Differentiation of Restrictive Cardiomyopathy from Pericardial Constriction: Assessment of Diastolic Function by Radionuclide Angiography
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View metadata, citation and similar papers at core.ac.uk brought to you by CORE provided by Elsevier - Publisher Connector JACC Vol. 13. No. S 1007 April 1989:3007-Id CLINICAL STUDIES Differentiation of Restrictive Cardiomyopathy From Pericardial Constriction: Assessment of Diastolic Function by Radionuclide Angiography CONSTANTINE N. ARONEY, MBBS, FRACP, TERRENCE D. RUDDY, MD, HUMBERTO DIGHERO, MD, MICHAEL A. FIFER, MD. FACC, CHARLES A. BOUCHER, MD. FACC, IGOR F. PALACIOS. MD, FACC Diastolic filling variables were studied in 12 patients with constriction was shorter (110 2 14 ms) than in those with the hemodynamic features of constriction, of whom 5 had restrictive cardiomyopathy (195 +- 45 ms, p < 0.01) or in restrictive cardiomyopathy, 5 had pericardial constriction normal subjects (173 + 32 ms, p < 0.01). The percent of and 2 had combined pericardial constriction and restrictive atria1 contribution to left ventricular filling was higher in cardiomyopathy. The values were compared with those in those with restrictive cardiomyopathy (4.5 f 17%) than in 10 normal subjects of comparable age. The filling fractions those with pericardial constriction (21 + 69, p < 0.05) or between 10% and 70% of the diastolic time interval were in normal subjects (24 + 9%, p < 0.01). Peak filling rate in greater in patients with pericardial constriction than in stroke volumes/s, but not in end-diastolic volumes/s, was those with restrictive cardiomyopathy (p < 0.01 between significantly greater in patients with pericardial constric- 20% and SO%, p < 0.05 at lo%, 60% and 70%), with no tion (5.09 2 0.97) than in those with restrictive cardiomy- overlap. The filling fractions in patients with pericardial opathy (3.52 f 0.43, p < 0.01) or in normal subjects constriction were also greater than those in normal subjects (3.98 f 0.70, p < 0.05). between 10% and 60% of the diastolic time interval. The The pattern of left ventricular filling by radionuclide filling fraction was lower in patients with restrictive cardio- angiography helps differentiate restrictive cardiomyopathy myopathy than in normal subjects at 40% of the diastolic from pericardial constriction. time interval (p < 0.05). (J Am Co11Cardiol1989;13:1007-14) The time to peak filling rate in patients with pericardial Patients presenting with the hemodynamic features of con- (I ,4.5.7-10) however, the hemodynamic features of restric- striction (constrictive physiology*) (1) at cardiac catheter- tive cardiomyopathy may mimic precisely those of restric- ization represent an important diagnostic dilemma in cardi- tive cardiomyopathy. and exploratory thoracotomy may be ology. The differentiation of pericardial constriction from required to examine the pericardium for definitive diagnosis restrictive cardiomyopathy in this situation is critical be- (1). Accordingly, a noninvasive method that reliably distin- cause management and prognosis of these two conditions are guishes pericardial constriction from restrictive cardiomyop- different. The separation of such patients on clinical grounds athy would be useful. The pattern of left ventricular filling in is difficult, and noninvasive techniques have been unreliable patients with pericardial constriction may differ from that of (l-6). Cardiac catheterization and, in particular, right ven- patients with restrictive cardiomyopathy, with the constraint tricular biopsy can be helpful in establishing the diagnosis on filling occurring earlier in diastole with restrictive cardio- myopathy and later in diastole with pericardial constriction. From the Cardiac Unit. Department of Medicine, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts. Dr. Aroney is an Overseas Clinical Fellow of the National Heart Foundation of Australia. Canberra. Australian Capital Territory, Australia. Dr. Fifer is a Clinician- *Constrictive physiology (II: Equalization of the diastolic pressure of the Scientist of the American Heart Association, Dallas. Texas. v,entricles within 5 mm Hg. elevation of the mean atrial or ventricular diastolic Manuscript received July -75, 1988: revised manuscript received October pressure to ~10 mm Hg. “dip-plateau” pattern of the ventricular pressure 25. 1988, accepted November 17. 1988. curve. prominent Y descent in the right atria1 pressure curve. right ventricular Address for reorints: Igor F. Palacios. MD. Cardiac Unit. Massachusetts end-diastolic pressure 210 of right ventricular systolic pressure and Left General Hospital, Boston. Massachusretts 02114. ventricular ejection fraction zO.40. 