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Blood cyst with a calcium stone originating from the right atrial septum
Case reports in a newborn with severely depressed myocardial function after arterial switch operation. The importance of establishing adequate coronary perfusion cannot be over-emphasized, but even with good coronary blood flow extracorporeal membrane oxygenation may be required for a longer period to allow successful myocardial recovery.
6.
Mayer JE, Sanders SP,Jonas RA, Castaneda AR, Wernovsky G. Coronary artery pattern and outcome of arterial switch operation for transposition of the great arteries. Circulation
7.
Yamaguchi M, Hosokawa Y, Imai Y et al. Early and midterm results of the arterial switch operation for transposition of the great arteries in Japan. J Thoruc Curdiovusc Surg 1990; 100:
References
8.
Day RW, Laks H, Drinkwater DC. The influence of coronary anatomy on the arterial switch operation in neonates. J Thoruc
9.
Sim EKW, van Son JAM, Julsrud PR, Puga FJ. Aortic intramural course of the left coronary artery in dextro-transposition of the great arteries. Ann Tboruc Surg 1994; 57: 458-60. Kuroczynski W, Schmid FX, Lazar U, Huth R, Schranz D, Oelert H. Linksventrikulire Postkardiotomie-Kreislaufassistenz bei
1990; 52(suppl. IV): IV139-45.
261-9.
1. Gittenberger-de Groot AC, Sauer U, Oppenheimer-Dekker A, Quaegebeur J. Coronary arterial anatomy in transposition of the great arteries: a morphologic study. Pediutr Cardiol 1983;
Cardiovusc Surg 1992; 104: 706-12.
4(suppl. 1): 15-24.
Moat NE, Pawade A, Lamb RK. Complex coronary arterial anatomy in transposition of the great arteries: arterial switch procedure without coronary relocation. / Thoruc Curdiouasc
2.
10.
Surg 1992; 103: 872-6.
Day RW. Unusual coronary patterns and arterial switch outcome. Ann Thorac Surg 1994; 57: 283-S. De Leval MR, Franqois K, Bull C, Brawn W, Spiegelhalter D. Analysis of a cluster of surgical failures: application to a seriesof neonatal arterial switch operations. J Thorac Cardiouasc Surg
3. 4.
1994; 107: 914-24.
Asou T, Karl TR, Pawade A, Mee RBB. Arterial switch: translocation of the intramural coronary artery. Ann Tboruc
5.
Surg 1994; 57: 461-5.
11. 12.
einem 7 Tage alten Neugeborenen. Z Herz- Thorax-Gefiifkhir 1993; 7: 104-7. Raithel SC, Pennington DG, Boegner E, Fiore A, Weber TR. Extracorporeal membrane oxygenation in children after cardiac surgery. Circtrlution 1992; 86(suppl. II): 11305-10. Cherian KM, Rao SG. Significance of intraoperative ST segment monitoring during arterial switch operation. I Tboruc Cardiovasc Surg 1991; 102: 160.
Paper accepted 19 October 1994
Copyright
Blood cyst with a calcium stone originating from the right atria1 septum H. Niinami*,S.Ishihara,S.Hara,S.Tanaka,E. Yamagut?andS.Takase+ Departments of c;7rdiovascu/ar Surgery and tCardiof~~ S%eikai Maebasbi Hospiital, Maebashi and *Depamen t of Cardiovascular Surgery, The Heart fnst&te of Japan, Tokyo Women 3 Medica/ College, 8- 1 Ka wa&-cho, Shivtiku-ku, Tokyo 162 Japan Blood cysts of the heart in adults are extremely rare, and their origin is usually from the valvular apparatus. However, a case was encountered of a blood cyst originating from the right atrial septum of a SD-year-old woman. Furthermore the cyst contained calcium stone. The cyst with the stone was successfully excised. Neither situation has been reported previously in the literature. Keywords:
260
blood cyst, right atrium, calcium stone
Curdmvuscular Surgq, Vol. 4, No. 2. pp. 260-262. 1996 0 1996 The International Society for Cardiovascular Surgery Published by Elsevier Science Ltd. Printed in Great Britain W67-2lG9/96 $15.00 + 0.00
Although small blood cysts of the heart valves are frequently seen in the newborn and in infants as a post-mortem findin the occurrence of such cysts after the first year of life is rare’- 8,. These cysts are usually found on the mitral and tricuspid valves along the atria1 surfaces and also occur less commonly on the ventricular surfaces of the pulmonary valve and papillary muscle 4-6 . However, there have been no reports in the literature of a blood cyst originating from the atria1 septum in an adult. Nor has there been any report of a stone floating inside a blood cyst. The following is a report of a 59-year-old woman who underwent successful surgical excision of a unique blood cyst with a floating calcium stone attached to the right atria1 septum.
