COLLECTIVE REVIEW
Congenital Left Aortic Sinus-Left Ventricle Fistula and Review of Aortocardiac Fistulas Edward R. Nowicki, M.D., Eoin Aberdeen, F.R.C.S., Sidney Friedman, M.D., and William J. Rashkind, M.D. ABSTRACT A fistula from the left aortic sinus to the left ventricle is reported that was successfully repaired using cardiopulmonary bypass. In addition, 175 cases of fistula to the heart from the aortic sinuses published in the English literature from 1839 through 1972 were studied, with particular emphasis on the 126 patients who underwent operative repair. The major cause of the fistulas (76%)was found to be rupture of a congenital aortic sinus aneurysm, usually during the third or fourth decade of life. The remainder of these fistulas to the heart consisted of simply a tract in an otherwise normal sinus. Ventricular septa1 defect was the most common associated defect and, when present, was nearly always related to a fistula arising from the right aortic sinus. Repair was successfulin 86% of the 126 operatedpatients. The principles of operative treatment of these fistulas are reviewed.
Fistulas from the root of the aorta to a chamber of the heart lead to gross disturbance of cardiac function. The large gradient between the highpressure aorta and low-pressure cardiac chamber causes a high flow, for which the heart must compensate to maintain adequate perfusion. These fistulas may result from rupture of congenital or acquired aneurysms of the aortic sinuses as well as from penetrating trauma [6, 33, 56, 65, 75, 79, 871. While aneurysms due to arteriosclerosis, syphilis, infectious endocarditis, or Marfan’s syndrome may be more common than their congenital counterparts, they are less often limited to just the sinuses. They tend to involve the ascending aorta, and when rupture takes place it may be into the pericardial, From the Department of Surgery, Hospital of the University of Pennsylvania, the Department of Pediatrics, University of Pennsylvania School of Medicine, the Division of Cardiology, The Children’s Hospital, Philadelphia, PA, and the Division of Cardiothoracic Surgery, Mt Sinai Medical Center, New York, NY. Address reprint requests to Dr. Nowicki, 7 Bramhall St, Portland, ME 04102.
378
mediastinal, or pleural space as well as into the heart. Because of the intracardiac location of the aortic sinuses, on the other hand, congenital weaknesses at this site cause the aneurysm to arise in a chamber of the heart, so that rupture nearly always causes a fistula between the aorta and the heart [56]. Not all congenital aneurysms of the aortic sinuses cause symptoms by rupture and fistula formation. Some aneurysms that have not ruptured are associated with aortic regurgitation because of related cusp abnormalities or inadequate support for the aortic annulus [31, 51, 58, 78,891. The left sinus aneurysms, because of their location and size, may press on the left coronary artery, with resultant obstruction to coronary flow and myocardial infarction [28,29]. Other aneurysms may affect various parts of the atrioventricular conduction system, either by direct pressure [25, 651 or by rupture into the interventricular septum without fistula formation [47]. Also, there is one report of relative pulmonary stenosis secondary to congenital aortic sinus aneurysm arising in the pulmonary outflow tract of the right ventricle [65]. Another related but less common defect of the aortic sinus is a fistulous tract without aneurysm. This tract may very well exist prior to birth [641. The least common aortic root lesions associated with fistulas are the aortoventricular tunnels. These lesions are not actually located in the aortic sinus but have their opening in the aorta just above the supravalvular ridge distal to the aortic sinuses. The channel bypasses the aortic valve and passes either through the interventricular septum to the left ventricle or directly into the right ventricle [8, 19, 48, 631. We wish to direct attention to the fistula associated with ruptured congenital aneurysm of an aortic sinus and to a less common variation, the fistulous tract without an aneurysmal sac. Nearly all possible variations of fistula between an aortic sinus and a chamber of the heart have
379 Collective Review: Nowicki et al: Aortocardiac Fistulas