1008 ARONEY ET AL. JACC Vol. 13, No. 5 DIASTOLIC FILLING BY RADIONUCLIDE ANGIOGRAPHY April 1989:1007-14 Table 1. Clinical, Histologic and Hemodynamic Data in 12 Patients Pt Age (yr)i RVEDP LVEDP Cl No. Gender NYHA RV Biopsy Pericardium (mm Hg) (mm Hg) (literslmin per mj Restrictive Cardiomyopathy 1 40/F III MHIIFIRad Normal 25 29 I so (EchoiThoriNMR) 2 65/M II Myocarditis Normal 11 13 2.14 (Echo) 3 SBIF III Amyloid Normal IO IO 2.40 (Echo) 4 55/F IV MHiIF Normal 16 20 3.90 (Echo/Thor) 5 67iF IV Amyloid Normal 16 16 1.66 (Echo/Thor) Pericardial Constriction 6 58/M III Normal tThor/Echo II 12 3.20 7 71/M III Normal tThoriEcho/NMR 20 22 2.30 8 62/M III Normal +Thor/Echo/NMR 20 20 1.80 9 52/M IV Normal tThor/Cazf II I1 2.50 IO 74/M IV Normal Echo 30 30 2.20 Combined Constriction/Restrictive 11 52/M IV MHIIFIRF tThor/Ca2’ 12 16 2.00 12 37/F III IFiRad tThor/NMR 19 21 2.56 Ca2+ = calcified pericardium on chest X-ray film; Cl = cardiac index: Echo = two-dimensional echocardiography (pericardial thickening); F = female: IF = interstitial fibrosis; LVEDP = left ventricular end-diastolic pressure; M = male; MH = myocyte hypertrophy; NMR = nuclear magnetic resonance imaging: NYHA = New York Heart Association functional class; PCWP = pulmonary capillary wedge pressure; RAP = right atrial pressure; Rad = radiation changes; RF = replacement fibrosis; RV = right ventricular; RVEDP = right ventricular end-diastolic pressure: Thor = thoracotomy; t = positive. We studied 12 patients with constrictive physiology, and fibrillation (Patients 3,9 and 11) with a heart rate at rest < 110 analyzed their diastolic filling patterns at rest, as measured beatslmin. Symptoms were rated according to the New York by radionuclide angiography, to determine whether pericar- Heart Association functional classification for heart failure. dial constriction could be differentiated from restrictive The normal group included five men and five women with cardiomyopathy. The results were also compared with those a mean age of 50 2 10 years (range 35 to 64) who had normal of 10 normal subjects of comparable age. findings on physical examination, electrocardiogram (ECG) and rest and exercise first pass radionuclide ventriculogram and no history of cardiovascular disease. There was no Methods difference in age and heart rate between the constrictive Study patients. Over a 30 month period, all 12 patients physiology and normal groups (Table 2). whose hemodynamic profile met the criteria for constrictive Pericardialconstriction group. The diagnosis of pericar- physiology underwent first pass radionuclide angiography at dial constriction required hemodynamic evidence of con- rest. This group included three patients in whom leg raising strictive physiology, no evidence of a specific form of heart or fluid challenge was performed and diastolic equalization muscle disease on right ventricular endomyocardial biopsy was maintained. Ten normal subjects were also studied. The and pericardial constriction on thoracotomy (Table 1). One constrictive physiology group included five patients with of the five patients (Patient 10) had previous aortic valve pericardial constriction, five with restrictive cardiomyopa- replacement, a normal endomyocardial biopsy and pericar- thy and two with a combination of myocardial fibrosis and dial thickening on echocardiography, but died of sepsis pericardial constriction. The diagnosis in these 12 patients before thoracotomy could be performed. The cause of peri- was made by right ventricular endomyocardial biopsy and cardial constriction in the other four patients was pericardial pericardial evaluation by thoracotomy (Table I). There were constriction after coronary bypass surgery in one, rheuma- seven men and five women with a mean age of 58 + 11 years toid arthritis in one and idiopathic pericardial constriction in (range 37 to 74). Age, gender, diagnosis, result of endomyo- two. Additional corroborative evidence of pericardial dis- cardial biopsy and hemodynamic data are shown in Table 1. ease included the presence of pericardial thickening at Nine of the 12 patients had sinus rhythm, and 3 had atria1 two-dimensional echocardiography, pericardial calcification JACC Vol. 13. No. 5 ARONEY ET AL. 1009 April 1989:1007-14 DLGTOLIC FILLING BY RADIONUCLIDE ANGIOGRAPHY Table 2. Standard Left Ventricular Systolic and Filling Indexes in 12 Patients and 10 Normal Subjects p Value RCM PC Norm RCM v\. PC Norm vs. PC Norm vs. RCM No. 5 IO Age (yr) 63 i- 9 50 2 II NS NS YS HR (beats/mint 76 ? 6 XI 2 I4 NS NS NS LVEF (%I 53 + 6 66 t x NS 10.05 <0.05 LVEDVI (ml/m’) X0? 17 iI I I6 NS NS NS TTPFR (ms) 110 2 14 I73 2 32 <O.Ol 10.01 NS AFC (%‘o) 21 2 6 24 t 9 <0.05 NS co.01 PFR EDVls 1.90 t 0.44 2.58 ? 0.45 I.65 t 0.63 NS NS NS SViS 3.52 2 0.43 5.09 + 0.97 3.98 -+ 0.70 <O.Ol <0.05 NS AFC = atria1 contrtbution to filling; EDV = end-diastolic volumes: HR = heart rate: LVEDVI = left ventricular volume index: LVEF = left ventricular ejection fraction; Norm = normal subjects: NS = not significant: PC = pericardial constriction: PFR = peak filling rate: RCM = restrictive cardiomyopathy: SV = stroke volumes; ‘TTPFR= time to peak filling rate on chest X-ray film and an abnormal thickened pericardium (Baird-Atomic System 77) with the patient at rest. All studies (>4 mm) using magnetic resonance imaging (Table 1) (I I). were performed with the patient upright in the anterior Restrictive cardiomyopathy group. The diagnosis of re- projection.