Advances in Research on the Cardiovascular Complications of Acromegaly

Cardiovascular-related complications are one of the most common complications in patients with acromegaly, and can lead to an increased risk of death. Hypertension and cardiomyopathy are the main cardiovascular complications. The characteristics of acromegalic cardiomyopathy are concentric biventricular hypertrophy and diastolic dysfunction. In addition, arrhythmia and heart valve disease are common cardiac complications in acromegaly. Although the underlying pathophysiology has not been fully elucidated, the spontaneous overproduction of GH and IGF-1, increasing age, prolonged duration of disease and the coexistence of other cardiovascular risk factors are crucial to cardiac complications in patients with acromegaly. Early diagnosis and appropriate treatment of acromegaly might be beneficial for the prevention of cardiomyopathy and premature death.

P1253 A case report of acromegalic cardiomyopathy with severe left ventricular hypertrophy

Introduction Acromegaly is a rare disease, mostly caused by a growth hormone (GH)-secreting benign pituitary tumor, with an increased production of GH and insulin-like growth factor 1 (IGF-1). One hallmark feature of the disease is acromegalic cardiomyopathy, a syndrome of progressive cardiac dysfunction characterized by left ventricular hypertrophy, diastolic dysfunction, and combined systolic and diastolic dysfunction in the very advanced stage. Clinical case A 54-year-old male with history of arterial hypertension and abnormal electrocardiogram (ECG) for more than 10 years was diagnosed with active stage of acromegaly (IGF-1-1711ng/ml, cardiac magnetic resonance (CMR) – pituitary tumor). Surgical treatment was recommended. He was referred for preoperative cardiac evaluation preceding transsphenoidal resection of a pituitary adenoma. He denied syncope or any chest pain. The standard 12-lead ECG demonstrated sinus rhythm, left atrial enlargement and left ventricular (LV) hypertrophy with deep negative T waves in V3-V6 leads. Holter monitor demonstrated episodes of non-sustained ventricular tachycardia. Transthoracic echocardiography revealed severe asymmetric LV hypertrophy without LVOT obstruction at rest and maneuver Valsalva (max. grad. – 19mm. Hg) with mildly abnormal LV ejection fraction (48%, range ≥52%), severe reduced global longitudinal strain (-8,2%, range <-18%) and grade II diastolic dysfunction. CMR imaging was performed. According to CMR suggested hypertrophic cardiomyopathy (HCM) demonstrating area of myocardial fibrosis on extensive late gadolinium enhancement, maximal LV wall thickness of 40 mm, increased myocardial mass (index 277g/m2, range 68–103g/m2) and mildly reduced systolic LV function (LVEF - 54%, range 57-74%). Coronary angiography did not show significant stenosis. After cardiac examination, transsphenoidal adenomectomy was done. There are currently no algorithms sudden cardiac death (SCD) for patients with acromegalic cardiomyopathy, but he was recommended implantation of cardioverter defibrillator devices on a scale for HCM (Risc SCD – 4,17%). Acromegaly-induced cardiomyopathy can mimic HCM. He was also recommended genetic typing for HCM, considering the results of MRI. Conclusion/Discussion: Reduction GLS might be expected to result in a fall in LVEF, however this is often not the case in hypertrophic LV diseases. It is important to note that measures of regional function such as myocardial strain may actually reflect global systolic function better than the ejection fraction. The results may improve our ability to provide a more accurate prognosis and better assessment of actual systolic function. Because cardiomyopathy is an important cause of mortality in acromegaly, diagnosis and appropriate management are critical to improve survival. Abstract P1253 Figure.

Heart in Acromegaly: The Echocardiographic Characteristics of Patients Diagnosed with Acromegaly in Various Stages of the Disease

To determine whether the echocardiographic presentation allows for diagnosis of acromegalic cardiomyopathy. 140 patients with acromegaly underwent echocardiography as part of routine diagnostics. The results were compared with the control group comprising of 52 age- and sex-matched healthy volunteers. Patients with acromegaly presented with higher BMI, prevalence of arterial hypertension, and glucose metabolism disorders (i.e., diabetes and/or prediabetes). In patients with acromegaly, the following findings were detected: increased left atrial volume index, increased interventricular septum thickness, increased posterior wall thickness, and increased left ventricular mass index, accompanied by reduced diastolic function measured by the following parameters: E’med., E/E’, and E/A. Additionally, they presented with abnormal right ventricular systolic pressure. All patients had normal systolic function measured by ejection fraction. However, the values of global longitudinal strain were slightly lower in patients than in the control group; the difference was statistically significant. There were no statistically significant differences in the size of the right and left ventricle, thickness of the right ventricular free wall, and indexed diameter of the ascending aorta between patients with acromegaly and healthy volunteers. None of 140 patients presented systolic dysfunction, which is the last phase of the so-called acromegalic cardiomyopathy. Some abnormal echocardiographic parameters found in acromegalic patients may be caused by concomitant diseases and not elevated levels of GH or IGF-1 alone. The potential role of demographic parameters like age, sex, and/or BMI requires further research.