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Congenital coronary artery fistula from supernumerary coronary artery
Congenital coronary artery fistula from supernumerary coronary artery Case report and review of the literature Frederick B. Parker, Jr., M.D., John F. Neville, Jr., M.D., Lewis W. Johnson; M.D., Joseph V. Scrivani, M.D., and Watts R. Webb, M.D., Syracuse, N. Y.
This report concerns a patient with a supernumerary coronary artery-right atrial fistula which has been surgically corrected. The vessel involved was an accessory right coronary artery that arose from the right coronary sinus of Valsalva. Six other cases of fistulas from supernumerary coronary arteries have been reported upon, but only 1 involved an operation. The rest were classified as interesting autopsy findings. This anatomy is in contrast to congenital fistulas which enter directly into cardiac chambers from normal coronary arteries; the latter have become a commonly recognized entity since the advent of coronary arteriography. At this writing, over 200 cases of congenital coronary artery fistulas arising from normal coronary arteries have been reported." Ninety per cent of these shunts enter the right heart, most frequently the right ventricle, followed by the right atrium and pulmonary artery. The remainder enter the left atrium or left ventricle. The significance of these fistulas varies tremendously. Some produce only a small shunt undetectable by routine cardiac catheterization, whereas From the Departments of Surgery, Medicine, and Radiology, Upstate Medical Center, Syracuse, N. Y. 13210. Received for publication Jan. 2, 1973.
others cause large shunts of hemodynamic importance.
Case report An asymptomatic 18-year-old black girl was discovered to have a heart murmur on routine physical examination. She was admitted to the Upstate Medical Center on July 4, 1972, for examination and diagnosis on the Cardiology Service. Physical examination revealed a systemic blood pressure of 98/58 mm. Hg, Her pulse was 60 beats per minute and regular. A continuous murmur was heard along the right sternal border in the third and fourth intercostal spaces. The pulmonic component of the second heart sound was accentuated with physiologic splitting of the second sound. An electrocardiogram was within normal limits. Cardiac roentgenograms revealed slight biventricular enlargement. Cardiac catheterization showed a left-to-right shunt of 2.8 L. per minute at the level of the right atrium and a normal pulmonary vascular resistance. A retrograde thoracic aortogram demonstrated an elongated fistulous tract with marked aneurysmal dilation which arose from an aortic sinus and extended in a circuitous manner into the right atrium. Both left and right coronary arteries and their branches appeared to be normal and intact. The size of the fistula and its potential complications prompted surgical intervention. At operation on Aug. 17, 1972, a soft, cystic, vascular lesion (2 em. in size) was found anterior to the root of the ascending aorta. The aorta was opened transversely, and two separate ostia were seen situated parallel to one another inside the
569
The Journal of
570
Parker et al.
Thoracic and Cardiovascular Surgery
Fig, 1. A, Arrow denotes the anomalous coronary artery fistula demonstrated by aortography. B, Aortography denotes the marked aneurysmal dilatation of the fistula (arrow). LA D, Left anterior descending coronary artery. LCA, Left circumflex artery. RCA, Right coronary artery. right coronary sinus, with the ostium on the right draining directly into the cystic lesion'; the ostium on the left supplied the normal right coronary artery. There was no dilation of the coronary sinus to suggest a sinus of Valsalva aneurysm. The fistulous tract coursed posterior to the right atrial appendage and followed the interatrial groove to empty into the right atrium. A right atriotomy revealed that the opening was just lateral to the interatrial septum in the pectinate musculature of the atrium. The accessory coronary ostium, the fistulous tract, and the opening in the atrium were all obliterated by multiple suture ligatures. Postoperatively, the patient has done extremely well, with obliteration of the murmur and no change in the electrocardiogram.
