Catheterization and Cardiovascular Diagnosis 25:132-134 (1992)
Case ReDorts
Thrombus Causing Fatal Left Vent ricular Outflow Tract Obstruction in a Heart Transplant Patient Vivian L. Clark, MD, FACC, and T. Barry Levine, MD, FACC A 60 year male, orthotoplc heart transplant recipient developed a fatal left Ventricular outflow obstruction secondary to thrombus at 38 months post transplant. Although he had episodes of mild to moderate rejection at 2 and 16 months post transplant, subsequent biopsies were negative and annual cardiac catheterizatlons showed mild left ventricular hypoklnesls and normal coronary arteries. This case represents a catastrophic complication of transplant rejection and illustrates the problems with Identifying rejection using current diagnostic methods. Key words: cardiac thrombus, cardiac transplant rejection, orthotopic heart transplant
INTRODUCTION
post transplantation showed mild apical hypokinesis and normal coronary arteries. Endomyocardial biopsy done Rejection, graft atherosclerosis, and infection are the 16 months post transplant showed mild to moderate acute major causes of morbidity and mortality in the heart rejection and a follow-up biopsy 1 month later showed transplant population [ 11. The frequency of intracardiac no evidence of rejection. Subsequent biopsies were also thrombus formation in this population is unknown, but negative. Throughout his post-transplant course trough nevertheless is a common occurrence among patients cyclosporine levels by radioimmunoassay ranged from with cardiovascular disease and sometimes leads to em120 to 190 kg/dl. bolization and associated vascular events [2]. ObstrucThirty-nine months post transplant he presented with tion of valves or outflow tract by a thrombus, however, congestive heart failure. An echocardiogram showed is a rare phenomenon [3,4]. Herein, a case of fatal left generalized left ventricular hypokinesis with an ejection ventricular outflow tract obstruction by a thrombus in a fraction of 40%. Biopsy, however, was negative for reheart transplant recipient is described. jection. He improved with medical therapy. Two months later he again presented with congestive heart failure. Electrocardiogram showed left atrial enlargement, right CASE REPORT axis deviation and anterolateral and inferior T wave abG.N. is a 60-yr-old white male who underwent ortho- normalities, unchanged from previous tracings. Again, topic cardiac transplant in June 1986 for ischemic cardi- biopsy showed no rejection, but echocardiogram again omyopathy. The ischemic time for the donor heart was showed generalized left ventricular hypokinesis, as well 105 minutes. The immediate post-operative course was as biatrial enlargement and a large left atrial thrombus. complicated by a sternal wound infection which required He was treated with diuretics and anticoagulants and parenteral antibiotics and debridement. He received a again improved. Retransplant was considered but not felt standard immuno-suppressive protocol consisting of to be an option due to technical considerations, primarily prednisone and cyclosporine at a dose of 80 mg every 12 marked intrathoracic fibrosis related to prior surgical hours. His first three postoperative biopsies were nega- procedures. tive, but the the fourth biopsy showed mild acute rejection. His post transplant course was uncomplicated except From the Heart Failure and Cardiac Transplant, Henry Ford Hospital, for a loculated left pleural empyema, felt to be related to Heart and Vascular Institute, Detroit, Michigan. previous bypass surgery, which required drainage and Received June 24, 1991; revision accepted August 27, 1991 antibiotic therapy. Two additional biopsies done during the 1st year post transplant showed no evidence of re- Address reprint requests to Vivian Clark, M.D., Henry Ford Hospital, jection. Cardiac catheterizations done 1, 2, and 3 years K2, 2799 West Grand Blvd., Detroit, MI 48202. 0 1992 Wiley-Liss, Inc.
LV Outflow Obstruction
133
Fig. 1. Left coronary anglography In right anterior oblique projection showing contrast, regurgitating across an incompetent aortic valve, outlining a radiolucent mass (arrows) lying in the left ventricular outflow tract.
