- Email: [email protected]
Anomalous origin of the right coronary artery from the pulmonary artery: an unusual cause of angina
Curdiovascular
UTTERWORTH E ! NEMANN
Surgery, Vol. 3, No. 1. pp. 81-83. 1995
Copyright 0 1995 Elsevier Science Ltd Printed in Great Britain. All rights reserved Oh?-2109!95
f10.0(! + 0.00
AnumWws origin of the ri the pulmonary artery: an J. S. Ladowski, D. A. Belvedere* and L. F. Wuest+ Departments of Cardiovascular and Thoracic Surgery. *Cardiology and ‘Family Practice, St Jweph Medical Center, Fort Wayne, Indiana, USA
Origin of the right coronary artery from the pulmonary trunk rather than the aorta is a rare congenital anomaly. Only five cases of this anomaly have presented with signs and symptoms of ischemia. One of these cases, along with objective evidence for the cause of the ischemia and recommendations regarding surgical repair, is presented here. Keywords: coronary anomaly,congenital defect. angina _-
_.....__ --. -...l_.--.---__
Although anomalous origin of the right coronary artery from the pulmonary artery is the second most common type of congenital coronary anomaly, it has only rarely caused documented ischemia of the myocardium. Furthermore, the location of such ischemia is unclear. This unusual experiment of nature allows a unique opportunity to study the effect of very efficient collaterals from the left coronary system (if present) which are drained into a low-pressure vascular space. Anterior and anterolateral ischemia in a patient with anomalous right coronary artery arising from the pulmonary artery was documented, and correction of this ischemia with surgical relocation of the right coronary artery to the aorta confirmed.
Case report A 59-year-old black woman presented with a severalyear history of chest tightness which radiated to the left arm. The pain was initially brought on by exertion, and for the 2 months before presentation had been increasing in frequency and intensity. She noted recent dyspnea as well as chest pain with these episodes and experienced relief with rest. She denied symptoms of congestive heart failure. Her risk factors included insulin-dependent diabetes mellitus and a distant history of tobacco abuse. Her father died at an early age of a ‘heart attack’. Physical examination was remarkable for a l/6 holosystolic murmur at the apex of the heart and an S4 gallop.
Correspondence to: Dr J. S. Ladowski, Indiana/Ohio Heart, 7910 West Jefferson Blvd - Suite 102, Fort Wayne, In 46 804, USA
CARDIOVASCULAR SURGERY FEBRUARY1995 VOL3 NO 1
Figure 1 Injection of contrast into left anterior showing the generous size of the coronary artery
descending artery
An adenosine thallium study demonstrated reversible ischemia in the anterior and anterolateral walls. Cardiac catheterization revealed an enormous left anterior descending artery arising from the usual aortic position (Figure I), with prompt filling of the large right coronary artery via collaterals. The right coronary artery, filled during the venous phase of the injection, drained into the root of the pulmonary artery (Figure 2). In April 1992, the patient underwenr surgery via a midline sternotomy. The left anterior descending artery was very tortuous, with a diameter in excessof l.Ocm. Cardiopulmonary bypass was employed and continuous warm retrograde cardioplegia used to insure a quiet operative field during the dissection of the right coronary artery, removal of the right coronary artery 81
Cardiac case repor&
Figure 2 ‘Venous-phase’ of the left anterior descending injection showing the large right coronary artery draining into the pulmonary artery
from the pulmonary artery, and repair of the resultant defect with a pericardial patch. The Carrel1 patch of pulmonary artery (with the origin of right coronary artery) was then anastomosed to the ventral aspect of the aorta, with the aid of a partial clamp. The large (l.O-cm) right coronary artery was thus transposed to the ascending aorta. The postoperative course was unremarkable. The patient has had no further angina and her dyspnea is entirely resolved. A follow-up thallium scan in August 1993 demonstrated no evidence of ischemia.
