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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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Case 604 Atrioventricular Septal Defect
(Primum Atrial Septal Defect) with Prolonged
Survival (Despite Severe Mitral Regurgitation
and Pulmonary Hypertension) and Associated
Cardiac Calcification (Mitral Anulus,
Coronary Artery and Pulmonary Trunk)
Carole A. Warnes, MB, BS, MRCP, Gerald I. Shugoll, MD,
Robert B. Wallace, MD and William C. Roberts, MD
Atrioventricular (AV) septal defect (formerly called AV canal or endocardial cushion
defect or primum atrial septal defect [ASD] with cleft mitral valve) may be either
complete (single AV valve) or partial (2 well formed AV valves). Patients with complete AV septal defect rarely survive for more than a few years without operation.
However, patients with partial AV septal defect often survive for many years, but
not as long as patients with secundum ASD. Prolonged survival with partial AV
septal defect (primum ASD), however, is unusual, particularly in the presence of
severe mitral regurgitation and pulmonary hypertension, but such was the case in
the patient described herein.
S.C., a 59-year- old woman, was found to have a precordial murmur in childhood. Episodic
palpitations began at age 20years and an episode of atrial brillation was documented when
she was 37years old. Although easily fatigued, she performed housework until her early 50s,
when palpitations associated with dyspnea became more frequent and pedal edema developed.
At age 59years, orthopnea and angina pectoris appeared. The blood pressure was 110/70mm
Hg. Aright ventricular lift was palpated, P
was wider than normal and did not vary with respiration. Agrade 3/6 systolic murmur was
present at the apex and it radiated into the left axilla. A grade 2/6 systolic murmur along
the lower left sternal border increased in intensity with inspiration. The electrocardiogram
showed sinus rhythm with prolonged PR interval (0.24 s), right bundle branch block and
left-axis deviation. The Holter monitor tracing showed both supraventricular and ventricular ectopic complexes with episodes of bigeminy and a 4-beat run of ventricular tachycardia. Chest radiography showed cardiomegaly with pulmonary congestion. The ndings on
cardiac catheterization are summarized in Table 1. Left ventriculography demonstrated a
normally contracting left ventricle, a narrowed outow tract with the characteristic “gooseneck” deformity, no ventricular septal defect, severe (grade 3+/4+) mitral regurgitation, and
thickened, deformed, relatively immobile mitral leaets. Calcium was visible in the mitral
anular region. Pulmonary venogram demonstrated an ostium primum ASD. The pulmonary to systemic ow ratio was 1.7 and the net left-to-right shunt was 2.5 liters/min. The
pulmonary resistance was 7 Wood units/m
2
. Coronary arteriography and, later, necropsy demonstrated a 50 to 75% diameter reduc-
m
tion in the midportion of the left anterior descending coronary artery. The ostium of the right
coronary artery was located in the left coronary sinus (conrmed at necropsy).
From the Pathology Branch, National Heart, Lung, and Blood Institute, National Institutes
of Health, Bethesda, Maryland, and Georgetown University Medical Center, Washington, D.C.
Manuscript received May8, 1984, accepted May16, 1984.
was loud and the interval between A2 and P2
2
2
and the systemic resistance was 28 Wood units/
222 DOI: 10.1201/9781003409342-34
Case 604 aTrioVenTriCular sePTal deFeCT (Primum aTrial sePTal deFeCT)
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Table 1: Hemodynamic data
Chamber Pressure (mm Hg) s/d Oxygen Saturation (%)
IVC ... 69
SVC ... 53
RA mean 22 70
RV (s/d) 95/15 77
PA (s/d) 95/30 77
LA mean 20 85
LV (s/d) 120/20 83
Aorta (s/d) 120/65 83
IVC=inferior vena cava; LA=left atrium; LV=left ventricle; PA=pulmonary artery;
RA=right atrium; RV=right ventricle; s/d=peak systole/end-diastole; SVC=superior
vena cava.
