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and coronary arteries may be involved either singly or in various combinations In most cases the cardiac manifestations are not the dominant feature, but in some it is the primary cause of symptoms and may be fatal The most common type of myocardial involvement is an infiltrative cardiomyopathy, such as systemic amyloidosis, sarcoidosis, hemochromatosis, Fabry s or glycogen storage disease These result in a restrictive cardiomyopathy (see above) Cardiac calcinosis can occur in hyperparathyroidism (usually the secondary form) and in primary oxalosis A number of muscular dystrophies can cause a cardiomyopathic picture (particularly Duchenne s, less frequently myotonic dystrophy, and several rarer forms) Involvement of the heart in Duchenne s dystrophy results in a focal cardiomyopathy of the posterior wall; the classic ECG has prominent anterior precordial forces In addition to LV dysfunction and heart failure, all of these conditions frequently cause conduction abnormalities, which may be the presenting or only feature The myocardium may also be involved in inflammatory and autoimmune diseases It is commonly affected in polymyositis and dermatomyositis, but usually this is subclinical Systemic lupus erythematosus, scleroderma, and mixed connective tissue disease may cause myocarditis, but these more commonly involve the pericardium, coronary arteries, or valves Several endocrinopathies, including acromegaly, thyrotoxicosis, myxedema, and pheochromocytoma, can produce dilated cardiomyopathies that resolve when the underlying disease is appropriately treated Pericardial involvement is quite common in many of the connective tissue diseases Systemic lupus erythematosus may present with pericarditis, and pericardial involvement is not uncommon (but is less frequently symptomatic) in active rheumatoid arthritis, systemic sclerosis, and mixed connective tissue disease Endocardial involvement takes the form of patchy fibrous predominantly on the right side or inflammatory or sclerotic changes of the heart valves Carcinoid heart disease results from the layering of plaque-like material over the tricuspid valve, RV endocardium, and pulmonic valve and presents with right heart failure due to tricuspid and pulmonic regurgitation The hypereosinophilic syndromes involve the endocardium, leading to restrictive cardiomyopathy A variety of arthritic syndromes are associated with aortic valvulitis or aortitis with resulting aortic regurgitation These include ankylosing spondylitis, rheumatoid arthritis, and Reiter s syndrome Disorders of collagen (Marfan syndrome is the most frequent, followed by Ehlers Danlos syndrome) often affect the ascending aorta, with resulting aneurysmal dilation and aortic regurgitation Mitral valve prolapse is also a common finding in these disorders Almost any vasculitic syndrome can involve the coronary arteries, leading to myocardial infarction This is most common with polyarteritis nodosa and systemic lupus erythematosus Two vasculitic syndromes have a particular predilection for the coronary arteries Kawasaki s disease and Takayasu s disease Kawasaki s disease can result in coronary aneurysms, occasionally of huge size Takayasu s disease affects the great vessels more than the coronaries and smooth, tapering lesions are usually seen, particularly at the vessel ostia In these, myocardial infarction may be the presenting symptom.

Before you can proceed with the determination rules, you need to maintain the customer pricing procedure (CuPP) indicator Use the following menu path

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location of the tumor) This is often confused with infective endocarditis, lymphoma, other cancers, or autoimmune diseases In other cases, the tumor may grow to considerable size and produce symptoms by obstructing mitral flow Episodic pulmonary edema (classically occurring when an upright posture is assumed) and signs of low output may result Physical examination may reveal a diastolic sound related to motion of the tumor ( tumor plop ) or a diastolic murmur similar to that of mitral stenosis Right-sided myxomas may cause symptoms of right-sided failure The diagnosis is established by echocardiography or by pathologic study of embolic material Cardiac MRI is useful as an adjunct only Contrast angiography is frequently not necessary Surgical excision is usually curative, though recurrences do occur and serial echocardiographic follow-up, on at least a yearly basis, is recommended The second most common cardiac tumors are valvular papillary fibroelastomas and atrial septal lipomas These tend to be benign and usually require no therapy Other primary cardiac tumors include rhabdomyomas (that often appear multiple in both the RV and LV), fibrous histiocytomas, hemangiomas, and a variety of unusual sarcomas The diagnosis may be supported by an abnormal cardiac contour on radiograph Echocardiography is usually helpful but may miss tumors infiltrating the ventricular wall It is likely that MRI will prove useful as well Metastases from malignant tumors can also affect the heart Most often this occurs in malignant melanoma, but other tumors involving the heart include bronchogenic carcinoma, carcinoma of the breast, the lymphomas, renal cell carcinoma, and, in patients with AIDS, Kaposi s sarcoma These are often clinically silent but may lead to pericardial tamponade, arrhythmias and conduction disturbances, heart failure, and peripheral emboli The diagnosis is often made by echocardiography, but cardiac MRI and CT scanning are also helpful ECG may reveal regional Q waves The prognosis is dismal; effective treatment is not available On rare occasions, surgical resection or chemotherapy is warranted.

Bennett KR et al The Carney complex: unusual skin findings and recurrent cardiac myxoma Arch Dermatol 2005 Jul;141 (7):916 8 [PMID: 16027322] Butany J et al Cardiac tumours: diagnosis and management Lancet Oncol 2005 Apr;6(4):219 28 [PMID: 15811617] Restrepo CS et al CT and MR imaging findings of benign cardiac tumors Curr Probl Diagn Radiol 2005 Jan Feb;34 (1):12 21 [PMID: 15644859] Sun JP et al Clinical and echocardiographic characteristics of papillary fibroelastomas: a retrospective and prospective study in 162 patients Circulation 2001 Jun 5;103(22):2687 93 [PMID: 11390338]

The heart may be involved in a number of systemic syndromes Many of these have been mentioned briefly in this chapter The pericardium, myocardium, heart valves,

Magnani JW et al Myocarditis: current trends in diagnosis and treatment Circulation 2006 Feb 14;113(6):876 90 [PMID: 16476862] Riboldi P et al Cardiac involvement in systemic autoimmune diseases Clin Rev Allergy Immunol 2002 Dec;23(3):247 61 [PMID: 12402411] Steen V The heart in systemic sclerosis Curr Rheumatol Rep 2004 Apr;6(2):137 40 [PMID: 15016344]

Bansal MK et al Myocardial contusion injury: redefining the diagnostic algorithm Emerg Med J 2005 Jul;22(7):465 9 [PMID: 15983078] Wall MJ Jr et al Trauma to cardiac valves Curr Opin Cardiol 2002 Mar;17(2):188 92 [PMID: 11981254]

The management of cardiac disease in pregnancy is discussed in detail in the references listed below Only a few major points can be covered in this brief section

Functions | Pricing | Pricing Control | Define and Assign Pricing Procedures | Define Customer Pricing Procedure [OVKP] In this step one needs to assign a single character alphanumeric key with a short description, as seen in Figure 8-18

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