A new approach to the assessment of aortic cusp geometry

Abstract

perfusion, thus resulting in clinically unspecific symptoms of dyspnea, cough, or impaired right ventricular function mimicking pulmonary thromboembolism in the majority of cases. The radiologic diagnosis can still be difficult despite the technologic advances in imaging techniques. Preoperative biopsy is the only way to establish a proper diagnosis, and it is essential to accurately plan the surgical approach. The only treatment of choice is radical surgical resection. Depending on tumor localization and distal extension, surgical treatment includes PA resection, a polytetrafluoroethylene (Gore-Tex) homograft, or even xenograft reconstruction and pneumonectomy. The use of cardiopulmonary bypass may be essential in patients in whom the tumor originates from the PA trunk or the pulmonary valve. As a palliative treatment, endovascular stent grafting may be an alternative therapeutic option in inoperable cases. Furthermore, neoadjuvant or adjuvant chemotherapy has been mentioned in the literature with doubtful results. Prolonged tumor-free survival is rare. A 10-month survival is reported in the literature for different sarcomas of PA, whereas in our series survival was 7 and 2.5 years, respectively. Conclusion Early diagnosis along with aggressive and extensive surgical treatment in patients with PA leiomyosarcoma without any extrathoracic metastases may provide an opportunity for prolonged survival in addition to palliation.