Discussion
Despite the fairly common occurrence of coronary arteriovenous communications, reports of a fistula from an accessory coronary artery are rare. Supernumerary coronary vessels in the right coronary sinus are common. Schlesinger," in a careful autopsy review of 651 hearts, found that 51 per cent had accessory arteries in the right coronary
sinus of Valsalva. Studies by Banchi,> Symmers," and Crainicianu> revealed the incidence of right accessory coronary arteries to vary from 33 to 65 per cent. In direct contrast, accessory left coronary arteries are quite rare. Schlesinger found only four in an autopsy series of 1,000 hearts. The right accessory coronary artery is usually referred to as the conus artery, as it supplies the outflow tract of the right ventricle. Normally its ostium is to the left of the ostium of the right coronary artery. In the case reported above, the accessory vessel began parallel to and to the right of the right coronary ostium, coursed posterior to the right atrial appendage, and followed the interatrial groove to enter the right atrium. We feel that the accessory coronary vessel in our case does not resemble the conus artery but is a separate variation of an accessory coronary artery. Only 6 other cases of fistula from an accessory coronary artery have been reported (Table I). Allenby- reported an
Volume 65
Supernumerary coronary artery-right atrial fistula
Number 4
571
April,1973
Fig. 2. Opening (arrow) of the anomalous right coronary artery fistula into the posterolateral wall of the right atrium.
Table I Author (year) Allenby et al. (1950) Scott (1948) Brown and Burnett (1942) Essenberg (1950) Gerisch et al. (1963 ) Currarino et al. (1959) Parker et al. (1972)
Location of supernumerary vessel
I
Structure involved
Diagnosis
Right coronary sinus Ventricular septal de- Main pulmonary arfect and pulmonary tery atresia Right coronary sinus None Main pulmonary artery Right ventricle Left coronary sinus None (infundibulum)
Autopsy
Other anomalies
Autopsy Autopsy
Multiple coronary artery anomalies Right coronary sinus None
Right atrium
Autopsy
Right atrium
Surgery
Noncoronary sinus
Right atrium
Autopsy
Right atrium
Surgery
Left coronary sinus
Esophageal atresia
Right coronary sinus None
autopsy finding of pulmonary atresia with an arteriovenous connection between an accessory conal artery and the pulmonary artery. It appeared that the vessel's function was to increase pulmonary blood flow by way of coronary collaterals into the atretic pulmonary artery. In 1948, Scott> recorded the autopsy findings in an 82-year-old man who had an accessory right coronary artery which dilated aneurysmally and emptied into the pulmonary artery. This also appeared to represent a conal branch. The only success-
ful surgical closure of an accessory coronary artery fistula was by Gerisch' in 1963, who transected a supernumerary right coronary artery which drained into the right atrium. The source of this vessel was never actually seen, but, by gross inspection, it appeared to rise from a coronary sinus, presumably the right. There have been 2 reported cases involving an accessory vessel from the left coronary sinus, both found at autopsy. Brown- reported on an accessory left coronary artery which drained into the infundib-
572
The Journal of
Parker et al.
Thorocic and Cardiovoscular Surgery
Fig. 3. Artist's sketch of the anomalous right coronary fistula (arrows) entering the right atrium. Ao, Aorta. IVC, Inferior vena cava. PA, Pulmonary artery. PV, Pulmonary veins. RCA, Right coronary artery. SVC, Superior vena cava.
ular region of the right ventricle. Essenberg," examining a fetal heart, found an aberrant vessel which arose from the left coronary sinus and drained into the right atrium. Currarino: reported the case of a patient with esophageal atresia who, at autopsy, had an accessory vessel from the noncoronary sinus which drained into the right atrium. Thus all reported supernumerary coronary fistulas have drained into the right heart, usually into the right atrium. In theory, fistulas between the coronary arteries and intracardiac chambers develop after arrested formation of the vascular network of the epicardium and the myocardium. Initially, in the embryo, there is a communication between the cardiac chambers and the intertrabecular spaces and sinusoids of the developing myocardium. These also enter into the newly formed epicardial vascular network. As the myocardium grows, it compresses these communicating spaces into capillary size or, in many instances, obliterates them. Failure to close these connections completely may result in an abnormal fistulous communication. The embryology of accessory coronary vessels is more conjectural. The main coronary ar-
