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Inverted takotsubo syndrome complicated with cardiogenic shock requiring veno-arterial extracorporeal membrane oxygenation in a patient with bilateral pheochromocytoma: a case report

Inverted takotsubo syndrome complicated with cardiogenic shock requiring veno-arterial... Downloaded from https://academic.oup.com/ehjcr/article-abstract/4/2/1/5807903 by guest on 28 April 2020 CASE REPORT European Heart Journal - Case Reports (2020) 4, 1–5 doi:10.1093/ehjcr/ytaa055 Heart failure Inverted takotsubo syndrome complicated with cardiogenic shock requiring veno-arterial extracorporeal membrane oxygenation in a patient with bilateral pheochromocytoma: a case report 1 1 Bebiana Manuela Monteiro Faria *, Joao Portugueˆs , 2 2 Roberto Roncon-Albuquerque Jr , and Rodrigo Pimentel 1 o 2 Cardiology Department, Hospital Senhora da Oliveira—Guimar~ aes, Rua dos Cutileiros, n 11, 44853-044 Guimar~ aes, Portugal; and Intensive Care Unit, Centro Hospitalar de S~ ao Jo~ ao, Porto, Portugal Received 25 September 2019; first decision 17 October 2019; accepted 13 February 2020; online publish-ahead-of-print 16 March 2020 Background Takotsubo syndrome (TS) is characterized by a transient left ventricular (LV) dysfunction and rarely presents with cardiogenic shock (CS). Inverted TS (ITS) is a rare entity associated with the presence of a pheochromocytoma. ................................................................................................................................................................................................... Case summary We present a case of a young woman was admitted to the emergency department due to intense headache, chest discomfort, palpitations, and breathlessness. An ITS secondary to a pheochromocytoma crisis presenting with CS was diagnosed. The patient was managed with veno-arterial extracorporeal membrane oxygenation, until recovery of LV function. On the 35th day of hospitalization, open bilateral adrenalectomy was performed. ................................................................................................................................................................................................... Discussion Takotsubo syndrome patients presenting with CS are challenging and clinicians should be aware of underlying causes. Specific triggers such as pheochromocytoma should systematically be considered particularly if ITS was pre- sented. Extracorporeal life support devices could provide temporary mechanical circulatory support in patients with TS on refractory CS and help to manage complex cases with TS due to pheochromocytoma. Keywords Takotsubo syndrome Pheochromocytoma Veno-arterial extracorporeal membrane oxygenation � � � Case report Learning points Recognition of a rare entity such, as inverted takotsubo syndrome, and its association with specific aetiologies, which should be systematic- ally investigated. The therapeutic management of a patient in cardiogenic shock (CS) secondary to the catecholaminergic crisis. The importance of mechanical circulatory support in patients with refractory CS, particularly in those with a potentially reversible cause. * Corresponding author. Tel: þ351 934899908, Email: bebianafaria@gmail.com Handling Editor: Domenico D’Amario Peer-reviewers: Albert Galyavich, Luca Arcari, and Hatem Soliman Aboumarie Compliance Editor: Carlos Minguito Carazo Supplementary Material Editor: Peregrine Green V The Author(s) 2020. Published by Oxford University Press on behalf of the European Society of Cardiology. This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com Downloaded from https://academic.oup.com/ehjcr/article-abstract/4/2/1/5807903 by guest on 28 April 2020 2 B.M.M. Faria et al. The electrocardiogram (EKG) revealed sinus tachycardia, left axis Introduction deviation, V4–V6 ST-segment depression, maximum 2 mm at V5, and Takotsubo syndrome (TS) is characterized by transient wall motion . an inverted T wave in aVL (Figure 1). abnormalities of the left ventricle that typically involve the apical and . Transthoracic echocardiography showed severe LV systolic dys- 1 . midventricular segments (apical ballooning). A variant, with akinesia . function with midbasal segments akinesia and hypercontractility of the of the basal and mid left ventricular (LV) segments and sparing of the . apical segments (Figure 2, Supplementary material online, Video S1). . The patient underwent aortic computed tomography angiography apex, has been called inverted TS (ITS) and is associated with the presence of a pheochromocytoma. This variant is important to iden- that excluded an acute aortic syndrome, but identified signs of severe pulmonary oedema and two heterogeneous solid masses in both ad- tify as it tends not to be recognized as readily as the apical 1,2 renal glands, the largest on the right adrenal gland, measuring presentation. 