International Journal of Cardiology 186 (2015) 10–12
Contents lists available at ScienceDirect
International Journal of Cardiology journal homepage: www.elsevier.com/locate/ijcard
Letter to the Editor
A case of pheochromocytoma with secondary dilated cardiomyopathy, ventricular fibrillation and mural thrombus of left ventricle Chao Feng a, Shu-Dong Xia a,b,⁎ a b
The Department of Cardiology, The Fourth Affiliated Hospital of Zhejiang University, Yiwu, China The Department of Cardiology, The First Affiliated Hospital of Zhejiang University, Hangzhou, China
a r t i c l e
i n f o
Article history: Received 14 February 2015 Accepted 17 March 2015 Available online 18 March 2015 Keywords: Pheochromocytoma Cardiomyopathy Ventricular fibrillation Thrombus
Pheochromocytoma is an important but sometimes untraceable cause of secondary hypertension. The pulsatile secretion of catecholamine not only leads to hypertension, but also leads to a series of cardiac complications including cardiomyopathy and arrhythmia [1–4], which sometimes are the initial manifestations of pheochromocytoma, and also make the diagnosis more intriguing. We describe a 19-year-old male who presented to hospital with tachypnea, palpation and edema in legs for 2 years and aggravated two days ago after a cold. Two years ago the echocardiogram showed a dilated left ventricular with an internal diameter at end-diastole (LVIDd) of 6.15 cm, and a decreased ejection fraction of left ventricular (LVEF) of 35%, then he regularly took digoxin, carvedilol, hydrochlorothiazide and spirolactone. On arrival, the physical examination was notable for an elevated blood pressure of 159/100 mm Hg, heart rate 120 bpm, temperature 37.9 °C, jugular venous distension, crackles of right lower pulmonary lobe, enlargement of cardiac dullness border, and mild edema of both two legs. Laboratory values and examinations were notable for an elevated white cell count of 13.1 ∗ 10^9/L, neutrophil proportion of 74.7%, platelet count of 436 ∗ 10^9/L, sinus tachycardia, enlarged left ventricular with LVIDd of 6.25 cm, decreased LVEF of 25% and diffusive contractive dysfunction for left ventricular on transthoracic echocardiogram, and increased lung markings on chest X-ray. He received the diagnosis of idiopathic dilated cardiomyopathy and probable pulmonary ⁎ Corresponding author at: Department of Cardiology, The Fourth Affiliated Hospital of Zhejiang University, Shangcheng Avenue N1#, Yiwu, China. E-mail address: [email protected] (S.-D. Xia).
http://dx.doi.org/10.1016/j.ijcard.2015.03.206 0167-5273/© 2015 Elsevier Ireland Ltd. All rights reserved.
infection, and was administrated with oral furosemide, spirolactone, digoxin, candesartan and intravenous ceroxime. Day 2, he had a sudden loss of consciousness, when the electrocardiograph monitoring showed a ventricular fibrillation. After cardiopulmonary resuscitation and electric defibrillation he was transferred to ICU. Next day, the echocardiogram detected a stripe of strong echo with the size of 2.2 ∗ 1.25 cm attaching on the endocardium of left ventricular adjacent to the root of anterior papillary muscle, oscillating with the cardiac cycle (Fig. 1-A). Meanwhile his temperature was elevated to 39.8 °C. He was diagnosed as probable infective endocarditis (IE). After venous blood sampled for blood culture which was later reported to be positive for Methicillin Resistant Staphylococcus Epidermidis (MRSE) in one of three samples, intravenous vancomycin and piperacillintazobactam were administrated. Then his temperature gradually dropped to 38 °C. On day 7, the echocardiogram reported a stripe of strong echo with the diameter of 1.16 cm attaching on the inferior wall of left ventricular near the apex, oscillating with the cardiac circle, while the previously detected vegetation disappeared (Fig. 1-B). A chest CT scan was also obtained and showed multiple patchy and cloudy infiltrating shadows in both sides. Day 8, he was transferred back to the Department of Cardiology. Echocardiography and magnetic resonance imaging were performed but found no vegetations. His temperature gradually returned to normal while his blood pressure was still fluctuating between 120/80–150/ 100 mm Hg. An abdominal CT scan was performed, and showed a huge mass in the left adrenal gland region, possibly pheochromocytoma (Fig. 2). Day 31, the mass was resected. Microscopically, the tumor cells demonstrated in acinar pattern, with nuclear atypia, basophilic cytoplasm and large necrotic areas. The pathological diagnosis of the mass was adrenal pheochromocytoma with massive necrosis. Day 36, he was discharged from hospital, with all vital signs returned to normal. After discharge, he had regular evaluation by echocardiogram, which showed that the LVIDd gradually decreased with LVEF gradually elevated. On 30th July 2012, the LVIDd was 5.1 cm, and LVEF was 56%. This was a complex case of pheochromocytoma which was first miss-diagnosed as idiopathic dilated cardiomyopathy and infectious endocarditis. Retrospectively, the presence of pheochromocytoma should be the cause of all the subsequent cardiovascular issues, including the cardiomyopathy, ventricular fibrillation and cardiac vegetation. The favorable outcome of this patient after surgery is an explicit indication to the diagnosis of secondary cardiomyopathy, which
C. Feng, S.-D. Xia / International Journal of Cardiology 186 (2015) 10–12
11
Fig. 1. A: The vegetation attaching on the endocardium of left ventricular adjacent to the root of anterior papillary muscle, marked by the crosses. B: The vegetation attaching on the inferior wall of left ventricular near the apex, marked by the crosses.