On January26, 1984, the deformed mitral valve was replaced with a 3M Starr-Edwards
prosthesis, the primum ASD was closed with a pericardial patch, a de Vega tricuspid valve
anuloplasty was performed, and a saphenous vein was inserted between the aorta and left
anterior descending coronary artery (done rst). Although atrial brillation developed, the
early postoperative course was uneventful. She went home on February4, 1984. Two days
later she had an acute episode of dyspnea, and she died about 12hours later.
At necropsy, the heart weighed 650g. The defect (4cm in maximal diameter) in the lower
most portion of the atrial septum had been closed securely, 1 of the 4 stents of the prosthesis
in the mitral position contacted the left ventricular wall, and the aortocoronary conduit was
patent (Figures 1 and 2). Heavy calcic deposits were present in the mitral anulus, pulmonary trunk and left anterior descending coronary artery (Figure 3). No clear anatomic cause
of death was discernible at necropsy.
Few reported patients with partial AV septal defect have survived for more than
50years. One man with this defect, conrmed at necropsy, lived 69years.
2
reported 52 such patients aged 20years or older, 18 of whom were 45years or
et al
older and 6 of whom were 60years or older. Pulmonary hypertension was rare; the
highest mean pulmonary artery pressure was 55mm Hg. Somerville
patients aged 30years or older with primum ASD, 2 of whom had pulmonary hypertension (pulmonary vascular resistance ≥3 Wood units). Severe mitral regurgitation
also was unusual in the patients of Hynes et al: Of 33 patients who underwent left
ventricular angiography, mitral regurgitation was found in 4 (12%). In Somerville’s
14 patients, severe mitral regurgitation was present in 2. Martin et al
adults, aged 34 to 48years, with primum ASD; 1 had an elevated pulmonary artery
pressure (86/33mm Hg), and mitral regurgitation was minimal in all 7. Goodman et
5
reported 12 patients aged 15 to 53years (mean 30) with primum ASD: 1 had a pul-
al
monary artery systolic pressure >50mm Hg, and 2 had severe mitral regurgitation.
Although recognized as frequent in patients with mitral valve prolapse, which
in turn is recognized as frequent in older patients with secundum type ASD, mitral
anular calcium is infrequent in patients with secundum ASD,
survival longer than 65years is relatively uncommon) and it has never been reported
in a patient with primum ASD. The occurrence of signicant coronary atherosclerosis, also present in our patient, has not been described previously in a patient with
primum ASD, and obviously, aortocoronary bypass grafting, as performed on our
patient, has not been reported previously in such a case. Moreover, the occurrence
of a calcied mass in the pulmonary trunk has not been reported previously in a
1
Hynes
3
reported 14
4
described 7
6
(presumably because
223
Case rePorTs in Cardiology
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Figure 1 Opened right side of the heart (left) exposing the operatively closed
atrial septal defect (“ASD”). The right atrium (RA) is dilated and the wall of right
ventricle (RV) is markedly thickened. Right, opened left atrium (LA) showing the
operatively closed ASD with a portion of the prosthetic ring attached to the most
caudal margin of the ASD. The arrow points toward the aortic valve. CS=coronary
sinus; LV=left ventricle.
Figure 2 Left side of the heart. a, radiograph. Calcium (Ca++) is present in the
mitral anular region. b, long-axis view showing relative sizes of the right ventricle
(RV), left ventricle (LV), left atrium (LA) and the relative thicknesses of the ventricular walls. The aortocoronary artery conduit is visible. c, close-up of the prosthesis. d, close-up of left ventricular outow tract. The anterior mitral leaet (broken
arrows) is displaced anteriorly toward the ventricular septum (VS), and this displacement appears to have diminished the area of left ventricular outow (solid
arrow). Ao=ascending aorta; AV=aortic valve.
224
Case 604 aTrioVenTriCular sePTal deFeCT (Primum aTrial sePTal deFeCT)
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Figure 3 a, calcic deposit (Ca++) in pulmonary trunk (PT), probably originally a
thrombus, and in the mitral valve anulus (MVA) and left anterior descending coronary artery (LAD). b, close-up of the calcic deposit in the pulmonary trunk located
just above the pulmonic valve (PV).
patient with primum ASD. Thus, the patient described herein is unusual on 7 counts
(prolonged survival, severe mitral regurgitation, severe pulmonary hypertension,
mitral anular calcium, pulmonary truncal calcium, coronary arterial calcium and
severe coronary atherosclerosis necessitating aortocoronary bypass grafting).
