Pheochromocytoma Cardiomyopathy Research Articles

SAT333 A Case Of Pheochromocytoma Initially Presenting As Takotsubo-like Cardiomyopathy

Abstract Disclosure: J.S. Figueroa: None. S.W. Holland: None. J. Cheng: None. M.C. Sullivan: None. Background: Takotsubo-like cardiomyopathy (TCM) is a known but rarely seen clinical complication associated with pheochromocytoma that is important to recognize. Case Presentation: A 68 year old woman with a history of hypertension on verapamil, lisinopril-hydrochlorothiazide, clonidine, hyperlipidemia, asthma, and chronic kidney disease presents for nausea and vomiting associated with pressure like chest pain. Cardiac work up revealed elevated troponins and T wave inversion in the anterolateral leads concerning for non-ST segment elevation myocardial infarction (NSTEMI). CT Abdomen study revealed two right sided adrenal nodules 2.4 x 1.8 cm and 1.9 x1.1 cm. Coronary angiography was normal but study was concerning for TCM. She developed new onset atrial fibrillation and was treated with calcium channel blocker and beta blocker. Transthoracic echocardiography showed apical hypokinesis and a mildly decreased ejection fraction of 41%. She was discharged home on beta blocker and losartan therapy. The patient presented 5 days later with similar symptoms and a maximum elevated blood pressure 170/110 mm Hg. Labs revealed elevated plasma free metanephrines level of 505 pg/ml (ref range ≤57 pg/ml) with normal renin, aldosterone, and cortisol levels. CT adrenal protocol study showing pre-contrast density of largest nodule 39 Hounsfield units (HU), postcontrast density 81 HU, and washout density 56 HU with washout at 59.5%. The smaller nodule had pre-contrast density of 31 HU, postcontrast of 65 HU, washout density of 61 HU with washout at 11.8%. Endocrine and Endocrine surgery were consulted at this time. Alpha blocker tamsulosin was initiated and beta blocker therapy was discontinued. Patient underwent right adrenalectomy with subsequent improvement of her ejection fraction two months later. Histology was concerning for malignancy for which she is currently being followed. Conclusion: This case highlights TCM as a presenting sign for a pheochromocytoma. A study by Agarwal et al. found that a minority of patients who had TCM due to pheochromocytoma developed symptoms typical of the tumor such as headache, hypertension, palpitations, and diaphoresis. The study also found that complications such as cardiogenic shock and heart failure were higher in pheochromocytoma related TCM.¹ Consider catecholamine producing tumors as a potential cause of TCM where appropriate alpha and beta blockade may help to reduce symptoms. Earlier recognition of a potential pheochromocytoma would also have discouraged the use of a beta blocker initially until pheochromocytoma was ruled out. An individualized, multidisciplinary approach helps lead to a timely diagnosis and appropriate treatment decisions for these rare tumors. References (1) Agarwal, V.,Kant, G.,Hans N., Messerli F.H. Takotsubo-like Cardiomyopathy in Pheochromocytoma. International Journal of Cardiology 2011; Vol 153,3,241-248 Presentation: Saturday, June 17, 2023

Predictive Factors for Catecholamine-Induced Cardiomyopathy in Patients with Pheochromocytoma and Paraganglioma.

ObjectiveTo investigate possible predictive factors of catecholamine-induced cardiomyopathy in pheochromocytoma and paraganglioma (CICMPP) patients.MethodsIn all, 50 CICMPP patients and 152 pheochromocytoma and paraganglioma (PPGL) patients without CICMPP who were treated in our institution between August 2012 and April 2018 were included in this retrospective study to assess predictors of CICMPP.ResultsPatients with CICMPP reported younger onset age, more clinical symptoms and signs, more family history of hypertension, and higher maximum systolic, diastolic, and mean BP and maximum HR. Medical evaluation also showed higher level of blood hematocrit, blood glucose, 24-h urine catecholamines, larger diameter of the tumor and more comorbidities, von Hippel-Lindau syndromes, and metastatic tumors in these patients. Multivariable analysis identified maximum resting HR over 115 beats/min (OR 10.05, 95% CI 3.71–27.20), maximum resting systolic BP over 180 mmHg (OR 7.17, 95% CI 2.22–23.23), blood glucose over 8.0 mmol/L (OR 6.52, 95% CI 2.25–18.86), more than 3 symptoms and signs (OR 6.05, 95% CI 1.86–19.64), and onset age under 40 years (OR 3.74, 95% CI 1.37–10.20) as independent predictors of CICMPP. Female sex (OR 5.06, 95% CI 1.19–21.54), complaint of chest pain (OR 5.84, 95% CI 1.27–26.90), and extra-adrenal tumor (OR 8.64, 95% CI 1.82–40.94) were independent predictors of Takotsubo cardiomyopathy in CICMPP.ConclusionMaximum resting HR ≥115 beats/min, maximum resting systolic BP ≥180 mmHg, blood glucose ≥8.0 mmol/L, number of symptoms and signs ≥3, and onset age ≤40 years were found to be predictive factors for CICMPP.

