acromegaly cardiomyopathy

Introduction

Introduction to acromegaly cardiomyopathy Acromegaly is a group of clinical syndromes in which the pituitary gland secretes too much growth hormone, leading to systemic tissue hyperplasia, hypertrophy and metabolic disorders. Recently reported, the disease involves a lot of cardiovascular damage, with the prolongation of the disease, the heart is enlarged, especially in patients who are 50 years old, the affected person can be diagnosed as acromegalic heart disease (acromegalic heart disease). basic knowledge The proportion of sickness: 0.002%-0.003% Susceptible people: no specific population Mode of infection: non-infectious Complications: diabetes, hypertension, arrhythmia, heart failure

Cause

Causes of acromegaly cardiomyopathy

(1) Causes of the disease

The etiology of acromegaly is unknown. The main pathology is pituitary growth hormone cell adenoma or hyperplasia, which secretes growth hormone, causing soft tissue, hypertrophy of bone and viscera, and endocrine and metabolic disorders. More than 99% of acromegaly is caused by pituitary gland. Growth hormone cell tumors are caused by a small number of growth hormone releasing hormone secreted by ganglion cell tumors of the hypothalamus; ectopic growth hormone secreting hormones or growth hormone secreted by peripheral tissues cause acromegaly rare.

(two) pathogenesis

Most patients with acromegaly are caused by growth hormone cell adenomas, a few are caused by hyperplasia and adenocarcinoma, but there are also mixed tumors. The diameter of the tumor is generally about 2cm or less, and the larger one is up to 4cm. The tissue around the saddle is often subjected to compression and erosion, sometimes causing an increase in intracranial pressure. Hemorrhage and cystic changes in the advanced tumor cause the function to drastically decrease from hyperthyroidism. Excessive secretion of growth hormone (GH) acts on various tissues and organs throughout the body. Through the signal transmission system, the protein synthesis and proliferation of the cells are stimulated. In the lesions of the internal organs, the skeletal system is most obvious, and the effect on the heart is divided into two stages as the course progresses.

1. Formation period: The main manifestations at this stage are cardiomyocyte proliferation, increased heart volume, thickened ventricular wall, symmetry hypertrophy in the whole heart, narrow heart chamber and diastolic function.

2. The recession period: As the disease progresses to the recession period, heart failure is the main manifestation. At this time, the myocardial contractility is weakened and the cardiac output is reduced.

Patients with acromegaly can be clinically evidenced by subclinical heart disease, especially diastolic dysfunction, which can be used to indicate that ventricular wall hypertrophy in patients with acromegaly is in the absence of any increase in wall tension. What happens is mainly centripetal hypertrophy, which is different from simple centripetal hypertrophy. It does not cause changes in the intraventricular diameter. Unless there is heart failure in the late stage of the disease, such patients have multiple left and right ventricles at the same time, and biventricular diastolic The filling was impaired. At rest, the contractile function did not change much, but the left ventricular ejection fraction did not increase correspondingly during exercise, suggesting that the cardiac function reserve was impaired.

In the early stage of acromegaly, the cardiac contractility is enhanced, the cardiac output is increased, the peripheral vascular resistance is decreased, and the dynamic state is high. At that time, the heart has no obvious morphological changes, and the progression of the disease, cardiac hypertrophy, interstitial hyperplasia, and ventricular diastolic dysfunction , cardiac output decreased, peripheral tissue perfusion decreased, clinical dyspnea may occur, more severe, ventricular diastolic dysfunction, cardiac output decreased significantly, histological examination showed extensive myocardial fibrosis, partial myocardial necrosis, with lymphatic and Monocyte infiltration.

Prevention

Acromegaly cardiomyopathy prevention

The cardiovascular system damage of this disease comes from pituitary diseases, so active treatment of primary disease is the key to prevent this disease.

Complication

Acromegaly cardiomyopathy complications Complications diabetes hypertensive arrhythmia heart failure

In the advanced stage of the disease, complications such as pituitary dysfunction, metabolic disorders, diabetes, hypertension, arrhythmia, heart failure or secondary infection may be complicated.

Symptom

Symptoms of acromegaly cardiomyopathy common symptoms headache

The disease has a slow onset, and the course of disease lasts for more than 20 years. The course of the disease can be divided into two phases.

1. Formation period: generally from the age of 20 to 30 years old, the earliest manifestations of thick hands and feet, rough face, headache, fatigue, back pain and diabetes symptoms, then the typical appearance gradually appeared, head soft tissue hyperplasia, scalp, rough skin Thickening, multiple folds, thick lips, enlarged ear and nose, large and thick tongue, blurred words, low tone, followed by head bone hyperplasia, resulting in enlarged face, mandibular growth, upper eyelid, forehead bone, tibia The bows are enlarged and prominent, while the limbs are thickened, the fingers and toes are short and thick, and the back of the back of the hand is thick and wide.

This period of disease is longer, up to 5 to 10 years.

2. The recession period: When the disease develops to the peak, it gradually becomes mentally debilitated and mentally metamorphosis. Then, multiple organ dysfunction begins to appear. The symptoms of pituitary and its surrounding compression are more common in the later stage, and half of the visual field is impaired. The hypothalamus is compressed. After sleepiness, obesity, diabetes insipidus and other manifestations, but increased intracranial pressure is rare, advanced often due to pituitary dysfunction, metabolic disorders, diabetic complications, heart failure or secondary infection and death.

