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Conditions of the ascending aorta simulating aortic valvular incompetence
Review Conditions of the Ascending Aorta Simulating Aortic Valvular
Incompetence*
ROBERT S. ELIOT, M.D., ROBERT L. WOODBURN, 11.~. and JESSE E. EDWARDS, St.
Paul, Minnesota
SOME patients with signs of aortic valvular incompetence, the fundamental process may lie not in the aortic valve but rather in the ascending aorta. Certain cases in the collection of the cardiovascular registry of the Charles T. Miller Hospital are examples of this phenomenon, which is the subject of this report. For completeness and for reference on the basis for aortic incompetence caused by diseases of the ascending aorta, we will first consider the normal aortic valve and then the intrinsic diseases of the aortic valve which make it incompetent. Following this we will describe those diseases of the ascending aorta that, by allowing abnormal run-off of blood either through the aortic valve or through some other route, cause a functional state comparable to that resulting from intrinsic incompetence of the aortic valve.
I
M.D.
Valsalva) lies between the cusp and the related aortic wall. The upper aspect of the sinus is open and lies at an imaginary horizontal plane corresponding with the upper level of the cusp. The coronary arterial ostia lie just above the level of the aortic sinuses, although it is frequently incorrectly stated that the coronary arteries arise from their respective aortic sinuses.
N
INCOMPETENCE DUE TO INTRINSIC AORTIC VALVE DISEASE The common causes of aortic valvular incompetence are rheumatic endocarditis, bacterial endocarditis and congenital disease.l Uncommonly, trauma resulting from rupture of a cusp may be a basis for aortic incompetence. usually, that which follows trauma is the result of a tear in the ascending aorta rather than in the valve itself. Aortic valvular incompetence of rheumatic origin may either be pure or be associated with varying degrees of stenosis. Classically, when pure valvular incompetence is present, the rheumatic process has spared the commissures, while the cusps show intrinsic change (Fig. 2), characterized by contracture from scarring, causing shortening of the cusps in both length and breadth. Because of these changes the surface area of the cusps is inadequate to guard the valvular orifice. Classically, at the center of the aortic orifice, there is a triangular defect through which blood regurgitates. In addition, rheumatic endocarditis may cause fusion between adjacent cusps at the commissures. Those cusps involved in the process of adhesion
THE NORMAL AORTIC VALVE Each of the three aortic cusps may be looked upon as an independent unit connected to the aortic wall (Fig. 1). The length of the free aspect of each cusp is greater than the straightline distance between its two lateral extremities. The extra length is such that during systole the central point of each cusp extends to the center of the aortic orifice. In this way the three cusps together may close the orifice. The space between a lateral attachment of one cusp and that of the adjacent cusp is referred to as a commissure. In the normal aortic valve, three such functional zones exist. Each of the three aortic sinuses (sinuses of
* From the Department of Pathology, The Charles T. Miller Hospital, St. Paul, Minn., and the Departments of Medicine and Pathology, University of Minnesota, Minneapolis, Minn. This study was supported by Research Grant HE 5694 and Research Training Grant 5 Tl HE 5570 of the National Heart Institute, National Institutes of Health, U. S. Public Health Service. VOLUME
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Eliot,
\~\‘oodl)urrl and
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Nocteriaf
rndoccuditis results in aortic
incompetence
through
upon the tissue.
its
Perforation
destructive
val\ular effects
of one or several
cusps may occur (Fig. 3), or valvular tissue ma) If the latter process is exbe lost by ulceration. tensive, support for the involved cusp or cusps is lost, and prolapse
FIG. 1 Ihnwtny II] m ml NWII<-w/w viewed from ahove. Each of the three aortic cusps is an indcpcndrnt unit connected to its respecti\r segment of the aortir wall. There arc two lateral attachments which are continuous through the inferior attachment of the cusp to the root of the aorta. Between the points of lateral attachment of each cusp is an excessive amount of tissue, which allows the central part of each cusp to extend to the center of The centers of each leaflet are in conthe aortic orifice. tact during closure of the valve. The space between the lateral attachments of two adjacent cusps is referred to as a commissurc.
at the commissures are prevented from full latIf the eral excursion during ventricular systole. change is sufficient, significant valvular stenosis may accompany the insufficiency.
of one or more of the cusps
may occur. As the destructive process takes place during the active stages of bacterial endocarditis, the aortic incompetence may complicate the state of the patient at this time. In some cases the bacterial stage of the disease may be overcome, but the mechanical residua continue to complicate the course of the illness. We have observed examples of ulceration or perforation of aortic cusps in patients with no history of antecedent bacterial endocarditis The lesions resemble those in cases of (Fig. 36). cured” bacterial enknown “bacteriologically For this reason it is our opinion docarditis. that such cases represent cure of bacterial endocarditis that was never diagnosed clinically. Cure may have been spontaneous or, it is more likely, occurred in response to the current procedure of liberal administration of antibiotics. Congenital feflestration of an aortic cusl) is so common an entity that it is usually considered a variant of the normal. The fenestration (or fenestrations) are usually confined to that part of the cusp which lies between the line of closure The below and the free edge of the cusp above.
FIG. 2. Rheumatic aortic incompetence. a, unopened aortic valve viewed from above. The valvular cusps are thickened and short. Two of the commissures are fused. The result of the shortening is 6, diagrammatic representation of rheumatic a triangular defect in the center of the aortic orifice. aortic incompetence demonstrating the thickening and the shortening of the aortic valvular cusps, which accounts for valvular incompetence. THE
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FIG. 3. Bacterial endocarditis. a, perforations (arrows) of aortic cusps resulting from bacterial endocarditis. 6, Perforation (arrow) of the posterior cusp of the aortic valve in the heart of a 35 year old woman. Although no history of bacterial endocarditis had been obtained, the perforation is considered to represent the healed lesion. Jet lesions (circle) secondary to regurgitation through the perforated aortic cusp are present upon the subaortic area of the ventricular septum.
fenestration is, therefore, usually above that part of the cusp that contributes to the closure of the valve. Only rarely is a fenestration large enough to form a basis for valvular incompetence. In a rare case reported from our institutions by Levy and associates,2 idiopathic dilatation of the aorta caused tension upon the cusps. As a result, enlargement of a congenital fenestration in one of the cusps was of such a degree that the cusp appeared to have been incompetent (a second cusp had ruptured as a result of the unusual mechanical tension). Maldevelopment in the number of aortic cusps is more common than fenestration as a congenital cause of intrinsic incompetence of the valve. Particularly significant is the congenital bicuspid aortic valve. Less common is the congenital “dome-shaped” valve. The congenital bicuspid aortic valve has been analyzed on theoretic grounds by one of us (J.E.Es), and it was emphasized that there are reasons for its malfunction. The valve with this anomaly is subject to (1) acquired aortic stenosis, often, with coexisting incompetence present, (2) bacterial endocarditis, sometimes with aortic incompetence as a secondary complication and (3) intrinsic incompetence (Fig. 4). The basis for intrinsic incompetence of the congenital bicuspid aortic valve seems to be that the two cusps are usually of unequal size (Fig. 5). The larger cusp may prolapse toward the left VOLUME
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ventricle and so fail to play a role in guarding the orifice. The frequent coexistence of a congenital bicuspid aortic valve in cases of coarctation of the aorta is recognized.4 Moreover, in such cases the bicuspid aortic valve may be incompetent. Less commonly realized is that incompetence of the bicuspid aortic valve may occur in patients without aortic coarctation. We have observed two examples of sudden death in
FIG. 4. Congenital bicuspid aortic valve from a 50 year old man. Chronic aortic insufficiency had been present, and death was sudden. Incompetence of the valve resulted from prolapse of the larger cusp.
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FIG. 5. Coqrn~M b~u$rd ~CU//C N/W tl(~monstratinq two possible functional characteristics. In r,ach, one cusp is large] than the other. o. the two cusps close thr orificc. h. the larger cusp prolapses and accounts for incompetence of the valve.
FIG. 6. 1Jnicommissuralnortic valuewhich is both stenotic and incompetent. n, semidiagrammatic drawing. The unicommissural valve haa but one cusp constructed in such a manner to give the orifice the shape of an exclamation point. The narrow part of the orifice is at the single commissure, while the widest part lies opposite the commissure. The single leaflet arises from the aortic wall adjacent to the commissure and extends across the orifice without making contact with the aortic wall. It then bends upon itself to make a second connection with the aortic wall near the first, thereby creating a single commissure. Although raphes are present in dome-shaped stenosis, these do not usually extend far from the base toward the free edge of the valvular tissue. b, a unicommissural aortic valve from above in a 54 year old woman. The valve orifice, which is fixed, is both stenotic and incompetent.
