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Case rePorts in Cardiology
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Figure 5 Patient No. 3 (A61–256). Diagram showing atresia of the right atrial
ostium of the coronary sinus associated with tricuspid atresia, foramen ovale type
of atrial septal defect (ASD), spontaneously closed ventricular septal defect, and a
left atrial ostium of the coronary sinus.
Figure 6 Patient No. 3. Opened right atrium (a) showing absent ostium of the
coronary sinus (dashed circle), atresia of the tricuspid valve and a large atrial septal
defect (SVC=superior vena cava; IVC=inferior vena cava), and opened left atrium,
mitral valve, and left ventricle (b). The small ostium of the coronary sinus is indicated by the dashed circle in the left atrium (b).
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Case 145 atresia of the right atrial ostium of the Coronary
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Figure 7 Patient No. 4 (GT No. 70A-353). Drawing depicting dilated coronary veins
and coronary sinus. a, anterior view; b, posterior view. The veins, particularly the posterior vein of left ventricle (PVLV) and the middle cardiac vein (MCV), and the coronary
sinus (CS) are greatly dilated. The small cardiac vein (SCV) drains into the left atrial
appendage (LAA). The site of the atretic right atrial ostium of the coronary sinus is designated by the dashed circle in b. GCV = great cardiac vein; ACV = anterior cardiac vein;
SVC=superior vena cava; IVC=inferior vena cava; Ao = aorta; PT=pulmonary trunk;
and RAA = right atrial appendage. This 63-year-old man had overt congestive cardiac
failure for 6 years before death and became functionally Class IV (New York Heart
Association Classication). He never had chest pain, precordial murmur, or systemic
hypertension. He ingested large quantities of alcohol chronically. Electrocardiogram
showed a prolonged P-R interval and complete left bundle branch block.
Figure 8 Patient No. 4. Posterior view of the heart showing the huge coronary
sinus (CS) (now opened) and marked dilatation of the posterior (P) and middle (M)
cardiac veins. The heart weighed 860 grams. The lumina of the coronary arteries
were narrowed <25 per cent. No foci of brosis or necrosis were noted in the walls
of either cardiac ventricle, both of which were quite dilated and hypertrophied. The
4 cardiac valves were normal.
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Figure 9 Patient No. 4. Opened right (a) and left sides (b) of the heart. a, The dashed
circle designates the site of the absent right atrial ostium of the coronary sinus. b, The
dashed circle designates the ostium of the coronary sinus in the left atrium.
Figure 10 Diagram showing atresia of the right atrial ostium of the coronary sinus
(CS) associated with persistence of the left superior vena cava (LSVC). Coronary
venous blood is delivered to right atrium (RA) via retrograde ow in the LSVC and
via forward ow in the innominate vein and right superior vena cava (RSVC). No
patient described herein had a persistent left SVC, but this is the most common
means of draining coronary sinus blood when the ostium in the right atrium is
absent. PV = pulmonary vein.
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REFERENCES
1. Meckel, J. F.: Über die Bildungsfehler des Herzens, Arch. Physiol. 6:549, 1805.
2. Jeffray, J.: Observations on the heart, and on peculiarities of the fetus, Glasgow, John
Smith and Son, Publisher, 1835, p.1.
3. Bauer, K.: Ein Fall von Verdoppelung der oberen Hohlvene und ein Fall von ein
Mündung des Sinus coronarius in den linken Vorhof, Morphol. Arb. 6:221, 1896.
4. Ott, M.: Ein Fall von ein Mündung des Sinus coronarius in den linken Vorhof,
Arch. Entwick-lungsmechan Organ 29:33, 1910.
5. Bredt, H.: Formdeutung und Entstehung des missgebildeten menschlichen
Herzens, Virchows Arch. 296:114, 1935.
6. Fieldstein, L. E., and Pick, J.: Drainage of the coronary sinus into the left auricle,
Am. J. Clin. Pathol. 12:66, 1942.
7. MacMahon, H. E.: Communication of the coronary sinus with the left atrium,
Circulation 28:947, 1963.
