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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_3702_Библиотеки_им_академика_М_И_Перельмана
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Case rePorTs in Cardiology
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Figure 1 Heart from above in the 87-year-old man. The right coronary artery
(RCA) arises from the right (R) sinus of Valsalva and within a centimeter of its origin, it gives rise to the left circumex coronary artery (LCCA) and the left anterior
descending coronary artery (LADCA). The latter artery courses caudally to penetrate into the ventricular septum located posteriorly to the right ventricular outow
tract (RVOT). The LADCA enters epicardium again anterior to the ventricular septum and quickly divides into 3 branches, one of which courses in the usual location
of the LADCA. The initial portion of the RCA is very large and after origin of the
LCCA and the LADCA, the RCA is very tortuous, so tortuous that R loops around
itself. In contrast, neither the retroaortic LCCA nor the intramyocardial portion of
the LADCA is tortuous. P=posterior and L=left sinus of Valsalva.
Table 1: Clinical ndings and anomaly class of six previously reported necropsy
patients with retroaortic epicardial course of the left circumex and
anteroaortic intramyocardial course of the left anterior descending
coronary artery
Study
(Reference)
Bachdalek
Sanes
White
Schulte
Virmani
Virmani
*
See Figure2.
+=present; 0 = absent; — = no information available.
5
6
7
8
†9
9
sclerosis with severe luminal narrowing.
252
Year Pt Age (yrs),
Sex
1867 60, F
Cardiac Cause of
Death
—
Anomaly
*
Class
1
1937 4, M 0 1
1948 39, M 0 1
1985 71, F 0 1
1989 64, M + III
1989 62, M 0 IIa
†
This patient died suddenly in bed at home and at necropsy had extensive coronary athero-
Case 848 reTroaorTiC ePiCardial Course oF The leFT CirCumFlex
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Figure 2 Four drawings of the complex of retroaortic epicardial left circumex
(LC) and anteroaortic intramyocardial left anterior descending (LAD) coronary
arteries showing the possible origins of the LC and LAD from either the aorta or
from the right (R) coronary artery. In type I (left) only a single coronary artery arises
from the aorta and both LC and LAD coronary arteries arise from the right. In type
II, there are 2 coronary ostia in the right sinus of Valsalva of the aorta, the right
coronary artery and either the LAD (Type IIA) or the LC (Type IIB). No reports are
available describing the type IIB variety. In type III, there are 3 coronary ostia in the
aorta, LC, right and LAD coronary arteries.
REFERENCES
1. Voldaver Z, Neufeld HN, Edwards JE. Coronary Arterial Variations in the Normal
Heart and in Congenital Heart Disease. New York: New York Academic Press,
1975:171.
2. Neufeld HN, Schneeweiss A. Coronary Artery Disease in Infants and Children.
Philadelphia: Lea and Febiger, 1983:189.
3. Roberts WC. Major anomalies of coronary arterial origin seen in adulthood. Am
Heart J 1986;111:941–963.
4. Virmani R, Rogan K, Cheitlin MD. Congenital coronary artery anomalies:
Pathologic aspects. In: Virmani R, Forman MB, eds. Nonatherosclerotic Ischemic
Heart Disease. New York: Raven Press, 1989:153–183.
5. Bachdalek H. Anomaler Verlauf der Kranzarterien des Herzens. Virchow Arch
Pathol Anat 1867;41:260.
253
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6. Sanes S. Anomalous origin and course of the left coronary artery in a child:
So-called congenital absence of the left coronary artery. Am Heart J 1937:14:219–229.
7. White NK, Edwards JE. Anomalies of the coronary arteries: Report of four cases.
Arch Pathol Lab Med 1948:45:766–771.
8. Schulte MA, Waller BF, Hull MT, Pless JE. Origin of the left anterior descending
coronary artery from the right aortic sinus with intramyocardial tunneling to the
left side of the heart via the ventricular septum: Acase against clinical and morphologic signicance of myocardial bridging. Am Heart J 1985;110:499–501.
9. Virmani R, Chun PKC, Rogan K, Riddick L. Anomalous origin of four coronary
ostia from the right sinus of Valsalva. Am J Cardiol 1989;63:760–761.
254
Case 897 Fatal IntraperICardIal rupture oF sInus oF ValsalVa aneurysm
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Case 897 Fatal Intrapericardial Rupture of Sinus of
Valsalva Aneurysm
Katrina R. Brabham and William C. Roberts, MD
Bethesda, MD
Aneurysmal dilatation of one sinus of Valsalva unassociated with infective endocarditis is rare. Its occurrence generally is attributed to a congenital absence of media
in the aortic wall behind the sinus. When a sinus of Valsalva aneurysm ruptures, it
does so almost always into an intracardiac chamber. An exception is the case to be
described herein, where a sinus of Valsalva aneurysm ruptured outside the heart.
