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Pericardial tamponade, a new complication of amyloid heart disease
Pericardial Tamponade, a New Complication of Amyloid Heart Disease
SCOTT BRODARICK, M.D. ROBERT PAINE, M.D., F.A.C.C. ENRIQUE HIGA, M.D. KIM A. CARMICHAEL, M.D. St. Louis, Missouri
With advancing age of the population and with echouardlographic means of diagnosls, amyloid dlsease of the heart is of increasing clinical interest. Advanced age, restrictive myocardiopathy, arrhythmias, and conduction disorders are familiar features of this disease. A 92 year old man with past history of hemibtock followed by complete heart block and transvenous pacemaker was admitted to the hospital because of increastng fatigue and the abrupt development of dyspnea. Examination revealed paradoxid pulse, markedly elevated central venous pressure, and echocartltographically demonstrated large perlcardial effusion. Shortly after admission signs of tamponade developed; 1,000 ml of perkaldial fluid was removed wlth prompt relief of dyspnea and disappearance of paradoxk pulse and return of central venous pretie to normal. However, dyspnea soon recurred and subsequent hemodynamic measurements indicated increased right ventricular and left ventricular filling pressures. Echocardiography reveated no recurrent effusion or ventrkular hypokinesis. Left ventricular ejection fraction by radionuclide ventricutogram was 64 percent. Echocardiography revealed ventricular wafi thickenlng, normal chamber size, and glittering, sparkling myocardial echoes. On po&mortsrtt examination, there was extensive myocardial amytotdo&. There was no evktence of constrictive pericardttis or recurrent effudon. The unique aspect of this case was the combined presence of restricttve myocardiopathy and pericardial tamponade. To our knowledge, no previous case of tamponade due to amyloid heart disease has been reported. Increasing frequency of amyloid heart disease in our aging population and echocardiographic means of diagnosis have heightened clinical awareness of this disorder. Its pathophysiology and farms of clinical presentation have been elucidated. Our purpose is to describe a manifestation of amyloid heart disease that has not been previously reported. CASE REPORT
From the Department of Internal Medicine and Pathology,St. L*e’s Hospital,St. Louis,Missouri. Requestsfor reprintsshouldbe addressedto Dr. RobertPaine, 5505 Delrnar Blvd.,St. Louis,Missouri63 112. Manuscriptaccepted on November 4, 1961.
A 92 year old practicinglawyerwas hospitalizedbecauseof extreme fatigue. Marked pedal edema had been present for 20 years but there had been no dyspnea, chest pain, hypertension, or evidence of myocardial infarction or valvular disease. Left anterior hemiblock had been detected at age 78 and complete atrioventricular block had led to transvenous pacemaker installation at age 86. One month before hospitalization, massive edema of the left arm had appeared and progressive fatigue had developed thereafter.
July 1982
The American Journalof Medicine Volume 73
133
PERICARDIAL
TAMPONADE-BRODARICK
ET AL.
