Endocrine diseases

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Acromegaly

Acromegaly and pituitary-dependent human gigantism are associated with markedly increased morbidity and mortality primarily from cardiovascular disease. Untreated acromegaly, identified by its characteristic clinical signs and symptoms and by increased hGH secretion, markedly shortens life expectancy with less than 20 percent of patients surviving beyond 60 years.

Cardiovascular involvement

  • is a chronic insidious process;
  • hypertension, cardiomegaly, congestive heart failure serve as the major events that limit survival.

Echocardiographic features in acromegaly

- up to 2/3 of acromegalic patients have echocardiographic criteria for left ventricular hypertrophy.[1][2];

- in untreated acromegaly, left ventricular diastolic dysfunction accompanies cardiac hypertrophy;

- progressive mitral valve regurgitation and left ventricular strain occur in uncontrolled acromegaly;

- regional myocardial systolic strain [3] abnormalities identified by Doppler imaging reversed with treatment;

- the right ventricle also increases in mass (indicating a more generalized process beyond systemic hypertension;

- asymmetrical septal hypertrophy (initially thought to be common in these patients) is an unusual finding;

- the prevalence of both aortic and mitral valve disease is increased and persists despite disease cure;

- the dilatation of the aortic root may be present;

- vascular changes are common in acromegaly: the arterial intimal thickness is increased.


References

  1. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine, 9th ed; 86: 1829;
  2. Bruch C, Herrmann B, Schmermund A, et al. „Impact of disease activity on left ventricular performance in patients with acromegaly ". Am Heart J 2002; 144:538;
  3. Di Bello V, Bogazzi F, Di Cori A, et al. „Myocardial systolic strain abnormalities in patients with acromegaly: A prospective color Doppler imaging study”. J Endocrinol Invest 2006; 29:544


Cushing Disease/Syndrome

Excess cortisol secretion and its attendant clinical disease states can arise either from excess pituitary release of ACTH (Cushing disease) or through the adenomatous or rarely malignant neoplastic process arising in the adrenal gland itself (Cushing syndrome).

Cardiovascular involvement

  • Cardiovascular complications of Cushing’s syndrome cause a mortality rate that is fourfold higher than that expected in the normal population. Cortisol hypersecretion causes central obesity, hypertension, impairment of glucose tolerance or diabetes, hyperlipidemia, and a pro-thrombotic state.
  • The increased cardiovascular morbidity and mortality can be explained by cerebrovascular disease, peripheral vascular disease, coronary artery disease with myocardial infarction, and chronic congestive heart failure [1].

Echocardiographic features

- left ventricular hypertrophy (echocardiographic findings of severe left ventricular hypertrophy or hypertrophic cardiomyopathy without obvious cause should raise the clinical suspicion of glucocorticoid excess, possibly from Cushing's syndrome);

- left ventricular concentric remodeling can be observed;

- left ventricular impaired contractility; midwall fractional shortening is reduced;

- diastolic left ventricular dysfunction with abnormal relaxation pattern (the ratio of E/A velocities is reduced and the E-wave deceleration is prolonged in Cushing patients)[2]

- dilated cardiomyopathy;

- cardiac myxoma (as Carney complex: Cushing syndrome, cardiac myxoma, and a variety of pigmented dermal lesions), most commonly occurring in the left atrium.


References

  1. Boscaro M, Barzon L, Fallo F, Sonino N. „Cushing’s syndrome”. Lancet. 2001;357:783–791
  2. Muiesan ML, Lupia M, Salvetti M, et al. „Left ventricular structural and functional characteristics in Cushing's syndrome”. J Am Coll Cardiol 2003; 41:2275


Hyperaldosteronism

Primary hyperaldosteronism, or Conn syndrome, a common curable disease of hypertension, is characterized by inappropriate production of aldosterone, regulated in part independently of the renin-angiotensin system. Idiopathic bilateral adrenal hyperplasia and aldosterone-producing adenomas are the leading causes of primary hyperaldosterism.

Cardiovascular involvement

Many of the changes in the heart and cardiovascular system in hyperaldosteronism result from the associated hypertension. Primary hyperaldosteronism is one of the most frequent cause of secondary hypertension.

