The Physiologic and Clinical Similarity Between Primary Amyloid of the Heart and Constrictive Pericarditis

Abstract

When primary amyloid infiltrates the my-oeaidium, there is a loss of distensibility and a resistance to contraction similar to that seen in constrictive pericardlitis. The clinical similarity between these two entities has been noted previously on only a few occasions and only one previous case of amynloid has had catheterization studies. A case of amyloid of the heart is presented with catheterization studies and the reasons for the clinical and physiologic similarity to constrictive pericarlditis are discussed.T HE essential pathology of constrictive pericarditis is the partial replacement of the epicardium and superficial myocardial layers by inelastic fibrous tissue. This interferes with diastolic filling of the ventricles and is recorded during right ventricular catheterization as a high end-diastolic plateau. In addition, the encasing and infiltrating lesion interferes with contractility and thereby reduces, somewhat, the systolic pressure anticipated in relation to the high diastolic filling pressure. This results in a pressure pattern in which the end-diastolic pressure is often greater than one-third the systolic pressure: a finding which has been considered diagnostic by some authors.' The early diastolic dip seen in the right ventricular curve is due to the "drag" effect of the fibrous exterior and its tendency to resist change and to reach the resting state more quickly. It is fairly sharply demarcated because of rapid filling caused by the high pressure within the auricle.Recently it has been shown by Hertzel, Wood and Burchell2 that similar pressure patterns can be found in other conditions and in amyloid of the heart specifically. In 1950, Fisher3 had showni the inability of the amyloid heart to augment its diastolic volume. Clinically this was not a new concept, for Couter and Reichert4 and Findley and Adams5 had noted the clinical

Amyloidosis: Five Cases of Primary Generalized Amyloidosis and Some Other Unusual Cases

“…Gunnar, Dillon, Wallyn, and Elisberg (1955) found that cardiac catheterization studies confirmed the clinical observation of a similarity between the cardiovascular disturbances of myocardial amyloidosis and constrictive pericarditis; they attributed the resemblance to the lowered elasticity of the amyloid myocardium. The later stages of heart failure in the case described here were associated with massive accumulations of fluid in the serosal cavities, more suggestive of a nephrotic state than of cardiac dropsy, particularly as there was persistently heavy proteinuria necropsy showed that there was in fact only slight renal damage.…”

“…FIVE CASES OF PRIMARY GENERALIZED AMYLOIDOSIS distribution of the amyloid deposits was that commonly seen in primary amyloidosis and in the other the picture was that of classical secondary amyloidosis. Amyloidosis is seldom found in association with rheumatic fever or its sequelae, but such cases have been described from time to time, for instance, by Wilks (1856), Beattie (1906), Dillon andEvans (1942), andJackson (1954). Amyloidosis is much oftener seen as a complication of rheumatoid arthritis (Reece and Reynolds, 1954).…”

Cardiac Amyloidosis: Combined Use of Two‐Dimensional Echocardiography and Electrocardiography in Noninvasive Screening Before Biopsy

“…The functional defects can be characterized at cardiac catheterization (Chew et (Couter and Reichert, 1950;Findley and Adams, 1948;Gunnar et al, 1953;Przybojewski el al., 1980;Von Hoyningen-Huene, 1964) and has led to thoracotomy in several cases (Brandt et al, 1968;Brigden, 1964 …”

“…Modern hemodynamic techniques can distinguish the restrictive picture from that of constrictive pericarditis (Meaney et al, 1976;Swanton et al, 1977;Tyberg et al, 1981). However, this differentiation often proved difficult (Couter and Reichert, 1950;Findley and Adams, 1948;Gunnar et al, 1953;Przybojewski el al., 1980;Von Hoyningen-Huene, 1964) and has led to thoracotomy in several cases (Brandt et al, 1968;Brigden, 1964;Chew et af., 1975;Meaney et al, 1976;Roberts and Waller, 1983;Sigueira-Filho et ul., 1981;St. John Sutton et al, 1982).…”

The Function of the Heart in Endomyocardial Fibrosis of the Right Ventricle

“…PARRY AND ABRAHAMS LOW R.V Wood, and Burchell, 1953;Gunnar et al, 1955), in diffuse myocardial fibrosis (Burwell and Robin, 1954), and in cardiomyopathy I > \,~f\J\~ (Goodwin et al, 1961). Evidently the pattern is non-specific, and is of little use for diagnostic purposes.…”