been reported except for a fistula between the vealed left ventricular hypertrophy with a left right sinus and left atrium and that between the ventricular strain pattern. The chest roentgenoleft sinus and left ventricle. We report a patient gram showed cardiomegaly due to left ventricuwith a fistula connecting the left sinus of Val- lar enlargement, with no increase in the size of salva and left ventricle that was successfully re- the left atrium. Results of cardiac catheterization paired using cardiopulmonary bypass. In addi- and angiocardiography were interpreted as tion, enough reports on operative repair of all showing an aneurysm of the left aortic sinus types of congenital sinus of Valsalva fistulas with aortic regurgitation and possible aneuryshave appeared to warrant a review of the opera- mal dilatation of the anterior wall of the left tive experience with this abnormality. ventricle. On December 1, 1971, at the age cf S1/2 years, Case History she was again admitted for evaluation because A 5-year-old white girl, found to have a marked of electrocardiographic signs of progressive left cardiac murmur on routine physical examina- ventricular hypertrophy and T-wave changes. tion, was admitted to The Children’s Hospital of She had developed no symptoms in the interim. Philadelphia on October 30, 1968, for evalua- The clinical examination was unchanged, with tion. She had grown normally and had no car- persistence of systolic and diastolic murmurs diac symptoms. O n physical examination her and a heightened left ventricular impulse. The carotid pulses had a collapsing quality, and the chest roentgenogram revealed an increase in the blood pressure was 110/50. A systolic ejection heart size. The angiocardiogram at this time murmur was heard at the aortic area and a identified a long tunnel from the aorta to the high-pitched, blowing, diastolic murmur at the body of the left ventricle without aortic regurgiapex. There were no signs of cardiac failure or tation (Fig 1).The left coronary artery was norother abnormality. The electrocardiogram re- mal. Operation was performed on February 2, Fig 1 . ( A ) Cineaortogram (retouched for clarity) 1972. The chest was entered through a midline w i t h (B) line interpretation of a fistulous tract f r o m sternotomy, and after the pericardium was the left aortic sinus to the left ventricle in a opened, a thrill was palpable over the left ventri9-year-old girl. (Ao = ascending aorta; LSV = left sinus of Valsalva; T = tract; LV = left ventricle.) cle and the root of the aorta. The ascending aorta
A
B
380 The Annals of Thoracic Surgery Vol 23
No 4
April 1977
was dilated for 4 cm starting 1 cm above the valve ring. The aortic sinuses were not dilated. The coronary arteries appeared normal in size on the surface of the heart. After the cavae and aorta were cannulated and cardiopulmonary bypass had been commenced, the patient was cooled to 22°C. The aorta was cross-clamped and opened by a transverse incision. The aortic valve was normal, but a fistulous tract led from the left sinus of Valsalva through the posterolateral portion of the left ventricular outflow tract to the cavity of the left ventricle (Fig 2A). It was a wide tract, measuring 8 mm in diameter. The opening, near the middle of the sinus, was separated from the valve ring and cusp tissue by 6 mm of aortic wall. The right coronary artery orifice appeared normal in the right aortic sinus. The left coronary artery lay superior and anterior to the orifice of the fistula. The artery had two openings. The superior one was the larger, and the lower one was separated from the fistula by 3 mm. The course of the left coronary artery was identified with a probe in
place while mattress sutures effectively closed the tract opening, uniting the aortic media of the superior margin of the orifice to the valve ring tissue (Fig 2B). The aortic incision was closed. Bypass was discontinued without difficulty after rewarming. No murmur or thrill was present after the repair. The postoperative course was uncomplicated, and the patient was discharged on the ninth postoperative day. She remained well four years after her operation without murmurs or any other evidence of recurrence of her fistula.
Fig 2.( A )The aorta has been opened. T h e probe is in the left coronary artery and the coronary sucker is i n theostium ofthefistulous tract in theleft sinus. ( B ) After repair the mattresssuturesare tied, closing the opening in thefistula. (L = left aortic cusp; NC = noncoronary cusp; R = right aortic cusp; LC = left coronary artery.)
