Unruptured
MICHAEL
C.
FISHBEIN,
ROBERT
OBMA,
WILLIAM
C.
Bethesda, La Crosse,
Sinus of Valsalva Aneurysm
MD*
MD+
ROBERTS,
MD,
FACC
Maryland Wisconsin
From the Section of Pathology, National Heart and Lung Institute, National Institutes of Health, Bethesda, Md. and The Skemp-Grandview Clinic, La Crosse, Wise. Manuscript accepted August 28, 1974. * Present address: Department of Pathology, Harbor General Hospital, Torrance, Calif. 90509. + Present address: Skemp-Grandview Clinic, La Crosse, Wise. Address for reprints: William C. Roberts, MD, Bldg. IOA, Rm. 3E30, National Institutes of Health, Bethesda, Md. 20014.
918
June 1975
An unruptured congenital sinus of Valsalva aneurysm (behind the right aortic valve cusp) is described as an incidental necropsy finding in an 82 year 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 fibers 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,lJ (2) the Marfan and Marfan-like syndromes,1,2 and (3) ankylosing spondylitis.3,4 Although all three conditions may be associated with 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.6 Virtually 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 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. s-l2 Surprisingly, few patients with an unruptured sinus of Valsalva aneurysm have been described (Table I). 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.
The American Journal of CARDIOLOGY
Volume 35
UNRUPTURED
TABLE
SINUS
OF VALSALVA
ANEURYSM-FISHBEIN
ET AL.
I
Data in Previously Reported Patients with an Unruptured Congenital Valsalva Aneurysm Affecting Only One or Two Aortic Sinuses
Case no.
Year & Reference no.
Age WI & Sex
1
19202’
23M
AR
2 3 4
19202’
1944’9 194922
23M 49M 29M
19532::
17F
1957’” 1963g4 196324 1963z6
75M 7F 38M 54F
AR CHF, A-V block CHF, A-V block, RVOT obst. Failure to thrive as child ?
196912 1969’2 197220
14F 62M
10 11 12
AMI = acute myocardial ductus tricular septal defect.
ure; PDA = patent
infarction; arteriosus;
Clinical Problem
CHF CHF Angina,
AMI
AR AR RVOT obst.
Sinus of
Associated Congenital Abnormalities
Aortic Sinus Involved
Cause of Death
Discrete su baortic stenosis Malformed AV Aortic stenosis 0
CHF
Right
CHF Arrhythmia CHF
Right + posterior Posterior Right + left
0
Alive
Right
0
?
Congenital PS, VSD Acquired PS, VSD Bicuspid AV
Operation Operation Compression of left coronary artery by aneurysm “Myocardial failure” Cerebral embolism Alive
Right Left Right + posterior Left
PDA 0 0
AR = aortic regurgitation; AV = aortic valve; A-V = atrioventriculal, PS = pulmonary stenosis; RVOT obst. = right ventricular outflow
Case Report An 82 year 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 70 years. At age 71 years he had an acute myocardial infarction. The frequency of angina pectoris decreased progressively thereafter and by age 73 years it had disappeared entirely; the blood pressure, however, remained elevated (180/105 mm Hg), exertional and nocturnal dyspnea appeared and the P-R interval widened to
Right Right Right
CHF = congestive tract
obstruction;
heart fail-
VSD = ven-
more than 0.20 second. By age 74 years, the blood pressure had decreased to 90/60 mm Hg and antihypertensive therapy with reserpine, given for 2 years, was discontinued. At age 76 years atria1 fibrillation developed and digitalis therapy was begun. Chest roentgenograms revealed a larger heart than that previously recorded. At age 79 years, pedal edema appeared; the blood pressure was 110/60 mm Hg, and a grade 216 apical blowing systolic murmur and a third heart sound were audible. In retrospect this murmur was probably due to tricuspid regurgitation, caused by pulmo-
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. A and S = anterior and septal tricuspid valve leaflets, respectively; OT = right ventricular outflow tract; RA = right atrium; RV = right ventricle.
June 1975
The American Journal of CARDIOLOGY
Volume 35
919
UNRUPTURED
SINUS
OF VALSALVA
ANEURYSM-FISHBEIN
ET AL.
wall. In addition to the posterobasal scar, the walls of all four cardiac chambers were infiltrated by small amounts of amyloid.