men with congenital bicuspid aortic valve but without coarctation. In each patient, aortic incompetence had been known to be present for many years. Th “dome-shaped” aortic valve closely resembles the pulmonary valve in congenital “domeshaped” stenosis. In its fully developed state the valve has no commissures, the cuspid tissue
simply being fused and resembling a truncated cone. A variant of this malformation is the socalled unicuspid aortic valve’ (Fig. 6). Then ature of the dome-shaped aortic valve is such that there is either inadequate material or no material to serve as flaps. The orifice is narrow and relatively fixed. Such valves are invariably stenotic and concomitantly incompetent. In THE
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the untreated state, the stenotic element usually dominates the clinical picture, but surgical attempts at relief of the stenosis may be followed by greater evidence of incompetence because, following incision, the cuspid tissue becomes flail. Aortic incompetence associated with ventricular septal defect is a well recognized entity. The defect lies somewhat anterior to the position of the usual ventricular septal defect, is immediately inferior to the right aortic cusp, and is associated with faulty support of the root of the The involved part of the aorta slides aorta. away from the plane of the ventricular septum. As a result of this process, the right aortic cusp tends to tip into a horizontal position relative to the left ventricular outflow tract. Therefore, the cusp fails to make proper contact with the other two cusps, and incompetence of the aortic As the right aortic cusp lies in an valve results. abnormal position and functions abnormally, it may become elongated, prolapse and accentuate the valvular incompetence. DISEASES OF ASCENDING
AORTA CAUSING ABNORMAL RUN-OFF OF BLOOD
The diseases of the ascending aorta responsible for abnormal run-off of blood fall into two major categories: (1) those causing secondary incompetence of the valve, thus allowing return of blood to the left ventricle through the orifice of the valve and (2) those characterized by an abnormal communication between the ascending aorta on one hand and a cardiac chamber or a large mediastinal vessel on the other. ESCAPE OF BLOOD THROUGHAORTIC VALVE Either of two fundamental processes, dilatation or tearing of the aorta, may be involved when a condition of the ascending aorta causes incompetence of the aortic valve. The more common is abnormal dilatation of the aorta as in (1) syphilitic disease, (2) rheumatoid spondylitis, (3) dissecting aneurysm, (4) the congenital weakness of the aortic media in Marfan’s syndrome or in idiopathic dilatation of the aorta and (5) senile dilatation of the aorta. Undetermined inflammatory diseases of the aorta5 may, rarely, also be responsible for aortic dilatation. The other and the less common process is a tear of the ascending aorta, so located as to cause loss of support of one or more of the aortic cusps. Dissecting aneurysm and traumatic rupture of the ascending aorta are the two usual causes of such a process. Syphilitic aortitis is the classical condition in VOLUME
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FIG. 7. Sy,bhilitic aortitis. Sagittal section through the thoracic aorta and the ventricular portion of the heart showing characteristic gross dilatation of the ascending aorta and aortic arch. Dilatation of the aorta under certain circumstances may be responsible for aortic incompetence. which aortic valvular incomoetence results from The primary process lies in aortic dilatation. the aortic media. As the aorta dilates, the straight-line distance between the two lateral attachments of each aortic cusp increases (Fig. While the cusps are not primarily involved, 7). the dilatation of the aorta causes reduction of the extra length of the cusps relative to the diameter of the aorta. As a result the cusps cannot make contact at the center of the aortic As the aorta orifice, and incompetence results. dilates, the spaces between adjacent cusps (the commissures) widen. This process accentuates the degree of valvular incompetence but is probably of secondary importance relative to the tension upon the cusps. As incompetence of the valve is set into motion by these processes, the cusps may exhibit changes secondary to the mechanical effects of the incompetence. These changes include rolling of the free aspect of the cusp and diffuse fibrosis which, in turn, may contribute to even greater degrees of valvular incompetence. Rheumatoid spondylitis may be associated with
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showing FIG. 8. Idiqbnthic dilatation of thrzuortnreithaortirin,wJkirnrvin a 52 )-carold man. (1,thwack rocntgcnogram No calcification of the aorta is present. promincncc of the ascendinq aorta as well as Aarqement of the left xzentriclr. 6, photomicrograph of the ascending aorta showing arcas of cystic medial necrosis with disruption of the normal_eler, photograph of the unopened aortic ments of thr media (hematolyxin and cosin stains. 110 reduced by 10%). valve from above. Dilatation of the aorta has caused bowing of the cusps and a resultant triangular defect at the center of the valvular orifice. d, scmidiagrammatic drawing of the valve to demonstrate the primary process of aortic dilataRolling of the free edges tion. Bowing of the cusps and separation at the commissures result in aortic incompetence. of the cusps is, in turn, considered to be secondary to aortic regurgitation.
medial disease of the ascending aorta, as obIn the asserved by Bauer and associates.6 tending aorta the pathologic changes bear close resemblance to those in syphilitic aortitk’fs
One feature which tends to distinguish the aortic disease of rheumatoid spondylitis from syphilitic aortitis is the distribution of involvement. In .. . . . syphlhtx aortltls the entire thoracic aorta is inTHE
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Conditions
FIG. 9.
Senile dilatation
of
of Ascending
Aorta
the aorta with aortic insujkiency.
Dilatation of the aorta has caused bowing of the aortic cusps, which now cannot approximate sufficiently to close the orifice. The mechanical effects of the aortic dilatation upon the cusps are like those in syphihtic aortitis and idiopathic dilatation of the aorta.
volved ; in rheumatoid spondylitis the lesions tend to be confined to the ascending aorta. Cystic medial necrosis of the aorta in varying deIt may be observed in hypergrees is common. tensive patients with and without dissecting aneurysm of the aorta. Under uncommon circumstances it occurs to such a degree that the resulting weakness of the aortic wall causes the aorta to dilate abnormally (Fig. 8). Patients in whom this phenomenon occurs are usually Some show the classic body normotensive. physique of arachnodactyly (Marfan’s syndrome).g-12 In those patients without signs of
Simulating
Aortic
Incompetence
FIG. 10. Dissecting aneurysm of aorta. Region of aortic valve in a 54 year old hypertensive man with a recent Vertical tear (between arrows) dissecting aneurysm. of inner aspect of aorta and dissecting hematoma (D.A.) within wall of ascending aorta are present. Death reIn cases of this type, sulted from hemopericardium. dilatation of the ascending aorta incident to the internal tear may cause aortic valvular incompetence.
Marfan’s syndrome but with aortic lesions and dilatation indistinguishable from those in Marfan’s syndrome, the process is called idiopathic dilatation of the aorta.13 The fundamental mechanical basis for aortic valvular incompetence in dilatation of the aorta from cystic medial necrosis is similar to that in syphilitic aortitis. There is a tendency for the dilated aorta with cystic medial necrosis to develop a tear in its wall. As in other instances of tearing in the aorta, either a dissecting or a saccular aneurysm results. Each of these complicating lesions
FIG. 11. Marfnn’s syndrome, aortic dissecting aneurysm and aortit in.m@ciency in a 32 year old woman (reported by Husebye et al.is). (I, roentgenogram of the right foot. b, roentgenogram of right hand. c, close-up view of aortic Both show prominent features of arachnodactyly (Marfan’s syndrome). valve (AV) and proximal part of ascending aorta. Retracted edges (A) represent retracted edges of the primary rupture in ascending aorta. The rupture extends almost completely around the (Roentaortic circumference leaving only one narrow segment of aortic wall uninvolved (0). genograms (1 and b are reprinted from Husebye and associatesis in the Am. Heart J. by permission of the authors and C. V. Mosby Company, publisher.) VOLUME
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Outflow tract of tllr FIG. 12. .iortictern in ity/~rr/rv~.~;rm. left ventricle and aortic valve in a 41 year old man with hypertension. In the ascending aorta is a longitudinal tear (arrowj. The lesion is thr primary tear like that seen in typical dissecting aneurysm of the aorta although in this cast3 extensive intramural hemorrhage did not occur. Through-and-through rupture resulted in fatal hemopericardium. Had the latter complication not occurred, it is possible that chronic aortic insufficiency might have resulted from the dilatation of the aorta following the tear. causes further dilatation of the aorta and comSuch pounds the distortion of the aortic cusps. a case in our collection was reported by Levy and co-workers.2 In a patient who had a saccular aneurysm of the ascending aorta, distortion of the aortic cusps resulted in rupture with prolapse in one and attenuation with extensive fenestration in the other. Cystic medial necrosis of the aorta has been described in yet another heritable disorder of
tissue,
the
so-called
Ehlers-Danlos
syndrome.‘4 Dilatation of the aorta with advancing age, a commonly recognized phenomenon, is often wrongly interpreted as secondary to atherosclerosis. The process, though poorly understood, is to be charged to progressive weakness of the media. Though some degree of aortic dilatation is universal in persons of advanced age, the degree of dilatation is usually not great enough to be responsible for aortic valvular insufficiency.16 Nevertheless, we have observed established examples of aortic insufficiency resulting from “senile dilatation” of the aorta (Fig. 7). We have also studied an example of rupture of the aorta as a complication of extreme dilatation in this condition. Tearing of the ascending aorta may be observed under several circumstances. The most common of these is typical dissecting aneurysm of the aorta in the hypertensive patient (Fig. 10),16,17 In the patient with Marfan’s syndrome this aneurysm may yield a gross picture similar to that in the more common circumstance of dissecting aneurysm (Fig. 11).i2~‘8 A tear in the ascending aorta may occur without intramural The process resembles that dissection of blood. of typical dissecting aneurysm save for the absence of the element of intramural dissection of This type of tear may be blood (Fig. 12). termed “incomplete dissecting aneurysm” of the aorta. The other circumstance that causes a tear in the ascending aorta is external trauma to the thorax (Fig. 13). These tears vary in their significance for aortic incompetence. Those involving that part immediately above the valve are more likely to result in valvular incompetence than are a, left FIG. 13. Traumatic tear in aorta. ventricle and aortic valve, including the proximal portion of the ascending aorta, showing a transverse tear (between arrows) 1 cm. above the left aortic valve, a result of an automobile accident. Rupture of the aorta led to hemopericardium. The tear appears to be sufficiently removed from the aortic valve so as not to cause loss of support for the valve. b, a second example of traumatic tear (arrows) in the ascending aorta from a 70 year old man who suffered multiple injuries when struck by a car. Immediately above the aortic valve is a tear of the ascending aorta. This involved the attachment of aortic cusps and caused prolapse of the related aortic cusps.
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Im 14. hpnitd flOrtiCSinUSaneurysm. a, tricuspid valve and adjacent portions of right atrium and ventricle. mediately above the tricuspid valve is a large aneurysm (between arrows) which has ruptured into the right atriumAneurysm originated from posterior aortic sinus. b, the right (R.) and posterior (P.) aortic sinuses exposed by division and retraction of the aortic leaflets. The circular defect at the depths of the posterior sinus is the mouth of the aneurysm which appeared in the right atrium and was illustrated in a. The aortic tissue forming the upper edge of the mouth of In the right aortic sinus there is a the aneurysm ends abruptly, having been avulsed from the aortic valvular annulus. similar change in the aortic wall, which has retracted upward, exposing the muscle of the right ventricular wall. In the right sinus the early stages in the formation of a so-called congenital aortic sinus aneurysm are portrayed. FIG.