Previous Cases of Aortocardiac Fistula Our review of the English literature from 1839 through 1972 identified 175 cases of fistula from a congenital aortic sinus (Valsalva) aneurysm to the heart, with particular emphasis on the 126 patients treated by operation [l-5, 7, 9, 10-22, 24,26,28,30,32,34,35-41,43-46,49,50,52-62, 65, 66, 69-73, 77, 80-85, 88-901. We eliminated the cases of fistula from rupture of acquired aneurysm. When there was associated infectious endocarditis, we included only cases in which the infection was secondary to the fistula or an associated ventricular septa1 defect. Data on some patients were incomplete, and we have included only cases with sufficient reported information to permit an anatomical diagnosis. We examined up to 37 items of information for
A
R
381 Collective Review: Nowicki et al: Aortocardiac Fistulas
each patient. The data were analyzed by a Digital Equipment Corporation PDP-10 Computer at the University of Pennsylvania Medical School Computer Facility using the statistical program package from the Health Science Computer Facility at the University of California, Los Angeles [23]. Analysis of variance (Snedecor f-test) was used to investigate the significance of variations observed [741. The age at operation or at death in the nonoperated cases was recorded for 145 patients and ranged from 6 weeks to 79 years (mean, 30.8 years) (Fig 3). Of the 145 patients for whom the sex was reported, 96 (66.2%) were male and 49 (33.8%) were female. The absence of symptoms or nature of their onset in 125 patients was grouped into three categories: no symptoms, 22 patients (17.6%); gradual onset of symptoms, 57 patients (45.6%); and sudden onset of symptoms, 46 patients (36.8%). Gradual onset was defined as symptoms developing over months or years. In the group with sudden onset the symptoms developed acutely, often catastrophically, usually within days. The mean age was 21.4 years for patients without symptoms, 33.9 years for those in whom the onset of symptoms was gradual, and 31.6 years for those in whom the onset was sudden. The difference in age between the group without symptoms and each of the groups with symptoms was significant ( p < 0.005); the age difference between the two groups with symptoms was not statistically significant. The symptoms at onset were usually dyspnea, pain, or both. Of 91 patients in whom the findings were reported, 77 (84.6%) had dyspnea and 14 Fig3. A g e distribution, b y decade, of 145 patients. 80-89
Range = 6 Weeks to 79 years Mean = 3 0 . 8 years
70-79
-c t w 0
60-69
(15.4%) did not. In 71 patients in whom the findings were reported, 23 (32.4%) had pain in the chest or abdomen, or both, at onset and 48 patients (67.6%) did not. Of the 23 patients with pain, 21 (91.3%) also had dyspnea. The mean blood pressure in 90 patients was 141 f 26 mm Hg systolic (range, 90-250) and 45 f 26 mm Hg diastolic (range, 0-105). The pulse character in 63 patients was described as collapsing or bounding in 52 (82.5%) and normal in 11 (17.5%) (Fig 4). A precordial thrill was palpated in 74 (78.7%) of 94 patients, not palpable in 20 (21.3%). Of 122 patients, 114 (93.4%) had a continuous murmur and 8 (6.6%) did not (Fig 4). Clinical signs of heart failure (rales, cardiomegaly, hepatomegaly) were observed in 55 of 91 patients (60%). The mean age of the patients with signs of failure was 34.4 years; it was 25.3 years in those with no signs of failure ( p < 0.005). Roentgenograms showed increased pulmonary vascular markings in 78 of 103 patients (76%) and enlarged cardiac size in 97 of 105 patients (92%). In 90 patients for whom electrocardiographic results were reported, 71 (79%)had abnormal patterns. Of these patients, 57 (80%) had some sign of left ventricular abnormality such as hypertrophy or strain. The ratio of calculated pulmonarylsystemic blood flow (QJQJ ranged from 1.2 to 5.0 (mean, 2.2 f 0.92) in the 30 patients in which this figure was recorded. In 19 patients the left-to-right shunt was measured in liters per minute and ranged from 3.0 to 9.9 (mean, 6.5 f 2.6). The pulmonary artery pressure was recorded in 26 patients; the systolic pressure ranged from 17 to 72 mm Hg (mean, 42 f 18) and the diastolic pressure from 5 to 40 mm Hg (mean, 16 f 7). The Fig4. Cardiac murmur in 122 patients and pulse character i n 63 patients.