9.2 9.2 10.8 cm, suggestive of a bilateral pheochromocytoma Pheochromocytoma crisis (PC) is an endocrine emergency with a 3,4 . (Figure 3). high mortality rate. Inverted TS associated with PC is an unusual . The patient progressed with respiratory failure and shock signs but reversible presentation and should be recognized as early as pos- . with poor peripheral perfusion, oligoanuria, and arterial hyperlactaci- sible. There is a lack of evidence on the management of patients with demia (41 mg/dL; normal range< _ 18 mg/dL), despite her hypertensive TS and concomitant pheochromocytoma but proper and fast sup- profile (mean arterial pressure 110–120 mmHg). At this stage, she port could impact the patient’s prognosis. was submitted to mechanical ventilation. Due to the rapidly deterio- . rated clinical status, she was referred and transported to the closest hospital with extracorporeal membrane oxygenation (ECMO) cap- Timeline ability for immediate mechanical circulatory support. The patient remained with veno-arterial ECMO (VA-ECMO) sup- port for 14 days and progressive recovery of LV dysfunction was observed, enabling the weaning of the VA-ECMO support from the Timeline Description 10th day, without use of inotropics. There were no significant Day 0 Patient admission in emergency department in a changes on the EKG on the following 2 days, with regression of repo- peripheral hospital. . larization changes at follow-up. The peak troponin I level was 1.73 ng/ Probable diagnosis was made: inverted . mL (normal range < _0.015 mg/dL), on the first day of hospitalization Takotsubo syndrome complicated with car- . with a slow but progressive fall and subsequent normalization. diogenic shock secondary to pheochromocy- . Urinary catecholamines and metanephrines in 24-h urine were ele- toma crisis . . vated (Table 1). Alpha-blockade was started with phenoxybenzamine Day 0–6 h after Transfer to hospital with extracorporeal mem- . and subsequently beta-blockade with metoprolol. admission brane oxygenation (ECMO) capability . On the 35th day of hospitalization, open bilateral adrenalectomy Day 0–8 h after Patient started ECMO support . was performed, without complications, and she began supplementa- admission . tion with gluco and mineralocorticoids. Anatomopathological exam- Day 10 Weaning ECMO support . ination confirmed the diagnosis of bilateral pheochromocytomas. Day 14 ECMO support removed The patient was discharged on the 49th day of hospitalization with Day 35 Open bilateral adrenalectomy complete recovery of the left ventricular systolic function and pre- Day 49 Hospital discharge served neurologic status. After discharge, the patient performed body scintigraphy with MIBG-123 that excluded pheochromocytoma metastases or syn- chronous tumours, but she was diagnosed with bilateral thyroid me- dullary carcinoma. Total thyroidectomy was performed and the Case presentation diagnose of Type 2 multiple endocrine neoplasia (MEN) syndrome was established. A 26-year-old woman was admitted to the emergency department due to intense headache, chest discomfort, palpitations, and breath- lessness, with 4 h of evolution. Discussion Physical examination showed tachypnoea, tachycardia (145 . b.p.m.), blood pressure of 158/95 mmHg, no fever, and oxygen satur- . Takotsubo syndrome is a reversible LV dysfunction triggered by a ation on room air of 92%. Cardiac auscultation was normal but pul- precipitating factor (emotional, physical, or combined) in about 70% monary auscultation revealed bilaterally dispersed crackles. She was of the patients and is thought to be caused by catecholamine- pale, with cold and clammy extremities and decreased capillary refill mediated injury. The exact mechanism is less well-understood, but it time but peripheral pulses were easily palpable. . is probably the result of a combination of various mechanisms: cor- The patient had a personal history of migraine and had been sub- . onary artery or microvascular spasms, direct cellular toxicity, and 1,2,5 mitted to periodontal surgery on the previous day. She was medi- . myocardial stunning due to receptor desensitization. cated with amoxicillin/clavulanic acid and prednisolone. No relevant . Inverted TS has been reported in various situations such as anti- family history was reported. depressant overdose, subarachnoid bleed, anaphylactic shock, and Downloaded from https://academic.oup.com/ehjcr/article-abstract/4/2/1/5807903 by guest on 28 April 2020 Inverted TS complicated with cardiogenic shock requiring VA-ECMO 3 Figure 1 Admission electrocardiogram revealed sinus tachycardia, left axis deviation, V4–V6 ST-segment depression, maximum 2 mm at V5, and an inverted T wave in aVL. . 