Pheochromocytoma With Brown Adipose Tissue Stimulation: A Case Report.

Brown adipose tissue represents about 1% of the adult body mass and decreases with age. Under variable circumstances, this amount changes, for example, with age or environmental conditions. Pathological states with hypersecretion of catecholamines can induce hypertrophy and hyperplasia in mature brown adipocytes. Consequently, this response can have imaging representation as pseudonodules, a pitfall in imaging interpretation, and may be confused with neoplastic involvement. A case of pheochromocytoma with brown fat stimulation and catecholamine cardiomyopathy is presented.

Case report: Catecholamine cardiomyopathy in children with neuroblastoma.

Introduction: Many endocrine diseases, such as neuroblastoma (NB), can be linked with acquired cardiomyopathy and heart failure. Neuroblastoma's cardiovascular manifestations are typically hypertension, electrocardiogram (ECG) changes, and conduction disturbances. Case Presentation: A 5-year-old 8-month-old girl was admitted to the hospital with ventricular hypertrophy and hypertension (HT) and heart failure. She had no previous history of HT. On color doppler echocardiography, the left atrium and left ventricle were enlarged. The left ventricular ejection fraction (EF) was as low as 40%, and the ventricular septum and left ventricular free wall were thickened. The internal diameters of both coronary arteries were widened. Abdominal computed tomography scan (CT) demonstrated an 8.7 cm × 7.1 cm × 9.5 cm tumor behind the left peritoneum. In urine catecholamines analysis, free-norepinephrine (f-NE), free-dopamine (f-DA), free-normetanephrine (f-NMN), free-3-methoxytyramine (f-3MT), vanillylmandelic acid (VMA), and homovanillic acid (HVA) levels were all greater than the normal range for 24 h except free-metanephrine (f-MN) and free-epinephrine (f-E). Based on these findings, we diagnosed her as NB complicated by catecholamine cardiomyopathy manifested by hypertrophic cardiomyopathy (HCM). Oral metoprolol, spironolactone, captopril and amlodipine furosemide, and intravenously injected sodium nitroprusside and phentolamine were employed for treating HT. After the tumor resection, the blood pressure (BP) and urinary catecholamine levels were all restored. After a follow-up of 7 months, echocardiography indicated normalization of ventricular hypertrophy and function. Conclusion: This is a rare report showing catecholamine cardiomyopathy in NB children. Tumor resection leads to a return to normal of the catecholamine cardiomyopathy manifested as HCM.

Cardiogenic Shock With Reverse Takotsubo Pattern Secondary to Pheochromocytoma: A Case Report.

Pheochromocytoma is a rare catecholamine-secreting neuroendocrine tumor arising from chromaffin cells. Acute catecholamine-mediated cardiomyopathy secondary to pheochromocytoma is rare, but life-threatening. We report a case of a 50-year-old man who presented with chest pain with electrocardiography showing ST elevation in V2-4. He was transferred to cardiac catheterization laboratory for coronary angiography immediately. However, the results showed no evidence of coronary artery occlusions and the left ventriculography revealed hypokinesia of basal part with poor left ventricular ejection fraction. Further investigation confirmed pheochromocytoma-related reversible cardiomyopathy.

Case Report: Malignant Pheochromocytoma Without Hypertension Accompanied by Increment of Serum VEGF Level and Catecholamine Cardiomyopathy.

Introduction: Pheochromocytoma is a catecholamine-producing tumor in the adrenal medulla and is often accompanied by hypertension, hyperglycemia, hypermetabolism, headache, and hyperhidrosis, and it is classified as benign and malignant pheochromocytoma. In addition, persistent hypertension is often observed in subjects with malignant pheochromocytoma. Case Presentation: A 52-year-old Japanese male was referred and hospitalized in our institution. He had a health check every year and no abnormalities had been pointed out. In addition, he had no past history of hypertension. In endocrinology markers, noradrenaline level was as high as 7,693 pg/ml, whereas adrenaline level was within normal range. Abdominal contrast-enhanced computed tomography revealed a 50-mm hyper-vascularized tumor with calcification in the right adrenal gland and multiple hyper-vascularized tumors in the liver. In 131I MIBG scintigraphy, there was high accumulation in the right adrenal gland and multiple accumulation in the liver and bone. In echocardiography, left ventricular ejection fraction was as low as 14.3%. In coronary angiography, however, there was no significant stenosis in the coronary arteries. Based on these findings, we finally diagnosed him as malignant pheochromocytoma accompanied by multiple liver and bone metastases and catecholamine cardiomyopathy. However, blood pressure was continuously within normal range without any anti-hypertensive drugs. Right adrenal tumor resection was performed together with left hepatic lobectomy and cholecystectomy. Furthermore, serum levels of vascular endothelial growth factor (VEGF) and parathyroid (PTH)-related protein were very high before the operation but they were markedly reduced after the operation. Conclusions: This is the first report showing the time course of serum VEGF level in a subject with malignant pheochromocytoma, clearly showing that malignant pheochromocytoma actually secreted VEGF. In addition, this case report clearly shows that we should bear in mind once again that malignant pheochromocytoma is not necessarily accompanied by hypertension.