Past medical history revealed orchidectomy for carcinoma of the prostate three years earlier; there had been no sign of recurrence. Physical examination revealed a pale, lethargic, elderly man who appeared chronically ill. Blood pressure was 120/60 mm Hg, respiratory rate 20 beats/min, heart rate (paced) 72 beats/min. Jugular veins were markedly distended, heart sounds were distant, and the point of maximal impulse could not be located. There was no murmur or gallop rhythm. Basal rales and signs of a left pleural effusion were noted. Both legs and the left arm were massively edematous. Shortly after admission, a 15 mm paradoxic pulse was observed. Laboratory data included: hemoglobin 11.5 g/dl, hematocrit 33 percent, platelet count 890,000, l-l- proteinuria, lactic dehydrogenase 433 mp/ml, alkaline phosphatase 130 m@ml, creatinine 2.1 mg/dl, and blood urea nitrogen 66 mg/dl. Serum albumin ranged from 3.2 to 3.7 g/dl, and globulin from 1.9 to 2.6 g/dl, rheumatoid factor, antinuclear antibody, thyroid function results, Coombs’ test results, and acid phosphatase were normal. Chest x-ray revealed slight cardiomegaly and a large left pleural effusion that, on subsequent examination, presented the characteristics of a sterile nonmalignant transudate. Electrocardiography revealed pacemaker rhythm. M-mode and two-dimensional echocardiography showed a large pericardial effusion with “swinging heart” motion; left ventricular posterior wall and septal end-diastolic thickness was 1.2 to 1.1 cm, left ventricular end-diastolic diameter was 3.8 cm. The apical, septal, and right ventricular myocardium had a glittering, granular appearance, and right ventricular wall was 0.5 cm thick. The left atrial diameter was 3.8 cm; the right atrial size and right ventricular chamber size were normal. Left ventricular ejection fraction determined by radionuelide ventriculogram was normal (64 percent) with normal regional wall motion. Shortly after admission, marked shortness of breath, diffuse rales, and wheezing developed. Symptoms did not respond to digoxin and furosemide. Central venous pressure was greater than 25 cm of water. A I5 mm paradoxic pulse was observed. Following thoracentesis. the pericardial sac was opened through a subxyphoid window and 1,000 ml of straw-colored fluid with characteristics of a nonmalignant transudate was removed. Systolic blood pressure promptly rose from 110 to 150 mm Hg and central venous pressure immediately fell from greater than 25 cm of water to 8 cm of water. Dyspnea subsided. Three to 4 hours later, central venous pressure rose again to greater than 25 cm of water despite the continued drainage of fluid from the pericardium. Paradoxic pulse and hypotension did not recur, but shortness of breath reappeared. The following day, respiratory arrest occurred. On assisted ventilation, right atrial pressure was 24/12 mm Hg (mean 17) right ventricular pressure was 40/ 15 mm Hg, pulmonary artery pressure 36/ 17 mm Hg (mean 251, and pulmonary wedge pressure was 20 mm Hg. A dip and plateau pattern (square root sign) was not seen. Cardiac index was 2.4 liter/min/m2. Subsequently, oliguric renal failure culminated in ventricular fibrillation; after defibrillation, hypotension was unresponsive to dopamine and norepinephrine. At this time, echocardi-
134
July 1982
The American Journal of Medlclne
Volume 73
ography was repeated; no pericardial effusion was observed; left ventricular posterior systolic motion was 0.7 cm, septal motion was 0.6 cm, and enddiastolic left ventricular diameter was 3.8 cm. At postmortem examination, the heart weighed 500 g with dilatation of all four chambers. The left ventricular myocardium was firm and had a glassy appearance. Thickness of right and left ventricular walls was 0.5 cm and 1.6 cm. There were loose adhesions between the visceral and parietal pericardium, but no effusion was present. Endocardium near the aortic and mitral valve was moderately fibrotic and the valve leaflets contained small calcified atheromatous plaques and fibrosis. Coronary arteries were widely patent. Microscopic examination revealed extensive interstitial deposits of a hyaline, eosinophilic, acellular material in the myocardium. This material frequently surrounded myocardial fibers and formed small nodules and was found in the walls of small intramyocardial arterioles. It stained vividly with Congo red and produced a bright greenish-yellow birefringency under polarized light. No other deposits of amyloid were present except in the walls of small and medium-sized arteries of the liver, small bowel, periadrenal tissue, pancreas, and lung. No amyloid was found in the kidneys or in hepatic parenchyma. Bone marrow appeared normal. In view of the prolonged course of this patient’s disease and the absence of major deposits of amyloid in other organs, bone marrow abnormalities, and chronic systemic disease, this case probably should be classified as senile amyloidosis. The presence of depositsin the walls of intramyocardialar-