Discussion Soloff’ described four types of anomalous origination of coronary arteries from the pulmonary artery. Of these, origin of right coronary artery was the second most common type, following anomalous origin of the left coronary artery from the pulmonary artery but more common than origin of both coronaries from the pulmonary artery or presence of an accessory coronary arising from the pulmonary artery. Although more than 20 cases of anomalous origin of the right coronary artery from the pulmonary artery have been reported, very few documented cases of ischemia arising from this anomaly have been noted. Pribble2 reported angina in a patient with a history of rheumatic heart disease. Autopsy confirmed the presence of ischemic heart disease, without coronary occlusions. A right coronary artery arising from the pulmonary artery was found, and the ischemic changes were thought to be ‘attributed to an inadequate supply of oxygen by an anomalous right coronary artery to a myocardium overburdened with rheumatic involvement’. Wald et a1.3 reported a 17-year-old woman who suffered sudden death and was found on autopsy to have a right coronary artery arising from the pulmonary artery. Fresh thrombus was noted in the left coronary 82
artery system. Bregman and colleagues4 reported successful relocation of the anomalous right coronary artery to the aorta. This was the first such relocation to be performed for signs of ischemia (cardiac arrest in a 25-year-old woman). Lerberg et al.’ noted crib death in a 2-year-old boy found on autopsy to have a right coronary artery arising from the pulmonary artery. Most recent reported cases of right coronary artery arising from the pulmonary artery without an associated congenital defect have been discovered because of a symptom-free murmur3>5-10. Unlike anomalous origin of the left main coronary from the pulmonary artery, anomalous origin of only the left anterior descending or right coronary artery from the pulmonary artery is less likely to cause ischemia. The location of ischemia is usually anterior in anomalous origin of the left anterior descending from the pulmonary artery”. This is thought to result from inadequate perfusion of the anterior portion of the thicker-walled left ventricle via the right coronary artery and circumflex systems. Interestingly, the location of ischemia in the present patient was also anterior. Because the blood supply to this area is from the normal left anterior descending artery, the authors are forced to speculate that this ischemia represents ‘steal’ from the anterior wall of the left ventricle via the right coronary artery-to-pulmonary artery channel. The authors’ finding of a massively dilated right coronary artery (as noted by others) seems to support the concept that the large-caliber artery is capable of ‘stealing’ from the left anterior descending capillary system. The reason for anterior as opposed to inferior ischemia is unclear. Presumably, the inferior wall has the same thickness, wall stress and oxygen consumption perg of myocardium as does the anterior wall. Why, then, should the inferior wall steal from the anterior wall? Most likely, the steal phenomenon affects the entire left ventricle. This is not a steal from the left anterior descending to the right coronary artery, but truly it is from the aorta to the pulmonary artery (it is, in fact, a long arteriovenous fistula). In the present patient, the least likely distribution to be ischemic is that of the circumflex, since it was least involved in the collateral supply of the posterior descending artery. Indeed, at cardiac catheterization the contrast remained in the circumflex system for a greater time than it did in the left anterior descending system. The relative abundance of circumflex collaterals to the inferior wall, perhaps combined with a relative abundance of septal collaterals between the left anterior descending and right coronary artery may account for anterior ischemia in this patient. If this is the case, the ‘most ischemic wall’ could vary from patient to patient depending on the small vessel and collateral anatomy of that individual. Unfortunately, there are not enough of these patients available to make the observations that would confirm or deny this impression. Although relocation of the anomalous right coronary artery to the aorta has been described without CARDIOVASCULAR SURGERY FEBRUARY 199s VOL 3 NO 1
cardiopulmonary bypass4, the authors feel that the relative safety of current bypass techniques, with cardioplegia to achieve a quiet operative field, more than outweigh the potential complications of the surgery. The technique of relocation of the right coronary artery to the aorta with pericardial patch closure of the pulmonary artery defect has a strong precedent in normal anatomy to recommend it, and should be used in preference to bypass with ligation of the right coronary artery. A theoretical disadvantage to the use of a pericardial patch is the possibility of aneurysmal dilatation. In the low-pressure pulmonary artery system, this is not likely to be a concern, Ligation of the right coronary artery without bypass, as reported in 1960’, should no longer be considered a surgical option. In conclusion, a case of right coronary artery arising from the pulmonary artery, presenting with ischemia in the anterior and anterolateral walls, is presented. The likely cause of these ischemic changes is ‘steal’ from the normal left anterior descending artery through the aberrant right coronary artery to the pulmonary artery shunt. Because it restores a dual coronary system, surgical correction should consist of relocation of the right coronary artery to the aorta.
