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Aorticoright atrial tunnel
Ann Thorac Surg 2000;69:635–7
with resolution of pulmonary AV malformations subsequent to a completion Fontan procedure which incorporated inferior vena cava and hepatic vein effluent into the pulmonary circulation.
Comment We add this report to the similar reports in patients with heterotaxy syndrome, as we are unaware of reported instances of pulmonary AV malformations and their subsequent resolution in patients undergoing staged Fontan reconstruction for malformations other than those associated with heterotaxy syndromes. This information may have significant implications with regard to the timing of staged reconstructive procedures culminating in a completed Fontan procedure, and with respect to those patients for whom superior cavopulmonary connection (without antegrade pulmonary blood flow) is viewed as long term palliation.
References 1. Srivastava D, Preminger T, Lock JE, et al. Hepatic venous blood and the development of pulmonary arteriovenous malformation in congenital heart disease. Circulation 1995;92: 1217–22. 2. Shah MJ, Rychik J, Fogel MA, Murphy JD, Jacobs ML. Pulmonary AV malformations after superior cavopulmonary connection: resolution after inclusion of hepatic veins in the pulmonary circulation. Ann Thorac Surg 1997;63:960–3. 3. Knight WB, Mee RBB. A cure for pulmonary arteriovenous fistulas? Ann Thorac Surg 1995;59:999 –1001. 4. Silverman A, Cooper MD, Moller JH, Good RA. Syndrome of cyanosis, digital clubbing, and hepatic disease in siblings. J Pediatr 1968;72:70– 80. 5. Krowka MJ, Cortese DA. Hepatopulmonary syndrome: an evolving perspective in the era of liver transplantation. Hepatology 1990;11:138 –142.
Aorticoright Atrial Tunnel Afksendiyos Kalangos, MD, PhD, Maurice Beghetti, MD, Dominique Vala, MD, Said Chraibi, MD, and Bernard Faidutti, MD Clinics for Cardiovascular Surgery and Pediatric Cardiology, University Cantonal Hospital of Geneva, Geneva, Switzerland
Two unusual cases of aorticoright atrial tunnel are described. Both patients were referred to our institution for evaluation of a continuous heart murmur best heard along the right upper sternal border. Ascending aortography showed the tunnel taking its origin from the aortic root and entering the right atrium through a tortuous link. Both patients underwent surgical closure. In addition, a review of similar cases in the literature is presented. (Ann Thorac Surg 2000;00:635–7) © 2000 by The Society of Thoracic Surgeons Accepted for publication July 1, 1999. Address reprint requests to Dr Kalangos, Clinic For Cardiovascular Surgery, University Cantonal Hospital of Geneva, 24, rue Micheli-duCrest, 1211 Geneva 14, Switzerland.
© 2000 by The Society of Thoracic Surgeons Published by Elsevier Science Inc
CASE REPORT KALANGOS ET AL AORTICORIGHT ATRIAL TUNNEL
635
T
he aorticoright atrial tunnel is a rare congenital anomaly first described by Otero Coto and colleagues in 1980 [1]. In this pathology, contrary to congenital coronary arteriovenous and coronary– cameral fistulas, the shunt is established through a vascular link arising from the aortic root and terminating in the right atrium (RA). We present two new patients with this unusual communication and review the five previously reported patients to better clarify its characteristics.
Case Reports Patient 1 An 18-year-old Ethiopian man was referred to our institution for evaluation of a continuous grade 4/6 heart murmur best heard along the right upper sternal border. A two-dimensional echocardiography identified a structure coursing from the left sinus of Valsalva toward the dilated RA. Aortography revealed moderate aortic insufficiency and the presence of a large tunnel originating from the left sinus of Valsalva, passing posterior to the aortic root, and terminating in the roof of the RA. QP/QS was estimated at 1.5/1. Coronary angiography revealed normal coronary arteries, although no sinus node artery could be identified. During operation, exploration of the aortic root revealed a tricuspid aortic valve with a moderately dilated aortic annulus and left sinus of Valsalva. The orifice of the tunnel, 7 mm in diameter, was located in the left sinus of Valsalva, 7 mm to the right of the left coronary ostium. An oblique right atriotomy revealed an orifice 5 mm in diameter in the superior aspect of the interatrial septum, 1.5 cm below the orifice of the superior vena cava (SVC). This orifice was obliterated by a 4-0 monofilament running suture. Thereafter, the tunnel was completely dissected up to its aortic origin and cut from the wall of the dilated sinus of Valsalva. The defect was then sutured with a 4-0 monofilament running suture.