10 also in association with pheochromocytoma, so these conditions 12.4%. In this case, although haemodynamic measurements of car- 1,6 should be considered in this particular setting. Even though the ori- diac output or index could not be obtained, the presence of severe ginal Mayo Clinic Diagnosis Criteria considered pheochromocytoma systolic dysfunction and signs of peripheral poor perfusion, oligoanu- as an exclusion criterium for the diagnosis of TS, this was never uni- ria, and hyperlactacidemia corroborate the diagnosis of CS. High versally accepted. The diagnostic criteria of the Heart Failure . blood pressure can be explained by the intense vasoconstriction Association of the European Society of Cardiology (ESC) and more caused by catecholaminergic discharge, increasing the afterload and recently, the new InterTAK Diagnosis Criteria, identified pheochro- contributing to the shock. mocytoma as a trigger for TS and this concept was integrated in the In TS presenting with CS, it is crucial to assess whether the shock 1,7 current ESC consensus position paper on TS. is caused by LV outflow tract obstruction or primary pump failure. In This clinical case highlights that, despite similar pathophysiological primary pump failure, treatment with VA-ECMO or an LV assist de- 5,7 causes to typical TS, ITS is associated with a different patient profile vice should be considered as a bridge to recovery. Catecholamine and presenting symptoms. Compared with typical TS, patients with inotropics should be avoided, especially if the TS is triggered by PC, 5,7 ITS more frequently have a stress trigger and present at a younger as they could exacerbate the clinical condition. age, which may be due to the abundance of adrenoreceptors at the In this patient, the presence of headaches, palpitations, a large ab- base of the heart in young individuals and the apex in older ones. dominal mass, and an ITS complicated with CS, despite sustained Contrarily to previous data that shows that ITS patients may pre- hypertension, constituted red flags to the diagnosis of pheochromo- sent with less pulmonary oedema and cardiogenic shock (CS) than cytoma and a PC precipitated by an elective surgery and the gluco- patients with classic TS, our case presented with CS, highlighting that corticoid therapy. 1,2 CS in ITS should also raise the suspicion of a PC. . Although coronary angiography should be considered in every pa- Pheochromocytomas are rare catecholamine-secreting neuroen- . tient with TS and/or unexplained CS, especially in cases of ST- docrine tumours in the adrenal medulla, with an incidence of . segment deviation, in this case, coronary heart disease was not 2-9 cases per 1 000 000 adults. Usually sporadic (90%), they also can . excluded. The patient was a very young female with no cardiovascu- appear earlier in the context of neurofibromatosis, Von Hippel– . lar risk factors, an almost normal EKG on admission, no electrocar- 8 . Lindau or MEN syndromes. . diographic evolution, or troponin rise compatible with an acute Pheochromocytoma crisis has reported mortality as high as 85% coronary syndrome and there was an early suspicion of pheochro- 3,4 and about 10–20% of cases present with TS. mocytoma with abdominal imaging confirmed afterwards with ele- CS is defined as systemic tissue hypoperfusion due to inadequate vated urinary catecholamines. cardiac output despite the adequate circulatory volume. CS is a life- Elective adrenalectomy is the ideal option for pheochromocytoma threatening complication in TS, with an incidence between 2.8% and treatment, accompanied by appropriate preoperative medical Downloaded from https://academic.oup.com/ehjcr/article-abstract/4/2/1/5807903 by guest on 28 April 2020 4 B.M.M. Faria et al. Table 1 Urinary catecholamines and metanephrines in patient with bilateral pheochromocytoma Catecholamines and Results Reference metanephrines in 24-h urine (mg/h) range (mg/h) ................................................................................................. Norepinephrine 13 144/24 <97/24 Epinephrine 3064/24 <27/24 Dopamina 1118/24 <500/24 Normetanephrine 15 689/24 <390/24 Metanephrine 15 000/24 <320/24 without appropriate alpha-blockade as this could worsen hyperten- sive episodes by exacerbating vasoconstriction while inhibiting vasodilation. In our case, alpha-blockers were gradually introduced, followed by . beta-blockers once cardiac systolic function had recovered and . ECMO