teries develop by epithelial buds that sprout from the coronary sinuses and either extend out to form the coronary vessels or join with already formed coronary vessels. An extra coronary artery may represent an accessory coronary anlage in the coronary sinus which undergoes the same development as a normal coronary artery. The accessory coronary may develop a fistula in a manner similar to normal coronary arteries. The presenting symptom in the majority of patients who have a coronary artery fistula is a continuous heart murmur, generally confused with a patent ductus arteriosus. A significant clue is the location of the murmur. This is dependent on the route of the fistula but usually is loudest along the right sternal border. Electrocardiography is not diagnostic but may reveal either right or left ventricular enlargement. Chest roentgenography may portray changes consistent with a left-to-right shunt in fistulas to the right heart or a left-to-left shunt with fistulas to the left heart. The definitive diagnostic study is arteriography. A root injection in the ascending aorta will usually suffice, but selective coronary arteriography is even more precise. Most of these fistulas will be dilated in an aneurysmal fashion due to the high blood flow coursing through them, and it is usually possible to define the exact route of these shunts. As patients grow older, these fistulas not only eventuate in congestive heart failure secondary to left heart overload or pulmonary hypertension, but in rare cases they may rupture or become the site for subacute bacterial endocarditis. It has also been postulated that myocardial ischemia may occur in the region of this shunt. The coronary blood can be shunted away from the area that it should supply due to the low resistance in the intracardiac chamber. For these reasons, surgical interruption of these shunts is recommended. The first ligation of a coronary artery fistula was accomplished by Biorck and Crafoord- in 1947. They operated to ligate a patent ductus arteriosus only to find a coronary artery fistula. Since then many cases have been successfully diagnosed and
Volume 65 Number 4
Supernumerary coronary artery-right atrial fistula
573
April,1973
managed surgically. The important aspect of corrective surgery is not only to interrupt the shunt completely but to preserve the surrounding myocardial blood supply as well. If isolating and exposing the lesion is difficult, cardiopulmonary bypass should be utilized to dissect out the pathology completely and close the shunt. Summary A case involving the successful surgical closure of a supernumerary right coronary artery with a fistula to the right atrium is reported. Only 1 other similar surgical case has been reported, and there are only six other reports of coronary artery fistulas arising from accessory coronary arteries in the literature. A brief discussion of the embryology, pathophysiology, diagnosis, and treatment of these lesions is presented. We are indebted to Dr. Bernard Schneider for aid in interpreting the radiologic findings. REFERENCES 1 Allenby, D. K., Brinton, W. D., Campbell, M., and Gardner, F.: Pulmonary Atresia and the Collateral Circulation to the Lungs, Guys Hosp. Rep. 99:110, 1950. l a Banchi, A.: Morfologia delle arteriae coronariae cordis, Arch. hal. Anal. Embriol. 3: 87, 1904. 2 Biorck, G., and Crafoord, C.: Arteriovenous Aneurysm of the Pulmonary Artery Simulating Patent Ductus Botalli, Thorax 2: 65, 1947.
3 Brown, R. C., and Burnett, J. D.: Anomalous Channel Between Aorta and Right Ventricle, Pediatrics 3: 597, 1942. 3a Crainicianu, A.: Anatomische Studien tiber die Coronararterien und Experimentelle Unter suchungen tiber Ihre Durchlassigkeit, Virchows Arch. (Pathol. Anat.) 238: I, 1922. 4 Currarino, G., Silverman, F. N., and Landing, B. H.: Abnormal Congenital Fistulous Communications of the Coronary Arteries, Am. J. Roentgeno!. Radium Ther. Nucl. Med. 82: 392, 1959. 5 Edwards, J. E.: Personal communication, 1972. 6 Essenberg, J. M.: An Anomalous Left Coronary Artery in a Human Fetus, Anat. Rec. 108: 709, 1950. 7 Gerisch, R. A., Dodrill, F. D., Carrnoney, W. J., and Krabbenhoft, K. L.: Anomalous Coronary Artery Communicating With Right Atrium: Case Report, Harper Hosp. Bull. 21: 215, 1963. 8 Liotta, D., Hallman, G. J., Hall, R. 1., and Cooley, D. A.: Surgical Treatment of Congenital Coronary Artery Fistula, Surgery 70: 856, 1971. 9 Ogden, J. A.: Congenital Variations of the Coronary Arteries: A Clinicopathologic survey. Thesis, Yale University, 1968. 10 McNamara, J. J., and Gross, R. E.: Congenital Coronary Artery Fistula, Surgery 65: 59, 1969. 11 Schlesinger, M., Zoll, P., and Wessler, S.: The Conus Artery: A Third Coronary Artery, Am. Heart J. 38: 823, 1949. 12 Scott, D. H.: Aneurysm of the Coronary Arteries, Am. Heart J. 36: 403, 1948. 13 Syrnmers, W. S. C.: Note on Accessory Coronary Arteries, J. Anal. Physio!. 41: 14, 1907.