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 leaflet: LA = left atrium; LV = left ventricle; NC = noncoronary sinus of Valsalva; P = posterior mitral leaflet; RAA = right atrial appendage; RV = right ventricle; VS = ventricular septum.
nary 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 fields 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 76 years. At age 80 years the patient had a cerebravascular accident, with residual right hemiparesis. The blood pressure was 130/90 mm 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-fixed heart weighed 600 g. The sinus of Valsalva behind the right coronary (right anterior) aortic valve cusp was aneurysmally dilated (Fig. 1 to 4). It protruded into the crista supraventricularis muscle of the right ventricular outflow tract. The right sinus was 2.5 cm deep and held 15 ml of fluid; the left coronary (left anterior) and posterior (noncoronary) sinuses were each 1.5 cm deep and each held only 5 ml of fluid. All four cardiac chambers were dilated. The atria1 walls contained focal endocardial waxy deposits that proved to be amyloid. The left ventricular wall, which measured up to 1.9 cm in thickness, was firm and rubbery, and a transmural posterobasal scar was present. The lumen of the right coronary artery was more than 75 percent 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 (Fig. 4). There was no aortic media within the aneurysmal
920
June 1975
The American Journal of CARDIOLOGY
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 the wall of the aorta behind the sinus’s (Fig. 5). Just because the media is deficient, however, does not assure the appearance of an aortic sinus aneurysm., 13~14Thus, 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 deficiency of aortic media occurs, the wall involved most commonly (about 70 percent of cases8) is that behind the right coronary (right anterior) cusp; occasionally (about 29 percent of cases+) it is the portion behind the noncoronary (posterior) cusp and virtually never (less than 1 percent of caseslS) that portion behind the left coronary (left anterior) 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 4 years old.16 Although the aneurysm itself may or may not be “congenital,” the deficiency in aortic media behind the sinus must be congenital. Incidence of rupture: Both the natural history and the frequency of media-deficient aortic sinus wall are uncertain.17 The number of persons with a media-deficient 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,‘s 59 (76 percent) had ruptured and 19 (24 percent) 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 five single (one of three sinuses involved) aortic sinus aneurysms studied at necropsy by us, four had ruptured and only one, that of the present case, was unruptured. These figures indicate that among reported cases of aortic sinus aneurysm, rupture is more common than nonrupture, but the data are probably considerably biased in favor of ruptured aneurysms. The nonruptured sinus aneurysm, for example, may be missed at necropsy (it was missed by the prosector in our case). The nonruptured aneurysm, with few exceptions,1g,20 produces no evidence of cardiac dysfunction, and therefore at necropsy there may be no clinical sign or symptom to
Volume 35
UNRUPTURED
FIGURE ure 2.
3. Close-up
view of aneurysm
of right sinus of Valsalva.
SINUS
OF VALSALVA
The aor tic wall behind the sinus aneurysm
ANEURYSM-FISHBEIN
is very thin. Abbreviations
ET AL.
as in Fig-
CONGENITAL ABSENCE OF A PORTION OF MEDIA IN WALL OF AORTA BEHIND THE SINUS OF VALSALVA
J
lhieurysm
Aneurysm (of
Sinus
J Rupture
Sympioms
FIGURE 4. Photomicrograph of wall of sinus of Valsalva aneurysm. The aortic media is not present in the wall behind the sinus. Onlv 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 X4.5, reduced by 33 percent.)
of
Valsalva)
\ Never
Rupture
of Symptoms Right Ventricular Outflow Obstruction or Conduction Disturbance
Never Symptoms
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 aneurvsm 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 outflow obstruction or conduction disturbances.
June 1975
The American
Journal
of CARDIOLOGY
Volume 35
921
UNRUPTURED
SINUS OF VALSALVA
ANEURYSM-FISHBEIN
ET AL
alert the pathologist to t,he presence of an aortic sinus incidental necropsy findaneurysm. Furthermore, ings, which an unruptured aortic sinus aneurysm usually represents, are infrequently reported. Thus, it is likely that the frequency of unruptured aortic sinus aneurysm is considerably greater than has been reported. Clinical features: An unruptured single aortic sinus aneurysm on rare occasion may produce evidence of cardiac dysfunction (Table I). Kerber et a1.20 described clinical and operative findings in a 62 year
old man who had evidence of right ventricular outflow obstruction as the result of an aortic sinus aneurysm’s bulging into the crista supraventricularis. The aneurysm in our patient also bulged into the right ventricular outflow tract but not enough to cause outflow obstruction. Complete heart block has also been described in a patient with unruptured aortic sinus aneurysm. I9 The latter patient, however, also had calcific aortic stenosis, and thus it is not certain that the sinus aneurysm was responsible for the conduction defect.
