Assessment Amyloidosis
of Relative Sensitivities of Noninvasive in Documented Cardiac Amyloidosis
Tests for Cardiac
Michael Simons, MD, and Jeffrey M. Isner, MD oninvasivediagnosisof cardiac amyloidosisis a considerable diagnostic challenge. A variety of apN proaches including 2-dimensional echocardiography,
the papillary muscle level. Left ventricular mass (muscle cross-sectional area or CSA) was calculated according to the following formula: CSA (cm2/mJ) = computerized tomography, magnetic resonanceimaging, [r(LVEDD/2 + Th)* - r(LVEDD/2)*]/BSA, where electrocardiography and nuclear scintigraphy are used LVEDD = left ventricular end-diastolic pimension, with varying degreesof effectiveness.The present study Th = left ventricular wall thickness, and BSA = body comparesthe relative sensitivity of thesenoninvasivetests surface area. Results of technetium-99m pyrophosphate in 15 patients with proven diagnosis of cardiac amyloi- and diphosphonate scans were reviewed. The degree of myocardial uptake was graded on a I-point scale: 0 = no dosis. To be included in the study, patients had to satisfy I uptake; 1+ = equivocal uptake; 2+ = definite uptake, of the following 3 criteria: (I ) tissue diagnosis of cardiac but less intense than that ofribs; 3+ = intensity equal to that of ribs, but less than that of sternum: 4+ = intensity amyloidosis obtained by transvenous endomyocardial biopsy, (2) tissue evidence of cardiac amyloidosis ob- equal or greater than that of sternum. Clinicalfindings in the 15patients are summarized in tained at necropsy, or (3) catheterization-documented hemodynamic evidence of restrictive cardiomyopathy in Table I, and the results of noninvasive studies are prea patient with extracardiac tissue evidence of sys- sented in Table II. Pathologic Q waves were the most frequently seen electrocardiographic abnormality and temic amyloidosis. Fifteen such patients were identified were present in I4 of 15 (93%) patients. Low voltage was through a retrospective review of medical records. Six were selected on the basis of endomyocardial biopsy, 8 present in 12 patients (80%). M-mode echocardiograms because of necropsyfindings, and 1 because of evidence were available for review in all 15 patients. In addition, of cardiac catheterization. Standard 12-lead electrocar- 11 patients had 2-dimensional echocardiogratis. Ltft ventricular wall thickness was 212 mm in all patients diograms were reviewed in all patients. Voltage in limb and precordial leads was measured, and presence of (mean 15 f 1.8 mmfor the left ventricular free wall, and pathological Q waves was noted. Measurements were 15.1 f 2 mm for the septum). Left ventricular endobtained using the electrocardiogram closest to the time diastolic diameter was normal (K5.5 cm) in all patients. of diagnosis of cardiac amyloidosis or death. M-mode Left ventricular systolic function was normal in all I1 echocardiograms were used for measurements of ven- patients with 2-dimensional echocardiograms available tricular wall thickness and cardiac chamber dimensions. for review. Four patients had a “‘sparkling” appearance of the myocardium. The “‘sparkle” was most pronounced The presence of sparkle on 2-dimensional echocardiograms was assessedin the parasternal short-axis view at in the interventricular septum and less so in the ventricular free wall. There was no correlation between left venFrom the Charles A. Dana Institute and the Harvard-Thomdike Labotricular wall thickness and presence of ‘kparkling” myoratory of the Department of Medicine (Cardiovascular Division), Beth cardium. Thirteen patients had pyrophosphate scans. Israel Hospital, 330 Brookline Avenue, Boston, Massachusetts 02215, Only 3 patients had a significantly (22+) positive myoand the Division of Cardiology, St. Elizabeth Hospital, Boston, Massacardial uptake. There was no correlation between myochusetts. Manuscript received August 20, 1991; revised manuscript received October 10, 199 1, and accepted October 13. cardial thickness (by ultrasound) and myocardial up-
TABLE I Clinical Findings in 15 Patients with Cardiac Amyloidosis Age (yr) Pt.
