382
Congenital Intrapericardial Aneurysm of the Left-Atrial Appendage K. I. Ganeshakrishnan, 1. M. S. Khandeparkar. V M. Na traj an, N. B. Agra wal. D. H. Oswal. and R. A. Magotra Department of Cardiovascular and Thora cic Surgery. K. E. M. Hospit al. Bombay. India
Key wor ds
Introduction Acquired ane urysmal dilatation of the left atrium may occur in mitral valve disease. tuberculosis. or syphilis (6). A possibility of viral infection and damage to the left atrium has been suggested by Mo le (8). However. congenital aneurysm of the left atrium is a ra re patho logical curiosity which can be of considerable clinical importance (4). Two varieti es are descri bed viz. intrapericardial and extrapericard ial (12). True aneurysm of the right atrium is still rare (9). We present here a rare case of congenital intrapericardial aneurysm of the left atrial appendage an d briefly review of the literature. Case Report A 35-year-old fema le presented with paroxysmal palpitations since 4 years and progressive exercise intolerance during the last 3 years. A physical examination was unre markable except for a mild increase in cardiac dullness on percussion. She had normal heart sounds and no murmur. Her electrocardiogram revea led atrial fibrillation with a controlle d ventricular rate of 80 per minute . Her chest ra diograph showed a cardiomegaly with a cardiothora cic ratio 0.6 and a prominent left heart border. A larger echolucent mass of 10 em x 10 em situated posterolateral to the left ventricle extending superiorly upto the ma in pulmonary artery and causing compression of the left ventricle was detected on cross-sectional echocardiography. A CTscan of Thorac. cardiovasc. Surgeon 40 (1992) 382 - 384 © Georg Thieme Verlag Stutt gart New York
Kon gen ital es intrape r ikardia les Ane urys ma des Iinken Herzohres Es wird tiber eine 35jiihrige Frau berichtet, die seit 4 Ja hren tiber paroxysma les Herzklopfen und eine zunehmende korp er Iiche Leistungseinschriinkun g klagte. 1m EKG fand sich Vorhofflimmern , die Bontgen-Thoraxaufn ahme zeigte eine Kardi omegalie und die Echokardiogra phie sowie das CT ergaben ein 10 x 10 em grofies, dem Iinken Ventrikel aufsitzendes Gebilde . Bei der Operation via Iinksseitiger Thorak otomie fand sich dann ein diinnwandiges. 10 x 15 em groBes. thrombenfreies Aneurysma des Iinken Her zohr es, das ohn e extra korporalen Kreislauf resiziert werd en konnt e. Postoperativ bestand Sinusrh ythmus und die Patientin war beschwerd efrei. Die einsc hliigige Literatur wird diskutiert.
her chest confirmed the findings of the echocardiography and suggested a homogenous fluid collection. A provisional diagnosis of encysted pericardial effusion was contemplated. However in view of the history of palpitations. the typical location of the mass and compression of the left ventricle. the possibility of an eurysm of the left atrial app endage was also consid ered. She was explored via a left later al thoracotomy thro ugh the fourth interspace. The pleural space was free. Left pericardium could be picked up freely from the underlying cystic mass which was compressible and reducib le. These mano euvres pr ecipitated a transi ent supraventri cular tachycardia. The mass was evidently communicating with some cardiac chamber. A generous lateral pericardio tomy revealed a red. thin-walled. dilated left-atrial app endage (Fig. 1) measuring 15 ern x 10 em. As her echocardiogram and chest CT scan had revealed a homogenous mass consisting of fluid only. it was decided to excise it without cardiopulmonary byass. The bas e of the appendage was thick-walled and 3 ern broad. This was occluded with two vascular clamps . The an eurysm was incised at its summ it and the blood was sucked out. There was no evidence of any thrombus. The redun dant wall of the an eurysm was excised (Fig. 2) and the edges were overswn with a continuous 5-0 prolene suture. Excess pericardium was excided and the per icardial incision was repair ed using a continuous 3-0 prolene suture . The chest was closed after maintaining hemostasis.
Received for Pub lication: July 20. 1992
Downloaded by: National University of Singapore. Copyrighted material.
