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Rupture of a sinus of Valsalva aneurysm∗
Case Reports Rupture
of a Sinus of Valsalva
BERNARD L. SEGAL, bin.,
b’lLLIAhf
LIKOFF, M.D., F.A.C.C.
Philadelphia,
N 1839 Hope described a “case of aneurysmal pouch of the aorta, bursting into the right ventricle,” the first published account of a ruptured congenital aortic sinus aneurysm.’ In the following one hundred years, 18 similar cases were reported. During the past decade, there has been a sharp rise in the reported incidence of this abnormality which may be attributed to the great advances in early diagnosis. There has been an increasing number of reports describing successful repairs of ruptured congenital aneurysms of the sinus of Valsalva, using various technices and approaches.2J The most recent and successful procedures have been performed with extracorporeal circulation, using an approach through the right atrium and repairing the defect anatomically with either a prosthesis or a suture of aortic wall to the aortic valvular ring. Morrow et a1.4 effected a cure in 1 case by fitting a mushroom-shaped prosthesis into the aneurysm and securing it to the right atria1 wall by neans of a pursestring suture to the base of the prosthesis. Lillehei et al.5 described the operative procedure performed on 3 patients, 2 of whom had rupture of the aneurysm into the right atrium, and the third into the right venAll three defects were closed by aptricle. proximation of the edges of the firm, fibrous ring at the base of the aneurysm with several interrupted stitches. It is now a matter of paramount importance not only that this lesion be considered in the differential diagnosis, but also that the definitive diagnosis be established with minimal delay. The sinus usually penetrates into the right atrium or right ventricle,6-g occasionally into Section
of the Department
and
PAUL Nov.4~6,
hf.D.
Pennsylvania
I
* From the Cardiovascular Philadelphia, Pa.
Aneurysm*
the pulmonary artery,“’ and rarely- into the left ventricle,il left atriumI or free pericardial cavity.i3 Rupture is heralded by a sudden onset of chest pain and dyspnea accompanied by the appearance of a loud, continuous murmur of the “machinery” type and by the peripheral signs of aortic and tricuspid regurgitation. Such patients commonly survive for weeks or months with evidence of congestive heart failure. This report describes a case clinically characteristic of ruptured aneurysm of the sinus of Valsalva, confirmed by right heart catheterization and retrograde aorto,graphy, which was successfully repaired by using bypass circulation. Interesting intraand extracardiac phonocardiographic recordings of the murmur are reported. CASE REPORT A 39 year old white male magazine
salesman was in good health until July 1961, when he had a bilateral inguinal herniorrhaphy. In September 1961, his left leg became swollen; this was followed by swelling of the scrotum and penis, and a severe abdominal distention. He was increasingly troubled by fatigue and progressive exertional dyspnea in October 1961. He denied recent infection, fever or chills and had suffered no injury to his chest. He was admitted to the Hahnemann Medical College and Hospital on January 14, 1962, with orthopnocturnal dyspnea, peripheral nea, paroxysmal edema, abdominal distention and pulsation of the neck veins. On examination, his blood pressure was 130/40 mm. His carotid and brachial pulses were bounding Hg. in quality. The neck veins lvere greatly distended in the sitting position and both giant a and u waves The right were noted; the D waves were dominant. ventricle was palpable and markedly hyperkinetic. ‘l‘here was a slight left ventricular lift at the apex and of Medicine,
544
Hahnemann
TIII!
Medical
AMERICAN
College and Hospital,
JOURSAL
OF
CARDIOLOGY
Rupture
of Sinus of Valsalva
545
Aneurysm
FIG. 1. Phonocardiogram taken from the pulmonary area (PA) and aortic area (AA), There is a continuous murmur at the showing a normal first and second heart sound. aortic
area.
The
indirect
carotid
pulse tracing
no thrills were palpable. A loud. grade 4 (grading 1-6) continuous murmur was best heard at the A grade 3 third and fourth right parasternal areas. high-pitched, blowing pansystolic murmur, increasing in intensity during inspiration, was noted in the Pulmonary valve area of the fifth left interspace. closure at the pulmonary area was not accentuated. The abdomen was slightly distended with fluid; the liver edge was palpable four fingerbreadths below Peripheral the right costal margin and not tender. edema was present bilaterally. ,4 phonocardiogram sho\ved a normal first and second heart sound, a continuous murmur at the right parasternal area, as well as in the aortic area (Fig. I), and an apical pans)-stolic murmur (Fig. 2). The indirect carotid pulse tracing showed a rapid upstroke consistent with an increased pulse pressure. The electrocardiogram demonstrated prominent P waves, suggestive of biatrial hypertrophy. The P-R There was right interval measured 0.16 sec. The S-T segment was slightly deaxis deviation. pressed and T wave inversion was present in leads Increased voltage was noted in the a\;F, Vg and l-6. precordial leads, suggesting right and left ventricular hypertrophy as well. OCTOBER
1963
(CAR)
shows
a rapid
upstroke.
7‘hr chest roent~yenogram revealed enlargement of both ventricles and atria, and the pulmonary vascular shadows were exaggerated throughout both lung fields. On fluoroscopy, the border of the right atrium and hilar areas showed increased pulsation. TABLE
Heart
Right
Location Superior
I
Catheterization 02 Content (vol. c’ /o1
Data
O? Saturation
(5) _~~___.._~~_~~~ ---.
vcna cava
Pressure
(mm. fk) ~-. -~ -...
11.5 (11))
Right atrium
17.6
Right ventricle
18.1
47:‘16
Pulmonary
18.3
47j17
Brachial Coronary
artrry artery sinus
Pulmonary capillary
venous (wedge)
Pulmonary
blood
Systemic
blood
flow flow
20.2
39
2.5
12
110147
(15) 11 .6 L./min. 2.2
L./min.