& Sex.
Amyloid Type
1 2 3 4 5 6 7 8 9 10 11 12 13 14 15
30 M 73 M 69 M 69 M 46 F 63 F 79 M 75 M 48M 64 F 56 M 73 F 47 M 67 M 47 M
AA AA AL/AA AL AL AL AL AL AL AL AL AL AL AL AL
AA = secondaly
Diagnosis by Autopsy Biopsy Autopsy Biopsy Cath. Autopsy Autopsy Autopsy Autopsy Biopsy Biopsy Autopsy Biopsy Biopsy Autopsy
Associated Disease
Clinical Presentation
Pulmonary Congestion
Postural Hypotension
JRA 0 0 0 0 0 0 MM 0 0 0 0 0 0 MM
SBO Neuropathy NS NS NS NS CRF NS NS CHF NS NS Hematuria NS NS
+ + + + + + + + + + + + + + +
+ + + + + + 0 0 + + + 0 + + 0
amyloidosis; AL = primary amyloidosis; Cath. = catheterization; CHF = congestive NS = nephrotic syndrome; SBO = small bowel obstruction.
MM = multiple my&ma;
heart failure; CRF = chronic renal failure; JRA = juvenile
rheumatoid
BRIEF REPORTS
arthritis;
425
TABLE II Results of Noninvasive Studies EGG/LowVoltage
ECG Voltage SV1 + RV5
1 2 3 4 5 6 7 8
+ 0 + + + + 0 +
13 18 7 5 8 10 27 10
9 10 11 12 13 14 15
+ + + + 0 + f
13 7 2 18 17 11 6
Pt.
ECG Q Waves
ECG Conduction
No VI 3, aVF, VI-Z 2,3, aVF Vl Vl-3 Vl
3, aVF, VI 3, aVF, VI-3 Vl-2
2,3, aVF, VI-Z 2,3, aVF, VI-4 3, AVF, VI-Z Vl-2 Vl-4
ECG Arrhythmia
VS (mm)
LVFW (mm)
LVEDD (mm)
LA (mm)
LV Mass (cm2/m?
Sparkle
TC Scan
NI NI NI NI 1”AVB NI LAH NI
0 0 SVT MAT 0 AF AF 0
17 19 16 15 12 17 16 13
16 19 14 15 13 17 16 15
46 37 47 26 35 40 51 45
30 32 36 26 40 32 30 48
17 14 13 11 12 18 21 16
+ 0 0 + 0 0 0 -
0 0 1 0 1 2 -
1”AVB 1”AVB LAH LAH NI NI NI
0 0 AF AF 0 SVT VT, SVT
15 16 14 15 12 15 22
13 16 14 15 12 15 21
33 35 45 47 32 45 42
36 35 38 43 38 50 46
8 13 17 19 20 9 22
0 + + +
0* 3+ 0 0* 0 0* 2
AF = atrial fibrillation; 1”AVB = first-degree atrioventricular block; ECG = electrocardiographic; LA = left atrium; LAH = left anterior hemiblock; LV = left ventricular; LVEDD = left ventricular end-diastolic diameter; LVFW = left ventricular free wall; MAT = multifocal atria tachycardia;, NI = normal; RVs = R wave in lead Vg; SV1 = S wave in lead VI; SVT = supraventriculartachycardia; TC = technetium scan; VS = ventricular septum; VT = ventriculartachycardla. *Technetium-99m diphosphonate scan.
take of pyrophosphate. Similarly, there was no correlation between myocardial sparkle and pyrophosphate uptake. Voltage/mass analysis was performed for each study patient using the electrocardiogram recorded on the day of echocardiographic examination. Voltage was
Voltage/Mass
Relation
30
20 0
0 ”
10
0
Muscle CSA /%A (cm2/m2) FIGURE 1. Electrocardiigraphic voltage is expressed as sum of height of S wave in lead VI (SVl) and of R wave in lead V5 (RIB). Muscle mass is expressed as cross-sectienalbody sur, face area (CSA/BSA) ratio. Box defines patkts in symptomatic amybid group according to criteria of Carrel et al.’