REFERENCES
1. Heath D. Long survival in partial persistent common atrioventricular canal. Br J
Dis Chest 1968;62:207–210.
2. Hynes JK, Tajik AJ, Seward JB, Fuster V, Ritter DG, Brandenburg RO, Puga
FJ, Danielson GK, McGoon DC. Partial atrioventricular canal defect in adults.
Circulation 1982;66:284–287.
3. Somerville J. Ostium primum defect: Factors causing deterioration in the natural
history. Br Heart J 1965;27:413–419.
4. Martin CE, Thomas CS, Bender HW. Ostium primum atrial septal defect in the
adult. South Med J 1976;69:1058–1060.
5. Goodman DJ, Harrison DC, Schroeder JS. Ostium primum defect in the adult:
Postoperative follow-up studies. Chest 1975;67:185–189.
6. Roberts WC. Morphologic features of the normal and abnormal mitral valve. Am
J Cardiol 1983;51:1005–1028.
225
CASE REPORTS IN CARDIOLOGY
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Case 605 “Massive” Calcification of a Right
Ventricular Outflow Parietal Pericardial
Patch in Tetralogy of Fallot
Elizabeth M. Ross, MD, Charles L. Mclntosh,
MD, PhD and William C. Roberts, MD
A variety of materials have been used for patches to widen obstructed right ventricular (RV) outow tracts in patients with tetralogy of Fallot. These materials
have included Teon®, preclotted Dacron®, parietal pericardium and dura mater.
Autologous parietal pericardium has the advantage of being readily available, and
does not present the problem of suture-line bleeding, which often occurs with the
synthetic patches. Both tissue and synthetic patches utilized in the RV outow tract
may become aneurysmal if the RV peak systolic pressure is not returned to normal
or near normal levels after operation. Although the intimal lining tissue of the synthetic patch may calcify, the synthetic material itself does not. In contrast, autologous patch material may calcify. Such was the case in the patient described here.
D.H. (#11-37-22), a 16-year-old boy with tetralogy of Fallot, had a Blalock-Taussig anastomosis at age 5years. He had closure of the ventricular septal defect with insertion of a parietal pericardial RV outow widening patch at age 9 after catheterization had shown the peak
systolic pressure gradient between RV body (105/12mm Hg) and pulmonary trunk (16/6mm
Hg) to be 89mm Hg. Another cardiac catheterization was not done until age 16years, when
the peak systolic pressure gradient between RV body (70/2mm Hg) and pulmonary trunk
(15/3mm Hg) was 55mm Hg. Chest radiograph showed a large calcic deposit in the RV outow tract (Figure 1). At reoperation, the RV outow patch was heavily calcied (Figure2). It
was excised and replaced with a larger patch consisting of Teon.
Review of several reports
or a tissue patch into the RV outow tract months to years earlier disclosed no mention of grossly visible calcic deposits of the material of the patch itself. Such heavy
calcic deposits in our patient may have resulted, in part, from the residual RV outow gradient which persisted after operation.
From the Pathology and Surgery Branches, National Heart, Lung, and Blood Institute,
National institutes of Health, Bethesda, Maryland 20205. Manuscript received and accepted
May30, 1984.
226 DOI: 10.1201/9781003409342-35
1–6
of patients who underwent insertion of a synthetic
Case 605 “massiVe” CalCiFiCaTion oF a righT VenTriCular ouTFlow PaTCh
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Figure 1 Chest radiograph (left, posteroanterior and right, lateral) at age 16years
showing calcium in the area of the ventricular outow tract.
Figure 2 Photograph and radiograph of calcied, operatively excised right ventricular outow patch.
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REFERENCES
1. Chiariello L, Meyer J, Wukasch DC, Hallman GL, Cooley DA. Intracardiac
repair of tetralogy of Fallot. Five-year review of 403 patients. J Thorac Cardiovasc
Surg 1975;70:529–535.
2. Seybold-Epting W, Chiariello L, Hallman GL, Cooley DA. Aneurysm of pericar-
dial right ventricular outow tract patches. Ann Thorac Surg 1977;24:237–240.