50-59 40-49
30-39 CHARACTER-63 PULSE
20-29
Bounding 8 2 . 5 % Normal 17.5%
10-19
1
1-9
0
Congenital Left Aortic Sinus-Left Ventricle Fistula and Review of Aortocardiac Fistulas Edward R. Nowicki, M.D., Eoin Aberdeen, F.R.C.S., Sidney Friedman, M.D., and William J. Rashkind, M.D. ABSTRACT A fistula from the left aortic sinus to the left ventricle is reported that was successfully repaired using cardiopulmonary bypass. In addition, 175 cases of fistula to the heart from the aortic sinuses published in the English literature from 1839 through 1972 were studied, with particular emphasis on the 126 patients who underwent operative repair. The major cause of the fistulas (76%)was found to be rupture of a congenital aortic sinus aneurysm, usually during the third or fourth decade of life. The remainder of these fistulas to the heart consisted of simply a tract in an otherwise normal sinus. Ventricular septa1 defect was the most common associated defect and, when present, was nearly always related to a fistula arising from the right aortic sinus. Repair was successfulin 86% of the 126 operatedpatients. The principles of operative treatment of these fistulas are reviewed.
Fistulas from the root of the aorta to a chamber of the heart lead to gross disturbance of cardiac function. The large gradient between the highpressure aorta and low-pressure cardiac chamber causes a high flow, for which the heart must compensate to maintain adequate perfusion. These fistulas may result from rupture of congenital or acquired aneurysms of the aortic sinuses as well as from penetrating trauma [6, 33, 56, 65, 75, 79, 871. While aneurysms due to arteriosclerosis, syphilis, infectious endocarditis, or Marfan’s syndrome may be more common than their congenital counterparts, they are less often limited to just the sinuses. They tend to involve the ascending aorta, and when rupture takes place it may be into the pericardial, From the Department of Surgery, Hospital of the University of Pennsylvania, the Department of Pediatrics, University of Pennsylvania School of Medicine, the Division of Cardiology, The Children’s Hospital, Philadelphia, PA, and the Division of Cardiothoracic Surgery, Mt Sinai Medical Center, New York, NY. Address reprint requests to Dr. Nowicki, 7 Bramhall St, Portland, ME 04102.
378
mediastinal, or pleural space as well as into the heart. Because of the intracardiac location of the aortic sinuses, on the other hand, congenital weaknesses at this site cause the aneurysm to arise in a chamber of the heart, so that rupture nearly always causes a fistula between the aorta and the heart [56]. Not all congenital aneurysms of the aortic sinuses cause symptoms by rupture and fistula formation. Some aneurysms that have not ruptured are associated with aortic regurgitation because of related cusp abnormalities or inadequate support for the aortic annulus [31, 51, 58, 78,891. The left sinus aneurysms, because of their location and size, may press on the left coronary artery, with resultant obstruction to coronary flow and myocardial infarction [28,29]. Other aneurysms may affect various parts of the atrioventricular conduction system, either by direct pressure [25, 651 or by rupture into the interventricular septum without fistula formation [47]. Also, there is one report of relative pulmonary stenosis secondary to congenital aortic sinus aneurysm arising in the pulmonary outflow tract of the right ventricle [65]. Another related but less common defect of the aortic sinus is a fistulous tract without aneurysm. This tract may very well exist prior to birth [641. The least common aortic root lesions associated with fistulas are the aortoventricular tunnels. These lesions are not actually located in the aortic sinus but have their opening in the aorta just above the supravalvular ridge distal to the aortic sinuses. The channel bypasses the aortic valve and passes either through the interventricular septum to the left ventricle or directly into the right ventricle [8, 19, 48, 631. We wish to direct attention to the fistula associated with ruptured congenital aneurysm of an aortic sinus and to a less common variation, the fistulous tract without an aneurysmal sac. Nearly all possible variations of fistula between an aortic sinus and a chamber of the heart have
379 Collective Review: Nowicki et al: Aortocardiac Fistulas