426
THE AMERICAN
JOURNAL
OF CARDIOLOGY
VOLUME
69
calculated as the sum of the S wave in lead VI and the R wave in lead V5 or V6 (whichever was taller), and left ventricular mass was assessed by echocardiography. A diagram developed by Carrol et al1 was used to plot the individual voltage/mass relations. In 12 uatients (SO”roo), the voltage/ma& relation was in the range (musclk CSi >I0 cm2/m2 BSA, voltage similar to that of symptomatic patients with cardiac amyloid in Carrol et al. The patient outside of this range because of a high voltage had history of severe hypertension. The other 2 patients outside of this range had low muscle crosssectional areas.
Diagnosisof cardiac amyloidosisfrequently presentsa challenge to clinicians despite the availability of a number of noninvasivetechniques.A variety of electrocardiographic, echocardiographic and nuclear scintigraphic findings were describedin thesepatients. However, there are few studiesdirectly comparing different techniquesin a well-defined group of patients. In this study we compared electrocardiographic,echocardiographicand scintigraphic findings in 15 patients with tissue or hemodynamic evidence of cardiac amyloidosis. Electrocardiographic documentation of low voltage2is often 1 of the first cluesto the presenceof cardiac amyloidosisand was seenin 80%of patients in the present,study.This finding iBnonspecific,occurring in a variety of conditions including pericardial and pleural effusions, emphysema,myxedema,obesity and coronary artery disease.3Echocardiography in patients with cardiac amyloidosisis frequently reported to showsymmetric left ventricular wall thickening4 and the sparkling appearanceof the myocardium.5 Both of these findings are nonspecific, with the latter describedin Pompe’sdisease,hypoplastic left heart syndrome, hypertrophic cardiomyopathy and chronic renal failure, as well as in left ventricular hypertrophy of any cause.6Myocardial uptake of technetium-99m-labeled pyrophosphatewas proposedas another marker of cardiac amyloidosis.However, results of the presentstudy and
FEBRUARY
1, 1992
similar results reported by Gertz et al7 strongly suggest that technetium pyrophosphate scintigraphy has a very low sensitivity for cardiac amyloidosis. Our results suggest that none of the noninvasiveparametersevaluatedin this study for diagnosisof cardiac amyloidosis are highly sensitive. Thus, low voltage had a sensitivity of 80%, myocardial sparkling 45%, and technetium-99m-pyrophosphateuptake 23%.This low sensitivity, coupledwith the previously mentioned low specificity, makes these findings unattractive for diagnosis of amyloidosis. An alternative approach to noninvasive diagnosis of cardiac amyloidosis was suggestedby Carrol et al’ who noted that patients with cardiac amyloidosistend to have low electrocardiographic voltages and high echocardiographic estimatesof left ventricular mass.When the voltage/mass relation for cardiac amyloid patients is compared with that for patients with aortic valve (high voltage, high mass) or pericardial (low voltage, low mass) disease,clear differences emerge.Plotting voltage/mass relations for the patients in this study on a diagram developed by Carrol et al (Figure 1) would place 12 patients (80%) in a cardiac amyloid range (voltage 10 cm2/m2). This result makes the voltage/mass relation an attractive approach for diagnosisof cardiac amyloidosis.However, there are no reliable estimatesof specificity for this technique.