Inhibitor of Tyrosine Hydroxylase and Catecholamine-induced Cardiomyopathy in Pheochromocytoma

Usual clinical presentation of secreting pheochromocytoma is headache, palpitations, sweating and paroxysmal hypertension but emergency situations are also described. We herein reported a case of pheochromocytoma revealed by cardiogenic shock on dilated cardiomyopathy with improvement of left ventricular ejection fraction (LVEF) after the introduction of an inhibitor of tyrosine hydroxylase. A 41-year-old patient was admitted in the ICU for cardiogenic shock with initially hypertension emergency and tachycardia. Transthoracic echocardiography revealed a dilated cardiomyopathy with estimated 2-dimensional LVEF at 15 %. A computed tomography angiography was performed to rule out a pulmonary embolism and led to discover bilateral adrenal tumors measured at 3 cm and 2 cm respectively on the left and right sides. Plasmatic noradrenaline was increased 5.5 μg/L ( N < 0.60). 123I-metaiodobenzylguanidine scintigraphy, 18F-fluorodeoxyglucose and FDOPA positron emission tomography showed uptake of the 2 lesions. Despite an optimized heart failure treatment including an angiotensin-converting-enzyme inhibitor, a diuretic, an inhibitor of aldosterone and increasing doses of beta-blocker in addition to a calcic-blocker and alpha-blocker for hypertension, a stagnation of LVEF around 20 % was still observed ten weeks after diagnosis, contraindicating the patient for adrenal surgery. The addition of an inhibitor of tyrosine hydroxylase (metyrosine, 1500 mg/day) led to a dramatic decrease of blood metanephrines (0.21 versus 1.42 μg/L before the introduction), even if results must be interpreted with caution regarding possible interference with this different medicine. Moreover, LVEF improved up to 40 % within 22 weeks allowing the bilateral adrenelactomy. Pathology results confirmed the diagnosis of benign bilateral pheochromocytomas. Genetic analysis showed a MAX mutation (c.145T > C, p.S49P). Congestive heart failure has a poor prognosis unless a correctable cause is identified. For the treatment of pheochromocytoma, surgical removal of tumor is the first choice but in particular cases, using an inhibitor of tyrosine hydroxylase could be a short-term alternative in preparation to surgery.

Pheochromocytoma Presenting With Cardiomyopathy in an Otherwise Healthy Young Patient: A Rare Case Report

Background: Pheochromocytomas are rare neuroendocrine tumors. The classic triad of symptoms includes diaphoresis, episodic headache, and tachycardia. Rarely, patients may develop cardiomyopathy associated with pheochromocytoma. It is uncommon for cardiomyopathy to be the initial presenting symptom of pheochromocytoma and can lead to a delayed diagnosis.Clinical Case: The patient is a 30-year old female with no significant medical history who presented with complaints of dyspnea, fever and malaise. The patient had no known prior history of hypertension and did not report complaints of headache, diaphoresis or palpitations. Patient was hypertensive and tachycardic on admission. Upon treatment with intravenous fluids, the patient acutely decompensated and developed respiratory failure requiring emergent intubation. CT of the chest, abdomen and pelvis was performed which showed diffuse bilateral pulmonary edema as well as a circumscribed, heterogeneously enhancing 4.4 cm mass involving the right adrenal gland. An echocardiogram was performed which showed significant left ventricular dysfunction with ejection fraction of 5%. Patient had wide fluctuations in her blood pressure during admission ranging from hypotension to extreme hypertension. She did require the use of epinephrine and milrinone when hypotensive. Due to the use of vasopressors, plasma fractionated metanephrines and 24-hour urine fractionated metanephrines and catecholamines were not recommended to be obtained as these medications may contribute to false positive testing. A dedicated CT of the abdomen with and without contrast was performed to further assess the patient’s right adrenal mass; this revealed a 3.9 x 2.4 x 4.9 cm right adrenal mass with 33% absolute washout and 17% relative washout concerning for pheochromocytoma. The patient underwent an open right adrenalectomy during admission. Surgical pathology results were consistent with a pheochromocytoma measuring 5.2 cm at greatest dimension; the tumor was confined to the adrenal gland and completely excised. Following her surgery, the patient’s clinical status improved significantly over the next several days. She was discharged home in stable condition on hospital day #18. An echocardiogram was repeated 2 months after her hospitalization with ejection fraction improved to 58%.Conclusion: Catecholamine-induced cardiomyopathy in pheochromocytoma is rare, occurring in approximately 8–11% of patients with pheochromocytoma. Cardiomyopathy as the initial presentation of pheochromocytoma is very uncommon. It is important to accurately diagnose pheochromocytoma-related cardiomyopathy as early detection and resection can lead to marked improvement in cardiac function and prevent catastrophic consequences including death.