support had been removed. Adrenalectomy was deferred . until myocardial recovery. . Conclusion . Takotsubo syndrome patients presenting with CS are challenging and . clinicians should be aware of possible underlying causes. Specific trig- . gers such as PC should systematically be considered for patients with . TS, particularly ITS complicated by CS. For the most severe cases, ECMO support can be a life-saving Figure 2 Admission echocardiography showing subcostal view of therapy, allowing myocardial recovery. After haemodynamic stabil- the left ventricle at end-diastole (A), and end-systole (B). ization, treatment should include alpha-blockade followed by beta- blockade, with elective adrenalectomy after myocardial recovery. Lead author biography Bebiana Manuela Monteiro Faria was born in Guimar~ aes, Portugal on 7 April 1988. She studied Medicine at University of Porto—Institute of Biomedical Sciences Abel Salazar where completed her Mastersde- gree in 2013. Since 2015 she is Cardiology resident at Hospital Senhora da Oliveira Guimar~ aes. With a particular interest in Emergency Medicine she also works as an Emergency Physician in the National Institute of Medical Emergency since 2017. Figure 3 Computed tomography scan showing two heteroge- neous solid masses (*), on the right (9.2 9.2 10.8 cm) and left (2.3 2.8 3.3 cm) adrenal gland. Supplementary material Supplementary material is available at European Heart Journal - Case Reports online. treatment. Alpha-blockade before elective surgery is associated with Slide sets: A fully edited slide set detailing this case and suitable for reduced mortality, shorter hospital stays, and fewer post-operative 5,8 complications. Beta-blockers should never be administered local presentation is available online as Supplementary data. Downloaded from https://academic.oup.com/ehjcr/article-abstract/4/2/1/5807903 by guest on 28 April 2020 Inverted TS complicated with cardiogenic shock requiring VA-ECMO 5 4. Ibrahim M, Banga S, Venkatapuram S, Mungee S. Transient cardiogenic shock Consent: The author/s confirm that written consent for submission . during a crisis of pheochromocytoma triggered by high-dose exogenous cortico- and publication of this case report including image(s) and associated . steroids. BMJ Case Rep 2015;doi:10.1136/bcr-2014-208683. text has been obtained from the patient in line with COPE guidance. . 5. Hekimian G, Kharcha F, Bre ´ chot N, Schmidt M, Ghander C, Lebreton G, Girerd W, Tresallet C, Trouillet JL, Leprince P, Chastre J, Combes A, Luyt CE. . Extracorporeal membrane oxygenation for pheochromocytoma-induced cardio- Conflict of interest: none declared. . genic shock. Ann Intensive Care 2016;6:117. 6. Banerjee S. Inverted Takotsubo cardiomyopathy: a rare entity often missed! . Indian Heart J 2016;68:s8–s9. References 7. Lyon AR, Bossone E, Schneider B, Sechtem U, Citro R, Underwood SR, Sheppard MN, Figtree GA, Parodi G, Akashi YJ, Ruschitzka F, Filippatos G, 1. Ghadri J-R, Wittstein IS, Prasad A, Sharkey S, Dote K, Akashi YJ, Cammann VL, Mebazaa A, Omerovic E. Current state of knowledge on takotsubo syndrome: a Crea F, Galiuto L, Desmet W, Yoshida T, Manfredini R, Eitel I, Kosuge M, Nef . . Position Statement from the Taskforce on Takotsubo Syndrome of the Heart HM, Deshmukh A, Lerman A, Bossone E, Citro R, Ueyama T, Corrado D, Kurisu Failure Association of the European Society of Cardiology. Eur J Heart Fail 2016; S, Ruschitzka F, Winchester D, Lyon AR, Omerovic E, Bax JJ, Meimoun P, 18:8–27. Tarantini G, Rihal C, Y.-Hassan S, Migliore F, Horowitz JD, Shimokawa H, . 8. Farrugia FA, Charalampopoulos A. Pheochromocytoma—review. Endocr Regul Lu ¨ scher TF, Templin C. International Expert Consensus Document on Takotsubo Syndrome (Part I): clinical characteristics, diagnostic criteria, and 2019;53:191–212. pathophysiology. Eur Heart J 2018;39:2032–2046. 9. Bellumkonda L, Gul B, Masri S. Evolving concepts in diagnosis and management 2. Patankar GR, Choi JW, Schussler JM. Reverse takotsubo cardiomyopathy: two . of cardiogenic shock. AmJCardiol 2018;122:1104–1110. case reports and review of the literature. J Med Case Rep 2013;7:84. 10. El-Battrawy I, Lang S, Ansari U, Sattler K, Behnes M, Schramm K, Fastner C, 3. Reyes HA, Paquin JJ, Harris DM. Pheochromocytoma, “the Great Masquerader,” Tu ¨lu ¨ men E, Zhou X, Hoffmann U, Borggrefe M, Akin I. Incidence and prognostic presenting as severe acute decompensated heart failure in a young patient. Case relevance of cardiopulmonary failure in takotsubo cardiomyopathy. Sci Rep 2017; Rep Cardiol 2018;2018:8767801. . 7:14673. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png European Heart Journal - Case Reports Oxford University Press

