Case Reports
Total Artificial Heart Implantation after Excision of Right Ventricular Angiosarcoma
Brian A. Bruckner, MD Walid K. Abu Saleh, MD Odeaa Al Jabbari, MD Jack G. Copeland, MD Jerry D. Estep, MD Matthias Loebe, MD, PhD Michael J. Reardon, MD
Primary cardiac sarcomas, although rare, are aggressive and lethal, requiring thorough surgical resection and adjuvant chemotherapy for the best possible outcome. We report the case of a 32-year-old woman who underwent total artificial heart implantation for rightsided heart failure caused by right ventricular angiosarcoma. For the first several weeks in intensive care, the patient recovered uneventfully. However, a postoperative liver biopsy indicated hepatocellular injury consistent with preoperative chemotherapy. She developed continuing liver failure, from which she died despite good cardiac function. (Tex Heart Inst J 2016;43(3):252-4)
O Key words: Angiosarcoma; antineoplastic agents; heart neoplasms; right ventricular tumors; metastatic sarcoma; total artificial heart From: Department of Cardiovascular Surgery (Drs. Abu Saleh, Al Jabbari, and Reardon) and Division of Transplant Surgery and Assist Devices (Drs. Bruckner, Copeland, Estep, and Loebe), Houston Methodist DeBakey Heart & Vascular Center, Houston Methodist Hospital, Houston, Texas 77030 Address for reprints: Brian A. Bruckner, MD, Department of Cardiovascular Surgery, Houston Methodist Hospital, 6550 Fannin St., Suite 1401, Houston, TX 77030 E-mail: babruckner@ houstonmethodist.org © 2016 by the Texas Heart ® Institute, Houston
252
f all primary cardiac tumors, approximately 75% are benign and 25% malignant; of the malignant tumors, 75% are sarcomas.1 Primary cardiac sarcomas often afflict young patients who have no predisposing factors, and these cancers tend to have a dismal prognosis. In studies involving treatment by medical therapy alone, 90% of primary cardiac sarcoma patients have died within 9 to 12 months. Surgical series of soft-tissue sarcomas outside the heart have shown that complete resection and adjuvant chemotherapy, when possible, yields the best possible outcome. We report the case of a 32-year-old woman who underwent total artificial heart (TAH) implantation for right-sided heart failure caused by right ventricular (RV) angiosarcoma.
Case Report A 32-year-old woman was referred to us from another hospital for treatment of an RV angiosarcoma. Originally, she had presented with worsening dyspnea, syncope, and fatigue, and cardiac magnetic resonance imaging had revealed a large RV mass that extended through the tricuspid valve (TV). Computed tomograms of the chest and abdomen did not reveal any extracardiac metastases at that time. A partial debulking was performed at the other hospital, and the patient was started on paclitaxel. Upon her 2-month follow-up evaluation, cardiac magnetic resonance imaging revealed an RV mass the same size as the initial mass, with extension to the TV. The patient was then started on adriamycin and ifosfamide but was able to tolerate only one round of chemotherapy because of thrombocytopenia, anorexia, and mucositis. The patient was referred to our practice and was admitted to the hospital for abdominal distention and pain. The results of her clinical examination were consistent with ascites. Upon paracentesis, 3.5 L of fluid, thought to be due to right-sided heart failure, was removed from her abdomen. She was started on doxorubicin and gemcitabine, with intermittent doses of docetaxel. After 14 cycles, she began reaching the upper limits of chemotherapy because of bone marrow depression, and scans at that time did not show any definitive disease outside her heart. The tumor occupied most of the RV apex and involved the interventricular septum (Fig. 1). At this time, our patient’s functional status was quite good. She was able to climb a flight of stairs despite chronic right-sided heart failure, valve destruction secondary to the tumor, and high venous pressures. Her condition was discussed during multiple medical review board meetings, and the decision was made to proceed with tumor resection and implantation of a TAH. The procedure was to be performed on an Institutional Review Board-approved protocol, and the patient gave full consent.
http://dx.doi.org/10.14503/THIJ-15-5131
Texas Heart Institute Journal • June 2016, Vol. 43, No. 3
Fig. 2 Photograph shows the resected right ventricle (with the tumor), the tricuspid valve, and a portion of the annulus. Fig. 1 Cardiac magnetic resonance image shows a right ventricular angiosarcoma (arrow).
