- Email: [email protected]
Congenital aneurysm of the left sinus of valsalva with an aortopulmonary tunnel
443
lACC Vol 7, No 2 February 1986.443-5
Congenital Aneurysm of the Left Sinus of Valsalva With an Aortopulmonary Tunnel DANIEL SCAGLIOTTL MD, ELIZABETH A. FISHER, MD, FACC, BARBARA 1. DEAL, MD, DAVID GORDON, MD, EVA V, CHOMKA, MD, BRUCE H. BRUNDAGE, MD, FACC Chicago, Illinois
Aneurysm of the left sinus of Valsalva is rare, and there is only one previous report of rupture into the pulmonary artery. This report describes a patient with valvular pulmonary atresia and ventricular septal defect in whom a portion of his pulmonary blood flow was supplied by
Congenital aneurysms of the sinus of Valsalva usually in• volve the noncoronary sinus, less frequently the right coro• nary sinus and rarely the left coronary sinus (I). Rupture of a sinus of Val salva aneurysm generally results in a fis• tulous connection between the aorta and a contiguous struc• ture, most often the right atrium or right ventricle. An aor• toatrial or aortoventricular tunnel may result from organization of fistulous connections created by rupture of the sinus of Val salva aneurysm occurring in utero (2). We report an unusual case with a left sinus of Valsalva aneurysm and an aortopulmonary tunnel.
Case Report History and physical examination. The patient is an 8 year old white boy first seen at our hospital at 2 years of age. He had undergone cardiac catheterization at another hospital at 3 months of age and a diagnosis of pulmonary atresia with ventricular septal defect was made. A com• munication between the aorta and the main pUlmonary artery was also found and was considered to be a left coronary artery to pUlmonary artery fistula. He was asymptomatic with normal growth and development. There was mild cy• anosis and clubbing. Cardiac examination showed a prominent right ventricFrom the University of Illinoi, College of MedIcine at ChIcago, ChI• cago, IllinOIS. Manu,cript receIved June II. 1985; revi,ed manuscript receIved July 30. 1985, accepted Augu:,t 12, 1985. Address for reprints: Daniel Scagliotti, MD, Pedlatnc CardIOlogy, UnI• versIty of IllInois HospIlal, 840 South Wood Street, Chicago. IllinOl, 60612 © 1986 by the Amencan College of CardIology
an aortopulmonary tunnel arising from a left sinus of Valsalva aneurysm. The surgical implications of precise definition of the type of aortopulmonary communication are discussed. (J Am Coll CardioI1986;7:443-S)
ular impulse, normal first heart sound and a loud and single second heart sound. A grade 3/6 harsh pansystolic murmur and a grade 2/6 continuous murmur were heard best at the mid-left sternal border and upper left sternal border, re• spectively. Continuous bruits were present over both lung fields. Liver and spleen were not palpable. Peripheral pulses were bounding. Cardiac catheterization. Repeat cardiac catheterization at 8 years of age showed a small left to right atrial shunt, a bidirectional ventricular shunt and large left to right aor• topulmonary shunts from both the ascending and descending aorta. Aortic saturation was 90%. Right ventricular pressure was at systemic level. The pUlmonary artery was not en• tered. but bilateral pulmonary venous wedge pressures were normal. Cineangiography showed valvular pulmonary atre• sia, large subaortic ventricular septal defect, overriding aorta, small atrial septal defect and left aortic arch. A typical windsock appearance of a large left sinus of Valsalva an• eurysm was demonstrated (Fig. I). The pulmonary blood supply was given by four large bronchial collateral arteries as well as by a fistulous connection between the aneurysm and the small main pulmonary artery (Fig. 2). Coronary angiography showed a normal left coronary artery (Fig. 3) that arose from the sinus of Valsalva aneurysm, 2.9 em from the aortic root. Other studies. Cross-sectional images of the great ar• teries obtained by two-dimensional echocardiography (Fig. 4) and ultrafast computed tomography (Fig. 5) also dem• onstrated the left sinus of Valsalva aneurysm and the aor• topulmonary tunnel. Current status. The patient remains asymptomatic and has not yet undergone surgery. 0735-1097/86/$3 50
444
-"
Ao
Discussion
,
'''.
J''';''
..,
.~~
~." _.h.,; ...
lACC Vol 7, No 2 February 1986443-5
SCAGLIOTTI ET AL LEFf SINUS OF V ALSAL VA ANEURYSM AND TUNNEL
..
;').
•.t_,'~".' _ ..c.
R
~:.:
Figure 1. Angiogram with contrast injection into the right ven• tricle (RV). frontal projection. The aorta (Ao) opacified from a large right to left shunt through the ventricular septal defect. The left sinus of Val salva aneurysm (An) was to the left of the aortic root. L = left pulmonary artery; R = right pulmonary artery.