Simultaneous Thrombolysis
In conclusion,the presentstudy examinedseveralfrequently usedfindings obtained by noninvasivetechniques with regard to their sensitivity for diagnosis of cardiac amyloidosis. Our results suggest that the sensitivity ‘of both myocardial technetium uptake and echocardiographic observation of sparkling are low. However, voltage/mass relation diagrams appear to be a very promising technique for noninvasive diagnosis of these patients. The use and specificity of this technique need further study.
1. Carrel JD, Gaasch WH, McAdam KPWJ. Amyloid cardiomyopathy: characterization by a distinct voltage/mass relation. Am J Cardiol 1982;49:9-13. 2. Roberts WC, Wailer BF. Cardiac amyloidosis causing cardiac dysfunction. Analysis of 54 necropsy patients. Am J Cardiol 1983;52:137-146. 3. Marriot HJL. Practical Electrocardiography. 7th ed. Baltimore: Williams and Wilkins, 1983:19. 4. Borer JS, Henry WL, Epstein SE. Echocardiographic observations in patients with systemic infiltrative disease involving the heart. Am J Cardiol 1977;39: 183-188. 5. Siqueira-Filho ACi, Cuhna CLP, Tajik AJ, Seward JB, Schattenberg TT, Giugliani ER. M-mode and two-dimensional echocardiographic features in cardiac amyloidosis. Circulation 1981;63:188-196. 6. Falk RH, Plenn JF, Deering T, Schick EC, Boinay P, Rubinow A, Skinner M, Cohen AS. Sensitivity and specificity of echocardiographic features of cardiac amyloidosis. Am J Cardiol 1987;59:418-422. 7. Gertz MA, Brown ML, Hauser MF, Kyle RA. Utility of technetium 99m pyrophosphate scanning in cardiac amyloidosis. Arch Intern Med 1987;147: 1039-1044.
Mechanical Clot Fragmentation and Pharmacologic in Acute Massive Pulmonary Embolism
Mohammed Rafique Essop, MCRP, FCP, Shirley Middlemost, FCP, John Skoularigis, MD, and Pinhas Sareli, MD atients with acute massive pulmonary embolism P have a high risk of death within a few hours after the major episode.’ A number of studies have demon-
peripheral intravenous8,gor intrapulmonary9 route. Despite the establishedsuperiority of thrombolytic therapy over heparin anticoagulation for hemodynamically comstrated acceleratedclot lysis using urokinase2,3or strepto- promised patients with massive pulmonary embolism, kinase.4-7More recently tissue-type plasminogenactiva- there remains a group of patients in whom survival detor has been shown to rapidly and, for the most part, pends on more rapid relief of pulmonary vascular obsafely lyse pulmonary emboli when given either by the struction and right ventricular afterload. Surgical pulmonary embolectomyis associatedwith a prohibitive operative mortality and is usually regarded as a treatment of From the Division of Cardiology, Baragwanath Hospital, P.O. Bertlast resort. Becausepulmonary vascular resistance insham 2013, and University of the Witwatersrand, Johannesburg, South creaseswith greater proximity of the thrombus to the Africa. Manuscript received June 11,199l; revised manuscript received and accepted October 7, 199 1. main pulmonary artery, we reasonedthat forceful adTABLE I Clinical and Biochemical Features in Patients with Acute Massive Pulmonary Embolism Pt. No.
Age (yr) & Sex
Interval* (hours)
Symptoms
Predisposing Factors
1 2 3 4 5
31F 40M 47F 38F 21F
18 36 12 6 21
Dyspnea, chest pain Dyspnea, phlebitis Syncope, chest pain Hemoptysis, chest pain Dyspnea
Postpartum lliofemoral thrombosis Calf vein thrombosis Pelvic mass 0
Systemic BP (mm Hg)
PO2lPCO2 (mm Hg)
80160 90/60
60128 52140 63132 59133 65126
601 70140 95165
*Interval = from symptom onset to presentation. Blood gases measured on 40% oxygen. BP = blood pressure; POz/PCOz = oxygen pressure/carbon dioxide pressure.