When valvular those lying at more distal levels. incompetence complicates a tear of the ascending aorta, the functional abnormality results either from dilatation of the aorta, resulting in basic valvular distortion, as in syphilitic aortitis, In or from loss of support of valvular tissue. the latter circumstance, the inadequately supported cusp prolapses into the left ventricle. Mycotic aneurysm of the ascending aorta represents yet another form of loss of continuity of Usually, the underlying lesion the aortic wall. is bacterial endocarditis of a left-sided valve, Abnormal run-off more often the aortic valve. of blood from the aorta in association with a mycotic aneurysm is not usually a direct result of the mere presence of the mycotic aneurysm but of one of two features that may be associated with it: destruction of aortic valvular tissue caused by the bacterial endocarditis or communication of the mycotic aneurysm with a Comcardiac chamber or a mediastinal vessel. plicated situations of this type are discussed more fully here. RUN-OFF THAN
OF BLOOD
AORTIC
THROUGH
CHANNELS
OTHER
VALVE
Incompetence of the aortic valve may be simulated when there is an abnormal communication between the ascending aorta on one hand VOLUME 14, NOVEMBER 1964
and a cardiac chamber, a pulmonary artery or a thoracic vein on the other. Abnormal communications in which a coronary artery is involved may yield similar results. Abnormal communications involving the ascending aorta may arise either from the beginning of the aorta at the sinuses or at more distal levels in this segment of the vessel. Congenital and inflammatory processes are the most common causes. Aneurysms of the aortic sinuses are of two types, the congenital and the acquired.rg Congenital aneurysms are characterized by separation of the aortic media from the annulus of the aortic valve.20 Following this event, the hydrostatic pressure within the aorta is supported only by that tissue lying against that segment of the As this tissue is aorta that is devoid of media. weaker than the aortic media, an aneurysm forms and may rupture. Acquired aneurysms of the aortic sinuses usually are mycotic aneurysms secondary to bacterial endocarditis. These, too, may rupture into a cardiac chamber or, in contrast to the usual congenital aneurysm, into the pericardial cavity. It should be emphasized that after a congenital aneurysm of an aortic sinus has ruptured, it may then become the seat of bacterial infection. The lesions resulting from such a sequence of events may be difficult to distinguish
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and Edwards FIG. 15. .4n,lzco-lrf~ventricular tunnd in 5 year old boy (illustrations from Levy and associate9”). a, the aortic valve and proximal part of interior of ascending aorta viewed from above. The aortic valve (A.V.) is bicuspid. In the anterior wall of the ascending aorta is a defect (CM.) which represents the aortic mouth of the aorticoleft ventricular tunnel. b, sagittal section through the ascending aorta and the ventricular septum, exposing the left ventricular cavity (L.V.) and a portion of the outflow tract of the right ventricle (Inf.). Part of this outflow tract, the pulmonary valve and part of the pulmonary trunk (P.T.) has been removed by this plane of section. The aortico-left ventricular tunnel (arrow) begins in the ascending aorta, enters the ventricular septum and, bypassing the aortic valve, then joins the left ventricular cavity. (Reprinted from Circulation24 by permission of the American Heart Association, Inc., publisher.)
from a ruptured aneurysm in which the primary process was bacterial infection. Congenital aneurysms of the aortic sinuses patanatomicofunctional certain assume terns.20~21 Aneurysms involving the left aortic sinus (that related to the origin of the left coronary artery) do not rupture into a cardiac chamber. By virtue of the anatomic relationships of the left aortic sinus, the rare aneurysm in this location may cause narrowing of the left coro-
nary artery,22 or it may rupture into the pericardial cavity. In this location the aneurysm does not usually establish a condition that simulates aortic incompetence. On the contrary, aneurysms of either of the other two aortic sinuses do, upon rupture, cause abnormal runoff into a cardiac chamber.1g-21J3 Aneurysms of the posterior (noncoronary) sinus classically rupture into the right atrium and are not associated with other malformations (Fig. 14). Aneurysms of the right aortic sinus (related to the right coronary arterial ostium) may rupture into the right atrium, although this is rare; more commonly, they rupture into the outflow tract of the right ventricle. Such aneurysms have a tendency to be associated with a ventricular septal defect. We have observed an exceptional example of a congenital aneurysm involving the right aortic sinus. The lower aspect of the aneurysm classically bulged into the right ventricular outflow tract. The upper portion appeared in the right atrioventricular sulcus, where it pushed the right coronary artery upward and ruptured into the pericardial cavity. Patients in whom an aortic sinus aneurysm has ruptured into a cardiac chamber usually exhibit classic signs of aortic incompetence. In some, the time of rupture may be clearly identified by the sudden onset of thoracic pain, dyspnea and signs of aortic incompetence. Length of survival following the rupture varies from minutes to years. Those patients surviving for longer periods usually manifest congestive cardiac failure. The so-called aortico-left ventricular tunnel is to be distinguished from aortic sinus aneurysm. It is a congenital communication between the aorta and the left ventricle, a vessel-like structure THE
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16. Healed bacterial endocurditis and mycotic anmrysm of aorta communicatzng with Ifft urntricle in a 54 year old woman. ~2, aortic valve. Fibrous thickening of cusps and an attached vegetation of healed bacterial endocarditis are seen. The probe is in a perforation of an aortic cusp. In the background beneath the probe is a defect in the septal wall of the subaortic region of the left ventricle. This is the lower mouth of a mycotic aneurysm of the ascending aorta illusAbove the trated in 6. b, sagittal section through ascending aorta (A.), aortic valve and ventricular septum (V.S.). valve, two perforations are present in the wall of the right aspect of the ascending aorta (one containing a probe; section made through the second opening). These openings lead into an aneurysm in the base of the ventricular septum. The lowermost part of the aneurysm communicates with the left ventricular cavity (L.V.). The peculiarities of the aneurysm provide for a channel that bypasses the aortic valve and connects the aorta with the left ventricle. FIG.
which originates from the anterior aspect of the aorta just above the levels of the coronary arterial ostia (Fig. 15). The channel enters the base of the ventricular septum, bypasses the aortic valve and enters the left ventricle. In its position within the ventricular septum, the tunnel lies in the posterior wall of the right ventricular infundibulum. The distortion of this wall suggests that a tendency toward right ventricular infundibular stenosis may be created. In 1 of the 3 cases reported by Levy and associates,24 and in another case observed by one of us, the extracardiac portion of the tunnel exhibited a saccular aneurysm. Bacterial infection of the ascending aorta, with the formation of a myocotic aneurysm may, through rupture of the aneurysm, lead to abnormal escape of blood from the ascending aorta.26 Those mycotic aneurysms which involve the aortic sinuses may lead either into the right atrium or the right ventricle. Because of the potential complexity of the path an infectious process may take, certain exotic channels may VOLUME
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result from bacterial infection. collection illustrates this :
A case in our
The patient was a 54 year old woman with evidence of healed bacterial endocarditis in the form of a perforation in one aortic cusp. In addition, there were two perforations in the posterior wall of the ascending aorta. These, in turn, led into an aneurysmal-like cavity within the ventricular septum. In this position the aneurysm pushed the infundibular wall of the right ventricle forward. The lower aspect of the aneurysm, which bypassed the aortic valve, communicated with the left ventricular cavity (Fig. 16). In a second case, involving a 14 year old patient, the clinical and angiocardiographic findings suggested presence of an aortic sinus aneurysm which had ruptured into the right ventricle. Moreover, as there was no history of bacterial endocarditis, the assumed aortic sinus aneurysm was considered to be of congenital origin. The pathologic examination revealed a ventricular septal defect and signs of healed bacterial endocarditis of the aortic valve (Fig. 17). The right aortic cusp was deformed in an aneurysmal manner. Its aneurysm, which was perforated, had prolapsed and passed through the ventricular septal
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FIG. 17.
venti
in 14 year old boy.
n, region of aortic valve. ‘f’he aortic valvular tissue is bulbous on the basis of healed vegetations of bacterial endocarditis. :Zn aneurysm of the right aortic cusp protrudes through a small ventricular septal defect (point of arrow) into the right ventricle. b, sagittal section through the ventricular septum (V.S.) and the aortic valve. The aneurysm of the right aortic cusp (An.) has herniated through the ventricular septal defect into the right ventricular cavity (R.V.). Perforations in the aneurysm of the valve allowed abnormal communication between the aorta and the right ventricular cavity. C, right ventricle. Immediately distal to the tricuspid valve is the aneurysm (An.) of the right aortic cusp. Through this, the aorta communicated with the right ventricle (R.V.). d, diagrammatic portrayal of the ventricular septal defect and the perforated aneurysm of the right aortic cusp which herniated through the ventricular septal defect to allow communication between the aorta and the right ventricle.
defect, thereby establishing a route between the astending aorta and the right ventricle. Syphilitic aortitis, as has been indicated, classitally causes incompetence of the aortic valve. Under other circumstances, syphiliticaortitismay underlie a situation whereby blood from the ascending aorta escapes through routes other
than the aortic valvular orifice. Such communications usually complicate syphilitic aneurysms. Charcteristically, these lie at levels distal to the aortic sinuses. The positions of syphilitic aneurysms of the aorta are such that when they rupture, the aortic lumen communicates with the pericardial cavity, the pulmonary trunk or right pulmonary THE
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FIG. 18. Anonudous connections of coronary arteries. Diagrammatic representation of conditions in which two coronary arteries arise from the aorta and in which one or both vessels make anomalous connection with a cardiac chamber or a vessel. O, right coronary artery joins coronary sinus. 6, right coronary artery connects with right atrium. C, right ventricle receives a branch of right coronary artery. d, both coronary arteries connect through an “accessory coronary artery” with the pulmonary trunk.
artery or the superior vena cava. Rupture into either of the last two sites sets the stage for a functional condition simulating aortic valvular insufficiency. Usually, such communications cause major and acute vascular catastrophe and early death. Less commonly, a chronic state may result. VOLUME
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1964
A congenital communication between the ascending aorta and the pulmonary arterial system occurs in the
form of the classic aorticopulmonary septal defeet (aorticopulmonary window ; partial persistent truncus arteriosus). This lesion is characterized by a wide opening in the left anterior wall of the aorta just above the levels of the coronary
692
FIG. 19.