8. Mantini, E., Grondin, C. M., Lillehei, C. W., and Edwards, J. E.: Congenital anom-
alies involving the coronary sinus, Circulation 33:317, 1966.
9. Gregg, D. E., and Dewald, D.: The immediate effects of the occlusion of the coronary
veins on the dynamics of the coronary circulation, Am. J. Physiol. 124:444, 1938.
10
. Gregg, D. E.: Some problems of the coronary circulation, Verh. Dtsch. Ges.
Kreislaufforsch. 21:22, 1955.
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Case 215 Unruptured Sinus of Valsalva Aneurysm
Michael C. Fishbein, MD*, Robert Obma, MD†
and William C. Roberts, MD, FACC
Bethesda, Maryland
La Crosse, Wisconsin
An unruptured congenital sinus of Valsalva aneurysm (behind the right aortic
valve cusp) is described as an incidental necropsy nding in an 82year old man.
Review of previous reports on aneurysms involving only one of the three aortic
sinuses discloses that few cases have been described, and that these lesions are
rarely diagnosed during life. It is probable, however, that unruptured aortic
sinus aneurysm (involving only one sinus) is more common than previous
reports indicate, but that, among patients with congenital sinus aneurysm,
rupture is likely to occur.
One of the consequences of aging is dilatation of the aorta. The dilatation generally affects the ascending portion more than the descending portion, presumably
because more elastic bers are present in the proximal aorta. Included in the ascending aorta are the sinuses of Valsalva, that is, that portion of the aorta immediately
behind the aortic valve cusps. Thus, with aging each of the three aortic sinuses
dilates, and each sinus is affected more or less to a similar degree. This “senile-type
dilatation” appears to be more pronounced in hypertensive than in normotensive
persons, and tends to occur earlier in life in the hypertensive individual.
In addition to aging, there are at least three other causes of dilatation of all three
aortic sinuses: (1) syphilis,
ankylosing spondylitis.
severe aortic regurgitation, each of the three sinuses may be dilated in the absence
of aortic regurgitation. A congenital type of dilatation of all three aortic sinuses
unassociated with other congenital anomalies of the heart or great vessels has been
recorded,
5
but its occurrence, if indeed of congenital origin, must be unique.
In contrast to the common occurrence of dilatation of all three aortic sinuses,
aneurysmal dilatation of only one or two of the three sinuses is unusual. The most
common cause is probably infectious endocarditis with spread of the infective process into adjacent structures and formation of one or more ring abscesses.
always when this occurs one or more aortic valve cusps are severely damaged by the
infective process so that severe aortic regurgitation results.
Aneurysmal dilatation of only one aortic sinus unassociated with infectious
endocarditis is extremely rare. Generally, in this circumstance the localized aneurysmal dilatation is attributed to a congenital absence of media in the wall of the
1, 2
(2) the Marfan and Marfan-like syndromes,
3, 4
Although all three conditions may be associated with
1, 2
and (3)
6
Virtually
From the Section of Pathology, National Heart and Lung Institute, National Institutes of
Health, Bethesda, Md. and The Skemp-Grandview Clinic, La Crosse, Wisc. Manuscript
accepted August28, 1974.
Address for reprints: William C. Roberts, MD, Bldg. 10A, Rm. 3E30, National Institutes of
Health, Bethesda, Md. 20014.
* Present address: Department of Pathology, Harbor General Hospital, Torrance, Calif.
90509.
†
Present address: Skemp-Grandview Clinic, La Crosse, Wisc.
146 DOI: 10.1201/9781003409342-19
Case 215 unruPtured sinus of ValsalVa aneurysm
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aorta behind a sinus of Valsalva.7 The sinus aneurysm may rupture, and several
reports have described clinical consequences of the rupture and surgical procedures
for correcting it.
8–12
Surprisingly, few patients with an unruptured sinus of Valsalva
aneurysm have been described (Table 1). This report describes another patient with
a large but unruptured sinus of Valsalva aneurysm, reviews previous reports of
unruptured congenital aortic sinus aneurysms and poses certain questions, still
unanswered, about this entity.
