Acromegalic Cardiomyopathy With Dynamic Obstruction of the Left Ventricle Outflow Tract

Abstract

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.

The clinical use of the MOGE(S) classification in the differential diagnosis between idiopathic hypertrophic cardiomyopathy and its phenocopies

“…Второй по частоте фенокопией ГКМП является AL-амилоидоз (n=6, 4%). У 1 пациентки имела место акромегалическая кардиомиопатия (1%), имитирующая ГКМП [4]. В данной статье представляем 3 клинических случая, демонстрирующих трудности проведения дифференциального диагноза между идиопатической ГКМП и различными фенокопиями ГКМП, особенно при стертой внесердечной симптоматике (№ 1) или изолированном поражении сердца (№ 2, № 3).…”