Texas Two-Step Procedure for a Patient With Pulmonary Artery Sarcoma

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The patient was a 21-year-old female with a past medical history of attention-deficit hyperactivity disorder (ADHD) and anxiety who presented to the emergency department (ER) with symptoms of shortness of breath, cough, and chest pain. She was initially diagnosed with pulmonary artery (PA) saddle thrombus by computed tomography (CT) and started on intravenous heparin; however, her symptoms did not improve. 

A positron emission tomography (PET) scan showed a PA mass with high metabolic activity and no signs of distant metastasis. Magnetic resonance imaging (MRI) of the chest revealed a soft tissue density with a maximal thickness of 2.2 cm/s, completely filling the right main PA and extending into the lobar, segmental, and subsegmental branches. The mass filled almost the entirety of the left PA, extending into the proximal segmental branches of the left upper and lower lobe. 

The potential diagnosis of a PA tumor was raised. Transthoracic echocardiography revealed normal left ventricular function; however, the right ventricular (RV) systolic function was moderately depressed. A ventilation/perfusion (V/Q) scan showed an absent perfusion to the right lung, indicating a nonfunctioning right lung. 

After a multidisciplinary tumor team discussion, the patient was deemed an urgent surgical candidate with no time for neoadjuvant chemotherapy. The surgical plan was to perform a complete anatomical tumor resection using PA and pulmonary root replacement, PA endarterectomy, and a staged right pneumectomy. Based on prior experience, survival after cardiac tumor resection requiring a pneumectomy are far superior when performed in a staged fashion rather than concomitantly. 
 
After going on total bypass with direct superior vena cava (SVC) and inferior vena cava (IVC) cannulation, the PA and aortic root were carefully separated. The aorta was cross-clamped, and cardiac arrest was implemented using antegrade del Nido cardioplegia. Then the PA and aorta were transected to improve visualization of the right and left pulmonary arteries. The tumor was identified and resected from the proximal left PA, and further dissection of the left PA was performed before continuing with the right PA. 

After satisfactory preparation, the right PA was divided. The extensive tumor was visible, filling the whole lumen of the vessel. An endarterectomy was then performed on the right side to leave a cuff that could be oversewn and closed in preparation for the later planned right pneumectomy. The vessel was closed with two continuous suture lines. 

Attention was turned back to the left PA, where the large intimal tumor was observed. This vessel was exposed with interrupted pledgeted sutures. Careful intimal tumor resection was performed using a Jamison sucker, extending all the way out to the left pulmonary artery bifurcation. All tumors were grossly removed. Intraoperative frozen section had shown spindle cell neoplasm, confirming the mass as sarcoma. 

The left main PA was then transected more distally over a drop sump, and the remaining vessel was exposed with interrupted sutures. Approximately a 3-4 mm mobilized segment of the pulmonary artery was left in front of the two pulmonary artery branches as a satisfactory cuff for the distal anastomosis. The removed segment was sent for permanent section. 
 
A 16 mm Dacron graft was carefully sewn to the remaining left PA with a 5-0 Prolene suture. The remaining pulmonary artery going down to the root was examined, revealing tumor extension proximally in the main PA to approximately 1 cm above the pulmonic valve. Therefore, the back wall of the PA was mobilized. A right angle was pplaced 5 mm underneath the pulmonic valve and passed anteriorly into the right ventricular muscle. The pulmonary root was then resected using a blade followed by electrocautery. The muscle was skived posteriorly to avoid damaging the first septal perforator. 
 
A 29 mm cadaveric pulmonic homograft was prepared as the right main PA was closed with surgical staplers. The muscle part was carefully trimmed, and the homograft was sutured to the pulmonary root with 4-0 Prolene sutures. Once the anastomosis was completed, the Dacron graft and homograft were stretched out and trimmed adequately. The Dacron graft was beveled to match the diameter of the pulmonary root, and they were sutured together with 5-0 Prolene sutures. 
 
Prior to completing the suture line, the heart was filled after the caval snares were removed, and the pulmonary root was de-aired. After that, the aorta was sutured back together with 4-0 SH Prolene sutures. Following the completion of the suture line, the heart was deaired in the usual fashion through the vent, and the cross-clamp was removed. A pacing wire was placed on the inferior RV surface. The patient was weaned from cardiopulmonary bypass with the use of dobutamine, without complications. After decannulation and the achievement of hemostasis, the chest was closed, and two chest tubes were left in place.  

The patient was kept on the ventilator and underwent a right robotic pneumectomy on postoperative day four. The pathology report revealed high-grade spindle cell sarcoma with an R0 resection margin. The postoperative course was complicated with biventricular dysfunction; however, the patient could be discharged on postoperative day 11. Since then, adjuvant chemotherapy was started, and postoperative pulmonary hypertension was treated with phosphodiesterase-5 inhibitors and endothelin-receptor antagonists. Two months after the procedure, the patient was doing well on optimal medical therapy. 

References

1. Chan EY, Ali A, Zubair MM, Nguyen DT, Ibarra-Cortez SH, Graviss EA, Shapira OM, Ravi V, MacGillivray TE, Reardon MJ. Primary cardiac sarcomas: Treatment strategies. J Thorac Cardiovasc Surg. 2023 Sep;166(3):828-838.e2. doi: 10.1016/j.jtcvs.2021.10.070. Epub 2022 Feb 1. PMID: 35219517.
2. Chan EY, Reul RM, Kim MP, Reardon MJ. The "Texas Two-Step" procedure. J Thorac Cardiovasc Surg. 2018 Jan;155(1):285-287. doi: 10.1016/j.jtcvs.2017.08.117. Epub 2017 Sep 13. PMID: 28964495.
3. Ramlawi B, Al-Jabbari O, Blau LN, Davies MG, Bruckner BA, Blackmon SH, Ravi V, Benjamin R, Rodriguez L, Shapira OM, Reardon MJ. Autotransplantation for the resection of complex left heart tumors. Ann Thorac Surg. 2014 Sep;98(3):863-8. doi: 10.1016/j.athoracsur.2014.04.125. Epub 2014 Jul 31. PMID: 25086947.

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