SAT-313 Catecholamine-Induced Cardiomyopathy in Pheochromocytoma during Pregnancy

Introduction: This case illustrates an uncommon initial presentation of cardiomyopathy in a pregnant woman who was later found to have Pheochromocytoma. Case: A 33-year-old female who is 19 weeks pregnant with no significant past medical or family history presented to the hospital with pleuritic chest pain associated with palpitations and dyspnea. Echocardiogram showed mildly dilated left ventricle with mild global left ventricular (LV) systolic dysfunction and an ejection fraction (EF) of 40-50%. Electrocardiogram (EKG) showed Q waves, with peak troponin of 1.8. Her symptoms were attributed to a post-viral perimyocarditis, was treated symptomatically and discharged home. She presented again at 22 weeks of pregnancy due to the same symptoms. Repeat echocardiogram showed improvement of cardiac function with normal EF and LV function; hence, she was once again treated symptomatically and discharged to home. The rest of her pregnancy was unremarkable. She carried to term and had an uncomplicated vaginal delivery. One week postpartum she presented to the hospital this time with complaints of intermittent headaches, episodic palpitations and new onset hypertension with systolic blood pressure (BP) in the 170s (<130mmHg). Pre pregnancy and BP during pregnancy were within normal limits. Diagnostic testing for secondary causes of hypertension were done which were all negative except for plasma catecholamines: metanephrine 24 nmol/L (<.50), normetanephrine 8.3nmol/L (<.9), epinephrine >1354 pg/ml (<642), and norepinephrine 2669 pg/ml (<642). 24 hour urine results showed: metanephrine 20 600mcg/24hr (<400), normetanephrine 3540 mcg/24hr (<900), total metanephrines 24 140 mcg/24hr (<1300), epinephrine 583 mcg/24hr(<21), norepinephrine 645 mcg/24hr (15-80). CT of the abdomen showed a 7.9x7x8.2 cm right adrenal mass. She underwent a laparoscopic right adrenalectomy and histopathology confirms the diagnosis of Pheochromocytoma. Her symptoms resolved after surgery with BP returning to normal. Repeat plasma catecholamines post surgery also returned to normal limits. Discussion: Pheochromocytoma during pregnancy is an extremely rare event with a reported incidence of .007% with an antepartum diagnosis that is only made 75% of the time (Van der Weerd et al., 2017). It is most often found during the third trimester of pregnancy and patients are more symptomatic during this time due to the compression of the enlarging gravid uterus on the tumor causing catecholamine release. Diagnosis is difficult especially in this case because the patient did not initially present with the classic symptoms until postpartum. Reference: Weerd, K. V., Noord, C. V., Loeve, M., Knapen, M. F., Visser, W., Herder, W. W., Feelders, R. A. (2017). ENDOCRINOLOGY IN PREGNANCY: Pheochromocytoma in pregnancy: Case series and review of literature. European Journal of Endocrinology,177(2). doi:10.1530/eje-16-0920

Catecholamine-Induced Cardiomyopathy in Pheochromocytoma: How to Manage a Rare Complication in a Rare Disease?