Patient 2 An asymptomatic 7-year-old Moroccan boy was referred to our institution for evaluation of a heart murmur. Physical examination was normal except for a grade 2/6 continuous murmur best heard along the right upper sternal border. A two-dimensional echocardiogram revealed a large tunnel originating from the left sinus of Valsalva, then taking an abnormal course to end in the RA. Diagnosis was confirmed by aortography and coronary angiography. Injection of contrast material into the aortic root resulted in rapid filling of a large canal taking origin from the left sinus of Valsalva (Fig 1A). This canal passed posterior to the aortic root, terminating in the roof of the RA. Selective injection of contrast material into both coronary arteries appeared normal, although no sinus node artery could be identified. QP/QS was estimated at 1.3/1. During operation, exploration of the aortic root revealed a normal tricuspid aortic valve and a slightly dilated left sinus of Valsalva. From the wall of the left sinus of Valsalva, an orifice located 3 mm to the right of the left coronary artery ostium was identified. This orifice, 5 mm in diameter, was the origin of a tunnel running posterior to the aortic root along the roof of the left atrium before disappearing near the RA–SVC junc0003-4975/00/$20.00 PII S0003-4975(99)01346-6
636
CASE REPORT KALANGOS ET AL AORTICORIGHT ATRIAL TUNNEL
Ann Thorac Surg 2000;69:635–7
Fig 1. (A) Selective injection of contrast material into the aorta shows a large tortuous communication (white arrow) coursing rightward and posteriorly to connect with the right atrium. Note the aneurysmal dilatation of the tunnel (black arrows) before it enters the right atrium. (B) Intraoperative photograph showing the aorticoright atrial tunnel (white arrow) dissected along the left atrial roof. (AA ⫽ ascending aorta; VCS ⫽ cannulated superior vena cava.)
tion (Fig 1B). An oblique right atriotomy revealed a windsocklike orifice 3 mm in diameter, in the superior aspect of the interatrial septum, 1 cm below the orifice of the SVC. This orifice was then longitudinally incised, revealing an aneurysm of 2 to 2.5 cm in diameter located in the superior aspect of the interatrial septum, extending posteriorly toward the junction of the SVC with the right atrial roof. First, the aneurysmal formation was obliterated by sewing its anterior and posterior walls together with a 4-0 monofilament running suture. Thereafter, the tunnel was completely dissected from its origin at the left sinus of Valsalva to the RA–SVC junction and then ligated by a 5-0 monofilament at both ends.
Comment Literature concerning aorticoright atrial communications is limited to 7 patients, including both of ours. The communication between the aortic root and the RA arose from above the left sinus of Valsalva in 4 patients [2], from above the noncoronary sinus in 1 patient [1], and from inside the left sinus in both of our patients. The communication terminated in the lateral aspect of the RA in 1 patient, in the SVC near its junction with the RA in 2 patients, and in the superior aspect of the RA in 4 patients, including ours. We believe that the communication arising from a diverticulum of the left sinus of Valsalva, which also gave rise to the left main coronary artery in 2 patients and independently to the left circumflex and anterior descending arteries in 2 other patients [2], cannot be considered as a true connection of the left
coronary artery with the RA, therefore, contesting the use of the term coronary-cameral fistula. Moreover, the communication arose independently from the left coronary artery in the other 3 patients and the histologic examination in our patients demonstrated that its tissue is similar to the aortic one. The distribution of both coronary arteries appeared normal in all patients, although no sinus node artery could be identified in 4 patients on selective coronary angiograms, including our patients [1, 2]. No comment about its presence was made in the remaining 3 patients. This sinus node artery, originating in about 55% of hearts from the right coronary artery and in 45% of hearts from the left circumflex artery, in rare instances may take its origin directly from the aortic root [3]. Although no sinus node dysfunction was present in any patient, fistulous involvement of the sinus node artery arising from the left sinus of Valsalva