Inverted takotsubo syndrome complicated with cardiogenic shock requiring veno-arterial extracorporeal membrane oxygenation in a patient with bilateral pheochromocytoma: a case report

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Downloaded from https://academic.oup.com/ehjcr/article-abstract/4/2/1/5807903 by guest on 28 April 2020 CASE REPORT European Heart Journal - Case Reports (2020) 4, 1–5 doi:10.1093/ehjcr/ytaa055 Heart failure Inverted takotsubo syndrome complicated with cardiogenic shock requiring veno-arterial extracorporeal membrane oxygenation in a patient with bilateral pheochromocytoma: a case report 1 1 Bebiana Manuela Monteiro Faria *, Joao Portugueˆs , 2 2 Roberto Roncon-Albuquerque Jr , and Rodrigo Pimentel 1 o 2 Cardiology Department, Hospital Senhora da Oliveira—Guimar~ aes, Rua dos Cutileiros, n 11, 44853-044 Guimar~ aes, Portugal; and Intensive Care Unit, Centro Hospitalar de S~ ao Jo~ ao, Porto, Portugal Received 25 September 2019; first decision 17 October 2019; accepted 13 February 2020; online publish-ahead-of-print 16 March 2020 Background Takotsubo syndrome (TS) is characterized by a transient left ventricular (LV) dysfunction and rarely presents with cardiogenic shock (CS). Inverted TS (ITS) is a rare entity associated with the presence of a pheochromocytoma. ................................................................................................................................................................................................... Case summary We present a case of a young woman was admitted to the emergency department due to intense headache, chest discomfort, palpitations, and breathlessness. An ITS secondary to a pheochromocytoma crisis presenting with CS was diagnosed. The patient was managed with veno-arterial extracorporeal membrane oxygenation, until recovery of LV function. On the 35th day of hospitalization, open bilateral adrenalectomy was performed. ................................................................................................................................................................................................... Discussion Takotsubo syndrome patients presenting with CS are challenging and clinicians should be aware of underlying causes. Specific triggers such as pheochromocytoma should systematically be considered particularly if ITS was pre- sented. Extracorporeal life support devices could provide temporary mechanical circulatory support in patients with TS on refractory CS and help to manage complex cases with TS due to pheochromocytoma. Keywords Takotsubo syndrome Pheochromocytoma Veno-arterial extracorporeal membrane oxygenation � � � Case report Learning points Recognition of a rare entity such, as inverted takotsubo syndrome, and its association with specific aetiologies, which should be systematic- ally investigated. The therapeutic management of a patient in cardiogenic shock (CS) secondary to the catecholaminergic crisis. The importance of mechanical circulatory support in patients with refractory CS, particularly in those with a potentially reversible cause. * Corresponding author. Tel: þ351 934899908, Email: bebianafaria@gmail.com Handling Editor: Domenico D’Amario Peer-reviewers: Albert Galyavich, Luca Arcari, and Hatem Soliman Aboumarie Compliance Editor: Carlos Minguito Carazo Supplementary Material Editor: Peregrine Green V The Author(s) 2020. Published by Oxford University Press on behalf of the European Society of Cardiology. This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com Downloaded from https://academic.oup.com/ehjcr/article-abstract/4/2/1/5807903 by guest on 28 April 2020 2 B.M.M. Faria et al. The electrocardiogram (EKG) revealed sinus tachycardia, left axis Introduction deviation, V4–V6 ST-segment depression, maximum 2 mm at V5, and Takotsubo syndrome (TS) is characterized by transient wall motion . an inverted T wave in aVL (Figure 1). abnormalities of the left ventricle that typically involve the apical and . Transthoracic echocardiography showed severe LV systolic dys- 1 . midventricular segments (apical ballooning). A variant, with akinesia . function with midbasal segments akinesia and hypercontractility of the of the basal and mid left ventricular (LV) segments and sparing of the . apical segments (Figure 2, Supplementary material online, Video S1). . The patient underwent aortic computed tomography angiography apex, has been called inverted TS (ITS) and is associated with the presence of a pheochromocytoma. This variant is important to iden- that excluded an acute aortic syndrome, but identified signs of severe pulmonary oedema and two heterogeneous solid masses in both ad- tify as it tends not to be recognized as readily as the apical 1,2 renal glands, the largest on the right adrenal gland, measuring presentation. 