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Enlargement of the right atrium associated with dermatomyositis
Ann Thorac Surg 1995;59:257-68
appropriate solution to primary cardiac tumor, it probably should be undertaken as soon as the diagnosis is made.
Jean Bachet, MD Carlo Banff, MD Luca Martinelli, MD Denis Brodaty, MD Daniel Guilmet, MD Service de Chirurgie Cardio-Vasculaire H6pital Foch 40 rue Worth 92151 Suresnes France References 1. Aufiero TX, Pae WE Jr, Clemson BS, Pawlush DG, Davis D. Heart transplantation for tumor. A n n Thorac Surg 1993;56: 1174-6. 2. Aravot DJ, Banner NR, M a d d e n B, et al. Primary cardiac tumors--is there a place for cardiac transplantation? Eur J Cardiothorac Surg 1989;3:521-4. 3. Dreyfus G, Jebara V, Mihaileanu S, Carpentier AF. Total orthotopic heart transplantation: an alternative to the standard technique. Ann Thorac Surg 1991;52:1181-4. 4. Siebenmann R, Jenni R, Makek M, Oelz O, Turina M. Primary synovial sarcoma of the heart treated by heart transplantation [Letter]. J Thorac Cardiovasc Surg 1990;99:567-8.
Reply To the Editor: We appreciate the opportunity to respond to the letter written by Bachet and associates. There are several important differences between the patient they report and our patient. First, their patient underwent three attempts at resection before transplantation, whereas ours had operative staging of her lesion and no attempts at resection before transplantation. Fortunately, the time lag between the staging operation and the transplantation was less than 72 hours. Since our last publication, our patient has indeed had evidence of tumor recurrence, and has undergone a pulmonary metastectomy. She also has undergone a course of chemotherapy with ifosfamide, doxorubicin, and dacarbazine. Four years after cardiac transplantation she continues to do very well. We would agree that heart transplantation after a third recurrence of a tumor is inappropriate, as well as cardiac transplantation for tumors that are metastatic to the heart. However, we firmly believe that cardiac transplantation for primary cardiac tumors in patients with no evidence of disseminated disease at the time of transplantation is indeed an appropriate and viable option.
CORRESPONDENCE
263
Enlargement of the Right Atrium Associated With Dermatomyositis To the Editor: Idiopathic enlargement of the right atrium (IERA) generally is considered to be congenital in origin [1-5]. In the previously reported cases, the most c o m m o n r h y t h m is normal sinus rhythm with some cases of atrial arrhythmias reported [2-6]. Here we describe a case of IERA associated with dermatomyositis of adult onset in which the right atrium was at a complete standstill and unexcitable. We also discuss the relations between IEILA and dermatomyositis. A 51-year-old housewife was admitted to the hospital because of progressive weakness of the proximal limb muscles for 15 years. At the age of 37 years she underwent pacemaker implantation for bradycardia. She complained of dysphagia a n d difficulty in walking, and noticed dyspnea even with slight exertion for the last several years. The clinical features, muscle biopsy, and electromyogram were consistent with the diagnosis of primary idiopathic dermatomyositis. A biopsy specimen obtained from the biceps brachi revealed degeneration a n d necrosis of muscle fibers, interstitial infiltration with monocytes, a n d perivascular infiltrates of inflammatory cells such as lymphocytes a n d plasma cells (Fig 1). An electrocardiogram showed an artificial ventricular paced rhythm with a rate of 70 beats/min. No P waves of sinus origin were noticed. A chest roentgenogram revealed cardiomegaly with a cardiothoracic ratio of 0.72 and marked right atrial enlargement. On echocardiography, the valves were intact a n d the right atrium was enlarged markedly. Cardiac catheterization disclosed that pressures in both vessels and chambers were within normal limits. An angiocardiogram showed a large, dilated right atrium and noncontractile atria. The diagnoses were IERA, atrial standstill, and primary idiopathic dermatomyositis. To increase cardiac output, dualchamber pacing and excision of the right atrium were performed on November 27, 1986. Through a median sternotomy, the pericardium was adherent to the markedly enlarged and dilated right atrium. The other chambers were not remarkable. Atrial electrical activity measured with epicardial electrodes was absent. Atrial pacing was attempted at multiple sites as high as 10
Thomas X. Auffero, MD Walter E. Pae, Jr, MD Division of Cardiothoracic Surgery Department of Surgery The Milton S. Hershey Medical Center The Pennsylvania State University PO Box 850 Hershey, PA 17033 © 1995 by The Society of Thoracic Surgeons
Fig 1. Specimen of the biceps brachi revealed degeneration and necrosis of muscle fibers with interstitial inflammatory cell infiltration. (Hematoxylin and eosin; ×50 before 47% reduction.) 0003-4975/95/$9.50
264
CORRESPONDENCE
Ann Thorac Surg 1995;59:257-68
larged right atrium and ventricular pacing controlled the heart failure well.
