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Anomalous origin of the right coronary artery from the left aortic sinus and sudden infant death
International Journal of Cardiology 45 (1994) 147-149
Anomalous origin of the right coronary artery from the left aortic sinus and sudden infant death Ana C. Duran, Annalisa Angelini, Carla Frescura, Cristina Basso, Gaetano Thiene* Institute of Pathological
Anatomy,
University of Padka Medical School, Via A. Gabelli 61. 35121 Padua. Italy
(Received 14 February 1994; revision accepted 30 March 1994)
Abstract
The origin of the right coronary artery from the left aortic sinus is an uncommon anomaly, which has been shown to be a cause of sudden cardiac death in young and adult patients, often in association with physical exertion. So far, to the best of our knowledge, only six cases of sudden death related to this anomaly have been reported in newborns and infants. We describe here a further case which reinforces the need of precise postmortem examination in these fatalities. Key words: Coronary artery anomalies; Sudden death
1. Introduction
2. Case report
The anomalous origin of the right coronary artery from the left aortic sinus of Valsalva has been shown to be a cause of sudden cardiac death in young and adult patients, often in association with physical exertion [1,2]. So far, to the best of our knowledge, only six cases of sudden death related to this anomaly have been reported in newborns and infants [3-71 (Table 1). We describe here a further case which reinforces the need of precise postmortem examination in these fatalities.
A 3-month-old male baby, previously healthy, was found dead in his crib. At postmortem evaluation, the infant weighed 6.570 kg and no anomalies were found except for the heart. There was leyocardia with atria1 situs solitus, atrioventricular and ventriculoarterial concordance. Venous return, septa and arterial outflow tracts were normal. The heart weighed 35.5 g (normal values for age and weight, 30 i 7 g). The maximal thickness of the left ventricular free wall and interventricular septum was 5 mm, while that of the right ventricular free wall was 3 mm. No grossly evidence of myocardial infarction was observed. The left main and right coronary arteries arose from two separate ostia, above
* Corresponding author.
0167-5273/94/$07.00 0 1994 Elsevier Science Ireland Ltd. All rights reserved. SSDI 0167-5273(94)02059-R
A.C. Duran et al. /hr.
148
J. Cardiol. 45 (1994)
147-149
Table I Previously reported cases of sudden death in neonates and infants related to the anomalous origin of the right coronary artery from the left aortic sinus Authors
Case
Sex/age
Circumstance of death
Coronarv artery pattern
Liberthson et al. [3] Muus and McManus [4]
I 2
M/9 months M/Stillborn
Right coronary dominance Balanced coronary pattern
Barth et al. [5] Ness and McManus ]6] Lip&t et al. [7] Lipsett et al. [7]
3 4 5 6
F/4 months F/2 months M/3 months M/7 months
Cardiac arrest while sleeping With the progression of spontaneous labor Found dead in his crib Cardiac arrest in her crib Found dead in his parents’ bed Cardiac arrest after a bath
the left sinus of Valsalva, just over the sinotubular junction (Fig. 1). The right coronary artery was dominant, originated from a circular orifice and had an acute angle takeoff. The right coronary artery, the proximal portion of which coursed intramurally within the aortic tunica media, passed between the aorta and the pulmonary trunk, in a rightward direction to attain its usual location in the right atrioventricular sulcus. The orifice of the left coro-
Right coronary dominance Right coronary dominance No information No information
nary artery was round and adjacent to that of the right coronary artery. Branching and distribution of the left coronary artery was normal. 3. Discussion The origin of the right coronary artery from the left aortic sinus is an uncommon anomaly, with an estimated frequency at angiography of about 0.19%
Fig. I. Anomalous origin of the right coronary artery from the left aortic sinus. The left ventricular outflow tract and the ascending aorta have been opened to show the aortic valve. Both coronary arteries arise above the left coronary sinus, just over the sinotubular junction. Abbreviations: AM, anterior cusp of the mitral valve; L, left aortic leaflet; LC, left coronary orifice; P, posterior aortic leaflet; R, right aortic leaflet; RC, right coronary orifice.
A.C. Duran et al. /ht.