(30) (68)
546
Segal, Likoff and Novack
FIG. 2. Phonocardzogram taken from the pulmonary area (PA) and mitral arca (MA), showing a pansystolic murmur at the apex.
FIG. 3. Dye-diMon cwve consistent with the presence of a left to right cardiac shunt. Cardio-green was injected in the right peripheral vein and sampling performed from the brachial artery. Right heart cathe erization was performed (Table I); the oxygen content of blood samples obtained from the right atrium was greatly increased. The pressures in the right heart chambers were elevated. A dye dilution curve was also consistent with the presence of a left to right intracardiac shunt (Fig. 3). The flows were estimated as follows: pulmonary blood flow, 11.6 L./min.; systemic blood flow, 2.2 L./min.; and left to right shunt, 9.4 L./min.
Intracardiac phonocardiography was performed with the Allard-Laurens micromanometer and phonocatheter. A continuous murmur was heard and demonstrated at the bifurcation of the main pulmonary artery. The louder diastolic component of this murmur was initiated with the second heart sound and continued almost throughout diastole (Fig. 4A). When the catheter was withdrawn to the pulmonary valve, the systolic component of this murmur became THE
AMERICAN
,JOURNAL
OF CARDIOLOGY
Rupture
547
of Sinus of Lralsalva Aneurysm
B
A
A, there is a loud, contmuous murmur at the bifurcation of the pulmonary artery Intracardiac phonocardiogram. B, withdrawal of the catheter to the region just above the pulmonary valve (PA), with a loud diastolic component. (PA) shows a louder systolic component. FIG.
4.
A
B
Intracardiac phonocardiogran~. A, the phonocatheter was withdrawn’ from the right ventricle (RV) into the right atrium (RA), showing the murmur in the right ventricle (A) which is probably related to increased flow from the large left to right shunt; B, the murmur is absent in the right atrium. FIG.
5.
louder and the diastolic component softer (Fig. 4B). The systolic murmur was also demonstrated in the right ventricle but disappeared in the right atrium (Fig. 5). The phonocatheter was advanced up the right brachial artery and positioned in the region above the aortic valves; here a short ejection systolic murmur was demonstrated. When the catheter was advanced through the fistulous communication, a OCTOBER
1963
loud continuous murmur was heard and well defined. This murmur disappeared when the catheter was advanced into the low right atrium. The continuous murmur began abruptly in the region of the fistula and disappeared on withdrawal of the catheter into the aorta. Right-sided angiography was accomplished by injection of radiopaque material into the right atrium.
Segal,
Likoff and Novack
FE. 7. -4, the left ventriculogram, showing enlargement outflow tract. Thrre is simultaneous opacification of the lrft to right shunt. B, the catheter is advanced from the into the right atrium. There is enlargement of the right trry. The fiitulous communication is in close anatomical monary
of the lpft ventricular cavity, with a normal pulmonary artery and aorta by virtue of a aorta through the fistulous communication atrium, right vrntriclr and pulmonary arproximity with thr bifurcation of the pul-
artery.
right ventricle and pulmonary The right atrium. there was no evidence of a artel y were enlarged; to kfl shunt. A left-sided angiogram was right Nrrnrd with the catheter tip above the aortic pd0 revealed excrllent opacification valve ‘_ The study The right aorta, with no dilatation. of tht 1 ascending sinus was slightly dilated, Arising poste rior aortic was an obvious fistula. ‘The stream of l‘l.0111 this sinus issued from this fkula and preradic kpaqur material
srnted as a bulbous enlargement which was acts tally an aneurysmal extension of the aortic sinus. ’This measured about the size of a small marble, 1.4 cm. From this bulb-like struct ure, in diameter (Fig. 6). a stream of radiopaque material issued into the right atrium to rapidly opacify the right atrium, and It was estim, ated partially opacify the right ventricle. that about 30 per cent of the total volume of opa lque material in.jertrd into the ascending aorta pa .ssed THE
AMERICAN
JOURNAL
OF
CARDIOLOGY
Kuptur
e of Sirius
of \-alsalva
AAi~eurysrtl
FIG. 8. Portofie~nficr phonocnrdioqrrzm taken from the pulmonary iMA showine a normal first heart sound and normal splitting ~l‘he indirect ;.‘arotid pulse tracing is normal through the aortic sinus fistula into the right atrium. The catheter was then manipulated across the aortic valve and positioned rather deeply in the left ventricle. The study revealed c’xcc.llent oparifiration of the somewhat enlarged left ventricle with a normal out‘The ascending aorta and the pulmonary flow tract. arteries became opacified by virtue of the left to right shunt (Fig. 7A). When the catheter was manipulated into the right arrium through the fistula, injection of radiopaque material showed an enlarged right atrium and ventricle (Fig. 7B). In summary, angiography revealed a fistulous communication between the right posterior aortic sinus and right atrium. l’he appearanc !’ suggested a small ancurysma1 fistula, with right atrial and ventricular enlargement and some left ventricular enlargement. Surgical Treatment and R~su1t.r: ‘l‘he patient was prepared for surgery, and a simple excision of the defect was carried out through the right atria1 approach. The postoperatiue cour.v~ remained completely uneventful except for episodes of paroxysmal atria1 tachycardia which \vere converted to normal sinus rhythm with left carotid sinus pressure. By the end of the second week it was noted that the giant (I and prominent u waves in the neck were disappearing. The right ventricle was less hyperkinetic. The continuous murmur disappeared immediately. The first heart sound remained normal and the second heart sound split normally at the pulmonic area, with no accentuation of pulmonary valve closure. OCTOBER1963
arra (PA) and mitral arca of the second heart sound.
