Diverential Diagnosis of Restrictive Cardiomyopathy and Constrictive
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Heart 2001;86:343–349 Varieties of constrictive pericarditis CARDIOMYOPATHY Heart: first published as 10.1136/heart.86.3.343 on 1 September 2001. Downloaded from x Typical forms DiVerential diagnosis of restrictive –chronic (calcific, rigid shell) cardiomyopathy and constrictive –subacute (non-calcific, elastic) pericarditis x EVusive-constrictive 343 x Localised E William Hancock x Occult Stanford University School of Medicine, Stanford, California, USA predominant clinical feature of ascites, simulat- he diVerentiation of restrictive cardio- ing liver disease, were notable in White’s series. myopathy and constrictive pericarditis Haemodynamic features delineated in the Thas been a perennial problem in clinical 1940s and ’50s included the narrow pulse pres- cardiology. Constrictive pericarditis requires sure in the right ventricle with normal systolic surgical treatment and is usually curable, while pressure and greatly increased diastolic pres- restrictive cardiomyopathy, short of cardiac sure, a prominent early diastolic dip and later transplantation, is treatable only by medical diastolic plateau in right ventricular pressure means and often responds unsatisfactorily. The waveforms, and an additional prominent systo- opinion has often been expressed that there are lic dip in the right atrial waveform, giving a “W” diYcult cases in which only an exploratory atrial waveform. Comments on the difficulty of operation will allow the two conditions to be distinguishing constrictive pericarditis from distinguished. However, such cases were rela- restrictive cardiomyopathy began to appear in tively rare in the past and should be extremely the medical literature only after the pressure so in the present era. Many diVerences exist recordings from cardiac catheterisation began between the two conditions, even though no to be used in the diagnosis of constrictive peri- one diagnostic method can be relied upon to carditis. One may suspect that cardiac catheteri- make the distinction by itself. sation data in the two conditions were more similar than the clinical features. Constrictive pericarditis Since 1960 the clinical profile of constrictive pericarditis has changed greatly. Tuberculous aetiology has become rare in developed coun- Constrictive pericarditis was recognised in the tries, while new aetiologies have appeared. Two 19th century and its surgical treatment was of them, previous cardiac surgery and previous developed early in the 20th century. Paul Wood radiotherapy, are now responsible for up to one noted in 1961 that only details had been added third of cases in some centres.3 The term http://heart.bmj.com/ to the picture presented to the English speaking “chronic” is often no longer included in the title, world by Paul Dudley White in his 1935 St because so many cases are now more appropri- 12 Cyres lecture. White described a “chronic ately considered to be acute or subacute. fibrous or callous thickening of the wall of the Subacute constrictive pericarditis diVers in sev- Correspondence to: pericardial sac that is so contracted that the eral respects from the chronic cases, as Paul E William Hancock normal diastolic filling of the heart is pre- Wood noted in his delineation of the diVerences MD, Stanford vented . There may or may not be calcifica- University Medical between active and inactive tuberculous con- Center, Division of tion ...Parietal pericardium or epicardium strictive pericarditis. A distinction between elas- on October 2, 2021 by guest. Protected copyright. Cardiovascular may be preponderantly involved . one area tic (subacute) and rigid shell (chronic) constric- Medicine, H-2157, 300 may be involved, other areas free . associated tion has been proposed to help to rationalise Avenue Louis Pasteur, heart disease is extremely rare . insidious these diVerences4 (table 1). Other additions to Stanford, CA evolution makes diagnosis more diYcult than the clinical profile include the recognition of 94305-5233, USA 5 whancock@cvmed. that of active constrictive pericarditis”. A eVusive–constrictive pericarditis, occult con- stanford.edu history of several years duration and a striction,6 localised constriction,7 and reversible constriction.8 These variant forms of constric- Table 1 Comparison of certain features in subacute (elastic) and chronic (rigid shell) constrictive pericarditis tive pericarditis each have some features that diVer from the classic chronic constrictive Subacute (elastic) Chronic (rigid shell) pericarditis of the past (table 2). Paradoxical pulse usually present, other signs of Paradoxical pulse usually minimal or absent, interdependence usually prominent other signs of interdependence less prominent Restrictive cardiomyopathy Usually an