer treatment, and the ISHLT advocates for individualized risk stratification for cancer recurrence in collaboration with oncologists. 15 In this manuscript, we review our single-center experience of durable VAD support for advanced HF in 6 children with chemotherapy-induced cardiomyopathy.…”