Severe Left Ventricular Dysfunction in a Patient with Scleroderma.

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Case report and discussion.

Reprinted with permission from Primary Cardiology, April 1995.

A 53 year old female with long standing history of scleroderma presented with congestive heart failure. She was found to have epicardial coronary artery disease as well as severely decreased systolic left ventricular function. She was referred for dobutamine stress echocardiography. This was done in an attempt to identify whether angioplasty of the epicardial coronary arteries had any role in her management.

The patient was given an incremental infusion of intravenous dobutamine 5, 10, 20 and 30 micrograms per kilogram per minute (we use three minute stages) during echocardiographic and electrocardiographic monitoring. The resting echocardiographic images of the left ventricle revealed diffuse hypokinesis. There was mild improvement in contractility of the lateral left ventricular wall only at the high dose. Unfortunately, the antero septal region (corresponding to the territory of her significantly stenotic left anterior descending artery) remained akinetic. The left ventricular apex, in fact, actually became dyskinetic at peak dobutamine stress. She did not develop chest pain. The ST segments did not change with stress. Based on these findings it was decided to forego the angioplasty. The fact that low dose dobutamine did not elicit any improvement was interpreted as evidence of diffuse myocardial scarring with limited viability.(1)

Progressive systemic sclerosis (scleroderma) is a generalized disorder of connective tissue with thickening and fibrosis of the skin (scleroderma) as well as some involvement of heart, lungs, kidney and gastrointestinal tract. Cardiac involvement has been recognized long before the advent of echocardiography. (2-8) In the heart, any combination of vascular obliteration, fibrosis and inflammation may be present. (9-22) This patient had an extreme degree of left ventricular scarring with severe systolic dysfunction.

Scarring in the form of patchy myocardial fibrosis is present in as many as 81% of these patients. (23) Intermittent ischemia is presumed to be the cause since the histopathologic findings in scleroderma show evidence of myocardial contraction band necrosis. Contraction band necrosis is the pathologic hallmark of transient myocardial ischemia followed by reperfusion.

Raynaud's phenomenon (24) is the familiar scleroderma manifestation of hand ischemia followed by reperfusion. The digital arteries of these patients exhibit marked intimal hyperplasia. Normal vasoconstrictor responses to cold or emotional stimuli, superimposed on the luminal narrowing, could cause complete or near complete vessel occlusion. Similar changes are evident in the small arteries and arterioles of affected organs and may explain certain important clinical syndromes such as scleroderma renal crisis and pulmonary hypertension. (25-28) Myocardial involvement has been documented clinically in the form of both fixed and reversible thallium perfusion abnormalities. (29-30) Dobutamine stress echocardiography may also have clinical utility, as shown in this case. The thallium perfusion abnormalities have been demonstrated to improve in response to calcium channel blocking agents. (31) In this patient however, the vasodilator prescribed was an angiotensin converting enzyme inhibitor. (32) It was given for the left ventricular dysfunction. Although the stress test she underwent consisted of dobutamine infusion, another consideration was to use cold. Transient regional wall motion abnormalities have been demonstrated to occur during cold pressor challenge by both echocardiographic and scintigraphic techniques suggesting a Raynaud like reactivity of the coronary microvasculature.(33-35)

Echocardiography has many uses in the patient with scleroderma. (36-40) This patient did not develop chest pain during the dobutamine stress in spite of proven coronary artery disease. Her dyspnea had both cardiac and pulmonary components. Doppler measurement of her tricuspid insufficiency peak velocity showed moderate pulmonary hypertension which did not worsen during dobutamine stress. She did have a small pericardial effusion. Pericardial disease only causes symptoms in a minority of patients with scleroderma. Asymptomatic pericardial effusions, on the other hand, can be diagnosed in as many as half of patients. The ready availability of echocardiography has resulted in increasing detection of effusions.(41-43) The mechanism producing the effusions in scleroderma is not elucidated but may be different from other rheumatic diseases. The clinical pattern may be either an acute onset of pericarditis or an indolent accumulation of pericardial fluid with few symptoms. A newly diagnosed effusion, especially a large one, may presage the onset of renal failure. Echocardiography remains important as the optimal method for diagnosing the presence and size of pericardial effusion in patients with scleroderma. The addition of Doppler to the echocardiographic examination adds hemodynamic information to the anatomic information. Left ventricular dimensions and indices of central hemodynamics are frequently abnormal. The pulmonary artery systolic pressure can be accurately estimated by measuring the peak velocity of a tricuspid insufficiency jet. Dobutamine infusion allowed assessment of the pulmonary hypertension during stress.

Rare patients with scleroderma may have abnormal left ventricular outflow Doppler patterns indicating dynamic obstruction similar to asymmetric septal hypertrophy. Mitral stenosis, mitral insufficiency, mitral valve prolapse and aortic insufficiency have also been reported. Impaired diastolic rather than the impaired systolic left ventricular function of our patient has also been described.(44-47)


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