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Table 1: Certain observations in 7 previously reported patients (patients 1 to 7) and in our patient (no. 8) in whom the left anterior descending
coronary artery arose from the pulmonary trunk
Pt First
Author
Year Age
(yr)&
Sex
1 Schwartz11971 7 moF — — 0 0 0 + + + 0 0 —
2
2 Probst
3 Baltaxe
4 Donaldson
5 Donaldson
6 Singh
7 Evans
8 Roberts 1984 32M — 0 0 + + 0 0 + + + 0
A=angiogram; AMI=acute myocardial infarction; AP=angina pectoris; CA=coronary artery; CE=cardiac enlargement; ECG=electrocardiogram; LAD=left anterior descending; PO=postoperatively; +=positive, present or done; 0=negative or absent;—= no information available or not done or not applicable.
1976 35F 18 AP 0 + + + 0 + 0 0 +
3
1977 18F 18 Fatigue — + 0 — + + — — —
4
1979 24F 24 AP 0 + 0 + 0 + + 0 + (12)
4
1979 26F — AP — — — — — + + 0
5
1983 45F 36 AP 0 + 0 + + + + + + (24)
6
1984 55F 37 AP + 0 0 + + + + + + (5)
Age (yr)
Onset
Symptoms
First
Symptom
AMI Precordial
Murmur
Systole Diastole
Anterior Wall
Ischemia
(ECG)
CE by
X-Ray
CA A Ligation
LAD
Conduit
Aorta to
LAD
Alive
(mo
PO)
Case rePorTs in Cardiology
Case 616 anomalous origin oF The leFT anTerior desCending Coronary arTery
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angiography 24 and 36 months, respectively, after operation; in each, the right and
LC arteries were much smaller than they had been preoperatively and the collateral
vessels between the right coronary artery and LC and the LAD had disappeared;
patient 7 had persistent angina postoperatively and the distal portions of both the
right coronary artery and LC (36 months postoperatively) were quite narrowed; the
cause of narrowing was unclear. Left ventricular angiograms, performed in 4 adults,
were normal in 2 (patients 2 and 4), and in the other 2 the apical portion of the left
ventricle was akinetic in 1 (patient 6) and aneurysmal in 1 (patient 7). Repeat left
ventricular angiography in these latter 2 patients disclosed better overall contractions in 1 and no change in 1 (patient 7).
The presence of both subjective and objective evidence of myocardial ischemia
in the 6 previously reported adults and the disappearance of angina and of the collateral vessels between the 2 coronary arteries arising from the aorta and the LAD
arising from the PT supports the view that operative treatment is proper for patients
with this coronary anomaly. Whether LAD ligation alone is enough or whether ligation plus insertion of a graft between the aorta and LAD is preferable is unclear.
Direct connection of the LAD to aorta in this situation appears technically inadvisable. All 4 patients reported in whom operation was performed had angina pectoris.
Our patient was asymptomatic preoperatively. Obviously, no data are available on
the advisibility of operation in an asymptomatic person in whom the LAD arises
from the PT, but it appears reasonable to believe that the operative therapy in this
circumstance is proper. If operation is to be performed, however, clear identication
of the anomalous artery before ligation is mandatory.
REFERENCES
1. Schwartz RP, Robicsek F. An unusual anomaly of the coronary system: Origin
of the anterior (descending) interventricular artery from the pulmonary trunk. J
Pediatr 1971;78:123–126.
2. Probst P, Pachinger O, Koller H, Niederberger M, Kaindl F. Origin of anterior
descending branch of left coronary artery from pulmonary trunk. Br Heart J
1976;38:523–525.
3. Baltaxe HA, Wixson D. The incidence of congenital anomalies of the coronary
arteries in the adult population. Radiology 1977;122:47–52.
4. Donaldson RM, Thornton A, Raphael MJ, Sturridge MF, Manuel RW.
Anomalous origin of the left anterior descending coronary artery from the pulmonary artery. Eur J Cardiol 1979;10:295–300.
5. Singh RN, Taylor PC. Anomalous origin of the left anterior descending coro-
nary artery from the pulmonary artery: Surgical correction in an adult. Cathet
Cardiovasc Diagn 1983;9:411–416.
6. Evans JJ, Phillips JF. Origin of the left anterior descending coronary artery from
the pulmonary artery. Three year angiographic follow-up after saphenous vein
bypass graft and proximal ligation. JACC 1984;3:219–224.