Yasushi Terada, MD Toshio Mitsui, MD Tatsuo Yamamoto, MD Institute of Clinical Medicine University of Tsukuba Tsukuba 305, Japan References
Fig 2. The section of the wall of right atrium showing diffusely fibrotic epicardium and scarce muscle fibers. (PTAH stain; × 10 before 47% reduction.)
V and 20 mA with no response. A large portion of right atrial wall was excised, and two epicardial leads were placed on the right ventricle for ventricular pacing. Section of the wall of the right atrium revealed that the epicardium was fibrotic diffusely and muscle fibers were scarce. Infiltration of inflammatory cells was not observed (Fig 2). Muscular weakness has progressed, but no other problems have developed in 7 years of follow-up study. Idiopathic enlargement of the right atrium generally is considered a congenital abnormality [1-5]. Pathohistologic appearance has been variable; the findings have included hypertrophy of muscle fibers, irregular thickness and loss of striation of muscle fibers, widely separated muscle fibers with no other abnormalities, and lipomatous degeneration [2, 4]. Electrophysiologically, the P waves usually were normal in IERA [1]. Supraventricular arrhythmias such as coronary sinus rhythm, atrial flutter, paroxysmal atrial tachycardia, and atrial premature beats also existed on some occasions [2-5]. The case presented herein is of interest in that the atrial electrical activity was absent with fibrotic atrial rnyocardium. We speculate that fibrotic myocardium in IERA in this case may be the result of postinflammatory fibrosis in the atrium. This case adds to a fairly sizable preexisting literature on the clinical entity of right atrial standstill. Atrial standstill has been noted in three clinical settings, one of which is underlying neuromuscular disease [7]. The association between heart disease including atrial standstill and muscular dystrophy also has been documented [8]. The dermatomyositis and the postinflammatory fibrosis in the atrium may be a related syndrome that affected different organ systems at different times. The fact that the patient's clinical symptomatology of muscle weakness dated back 15 years and actually preceded the implantation of a pacemaker also supports our speculation. We conclude that the etiology of IERA may not be uniform. In this case IERA presented a clinical feature of p e r m a n e n t atrial standstill associated with dermatomyositis. Excision of the en© 1995 by The Society of Thoracic Surgeons
1. Tajik AJ, Broadbent JC, Schattenberg TT. Idiopathic right atrial enlargement with pericardial effusion. Chest 1973;63: 450-3. 2. Pastor BH, Forte AL. Idiopathic enlargement of the right atrium. Am J Cardiol 1961;8:513-8. 3. Morrow AG, Behrendt DM. Congenital aneurysm (diverticulum) of the right atrium. Circulation 1968;38:124-8. 4. Tenckhoff L, Stamm SJ, Beckwith JB. Sudden death in idiopathic (congenital) right atrial enlargement. Postmortem findings and review of cases. Circulation 1969;40:227-35. 5. Asayama J, Matsuura T, Endo N, Matsukuba H, Furukawa K. Idiopathic enlargement of the right atrium. Am J Cardiol 1977;40:620 -3. 6. Burch GE, Giles TD, Shewey LL, Cook GW. Idiopathic enlargement of the right atrium of adult onset. Am J Cardiol 1972;30:87-90. 7. Woolliscroft J, Tuna N. Permanent atrial standstill. The clinical spectrum. Am J Cardiol 1982;49:2037-41. 8. Perloff JK, Leon AC Jr, O'Doherty D. The cardiomyopathy of progressive muscular dystrophy. Circulation 1966;33:625-48.
