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Role of transesophageal echocardiography in the diagnosis of ruptured aneurysm of sinus of Valsalva
m
,II~ilINO&
ULTI~SOUND ELSEVIER
European Journal of Ultrasound 4 (1996) 129-133
Clinical report
Role of transesophageal echocardiography in the diagnosis of ruptured aneurysm of sinus of Valsalva Christophe Tron*, Genevi6ve Derumeaux, Alain Cribier, Brice Letac Service de Cardiologie, VACOMED Research Group, Centre Hospitalo-Universitaire, H6pital Charles-Nicolle, 1 rue de Gerrnont 76000 Rouen France
Received 3 October 1995; revised 17 May 1996; accepted 17 May 1996
Abstract
Diagnosis of aorto to right cameral fistula resulting from rupture of an aneurysm of sinus of Valsalva may be difficult by transthoracic echocardiography. The authors report two cases of ruptured aneurysms of sinus of Valsalva, one in the right atrium and one in the right ventricle. In both cases, transesophageal echocardiography provided a precise definition of the anatomic abnormalities which might allow to proceed to surgery without cardiac catheterization. Keywords: Valsalva; Aneurysm; Transesophageal echocardiography
1. I n t r o d u c t i o n
Congenital aneurysms of sinus of Valsalva are rare and usually involve the right coronary sinus or the noncoronary sinus. They may rupture into the right heart chambers resulting in an aortocameral fistula. The differential diagnosis of these ruptured sinus of Valsalva aneurysms may be difficult and, until recently, required car* Corresponding author.
diac catheterization. Transthoracic bidimensional echocardiography and color flow Doppler have proved to be useful in this indication, but it may be at times difficult to fully define the anatomic abnormalities (Hoadley, 1987; Nishimura et al., 1976; Terdjman et al., 1984). We report two patients with aorto-right cameral fistula in which the use of transesophageal echocardiography allowed a precise diagnosis which would have been missed by transthoracic echocardiography or by aortography.
0929-8266/96/$15.00 © 1996 Elsevier Science Ireland Ltd. All rights reserved PII S0929-8266(95)001 84-X
130
C. Tron et al./ European Journal of Ultrasound 4 (1996) 129-133
2. Case reports
Two patients were seen at our institution with suspected aorto-right cameral fistula. Both patients underwent two-dimensional transthoracic echocardiography, transesophageal echocardiography and cardiac catheterization including an aortogram. All patients were subsequently operated on and lesions were confirmed at surgery. Two-dimensional echocardiography was performed with an Acuson 128 XP 10 equipped with a 2.5-MHz transducer. Transesophageal echocardiography was performed with the same equipment using a high frequency biplane transesophageal transducer. 2.1. Case 1
A 46-year-old man was referred to our hospital for investigation of a dyspnea. His past medical history was remarkable for a bilateral heart failure 1 year before. Other antecedents were chronic alcoolism and smoking. Despite a treatment with diuretics and ACE inhibitors, exertional dyspnea persisted. On examination, a grade 4/6 pansystolic murmur was heard over the entire precordium. No diastolic murmur was heard. There were no signs of congestive heart failure. The electrocardiogram was normal. Two-dimensional transthoracic echocardiography showed normal left ventricular function with no evidence of valve disease. In the parasternal short-axis view, a continuous flow with multiple aliasing was detected by color flow Doppler between the aorta and the right atrium. Transesophageal echocardiography clearly showed a tube-like structure originating from the right coronary cusp and directing toward the right atrium demonstrating the presence of a fistula between the aorta and the right atrium (Fig. 1). Addition of color flow Doppler revealed continuous turbulent flow passing between the aorta and the right atrium. Right and left cardiac catheterization was subsequently performed and the diagnosis was a fistula between the aorta and the right ventricle with an output of 3 1 per minute, disagreeing with transesophageal echocardiography constatations. Coronary angiography was normal. The patient