References 1. Roberts WC, bangel JC, Bulkley BH: Non-rheumatic valvular cardiac disease: a clinicopathologic survey of 27 different conditions causing valvular dysfunction. Cardiovasc Clin 5:333-446, 1973 2. Roberts WC: Left ventricular outflow tract obstruction and aortic regurgitation, chap 7. In, The Heart (Edwards JE, Lev M, Abell MR, ed). Baltimore, Williams & Wilkins, 1974, p 1 lo-175 3. Bulkley BH, Roberts WC: Ankylosing spondylitis and aortic regurgitation. Description of the characteristic cardiovascular lesion from study of eight necropsy patients. Circulation 48: 1014-1027, 1973 4. Roberts WC, Holllngsworth JF, Bulkley BH, et al: Combined mitral and aortic regurgitation in ankylosing spondylitis. Angiographic and anatomic features. Am J Med 56: 237-243, 1974 5. Pomerance A, Davies MJ: Congenital aneurysms of all three sinuses of Valsalva. J Pathol Bact 89:607-610, 1965 6. Buchbinder NA, Roberts WC: Left-sided valvular active infective endocarditis. A study of forty-five necropsy patients. Am J Med 53:20-35.1972 7. Edwards JE, Burchell HB: Specimen exhibiting the essential lesion in aneurysm of the aortic sinus. Proc Mayo Clin 31:407412, 1956 8. Sawyers JL, Adams JE, Scott W: Surgical treatment for aneurysms of the aortic sinuses with aorticoatrial fistula. Surgery 41:26-42, 1957 9. Morrow AG, Baker RR, Hanson HE, et al: Successful surgical repair of a ruptured aneurysm of the sinus of Valsalva. Circulation 16:533-538, 1957 10. McGoon DC, Edwards JE, Klrklin JW: Surgical treatment of ruptured aneurysm of aortic sinus. Ann Surg 147:387-392, 1958 11. Spencer FC, Blake HA, Bahnson HT: Surgical repair of ruptured aneurysm of sinus of Valsalva in two patients. Ann Surg 152:963-968, 1960 12. Taguchi K, Sasaki N, Matsuura Y, et al: Surgical correction of aneurysm of the sinus of Valsalva: a report of forty-five consec-
922
June 3975
The American Journal of CARDIOLOGY
13.
14.
75.
16. 17. 18. 19. 20.
21. 22. 23.
24.
25.
Volume 35
utive patients including eight with total replacement of the aortic valve. Am J Cardiol 23:180-191, 1969 Edwards JE, Burchell HB: The pathological anatomy of deficiencies between the aortic root and the heart including aortic sinus aneurysms. Thorax 12:125-139, 1957 Kwittken J, Christoponlos P, Dua NK, et al: Congenital and acquired aortic sinus aneurysm. A case report of each with histologic study. Arch Intern Med 115:684-691, 1965 Sakakibara S, Konno S: Congenital aneurysm of the sinus of Valsalva. Anatomy and classification. Am Heart J 63:405-424, 1962 Fowler REL, Bevel HH: Aneurysms of the sinus of Valsalva. Pediatrics 81340-348. 1951 Steinberg I, Flnby N: Clinical manifestations of the unperforated aortic sinus aneurysm. Circulation 14:115-124, 1956 Kieffer SA, Wlnchell P: Congenital aneurysms of the aortic sinuses with cardioaortic fistula. Dis Chest 38:79-96, 1960 Duras PF: Heart block with aneurysm of the aortic sinus. Br Heart J 6:61-65, 1944 Kerber RE, Ridges JD, Kriss JP, et at Unruptured aneurysm of the sinus of Valsalva producing right ventricular outflow obstruction. Am J Med 53:775-783, 1972 Goehrlng C: Congenital aneurysm of the aortic sinus of Valsalva. J Med Res 42:49-60, 1920 Raman TK, Menon TB: Aneurysms of the sinuses of Valsalva. Indian Heart J l:l-14, 1949 Falholt W, Thomsen G: Congenital aneurysm of the right sinus of Valsalva diagnosed by aortography. Circulation 8:549-553, 1953 Gialloreto OP, Lolselle 0: Aneurysm of aortic sinus of Valsalva associated with high ventricular septal defect. Am J Cardiol 11: 537-546, 1963 Eliot RB, Wolbrlnk A, Edwards JE: Congenital aneurysm of the left aortic sinus. A rare lesion and a rare cause of coronary insufficiency. Circulation 28:951-956. 1963
MICHAEL
C.