This report concerns a patient with a supernumerary coronary artery-right atrial fistula which has been surgically corrected. The vessel involved was an accessory right coronary artery that arose from the right coronary sinus of Valsalva. Six other cases of fistulas from supernumerary coronary arteries have been reported upon, but only 1 involved an operation. The rest were classified as interesting autopsy findings. This anatomy is in contrast to congenital fistulas which enter directly into cardiac chambers from normal coronary arteries; the latter have become a commonly recognized entity since the advent of coronary arteriography. At this writing, over 200 cases of congenital coronary artery fistulas arising from normal coronary arteries have been reported." Ninety per cent of these shunts enter the right heart, most frequently the right ventricle, followed by the right atrium and pulmonary artery. The remainder enter the left atrium or left ventricle. The significance of these fistulas varies tremendously. Some produce only a small shunt undetectable by routine cardiac catheterization, whereas From the Departments of Surgery, Medicine, and Radiology, Upstate Medical Center, Syracuse, N. Y. 13210. Received for publication Jan. 2, 1973.
others cause large shunts of hemodynamic importance.
Case report An asymptomatic 18-year-old black girl was discovered to have a heart murmur on routine physical examination. She was admitted to the Upstate Medical Center on July 4, 1972, for examination and diagnosis on the Cardiology Service. Physical examination revealed a systemic blood pressure of 98/58 mm. Hg, Her pulse was 60 beats per minute and regular. A continuous murmur was heard along the right sternal border in the third and fourth intercostal spaces. The pulmonic component of the second heart sound was accentuated with physiologic splitting of the second sound. An electrocardiogram was within normal limits. Cardiac roentgenograms revealed slight biventricular enlargement. Cardiac catheterization showed a left-to-right shunt of 2.8 L. per minute at the level of the right atrium and a normal pulmonary vascular resistance. A retrograde thoracic aortogram demonstrated an elongated fistulous tract with marked aneurysmal dilation which arose from an aortic sinus and extended in a circuitous manner into the right atrium. Both left and right coronary arteries and their branches appeared to be normal and intact. The size of the fistula and its potential complications prompted surgical intervention. At operation on Aug. 17, 1972, a soft, cystic, vascular lesion (2 em. in size) was found anterior to the root of the ascending aorta. The aorta was opened transversely, and two separate ostia were seen situated parallel to one another inside the
569
The Journal of
570
Parker et al.
Thoracic and Cardiovascular Surgery
Fig, 1. A, Arrow denotes the anomalous coronary artery fistula demonstrated by aortography. B, Aortography denotes the marked aneurysmal dilatation of the fistula (arrow). LA D, Left anterior descending coronary artery. LCA, Left circumflex artery. RCA, Right coronary artery. right coronary sinus, with the ostium on the right draining directly into the cystic lesion'; the ostium on the left supplied the normal right coronary artery. There was no dilation of the coronary sinus to suggest a sinus of Valsalva aneurysm. The fistulous tract coursed posterior to the right atrial appendage and followed the interatrial groove to empty into the right atrium. A right atriotomy revealed that the opening was just lateral to the interatrial septum in the pectinate musculature of the atrium. The accessory coronary ostium, the fistulous tract, and the opening in the atrium were all obliterated by multiple suture ligatures. Postoperatively, the patient has done extremely well, with obliteration of the murmur and no change in the electrocardiogram.
Discussion
Despite the fairly common occurrence of coronary arteriovenous communications, reports of a fistula from an accessory coronary artery are rare. Supernumerary coronary vessels in the right coronary sinus are common. Schlesinger," in a careful autopsy review of 651 hearts, found that 51 per cent had accessory arteries in the right coronary
sinus of Valsalva. Studies by Banchi,> Symmers," and Crainicianu> revealed the incidence of right accessory coronary arteries to vary from 33 to 65 per cent. In direct contrast, accessory left coronary arteries are quite rare. Schlesinger found only four in an autopsy series of 1,000 hearts. The right accessory coronary artery is usually referred to as the conus artery, as it supplies the outflow tract of the right ventricle. Normally its ostium is to the left of the ostium of the right coronary artery. In the case reported above, the accessory vessel began parallel to and to the right of the right coronary ostium, coursed posterior to the right atrial appendage, and followed the interatrial groove to enter the right atrium. We feel that the accessory coronary vessel in our case does not resemble the conus artery but is a separate variation of an accessory coronary artery. Only 6 other cases of fistula from an accessory coronary artery have been reported (Table I). Allenby- reported an
Volume 65
Supernumerary coronary artery-right atrial fistula
Number 4
571
April,1973
Fig. 2. Opening (arrow) of the anomalous right coronary artery fistula into the posterolateral wall of the right atrium.
