ACROMEGALIC CARDIOMYOPATHY WITH DYNAMIC OBSTRUCTION OF THE LEFT VENTRICLE OUTFLOW TRACT

Aim. To explore genesis of the left ventricle hypertrophy in acromegaly patient, with the method of next generation sequencing.

Material and methods. Standard clinical and laboratory minimum was done, with electrocardiography, 24 hour ECG monitoring, echocardiography, magnete resonance tomography of the heart, new generation sequencing on the IlluminaHiSeq 2000 equipment with simultaneous analysis of 108 genes associated with idiopathic hypertrophic cardiomyopathy (HCMP) and phenocopies of HCMP.

Results. At the age 59 y. o. the female patient had beed first time diagnosed with asymmetric HCMP, non-obstructive type (interventricular septum 19 mm, posterior wall 11 mm, pressure gradient in outflow tract of the left ventricle (OTLV) — 25 mmHg). At the age 62 y. o. she developed HCMP with dynamic obstruction of OTLV (pressure gradient in OTLV up to 80 mmHg) with progressing dyspnea on exertion, and required non-surgical reduction of interventricular septum. By the computed tomography data, at the age 63 y. o. the patient was diagnosed with endocellar hypophysis microadenoma (a tumor 6,7*7,3 mm), somatotropic hormone — 53,39 mU/L (normal: 0,1-20 mU/L), insulin-like growth factor 1 — 359 ng/mL (normal: 118-314 ng/mL). However, with retrospective analysis of her photos, even from the age 40 y. o. there were enlarged hands, feet, nasal cartilages, ears, lips and eyebrow arcs that witness for long lasting disease course. With the new generation sequencing, there were no pathological mutations revealed.

Conclusion. The case represents hypertrophic cardiomyopathy as a leading clinical sign in acromegalic cardiomyopathy that imitated idiopathic HCMP. Patient management in such case should include on-time etiopathogenetic therapy that works against disease progression, and in some cases even for regression of the left ventricle hypertrophy.

1. Elliott PM, Anastasakis A, Borger MA, et al. 2014 ESC Guidelines on diagnosis and management of hypertrophic cardiomyopathy. European Heart Journal. 2014; 35: 273379. DOI: 10.1093/eurheartj/ehu284.

2. Abdelsalam MA, Nippoldt TB, Geske JB. Acromegaly-induced cardiomyopathy with dobutamine-induced outflow tract obstruction. BMJ Case Rep. 2016. DOI: 10.1136/bcr2015-213463.

3. Viveiros Monteiro A, Fiarresga A, Cacela D. Alcohol septal ablation in obstructive acromegalic hypertrophic cardiomyopathy — a first case report. Rev Port Cardiol. 2016; 35 (9): 499.

4. Hradec J, Marek J, Petrásek J. The nature of cardiac hypertrophy in acromegaly: an echocardiographic study. Cor Vasa. 1988; 30 (3): 186-99.

5. Kawanishi H, Suda K, Mori T, et al. The heart in acromegaly: an echocardiographic study. J. Cardiogr. 1984; 14 (3): 459-69.

6. Li G, Borger MA, Williams WG et al. Regional overexpression of insulin-like growth factor-I and transforming growth factor-beta1 in the myocardium of patients with hypertrophic obstructive cardiomyopathy. J. Thorac Cardiovasc Surg. 2002; 123 (1): 89-95.

7. Li G, Li RK, Mickle DA, et al. Elevated insulin-like growth factor-I and transforming growth factor-beta 1 and their receptors in patients with idiopathic hypertrophic obstructive cardiomyopathy. A possible mechanism. Circulation. 1998; 98 (19): II144-9.

8. Tokita N, Hasegawa S, Hirata M, et al. Fluorine-18 FDG myocardial positron emission tomographic findings before and after pituitary adenoma resection in a patient with acromegalic cardiomyopathy. Clin Nucl Med. 2000; 25 (8): 619-21.