Six days later he developed profound hypotension and
outflow tract and the obstruction relieved. At surgery no
ECG showed anterolateral ST segment elevation and loss thrombus was found. Unfortunately, the patient continof R waves in the precordial and lateral leads consistent with acute anterolateral myocardial infarction. He failed to improve with vasopressors and underwent emergency cardiac catheterization. Angiography of the left coronary artery showed extremely slow flow without evidence of coronary thrombus or obstruction. During coronary injection there was regurgitation of contrast across an incompetent aortic valve which outlined a radiolucent mass, which appeared to be a thrombus, in the left ventricular outflow tract (Fig. 1). This mass was visible throughout the entire cineangiographic run. Because of continued hemodynamic deterioration, after consultation with the cardiothoracic surgeons, the patient was sent for an emergency cardiac embolectomy. During transport to the operating room the patient’s blood pressure, which had been 80/50 torr, rose dramatically to 150/80 torr and his color improved. The clinical impression was that the clot may have become dislodged from the ventricular
ued to deteriorate and did not survive the procedure. At post-mortem examination no thrombus was found within the left ventricular cavity. A 2 x 4 x 0.8 cm thrombus was found within the left atrium near the mitral valve. Autopsy also revealed evidence of acute and chronic rejection and severe diffuse coronary obstructive disease, as well as old anterior infarction with aneurysm formation and acute necrosis of the septum and posterolateral wall. The distal arterial beds were not examined for evidence of emboli. DISCUSSION This patient suffered a catastrophic event as a result of large intracavitary cardiac thrombus. Outflow tract obstruction usually presents with syncope, chest pain, congestive heart failure, or arrhythmias and symptoms can be intermittent, as is often the case with cardiac tumors.
134
Clark and Levine
Obstructive shock is most often seen in the setting of massive pulmonary emboli but has been reported with ventricular outflow tract obstruction [3]. In this case it is most likely that the thrombus migrated from the left atrium to the left ventricular cavity. A remnant of the atrial thrombus was found at post mortem. Although cardiac thrombus formation is common, clinical embolic events occur in only a small percentage of cases, and emboli large enough to compromise cardiac outflow are exceedingly rare [2]. Previous echocardiographic studies in heart transplant recipients have demonstrated atrial thrombus formation in a significant number [5].Diminished left ventricular function as a result of chronic rejection has been associated with increased left atrial spontaneous echo contrast and probably contributed to this patient’s thrombus formation [6]. This patient was not known to have any other predisposing factors for thromboembolic disease. This case also illustrates a number of other important points. First, it demonstrates that endomyocardial biopsy can be an unreliable method for detecting acute rejection. Second, serial cardiac catheterization may fail to identify coronary occlusive disease, particularly if it is of a dif-
fuse nature. Progressive deterioration in left ventricular function, as shown by left ventriculography and echocardiography, was the most important clue in this individual that significant cardiac pathology was present.
REFERENCES I . Heck CF, Shumway SJ, Kaye MP: The registry of the International Society for Heart Transplantation: Sixth official report 1989. J Heart Transplant 8:271-276. 1989. 2. Meltzer RS, Visser CA, Fuster V: lntracardiac thrombi and systemic embolization. Ann Intern Med 104:689-698, 1986. 3. Waller BF, Dean PJ, Mann 0, Rosen JH and Roberts WC: Right ventricular outflow obstruction from thrombus with small peripheral pulmonary emboli. Chest 79:224-225, 1981. 4. Gultekin N , Dogar H. Turkoglu C, Ozturk S, Gokhan N and Demiroglu C: Giant right atrial thrombus causing ventricular inflow and outflow obstruction. Am Heart J 1161367-1369, 1988. 5. Angerman C. Spes C, Stempfle U, et al.: Morphological and functional characteristics pf the transplanted heart: transthoracic versus transesophageal echocardiographic findings. (Abstr) Circulation 80 (Suppl II):474. 1989. 6. Polanco G, Jafn SM, Click CL, Alam M, Levine TB: Transesophageal versus transthoracic echocardiography in the heart transplant population. (Abstr) Clin Res 38(3):884A, 1990.