mimicked idiopathic dilated cardiomyopathy in this case. The mechanism of the ventricular fibrillation was more intricate. Arrhythmia is one of the cardiac complications of heart failure, also not rare for pheochromocytoma with pulsatile secretion of catecholamine [1]. Besides, the large necrotic areas in the tumor as shown by the pathological exam suggested a probable hemorrhagic necrosis which might lead to a sudden hypotension or shock, followed by ventricular fibrillation. It indicated another path from pheochromocytoma to the ventricular fibrillation. The most controversial issue in this case was the cause of the cardiac vegetation. At the first the presence of the vegetation together with the
fever, our top diagnostic consideration was IE based on the modified Duke criteria. The MRSE reported in one sample of blood culture further supported this diagnosis. However, more features didn't fit with IE but indicated to the diagnosis of thrombus. First, the patient had a fever and elevated white cell count on arrival but had no vegetations; just 1 day after the ventricular fibrillation when he had serious hemodynamic disturbance, the vegetation was first reported. Second, the vegetation had changeable locations and disappeared only after 5 days, with the temperature going down rapidly. Third, the patient had no valvular heart disease or other conditions as the predisposing factors of IE, but had thrombocytosis which is a contributor for thrombosis. Actually thrombosis has been reported as a rare presentation of pheochromocytoma in several cases [2,5]. It was also reported that pheochromocytoma was associated with abnormal platelet aggregation, probably due to the increased catecholamine level which has been doubted to have an influence on hemostasis [6]. On the other hand, the fever and bacteremia were probably caused by pulmonary infection, the diagnosis of which was supported by the crackles and the chest CT scan which showed multiple infiltrating shadows. Finally, the patient was diagnosed as pheochromocytoma, secondary hypertension, secondary dilated cardiomyopathy, ventricular fibrillation, cardiac mural thrombus, heart failure, pulmonary infection, and thrombocytosis. We reported this case because of the varied and typical cardiac complications of pheochromocytoma, and the diagnostic challenges about the secondary cardiomyopathy and cardiac thrombus which respectively mimicked idiopathic dilated cardiomyopathy and infectious endocarditis. As we know, no such case has been reported so far. Conflict of interest
Fig. 2. The huge mass in the left adrenal gland region shown by CT scan.
The authors report no relationships that could be construed as a conflict of interest.
12
C. Feng, S.-D. Xia / International Journal of Cardiology 186 (2015) 10–12
References [1] T. Zelinka, O. Petrak, H. Turkova, et al., High incidence of cardiovascular complications in pheochromocytoma, Horm. Metab. Res. 44 (5) (2012) 379–384. [2] R. Yu, N.N. Nissen, S.I. Bannykh, Cardiac complications as initial manifestation of pheochromocytoma: frequency, outcome, and predictors, Endocr. Pract. 18 (4) (2012) 483–492. [3] A. Prejbisz, J.W. Lenders, G. Eisenhofer, A. Januszewicz, Cardiovascular manifestations of phaeochromocytoma, J. Hypertens. 29 (11) (2011) 2049–2060.
[4] T. Yoshikawa, Takotsubo cardiomyopathy, a new concept of cardiomyopathy: clinical features and pathophysiology, Int. J. Cardiol. 182C (2014) 297–303. [5] R. Hou, A.M. Leathersich, B.T. Ruud, Pheochromocytoma presenting with arterial and intracardiac thrombus in a 47-year-old woman: a case report, J. Med. Case Rep. 5 (2011) 310. [6] A. Squizzato, V.E. Gerdes, W. Ageno, H.R. Buller, The coagulation system in endocrine disorders: a narrative review, Intern. Emerg. Med. 2 (2) (2007) 76–83.
