Catecholamine-induced Cardiomyopathy Research Articles

Histologic characterization of spontaneous catecholamine-induced cardiomyopathy in laboratory New Zealand White rabbits.

Catecholamine-induced cardiomyopathy (CCM) is an entity associated with increased levels of catecholamines causing subendocardial and papillary muscle cardiomyocyte degeneration and necrosis. In 2020, 49 autopsies from early rabbit deaths in a colony used for medical device biocompatibility studies were submitted for microscopic examination. Of the 49 rabbits, 26 had histologic changes consistent with CCM. No common stressor for CCM was determined in affected rabbits. Animals were generally male, were 12-16-wk-old, and were found dead or had bloating, lethargy, and/or diarrhea. Those observed with clinical signs were euthanized and autopsied per the organization's standard operating procedures. Heart lesions consisted of various degrees of apical subendocardial myocardial degeneration and necrosis. Common non-cardiac lesions included pulmonary congestion and edema, hepatic congestion and centrilobular hepatocellular degeneration, and/or variable intestinal submucosal edema.

P-06 Catecholamine-induced cardiomyopathy: A rare presentiation of pheochromocytoma

Abstract Introduction Pheochromocytoma and paraganglioma (PPGL) are rare neuroendocrine tumors. Clinical manifestations consist of various cardiovascular signs and symptoms related to excessive secretion of catecholamines. Catecholamine-induced cardiomyopathy is a rare but life-threatening complication of PPGL. Excessive discharge of catecholamines results in vasoconstriction, coronary vasospasm and myocardial ischemia. Three types of cardiomyopathies can be observed in patients with PPGL; dilated cardiomyopathy, Takotsuba cardiomyopathy and hypertrophic cardiomyopathy. Clinical Case Herein, we present a case with PPGL complicated with heart failure probably due to excessive secretion of catecholamines. A 19 years-old man was admitted to emergency department with dyspnea and hypotension. He exhibited severe signs of heart failure with very high liver function tests; ALT: 3647 U/L (8-65) and AST: 4542U/L (7-40), probably due to hepatic congestion. Echocardiogram performed in ICU reported %10 left venticular ejection fraction with diffuse akinesia. Remarkable improvement of liver function was recorded following the introduction of heart failure medicines. A computed tomography scan that was performed for abdominal pain and distension revealed a six cm tumour at the left adrenal gland compatible with pheochromocytoma. Laboratory analyses were; 24-hour urine metanephrines: 1938mcg (44-261), normetanephrines: 17934mcg (103-390). Following necessary preoperative preparations, laparoscopic left adrenalectomy was performed and a 70 mm adrenal tumor, histopathologically compatible with pheochromocytoma, was excised. His postoperative laboratory findings were; 24-hour urine metanephrines: 248 mcg (44-261), normetanephrines: 4368 mcg (103-390), chromogranin A: 554.9 ng/ml (0-100), 3-methoxytyramine: 0.34 nmol/l (0-0.17). A 68-Ga-DOTA-Peptide PET/CT following 12 weeks of surgery demonstrated hypermetabolic lesions at left suprarenal area. A second open surgery was performed and lymph node metastases were detected (Figure 1). His cardiac functions ameliorated significantly following the removal of tumors. Genetic analysis for PPGL syndromes were negative for KIF1B, SDHB, NTRK1, TMEME127, VHL, RET, SDHD genes. Work-up is still pending for the mutations of SDHA, SDHC and SDHAF2 genes. Conclusion Cathecolamine-induced cardiomyopathy is a rare entity associated with PPGL. Urgent recognition of related clinical signs and symptoms and detection of PPGL is lifesaving. It should be kept in mind in patients presenting with symptoms of heart failure, hypotension and multisystem crisis in the absence of coronary or valvular heart disease.Figure 1.Pheochromocytoma with lymph node metastasis. (H&E x100)

Case series of catecholamine-induced Takotsubo cardiomyopathy secondary to pheochromocytoma and discussion of relevant literature

Case series of catecholamine-induced Takotsubo cardiomyopathy secondary to pheochromocytoma and discussion of relevant literature