or above was speculated on explaining the course of the tunnel in some patients [2]. The tunnel showed aneurysmal dilatation in 2 patients. In our second patient, the tunnel presented an aneurysmal dilatation in the superior aspect of the interatrial septum before entering the RA through a stenotic ostium of 3 mm in diameter. In the remaining patients, the tunnel was large and tortuous. The wall of the tunnel presented calcifications in 2 patients [1, 2]. In only 1 patient was there an associated cardiac lesion, which was that of an absent right SVC and the presence of a large left SVC [1]. All patients were essentially asymptomatic. A continuous murmur was best heard at the right upper sternal border in 6 patients including both of ours, and in the left
Ann Thorac Surg 2000;69:637–9
infraclavicular area in 1 patient [1, 2]. This murmur led cardiologists to further investigate and hence establish diagnosis in 6 patients, including ours [2]. In the other patient, the discovery of an anomalous mediastinal structure on chest roentgenogram forced surgeons to perform exploratory thoracotomy during which they noted the presence of a mediastinal mass with a continuous thrill requiring additional investigations [1]. Although the tunnel could be readily identified by two-dimensional echocardiography, retrograde aortography combined with selective coronary angiography was the most appropriate diagnostic tool [2]. Surgical treatment was performed in 6 patients, including ours [1, 2]. Although the need for operative closure in asymptomatic patients remains controversial, we recommend the closure of an aorticoright atrial tunnel, as we do for all congenital coronary fistulas. We stress the low rate of surgical complications in the asymptomatic patient. The risk of continued patency of the communication with age could become manifest by volume overload of both ventricles, bacterial endocarditis, aneurysm formation, or spontaneous rupture. Although coil embolization could be a possible alternative treatment for this condition, surgical treatment also has the benefit of reconstructing the dilated sinus of Valsalva or minimizing the likelihood of further sinus dilatation by ligating the tunnel close to the sinus. The aortic origin of the tunnel was confirmed by aortotomy and the atrial end by right atriotomy in 5 patients, including ours [2]. In 1 patient with aneurysmal dilatation of the tunnel, the aortic and right atrial orifices were confirmed and closed through aneurysmotomy [1]. In 2 patients, the tunnel was opened to the right of the aorta and the incision was then extended toward its aortic origin [2]. The orifice and tunnel were oversewn flush with the sinus, with care taken to protect the coronary ostium. In none of the patients was the dilated sinus of Valsalva obliterated to avoid compromising the coronary blood flow or distorting the adjacent aortic valve [1, 2]. However, the persistence of a dilated sinus of Valsalva constitutes concern and requires further follow-up to determine its evolution. According to the above described anatomic grounds of an aorticoright atrial tunnel, the differential diagnosis can only include the possibility of a ruptured aneurysm of the sinus of Valsalva or the right coronary artery [4], which can become more confounding if the tunnel presents aneurysmal dilatation and has its origin inside the sinus. In conclusion, the aorticoright atrial tunnel should be included in the clinical differential diagnosis of continuous murmurs usually best heard at the right upper sternal border. Ascending aortography combined with selective coronary angiography is the best diagnostic tool, showing the tunnel taking its origin from the aortic root and entering the RA through a tortuous link. Surgical closure should be recommended soon after diagnosis, as the continued patency of the tunnel may predispose patients to increasing risks and surgical mortality with age.
CASE REPORT KALIMI ET AL LEFT CORONARY OSTIA OCCLUSION
637
2. Rosenberg H, Williams WG, Trusler GA, et al. Congenital aortico-right atrial communication. The dilemma of differentiation from coronary-cameral fistula. J Thorac Cardiovasc Surg 1986;91:841–7. 3. James TN. The sinus node. Am J Cardiol 1977;40:965– 86. 4. Gray LA, McMartin DE. Surgical treatment of coronary artery aneurysm with rupture into the right atrium. J Thorac Cardiovasc Surg 1977;74:455– 60.