J. Cardiol. 45 (1994)
149
147-149
[S]. Our group has previously described two cases of this coronary anomaly in young patients, both associated with sudden death occurring during effort ]2]. It has been postulated that the reduction of coronary blood flow may produce acute myocardial ischaemia in this condition resulting in cardiac arrhythmia and sudden death [I ,2,9]. Several mechanisms have been proposed to explain how the anomalous origin of the right coronary artery from the left sinus of Valsalva can cause myocardial ischaemia and cardiac death. Virmani et al. suggested that the acute takeoff at the origin of the coronary artery can be worsened when dilatation of the aorta occurs, therefore producing a fall in flow blood [9], According to Roberts et al., since the anomalous artery courses between the aorta and the pulmonary trunk, it might be compressed when pressure increases in these vessels such as during exercise [ 11. The intramural course of the anomalous coronary artery in the aortic tunica media may be crucial because it produces a slit-like lumen; when the diastolic filling time of the coronary arteries is shortened such as during effort tachycardia, the adequate blood flow through the anomalous artery may be compromised [2]. Even a prolonged spasm can not be overlooked as a possible cause of reduction of blood flow in the anomalous artery. More recently, Kragel and Roberts have pointed out that the right coronary dominance can be a determinant in the patient’s outcome [IO]. In our case, all the anatomic features which had been previously related to an increase risk of sudden death in patients with anomalous origin of the right coronary artery were present, such as sharp angle origin, intramural aortic course with the subsequent running between aorta and pulmonary trunk, and right dominance. We think that the ultimate event was cardiac arrhythmia precipitated by an episode of acute ischaemia. How ischaemia can be precipitated while sleeping is intriguing. We can speculate that either the baby could have cried with a consequent rise in pulmonary pressure and heart rate or sympathetic stimulation during REM sleep might have caused a cardiac stress, namely tachycardia and increased blood pressure. The anomalous origin of the right coronary artery from the left sinus of Valsalva is not a minor entity
at all. Sudden infant death syndrome, by definition, should not exhibit an organic substrate, but there is a strong suspicion that coronary arterial anomalies are overlooked by paediatric pathologists. Dissection down onto the aortic valve from above and checking each coronary arterial orifice before doing anything to the heart is highly advisable. Careful inspection of the origin of the coronary arteries should be carried out in cases of apparent sudden infant death syndrome to rule out a possible role of a coronary artery anomaly as an anatomic substrate for this fatal event. 4. References 1
Roberts WC, Siegel RJ, Zipes DF. Origin of the right coronary artery from the left sinus of Valsalva and its functional consequences: analysis of 10 necropsy patiens. Am J Cardiol 1982: 49: 863-868.
2
Corrado
D. Thiene G, Nava A, Rossi L, Fennelli N. Sud-
den death in young competitive athletes: clinicopathologic correlations 3
Liberthson
in 22 cases. Am J Med 1990; 89: 588-596. RR, Gang DL, Custer J. Sudden death in an
infant with aberrant origin of the right coronary artery from the left sinus of Valsalva of the aorta: case report and re4
view of the literature.
Pediatr Cardiol
Muus CJ. MacManus
BM. Common
left coronary Valsalva:
arteries
association
from
1983; 4: 45-48. origin of right and
the region
of left sinus of
with unexpected
intrauterine
fetal
death. Am Heart J 1984: 107: 1285-1286. 5
Barth III CW. Bray M, Roberts WC. Sudden death in infancy associated with origin of both left main and right coronary arteries from a common ostium above the left sinus of Valsalva.
6
Am J Cardiol
Ness MJ. MacManus
1986: 57: 365-366.
BM. Anomalous right coronary artery
origin in otherwise unexplained infant death. Arch Path01 Lab Med 1988; 112: 626-629. 7
Lips&t JL. Byard RW, Carpenter BF, Jimenez CL, Bourne AJ. Anomalous coronary arteries arising from the aorta associated with sudden death in infancy and early childhood. An
autopsy
series. Arch
Pathol
Lab
Med
1991;
115:
770-773. 8
Chaitman BR, Lesperance J, Saltiel J. Bourassa MG. Clinical, angiographic. with anomalous
and hemodynamic
findings in patients
origin of the coronary
arteries. Circula-
tion 1976: 53: 122-131. 9
Virmani
R. Chun
McAllister
HA.
FK,
Goldstein
Acute takeoffs
RE,
Robinowitz
of the coronary
M.
arteries
along the aortic wall and congenital coronary ostial valvelike ridges: association with sudden death. J Am Coll Cardiol 1984: 766-771. IO
Kragel AH.
Roberts WC. Anomalous
origin of either the
right or left main coronary artery from the aorta with subsequent coursing between aorta and pulmonary trunk: analysis of 32 necropsy cases. Am J Cardiol
1988: 62: 771-777.
