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Cardiovascular Manifestations of the Systemic Sclerosis

A. Nikparvar, M.D.
Department of Internal Medicine, SUMS

 Abstract: 

Systemic sclerosis (SS) was first identified over two centuries ago. It is characterized by its skin manifestations. However, cardiac signs were described in 1943 that were results of the underlying vascular involvement or sclerotic involvement of the kidney or lung. Cardiac involvement is a poor prognostic factor, but diagnosis may be late or missing because of the frequent discrepancy between clinical manifestation and cardiac involvement, for this reason, resort to all available diagnostic procedures is recommended to achieve an early diagnosis. Cardiac signs include myocardial fibrosis, myocardial necrosis (ischemia), Conduction abnormalities such as left anterior hemiblock, systolic and diastolic dysfunction, pericarditis and pericardial effusion/tamponade (which is usually secondary to renal involvement). These abnormalities usually present with biventicular congestive heart failure, atrial and ventricular arrhythmias, ischemic heart disease and sudden cardiac death. Antimyosin scintigraphy is a valuable non-invasive method for early detection of clinically silent cardiac involvement in patients with systemic sclerosis, even in the absence of left ventricular dysfunction. In patients with positive antimyosin study, intense pharmacologic treatment with vasodilators may be warranted. Cardiac score is a semiquantitative measure of cardiac involvement that improves prediction of prognosis in systemic sclerosis. Using this method, cardiac involvement assessed yearly by a semi quantitative cardiac scoring technique (the sum of 2 variables, scored 0 or 1 for left axis deviation and 0 or 2 for moderate-large pericardial effusion) and outcome at 10 years were evaluated in 90 systemic sclerosis patients enrolled in a 3 years prospective drug trial. Higher cardiac score was more significantly related to survival than any other individual cardiopulmonary variable. Predicted 6 year survival was 79% in patients with a cardiac score of 0, 51% in those with a score of 1, 15% in those with a score of 2 and 0% in those with a score of 3.

Introduction:

Systemic sclerosis (SS) was first identified over two centuries ago. It is characterized by its skin manifestations, hence the name scleroderma⁽¹⁾. The systemic nature of the disease and in particular its ability to affect the heart become apparent much later. In 1943 Weiss et al described a pattern in the cardiac dysfunction of nine patients with sclerodema and correlated these changes with abnormality in the heart at autopsy in two of the patients. Moreover, they recognized that the cardiac disease was a manifestation of underlying primary vascular disorder⁽¹⁾.

Cardiovascular involvement: 

Cardiovascular disease in patients with SS may be due to either primary involvement of the heart by sclerosing disease or a secondary involvement from disease of the kidney or lungs. Cardiac involvement is a poor prognostic factor, but diagnosis may be late or missing because of the frequent discrepancy between clinical manifestation and cardiac involvement, for this reason, resort to all available diagnostic procedures is recommended to achieve an early diagnosis⁽²⁾.

1- Myocardial involvement is a principal determinant of survival in SS. The fibrosis that occurs bears no direct relation to large or small vessel occlusion or other anatomic abnormalities. The fibrosis tends to be patchy, involving all levels of the myocardium unpredictably and the right ventricle as often as the left. The cause of myocardial necrosis and fibrosis that develops in the setting of patent extramural and intramural vessels is also unclear⁽¹⁾.
 The present evidence suggests that vascular system and particularly the smaller arteries and arterioles are primary target organs of SS, and cardiac sclerosis of scleroderma may be a consequence of focal, intermittent and progressive ischemic injury⁽²⁾. Several functional studies have also suggested that microvascular spasm occurs⁽¹⁾. In 17 patients with cardiac scleroderma and clinical profile of systemic sclerosis, bedside routine investigations, echocardiography, radionuclide ventriculography and computerized stress test both before and after cold pressure test were done. Ten patients had abnormal ECG findings, two patients had mild pericardial effusion and five patients had moderate pulmonary hypertension. Echocardiography and radionuclide ventriculography didn't reveal wall motion abnormality either before or after cold stimulation. Computerized stress test was positive for ischemia following cold stimulation in one patient⁽³⁾.