Case ReDorts
Thrombus Causing Fatal Left Vent ricular Outflow Tract Obstruction in a Heart Transplant Patient Vivian L. Clark, MD, FACC, and T. Barry Levine, MD, FACC A 60 year male, orthotoplc heart transplant recipient developed a fatal left Ventricular outflow obstruction secondary to thrombus at 38 months post transplant. Although he had episodes of mild to moderate rejection at 2 and 16 months post transplant, subsequent biopsies were negative and annual cardiac catheterizatlons showed mild left ventricular hypoklnesls and normal coronary arteries. This case represents a catastrophic complication of transplant rejection and illustrates the problems with Identifying rejection using current diagnostic methods. Key words: cardiac thrombus, cardiac transplant rejection, orthotopic heart transplant
INTRODUCTION
post transplantation showed mild apical hypokinesis and normal coronary arteries. Endomyocardial biopsy done Rejection, graft atherosclerosis, and infection are the 16 months post transplant showed mild to moderate acute major causes of morbidity and mortality in the heart rejection and a follow-up biopsy 1 month later showed transplant population [ 11. The frequency of intracardiac no evidence of rejection. Subsequent biopsies were also thrombus formation in this population is unknown, but negative. Throughout his post-transplant course trough nevertheless is a common occurrence among patients cyclosporine levels by radioimmunoassay ranged from with cardiovascular disease and sometimes leads to em120 to 190 kg/dl. bolization and associated vascular events [2]. ObstrucThirty-nine months post transplant he presented with tion of valves or outflow tract by a thrombus, however, congestive heart failure. An echocardiogram showed is a rare phenomenon [3,4]. Herein, a case of fatal left generalized left ventricular hypokinesis with an ejection ventricular outflow tract obstruction by a thrombus in a fraction of 40%. Biopsy, however, was negative for reheart transplant recipient is described. jection. He improved with medical therapy. Two months later he again presented with congestive heart failure. Electrocardiogram showed left atrial enlargement, right CASE REPORT axis deviation and anterolateral and inferior T wave abG.N. is a 60-yr-old white male who underwent ortho- normalities, unchanged from previous tracings. Again, topic cardiac transplant in June 1986 for ischemic cardi- biopsy showed no rejection, but echocardiogram again omyopathy. The ischemic time for the donor heart was showed generalized left ventricular hypokinesis, as well 105 minutes. The immediate post-operative course was as biatrial enlargement and a large left atrial thrombus. complicated by a sternal wound infection which required He was treated with diuretics and anticoagulants and parenteral antibiotics and debridement. He received a again improved. Retransplant was considered but not felt standard immuno-suppressive protocol consisting of to be an option due to technical considerations, primarily prednisone and cyclosporine at a dose of 80 mg every 12 marked intrathoracic fibrosis related to prior surgical hours. His first three postoperative biopsies were nega- procedures. tive, but the the fourth biopsy showed mild acute rejection. His post transplant course was uncomplicated except From the Heart Failure and Cardiac Transplant, Henry Ford Hospital, for a loculated left pleural empyema, felt to be related to Heart and Vascular Institute, Detroit, Michigan. previous bypass surgery, which required drainage and Received June 24, 1991; revision accepted August 27, 1991 antibiotic therapy. Two additional biopsies done during the 1st year post transplant showed no evidence of re- Address reprint requests to Vivian Clark, M.D., Henry Ford Hospital, jection. Cardiac catheterizations done 1, 2, and 3 years K2, 2799 West Grand Blvd., Detroit, MI 48202. 0 1992 Wiley-Liss, Inc.
LV Outflow Obstruction
133
Fig. 1. Left coronary anglography In right anterior oblique projection showing contrast, regurgitating across an incompetent aortic valve, outlining a radiolucent mass (arrows) lying in the left ventricular outflow tract.