Recurrent pheochromocytoma with catecholamine cardiomyopathy and left ventricular thrombus: a case report.

Pheochromocytoma is a rare and usually benign tumor of the adrenal glands. We report a case of a 40-year-old woman with recurrent pheochromocytoma and catecholamine cardiomyopathy. She had no history of other types of tumors or connective tissue disease. She had already undergone surgery twice to remove the pheochromocytoma, which had now recurred for the second time. A thrombus in the left ventricle was also noted upon imaging examination, which dissipated after anticoagulation therapy using dabigatran, allowing the patient to opt for an elective third surgery. This paper describes the clinical outcome of using the anticoagulant dabigatran to treat left ventricular thrombosis in this rare case of recurrent pheochromocytoma, and thus further contributing to the knowledge of the clinical management of this rare and complicated disease.

Giant Paraganglioma Complicated With Catecholamine Crisis and Catecholamine Cardiomyopathy: A Case Report and Review of the Literature.

Background: Pheochromocytomas and paragangliomas (PPGL) are rare neuroendocrine tumors which overproduce catecholamines. Heart failure and myocardial infarction caused by paraganglioma complicated with catecholamine crisis are the most common causes of death in PPGL patients before surgery. When giant paraganglioma is complicated with catecholamine crisis, treatment brooks no delay. Case Summary: A 49-year-old man had episodic sweating, tachycardia with irregular pulse, and headaches 5 days before, and then showed up with chest pain and wheezing for 1 day. Meanwhile, he developed symptoms of recurrent severe abdominal pain and loss of consciousness, and his blood pressure was severely unstable (from 70/40 to 300/200 mmHg). First, the electrocardiogram showed ventricular tachycardia, and then we noticed the waves of ST-segment elevation, but we did not find significant abnormalities in coronary angiography. Abdominal CT and MRI revealed a giant lesion with bleeding or infection in the retroperitoneal adrenal area. These imaging findings were confirmed during surgery, and there was vascular adhesion between the retroperitoneal tumor and the inferior vena cava and left and right renal vein. After the successful resection of the tumor, postoperative pathology confirmed paraganglioma, and the patient pulled through and was discharged quickly. Discussion: This is a rare case of giant paraganglioma complicated with catecholamine crisis and catecholamine cardiomyopathy. We can diagnosis this disease greatly by elevated norepinephrine, and it is a gold biochemical standard at present. Standard treatment is surgical resection, which is effective in treating this rare neuroendocrine tumor.

Stress kardiomyopathy triggered by unusual situation.

Stress - “takotsubo” cardiomyopathy - is a reversible mimicking acute myocardial infarction. The trigger is extreme mental or physical stress. The main diagnostic examination is ventriculography with typical left ventricle apical ballooning wall motion abnormality. We present a case report of 63 years old woman, hospitalized at the Department of IV. internal Clinic Medical Faculty and University Hospital Bratislava due to angina. The main trigger was atypical stress situation - urgent need to urinate. On admission, the patients condition was dominated by the hypertension emergency, tachycardia and psychic tension. The ECG on admission revealed the sinus tachycardia and only marked ST elevation in leads I, II, V3-V6. The negative T wave in the leads I, II, V1-V6 was documented on latter ECG. Following the dynamics of troponin levels we assumed the non-STEMI, but due to psychic stress we also considered stress cardiomyopathy. Our patient underwent the coronary angiography and only marginal changes were present. The catecholamine cardiomyopathy with left ventricular apical wall motion abnormality, mild reduction of ejection fraction (48-50 %) and 1st degree of diastolic dysfunction was proved by ventriculography and echocardiography. After the 2 months follow-up, echocardiography confirmed the physiologic finding. This case report points out to the atypical urgent situation that provoked the stress cardiomyopathy.

Cardiac arrest and catecholamine cardiomyopathy secondary to a misdiagnosed ectopic pheochromocytoma.

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Coronary artery myointimal dysplasia in patients with pheochromocytoma-possible causal relationship: pathophysiology and clinical implication with reference to Takotsubo cardiomyopathy and spontaneous coronary dissection.

