Cytoreductive surgical resection of a rare pulmonary artery intimal sarcoma involving the pulmonary valve and right ventricle: a case report.
Michael L WilliamsCharis TanMartin MisfeldTristan D YanPublished in: Journal of surgical case reports (2021)
Pulmonary artery intimal sarcoma (PAIS) is an extremely rare malignant tumour. It is often misdiagnosed as chronic pulmonary thromboembolism. We describe a complex case in a 70-year-old man with PAIS extending into his right ventricle undergoing salvage cytoreductive surgical resection utilizing bivalirudin for cardiopulmonary bypass anticoagulation due to heparin-induced thrombocytopenia and thrombosis syndrome. The prognosis for PAIS is extremely poor, with a median survival of 1.5 months without surgical resection. Cytoreductive surgical debulking can improve the median survival time to 17 months. The main aim of palliative surgical resection is to improve ventilation-perfusion mismatch and prevent haemodynamic collapse.
Keyphrases
- pulmonary artery
- pulmonary hypertension
- pulmonary arterial hypertension
- coronary artery
- metastatic renal cell carcinoma
- venous thromboembolism
- mitral valve
- free survival
- aortic valve
- high glucose
- palliative care
- neoadjuvant chemotherapy
- drug induced
- pulmonary embolism
- atrial fibrillation
- percutaneous coronary intervention
- case report
- heart failure
- magnetic resonance imaging
- squamous cell carcinoma
- contrast enhanced
- magnetic resonance
- growth factor
- endothelial cells
- advanced cancer
- left ventricular
- mechanical ventilation
- computed tomography
- lymph node
- transcatheter aortic valve replacement
- congenital heart disease
- acute respiratory distress syndrome