FRI194 Pheochromocytoma Presenting As Catecholamine-Induced Cardiomyopathy: A Case Report

Abstract Disclosure: M. Tsikala Vafea: None. D. Lee: None. Introduction: Pheochromocytoma (PCC) is a rare catecholamine-secreting neuroendocrine tumor presenting with the classic triad of episodic headache, sweating and tachycardia. Catecholamine-induced cardiomyopathy (CICMP) can occur as a complication of PCC in 7-11% of patients. It presents more commonly as dilated cardiomyopathy and inverted Takotsubo cardiomyopathy but also as hypertrophic or unspecified cardiomyopathy. Following appropriate pharmacologic treatment and resection of PCC, these cardiac complications usually resolve. Case report: 36 year old man with history of hypertension presented to the hospital with worsening orthopnea and cough. Following antibiotic treatment failure for suspected pneumonia, he was re-admitted with hemoptysis, tachycardia, hypoxia, and hypertensive emergency. Troponin and brain natriuretic peptide were found to be elevated. CT chest and abdomen showed pulmonary nodules and a large left adrenal mass measuring 9.6x9.3x9.4 cm, concerning for metastatic PCC. He was subsequently started on doxazosin. Transthoracic echo showed bi-atrial enlargement, diffuse severe left ventricle hypokinesis, reduced ejection fraction 25% and diastolic dysfunction. Stress cardiac MRI showed non-ischemic cardiomyopathy, corroborating the diagnosis of CICMP. After achieving adequate alpha-adrenergic blockade, he was started on goal-direct medical therapy consisting of valsartan, metoprolol and empagliflozin. His pulmonary nodular opacities improved on repeat imaging, congruent with pulmonary edema rather than metastases. Plasma and 24-hour urine metanephrines (2,964 pg/mL and 23,310 mcg/24 hr, respectively) and normetanephrines (9,266 pg/mL and 33,927 mcg/24 hr, respectively), as well as dopamine (493 pg/mL), epinephrine (3,205 pg/mL) and norepinephrine (above reportable range of 4,800 pg/mL) eventually came back significantly elevated, confirming his diagnosis of PCC. A month after presentation, he underwent left adrenalectomy, which revealed an 11 cm composite PCC ganglioneuroma. One-month postoperatively, urine metanephrines and normetanephrines were normal but his ejection fraction remained unchanged. Discussion: CICMP should be considered in patients presenting with acute heart failure and an incidental adrenal mass to avoid delays in treatment. Acute pulmonary edema may resemble pulmonary nodules on CT, so interpretation of these findings should correlate with the clinical presentation. Timely initiation of alpha-adrenergic blockade upon suspicion of PCC is crucial as it can significantly improve hemodynamics, and biochemical markers take time to result. 96% of CICMP cases improve after resection of PCC within a variable time frame ranging from one week to several months. Our patient’s CICMP had not improved one month after his adrenalectomy, but it remains to be seen whether he eventually recovers his cardiac function. Presentation: Friday, June 16, 2023

FRI227 Functional Transformation Of An Initially Non-functional Paraganglioma In A Patient With Cyanotic Congenital Heart Disease