Eliot, Woodburn and Edwards
coronnrv arte?y-ryhl
urntrmln,_J5.aLln.
in) Frontal
communication between the left coronarv artrrv channel between the aorta and the right vcntriclc.
and
and ih I left lateral view of aortogram on a patient having a The fistula is demonstrated as a tortuous the right ventricle.
arterial ostia.Z6T” The opening represents a window-like communication between the aorta In the latter vessel, and the pulmonary trunk. the opening is located in its right wall just anterior to the ostium of the right pulmonary arAs the opening is large, there is a tendtery. ency for equalization of pressures between the systemic and pulmonary arterial systems. The functional characteristics of this condition are similar to those in wide patent ductus arteriosus. Anomalous communication of coronary arteries may cause functional derangements that in some aspects are like those in certain conditions of the Two anatomic entities perascending aorta.28 tain. In the first, both coronary arteries arise from the aorta, and one communicates anomalously either with the coronary sinus, a cardiac chamber or the pulmonary trunk (Fig. 18).2g In any of these situations an arteriovenouslike arrangement involves the coronary arterial system, and this may be reflected as an abnormal escape of aortic blood (Fig. 19) and as myocardial ischemia. The dilated artery or arteries involved are tortuous and wide and are subject to the development of complicating saccular aneurysms. In the second anatomic entity of anomalous communication of coronary arteries, one arises
from the aorta and the other arises from the pulWith the development of wide monary trunk. and numerous collaterals between the two, blood from the artery arising from the aorta will escape into the abnormally arising artery and, through it, into the pulmonary trunk (Fig. 20). Here, also, an arteriovenous fistulous arrangement is present, and the characteristics of aortic blood pressure and myocardial perfusion are like those in the first anomalous coronary arterial arrangement.
DISCUSSION We have considered two major anatomic forms of aortic incompetence : one through the aortic valve itself and the other, through chanClinically, nels other than the aortic valve. this distinction may be made by the location and duration of the murmurs. A to-and-fro murmur usually indicates aortic incompetence through the valve itself or through a communicaOn the tion from the aorta to the left ventricle. other hand, the continuous murmur, regardless of its location, is usually associated with some form of communication between the aorta and a right-sided cardiac structure such as the coronary sinus, right atrium, right ventricle or pulmonary trunk. THE
AMERICAN
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of Ascending
Aorta
Simulating
Aortic
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allow abnormal escape of blood from the aorta The first includes those fall into two categories. conditions which, by virtue of the distortion or dilatation of the ascending aorta, cause the aortic valve to become incompetent. The route for abnormal escape of blood in these In the situations is through the aortic valve. second the aorta communicates abnormally with a cardiac structure or a mediastinal blood vessel. Congenital, traumatic and infectious conditions constitute the major backgrounds for this second type of functional derangement.
REFERENCES Pathologic aspects of cardiac 1. EDWARDS, J. E. valvolar insufficiencies. A.M.A. Arch. Sk..., 77:
634, 1958. 2. LEVY, M. J., SIEGAL, D. L., WANG, Y. and EDWARDS, J. E. Rupture of aortic valve secondary to aneurysm of ascending aorta. Circulation, 27 : 422,
1963. 3. EDWARDS, J. E. The congenital bicuspid aortic valve. Circulation, 23: 485, 1961. 4. EDWARDS, J. E., CHRISTENSEN,N. A., CLAGETT, 0. T. and MCDONALD, J. R. Pathologic considerations in coarctation of the aorta. PIOC. Staff Meet.
FIG. 20. Anomalous origin of the left coronary artery. When collaterals between the two coronary arteries become established, run-off of aortic blood occurs from the right coronary artery through the anomalously arising left artery and into the pulmonary trunk.
In general, the location of a murmur and its point of maximal intensity are of additional importance in differentiation. Murmurs of incompetence of the aortic valve are usually loudest in the so-called aortic area. If the murmur is located anywhere except in the aortic area, this finding is highly suggestive of some form of aortic incompetence which bypasses the aortic valve to communicate with a right-sided cardiac structure. The electrocardiogram may also aid in differentiating between the two anatomic forms of Signs of right ventricular aortic incompetence. (in addition to left) hypertrophy are of primary significance, since such hypertrophy would suggest a shunt from the ascending aorta to the right side of the heart. SUMMARY
Abnormal run-off of blood from the ascending aorta may result either from primary diseases of the aortic valve or from primary diseases of the ascending aorta. Primary diseases of the ascending aorta which VOLUME
14, NOVEMBER 1964
Mayo Clin., 23: 324, 1948. 5. MCGUIRE, J., SCOTT, R. C.
and GALL, E. A. Chronic aortitis of undetermined cause with severe and fatal aortic insufficiency. Am. J. M.
SC., 235: 394, 1958. 6. BAUER, W., CLARK, W. S. and KULKA, J. P. Aortitis and aortic endocarditis, an unrecognized manifestation of rheumatoid arthritis. Ann. Rhcumat.
Dis., 10: 470, 1951. 7. CLARK, W. S., KULKA, J. P. and BAUER, W.
Rheumatoid aortitis with aortic regurgitation. An unusual manifestation of rheumatoid arthritis (including spondylitis). Am. J. Med., 57: 580, 1957. 8. DAVIDSON, P., BAGGENSTOSS,A. H., SLOCUMB, C. H. and DAUGHERTY, G. W. Cardiac and aortic lesions in rheumatoid spondylitis. Proc. Staff Meet.
Mayo Clin., 38: 427, 1963. 9. TUNG, H. L. and LIEBOW, A. A.
Marfan’s syndrome. Observation at necropsy with special reference to medionecrosis of the great vessels. Lab.
Invest., 1 : 382, 1952. The cardiovascular 10. MCKUSICK, V. A.
aspects of Marfan’s syndrome: A heritable disorder of connective tissue. Circulation, 11: 321, 1955. 11. PAPAIOANNOU.A. C.. AGUSTSSON.M. H. and GASUL. B. M. Early manifestations of the cardiovascul& disorders in Marfan syndrome. Pediatrics, 27:
255 1961. 12. WAGENVOORT, C. A., NEUFELD, H. N. and EDWARDS, J. E. Cardiovascular system in Marfan’s syndrome and in idiopathic dilatation of the ascending aorta. Am. J. Cardiol., 9: 496, 1962. 13. WEAVER, W. F., EDWARDS, J. E. and BRANDENBURG, R. 0. Idiopathic dilatation of the aorta with aortic valvular insufficiency: A possible forme fruste of Marfan’s syndrome. Proc. Sta# Meet.
Mayo Clin., 34: 518, 1959.
Eliot,
694
Woodburn
14. FRIEDEN, J., HURWITT, E. S. and LEADER, E. Kuptured aortic cusp associated with an heritable disorder of connective tissue. Am. .J. Med., 33: 61.5, 1962. 15. EDWARDS, J. E. An Atlas of Acquired Diseases of the Heart and Great Vessels, p. 1126. Philadelphia, 1961. W. B. Saunders Co. 16. GORE, 1. and SEIWERT, V. J. Dissecting aneurysm of the aorta. Arch. Path., 53: 121, 1952. 17. BURCHELL, H. B. Aortic dissection (dissecting hematoma;
dissecting
aneurysm
of the aorta).
Circulation, 12: 1068, 1955. 18. HUSEBYE, K. O., WOLFF, H. J. and FRIEDMAN,L. L. Aortic dissection in pregnancy: A case of Marfan’s syndrome. Am. Heart J., 55: 662, 1958. 19. JONES, A. M. and LANGLEY, F. A. Aortic sinus aneurysms. Brit. Heart J., 11: 325, 1949. 20. EDWARDS, J. E. and BURCHELL, H. B. The pathologic anatomy of deficiencies between the aortic root and the heart, including aortic sinus aneurysms. Thorax, 12 : 125, 1957. 21. SAKAKIBARA, S. and KONNO, S. Congenital aneurysm of the sinus of Valsalva. Anatomy and classification. Am. Hemt J., 63: 405, 1962. 22. ELIOT, R. S., WOLBRINK, A. and EDWARDS, J. E.
and Edwards Congenital aneurysm of the left aortic sinus. A rare lesion and a rare cause of coronary insufficiency. Circulation, 28: 951, 1963. 23. MACLEOD, A. Cardio-aortic fstula. &it. Heart J., 6: 194, 1944. 24. LEVY, M. J., LILLEHIII, c. \v., ANDERSON, R. C.,
25.
AMPLATZ, K. and EDWARDS, J. E. Aortico-left xrentricular tunnel. Circulation, 27: 841, 1963. SEGAL, E. L., BROADBENT,J. C. and EDWARDS, J. E. Cardio-aortic fI.stulas complicating bacterial endo-
carditis in a case of calcific aortic stenosis. Pm. Staf Meet. Mayo Clin., 33: 209, 1958. 26. GASUL, B. M., FELL, E. H. and CASAS, R.: The diagnosis of aortic septal defect by retrograde Report of a case. Circulation, 4: aortography. 251, 1951. 27. NEUFELD, H. N., et al. Aorticopulmonary septal defect. Am. J. Cardiol., 9: 12, 1962. 28. EDWARDS, J. E. Anomalous coronary arteries with special reference to arteriovenous-like communications. Circulation, 17: 1001, 1958. 29. NEUFELD, H. N., et al. Congenital communication of a coronary artery with a cardiac chamber or the pulmonary trunk (“coronary artery fistula”). Circulation, 24: 171, 1961.