2.
3. 4. 5. 6.
7.
8. 9.
10.
11.
Pribble RH. Anatomic variations of the coronary arteries and their clinical significance. 1 Indiana State Med Assnc 1961; 54: 329-33. Wald S, Stonecipher K, Baldwin BJ et al. .4nomalous origin of the right coronary artery from the puhnonary artery. Am / Curdiol 1971; 27: 677-81. Bregman D, Brennan F, Singer A et al. Anomalous orrgm of the right coronary artery from the pulmonary arrery. j Tboruc Cardiovasc Surg 1976; 72: 626-30. Lerberg DB, Ogden JA, Zuberbuhler JR et al. Anomalous or+ of the right coronary artery from the pulmonary artery. Ann Tborac Surg 1978; 27: 87-94. Cronk ES, Sinclair JG, Rigdon RH. An anomalous right coronary artery arising from the pulmonary artery. Am Heart r 1951; 42: 906-11. Rowe GG, Young WP. Anomalous origtn of the coronary arteries with special reference to surgical treatment. / ThorJc Cardiovasc Surg 1960; 39: 777-80. Raminger K, Thilenius 0, Cassels DC. Angiographic diagnosis of an anomalous right coronary artery arising from the pulmonary artery. Radiology 1967; 88: 29-3 1, Tingelstad JB, Lower RR, Eldredge WJ. Anomalous origin of the right coronary artery from the main pulmonary artery. Am / Cmdiol 1972; 30: 670-3. Worsham C, Sanders SP, Burger BM. Ortgin of the right coronary artery from the pulmonary trunk: diagnosis by two-dimensional echocardiography. Am 1 Car&o/ 1985; 55: 232-8. Derrick MJ, Moreno-Cabral RJ. Anomalous origin of the left anterior descending artery from the pulmonarv artery. i Crrdinr Surgery 1992: 6: 24-8.
References 1.
Soloff LA. Anomalous coronary arteries arising pulmonary artery. Am Heart ] 1942; 24: 118-25.
CARDIOVASCULAR SURGERY FEBRUARY 1995 VOL 3 NO 1
from
the Paper accepted 9 March 1994
83
UTTERWORTH E ! NEMANN
Surgery, Vol. 3, No. 1. pp. 81-83. 1995
Copyright 0 1995 Elsevier Science Ltd Printed in Great Britain. All rights reserved Oh?-2109!95
f10.0(! + 0.00
AnumWws origin of the ri the pulmonary artery: an J. S. Ladowski, D. A. Belvedere* and L. F. Wuest+ Departments of Cardiovascular and Thoracic Surgery. *Cardiology and ‘Family Practice, St Jweph Medical Center, Fort Wayne, Indiana, USA
Origin of the right coronary artery from the pulmonary trunk rather than the aorta is a rare congenital anomaly. Only five cases of this anomaly have presented with signs and symptoms of ischemia. One of these cases, along with objective evidence for the cause of the ischemia and recommendations regarding surgical repair, is presented here. Keywords: coronary anomaly,congenital defect. angina _-
_.....__ --. -...l_.--.---__
Although anomalous origin of the right coronary artery from the pulmonary artery is the second most common type of congenital coronary anomaly, it has only rarely caused documented ischemia of the myocardium. Furthermore, the location of such ischemia is unclear. This unusual experiment of nature allows a unique opportunity to study the effect of very efficient collaterals from the left coronary system (if present) which are drained into a low-pressure vascular space. Anterior and anterolateral ischemia in a patient with anomalous right coronary artery arising from the pulmonary artery was documented, and correction of this ischemia with surgical relocation of the right coronary artery to the aorta confirmed.
Case report A 59-year-old black woman presented with a severalyear history of chest tightness which radiated to the left arm. The pain was initially brought on by exertion, and for the 2 months before presentation had been increasing in frequency and intensity. She noted recent dyspnea as well as chest pain with these episodes and experienced relief with rest. She denied symptoms of congestive heart failure. Her risk factors included insulin-dependent diabetes mellitus and a distant history of tobacco abuse. Her father died at an early age of a ‘heart attack’. Physical examination was remarkable for a l/6 holosystolic murmur at the apex of the heart and an S4 gallop.