Table I Author (year) Allenby et al. (1950) Scott (1948) Brown and Burnett (1942) Essenberg (1950) Gerisch et al. (1963 ) Currarino et al. (1959) Parker et al. (1972)
Location of supernumerary vessel
I
Structure involved
Diagnosis
Right coronary sinus Ventricular septal de- Main pulmonary arfect and pulmonary tery atresia Right coronary sinus None Main pulmonary artery Right ventricle Left coronary sinus None (infundibulum)
Autopsy
Other anomalies
Autopsy Autopsy
Multiple coronary artery anomalies Right coronary sinus None
Right atrium
Autopsy
Right atrium
Surgery
Noncoronary sinus
Right atrium
Autopsy
Right atrium
Surgery
Left coronary sinus
Esophageal atresia
Right coronary sinus None
autopsy finding of pulmonary atresia with an arteriovenous connection between an accessory conal artery and the pulmonary artery. It appeared that the vessel's function was to increase pulmonary blood flow by way of coronary collaterals into the atretic pulmonary artery. In 1948, Scott> recorded the autopsy findings in an 82-year-old man who had an accessory right coronary artery which dilated aneurysmally and emptied into the pulmonary artery. This also appeared to represent a conal branch. The only success-
ful surgical closure of an accessory coronary artery fistula was by Gerisch' in 1963, who transected a supernumerary right coronary artery which drained into the right atrium. The source of this vessel was never actually seen, but, by gross inspection, it appeared to rise from a coronary sinus, presumably the right. There have been 2 reported cases involving an accessory vessel from the left coronary sinus, both found at autopsy. Brown- reported on an accessory left coronary artery which drained into the infundib-
572
The Journal of
Parker et al.
Thorocic and Cardiovoscular Surgery
Fig. 3. Artist's sketch of the anomalous right coronary fistula (arrows) entering the right atrium. Ao, Aorta. IVC, Inferior vena cava. PA, Pulmonary artery. PV, Pulmonary veins. RCA, Right coronary artery. SVC, Superior vena cava.
ular region of the right ventricle. Essenberg," examining a fetal heart, found an aberrant vessel which arose from the left coronary sinus and drained into the right atrium. Currarino: reported the case of a patient with esophageal atresia who, at autopsy, had an accessory vessel from the noncoronary sinus which drained into the right atrium. Thus all reported supernumerary coronary fistulas have drained into the right heart, usually into the right atrium. In theory, fistulas between the coronary arteries and intracardiac chambers develop after arrested formation of the vascular network of the epicardium and the myocardium. Initially, in the embryo, there is a communication between the cardiac chambers and the intertrabecular spaces and sinusoids of the developing myocardium. These also enter into the newly formed epicardial vascular network. As the myocardium grows, it compresses these communicating spaces into capillary size or, in many instances, obliterates them. Failure to close these connections completely may result in an abnormal fistulous communication. The embryology of accessory coronary vessels is more conjectural. The main coronary ar-
teries develop by epithelial buds that sprout from the coronary sinuses and either extend out to form the coronary vessels or join with already formed coronary vessels. An extra coronary artery may represent an accessory coronary anlage in the coronary sinus which undergoes the same development as a normal coronary artery. The accessory coronary may develop a fistula in a manner similar to normal coronary arteries. The presenting symptom in the majority of patients who have a coronary artery fistula is a continuous heart murmur, generally confused with a patent ductus arteriosus. A significant clue is the location of the murmur. This is dependent on the route of the fistula but usually is loudest along the right sternal border. Electrocardiography is not diagnostic but may reveal either right or left ventricular enlargement. Chest roentgenography may