Intraoperative Autohemotransfusion
and Open Heart
Reoperation To the Editor: The interesting article by Sch6nberger and associates [1] highlights, by a retrospective study of 100 patients undergoing myocardial revascularization with internal m a m m a r y artery, the role of intraoperative autotransfusion in reduction of blood loss and need of homologous b a n k e d blood products. We started to use a similar technique in elective cardiac operations some years ago, and the encouraging results of our first study [2] persuaded us to use it routinely during all cardiac procedures, and particularly during reoperations. We recently presented a retrospective study of 169 patients (1981 to 1990) operated on for single or double heart valve replacement after a previous mitral commissurotomy and radical correction after a previous Blalock-Taussig shunt [3], and a prospective, randomized study in 42 patients (75% of whom h a d reoperations) [4]. The technique of blood collection is similar to that proposed by Sch6nberger and associates, using a cardiotomy reservoir maintained at room temperature to store the blood, obtained 3 minutes after the anticoagulation with heparin (5 mg/kg of body weight) and before the start of cardiopulmonary bypass. Crystalloid solution is infused after blood withdrawal to maintain hematocrit values as low as 20% to 28% during the operation. After the surgical procedure, autologus blood is gradually reinfused to the patient. We did not observe any significant change in arterial blood pressure or cardiac rhythm during and after autohemotransfusion. Prothrombin time, partial thromboplastin time, t h r o m b i n time, t h r o m b i n time coagulase, fibrin/fibrinogen degradation products, platelet number, arterial blood gases, and activated clotting time were measured on admission for operation and then during and after operation. We also calculated blood losses 0003-4975/95/$9.50
appropriate solution to primary cardiac tumor, it probably should be undertaken as soon as the diagnosis is made.
Jean Bachet, MD Carlo Banff, MD Luca Martinelli, MD Denis Brodaty, MD Daniel Guilmet, MD Service de Chirurgie Cardio-Vasculaire H6pital Foch 40 rue Worth 92151 Suresnes France References 1. Aufiero TX, Pae WE Jr, Clemson BS, Pawlush DG, Davis D. Heart transplantation for tumor. A n n Thorac Surg 1993;56: 1174-6. 2. Aravot DJ, Banner NR, M a d d e n B, et al. Primary cardiac tumors--is there a place for cardiac transplantation? Eur J Cardiothorac Surg 1989;3:521-4. 3. Dreyfus G, Jebara V, Mihaileanu S, Carpentier AF. Total orthotopic heart transplantation: an alternative to the standard technique. Ann Thorac Surg 1991;52:1181-4. 4. Siebenmann R, Jenni R, Makek M, Oelz O, Turina M. Primary synovial sarcoma of the heart treated by heart transplantation [Letter]. J Thorac Cardiovasc Surg 1990;99:567-8.
Reply To the Editor: We appreciate the opportunity to respond to the letter written by Bachet and associates. There are several important differences between the patient they report and our patient. First, their patient underwent three attempts at resection before transplantation, whereas ours had operative staging of her lesion and no attempts at resection before transplantation. Fortunately, the time lag between the staging operation and the transplantation was less than 72 hours. Since our last publication, our patient has indeed had evidence of tumor recurrence, and has undergone a pulmonary metastectomy. She also has undergone a course of chemotherapy with ifosfamide, doxorubicin, and dacarbazine. Four years after cardiac transplantation she continues to do very well. We would agree that heart transplantation after a third recurrence of a tumor is inappropriate, as well as cardiac transplantation for tumors that are metastatic to the heart. However, we firmly believe that cardiac transplantation for primary cardiac tumors in patients with no evidence of disseminated disease at the time of transplantation is indeed an appropriate and viable option.