Myocardial damage in catecholamine cardiomyopathy, characterized by patchy myocyte necrosis commonly with contraction band appearances, interstitial fibrosis, and varying degrees of inflammatory infiltrates, has been well documented. However, coronary vascular pathology has not been recognized. Autopsy of a 43-year-old housewife who died of acute apical anteroseptal myocardial infarction revealed the incidental finding of a left adrenal pheochromocytoma. The epicardial and intramyocardial median- and small-sized coronary arteries exhibited myointimal dysplasia in the form of fibroblastic proliferation in the intima and media, resulting in thickened dysplastic vessels with marked luminal narrowing, occasionally leading to near-total occlusion. The distal left anterior descending artery showed features of recanalization after prior embolic occlusion. The density and severity of vascular involvement revealed a decreasing gradient from apical to basal region, mainly affecting the left ventricle, but the proximal coronary arteries were minimally affected. Myointimal dysplasia was not seen in control cases of hypertensive heart, and despite its presence in hearts with hypertrophic cardiomyopathy, it lacked the distinctive pattern of distribution and the epicardial vessels are uninvolved. Myointimal dysplasia probably results from reactive fibroplasia in response to the vasoconstrictive effect of circulating or local neurosecretory catecholamine and appears crucial in the pathogenesis of various types of catecholamine cardiomyopathy, including Takotsubo or stress cardiomyopathy. Together with the direct catecholamine cardiotoxicity, they result in diffuse microscopic ischemic necrosis and fibrosis. Depending on the type of catecholamine overproduction and action, together with the characteristic anatomic distribution and density of the various types of adrenergic receptors in the ventricles, different regions of the heart are variously affected so that different patterns of ventricular dysfunction are produced, with the subsequent angiographic appearances ranging from apical through midventricular to basal ballooning. Additional complications from the myointimal dysplasia include spontaneous coronary dissection, acute myocardial infarction, and superimposed thrombus formation in the dysplastic vessels and dyskinetic ventricle, with the risk of further damage from embolic events.

Prevalence and patterns of left ventricular dysfunction in patients with pheochromocytoma.

BACKGROUND: Excessive catecholamine release in pheochromocytoma is known to cause transient reversible left ventricular (LV) dysfunction, such as in the case of pheochromocytoma-associated catecholamine cardiomyopathy. We investigated patterns of clinical presentation and incidence of LV dysfunction in patients with pheochromocytoma. METHODS: From January 2004 to April 2011, consecutive patients with pheochromocytoma were retrospectively studied with clinical symptoms, serum catecholamine profiles, and radiologic findings. Patterns of electrocardiography and echocardiography were also analyzed. RESULTS: During the study period, a total of 36 patients (21 males, 49.8 ± 15.8 years, range 14-81 years) with pheochromocytoma were included. In the electrocardiographic examinations, normal findings were the most common findings (19, 52.8%). LV hypertrophy in 12 cases (33.3%), sinus tachycardia in 3 (8.3%), ischemic pattern in 1 (2.8%) and supraventricular tachycardia in 1 (2.8%). Echocardiographic exam was done in 29 patients (80.6%). Eighteen patients (62.1%) showed normal finding, 8 (27.6%) revealed concentric LV hypertrophy with normal LV systolic function, and 3 (10.3%) demonstrate LV systolic dysfunction (LV ejection fraction < 50%). Three showed transient LV dysfunction (2 with inverted Takotsubo-type cardiomyopathy and 1 with a diffuse hypokinesia pattern). Common presenting symptoms in the 3 cases were new onset chest discomfort and dyspnea which were not common in the other patients. Their echocardiographic abnormalities were normalized with conventional treatment within 3 days. CONCLUSION: Out of total 36 patients with pheochromocytoma, 3 showed transient LV systolic dysfunction (catecholamine cardiomyopathy). Pheochromocytoma should be included as one of possible causes of transient LV systolic dysfunction.

Prevalence and Patterns of Left Ventricular Dysfunction in Patients with Pheochromocytoma

BACKGROUND: Excessive catecholamine release in pheochromocytoma is known to cause transient reversible left ventricular (LV) dysfunction, such as in the case of pheochromocytoma-associated catecholamine cardiomyopathy. We investigated patterns of clinical presentation and incidence of LV dysfunction in patients with pheochromocytoma. METHODS: From January 2004 to April 2011, consecutive patients with pheochromocytoma were retrospectively studied with clinical symptoms, serum catecholamine profiles, and radiologic findings. Patterns of electrocardiography and echocardiography were also analyzed. RESULTS: During the study period, a total of 36 patients (21 males, 49.8 +/- 15.8 years, range 14-81 years) with pheochromocytoma were included. In the electrocardiographic examinations, normal findings were the most common findings (19, 52.8%). LV hypertrophy in 12 cases (33.3%), sinus tachycardia in 3 (8.3%), ischemic pattern in 1 (2.8%) and supraventricular tachycardia in 1 (2.8%). Echocardiographic exam was done in 29 patients (80.6%). Eighteen patients (62.1%) showed normal finding, 8 (27.6%) revealed concentric LV hypertrophy with normal LV systolic function, and 3 (10.3%) demonstrate LV systolic dysfunction (LV ejection fraction < 50%). Three showed transient LV dysfunction (2 with inverted Takotsubo-type cardiomyopathy and 1 with a diffuse hypokinesia pattern). Common presenting symptoms in the 3 cases were new onset chest discomfort and dyspnea which were not common in the other patients. Their echocardiographic abnormalities were normalized with conventional treatment within 3 days. CONCLUSION: Out of total 36 patients with pheochromocytoma, 3 showed transient LV systolic dysfunction (catecholamine cardiomyopathy). Pheochromocytoma should be included as one of possible causes of transient LV systolic dysfunction.