THE AMERICAN JOURNAL OF CARDIOLOGY
Incompetence*
ROBERT S. ELIOT, M.D., ROBERT L. WOODBURN, 11.~. and JESSE E. EDWARDS, St.
Paul, Minnesota
SOME patients with signs of aortic valvular incompetence, the fundamental process may lie not in the aortic valve but rather in the ascending aorta. Certain cases in the collection of the cardiovascular registry of the Charles T. Miller Hospital are examples of this phenomenon, which is the subject of this report. For completeness and for reference on the basis for aortic incompetence caused by diseases of the ascending aorta, we will first consider the normal aortic valve and then the intrinsic diseases of the aortic valve which make it incompetent. Following this we will describe those diseases of the ascending aorta that, by allowing abnormal run-off of blood either through the aortic valve or through some other route, cause a functional state comparable to that resulting from intrinsic incompetence of the aortic valve.
I
M.D.
Valsalva) lies between the cusp and the related aortic wall. The upper aspect of the sinus is open and lies at an imaginary horizontal plane corresponding with the upper level of the cusp. The coronary arterial ostia lie just above the level of the aortic sinuses, although it is frequently incorrectly stated that the coronary arteries arise from their respective aortic sinuses.
N
INCOMPETENCE DUE TO INTRINSIC AORTIC VALVE DISEASE The common causes of aortic valvular incompetence are rheumatic endocarditis, bacterial endocarditis and congenital disease.l Uncommonly, trauma resulting from rupture of a cusp may be a basis for aortic incompetence. usually, that which follows trauma is the result of a tear in the ascending aorta rather than in the valve itself. Aortic valvular incompetence of rheumatic origin may either be pure or be associated with varying degrees of stenosis. Classically, when pure valvular incompetence is present, the rheumatic process has spared the commissures, while the cusps show intrinsic change (Fig. 2), characterized by contracture from scarring, causing shortening of the cusps in both length and breadth. Because of these changes the surface area of the cusps is inadequate to guard the valvular orifice. Classically, at the center of the aortic orifice, there is a triangular defect through which blood regurgitates. In addition, rheumatic endocarditis may cause fusion between adjacent cusps at the commissures. Those cusps involved in the process of adhesion
THE NORMAL AORTIC VALVE Each of the three aortic cusps may be looked upon as an independent unit connected to the aortic wall (Fig. 1). The length of the free aspect of each cusp is greater than the straightline distance between its two lateral extremities. The extra length is such that during systole the central point of each cusp extends to the center of the aortic orifice. In this way the three cusps together may close the orifice. The space between a lateral attachment of one cusp and that of the adjacent cusp is referred to as a commissure. In the normal aortic valve, three such functional zones exist. Each of the three aortic sinuses (sinuses of
* From the Department of Pathology, The Charles T. Miller Hospital, St. Paul, Minn., and the Departments of Medicine and Pathology, University of Minnesota, Minneapolis, Minn. This study was supported by Research Grant HE 5694 and Research Training Grant 5 Tl HE 5570 of the National Heart Institute, National Institutes of Health, U. S. Public Health Service. VOLUME
14.
NOVEMBER
1964
679
680
Eliot,
\~\‘oodl)urrl and
Edwards
Nocteriaf
rndoccuditis results in aortic
incompetence
through
upon the tissue.
its
Perforation
destructive
val\ular effects
of one or several
cusps may occur (Fig. 3), or valvular tissue ma) If the latter process is exbe lost by ulceration. tensive, support for the involved cusp or cusps is lost, and prolapse
FIG. 1 Ihnwtny II] m ml NWII<-w/w viewed from ahove. Each of the three aortic cusps is an indcpcndrnt unit connected to its respecti\r segment of the aortir wall. There arc two lateral attachments which are continuous through the inferior attachment of the cusp to the root of the aorta. Between the points of lateral attachment of each cusp is an excessive amount of tissue, which allows the central part of each cusp to extend to the center of The centers of each leaflet are in conthe aortic orifice. tact during closure of the valve. The space between the lateral attachments of two adjacent cusps is referred to as a commissurc.
at the commissures are prevented from full latIf the eral excursion during ventricular systole. change is sufficient, significant valvular stenosis may accompany the insufficiency.
of one or more of the cusps
may occur. As the destructive process takes place during the active stages of bacterial endocarditis, the aortic incompetence may complicate the state of the patient at this time. In some cases the bacterial stage of the disease may be overcome, but the mechanical residua continue to complicate the course of the illness. We have observed examples of ulceration or perforation of aortic cusps in patients with no history of antecedent bacterial endocarditis The lesions resemble those in cases of (Fig. 36). cured” bacterial enknown “bacteriologically For this reason it is our opinion docarditis. that such cases represent cure of bacterial endocarditis that was never diagnosed clinically. Cure may have been spontaneous or, it is more likely, occurred in response to the current procedure of liberal administration of antibiotics. Congenital feflestration of an aortic cusl) is so common an entity that it is usually considered a variant of the normal. The fenestration (or fenestrations) are usually confined to that part of the cusp which lies between the line of closure The below and the free edge of the cusp above.
FIG. 2. Rheumatic aortic incompetence. a, unopened aortic valve viewed from above. The valvular cusps are thickened and short. Two of the commissures are fused. The result of the shortening is 6, diagrammatic representation of rheumatic a triangular defect in the center of the aortic orifice. aortic incompetence demonstrating the thickening and the shortening of the aortic valvular cusps, which accounts for valvular incompetence. THE
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FIG. 3. Bacterial endocarditis. a, perforations (arrows) of aortic cusps resulting from bacterial endocarditis. 6, Perforation (arrow) of the posterior cusp of the aortic valve in the heart of a 35 year old woman. Although no history of bacterial endocarditis had been obtained, the perforation is considered to represent the healed lesion. Jet lesions (circle) secondary to regurgitation through the perforated aortic cusp are present upon the subaortic area of the ventricular septum.
fenestration is, therefore, usually above that part of the cusp that contributes to the closure of the valve. Only rarely is a fenestration large enough to form a basis for valvular incompetence. In a rare case reported from our institutions by Levy and associates,2 idiopathic dilatation of the aorta caused tension upon the cusps. As a result, enlargement of a congenital fenestration in one of the cusps was of such a degree that the cusp appeared to have been incompetent (a second cusp had ruptured as a result of the unusual mechanical tension). Maldevelopment in the number of aortic cusps is more common than fenestration as a congenital cause of intrinsic incompetence of the valve. Particularly significant is the congenital bicuspid aortic valve. Less common is the congenital “dome-shaped” valve. The congenital bicuspid aortic valve has been analyzed on theoretic grounds by one of us (J.E.Es), and it was emphasized that there are reasons for its malfunction. The valve with this anomaly is subject to (1) acquired aortic stenosis, often, with coexisting incompetence present, (2) bacterial endocarditis, sometimes with aortic incompetence as a secondary complication and (3) intrinsic incompetence (Fig. 4). The basis for intrinsic incompetence of the congenital bicuspid aortic valve seems to be that the two cusps are usually of unequal size (Fig. 5). The larger cusp may prolapse toward the left VOLUME
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1964
ventricle and so fail to play a role in guarding the orifice. The frequent coexistence of a congenital bicuspid aortic valve in cases of coarctation of the aorta is recognized.4 Moreover, in such cases the bicuspid aortic valve may be incompetent. Less commonly realized is that incompetence of the bicuspid aortic valve may occur in patients without aortic coarctation. We have observed two examples of sudden death in
FIG. 4. Congenital bicuspid aortic valve from a 50 year old man. Chronic aortic insufficiency had been present, and death was sudden. Incompetence of the valve resulted from prolapse of the larger cusp.
682
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h
FIG. 5. Coqrn~M b~u$rd ~CU//C N/W tl(~monstratinq two possible functional characteristics. In r,ach, one cusp is large] than the other. o. the two cusps close thr orificc. h. the larger cusp prolapses and accounts for incompetence of the valve.
FIG. 6. 1Jnicommissuralnortic valuewhich is both stenotic and incompetent. n, semidiagrammatic drawing. The unicommissural valve haa but one cusp constructed in such a manner to give the orifice the shape of an exclamation point. The narrow part of the orifice is at the single commissure, while the widest part lies opposite the commissure. The single leaflet arises from the aortic wall adjacent to the commissure and extends across the orifice without making contact with the aortic wall. It then bends upon itself to make a second connection with the aortic wall near the first, thereby creating a single commissure. Although raphes are present in dome-shaped stenosis, these do not usually extend far from the base toward the free edge of the valvular tissue. b, a unicommissural aortic valve from above in a 54 year old woman. The valve orifice, which is fixed, is both stenotic and incompetent.