Summary An interesting case of congenital intrapericardial an eurysm of the left-atrial app endage is reported . The pat ient pr esented with re current supraventi cular arrhythmias and progressive exer cise intolerance and fatigue. Computeris ed axial tomography aide d in the diagn osis by excluding the presence of any thrombus in the sac . The an eur ysm could be safely excised via a left thoracotomy without cardiopulmonary bypas s. The relevant literature on this prob lem is briefly discuss ed.
Thorae. eardiovase . Surg eon 40 (1992)
Fig. 1 Large aneurysm of left-atrial appendage is seen afterpericardiotomy.
Fig. 2 The excised wall of the aneurysm of the left-atrial appendage showing a smooth inner lining traversed by a fewtrabeculae.
Postoperatively th e patient maintained in sinus rh ythm and mad e an un eventful recovery. She was discharged on seventh postop erativ e day in sinus rhythm. At the end of 3 months' follow-up, she has no episodes of palpitatio ns and has improved exercise tolerance.
echocardiographic apical 4-chamber and long-axis parasternal views, these an eurysms ar e seen to originate from an otherwis e normal atrial chamber and have a distinct comm unication with the atrial cavity and caus e distortion of the left ventricle by being situated intraperica rdially (4). The extra perica rdial herni ations through part ial pericardial defects are distin guished by their location outside the pericard ial space and lack of ventricular distortion (4). Besides, the echocardiography should also differentiate the following conditions viz. acq uired aneurysm of the left atrium , pericardial and extrapericardial fluid, and solid or cystic paracardiac tumors (6, 7, 12). Atrial septal defect has been rarely reported in association with congenital an eurysm of the left-atrial app endage (0) and is identifiabl e at echocardiography. The demonstration of a distinct communication with the atrial cavity may be difficult at times in patie nts with poor echogenic window e. g. obesity. Probably transesoph ageal echocardiography may be more helpful, parti cularly in defining the presence of any thrombus in the aneurysm . Though angioca rdiograp hy is the definitive metho d of the diagnosis, it may be inconclusi ve at times, particular ly a thrombus-filled an eurysm making its depiction difficult 0). The angiocardiographic demonstration of this entity would depend upon the app earance of the contrast materi al in the left atrium from the levophase of pulm ona ry arterial injection or trans-septal left-atria l injection. However, neither of these is rout ine at cardiac catheterisation, and the latter technique is potentially dangerous as it may dislodge an unsuspected throm bus (4). Computed axial tomogra phy of the chest as has been previously report ed (2) and as also shown in this case can be of considerab le diagnostic value by demonstrating th e consistency of the interrogated mass, whether uniformly cystic (as in this case) or partly solid and partly fluid (as in the pr eviously reported case) (2). This differentiation is of importance in the furth er man agem ent of the patient with a congenital intrapericardial an eurysm of the left-atrial appendage. Thos e witho ut thrombi may be treat ed by left thoracotomy and excision of the an eurysmal appendage as in our cas e. However, a temp orary cardiopulmonary bypas s is indicated in the management of the an eurysm with throm -
Discu ssion The extr aperica rdial congenita l aneurysm of the left at rium is due to th e herniation of the dilated left atrial appendage through a pericardial defect (5, 12). The intrapericardial an eurysm of the left atrium is, in the absence of a definitive etiology, most probably congenital and is due to a developmental weakn ess in th e atrial wall (1) . Such an an eurysm ma y aris e from the body of the left atrium or its appendage (2) .
Though it is congenital. it is identified mostly in adults in the secon d to the fourth decad es of life (1) and has fair ly equa l sex distribution (2). A large mass occupying limited pericard ial space and thereby compressi ng the ventricles may caus e a state of compro mise d cardiac output, as in the restrictiv e and constrictive diseases of the peri - an d myoca rdium (9). This may be responsible for exercise intolerance and fatigue (9) as noted in our patient. Supraventricu lar arrhythmias observed in th e cas e occur in 75 % of the patients (2). Thrombosis in the an eurysma l sac and subsequ ent systemic embolisation could be life-threatening 0). Chest pain ( 2) and left-heart failure (6) may be presenting features . Rupture of this aneurysm see ms rare 0). A physical exam ination may be noncontributory and norm al or reveal nondiagn ostic systolic murmur (2). Our patient ha d only an increased cardiac dullness on percussion . An electrocardiogram at rest may revea l only sinus rhythm, although som etimes atrial fibrillation an d systemic embolisation has been reported in patients in sinus rhythm (2). Arrhythmias may be produced by an incr eased atrial irritability due to dilatation with ectopic focus formation (6). Non-specific intraventri cular conduction defects, inverted T wave in lea ds I an d aVL and a peculiar rise before the QRS complex are also reported (3, 10). A chest rad iograph may reveal only a cardiomegaly (7) or a ventricular aneurysm or extracardiac ma ss (2). Using
383
Downloaded by: National University of Singapore. Copyrighted material.