Abstract Disclosure: M. McConnell: None. M. Kanin: None. M.S. Auerbach: None. R. Yu: None. Background: Most pheochromocytomas and about half of paragangliomas (P-PGLs) are functional in that they produce catecholamines. Transformation of non-functional to functional P-PGLs is exceedingly rare. We present a case of such transformation in a young male with cyanotic congenital heart disease (CCHD), resulting in catecholamine-induced cardiomyopathy. Clinical Case: A 27-year-old male with CCHD presented to the hospital with tachycardia, hypertension, and hypoxia. He had a newly depressed ejection fraction of <20% and was in florid heart failure. Despite intervention, he decompensated rapidly, requiring extracorporeal membrane oxygenation (ECMO). Five years prior to this presentation, he had had normal 24-hour urine fractionated metanephrines, vanillylmandelic acid (VMA), and homovanillic acid (HVA) levels; on imaging, he had been incidentally found to harbor a 3.3 x 1.6 x 2.5-cm nodule in the upper aortocaval space which could represent a paraganglioma. Subsequent imaging showed slow growth of this mass. Given this pertinent history, urine and plasma free metanephrine studies were repeated during the hospitalization while he was not on vasopressors. 24-hour urine normetanephrines were elevated at 2,644 mcg/d (81-667 mcg/d), metanephrines at 405 mcg/d (55-320 mcg/d) while plasma free metanephrines were elevated at 0.92 nmol/L (0-0.49 nmol/L) and normetanephrines at 11.85 nmol/L (0-0.89 nmol/L). DOTATATE PET/CT revealed a soft tissue mass measuring 4.3 x 3.1 x 4.9 cm with intense DOTATATE activity in the right paravertebral space between the aorta and cava. He was treated with alpha-adrenergic blockade with improvement in blood pressure. Two months later, he underwent resection of the mass with pathology confirming a 4.9 cm paraganglioma. Conclusion: It is very rare for a paraganglioma to undergo transformation from non-functional to functional status although it is reported in the literature. This scenario is supported by our patient’s normal 24-hour urine metanephrines, VMA, and HVA results on initial presentation followed by marked elevation in urine and plasma metanephrines 5 years later when he presented with heart failure. The functional transformation in this patient was accompanied by significant growth of the paraganglioma. We surmise that alterations in gene expression during tumor growth may be responsible for the functional transformation. It is crucial to reassess the functionality of a paraganglioma if the clinical condition suggests catecholamine excess. Presentation: Friday, June 16, 2023

Phaeochromocytoma associated with cardiomyopathy and leukocytoclastic vasculitis in a dog

Phaeochromocytomas are rare tumours of the adrenal medulla that can be associated with various presentations. Many of the better characterized clinical signs, including weakness, tachycardia and tachypnoea, are attributable to excessive and unregulated catecholamine secretion from functional tumours. In addition to catecholamine-induced cardiomyopathy and vasospasm, the invasive nature of phaeochromocytomas can lead to occlusion of the caudal vena cava contributing to systemic cardiovascular compromise. In humans, leukocytoclastic vasculitis is a rarely reported manifestation of catecholamine excess associated with phaeochromocytomas. We now describe a dog that had an invasive unilateral phaeochromocytoma with histological evidence of myocardial damage, consistent with catecholamine-induced cardiomyopathy, and leukocytoclastic vasculitis of small vessels in a range of tissues. We conclude that catecholamine excess may have played a role in the pathogenesis of vasculitis in this case. To the best of our knowledge, this is the first documented association between phaeochromocytoma and leukocytoclastic vasculitis in a non-human species.

Myocardial infarction as a mask for catecholamine-induced cardiomyopathy against the background of pheochromocytoma

Myocardial infarction as a mask for catecholamine-induced cardiomyopathy against the background of pheochromocytoma

Two cases of cardiomyopathy associated with phaeochromocytoma successfully managed with veno-arterial extracorporeal membrane oxygenation (V-A ECMO).

Phaeochromocytoma, a rare neuroendocrine tumour of chromaffin cell origin, is characterised by catecholamine excess. Clinical presentation ranges from asymptomatic disease to life-threatening multiorgan dysfunction. Catecholamine-induced cardiomyopathy is a dreaded complication with high lethality. While there is lack of evidence-based guidelines for use of veno-arterial extracorporeal membrane oxygenation (V-A ECMO) in the management of this condition, limited to case reports and small case series, V-A ECMO has been reported as 'bridge to recovery' therapy, providing circulatory support in the initial period of stabilisation prior to surgery. We report on two patients presenting with catecholamine-induced cardiomyopathy and circulatory collapse who were successfully treated with V-A ECMO for 5 and 6 days, respectively, providing initial haemodynamic support. After stabilisation and introduction of alpha-blockade, both cases had favourable outcomes, with successful laparoscopic adrenalectomies on days 62 and 83 of admission, respectively. Our case reports provide further support for the use of V-A ECMO in the treatment of such gravely ill patients. Phaeochromocytoma should be considered in the diagnosis of patients presenting with acute cardiomyopathy. Management of catecholamine-induced cardiomyopathy is complex and requires multidisciplinary specialist input. Pre-operative management of phaeochromocytoma involves alpha-blockade; however, haemodynamic instability in the setting of cardiogenic shock can preclude alpha-blockade use. Veno-arterial extracorporeal membrane oxygenation is a life-saving intervention which may be considered in cases of acute catecholamine-induced cardiomyopathy and cardiogenic shock in order to provide the required haemodynamic support in the initial phase of treatment, enabling the administration of traditional pharmacological agents, including alpha-blockade.