terioles deserves comment however since this has been consideredto be associatedwith primaryratherthan senile amyloidosis[I]. COMMENTS Advanced age, ventricular dysfunction, arrhythmias, and conduction disorders are familiar features of amyloid heart disease [2-lo]. In this patient, persistent leg edema developed at age 72, left anterior hemiblock at 78, and complete atrioventricular block at 86. The extensive infiltration of the myocardium produced the clinical signs of restrictive myocardiopathy. Despite the presence of edema for 20 years, dyspnea and signs of pulmonary congestion were strikingly absent until the final days of his chronic illness; digitalis and diuretics were ineffective; radionuclide ventriculography and echocardiography showed no evidence of systolic contractile deficit; hemodynamic measurements revealed increased right and left ventricular filling pressures with borderline normal cardiac index. Ventricular wall thickening, normal chamber size, and the glittering “granular sparkling” appearance of the myocardium were characteristic echocardiographic signs of amyloid heart disease [ 111. The unique aspect of this case was the combined presence of restrictive myocardiopathy and pericardial tamponade. To our knowledge, no previous case of tamponade due to amyloid heart disease has been reported. Small to moderate pericardial effusion are
PERICARDIAL
common in this disorder and occasional examples of large effusions have been reported [ 1,8,9,12,13]. In no instance, however, has tamponade been documented. The abrupt restoration of normal central venous pressure and rise in systemic blood pressure and disappearance of paradoxic pulse following removal of a liter of pericardial fluid gave dramatic confirmation of the diagnosis. The presence of the underlying restrictive myocardiopathy became apparent when the clinical hemodynamic improvement after pericardial drainage lasted only a few hours. Central venous hypertension and dyspnea returned, and right and left ventricular filling pressures elevated despite continuing drainage of the pericardial sac and absence of pericardial effusion on
TAMPONADE-BRODARICK
ET AL.
repeated echocardiography (later confirmed by postmortem examination). Cardiac function did not respond to vigorous administration of inotropic agents. The distinction between restrictive myocardiopathy and pericardial disease has long been a subject of clinical interest. This case appears to be unique in that it combines both disorders. The course of the patient demonstrated clearly the unrelenting nature of restrictive myocardiopathy despite the transient improvement following the relief of the cardiac compression. ACKNOWLEDGMENT We wish to thank Kathleen A. Mullen for library assistance and Mabel A. Siegel for typing the manuscript.
REFERENCES 1. 2.
3. 4. 5.
6.
7.
Calkins E, Wright JR: Personal communication. Chew C, Ziady GM, Raphael MJ, et al.: The functional defect in amyloid heart disease. Am J Cardiol 1975; 36: 436444. Kyle RA, Bayrd ED: Amyloidosis: review of 236 cases. Medicine (Baltimore) 1975; 54: 271-299. Buja LM, Khoi NB. Roberts WC: Clinically significant cardiac amyloidosis. Am J Cardiol 1970; 26: 394-405. Hodkinson HM, Pomerance A: The clinical significance of senile cardiac amyloidosis: a prospective clinicopathological study. 0 J Med 1977: XLVI: 361-367. Ridolfi RL, Bulkley BH, Hutchins GM: The conduction system in cardiac amyloidosis. Am J Med 1977; 62: 677-666. Garcia R, Saeed SM: Amylotisis. Arch Intern Med 1966; 12 1: 259-266.
6.
9. 10.
11.
12. 13.
July 1992
Kilpatrick TFt, Horack HM, Moore CB: ‘Stiff Heart’ syndrome an uncommon cause of heart failure. Med Clin North Am 1967; 51: 959-966. Crockett LK, Thompson M, Dekker A: A review of cardiac amyloidosis. Am J Med Sci 1972; 264: 149-156. Meaney E, Shabetai R, Bhargava V, et al.: Cardiac amylokkrsis, constrictive pericarditis and restrictive cardiomyopathy. Am J Cardiol 1976; 36: 547-556. Siqueira-Filho AG, Cunha CLP, Tajik AJ, et al.: M-mode and two-dimensional echocardiographic features in cardiac amyloidosis. Circulation 1961; 63: 166-196. Kitterage RD, Finby N: Amyloid heart disease. Am J Roentgenol 1965; 95: 662-666. Giles TD. Leon-Galindo J, Burch GE: Echocardiographic findings in amyloid cardiomyopathy. South Med J 1976; 71: 1393-1396.