FISHBEIN,
ROBERT
OBMA,
WILLIAM
C.
Bethesda, La Crosse,
Sinus of Valsalva Aneurysm
MD*
MD+
ROBERTS,
MD,
FACC
Maryland Wisconsin
From the Section of Pathology, National Heart and Lung Institute, National Institutes of Health, Bethesda, Md. and The Skemp-Grandview Clinic, La Crosse, Wise. Manuscript accepted August 28, 1974. * Present address: Department of Pathology, Harbor General Hospital, Torrance, Calif. 90509. + Present address: Skemp-Grandview Clinic, La Crosse, Wise. Address for reprints: William C. Roberts, MD, Bldg. IOA, Rm. 3E30, National Institutes of Health, Bethesda, Md. 20014.
918
June 1975
An unruptured congenital sinus of Valsalva aneurysm (behind the right aortic valve cusp) is described as an incidental necropsy finding in an 82 year 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 fibers 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,lJ (2) the Marfan and Marfan-like syndromes,1,2 and (3) ankylosing spondylitis.3,4 Although all three conditions may be associated with 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.6 Virtually 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 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. s-l2 Surprisingly, few patients with an unruptured sinus of Valsalva aneurysm have been described (Table I). 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.
The American Journal of CARDIOLOGY
Volume 35
UNRUPTURED
TABLE
SINUS
OF VALSALVA
ANEURYSM-FISHBEIN
ET AL.
I
Data in Previously Reported Patients with an Unruptured Congenital Valsalva Aneurysm Affecting Only One or Two Aortic Sinuses
Case no.
Year & Reference no.
Age WI & Sex
1
19202’
23M
AR
2 3 4
19202’
1944’9 194922
23M 49M 29M
19532::
17F
1957’” 1963g4 196324 1963z6
75M 7F 38M 54F
AR CHF, A-V block CHF, A-V block, RVOT obst. Failure to thrive as child ?
196912 1969’2 197220
14F 62M
10 11 12
AMI = acute myocardial ductus tricular septal defect.
ure; PDA = patent
infarction; arteriosus;
Clinical Problem
CHF CHF Angina,
AMI
AR AR RVOT obst.
Sinus of
Associated Congenital Abnormalities
Aortic Sinus Involved
Cause of Death
Discrete su baortic stenosis Malformed AV Aortic stenosis 0
CHF
Right
CHF Arrhythmia CHF
Right + posterior Posterior Right + left
0
Alive
Right
0
?
Congenital PS, VSD Acquired PS, VSD Bicuspid AV
Operation Operation Compression of left coronary artery by aneurysm “Myocardial failure” Cerebral embolism Alive
Right Left Right + posterior Left
PDA 0 0
AR = aortic regurgitation; AV = aortic valve; A-V = atrioventriculal, PS = pulmonary stenosis; RVOT obst. = right ventricular outflow
Case Report An 82 year 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 70 years. At age 71 years he had an acute myocardial infarction. The frequency of angina pectoris decreased progressively thereafter and by age 73 years it had disappeared entirely; the blood pressure, however, remained elevated (180/105 mm Hg), exertional and nocturnal dyspnea appeared and the P-R interval widened to
Right Right Right
CHF = congestive tract
obstruction;
heart fail-
VSD = ven-
more than 0.20 second. By age 74 years, the blood pressure had decreased to 90/60 mm Hg and antihypertensive therapy with reserpine, given for 2 years, was discontinued. At age 76 years atria1 fibrillation developed and digitalis therapy was begun. Chest roentgenograms revealed a larger heart than that previously recorded. At age 79 years, pedal edema appeared; the blood pressure was 110/60 mm Hg, and a grade 216 apical blowing systolic murmur and a third heart sound were audible. In retrospect this murmur was probably due to tricuspid regurgitation, caused by pulmo-
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. A and S = anterior and septal tricuspid valve leaflets, respectively; OT = right ventricular outflow tract; RA = right atrium; RV = right ventricle.
June 1975
The American Journal of CARDIOLOGY
Volume 35
919
UNRUPTURED
SINUS
OF VALSALVA
ANEURYSM-FISHBEIN
ET AL.
wall. In addition to the posterobasal scar, the walls of all four cardiac chambers were infiltrated by small amounts of amyloid.