9.2 9.2 10.8 cm, suggestive of a bilateral pheochromocytoma Pheochromocytoma crisis (PC) is an endocrine emergency with a 3,4 . (Figure 3). high mortality rate. Inverted TS associated with PC is an unusual . The patient progressed with respiratory failure and shock signs but reversible presentation and should be recognized as early as pos- . with poor peripheral perfusion, oligoanuria, and arterial hyperlactaci- sible. There is a lack of evidence on the management of patients with demia (41 mg/dL; normal range< _ 18 mg/dL), despite her hypertensive TS and concomitant pheochromocytoma but proper and fast sup- profile (mean arterial pressure 110–120 mmHg). At this stage, she port could impact the patient’s prognosis. was submitted to mechanical ventilation. Due to the rapidly deterio- . rated clinical status, she was referred and transported to the closest hospital with extracorporeal membrane oxygenation (ECMO) cap- Timeline ability for immediate mechanical circulatory support. The patient remained with veno-arterial ECMO (VA-ECMO) sup- port for 14 days and progressive recovery of LV dysfunction was observed, enabling the weaning of the VA-ECMO support from the Timeline Description 10th day, without use of inotropics. There were no significant Day 0 Patient admission in emergency department in a changes on the EKG on the following 2 days, with regression of repo- peripheral hospital. . larization changes at follow-up. The peak troponin I level was 1.73 ng/ Probable diagnosis was made: inverted . mL (normal range < _0.015 mg/dL), on the first day of hospitalization Takotsubo syndrome complicated with car- . with a slow but progressive fall and subsequent normalization. diogenic shock secondary to pheochromocy- . Urinary catecholamines and metanephrines in 24-h urine were ele- toma crisis . . vated (Table 1). Alpha-blockade was started with phenoxybenzamine Day 0–6 h after Transfer to hospital with extracorporeal mem- . and subsequently beta-blockade with metoprolol. admission brane oxygenation (ECMO) capability . On the 35th day of hospitalization, open bilateral adrenalectomy Day 0–8 h after Patient started ECMO support . was performed, without complications, and she began supplementa- admission . tion with gluco and mineralocorticoids. Anatomopathological exam- Day 10 Weaning ECMO support . ination confirmed the diagnosis of bilateral pheochromocytomas. Day 14 ECMO support removed The patient was discharged on the 49th day of hospitalization with Day 35 Open bilateral adrenalectomy complete recovery of the left ventricular systolic function and pre- Day 49 Hospital discharge served neurologic status. After discharge, the patient performed body scintigraphy with MIBG-123 that excluded pheochromocytoma metastases or syn- chronous tumours, but she was diagnosed with bilateral thyroid me- dullary carcinoma. Total thyroidectomy was performed and the Case presentation diagnose of Type 2 multiple endocrine neoplasia (MEN) syndrome was established. A 26-year-old woman was admitted to the emergency department due to intense headache, chest discomfort, palpitations, and breath- lessness, with 4 h of evolution. Discussion Physical examination showed tachypnoea, tachycardia (145 . b.p.m.), blood pressure of 158/95 mmHg, no fever, and oxygen satur- . Takotsubo syndrome is a reversible LV dysfunction triggered by a ation on room air of 92%. Cardiac auscultation was normal but pul- precipitating factor (emotional, physical, or combined) in about 70% monary auscultation revealed bilaterally dispersed crackles. She was of the patients and is thought to be caused by catecholamine- pale, with cold and clammy extremities and decreased capillary refill mediated injury. The exact mechanism is less well-understood, but it time but peripheral pulses were easily palpable. . is probably the result of a combination of various mechanisms: cor- The patient had a personal history of migraine and had been sub- . onary artery or microvascular spasms, direct cellular toxicity, and 1,2,5 mitted to periodontal surgery on the previous day. She was medi- . myocardial stunning due to receptor desensitization. cated with amoxicillin/clavulanic acid and prednisolone. No relevant . Inverted TS has been reported in various situations such as anti- family history was reported. depressant overdose, subarachnoid bleed, anaphylactic shock, and Downloaded from https://academic.oup.com/ehjcr/article-abstract/4/2/1/5807903 by guest on 28 April 2020 Inverted TS complicated with cardiogenic shock requiring VA-ECMO 3 Figure 1 Admission electrocardiogram revealed sinus tachycardia, left axis deviation, V4–V6 ST-segment depression, maximum 2 mm at V5, and an inverted T wave in aVL. . 