‘I’he pansystolic murmur at the tricuspid area \vas then absent. The liver decreased in size; the ascitrs disappeared and the scrotal and peripheral edema improved. 7%8 fhnocu~dir~,gu~n showed a normal first heart sound. composed of mitral and tricuspid components, normal splitting of the second heart sound and no murmurs (Fig. 8). ‘rhe electrocardiogram showed a return to more normal axis. with nonspecific S’r-‘T wave changes, consistent with digitalis effect. The Suoroscopic examination showed considerable decrease in the cardiac size with no abnormal pulsation of the hilum or right atrium. ‘4 i)oxtopPrativ left-sided angiopmm u as essentially normal. ‘I’he aortic valve cusps appeared to move in a normal fashion. with no reflux of radiopaque material into the left ventricle (Fig. 9). ‘I-he fistulous communication between the right posterior aortic sinus and right atrium was no longer seen. The operative repair had effectiveI>. closed the fistula The pa\+?th no distortion of the root of the aorta. tient was seen six months later; hc- remained completely asymptomatic. DISCUSSION
Communications between the aorta and rightsided heart chambers constitute a great threat to cardiac function. Life expectancy depends In 1955, upon the size and site of the shunt. Oram and East8 found only 23 published cases
Segal,
FIG. 9. Postoperativeaortogramdemonstrates
Likoff and Novack
normal
of perforation of aortic sinus aneurysm into the right atrium, including two of their own. These patients lived from seven days to 17 years (usually weeks to months) after onset of symptoms or signs. The etiology of the aneurysm was considered to be congenital in 14 patients, caused by bacterial endocarditis in 4, by in 2. ,411 syphilis in 3, and was uncertain patients were 20 years of age or older and even congenital aneurysms apparently did not rupture until early adult life. Rupture into the pulmonary artery simulates an acute form of patent ductus arteriosus. Because the lungs and left heart are immediately involved, severe symptoms result and lead to death within days or weeks. The outlook is better when the Estula ruptures more proximally between the aorta and the right atrium or superior vena cava . The latter two structures dilate and thus partially dissipate the kinetic energy and volume effect of an influx of arterialized blood under high pressure, relieving the strain on the pulmonary circuit and left heart.‘? Systemic venous engorgement results from the elevated venous pressure and the neck veins are distended. A prominent v wave suggests dilatation of the tricuspid valve ring with tricuspid
coronary
sinuses with no fistulous
communication.
regurgitation. The hyperkinetic right ventricle is the result of the large left to right shunt with increased flow in the right heart and pulmonary circuit. The one constant feature of the murmur following rupture is its presence in both systole It is usually continuous, but not and diastole. infrequently is composed of separate systolic and diastolic components, resembling a toand-fro murmur. It is of moderate to great intensity, with the maximal intensity occurring either in both phases or in systole. Rarely, the murmur is of maximal intensity during The quality of the murmur is generdiastole. ally harsh and coarse, and a “machinery” character is often imparted when it is conThis is due to the constant difference tinuous. in pressure between the systemic and venous sides of the perforation. The aortic diastolic reflux may also produce a water-hammer pulse.i6 The maximal site of the murmur is usually between the third and fourth ribs and the xiphoid area, located over or adjacent to either side of the sternum. There is widespread transmission of the murmur, and occasionally and diastolic components the systolic are transmitted differentially.16 THE
AMERICAN
JOURNAL
OF
CARDIOLOGY
Rupture
of Sinus
The site of maximal intensity of the murmur and the transmission of the diastolic component may provide clues to the location of the shunt. The maximal intensity is often higher in the second and third intercostal space, particularly when the left to right shunt enters the outflow tract of the right ventricle. When the shunt enters the inflow tract or the right atrium, the maximal intensity is usually located lower on the chest wall. The murmur in our case was continuous, with the site of maximal intensity The in the third to fifth right parasternal area. systolic and diastolic components were equally well heard on the chest wall (Fig. l), and the expected differential transmission of the diastolic component to the right fourth and fifth parasternal areas was not found in our patient. Left-sided intracardiac phonocardiography showed the loud continuous murmur to be well localized in the fistulous communication. A soft, short ejection systolic murmur in the aorta was replaced by the continuous murmur when the phonocatheter was advanced into the fistula and disappeared as the catheter entered the right atrium. It would appear that the continuous murmur, if loud enough, can be transmitted directly to adjoining vessels. Rightsided intracardiac phonocardiograms (Fig. 4) showed this murmur in the bifurcation of the pulmonary artery. The fistulous communication was in close proximity with this area (Fig. 7B). The loud systolic murmur in the main pulmonary artery above the valve and in the right ventricle was related to increased flow from the large left to right shunt. Again, no murmur was noted in the right atrium. ‘The diagnosis of rupture is confirmed through specific information gained by right heart catheterization, retrograde aortography and In the case selective dye-dilution methods. presented, the aneurysm ruptured from the right posterior aortic sinus into the right atrium. In a review of 47 cases by Sawyers et al., 14 (30 per cent) were in the noncoronary sinus The and all ruptured into the right atrium.” remaining 33 (70 per cent) were in the right coronary sinus ; only 3 of these ruptured into the right atrium, while 19 ruptured into the right ventricle and one each into the left ventricle, pulmonary artery and pericardium. In another review of 23 cases, 13 aneurysms were in the right coronary sinus ; 4 of these ruptured into the right atrium, 7 into the right ventricle, 1 into both right atrium and ventricle, and 1 into both ventricles.8 OCTOBER