Differential diagnosis. The differential diagnosis of nonsurgical communications between the ascending aorta and main pulmonary artery includes truncus arteriosus and aortopulmonary window, both of which are direct connec• tions, Indirect connections include anomalous origin of the left or right coronary artery from the pulmonary artery, coronary artery fistula, rupture of sinus of Valsalva aneu• rysm into the pulmonary artery and aortopulmonary tunnel. There are two previous reports of coronary artery fistula to the pulmonary artery (3,4), both in patients with pul• monary atresia and ventricular septal defect. In each, the sinus of Valsalva was normal and the connecting fistula arose from a coronary artery, The first report of rupture of a sinus of Valsalva aneurysm into the pulmonary artery in an adult with an otherwise normal heart appeared recently (5), The aneurysm involved the left sinus of Valsalva; the coronary artery anatomy was not reported, The present case is the first report of a left sinus of Valsalva aneurysm and aortopulmonary tunnel. The normal coronary anatomy ruled out a coronary artery fistula,
B
Figure 2. Angiograms with contrast injection into the left sinus of Valsalva aneurysm (An), A, Lat• eral projection, B, corresponding line drawing and C, frontal projection with cranial tilt. The aneu• rysm was posterior (A,B) and to the left (C) of the small main pulmonary artery (MPA), The aor• topulmonary tunnel (T) is best seen in A. The left coronary artery (LCA) also opacified from the aneurysm. CA = coronary artery; L = left pul• monary artery; R = right pulmonary artery,
•••"r....... "
JACC Vol 7, No 2
February 1986.443-5
Figure 3. Selective left coronary artery angiogram. lateral pro• jection, The left coronary artery (LCA) was normal. Cath = catheter.
Surgical implications. The distinction between an aor• topulmonary tunnel arising from a sinus of Valsalva an• eurysm and a coronary artery fistula communicating with the pulmonary artery has important surgical implications, Rastelli et aL (4), described a coronary artery fistula that could be ligated with impunity, In the present case there is Figure 4. Two-dimensional echocardiogram, parasternal short• axis view, great artery level. The large left SInUS of Valsalva aneurysm (AN) was posterior to the small main pulmonary artery (MPA) and connected to it by a short, linear aortopulmonary tunnel (T), The neck of the aneurysm is indicated by the open arrow. aortic root; L = left; P = posterior; R A = anterior; AO right.
SCAGLIOTII ET AL LEFT SINUS OF V ALSALV A ANEURYSM AND TUNNEL
445
Figure 5. Ultrafast computed tomography with contrast enhance• ment. cross-sectional view, great artery level. The large left sinus of Valsalva aneurysm (An), aortopulmonary tunnel (T) and main pulmonary artery (MP A) opacified from the aortic root after pe• ripheral venous injection of contrast medium. Ao = aorta (as• cending at upper right, descending at lower left); C = superior vena cava; LA = left atrium; other abbreviations as in Figure 4.
a high level of flow through the aneurysm and tunnel into the pulmonary artery. Ligation of the tunnel without repair of the aneurysm might result in sluggish flow in the aneu• rysm, thrombus formation and, possibly, severe compro• mise of left coronary artery flow. The potential for rupture of the unrepaired aneurysm must also be considered. Trans• aortic repair of the aneurysm would require considerable mobilization of the left coronary artery. If that proved im• possible, the surgeon might need to reroute flow to the left coronary artery through a graft. The long-term prognosis of coronary bypass grafts in children is unknown. This case is, to our knowledge, the first of its type re• ported. It illustrates the importance of careful delineation of the nature of an aortopulmonary communication before attempted surgical repair,
References Sakalbara S. Konno S. Congenital aneurysm of the smus of Valsalva. Anatomy and classification. Am Heart J 1962;63:405-24. 2. Heiner DC, Hara M, White HJ. Cardioaortlc fistulas and aneurysms of smw, of Valsalva in infancy. A report of an aortic-left atrial commu• nication indistinguishable from a ruptured aneurysm of the aortic sinus. Pediatncs 1961;27:415-26. 3. Allanby KD. Brinton WD, Campbell M. Gardner F. Pulmonary atresia and the collateral ClfculatJon to the lung. Guy's Hosp Rep 1950;99:110-52. 4. Rastelli GC. Ongley PA. DaVIS GD. Kirklin JW. Surgical repair for pulmonary valve atresia With coronary to pulmonary artery fistula. Report of a case. Mayo Clm Proc 1965;40:521-27. 5. Heilman KJ III. Groves BM. Campbell D. Blount SG Jr. Rupture of left sinus of Val salva aneurysm mto the pulmonary artery J Am Coli CardIOI1985;5:1005-7.
