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Address for correspondence: Mikalai Shchatsinka, Republican Scientific and Practical Centre “Cardiology”, 110B R. Luxemburg St, 220036 Minsk, Belarus, phone: +37 544 714 12 52, e-mail: mikalai.shchatsinka@gmail.com Received: 5.08.2018, accepted: 31.08.2018. Primary pulmonary artery sarcoma (PPAS) is a rare tumor originating from the intimal mesenchymal cells of the pulmonary artery with an incidence of 0.001–0.03% and is usually fatal [1]. The diagnosis is difficult and delayed in most cases. It usually presents in adults (almost equally in males and females), with median age of presentation of 50 years [2]. Newer imaging techniques could allow early diagnosis in patients with symptoms of pulmonary vascular obstruction [3]. Surgical resection improves clinical symptoms, offers the only chance of cure and is performed in many cardiothoracic clinics. The PPAS spreading to lobular branches of pulmonary artery (PA) represents an especially serious surgical challenge. A 58-year-old female patient was referred to our unit in December 2016 complaining of dyspnea on moderate physical exertion and unproductive cough starting 2 months prior to admission. Transthoracic echocardiography revealed a mass formation of 62/24 mm in the pulmonary trunk, fused with the valvular leaflets. Third-degree tricuspid valve regurgitation was recorded as well. Aortic and mitral valves showed no significant change, with left ventricular ejection fraction (LVEF) of 62%, and right ventricular ejection fraction (RVEF) of 42%. Laboratory findings revealed no significant changes. Computed tomography angiography (CTA) showed a negative dynamic in comparison to CT data of a month earlier: the tumor spread from the bifurcation of the pulmonary trunk by 5 mm to the left branch of the pulmonary artery, to the bifurcation of the right branch of the PA and up to 10 mm to the valvular space of the right ventricular outflow tract (Fig. 1 A). Tumor emboli in the segmental branches of the PA were also seen. In the lung parenchyma, foci appeared to have increased in size: 4 mm in S6 on the right (+3 mm), 6 mm in S3 on the left (+1–2 mm), 7 mm in S6 on the left (newly formed). Positron emission tomography (PET) confirmed the presence of metabolic-active formation in the pulmonary truck spreading to both PA and secondary changes in S6 of the right and in S3 and S6 of the left lung. In this case changes in the lungs were assumed as secondary to primary sarcoma of the PA. Surgery was performed on December 28, 2016 through median sternotomy. Before institution of cardiopulmonary bypass (CPB) and following a revision of the lungs, an atypical resection of the foci in S6 of the right lung and in S3 and S6 of the left lung was performed. Upon opening the pericardium, about 150 ml of fluids was drained with subsequent sudden disturbance in hemodynamics with systolic pressure dropping to less than 50 mm Hg, followed by an increase in the central venous pressure and asystole. Direct cardiac massage was performed and CPB was started. Under a total operative time of 380 min (myocardial ischemia time of 181 min) and hypothermia of 26°C, thromboendarterectomy from both PA branches with complete replacement of the pulmonary trunk by pulmonary trunk allograft (PTA, ring diameter 28 mm) was performed. Annuloplasty repair of the tricuspid valve by De Vega was also performed. Due to insufficient length of the right branch of the PTA, around 85 mm in length of bifurcation of the abdominal aorta allograft (BAAA, 20 mm in diameter) was anastomosed to the lobular branch of the PTA. To fit the diameter of one iliac branch of the BAAA, the right upper and lower lobular PA branches were sutured together for a suitable anastomosis, while the other iliac branch of the BAAA was ligated (Figs. 1 B, C). Perioperative blood replacement therapy – 5 doses of erythrocyte mass, 5 doses of 10% albumin solution, 3 doses of Octaplas (a pooled human plasma solution, Octapharma Pharmazeutika Produktionsges m.b.H., Vienna, Austria). The patient was extubated after 13 h but remained in the intensive care unit for 5 days due to moderate respiratory failure. Postoperative chest X-ray showed subsegmental atelectases of both lobes on both sides, as a consequence Surgical treatment of primary pulmonary sarcoma disseminated to both pulmonary arteries using composite pulmonary trunk and bifurcation of abdominal aorta allograft

kardiochirurgia i torakochirurgia polska

Surgical treatment of primary pulmonary sarcoma disseminated to both pulmonary arteries using composite pulmonary trunk and bifurcation of abdominal aorta allograft