In another study 19 patients with limited systemic sclerosis (SS) and without any cardiac symptoms were evaluated noninvasively for silent cardiac involvement using electrocardiography, M-Mode, two-dimensional and Doppler echocardiography, resting and post-exercise radionuclide ventriculography. Left anterior hemiblock and mild pericardial effusion were seen in two of patients. The interventricular septal thickness and left ventricular posterior wall thickness were greater in test patients compared with controls. Patients with limited SS also exhibited low early diastolic filling and a low early diastolic to atrial filling ratio (E/A). However, these values didn't correlate with the age of the patients or disease duration. Eight patients (42.1%) were found to have cardiac dysfunction, of which, four had combined systolic and diastolic dysfunction, isolated systolic dysfunction was seen in three patients and isolated diastolic dysfunction in one patient⁽⁴⁾. 

2- Pericardial involvement: pericardial involvement may occur in about 20 percent of patients with SS. Although pericardial involvement is due to renal failure in as many as two-thirds of patients, some patients develop a fibrofibrinous or fibrinous pericarditis for which no other apparent cause is evident. Exudative pericardial effusion may accompany scleroderma pericardial disease and can be massive. Pericardial tamponade may occur and may precede cutaneous thickening. Rarely constrictive pericardial disease may result from pericardial involvement⁽¹⁾.

In a study of frequency of pericardial and pleural effusion in sclerodema it was reported that pericardial effusion do occur in scleroderma without evidence of clinical cardiac dysfunciton and are more common in diffuse scleroderma. Pleuropericarditis in systemic sclerosis may occur on the basis of vasculitis⁽⁵⁾. 

Clinical manifestation:

The clinical features of myocardial SS include biventricular congestive heart failure, atrial and ventricular arrhythmias, myocardial infarction, angina pectoris, sudden cardiac death⁽¹⁾. Three patients with advanced systemic sclerosis and recurrent or incessant monomorphic ventricular tachycardia underwent cardiac electrophysiologic studies. Biventricular transcatheter mapping showed findings most compatible with a reentrant mechanism which was effectively treated with transcatheter ablation.

These clinical manifestations reflect the underlying conditions of myocardial necrosis and fibrosis and may at times mimic ischemic heart disease due to atherosclerosis. If myocardial injury is extensive enough, leading to dilated hypodynamic ventricles, a syndrome resembling idiopathic dilated cardiomyopathy may be simulated. Patients with SS may have cardiac involvement but no cardiac symptoms ⁽¹⁾.

Antimyosin scintigraphy is a valuable non-invasive method for early detection of clinically silent cardiac involvement in patients with systemic sclerosis, even in the absence of left ventricular dysfunction. In patients with positive antimyosin study, intense pharmacologic treatment with vasodilators may be warranted.  Although the course of progressive systemic sclerosis is frequently indolent, it may be fulminant, leading to death before diagnosis is determined⁽⁶⁾. Cardiac score is a semiquantitative measure of cardiac involvement that improves prediction of prognosis in systemic sclerosis. Using this method, cardiac involvement assessed yearly by a semi quantitative cardiac scoring technique (the sum of 2 variables, scored 0 or 1 for left axis deviation and 0 or 2 for moderate-large pericardial effusion) and outcome at 10 years were evaluated in 90 systemic sclerosis (SS) patients enrolled in a 3 years prospective drug trial. Higher cardiac score was more significantly related to survival than any other individual cardiopulmonary variable. Predicted 6 year survival was 79% in patients with a cardiac score of 0, 51% in those with a score of 1, 15% in those with a score of 2 and 0% in those with a score of 3⁽⁷⁾. 
  Several cases reported with cardiac tomponade and limited systemic sclerosis (CREST). In a review of five cases with scleroderma, each of whom had pericardial effusion with an estimated volume of more than 200ml, high protein and LDH and low white blood cell count were the characteristics of pericardial fluid. None of these patients had signs of acute pericarditis. Four of the five cases died within 9mo of diagnosis of pericarditis, two with renal failure, one with cardiac tomponade, and another with sudden death. Pericarditis of diffuse SS especially in cases with anti-topoisomerase-I may present as a chronic form of pericarditis with poor prognosis⁽¹⁾. To determine the frequency and histological characteristics of pericardial involvement in systemic sclerosis, necropsy sections of pericardium from 44 patients with SS were studied. Chronic pericarditis was seen in 31 (77.5%) of cases but in only one of the controls. The characteristic changes of uremic pericarditis wasn't seen. The results indicate that pericarditis is a primary disease (rather than secondary to uremia)⁽⁹⁾.

One report described a patient with limited cutaneous sclerodema in whom calcific constrictive pericarditis was seen⁽⁹⁾. The role of vasodilators and immunosuppressive therapy in cardiac scleroderma needs further investigation⁽¹⁰⁾.

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