233
CASE REPORTS IN CARDIOLOGY
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Case 617 Asymptomatic Sinus of Valsalva
Aneurysm Causing Right Ventricular Outflow
Obstruction Before and After Rupture
Carole A. Warnes, MB, BS, MRCP, Barry J. Maron, MD,
Michael Jones, MD and William C. Roberts, MD
Sinus of Valsalva aneurysm (SVA) unassociated with ventricular septal defect does
not produce symptoms of cardiac dysfunction until the wall of the SVA ruptures
or the aneurysm itself obstructs right ventricular (RV) outow. If rupture occurs,
usually symptoms appear abruptly and congestive heart failure progresses rapidly
thereafter. Obstruction to RV outow by bulging of the SVA into the RV outow
tract has been demonstrated hemodynamically in only 2, or possibly 3, patients.
The patient described herein is unique because the degree of RV outow tract
obstruction by a SVA was observed to increase with time and symptoms of cardiac
dysfunction never occurred despite rupture of the wall of the SVA.
G.P. (#16-40-13-6), a 23-year-old man, had a precordial murmur detected at age 6 weeks.
At age 13years, a grade 4/6 systolic murmur was audible along the left sternal border. At
age 16years, the rst cardiac catheterization was performed, and RV outow obstruction
was observed (Figure 1). An aortogram showed aneurysm of the right sinus of Valsalva. The
patient remained asymptomatic and worked outdoors in construction. At age 22years, the
presence of the precordial murmur prompted a second cardiac catheterization. RV oxygen
content had increased, and the aortogram showed a perforation in the wall of the aneurysm.
Five months later, a third catheterization revealed a Qp:Qs ratio of 2.6:1 by Krypton and
3.3:1 by oximetry. Aortogram (Figure 2) conrmed the aortico-RV shunt through the ruptured SVA. Electrocardiogram was compatible with left ventricular hypertrophy. The total
QRS voltage in all 12 leads was 376mm (10mm=1mV). M-mode and 2-D echocardiograms
(Figure 2) showed discontinuity between the anterior wall of the aorta and the ventricular
septum. In May1983 the wall of the sinus of Valsalva aneurysm was excised (Figure 1) and
aortic valvuloplasty was performed. Six months later, the patient was asymptomatic, and
catheterization showed that the RV outow obstruction had been abolished. He had no leftto-right shunt, but he did have signicant regurgitation from the aorta into the left ventricle,
a nding not present preoperatively. Total 12-lead QRS voltage 6 months after operation was
379mm.
1–3
Although Sakakibara and Konno
patients in whom 1 or more sinuses of Valsalva protruded into the RV outow tract,
actual obstruction to RV outow was unconrmed because none of the 7 patients
had cardiac catheterization, or if performed, the data were not presented. It appears
that the 3 patients reported by Bulkley et al had syphilis. Only 2 patients (possibly 3)
with hemodynamically documented RV outow obstruction associated with aneurysm of 1 of the 3 sinuses, as occurred in our patient, have been reported. Gerbode et
3
described a 47-year-old woman with a 106-mm Hg peak systolic gradient across
al
From the Pathology, Cardiology and Surgery Branches, National Heart, Lung, and Blood
Institute, National Institutes of Health, Bethesda, Maryland 20205. Manuscript received and
accepted July25, 1984.
234 DOI: 10.1201/9781003409342-37
1
described 4 patients and Bulkley et al2 3
Case 617 asymPTomaTiC sinus oF ValsalVa aneurysm
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Figure1 Sinus of Valsalva aneurysm. Left, drawing of how the aneurysm obstructs
right ventricular (RV) outow. LA=left atrium; LV=left ventricle; R=right coronary
sinus. Right, wall of sinus of Valsalva aneurysm excised at operation. A small
perforation is visible.
Figure 2 a, M-mode echocardiogram showing discontinuity in the anterior aor-
tic wall; b, aortogram showing the sinus of Valsalva aneurysm (arrows); c and c
2-dimensional echocardiogram (parasternal long-axis view) showing area of discontinuity between anterior aortic wall and ventricular septum (×). The margins of the
aneurysm were not imaged in their entirety in any cross-sectional plane. The discontinuity in a almost certainly represents the mouth of the sinus of Valsalva aneurysm.
This discontinuity is present in only a portion of the cardiac cycle because movement of the heart allows the intact wall of aorta to pass into the path of the M-mode
beam. AAW=anterior aortic wall; Ao= aorta; AV=aortic valve cusp; LA=left
atrium; PAW=posterior aortic wall; RVOT=right ventricular outow tract.
