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Instrument No.9319 of 1998

Revocation and Determination

Statement of Principles

concerning

CARDIOMYOPATHY

ICD CODES: 086.0, 425086.0, 425

Veterans’ Entitlements Act 1986

1. The Repatriation Medical Authority under subsection 196B(2) of the Veterans’ Entitlements Act 1986 (the Act):

(a) revokes Instrument No.93 of 1996; and

(b) determines in its place the following Statement of Principles.

Kind of injury, disease or death

2. (a) This Statement of Principles is about cardiomyopathycardiomyopathy and death from cardiomyopathy.

(b) For the purposes of this Statement of Principles, “cardiomyopathy” means a non-inflammatory disorder of heart muscle, other than ischaemic or hypertensive disease, being described as dilated, restrictive or hypertrophic in type, in which the pathological process involves solely the myocardium, or the myocardium and the endocardium, including primary and secondary forms, attracting ICD code 086.0 or 425.

Basis for determining the factors

3. The Repatriation Medical Authority is of the view that there is sound medical-scientific evidence that indicates that cardiomyopathy and death from cardiomyopathy can be related to relevant service rendered

by veterans, members of Peacekeeping Forces, or members of the Forces.

Factors that must be related to service

4. Subject to clause 6, at least one of the factors set out in clause 5 must be related to any relevant service rendered by the person.

Factors

5. The factors that must as a minimum exist before it can be said that a reasonable hypothesis has been raised connecting cardiomyopathy or death from cardiomyopathy with the circumstances of a person’s relevant service are:

(a) being a prisoner-of-war of Japan before the clinical onset of secondary cardiomyopathy; or

(b) for men, drinking at least 250kg of alcohol (contained within alcoholic drinks) within any 10 year period before the clinical onset of secondary cardiomyopathy; or

(c) for women, drinking at least 200kg of alcohol (contained within alcoholic drinks) within any 10 year period before the clinical onset of secondary cardiomyopathy; or

(d) undergoing treatment with anthracyclines within the 12 months immediately before the clinical onset of secondary cardiomyopathy; or

(e) being infected with Human Immunodeficiency Virus (HIV) before the clinical onset of secondary cardiomyopathy; or

(f) suffering from coxsackie myocarditis before the clinical onset of secondary cardiomyopathy; or

(g) suffering from chagasic myocarditis before the clinical onset of secondary cardiomyopathy; or

(h) suffering from Lyme disease myocarditis before the clinical onset of secondary cardiomyopathy; or

(j) suffering from rickettsial myocarditis before the clinical onset of secondary cardiomyopathy; or

(k) suffering from diabetes mellitus at the time of the clinical onset of secondary cardiomyopathy; or

(m) suffering from thyrotoxicosis or hypothyroidism at the time of the clinical onset of secondary cardiomyopathy; or

(n) suffering from sarcoidosis at the time of the clinical onset of secondary cardiomyopathy; or

(o) suffering from total lipodystrophy at the time of the clinical onset of secondary cardiomyopathy; or

(p) suffering from phaeochromocytoma at the time of the clinical onset of secondary cardiomyopathy; or

(q) suffering from systemic lupus erythematosus at the time of the clinical onset of secondary cardiomyopathy; or

(r) suffering from polyarteritis nodosa at the time of the clinical onset of secondary cardiomyopathy; or

(s) suffering from dermatomyositis at the time of the clinical onset of secondary cardiomyopathy; or

(t) suffering from systemic sclerosis at the time of the clinical onset of secondary cardiomyopathy; or

(u) suffering from haemochromatosis at the time of the clinical onset of secondary cardiomyopathy; or

(v) being pregnant or being within the six months immediately postpartum at the time of the clinical onset of secondary cardiomyopathy; or

(w) undergoing a course of therapeutic radiation involving the mediastinum before the clinical onset of secondary cardiomyopathy; or

(x) suffering an idiosyncratic drug reaction which has resulted in the clinical onset of secondary cardiomyopathy; or

(y) suffering clinically apparent thiamine deficiency (beriberi) before the clinical onset of secondary cardiomyopathy; or

(z) for men, drinking at least 250kg of alcohol (contained within alcoholic drinks) within any 10 year period before the clinical worsening of primary or secondary cardiomyopathy; or

(za) for women, drinking at least 200 kg of alcohol (contained within alcoholic drinks) within any 10 year period before the clinical worsening of primary or secondary cardiomyopathy; or

(zb) undergoing treatment with anthracyclines within the 12 months immediately before the clinical worsening of primary or secondary cardiomyopathy; or

(zc) being infected with Human Immunodeficiency Virus (HIV) before the clinical worsening of primary or secondary cardiomyopathy; or