Anomalous origin of the right coronary artery from the left aortic sinus and sudden infant death Ana C. Duran, Annalisa Angelini, Carla Frescura, Cristina Basso, Gaetano Thiene* Institute of Pathological
Anatomy,
University of Padka Medical School, Via A. Gabelli 61. 35121 Padua. Italy
(Received 14 February 1994; revision accepted 30 March 1994)
Abstract
The origin of the right coronary artery from the left aortic sinus is an uncommon anomaly, which has been shown to be a cause of sudden cardiac death in young and adult patients, often in association with physical exertion. So far, to the best of our knowledge, only six cases of sudden death related to this anomaly have been reported in newborns and infants. We describe here a further case which reinforces the need of precise postmortem examination in these fatalities. Key words: Coronary artery anomalies; Sudden death
1. Introduction
2. Case report
The anomalous origin of the right coronary artery from the left aortic sinus of Valsalva has been shown to be a cause of sudden cardiac death in young and adult patients, often in association with physical exertion [1,2]. So far, to the best of our knowledge, only six cases of sudden death related to this anomaly have been reported in newborns and infants [3-71 (Table 1). We describe here a further case which reinforces the need of precise postmortem examination in these fatalities.
A 3-month-old male baby, previously healthy, was found dead in his crib. At postmortem evaluation, the infant weighed 6.570 kg and no anomalies were found except for the heart. There was leyocardia with atria1 situs solitus, atrioventricular and ventriculoarterial concordance. Venous return, septa and arterial outflow tracts were normal. The heart weighed 35.5 g (normal values for age and weight, 30 i 7 g). The maximal thickness of the left ventricular free wall and interventricular septum was 5 mm, while that of the right ventricular free wall was 3 mm. No grossly evidence of myocardial infarction was observed. The left main and right coronary arteries arose from two separate ostia, above
* Corresponding author.
0167-5273/94/$07.00 0 1994 Elsevier Science Ireland Ltd. All rights reserved. SSDI 0167-5273(94)02059-R
A.C. Duran et al. /hr.
148
J. Cardiol. 45 (1994)
147-149
Table I Previously reported cases of sudden death in neonates and infants related to the anomalous origin of the right coronary artery from the left aortic sinus Authors
Case
Sex/age
Circumstance of death
Coronarv artery pattern
Liberthson et al. [3] Muus and McManus [4]
I 2
M/9 months M/Stillborn
Right coronary dominance Balanced coronary pattern
Barth et al. [5] Ness and McManus ]6] Lip&t et al. [7] Lipsett et al. [7]
3 4 5 6
F/4 months F/2 months M/3 months M/7 months
Cardiac arrest while sleeping With the progression of spontaneous labor Found dead in his crib Cardiac arrest in her crib Found dead in his parents’ bed Cardiac arrest after a bath
the left sinus of Valsalva, just over the sinotubular junction (Fig. 1). The right coronary artery was dominant, originated from a circular orifice and had an acute angle takeoff. The right coronary artery, the proximal portion of which coursed intramurally within the aortic tunica media, passed between the aorta and the pulmonary trunk, in a rightward direction to attain its usual location in the right atrioventricular sulcus. The orifice of the left coro-
Right coronary dominance Right coronary dominance No information No information
nary artery was round and adjacent to that of the right coronary artery. Branching and distribution of the left coronary artery was normal. 3. Discussion The origin of the right coronary artery from the left aortic sinus is an uncommon anomaly, with an estimated frequency at angiography of about 0.19%
Fig. I. Anomalous origin of the right coronary artery from the left aortic sinus. The left ventricular outflow tract and the ascending aorta have been opened to show the aortic valve. Both coronary arteries arise above the left coronary sinus, just over the sinotubular junction. Abbreviations: AM, anterior cusp of the mitral valve; L, left aortic leaflet; LC, left coronary orifice; P, posterior aortic leaflet; R, right aortic leaflet; RC, right coronary orifice.
A.C. Duran et al. /ht.