CORRESPONDENCE
263
Enlargement of the Right Atrium Associated With Dermatomyositis To the Editor: Idiopathic enlargement of the right atrium (IERA) generally is considered to be congenital in origin [1-5]. In the previously reported cases, the most c o m m o n r h y t h m is normal sinus rhythm with some cases of atrial arrhythmias reported [2-6]. Here we describe a case of IERA associated with dermatomyositis of adult onset in which the right atrium was at a complete standstill and unexcitable. We also discuss the relations between IEILA and dermatomyositis. A 51-year-old housewife was admitted to the hospital because of progressive weakness of the proximal limb muscles for 15 years. At the age of 37 years she underwent pacemaker implantation for bradycardia. She complained of dysphagia a n d difficulty in walking, and noticed dyspnea even with slight exertion for the last several years. The clinical features, muscle biopsy, and electromyogram were consistent with the diagnosis of primary idiopathic dermatomyositis. A biopsy specimen obtained from the biceps brachi revealed degeneration a n d necrosis of muscle fibers, interstitial infiltration with monocytes, a n d perivascular infiltrates of inflammatory cells such as lymphocytes a n d plasma cells (Fig 1). An electrocardiogram showed an artificial ventricular paced rhythm with a rate of 70 beats/min. No P waves of sinus origin were noticed. A chest roentgenogram revealed cardiomegaly with a cardiothoracic ratio of 0.72 and marked right atrial enlargement. On echocardiography, the valves were intact a n d the right atrium was enlarged markedly. Cardiac catheterization disclosed that pressures in both vessels and chambers were within normal limits. An angiocardiogram showed a large, dilated right atrium and noncontractile atria. The diagnoses were IERA, atrial standstill, and primary idiopathic dermatomyositis. To increase cardiac output, dualchamber pacing and excision of the right atrium were performed on November 27, 1986. Through a median sternotomy, the pericardium was adherent to the markedly enlarged and dilated right atrium. The other chambers were not remarkable. Atrial electrical activity measured with epicardial electrodes was absent. Atrial pacing was attempted at multiple sites as high as 10
Thomas X. Auffero, MD Walter E. Pae, Jr, MD Division of Cardiothoracic Surgery Department of Surgery The Milton S. Hershey Medical Center The Pennsylvania State University PO Box 850 Hershey, PA 17033 © 1995 by The Society of Thoracic Surgeons
Fig 1. Specimen of the biceps brachi revealed degeneration and necrosis of muscle fibers with interstitial inflammatory cell infiltration. (Hematoxylin and eosin; ×50 before 47% reduction.) 0003-4975/95/$9.50
264
CORRESPONDENCE
Ann Thorac Surg 1995;59:257-68
larged right atrium and ventricular pacing controlled the heart failure well.
Yasushi Terada, MD Toshio Mitsui, MD Tatsuo Yamamoto, MD Institute of Clinical Medicine University of Tsukuba Tsukuba 305, Japan References
Fig 2. The section of the wall of right atrium showing diffusely fibrotic epicardium and scarce muscle fibers. (PTAH stain; × 10 before 47% reduction.)