235
1
,
Case rePorTs in Cardiology
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the RV outow tract associated with an SVA; although the obstruction may have been
caused in part by the SVA, an additional “infundibular stenosis” also was present.
Taguchi et al
ruptured, produced a 17-mm Hg peak systolic pressure difference between RV body
and pulmonary trunk. Kerber et al
4
described a 35-year-old man in whom the wall of the SVA, which had
5
described a 62-year-old man with a 60-mm Hg
peak systolic pressure gradient caused by an unruptured SVA. Our patient is unique
in having hemodynamic documentation of progression of RV outow obstruction
by the SVA in the RV outow tract, hemodynamic proof of elimination of the gradient after excision of the aneurysm, and no symptoms of cardiac dysfunction despite
rupture of the wall of the SVA. The lack of symptoms probably was a result of the
small size of the perforation in the aneurysm’s wall.
REFERENCES
1. Sakakibara S, Konno S. Congenital aneurysms of sinus of Valsalva. Anatomy and
classication. Am Heart J 1962;63:405–424.
2. Bulkley BH, Hutchins GM, Ross RS. Aortic sinus Valsalva aneurysms simulating
primary right-sided valvular heart disease. Circulation 1975;52:696–699.
3. Gerbode F, Osborn JJ, Johnston JB, Kerth WJ. Ruptured aneurysms of the aortic
sinuses of Valsalva. Am J Surg 1961;102:268–279.
4. Taguchi K, Sasaki N, Matsuura Y, Uemura R. Surgical correction of aneurysm of
the sinus of Valsalva. Areport of forty-ve consecutive patients including eight
with total replacement of the aortic valve. Am J Cardiol 1969;23:180–191.
5. Kerber RE, Ridges JD, Kriss JP, Silverman JF, Anderson ET, Harrison DC.
Unruptured aneurysm of the sinus of Valsalva producing right ventricular outow obstruction. Am J Med 1972;53:775–783.
236
Case 635 origin oF The righT From The leFT main Coronary
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Case 635 Origin of the Right from the Left Main
Coronary Artery (Single Coronary Ostium in Aorta)
Deborah J. Barbour, MD and William C. Roberts, MD
Origin of both the right and left main (LM) coronary arteries from the aortic wall
of the right sinus of Valsalva frequently is a lethal anomaly.1 Origin of both the right
and LM coronary arteries from the aortic wall of the left sinus of Valsalva, in contrast, usually is a benign anomaly.
of both LM and right coronary arteries from the aortic wall of the same sinus of
Valsalva, few
Husaini et al
1, 2
have described origin of the right coronary artery from the LM.
3
described angiographic features of this anomaly in a 52-year-old man
who underwent selective coronary angiography after probable acute myocardial
infarction. Muus and McManus
infant who also had a bicuspid aortic valve. Whether the coronary anomaly played a
role in the stillbirth is uncertain. In both of these previously described patients, the
anomalously arising right coronary artery coursed between aorta posteriorly and
the pulmonary trunk anteriorly.
J.G., a 65-year-old man, never had signs or symptoms of cardiac dysfunction. He did
have systemic hypertension and electrocardiographic voltage criteria consistent with left ventricular hypertrophy. He died from carcinoma of the lung. At necropsy, the heart weighed 380
g. The cardiac cavities were of normal size. No foci of myocardial brosis or necrosis were
present. The origin and courses of the coronary arteries are shown in Figure1. The anomalous right coronary artery was much smaller than either the left circumex or left anterior
descending branches; it burrowed into the myocardium of the crista supraventricularis for
about 2cm of its length shortly after its origin from the LM. All epicardial coronary arteries
were free of atherosclerotic plaque.
2
Although many studies have described origin
4
described this anomaly in a full-term stillborn
This case demonstrates that origin of the right coronary artery from the LM may
be a benign anomaly unassociated with either functional or anatomic evidence of
myocardial ischemia.
From The Pathology Branch, National Heart, Lung, and Blood Institute, National Institutes
of Health, Bethesda, Maryland 20205. Manuscript received and accepted November7, 1984.