Operative Technique
We took a midline-sternotomy approach, removing sternal wires and releasing dense adhesions. Bilaterally, the lungs were entrapped by numerous adhesions from loculated effusions, so we decorticated both lungs. An arterial cannula was placed in the distal aspect of the ascending aorta, and both the superior and inferior venae cavae were directly cannulated to establish cardiopulmonary bypass. The aorta and the pulmonary artery were then transected, and the left ventricle was removed. The native left atrium and the mitral annulus were left in place, but the mitral leaflets were removed along with the left atrial appendage. The tumor was noted to primarily involve the RV with invasion through the TV. The RV, TV, and a portion of the annulus were removed (Fig. 2), but the right atrium was left intact, because no tumor was seen on images or by direct viewing. Attention was now directed at implantation of the TAH (Fig. 3). Sewing rings for the left and right ventricles of the TAH were sutured with running 3-0 Prolene to the mitral and tricuspid annular tissue, respectively. Once secured into place, the rings were tested for leaks and reinforced as needed. The pulmonary artery outflow graft was sewn to the main pulmonary artery trunk, and the aortic graft was sewn to the native aorta, both with running 4-0 Prolene suture. For the first several weeks in the intensive care unit, the patient recovered uneventfully, was extubated, and ambulated with assistance, but with good hemodynamic function. However, she developed progressive ascites and liver failure that required multiple paracentesis procedures. Our hepatology service monitored her closely and Texas Heart Institute Journal
Fig. 3 Intraoperative photograph shows the implanted SynCardia total artificial heart after the complete resection of the right ventricular sarcoma. Both ventricles, which contained the sarcoma, have been completely resected. The atria remained intact and this enabled the placement of the artificial heart ventricles as shown.
performed a liver biopsy, which indicated hepatocellular injury consistent with the patient’s previous rounds of chemotherapy; she did not appear to be fully recovering. Indeed she developed continuing liver failure, from which she died despite good cardiac function.
Discussion Because primary cardiac sarcomas are rare, few programs have substantial experience. We have a longstanding cardiac sarcoma program and have published Excision of Right Ventricular Angiosarcoma
253
extensively on our surgical technique and outcomes.2-6 We can resect and reconstruct the entire left and right atria, but intraventricular tumors place restrictions on resection if reconstruction is to be successful. For pulmonary cardiac sarcoma, complete resection remains the cornerstone of treatment. For primary cardiac sarcoma that involves a large portion of either ventricle, complete excision of the ventricles and cardiac replacement are necessary if a surgical option is to be pursued. To our knowledge, this is the first report of a TAH implantation in a patient after resection of a malignant cardiac tumor. We previously reported our experience with resection, in a young patient, of a similar tumor that was confined to the RV and outflow tract; that procedure necessitated a total cardiectomy, together with the reconstruction and replacement of 2 biventricular assist devices.7 That patient survived for more than 30 days but eventually died of compression (by inflow-graft tamponade) of the atrial reconstruction that had been needed to interface with the 2 extracorporeal ventricular assist devices. In this report, we describe our use of the SynCardia® temporary Total Artificial Heart (SynCardia Systems, Inc.; Tucson, Ariz) as a cardiac replacement in a patient who had a large obstructive ventricular sarcoma. The advantages of using the SynCardia are the durability of the device and the ability to bridge a patient to a possible cardiac transplantation or even a destination therapy. However, atrial reconstruction can be challenging to combine with the SynCardia device, which requires relatively intact atria. Because our current patient had no atrial involvement, it was possible to resect both ventricles and implant the SynCardia as a cardiac replacement. By removing the RV tumor that had been causing right-sided outflow obstruction and therefore hepatic congestion, we expected immediate improvement