underwent surgery at which time the transesophageal echocardiographic findings of a fistula between the aorta and the right atrium were confirmed. The ruptured aneurysm was excised and the fistula was closed with a pericardial patch reinforced with Teflon. The post operative course was uneventful. 2.2. Case 2
The second patient was a 29-year-old man, known to be having a systolic murmur since the age of 7. This murmur had been presumed to be functional and no further explorations had been done previously. Clinical examination revealed a grade 4/6 continuous murmur heard best at the fourth left intercostal space. The electrocardiogram was normal. The transthoracic echocardiography showed normal chamber sizes. Continuous Doppler showed a continuous flow near the aortic root but precise location of this flow was not possible. Transesophageal echocardiography with color Doppler allowed the identification of a shunt between the right sinus of Valsalva and the right ventricle without other abnormalities. Subsequent aortography in the anterior oblique view confirmed the aorta to right ventricle fistula. Right chambers pressures were normal. Coronary arteries were found to be normal. Patient underwent surgery. An aneurysm of the right coronary sinus of Valsalva was found with rupture in the pulmonary infundibulum. There was a small (5 mm diameter) infundibulum ventricular septal defect which was closed with a Dacron patch. This associated ventricular septal defect have not been diagnosed neither by transesophageal echocardiography nor cardiac catheterization. The aneurysm was excised and the fistula closed with a Dacron patch. The postoperative course was uneventful.
3. Discussion
Aorto to right cameral fistula due to rupture of sinus of Valsalva are rare but their diagnosis is important since long term survival after surgical treatment is excellent (Matti!a et al., 1987; Van Son et al., 1994). Although the sinus aneurysms
C. Tron et al. / European Journal o f Ultrasound 4 (1996) 129-133
131
~is Fistula
N
1
Right atrium Right atrium Fig. 1. Transesophageal echocardiographyshowing a tube-like structure originating from the right coronary cusp and directing toward the right atrium (left). On the right, demonstration of the fistula between the aorta and the right atrium with addition of color flow Doppler.
are mostly congenital, rupture rarely occurs in infancy, and the majority of patients are operated on between 20 and 40 years of age, as our patients. In this report, we stress the value of transesophageal echocardiography for diagnosis of the rupture of a sinus of Valsalva aneurysm and precise localization of the fistulous communications between the aorta and the right heart chambers. Diagnosis of aortic to right cameral fistula by transesophageal echocardiography have been rarely reported in the litterature (Alam, 1993; Thomas et al., 1993). Indeed, several reports have documented the usefulness of transthoracic echocardiography for the diagnosis of sinus of Valsalva aneurysms and rupture (Nishimura et
al., 1976; Terdjman et al., 1984; Weyman et al., 1975). Injection of contrast in the aortic root during cardiac catheterization has been suggested to determine the location of the shunt with twodimensional echocardiography (Vered et al., 1985). However, good quality images may be difficult to obtain with transthoracic echocardiography leaving uncertainty in the diagnosis. In our second case, although continuous Doppler revealed a continuous flow near the aortic root, precise location of this flow was impossible, while transesophageal echocardiography easily and clearly showed the right coronary sinus aneurysm and its fistulous connection with the right atrium. Our study was performed using a biplane trans-
132
C. Tron et al. / European Journal o f Ultrasound 4 (1996) 129-133