Occlusion of Left Coronary Artery Ostium by an Aortic Valve Cusp Robert Kalimi, MD, Robert S. Palazzo, MD, and L. Michael Graver, MD Division of Cardiothoracic Surgery, Department of Surgery, Long Island Jewish Medical Center, New Hyde Park, New York
Congenital anomalies of the aortic valve can be associated with other cardiac anomalies. In this report, we present a patient with an aortic valve anomaly associated with occlusion of left coronary ostia. In addition, we reviewed the literature and found 10 similar cases. Although compatible with life, this anomaly can lead to significant symptoms. Preoperative diagnosis as well as proper therapeutic planning should be tailored to correct valvular competence and restore coronary blood flow. (Ann Thorac Surg 2000;69:637–9) © 2000 by The Society of Thoracic Surgeons
T
here is evidence to suggest that occlusion of left coronary ostia by an aortic valve cusp may be congenital in nature and may be associated with other malformations. Review of ten similar cases from the literature suggests that this condition may be related to an anomaly in the development of the aortic valve. A 42-year-old woman with no significant past history presented to our institution with chest pain, dyspnea on exertion, and palpitations. On physical exam a IV/VI diastolic murmur was present. An echocardiogram disclosed severe aortic insufficiency and a dilated hypokinetic left ventricle. During cardiac catheterization, the left coronary (LC) ostium could not be cannulated. The right coronary (RC) artery was of normal caliber with a large conal branch to the left anterior descending artery. Retrograde blood flow was present through the left main artery to a pouch in the area of the left coronary ostium (Fig 1). The operative approach was via a median sternotomy. Cardiopulmonary bypass (CPB) was established using a bicaval and ascending aortic cannulation. Retrograde cardioplegia was administered via the coronary sinus. The left ventricle was vented via the right superior
References
Accepted for publication July 3, 1999.
1. Otero Coto E, Caffarena JM, Such M, Marques JL. Aorta-right atrial communication. Report of an unusual case. J Thorac Cardiovasc Surg 1980;80:941– 4.
Address reprint requests to Dr Kalimi, Department of Surgery, Long Island Jewish Medical Center, 270-05 76th Ave, New Hyde Park, NY 11040.
© 2000 by The Society of Thoracic Surgeons Published by Elsevier Science Inc
0003-4975/00/$20.00 PII S0003-4975(99)01392-2
with resolution of pulmonary AV malformations subsequent to a completion Fontan procedure which incorporated inferior vena cava and hepatic vein effluent into the pulmonary circulation.
Comment We add this report to the similar reports in patients with heterotaxy syndrome, as we are unaware of reported instances of pulmonary AV malformations and their subsequent resolution in patients undergoing staged Fontan reconstruction for malformations other than those associated with heterotaxy syndromes. This information may have significant implications with regard to the timing of staged reconstructive procedures culminating in a completed Fontan procedure, and with respect to those patients for whom superior cavopulmonary connection (without antegrade pulmonary blood flow) is viewed as long term palliation.
References 1. Srivastava D, Preminger T, Lock JE, et al. Hepatic venous blood and the development of pulmonary arteriovenous malformation in congenital heart disease. Circulation 1995;92: 1217–22. 2. Shah MJ, Rychik J, Fogel MA, Murphy JD, Jacobs ML. Pulmonary AV malformations after superior cavopulmonary connection: resolution after inclusion of hepatic veins in the pulmonary circulation. Ann Thorac Surg 1997;63:960–3. 3. Knight WB, Mee RBB. A cure for pulmonary arteriovenous fistulas? Ann Thorac Surg 1995;59:999 –1001. 4. Silverman A, Cooper MD, Moller JH, Good RA. Syndrome of cyanosis, digital clubbing, and hepatic disease in siblings. J Pediatr 1968;72:70– 80. 5. Krowka MJ, Cortese DA. Hepatopulmonary syndrome: an evolving perspective in the era of liver transplantation. Hepatology 1990;11:138 –142.