portray changes consistent with a left-to-right shunt in fistulas to the right heart or a left-to-left shunt with fistulas to the left heart. The definitive diagnostic study is arteriography. A root injection in the ascending aorta will usually suffice, but selective coronary arteriography is even more precise. Most of these fistulas will be dilated in an aneurysmal fashion due to the high blood flow coursing through them, and it is usually possible to define the exact route of these shunts. As patients grow older, these fistulas not only eventuate in congestive heart failure secondary to left heart overload or pulmonary hypertension, but in rare cases they may rupture or become the site for subacute bacterial endocarditis. It has also been postulated that myocardial ischemia may occur in the region of this shunt. The coronary blood can be shunted away from the area that it should supply due to the low resistance in the intracardiac chamber. For these reasons, surgical interruption of these shunts is recommended. The first ligation of a coronary artery fistula was accomplished by Biorck and Crafoord- in 1947. They operated to ligate a patent ductus arteriosus only to find a coronary artery fistula. Since then many cases have been successfully diagnosed and
Volume 65 Number 4
Supernumerary coronary artery-right atrial fistula
573
April,1973
managed surgically. The important aspect of corrective surgery is not only to interrupt the shunt completely but to preserve the surrounding myocardial blood supply as well. If isolating and exposing the lesion is difficult, cardiopulmonary bypass should be utilized to dissect out the pathology completely and close the shunt. Summary A case involving the successful surgical closure of a supernumerary right coronary artery with a fistula to the right atrium is reported. Only 1 other similar surgical case has been reported, and there are only six other reports of coronary artery fistulas arising from accessory coronary arteries in the literature. A brief discussion of the embryology, pathophysiology, diagnosis, and treatment of these lesions is presented. We are indebted to Dr. Bernard Schneider for aid in interpreting the radiologic findings. REFERENCES 1 Allenby, D. K., Brinton, W. D., Campbell, M., and Gardner, F.: Pulmonary Atresia and the Collateral Circulation to the Lungs, Guys Hosp. Rep. 99:110, 1950. l a Banchi, A.: Morfologia delle arteriae coronariae cordis, Arch. hal. Anal. Embriol. 3: 87, 1904. 2 Biorck, G., and Crafoord, C.: Arteriovenous Aneurysm of the Pulmonary Artery Simulating Patent Ductus Botalli, Thorax 2: 65, 1947.
3 Brown, R. C., and Burnett, J. D.: Anomalous Channel Between Aorta and Right Ventricle, Pediatrics 3: 597, 1942. 3a Crainicianu, A.: Anatomische Studien tiber die Coronararterien und Experimentelle Unter suchungen tiber Ihre Durchlassigkeit, Virchows Arch. (Pathol. Anat.) 238: I, 1922. 4 Currarino, G., Silverman, F. N., and Landing, B. H.: Abnormal Congenital Fistulous Communications of the Coronary Arteries, Am. J. Roentgeno!. Radium Ther. Nucl. Med. 82: 392, 1959. 5 Edwards, J. E.: Personal communication, 1972. 6 Essenberg, J. M.: An Anomalous Left Coronary Artery in a Human Fetus, Anat. Rec. 108: 709, 1950. 7 Gerisch, R. A., Dodrill, F. D., Carrnoney, W. J., and Krabbenhoft, K. L.: Anomalous Coronary Artery Communicating With Right Atrium: Case Report, Harper Hosp. Bull. 21: 215, 1963. 8 Liotta, D., Hallman, G. J., Hall, R. 1., and Cooley, D. A.: Surgical Treatment of Congenital Coronary Artery Fistula, Surgery 70: 856, 1971. 9 Ogden, J. A.: Congenital Variations of the Coronary Arteries: A Clinicopathologic survey. Thesis, Yale University, 1968. 10 McNamara, J. J., and Gross, R. E.: Congenital Coronary Artery Fistula, Surgery 65: 59, 1969. 11 Schlesinger, M., Zoll, P., and Wessler, S.: The Conus Artery: A Third Coronary Artery, Am. Heart J. 38: 823, 1949. 12 Scott, D. H.: Aneurysm of the Coronary Arteries, Am. Heart J. 36: 403, 1948. 13 Syrnmers, W. S. C.: Note on Accessory Coronary Arteries, J. Anal. Physio!. 41: 14, 1907.