lACC Vol 7, No 2 February 1986.443-5
Congenital Aneurysm of the Left Sinus of Valsalva With an Aortopulmonary Tunnel DANIEL SCAGLIOTTL MD, ELIZABETH A. FISHER, MD, FACC, BARBARA 1. DEAL, MD, DAVID GORDON, MD, EVA V, CHOMKA, MD, BRUCE H. BRUNDAGE, MD, FACC Chicago, Illinois
Aneurysm of the left sinus of Valsalva is rare, and there is only one previous report of rupture into the pulmonary artery. This report describes a patient with valvular pulmonary atresia and ventricular septal defect in whom a portion of his pulmonary blood flow was supplied by
Congenital aneurysms of the sinus of Valsalva usually in• volve the noncoronary sinus, less frequently the right coro• nary sinus and rarely the left coronary sinus (I). Rupture of a sinus of Val salva aneurysm generally results in a fis• tulous connection between the aorta and a contiguous struc• ture, most often the right atrium or right ventricle. An aor• toatrial or aortoventricular tunnel may result from organization of fistulous connections created by rupture of the sinus of Val salva aneurysm occurring in utero (2). We report an unusual case with a left sinus of Valsalva aneurysm and an aortopulmonary tunnel.
Case Report History and physical examination. The patient is an 8 year old white boy first seen at our hospital at 2 years of age. He had undergone cardiac catheterization at another hospital at 3 months of age and a diagnosis of pulmonary atresia with ventricular septal defect was made. A com• munication between the aorta and the main pUlmonary artery was also found and was considered to be a left coronary artery to pUlmonary artery fistula. He was asymptomatic with normal growth and development. There was mild cy• anosis and clubbing. Cardiac examination showed a prominent right ventricFrom the University of Illinoi, College of MedIcine at ChIcago, ChI• cago, IllinOIS. Manu,cript receIved June II. 1985; revi,ed manuscript receIved July 30. 1985, accepted Augu:,t 12, 1985. Address for reprints: Daniel Scagliotti, MD, Pedlatnc CardIOlogy, UnI• versIty of IllInois HospIlal, 840 South Wood Street, Chicago. IllinOl, 60612 © 1986 by the Amencan College of CardIology
an aortopulmonary tunnel arising from a left sinus of Valsalva aneurysm. The surgical implications of precise definition of the type of aortopulmonary communication are discussed. (J Am Coll CardioI1986;7:443-S)
ular impulse, normal first heart sound and a loud and single second heart sound. A grade 3/6 harsh pansystolic murmur and a grade 2/6 continuous murmur were heard best at the mid-left sternal border and upper left sternal border, re• spectively. Continuous bruits were present over both lung fields. Liver and spleen were not palpable. Peripheral pulses were bounding. Cardiac catheterization. Repeat cardiac catheterization at 8 years of age showed a small left to right atrial shunt, a bidirectional ventricular shunt and large left to right aor• topulmonary shunts from both the ascending and descending aorta. Aortic saturation was 90%. Right ventricular pressure was at systemic level. The pUlmonary artery was not en• tered. but bilateral pulmonary venous wedge pressures were normal. Cineangiography showed valvular pulmonary atre• sia, large subaortic ventricular septal defect, overriding aorta, small atrial septal defect and left aortic arch. A typical windsock appearance of a large left sinus of Valsalva an• eurysm was demonstrated (Fig. I). The pulmonary blood supply was given by four large bronchial collateral arteries as well as by a fistulous connection between the aneurysm and the small main pulmonary artery (Fig. 2). Coronary angiography showed a normal left coronary artery (Fig. 3) that arose from the sinus of Valsalva aneurysm, 2.9 em from the aortic root. Other studies. Cross-sectional images of the great ar• teries obtained by two-dimensional echocardiography (Fig. 4) and ultrafast computed tomography (Fig. 5) also dem• onstrated the left sinus of Valsalva aneurysm and the aor• topulmonary tunnel. Current status. The patient remains asymptomatic and has not yet undergone surgery. 0735-1097/86/$3 50
444
-"
Ao
Discussion
,
'''.
J''';''
..,
.~~
~." _.h.,; ...
lACC Vol 7, No 2 February 1986443-5
SCAGLIOTTI ET AL LEFf SINUS OF V ALSAL VA ANEURYSM AND TUNNEL
..
;').
•.t_,'~".' _ ..c.
R
~:.:
Figure 1. Angiogram with contrast injection into the right ven• tricle (RV). frontal projection. The aorta (Ao) opacified from a large right to left shunt through the ventricular septal defect. The left sinus of Val salva aneurysm (An) was to the left of the aortic root. L = left pulmonary artery; R = right pulmonary artery.