Aorticoright Atrial Tunnel Afksendiyos Kalangos, MD, PhD, Maurice Beghetti, MD, Dominique Vala, MD, Said Chraibi, MD, and Bernard Faidutti, MD Clinics for Cardiovascular Surgery and Pediatric Cardiology, University Cantonal Hospital of Geneva, Geneva, Switzerland
Two unusual cases of aorticoright atrial tunnel are described. Both patients were referred to our institution for evaluation of a continuous heart murmur best heard along the right upper sternal border. Ascending aortography showed the tunnel taking its origin from the aortic root and entering the right atrium through a tortuous link. Both patients underwent surgical closure. In addition, a review of similar cases in the literature is presented. (Ann Thorac Surg 2000;00:635–7) © 2000 by The Society of Thoracic Surgeons Accepted for publication July 1, 1999. Address reprint requests to Dr Kalangos, Clinic For Cardiovascular Surgery, University Cantonal Hospital of Geneva, 24, rue Micheli-duCrest, 1211 Geneva 14, Switzerland.
© 2000 by The Society of Thoracic Surgeons Published by Elsevier Science Inc
CASE REPORT KALANGOS ET AL AORTICORIGHT ATRIAL TUNNEL
635
T
he aorticoright atrial tunnel is a rare congenital anomaly first described by Otero Coto and colleagues in 1980 [1]. In this pathology, contrary to congenital coronary arteriovenous and coronary– cameral fistulas, the shunt is established through a vascular link arising from the aortic root and terminating in the right atrium (RA). We present two new patients with this unusual communication and review the five previously reported patients to better clarify its characteristics.
Case Reports Patient 1 An 18-year-old Ethiopian man was referred to our institution for evaluation of a continuous grade 4/6 heart murmur best heard along the right upper sternal border. A two-dimensional echocardiography identified a structure coursing from the left sinus of Valsalva toward the dilated RA. Aortography revealed moderate aortic insufficiency and the presence of a large tunnel originating from the left sinus of Valsalva, passing posterior to the aortic root, and terminating in the roof of the RA. QP/QS was estimated at 1.5/1. Coronary angiography revealed normal coronary arteries, although no sinus node artery could be identified. During operation, exploration of the aortic root revealed a tricuspid aortic valve with a moderately dilated aortic annulus and left sinus of Valsalva. The orifice of the tunnel, 7 mm in diameter, was located in the left sinus of Valsalva, 7 mm to the right of the left coronary ostium. An oblique right atriotomy revealed an orifice 5 mm in diameter in the superior aspect of the interatrial septum, 1.5 cm below the orifice of the superior vena cava (SVC). This orifice was obliterated by a 4-0 monofilament running suture. Thereafter, the tunnel was completely dissected up to its aortic origin and cut from the wall of the dilated sinus of Valsalva. The defect was then sutured with a 4-0 monofilament running suture.
Patient 2 An asymptomatic 7-year-old Moroccan boy was referred to our institution for evaluation of a heart murmur. Physical examination was normal except for a grade 2/6 continuous murmur best heard along the right upper sternal border. A two-dimensional echocardiogram revealed a large tunnel originating from the left sinus of Valsalva, then taking an abnormal course to end in the RA. Diagnosis was confirmed by aortography and coronary angiography. Injection of contrast material into the aortic root resulted in rapid filling of a large canal taking origin from the left sinus of Valsalva (Fig 1A). This canal passed posterior to the aortic root, terminating in the roof of the RA. Selective injection of contrast material into both coronary arteries appeared normal, although no sinus node artery could be identified. QP/QS was estimated at 1.3/1. During operation, exploration of the aortic root revealed a normal tricuspid aortic valve and a slightly dilated left sinus of Valsalva. From the wall of the left sinus of Valsalva, an orifice located 3 mm to the right of the left coronary artery ostium was identified. This orifice, 5 mm in diameter, was the origin of a tunnel running posterior to the aortic root along the roof of the left atrium before disappearing near the RA–SVC junc0003-4975/00/$20.00 PII S0003-4975(99)01346-6
636
CASE REPORT KALANGOS ET AL AORTICORIGHT ATRIAL TUNNEL
Ann Thorac Surg 2000;69:635–7
Fig 1. (A) Selective injection of contrast material into the aorta shows a large tortuous communication (white arrow) coursing rightward and posteriorly to connect with the right atrium. Note the aneurysmal dilatation of the tunnel (black arrows) before it enters the right atrium. (B) Intraoperative photograph showing the aorticoright atrial tunnel (white arrow) dissected along the left atrial roof. (AA ⫽ ascending aorta; VCS ⫽ cannulated superior vena cava.)