1963
of Valsalva
551
Aneurysm
The basic etiology of the congenital type of aneurysm has been well demonstrated by Burchell and Edwards as a defect between the aortic root and the heart, manifested as a failure of union of the elastic media of the aortic wall above and the annulus fibrosis of the aortic valve ring below.18~*g This same type of defect may occur below the valve ring, giving rise to a ventricular septal defect which is a frequently associated anomaly. Other cases have been reported with coarctation of the aorta9 JO fzl and stenosis of the pulmonary conus The high pressure in the aorta produces an aneurysmal sac from the weakened section of the aortic wall, with rupture into the low pressure chamber of the right heart. Our patient showed the locus of protrusion from the right coronary sinus, bulging into the right atrium with rupture. With the advent of corrective surgical technics, the recognition of aortico-right atria1 communication has assumed a new significance. The simple and logical approach for repair of this defect is to bridge the defect either by direct suture or by prosthesis via the right atrium or right ventricle, depending upon the location of the rupture.22 Morrow et a1.4 reported the successful use of a prosthetic tampon to occlude the fistulous tract under hypothermia, and McGoon, Edwards, and Kirklin3 have also reported the use of a polyvinyl sponge prosthesis in the successful repair of a ruptured aneurysm of the sinus of Valsalva occurring in combination with a ventricular septal defect, using bypass circulation. Direct suture, using bypass circulation and superficial hypothermia, were employed in the successful repair of the defect in our patient. SUMMARY
Rupture of an aneurysm of the sinus of Valsalva into the right atrium followed by successful surgical repair is described. The clinical aspects of the case are emphasized, and the and angiocardiographic phonocardiographic findings are presented. !SKNOWLEDGMENT The authors are grateful to Dr. Henry Nichols, who performed the surgical procedure, and Dr. J. Stauffer Lehman, who was responsible for the angiographic procedures. REFERENCES 1. HOPE, J. Diseases of the Heart and Great Vessels, ed. 3. London, 1839. John Churchill.
552
&gal,
Likoff
2. BIGELOW, W. G. and BARNES, W. T. Ruptured aneurysm of aortic sinus. Ann. Surg., 150: 117, 1959. 3. MCGOON, D. C., EDWARDS,J. E. and KIRKLIN, J. W. Surgical treatment of ruptured aneurysm of aortic sinus. Ann. S’urg., 147: 387, 1958. 4. MORROW, A. G., BAKER, R. R., HANSON,H. F. and MATTINGLY, T. W. Successful surgical repair of ruptured aneurysm of the sinus of Valsalva. Circulation, 16: 533, 1957. 5. LILLEHEI, C. W., STANLEY, P. and VARCO, R. L. Surgical treatment of ruptured aneurysms of the sinus of Valsalva. Ann. Surg., 146: 459, 1957. 6. JONES, .4. M. and LANGLEY, F. A. Aortic sinus aneurysms. Brit. Heart J., 11: 325, 1949. 7. VENNING, G. R. Aneurysms of sinuses of Valsalva. Am. Heart J., 42: 57, 1951. 8. ORAM, S. and EAST, T. Rupture of aneurysms of aortic sinus into the right side of the heart. Brit. Heart J., 17: 541, 1955. 9. HALL, B. and PIC~ARD, S. D. Unsuspected rupture of aortic sinus aneurysm into the right atrium. Am. J. Cardiol., 3: 404, 1959. 10. BROWN, J. W., HEATH, D. and WHITTAKER, W. Cardio-aortic fistula. Circulation, 12 : 819, 1955. 11. WARTHEN, R. Congenital aneurysm of right anterior sinus of Valsalva with rupture into the left ventricle. Am. Heart J., 37: 975, 1949. 12. THURNAM, J. Spontaneous varicose aneurysms of the ascending aorta and sinuses of Valsalva. TY. Royal Med. Chir. Sac. Glasgow, 23 : 323, 1840. 13. WOOD, P. Diseases of the Heart and Circulation, p. 925. Philadelphia, 1956. J. B. Lippincott Co.
and Novack 14. SWANEPOEL,A4., SCHRIRE, V.. ~YELLEN, M., VOGELPOEL, 1,. and BARNARD, C. Traumatic aorticoright atria1 fistula. Report of a case corrected by operation. Am. Heart J.. 61 : 120. 1961. 15. PERLObF, J. Sinus of Valsalva-right heart communications due to congenital aortic sinus defects. Am. Heart J., 59: 318, 1960. 16. EVANS, J. W., HARRIS, 7‘. R. and BRODY, D. .\. Ruptured aortic sinus aneurysm. Case report with review of clinical features. Am. Heart J., 61 : 408, 1961. 17. SAWYERS, J. L., ADAMS, J. E. and SCOTT, H. W., .JR. A method of surgical repair for ruptured aortic sinus aneurysms with aortic-atria1 fistula. Southwz M. J., 50: 1075, 1957. 18. EDWARDS, J. E. and BURCI~ELL,H. B. Specimen exhibiting the essential lesion in aneurysm of the aortic sinus. Proc. Staf .Weet. AMayo Clin., 31: 407, 1956. 19. EDWARDS, J. E. and BURCHELI..H. B. The pathological anatomy of deficiencies between the aortic root and the heart, including aortic sinus aneurysms. Thorax, 12: 125, 1957. 20. DUBILIER, W., TAYLOR, T. and STEINBERG, I. Aortic sinus aneurysm associated with coarctation of the aorta. Am. J. Roentgenol., 73: 10, 1955. 21. STEINBERG,I. and FINBY, N. Congenital aneurysm of right aortic sinus associated with coarctation of the aorta. New England J. Med., 253: 549, 1955. 22. LIPPSCHUTZ,E. J. and WOOD, L. W. Rupture of an aneurysm of the sinus of Valsalva. ilm. J. Med., 28 : 859, 1960.