Pheochromocytomas and paragangliomas (PHEOs) are rare neuroendocrine tumors. Clinical manifestations include different cardiovascular signs and symptoms, which are related to excessive secretion of catecholamines. Catecholamine-induced cardiomyopathy in PHEO (CICMPP) is a rare but dreaded complication of PHEO. Once patient is diagnosed with this condition, the prognosis is worse and a surgical risk is much higher than expected. This article focuses on how catecholamines affect the heart and the pathophysiologic mechanism of CICMPP. The cardiovascular responses to catecholamine depend mostly on which catecholamine is released as well as the amount of catecholamine that is released. The acute release of norepinephrine and epinephrine from PHEO increases heart rate, systemic vascular resistance, myocardial contractility, and reduces venous compliance. The excessive adrenergic stimulation by catecholamine results in severe vasoconstriction and coronary vasospasm, myocardial ischemia, and subsequently damage, and necrosis. Chronically elevated catecholamine levels lead to significant desensitization of cardiac β-adrenoceptors. The increased levels of the enzyme β-adrenoceptors kinase (βARK) in the heart seems to mediate these biochemical and physiological changes that are consistently correlated with attenuated responsiveness to catecholamine stimulation. Through these mechanisms different types of cardiomyopathy (CMP) can be formed. This review discusses extensively the 3 types of cardiomyopathies that can be present in a PHEO patient. It also provides the clinical presentation and diagnostic and therapeutic algorithm in managing patients with CICMPP.

Catecholamine induced cardiomyopathy in pheochromocytoma

Catecholamine induced cardiomyopathy in the setting of pheochromocytoma is an unusual clinical entity. Earlier studies have reported left ventricular dysfunction in around 10% of subjects with pheochromocytoma.[1] Catecholamine induced vasoconstriction, direct toxic effect of byproducts of catecholamine degradation and direct receptor-mediated mechanisms are thought to contribute to cardiomyopathy in subjects with pheochromocytoma. The presentation remains a diagnostic challenge as patients may already have hypertensive heart disease and acute coronary syndrome on account of uncontrolled secondary hypertension. We report a case of a 42-year-old male, who presented with features of pheochromocytoma induced cardiomyopathy.

Asymmetric blood pressure caused by extremely rapid cyclic fluctuations and reversible cardiomyopathy in pheochromocytoma

Asymmetric blood pressure caused by extremely rapid cyclic fluctuations and reversible cardiomyopathy in pheochromocytoma

Pheochromocytoma Presenting With Myocardial Infarction, Cardiomyopathy, Renal Failure, Pulmonary Hemorrhage, and Cyclic Hypotension: Case Report and Review of Unusual Presentations of Pheochromocytoma

Pheochromocytoma Presenting With Myocardial Infarction, Cardiomyopathy, Renal Failure, Pulmonary Hemorrhage, and Cyclic Hypotension: Case Report and Review of Unusual Presentations of Pheochromocytoma

Captopril improves hypertension and cardiomyopathy in rats with pheochromocytoma.

Hypertension and cardiomyopathy are prominent findings in humans and rats harboring pheochromocytomas, tumors that can secrete enormous quantities of catecholamines. We have previously found that alpha- and beta-adrenergic receptor antagonists may ameliorate the hypertension and cardiomyopathy found in New England Deaconess Hospital rats implanted with pheochromocytoma. The present studies were designed to determine the possible action of the angiotensin converting enzyme inhibitor captopril on these changes in rats harboring pheochromocytomas. Rats were implanted with transplantable pheochromocytomas and treated with captopril dissolved in the drinking water (1 mg/ml) for 4-6 weeks. Systolic blood pressure was monitored by using the tail-cuff technique. In the rats with pheochromocytoma, blood pressure progressively increased to 184 +/- 3 mm Hg after the tumor was implanted. However, in rats with pheochromocytoma treated with captopril in the drinking water before the development of hypertension, blood pressure did not increase (137 +/- 3 mm Hg). In rats with pheochromocytoma with established hypertension, captopril normalized the systolic blood pressure. Plasma norepinephrine was markedly elevated to a similar extent in both groups compared with unimplanted control rats. Plasma renin activities were slightly lower in rats with pheochromocytoma compared with unimplanted control rats. Treatment with captopril of rats with pheochromocytoma did not modify contraction of isolated rings of thoracic aorta exposed in vitro to either phenylephrine or angiotensin II. Treatment with captopril markedly attenuated the cardiomyopathy induced by pheochromocytoma. These results demonstrate that captopril prevents the development of hypertension despite markedly elevated concentrations of catecholamines. In addition, captopril attenuates catecholamine-induced cardiomyopathy in pheochromocytoma.(ABSTRACT TRUNCATED AT 250 WORDS)