A rare case report of hypertrophic cardiomyopathy induced by catecholamine-producing tumor

Abstract

Rationale: Catecholamine-producing tumors are rare, occurring in less than 0.2% of patients with hypertension, but can have relevant cardiovascular morbidity and mortality.

Patient concerns: A 37-year-old woman presented with a history of dyspnea, chest pain, palpitations, and paroxysmal hypertension. Electrocardiogram, echocardiogram, and cardiac magnetic resonance showed severe LVH with a prevalent involvement of the anterior portion of interventricular septum. Endomyocardial biopsy found severe hypertrophy with disarray of cardiomyocytes and ultrastructural evidence of contraction and necrosis of myocytes. Hormone investigations revealed high values of 24-hours urinary metanephrines. Abdominal computed tomography (CT) showed an enlarged left adrenal gland with a strong uptake of I-metaiodobenzylguanidine at scintigraphy scan.

Interventions: Thus, the adrenal tumor was surgically removed.

Outcomes: At follow-up examination, the patient's metanephrines levels were normalized and the transthoracic echocardiogram showed a reduction of LVH.

Diagnosis and lessons: We report a rare case of catecholamine-induced cardiomyopathy due to an adrenal adenoma mixed with nodules enriched in epinephrine-types secreting granules.

Figure 1

Cardiac magnetic resonance (MR) showing moderate symmetric left ventricular hypertrophy (LVH), which mostly involves the anterior section of interventricular septum (IVS) in its basal and medial part (sectal thickness of anterior-basal part is 19 mm; inferior-basal part is 14 mm; anterior-medial part is 16 mm; inferior-basal part is 14 mm), in basal portion of anterior wall (thickness 18 mm) and in middle portion of lateral wall (thickness 16 mm). The remaining part of myocardium has a thickness between 10 and 15 mm. This hypertrophy is responsible for a mild tightening/obstruction of the left ventricular outflow tract and a modest acceleration of flow, without an atypical systolic anterior motion of the mitral valve (SAM). Mild aortic and mitral valve regurgitation can been seen. LVH = left ventricular hypertrophy, IVS = interventricular septum, MR = magnetic resonance.

Figure 2

Microscopic findings of biopsy specimen from the endomyocardium of left ventricle showing markedly hypertrophied fibers, contraction bands and replacement fibrosis.

Figure 3

Scintigraphy with ¹²³I-metaiodobenzilguanidine (¹²³I—MIBG) showed raised activity within the left adrenal gland, concordant with the mass.

Figure 4

Immunohistochemical staining for synaptophysin in cortical adrenal gland specimen.

Figure 5

Electron microscopy showed neurosecretory granules in corticomedulla nodules.