I
-I
BRIEF REPORTS 427
of Relative Sensitivities of Noninvasive in Documented Cardiac Amyloidosis
Tests for Cardiac
Michael Simons, MD, and Jeffrey M. Isner, MD oninvasivediagnosisof cardiac amyloidosisis a considerable diagnostic challenge. A variety of apN proaches including 2-dimensional echocardiography,
the papillary muscle level. Left ventricular mass (muscle cross-sectional area or CSA) was calculated according to the following formula: CSA (cm2/mJ) = computerized tomography, magnetic resonanceimaging, [r(LVEDD/2 + Th)* - r(LVEDD/2)*]/BSA, where electrocardiography and nuclear scintigraphy are used LVEDD = left ventricular end-diastolic pimension, with varying degreesof effectiveness.The present study Th = left ventricular wall thickness, and BSA = body comparesthe relative sensitivity of thesenoninvasivetests surface area. Results of technetium-99m pyrophosphate in 15 patients with proven diagnosis of cardiac amyloi- and diphosphonate scans were reviewed. The degree of myocardial uptake was graded on a I-point scale: 0 = no dosis. To be included in the study, patients had to satisfy I uptake; 1+ = equivocal uptake; 2+ = definite uptake, of the following 3 criteria: (I ) tissue diagnosis of cardiac but less intense than that ofribs; 3+ = intensity equal to that of ribs, but less than that of sternum: 4+ = intensity amyloidosis obtained by transvenous endomyocardial biopsy, (2) tissue evidence of cardiac amyloidosis ob- equal or greater than that of sternum. Clinicalfindings in the 15patients are summarized in tained at necropsy, or (3) catheterization-documented hemodynamic evidence of restrictive cardiomyopathy in Table I, and the results of noninvasive studies are prea patient with extracardiac tissue evidence of sys- sented in Table II. Pathologic Q waves were the most frequently seen electrocardiographic abnormality and temic amyloidosis. Fifteen such patients were identified were present in I4 of 15 (93%) patients. Low voltage was through a retrospective review of medical records. Six were selected on the basis of endomyocardial biopsy, 8 present in 12 patients (80%). M-mode echocardiograms because of necropsyfindings, and 1 because of evidence were available for review in all 15 patients. In addition, of cardiac catheterization. Standard 12-lead electrocar- 11 patients had 2-dimensional echocardiogratis. Ltft ventricular wall thickness was 212 mm in all patients diograms were reviewed in all patients. Voltage in limb and precordial leads was measured, and presence of (mean 15 f 1.8 mmfor the left ventricular free wall, and pathological Q waves was noted. Measurements were 15.1 f 2 mm for the septum). Left ventricular endobtained using the electrocardiogram closest to the time diastolic diameter was normal (K5.5 cm) in all patients. of diagnosis of cardiac amyloidosis or death. M-mode Left ventricular systolic function was normal in all I1 echocardiograms were used for measurements of ven- patients with 2-dimensional echocardiograms available tricular wall thickness and cardiac chamber dimensions. for review. Four patients had a “‘sparkling” appearance of the myocardium. The “‘sparkle” was most pronounced The presence of sparkle on 2-dimensional echocardiograms was assessedin the parasternal short-axis view at in the interventricular septum and less so in the ventricular free wall. There was no correlation between left venFrom the Charles A. Dana Institute and the Harvard-Thomdike Labotricular wall thickness and presence of ‘kparkling” myoratory of the Department of Medicine (Cardiovascular Division), Beth cardium. Thirteen patients had pyrophosphate scans. Israel Hospital, 330 Brookline Avenue, Boston, Massachusetts 02215, Only 3 patients had a significantly (22+) positive myoand the Division of Cardiology, St. Elizabeth Hospital, Boston, Massacardial uptake. There was no correlation between myochusetts. Manuscript received August 20, 1991; revised manuscript received October 10, 199 1, and accepted October 13. cardial thickness (by ultrasound) and myocardial up-
TABLE I Clinical Findings in 15 Patients with Cardiac Amyloidosis Age (yr) Pt.