237DOI: 10.1201/9781003409342-38
Case rePorTs in Cardiology
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Figure 1 Course of the right coronary artery after its anomalous origin from the
left main (LM) coronary artery. It burrowed into the myocardium of the crista supraventricularis for 2cm shortly after its origin. The entire right coronary artery is small
and it virtually disappeared after reaching the right margin of the heart. L=left
sinus of Valsalva; LAD= left anterior descending coronary artery; LC= left circumex coronary artery; P=posterior sinus of Valsalva; R=right sinus of Valsalva;
RVOT=right ventricular outow tract.
REFERENCES
1. Cheitlin MD, DeCastro CM, McAllister HA. Sudden death as a complication
of anomalous left coronary origin from the anterior sinus of Valsalva. Anot-sominor congenital anomaly. Circulation 1974;50:780–787.
2. Roberts WC, Siegel RJ, Zipes DP. Origin of the right coronary artery from the
left sinus of Valsalva and its functional consequences: Analysis of 10 necropsy
patients. Am J Cardiol 1982;49:863–868.
3. Husaini SN, Beaver WL, Wilson IJ, Lach RD. Anomalous right coronary artery
arising from left mainstem. Cathet Cardiovasc Diagn 1983;9:407–409.
4. Muus CJ, McManus BM. Common origin of right and left coronary arteries from
the region of left sinus of Valsalva: Association with unexpected intrauterine fetal
death. Am Heart J 1984;107:1285–1286.
238
Case 642 miTral ValVe CleFT wiThouT CardiaC sePTal deFeCT
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Case 642 Mitral Valve Cleft Without
Cardiac Septal Defect Causing Severe Mitral
Regurgitation but Allowing Long Survival
Charles W. Barth, III, MD, James D. Dibdin, MD,
LLB and William C. Roberts, MD
Partial atrioventricular “defect” includes a spectrum of 5 anatomic anomalies. Some
patients have all 5 and others have only 1 or 2. The 5 are the following: (1) defect in the
lower portion of the atrial septum, so-called primum atrial septal defect; (2)defect
in, or absence of, the posterobasal portion of ventricular septum; (3) cleft, anterior
mitral leaet; (4) anomalous chordae tendineae from the anterior mitral leaet to
the crest of the ventricular septum; and (5) partial or complete absence of the septal
tricuspid valve leaet. There are at least 4 potential functional consequences of these
5 anatomic anomalies: (1) shunt at the atrial level, (2) shunt at the ventricular level,
(3) mitral regurgitation (MR), and (4) obstruction to left ventricular outow. Well
over 95% of patients with partial atrioventricular defect have a primum type atrial
septal defect, and most of those without a primum defect have a shunt at the ventricular level. The occurrence of MRfrom a cleft in the anterior mitral leaet unassociated with a defect in either atrial or ventricular septa is extremely rare. Such was
the case, however, in the patient to be described herein.
B.P., a 27-year-old man, was found to have a precordial murmur consistent with MRat
age 16years. Atrial brillation was also present. Left ventricular angiogram at the time
showed 2+/4+ MR. He continued to play recreational basketball and work as a service station attendant without difculty. At age 27, ve months before death, he had a respiratory
infection followed by evidence of congestive heart failure. Agrade 4/6 holosystolic murmur
consistent with MRand a third heart sound were heard. M-mode echocardiogram showed
thickened but mobile mitral leaets; the left atrial dimension was 70mm, and the left ventricular cavity was 82mm in diastole and 58mm in systole. The electrocardiogram showed
atrial brillation, QRS voltage of left ventricular hypertrophy and inverted T waves in leads
II, III and aVF. The pressures in mm Hg were as follows: pulmonary artery wedge mean 15,
v wave 16; pulmonary artery 28/14 (mean 18); right ventricle 28/5; right atrial mean 5; left
ventricle 120/8; and aorta 120/80 (mean 90). Cardiac index (thermodilution) was 2.3 liters/
2
. Left ventricular angiography now disclosed 4+/4+ MR. After discharge he returned
min/m
to an active life. He died suddenly from an overdose of cocaine and phencyclidine.
At necropsy the heart weighed 900 g. All 4 cardiac chambers were dilated. Ahuge cleft
was present in the anterior mitral leaet (Figure 1). The mitral anulus measured 17cm in
circumference and the tricuspid anulus measured 15cm. The edges of the cleft were thick. The
atrial and ventricular septa were intact. The tricuspid, pulmonary and aortic valve leaets
were normal.
We found studies describing 10 patients at necropsy with a cleft in the anterior
mitral leaet unassociated with a defect in either the atrial or ventricular septum.