Abstract 13939: Catecholamine-Induced Cardiomyopathy and Limb Ischemia in Pheochromocytoma

Case Presentation: A 40-year-old woman who was 4 weeks pregnant presented with nausea and vomiting. Blood pressure (BP), heart rate, and temperature were 180/104 mmHg, 85 beats/min, 36.1℃ (96.98 ℉), Hypoxemia and mild pulmonary edema were observed. Echocardiography showed severe left ventricular dysfunction and normal dimension with ejection fraction < 10 %. As BP was decreased despite inotropic agents with aggravated hypoxemia, deteriorated acute kidney injury, mechanical ventilation, venous arterial extracorporeal membrane oxygenation (VA-ECMO), and continuous renal replacement therapy were initiated. Coronary angiography revealed normal coronary arteries and the possibility of myocarditis was low because endomyocardial biopsy showed rare inflammatory infiltration. After a few days, other parameters including cardiac function were improved and VA-ECMO was stopped, however, ischemic signs of the right lower leg progressed. To rule out rhabdomyolysis and compartment syndrome, CT angiography was done and about 4.9 cm sized left adrenal mass was found (A). The results of catecholamines are as follows: plasma metanephrine, 1862.6 (< 98.6 pg/mL); normetanephrine, 993.8 (< 164.9 pq/mL); 24hr urine metanephrine, 1396.2 (36~229 mcg/24hr); normetanephrine, 835.8 (95~650 mcg/24hr). A diagnosis of pheochromocytoma was made and left adrenalectomy was done, Pheochromocytoma was demonstrated in biopsy. Unfortunately, due to severe vasoconstriction and rhabdomyolysis, her right leg was not completely recovered, so amputation was done below knee (B). Discussion: Pheochromocytomas are catecholamine secreting tumors derived from chromaffin cells in the adrenal medulla. Catecholamine-induced vasoconstriction, microvascular hypoxia, muscle ischemia, direct toxic effects are considered to contribute to cardiomyopathy and limb ischemia in rare situations. Timely clinical suspicion, prompt diagnosis, and therapeutic intervention are critical.

PSAT021 Hemorrhagic Pheochromocytoma Presenting With Cardiogenic Shock: Challenges in Diagnosis and Treatment.

