Aortocardiac fistula Robert L. Treasure, M.D., Lieutenant Colonel, MC, David C. Green, M.D., Colonel, MC,
USA*
USA*
Julius L. Bedynek, Jr., M.D., Lieutenant Colonel, MC, USA,** and Alan R. Hopeman, M.D.,*** Washington, D. C, and Columbia, Mo.
zVortocardiac fistula is an unusual entity. The diverse etiology of this condition, in cluding bacterial endocarditis, syphilis, rup ture of aneurysms of an aortic sinus of Valsalva, operative trauma during repair of a high ventricular septal defect, as well as penetrating and blunt trauma, has been emphasized in the literature. 16 Bacterial en docarditis and syphilis rarely initiate fistulization because of effective treatment with antimicrobial agents. With improved knowl edge and techniques, operative injury is now an unusual reason for fistula formation. Ruptured aneurysm of the sinus of Valsalva and trauma remain the most important causes of aortocardiac fistula. Gerbode, 7 in 1957, reported a case of aortoatrial fistula secondary to a bullet wound, and King and Shumacker,8 in 1958, reported a traumatic aortoventricular fistula with successful repair. Since that time, sucFrom the Department of Thoracic and Cardiovascular Surgery and the Section of Cardiology, Walter Reed General Hospital, Washington, D.C. 20012, and the Department of Surgery, Section of Thoracic and Cardiovascular Surgery, University of Missouri, Columbia, Mo. 65201. Received for publication April 2, 1971. •Assistant Chief, Thoracic and Cardiovascular Surgery, Walter Reed General Hospital, Washington, D. C. 20012. Present address: Box 6194, Fitzsimons General Hospital, Denver, Colo. 80240. »»Assistant Chief, Cardiology Service, Walter Reed General Hospital, Washington, D. C. 20012. •••Department of Surgery, Section of Thoracic and Cardiovascular Surgery, University of Missouri, Columbia, Mo. 65201.
8 18
cessful surgical repair of traumatic aorto cardiac fistula was reported approximately eight times as of 1968. 9 Ruptured aneurysms of the sinus of Val salva have been recognized as a cause of aortocardiac fistula since the initial descrip tion by James Hope in 1839. This lesion was first successfully repaired surgically by Lillehei and associates10 in 1957. The embryology, pathophysiology, and principles of surgical repair have been presented in detail in the literature. 111 ' 1 Due to the rela tive infrequency of other causes, ruptured Valsalva aneurysm represents the most com mon cause of aortocardiac fistula. We have had the opportunity to treat 6 patients with aortocardiac fistula during the past 3 years. This report summarizes this experience. Clinical material Table I shows the age, sex, cause of fistula, and the preoperative clinical status of the patients. There were 5 men and 1 woman. All were young adults. The etiology of the fistula varied. Patient 1 underwent openheart repair of a ventricular septal defect in 1960. Three days postoperatively, she developed a loud precordial murmur. Re peat catheterization in 1967 demonstrated an aortocardiac fistula, which was thought to be a result of direct trauma at the time of the original operation. In Patients 2 and
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Table I Age
Sex
26
Female
Previous VSD closure
Clinical status Exertional dyspnea, congestive heart failure
15
Male
Aneurysm of sinus of Valsalva associated with VSD
Exertional dyspnea, congestive heart failure
3
22
Male
Aneurysm of sinus of Valsalva associated with VSD
Exertional dyspnea
4
20
Male
Shell fragment wound
Asymptomatic
5
20
Male
Shell fragment wound
Asymptomatic
6
19
Male
Stab wound
Exertional dyspnea
Patient 1
Cause of fistula
Legend: VSD, Ventricular septal defect.
3, small supracristal ventricular defects and aneurysms of the aortic sinus of Valsalva were the basic cardiac defects. They de veloped aortocardiac fistula spontaneously as young adults. Patients 4, 5, and 6 de veloped the fistula as a result of penetrating injury. Patient 6 also sustained lacerated aortic valve cusps with resultant severe aortic régurgitation and is the subject of a separate report. Two patients were essen tially asymptomatic. The remainder com plained of exertional dyspnea. Two patients were successfully treated for congestive heart failure prior to operation. All patients underwent preoperative evaluation including cardiac catheterization with cine- or biplane angiography. Table II shows the localization of the fistula and the size of the shunt as calculated from oxygen studies. The ratio of pulmonary blood flow (PBF) to systemic blood flow (SBF) was from 1.2:1 to 2.0:1. In 4 patients the fistula was from the right coronary sinus of Valsalva to the right ventricle. In 1 patient, the fistula coursed from the noncoronary sinus to the right atrium. In the remaining patients, the exact sinus of origin could not be determined be cause the aorta was not opened. Surgical therapy A midline sternotomy was used in all cases. Cardiopulmonary bypass was estab lished by means of hemodilution with Ring er's lactate as the priming solution in a dis-
Table II Patient Localization of fistula PBF/SBF 1 Right coronary sinus to right 1.2:1 ventricle 2
Right coronary sinus to right ventricle
1.7:1
3
Right coronary sinus to right ventricle
1.7:1
4
Noncoronary sinus to atrium
5
Right coronary sinus to right ventricle
1.5:1
6
Right coronary sinus to right ventricle
1.5:1
right
2:1
Legend: PBF/SBF, Pulmonary blood flow to systemic blood flow.