(zd) being infected with coxsackie B virus before the clinical worsening of primary or secondary cardiomyopathy; or

(ze) being infected with the parasite Trypanosoma cruzi before the clinical worsening of primary or secondary cardiomyopathy; or

(zf) being infected with the spirochaete Borrelia burgdorferi before the clinical worsening of primary or secondary cardiomyopathy; or

(zg) suffering from rickettsial myocarditis before the clinical worsening of primary or secondary cardiomyopathy; or

(zh) suffering from diabetes mellitus at the time of the clinical worsening of primary or secondary cardiomyopathy; or

(zj) suffering from thyrotoxicosis or hypothyroidism at the time of the clinical worsening of primary or secondary cardiomyopathy; or

(zk) suffering from sarcoidosis at the time of the clinical worsening of primary or secondary cardiomyopathy; or

(zm) suffering from phaeochromocytoma at the time of the clinical worsening of primary or secondary cardiomyopathy; or

(zn) suffering from systemic lupus erythematosus at the time of the clinical worsening of primary or secondary cardiomyopathy; or

(zo) suffering from polyarteritis nodosa at the time of the clinical worsening of primary or secondary cardiomyopathy; or

(zp) suffering from dermatomyositis at the time of the clinical worsening of primary or secondary cardiomyopathy; or

(zq) suffering from systemic sclerosis at the time of the clinical worsening of primary or secondary cardiomyopathy; or

(zr) suffering from haemochromatosis at the time of the clinical worsening of primary or secondary cardiomyopathy; or

(zs) being pregnant or being within the six months immediately postpartum at the time of the clinical worsening of primary or secondary cardiomyopathy; or

(zt) undergoing a course of therapeutic radiation involving the mediastinum before the clinical worsening of primary or secondary cardiomyopathy; or

(zu) suffering an idiosyncratic drug reaction which has resulted in the clinical worsening of primary or secondary cardiomyopathy; or

(zv) suffering clinically apparent thiamine deficiency (beriberi) before the clinical worsening of primary or secondary cardiomyopathy; or

(zw) inability to obtain appropriate clinical management for cardiomyopathycardiomyopathy.

Factors that apply only to material contribution or aggravation

6. Paragraphs 5(z) to 5(zw) apply only to material contribution to, or aggravation of, cardiomyopathy where the person’s cardiomyopathy was suffered or contracted before or during (but not arising out of) the person’s relevant service; paragraph 8(1)(e), 9(1)(e), 70(5)(d) or 70(5A)(d) of the Act refers.

Other definitions

7. For the purposes of this Statement of Principles:

“a course of therapeutic radiation” means one or more fractions (treatment portions) of ionising radiation administered with the aim of achieving palliation or cure with gamma rays, x-rays, alpha particles or beta particles;

“alcohol (contained within alcoholic drinks)” is measured by the alcohol consumption calculations utilising the Australian Standard of 10 grams of alcohol per standard alcoholic drink;

“being infected with Human Immunodeficiency Virus (HIV)” means serological evidence of infection with Human Immunodeficiency Virus, attracting ICD code 042 or 079.53;

“beriberi” means a state of thiamine deficiency which may be due to decreased thiamine intake, impaired thiamine absorption and storage, and/or accelerated destruction of thiamine diphosphate in the body. The two major manifestations of thiamine deficiency involve the cardiovascular (wet beriberi) and nervous systems (dry beriberi and Wernicke-Korsakoff syndrome). Typically the features are mixed with both systems affected to a greater or lesser extent;

“chagasic myocarditis” means inflammation of the muscular walls of the heart due to infection with the parasite Trypanosoma cruzi, attracting ICD code 086.0;

“coxsackie B virus” means one of a heterogeneous group of viruses of the genus Enterovirus, that in humans has a tendency to affect the meninges and occasionally the cerebrum, but which can cause a wide spectrum of clinical illness;

“coxsackie myocarditis” means myocarditis due to infection with the coxsackie B virus, attracting ICD code 074.23;

“dermatomyositis” means a condition in which characteristic inflammatory skin changes occur in conjunction with a chronic, nonsuppurative, progressive, inflammatory lymphocyte infiltration of skeletal muscle, attracting ICD code 710.3;

“diabetes mellitus” means an endocrine disease characterised by:

(a) a fasting venous plasma glucose concentration equal to or greater than 7.8 millimoles per litre on at least two separate occasions; or

(b) a venous plasma glucose concentration equal to or greater than 11.1 millimoles per litre both within two hours and at two hours after ingestion of 75 grams of glucose,

attracting ICD code 250;