V and 20 mA with no response. A large portion of right atrial wall was excised, and two epicardial leads were placed on the right ventricle for ventricular pacing. Section of the wall of the right atrium revealed that the epicardium was fibrotic diffusely and muscle fibers were scarce. Infiltration of inflammatory cells was not observed (Fig 2). Muscular weakness has progressed, but no other problems have developed in 7 years of follow-up study. Idiopathic enlargement of the right atrium generally is considered a congenital abnormality [1-5]. Pathohistologic appearance has been variable; the findings have included hypertrophy of muscle fibers, irregular thickness and loss of striation of muscle fibers, widely separated muscle fibers with no other abnormalities, and lipomatous degeneration [2, 4]. Electrophysiologically, the P waves usually were normal in IERA [1]. Supraventricular arrhythmias such as coronary sinus rhythm, atrial flutter, paroxysmal atrial tachycardia, and atrial premature beats also existed on some occasions [2-5]. The case presented herein is of interest in that the atrial electrical activity was absent with fibrotic atrial rnyocardium. We speculate that fibrotic myocardium in IERA in this case may be the result of postinflammatory fibrosis in the atrium. This case adds to a fairly sizable preexisting literature on the clinical entity of right atrial standstill. Atrial standstill has been noted in three clinical settings, one of which is underlying neuromuscular disease [7]. The association between heart disease including atrial standstill and muscular dystrophy also has been documented [8]. The dermatomyositis and the postinflammatory fibrosis in the atrium may be a related syndrome that affected different organ systems at different times. The fact that the patient's clinical symptomatology of muscle weakness dated back 15 years and actually preceded the implantation of a pacemaker also supports our speculation. We conclude that the etiology of IERA may not be uniform. In this case IERA presented a clinical feature of p e r m a n e n t atrial standstill associated with dermatomyositis. Excision of the en© 1995 by The Society of Thoracic Surgeons
1. Tajik AJ, Broadbent JC, Schattenberg TT. Idiopathic right atrial enlargement with pericardial effusion. Chest 1973;63: 450-3. 2. Pastor BH, Forte AL. Idiopathic enlargement of the right atrium. Am J Cardiol 1961;8:513-8. 3. Morrow AG, Behrendt DM. Congenital aneurysm (diverticulum) of the right atrium. Circulation 1968;38:124-8. 4. Tenckhoff L, Stamm SJ, Beckwith JB. Sudden death in idiopathic (congenital) right atrial enlargement. Postmortem findings and review of cases. Circulation 1969;40:227-35. 5. Asayama J, Matsuura T, Endo N, Matsukuba H, Furukawa K. Idiopathic enlargement of the right atrium. Am J Cardiol 1977;40:620 -3. 6. Burch GE, Giles TD, Shewey LL, Cook GW. Idiopathic enlargement of the right atrium of adult onset. Am J Cardiol 1972;30:87-90. 7. Woolliscroft J, Tuna N. Permanent atrial standstill. The clinical spectrum. Am J Cardiol 1982;49:2037-41. 8. Perloff JK, Leon AC Jr, O'Doherty D. The cardiomyopathy of progressive muscular dystrophy. Circulation 1966;33:625-48.
Intraoperative Autohemotransfusion
and Open Heart
Reoperation To the Editor: The interesting article by Sch6nberger and associates [1] highlights, by a retrospective study of 100 patients undergoing myocardial revascularization with internal m a m m a r y artery, the role of intraoperative autotransfusion in reduction of blood loss and need of homologous b a n k e d blood products. We started to use a similar technique in elective cardiac operations some years ago, and the encouraging results of our first study [2] persuaded us to use it routinely during all cardiac procedures, and particularly during reoperations. We recently presented a retrospective study of 169 patients (1981 to 1990) operated on for single or double heart valve replacement after a previous mitral commissurotomy and radical correction after a previous Blalock-Taussig shunt [3], and a prospective, randomized study in 42 patients (75% of whom h a d reoperations) [4]. The technique of blood collection is similar to that proposed by Sch6nberger and associates, using a cardiotomy reservoir maintained at room temperature to store the blood, obtained 3 minutes after the anticoagulation with heparin (5 mg/kg of body weight) and before the start of cardiopulmonary bypass. Crystalloid solution is infused after blood withdrawal to maintain hematocrit values as low as 20% to 28% during the operation. After the surgical procedure, autologus blood is gradually reinfused to the patient. We did not observe any significant change in arterial blood pressure or cardiac rhythm during and after autohemotransfusion. Prothrombin time, partial thromboplastin time, t h r o m b i n time, t h r o m b i n time coagulase, fibrin/fibrinogen degradation products, platelet number, arterial blood gases, and activated clotting time were measured on admission for operation and then during and after operation. We also calculated blood losses 0003-4975/95/$9.50