& Sex.
Amyloid Type
1 2 3 4 5 6 7 8 9 10 11 12 13 14 15
30 M 73 M 69 M 69 M 46 F 63 F 79 M 75 M 48M 64 F 56 M 73 F 47 M 67 M 47 M
AA AA AL/AA AL AL AL AL AL AL AL AL AL AL AL AL
AA = secondaly
Diagnosis by Autopsy Biopsy Autopsy Biopsy Cath. Autopsy Autopsy Autopsy Autopsy Biopsy Biopsy Autopsy Biopsy Biopsy Autopsy
Associated Disease
Clinical Presentation
Pulmonary Congestion
Postural Hypotension
JRA 0 0 0 0 0 0 MM 0 0 0 0 0 0 MM
SBO Neuropathy NS NS NS NS CRF NS NS CHF NS NS Hematuria NS NS
+ + + + + + + + + + + + + + +
+ + + + + + 0 0 + + + 0 + + 0
amyloidosis; AL = primary amyloidosis; Cath. = catheterization; CHF = congestive NS = nephrotic syndrome; SBO = small bowel obstruction.
MM = multiple my&ma;
heart failure; CRF = chronic renal failure; JRA = juvenile
rheumatoid
BRIEF REPORTS
arthritis;
425
TABLE II Results of Noninvasive Studies EGG/LowVoltage
ECG Voltage SV1 + RV5
1 2 3 4 5 6 7 8
+ 0 + + + + 0 +
13 18 7 5 8 10 27 10
9 10 11 12 13 14 15
+ + + + 0 + f
13 7 2 18 17 11 6
Pt.
ECG Q Waves
ECG Conduction
No VI 3, aVF, VI-Z 2,3, aVF Vl Vl-3 Vl
3, aVF, VI 3, aVF, VI-3 Vl-2
2,3, aVF, VI-Z 2,3, aVF, VI-4 3, AVF, VI-Z Vl-2 Vl-4
ECG Arrhythmia
VS (mm)
LVFW (mm)
LVEDD (mm)
LA (mm)
LV Mass (cm2/m?
Sparkle
TC Scan
NI NI NI NI 1”AVB NI LAH NI
0 0 SVT MAT 0 AF AF 0
17 19 16 15 12 17 16 13
16 19 14 15 13 17 16 15
46 37 47 26 35 40 51 45
30 32 36 26 40 32 30 48
17 14 13 11 12 18 21 16
+ 0 0 + 0 0 0 -
0 0 1 0 1 2 -
1”AVB 1”AVB LAH LAH NI NI NI
0 0 AF AF 0 SVT VT, SVT
15 16 14 15 12 15 22
13 16 14 15 12 15 21
33 35 45 47 32 45 42
36 35 38 43 38 50 46
8 13 17 19 20 9 22
0 + + +
0* 3+ 0 0* 0 0* 2
AF = atrial fibrillation; 1”AVB = first-degree atrioventricular block; ECG = electrocardiographic; LA = left atrium; LAH = left anterior hemiblock; LV = left ventricular; LVEDD = left ventricular end-diastolic diameter; LVFW = left ventricular free wall; MAT = multifocal atria tachycardia;, NI = normal; RVs = R wave in lead Vg; SV1 = S wave in lead VI; SVT = supraventriculartachycardia; TC = technetium scan; VS = ventricular septum; VT = ventriculartachycardla. *Technetium-99m diphosphonate scan.
take of pyrophosphate. Similarly, there was no correlation between myocardial sparkle and pyrophosphate uptake. Voltage/mass analysis was performed for each study patient using the electrocardiogram recorded on the day of echocardiographic examination. Voltage was
Voltage/Mass
Relation
30
20 0
0 ”
10
0
Muscle CSA /%A (cm2/m2) FIGURE 1. Electrocardiigraphic voltage is expressed as sum of height of S wave in lead VI (SVl) and of R wave in lead V5 (RIB). Muscle mass is expressed as cross-sectienalbody sur, face area (CSA/BSA) ratio. Box defines patkts in symptomatic amybid group according to criteria of Carrel et al.’