Contents lists available at ScienceDirect
International Journal of Cardiology journal homepage: www.elsevier.com/locate/ijcard
Letter to the Editor
A case of pheochromocytoma with secondary dilated cardiomyopathy, ventricular fibrillation and mural thrombus of left ventricle Chao Feng a, Shu-Dong Xia a,b,⁎ a b
The Department of Cardiology, The Fourth Affiliated Hospital of Zhejiang University, Yiwu, China The Department of Cardiology, The First Affiliated Hospital of Zhejiang University, Hangzhou, China
a r t i c l e
i n f o
Article history: Received 14 February 2015 Accepted 17 March 2015 Available online 18 March 2015 Keywords: Pheochromocytoma Cardiomyopathy Ventricular fibrillation Thrombus
Pheochromocytoma is an important but sometimes untraceable cause of secondary hypertension. The pulsatile secretion of catecholamine not only leads to hypertension, but also leads to a series of cardiac complications including cardiomyopathy and arrhythmia [1–4], which sometimes are the initial manifestations of pheochromocytoma, and also make the diagnosis more intriguing. We describe a 19-year-old male who presented to hospital with tachypnea, palpation and edema in legs for 2 years and aggravated two days ago after a cold. Two years ago the echocardiogram showed a dilated left ventricular with an internal diameter at end-diastole (LVIDd) of 6.15 cm, and a decreased ejection fraction of left ventricular (LVEF) of 35%, then he regularly took digoxin, carvedilol, hydrochlorothiazide and spirolactone. On arrival, the physical examination was notable for an elevated blood pressure of 159/100 mm Hg, heart rate 120 bpm, temperature 37.9 °C, jugular venous distension, crackles of right lower pulmonary lobe, enlargement of cardiac dullness border, and mild edema of both two legs. Laboratory values and examinations were notable for an elevated white cell count of 13.1 ∗ 10^9/L, neutrophil proportion of 74.7%, platelet count of 436 ∗ 10^9/L, sinus tachycardia, enlarged left ventricular with LVIDd of 6.25 cm, decreased LVEF of 25% and diffusive contractive dysfunction for left ventricular on transthoracic echocardiogram, and increased lung markings on chest X-ray. He received the diagnosis of idiopathic dilated cardiomyopathy and probable pulmonary ⁎ Corresponding author at: Department of Cardiology, The Fourth Affiliated Hospital of Zhejiang University, Shangcheng Avenue N1#, Yiwu, China. E-mail address: [email protected] (S.-D. Xia).
http://dx.doi.org/10.1016/j.ijcard.2015.03.206 0167-5273/© 2015 Elsevier Ireland Ltd. All rights reserved.
infection, and was administrated with oral furosemide, spirolactone, digoxin, candesartan and intravenous ceroxime. Day 2, he had a sudden loss of consciousness, when the electrocardiograph monitoring showed a ventricular fibrillation. After cardiopulmonary resuscitation and electric defibrillation he was transferred to ICU. Next day, the echocardiogram detected a stripe of strong echo with the size of 2.2 ∗ 1.25 cm attaching on the endocardium of left ventricular adjacent to the root of anterior papillary muscle, oscillating with the cardiac cycle (Fig. 1-A). Meanwhile his temperature was elevated to 39.8 °C. He was diagnosed as probable infective endocarditis (IE). After venous blood sampled for blood culture which was later reported to be positive for Methicillin Resistant Staphylococcus Epidermidis (MRSE) in one of three samples, intravenous vancomycin and piperacillintazobactam were administrated. Then his temperature gradually dropped to 38 °C. On day 7, the echocardiogram reported a stripe of strong echo with the diameter of 1.16 cm attaching on the inferior wall of left ventricular near the apex, oscillating with the cardiac circle, while the previously detected vegetation disappeared (Fig. 1-B). A chest CT scan was also obtained and showed multiple patchy and cloudy infiltrating shadows in both sides. Day 8, he was transferred back to the Department of Cardiology. Echocardiography and magnetic resonance imaging were performed but found no vegetations. His temperature gradually returned to normal while his blood pressure was still fluctuating between 120/80–150/ 100 mm Hg. An abdominal CT scan was performed, and showed a huge mass in the left adrenal gland region, possibly pheochromocytoma (Fig. 2). Day 31, the mass was resected. Microscopically, the tumor cells demonstrated in acinar pattern, with nuclear atypia, basophilic cytoplasm and large necrotic areas. The pathological diagnosis of the mass was adrenal pheochromocytoma with massive necrosis. Day 36, he was discharged from hospital, with all vital signs returned to normal. After discharge, he had regular evaluation by echocardiogram, which showed that the LVIDd gradually decreased with LVEF gradually elevated. On 30th July 2012, the LVIDd was 5.1 cm, and LVEF was 56%. This was a complex case of pheochromocytoma which was first miss-diagnosed as idiopathic dilated cardiomyopathy and infectious endocarditis. Retrospectively, the presence of pheochromocytoma should be the cause of all the subsequent cardiovascular issues, including the cardiomyopathy, ventricular fibrillation and cardiac vegetation. The favorable outcome of this patient after surgery is an explicit indication to the diagnosis of secondary cardiomyopathy, which
C. Feng, S.-D. Xia / International Journal of Cardiology 186 (2015) 10–12
11
Fig. 1. A: The vegetation attaching on the endocardium of left ventricular adjacent to the root of anterior papillary muscle, marked by the crosses. B: The vegetation attaching on the inferior wall of left ventricular near the apex, marked by the crosses.