3. Kirklin JW, Bargeron LM, Pacico AD. The enlargement of small pulmonary
arteries by preliminary palliative operations. Circulation 1977;56:612–617.
4. Arciniegas E, Farooki ZQ, Hakimi M, Perry BL, Green EW. Early and late results
of total correction of tetralogy of Fallot. J Thorac Cardiovasc Surg 1980;80:770–778.
5. Fuster V, McGoon DG, Kennedy MA, Ritter DG, Kirklin JW. Long-term evalu-
ation (12 to 22years) of open heart surgery for tetralogy of Fallot. Am J Cardiol
1980;46:635–642.
6. Lane I, Treasure T, Leijala M, Shinebourne E, Lincoln C. Diminutive pulmonary
artery growth following right ventricular outow tract enlargement. Int J Cardiol
1983;3:175–189.
228
Case 616 anomalous origin oF The leFT anTerior desCending Coronary arTery
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Case 616 Anomalous Origin of the Left Anterior
Descending Coronary Artery from the Pulmonary
Trunk with Origin of the Right and Left
Circumflex Coronary Arteries from the Aorta
William C. Roberts, MD and Max Robinowitz, MD
Origin of both right and left main (LM) coronary arteries from the pulmonary trunk
(PT) rarely allows survival for more than 2 weeks after birth. Origin of the LM from
the PT with origin of the right coronary artery from the aorta allows longer survival,
but usually (80%) for no more than 1year after birth. Origin of the right coronary
artery from the PT and the LM from the aorta, in contrast, allows survival into adulthood and maybe a normal life span. Because the LM is equivalent to 2 major coronary arteries, whenever the LM arises from the PT, whether in association with the
right artery or when isolated, survival is short. However, when 1 major branch of
the LM arises from the PT and both the right and the other major LM branch arises
from the aorta, the survival rate should be similar to that in patients in whom the
right coronary artery arises from the PT and the LM from the aorta. In this report we
describe a man in whom the left anterior descending coronary artery (LAD) arose
from the PT and the right and left circumex (LC) coronary arteries from the aorta.
T.J., a 32-year-old man, died suddenly soon after jogging on December29, 1981. In
November1979 a continuous murmur ‘‘similar to that of a patent ductus arteriosus” had
been heard during routine physical examination. He was and always had been asymptomatic. The murmur was grade 3/6 in intensity, and loudest in the third left intercostal space.
The systemic blood pressure was 120/50mm Hg. The electrocardiogram was normal. Chest
radiograph showed the cardiac silhouette to be at the upper limits of normal. On the M-mode
echocardiogram, the left ventricular cavity in end-diastole was 65mm and the left atrium
was 40mm. Coronary angiography disclosed the origin of the right artery from the right
sinus and the origin of only the LC from the left sinus of Valsalva. Both the right coronary
artery and LC communicated through collateral vessels with the LAD, which arose from the
PT; it was dilated and tortuous. The ejection fraction on the left ventricular angiogram was
48%. The pulmonary to systemic ow ratio was 1.6:1.
On March9, 1981, thoracotomy was performed for the purpose of ligating the LAD close
to its origin from the PT and insertion of saphenous vein graft from aorta to the more distal
portion of the LAD. At operation, rather than ligating the LAD, the proximal LC was ligated
instead (Figure 1). On the second postoperative day, the continuous precordial murmur was
still audible and repeat angiography disclosed that both the LAD and the graft were widely
patent. Reoperation was performed on March12, 1981, and this time the LAD near the PT
was ligated (Figure 1).
Reevaluation in May1981 disclosed mild exertional dyspnea postoperatively, but never
chest pain. The patient was working full days as a dentist. The blood pressure was 130/85mm
Hg. No precordial murmurs were present. Electrocardiography disclosed Q waves in leads
From the Pathology Branch, National Heart, Lung, and Blood Institute, National Institutes
of Health, Bethesda, Maryland, and the Cardiovascular Pathology Department, Armed Forces
Institute of Pathology, Washington, DC. Manuscript received and accepted July24, 1984.