men with congenital bicuspid aortic valve but without coarctation. In each patient, aortic incompetence had been known to be present for many years. Th “dome-shaped” aortic valve closely resembles the pulmonary valve in congenital “domeshaped” stenosis. In its fully developed state the valve has no commissures, the cuspid tissue
simply being fused and resembling a truncated cone. A variant of this malformation is the socalled unicuspid aortic valve’ (Fig. 6). Then ature of the dome-shaped aortic valve is such that there is either inadequate material or no material to serve as flaps. The orifice is narrow and relatively fixed. Such valves are invariably stenotic and concomitantly incompetent. In THE
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the untreated state, the stenotic element usually dominates the clinical picture, but surgical attempts at relief of the stenosis may be followed by greater evidence of incompetence because, following incision, the cuspid tissue becomes flail. Aortic incompetence associated with ventricular septal defect is a well recognized entity. The defect lies somewhat anterior to the position of the usual ventricular septal defect, is immediately inferior to the right aortic cusp, and is associated with faulty support of the root of the The involved part of the aorta slides aorta. away from the plane of the ventricular septum. As a result of this process, the right aortic cusp tends to tip into a horizontal position relative to the left ventricular outflow tract. Therefore, the cusp fails to make proper contact with the other two cusps, and incompetence of the aortic As the right aortic cusp lies in an valve results. abnormal position and functions abnormally, it may become elongated, prolapse and accentuate the valvular incompetence. DISEASES OF ASCENDING
AORTA CAUSING ABNORMAL RUN-OFF OF BLOOD
The diseases of the ascending aorta responsible for abnormal run-off of blood fall into two major categories: (1) those causing secondary incompetence of the valve, thus allowing return of blood to the left ventricle through the orifice of the valve and (2) those characterized by an abnormal communication between the ascending aorta on one hand and a cardiac chamber or a large mediastinal vessel on the other. ESCAPE OF BLOOD THROUGHAORTIC VALVE Either of two fundamental processes, dilatation or tearing of the aorta, may be involved when a condition of the ascending aorta causes incompetence of the aortic valve. The more common is abnormal dilatation of the aorta as in (1) syphilitic disease, (2) rheumatoid spondylitis, (3) dissecting aneurysm, (4) the congenital weakness of the aortic media in Marfan’s syndrome or in idiopathic dilatation of the aorta and (5) senile dilatation of the aorta. Undetermined inflammatory diseases of the aorta5 may, rarely, also be responsible for aortic dilatation. The other and the less common process is a tear of the ascending aorta, so located as to cause loss of support of one or more of the aortic cusps. Dissecting aneurysm and traumatic rupture of the ascending aorta are the two usual causes of such a process. Syphilitic aortitis is the classical condition in VOLUME
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FIG. 7. Sy,bhilitic aortitis. Sagittal section through the thoracic aorta and the ventricular portion of the heart showing characteristic gross dilatation of the ascending aorta and aortic arch. Dilatation of the aorta under certain circumstances may be responsible for aortic incompetence. which aortic valvular incomoetence results from The primary process lies in aortic dilatation. the aortic media. As the aorta dilates, the straight-line distance between the two lateral attachments of each aortic cusp increases (Fig. While the cusps are not primarily involved, 7). the dilatation of the aorta causes reduction of the extra length of the cusps relative to the diameter of the aorta. As a result the cusps cannot make contact at the center of the aortic As the aorta orifice, and incompetence results. dilates, the spaces between adjacent cusps (the commissures) widen. This process accentuates the degree of valvular incompetence but is probably of secondary importance relative to the tension upon the cusps. As incompetence of the valve is set into motion by these processes, the cusps may exhibit changes secondary to the mechanical effects of the incompetence. These changes include rolling of the free aspect of the cusp and diffuse fibrosis which, in turn, may contribute to even greater degrees of valvular incompetence. Rheumatoid spondylitis may be associated with
684
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showing FIG. 8. Idiqbnthic dilatation of thrzuortnreithaortirin,wJkirnrvin a 52 )-carold man. (1,thwack rocntgcnogram No calcification of the aorta is present. promincncc of the ascendinq aorta as well as Aarqement of the left xzentriclr. 6, photomicrograph of the ascending aorta showing arcas of cystic medial necrosis with disruption of the normal_eler, photograph of the unopened aortic ments of thr media (hematolyxin and cosin stains. 110 reduced by 10%). valve from above. Dilatation of the aorta has caused bowing of the cusps and a resultant triangular defect at the center of the valvular orifice. d, scmidiagrammatic drawing of the valve to demonstrate the primary process of aortic dilataRolling of the free edges tion. Bowing of the cusps and separation at the commissures result in aortic incompetence. of the cusps is, in turn, considered to be secondary to aortic regurgitation.
medial disease of the ascending aorta, as obIn the asserved by Bauer and associates.6 tending aorta the pathologic changes bear close resemblance to those in syphilitic aortitk’fs
One feature which tends to distinguish the aortic disease of rheumatoid spondylitis from syphilitic aortitis is the distribution of involvement. In .. . . . syphlhtx aortltls the entire thoracic aorta is inTHE
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Conditions
FIG. 9.
Senile dilatation
of
of Ascending
Aorta
the aorta with aortic insujkiency.
Dilatation of the aorta has caused bowing of the aortic cusps, which now cannot approximate sufficiently to close the orifice. The mechanical effects of the aortic dilatation upon the cusps are like those in syphihtic aortitis and idiopathic dilatation of the aorta.
volved ; in rheumatoid spondylitis the lesions tend to be confined to the ascending aorta. Cystic medial necrosis of the aorta in varying deIt may be observed in hypergrees is common. tensive patients with and without dissecting aneurysm of the aorta. Under uncommon circumstances it occurs to such a degree that the resulting weakness of the aortic wall causes the aorta to dilate abnormally (Fig. 8). Patients in whom this phenomenon occurs are usually Some show the classic body normotensive. physique of arachnodactyly (Marfan’s syndrome).g-12 In those patients without signs of
Simulating
Aortic
Incompetence
FIG. 10. Dissecting aneurysm of aorta. Region of aortic valve in a 54 year old hypertensive man with a recent Vertical tear (between arrows) dissecting aneurysm. of inner aspect of aorta and dissecting hematoma (D.A.) within wall of ascending aorta are present. Death reIn cases of this type, sulted from hemopericardium. dilatation of the ascending aorta incident to the internal tear may cause aortic valvular incompetence.
Marfan’s syndrome but with aortic lesions and dilatation indistinguishable from those in Marfan’s syndrome, the process is called idiopathic dilatation of the aorta.13 The fundamental mechanical basis for aortic valvular incompetence in dilatation of the aorta from cystic medial necrosis is similar to that in syphilitic aortitis. There is a tendency for the dilated aorta with cystic medial necrosis to develop a tear in its wall. As in other instances of tearing in the aorta, either a dissecting or a saccular aneurysm results. Each of these complicating lesions
FIG. 11. Marfnn’s syndrome, aortic dissecting aneurysm and aortit in.m@ciency in a 32 year old woman (reported by Husebye et al.is). (I, roentgenogram of the right foot. b, roentgenogram of right hand. c, close-up view of aortic Both show prominent features of arachnodactyly (Marfan’s syndrome). valve (AV) and proximal part of ascending aorta. Retracted edges (A) represent retracted edges of the primary rupture in ascending aorta. The rupture extends almost completely around the (Roentaortic circumference leaving only one narrow segment of aortic wall uninvolved (0). genograms (1 and b are reprinted from Husebye and associatesis in the Am. Heart J. by permission of the authors and C. V. Mosby Company, publisher.) VOLUME
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685
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Eliot,
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and Edwards connective
Outflow tract of tllr FIG. 12. .iortictern in ity/~rr/rv~.~;rm. left ventricle and aortic valve in a 41 year old man with hypertension. In the ascending aorta is a longitudinal tear (arrowj. The lesion is thr primary tear like that seen in typical dissecting aneurysm of the aorta although in this cast3 extensive intramural hemorrhage did not occur. Through-and-through rupture resulted in fatal hemopericardium. Had the latter complication not occurred, it is possible that chronic aortic insufficiency might have resulted from the dilatation of the aorta following the tear. causes further dilatation of the aorta and comSuch pounds the distortion of the aortic cusps. a case in our collection was reported by Levy and co-workers.2 In a patient who had a saccular aneurysm of the ascending aorta, distortion of the aortic cusps resulted in rupture with prolapse in one and attenuation with extensive fenestration in the other. Cystic medial necrosis of the aorta has been described in yet another heritable disorder of
tissue,
the
so-called
Ehlers-Danlos
syndrome.‘4 Dilatation of the aorta with advancing age, a commonly recognized phenomenon, is often wrongly interpreted as secondary to atherosclerosis. The process, though poorly understood, is to be charged to progressive weakness of the media. Though some degree of aortic dilatation is universal in persons of advanced age, the degree of dilatation is usually not great enough to be responsible for aortic valvular insufficiency.16 Nevertheless, we have observed established examples of aortic insufficiency resulting from “senile dilatation” of the aorta (Fig. 7). We have also studied an example of rupture of the aorta as a complication of extreme dilatation in this condition. Tearing of the ascending aorta may be observed under several circumstances. The most common of these is typical dissecting aneurysm of the aorta in the hypertensive patient (Fig. 10),16,17 In the patient with Marfan’s syndrome this aneurysm may yield a gross picture similar to that in the more common circumstance of dissecting aneurysm (Fig. 11).i2~‘8 A tear in the ascending aorta may occur without intramural The process resembles that dissection of blood. of typical dissecting aneurysm save for the absence of the element of intramural dissection of This type of tear may be blood (Fig. 12). termed “incomplete dissecting aneurysm” of the aorta. The other circumstance that causes a tear in the ascending aorta is external trauma to the thorax (Fig. 13). These tears vary in their significance for aortic incompetence. Those involving that part immediately above the valve are more likely to result in valvular incompetence than are a, left FIG. 13. Traumatic tear in aorta. ventricle and aortic valve, including the proximal portion of the ascending aorta, showing a transverse tear (between arrows) 1 cm. above the left aortic valve, a result of an automobile accident. Rupture of the aorta led to hemopericardium. The tear appears to be sufficiently removed from the aortic valve so as not to cause loss of support for the valve. b, a second example of traumatic tear (arrows) in the ascending aorta from a 70 year old man who suffered multiple injuries when struck by a car. Immediately above the aortic valve is a tear of the ascending aorta. This involved the attachment of aortic cusps and caused prolapse of the related aortic cusps.
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Im 14. hpnitd flOrtiCSinUSaneurysm. a, tricuspid valve and adjacent portions of right atrium and ventricle. mediately above the tricuspid valve is a large aneurysm (between arrows) which has ruptured into the right atriumAneurysm originated from posterior aortic sinus. b, the right (R.) and posterior (P.) aortic sinuses exposed by division and retraction of the aortic leaflets. The circular defect at the depths of the posterior sinus is the mouth of the aneurysm which appeared in the right atrium and was illustrated in a. The aortic tissue forming the upper edge of the mouth of In the right aortic sinus there is a the aneurysm ends abruptly, having been avulsed from the aortic valvular annulus. similar change in the aortic wall, which has retracted upward, exposing the muscle of the right ventricular wall. In the right sinus the early stages in the formation of a so-called congenital aortic sinus aneurysm are portrayed. FIG.