posable bubble oxygenator. Hypothermia to 31 ° C. with coronary perfusion was utilized when a prolonged period of cardiac hypoxia was anticipated. Except in Patient 1, the fistula was ex posed from both ends by aortotomy and ap propriate cardiotomy. With the anatomy of the problem clearly demonstrated, closure of the fistula could be effected without dis torting the aortic valve or endangering coro nary arteries. Table III gives the surgical approach used in each patient. Results There were no deaths in this series. Pa tient 6 underwent an early secondary tho-
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Thoracic and Cardiovascular Surgery
Table III Patient 1 2 3 4 5 6
Date of operation Nov. 21, 1967 Jan. 9, 1969 May 28, 1969 April 10, 1968 April 16, 1969 Oct. 15, 1969
Procedure Ventriculotomy and closure of fistula from ventricular side only Ventriculotomy, aortotomy closure of fistula from both sides Ventriculotomy, aortotomy closure of fistula from both sides Ventriculotomy, aortotomy closure of fistula from both sides Ventriculotomy, aortotomy closure of fistula from both sides Ventriculotomy, aortotomy closure of fistula and repair of aortic cusps
racotomy for excessive bleeding. Patient 1 has a Grade 1 of 6 murmur of aortic in sufficiency but no evidence of insufficiency on biplane angiography. Patient 2 has a Grade 2 of 6 murmur of insufficiency but no evidence of hemodynamically significant insufficiency. All are asymptomatic. Discussion This series adds evidence to the concept that aortocardiac fistula can be treated with low mortality and morbidity. The presence of 3 patients with a fistula secondary to trauma reflects the large vol ume of traumatic heart disease seen in a major military medical center and does not alter the previous statement that rupture of aneurysms of the sinus of Valsalva is the most frequent cause of fistula formation. The necessity of visualizing the aortic ori fice of the defect as well as the cardiac ori fice remains controversial.15"17 The aortot omy undoubtedly makes the operative pro cedure somewhat more complex, but this disadvantage is often outweighed by the ex cellent exposure obtained. Each case must be individualized, but in general we believe that it is important to visualize the aortic side of the defect as well as the cardiac side for the following reasons: (1) A more secure obliteration of the fistula can be ob tained by suturing both ends, and thus, theoretically, the possibility of recurrence is reduced; (2) visualization of the aortic valve and coronary arteries reduces the chance of injury to these structures; and (3) the status of the aortic valve can be evaluated, and valvuloplasty or replace ment can be performed if indicated. This is especially important when aortic insuf
ficiency is secondary to an aneurysm of the sinus of Valsalva in association with a ven tricular septal defect. The association of ruptured aneurysms of the right sinus of Valsalva with supracristal ventricular septal defect and aortic insuf ficiency has been emphasized recently by Sakakibara and Konno. 18 The pathologic explanation in terms of a defect in the conal septum has been advanced by VanPraagh.10 The ventricular septal defect may be small or occluded by the prolapsing aortic cusp, and its presence may be easily missed by preoperative catheterization data. Further, the frequent association of serious compli cations, such as fistulization of aneurysms of the sinus of Valsalva or progressive aortic insufficiency with supracristal defects, suggests that these should be closed even if the calculated shunt is small. Accurate preoperative diagnosis by angio cardiography is of paramount importance in planning and executing a successful op eration. We have encountered some dif ficulty in obtaining diagnostic angiocardio grams due to the rapid runoff of contrast materials. Therefore, large-bolus, rapid-in jection, selective aortic root studies are necessary. Since the patients in this study were operated upon recently, no long-term fol low-up data are available. At the present time, all patients are considered to have been cured, and none has evidence of re current fistula. Summary A series of 6 patients with aortocardiac fistula of diverse etiology who underwent successful operative closure at Walter Reed