THE AMERICANJOURNAL OF CARDIOLOGY
of a Sinus of Valsalva
BERNARD L. SEGAL, bin.,
b’lLLIAhf
LIKOFF, M.D., F.A.C.C.
Philadelphia,
N 1839 Hope described a “case of aneurysmal pouch of the aorta, bursting into the right ventricle,” the first published account of a ruptured congenital aortic sinus aneurysm.’ In the following one hundred years, 18 similar cases were reported. During the past decade, there has been a sharp rise in the reported incidence of this abnormality which may be attributed to the great advances in early diagnosis. There has been an increasing number of reports describing successful repairs of ruptured congenital aneurysms of the sinus of Valsalva, using various technices and approaches.2J The most recent and successful procedures have been performed with extracorporeal circulation, using an approach through the right atrium and repairing the defect anatomically with either a prosthesis or a suture of aortic wall to the aortic valvular ring. Morrow et a1.4 effected a cure in 1 case by fitting a mushroom-shaped prosthesis into the aneurysm and securing it to the right atria1 wall by neans of a pursestring suture to the base of the prosthesis. Lillehei et al.5 described the operative procedure performed on 3 patients, 2 of whom had rupture of the aneurysm into the right atrium, and the third into the right venAll three defects were closed by aptricle. proximation of the edges of the firm, fibrous ring at the base of the aneurysm with several interrupted stitches. It is now a matter of paramount importance not only that this lesion be considered in the differential diagnosis, but also that the definitive diagnosis be established with minimal delay. The sinus usually penetrates into the right atrium or right ventricle,6-g occasionally into Section
of the Department
and
PAUL Nov.4~6,
hf.D.
Pennsylvania
I
* From the Cardiovascular Philadelphia, Pa.
Aneurysm*
the pulmonary artery,“’ and rarely- into the left ventricle,il left atriumI or free pericardial cavity.i3 Rupture is heralded by a sudden onset of chest pain and dyspnea accompanied by the appearance of a loud, continuous murmur of the “machinery” type and by the peripheral signs of aortic and tricuspid regurgitation. Such patients commonly survive for weeks or months with evidence of congestive heart failure. This report describes a case clinically characteristic of ruptured aneurysm of the sinus of Valsalva, confirmed by right heart catheterization and retrograde aorto,graphy, which was successfully repaired by using bypass circulation. Interesting intraand extracardiac phonocardiographic recordings of the murmur are reported. CASE REPORT A 39 year old white male magazine
salesman was in good health until July 1961, when he had a bilateral inguinal herniorrhaphy. In September 1961, his left leg became swollen; this was followed by swelling of the scrotum and penis, and a severe abdominal distention. He was increasingly troubled by fatigue and progressive exertional dyspnea in October 1961. He denied recent infection, fever or chills and had suffered no injury to his chest. He was admitted to the Hahnemann Medical College and Hospital on January 14, 1962, with orthopnocturnal dyspnea, peripheral nea, paroxysmal edema, abdominal distention and pulsation of the neck veins. On examination, his blood pressure was 130/40 mm. His carotid and brachial pulses were bounding Hg. in quality. The neck veins lvere greatly distended in the sitting position and both giant a and u waves The right were noted; the D waves were dominant. ventricle was palpable and markedly hyperkinetic. ‘l‘here was a slight left ventricular lift at the apex and of Medicine,
544
Hahnemann
TIII!
Medical
AMERICAN
College and Hospital,
JOURSAL
OF
CARDIOLOGY
Rupture
of Sinus of Valsalva
545
Aneurysm
FIG. 1. Phonocardiogram taken from the pulmonary area (PA) and aortic area (AA), There is a continuous murmur at the showing a normal first and second heart sound. aortic
area.
The
indirect
carotid
pulse tracing
no thrills were palpable. A loud. grade 4 (grading 1-6) continuous murmur was best heard at the A grade 3 third and fourth right parasternal areas. high-pitched, blowing pansystolic murmur, increasing in intensity during inspiration, was noted in the Pulmonary valve area of the fifth left interspace. closure at the pulmonary area was not accentuated. The abdomen was slightly distended with fluid; the liver edge was palpable four fingerbreadths below Peripheral the right costal margin and not tender. edema was present bilaterally. ,4 phonocardiogram sho\ved a normal first and second heart sound, a continuous murmur at the right parasternal area, as well as in the aortic area (Fig. I), and an apical pans)-stolic murmur (Fig. 2). The indirect carotid pulse tracing showed a rapid upstroke consistent with an increased pulse pressure. The electrocardiogram demonstrated prominent P waves, suggestive of biatrial hypertrophy. The P-R There was right interval measured 0.16 sec. The S-T segment was slightly deaxis deviation. pressed and T wave inversion was present in leads Increased voltage was noted in the a\;F, Vg and l-6. precordial leads, suggesting right and left ventricular hypertrophy as well. OCTOBER
1963
(CAR)
shows
a rapid
upstroke.
7‘hr chest roent~yenogram revealed enlargement of both ventricles and atria, and the pulmonary vascular shadows were exaggerated throughout both lung fields. On fluoroscopy, the border of the right atrium and hilar areas showed increased pulsation. TABLE
Heart
Right
Location Superior
I
Catheterization 02 Content (vol. c’ /o1
Data
O? Saturation
(5) _~~___.._~~_~~~ ---.
vcna cava
Pressure
(mm. fk) ~-. -~ -...