Abstract Introduction Pheochromocytomas are rare catecholamine producing tumors with heterogenous clinical presentations. Although acute catecholamine-induced cardiomyopathy is well described in the literature, it is rarely suspected as a cause of cardiogenic shock. Timely diagnosis is challenging and appropriate preoperative treatment is crucial for successful management, as this condition carries a high mortality rate. We describe a case of hemorrhagic pheochromocytoma presenting with refractory hypotension and discuss the difficulties in diagnosis and treatment we encountered. Case Presentation A 57-year-old man presented with pallor, severe headache, and hypotension. After administration of intravenous fluids, the patient developed flash pulmonary edema and was found to have acute systolic heart failure. He was taken for emergent left heart catheterization, which did not show any evidence of coronary obstruction. Therefore, he was treated for presumed myocarditis and received stress dose steroids. His condition quickly deteriorated to cardiogenic shock and was further complicated by cardiac arrest. He required extracorporeal membrane oxygenation (ECMO) treatment for 2 days, during which time his systolic blood pressures were extremely labile (47–270 mmHg). CT angiography incidentally noted a 4.3 cm right para-adrenal lesion, at which time urine metanephrines were ordered and noted to be markedly high. Ga-68 DOTATATE scan was negative, however, dedicated abdominal CT revealed a likely hemorrhagic right adrenal mass. With supportive care, the patient's cardiac function recovered by the fifth hospital day, and labetalol was slowly introduced, followed by doxazosin. Sixteen days after the initial presentation, laparoscopic right adrenalectomy was successfully performed. Pathology confirmed pheochromocytoma with few positive cells in a hemorrhagic and necrotic adrenal lesion. Discussion Our case underlines several important points in the diagnosis and management of pheochromocytoma. First, it is crucial to have a high level of suspicion for a catecholamine-secreting tumor in the setting of labile blood pressures and acute cardiomyopathy, especially in cases without evidence of coronary artery or valvular disease. Notably, steroids should be used cautiously, as they may further precipitate catecholamine crisis and have been associated with hemorrhagic pheochromocytomas. While catecholamine-induced cardiomyopathy is associated with a high mortality rate, the majority of cases are reversible with timely management. Treatment is complex, as standard initial pheochromocytoma management with alpha-blockers and hydration is often precluded due to cardiogenic shock, and vasopressors such as norepinephrine and epinephrine can worsen cardiomyopathy. Furthermore, early initiation of mechanical circulatory support (specifically ECMO) was a life-saving modality, as it facilitated cardiac function recovery and enabled bridging with alpha blockers. Better prognosis is associated with surgery performed after improvement of cardiomyopathy and alpha blockade. And finally, a multidisciplinary approach is crucial for the successful treatment of pheochromocytoma in emergency situations. Given this rare presentation of pheochromocytoma more, data are needed to define an optimal strategy. Presentation: Saturday, June 11, 2022 1:00 p.m. - 3:00 p.m.

Complications of phaeochromocytoma: a case of catecholamine-induced cardiomyopathy

Searchable abstracts of presentations at key conferences in endocrinology ISSN 1470-3947 (print) | ISSN 1479-6848 (online)

Genetic biomarkers of life-threatening pheochromocytoma-induced cardiomyopathy.

The release of excessive amounts of catecholamine by pheochromocytoma-paragangliomas (PPGL) can lead to life-threatening catecholamine-induced cardiomyopathy (CIC). Single-nucleotide polymorphisms of the beta1 and alpha-2c adrenergic receptors alter myocyte receptor function and enhanced norepinephrine release. We tested the hypothesis that such genetic variations may impact the risk of developing CIC in the context of PPGL. Thirty-one patients with PPGL, including nine with a history of CIC, were analyzed for alpha-2-adrenergic receptors: ADRA2C, beta-1 and beta-2 adrenergic receptors: ADRB1 and ADRB2 genotyping. CIC was defined either by a history of heart failure or cardiogenic shock associated with dilated or Takotsubo cardiomyopathy. Subjects were genotyped for ADRA2C (rs61767072 for del322_325), ADRB1 (rs1801252 for Ser49Gly and rs1801253 for Arg389Gly) and ADRB2 (rs1042713 for Arg16Gly and rs1042714 for Gln27Glu). Single-locus analysis revealed that variant in ADRA2C (alpha 2CDel322-325) was more common among patients with CIC than among controls (allele frequency, 0.44 vs 0.05; P< 0.001). The lack of alpha 2CDel322-325 polymorphism has a negative predictive value of 95% for the onset of CIC. In a replication cohort including 26 patients with PPGL whom eight have developed a CIC, the association between Alpha 2CDel322-325 and CIC was confirmed (allele frequency, 0.33 vs 0.; P= 0.0001). In conclusion, Alpha 2CDel322-325 through the identification of patients at low risk of developing CIC can help physicians to better determine the most appropriate therapeutic approach, notably in patients at high risk of surgical complications.

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.

