Noncardiogenic pulmonary edema as the sole manifestation of pheochromocytoma.

Abstract

SUMMARY A 40-year-old man was admitted to the hospital with pulmonary edema without signs of left ventricular failure. Noncardiogenic pulmonary edema was diagnosed, and a subsequent workup identified a pheochromocytoma as the cause of this condition. The clinical picture could be mimicked by infusion of exogenous norepinephrine. It is concluded that surges of catecholamines from a pheochromocytoma may provoke pulmonary edema in a manner similar to that by which neurogenic pulmonary edema related to cerebral disorders occurs. (Hypertension 8: 810-812, 1986) KEY WORDS • catecholamines • heart failure * hypertension P RESSOR crises, excessive sweating, and headache are among the most frequent symptoms of pheochromocytoma. 1 Furthermore, pulmonary edema may develop as a result of cardiac failure. The latter usually is due to a sudden or sustained rise in arterial pressure or the result of catecholamine-induced cardiomyopathy.1 ' 2 We report on a patient with pheochromocytoma in whom several episodes of pulmonary edema occurred in the absence of hypertension and without signs of left ventricular dysfunction.Case Report A 40-year-old man was admitted to the coronary care unit because of acute, severe dyspnea and production of blood-stained sputum. He had been well until 8 years earlier when he was involved in a car accident and remained comatose for 24 hours. Since that time he had had several bouts of mild dyspnea accompanied by feelings of unease and restlessness. Although such episodes were short-lived, the patient had noticed an increased frequency over the previous 2 or 3 months.On examination he appeared tachypneic, cyanotic, and clammy. Supine blood pressure was 80/60 mm Hg, and the heart rate was 115 beats/min. The heart was normal, and no ventricular gallop was heard, but numerous rales were audible in both lungs. No other abnormalities were evident. Laboratory investigation disclosed slightly elevated hemoglobin levels (12 mmol/L), hematocrit (59%), and creatinine levels (135 /xmol/L). The levels of serum glutamic-oxalacetictransaminase, serum glutamic-pyruvate transaminase, and lactic dehydrogenase were modestly increased, but the creatinine kinase level was normal. A chest roentgenogram showed massive pulmonary edema but a normal-sized heart; an electrocardiogram revealed sinus tachycardia and signs of right ventricular strain with no evidence of myocardial infarction. After a Swan-Ganz thermodilution catheter had been introduced, the following hemodynamic data were obtained: pulmonary artery pressure, 21/3 mm Hg (mean, 12 mm Hg); pulmonary capillary wedge pressure, 2 mm Hg; right atrial pressure, 0 mm Hg. Cardiac output was 3.45 L/min, while intra-arterial pressure was still low: 75/55 mm Hg. All measurements were done while the patient was still recumbent. Calculated systemic vascular resistance was normal, but pulmonary vascular resistance was increased. Treatment consisted of oxygen by mask and infusion of plasma and the patient recovered within a few hours. Cardiac output then stabilized at 7 L/min...

Emergencies Caused by Pheochromocytoma, Neuroblastoma, or Ganglioneuroma

“…Although hypotension was thought to occur only in patients with predominantly epinephrine-secreting tumors [29,30], it has also been described in norepinephrine-secreting tumors [31]. The mechanisms that lead to hypotension and shock in patients with pheochromocytoma are not understood.…”

Pulmonary Edema in Pheochromocytoma

“…Pulmonary edema as the first presentation of pheochromocytoma is uncommon and usually rapidly fatal (7,8). It can be seen not only during the course of the disease, but also after tumor resection (9).…”

“…In most pheochromocytoma patients pulmonary edema is cardiogenic in origin. Nonetheless, noncardiogenic pulmonary edema is also seen (7). It is thought to occur as a result of catecholamine-induced transient increase in pulmonary capillary pressure due to pulmonary venoconstriction and altered pulmonary capillary permeability (10).…”

De novo Multiple Endocrine Neoplasia Type 2B with Noncardiogenic Pulmonary Edema as the Presenting Symptom

“…Pulmonary edema as the first sign of MEN 2B is uncommon, although some reports have suggested a relationship between pulmonary edema and pheochromocytoma. Pulmonary edema as the first presentation of pheochromocytoma is rare and usually rapidly fatal [5,6]. In most pheochromocytoma patients, pulmonary edema is cardiogenic in origin, because marked catecholamine release from the tumor may cause cardiovascular emergencies, such as hypertensive crisis, myocarditis, cardiomyopathy, and myocardial ischemia [7,8].…”

“…It was suggested that a rise in the pulmonary-capillary hydrostatic pressure result from postcapillary venoconstriction secondary to local effects of catecholamines and cause upstream transudation of fluid in the alveoli of interstitial space in animal study [9]. Increased permeability of the alveolocapillary membrane secondary to an elevation in catecholamines has been invoked [5]. It was shown that catecholamines dramatically increase after a variety of central nervous system insults [10].…”