Cardiomyopathy and phaeochromocytoma

Abstract

SummaryA cnse is presented of phaeoc~hromocytomu with cuteuholumine-induced cardiomyopath). The dlfeculties in the rnanugenient when left ventricuiar failure occurred are reported and the importance of nionitoring pulmonary artery pressure during the unaesthetic procedure is strrssed. Key wordsAnue.ctkesia; phaeochromocytoma. C'onipliutions; pulmonary oedema. cardiomyopathy.The anaesthetic management of patients with phaeochromocytoma is notoriously difficult even when the diagnosis has been established prior to surgery. One of the factors causing this difficulty is the presence of cardiom yopathy which, despite being found in 2&309;, of patients with phaeochromocytoma, has received little attention in the literature.'-' Small necrotic foci are found in the myocardium which are apparently unrelated to vascular distribution. There is segmentation of the myocyte cytoplasm within these foci into cosinophilic transverse bands, and interstitial proliferation of mononuclear cells whilst polymorphonuclear cells are unusual. Areas of calcification are found within the fibres. This combination is termed myofibrillar degeneration or myofibrillar damage.4 The myofibrillar damage is due to direct inflammatory cffects of the catecholamines, ischaemia caused by coronary vasoconstriction, relative ischaemia due to increased oxygen demand. and to increased platelet aggregation. ~ -~The clinical manifestations are variable and non-specific, and include disturbances of the cardiac rhythm, congestive heart failure, acute pulmonary oedema coincident with hypertensive crises. and non-specific electrocardiographic changes.6 Case historyThe anaesthetic management and the complications in one patient who presented with phaeochromocytoma and cardiomyopathy are described. The patient was a Caucasian female aged 45 years who had an 1 1-year history of headache. palpitations. hyperhidrosis. pallor and occasional dyspnoea. In the past 3 years she had developed dyspnoea on exertion and had several episodes of pulmonary oedema. She was admitted to hospital with supraventricular tachycardia, left ventricular failure and acute pulmonary oedema which coincided with a hypertensive crisis. This settled a few minutes ~ F

A Case of a Composite Adrenal Medullary Tumor of Pheochromocytoma and Ganglioneuroma Masquerading as Acute Pancreatitis

“…The signs and symptoms are often absent and can be unusually presented as catecholamine-induced cardiomyopathy4-6) or hyperamylasemia7-11). In review of the literature, the amylase was almost exclusively of the S-type in the pheochromocytoma- associated hyperamylasemia.…”

“…It is apparent that composite tumors of that type may display symptoms referable to hormonal hypersecretion by either portion of the tumor1). Yet, pheochromocytomas commonly act in the typical manner and presented as cardiomyopathy4-6) or pancreatitis7-11) have been rarely documented. The non-specific nature of its manifestations may render prompt recognition elusive12).…”

The perioperative management of children with phaeochromocytoma

“…Making the diagnosis of cardiomyopathy is important for several reasons: (i) There is an increased morbidity and mortality associated with phaeochromocytomas complicated by the presence of cardiomyopathy ( 1, 6, 26); (ii) The myocardial dysfunction associated with the condition may be reversible with medical ( 24, 27) or surgical ( 23) treatment of the underlying phaeochromocytoma; (iii) the discovery of a cardiomyopathy has important implications for the child's subsequent management, especially by the anaesthetist. The choice of anaesthetic technique, including the possible use of additional monitoring such as pulmonary artery catheters (vida infra) may be altered ( 26, 28). The choice of drugs used will be affected, in particular the avoidance of negative inotropes such as β‐blockers that can precipitate pulmonary oedema and severe hypotension ( 28, 29) and perhaps a more cautious approach to fluid administration to help reduce morbidity ( 26).…”

Undiagnosed Pheochromocytoma Complicated with Perioperative Hemodynamic Crisis and Multiple Organ Failure

“…The splanchnic vessels are highly susceptible to catecholamineinduced vasoconstriction, and hence the ischemic gut mucosa may allow bacterial translocation or the passage of endotoxins across the intestinal barrier to extraintestinal sites including the lung which is the first organ to fail. Also, acute renal failure can be attributed to acute tubular necrosis because of the combination of cardiogenic shock reducing the renal perfusion, associated with renal vasoconstriction induced by a surge of catecholamines (Sardesai et al,1990;Gilsanz et al., 1983;Takaror et al, 1987;Fripp et al, 1981). Gut-derived factors, contained primarily in the mesenteric lymph rather than the portal system, potentiate the development of distant multiple organ failure.…”

“…Excess noradrenaline also induces changes in permeability of the sarcolimmal membrane leading to increased calcium influx. Also, it has been proposed that the injury process might involve release of free radicals (Sardesai et al,1990;Gilsanz et al., 1983;Takaror et al, 1987;Fripp et al, 1981). Left atrial enlargement secondary to catecholamine-induced left ventricular diastolic dysfunction is quite common (around 20%), but seldom poses a management problem unless accompanied by left ventricular systolic dysfunction.…”