Case report A 59-year-old Japanese woman was admitted to Saiseikai Maebashi Hospital in September 1991. Her chief complaints were periodic episodes of substernal pressure and dry cough. There was no history of syncope. She had not had rheumatic fever. Physical examination revealed a well-developed, wellnourished woman. Vital signs were normal and there was no evidence of cyanosis. On cardiac examination the palpable maximum impulse was normal and in the midclavicular line. A l/6 holosystolic
Correspondence to: Dr H. Niinami
CARDIOVASCULAR SURGERY APRIL 1996 VOL 4 NO 2
murmur was heard along the left sternal border. Electrocardiography and chest radiography were unremarkable. Transthoracic two-dimensional echocardiography revealed mild right atria1 enlargement, mild tricuspid valve insufficiency, and a mobile tumour fixed to the atria1 septum near the right atrioventricular junction. This tumour was a round, unilocular, ball-like cystic mass, approximately 2cm in diameter. A left ventriculogram and coronary arteriogram were normal. No feeding arteries to the tumour were observed. However, a small radiopaque mass, approximately 1 cm in diameter, was visible on a cineangiogram. The final clinical diagnosis was thought to be myxoma of the right atrium with attachment near the base of the tricuspid valve. Surgical intervention was recommended. The patient then underwent open-heart surgery. The heart was exposed through a median sternotomy with the use of cardiopulmonary bypass. Bicaval cannulations were established directly into the superior vena cava and right femoral vein so as not to manipulate the right atrium. From the right atriotomy, a dark cystic mass was seen on the atria1 septum between the fossa ovalis and tricuspid valvular annulus. It was revealed as a circumscribed, smoothly outlined, unilocular cystic mass, 2.5 x 2.0 cm in diameter and completely different from the myxoma (Figure 2). In the process of excision from the atria1 septum, the cystic tumour was ruptured, bloody fluid drained out, and it became flaccid. A stone-like mass, 0.8 x 0.6 cm in diameter, was then seeninside the cyst, the stone was not attached to the wall of the cyst (Figure 2). The cyst with the stone was excised, together with a small part of the atria1 septum, and the septum was repaired by patch. The patient’s postoperative course was uneventful and her presenting symptoms have resolved. The cystic wall was 1 mm thick and its histological examination showed oedematous connective tissue rich in collagenous fibres and fibroblasts and scarce in elastic fibres and smooth muscle cells. Both the inner and outer sides of the cystic wall were lined with a thin endothelium shown by Factor VIII staining. Pathological diagnosis of this cystic mass was blood cyst. The stone was found to consist of calcium.
Discusssion Blood cyst of the heart was first reported by Elsasserin 1844’. Since then many observers have reported these cysts of the heart in small infants under 6 months of agerm3.They are usually found as small, rounded, multiple nodules varying in size from a pinpoint to a pinhead, on the endocardium, particularly the valvular endocardium. These cysts have been observed in up to 56% of autopsy specimensof neonates and infants’ -3 The single, large blood cyst that occurs in older children and adults is a different entity and has been infrequently reported in the literature. The reported blood cysts in adult cases all originated from the valvular apparatus, such as the pulmona valve, mitral valve, tricuspid valve and papillary muscle4-7 . However, the cyst reported here differed from those reported in the literature with regard to two aspects.First, the origin of the cyst was not from the valvular apparatus, but from the right atria1 septum. Secondly, it contained a floating stone, which was composed of calcium, within the cystic cavity. Several hypotheses have been expressed regarding the development of these blood cysts. The most well-known theory is that they are formed when blood enters into crevices on the surface of the valvular cusp with subsequent sealing off
CARDIOVASCULAR SURGERY APRIL 1996 VOL 4 NO 2
..-. ., --_ Figure 1
Operative view of the cyst through right arriotomy
Figure 2
Collapsed cyst with atrial septum and
CaiCliJrii
_.
