Acute Dyspnoea Resulting from Pulmonary Oedema as the First Sign of a Phaeochromocytoma

Abstract

The day after undergoing neck dissection, a 42-year-old woman developed acute dyspnoea due to pulmonary oedema. Measurements with a Swan-Ganz catheter revealed not only cardiac depression but also a greatly increased peripheral vascular resistance: 5,400 dyn·s· cm^–5/m². A phaeochromocytoma with acute cardiac failure leading to pulmonary oedema was considered. Treatment with α- and β-blockers was complicated by severe hypotension and later ventricular fibrillation. Mechanical ventilation was required for 6 days following resuscitation. Investigation of the urine subsequently showed greatly increased catecholamine concentrations, while imaging revealed bilateral adrenal tumours. Our case history shows that acute pulmonary oedema may be the presenting manifestation of a phaeochromocytoma. The pulmonary oedema resulted partly from backward failure following tachycardia, myocyte necrosis and the greatly increased peripheral vascular resistance, and partly from increased permeability of the capillary network in the lungs.

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

“…PHEO patients could also present with symptoms and signs of pulmonary edema and/or hypertensive crisis, and rarely with refractory cardiogenic shock requiring mechanical circulatory support [51,71]. Acute onset of dyspnea due to pulmonary edema has been described as the initial presentation of PHEO [72,73]. Pulmonary edema is believed to be caused by decompensation in the setting of CMP and severely increased peripheral vascular resistance [72].…”

Contribution ofα- andβ-Adrenergic Mechanisms to the Development of Pulmonary Edema

“…Cardiogenic PE is associated with left ventricular (LV) dysfunction, while noncardiogenic edema presents with normal LV function and without signs of cardiopathy. Several mechanisms are proposed for pathogenesis of noncardiogenic PE which all can be induced by elevated CA levels or sympathetic discharge, mainly by predominant α -adrenergic stimulation: (1) vasoconstriction, especially postcapillary venoconstriction in the lung resulting in a rise in pulmonary-capillary hydrostatic pressure, (2) elevated pulmonary blood volume, (3) increased alveolocapillary permeability, and (4) inflammation with neutrophil accumulation in the lung [2, 6, 7, 9]. The latter may be caused by interleukin-6 (IL-6) overproduction that is thought to be a consequence of high CA levels [11, 12].…”

The Role of Catecholamines in Formation and Resolution of Pulmonary Oedema

“…Systemic hypertension due to vasoconstriction increases left ventricular afterload and finally, causes pulmonary congestion [13]. A similar pathogenesis combined with overfilling in the pulmonary circulation is discussed to cause non-cardiogenic oedema in phaeochromocytoma [14,15]. When the pulmonary blood flow is high, the endothelial surface area, one of the determinants of the capillary filtration coefficient, is increased [16].…”

“…It may concern the capillary endothelial layer, the alveolar epithelial layer, or, sometimes, all layers of the wall [26,28]. In NPE or phaeochromocytoma-related PO, effects of CAs, particularly -adrenergic effects with strong increase in TPR and elevation of cardiac afterload are discussed to finally cause a permeability defect of the alveolo-capillary barrier [3,14,29]. Ultrastructural changes in the walls of pulmonary capillaries identical to those observed in stress failure have been demonstrated in a rabbit model of NPE [30].…”

“…A study on the effects of different -adrenergic agonists and antagonists on formation and prevention of PO revealed that induction of oedema strictly depended on activation of 1 -adrenoceptors [67]. 1 -Adrenergic antagonists were successfully administered in phaeochromocytoma patients for treatment of PO [14,83]. In contrast, administration of the -blocker propranolol even induced PO in phaeochromocytoma patients [88].…”