Cardiac intimal sarcoma presenting with pseudo-pulmonary embolism: surgical debulking, RVOT reconstruction and pulmonary valve replacement

Intimal sarcoma is a malignant mesenchymal tumour arising in large blood vessels of systemic and pulmonary circulation, depicted as undifferentiated and aggressive. Clinical presentation of these cardiac masses is often subtle, making misdiagnosis possible. We discuss the case of a 27-year-old woman with a recent history of delivery, admitted for dyspnoea and signs of right-heart failure. A diagnosis of pulmonary embolism was made on a computed tomography angiogram and transoesophageal echocardiography basis. Emergency surgery was performed due to haemodynamic instability. Pulmonary arteriotomy revealed a large solid polylobate mass instead of a thrombus. Surgical debulking, with reconstruction of the right ventricle outflow tract, and pulmonary valve replacement were therefore necessary. Partial pulmonary annulus reconstruction was furthermore performed with a strip of heterologous pericardium with a 'sandwich technique'. Prompt diagnosis of intimal sarcoma was made, permitting initiation of timely treatment with anthracycline. Surgical resection of malignancies involving either the heart or great vessels is uncommonly performed because of the potential morbidity and mortality and the unknown probability of significant cure. Preoperative imaging is then of paramount importance in determining the diagnosis and planning for surgery. In this specific case, the haemodynamic instability, the high suspicion of a thrombotic nature of the mass, and young age precluded the performing of any second-level examination, leading surgeons to an unexpected reconstruction.