“haemochromatosis” means a genetic disorder of iron storage in which an inappropriate increase in intestinal iron absorption results in the deposition of excessive quantities of iron in parenchymal cells, with eventual tissue damage and functional impairment of the organs involved, especially the liver, pancreas, heart and pituitary, and excluding acquired haemosiderosis, attracting ICD code 275.0;

“hypothyroidism” means the functional state resulting from insufficiency of thyroid hormones, attracting ICD code 243 or 244;

“ICD code” means a number assigned to a particular kind of injury or disease in the Australian Version of The International Classification of Diseases, 9th revision, Clinical Modification (ICD-9-CM), effective date of 1 July 1996, copyrighted by the National Coding Centre, Faculty of Health Sciences, University of Sydney, NSW, and having ISBN 0 642 24447 2;

“idiosyncratic drug reaction” means an abnormal susceptibility to some drug, which is peculiar to the individual. For a drug to be implicated as the cause of an idiosyncratic reaction, for practical purposes it must be recognised as causing such in the medical-scientific literature, as idiosyncratic drug reactions are not dose dependent or involve a consistent temporal relationship. The medical-scientific literature to be consulted should be adequately referenced and include computer databases such as Stat-Ref or standard pharmacology textbooks such The Medical Letter on Drugs and Therapeutics (1988-1996), Basic and Clinical Pharmacology (1998), Ellenhorn Medical Toxicology (1997), Handbook of Adverse Drug Interactions (1996), Physicians GenRx - The Complete Drug Reference (1996), Merck Manual of Diagnosis and Therapy (16th ed. 1992), Goodman and Gilman's The Pharmacological Basis of Therapeutics (9th ed. 1996) and Martindale The Extra Pharmacopoeia (30th ed. 1993). The Australian Adverse Drug Reaction Advisory Committee is also another source of medical-scientific information;

“Lyme disease myocarditis” means myocarditis due to infection with the spirochaete Borrelia burgdorferi, attracting ICD code 088.81 or 422.0;

“myocarditis” means inflammation of the muscular walls of the heart, attracting ICD code 032.82, 036.43, 074.23, 093.82, 130.3, 391.2, 398.0, 422 or 429.0;

“phaeochromocytoma” means a tumour which produces, stores and secretes catecholamines, which are usually derived from the adrenal medulla or chromaffin cells in or about sympathetic ganglia, attracting ICD code 194.0, 227.0, 237.2 or 237.3;

“polyarteritis nodosa” means a systemic, necrotizing vasculitis affecting small and medium-sized arteries, attracting ICD code 446.0;

“primary cardiomyopathy” means a non-inflammatory disorder of heart muscle, being described as dilated, restrictive or hypertrophic in type, in which the pathological process involves solely the myocardium, or the myocardium and the endocardium, arising because of no obvious or known cause and is not part of a disease affecting other organs attracting ICD code 425.0, 425.1, 425.2, 425.3 or 425.4;

“relevant service” means:

(a) operational service; or

(b) peacekeeping service; or

“rickettsial myocarditis” means myocarditis due to infection with Rickettsia rickettsii, attracting ICD code 082.0 or 422.0;

“sarcoidosis” means a chronic, progressive, systemic, granulomatous reticulosis involving almost any organ or tissue and characterized histologically by the presence in all affected organs or tissues, of noncaseating epithelioid cell tubercles, attracting ICD code 135;

“secondary cardiomyopathy” means a non-inflammatory disorder of heart muscle, being described as dilated, restrictive or hypertrophic in type, in which the pathological process involves solely the myocardium, or the myocardium and the endocardium, and which is the result of a known agent, disease process or condition, attracting ICD code 086.0, 425.5, 425.7, 425.8 or 425.9;

“systemic lupus erythematosus” means a connective tissue disease in which cells are damaged by pathogenic autoantibodies and immune complexes, attracting ICD code 710.0;

“systemic sclerosis” means a systemic disorder of the connective tissue characterised by induration and thickening of the skin, by fibrotic degenerative changes in various body organs, attracting ICD code 710.1;

“thyrotoxicosis” means a condition caused by excessive quantities of thyroid hormones, whether the excess results from overproduction by the thyroid gland (as in Graves' disease), originates outside the thyroid, or is due to loss of storage function and leakage from the gland, attracting ICD code 242;

“total lipodystrophy” means an autosomal recessive disorder of lipid storage, characterised by a generalised loss of subcutaneous fat and extracutaneous adipose tissue, attracting ICD code 272.6.

Application

8. This Instrument applies to all matters to which section 120A of the Act applies.

Dated this Twenty-seventh day of March 1998

The Common Seal of the )

Repatriation Medical Authority )

was affixed to this instrument )

in the presence of )

KEN DONALD

CHAIRMAN