in her right-sided heart failure and eventually in her liver function. However, this improvement was transient, for our patient appeared to have chronic irreversible damage to her liver, most likely from chemotherapy. We found her to be too high-risk to undergo any further surgical procedures, including a liver transplantation. Patients who have undergone substantial chemotherapy for cardiac tumors need extensive and thorough preoperative liver evaluation before any invasive therapy, including assist-device or TAH implantation. Ascites should also be considered a caution for surgical intervention. We were successful in completely removing the rightsided cardiac tumor, thereby obtaining surgically negative margins that enabled complete replacement of the heart. We conclude that complete excision of both ventricles and total cardiac replacement with the SynCardia device is possible and might be an option in carefully selected patients who have primary cardiac sarcoma with extensive ventricular involvement. 254
Excision of Right Ventricular Angiosarcoma
References 1. Bakaeen FG, Reardon MJ, Coselli JS, Miller CC, Howell JF, Lawrie GM, et al. Surgical outcome in 85 patients with primary cardiac tumors. Am J Surg 2003;186(6):641-7. 2. Kim MP, Correa AM, Blackmon S, Quiroga-Garza G, Weilbaecher D, Bruckner B, et al. Outcomes after right-side heart sarcoma resection. Ann Thorac Surg 2011;91(3):770-6. 3. Ramlawi B, Al-Jabbari O, Blau LN, Davies MG, Bruckner BA, Blackmon SH, et al. Autotransplantation for the resection of complex left heart tumors. Ann Thorac Surg 2014;98(3): 863-8. 4. Blackmon SH, Reardon MJ. Surgical treatment of primary cardiac sarcomas. Tex Heart Inst J 2009;36(5):451-2. 5. Blackmon SH, Patel A, Reardon MJ. Management of primary cardiac sarcomas. Expert Rev Cardiovasc Ther 2008;6(9): 1217-22. 6. Blackmon SH, Reardon MJ. Pulmonary artery sarcoma. Methodist DeBakey Cardiovasc J 2010;6(3):38-43. 7. Bruckner BA, Rodriguez LE, Bunge R, Motomura T, Estep JD, Loebe M, Reardon MJ. Large cardiac tumor managed with resection and two ventricular assist devices. Ann Thorac Surg 2014;97(1):321-4.
June 2016, Vol. 43, No. 3
Total Artificial Heart Implantation after Excision of Right Ventricular Angiosarcoma
Brian A. Bruckner, MD Walid K. Abu Saleh, MD Odeaa Al Jabbari, MD Jack G. Copeland, MD Jerry D. Estep, MD Matthias Loebe, MD, PhD Michael J. Reardon, MD
Primary cardiac sarcomas, although rare, are aggressive and lethal, requiring thorough surgical resection and adjuvant chemotherapy for the best possible outcome. We report the case of a 32-year-old woman who underwent total artificial heart implantation for rightsided heart failure caused by right ventricular angiosarcoma. For the first several weeks in intensive care, the patient recovered uneventfully. However, a postoperative liver biopsy indicated hepatocellular injury consistent with preoperative chemotherapy. She developed continuing liver failure, from which she died despite good cardiac function. (Tex Heart Inst J 2016;43(3):252-4)
O Key words: Angiosarcoma; antineoplastic agents; heart neoplasms; right ventricular tumors; metastatic sarcoma; total artificial heart From: Department of Cardiovascular Surgery (Drs. Abu Saleh, Al Jabbari, and Reardon) and Division of Transplant Surgery and Assist Devices (Drs. Bruckner, Copeland, Estep, and Loebe), Houston Methodist DeBakey Heart & Vascular Center, Houston Methodist Hospital, Houston, Texas 77030 Address for reprints: Brian A. Bruckner, MD, Department of Cardiovascular Surgery, Houston Methodist Hospital, 6550 Fannin St., Suite 1401, Houston, TX 77030 E-mail: babruckner@ houstonmethodist.org © 2016 by the Texas Heart ® Institute, Houston
252
f all primary cardiac tumors, approximately 75% are benign and 25% malignant; of the malignant tumors, 75% are sarcomas.1 Primary cardiac sarcomas often afflict young patients who have no predisposing factors, and these cancers tend to have a dismal prognosis. In studies involving treatment by medical therapy alone, 90% of primary cardiac sarcoma patients have died within 9 to 12 months. Surgical series of soft-tissue sarcomas outside the heart have shown that complete resection and adjuvant chemotherapy, when possible, yields the best possible outcome. We report the case of a 32-year-old woman who underwent total artificial heart (TAH) implantation for right-sided heart failure caused by right ventricular (RV) angiosarcoma.