Congenita l lntraperieardia l An eurysm of the Left-Atrial Appendag e
Thorae. eardiovase . S urgeon 40 (I 992)
bi, of dens e perianeurysmal adhesions , of an eurysms of the body of the left atrium with associated intracardiac defects, and perhaps of the "irritable appendage" (3,10), handling of which could indu ce serious arrhythmias. Surgery is indicated in the congenital aneurysm of the left atrium or its appendage to eliminate a potential source of systemtic emboli and arrhythmias (1,2) and relieve ventr icular compression. The results of surg ery have been mostly gratifying. There has been no death due to sur gery nor an y peri- or post-operative embolism. Acknowledgement We a re grateful to Dr. (Ms) P. M. Pai, Dean , Seth G. S. Medi cal College a nd K. E. M. Hospit al, Bomb ay (India) for her kind permission to report this case. We a re also gra teful to Dr. (Ms) J . Deshp and e for her kind advice .
References 1 2
3
4
Bramlet. D. A.. and 1. E. Edwards: Congenital ane urys m of left atr ial appe ndage . Brit. Hear t J . 45 (1981) 97-100 Coselli. J. S.. A. C. Beall. and G. M. Ziaddi: Congenital intr aper icard ial ane urysm al dilatation of the left-atri al appendage. Ann. Thorac. Surg . 39 (1985) 466-468 Diamond. E. G.. C. F. Kittle, and D. W Voth: Extreme hypertr ophy of th e left-atrial a ppendage, the case of the giant dog ca r. Amer. J. Cardio!. 5 (1960) 122- 125 Foale. R. A. . T. C. Gibson. D. E. Guye r. L. Gillam, M. E. King. and A. E. Weyman: Congenital a neurysms of the left atr ium: Recognition by cross sectional echoca rdiogra phy. Circ ulation 66 (1982 ) 1065 -1069
K. T. Ganeshakrishnan et al. 5 6
7 8
9
10 11
12
Fry. W : Herni ation of the left auricle. Am. J . Surg. 86 (1953) 736-738 Grinfeld, R. 1.. G. Trainin i. A. Roncoroni. F. Fabrykant, H. Cacheda, and G. Tripodi: Congenital aneurys m of the left atrium. Ann. Th ora c. Surg. 39 (1985) 469-471 Hougen. T. J.. D. G. Muld er. M. T. Gyepes. and A. J. Moss: Aneurys m of the left atrium . Am . J . Cardio!. 33 (1974) 557 -561 Mole. M. T.. P. Goldstraui and M. N. Sheppard: Desm oid tumour in thoracotomy sca r 5 yea rs after excision of a left giant atrial ap penda ge aneurysm in a femal e with family history of Gardener's syndrome. Thora c. cardiovasc. Surgeon 40 (1992) 300-302 Morrow. A. C. and D. M. Behr endt : Congen ital an eu rysm (Diverti culum) of the right at rium. Circulation 38 (1968) 124 128 Pramley. L. F.. Jr.: Congenital atriomega ly. Circulation 25 (1962) 553-558 Shaher; R. M.. W A nis. R. Alley. and J. Mintzer: Congenital enlar gement of the left atrium. J. Thorac. Ca rdiovasc. Surg. 62 (1972) 292 - 299 Williams. W G.: Dilatation of the left-at rial appendage . Brit. HeartJ . 25 (1963) 637- 643
Dr. 1. M. S. Khand eparkar Associate Professor Department of Cardiovasc ula r & Thoracic Surger y K. E. M. Hospital, Par el Bombay 40001 2 India
Downloaded by: National University of Singapore. Copyrighted material.