J. Cardiol. 45 (1994)
149
147-149
[S]. Our group has previously described two cases of this coronary anomaly in young patients, both associated with sudden death occurring during effort ]2]. It has been postulated that the reduction of coronary blood flow may produce acute myocardial ischaemia in this condition resulting in cardiac arrhythmia and sudden death [I ,2,9]. Several mechanisms have been proposed to explain how the anomalous origin of the right coronary artery from the left sinus of Valsalva can cause myocardial ischaemia and cardiac death. Virmani et al. suggested that the acute takeoff at the origin of the coronary artery can be worsened when dilatation of the aorta occurs, therefore producing a fall in flow blood [9], According to Roberts et al., since the anomalous artery courses between the aorta and the pulmonary trunk, it might be compressed when pressure increases in these vessels such as during exercise [ 11. The intramural course of the anomalous coronary artery in the aortic tunica media may be crucial because it produces a slit-like lumen; when the diastolic filling time of the coronary arteries is shortened such as during effort tachycardia, the adequate blood flow through the anomalous artery may be compromised [2]. Even a prolonged spasm can not be overlooked as a possible cause of reduction of blood flow in the anomalous artery. More recently, Kragel and Roberts have pointed out that the right coronary dominance can be a determinant in the patient’s outcome [IO]. In our case, all the anatomic features which had been previously related to an increase risk of sudden death in patients with anomalous origin of the right coronary artery were present, such as sharp angle origin, intramural aortic course with the subsequent running between aorta and pulmonary trunk, and right dominance. We think that the ultimate event was cardiac arrhythmia precipitated by an episode of acute ischaemia. How ischaemia can be precipitated while sleeping is intriguing. We can speculate that either the baby could have cried with a consequent rise in pulmonary pressure and heart rate or sympathetic stimulation during REM sleep might have caused a cardiac stress, namely tachycardia and increased blood pressure. The anomalous origin of the right coronary artery from the left sinus of Valsalva is not a minor entity
at all. Sudden infant death syndrome, by definition, should not exhibit an organic substrate, but there is a strong suspicion that coronary arterial anomalies are overlooked by paediatric pathologists. Dissection down onto the aortic valve from above and checking each coronary arterial orifice before doing anything to the heart is highly advisable. Careful inspection of the origin of the coronary arteries should be carried out in cases of apparent sudden infant death syndrome to rule out a possible role of a coronary artery anomaly as an anatomic substrate for this fatal event. 4. References 1
Roberts WC, Siegel RJ, Zipes DF. Origin of the right coronary artery from the left sinus of Valsalva and its functional consequences: analysis of 10 necropsy patiens. Am J Cardiol 1982: 49: 863-868.
2
Corrado
D. Thiene G, Nava A, Rossi L, Fennelli N. Sud-
den death in young competitive athletes: clinicopathologic correlations 3
Liberthson
in 22 cases. Am J Med 1990; 89: 588-596. RR, Gang DL, Custer J. Sudden death in an
infant with aberrant origin of the right coronary artery from the left sinus of Valsalva of the aorta: case report and re4
view of the literature.
Pediatr Cardiol
Muus CJ. MacManus
BM. Common
left coronary Valsalva:
arteries
association
from
1983; 4: 45-48. origin of right and
the region
of left sinus of
with unexpected
intrauterine
fetal
death. Am Heart J 1984: 107: 1285-1286. 5
Barth III CW. Bray M, Roberts WC. Sudden death in infancy associated with origin of both left main and right coronary arteries from a common ostium above the left sinus of Valsalva.
6
Am J Cardiol
Ness MJ. MacManus
1986: 57: 365-366.
BM. Anomalous right coronary artery
origin in otherwise unexplained infant death. Arch Path01 Lab Med 1988; 112: 626-629. 7
Lips&t JL. Byard RW, Carpenter BF, Jimenez CL, Bourne AJ. Anomalous coronary arteries arising from the aorta associated with sudden death in infancy and early childhood. An
autopsy
series. Arch
Pathol
Lab
Med
1991;
115:
770-773. 8
Chaitman BR, Lesperance J, Saltiel J. Bourassa MG. Clinical, angiographic. with anomalous
and hemodynamic
findings in patients
origin of the coronary
arteries. Circula-
tion 1976: 53: 122-131. 9
Virmani
R. Chun
McAllister
HA.
FK,
Goldstein
Acute takeoffs
RE,
Robinowitz
of the coronary
M.
arteries
along the aortic wall and congenital coronary ostial valvelike ridges: association with sudden death. J Am Coll Cardiol 1984: 766-771. IO
Kragel AH.
Roberts WC. Anomalous
origin of either the
right or left main coronary artery from the aorta with subsequent coursing between aorta and pulmonary trunk: analysis of 32 necropsy cases. Am J Cardiol
1988: 62: 771-777.