mimicked idiopathic dilated cardiomyopathy in this case. The mechanism of the ventricular fibrillation was more intricate. Arrhythmia is one of the cardiac complications of heart failure, also not rare for pheochromocytoma with pulsatile secretion of catecholamine [1]. Besides, the large necrotic areas in the tumor as shown by the pathological exam suggested a probable hemorrhagic necrosis which might lead to a sudden hypotension or shock, followed by ventricular fibrillation. It indicated another path from pheochromocytoma to the ventricular fibrillation. The most controversial issue in this case was the cause of the cardiac vegetation. At the first the presence of the vegetation together with the
fever, our top diagnostic consideration was IE based on the modified Duke criteria. The MRSE reported in one sample of blood culture further supported this diagnosis. However, more features didn't fit with IE but indicated to the diagnosis of thrombus. First, the patient had a fever and elevated white cell count on arrival but had no vegetations; just 1 day after the ventricular fibrillation when he had serious hemodynamic disturbance, the vegetation was first reported. Second, the vegetation had changeable locations and disappeared only after 5 days, with the temperature going down rapidly. Third, the patient had no valvular heart disease or other conditions as the predisposing factors of IE, but had thrombocytosis which is a contributor for thrombosis. Actually thrombosis has been reported as a rare presentation of pheochromocytoma in several cases [2,5]. It was also reported that pheochromocytoma was associated with abnormal platelet aggregation, probably due to the increased catecholamine level which has been doubted to have an influence on hemostasis [6]. On the other hand, the fever and bacteremia were probably caused by pulmonary infection, the diagnosis of which was supported by the crackles and the chest CT scan which showed multiple infiltrating shadows. Finally, the patient was diagnosed as pheochromocytoma, secondary hypertension, secondary dilated cardiomyopathy, ventricular fibrillation, cardiac mural thrombus, heart failure, pulmonary infection, and thrombocytosis. We reported this case because of the varied and typical cardiac complications of pheochromocytoma, and the diagnostic challenges about the secondary cardiomyopathy and cardiac thrombus which respectively mimicked idiopathic dilated cardiomyopathy and infectious endocarditis. As we know, no such case has been reported so far. Conflict of interest
Fig. 2. The huge mass in the left adrenal gland region shown by CT scan.
The authors report no relationships that could be construed as a conflict of interest.
12
C. Feng, S.-D. Xia / International Journal of Cardiology 186 (2015) 10–12
References [1] T. Zelinka, O. Petrak, H. Turkova, et al., High incidence of cardiovascular complications in pheochromocytoma, Horm. Metab. Res. 44 (5) (2012) 379–384. [2] R. Yu, N.N. Nissen, S.I. Bannykh, Cardiac complications as initial manifestation of pheochromocytoma: frequency, outcome, and predictors, Endocr. Pract. 18 (4) (2012) 483–492. [3] A. Prejbisz, J.W. Lenders, G. Eisenhofer, A. Januszewicz, Cardiovascular manifestations of phaeochromocytoma, J. Hypertens. 29 (11) (2011) 2049–2060.
[4] T. Yoshikawa, Takotsubo cardiomyopathy, a new concept of cardiomyopathy: clinical features and pathophysiology, Int. J. Cardiol. 182C (2014) 297–303. [5] R. Hou, A.M. Leathersich, B.T. Ruud, Pheochromocytoma presenting with arterial and intracardiac thrombus in a 47-year-old woman: a case report, J. Med. Case Rep. 5 (2011) 310. [6] A. Squizzato, V.E. Gerdes, W. Ageno, H.R. Buller, The coagulation system in endocrine disorders: a narrative review, Intern. Emerg. Med. 2 (2) (2007) 76–83.