Case Report A 32-year-old woman was referred to us from another hospital for treatment of an RV angiosarcoma. Originally, she had presented with worsening dyspnea, syncope, and fatigue, and cardiac magnetic resonance imaging had revealed a large RV mass that extended through the tricuspid valve (TV). Computed tomograms of the chest and abdomen did not reveal any extracardiac metastases at that time. A partial debulking was performed at the other hospital, and the patient was started on paclitaxel. Upon her 2-month follow-up evaluation, cardiac magnetic resonance imaging revealed an RV mass the same size as the initial mass, with extension to the TV. The patient was then started on adriamycin and ifosfamide but was able to tolerate only one round of chemotherapy because of thrombocytopenia, anorexia, and mucositis. The patient was referred to our practice and was admitted to the hospital for abdominal distention and pain. The results of her clinical examination were consistent with ascites. Upon paracentesis, 3.5 L of fluid, thought to be due to right-sided heart failure, was removed from her abdomen. She was started on doxorubicin and gemcitabine, with intermittent doses of docetaxel. After 14 cycles, she began reaching the upper limits of chemotherapy because of bone marrow depression, and scans at that time did not show any definitive disease outside her heart. The tumor occupied most of the RV apex and involved the interventricular septum (Fig. 1). At this time, our patient’s functional status was quite good. She was able to climb a flight of stairs despite chronic right-sided heart failure, valve destruction secondary to the tumor, and high venous pressures. Her condition was discussed during multiple medical review board meetings, and the decision was made to proceed with tumor resection and implantation of a TAH. The procedure was to be performed on an Institutional Review Board-approved protocol, and the patient gave full consent.
http://dx.doi.org/10.14503/THIJ-15-5131
Texas Heart Institute Journal • June 2016, Vol. 43, No. 3
Fig. 2 Photograph shows the resected right ventricle (with the tumor), the tricuspid valve, and a portion of the annulus. Fig. 1 Cardiac magnetic resonance image shows a right ventricular angiosarcoma (arrow).
Operative Technique
We took a midline-sternotomy approach, removing sternal wires and releasing dense adhesions. Bilaterally, the lungs were entrapped by numerous adhesions from loculated effusions, so we decorticated both lungs. An arterial cannula was placed in the distal aspect of the ascending aorta, and both the superior and inferior venae cavae were directly cannulated to establish cardiopulmonary bypass. The aorta and the pulmonary artery were then transected, and the left ventricle was removed. The native left atrium and the mitral annulus were left in place, but the mitral leaflets were removed along with the left atrial appendage. The tumor was noted to primarily involve the RV with invasion through the TV. The RV, TV, and a portion of the annulus were removed (Fig. 2), but the right atrium was left intact, because no tumor was seen on images or by direct viewing. Attention was now directed at implantation of the TAH (Fig. 3). Sewing rings for the left and right ventricles of the TAH were sutured with running 3-0 Prolene to the mitral and tricuspid annular tissue, respectively. Once secured into place, the rings were tested for leaks and reinforced as needed. The pulmonary artery outflow graft was sewn to the main pulmonary artery trunk, and the aortic graft was sewn to the native aorta, both with running 4-0 Prolene suture. For the first several weeks in the intensive care unit, the patient recovered uneventfully, was extubated, and ambulated with assistance, but with good hemodynamic function. However, she developed progressive ascites and liver failure that required multiple paracentesis procedures. Our hepatology service monitored her closely and Texas Heart Institute Journal
Fig. 3 Intraoperative photograph shows the implanted SynCardia total artificial heart after the complete resection of the right ventricular sarcoma. Both ventricles, which contained the sarcoma, have been completely resected. The atria remained intact and this enabled the placement of the artificial heart ventricles as shown.
performed a liver biopsy, which indicated hepatocellular injury consistent with the patient’s previous rounds of chemotherapy; she did not appear to be fully recovering. Indeed she developed continuing liver failure, from which she died despite good cardiac function.