been reported except for a fistula between the vealed left ventricular hypertrophy with a left right sinus and left atrium and that between the ventricular strain pattern. The chest roentgenoleft sinus and left ventricle. We report a patient gram showed cardiomegaly due to left ventricuwith a fistula connecting the left sinus of Val- lar enlargement, with no increase in the size of salva and left ventricle that was successfully re- the left atrium. Results of cardiac catheterization paired using cardiopulmonary bypass. In addi- and angiocardiography were interpreted as tion, enough reports on operative repair of all showing an aneurysm of the left aortic sinus types of congenital sinus of Valsalva fistulas with aortic regurgitation and possible aneuryshave appeared to warrant a review of the opera- mal dilatation of the anterior wall of the left tive experience with this abnormality. ventricle. On December 1, 1971, at the age cf S1/2 years, Case History she was again admitted for evaluation because A 5-year-old white girl, found to have a marked of electrocardiographic signs of progressive left cardiac murmur on routine physical examina- ventricular hypertrophy and T-wave changes. tion, was admitted to The Children’s Hospital of She had developed no symptoms in the interim. Philadelphia on October 30, 1968, for evalua- The clinical examination was unchanged, with tion. She had grown normally and had no car- persistence of systolic and diastolic murmurs diac symptoms. O n physical examination her and a heightened left ventricular impulse. The carotid pulses had a collapsing quality, and the chest roentgenogram revealed an increase in the blood pressure was 110/50. A systolic ejection heart size. The angiocardiogram at this time murmur was heard at the aortic area and a identified a long tunnel from the aorta to the high-pitched, blowing, diastolic murmur at the body of the left ventricle without aortic regurgiapex. There were no signs of cardiac failure or tation (Fig 1).The left coronary artery was norother abnormality. The electrocardiogram re- mal. Operation was performed on February 2, Fig 1 . ( A ) Cineaortogram (retouched for clarity) 1972. The chest was entered through a midline w i t h (B) line interpretation of a fistulous tract f r o m sternotomy, and after the pericardium was the left aortic sinus to the left ventricle in a opened, a thrill was palpable over the left ventri9-year-old girl. (Ao = ascending aorta; LSV = left sinus of Valsalva; T = tract; LV = left ventricle.) cle and the root of the aorta. The ascending aorta
A
B
380 The Annals of Thoracic Surgery Vol 23
No 4
April 1977
was dilated for 4 cm starting 1 cm above the valve ring. The aortic sinuses were not dilated. The coronary arteries appeared normal in size on the surface of the heart. After the cavae and aorta were cannulated and cardiopulmonary bypass had been commenced, the patient was cooled to 22°C. The aorta was cross-clamped and opened by a transverse incision. The aortic valve was normal, but a fistulous tract led from the left sinus of Valsalva through the posterolateral portion of the left ventricular outflow tract to the cavity of the left ventricle (Fig 2A). It was a wide tract, measuring 8 mm in diameter. The opening, near the middle of the sinus, was separated from the valve ring and cusp tissue by 6 mm of aortic wall. The right coronary artery orifice appeared normal in the right aortic sinus. The left coronary artery lay superior and anterior to the orifice of the fistula. The artery had two openings. The superior one was the larger, and the lower one was separated from the fistula by 3 mm. The course of the left coronary artery was identified with a probe in
place while mattress sutures effectively closed the tract opening, uniting the aortic media of the superior margin of the orifice to the valve ring tissue (Fig 2B). The aortic incision was closed. Bypass was discontinued without difficulty after rewarming. No murmur or thrill was present after the repair. The postoperative course was uncomplicated, and the patient was discharged on the ninth postoperative day. She remained well four years after her operation without murmurs or any other evidence of recurrence of her fistula.
Fig 2.( A )The aorta has been opened. T h e probe is in the left coronary artery and the coronary sucker is i n theostium ofthefistulous tract in theleft sinus. ( B ) After repair the mattresssuturesare tied, closing the opening in thefistula. (L = left aortic cusp; NC = noncoronary cusp; R = right aortic cusp; LC = left coronary artery.)