stone
of the portal entry3. However, this theory would seem to be untenable in this case, because the origin of the cyst was not from the valvular apparatus. Sakakibara et a1.smentioned the relation of the origin of the blood cyst to the sudden occlusion of branches of the circulation, such as that caused by inflammation, vagal stimulation causing anoxia and haemorrhagic diathesis. They also referred to the valvular apparatus. The authors hypothesized that this might occur in any circulation in the heart such as Thebesian circulation in either the atrium or the ventricle. Although there was no evidence to support this hypothesis, the present casesuggeststhat a blood cyst can be formed not only on the valvular apparatus, but also anywhere in the cardiac cavity. If this ‘sudden occlusion theory’ is the origin of the blood cyst, it would be reasonable that most reported blood cysts originated from the valvular apparatus. Because the valve is notably avascular, the limit between the endocardium and the valve tissue could be the end of the blood vessels.Furthermore, in the present case,it is very curious that there was a calcified stone floating in the cystic cavity. This stone was similar to those found in the gall bladder. Despite a search of the literature, no other reports of a blood cyst, either originating from the right atria1 septum or containing a stone, were found.
261
Case repor& In conclusion, although the mechanisms of occlusion are ambiguous, the present case could constitute evidence to support the ‘sudden occlusion theory’.
References 1. Levinson SA, Learner A. Blood cysts on the heart valves of newborn infants. Arch Puthol1932; 14: 810-17. 2. Dow DR, Harper WF. Blood cysts in human cardiac valves. J Anat 1936; 71: 117-23. 3. Boyd TAB. Blood cysts on the heart valves of infants. Am J Pathol 1949; 25: 757-9.
4. Leatherman L, Leachman RD, Hallman GL, Cooley DA. Cyst of
262
the mitral valve. Am J Curdiol 1968; 21: 428-30. 5. Galluci V, Stritoni P, Fasoli G, Thiene G. Giant blood cyst of tricuspid valve: successfulexcision in an infant. Br Heart J 1976; 38: 990-2.
Liese GJ, Brainward SC, Goto U. Giant blood cyst of the pulmonary valve. N Engl J Med 1963; 269: 465-7. Elsasser C. Bericht uber die Ereignisse in der Gebaranstalt des Catherinen-Hospital im lahre 1844. Med Correspondenzblutt 1844; 14: 297.. ” Sakakibara S, Katsuhara K, Iida Y, Nishida H. Pulmonary subvalvular tumor. Dis Chest 1967; 51: 637-41. raper accepted 26 October 1994
CARDIOVASCULAR SURGERY APRIL 1996 VOL4 NO2
6.
Mayer JE, Sanders SP,Jonas RA, Castaneda AR, Wernovsky G. Coronary artery pattern and outcome of arterial switch operation for transposition of the great arteries. Circulation
7.
Yamaguchi M, Hosokawa Y, Imai Y et al. Early and midterm results of the arterial switch operation for transposition of the great arteries in Japan. J Thoruc Curdiovusc Surg 1990; 100:
References
8.
Day RW, Laks H, Drinkwater DC. The influence of coronary anatomy on the arterial switch operation in neonates. J Thoruc
9.
Sim EKW, van Son JAM, Julsrud PR, Puga FJ. Aortic intramural course of the left coronary artery in dextro-transposition of the great arteries. Ann Tboruc Surg 1994; 57: 458-60. Kuroczynski W, Schmid FX, Lazar U, Huth R, Schranz D, Oelert H. Linksventrikulire Postkardiotomie-Kreislaufassistenz bei
1990; 52(suppl. IV): IV139-45.
261-9.
1. Gittenberger-de Groot AC, Sauer U, Oppenheimer-Dekker A, Quaegebeur J. Coronary arterial anatomy in transposition of the great arteries: a morphologic study. Pediutr Cardiol 1983;
Cardiovusc Surg 1992; 104: 706-12.
4(suppl. 1): 15-24.
Moat NE, Pawade A, Lamb RK. Complex coronary arterial anatomy in transposition of the great arteries: arterial switch procedure without coronary relocation. / Thoruc Curdiouasc
2.
10.
Surg 1992; 103: 872-6.
Day RW. Unusual coronary patterns and arterial switch outcome. Ann Thorac Surg 1994; 57: 283-S. De Leval MR, Franqois K, Bull C, Brawn W, Spiegelhalter D. Analysis of a cluster of surgical failures: application to a seriesof neonatal arterial switch operations. J Thorac Cardiouasc Surg
3. 4.
1994; 107: 914-24.
Asou T, Karl TR, Pawade A, Mee RBB. Arterial switch: translocation of the intramural coronary artery. Ann Tboruc
5.