The American Journal ol Yedlclne
Volume 73
135
SCOTT BRODARICK, M.D. ROBERT PAINE, M.D., F.A.C.C. ENRIQUE HIGA, M.D. KIM A. CARMICHAEL, M.D. St. Louis, Missouri
With advancing age of the population and with echouardlographic means of diagnosls, amyloid dlsease of the heart is of increasing clinical interest. Advanced age, restrictive myocardiopathy, arrhythmias, and conduction disorders are familiar features of this disease. A 92 year old man with past history of hemibtock followed by complete heart block and transvenous pacemaker was admitted to the hospital because of increastng fatigue and the abrupt development of dyspnea. Examination revealed paradoxid pulse, markedly elevated central venous pressure, and echocartltographically demonstrated large perlcardial effusion. Shortly after admission signs of tamponade developed; 1,000 ml of perkaldial fluid was removed wlth prompt relief of dyspnea and disappearance of paradoxk pulse and return of central venous pretie to normal. However, dyspnea soon recurred and subsequent hemodynamic measurements indicated increased right ventricular and left ventricular filling pressures. Echocardiography reveated no recurrent effusion or ventrkular hypokinesis. Left ventricular ejection fraction by radionuclide ventricutogram was 64 percent. Echocardiography revealed ventricular wafi thickenlng, normal chamber size, and glittering, sparkling myocardial echoes. On po&mortsrtt examination, there was extensive myocardial amytotdo&. There was no evktence of constrictive pericardttis or recurrent effudon. The unique aspect of this case was the combined presence of restricttve myocardiopathy and pericardial tamponade. To our knowledge, no previous case of tamponade due to amyloid heart disease has been reported. Increasing frequency of amyloid heart disease in our aging population and echocardiographic means of diagnosis have heightened clinical awareness of this disorder. Its pathophysiology and farms of clinical presentation have been elucidated. Our purpose is to describe a manifestation of amyloid heart disease that has not been previously reported. CASE REPORT
From the Department of Internal Medicine and Pathology,St. L*e’s Hospital,St. Louis,Missouri. Requestsfor reprintsshouldbe addressedto Dr. RobertPaine, 5505 Delrnar Blvd.,St. Louis,Missouri63 112. Manuscriptaccepted on November 4, 1961.
A 92 year old practicinglawyerwas hospitalizedbecauseof extreme fatigue. Marked pedal edema had been present for 20 years but there had been no dyspnea, chest pain, hypertension, or evidence of myocardial infarction or valvular disease. Left anterior hemiblock had been detected at age 78 and complete atrioventricular block had led to transvenous pacemaker installation at age 86. One month before hospitalization, massive edema of the left arm had appeared and progressive fatigue had developed thereafter.
July 1982
The American Journalof Medicine Volume 73
133
PERICARDIAL
TAMPONADE-BRODARICK
ET AL.
