Vol 73, No 2 (2022)
Clinical vignette
Published online: 2022-04-05

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An atypical cause leading to a diagnosis of acromegaly

Ivana Ságová12, Milan Dragula2, Marián Mokáň2, Peter Vaňuga1
Pubmed: 35381102
Endokrynol Pol 2022;73(2):385-386.

Abstract

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Clinical vignette

Endokrynologia Polska

DOI: 10.5603/EP.a2022.0024

ISSN 0423–104X, e-ISSN 2299–8306

Volume/Tom 73; Number/Numer 2/2022

An atypical cause leading to a diagnosis of acromegaly

Ivana Ságová12Milan Dragula2Marián Mokáň2Peter Vaňuga1
1Department of Endocrinology, National Institute of Endocrinology and Diabetology, Ľubochňa, Slovakia
21st Department of Internal Medicine, Comenius University Jessenius Faculty of Medicine, University Hospital Martin, Martin, Slovakia

Key words: acromegaly; heart failure; insulin-like growth factor 1; treatment

Ivana Ságová M.D., Ph.D., Department of Endocrinology, National Institute of Endocrinology and Diabetology, Kollárová 282/3, 034 91 Ľubochňa, Slovakia, tel: +421444306138; e-mail: ivana.sagova1@gmail.com

Submitted: 31.12.2021

Accepted: 06.02.2022

Early publication date: 05.04.2022

This article is available in open access under Creative Common Attribution-Non-Commercial-No Derivatives 4.0 International (CC BY-NC-ND 4.0) license, allowing to download articles and share them with others as long as they credit the authors and the publisher, but without permission to change them in any way or use them commercially

Acromegaly is characterized by a chronic hypersecretion of growth hormone (GH). Cardiovascular diseases are the most common comorbidity in this condition [1]. A specific acromegalic cardiomyopathy characterized by a biventricular hypertrophy is the result of a long-term exposure of excessive amounts of GH and insulin-like growth factor 1 (IGF-1) to the cardiomyocyte [2]. We report a case of a patient with an undiagnosed acromegaly, which was firstly presented with an acute heart failure.

A 39-year-old man with a history of arterial hypertension with exogenous obesity was admitted to the department of internal medicine for an acute heart failure. The patient was admitted to the hospital with orthopnoea (in the patient’s history there was exertional dyspnoea lasting 3 months, and an orthopnoea and a horizontal position intolerance was presented last night before the hospitalization) without anginal pain and palpitation. He suffered from excessive sweating and arthralgia lasting 1 year. There was no history of alcohol, smoking, or drug abuse. He had a tall stature [height 193 cm, weight 150 kg, body mass index (BMI) 40.5 kg/m2] and enlarged acral parts of the body (including enlargement of the hands, feet, facial bone, nose, and tongue, with wider space between his teeth). The physical examination showed a blood pressure of 145/90 mmHg, pulmonary auscultation revealed bilateral crackles to the middle of the lungs, cardiac auscultation was normal, and lower limbs were with pitting oedema. 12-lead ECG revealed sinus rhythm at 66 bpm, without ischaemic changes, and without arrhythmia. A chest X-ray showed enlargement of the cardiac silhouette and accentuation of the bronchovascular markings with a maximum in the perihilar and paracardiac regions on the right side. Blood count, coagulation, biochemistry, and inflammatory parameters were normal. At the beginning of the hospitalization, proper treatment of heart failure was started. Echocardiography showed dilated left ventricle and left atrium, hypertrophy of the interventricular septum (IVS 17 mm) with a diffuse hypokinesis, and an ejection fraction (EF) of 22%. Diastolic function was with a restrictive filling pattern. The right ventricle and atrium were also dilated, and the valves were normal (Fig. 1). Coronary angiography showed normal coronary arteries without a stenosis. 24-hour electrocardiographic (Holter) monitoring result was without arrhythmia. The condition was initially assessed as an acute heart failure in poorly treated arterial hypertension, and a diagnosis of a hypertrophic cardiomyopathy was also considered. During the complex treatment of the heart failure (angiotensin converting enzyme inhibitors, diuretics, b-blockers) the patient’s clinical condition improved (NYHA II during discharge). The patient was subsequently admitted to the National Endocrinology and Diabetology Institute in Ľubochňa for suspected acromegaly. Laboratory tests revealed a high level of GH not suppressed after glucose administration, and high levels of IGF-1 and IGF binding protein 3 (IGFBP3) (Supplementary File Tab. 1). Magnetic resonance imaging (MRI) scan showed a 14 × 14 × 12 mm macroadenoma of the pituitary gland and enlarged adenohypophysis extending the Turkish saddle. The infundibulum was without deviation, the chiasma opticum was not dilated. The distance of the visual pathway from the pituitary macroadenoma was 0.5 mm. (Fig. 2A, C). A diagnosis of acromegaly was established. A perimetry test ruled out visual field defects. An abdominal ultrasound showed hepatomegaly. Treatment by somatostatin analogues (lanreotide) was initiated at 120 mg every 28 days. Transsphenoidal resection of the pituitary macroadenoma was initially abandoned due to a cardiac finding. After 1 year of treatment with lanreotide the plasma IGF-1 and basal GH normalized. Oral GTT test with GH was with adequate suppression of GH after glucose administration (Tab. 1). A control MRI of the sella turcica demonstrated a regression of the pituitary macroadenoma to size 11 × 11 × 8.5 mm (Fig. 2 B, D). From a cardiological point of view, the clinical condition of the patient improved (functional stage NYHA I). A control echocardiography showed persistent dilatation of ventricles and ventricular hypertrophy, with an improved diastolic function to stage 1 and a slight improvement in systolic function as well as improvement of the cardiac output (Fig. 3). As part of the differential diagnosis of cardiomyopathy, a cardiac MRI was performed, which, due to the patient's size and numerous artifacts, did not further contribute to the diagnosis. From a cardiological point of view, a prospective consultation of the transplant centre is planned in case of insufficient success of the complex treatment.