Reversible Catecholamine Induced Cardiomyopathy

Background: Pheochromocytomas and paragangliomas (PPGL) are rare neuro-endocrine tumors associated with a myriad of poor outcomes as a result of long-term exposure to catecholamines. Although paragangliomas are less commonly associated with increased catecholamine production than adrenal pheochromocytomas, there have been a few reports of catecholamine-induced cardiomyopathy in patients diagnosed with PPGL. We report a case of a PPGL associated with hypercoagulability and cardiomyopathy. Clinical Case: 42-year-old man with uncontrolled hypertension presented to the emergency department with abdominal pain. On CT imaging, he was found to have hepatic lesions, aortocaval lymph node concerning for metastatic disease, left renal infarct, and a left ventricular thrombus. Soon after his admission, he developed acute ataxia, gaze palsies and left hemiparalysis. CTA of the head showed a basilar artery thrombus [FJ1] which was treated with emergent thrombectomy. In addition patient had absent distal pulse of the right foot[FJ2], and found to have thrombus of the popliteal artery, which was treated with thrombectomy. Further workup with abdominal MRI showed retroperitoneal mass[FJ3] and multiple hepatic lesions concerning for metastatic extra-adrenal neuroendocrine tumor. Plasma normetanephrine was 4.5 nmol/L (ULN 0.89), plasma metanephrine 0.3 nmol/L (ULN 0.49) Chromogranin A was 387 ng/ml (ULN 160). Ga-68 DOTATE scan was consistent with an extra adrenal paraganglioma with less prominent radiotracer activity in hepatic lesion concerning for dedifferentiated metastatic disease. In addition, echocardiogram showed reduced LV ejection fraction of 24% with global hypokinesis, and confirmed the LV thrombus. Cardiac MRI showed infiltrative nonischemic cardiomyopathy and mild dilation of left ventricle, as well as patchy delayed enhancement in the basal and inferoseptal walls suggestive of myocarditis. Treatment included rivaroxaban[FJ4], lisinopril, doxazosin, furosemide, and carvedilol. Several months after discharge, his EF improved to 48%. Hepatic lesions concerning for dediffertiated tumor vs unrelated malignancy was biopsied[FJ5] and consistent with neuroendocrine tumor. Future plan for his PPGL include revaluation for resection of retroperitoneal mass or DOTA Lutathera therapy. Conclusions: This case highlights a young man who was incidentally found to have metastatic paraganglioma with catecholamine-induced cardiomyopathy. The patient was asymptomatic until he developed significant heart failure. Cardiomyopathy in this setting is thought to be secondary to uncontrolled hypertension, as well as sympathetic overdrive from overstimulation of norepinephrine. We present the case to highlight the management challenges in a patient with PPGL with significant cardiovascular compromise and limited therapeutic options.

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.

Acute coronary syndrome: Uncommon presentation of multiple endocrine neoplasia.

IntroductionMyocardial infarction is usually due to thrombotic occlusion of a coronary vessel caused by rupture of a vulnerable atherosclerosis plaque. There is also the acute myocardial infarction with no evidence of relevant stenosis of the coronary artery, known as myocardial infarction with non-obstructive coronary arteries (MINOCA) such as Takotsubo, myocarditis and catecholamine induced cardiomyopathy. Pheochromocytoma is one of the causes of MINOCA. This association is rare but it may delay diagnosis and must be known in order to provide the best chance at early detection.This work has been reported in the line with the SCARE criteria.Presentation of the caseWe report a case of a 49 year-old man, admitted to our department for a recurrence of myocardial infarction with angiographically normal coronary arteries. During his hospitalization the patient complained of intestinal haemorrhage. The abdominal Computed tomographic scan revealed bilateral adrenal mass. The diagnosis of pheochromocytoma was made and confirmed by a high level of normetanephirnes and metanephrines.DiscussionThe coexistence of multiple endocrine neoplasia type 2 and myocardial infarction appears to be a rare association rather than a coincidence.ConclusionIn this case we highlight the importance of thorough history taking and investigation for the determining the aetiology of MINOCA. As a reversible cause of myocardial dysfunction, catecholamine-induced cardiomyopathy can occur as a feature of multiple endocrine neoplasia. The prognosis depends greatly on early diagnosis and prompt medical and surgical treatment, which are unfortunately often delayed because of the challenging diagnosis in many cases.

