Heart Transplant As Therapy For An Unresectable Recurrent Cardiac Fibroma

Abstract

<h3>Introduction</h3> Cardiac fibromas (CF) have a reported incidence of 0.03-0.32% and have an excellent prognosis. Most cardiac tumors are found incidentally, and symptoms are related to location and size. Most CF are amenable to surgical removal, but some are deemed unresectable due to their size and/or invasion of vital cardiac structures that warrant heart transplants. <h3>Case Report</h3> 66-year-old male with a history of prior cardiac fibroma resection at age 13, followed by dual-chamber pacemaker insertion due to complete heart block and recent ventricular tachycardia ablation. He came to our facility for a second opinion regarding heart transplants because he was previously considered high-risk by another facility. Echo showed an EF of 55%, with CF noted on the anterior wall. Heart CT reported a LV anterior wall mass measuring 6 × 8.5 × 8.9 cm, markedly displacing the LAD and first diagonal with a non-hypermetabolic mass on PET scan. He was presented during our multidisciplinary team rounds; the decision was to proceed with biopsy of the left ventricular cardiac mass showing CF with abundant collagen in the stroma separating the tumor cells (Figure 1A), and the CF is composed of slender spindle cells admixed with undulant and wavy collagenized stroma. No nuclear pleomorphism or mitotic activity is present (Figure 1B). Postoperatively, the patient developed incessant VT requiring amiodarone and lidocaine. EP was consulted, and due to prior VT ablation and limited options, the patient was listed for an orthotopic heart transplant (OHT). He underwent successful OHT without complications. The explanted heart has a whorled, well-circumscribed, tan appearance mass measuring 14 × 10 × 9 cm involving the LV, the interventricular septum, and epicardium (Figure 2). <h3>Discussion</h3> Cardiac fibromas are connective tissue tumors that originate from fibroblasts, typically grossly well demarcated. CF are considered benign tumors, but they are well known to be the most common arrhythmogenic tumors than other cardiac tumors; It is essential that cardiologists be aware of CF presentation, diagnosis and treatment. Careful selection of potential candidates based on patient characteristics, type of tumor, and life-threatening complications for OHT by a multidisciplinary team is imperative in such complex cases. We report a case that required orthotopic heart transplantation due to incessant ventricular tachycardia secondary to recurrent unresectable fibroma.