A 32-year-old black woman, who had been well all her life, suddenly collapsed and died
while vacuuming the oor of a house. Necropsy disclosed the pericardial sac to be lled with
blood. An aneurysm, which had perforated along its right border, was present at the aortic
root and it involved the right sinus of Valsalva (Figure 1). The wall of the aneurysm was
Figure 1 Photographs of the heart in the patient described. a, View of heart ante-
riorly. The sinus of Valsalva aneurysm (A) is located between the aorta (Ao) and right
atrium (RA). LV, Left ventricle; PT, pulmonary trunk; RV, right ventricle. b, View
after excising the anterior portions of both ventricles and of aorta. The thin-walled
aneurysm (A) is apparent. VS, Ventricular septum. c, View of the aneurysm from the
right side. Arrows show the perforation, which led to fatal hemopericardium, in the
wall of the aneurysm.
From the Pathology Branch, National Heart, Lung, and Blood Institute, National Institutes
of Health.
Reprint requests: William C. Roberts, MD, Bldg. 10, Room 2N258, National Institutes of
Health, National Heart, Lung, and Blood Institute, Bethesda, MD 20892.
4/4/24345
DOI: 10.1201/9781003409342-43
255
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much thinner than the wall of either of the other two normal-sized sinuses of Valsalva. The
aortic valve cusps were normal, as were each of the other three cardiac valves. None of the
four cardiac chambers was dilated. No myocardial foci of brosis or necrosis were present.
The coronary arteries were normal.
Most congenital sinus of Valsalva aneurysms involve only one or two sinuses.
The most common site for an aneurysm is the wall behind the right coronary cusp;
next is the wall behind the posterior cusp; the least common is the wall behind the
left coronary cusp. Rupture rarely occurs until adulthood. The subject is usually
asymptomatic because the nonruptured aneurysm usually causes no cardiac dysfunction. When rupture does occur, a large left-to-right shunt usually develops,
along with chest pain, cardiomegaly, plethoric lung elds, a continuous precordial
murmur, wide systemic arterial pulse pressures, and collapsing peripheral pulses.
Because the rupture of the aneurysm is nearly always intracardiac, death is delayed.
If, however, the sinus of Valsalva aneurysm ruptures into the pericardial sac, as in
the aforementioned patient, death is instantaneous. We are aware of only two previous reports of rupture of a congenital sinus of Valsalva aneurysm into the pericardial sac.
1, 2
REFERENCES
1. Killen DA, Wathanacharoen S, Pogson GW Jr. Repair of intrapericardial rupture of
left sinus of Valsalva aneurysm. Ann Thorac Surg 1987;44:310–311.
2. Defraigne JO, Dekoster G, Demoulin JC, Limet R. Rupture intrapéricardique d’un
anévrysme du sinus de Valsalva antéro-droit: Cas clinique et revue de la littérature. Acta Chir Belg 1988;88:369–374.
256
Case 968 prolonged surVIVal (74years) In unoperated tetralogy oF Fallot
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Case 968 Prolonged Survival (74Years)
in Unoperated Tetralogy of Fallot with
Associated Mitral Valve Prolapse
Daniel J. Fernicola, MD, Victor R. Boodhoo, MD and William C. Roberts, MD
Prolonged survival in tetralogy of Fallot is rare. About 5% of patients without operative therapy survive >25years. Recently, we studied at necropsy a man with tetralogy of Fallot who survived 74years without operation and at necropsy severe mitral
valve prolapse also was present. A description of pertinent ndings in him are
described herein.
S.D., a 74-year-old white man, who worked in a post ofce as a mail sorter, was cyanotic
at birth and had recurrent syncope until the age of 8years. He was then asymptomatic until
age 43 when he developed numbness in his left hand and it recurred. At age 46, the rst of
2 cardiac catheterizations was performed and the results are summarized in Table 1. Atrial
brillation began at age 58, and left ventriculogram at age 68 disclosed moderate mitral
regurgitation. From age 46 to 55 he had episodic upper gastrointestinal tract bleeding from a
gastric ulcer, and on 1 occasion his blood hematocrit was reduced to 24%. Signs and symptoms of congestive heart failure began at age 73, about 17 months before death. Examination
at that time disclosed a grade 4/6 systolic murmur, loudest along the upper left sternal border. His ngers and toes were clubbed and cyanotic. The blood hematocrit was 36% and
the systemic oxygen saturation was 80%. An electrocardiogram showed atrial brillation,
Table 1: Cardiac catheterization data in the patient described
Site Age (years) at Study
Right atrium (mean) (mm Hg) 6 10
Right ventricle (s/d) (mm Hg) 95/0
Pulmonary artery (s/d) (mm Hg) 25/0 35/12
Pulmonary artery wedge (mean) (mm Hg) 16 21
Left ventricle (s/d) (mm Hg) — 105/15
Systemic artery (s/d) (mm Hg) 110/50 105/70
Systemic O
Blood hematocrit (%) 49 40
Coronary angiogram — Single ostium
Hospitalized for upper gastrointestinal bleed 5 months before catheterization.
