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This article is for Medical Professionals

Professional Reference articles are designed for health professionals to use. They are written by UK doctors and based on research evidence, UK and European Guidelines. You may find one of our health articles more useful.

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Treatment of almost all medical conditions has been affected by the COVID-19 pandemic. NICE has issued rapid update guidelines in relation to many of these. This guidance is changing frequently. Please visit https://www.nice.org.uk/covid-19 to see if there is temporary guidance issued by NICE in relation to the management of this condition, which may vary from the information given below.

A wide variety of systemic diseases may affect the heart by a number of different mechanisms, including increasing demands on the heart, causing arrhythmias, affecting the structure of the heart or promoting cardiovascular disease and therefore coronary heart disease.

Common cardiac associations with systemic disease include:

  • Endocrine and metabolic:
  • Nutrition:
    • Malnutrition: dilated cardiomyopathy, heart failure.
    • Thiamine deficiency: high-output heart failure, dilated cardiomyopathy.
    • Hyperhomocysteinaemia: premature atherosclerosis.
    • Obesity: cardiomyopathy, heart failure.
  • Multisystem diseases:
  • HIV infection: myocarditis, dilated cardiomyopathy, pericardial effusion.

Heart failure may be caused or precipitated by any condition that puts a greater demand on the heart - eg, fever, severe anaemia, thyrotoxicosis and pregnancy.

  • 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]
  • There is also a strong relationship between coronary heart disease and rheumatoid arthritis.[2]

Any cause of secondary hypertension, such as renal disease (eg, glomerulonephritis, polyarteritis nodosa, systemic sclerosis, chronic pyelonephritis, or polycystic kidneys), or endocrine disease (eg, Cushing's syndrome, Conn's syndrome, phaeochromocytoma, acromegaly, hyperparathyroidism), may cause hypertensive heart disease, which may lead to left ventricular hypertrophy.

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

Rheumatic fever is now very uncommon in Western Europe, although it is still seen in other parts of the world, especially Africa.[3]

  • Rheumatic fever may cause disease of the mitral valve and/or the aortic valve. This is usually mitral stenosis or aortic stenosis but mitral regurgitation or aortic regurgitation may occur alone or in combination.
  • Acute rheumatic fever is also associated with myocarditis, which 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.

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.

Cardiomyopathy is discussed much more fully in the separate Cardiomyopathies article. They may be primary or due to other disease. Many systemic diseases may cause cardiomyopathy, including:

  • Sarcoidosis.
  • Metabolic: diabetes, amyloidosis, Wilson's disease, haemochromatosis, glycogen storage diseases.
  • Drugs and poisons:
    • Around 7 or 8 units of alcohol (>80g) a day for at least five years are required to develop cardiomyopathy.[5] However, it is probably an underdiagnosed cause and may represent 30% of dilated cardiomyopathy. Women are susceptible at a lower dose than men. High consumption of alcohol also leads to hypertension.
    • Many other substances have been implicated. Examples include cocaine, amphetamines, chemotherapy for malignancy.[6]
  • Cardiomyopathy may occur in patients on long-term dialysis.
  • Endocrine disease: acromegaly, phaeochromocytoma, diabetes mellitus (maternal diabetes can also have an adverse effect on the developing fetal heart),[7] hyperthyroidism, hypothyroidism.
  • Connective tissue disorders: systemic sclerosis (may cause myocarditis or pericardial effusion), rheumatoid arthritis (can cause pericardial effusion, valvulitis and myocardial fibrosis), SLE (is associated with pericarditis, hypertension, an increased risk of coronary heart disease and Libman-Sacks endocarditis).
  • Infections: acute viral infection (especially Coxsackie B), South American trypanosomiasis (Chagas' disease), hepatitis B, HIV infection.
  • Nutritional: malnutrition, vitamin B1 deficiency, obesity.
  • Myopathies: Duchenne muscular dystrophy, Becker's muscular dystrophy.
  • Metastatic spread of malignancy to the heart is far more common than primary cardiac tumours.[8] The most common clinical presentation is from pericardial effusion, tachyarrhythmias, atrioventricular block and congestive heart failure.
  • Tumours most likely to metastasise to the heart are malignant melanoma, leukaemia, malignant germ cell tumours 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 in the absence of metastatic spread to the heart.
  • ECG abnormalities and rhythm disorders often occur in patients with subarachnoid haemorrhage and in cases of ischaemic stroke, intracranial haemorrhage, head trauma, neurosurgical procedures, acute meningitis, intracranial space-occupying tumours and epilepsy.
  • New-onset atrial fibrillation has been reported in up to one third of patients with acute stroke.

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

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. In addition to any other investigation for the suspected underlying disease:

  • Cardiovascular history and examination and clinical assessment of other systems as applicable.
  • Blood tests for myocardial infarction (cardiac enzymes - particularly troponins) and/or heart failure (including brain natriuretic peptide (BNP)).
  • CXR 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.
  • 12-lead ECG.
  • Echocardiography.
  • Other investigations may be indicated - eg, cardiac catheterisation, MRI scan, Doppler flow studies, nuclear cardiology and other cardiac scans.

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Further reading and references

  1. Fahed G, Aoun L, Bou Zerdan M, et al; Metabolic Syndrome: Updates on Pathophysiology and Management in 2021. Int J Mol Sci. 2022 Jan 1223(2). pii: ijms23020786. doi: 10.3390/ijms23020786.

  2. Qiu S, Li M, Jin S, et al; Rheumatoid Arthritis and Cardio-Cerebrovascular Disease: A Mendelian Randomization Study. Front Genet. 2021 Oct 2112:745224. doi: 10.3389/fgene.2021.745224. eCollection 2021.

  3. Lahiri S, Sanyahumbi A; Acute Rheumatic Fever. Pediatr Rev. 2021 May42(5):221-232. doi: 10.1542/pir.2019-0288.

  4. Pan SY, Tian HM, Zhu Y, et al; Cardiac damage in autoimmune diseases: Target organ involvement that cannot be ignored. Front Immunol. 2022 Nov 2213:1056400. doi: 10.3389/fimmu.2022.1056400. eCollection 2022.

  5. Day E, Rudd JHF; Alcohol use disorders and the heart. Addiction. 2019 Sep114(9):1670-1678. doi: 10.1111/add.14703. Epub 2019 Jul 15.

  6. Arenas DJ, Beltran S, Zhou S, et al; Cocaine, cardiomyopathy, and heart failure: a systematic review and meta-analysis. Sci Rep. 2020 Nov 1310(1):19795. doi: 10.1038/s41598-020-76273-1.

  7. Hornberger LK; Maternal diabetes and the fetal heart. Heart. 2006 Aug92(8):1019-21. Epub 2006 May 12.

  8. Butany J, Nair V, Naseemuddin A, et al; Cardiac tumours: diagnosis and management. Lancet Oncol. 2005 Apr6(4):219-28.

  9. Law JP, Pickup L, Pavlovic D, et al; Hypertension and cardiomyopathy associated with chronic kidney disease: epidemiology, pathogenesis and treatment considerations. J Hum Hypertens. 2023 Jan37(1):1-19. doi: 10.1038/s41371-022-00751-4. Epub 2022 Sep 22.

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