Six days later he developed profound hypotension and
outflow tract and the obstruction relieved. At surgery no
ECG showed anterolateral ST segment elevation and loss thrombus was found. Unfortunately, the patient continof R waves in the precordial and lateral leads consistent with acute anterolateral myocardial infarction. He failed to improve with vasopressors and underwent emergency cardiac catheterization. Angiography of the left coronary artery showed extremely slow flow without evidence of coronary thrombus or obstruction. During coronary injection there was regurgitation of contrast across an incompetent aortic valve which outlined a radiolucent mass, which appeared to be a thrombus, in the left ventricular outflow tract (Fig. 1). This mass was visible throughout the entire cineangiographic run. Because of continued hemodynamic deterioration, after consultation with the cardiothoracic surgeons, the patient was sent for an emergency cardiac embolectomy. During transport to the operating room the patient’s blood pressure, which had been 80/50 torr, rose dramatically to 150/80 torr and his color improved. The clinical impression was that the clot may have become dislodged from the ventricular
ued to deteriorate and did not survive the procedure. At post-mortem examination no thrombus was found within the left ventricular cavity. A 2 x 4 x 0.8 cm thrombus was found within the left atrium near the mitral valve. Autopsy also revealed evidence of acute and chronic rejection and severe diffuse coronary obstructive disease, as well as old anterior infarction with aneurysm formation and acute necrosis of the septum and posterolateral wall. The distal arterial beds were not examined for evidence of emboli. DISCUSSION This patient suffered a catastrophic event as a result of large intracavitary cardiac thrombus. Outflow tract obstruction usually presents with syncope, chest pain, congestive heart failure, or arrhythmias and symptoms can be intermittent, as is often the case with cardiac tumors.
134
Clark and Levine
Obstructive shock is most often seen in the setting of massive pulmonary emboli but has been reported with ventricular outflow tract obstruction [3]. In this case it is most likely that the thrombus migrated from the left atrium to the left ventricular cavity. A remnant of the atrial thrombus was found at post mortem. Although cardiac thrombus formation is common, clinical embolic events occur in only a small percentage of cases, and emboli large enough to compromise cardiac outflow are exceedingly rare [2]. Previous echocardiographic studies in heart transplant recipients have demonstrated atrial thrombus formation in a significant number [5].Diminished left ventricular function as a result of chronic rejection has been associated with increased left atrial spontaneous echo contrast and probably contributed to this patient’s thrombus formation [6]. This patient was not known to have any other predisposing factors for thromboembolic disease. This case also illustrates a number of other important points. First, it demonstrates that endomyocardial biopsy can be an unreliable method for detecting acute rejection. Second, serial cardiac catheterization may fail to identify coronary occlusive disease, particularly if it is of a dif-
fuse nature. Progressive deterioration in left ventricular function, as shown by left ventriculography and echocardiography, was the most important clue in this individual that significant cardiac pathology was present.
REFERENCES I . Heck CF, Shumway SJ, Kaye MP: The registry of the International Society for Heart Transplantation: Sixth official report 1989. J Heart Transplant 8:271-276. 1989. 2. Meltzer RS, Visser CA, Fuster V: lntracardiac thrombi and systemic embolization. Ann Intern Med 104:689-698, 1986. 3. Waller BF, Dean PJ, Mann 0, Rosen JH and Roberts WC: Right ventricular outflow obstruction from thrombus with small peripheral pulmonary emboli. Chest 79:224-225, 1981. 4. Gultekin N , Dogar H. Turkoglu C, Ozturk S, Gokhan N and Demiroglu C: Giant right atrial thrombus causing ventricular inflow and outflow obstruction. Am Heart J 1161367-1369, 1988. 5. Angerman C. Spes C, Stempfle U, et al.: Morphological and functional characteristics pf the transplanted heart: transthoracic versus transesophageal echocardiographic findings. (Abstr) Circulation 80 (Suppl II):474. 1989. 6. Polanco G, Jafn SM, Click CL, Alam M, Levine TB: Transesophageal versus transthoracic echocardiography in the heart transplant population. (Abstr) Clin Res 38(3):884A, 1990.