*
O
=oxygen; psg=peak systolic gradient; s/d=peak systole/end diastole.
2
From the Pathology Branch, National Heart, Lung, and Blood Institute, National Institutes of
Health, Bethesda, Maryland, and the Parrish Medical Center, Titusville, Florida. Manuscript
received August18, 1992; revised manuscript received and accepted September21, 1992.
saturation (%) 89 83
2
46 68
105/10
}
psg=70
psg=70
}
*
257DOI: 10.1201/9781003409342-44
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Figure 1 Electrocardiogram of the patient recorded 17 months before death.
incomplete right bundle branch block, right ventricular hypertrophy, and nonspecic ST-Twave changes (Figure1). He died of progressive congestive heart failure.
At necropsy, the heart weighed 860 g and the typical features of tetralogy of Fallot, namely
ventricular septal defect and right ventricular outow obstruction were present (Figures2
and 3). Both mitral leaets were thickened and both, the posterior more than the anterior, protruded abnormally into the left atrium. Only 1 coronary ostium was present in the aorta and
it was located in the left aortic sinus (Figure4). Aradiograph of the heart specimen disclosed
heavy calcic deposits in the left circumex and in its continuation as the right coronary
artery, but insignicant luminal narrowing was present (Figure5). The epicardial coronary
arteries were much more dilated than expected (for age 74years). All 76 ve-mm segments of
the epicardial coronary arteries were narrowed <50% in cross-sectional area despite heavy
calcic deposits within some portions of the walls.
This patient had classic tetralogy of Fallot with a large ventricular septal defect
and severe subpulmonic and pulmonic valve obstruction. The pulmonic valve had
a unicuspid structure. Additionally, the patient had a single coronary ostium in the
aorta, and the very dilated epicardial coronary arteries were devoid of signicant
narrowing despite heavy calcic deposits. The mitral valve had typical anatomic
features of prolapse and the valve was incompetent. Both atria were quite dilated
and atrial brillation was present.
At least 20 patients >40years of age with unoperated tetralogy of Fallot and
studied at necropsy have been reported (Table 2).
1–18
In contrast to our patient, none
258
Case 968 prolonged surVIVal (74years) In unoperated tetralogy oF Fallot
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Figure 2 The heart at necropsy after an anteroposterior longitudinal cut. The ventricular septal defect is located just caudal to the overriding aorta. The right ventricular (RV) outow tract is narrowed. Both mitral leaets (arrows) prolapse into the
dilated left atrium (LA). The left ventricular (LV) cavity is moderately dilated. The
left circumex (LC) coronary artery is very dilated.
Figure 3 Close-up view of the stenotic pulmonic valve from the arterial side.
259
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Figure 4 Diagram illustrating the origin and course of the coronary arteries.
Asingle coronary ostium is in the left (L) anterior aortic sinus. The left main coronary artery (LMCA) bifurcates into the left circumex coronary artery (LCCA) and
left anterior decending coronary artery (LADCA). The LCCA continues in the left
atrioventricular groove to supply the posterior left ventricular wall and right ventricular wall. Aportion of the LADCA was tunnelled. The relative sizes of the aortic
valve (AV) and the unicuspid, unicommissural, stenotic pulmonic valve (PV) are
also shown. P=posterior; R=right.
260
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Figure 5 Coronary arteries. a, radiograph of the intact heart showing calcic
deposits in the epicardial coronary arteries, pulmonic valve (PV) and aortic valve.
The left circumex (LC) coronary artery is heavily calcied. b, radiograph of the
excised coronary arteries showing extensive calcic deposits in the LC coronary
artery. c, photomicrograph of a section indicated by the white line of the most dilated
portion of the LC coronary artery. (Original magnication ×9, reduced by 27%.)
Large calcic plaques are present, but the lumen is widely patent. LM=left main;
LAD=left anterior descending.
of the 20 patients at necropsy had mitral valve prolapse, and none had a coronary
artery anomaly; only 1 survived into the eighth decade, and only 3 had had atrial
brillation. One (no. 20, Table 2) of the 20 patients was reported to have had mitral
valve prolapse with severe mitral regurgitation by M-mode echocardiogram, but the
mitral prolapse was not conrmed at necropsy.
18
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