229DOI: 10.1201/9781003409342-36
Case rePorTs in Cardiology
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Figure 1 Sequence of development before, during and after operation in the
patient described. Ao=aorta; AV=aortic valve; PV=pulmonic valve; LAD, LC and
R=left anterior descending, left circumex and right coronary arteries.
Iand aVL and inverted T waves in lead aVL. The Bruce treadmill test to 10 minutes disclosed
a heart rate of 175 beats/min, blood pressure 155/75mm Hg, no symptoms and no ischemic electrocardiographic changes. No symptoms occurred thereafter and he began running
2 miles daily and playing tennis. On December29, 1981, he complained of dyspnea shortly
after a run and died.
Necropsy disclosed the heart weight at 450g. The proximal LAD was occluded by a
thrombus and ligature and the graft was patent (Figure 1). Atransmural scar involved the
lateral wall of the left ventricle and ventricular septum at the base and both papillary muscles
were focally scarred (Figure 2).
At least 7 patients (Table 1) have been reported in whom the LAD arose from the
PT and both right and LC arteries arose from the aorta.
230
1–6
At the time of the reports
Case 616 anomalous origin oF The leFT anTerior desCending Coronary arTery
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Figure 2 Transverse section of cardiac ventricles at level of left ventricular papillary muscles showing transmural left ventricular scar. LV=left ventricle; RV=right
ventricle.
1 patient (patient 1, Table 1), a 7-month old girl, had died of an anterior wall acute
myocardial infarct; the other 6 (patients 2 to 7, all women) were aged 18 to 55years
(mean 34). Our patient is the only male thus far described. Of the 6 adults, all were
symptomatic: 5 with angina pectoris, 1 of whom also had an anterior wall acute
myocardial infarct, and 1 with severe fatigue attributed to severe mitral regurgitation from papillary muscle dysfunction. The angina at some time in all 5 patients
was stable, but 3 of the 5 (patients 4, 6 and 7) had unstable angina just before cardiac
operation. The age at onset of symptoms of myocardial ischemia in the 6 adults
ranged from 18 to 37years (mean 27). Precordial murmurs were described in 4 of
5 previously reported adults. (No information was provided in the “addendum
case” of Donaldson et al.
4
): The murmur apparently was present only in systole in 4
patients, and also in diastole in 1. The intensity of the murmurs was mentioned in
2 patients: “soft” in 1 (patient 4) and grade 2/6 in 1 (patient 6). Findings on the electrocardiogram at rest were described in 4 adults: All had poor R-wave progression
in leads V
to V3 and at least 2 (patients 6 and 7) had ST–T-wave changes of ischemia
1
in more than 1 lead. Exercise stress tests in 2 patients (patients 2 and 7) disclosed
ST-segment ischemic changes in each. Chest x-rays in 5 of the 6 adults disclosed normal-sized cardiac silhouettes in 2 and cardiac enlargement in 3: mild in 2 (patients
4 and 6) and severe in 1 (patient 3). Right-sided cardiac catheterization, performed
in at least 5 patients (patients 2 and 4 to 7) disclosed normal pressures in each and
oxygen step-up in the pulmonary trunk in only 1 (patient 6). Coronary angiography
with injection of contrast material in the right coronary artery and LC in all 6 adults
disclosed that each of these 2 arteries in all 6 patients was large, occasionally also
tortuous, and that the LAD was lled with extensive collateral vessels from both
the right coronary artery and LC. Injection of contrast material into the PT did not
cause lling of the LAD; when the LAD, however, was lled by injections into either
the right coronary artery or LC, contrast material did enter the PT through the LAD,
which was lled by collateral vessels.
Of the 6 previously reported adults, operative treatment was carried out in 4,
all of whom preoperatively had angina: In 2 patients (patients 4 and 5) the PT was
opened and the ostium of the LAD was obliterated by sutures, and in 2 (patients 6
and 7) the LAD was ligated just proximal to its entrance into the PT and a reversed
saphenous vein was inserted from the ascending aorta to the LAD. Of the 4 patients
who had operative treatment, angina disappeared in 3 (patients 4 to 6) and persisted in 1 (patient 7). Patients 6 and 7 had repeat coronary and left ventricular
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