11.5 (11))
Right atrium
17.6
Right ventricle
18.1
47:‘16
Pulmonary
18.3
47j17
Brachial Coronary
artrry artery sinus
Pulmonary capillary
venous (wedge)
Pulmonary
blood
Systemic
blood
flow flow
20.2
39
2.5
12
110147
(15) 11 .6 L./min. 2.2
L./min.
(30) (68)
546
Segal, Likoff and Novack
FIG. 2. Phonocardzogram taken from the pulmonary area (PA) and mitral arca (MA), showing a pansystolic murmur at the apex.
FIG. 3. Dye-diMon cwve consistent with the presence of a left to right cardiac shunt. Cardio-green was injected in the right peripheral vein and sampling performed from the brachial artery. Right heart cathe erization was performed (Table I); the oxygen content of blood samples obtained from the right atrium was greatly increased. The pressures in the right heart chambers were elevated. A dye dilution curve was also consistent with the presence of a left to right intracardiac shunt (Fig. 3). The flows were estimated as follows: pulmonary blood flow, 11.6 L./min.; systemic blood flow, 2.2 L./min.; and left to right shunt, 9.4 L./min.
Intracardiac phonocardiography was performed with the Allard-Laurens micromanometer and phonocatheter. A continuous murmur was heard and demonstrated at the bifurcation of the main pulmonary artery. The louder diastolic component of this murmur was initiated with the second heart sound and continued almost throughout diastole (Fig. 4A). When the catheter was withdrawn to the pulmonary valve, the systolic component of this murmur became THE
AMERICAN
,JOURNAL
OF CARDIOLOGY
Rupture
547
of Sinus of Lralsalva Aneurysm
B
A
A, there is a loud, contmuous murmur at the bifurcation of the pulmonary artery Intracardiac phonocardiogram. B, withdrawal of the catheter to the region just above the pulmonary valve (PA), with a loud diastolic component. (PA) shows a louder systolic component. FIG.
4.
A
B
Intracardiac phonocardiogran~. A, the phonocatheter was withdrawn’ from the right ventricle (RV) into the right atrium (RA), showing the murmur in the right ventricle (A) which is probably related to increased flow from the large left to right shunt; B, the murmur is absent in the right atrium. FIG.
5.
louder and the diastolic component softer (Fig. 4B). The systolic murmur was also demonstrated in the right ventricle but disappeared in the right atrium (Fig. 5). The phonocatheter was advanced up the right brachial artery and positioned in the region above the aortic valves; here a short ejection systolic murmur was demonstrated. When the catheter was advanced through the fistulous communication, a OCTOBER
1963
loud continuous murmur was heard and well defined. This murmur disappeared when the catheter was advanced into the low right atrium. The continuous murmur began abruptly in the region of the fistula and disappeared on withdrawal of the catheter into the aorta. Right-sided angiography was accomplished by injection of radiopaque material into the right atrium.
Segal,
Likoff and Novack
FE. 7. -4, the left ventriculogram, showing enlargement outflow tract. Thrre is simultaneous opacification of the lrft to right shunt. B, the catheter is advanced from the into the right atrium. There is enlargement of the right trry. The fiitulous communication is in close anatomical monary
of the lpft ventricular cavity, with a normal pulmonary artery and aorta by virtue of a aorta through the fistulous communication atrium, right vrntriclr and pulmonary arproximity with thr bifurcation of the pul-
artery.
right ventricle and pulmonary The right atrium. there was no evidence of a artel y were enlarged; to kfl shunt. A left-sided angiogram was right Nrrnrd with the catheter tip above the aortic pd0 revealed excrllent opacification valve ‘_ The study The right aorta, with no dilatation. of tht 1 ascending sinus was slightly dilated, Arising poste rior aortic was an obvious fistula. ‘The stream of l‘l.0111 this sinus issued from this fkula and preradic kpaqur material
srnted as a bulbous enlargement which was acts tally an aneurysmal extension of the aortic sinus. ’This measured about the size of a small marble, 1.4 cm. From this bulb-like struct ure, in diameter (Fig. 6). a stream of radiopaque material issued into the right atrium to rapidly opacify the right atrium, and It was estim, ated partially opacify the right ventricle. that about 30 per cent of the total volume of opa lque material in.jertrd into the ascending aorta pa .ssed THE
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Kuptur
e of Sirius
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AAi~eurysrtl
FIG. 8. Portofie~nficr phonocnrdioqrrzm taken from the pulmonary iMA showine a normal first heart sound and normal splitting ~l‘he indirect ;.‘arotid pulse tracing is normal through the aortic sinus fistula into the right atrium. The catheter was then manipulated across the aortic valve and positioned rather deeply in the left ventricle. The study revealed c’xcc.llent oparifiration of the somewhat enlarged left ventricle with a normal out‘The ascending aorta and the pulmonary flow tract. arteries became opacified by virtue of the left to right shunt (Fig. 7A). When the catheter was manipulated into the right arrium through the fistula, injection of radiopaque material showed an enlarged right atrium and ventricle (Fig. 7B). In summary, angiography revealed a fistulous communication between the right posterior aortic sinus and right atrium. l’he appearanc !’ suggested a small ancurysma1 fistula, with right atrial and ventricular enlargement and some left ventricular enlargement. Surgical Treatment and R~su1t.r: ‘l‘he patient was prepared for surgery, and a simple excision of the defect was carried out through the right atria1 approach. The postoperatiue cour.v~ remained completely uneventful except for episodes of paroxysmal atria1 tachycardia which \vere converted to normal sinus rhythm with left carotid sinus pressure. By the end of the second week it was noted that the giant (I and prominent u waves in the neck were disappearing. The right ventricle was less hyperkinetic. The continuous murmur disappeared immediately. The first heart sound remained normal and the second heart sound split normally at the pulmonic area, with no accentuation of pulmonary valve closure. OCTOBER1963
arra (PA) and mitral arca of the second heart sound.
