Introduction: Primary cardiac tumors are rare at 0.001%-0.3% in the general population. Untreated, these tumors (particularly malignant) carry a terrible prognosis via local extension and cardiovascular compromise. Complete surgical resection via autotransplantation has proven to significantly improve outcome when compared to chemotherapy and other conservative management (Putnam). Simultaneous pneumonectomy has previously be linked to increase operative mortality from 11% to 43% and decreased median survival from 378 days to 55 days (Ramwali), who concluded that a need for simultaneous pneumonectomy is a strict contraindication for surgical resection. Another group found 50% operative mortality with simultaneous pneumonectomy as opposed to 0% operative mortality with autotransplantation alone (Blackmon).
Case Presentation: 20F who presented to OSH with atrial fibrillation with RVR found on echo to have myxoid and spindle cell sarcoma of left atrium. At OSH she underwent partial debulking but ultimately transferred to UCSD for higher level of care. She was found to be pregnant at this time and had her pregnancy terminated. Imaging modalities included: CT chest, CT head, Cardiac MRI and PET CT. Imaging revealed multilobulated mass in left atrium that extended to the posterior annulus of the mitral valve as well as into the left pulmonary veins with near-complete occlusion of the left sided pulmonary veins.
Surgical Intervention and Anesthesia Concerns: Induction with Etomidate 0.3mg/kg, Fentanyl 10mcg/kg, Midazolam 3mg, Rocuronium 100mg, Isoflurance for maintenance. Cordis and Swan Ganz catheter palced in Right IJ, TEE placed. Cardiopulmonary bypass time of 290 minutes with aortic clamp time of 153 minutes at 28⁰C. Cardiectomy performed and all visual signs of sarcoma were removed from left atrium. Left pneumonectomy performed with stapling of left pulmonary artery and main bronchus. Reanastamosis for cardiac autotransplantation performed. Patient received 3 units platelets and 2 units FFP at end of procedure. Required Dopamine 3mcg/kg/min and Phenylephrine 40 mcg/min for transport to ICU, with final Cardiac Index noted to be 2.8 L/min/m2. Patient was cared for in the ICU under CT Surgery, Anesthesia Critical Care and Cardiology. Patient was extubated POD#2. Developed infection of unknown origin requiring 10 days of broad spectrum antibiotics. Developed right heart dysfunction requiring short term milrinone, epoprostenol, sildenafil and diuretics. Left sided vocal cord dysfunction noted as well. Doing well at 1 year follow up, continuing care in Las Vegas.
Discussion: Prior data indicates that 30-day, 1 year and 2 year mortality for cardiac autotransplantation with simultaneous pneumonectomy is 43%, 86%, 86%, respectively (Ramwali). Mortality was primarily from bleeding into the pneumonectomy site with subsequent “coagulopathy, transfusion, volume overload, unilateral pulmonary edema, and ultimately death.” Although the data sets are small (7 combined with 26 total for Ramwali et al and 6 combined with 21 total for Blackmon et al) they have prevented these groups from continuing with performing surgery in patients with disease extending into lung tissue. The presented case demonstrates a successful combined approach, with no bleeding complications. Future examination of technique differences may allow for treatment options in patients required a combined approach.
