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

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. We present the first reported case of Impella 5.5 use in LVNC as a bridge to transplant in a highly sensitized patient.

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. Hospitalized, she escalated to Dobutamine and Milrinone due to progressive shock. Impella 5.5 was inserted, 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. 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.

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.