Past medical history revealed orchidectomy for carcinoma of the prostate three years earlier; there had been no sign of recurrence. Physical examination revealed a pale, lethargic, elderly man who appeared chronically ill. Blood pressure was 120/60 mm Hg, respiratory rate 20 beats/min, heart rate (paced) 72 beats/min. Jugular veins were markedly distended, heart sounds were distant, and the point of maximal impulse could not be located. There was no murmur or gallop rhythm. Basal rales and signs of a left pleural effusion were noted. Both legs and the left arm were massively edematous. Shortly after admission, a 15 mm paradoxic pulse was observed. Laboratory data included: hemoglobin 11.5 g/dl, hematocrit 33 percent, platelet count 890,000, l-l- proteinuria, lactic dehydrogenase 433 mp/ml, alkaline phosphatase 130 m@ml, creatinine 2.1 mg/dl, and blood urea nitrogen 66 mg/dl. Serum albumin ranged from 3.2 to 3.7 g/dl, and globulin from 1.9 to 2.6 g/dl, rheumatoid factor, antinuclear antibody, thyroid function results, Coombs’ test results, and acid phosphatase were normal. Chest x-ray revealed slight cardiomegaly and a large left pleural effusion that, on subsequent examination, presented the characteristics of a sterile nonmalignant transudate. Electrocardiography revealed pacemaker rhythm. M-mode and two-dimensional echocardiography showed a large pericardial effusion with “swinging heart” motion; left ventricular posterior wall and septal end-diastolic thickness was 1.2 to 1.1 cm, left ventricular end-diastolic diameter was 3.8 cm. The apical, septal, and right ventricular myocardium had a glittering, granular appearance, and right ventricular wall was 0.5 cm thick. The left atrial diameter was 3.8 cm; the right atrial size and right ventricular chamber size were normal. Left ventricular ejection fraction determined by radionuelide ventriculogram was normal (64 percent) with normal regional wall motion. Shortly after admission, marked shortness of breath, diffuse rales, and wheezing developed. Symptoms did not respond to digoxin and furosemide. Central venous pressure was greater than 25 cm of water. A I5 mm paradoxic pulse was observed. Following thoracentesis. the pericardial sac was opened through a subxyphoid window and 1,000 ml of straw-colored fluid with characteristics of a nonmalignant transudate was removed. Systolic blood pressure promptly rose from 110 to 150 mm Hg and central venous pressure immediately fell from greater than 25 cm of water to 8 cm of water. Dyspnea subsided. Three to 4 hours later, central venous pressure rose again to greater than 25 cm of water despite the continued drainage of fluid from the pericardium. Paradoxic pulse and hypotension did not recur, but shortness of breath reappeared. The following day, respiratory arrest occurred. On assisted ventilation, right atrial pressure was 24/12 mm Hg (mean 17) right ventricular pressure was 40/ 15 mm Hg, pulmonary artery pressure 36/ 17 mm Hg (mean 251, and pulmonary wedge pressure was 20 mm Hg. A dip and plateau pattern (square root sign) was not seen. Cardiac index was 2.4 liter/min/m2. Subsequently, oliguric renal failure culminated in ventricular fibrillation; after defibrillation, hypotension was unresponsive to dopamine and norepinephrine. At this time, echocardi-
134
July 1982
The American Journal of Medlclne
Volume 73
ography was repeated; no pericardial effusion was observed; left ventricular posterior systolic motion was 0.7 cm, septal motion was 0.6 cm, and enddiastolic left ventricular diameter was 3.8 cm. At postmortem examination, the heart weighed 500 g with dilatation of all four chambers. The left ventricular myocardium was firm and had a glassy appearance. Thickness of right and left ventricular walls was 0.5 cm and 1.6 cm. There were loose adhesions between the visceral and parietal pericardium, but no effusion was present. Endocardium near the aortic and mitral valve was moderately fibrotic and the valve leaflets contained small calcified atheromatous plaques and fibrosis. Coronary arteries were widely patent. Microscopic examination revealed extensive interstitial deposits of a hyaline, eosinophilic, acellular material in the myocardium. This material frequently surrounded myocardial fibers and formed small nodules and was found in the walls of small intramyocardial arterioles. It stained vividly with Congo red and produced a bright greenish-yellow birefringency under polarized light. No other deposits of amyloid were present except in the walls of small and medium-sized arteries of the liver, small bowel, periadrenal tissue, pancreas, and lung. No amyloid was found in the kidneys or in hepatic parenchyma. Bone marrow appeared normal. In view of the prolonged course of this patient’s disease and the absence of major deposits of amyloid in other organs, bone marrow abnormalities, and chronic systemic disease, this case probably should be classified as senile amyloidosis. The presence of depositsin the walls of intramyocardialar-