esophageal transducer and it is likely that use of multiplane transducers would have facilitated the diagnosis. As in a previous report (Thomas et al., 1993), cardiac catheterization provided no additive information, except for the coronary anatomy that was normal. Given that patients with ruptured aneurysms of sinus of Valsalva are often young, complete diagnosis by transesophageal echocardiography should allow to proceed to surgery without cardiac catheterization. In contrary, in patients older than 40 years or with risk factors for coronary artery disease, a coronary arteriography should be performed. Moreover, in our first case, angiography mistakenly suggested a fistula between the aorta and the right ventricle. The superiority of the transesophageal echocardiography over aortic angiography in this case is not really surprising since the transesophageal approach, with its enhanced quality of imaging, provides a much better delineation of the aortic root than angiography. Important observations given by transesophageal echocardiography include (1) the location of the sinus of Valsalva involved; (2) which cardiac chamber the fistula enters; and also (3) the presence and the importance of a possible associated aortic regurgitation; (4) potential associated anomalies including presence and type of ventricular septal defect, right ventricular outflow tract obstruction and bicuspid aortic valve; and (5) potential associated complications such as endocarditis vegetations. Furthermore, transesophageal echocardiography may be useful for the follow up of patients after surgery. The association of a subarterial ventricular septal defect is not rare in patients with ruptured sinus of Valsalva aneurysm (Van Son et al., 1994). It may be useful to perform transesophageal echocardiography during surgery as a small ventricular septal defect can be missed by preoperative transesophageal echocardiography as in our second case. It is likely that in our second patient the systolic murmur known since he was 7 years old was due to the presence of the ventricular septal defect and that the apparition of a diastolic murmur corresponded to the rupture of the sinus of Valsalva aneurysm in the right ventricle. Thus, the occurrence of a diastolic murmur in a patient with known ventricular septal defect might be
considered as an indication to perform a transesophageal echocardiography.
4. Conclusion This report illustrates the potential for transesophageal echocardiography with color flow Doppler to diagnose and precisely define aortic to right cameral fistula secondary to rupture of sinus of Valsalva aneurysms. This technique should, therefore, become the investigation of choice in case of suspicion of ruptured sinus of Valsalva aneurysm and be considered early when transthoracic echocardiography results are questionable. Furthermore, transesophageal echocardiography might allow to operate on the patients without pre-operative cardiac catheterization.
Acknowledgements The authors wish to thank Franqois Bouchart M.D. and Nadine David M.D. for helpful discussion.
References Alam M. Transesophageal color flow Doppler features of aorta to right ventriclefistula. Chest 1993; 103: 1907-1908. Hoadley SD. Aortic pseudoaneurysmwith right atrial communication shown by Doppler color flow imaging. Am Heart J 1987; 114: 1535-1536. Mattila SP, Kupari M, Harjula ALJ, Ventila M, Meurala H, Maamies TJ, Aho P. Ruptured aneurysm of sinus of Valsalva: Long-term postoperative follow-up. Scand J Thorac Cardiovasc Surg 1987; 2l: 233-238. Nishimura K, Hibi N, Kato T, et al. Real-time observation of ruptured right sinus of Valsalva aneurysm by high speed ultrason-cardiotomography. Report of a case. Circulation 1976; 53: 732-735. Terdjman M, Bourdarias JP, Farcot JP et al. Aneurysms of sinus of Valsalva: two-dimensional echocardiographicdiagnosis and recognition of rupture into the right heart cavities. J Am Coil Cardiol 1984; 3: 1227-1235. Thomas MR, Monaghan MJ, Michalis LK, Jewitt DE. Aortoatrial fistulae diagnosed by transthoracic and transesophageal echocardiography: advantages of the transesophageal approach. J Am Soc Echocardiogr 1993; 6: 21-29.
C. Tron et al. / European Journal of Ultrasound 4 (1996) 129-133 Van Son JAM, Danielson GK, Schaff HV, Orszulak TA, Edwards WD, Seward JB. Long-term outcome of surgical repair of ruptured sinus of Valsalva aneurysm. Circulation 1994; 90 [part 2]: II-20-I1-29). Vered Z, Rath S, Benjamin P, Motro M, Neufeld HN. Ruptured sinus of Valsalva: Demonstration by contrast
133
echocardiography during cardiac catheterization. Am Heart J 1985; 109: 365-366. Weyman AE, Dillon JC, Feigenbaum H, Chang S. Premature pulmonic valve opening following sinus of Valsalva aneurysm rupture into the right atrium. Circulation 1975; 51: 556-560.
