Impella 5.5 in left ventricular noncompaction syndrome as bridge to heart transplant

Introduction Left ventricular non-compaction syndrome (LVNC) is an uncommon congenital disease characterized by prominent trabeculations and deep inter-trabecular recesses of the left ventricle wall. Patients can have asymptomatic left ventricular dysfunction or have severe, incapacitating congestive heart failure, eventually needing a heart transplant. We present the first reported case of Impella 5.5 use in LVNC as a bridge to transplant in a highly sensitized female supported for 120 days. Case presentation A 50-year-old female with a family history of sudden cardiac death, LVNC cardiomyopathy associated with TPM1 gene mutation, and recurrent hospitalizations due to heart failure was listed for heart transplantation. She had severely reduced systolic function (EF 20%) and high panel reactive antibodies (PRA). She was admitted for desensitization therapy and underwent plasmapheresis and bortezomib therapy. Hospitalized, she escalated to Dobutamine and Milrinone due to progressive shock. With limited medical options and dismal cardiac index, mechanical support was utilized as a bridge to transplant. Due to size and sensitization issues, she was not eligible for a durable LVAD. Impella 5.5 was inserted via the right axillary artery, and inotropes were reduced as her condition steadied. She tolerated desensitization and eventually underwent heart transplantation after 120 days of continuous Impella 5.5 support without developing hemolysis or pump thrombosis. Post-transplant she was found to have concern for left sided stroke symtpoms but completely recovered and remians stable on follow up at 6 months. Discussion Due to the lack of prospective trials with large cohorts, managing patients with LVNC and cardiogenic shock is challenging. Novel approaches to complex patients with both Impella 5.5 support and desensitization therapies should be considered in the appropriate setting. This case highlights the stepwise approach and early escalation to Impella 5.5 in patients with cardiogenic shock and a heart-team pathway for managing desensitization in patients at high risk of thromboembolic complications while awaiting life saving organ replacement therapies. Conclusion Our case highlights the use of mechanical circulatory support in the management of cardiogenic shock within a small left ventricluar cavity in a sensitized patient. This approach should be considered in high-risk patients in whom durable left ventricular assist therapy is not feasible.