terioles deserves comment however since this has been consideredto be associatedwith primaryratherthan senile amyloidosis[I]. COMMENTS Advanced age, ventricular dysfunction, arrhythmias, and conduction disorders are familiar features of amyloid heart disease [2-lo]. In this patient, persistent leg edema developed at age 72, left anterior hemiblock at 78, and complete atrioventricular block at 86. The extensive infiltration of the myocardium produced the clinical signs of restrictive myocardiopathy. Despite the presence of edema for 20 years, dyspnea and signs of pulmonary congestion were strikingly absent until the final days of his chronic illness; digitalis and diuretics were ineffective; radionuclide ventriculography and echocardiography showed no evidence of systolic contractile deficit; hemodynamic measurements revealed increased right and left ventricular filling pressures with borderline normal cardiac index. Ventricular wall thickening, normal chamber size, and the glittering “granular sparkling” appearance of the myocardium were characteristic echocardiographic signs of amyloid heart disease [ 111. The unique aspect of this case was the combined presence of restrictive myocardiopathy and pericardial tamponade. To our knowledge, no previous case of tamponade due to amyloid heart disease has been reported. Small to moderate pericardial effusion are
PERICARDIAL
common in this disorder and occasional examples of large effusions have been reported [ 1,8,9,12,13]. In no instance, however, has tamponade been documented. The abrupt restoration of normal central venous pressure and rise in systemic blood pressure and disappearance of paradoxic pulse following removal of a liter of pericardial fluid gave dramatic confirmation of the diagnosis. The presence of the underlying restrictive myocardiopathy became apparent when the clinical hemodynamic improvement after pericardial drainage lasted only a few hours. Central venous hypertension and dyspnea returned, and right and left ventricular filling pressures elevated despite continuing drainage of the pericardial sac and absence of pericardial effusion on
TAMPONADE-BRODARICK
ET AL.
repeated echocardiography (later confirmed by postmortem examination). Cardiac function did not respond to vigorous administration of inotropic agents. The distinction between restrictive myocardiopathy and pericardial disease has long been a subject of clinical interest. This case appears to be unique in that it combines both disorders. The course of the patient demonstrated clearly the unrelenting nature of restrictive myocardiopathy despite the transient improvement following the relief of the cardiac compression. ACKNOWLEDGMENT We wish to thank Kathleen A. Mullen for library assistance and Mabel A. Siegel for typing the manuscript.
REFERENCES 1. 2.
3. 4. 5.
6.
7.
Calkins E, Wright JR: Personal communication. Chew C, Ziady GM, Raphael MJ, et al.: The functional defect in amyloid heart disease. Am J Cardiol 1975; 36: 436444. Kyle RA, Bayrd ED: Amyloidosis: review of 236 cases. Medicine (Baltimore) 1975; 54: 271-299. Buja LM, Khoi NB. Roberts WC: Clinically significant cardiac amyloidosis. Am J Cardiol 1970; 26: 394-405. Hodkinson HM, Pomerance A: The clinical significance of senile cardiac amyloidosis: a prospective clinicopathological study. 0 J Med 1977: XLVI: 361-367. Ridolfi RL, Bulkley BH, Hutchins GM: The conduction system in cardiac amyloidosis. Am J Med 1977; 62: 677-666. Garcia R, Saeed SM: Amylotisis. Arch Intern Med 1966; 12 1: 259-266.
6.
9. 10.
11.
12. 13.
July 1992
Kilpatrick TFt, Horack HM, Moore CB: ‘Stiff Heart’ syndrome an uncommon cause of heart failure. Med Clin North Am 1967; 51: 959-966. Crockett LK, Thompson M, Dekker A: A review of cardiac amyloidosis. Am J Med Sci 1972; 264: 149-156. Meaney E, Shabetai R, Bhargava V, et al.: Cardiac amylokkrsis, constrictive pericarditis and restrictive cardiomyopathy. Am J Cardiol 1976; 36: 547-556. Siqueira-Filho AG, Cunha CLP, Tajik AJ, et al.: M-mode and two-dimensional echocardiographic features in cardiac amyloidosis. Circulation 1961; 63: 166-196. Kitterage RD, Finby N: Amyloid heart disease. Am J Roentgenol 1965; 95: 662-666. Giles TD. Leon-Galindo J, Burch GE: Echocardiographic findings in amyloid cardiomyopathy. South Med J 1976; 71: 1393-1396.
The American Journal ol Yedlclne
Volume 73
135