‘I’he pansystolic murmur at the tricuspid area \vas then absent. The liver decreased in size; the ascitrs disappeared and the scrotal and peripheral edema improved. 7%8 fhnocu~dir~,gu~n showed a normal first heart sound. composed of mitral and tricuspid components, normal splitting of the second heart sound and no murmurs (Fig. 8). ‘rhe electrocardiogram showed a return to more normal axis. with nonspecific S’r-‘T wave changes, consistent with digitalis effect. The Suoroscopic examination showed considerable decrease in the cardiac size with no abnormal pulsation of the hilum or right atrium. ‘4 i)oxtopPrativ left-sided angiopmm u as essentially normal. ‘I’he aortic valve cusps appeared to move in a normal fashion. with no reflux of radiopaque material into the left ventricle (Fig. 9). ‘I-he fistulous communication between the right posterior aortic sinus and right atrium was no longer seen. The operative repair had effectiveI>. closed the fistula The pa\+?th no distortion of the root of the aorta. tient was seen six months later; hc- remained completely asymptomatic. DISCUSSION
Communications between the aorta and rightsided heart chambers constitute a great threat to cardiac function. Life expectancy depends In 1955, upon the size and site of the shunt. Oram and East8 found only 23 published cases
Segal,
FIG. 9. Postoperativeaortogramdemonstrates
Likoff and Novack
normal
of perforation of aortic sinus aneurysm into the right atrium, including two of their own. These patients lived from seven days to 17 years (usually weeks to months) after onset of symptoms or signs. The etiology of the aneurysm was considered to be congenital in 14 patients, caused by bacterial endocarditis in 4, by in 2. ,411 syphilis in 3, and was uncertain patients were 20 years of age or older and even congenital aneurysms apparently did not rupture until early adult life. Rupture into the pulmonary artery simulates an acute form of patent ductus arteriosus. Because the lungs and left heart are immediately involved, severe symptoms result and lead to death within days or weeks. The outlook is better when the Estula ruptures more proximally between the aorta and the right atrium or superior vena cava . The latter two structures dilate and thus partially dissipate the kinetic energy and volume effect of an influx of arterialized blood under high pressure, relieving the strain on the pulmonary circuit and left heart.‘? Systemic venous engorgement results from the elevated venous pressure and the neck veins are distended. A prominent v wave suggests dilatation of the tricuspid valve ring with tricuspid
coronary
sinuses with no fistulous
communication.
regurgitation. The hyperkinetic right ventricle is the result of the large left to right shunt with increased flow in the right heart and pulmonary circuit. The one constant feature of the murmur following rupture is its presence in both systole It is usually continuous, but not and diastole. infrequently is composed of separate systolic and diastolic components, resembling a toand-fro murmur. It is of moderate to great intensity, with the maximal intensity occurring either in both phases or in systole. Rarely, the murmur is of maximal intensity during The quality of the murmur is generdiastole. ally harsh and coarse, and a “machinery” character is often imparted when it is conThis is due to the constant difference tinuous. in pressure between the systemic and venous sides of the perforation. The aortic diastolic reflux may also produce a water-hammer pulse.i6 The maximal site of the murmur is usually between the third and fourth ribs and the xiphoid area, located over or adjacent to either side of the sternum. There is widespread transmission of the murmur, and occasionally and diastolic components the systolic are transmitted differentially.16 THE
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Rupture
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The site of maximal intensity of the murmur and the transmission of the diastolic component may provide clues to the location of the shunt. The maximal intensity is often higher in the second and third intercostal space, particularly when the left to right shunt enters the outflow tract of the right ventricle. When the shunt enters the inflow tract or the right atrium, the maximal intensity is usually located lower on the chest wall. The murmur in our case was continuous, with the site of maximal intensity The in the third to fifth right parasternal area. systolic and diastolic components were equally well heard on the chest wall (Fig. l), and the expected differential transmission of the diastolic component to the right fourth and fifth parasternal areas was not found in our patient. Left-sided intracardiac phonocardiography showed the loud continuous murmur to be well localized in the fistulous communication. A soft, short ejection systolic murmur in the aorta was replaced by the continuous murmur when the phonocatheter was advanced into the fistula and disappeared as the catheter entered the right atrium. It would appear that the continuous murmur, if loud enough, can be transmitted directly to adjoining vessels. Rightsided intracardiac phonocardiograms (Fig. 4) showed this murmur in the bifurcation of the pulmonary artery. The fistulous communication was in close proximity with this area (Fig. 7B). The loud systolic murmur in the main pulmonary artery above the valve and in the right ventricle was related to increased flow from the large left to right shunt. Again, no murmur was noted in the right atrium. ‘The diagnosis of rupture is confirmed through specific information gained by right heart catheterization, retrograde aortography and In the case selective dye-dilution methods. presented, the aneurysm ruptured from the right posterior aortic sinus into the right atrium. In a review of 47 cases by Sawyers et al., 14 (30 per cent) were in the noncoronary sinus The and all ruptured into the right atrium.” remaining 33 (70 per cent) were in the right coronary sinus ; only 3 of these ruptured into the right atrium, while 19 ruptured into the right ventricle and one each into the left ventricle, pulmonary artery and pericardium. In another review of 23 cases, 13 aneurysms were in the right coronary sinus ; 4 of these ruptured into the right atrium, 7 into the right ventricle, 1 into both right atrium and ventricle, and 1 into both ventricles.8 OCTOBER