18-Sagova-1.jpg
Figure 1. Echocardiogram on admission. LVPWsposterior wall thickness end systole; LVIDsleft ventricular internal diameter end systole; IVSsinterventricular septal wall thickness end systole; LVPWdposterior wall thickness end diastole; LVIDdleft ventricular internal diameter end diastole; IVSdinterventricular septal wall thickness end diastole; EDVLV end-diastolic volume; ESVend-systolic volume; FSfractional shortening; EFejection fraction
18-Sagova-2.jpg
Figure 2. Pituitary magnetic resonance imaging (MRI). A. T1VO in sagittal axison admission; B. T1VO in sagittal axis after 1 year under treatment with somatostatin analogues; C. T1VO in transversal axison admission; D. T1VO in transversal axisafter 1 year under treatment with a somatostatin analogues. The white arrow marks pituitary adenoma
18-Sagova-3.png
Figure 3. Echocardiogram after 1 year under treatment with somatostatin analogues. LVPWsposterior wall thickness end systole; LVIDsleft ventricular internal diameter end systole; IVSsinterventricular septal wall thickness end systole; LVPWdposterior wall thickness end diastole; LVIDd- left ventricular internal diameter end diastole; IVSdinterventricular septal wall thickness end diastole; EDVLV end-diastolic volume; ESVend-systolic volume; FSfractional shortening; EFejection fraction

Cardiovascular disease is a major cause of morbidity and mortality in patients with acromegaly [1]. An early diagnosis and a prompt treatment of acromegaly is essential to avert chronic cardiovascular damage [3]. A strict control of hormone excess in acromegaly is associated with a significant improvement in cardiac hypertrophy, as well as diastolic and systolic functions, leading to an overall improvement in cardiac structure and performance [4]. In the differential diagnosis of a heart failure based on a cardiomyopathy, we must not forget the possible endocrine cause not only thyroid dysfunction, but also acromegaly.

Conflict of interest

The authors declare that they have no conflicts of interest concerning this article. All authors have read and approved the final form of this article.

References

  1. Ságová I, Pavai D, Kantárová D, et al. Influence of Disease Activity and Body Composition Parameters on Cross-Sectional Area of the Median Nerve in Acromegalic Patients. Physiol Res. 2021; 70(6): 921929, doi: 10.33549/physiolres.934681, indexed in Pubmed: 34717059.
  2. Sharma MD, Nguyen AV, Brown S, et al. Cardiovascular Disease in Acromegaly. Methodist Debakey Cardiovasc J. 2017; 13(2): 6467, doi: 10.14797/mdcj-13-2-64, indexed in Pubmed: 28740584.
  3. Gadelha MR, Kasuki L, Lim DST, et al. Systemic Complications of Acromegaly and the Impact of the Current Treatment Landscape: An Update. Endocr Rev. 2019; 40(1): 268332, doi: 10.1210/er.2018-00115, indexed in Pubmed: 30184064.
  4. Colao A, Pivonello R, Galderisi M, et al. Impact of treating acromegaly first with surgery or somatostatin analogs on cardiomyopathy. J Clin Endocrinol Metab. 2008; 93(7): 26392646, doi: 10.1210/jc.2008-0299, indexed in Pubmed: 18445662.