10 also in association with pheochromocytoma, so these conditions 12.4%. In this case, although haemodynamic measurements of car- 1,6 should be considered in this particular setting. Even though the ori- diac output or index could not be obtained, the presence of severe ginal Mayo Clinic Diagnosis Criteria considered pheochromocytoma systolic dysfunction and signs of peripheral poor perfusion, oligoanu- as an exclusion criterium for the diagnosis of TS, this was never uni- ria, and hyperlactacidemia corroborate the diagnosis of CS. High versally accepted. The diagnostic criteria of the Heart Failure . blood pressure can be explained by the intense vasoconstriction Association of the European Society of Cardiology (ESC) and more caused by catecholaminergic discharge, increasing the afterload and recently, the new InterTAK Diagnosis Criteria, identified pheochro- contributing to the shock. mocytoma as a trigger for TS and this concept was integrated in the In TS presenting with CS, it is crucial to assess whether the shock 1,7 current ESC consensus position paper on TS. is caused by LV outflow tract obstruction or primary pump failure. In This clinical case highlights that, despite similar pathophysiological primary pump failure, treatment with VA-ECMO or an LV assist de- 5,7 causes to typical TS, ITS is associated with a different patient profile vice should be considered as a bridge to recovery. Catecholamine and presenting symptoms. Compared with typical TS, patients with inotropics should be avoided, especially if the TS is triggered by PC, 5,7 ITS more frequently have a stress trigger and present at a younger as they could exacerbate the clinical condition. age, which may be due to the abundance of adrenoreceptors at the In this patient, the presence of headaches, palpitations, a large ab- base of the heart in young individuals and the apex in older ones. dominal mass, and an ITS complicated with CS, despite sustained Contrarily to previous data that shows that ITS patients may pre- hypertension, constituted red flags to the diagnosis of pheochromo- sent with less pulmonary oedema and cardiogenic shock (CS) than cytoma and a PC precipitated by an elective surgery and the gluco- patients with classic TS, our case presented with CS, highlighting that corticoid therapy. 1,2 CS in ITS should also raise the suspicion of a PC. . Although coronary angiography should be considered in every pa- Pheochromocytomas are rare catecholamine-secreting neuroen- . tient with TS and/or unexplained CS, especially in cases of ST- docrine tumours in the adrenal medulla, with an incidence of . segment deviation, in this case, coronary heart disease was not 2-9 cases per 1 000 000 adults. Usually sporadic (90%), they also can . excluded. The patient was a very young female with no cardiovascu- appear earlier in the context of neurofibromatosis, Von Hippel– . lar risk factors, an almost normal EKG on admission, no electrocar- 8 . Lindau or MEN syndromes. . diographic evolution, or troponin rise compatible with an acute Pheochromocytoma crisis has reported mortality as high as 85% coronary syndrome and there was an early suspicion of pheochro- 3,4 and about 10–20% of cases present with TS. mocytoma with abdominal imaging confirmed afterwards with ele- CS is defined as systemic tissue hypoperfusion due to inadequate vated urinary catecholamines. cardiac output despite the adequate circulatory volume. CS is a life- Elective adrenalectomy is the ideal option for pheochromocytoma threatening complication in TS, with an incidence between 2.8% and treatment, accompanied by appropriate preoperative medical Downloaded from https://academic.oup.com/ehjcr/article-abstract/4/2/1/5807903 by guest on 28 April 2020 4 B.M.M. Faria et al. Table 1 Urinary catecholamines and metanephrines in patient with bilateral pheochromocytoma Catecholamines and Results Reference metanephrines in 24-h urine (mg/h) range (mg/h) ................................................................................................. Norepinephrine 13 144/24 <97/24 Epinephrine 3064/24 <27/24 Dopamina 1118/24 <500/24 Normetanephrine 15 689/24 <390/24 Metanephrine 15 000/24 <320/24 without appropriate alpha-blockade as this could worsen hyperten- sive episodes by exacerbating vasoconstriction while inhibiting vasodilation. In our case, alpha-blockers were gradually introduced, followed by . beta-blockers once cardiac systolic function had recovered and . ECMO support had been removed. Adrenalectomy was deferred . until myocardial recovery. . Conclusion . Takotsubo syndrome patients presenting with CS are challenging and . clinicians should be aware of possible underlying causes. Specific trig- . gers such as PC should systematically be considered for patients with . TS, particularly ITS complicated by CS. For the most severe cases, ECMO support can be a life-saving Figure 2 Admission echocardiography showing subcostal view of therapy, allowing myocardial recovery. After haemodynamic stabil- the left ventricle at end-diastole (A), and end-systole (B). ization, treatment should include alpha-blockade followed by beta- blockade, with elective adrenalectomy after myocardial recovery. Lead author biography Bebiana Manuela Monteiro Faria was born in Guimar~ aes, Portugal on 7 April 1988. She studied Medicine at University of Porto—Institute of Biomedical Sciences Abel Salazar where completed her Mastersde- gree in 2013. Since 2015 she is Cardiology resident at Hospital Senhora da Oliveira Guimar~ aes. With a particular interest in Emergency Medicine she also works as an Emergency Physician in the National Institute of Medical Emergency since 2017. Figure 3 Computed tomography scan showing two heteroge- neous solid masses (*), on the right (9.2 9.2 10.8 cm) and left (2.3 2.8 3.3 cm) adrenal gland. Supplementary material Supplementary material is available at European Heart Journal - Case Reports online. treatment. Alpha-blockade before elective surgery is associated with Slide sets: A fully edited slide set detailing this case and suitable for reduced mortality, shorter hospital stays, and fewer post-operative 5,8 complications. Beta-blockers should never be administered local presentation is available online as Supplementary data. Downloaded from https://academic.oup.com/ehjcr/article-abstract/4/2/1/5807903 by guest on 28 April 2020 Inverted TS complicated with cardiogenic shock requiring VA-ECMO 5 4. Ibrahim M, Banga S, Venkatapuram S, Mungee S. Transient cardiogenic shock Consent: The author/s confirm that written consent for submission . during a crisis of pheochromocytoma triggered by high-dose exogenous cortico- and publication of this case report including image(s) and associated . steroids. BMJ Case Rep 2015;doi:10.1136/bcr-2014-208683. text has been obtained from the patient in line with COPE guidance. . 5. Hekimian G, Kharcha F, Bre ´ chot N, Schmidt M, Ghander C, Lebreton G, Girerd W, Tresallet C, Trouillet JL, Leprince P, Chastre J, Combes A, Luyt CE. . Extracorporeal membrane oxygenation for pheochromocytoma-induced cardio- Conflict of interest: none declared. . genic shock. Ann Intensive Care 2016;6:117. 6. Banerjee S. Inverted Takotsubo cardiomyopathy: a rare entity often missed! . Indian Heart J 2016;68:s8–s9. References 7. Lyon AR, Bossone E, Schneider B, Sechtem U, Citro R, Underwood SR, Sheppard MN, Figtree GA, Parodi G, Akashi YJ, Ruschitzka F, Filippatos G, 1. Ghadri J-R, Wittstein IS, Prasad A, Sharkey S, Dote K, Akashi YJ, Cammann VL, Mebazaa A, Omerovic E. Current state of knowledge on takotsubo syndrome: a Crea F, Galiuto L, Desmet W, Yoshida T, Manfredini R, Eitel I, Kosuge M, Nef . . Position Statement from the Taskforce on Takotsubo Syndrome of the Heart HM, Deshmukh A, Lerman A, Bossone E, Citro R, Ueyama T, Corrado D, Kurisu Failure Association of the European Society of Cardiology. Eur J Heart Fail 2016; S, Ruschitzka F, Winchester D, Lyon AR, Omerovic E, Bax JJ, Meimoun P, 18:8–27. Tarantini G, Rihal C, Y.-Hassan S, Migliore F, Horowitz JD, Shimokawa H, . 8. Farrugia FA, Charalampopoulos A. Pheochromocytoma—review. Endocr Regul Lu ¨ scher TF, Templin C. International Expert Consensus Document on Takotsubo Syndrome (Part I): clinical characteristics, diagnostic criteria, and 2019;53:191–212. pathophysiology. Eur Heart J 2018;39:2032–2046. 9. Bellumkonda L, Gul B, Masri S. Evolving concepts in diagnosis and management 2. Patankar GR, Choi JW, Schussler JM. Reverse takotsubo cardiomyopathy: two . of cardiogenic shock. AmJCardiol 2018;122:1104–1110. case reports and review of the literature. J Med Case Rep 2013;7:84. 10. El-Battrawy I, Lang S, Ansari U, Sattler K, Behnes M, Schramm K, Fastner C, 3. Reyes HA, Paquin JJ, Harris DM. Pheochromocytoma, “the Great Masquerader,” Tu ¨lu ¨ men E, Zhou X, Hoffmann U, Borggrefe M, Akin I. Incidence and prognostic presenting as severe acute decompensated heart failure in a young patient. Case relevance of cardiopulmonary failure in takotsubo cardiomyopathy. Sci Rep 2017; Rep Cardiol 2018;2018:8767801. . 7:14673.

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European Heart Journal - Case Reports – Oxford University Press

Published: Jun 1, 2020

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