Surg 1994; 57: 461-5.
11. 12.
einem 7 Tage alten Neugeborenen. Z Herz- Thorax-Gefiifkhir 1993; 7: 104-7. Raithel SC, Pennington DG, Boegner E, Fiore A, Weber TR. Extracorporeal membrane oxygenation in children after cardiac surgery. Circtrlution 1992; 86(suppl. II): 11305-10. Cherian KM, Rao SG. Significance of intraoperative ST segment monitoring during arterial switch operation. I Tboruc Cardiovasc Surg 1991; 102: 160.
Paper accepted 19 October 1994
Copyright
Blood cyst with a calcium stone originating from the right atria1 septum H. Niinami*,S.Ishihara,S.Hara,S.Tanaka,E. Yamagut?andS.Takase+ Departments of c;7rdiovascu/ar Surgery and tCardiof~~ S%eikai Maebasbi Hospiital, Maebashi and *Depamen t of Cardiovascular Surgery, The Heart fnst&te of Japan, Tokyo Women 3 Medica/ College, 8- 1 Ka wa&-cho, Shivtiku-ku, Tokyo 162 Japan Blood cysts of the heart in adults are extremely rare, and their origin is usually from the valvular apparatus. However, a case was encountered of a blood cyst originating from the right atrial septum of a SD-year-old woman. Furthermore the cyst contained calcium stone. The cyst with the stone was successfully excised. Neither situation has been reported previously in the literature. Keywords:
260
blood cyst, right atrium, calcium stone
Curdmvuscular Surgq, Vol. 4, No. 2. pp. 260-262. 1996 0 1996 The International Society for Cardiovascular Surgery Published by Elsevier Science Ltd. Printed in Great Britain W67-2lG9/96 $15.00 + 0.00
Although small blood cysts of the heart valves are frequently seen in the newborn and in infants as a post-mortem findin the occurrence of such cysts after the first year of life is rare’- 8,. These cysts are usually found on the mitral and tricuspid valves along the atria1 surfaces and also occur less commonly on the ventricular surfaces of the pulmonary valve and papillary muscle 4-6 . However, there have been no reports in the literature of a blood cyst originating from the atria1 septum in an adult. Nor has there been any report of a stone floating inside a blood cyst. The following is a report of a 59-year-old woman who underwent successful surgical excision of a unique blood cyst with a floating calcium stone attached to the right atria1 septum.
Case report A 59-year-old Japanese woman was admitted to Saiseikai Maebashi Hospital in September 1991. Her chief complaints were periodic episodes of substernal pressure and dry cough. There was no history of syncope. She had not had rheumatic fever. Physical examination revealed a well-developed, wellnourished woman. Vital signs were normal and there was no evidence of cyanosis. On cardiac examination the palpable maximum impulse was normal and in the midclavicular line. A l/6 holosystolic
Correspondence to: Dr H. Niinami
CARDIOVASCULAR SURGERY APRIL 1996 VOL 4 NO 2
murmur was heard along the left sternal border. Electrocardiography and chest radiography were unremarkable. Transthoracic two-dimensional echocardiography revealed mild right atria1 enlargement, mild tricuspid valve insufficiency, and a mobile tumour fixed to the atria1 septum near the right atrioventricular junction. This tumour was a round, unilocular, ball-like cystic mass, approximately 2cm in diameter. A left ventriculogram and coronary arteriogram were normal. No feeding arteries to the tumour were observed. However, a small radiopaque mass, approximately 1 cm in diameter, was visible on a cineangiogram. The final clinical diagnosis was thought to be myxoma of the right atrium with attachment near the base of the tricuspid valve. Surgical intervention was recommended. The patient then underwent open-heart surgery. The heart was exposed through a median sternotomy with the use of cardiopulmonary bypass. Bicaval cannulations were established directly into the superior vena cava and right femoral vein so as not to manipulate the right atrium. From the right atriotomy, a dark cystic mass was seen on the atria1 septum between the fossa ovalis and tricuspid valvular annulus. It was revealed as a circumscribed, smoothly outlined, unilocular cystic mass, 2.5 x 2.0 cm in diameter and completely different from the myxoma (Figure 2). In the process of excision from the atria1 septum, the cystic tumour was ruptured, bloody fluid drained out, and it became flaccid. A stone-like mass, 0.8 x 0.6 cm in diameter, was then seeninside the cyst, the stone was not attached to the wall of the cyst (Figure 2). The cyst with the stone was excised, together with a small part of the atria1 septum, and the septum was repaired by patch. The patient’s postoperative course was uneventful and her presenting symptoms have resolved. The cystic wall was 1 mm thick and its histological examination showed oedematous connective tissue rich in collagenous fibres and fibroblasts and scarce in elastic fibres and smooth muscle cells. Both the inner and outer sides of the cystic wall were lined with a thin endothelium shown by Factor VIII staining. Pathological diagnosis of this cystic mass was blood cyst. The stone was found to consist of calcium.