Echocardiographic features

Left ventricular wall thickening without asymmetric septal hypertrophy is a feature of primary hyperaldosteronism. Patients with primary hyperaldosteronism (Conn syndrome) may also have :

- thicker interventricular septal thickness
- left ventricular posterior wall thickness
- higher left ventricular mass index [1]
- left ventricular concentric remodeling [1]
- the E wave flow velocity and the E/A ratio are lower in patients with primary hyperaldosteronism compared with essential hypertension patients [2]

Both left ventricular hypertrophy and left ventricular diastolic dysfuntion have been shown to be independent predictors of cardiovascular events.

References

  1. 1.0 1.1 Lin YH, Lee HH, Liu KL et al. „Reversal of myocardial fibrosis in patients with unilateral hyperaldosteronism receiving adrenalectomy”. Surgery. 2011 Sep;150(3):526-33
  2. Rossi G P, Sacchetto A, Visentin P, et al. „Changes in Left Ventricular Anatomy and Function in Hypertension and Primary Aldosteronism.Hypertension”.Hypertesion 1996;27:1039-1045


Addison Disease

Adrenal insufficiency (Adisson disease) most commonly arises from bilateral loss of adrenal function on an autoimmune basis, as a result of infection, hemorrhage, or metastatic malignancy. In contrast, secondary adrenal insufficiency, which results from pituitary-dependent loss of ACTH secretion, leads to a fall in glucocorticoid production, whereas mineralocorticoid production including aldosterone remains at relatively normal levels.

Cardiovascular involvement

  • tachycardia, hypotension, and electrolyte abnormalities herald impending cardiovascular collapse and crisis

Echocardiographic features

→ reduced left ventricular end-systolic dimensions
→ reduced left ventricular end-diastolic dimensions
→ sometimes, echocardiographic signs of mitral valve prolapse at the anterior leaflet may be observed (because of a disproportion between the mitral valve and left ventricular chamber in these patients, concept of volume - induced valvular - ventricular disproportion [1])


References

  1. Fallo F, Betterle C, Budano S et al. „Regression of cardiac abnormalities after replacement therapy in Addison’s disease”. European Journal of Endocrinology 1999;140: 425–428


Hyperparathyroidism

Classical primary hyperparathyroidism producing hypercalcemia most often arises as a result of the adenomatous enlargement of one of four parathyroid glands. About 1% of the adult population has been suggested to be affected, and there is an undisputed female predominance and increase in prevalence with increasing age of both genders.

Cardiovascular involvement

The major cause of death in primary hyperparathyroidism patients is cardiovascular disease. Hypercalcemia has been linked to pathological changes in the heart including the myocardial interstitium, the conducting system, and calcific deposits in the valve cusps and annuli.

Echocardiographic features

- a high incidence of left ventricular hypertrophy (a powerful predictor of cardiovascular mortality). Partial regression of left ventricular hypertrophy may occur 6 months to 1 year after parathyroidectomy in these patients [1];

- cardiac calcific deposits in the myocardium;

- aortic and mitral valve calcification;

- severe or chronic disease may impair diastolic function [2]; a reduced transmitral valve E/A ratio by pulsed Doppler echocardiography has been reported in patients with primary hyperparathyroidism and suggests that left ventricular diastolic dysfunction may be a feature of this disease (the diastolic dysfunction has been related to decreased compliance from calcium deposition in the myocardium, decreased mitochondrial energy production and chronic calcium excess in the myocytes;

- left ventricular systolic function does not seem to be affected in patients with primary hyperparathyroidism [2].

References

  1. Näppi S, Saha H, Virtanen V, et al. „Left ventricular structure and function in primary hyperparathyroidism before and after parathyroidectomy”. Cardiology 2000;93:229-233
  2. 2.0 2.1 Andersson P, Rydberg E, Willenheimer R. „Primary hyperparathyroidism and heart disease—a review”. Eur Heart J 2004; 25:1776


Hyperthyroidism

High circulating levels of thyroid hormones can alter almost all organ systems of the body.