Previous Cases of Aortocardiac Fistula Our review of the English literature from 1839 through 1972 identified 175 cases of fistula from a congenital aortic sinus (Valsalva) aneurysm to the heart, with particular emphasis on the 126 patients treated by operation [l-5, 7, 9, 10-22, 24,26,28,30,32,34,35-41,43-46,49,50,52-62, 65, 66, 69-73, 77, 80-85, 88-901. We eliminated the cases of fistula from rupture of acquired aneurysm. When there was associated infectious endocarditis, we included only cases in which the infection was secondary to the fistula or an associated ventricular septa1 defect. Data on some patients were incomplete, and we have included only cases with sufficient reported information to permit an anatomical diagnosis. We examined up to 37 items of information for
A
R
381 Collective Review: Nowicki et al: Aortocardiac Fistulas
each patient. The data were analyzed by a Digital Equipment Corporation PDP-10 Computer at the University of Pennsylvania Medical School Computer Facility using the statistical program package from the Health Science Computer Facility at the University of California, Los Angeles [23]. Analysis of variance (Snedecor f-test) was used to investigate the significance of variations observed [741. The age at operation or at death in the nonoperated cases was recorded for 145 patients and ranged from 6 weeks to 79 years (mean, 30.8 years) (Fig 3). Of the 145 patients for whom the sex was reported, 96 (66.2%) were male and 49 (33.8%) were female. The absence of symptoms or nature of their onset in 125 patients was grouped into three categories: no symptoms, 22 patients (17.6%); gradual onset of symptoms, 57 patients (45.6%); and sudden onset of symptoms, 46 patients (36.8%). Gradual onset was defined as symptoms developing over months or years. In the group with sudden onset the symptoms developed acutely, often catastrophically, usually within days. The mean age was 21.4 years for patients without symptoms, 33.9 years for those in whom the onset of symptoms was gradual, and 31.6 years for those in whom the onset was sudden. The difference in age between the group without symptoms and each of the groups with symptoms was significant ( p < 0.005); the age difference between the two groups with symptoms was not statistically significant. The symptoms at onset were usually dyspnea, pain, or both. Of 91 patients in whom the findings were reported, 77 (84.6%) had dyspnea and 14 Fig3. A g e distribution, b y decade, of 145 patients. 80-89
Range = 6 Weeks to 79 years Mean = 3 0 . 8 years
70-79
-c t w 0
60-69
(15.4%) did not. In 71 patients in whom the findings were reported, 23 (32.4%) had pain in the chest or abdomen, or both, at onset and 48 patients (67.6%) did not. Of the 23 patients with pain, 21 (91.3%) also had dyspnea. The mean blood pressure in 90 patients was 141 f 26 mm Hg systolic (range, 90-250) and 45 f 26 mm Hg diastolic (range, 0-105). The pulse character in 63 patients was described as collapsing or bounding in 52 (82.5%) and normal in 11 (17.5%) (Fig 4). A precordial thrill was palpated in 74 (78.7%) of 94 patients, not palpable in 20 (21.3%). Of 122 patients, 114 (93.4%) had a continuous murmur and 8 (6.6%) did not (Fig 4). Clinical signs of heart failure (rales, cardiomegaly, hepatomegaly) were observed in 55 of 91 patients (60%). The mean age of the patients with signs of failure was 34.4 years; it was 25.3 years in those with no signs of failure ( p < 0.005). Roentgenograms showed increased pulmonary vascular markings in 78 of 103 patients (76%) and enlarged cardiac size in 97 of 105 patients (92%). In 90 patients for whom electrocardiographic results were reported, 71 (79%)had abnormal patterns. Of these patients, 57 (80%) had some sign of left ventricular abnormality such as hypertrophy or strain. The ratio of calculated pulmonarylsystemic blood flow (QJQJ ranged from 1.2 to 5.0 (mean, 2.2 f 0.92) in the 30 patients in which this figure was recorded. In 19 patients the left-to-right shunt was measured in liters per minute and ranged from 3.0 to 9.9 (mean, 6.5 f 2.6). The pulmonary artery pressure was recorded in 26 patients; the systolic pressure ranged from 17 to 72 mm Hg (mean, 42 f 18) and the diastolic pressure from 5 to 40 mm Hg (mean, 16 f 7). The Fig4. Cardiac murmur in 122 patients and pulse character i n 63 patients.
50-59 40-49
30-39 CHARACTER-63 PULSE
20-29
Bounding 8 2 . 5 % Normal 17.5%
10-19
1
1-9
0