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 leaflet: LA = left atrium; LV = left ventricle; NC = noncoronary sinus of Valsalva; P = posterior mitral leaflet; RAA = right atrial appendage; RV = right ventricle; VS = ventricular septum.
nary 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 fields 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 76 years. At age 80 years the patient had a cerebravascular accident, with residual right hemiparesis. The blood pressure was 130/90 mm 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-fixed heart weighed 600 g. The sinus of Valsalva behind the right coronary (right anterior) aortic valve cusp was aneurysmally dilated (Fig. 1 to 4). It protruded into the crista supraventricularis muscle of the right ventricular outflow tract. The right sinus was 2.5 cm deep and held 15 ml of fluid; the left coronary (left anterior) and posterior (noncoronary) sinuses were each 1.5 cm deep and each held only 5 ml of fluid. All four cardiac chambers were dilated. The atria1 walls contained focal endocardial waxy deposits that proved to be amyloid. The left ventricular wall, which measured up to 1.9 cm in thickness, was firm and rubbery, and a transmural posterobasal scar was present. The lumen of the right coronary artery was more than 75 percent 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 (Fig. 4). There was no aortic media within the aneurysmal
920
June 1975
The American Journal of CARDIOLOGY
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 the wall of the aorta behind the sinus’s (Fig. 5). Just because the media is deficient, however, does not assure the appearance of an aortic sinus aneurysm., 13~14Thus, 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 deficiency of aortic media occurs, the wall involved most commonly (about 70 percent of cases8) is that behind the right coronary (right anterior) cusp; occasionally (about 29 percent of cases+) it is the portion behind the noncoronary (posterior) cusp and virtually never (less than 1 percent of caseslS) that portion behind the left coronary (left anterior) 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 4 years old.16 Although the aneurysm itself may or may not be “congenital,” the deficiency in aortic media behind the sinus must be congenital. Incidence of rupture: Both the natural history and the frequency of media-deficient aortic sinus wall are uncertain.17 The number of persons with a media-deficient 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,‘s 59 (76 percent) had ruptured and 19 (24 percent) 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 five single (one of three sinuses involved) aortic sinus aneurysms studied at necropsy by us, four had ruptured and only one, that of the present case, was unruptured. These figures indicate that among reported cases of aortic sinus aneurysm, rupture is more common than nonrupture, but the data are probably considerably biased in favor of ruptured aneurysms. The nonruptured sinus aneurysm, for example, may be missed at necropsy (it was missed by the prosector in our case). The nonruptured aneurysm, with few exceptions,1g,20 produces no evidence of cardiac dysfunction, and therefore at necropsy there may be no clinical sign or symptom to
Volume 35
UNRUPTURED
FIGURE ure 2.
3. Close-up
view of aneurysm
of right sinus of Valsalva.
SINUS
OF VALSALVA
The aor tic wall behind the sinus aneurysm
ANEURYSM-FISHBEIN
is very thin. Abbreviations
ET AL.
as in Fig-
CONGENITAL ABSENCE OF A PORTION OF MEDIA IN WALL OF AORTA BEHIND THE SINUS OF VALSALVA
J
lhieurysm
Aneurysm (of
Sinus
J Rupture
Sympioms
FIGURE 4. Photomicrograph of wall of sinus of Valsalva aneurysm. The aortic media is not present in the wall behind the sinus. Onlv 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 X4.5, reduced by 33 percent.)
of
Valsalva)
\ Never
Rupture
of Symptoms Right Ventricular Outflow Obstruction or Conduction Disturbance
Never Symptoms
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 aneurvsm 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 outflow obstruction or conduction disturbances.
June 1975
The American
Journal
of CARDIOLOGY
Volume 35
921
UNRUPTURED
SINUS OF VALSALVA
ANEURYSM-FISHBEIN
ET AL
alert the pathologist to t,he presence of an aortic sinus incidental necropsy findaneurysm. Furthermore, ings, which an unruptured aortic sinus aneurysm usually represents, are infrequently reported. Thus, it is likely that the frequency of unruptured aortic sinus aneurysm is considerably greater than has been reported. Clinical features: An unruptured single aortic sinus aneurysm on rare occasion may produce evidence of cardiac dysfunction (Table I). Kerber et a1.20 described clinical and operative findings in a 62 year
old man who had evidence of right ventricular outflow obstruction as the result of an aortic sinus aneurysm’s bulging into the crista supraventricularis. The aneurysm in our patient also bulged into the right ventricular outflow tract but not enough to cause outflow obstruction. Complete heart block has also been described in a patient with unruptured aortic sinus aneurysm. I9 The latter patient, however, also had calcific aortic stenosis, and thus it is not certain that the sinus aneurysm was responsible for the conduction defect.