Correspondence to: Dr J. S. Ladowski, Indiana/Ohio Heart, 7910 West Jefferson Blvd - Suite 102, Fort Wayne, In 46 804, USA
CARDIOVASCULAR SURGERY FEBRUARY1995 VOL3 NO 1
Figure 1 Injection of contrast into left anterior showing the generous size of the coronary artery
descending artery
An adenosine thallium study demonstrated reversible ischemia in the anterior and anterolateral walls. Cardiac catheterization revealed an enormous left anterior descending artery arising from the usual aortic position (Figure I), with prompt filling of the large right coronary artery via collaterals. The right coronary artery, filled during the venous phase of the injection, drained into the root of the pulmonary artery (Figure 2). In April 1992, the patient underwenr surgery via a midline sternotomy. The left anterior descending artery was very tortuous, with a diameter in excessof l.Ocm. Cardiopulmonary bypass was employed and continuous warm retrograde cardioplegia used to insure a quiet operative field during the dissection of the right coronary artery, removal of the right coronary artery 81
Cardiac case repor&
Figure 2 ‘Venous-phase’ of the left anterior descending injection showing the large right coronary artery draining into the pulmonary artery
from the pulmonary artery, and repair of the resultant defect with a pericardial patch. The Carrel1 patch of pulmonary artery (with the origin of right coronary artery) was then anastomosed to the ventral aspect of the aorta, with the aid of a partial clamp. The large (l.O-cm) right coronary artery was thus transposed to the ascending aorta. The postoperative course was unremarkable. The patient has had no further angina and her dyspnea is entirely resolved. A follow-up thallium scan in August 1993 demonstrated no evidence of ischemia.
Discussion Soloff’ described four types of anomalous origination of coronary arteries from the pulmonary artery. Of these, origin of right coronary artery was the second most common type, following anomalous origin of the left coronary artery from the pulmonary artery but more common than origin of both coronaries from the pulmonary artery or presence of an accessory coronary arising from the pulmonary artery. Although more than 20 cases of anomalous origin of the right coronary artery from the pulmonary artery have been reported, very few documented cases of ischemia arising from this anomaly have been noted. Pribble2 reported angina in a patient with a history of rheumatic heart disease. Autopsy confirmed the presence of ischemic heart disease, without coronary occlusions. A right coronary artery arising from the pulmonary artery was found, and the ischemic changes were thought to be ‘attributed to an inadequate supply of oxygen by an anomalous right coronary artery to a myocardium overburdened with rheumatic involvement’. Wald et a1.3 reported a 17-year-old woman who suffered sudden death and was found on autopsy to have a right coronary artery arising from the pulmonary artery. Fresh thrombus was noted in the left coronary 82
artery system. Bregman and colleagues4 reported successful relocation of the anomalous right coronary artery to the aorta. This was the first such relocation to be performed for signs of ischemia (cardiac arrest in a 25-year-old woman). Lerberg et al.’ noted crib death in a 2-year-old boy found on autopsy to have a right coronary artery arising from the pulmonary artery. Most recent reported cases of right coronary artery arising from the pulmonary artery without an associated congenital defect have been discovered because of a symptom-free murmur3>5-10. Unlike anomalous origin of the left main coronary from the pulmonary artery, anomalous origin of only the left anterior descending or right coronary artery from the pulmonary artery is less likely to cause ischemia. The location of ischemia is usually anterior in anomalous origin of the left anterior descending from the pulmonary artery”. This is thought to result from inadequate perfusion of the anterior portion of the thicker-walled left ventricle via the right coronary artery and circumflex systems. Interestingly, the location of ischemia in the present patient was also anterior. Because the blood supply to this area is from the normal left anterior descending artery, the authors are forced to speculate that this ischemia represents ‘steal’ from the anterior wall of the left ventricle via the right coronary artery-to-pulmonary artery channel. The authors’ finding of a massively dilated right coronary artery (as noted by others) seems to support the concept that the large-caliber artery is