Cardiovascular involvement

  • Cardiovascular symptoms are often the predominant clinical presentation of patients with hyperthyroidism.
  • Thyroid hormone have positive chronotropic and inotrophic effects on the heart, with the result that in hyperthyroidism cardiac output and cardiac rate rise.
  • Hyperthyroid cardiovascular system is highly stressed at rest and its functional reserve is reduced.
  • The importance of the recognition of the effects of thyroid disease on the heart also derives from the observation that restoration of normal thyroid function most often reverses the abnormal cardiovascular hemodynamics.

Echocardiographic features in hyperthyroidism

• at rest, the stroke volume index, ejection fraction, and cardiac index are significantly increased;

• hyperkinetic left ventricular wall motion is noted;

• using stress echocardiography, exercise capacity and global left ventricular function can be assessed noninvasively;

• during exercise, the left ventricular ejection fraction may actually fall [1], but despite this apparently pathological response, frank congestive heart failure is not common in hyperthyroidism, unless the patient has underlying cardiac disease;

• thyroid hormones enhance the diastolic relaxation rate, which contributes to the increase in left ventricular end-diastolic volume;

• the E/A ratio is lower in patients with overt hyperthyroidism (consequence of the increased left ventricular stiffness)[2];

• findings of cardiac hypertrophy;

• congestive heart failure however is very rare and, when present, is generally associated with atrial fibrillation, more advanced age, persistent systolic left ventricular dysfunction [3];

• pulmonary hypertension has been associated with hyperthyroidism (autoimmune disease may play a role in this);

Graves’ and Hashimoto’s diseases are reported to be associated with an increased prevalence of mitral valve prolapse (which in turn may predispose to enlargement of the left atrium and atrial fibrillation)[4].


References

  1. Forfar JC, Muir AL, Sawers SA et al. „Abnormal left ventricular function in Hyperthyroidism: evidence for a possible reversible cardiomyopathy”. N eng J Med. 1982; 307: 1165-1170
  2. Mario Petretta, Domenico Bonaduce, Letizia Spinelli,et al. „Cardiovascular haemodynamics and cardiac autonomic control in patients with subclinical and overt hyperthyroidism”. European Journal of Endocrinology (2001) 145 691-696
  3. Siu CW, Yeung CY, Kung AW, Tse HF. „Incidence, clinical characteristics and outcome of congestive heart failure as the initial presentation in patients with primary hyperthyrpoidism”. Heart 2007, 93:483-7
  4. Evangelopoulou ME, Alevizaki M, Toumanidis S,et al. „Mitral valve prolapse in autoimmune thyroid disease: an index of systemic autoimmunity?”Thyroid 1999; 9: 973–977


Hypothyroidism

It is a common endocrine disorder resulting from deficiency of thyroid hormones.

Cardiovascular involvement

  • The most common cardiovascular signs and symptoms of hypothyroidism may include bradycardia, mild hypertension (diastolic), narrowed pulse pressure, cold intolerance, and fatigue.
  • It is less well recognized that hypothyroidism can predispose to ventricular dysrhythmias.
  • The cardiovascular effects manifestations of hypothyroidism include the following:
♦ Decreased total volume;
♦ Decreased contractility;
♦ Decreased heart rate;
♦ Increased systemic vascular resistance (leading to increased diastolic blood pressure);
♦ Increased capillary permeability (leading to pericardial effusion).

As an non-invasive method, echocardiography can play important role in recognizing the cardiac pathology as well as to follow up effect of the therapy.