,II~ilINO&
ULTI~SOUND ELSEVIER
European Journal of Ultrasound 4 (1996) 129-133
Clinical report
Role of transesophageal echocardiography in the diagnosis of ruptured aneurysm of sinus of Valsalva Christophe Tron*, Genevi6ve Derumeaux, Alain Cribier, Brice Letac Service de Cardiologie, VACOMED Research Group, Centre Hospitalo-Universitaire, H6pital Charles-Nicolle, 1 rue de Gerrnont 76000 Rouen France
Received 3 October 1995; revised 17 May 1996; accepted 17 May 1996
Abstract
Diagnosis of aorto to right cameral fistula resulting from rupture of an aneurysm of sinus of Valsalva may be difficult by transthoracic echocardiography. The authors report two cases of ruptured aneurysms of sinus of Valsalva, one in the right atrium and one in the right ventricle. In both cases, transesophageal echocardiography provided a precise definition of the anatomic abnormalities which might allow to proceed to surgery without cardiac catheterization. Keywords: Valsalva; Aneurysm; Transesophageal echocardiography
1. I n t r o d u c t i o n
Congenital aneurysms of sinus of Valsalva are rare and usually involve the right coronary sinus or the noncoronary sinus. They may rupture into the right heart chambers resulting in an aortocameral fistula. The differential diagnosis of these ruptured sinus of Valsalva aneurysms may be difficult and, until recently, required car* Corresponding author.
diac catheterization. Transthoracic bidimensional echocardiography and color flow Doppler have proved to be useful in this indication, but it may be at times difficult to fully define the anatomic abnormalities (Hoadley, 1987; Nishimura et al., 1976; Terdjman et al., 1984). We report two patients with aorto-right cameral fistula in which the use of transesophageal echocardiography allowed a precise diagnosis which would have been missed by transthoracic echocardiography or by aortography.
0929-8266/96/$15.00 © 1996 Elsevier Science Ireland Ltd. All rights reserved PII S0929-8266(95)001 84-X
130
C. Tron et al./ European Journal of Ultrasound 4 (1996) 129-133
2. Case reports
Two patients were seen at our institution with suspected aorto-right cameral fistula. Both patients underwent two-dimensional transthoracic echocardiography, transesophageal echocardiography and cardiac catheterization including an aortogram. All patients were subsequently operated on and lesions were confirmed at surgery. Two-dimensional echocardiography was performed with an Acuson 128 XP 10 equipped with a 2.5-MHz transducer. Transesophageal echocardiography was performed with the same equipment using a high frequency biplane transesophageal transducer. 2.1. Case 1
A 46-year-old man was referred to our hospital for investigation of a dyspnea. His past medical history was remarkable for a bilateral heart failure 1 year before. Other antecedents were chronic alcoolism and smoking. Despite a treatment with diuretics and ACE inhibitors, exertional dyspnea persisted. On examination, a grade 4/6 pansystolic murmur was heard over the entire precordium. No diastolic murmur was heard. There were no signs of congestive heart failure. The electrocardiogram was normal. Two-dimensional transthoracic echocardiography showed normal left ventricular function with no evidence of valve disease. In the parasternal short-axis view, a continuous flow with multiple aliasing was detected by color flow Doppler between the aorta and the right atrium. Transesophageal echocardiography clearly showed a tube-like structure originating from the right coronary cusp and directing toward the right atrium demonstrating the presence of a fistula between the aorta and the right atrium (Fig. 1). Addition of color flow Doppler revealed continuous turbulent flow passing between the aorta and the right atrium. Right and left cardiac catheterization was subsequently performed and the diagnosis was a fistula between the aorta and the right ventricle with an output of 3 1 per minute, disagreeing with transesophageal echocardiography constatations. Coronary angiography was normal. The patient
underwent surgery at which time the transesophageal echocardiographic findings of a fistula between the aorta and the right atrium were confirmed. The ruptured aneurysm was excised and the fistula was closed with a pericardial patch reinforced with Teflon. The post operative course was uneventful. 2.2. Case 2