426
THE AMERICAN
JOURNAL
OF CARDIOLOGY
VOLUME
69
calculated as the sum of the S wave in lead VI and the R wave in lead V5 or V6 (whichever was taller), and left ventricular mass was assessed by echocardiography. A diagram developed by Carrol et al1 was used to plot the individual voltage/mass relations. In 12 uatients (SO”roo), the voltage/ma& relation was in the range (musclk CSi >I0 cm2/m2 BSA, voltage similar to that of symptomatic patients with cardiac amyloid in Carrol et al. The patient outside of this range because of a high voltage had history of severe hypertension. The other 2 patients outside of this range had low muscle crosssectional areas.
Diagnosisof cardiac amyloidosisfrequently presentsa challenge to clinicians despite the availability of a number of noninvasivetechniques.A variety of electrocardiographic, echocardiographic and nuclear scintigraphic findings were describedin thesepatients. However, there are few studiesdirectly comparing different techniquesin a well-defined group of patients. In this study we compared electrocardiographic,echocardiographicand scintigraphic findings in 15 patients with tissue or hemodynamic evidence of cardiac amyloidosis. Electrocardiographic documentation of low voltage2is often 1 of the first cluesto the presenceof cardiac amyloidosisand was seenin 80%of patients in the present,study.This finding iBnonspecific,occurring in a variety of conditions including pericardial and pleural effusions, emphysema,myxedema,obesity and coronary artery disease.3Echocardiography in patients with cardiac amyloidosisis frequently reported to showsymmetric left ventricular wall thickening4 and the sparkling appearanceof the myocardium.5 Both of these findings are nonspecific, with the latter describedin Pompe’sdisease,hypoplastic left heart syndrome, hypertrophic cardiomyopathy and chronic renal failure, as well as in left ventricular hypertrophy of any cause.6Myocardial uptake of technetium-99m-labeled pyrophosphatewas proposedas another marker of cardiac amyloidosis.However, results of the presentstudy and
FEBRUARY
1, 1992
similar results reported by Gertz et al7 strongly suggest that technetium pyrophosphate scintigraphy has a very low sensitivity for cardiac amyloidosis. Our results suggest that none of the noninvasiveparametersevaluatedin this study for diagnosisof cardiac amyloidosis are highly sensitive. Thus, low voltage had a sensitivity of 80%, myocardial sparkling 45%, and technetium-99m-pyrophosphateuptake 23%.This low sensitivity, coupledwith the previously mentioned low specificity, makes these findings unattractive for diagnosis of amyloidosis. An alternative approach to noninvasive diagnosis of cardiac amyloidosis was suggestedby Carrol et al’ who noted that patients with cardiac amyloidosistend to have low electrocardiographic voltages and high echocardiographic estimatesof left ventricular mass.When the voltage/mass relation for cardiac amyloid patients is compared with that for patients with aortic valve (high voltage, high mass) or pericardial (low voltage, low mass) disease,clear differences emerge.Plotting voltage/mass relations for the patients in this study on a diagram developed by Carrol et al (Figure 1) would place 12 patients (80%) in a cardiac amyloid range (voltage 10 cm2/m2). This result makes the voltage/mass relation an attractive approach for diagnosisof cardiac amyloidosis.However, there are no reliable estimatesof specificity for this technique.
Simultaneous Thrombolysis
In conclusion,the presentstudy examinedseveralfrequently usedfindings obtained by noninvasivetechniques with regard to their sensitivity for diagnosis of cardiac amyloidosis. Our results suggest that the sensitivity ‘of both myocardial technetium uptake and echocardiographic observation of sparkling are low. However, voltage/mass relation diagrams appear to be a very promising technique for noninvasive diagnosis of these patients. The use and specificity of this technique need further study.