When valvular those lying at more distal levels. incompetence complicates a tear of the ascending aorta, the functional abnormality results either from dilatation of the aorta, resulting in basic valvular distortion, as in syphilitic aortitis, In or from loss of support of valvular tissue. the latter circumstance, the inadequately supported cusp prolapses into the left ventricle. Mycotic aneurysm of the ascending aorta represents yet another form of loss of continuity of Usually, the underlying lesion the aortic wall. is bacterial endocarditis of a left-sided valve, Abnormal run-off more often the aortic valve. of blood from the aorta in association with a mycotic aneurysm is not usually a direct result of the mere presence of the mycotic aneurysm but of one of two features that may be associated with it: destruction of aortic valvular tissue caused by the bacterial endocarditis or communication of the mycotic aneurysm with a Comcardiac chamber or a mediastinal vessel. plicated situations of this type are discussed more fully here. RUN-OFF THAN
OF BLOOD
AORTIC
THROUGH
CHANNELS
OTHER
VALVE
Incompetence of the aortic valve may be simulated when there is an abnormal communication between the ascending aorta on one hand VOLUME 14, NOVEMBER 1964
and a cardiac chamber, a pulmonary artery or a thoracic vein on the other. Abnormal communications in which a coronary artery is involved may yield similar results. Abnormal communications involving the ascending aorta may arise either from the beginning of the aorta at the sinuses or at more distal levels in this segment of the vessel. Congenital and inflammatory processes are the most common causes. Aneurysms of the aortic sinuses are of two types, the congenital and the acquired.rg Congenital aneurysms are characterized by separation of the aortic media from the annulus of the aortic valve.20 Following this event, the hydrostatic pressure within the aorta is supported only by that tissue lying against that segment of the As this tissue is aorta that is devoid of media. weaker than the aortic media, an aneurysm forms and may rupture. Acquired aneurysms of the aortic sinuses usually are mycotic aneurysms secondary to bacterial endocarditis. These, too, may rupture into a cardiac chamber or, in contrast to the usual congenital aneurysm, into the pericardial cavity. It should be emphasized that after a congenital aneurysm of an aortic sinus has ruptured, it may then become the seat of bacterial infection. The lesions resulting from such a sequence of events may be difficult to distinguish
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and Edwards FIG. 15. .4n,lzco-lrf~ventricular tunnd in 5 year old boy (illustrations from Levy and associate9”). a, the aortic valve and proximal part of interior of ascending aorta viewed from above. The aortic valve (A.V.) is bicuspid. In the anterior wall of the ascending aorta is a defect (CM.) which represents the aortic mouth of the aorticoleft ventricular tunnel. b, sagittal section through the ascending aorta and the ventricular septum, exposing the left ventricular cavity (L.V.) and a portion of the outflow tract of the right ventricle (Inf.). Part of this outflow tract, the pulmonary valve and part of the pulmonary trunk (P.T.) has been removed by this plane of section. The aortico-left ventricular tunnel (arrow) begins in the ascending aorta, enters the ventricular septum and, bypassing the aortic valve, then joins the left ventricular cavity. (Reprinted from Circulation24 by permission of the American Heart Association, Inc., publisher.)
from a ruptured aneurysm in which the primary process was bacterial infection. Congenital aneurysms of the aortic sinuses patanatomicofunctional certain assume terns.20~21 Aneurysms involving the left aortic sinus (that related to the origin of the left coronary artery) do not rupture into a cardiac chamber. By virtue of the anatomic relationships of the left aortic sinus, the rare aneurysm in this location may cause narrowing of the left coro-
nary artery,22 or it may rupture into the pericardial cavity. In this location the aneurysm does not usually establish a condition that simulates aortic incompetence. On the contrary, aneurysms of either of the other two aortic sinuses do, upon rupture, cause abnormal runoff into a cardiac chamber.1g-21J3 Aneurysms of the posterior (noncoronary) sinus classically rupture into the right atrium and are not associated with other malformations (Fig. 14). Aneurysms of the right aortic sinus (related to the right coronary arterial ostium) may rupture into the right atrium, although this is rare; more commonly, they rupture into the outflow tract of the right ventricle. Such aneurysms have a tendency to be associated with a ventricular septal defect. We have observed an exceptional example of a congenital aneurysm involving the right aortic sinus. The lower aspect of the aneurysm classically bulged into the right ventricular outflow tract. The upper portion appeared in the right atrioventricular sulcus, where it pushed the right coronary artery upward and ruptured into the pericardial cavity. Patients in whom an aortic sinus aneurysm has ruptured into a cardiac chamber usually exhibit classic signs of aortic incompetence. In some, the time of rupture may be clearly identified by the sudden onset of thoracic pain, dyspnea and signs of aortic incompetence. Length of survival following the rupture varies from minutes to years. Those patients surviving for longer periods usually manifest congestive cardiac failure. The so-called aortico-left ventricular tunnel is to be distinguished from aortic sinus aneurysm. It is a congenital communication between the aorta and the left ventricle, a vessel-like structure THE
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16. Healed bacterial endocurditis and mycotic anmrysm of aorta communicatzng with Ifft urntricle in a 54 year old woman. ~2, aortic valve. Fibrous thickening of cusps and an attached vegetation of healed bacterial endocarditis are seen. The probe is in a perforation of an aortic cusp. In the background beneath the probe is a defect in the septal wall of the subaortic region of the left ventricle. This is the lower mouth of a mycotic aneurysm of the ascending aorta illusAbove the trated in 6. b, sagittal section through ascending aorta (A.), aortic valve and ventricular septum (V.S.). valve, two perforations are present in the wall of the right aspect of the ascending aorta (one containing a probe; section made through the second opening). These openings lead into an aneurysm in the base of the ventricular septum. The lowermost part of the aneurysm communicates with the left ventricular cavity (L.V.). The peculiarities of the aneurysm provide for a channel that bypasses the aortic valve and connects the aorta with the left ventricle. FIG.
which originates from the anterior aspect of the aorta just above the levels of the coronary arterial ostia (Fig. 15). The channel enters the base of the ventricular septum, bypasses the aortic valve and enters the left ventricle. In its position within the ventricular septum, the tunnel lies in the posterior wall of the right ventricular infundibulum. The distortion of this wall suggests that a tendency toward right ventricular infundibular stenosis may be created. In 1 of the 3 cases reported by Levy and associates,24 and in another case observed by one of us, the extracardiac portion of the tunnel exhibited a saccular aneurysm. Bacterial infection of the ascending aorta, with the formation of a myocotic aneurysm may, through rupture of the aneurysm, lead to abnormal escape of blood from the ascending aorta.26 Those mycotic aneurysms which involve the aortic sinuses may lead either into the right atrium or the right ventricle. Because of the potential complexity of the path an infectious process may take, certain exotic channels may VOLUME
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result from bacterial infection. collection illustrates this :
A case in our
The patient was a 54 year old woman with evidence of healed bacterial endocarditis in the form of a perforation in one aortic cusp. In addition, there were two perforations in the posterior wall of the ascending aorta. These, in turn, led into an aneurysmal-like cavity within the ventricular septum. In this position the aneurysm pushed the infundibular wall of the right ventricle forward. The lower aspect of the aneurysm, which bypassed the aortic valve, communicated with the left ventricular cavity (Fig. 16). In a second case, involving a 14 year old patient, the clinical and angiocardiographic findings suggested presence of an aortic sinus aneurysm which had ruptured into the right ventricle. Moreover, as there was no history of bacterial endocarditis, the assumed aortic sinus aneurysm was considered to be of congenital origin. The pathologic examination revealed a ventricular septal defect and signs of healed bacterial endocarditis of the aortic valve (Fig. 17). The right aortic cusp was deformed in an aneurysmal manner. Its aneurysm, which was perforated, had prolapsed and passed through the ventricular septal
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FIG. 17.
venti
in 14 year old boy.
n, region of aortic valve. ‘f’he aortic valvular tissue is bulbous on the basis of healed vegetations of bacterial endocarditis. :Zn aneurysm of the right aortic cusp protrudes through a small ventricular septal defect (point of arrow) into the right ventricle. b, sagittal section through the ventricular septum (V.S.) and the aortic valve. The aneurysm of the right aortic cusp (An.) has herniated through the ventricular septal defect into the right ventricular cavity (R.V.). Perforations in the aneurysm of the valve allowed abnormal communication between the aorta and the right ventricular cavity. C, right ventricle. Immediately distal to the tricuspid valve is the aneurysm (An.) of the right aortic cusp. Through this, the aorta communicated with the right ventricle (R.V.). d, diagrammatic portrayal of the ventricular septal defect and the perforated aneurysm of the right aortic cusp which herniated through the ventricular septal defect to allow communication between the aorta and the right ventricle.
defect, thereby establishing a route between the astending aorta and the right ventricle. Syphilitic aortitis, as has been indicated, classitally causes incompetence of the aortic valve. Under other circumstances, syphiliticaortitismay underlie a situation whereby blood from the ascending aorta escapes through routes other
than the aortic valvular orifice. Such communications usually complicate syphilitic aneurysms. Charcteristically, these lie at levels distal to the aortic sinuses. The positions of syphilitic aneurysms of the aorta are such that when they rupture, the aortic lumen communicates with the pericardial cavity, the pulmonary trunk or right pulmonary THE
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FIG. 18. Anonudous connections of coronary arteries. Diagrammatic representation of conditions in which two coronary arteries arise from the aorta and in which one or both vessels make anomalous connection with a cardiac chamber or a vessel. O, right coronary artery joins coronary sinus. 6, right coronary artery connects with right atrium. C, right ventricle receives a branch of right coronary artery. d, both coronary arteries connect through an “accessory coronary artery” with the pulmonary trunk.
artery or the superior vena cava. Rupture into either of the last two sites sets the stage for a functional condition simulating aortic valvular insufficiency. Usually, such communications cause major and acute vascular catastrophe and early death. Less commonly, a chronic state may result. VOLUME
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A congenital communication between the ascending aorta and the pulmonary arterial system occurs in the
form of the classic aorticopulmonary septal defeet (aorticopulmonary window ; partial persistent truncus arteriosus). This lesion is characterized by a wide opening in the left anterior wall of the aorta just above the levels of the coronary
692
FIG. 19.