The second patient was a 29-year-old man, known to be having a systolic murmur since the age of 7. This murmur had been presumed to be functional and no further explorations had been done previously. Clinical examination revealed a grade 4/6 continuous murmur heard best at the fourth left intercostal space. The electrocardiogram was normal. The transthoracic echocardiography showed normal chamber sizes. Continuous Doppler showed a continuous flow near the aortic root but precise location of this flow was not possible. Transesophageal echocardiography with color Doppler allowed the identification of a shunt between the right sinus of Valsalva and the right ventricle without other abnormalities. Subsequent aortography in the anterior oblique view confirmed the aorta to right ventricle fistula. Right chambers pressures were normal. Coronary arteries were found to be normal. Patient underwent surgery. An aneurysm of the right coronary sinus of Valsalva was found with rupture in the pulmonary infundibulum. There was a small (5 mm diameter) infundibulum ventricular septal defect which was closed with a Dacron patch. This associated ventricular septal defect have not been diagnosed neither by transesophageal echocardiography nor cardiac catheterization. The aneurysm was excised and the fistula closed with a Dacron patch. The postoperative course was uneventful.
3. Discussion
Aorto to right cameral fistula due to rupture of sinus of Valsalva are rare but their diagnosis is important since long term survival after surgical treatment is excellent (Matti!a et al., 1987; Van Son et al., 1994). Although the sinus aneurysms
C. Tron et al. / European Journal o f Ultrasound 4 (1996) 129-133
131
~is Fistula
N
1
Right atrium Right atrium Fig. 1. Transesophageal echocardiographyshowing a tube-like structure originating from the right coronary cusp and directing toward the right atrium (left). On the right, demonstration of the fistula between the aorta and the right atrium with addition of color flow Doppler.
are mostly congenital, rupture rarely occurs in infancy, and the majority of patients are operated on between 20 and 40 years of age, as our patients. In this report, we stress the value of transesophageal echocardiography for diagnosis of the rupture of a sinus of Valsalva aneurysm and precise localization of the fistulous communications between the aorta and the right heart chambers. Diagnosis of aortic to right cameral fistula by transesophageal echocardiography have been rarely reported in the litterature (Alam, 1993; Thomas et al., 1993). Indeed, several reports have documented the usefulness of transthoracic echocardiography for the diagnosis of sinus of Valsalva aneurysms and rupture (Nishimura et
al., 1976; Terdjman et al., 1984; Weyman et al., 1975). Injection of contrast in the aortic root during cardiac catheterization has been suggested to determine the location of the shunt with twodimensional echocardiography (Vered et al., 1985). However, good quality images may be difficult to obtain with transthoracic echocardiography leaving uncertainty in the diagnosis. In our second case, although continuous Doppler revealed a continuous flow near the aortic root, precise location of this flow was impossible, while transesophageal echocardiography easily and clearly showed the right coronary sinus aneurysm and its fistulous connection with the right atrium. Our study was performed using a biplane trans-
132
C. Tron et al. / European Journal o f Ultrasound 4 (1996) 129-133