3. Cardiovascular damage in acromegaly

(1) Congestive heart failure: about 20% of patients with acromegaly have congestive heart failure, have heart palpitations, shortness of breath, lower extremity edema, increased heart sounds, and systolic murmurs in the apical region and pulmonary valve area. Pulmonary examination to obtain wet voice, caused by cardiac hypertrophy and interstitial fibrosis caused by contraction and diastolic dysfunction, especially in the future.

(2) arrhythmia: palpitations, chest tightness, dizziness, severe cases may have syncope, up to 50% of patients with acromegaly can detect abnormal ECG, is related to sinus node and atrioventricular node inflammation and degeneration, and ST segment depression Also more common, with or without T wave abnormalities, left ventricular hypertrophy, indoor conduction abnormalities, especially bundle branch block and supraventricular or ventricular ectopic rhythm, the incidence increases with the course of disease, a group of controls Studies have shown that the acromegaly group has a complex ventricular arrhythmia accounted for 48%, compared with only 12% in the normal group.

(3) Hypertension: more common elderly patients, the incidence rate is 25% to 50%, if the use of 24h blood pressure test records, the detection rate is higher, the disease and the duration of the disease are temporarily related, mostly mildly elevated, And no complications.

Examine

Examination of acromegaly cardiomyopathy

1. Increased growth hormone test: The concentration of serum growth hormone is significantly increased during the daytime, and the upper limit of normal growth hormone radioimmunoassay is 5g/L. Recently, the measurement of urinary growth hormone is a recently developed non-invasive growth hormone determination technique. The patient's urinary growth hormone is 50 to 100 times that of normal people.

2. IGF-I determination in blood is significantly increased: the level of IGF-I in serum can reflect the level of growth hormone, and the half-life of IGF-I is long, the serum concentration is stable, and the normal value of IGF-I is 0.38-26.0 g/ml.

3. Growth hormone releasing hormone assay: The endocrine excess of growth hormone releasing hormone used to identify pituitary growth hormone adenoma and hypothalamus or peripheral causes pituitary growth hormone cell proliferation, both of which have manifestations of acromegaly.

4. Glucose inhibition test: Plasma GH was measured 1 h after oral glucose 100 g, and the value of 10 g/L was diagnosed.

5. CT and MRI: It can effectively detect pituitary abnormalities in patients with acromegaly. Most patients have large pituitary tumors, so pituitary tumors can be found in almost all patients; and one third of patients There is an expansion outside the sella.

6. Electrocardiogram: ST segment rise, there may be T wave abnormalities, left ventricular hypertrophy, strain; indoor conduction block (bundle branch block).

7 echocardiography: ventricular septal asymmetry hypertrophy, left and right ventricular hypertrophy or simple interventricular hypertrophy, left ventricular ejection time (LVET) shortened, pre-ejection (PEP) time prolonged, PEP / LVET ratio increased.

Diagnosis

Diagnosis and diagnosis of acromegaly cardiomyopathy

diagnosis

First, the diagnosis of acromegaly is determined; on this basis, cardiac changes such as cardiac enlargement, arrhythmia, hypertension, atherosclerosis, cardiac insufficiency, etc., are excluded, and other types of heart disease are excluded.

The diagnostic criteria of the New York Heart Association Standards Committee are:

1 diagnosed as acromegaly,

2 The heart has the following manifestations: heart enlargement; hypertension; heart failure; electrocardiogram and echocardiography show left ventricular hypertrophy.

Differential diagnosis

1. Essential hypertension: patients with essential hypertension, the onset is more slow, early asymptomatic, even by physical examination, sometimes headache, vertigo, tinnitus, insomnia, fatigue, palpitations and other symptoms, late Blood pressure continues at a high level, with heart, brain, kidney and other target lesions, but the characteristic clinical signs of acromegaly in patients with essential hypertension, no increase in blood growth hormone, and growth hormone can be glucose Suppressed.

2. Coronary heart disease: Especially need to distinguish from ischemic cardiomyopathy, coronary atherosclerosis, long-term myocardial oxygen supply disorder, myocardial degeneration and necrosis, caused by the replacement of fibrous tissue, the heart can gradually increase, and repeated myocardial infarction, The clinical manifestations are angina pectoris, arrhythmia and/or congestive heart failure, but the characteristic appearance of coronary heart disease without acromegaly and no increase in growth hormone. However, acromegaly may be prone to atheroma due to metabolic disorders. Coronary heart disease can be complicated by sclerosis. In this case, coronary angiography can be identified.

3. Hypertrophic cardiomyopathy: autosomal dominant genetic disease, patients with chest pain, palpitations and labor dyspnea and other symptoms, echocardiography is of great value for diagnosis, showing ventricular septal asymmetry hypertrophy, diastolic ventricular septum The ratio of thickness to posterior wall is greater than 1:1.3. The obstructive outflow tract is narrow, the left ventricular cavity is smaller than normal, the anterior mitral anterior leaf is moved forward during contraction, and the acromegaly hypertensive heart disease has similar hypertrophy. Type of cardiomyopathy, however, can be identified according to the characteristic appearance and elevated growth hormone.

Was this article helpful?

The material in this site is intended to be of general informational use and is not intended to constitute medical advice, probable diagnosis, or recommended treatments.