Acute cardiac complications and subclinical myocardial injuries associated with pheochromocytoma and paraganglioma

BackgroundCatecholamine excess arising from pheochromocytomas and paragangliomas (PPGLs) can cause a wide spectrum of cardiac manifestations, including acute cardiac complications (ACCs) and subclinical myocardial injuries (SMIs). In this study, we aimed to conduct a comprehensive analysis of ACCs and SMIs in a large cohort of patients with PPGLs.MethodsWe retrospectively analyzed the clinical data of consecutive patients with PPGLs admitted between January 2013 and July 2020 (n = 189). The prevalence of ACCs and SMIs and characteristics of patients identified with ACCs and SMIs were investigated. Moreover, comparisons were performed between patients with and without ACCs.ResultsFourteen patients (7.4%) fulfilled the criteria for ACCs, including nine (4.8%) who presented with Takotsubo-like cardiomyopathy, four (2.1%) with heart failure with preserved ejection fraction, and finally one (0.5%) with catecholamine-induced cardiomyopathy. Compared to those without ACCs (n = 175), patients with ACCs had a higher prevalence of epinephrine-producing PPGLs (81.8% vs 33.9%, P = 0.006) and were more likely to show invasive behavior (61.5% vs 27.3%, P = 0.022) or hemorrhage/necrosis (53.9% vs 17.4%, P = 0.005) on histology. The apical sparing pattern (5/7, 71.4%) was the dominant impairment pattern of longitudinal strain (LS) for patients displaying Takotsubo-like cardiomyopathy. In patients without cardiac symptoms, a fairly high proportion (21/77, 27.3%) of patients who underwent screening for troponin and/or natriuretic peptide and/or echocardiography had SMIs.ConclusionsOne in every fourteen PPGL patients presented with ACCs, and in the patients with Takotsubo-like cardiomyopathy, the apical sparing pattern was the primary impairment pattern of LS. Additionally, nearly one-third of patients without symptoms had SMIs. The diagnosis of PPGLs should be considered in patients with acute reversible cardiomyopathy, especially in those exhibiting an apical sparing pattern of LS.

Catecholamine-induced cardiomyopathy: an endocrinologist's perspective.

Although many endocrine diseases can be associated with acquired cardiomyopathy and heart failure, conditions except hypothyroidism, hyperthyroidism, phaeochromocytoma-paraganglioma (PPGL), and primary hyperaldosteronism are rare. PPGL is a rare catecholamine-secreting neuroendocrine tumour arising from the adrenal gland in 80-85% or extra-adrenal chromaffin cells of the autonomic neural ganglia in the remainder. The annual incidence of PPGL is 3-8 cases per million per year in the general population. Catecholamine-induced cardiomyopathy (CICMP) has got a prevalence of 8-11% among patients with PPGL. Hypertension, either sustained or episodic, is present in the vast majority (95%) of PPGL patients. However, among patients with CICMP, hypertension is present only in 65% of cases and the classical triad of paroxysmal headache, sweating, and palpitation is present only in 4%. Based on the cardiac remodelling in response to endogenous catecholamine excess, PPGL patients might present with one of the three CICMPs, including dilated cardiomyopathy (DCM), hypertrophic cardiomyopathy (HCM), or Takotsubo cardiomyopathy (TCM). Regardless of the subtypes, all CICMPs have many features in common - a dramatic clinical presentation, reversible cardiomyopathy, similar repolarisation electrocardiography changes, mild-moderate cardiac biomarker elevation, and normal coronary arteries on coronary angiography. CICMP should be suspected in patients with non-ischaemic, non-valvular forms of cardiomyopathy, even in those without definite features of catecholamine excess. PPGL associated TCM should be suspected in all acute coronary syndrome (ACS) patients exhibiting pronounced blood pressure variability with no culprit lesions on coronary angiography. This article will provide a review of the various CICMPs, their pathophysiology, clinical features, and the management options.

A Case of Catecholamine-Induced Cardiomyopathy Associated With Pheochromocytoma Presenting With a Clinical Course of Fulminant Myocarditis

A Case of Catecholamine-Induced Cardiomyopathy Associated With Pheochromocytoma Presenting With a Clinical Course of Fulminant Myocarditis