384
Congenital Intrapericardial Aneurysm of the Left-Atrial Appendage K. I. Ganeshakrishnan, 1. M. S. Khandeparkar. V M. Na traj an, N. B. Agra wal. D. H. Oswal. and R. A. Magotra Department of Cardiovascular and Thora cic Surgery. K. E. M. Hospit al. Bombay. India
Key wor ds
Introduction Acquired ane urysmal dilatation of the left atrium may occur in mitral valve disease. tuberculosis. or syphilis (6). A possibility of viral infection and damage to the left atrium has been suggested by Mo le (8). However. congenital aneurysm of the left atrium is a ra re patho logical curiosity which can be of considerable clinical importance (4). Two varieti es are descri bed viz. intrapericardial and extrapericard ial (12). True aneurysm of the right atrium is still rare (9). We present here a rare case of congenital intrapericardial aneurysm of the left atrial appendage an d briefly review of the literature. Case Report A 35-year-old fema le presented with paroxysmal palpitations since 4 years and progressive exercise intolerance during the last 3 years. A physical examination was unre markable except for a mild increase in cardiac dullness on percussion. She had normal heart sounds and no murmur. Her electrocardiogram revea led atrial fibrillation with a controlle d ventricular rate of 80 per minute . Her chest ra diograph showed a cardiomegaly with a cardiothora cic ratio 0.6 and a prominent left heart border. A larger echolucent mass of 10 em x 10 em situated posterolateral to the left ventricle extending superiorly upto the ma in pulmonary artery and causing compression of the left ventricle was detected on cross-sectional echocardiography. A CTscan of Thorac. cardiovasc. Surgeon 40 (1992) 382 - 384 © Georg Thieme Verlag Stutt gart New York
Kon gen ital es intrape r ikardia les Ane urys ma des Iinken Herzohres Es wird tiber eine 35jiihrige Frau berichtet, die seit 4 Ja hren tiber paroxysma les Herzklopfen und eine zunehmende korp er Iiche Leistungseinschriinkun g klagte. 1m EKG fand sich Vorhofflimmern , die Bontgen-Thoraxaufn ahme zeigte eine Kardi omegalie und die Echokardiogra phie sowie das CT ergaben ein 10 x 10 em grofies, dem Iinken Ventrikel aufsitzendes Gebilde . Bei der Operation via Iinksseitiger Thorak otomie fand sich dann ein diinnwandiges. 10 x 15 em groBes. thrombenfreies Aneurysma des Iinken Her zohr es, das ohn e extra korporalen Kreislauf resiziert werd en konnt e. Postoperativ bestand Sinusrh ythmus und die Patientin war beschwerd efrei. Die einsc hliigige Literatur wird diskutiert.
her chest confirmed the findings of the echocardiography and suggested a homogenous fluid collection. A provisional diagnosis of encysted pericardial effusion was contemplated. However in view of the history of palpitations. the typical location of the mass and compression of the left ventricle. the possibility of an eurysm of the left atrial app endage was also consid ered. She was explored via a left later al thoracotomy thro ugh the fourth interspace. The pleural space was free. Left pericardium could be picked up freely from the underlying cystic mass which was compressible and reducib le. These mano euvres pr ecipitated a transi ent supraventri cular tachycardia. The mass was evidently communicating with some cardiac chamber. A generous lateral pericardio tomy revealed a red. thin-walled. dilated left-atrial app endage (Fig. 1) measuring 15 ern x 10 em. As her echocardiogram and chest CT scan had revealed a homogenous mass consisting of fluid only. it was decided to excise it without cardiopulmonary byass. The bas e of the appendage was thick-walled and 3 ern broad. This was occluded with two vascular clamps . The an eurysm was incised at its summ it and the blood was sucked out. There was no evidence of any thrombus. The redun dant wall of the an eurysm was excised (Fig. 2) and the edges were overswn with a continuous 5-0 prolene suture. Excess pericardium was excided and the per icardial incision was repair ed using a continuous 3-0 prolene suture . The chest was closed after maintaining hemostasis.
Received for Pub lication: July 20. 1992
Downloaded by: National University of Singapore. Copyrighted material.