Discusssion Blood cyst of the heart was first reported by Elsasserin 1844’. Since then many observers have reported these cysts of the heart in small infants under 6 months of agerm3.They are usually found as small, rounded, multiple nodules varying in size from a pinpoint to a pinhead, on the endocardium, particularly the valvular endocardium. These cysts have been observed in up to 56% of autopsy specimensof neonates and infants’ -3 The single, large blood cyst that occurs in older children and adults is a different entity and has been infrequently reported in the literature. The reported blood cysts in adult cases all originated from the valvular apparatus, such as the pulmona valve, mitral valve, tricuspid valve and papillary muscle4-7 . However, the cyst reported here differed from those reported in the literature with regard to two aspects.First, the origin of the cyst was not from the valvular apparatus, but from the right atria1 septum. Secondly, it contained a floating stone, which was composed of calcium, within the cystic cavity. Several hypotheses have been expressed regarding the development of these blood cysts. The most well-known theory is that they are formed when blood enters into crevices on the surface of the valvular cusp with subsequent sealing off
CARDIOVASCULAR SURGERY APRIL 1996 VOL 4 NO 2
..-. ., --_ Figure 1
Operative view of the cyst through right arriotomy
Figure 2
Collapsed cyst with atrial septum and
CaiCliJrii
_.
stone
of the portal entry3. However, this theory would seem to be untenable in this case, because the origin of the cyst was not from the valvular apparatus. Sakakibara et a1.smentioned the relation of the origin of the blood cyst to the sudden occlusion of branches of the circulation, such as that caused by inflammation, vagal stimulation causing anoxia and haemorrhagic diathesis. They also referred to the valvular apparatus. The authors hypothesized that this might occur in any circulation in the heart such as Thebesian circulation in either the atrium or the ventricle. Although there was no evidence to support this hypothesis, the present casesuggeststhat a blood cyst can be formed not only on the valvular apparatus, but also anywhere in the cardiac cavity. If this ‘sudden occlusion theory’ is the origin of the blood cyst, it would be reasonable that most reported blood cysts originated from the valvular apparatus. Because the valve is notably avascular, the limit between the endocardium and the valve tissue could be the end of the blood vessels.Furthermore, in the present case,it is very curious that there was a calcified stone floating in the cystic cavity. This stone was similar to those found in the gall bladder. Despite a search of the literature, no other reports of a blood cyst, either originating from the right atria1 septum or containing a stone, were found.
261
Case repor& In conclusion, although the mechanisms of occlusion are ambiguous, the present case could constitute evidence to support the ‘sudden occlusion theory’.
References 1. Levinson SA, Learner A. Blood cysts on the heart valves of newborn infants. Arch Puthol1932; 14: 810-17. 2. Dow DR, Harper WF. Blood cysts in human cardiac valves. J Anat 1936; 71: 117-23. 3. Boyd TAB. Blood cysts on the heart valves of infants. Am J Pathol 1949; 25: 757-9.
4. Leatherman L, Leachman RD, Hallman GL, Cooley DA. Cyst of
262
the mitral valve. Am J Curdiol 1968; 21: 428-30. 5. Galluci V, Stritoni P, Fasoli G, Thiene G. Giant blood cyst of tricuspid valve: successfulexcision in an infant. Br Heart J 1976; 38: 990-2.
Liese GJ, Brainward SC, Goto U. Giant blood cyst of the pulmonary valve. N Engl J Med 1963; 269: 465-7. Elsasser C. Bericht uber die Ereignisse in der Gebaranstalt des Catherinen-Hospital im lahre 1844. Med Correspondenzblutt 1844; 14: 297.. ” Sakakibara S, Katsuhara K, Iida Y, Nishida H. Pulmonary subvalvular tumor. Dis Chest 1967; 51: 637-41. raper accepted 26 October 1994
CARDIOVASCULAR SURGERY APRIL 1996 VOL4 NO2