Unexpected or Nonsuspected?

Abstract

Acute heart failure represents a challenge, especially in infrequent etiologies. We present the clinical case of a young woman diagnosed with acute heart failure and cardiogenic shock. Pheocromocytoma was identified as a reversible etiology. The surgical treatment led to a complete recovery of cardiac function. (Level of Difficulty: Beginner.)

Graphical abstract

Acute heart failure represents a challenge, especially in infrequent etiologies. We present the…

A 24-year-old woman without relevant past medical history presented to the emergency room after 6 days of flu-like symptoms. In the last 24 h, she also presented dyspnea, hot flashes, and palpitations. The physical examination was relevant for hypertension and tachycardia. The electrocardiogram showed supraventricular tachycardia that was treated with adenosine and labetalol returning to sinus rhythm. Two hours later, she had signs of peripheral hypoperfusion and low oxygen saturation. Laboratory tests were performed, and hypothyroidism, pneumonia, and pulmonary embolism were ruled out. The transthoracic echocardiography showed ejection fraction (EF) 20%, left atrial dilatation, and global hyperkinesia. The patient was transferred to the intensive care unit with cardiogenic shock after a cardiac arrest and developed multiorgan dysfunction; she required vasopressor, inotropic, and venoarterial extracorporeal membrane oxygenation support for 5 days. Her medical condition improved, but her blood pressure was difficult to control. A new echocardiography showed improvement of left ventricular EF to 60%, and cardiac magnetic resonance imaging ruled out myocarditis. The 24-h urine methanephrines tested positive, and the abdominal contrast magnetic resonance showed a left adrenal gland lesion compatible with pheochromocytoma (Figures 1A and 1B). The metaiodo- benzyl-guanidine scintigraphy showed signs of left adrenal gland neuroendocrine tumor (Figures 1C and 1D). The patient underwent radical left adrenalectomy and histopathology confirmed the diagnosis of pheochromocytoma. The patient improved her medical condition: after surgery she did not require antihypertensive medication, and was completely asymptomatic. During the follow-up period, she did not present heart failure manifestations, and the N-terminal pro–B-type natriuretic peptide and EF returned to normal values.

Figure 1.

Adrenal Mass: Pheochromocytoma

(A and B) Abdominal contrast magnetic resonance showed a left adrenal gland lesion. (C and D) The metaiodo-benzyl-guanidine scintigraphy showed signs of left adrenal gland neuroendocrine tumor.

Cardiomyopathy induced by pheochromocytoma represents a barely recognized entity. It has diverse clinical manifestations: Takotsubo syndrome, myocarditis, and dilated cardiomyopathy. The clinical presentation is variable: hypertension is the most frequent (65%), and the classical triad (headache, palpitations, and diaphoresis) is described in 4% (1). There are few reports of patients who present cardiogenic shock (2), but the predominance of multiorgan failure over catecholamine excess symptoms is frequently described (1). In this case, dobutamine could contribute to the evolution because of its potential to induce shock in patients with pheochromocytoma (2). The pheochromocytoma resection is associated with improvement of ventricular function in 96% of cases. The lack of intervention is related to an increase in mortality (33%), heart transplant (11%), and serious adverse events (44%) (1).

Pheocromocytoma-induced cardiomyopathy represents a potentially reversible disease. The diagnosis implies an early suspicion in cases of heart failure without clear etiology, even in the absence of classical symptoms of catecholamine excessive stimulation. Pheocromocytoma resection can prevent progression to irreversible cardiac remodeling and adverse outcomes.