XY waveform (“M” or “W” waveform) Y is predominant, X sometimes minimal Dip–plateau pattern less conspicuous, because Dip–plateau usually conspicuous, because early diastolic nadir may not approach zero early diastolic nadir often reaches zero As the haemodynamics of constrictive pericar- ditis became known in the 1940s and ’50s it Calcification usually absent Calcification often present quickly became apparent that amyloid and Pericardial eVusion sometimes present, Pericardial eVusion absent. The two layers of other forms of myocardial or endocardial generalised or loculated. Constriction is by pericardium are fused, and jointly constrict disease could have similar haemodynamic fea- the visceral pericardium the heart tures. At the same time, the concept of P waves usually normal P waves often wide, notched and low in amplitude idiopathic cardiomyopathy was evolving, lead- ing to Goodwin’s 1961 classification into three Atrial fibrillation or flutter rare Atrial fibrillation or flutter common types.9 The hypertrophic and dilated forms www.heartjnl.com Education in Heart Table 2 Features of variant forms of constrictive pericarditis Major forms of restrictive cardiomyopathy EVusive–constrictive pericarditis Pericardial eVusion is present, sometimes loculated, with Heart: first published as 10.1136/heart.86.3.343 on 1 September 2001. Downloaded from x Amyloid constriction by the visceral pericardium Occult constrictive pericarditis Haemodynamics are normal at rest, but assume the x Other infiltrative diseases features of constriction after an acute volume load Localised constrictive pericarditis Constriction limited to the right or left ventricle. x Endomyocardial fibrosis Ventricular interdependence reduced or absent 344 Transient constrictive pericarditis During the resolution of acute pericarditis with eVusion, constriction develops, but then resolves spontaneously over x Idiopathic restrictive cardiomyopathy a few weeks 11 quickly became well known, but the third type, years. When the definition is broadened to “constrictive cardiomyopathy” (later renamed permit what is considered to be a lesser degree “restrictive cardiomyopathy”), defined as con- of systolic dysfunction associated with pre- gestive heart failure with neither hypertrophy dominant diastolic dysfunction, diastolic heart nor dilatation, was less common and received failure becomes very common, and perhaps less attention. The definition of restrictive car- includes about one half of all cases of diomyopathy has varied considerably, and the congestive heart failure. It is particularly com- term has usually been used in a broad sense, to mon in elderly patients. Such patients may be include such entities as amyloidosis, tropical referred to as having restrictive cardiomyopa- endomyocardial fibrosis, endocardial fibroelas- thy, even though it appears likely that hyper- tosis, haemochromatosis, and eosinophilic en- tension and increased arterial stiVness are 12 domyocardial disease, as well as the idiopathic background factors in many of them. cases. Idiopathic restrictive cardiomyopathy, Whatever definition of restrictive cardiomy- strictly defined to include normal ventricular opathy is used, it is clear that patients who wall motion as well as normal wall thickness simulate constrictive pericarditis are relatively and ventricular chamber dimensions, proved to rare, and that cardiac amyloidosis is the most be relatively rare. Such a strict definition can be frequent diagnosis among them. The others carried a step further by defining restrictive have miscellaneous diagnoses, with a small cardiomyopathy as those patients who cannot number representing an idiopathic restrictive be diVerentiated from constrictive pericarditis cardiomyopathy. by means of physical examination, chest radio- graph, and cardiac catheterisation.10 No large Differentiating features series of patients with such a condition have been described. On the other hand, the concept of diastolic Table 3 lists 17 features, obtained by eight dif- heart failure, or diastolic dysfunction, has ferent clinical methods, that provide useful received a great deal of attention in the past 20 clues in diVerentiating constrictive pericarditis http://heart.bmj.com/ Table 3 Features useful in diVerentiating constrictive pericarditis from restrictive cardiomyopathy Feature Constrictive pericarditis Restrictive cardiomyopathy Past medical history Previous pericarditis, cardiac surgery, trauma, radiotherapy, These items rare connective tissue disease Jugular venous waveform X and Y dips brief and “flicking”, not conspicuous positive X and Y dips less brief, may have conspicuous A wave or V waves wave Extra sounds in diastole Early S3, high pitched “pericardial knock”. No S4 Later S3, low pitched, “triple rhythm”. S4 in some cases on October 2, 2021 by guest. Protected copyright.