1. Carrel JD, Gaasch WH, McAdam KPWJ. Amyloid cardiomyopathy: characterization by a distinct voltage/mass relation. Am J Cardiol 1982;49:9-13. 2. Roberts WC, Wailer BF. Cardiac amyloidosis causing cardiac dysfunction. Analysis of 54 necropsy patients. Am J Cardiol 1983;52:137-146. 3. Marriot HJL. Practical Electrocardiography. 7th ed. Baltimore: Williams and Wilkins, 1983:19. 4. Borer JS, Henry WL, Epstein SE. Echocardiographic observations in patients with systemic infiltrative disease involving the heart. Am J Cardiol 1977;39: 183-188. 5. Siqueira-Filho ACi, Cuhna CLP, Tajik AJ, Seward JB, Schattenberg TT, Giugliani ER. M-mode and two-dimensional echocardiographic features in cardiac amyloidosis. Circulation 1981;63:188-196. 6. Falk RH, Plenn JF, Deering T, Schick EC, Boinay P, Rubinow A, Skinner M, Cohen AS. Sensitivity and specificity of echocardiographic features of cardiac amyloidosis. Am J Cardiol 1987;59:418-422. 7. Gertz MA, Brown ML, Hauser MF, Kyle RA. Utility of technetium 99m pyrophosphate scanning in cardiac amyloidosis. Arch Intern Med 1987;147: 1039-1044.
Mechanical Clot Fragmentation and Pharmacologic in Acute Massive Pulmonary Embolism
Mohammed Rafique Essop, MCRP, FCP, Shirley Middlemost, FCP, John Skoularigis, MD, and Pinhas Sareli, MD atients with acute massive pulmonary embolism P have a high risk of death within a few hours after the major episode.’ A number of studies have demon-
peripheral intravenous8,gor intrapulmonary9 route. Despite the establishedsuperiority of thrombolytic therapy over heparin anticoagulation for hemodynamically comstrated acceleratedclot lysis using urokinase2,3or strepto- promised patients with massive pulmonary embolism, kinase.4-7More recently tissue-type plasminogenactiva- there remains a group of patients in whom survival detor has been shown to rapidly and, for the most part, pends on more rapid relief of pulmonary vascular obsafely lyse pulmonary emboli when given either by the struction and right ventricular afterload. Surgical pulmonary embolectomyis associatedwith a prohibitive operative mortality and is usually regarded as a treatment of From the Division of Cardiology, Baragwanath Hospital, P.O. Bertlast resort. Becausepulmonary vascular resistance insham 2013, and University of the Witwatersrand, Johannesburg, South creaseswith greater proximity of the thrombus to the Africa. Manuscript received June 11,199l; revised manuscript received and accepted October 7, 199 1. main pulmonary artery, we reasonedthat forceful adTABLE I Clinical and Biochemical Features in Patients with Acute Massive Pulmonary Embolism Pt. No.
Age (yr) & Sex
Interval* (hours)
Symptoms
Predisposing Factors
1 2 3 4 5
31F 40M 47F 38F 21F
18 36 12 6 21
Dyspnea, chest pain Dyspnea, phlebitis Syncope, chest pain Hemoptysis, chest pain Dyspnea
Postpartum lliofemoral thrombosis Calf vein thrombosis Pelvic mass 0
Systemic BP (mm Hg)
PO2lPCO2 (mm Hg)
80160 90/60
60128 52140 63132 59133 65126
601 70140 95165
*Interval = from symptom onset to presentation. Blood gases measured on 40% oxygen. BP = blood pressure; POz/PCOz = oxygen pressure/carbon dioxide pressure.
I
-I
BRIEF REPORTS 427
