Salvage Right Ventricular Outflow Tract Reconstruction for Pulmonary Embolization with Pulmonary Artery Sarcoma

TL;DR

A rare case of pulmonary artery intimal sarcoma causing severe heart issues was treated with emergency surgery, providing temporary relief but not a cure.

Contribution

This paper presents a novel surgical approach for palliative treatment of PAIS with pulmonary embolization.

Findings

Emergency salvage surgery improved symptoms and allowed the patient to be discharged asymptomatic.

Palliative reconstruction of the right ventricular outflow tract was feasible in this rare condition.

Despite successful surgery, tumor progression occurred, highlighting the aggressive nature of PAIS.

Abstract

Pulmonary artery intimal sarcoma (PAIS) is extremely rare and highly malignant. Although improved outcomes have been reported after complete surgical resection and chemotherapy, limited information is available regarding the indications, procedures, and prognosis of palliative surgery for PAIS. This report describes a successful salvage surgical case for rapid hemodynamic deterioration due to PAIS obstructing the pulmonary artery trunk.

A 64-year-old woman, complaining of dyspnea for a month, was referred for a pulmonary artery tumor. Imaging studies confirmed an intraluminal tumor that obstructs the pulmonary artery trunk and extends to the right ventricular wall and interventricular septum, suspecting a malignancy. During preoperative workups, she developed acute hemodynamic and respiratory deterioration due to pulmonary embolization, so emergency surgery was planned on a salvage basis. The tumor originated from the pulmonary artery intima just distal to the pulmonary valve, obstructed the pulmonary artery trunk, and extensively involved the left main coronary artery and the interventricular septum, where complete resection of the tumor was not achieved. Reconstruction of the pulmonary valve, the right ventricular outflow tract (RVOT), and bilateral pulmonary arteries were performed using a composite of a prosthetic valve and vascular grafts. The patient’s postoperative course was uneventful, and she was discharged home asymptomatic. Pathological diagnosis of the operative specimen confirmed pulmonary intimal sarcoma. After 4 months of postoperative chemotherapy, tumor progression was confirmed. The patient passed away at home 8 months after surgery.

We reported a case of PAIS presenting with RVOT obstruction and rapid hemodynamic and respiratory deterioration, who underwent succeeding emergent surgery and was discharged home asymptomatic. Palliative RVOT reconstruction can be a useful surgical option for PAIS accompanying pulmonary embolization on a salvage basis.

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