tion (Fig 1B). An oblique right atriotomy revealed a windsocklike orifice 3 mm in diameter, in the superior aspect of the interatrial septum, 1 cm below the orifice of the SVC. This orifice was then longitudinally incised, revealing an aneurysm of 2 to 2.5 cm in diameter located in the superior aspect of the interatrial septum, extending posteriorly toward the junction of the SVC with the right atrial roof. First, the aneurysmal formation was obliterated by sewing its anterior and posterior walls together with a 4-0 monofilament running suture. Thereafter, the tunnel was completely dissected from its origin at the left sinus of Valsalva to the RA–SVC junction and then ligated by a 5-0 monofilament at both ends.
Comment Literature concerning aorticoright atrial communications is limited to 7 patients, including both of ours. The communication between the aortic root and the RA arose from above the left sinus of Valsalva in 4 patients [2], from above the noncoronary sinus in 1 patient [1], and from inside the left sinus in both of our patients. The communication terminated in the lateral aspect of the RA in 1 patient, in the SVC near its junction with the RA in 2 patients, and in the superior aspect of the RA in 4 patients, including ours. We believe that the communication arising from a diverticulum of the left sinus of Valsalva, which also gave rise to the left main coronary artery in 2 patients and independently to the left circumflex and anterior descending arteries in 2 other patients [2], cannot be considered as a true connection of the left
coronary artery with the RA, therefore, contesting the use of the term coronary-cameral fistula. Moreover, the communication arose independently from the left coronary artery in the other 3 patients and the histologic examination in our patients demonstrated that its tissue is similar to the aortic one. The distribution of both coronary arteries appeared normal in all patients, although no sinus node artery could be identified in 4 patients on selective coronary angiograms, including our patients [1, 2]. No comment about its presence was made in the remaining 3 patients. This sinus node artery, originating in about 55% of hearts from the right coronary artery and in 45% of hearts from the left circumflex artery, in rare instances may take its origin directly from the aortic root [3]. Although no sinus node dysfunction was present in any patient, fistulous involvement of the sinus node artery arising from the left sinus of Valsalva or above was speculated on explaining the course of the tunnel in some patients [2]. The tunnel showed aneurysmal dilatation in 2 patients. In our second patient, the tunnel presented an aneurysmal dilatation in the superior aspect of the interatrial septum before entering the RA through a stenotic ostium of 3 mm in diameter. In the remaining patients, the tunnel was large and tortuous. The wall of the tunnel presented calcifications in 2 patients [1, 2]. In only 1 patient was there an associated cardiac lesion, which was that of an absent right SVC and the presence of a large left SVC [1]. All patients were essentially asymptomatic. A continuous murmur was best heard at the right upper sternal border in 6 patients including both of ours, and in the left
Ann Thorac Surg 2000;69:637–9
infraclavicular area in 1 patient [1, 2]. This murmur led cardiologists to further investigate and hence establish diagnosis in 6 patients, including ours [2]. In the other patient, the discovery of an anomalous mediastinal structure on chest roentgenogram forced surgeons to perform exploratory thoracotomy during which they noted the presence of a mediastinal mass with a continuous thrill requiring additional investigations [1]. Although the tunnel could be readily identified by two-dimensional echocardiography, retrograde aortography combined with selective coronary angiography was the most appropriate diagnostic tool [2]. Surgical treatment was performed in 6 patients, including ours [1, 2]. Although the need for operative closure in asymptomatic patients remains controversial, we recommend the closure of an aorticoright atrial tunnel, as we do for all congenital coronary fistulas. We stress the low rate of surgical complications in the asymptomatic patient. The risk of continued patency of the communication with age could become manifest by volume overload of both ventricles, bacterial endocarditis, aneurysm formation, or spontaneous rupture. Although coil embolization could be a possible alternative treatment for this condition, surgical treatment also has the benefit of reconstructing the dilated sinus of Valsalva or minimizing the likelihood of further sinus dilatation