P239 Total artificial heart implantation as a bridge to candidacy - an approach to treating primary cardiac sarcoma?
Authors: Atenska-Pawlowska J , Chrapowicka A , Juraszek A , Szymanski J , Kuriata J , Siudalska H , Kusmierczyk M 
Published: January 2020

Abstract

Background
Primary cardiac tumors are rare entities, only 25% of them are malignant. Most common malignant neoplasms of the heart are sarcomas - aggressive tumors with a high rate of local recurrence and systemic metastases. They may lead to a rapid clinical deterioration and sudden circulatory collapse due to obstruction of the intracardiac outflow tract. Most patients affected are younger than 65 years of age. Early diagnosis of cardiac tumors is challenging, echocardiography remains a mainstay of their evaluation. The prognosis is very poor, most patients succumb to disease within months of diagnosis. Complete surgical resection and adjuvant chemotherapy offers palliation and improves survival. Total artificial heart implantation (TAH) allows through, radical resection of large tumors yielding the best possible outcome.

Case presentation
A 58-year-old male patient with a history of hypertension was admitted to our hospital with a chief complaint of worsening dyspnea and fatigue for last several weeks. Echocardiogram demonstrated a large cardiac mass with abundant vascular supply that incorporated the whole right ventricle and intraventricular septum, extended to the tricuspid valve, infiltrated the left ventricle apex and the base of posterior tricuspid leaflet. The right ventricle cavity was slit-like with small gradient in the outflow track. Cardiac magnetic resonance suggested angiosarcoma or lymphoma. PET-CT did not reveal any extracardiac metastases at that time. The case was discussed at our multidisciplinary cardio-oncology conference. The radical resection of the mass with subsequent TAH implantation was decided upon in high-risk setting, with feasible long-term survival, including eventual consideration for cardiac transplantation (HTX). Native ventricles were excised at the atrioventricular level down to the annulus of the mitral and tricuspid valves. The artificial ventricles were connected to the atrial remnants. The ascending aorta and the main pulmonary artery were dissected close to the valves and connected to outflow grafts. Histopathologic examination of the mass demonstrated angiosarcoma. The patient was started on paklitaksel. After completion of his chemotherapy regimen his eligibility for HTX will be reevaluated.

Conclusion
Diagnosis and therapy of sarcomas are challenging. Their rarity makes it difficult to standardize therapy. There is few evidence available in literature to guide the treatment of such patients. Case-specific, heart-team-based strategy must be adopted to aid decision making in this rare cases. Sarcomas are lethal, develop rapidly and affect fairly young patients. Prompt surgical resection with subsequent TAH implantation and adjuvant chemotherapy seems ideal in this situation as it offers a possible cure. It gives a slim chance of long-term survival after consecutive HTX for chemotherapy responders.
Abstract P239 Figure. Angiosarcoma in TTE, MRI and TEE.