Echocardiographic features in hypothyroidism

• a relatively high incidence of asymmetrical septal or concentric left ventricular hypertrophy (interventricular septal dimensions may be significantly raised in moderate subclinical and in severe overt hypothyroidism);
• in elderly patients with hypothyroidism there are described low systolic function indices, but no such alteration in systolic function in younger patients (aged 20- 48 years);
• left ventricular systolic dysfunction in the absence of any other underlying cardiac disease is uncommon in these patients;
• in subclinical hypothyroidism, patients exhibit resting left ventricular diastolic dysfunction evidenced by delayed relaxation, and impaired systolic function on effort that results in poor exercise capacity [1];
• impaired diastolic function of hypothyroid patients is manifested by prolongation in isovolumetric relaxation time and reduction in early diastolic posterior wall thinning rate. These abnormalities can be assessed by Doppler and M-mode echocardiography;
pericardial effusion is reported to occur in 30 % to 80% of patients with hypothyroidism. Pericardial effusion is a common cardiac manifestation in hypothyroid subjects and all patients with unexplained pericardial effusion should be screened for hypothyroidism. Echocardiography is useful in assessing the response to replacement therapy in patients with pericardial effusion [2]. Substernal goiters rarely cause pericardial effusions [3]. Cardiac tamponade due to pericardial effusion from hypothyroidism is rare;
• right ventricular wall thickness usually have no changes;
• most of cardiac manifestations are reversible with adequate and timely replacement therapy with thyroxine (T4)

References

  1. Rodondi N, Aujesky D, Vittinghoff E, Cornuz J, Bauer D.C. „Subclinical hypothyroidism and the risk of coronary heart disease: a meta-analysis”. Am. J. Med. 2006;119 (7):541-551
  2. Rawat B, Satyal A. „An echocardiographic study of cardiac changes in hypothyroidism and the response to treatment”. Kathmandu University Medical Journal (2003) Vol. 2, No. 3, Issue 7, 182- 187
  3. Anders HJ. „Compression syndromes caused by substernal goitres”.Postgrad Med J. 1998 Jun;74(872):327-9


Pheochromocytoma

Pheochromocytomas are primarily benign tumors arising from neuroectodermal chromaffin cells primarily within the adrenal medulla and abdomen, but they may arise anywhere within plexi of sympathetic adrenergic nerves. Primary cardiac pheochromocytoma is very rare. When pheochromocytoma coexists with medullary thyroid carcinoma or occasionally with hyperparathyroidism, it is a designated multiple endocrine neoplasia (MEN) syndrome type II. Clinical presentation: headache, palpitations, excessive sweating, tremulousness, chest pain, weight loss, and a variety of other constitutional complaints. Hypertension may be episodic but is most commonly constant and is paradoxically associated with orthostatic hypotension on arising in the morning.

Echocardiographic findings

→ left ventricular hypertrophy (symmetric thickening of left ventricular walls); left ventricular hypertrophy simulating hypertrophic obstructive cardiomyopathy is a rare complication of pheochromocytoma;
→ catecholamine-induced myocarditis;
→ dilated cardiomyopathy.

¤ Pheochromocytoma should be included as one of possible causes of transient left ventricular systolic dysfunction [1].

¤ The increased incidence of diastolic dysfunction may be associated with injury of myocytes or increased oxidative stress by circulating catecholamines.


References

  1. Park JH, Kim KS, , Sul JY et al. „Prevalence and Patterns of Left Ventricular Dysfunction in Patients with Pheochromocytoma”.J Cardiovasc Ultrasound. 2011 June; 19(2): 76–82

Carcinoid syndrome

Carcinoid syndrome is an endocrinologic syndrome resulting in release of active metabolites of serotonin and tryptophan that have a toxic effect on the endothelium of the heart.

Echocardiographic findings

  • The classic abnormality in this syndrome is diffuse thickening and immobility of the tricuspid and less commonly the pulmonary valve.
♦ the tricuspid valve is thickened and retracted, with limited mobility, and coaptation is incomplete, resulting in severe tricuspid regurgitation. The severe tricuspid regurgitation produces a pattern of right ventricular volume overload, with an enlarged right ventricular and ventricular septal motion abnormalities.
♦ the pulmonary valve, whose increased thickness and retraction result in pulmonic stenosis, is occasionally difficult to image with transthoracic echocardiography. The transesophageal longitudinal-axis view of the right ventricular outflow tract may be needed to visualize the pulmonary valve.
♦ Doppler recordings from the tricuspid and pulmonic valves are characteristic for severe tricuspid regurgitation and a rapid increase in right ventricular diastolic pressure.
  • The subcostal view is very important for identifing liver metastases, and the hepatic vein Doppler recording demonstrates marked systolic flow reversal in severe tricuspid regurgitation.
  • A metastatic tumor may be seen in the myocardium, involving the RV in 40% of cases, the LV in 53%, and the ventricular septum in 7% [1].