capable of ‘stealing’ from the left anterior descending capillary system. The reason for anterior as opposed to inferior ischemia is unclear. Presumably, the inferior wall has the same thickness, wall stress and oxygen consumption perg of myocardium as does the anterior wall. Why, then, should the inferior wall steal from the anterior wall? Most likely, the steal phenomenon affects the entire left ventricle. This is not a steal from the left anterior descending to the right coronary artery, but truly it is from the aorta to the pulmonary artery (it is, in fact, a long arteriovenous fistula). In the present patient, the least likely distribution to be ischemic is that of the circumflex, since it was least involved in the collateral supply of the posterior descending artery. Indeed, at cardiac catheterization the contrast remained in the circumflex system for a greater time than it did in the left anterior descending system. The relative abundance of circumflex collaterals to the inferior wall, perhaps combined with a relative abundance of septal collaterals between the left anterior descending and right coronary artery may account for anterior ischemia in this patient. If this is the case, the ‘most ischemic wall’ could vary from patient to patient depending on the small vessel and collateral anatomy of that individual. Unfortunately, there are not enough of these patients available to make the observations that would confirm or deny this impression. Although relocation of the anomalous right coronary artery to the aorta has been described without CARDIOVASCULAR SURGERY FEBRUARY 199s VOL 3 NO 1
cardiopulmonary bypass4, the authors feel that the relative safety of current bypass techniques, with cardioplegia to achieve a quiet operative field, more than outweigh the potential complications of the surgery. The technique of relocation of the right coronary artery to the aorta with pericardial patch closure of the pulmonary artery defect has a strong precedent in normal anatomy to recommend it, and should be used in preference to bypass with ligation of the right coronary artery. A theoretical disadvantage to the use of a pericardial patch is the possibility of aneurysmal dilatation. In the low-pressure pulmonary artery system, this is not likely to be a concern, Ligation of the right coronary artery without bypass, as reported in 1960’, should no longer be considered a surgical option. In conclusion, a case of right coronary artery arising from the pulmonary artery, presenting with ischemia in the anterior and anterolateral walls, is presented. The likely cause of these ischemic changes is ‘steal’ from the normal left anterior descending artery through the aberrant right coronary artery to the pulmonary artery shunt. Because it restores a dual coronary system, surgical correction should consist of relocation of the right coronary artery to the aorta.
2.
3. 4. 5. 6.
7.
8. 9.
10.
11.
Pribble RH. Anatomic variations of the coronary arteries and their clinical significance. 1 Indiana State Med Assnc 1961; 54: 329-33. Wald S, Stonecipher K, Baldwin BJ et al. .4nomalous origin of the right coronary artery from the puhnonary artery. Am / Curdiol 1971; 27: 677-81. Bregman D, Brennan F, Singer A et al. Anomalous orrgm of the right coronary artery from the pulmonary arrery. j Tboruc Cardiovasc Surg 1976; 72: 626-30. Lerberg DB, Ogden JA, Zuberbuhler JR et al. Anomalous or+ of the right coronary artery from the pulmonary artery. Ann Tborac Surg 1978; 27: 87-94. Cronk ES, Sinclair JG, Rigdon RH. An anomalous right coronary artery arising from the pulmonary artery. Am Heart r 1951; 42: 906-11. Rowe GG, Young WP. Anomalous origtn of the coronary arteries with special reference to surgical treatment. / ThorJc Cardiovasc Surg 1960; 39: 777-80. Raminger K, Thilenius 0, Cassels DC. Angiographic diagnosis of an anomalous right coronary artery arising from the pulmonary artery. Radiology 1967; 88: 29-3 1, Tingelstad JB, Lower RR, Eldredge WJ. Anomalous origin of the right coronary artery from the main pulmonary artery. Am / Cmdiol 1972; 30: 670-3. Worsham C, Sanders SP, Burger BM. Ortgin of the right coronary artery from the pulmonary trunk: diagnosis by two-dimensional echocardiography. Am 1 Car&o/ 1985; 55: 232-8. Derrick MJ, Moreno-Cabral RJ. Anomalous origin of the left anterior descending artery from the pulmonarv artery. i Crrdinr Surgery 1992: 6: 24-8.
References 1.
Soloff LA. Anomalous coronary arteries arising pulmonary artery. Am Heart ] 1942; 24: 118-25.
CARDIOVASCULAR SURGERY FEBRUARY 1995 VOL 3 NO 1
from
the Paper accepted 9 March 1994
83