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PatientPlus articles are written for doctors and so the language can be technical. However, some people find that they add depth to the articles found in the other sections of this website which are written for non-medical people.

The Heart in Systemic Disease

The heart may be affected by diseases that affect the circulation and in doing so, they make greater demands upon the heart. Increased cardiac output is demanded by fever, severe anaemia, thyrotoxicosis and pregnancy. The last is a physiological condition and not a disease. Hypovolaemia will also stress the circulation and its pump. This article does not aim to examine primary diseases of the heart such as congenital heart disease but systemic diseases in which the heart may also be involved. The scope is such that frequent use will be made of links to other articles about those diseases.

Atherosclerosis

This is a very common disease that affects us all to a variable extent as the years go by. It produces coronary artery disease that is the commonest cause of death in developed countries, as well as affecting many other arteries. Atherosclerosis is discussed in its own article.

Coronary arteries may be involved in Kawasaki disease and, very rarely, in late syphilis.

The association between coronary heart disease and diabetes is as well known. It is also well known to be associated with abnormalities of lipid metabolism including the metabolic syndrome.1 Less well known is the strong relationship between CHD and rheumatoid arthritis.2

Hypertension

Hypertensive heart disease is less common now that hypertension is better diagnosed and more effectively treated. It can lead to left ventricular hypertrophy that carries a poor prognosis. There is evidence that ACE inhibitors and AT2 antagonists too, may be able to reverse this.3

Disease of the lungs can also lead to right ventricular hypertrophy and strain.

Rheumatic Fever

Rheumatic fever is now very uncommon in Western Europe, although it was frequent in the earlier part of the 20th century and is still seen in other parts of the world, especially Africa. A complication that is well known and develops some time after the infection, is disease of the mitral valve, the aortic valve, or both. This is usually mitral stenosis or aortic stenosis but mitral regurgitation or aortic regurgitation may occur alone or in combination.

It is less well appreciated that the acute disease is also associated with myocarditis. This myocarditis can be severe. A soft, rumbling, mid-diastolic murmur, called the Carey-Coombs murmur may be heard during active disease. Severe disease is associated with a greater risk of recurrence. The myocarditis leaves the heart weak for the rest of the patient's life and even in the absence of valvular disease, there should be cardiological follow up for life.

Other Causes of Valvular Heart Disease

As rheumatic fever appears to be confined to history, at least in the UK, other causes of disease of heart valves take importance. Many are congenital heart disease.

Any damage or abnormality of the heart or valves makes them susceptible to subacute bacterial endocarditis. Acute bacterial endocarditis can occur when drug addicts inject themselves with heavily infected material.

Pericardial Disease

Acute pericarditis, chronic pericarditis and pericardial effusion are all described in articles of their own. They are often associated with systemic disease. These are often inflammatory diseases or infections. Constrictive pericarditis may impair adequate filling of the heart. Tuberculosis is one possible cause. Pericardial effusion can further constrict the heart and cause cardiac tamponade.

Cardiomyopathy

Primary disease of the heart muscle, rather than coronary artery disease, is called cardiomyopathy. Cardiomyopathy is discussed much more fully elsewhere. They may be primary or due to other disease. Ischaemic heart disease tends to affect older people and so where there is heart failure, arrhythmias or thromboembolism in a young person, cardiomyopathy should be suspected.5 Many systemic diseases may cause cardiomyopathy:
There may be infiltration of the myocardium:

Cardiomyopathy can result from poisons:

  • Alcohol probably requires around 7 or 8 units a day for at least 5 years6 but is probably an underdiagnosed cause and may represent 30% of dilated cardiomyopathy.7 Women are susceptible at a lower dose than men.8 High consumption of alcohol also leads to hypertension.
  • Cobalt was found to induce cardiomyopathy in heavy beer drinkers in the 1960s.9 It is no longer used in beer production.
  • Cardiomyopathy may occur in patients on long term dialysis. Many substances have been implicated.10
  • Cocaine
  • Amphetamines
  • Chemotherapy for malignancy

There are causes related to endocrine disease, infections and malnutrition:

Cardiomyopathy can also occur and be the fatal outcome of myopathies, especially the two X-linked muscular dystrophies:

Various other systemic diseases that may affect the myocardium include:

Systemic Lupus Erythematosis

Systemic lupus erythematosis is a multisystem disease that has its own article. It is associated with pericarditis, hypertension, an increased risk of coronary heart disease and a condition called Libman-Sachs endocarditis. This also has its own record. It is classically described as like a cluster of mulberries on the ventricular surface of the posterior leaflet of the mitral valve and found at postmortem. The leaflet and the chordae tendinae are often adherent to the endocardium of the ventricular wall.

The disease is often missed at echocardiography. The spectrum of the disease has changed since the original description as steroids and other treatment has changed the course of the disease and life expectation, whilst limited, is much higher than it was. The left side of the heart is affected more often than the right but even the endocardium can be affected. Regurgitation is more frequent than stenosis.

Libman-Sachs endocarditis is usually described with SLE but it can also occur in anti-phospholipid syndrome.