CASE REPORT
An 82year old retired farmer, who was known to have systemic hypertension and
cardiomegaly (on chest roentgenogram) since about age 65, had the onset of typical
angina pectoris at age 70years. At age 71years he had an acute myocardial infarction. The frequency of angina pectoris decreased progressively thereafter and by
age 73years it had disappeared entirely; the blood pressure, however, remained
elevated (180/105mm Hg), exertional and nocturnal dyspnea appeared and the
P-R interval widened to more than 0.20 second. By age 74years, the blood pressure had decreased to 90/60mm Hg and antihypertensive therapy with reserpine,
given for 2years, was discontinued. At age 76years atrial brillation developed
and digitalis therapy was begun. Chest roentgenograms revealed a larger heart
than that previously recorded. At age 79years, pedal edema appeared; the blood
pressure was 110/60mm Hg, and a grade 2/6 apical blowing systolic murmur and
a third heart sound were audible. In retrospect this murmur was probably due
to tricuspid regurgitation, caused by pulmonary hypertension secondary to an
embolus. No precordial murmur had been heard previously on repeated examinations, and 2 weeks later no precordial murmur was present. The lung elds
were clear but there was marked subcutaneous pitting edema of the legs and
scrotum. Chest roentgenograms revealed an even larger heart than that recorded
at age 76years.
At age 80years the patient had a cerebrovascular accident with residual right
hemiparesis. The blood pressure was 130/90mm Hg and the edema was still severe.
In addition, a nodule that proved to be an adenocarcinoma was palpated in the prostate gland, and extensive distant metastases were found. He died suddenly several
months later in a nursing home.
At necropsy, the formalin-xed heart weighed 600 g. The sinus of Valsalva
behind the right coronary (right anterior) aortic valve cusp was aneurysmally
dilated (Figures 1 to 4). It protruded into the crista supraventricularis muscle of
the right ventricular outow tract. The right sinus was 2.5cm deep and held 15 ml
of uid; the left coronary (left anterior) and posterior (noncoronary) sinuses were
each 1.5cm deep and each held only 5 ml of uid. All four cardiac chambers were
dilated. The atrial walls contained focal endocardial waxy deposits that proved to
be amyloid. The left ventricular wall, which measured up to 1.9cm in thickness,
was rm and rubbery, and a transmural posterobasal scar was present. The lumen
of the right coronary artery was more than 75percent narrowed by atherosclerotic
plaques.
Histologic sections revealed that the wall of the aortic sinus aneurysm consisted of
endothelium covering a thin layer of connective tissue beneath which was myocardium (Figure 4). There was no aortic media within the aneurysmal wall. In addition
to the posterobasal scar, the walls of all four cardiac chambers were inltrated by
small amounts of amyloid.
COMMENTS
Pathogenesis: The basic defect in patients with aneurysms involving only one or
two of the three aortic valve sinuses is an absence of a portion or all of the media in
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Table 1: Data in previously reported patients with an unruptured congenital sinus of valsalva aneurysm affecting only one or two
aortic sinuses
Case no. Year &
Reference no.
1 1920
2 1920
3 1944
4 1949
5 1953
6 1957
7 1963
8 1963
9 1963
21
21
19
22
23
13
24
24
25
Age (yr) & Sex Clinical Problem Associated Congenital
Cause of Death Aortic Sinus Involved
Abnormalities
23M AR Discrete subaortic stenosis CHF Right
23M AR Malformed AV CHF Right + posterior
49M CHF, A-V block Aortic stenosis Arrhythmia Posterior
29M CHF, A-V block,
RVOT obst.
17F Failure to thrive as
child
75M ? 0 ? Right
7F CHF Congenital PS, VSD Operation Left
38M CHF Acquired PS, VSD Operation Right + posterior
54F Angina, AMI Bicuspid AV Compression of left
0 CHF Right + left
0 Alive Right
Left
coronary artery by
aneurysm
10 1969
11 1969
12 1972
12
12
20
16M AR PDA “Myocardial failure” Right
14F AR 0 Cerebral embolism Right
62M RVOT obst. 0 Alive Right
AMI=acute myocardial infarction; AR=aortic regurgitation; AV=aortic valve; A-V=atrioventricular CHF=congestive heart failure; PDA=patent ductus
arteriosus; PS=pulmonary stenosis; RVOT obst.=right ventricular outow tract obstruction; VSD=ventricular septal defect.