From the Pathology Branch, National Heart, Lung, and Blood Institute, National Institutes
of Health, Bethesda, Maryland 20205, and The Medical Examiner’s Ofce, Washington, D.C.
Manuscript received December19, 1984, accepted December31, 1984.
1–4
239DOI: 10.1201/9781003409342-39
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Figure 1 Mitral valve in the patient described. In each of the 4 views the space
bordering the cleft in the anterior mitral leaet is designated by dashed lines. a,
view from left atrium during simulated ventricular diastole; b, close-up view from
left atrium during simulated ventricular systole; c, view from left ventricle. The apex
of the cleft inserts into the crest of the ventricular septum (VS) just caudal to the
opened aortic valve (AV). The anterolateral (A-L) papillary muscle actually inserts
into the anterior one-half of the cleft anterior mitral leaet; d, opened left atrium,
mitral valve and left ventricle. The large amount of anterior mitral leaet actually
missing, i.e., that space bordered by the dashed lines, is huge. The circumference
of the mitral anulus is about twice normal. P-M=posteromedial papillary muscle.
(Photographs by M.M.M. Moore.)
Information on age and gender was available in 6 of the 10 patients: All were males;
5 were younger than age 10years and 1 was 74years old. Of the 10 patients, some
degree of MRwas present in 9; only the 74-year-old man had no MR.
3
Only 2 of the
10 patients were known to have a large cleft—such as occurred in our patient—and
both had severe MRand progressive, eventually fatal congestive heart failure.
Our 27-year-old patient appears to be the oldest necropsy patient thus far
reported with severe MRfrom a cleft in the anterior mitral leaet. Although he had
evidence of congestive heart failure for a few months before death, his death was not
the result of cardiac disease. What allowed his relatively long survival? Although he
had MRduring his entire 27years, the degree of MRworsened through the years.
Initially, MRwas probably due entirely to the regurgitation through the cleft in the
anterior mitral leaet, but with time the mitral anulus progressively dilated, and
this huge anular dilation contributed to the degree of MR, as occurs in patients with
mitral valve prolapse.
5
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Case 642 miTral ValVe CleFT wiThouT CardiaC sePTal deFeCT
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It is likely that our patient was able to tolerate the MRwell because the huge
size of the left atrial cavity was able to “absorb” the left ventricular systolic pressure
and prevent its reection into the pulmonary veins and from there into the pulmonary arteries. More than 2 decades ago, Braunwald and Awe
6
demonstrated that
patients with pure MRin whom huge left atrial cavities develop do not have pulmonary venous and subsequently pulmonary arterial hypertension; in contrast, those
patients with severe MRwhose left atrial cavities do not dilate do have severe elevation of their pulmonary venous and pulmonary arterial pressures.
7
The courses in
the former group are long and those in the latter group are short. The present patient
had a huge left atrial cavity, nearly normal pulmonary wedge pressures and normal
pulmonary arterial pressures, and therefore prolonged survival.
REFERENCES
1. Edwards JE, Dry TJ, Parker RL, Burchell HB, Wood EH, Bulbulian AH. An Atlas
of Congenital Anomalies of the Heart and Great Vessels. Springeld, IL: Charles
C Thomas, 1954:41–42.
2. Berghuis J, Kirklin JW, Edwards JE, Titus JL. The surgical anatomy of isolated
congenital mitral insufciency. J Thorac Cardiovasc Surg 1964;47:791–798.
3. Di Segni E, Edwards JE. Cleft anterior leaet of the mitral valve with intact septa.
Astudy of 20 cases. Am J Cardiol 1983;51:919–926.
4. Sellers RD, Lillehei CW, Edwards JE. Subaortic stenosis caused by anomalies of
the atrioventriciular valves. J Thorac Cardiovasc Surg 1964;48:289–302.
5. Waller BF, Morrow AG, Maron BJ, Del Negro AA, Kent KM, McGrath FJ,
Wallace RB, McIntosh CL, Roberts WC. Etiology of clinically isolated, severe,
chronic, pure mitral regurgitation. Analysis of 97 patients over 30years of age
having mitral valve replacement. Am Heart J 1982;104:276–288.
6. Braunwald E, Awe WC. The syndrome of severe mitral regurgitation with normal
left atrial pressure. Circulation 1963;27:29–35.
7. Roberts WC, Braunwald E, Morrow Ag. Acute severe mitral regurgitation sec-
ondary to ruptured chordae tendineae. Clinical, hemodynamic, and pathologic
considerations. Circulation 1966;33:58–70.
241
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