Context:Pheochromocytoma crisis is a feared and potentially lethal complication of pheochromocytoma.Objective:We sought to determine the best treatment strategy for pheochromocytoma crisis patients and hypothesized that emergency resection is not indicated.Design:Retrospective cohort study (1993-2011); literature review (1944-2011).Setting:Tertiary referral center.Patients:There were 137 pheochromocytoma patients from our center and 97 pheochromocytoma crisis patients who underwent adrenalectomy from the literature.Intervention:Medical management of pheochromocytoma crisis; adrenalectomy.Main Outcome Measure(s):Perioperative complications, conversion, and mortality.Results:In our database, 25 patients (18%) presented with crisis. After medical stabilization and α-blockade, 15 patients were discharged and readmitted for elective surgery and 10 patients were operated on urgently during the same hospitalization. None underwent emergency surgery. Postoperatively, patients who underwent elective surgery had shorter hospital stays (1.7 vs 5.7 d, P = 0.001) and fewer postoperative complications (1 of 15 [7%] vs 5 of 10 [50%], P = 0.045) and were less often admitted to the intensive care unit (1 of 15 [7%] vs 5 of 10 [50%], P = 0.045) in comparison with urgently operated patients. There was no mortality. Review of the literature (n = 97) showed that crisis patients who underwent elective or urgent surgery vs emergency surgery had less intraoperative (13 of 31 [42%] vs 20 of 25 [80%], P < 0.001) and postoperative complications (15 of 45 [33%] vs 15 of 21 [71%], P = 0.047) and a lower mortality (0 of 64 vs 6 of 33 [18%], P = 0.002).Conclusions:Management of patients presenting with pheochromocytoma crisis should include initial stabilization of the acute crisis followed by sufficient α-blockade before surgery. Emergency resection of pheochromocytoma is associated with high surgical morbidity and mortality.
"On the other hand, α-blockade is the first choice for the management of pheochromocytoma crisis  and to reduce the risk of hypertensive crises in patients with biochemically active pheochromocytoma . α-Blockade should be started before β-blockade because stimulation of α-adrenoceptors unopposed by β-adrenoceptor vasodilation can result in hypertensive crises  . Vasculitis is a well-described but rare autoimmune complication of pheochromocytoma, triggered by an excess of cathecolamine levels . "
"60 The mortality from phaeochromocytoma multisystem crisis was reported as 45% after the review of 11 published cases from 1988–2009. 61 Scholten et al. (2013) 6 reported a 0% mortality in their series of 25 cases of PCC, and a 2% (1 of 58) mortality from a literature review they conducted (1990–2013). However, in both instances, patients were only selected if they had proceeded to surgery, and so their cohorts would not have included any patient who died prior to surgery. "
[Show abstract][Hide abstract] ABSTRACT: Pheochromocytoma crisis is an endocrine emergency associated with significant mortality. There is little published guidance on the management of pheochromocytoma crisis. This clinical practice update summarises the relevant published literature, including a detailed review of cases published in the past five years, and a proposed classification system. We review the recommended management of pheochromocytoma crisis including the use of alpha blockade, which is strongly associated with survival of a crisis. Mechanical circulatory supportive therapy (such as intra-aortic balloon pump or extra corporeal membrane oxygenation) is strongly recommended for patients with sustained hypotension. Surgical intervention should be deferred until medical stabilisation is achieved. This article is protected by copyright. All rights reserved.
[Show abstract][Hide abstract] ABSTRACT: Phaeochromocytomas and paragangliomas (PPGL) can cause acute catecholamine cardiomyopathy (ACC). We assessed the prevalence of ACC and compared the presentation of cases with and without ACC in a large series of PPGL.
Single centre retrospective study.
Hypertension Unit, University Hospital, Paris.
140 consecutive patients with PPGL, referred from January 2003 to September 2012.
Left ventricular ejection fraction (LVEF), perioperative mortality.
Fifteen patients (11%) had suffered an ACC, occurring in 14 cases before the diagnosis of PPGL. Precipitating factors were identified in 11 cases. Twelve patients presented with acute pulmonary oedema, including 10 with cardiogenic shock, requiring life support in eight cases. Seven patients (five with pulmonary oedema) presented with acute chest pain and cardiac dysfunction. Electrocardiographic abnormalities were present in 14 cases: ST segment elevation or pathological Q waves, ST segment depression, and/or diffuse T wave inversion. Six patients displayed classical (apical ballooning) or inverted (basal/mid ventricular stunning) takotsubo-like cardiomyopathy. Coronary arteries were always normal on angiography. In patients with ACC, median LVEF rose from 30% (IQR 23-33%) during ACC to 71% (50-72%) before surgery (n=11, p<0.001). Median LVEF before PPGL surgery was 65% (51-72%) and 65% (60-70%) in patients with and without a history of ACC, respectively (not significant).
PPGL may present as ACC in 11% of cases, excluding patients dying from undiagnosed tumours. Left ventricular dysfunction is usually reversible before surgery. PPGL should be suspected in patients with acute heart failure without evidence of valvular or coronary artery disease.
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