Malignant Disease

Cardiac tumours are uncommon. Primary malignancy of the heart is very rare.12 Metastatic spread of malignancy is very rare as shown by unselected autopsy reports.13 The tumour most commonly implicated is malignant melanoma. However, large autopsy studies of patients with disseminated cancer showed intracardiac metastases in approximately 15%.14 These metastases are asymptomatic in the majority of cases. Tumours most likely to metastasise to the heart are malignant melanoma, leukemia, malignant germ cell tumors, and malignant thymoma. Although carcinoma of the lung and breast do not often metastasise to the heart, because of the very high numbers, they account for the greatest numbers of cardiac metastases. Carcinoma of the lung can also cause atrial fibrillation. Intracardiac metastases are more often on the right side of the heart.

The most common clinical presentation is from pericardial effusion, tachy-arrhythmias, atrioventricular block, and congestive heart failure.

Central Nervous System
Renal Disease

Abnormalities of renal function may affect the heart in a number of ways.

  • Renal inadequacy may impair the clearance of drugs that are potentially cardiotoxic such as digoxin.
  • Upset of electrolytes may cause cardiac abnormalities. Hypokalaemia, hyperkalaemia, hypercalcaemia and hypocalcaemia are the most notable but other electrolytes, including magnesium, may be important. Electrolyte disturbance from other causes such as parathyroid abnormalities are just as important.
  • Uraemia may result in pericardial effusion.
  • Long term dialysis may produce cardiomyopathy from various causes.10
  • Renal damage may lead to hypertensive cardiac disease.
Investigations

If there is any suspicion that the heart may be involved in systemic disease, this needs to be investigated or it may become apparent on other investigations:

  • Chest x-ray may show an enlarged heart although it may not be clear if this is due to hypertrophy of the myocardium or dilation of the chambers. It may also indicate heart failure.
  • Interpretation of the ECG is very important.
  • Echocardiography may be valuable.
  • Occasionally, other investigations including MRI scan or doppler flow studies are required.

Document References
  1. Olijhoek JK, van der Graaf Y, Banga JD, et al; The metabolic syndrome is associated with advanced vascular damage in patients with coronary heart disease, stroke, peripheral arterial disease or abdominal aortic aneurysm. Eur Heart J. 2004 Feb;25(4):342-8. [abstract]
  2. Kremers HM, Gabriel SE; Rheumatoid arthritis and the heart. Curr Heart Fail Rep. 2006 Jun;3(2):57-63. [abstract]
  3. Gonzalez A, Lopez B, Diez J; Fibrosis in hypertensive heart disease: role of the renin-angiotensin-aldosterone system. Med Clin North Am. 2004 Jan;88(1):83-97. [abstract]
  4. Maksimowicz-McKinnon K, Mandell BF; Understanding valvular heart disease in patients with systemic autoimmune diseases. Cleve Clin J Med. 2004 Nov;71(11):881-5. [abstract]
  5. Franz WM, Muller OJ, Katus HA; Cardiomyopathies: from genetics to the prospect of treatment. Lancet. 2001 Nov 10;358(9293):1627-37. [abstract]
  6. Piano MR; Alcoholic cardiomyopathy: incidence, clinical characteristics, and pathophysiology. Chest. 2002 May;121(5):1638-50. [abstract]
  7. Lee WK, Regan TJ; Alcoholic cardiomyopathy: is it dose-dependent? Congest Heart Fail. 2002 Nov-Dec;8(6):303-6. [abstract]
  8. Fernandez-Sola J, Nicolas-Arfelis JM; Gender differences in alcoholic cardiomyopathy. J Gend Specif Med. 2002 Jan-Feb;5(1):41-7. [abstract]
  9. Klatsky AL; Alcohol and cardiovascular diseases: a historical overview. Ann N Y Acad Sci. 2002 May;957:7-15. [abstract]
  10. Shik J, Parfrey PS; The clinical epidemiology of cardiovascular disease in chronic kidney disease. Curr Opin Nephrol Hypertens. 2005 Nov;14(6):550-7. [abstract]
  11. Hornberger LK; Maternal diabetes and the fetal heart.; Heart. 2006 Aug;92(8):1019-21. Epub 2006 May 12. [abstract]
  12. Reardon MJ, Walkes JC, Benjamin R; Therapy insight: malignant primary cardiac tumors. Nat Clin Pract Cardiovasc Med. 2006 Oct;3(10):548-53. [abstract]
  13. Majano-Lainez RA; Cardiac tumors: a current clinical and pathological perspective. Crit Rev Oncog. 1997;8(4):293-303. [abstract]
  14. Burke A, Virmani R: Tumors metastatic to the heart and pericardium, in Rosai J (ed): Atlas of tumor pathology.; Washington, DC, Armed Forces Institute of Pathology, 1996, pp 195-209
Acknowledgements EMIS is grateful to the Mentor authoring team for writing this article. The final copy has passed scrutiny by the independent Mentor GP reviewing team. ©EMIS 2007.
DocID: 3897
Document Version: 20
DocRef: bgp24479
Last Updated: 31 Jan 2007
Review Date: 30 Jan 2009






















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PS - Health and Poverty

Perhaps the biggest cause of ill health in the world is poverty. Help to Make Poverty History. For example, why not lend some of your money to disadvantaged communities to enable them to trade their way out of poverty through schemes such as Shared Interest.

See also MAKEPOVERTYHISTORY North East for details and links to campaigns against poverty.

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