Discussion Because primary cardiac sarcomas are rare, few programs have substantial experience. We have a longstanding cardiac sarcoma program and have published Excision of Right Ventricular Angiosarcoma
253
extensively on our surgical technique and outcomes.2-6 We can resect and reconstruct the entire left and right atria, but intraventricular tumors place restrictions on resection if reconstruction is to be successful. For pulmonary cardiac sarcoma, complete resection remains the cornerstone of treatment. For primary cardiac sarcoma that involves a large portion of either ventricle, complete excision of the ventricles and cardiac replacement are necessary if a surgical option is to be pursued. To our knowledge, this is the first report of a TAH implantation in a patient after resection of a malignant cardiac tumor. We previously reported our experience with resection, in a young patient, of a similar tumor that was confined to the RV and outflow tract; that procedure necessitated a total cardiectomy, together with the reconstruction and replacement of 2 biventricular assist devices.7 That patient survived for more than 30 days but eventually died of compression (by inflow-graft tamponade) of the atrial reconstruction that had been needed to interface with the 2 extracorporeal ventricular assist devices. In this report, we describe our use of the SynCardia® temporary Total Artificial Heart (SynCardia Systems, Inc.; Tucson, Ariz) as a cardiac replacement in a patient who had a large obstructive ventricular sarcoma. The advantages of using the SynCardia are the durability of the device and the ability to bridge a patient to a possible cardiac transplantation or even a destination therapy. However, atrial reconstruction can be challenging to combine with the SynCardia device, which requires relatively intact atria. Because our current patient had no atrial involvement, it was possible to resect both ventricles and implant the SynCardia as a cardiac replacement. By removing the RV tumor that had been causing right-sided outflow obstruction and therefore hepatic congestion, we expected immediate improvement in her right-sided heart failure and eventually in her liver function. However, this improvement was transient, for our patient appeared to have chronic irreversible damage to her liver, most likely from chemotherapy. We found her to be too high-risk to undergo any further surgical procedures, including a liver transplantation. Patients who have undergone substantial chemotherapy for cardiac tumors need extensive and thorough preoperative liver evaluation before any invasive therapy, including assist-device or TAH implantation. Ascites should also be considered a caution for surgical intervention. We were successful in completely removing the rightsided cardiac tumor, thereby obtaining surgically negative margins that enabled complete replacement of the heart. We conclude that complete excision of both ventricles and total cardiac replacement with the SynCardia device is possible and might be an option in carefully selected patients who have primary cardiac sarcoma with extensive ventricular involvement. 254
Excision of Right Ventricular Angiosarcoma
References 1. Bakaeen FG, Reardon MJ, Coselli JS, Miller CC, Howell JF, Lawrie GM, et al. Surgical outcome in 85 patients with primary cardiac tumors. Am J Surg 2003;186(6):641-7. 2. Kim MP, Correa AM, Blackmon S, Quiroga-Garza G, Weilbaecher D, Bruckner B, et al. Outcomes after right-side heart sarcoma resection. Ann Thorac Surg 2011;91(3):770-6. 3. Ramlawi B, Al-Jabbari O, Blau LN, Davies MG, Bruckner BA, Blackmon SH, et al. Autotransplantation for the resection of complex left heart tumors. Ann Thorac Surg 2014;98(3): 863-8. 4. Blackmon SH, Reardon MJ. Surgical treatment of primary cardiac sarcomas. Tex Heart Inst J 2009;36(5):451-2. 5. Blackmon SH, Patel A, Reardon MJ. Management of primary cardiac sarcomas. Expert Rev Cardiovasc Ther 2008;6(9): 1217-22. 6. Blackmon SH, Reardon MJ. Pulmonary artery sarcoma. Methodist DeBakey Cardiovasc J 2010;6(3):38-43. 7. Bruckner BA, Rodriguez LE, Bunge R, Motomura T, Estep JD, Loebe M, Reardon MJ. Large cardiac tumor managed with resection and two ventricular assist devices. Ann Thorac Surg 2014;97(1):321-4.
June 2016, Vol. 43, No. 3
