The Heart in Scleroderma

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The heart is one of the major organs involved in scleroderma, the involvement of which can be manifested by myocardial disease, conduction system abnormalities, arrhythmias, or pericardial disease. Additionally, scleroderma renal crisis and pulmonary hypertension lead to significant cardiac dysfunction secondary to damage in the kidney and lung. This article summarizes the types and mechanism of abnormalities in the heart in scleroderma. The concept of cardiac dysfunction in scleroderma and other rheumatologic conditions has received new interest with the advent of newer noninvasive imaging techniques, as well as the interest in detecting subclinical disease. With this increased interest in cardiac manifestations in scleroderma comes the realization that long-term studies are needed to better assess the appropriate screening and treatment in this patient population.

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Clinical presentation

Like any presentation of cardiac disease, the symptoms of patients with cardiac involvement in SSc are varied. In patients presenting with signs of left heart failure, pulmonary congestion, and elevated left heart filling pressures, the most common complaints are dyspnea with exertion, paroxysms of nocturnal dyspnea or orthopnea and, if chronic in nature, possible presentation with ascites and pedal edema. Patients with pulmonary hypertension and subsequent right heart failure usually present

Prevalence and prognosis of cardiac involvement in systemic sclerosis

The presence of cardiac involvement in SSc is often underestimated because of the occult nature of the signs and symptoms, and reports of the prevalence of cardiac disease vary depending on the methods used. Moreover, symptoms of cardiac manifestations are often attributed to noncardiac causes, such as pulmonary, musculoskeletal, or esophageal involvement. More recent studies suggest that clinical evidence of myocardial disease may be seen in 20% to 25% of patients with SSc [4], [6], [7], [8].

Direct myocardial involvement: fibrosis and myositis

Studies of the cardiac manifestations of SSc have been limited for a number of reasons. Most histochemic studies involve autopsy specimens and likely do not reflect the cardiac involvement in patients with subclinical disease. Moreover, imaging studies have been limited because of the fact that there were no endomyocardial biopsies performed to correlated histopathologic fibrosis, hypertrophy, or myositis with imaging techniques. In general, myocardial fibrosis is considered to be the hallmark

Left ventricular systolic and diastolic dysfuction

Left ventricular (LV) systolic dysfunction is not an uncommon finding in advanced scleroderma, but the time course and susceptibility for this is not well understood. Systolic and diastolic dysfunction can occur as a result of myocardial fibrosis, but the role of ongoing low-grade myocarditis in this process is less well characterized. Anecdotally, patients with reduced ejection fraction and normal coronary arteries may benefit from increasing the patient's immunosupression. It has been

Right heart failure

Right heart failure is most commonly the result of pulmonary hypertension. Pulmonary hypertension is a common manifestation of scleroderma and a poor prognostic sign. It is the ability of the right ventricle to function under this increased load that determines both the severity of symptoms and survival [39], [40], [41], [42]. Signs and symptoms of right heart failure by history, echocardiogram, and catheterization are associated with a significantly increased risk of death. In studies

Coronary vasculature and myocardial perfusion

The prevalence of atherosclerotic coronary artery disease is not increased in SSc [4], [6], However, in patients with scleroderma and coronary disease, the likelihood of coronary vasospasm is significantly higher than in the general population. It is possible that coronary involvement in scleroderma is not in epicardial vessels, but rests in small arterial segments. Normal coronary angiograms have been demonstrated in patients with exercise-induced perfusion defects, suggesting that abnormal

Pericardial disease

Pericardial abnormalities in scleroderma have noted fibrinous pericarditis, fibrous pericarditis, pericardial adhesions, and pericardial effusions at the time of autopsy [4]. However, clinically symptomatic pericardial disease (5%–16%) is much less frequent than autopsy-demonstrated pericardial involvement (33%–72%) [9], [11], [14], [22], [31], [48], [51], [52], [53]. Asymptomatic pericardial effusions commonly occur in scleroderma [54]. Moreover, there also have been large effusions causing

Conduction system disease

Conduction defects and arrhythmias are seen frequently in scleroderma patients and are thought to be a result of fibrosis or ischemia of the conduction system [50]. Zakopoulos and colleagues [57] found that there was no difference in the cumulative 24-hour heart rate and blood pressure when comparing scleroderma patients with control subjects, while Wranicz and colleagues [58] found that scleroderma patients had a higher mean heart rate. Depending on the underlying cardiac involvement,

Valvular disease

Prior studies using echocardiography, as well as studies on autopsy samples, have suggested a relatively minor valvular involvement in SSc [4]. Nodular thickening of the mitral valve was shown in 38% of their autopsy subjects with SSc [11]. Shortening of the chordae tendinae of the mitral valve has been noted, as well as mitral and tricuspid valve vegetations in some autopsy samples [11], [31], [52], [53]. Nodular thickening of the mitral and aortic valves with regurgitation and mitral valve

Summary

In summary, while the influence of scleroderma on cardiac function has been known for nearly a century, only recently have we begun to gain a new understanding of the prevalence and prognosis in this patient population. Through new and more refined imaging modalities, as well as more frequent use of invasive hemodynamics, we will be able to better assess patients for subclinical disease and gain new insight as to the long-term prognosis in patients with SSc. Moreover, early detection will allow

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    Dr. Champion is supported in part by the Bernard A. and Rebecca S. Bernard Foundation, a scientist development grant from the American Heart Association, the WW Smith Foundation, and National Institutes of Health grant P50 HL084946.

    Dr. Champion is a Fellow of the American Heart Association and the Pulmonary Vascular Research Institute. He is a recipient of the Zipes Distinguished Young Investigator Award of the American College of Cardiology, the Shin Chun-Wang Young Investigator Award and the Giles F. Filley Memorial Award from the American Physiologic Society.

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