Summary An interesting case of congenital intrapericardial an eurysm of the left-atrial app endage is reported . The pat ient pr esented with re current supraventi cular arrhythmias and progressive exer cise intolerance and fatigue. Computeris ed axial tomography aide d in the diagn osis by excluding the presence of any thrombus in the sac . The an eur ysm could be safely excised via a left thoracotomy without cardiopulmonary bypas s. The relevant literature on this prob lem is briefly discuss ed.
Thorae. eardiovase . Surg eon 40 (1992)
Fig. 1 Large aneurysm of left-atrial appendage is seen afterpericardiotomy.
Fig. 2 The excised wall of the aneurysm of the left-atrial appendage showing a smooth inner lining traversed by a fewtrabeculae.
Postoperatively th e patient maintained in sinus rh ythm and mad e an un eventful recovery. She was discharged on seventh postop erativ e day in sinus rhythm. At the end of 3 months' follow-up, she has no episodes of palpitatio ns and has improved exercise tolerance.
echocardiographic apical 4-chamber and long-axis parasternal views, these an eurysms ar e seen to originate from an otherwis e normal atrial chamber and have a distinct comm unication with the atrial cavity and caus e distortion of the left ventricle by being situated intraperica rdially (4). The extra perica rdial herni ations through part ial pericardial defects are distin guished by their location outside the pericard ial space and lack of ventricular distortion (4). Besides, the echocardiography should also differentiate the following conditions viz. acq uired aneurysm of the left atrium , pericardial and extrapericardial fluid, and solid or cystic paracardiac tumors (6, 7, 12). Atrial septal defect has been rarely reported in association with congenital an eurysm of the left-atrial app endage (0) and is identifiabl e at echocardiography. The demonstration of a distinct communication with the atrial cavity may be difficult at times in patie nts with poor echogenic window e. g. obesity. Probably transesoph ageal echocardiography may be more helpful, parti cularly in defining the presence of any thrombus in the aneurysm . Though angioca rdiograp hy is the definitive metho d of the diagnosis, it may be inconclusi ve at times, particular ly a thrombus-filled an eurysm making its depiction difficult 0). The angiocardiographic demonstration of this entity would depend upon the app earance of the contrast materi al in the left atrium from the levophase of pulm ona ry arterial injection or trans-septal left-atria l injection. However, neither of these is rout ine at cardiac catheterisation, and the latter technique is potentially dangerous as it may dislodge an unsuspected throm bus (4). Computed axial tomogra phy of the chest as has been previously report ed (2) and as also shown in this case can be of considerab le diagnostic value by demonstrating th e consistency of the interrogated mass, whether uniformly cystic (as in this case) or partly solid and partly fluid (as in the pr eviously reported case) (2). This differentiation is of importance in the furth er man agem ent of the patient with a congenital intrapericardial an eurysm of the left-atrial appendage. Thos e witho ut thrombi may be treat ed by left thoracotomy and excision of the an eurysmal appendage as in our cas e. However, a temp orary cardiopulmonary bypas s is indicated in the management of the an eurysm with throm -
Discu ssion The extr aperica rdial congenita l aneurysm of the left at rium is due to th e herniation of the dilated left atrial appendage through a pericardial defect (5, 12). The intrapericardial an eurysm of the left atrium is, in the absence of a definitive etiology, most probably congenital and is due to a developmental weakn ess in th e atrial wall (1) . Such an an eurysm ma y aris e from the body of the left atrium or its appendage (2) .
Though it is congenital. it is identified mostly in adults in the secon d to the fourth decad es of life (1) and has fair ly equa l sex distribution (2). A large mass occupying limited pericard ial space and thereby compressi ng the ventricles may caus e a state of compro mise d cardiac output, as in the restrictiv e and constrictive diseases of the peri - an d myoca rdium (9). This may be responsible for exercise intolerance and fatigue (9) as noted in our patient. Supraventricu lar arrhythmias observed in th e cas e occur in 75 % of the patients (2). Thrombosis in the an eurysma l sac and subsequ ent systemic embolisation could be life-threatening 0). Chest pain ( 2) and left-heart failure (6) may be presenting features . Rupture of this aneurysm see ms rare 0). A physical exam ination may be noncontributory and norm al or reveal nondiagn ostic systolic murmur (2). Our patient ha d only an increased cardiac dullness on percussion . An electrocardiogram at rest may revea l only sinus rhythm, although som etimes atrial fibrillation an d systemic embolisation has been reported in patients in sinus rhythm (2). Arrhythmias may be produced by an incr eased atrial irritability due to dilatation with ectopic focus formation (6). Non-specific intraventri cular conduction defects, inverted T wave in lea ds I an d aVL and a peculiar rise before the QRS complex are also reported (3, 10). A chest rad iograph may reveal only a cardiomegaly (7) or a ventricular aneurysm or extracardiac ma ss (2). Using
383
Downloaded by: National University of Singapore. Copyrighted material.