esophageal transducer and it is likely that use of multiplane transducers would have facilitated the diagnosis. As in a previous report (Thomas et al., 1993), cardiac catheterization provided no additive information, except for the coronary anatomy that was normal. Given that patients with ruptured aneurysms of sinus of Valsalva are often young, complete diagnosis by transesophageal echocardiography should allow to proceed to surgery without cardiac catheterization. In contrary, in patients older than 40 years or with risk factors for coronary artery disease, a coronary arteriography should be performed. Moreover, in our first case, angiography mistakenly suggested a fistula between the aorta and the right ventricle. The superiority of the transesophageal echocardiography over aortic angiography in this case is not really surprising since the transesophageal approach, with its enhanced quality of imaging, provides a much better delineation of the aortic root than angiography. Important observations given by transesophageal echocardiography include (1) the location of the sinus of Valsalva involved; (2) which cardiac chamber the fistula enters; and also (3) the presence and the importance of a possible associated aortic regurgitation; (4) potential associated anomalies including presence and type of ventricular septal defect, right ventricular outflow tract obstruction and bicuspid aortic valve; and (5) potential associated complications such as endocarditis vegetations. Furthermore, transesophageal echocardiography may be useful for the follow up of patients after surgery. The association of a subarterial ventricular septal defect is not rare in patients with ruptured sinus of Valsalva aneurysm (Van Son et al., 1994). It may be useful to perform transesophageal echocardiography during surgery as a small ventricular septal defect can be missed by preoperative transesophageal echocardiography as in our second case. It is likely that in our second patient the systolic murmur known since he was 7 years old was due to the presence of the ventricular septal defect and that the apparition of a diastolic murmur corresponded to the rupture of the sinus of Valsalva aneurysm in the right ventricle. Thus, the occurrence of a diastolic murmur in a patient with known ventricular septal defect might be
considered as an indication to perform a transesophageal echocardiography.
4. Conclusion This report illustrates the potential for transesophageal echocardiography with color flow Doppler to diagnose and precisely define aortic to right cameral fistula secondary to rupture of sinus of Valsalva aneurysms. This technique should, therefore, become the investigation of choice in case of suspicion of ruptured sinus of Valsalva aneurysm and be considered early when transthoracic echocardiography results are questionable. Furthermore, transesophageal echocardiography might allow to operate on the patients without pre-operative cardiac catheterization.
Acknowledgements The authors wish to thank Franqois Bouchart M.D. and Nadine David M.D. for helpful discussion.
References Alam M. Transesophageal color flow Doppler features of aorta to right ventriclefistula. Chest 1993; 103: 1907-1908. Hoadley SD. Aortic pseudoaneurysmwith right atrial communication shown by Doppler color flow imaging. Am Heart J 1987; 114: 1535-1536. Mattila SP, Kupari M, Harjula ALJ, Ventila M, Meurala H, Maamies TJ, Aho P. Ruptured aneurysm of sinus of Valsalva: Long-term postoperative follow-up. Scand J Thorac Cardiovasc Surg 1987; 2l: 233-238. Nishimura K, Hibi N, Kato T, et al. Real-time observation of ruptured right sinus of Valsalva aneurysm by high speed ultrason-cardiotomography. Report of a case. Circulation 1976; 53: 732-735. Terdjman M, Bourdarias JP, Farcot JP et al. Aneurysms of sinus of Valsalva: two-dimensional echocardiographicdiagnosis and recognition of rupture into the right heart cavities. J Am Coil Cardiol 1984; 3: 1227-1235. Thomas MR, Monaghan MJ, Michalis LK, Jewitt DE. Aortoatrial fistulae diagnosed by transthoracic and transesophageal echocardiography: advantages of the transesophageal approach. J Am Soc Echocardiogr 1993; 6: 21-29.
C. Tron et al. / European Journal of Ultrasound 4 (1996) 129-133 Van Son JAM, Danielson GK, Schaff HV, Orszulak TA, Edwards WD, Seward JB. Long-term outcome of surgical repair of ruptured sinus of Valsalva aneurysm. Circulation 1994; 90 [part 2]: II-20-I1-29). Vered Z, Rath S, Benjamin P, Motro M, Neufeld HN. Ruptured sinus of Valsalva: Demonstration by contrast
133
echocardiography during cardiac catheterization. Am Heart J 1985; 109: 365-366. Weyman AE, Dillon JC, Feigenbaum H, Chang S. Premature pulmonic valve opening following sinus of Valsalva aneurysm rupture into the right atrium. Circulation 1975; 51: 556-560.