Eliot, Woodburn and Edwards
coronnrv arte?y-ryhl
urntrmln,_J5.aLln.
in) Frontal
communication between the left coronarv artrrv channel between the aorta and the right vcntriclc.
and
and ih I left lateral view of aortogram on a patient having a The fistula is demonstrated as a tortuous the right ventricle.
arterial ostia.Z6T” The opening represents a window-like communication between the aorta In the latter vessel, and the pulmonary trunk. the opening is located in its right wall just anterior to the ostium of the right pulmonary arAs the opening is large, there is a tendtery. ency for equalization of pressures between the systemic and pulmonary arterial systems. The functional characteristics of this condition are similar to those in wide patent ductus arteriosus. Anomalous communication of coronary arteries may cause functional derangements that in some aspects are like those in certain conditions of the Two anatomic entities perascending aorta.28 tain. In the first, both coronary arteries arise from the aorta, and one communicates anomalously either with the coronary sinus, a cardiac chamber or the pulmonary trunk (Fig. 18).2g In any of these situations an arteriovenouslike arrangement involves the coronary arterial system, and this may be reflected as an abnormal escape of aortic blood (Fig. 19) and as myocardial ischemia. The dilated artery or arteries involved are tortuous and wide and are subject to the development of complicating saccular aneurysms. In the second anatomic entity of anomalous communication of coronary arteries, one arises
from the aorta and the other arises from the pulWith the development of wide monary trunk. and numerous collaterals between the two, blood from the artery arising from the aorta will escape into the abnormally arising artery and, through it, into the pulmonary trunk (Fig. 20). Here, also, an arteriovenous fistulous arrangement is present, and the characteristics of aortic blood pressure and myocardial perfusion are like those in the first anomalous coronary arterial arrangement.
DISCUSSION We have considered two major anatomic forms of aortic incompetence : one through the aortic valve itself and the other, through chanClinically, nels other than the aortic valve. this distinction may be made by the location and duration of the murmurs. A to-and-fro murmur usually indicates aortic incompetence through the valve itself or through a communicaOn the tion from the aorta to the left ventricle. other hand, the continuous murmur, regardless of its location, is usually associated with some form of communication between the aorta and a right-sided cardiac structure such as the coronary sinus, right atrium, right ventricle or pulmonary trunk. THE
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allow abnormal escape of blood from the aorta The first includes those fall into two categories. conditions which, by virtue of the distortion or dilatation of the ascending aorta, cause the aortic valve to become incompetent. The route for abnormal escape of blood in these In the situations is through the aortic valve. second the aorta communicates abnormally with a cardiac structure or a mediastinal blood vessel. Congenital, traumatic and infectious conditions constitute the major backgrounds for this second type of functional derangement.
REFERENCES Pathologic aspects of cardiac 1. EDWARDS, J. E. valvolar insufficiencies. A.M.A. Arch. Sk..., 77:
634, 1958. 2. LEVY, M. J., SIEGAL, D. L., WANG, Y. and EDWARDS, J. E. Rupture of aortic valve secondary to aneurysm of ascending aorta. Circulation, 27 : 422,
1963. 3. EDWARDS, J. E. The congenital bicuspid aortic valve. Circulation, 23: 485, 1961. 4. EDWARDS, J. E., CHRISTENSEN,N. A., CLAGETT, 0. T. and MCDONALD, J. R. Pathologic considerations in coarctation of the aorta. PIOC. Staff Meet.
FIG. 20. Anomalous origin of the left coronary artery. When collaterals between the two coronary arteries become established, run-off of aortic blood occurs from the right coronary artery through the anomalously arising left artery and into the pulmonary trunk.
In general, the location of a murmur and its point of maximal intensity are of additional importance in differentiation. Murmurs of incompetence of the aortic valve are usually loudest in the so-called aortic area. If the murmur is located anywhere except in the aortic area, this finding is highly suggestive of some form of aortic incompetence which bypasses the aortic valve to communicate with a right-sided cardiac structure. The electrocardiogram may also aid in differentiating between the two anatomic forms of Signs of right ventricular aortic incompetence. (in addition to left) hypertrophy are of primary significance, since such hypertrophy would suggest a shunt from the ascending aorta to the right side of the heart. SUMMARY
Abnormal run-off of blood from the ascending aorta may result either from primary diseases of the aortic valve or from primary diseases of the ascending aorta. Primary diseases of the ascending aorta which VOLUME
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Mayo Clin., 23: 324, 1948. 5. MCGUIRE, J., SCOTT, R. C.
and GALL, E. A. Chronic aortitis of undetermined cause with severe and fatal aortic insufficiency. Am. J. M.
SC., 235: 394, 1958. 6. BAUER, W., CLARK, W. S. and KULKA, J. P. Aortitis and aortic endocarditis, an unrecognized manifestation of rheumatoid arthritis. Ann. Rhcumat.
Dis., 10: 470, 1951. 7. CLARK, W. S., KULKA, J. P. and BAUER, W.
Rheumatoid aortitis with aortic regurgitation. An unusual manifestation of rheumatoid arthritis (including spondylitis). Am. J. Med., 57: 580, 1957. 8. DAVIDSON, P., BAGGENSTOSS,A. H., SLOCUMB, C. H. and DAUGHERTY, G. W. Cardiac and aortic lesions in rheumatoid spondylitis. Proc. Staff Meet.
Mayo Clin., 38: 427, 1963. 9. TUNG, H. L. and LIEBOW, A. A.
Marfan’s syndrome. Observation at necropsy with special reference to medionecrosis of the great vessels. Lab.
Invest., 1 : 382, 1952. The cardiovascular 10. MCKUSICK, V. A.
aspects of Marfan’s syndrome: A heritable disorder of connective tissue. Circulation, 11: 321, 1955. 11. PAPAIOANNOU.A. C.. AGUSTSSON.M. H. and GASUL. B. M. Early manifestations of the cardiovascul& disorders in Marfan syndrome. Pediatrics, 27:
255 1961. 12. WAGENVOORT, C. A., NEUFELD, H. N. and EDWARDS, J. E. Cardiovascular system in Marfan’s syndrome and in idiopathic dilatation of the ascending aorta. Am. J. Cardiol., 9: 496, 1962. 13. WEAVER, W. F., EDWARDS, J. E. and BRANDENBURG, R. 0. Idiopathic dilatation of the aorta with aortic valvular insufficiency: A possible forme fruste of Marfan’s syndrome. Proc. Sta# Meet.
Mayo Clin., 34: 518, 1959.
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14. FRIEDEN, J., HURWITT, E. S. and LEADER, E. Kuptured aortic cusp associated with an heritable disorder of connective tissue. Am. .J. Med., 33: 61.5, 1962. 15. EDWARDS, J. E. An Atlas of Acquired Diseases of the Heart and Great Vessels, p. 1126. Philadelphia, 1961. W. B. Saunders Co. 16. GORE, 1. and SEIWERT, V. J. Dissecting aneurysm of the aorta. Arch. Path., 53: 121, 1952. 17. BURCHELL, H. B. Aortic dissection (dissecting hematoma;
dissecting
aneurysm
of the aorta).
Circulation, 12: 1068, 1955. 18. HUSEBYE, K. O., WOLFF, H. J. and FRIEDMAN,L. L. Aortic dissection in pregnancy: A case of Marfan’s syndrome. Am. Heart J., 55: 662, 1958. 19. JONES, A. M. and LANGLEY, F. A. Aortic sinus aneurysms. Brit. Heart J., 11: 325, 1949. 20. EDWARDS, J. E. and BURCHELL, H. B. The pathologic anatomy of deficiencies between the aortic root and the heart, including aortic sinus aneurysms. Thorax, 12 : 125, 1957. 21. SAKAKIBARA, S. and KONNO, S. Congenital aneurysm of the sinus of Valsalva. Anatomy and classification. Am. Hemt J., 63: 405, 1962. 22. ELIOT, R. S., WOLBRINK, A. and EDWARDS, J. E.
and Edwards Congenital aneurysm of the left aortic sinus. A rare lesion and a rare cause of coronary insufficiency. Circulation, 28: 951, 1963. 23. MACLEOD, A. Cardio-aortic fstula. &it. Heart J., 6: 194, 1944. 24. LEVY, M. J., LILLEHIII, c. \v., ANDERSON, R. C.,
25.
AMPLATZ, K. and EDWARDS, J. E. Aortico-left xrentricular tunnel. Circulation, 27: 841, 1963. SEGAL, E. L., BROADBENT,J. C. and EDWARDS, J. E. Cardio-aortic fI.stulas complicating bacterial endo-
carditis in a case of calcific aortic stenosis. Pm. Staf Meet. Mayo Clin., 33: 209, 1958. 26. GASUL, B. M., FELL, E. H. and CASAS, R.: The diagnosis of aortic septal defect by retrograde Report of a case. Circulation, 4: aortography. 251, 1951. 27. NEUFELD, H. N., et al. Aorticopulmonary septal defect. Am. J. Cardiol., 9: 12, 1962. 28. EDWARDS, J. E. Anomalous coronary arteries with special reference to arteriovenous-like communications. Circulation, 17: 1001, 1958. 29. NEUFELD, H. N., et al. Congenital communication of a coronary artery with a cardiac chamber or the pulmonary trunk (“coronary artery fistula”). Circulation, 24: 171, 1961.
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