1963
of Valsalva
551
Aneurysm
The basic etiology of the congenital type of aneurysm has been well demonstrated by Burchell and Edwards as a defect between the aortic root and the heart, manifested as a failure of union of the elastic media of the aortic wall above and the annulus fibrosis of the aortic valve ring below.18~*g This same type of defect may occur below the valve ring, giving rise to a ventricular septal defect which is a frequently associated anomaly. Other cases have been reported with coarctation of the aorta9 JO fzl and stenosis of the pulmonary conus The high pressure in the aorta produces an aneurysmal sac from the weakened section of the aortic wall, with rupture into the low pressure chamber of the right heart. Our patient showed the locus of protrusion from the right coronary sinus, bulging into the right atrium with rupture. With the advent of corrective surgical technics, the recognition of aortico-right atria1 communication has assumed a new significance. The simple and logical approach for repair of this defect is to bridge the defect either by direct suture or by prosthesis via the right atrium or right ventricle, depending upon the location of the rupture.22 Morrow et a1.4 reported the successful use of a prosthetic tampon to occlude the fistulous tract under hypothermia, and McGoon, Edwards, and Kirklin3 have also reported the use of a polyvinyl sponge prosthesis in the successful repair of a ruptured aneurysm of the sinus of Valsalva occurring in combination with a ventricular septal defect, using bypass circulation. Direct suture, using bypass circulation and superficial hypothermia, were employed in the successful repair of the defect in our patient. SUMMARY
Rupture of an aneurysm of the sinus of Valsalva into the right atrium followed by successful surgical repair is described. The clinical aspects of the case are emphasized, and the and angiocardiographic phonocardiographic findings are presented. !SKNOWLEDGMENT The authors are grateful to Dr. Henry Nichols, who performed the surgical procedure, and Dr. J. Stauffer Lehman, who was responsible for the angiographic procedures. REFERENCES 1. HOPE, J. Diseases of the Heart and Great Vessels, ed. 3. London, 1839. John Churchill.
552
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2. BIGELOW, W. G. and BARNES, W. T. Ruptured aneurysm of aortic sinus. Ann. Surg., 150: 117, 1959. 3. MCGOON, D. C., EDWARDS,J. E. and KIRKLIN, J. W. Surgical treatment of ruptured aneurysm of aortic sinus. Ann. S’urg., 147: 387, 1958. 4. MORROW, A. G., BAKER, R. R., HANSON,H. F. and MATTINGLY, T. W. Successful surgical repair of ruptured aneurysm of the sinus of Valsalva. Circulation, 16: 533, 1957. 5. LILLEHEI, C. W., STANLEY, P. and VARCO, R. L. Surgical treatment of ruptured aneurysms of the sinus of Valsalva. Ann. Surg., 146: 459, 1957. 6. JONES, .4. M. and LANGLEY, F. A. Aortic sinus aneurysms. Brit. Heart J., 11: 325, 1949. 7. VENNING, G. R. Aneurysms of sinuses of Valsalva. Am. Heart J., 42: 57, 1951. 8. ORAM, S. and EAST, T. Rupture of aneurysms of aortic sinus into the right side of the heart. Brit. Heart J., 17: 541, 1955. 9. HALL, B. and PIC~ARD, S. D. Unsuspected rupture of aortic sinus aneurysm into the right atrium. Am. J. Cardiol., 3: 404, 1959. 10. BROWN, J. W., HEATH, D. and WHITTAKER, W. Cardio-aortic fistula. Circulation, 12 : 819, 1955. 11. WARTHEN, R. Congenital aneurysm of right anterior sinus of Valsalva with rupture into the left ventricle. Am. Heart J., 37: 975, 1949. 12. THURNAM, J. Spontaneous varicose aneurysms of the ascending aorta and sinuses of Valsalva. TY. Royal Med. Chir. Sac. Glasgow, 23 : 323, 1840. 13. WOOD, P. Diseases of the Heart and Circulation, p. 925. Philadelphia, 1956. J. B. Lippincott Co.
and Novack 14. SWANEPOEL,A4., SCHRIRE, V.. ~YELLEN, M., VOGELPOEL, 1,. and BARNARD, C. Traumatic aorticoright atria1 fistula. Report of a case corrected by operation. Am. Heart J.. 61 : 120. 1961. 15. PERLObF, J. Sinus of Valsalva-right heart communications due to congenital aortic sinus defects. Am. Heart J., 59: 318, 1960. 16. EVANS, J. W., HARRIS, 7‘. R. and BRODY, D. .\. Ruptured aortic sinus aneurysm. Case report with review of clinical features. Am. Heart J., 61 : 408, 1961. 17. SAWYERS, J. L., ADAMS, J. E. and SCOTT, H. W., .JR. A method of surgical repair for ruptured aortic sinus aneurysms with aortic-atria1 fistula. Southwz M. J., 50: 1075, 1957. 18. EDWARDS, J. E. and BURCI~ELL,H. B. Specimen exhibiting the essential lesion in aneurysm of the aortic sinus. Proc. Staf .Weet. AMayo Clin., 31: 407, 1956. 19. EDWARDS, J. E. and BURCHELI..H. B. The pathological anatomy of deficiencies between the aortic root and the heart, including aortic sinus aneurysms. Thorax, 12: 125, 1957. 20. DUBILIER, W., TAYLOR, T. and STEINBERG, I. Aortic sinus aneurysm associated with coarctation of the aorta. Am. J. Roentgenol., 73: 10, 1955. 21. STEINBERG,I. and FINBY, N. Congenital aneurysm of right aortic sinus associated with coarctation of the aorta. New England J. Med., 253: 549, 1955. 22. LIPPSCHUTZ,E. J. and WOOD, L. W. Rupture of an aneurysm of the sinus of Valsalva. ilm. J. Med., 28 : 859, 1960.
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