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General Hospital over the past 3 years is presented. Operative treatment is described with emphasis being given to the advisability of exposing the fistula by aortotomy and ap propriate cardiotomy and subsequently closing the fistula from both ends. Attention is called to those instances in which aortic régurgitation is secondary to fistulization of aneurysms in association with supracristal ventricular defects. Operative therapy is recommended for all patients with aortocardiac fistula. REFERENCES 1 Oram, S., and East, T.: Rupture of Aneurysm of Aortic Sinus of Valsalva into the Right Side of the Heart, Br. Heart J. 17: 541, 1955. 2 Kieffer, S. A., and Winchell, P.: Congenital Aneurysms of the Aortic Sinuses With Cardioaortic Fistula, Dis. Chest. 38: 79, 1960. 3 Magidson, O., and Kay, J. H.: Ruptured Aortic Sinus Aneurysms, Am. Heart J. 65: 597, 1963. 4 Agustsson, M. H., Weinberg, M., Jr., Gasul, B. M., Fell, E. H., Arcilla, R. A., Bicoff, J. P., Steiger, Z., and Iwa, T.: Aortic-Cardiac Fistula Following Corrective Operations for Ventricular Septal Defects and Tetralogy of Fallot, J. THORAC. CARDIOVASC. SURG. 43: 166,
1962. 5 Sawyers, J. L., Adams, J. E., and Scott, H. W.: Surgical Treatment of Aneurysms of the Aortic Sinuses With Aorticoatrial Fistula, Surgery 41: 26, 1957. 6 Summerall, C. P., Lee, W. H., and Boone, J. A.: Intracardiac Shunts After Penetrating Wounds of the Heart, N . Engl. J. Med. 272: 240, 1965. 7 Gerbode, F.: Discussion of Sawyers, J. L., Adams, J. E., and Scott, H. W.: Surgical Treat ment of Aneurysms of the Aortic Sinuses With Aorticoatrial Fistula: Experimental and Clini cal Study, Surgery 41: 42, 1957.
8 King, H., and Shumacker, H . B., Jr.: Surgical Repair of a Traumatic Aortic-Right Ventricular Fistula,
J.
THORAC.
CARDIOVASC.
SURG.
35:
734, 1958. 9 Villareal, R., Fries, C. C, Cheng, T. 0 . , and Potter, R. T.: Traumatic Aortico-Right Ven tricular Fistula, Ann. Thorac. Surg. 5: 36, 1968. 10 Lillehei, C. W., Stanley, P., and Varco, R. L.: Surgical Treatment of Ruptured Aneurysms of the Sinus of Valsalva, Ann. Surg. 146: 459, 1957. 11 Gibbs, N . M., and Harris, E. L.: Aortic Sinus Aneurysms, Br. Heart J. 23: 131, 1961. 12 Morgan-Jones, A., and Langley, F . A.: Aortic Sinus Aneurysms, Br. Heart J. 11: 325, 1949. 13 Edwards, J. E., and Burchell, H . B.: T h e Pathologic Anatomy of Deficiencies Between the Aortic Root and the Heart, Including Aortic Sinus Aneurysms, Thorax 12: 125, 1957. 14 Besterman, E. M. M., Goldberg, M . J., and Sellors, T. H.: Surgical Repair of Ruptured Sinus of Valsalva, Br. Med. J. 5354: 410, 1963. 15 Shumacker, H . B., King, H., and Waldhausen, J. A.: Transaortic Approach for the Repair of Ruptured Aneurysms of the Sinuses of Val salva, Ann. Surg. 161: 946, 1965. 16 Bosher, L. H . : The Combined Surgical A p proach for Correction of Congenital Aortic Sinus Fistula Into the Right Atrium, J. T H O R A C CARDIOVASC. SURG. 50: 243, 1965.
17 D e Bakey, M. E., Diethrich, E. B., Liddicoat, J. E., Kinard, S. A., and Garrett, H . E.: Abnormalities of the Sinuses of Valsalva, J. THORAC. CARDIOVASC. SURG. 54: 312, 1967.
18 Sakakibara, S., and Konno, S.: Congenital Aneurysm of the Sinus of Valsalva Associated With Ventricular Septal Defect, A m . Heart J. 75: 595, 1968. 19 VanPraagh, R., and McNamara, J. J.: Ana tomic Types of Ventricular Septal Defect With Aortic Insufficiency, A m . Heart J. 75: 604, 1968.