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Figure 1 Aneurysm of right sinus of Valsalva viewed from aorta (left) and right
ventricle (right). The sinus aneurysm (dashed circle, right) bulges into the crista
supraventricularis muscle. Aand S = anterior and septal tricuspid valve leaets,
respectively; OT=right ventricular outow tract; RA = right atrium; RV =right
ventricle.
Figure 2 Coronal section of heart showing aneurysm of the right (Rt) sinus of
Valsalva. If rupture had occurred, aorto-right ventricular communication would
have resulted. A = anterior mitral leaet; LA = left atrium; LV= left ventricle;
NC=noncoronary sinus of Valsalva; P=posterior mitral leaet; RAA=right atrial
appendage; RV=right ventricle; VS=ventricular septum.
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Figure 3 Close-up view of aneurysm of right sinus of Valsalva. The aortic wall
behind the sinus aneurysm is very thin. Abbreviations as in Figure2.
Figure 4 Photomicrograph of wall of sinus of Valsalva aneurysm. The aortic media is not present in the wall behind the sinus. Only thin connective tissue
over supraventricularis muscle (RV wall) separates the sinus of Valsalva and right
ventricular (RV) cavity. VS= ventricular septum. (Elastic-van Gieson stain × 4.5,
reduced by 33percent.)
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the wall of the aorta behind the sinus13 (Figure 5). Just because the media is decient,
however, does not assure the appearance of an aortic sinus aneurysm.
13, 14
Thus,
when a single sinus aneurysm occurs the sinus wall is defective, but the sinus wall
may be defective without the occurrence of an aneurysm. When a deciency of aortic media occurs, the wall involved most commonly (about 70percent of cases
that behind the right coronary (right anterior) cusp; occasionally (about 29percent of
8
) it is the portion behind the noncoronary (posterior) cusp and virtually never
cases
(less than 1percent of cases
15
) that portion behind the left coronary (left anterior)
8
) is
cusp. The explanation for these differences is uncertain.
Whether or not a sinus of Valsalva aneurysm is ever present from birth is uncertain; most likely the aneurysm is acquired, presumably the result of the aortic pressure. The higher the aortic pressure (for example, in patients with coarctation of the
aortic isthmus) the more likely is an aortic sinus aneurysm to develop. The youngest
child described with isolated sinus of Valsalva aneurysm was 4years old.
16
Although
the aneurysm itself may or may not be “congenital,” the deciency in aortic media
behind the sinus must be congenital.
Incidence of rupture: Both the natural history and the frequency of media-
decient aortic sinus wall are uncertain.
17
The number of persons with a mediadecient aortic sinus wall in whom a sinus aneurysm developed is uncertain, as
is the frequency of rupture of a developed sinus aneurysm. Among 78 cases of
sinus of Valsalva aneurysm collected from previous publications by Kieffer and
Winchell,
18
59 (76 percent) had ruptured and 19 (24percent) had not. In some of
the cases included by these authors, however, the aneurysms involved all three
sinuses and therefore were probably associated with the Marfan syndrome. Among
seven hearts with aneurysm of one aortic sinus studied at necropsy by Edwards
and Burchell,
7
four aneurysms had ruptured and three had not. Of ve single (one
of three sinuses involved) aortic sinus aneurysms studied at necropsy by us, four
Figure 5 Diagram showing possible outcome in patients with congenital absence
of a portion of media in aortic wall behind sinus of Valsalva. If no aneurysm occurs,
the congenital defect will go unnoticed. If an aneurysm does occur it may or may
not rupture. Rupture will virtually always produce clinical symptoms and signs of
cardiac dysfunction. If rupture does not occur, symptoms will usually be present
only if there is right ventricular outow obstruction or conduction disturbances.
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