Differential diagnosis. The differential diagnosis of nonsurgical communications between the ascending aorta and main pulmonary artery includes truncus arteriosus and aortopulmonary window, both of which are direct connec• tions, Indirect connections include anomalous origin of the left or right coronary artery from the pulmonary artery, coronary artery fistula, rupture of sinus of Valsalva aneu• rysm into the pulmonary artery and aortopulmonary tunnel. There are two previous reports of coronary artery fistula to the pulmonary artery (3,4), both in patients with pul• monary atresia and ventricular septal defect. In each, the sinus of Valsalva was normal and the connecting fistula arose from a coronary artery, The first report of rupture of a sinus of Valsalva aneurysm into the pulmonary artery in an adult with an otherwise normal heart appeared recently (5), The aneurysm involved the left sinus of Valsalva; the coronary artery anatomy was not reported, The present case is the first report of a left sinus of Valsalva aneurysm and aortopulmonary tunnel. The normal coronary anatomy ruled out a coronary artery fistula,
B
Figure 2. Angiograms with contrast injection into the left sinus of Valsalva aneurysm (An), A, Lat• eral projection, B, corresponding line drawing and C, frontal projection with cranial tilt. The aneu• rysm was posterior (A,B) and to the left (C) of the small main pulmonary artery (MPA), The aor• topulmonary tunnel (T) is best seen in A. The left coronary artery (LCA) also opacified from the aneurysm. CA = coronary artery; L = left pul• monary artery; R = right pulmonary artery,
•••"r....... "
JACC Vol 7, No 2
February 1986.443-5
Figure 3. Selective left coronary artery angiogram. lateral pro• jection, The left coronary artery (LCA) was normal. Cath = catheter.
Surgical implications. The distinction between an aor• topulmonary tunnel arising from a sinus of Valsalva an• eurysm and a coronary artery fistula communicating with the pulmonary artery has important surgical implications, Rastelli et aL (4), described a coronary artery fistula that could be ligated with impunity, In the present case there is Figure 4. Two-dimensional echocardiogram, parasternal short• axis view, great artery level. The large left SInUS of Valsalva aneurysm (AN) was posterior to the small main pulmonary artery (MPA) and connected to it by a short, linear aortopulmonary tunnel (T), The neck of the aneurysm is indicated by the open arrow. aortic root; L = left; P = posterior; R A = anterior; AO right.
SCAGLIOTII ET AL LEFT SINUS OF V ALSALV A ANEURYSM AND TUNNEL
445
Figure 5. Ultrafast computed tomography with contrast enhance• ment. cross-sectional view, great artery level. The large left sinus of Valsalva aneurysm (An), aortopulmonary tunnel (T) and main pulmonary artery (MP A) opacified from the aortic root after pe• ripheral venous injection of contrast medium. Ao = aorta (as• cending at upper right, descending at lower left); C = superior vena cava; LA = left atrium; other abbreviations as in Figure 4.
a high level of flow through the aneurysm and tunnel into the pulmonary artery. Ligation of the tunnel without repair of the aneurysm might result in sluggish flow in the aneu• rysm, thrombus formation and, possibly, severe compro• mise of left coronary artery flow. The potential for rupture of the unrepaired aneurysm must also be considered. Trans• aortic repair of the aneurysm would require considerable mobilization of the left coronary artery. If that proved im• possible, the surgeon might need to reroute flow to the left coronary artery through a graft. The long-term prognosis of coronary bypass grafts in children is unknown. This case is, to our knowledge, the first of its type re• ported. It illustrates the importance of careful delineation of the nature of an aortopulmonary communication before attempted surgical repair,
References Sakalbara S. Konno S. Congenital aneurysm of the smus of Valsalva. Anatomy and classification. Am Heart J 1962;63:405-24. 2. Heiner DC, Hara M, White HJ. Cardioaortlc fistulas and aneurysms of smw, of Valsalva in infancy. A report of an aortic-left atrial commu• nication indistinguishable from a ruptured aneurysm of the aortic sinus. Pediatncs 1961;27:415-26. 3. Allanby KD. Brinton WD, Campbell M. Gardner F. Pulmonary atresia and the collateral ClfculatJon to the lung. Guy's Hosp Rep 1950;99:110-52. 4. Rastelli GC. Ongley PA. DaVIS GD. Kirklin JW. Surgical repair for pulmonary valve atresia With coronary to pulmonary artery fistula. Report of a case. Mayo Clm Proc 1965;40:521-27. 5. Heilman KJ III. Groves BM. Campbell D. Blount SG Jr. Rupture of left sinus of Val salva aneurysm mto the pulmonary artery J Am Coli CardIOI1985;5:1005-7.