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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 Patient 1. ECG showing atrial brillation and complete right bundle
branch block.
Figure 2 Patient 1. Chest roentgenogram showing considerable cardiomegaly and
prominent main pulmonary arteries.
At operation (June1979), the dome-shaped stenotic pulmonic valve was incised, and
the large (4.5 × 3.0-cm) secundum type atrial septal defect was closed with a patch.
The early postoperative course was uneventful, and since the early postoperative
period she has been asymptomatic.
COMMENTS
Each of the two women described had pulmonic-valve stenosis (PS) and atrial septal defect (ASD) with large left-to-right shunts at the atrial level. Neither had ever
had cyanosis, even with strenuous exertion. One lived 69years without symptoms
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Case 399 PulmoniC ValVe stenosis with intaCt VentriCular sePtum
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Figure 3 Patient 1. Drawing showing the major cardiac ndings at necropsy.
Right atrial (RA) and right ventricular (RV) cavities severely dilated; left atrial (LA)
and left ventricular (LV) cavities normal in size. From anterior aspect (A), left ventricle was not visible, and only appendage of left atrium was visible. Secundum type
atrial septal defect (ASD) was approximately 2.7cm in largest diameter. Orice of
pulmonic valve was eccentric, stenotic, and valvular cusps, severely thickened by
brous tissue and calcic deposits. Pulmonic trunk (PT) dilated. Calcic deposits
also present in the tricuspid-valve annulus. IVC and SVC=inferior and superior
vena cava, respectively; Ao=ascending aorta.
Figure 4 Patient 2. ECG showing sinus rhythm and complete right bundle branch
block.
of cardiac dysfunction, and the second was asymptomatic until the age of 48years.
At necropsy or operation, both had stenotic pulmonic valves and large secundum
type ASD.
Each of our two patients demonstrated typical morphologic features produced
by the combination of PS and ASD with left-to-right shunting, but both are unique
because of their age. Recently, Roberts and associates
1
reviewed hemodynamic and
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Figure 5 Patient 2. Chest roentgenograms, posteroanterior view (left) and lateral
view (right) showing considerable enlargement of major pulmonary arteries.
Table 1: Hemodynamic ndings in patient 2
Pulmonary artery (s/d) 20/5 92.2
Right ventricle (s/d)
Outow 70/1 ...
Inow 77/1 78.7
Right atrium
Mean: A:V 0:5:1 76.2
Superior vena cava ... 68.6
Inferior vena cava ... 74.0
Left atrium
Mean: A:V 0:5:1 96.6
Left ventricle (s/d) 120/1 ...
Aorta (s/d) 120/56 94.4
Pulmonic valve area (index) 1.2cm2 (0.8cm2/M2)
Left-to-right shunt 1.8 liters/minute
operative ndings in 30 patients with PS and ASD: in 19 (63percent), the shunt at
the atrial level was exclusively, or nearly so, right-to-left, and in the other 11 (37percent), the shunt was entirely, or nearly so, left-to-right. The ages at operation of the
19 patients with right-to-left shunts ranged from 3 to 43years (mean, 19years), and
the ages of the 11 patients with left-to-right shunts ranged from 4 to 28years (mean,
14years). Of the 30 patients, 21 (70percent) were female, including seven of the 11
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Pressure, mm Hg Oxygen Saturation, %
Case 399 PulmoniC ValVe stenosis with intaCt VentriCular sePtum
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with entirely left-to-right shunts. The explanation for the differing direction of the
shunting at the atrial level in the 30 patients was the size of the defect in the atrial
septum and the degree of obstruction to right ventricular outow. The patients with
right-to-left interatrial shunting had small (average diameter, 1.1 ± 0.1-cm) sized
defects in the atrial septum (patent foramen ovale) and severe pulmonic valve stenosis (average peak systolic pressure gradient, 120 ± 11mm Hg). In contrast, the
patients with left-to-right shunts had mild to moderate pulmonic valve stenosis
(average peak systolic pressure gradient, 60 ± 5mm Hg) and relatively large (average diameter, 2.8 ± –.1cm) defects in the atrial septum (true atrial septal defect). Both
of our patients had secundum atrial septal defects larger than 2.5cm in diameter,
and both had moderate pulmonic valve stenosis (54mm Hg peak systolic pressure
gradient in patient 2). Surprisingly, the patients described earlier by Roberts and
associates
1
with right-to-left interatrial shunts had no signicant differences in right
vs left atrial pressures. The patients with left-to-right interatrial shunts, however,
had left atrial pressures (7 ± 0.5 vs 5 ± 0.5mm Hg, P <.05), signicantly greater than
right atrial pressures. No signicant differences in ventricular end-diastolic pressures were observed.
Although numerous reports have emphasized the frequency of right-to-left
interatrial shunting in patients with valvular PS with intact ventricular septa, few
have focused on patients with exclusive or nearly exclusive left-to-right shunting at
the atrial level. The rst to do so seems to have been Abrahams and Wood in 1951.