References 1. Roberts WC, bangel JC, Bulkley BH: Non-rheumatic valvular cardiac disease: a clinicopathologic survey of 27 different conditions causing valvular dysfunction. Cardiovasc Clin 5:333-446, 1973 2. Roberts WC: Left ventricular outflow tract obstruction and aortic regurgitation, chap 7. In, The Heart (Edwards JE, Lev M, Abell MR, ed). Baltimore, Williams & Wilkins, 1974, p 1 lo-175 3. Bulkley BH, Roberts WC: Ankylosing spondylitis and aortic regurgitation. Description of the characteristic cardiovascular lesion from study of eight necropsy patients. Circulation 48: 1014-1027, 1973 4. Roberts WC, Holllngsworth JF, Bulkley BH, et al: Combined mitral and aortic regurgitation in ankylosing spondylitis. Angiographic and anatomic features. Am J Med 56: 237-243, 1974 5. Pomerance A, Davies MJ: Congenital aneurysms of all three sinuses of Valsalva. J Pathol Bact 89:607-610, 1965 6. Buchbinder NA, Roberts WC: Left-sided valvular active infective endocarditis. A study of forty-five necropsy patients. Am J Med 53:20-35.1972 7. Edwards JE, Burchell HB: Specimen exhibiting the essential lesion in aneurysm of the aortic sinus. Proc Mayo Clin 31:407412, 1956 8. Sawyers JL, Adams JE, Scott W: Surgical treatment for aneurysms of the aortic sinuses with aorticoatrial fistula. Surgery 41:26-42, 1957 9. Morrow AG, Baker RR, Hanson HE, et al: Successful surgical repair of a ruptured aneurysm of the sinus of Valsalva. Circulation 16:533-538, 1957 10. McGoon DC, Edwards JE, Klrklin JW: Surgical treatment of ruptured aneurysm of aortic sinus. Ann Surg 147:387-392, 1958 11. Spencer FC, Blake HA, Bahnson HT: Surgical repair of ruptured aneurysm of sinus of Valsalva in two patients. Ann Surg 152:963-968, 1960 12. Taguchi K, Sasaki N, Matsuura Y, et al: Surgical correction of aneurysm of the sinus of Valsalva: a report of forty-five consec-
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utive patients including eight with total replacement of the aortic valve. Am J Cardiol 23:180-191, 1969 Edwards JE, Burchell HB: The pathological anatomy of deficiencies between the aortic root and the heart including aortic sinus aneurysms. Thorax 12:125-139, 1957 Kwittken J, Christoponlos P, Dua NK, et al: Congenital and acquired aortic sinus aneurysm. A case report of each with histologic study. Arch Intern Med 115:684-691, 1965 Sakakibara S, Konno S: Congenital aneurysm of the sinus of Valsalva. Anatomy and classification. Am Heart J 63:405-424, 1962 Fowler REL, Bevel HH: Aneurysms of the sinus of Valsalva. Pediatrics 81340-348. 1951 Steinberg I, Flnby N: Clinical manifestations of the unperforated aortic sinus aneurysm. Circulation 14:115-124, 1956 Kieffer SA, Wlnchell P: Congenital aneurysms of the aortic sinuses with cardioaortic fistula. Dis Chest 38:79-96, 1960 Duras PF: Heart block with aneurysm of the aortic sinus. Br Heart J 6:61-65, 1944 Kerber RE, Ridges JD, Kriss JP, et at Unruptured aneurysm of the sinus of Valsalva producing right ventricular outflow obstruction. Am J Med 53:775-783, 1972 Goehrlng C: Congenital aneurysm of the aortic sinus of Valsalva. J Med Res 42:49-60, 1920 Raman TK, Menon TB: Aneurysms of the sinuses of Valsalva. Indian Heart J l:l-14, 1949 Falholt W, Thomsen G: Congenital aneurysm of the right sinus of Valsalva diagnosed by aortography. Circulation 8:549-553, 1953 Gialloreto OP, Lolselle 0: Aneurysm of aortic sinus of Valsalva associated with high ventricular septal defect. Am J Cardiol 11: 537-546, 1963 Eliot RB, Wolbrlnk A, Edwards JE: Congenital aneurysm of the left aortic sinus. A rare lesion and a rare cause of coronary insufficiency. Circulation 28:951-956. 1963