by ligating the tunnel close to the sinus. The aortic origin of the tunnel was confirmed by aortotomy and the atrial end by right atriotomy in 5 patients, including ours [2]. In 1 patient with aneurysmal dilatation of the tunnel, the aortic and right atrial orifices were confirmed and closed through aneurysmotomy [1]. In 2 patients, the tunnel was opened to the right of the aorta and the incision was then extended toward its aortic origin [2]. The orifice and tunnel were oversewn flush with the sinus, with care taken to protect the coronary ostium. In none of the patients was the dilated sinus of Valsalva obliterated to avoid compromising the coronary blood flow or distorting the adjacent aortic valve [1, 2]. However, the persistence of a dilated sinus of Valsalva constitutes concern and requires further follow-up to determine its evolution. According to the above described anatomic grounds of an aorticoright atrial tunnel, the differential diagnosis can only include the possibility of a ruptured aneurysm of the sinus of Valsalva or the right coronary artery [4], which can become more confounding if the tunnel presents aneurysmal dilatation and has its origin inside the sinus. In conclusion, the aorticoright atrial tunnel should be included in the clinical differential diagnosis of continuous murmurs usually best heard at the right upper sternal border. Ascending aortography combined with selective coronary angiography is the best diagnostic tool, showing the tunnel taking its origin from the aortic root and entering the RA through a tortuous link. Surgical closure should be recommended soon after diagnosis, as the continued patency of the tunnel may predispose patients to increasing risks and surgical mortality with age.
CASE REPORT KALIMI ET AL LEFT CORONARY OSTIA OCCLUSION
637
2. Rosenberg H, Williams WG, Trusler GA, et al. Congenital aortico-right atrial communication. The dilemma of differentiation from coronary-cameral fistula. J Thorac Cardiovasc Surg 1986;91:841–7. 3. James TN. The sinus node. Am J Cardiol 1977;40:965– 86. 4. Gray LA, McMartin DE. Surgical treatment of coronary artery aneurysm with rupture into the right atrium. J Thorac Cardiovasc Surg 1977;74:455– 60.
Occlusion of Left Coronary Artery Ostium by an Aortic Valve Cusp Robert Kalimi, MD, Robert S. Palazzo, MD, and L. Michael Graver, MD Division of Cardiothoracic Surgery, Department of Surgery, Long Island Jewish Medical Center, New Hyde Park, New York
Congenital anomalies of the aortic valve can be associated with other cardiac anomalies. In this report, we present a patient with an aortic valve anomaly associated with occlusion of left coronary ostia. In addition, we reviewed the literature and found 10 similar cases. Although compatible with life, this anomaly can lead to significant symptoms. Preoperative diagnosis as well as proper therapeutic planning should be tailored to correct valvular competence and restore coronary blood flow. (Ann Thorac Surg 2000;69:637–9) © 2000 by The Society of Thoracic Surgeons
T
here is evidence to suggest that occlusion of left coronary ostia by an aortic valve cusp may be congenital in nature and may be associated with other malformations. Review of ten similar cases from the literature suggests that this condition may be related to an anomaly in the development of the aortic valve. A 42-year-old woman with no significant past history presented to our institution with chest pain, dyspnea on exertion, and palpitations. On physical exam a IV/VI diastolic murmur was present. An echocardiogram disclosed severe aortic insufficiency and a dilated hypokinetic left ventricle. During cardiac catheterization, the left coronary (LC) ostium could not be cannulated. The right coronary (RC) artery was of normal caliber with a large conal branch to the left anterior descending artery. Retrograde blood flow was present through the left main artery to a pouch in the area of the left coronary ostium (Fig 1). The operative approach was via a median sternotomy. Cardiopulmonary bypass (CPB) was established using a bicaval and ascending aortic cannulation. Retrograde cardioplegia was administered via the coronary sinus. The left ventricle was vented via the right superior
References
Accepted for publication July 3, 1999.
1. Otero Coto E, Caffarena JM, Such M, Marques JL. Aorta-right atrial communication. Report of an unusual case. J Thorac Cardiovasc Surg 1980;80:941– 4.
Address reprint requests to Dr Kalimi, Department of Surgery, Long Island Jewish Medical Center, 270-05 76th Ave, New Hyde Park, NY 11040.
© 2000 by The Society of Thoracic Surgeons Published by Elsevier Science Inc
0003-4975/00/$20.00 PII S0003-4975(99)01392-2