References

  1. Pandya UH, Pellikka PA, Enriquez-Sarano M et al. „Metastatic carcinoid tumor to the heart: Echocardiographic-pathologic study of 11 patients”. J Am Coll Cardiol. 2002 Oct 2; 40(7):1328-32


Diabetes mellitus

„A metabolic disorder of multiple etiology, characterized by chronic hyperglycaemia with disturbances of carbohydrate, fat and protein metabolism resulting from defects in insulin secretion, insulin action, or both. The effects of diabetes mellitus include long-term damage, dysfunction and failure of various organs” [1].

Cardiovascular involvement

  • Abundant evidence shows that patients with type 1 diabetes or type 2 diabetes are at high risk for several cardiovascular disorders: coronary heart disease, stroke, peripheral arterial disease, cardiomyopathy, and congestive heart failure.
  • Cardiovascular complications are the leading causes of diabetes-related morbidity and mortality.

Echocardiographic findings

♦ the presence of a dilated cardiomyopathy specifically due to diabetes mellitus was first suggested by Rubler in 1972 [2];
left ventricular diastolic dysfunction:
- several studies confirm an association between diabetes mellitus and [3] (apparently an earlier sign of diabetic cardiomyopathy);
- diastolic dysfunction in diabetes may consist of impaired left ventricular relaxation, increased compliance, or both conditions;
- in diabetic persons, as compared with control subjects, pulsed Doppler echocardiography of mitral valve inflow has shown decreased peak early inflow velocity, decreased peak early to peak late filling velocity (E/A) ratio, increased pressure half-time, increased atrial contribution to filling and prolonged isovolumetric relaxation time;
left ventricular systolic dysfunction:
- although a number of studies have confirmed the association of left ventricular systolic dysfunction with diabetes mellitus, this finding has not been uniformly reported [4];
- many of those who have normal LV systolic function at rest may show abnormalities during exercise or dobutamine stress [5] indicating that LV systolic reserve is reduced in these patients;
- more sensitive techniques for systolic assessment such as strain, strain rate, and myocardial tissue Doppler velocity can detect preclinical systolic abnormalities in diabetic patients [6];
- in young asymptomatic diabetic patients, abnormalities of left ventricular shortening fraction and velocity of circumferential fiber shortening measured by M-mode echocardiography may become evident immediately after exercise;
♦ myocardial hypertrophy, interstitial collagen accumulation, and fibrosis occur in diabetic cardiomyopathy and may partially account for increased compliance;
♦ left ventricular hypertrophy is a feature of diabetes mellitus and can be assessed by M-mode and two-dimensional echocardiography.


References

  1. „Definition, diagnosis and classification of diabetes mellitus and its complications”. Report of a WHO Consultation. Part 1: diagnosis and classification of diabetes mellitus. Geneva, World Health Organization, 1999 (WHO/NCD/NCS/99.2)
  2. Rubler S, Dlugash J, Yuceoglu YZ et al. „New type of cardiomyopathy associated with diabetic glomerulosclerosis”. Am J Cardiol 1972. 30(6):595-602
  3. Schannwell CM, Schneppenheim M, Perings S et al. „Left ventricular diastolic dysfunction as an early manifestation of diabetic cardiomyopathy”. Cardiology 2002. 98(1-2):33-39
  4. Fang ZY, Prins J B, Marwick TH. Diabetic Cardiomyopathy: Evidence, Mechanisms, and Therapeutic Implications. Endocrine Reviews, August 2004, 25(4):543–567
  5. Vered A, Battler A, Segal P, et al. „Exercise-induced left ventricular dysfunction in young men with asymptomatic diabetes mellitus (diabetic cardiomyopath)”. Am J Cardiol 1984;54:633–637
  6. Fang ZY, Yuda S, Andersen et al.„Echocardiographic detection of early diabetic myocardial disease”. J Am Coll Cardiol 2003; 41:611–617
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