Congenita l lntraperieardia l An eurysm of the Left-Atrial Appendag e
Thorae. eardiovase . S urgeon 40 (I 992)
bi, of dens e perianeurysmal adhesions , of an eurysms of the body of the left atrium with associated intracardiac defects, and perhaps of the "irritable appendage" (3,10), handling of which could indu ce serious arrhythmias. Surgery is indicated in the congenital aneurysm of the left atrium or its appendage to eliminate a potential source of systemtic emboli and arrhythmias (1,2) and relieve ventr icular compression. The results of surg ery have been mostly gratifying. There has been no death due to sur gery nor an y peri- or post-operative embolism. Acknowledgement We a re grateful to Dr. (Ms) P. M. Pai, Dean , Seth G. S. Medi cal College a nd K. E. M. Hospit al, Bomb ay (India) for her kind permission to report this case. We a re also gra teful to Dr. (Ms) J . Deshp and e for her kind advice .
References 1 2
3
4
Bramlet. D. A.. and 1. E. Edwards: Congenital ane urys m of left atr ial appe ndage . Brit. Hear t J . 45 (1981) 97-100 Coselli. J. S.. A. C. Beall. and G. M. Ziaddi: Congenital intr aper icard ial ane urysm al dilatation of the left-atri al appendage. Ann. Thorac. Surg . 39 (1985) 466-468 Diamond. E. G.. C. F. Kittle, and D. W Voth: Extreme hypertr ophy of th e left-atrial a ppendage, the case of the giant dog ca r. Amer. J. Cardio!. 5 (1960) 122- 125 Foale. R. A. . T. C. Gibson. D. E. Guye r. L. Gillam, M. E. King. and A. E. Weyman: Congenital a neurysms of the left atr ium: Recognition by cross sectional echoca rdiogra phy. Circ ulation 66 (1982 ) 1065 -1069
K. T. Ganeshakrishnan et al. 5 6
7 8
9
10 11
12
Fry. W : Herni ation of the left auricle. Am. J . Surg. 86 (1953) 736-738 Grinfeld, R. 1.. G. Trainin i. A. Roncoroni. F. Fabrykant, H. Cacheda, and G. Tripodi: Congenital aneurys m of the left atrium. Ann. Th ora c. Surg. 39 (1985) 469-471 Hougen. T. J.. D. G. Muld er. M. T. Gyepes. and A. J. Moss: Aneurys m of the left atrium . Am . J . Cardio!. 33 (1974) 557 -561 Mole. M. T.. P. Goldstraui and M. N. Sheppard: Desm oid tumour in thoracotomy sca r 5 yea rs after excision of a left giant atrial ap penda ge aneurysm in a femal e with family history of Gardener's syndrome. Thora c. cardiovasc. Surgeon 40 (1992) 300-302 Morrow. A. C. and D. M. Behr endt : Congen ital an eu rysm (Diverti culum) of the right at rium. Circulation 38 (1968) 124 128 Pramley. L. F.. Jr.: Congenital atriomega ly. Circulation 25 (1962) 553-558 Shaher; R. M.. W A nis. R. Alley. and J. Mintzer: Congenital enlar gement of the left atrium. J. Thorac. Ca rdiovasc. Surg. 62 (1972) 292 - 299 Williams. W G.: Dilatation of the left-at rial appendage . Brit. HeartJ . 25 (1963) 637- 643
Dr. 1. M. S. Khand eparkar Associate Professor Department of Cardiovasc ula r & Thoracic Surger y K. E. M. Hospital, Par el Bombay 40001 2 India
Downloaded by: National University of Singapore. Copyrighted material.
384
![[Giant congenital intrapericardial left atrial appendage aneurysm: about a case and review of the literature].](/assets/img/hold.png)