Among their 52 patients with “isolated” pulmonic stenosis, 15 had defects in the
atrial septum, producing exclusive right-to-left shunting in eight and left-to-right
shunting in seven. Each of the latter seven had only mild PS, whereas each of the
former eight had evidence of severe PS. Although not conrmed anatomically, these
authors reasoned that the patients with severe PS had venoarterial interatrial shunting via a patent foramen ovale, and that the patients with arteriovenous interatrial
shunting had a true atrial septal defect. This reasoning was conrmed anatomically
by the recent report by Roberts and associates
1
and by the morphologic and hemo-
dynamic ndings in the two patients just described.
2
REFERENCES
1 Roberts WC, Shemin RJ, Kent KM. Frequency and direction of interatrial shunt-
ing in valvular pulmonic stenosis with intact ventricular septum and without left
ventricular inow or outow obstruction. Am Heart J 1980; 99:142–48.
2 Abrahams DG, Wood P. Pulmonary stenosis with normal aortic root. Br Heart J
1971; 13:519–24.
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Case 406 Prolapsing Atrioventricular Valve
in Partial Atrioventricular Defect
Bruce F. Waller, MD, Mazhar U. Sheikh, MD,
and William C. Roberts, MD
Bethesda, Md. and Washington, D.C.
Prolapse of the posterior mitral leaet is now recognized to occur fairly frequently
in patients with secundum-type atrial septal defect.
1–5
In contrast, prolapse of an
atrioventricular (AV) valve in patients with primum-type atrial septal defect (AV
defect or canal) has not been reported to our knowledge. Such an occurrence, however, was observed in a woman with partial AV defect and clinical and morphologic
cardiac ndings are described in this report.
A 56-year-old mongoloid black woman, who died on September28, 1978, had
been in her usual state of health until 8 days before death. In October, 1977 (9 months
before death), she was seen at a cardiology clinic because of a precordial murmur.
The cardiac silhouette on chest radiograph was of normal size, but the major pulmonary arteries were mildly dilated. She remained asymptomatic until September20,
1978, when she became dyspneic and febrile. When hospitalized, she was lethargic
and the systemic blood pressure was 100/70mm Hg. The intensity of the second cardiac sound (S
) was slightly increased (Figure 1); a grade 3/6 holosystolic murmur,
2
loudest over the cardiac apical impulse and in the left axilla (Figure 1), was heard.
Asimultaneously recorded phonocardiogram and echocardiogram (Figure 1) disclosed “holosystolic” prolapse of a mitral leaet. Pulmonary parenchymal inltrates
were present on chest radiogram. An electrocardiogram (Figure 2) showed a heart
rate of 105 beats/min, a QRS axis of –75 degrees, QRS complexes typical of incomplete bundle branch block. Despite antibiotic therapy, her condition worsened, and
she died after aspirating gastric contents.
At necropsy, the heart weighed 340 gm. Partial AV defect with ballooning of
the AV leaets was present (Figure 3). Alarge defect was present in the lowermost
portion of the atrial septum (Figure 4). The right ventricle was mildly dilated.
Thromboembolic material was present in the intrapulmonary pulmonary arteries
and infarcts were present in the lower lobes of the lungs. Histologically, both the
media and intima of the pulmonary arteries were normal.
No previous report, to our knowledge, has described prolapse or oppiness
of one or more AV valve leaets in AV defect (canal). Long survival in our patient
appears to have been made possible because of the lack of development of evidence
of pulmonary arterial hypertension or overt evidence of chronic congestive heart
failure. The prolapsing AV valvular leaet may have played a role in reducing the
left-to-right shunt and therefore contributed to rather than detracted from prolonged survival.
From the Pathology Branch, National Heart, Lung, and Blood Institute, National Institutes of
Health, Bethesda, and District of Columbia General Hospital, Washington, D.C.
Reprint requests: William C. Roberts, M.D., Building 10A, Room 3E-30, National Institutes of
Health, Bethesda, MD 20205.
176 DOI: 10.1201/9781003409342-24
Case 406 ProlaPsing atrioventriCular valve in Partial av DeFeCt
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Figure 1 Phonocardiogram and echocardiogram obtained 8 days before death.
Apansystolic murmur (SM) was recorded during “holosystolic” prolapse of a mitral
leaet (“A ML”). ecg =electrocardiogram, LV = left ventricular cavity, LSB = left
sternal border, VS = ventricular septum.
Figure 2 Electrocardiogram recorded 1 week before death.
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Figure 3A Partial AV defect. Atrial view of ballooning AV valvular leaets (x, y,
z). A=anterior leaet, CS = ostium of the coronary sinus.
Figure 3B and C. b, View of prolapsed leaets (x, y, z) from right ventricle (RV).
c, outow tract of left ventricle (LV) showing cleft anterior (A) and prolapsed por-
tions (x, y) of the AV valve. R=right and P=posterior aortic cusps.
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Case 406 ProlaPsing atrioventriCular valve in Partial av DeFeCt
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Figure 4 Right (RV) and left (LV) ventricular views of anterior (A), septal (S), and
ballooning portions of the AV valve. An ostium primum atrial septal defect (ASD) is
seen. CS=ostium of the coronary sinus.
REFERENCES
1. McDonald A, Harris A, Jetterson K, Marshall J, McDonald L: Association of pro-
lapse of posterior cusp of mitral valve and atrial septal defect. Br Heart J 33:383,
1971.
2. Pocock WA, Barlow JB: An association between billowing posterior mitral leaet
syndrome and congenital heart disease, particularly atrial septal defect. Am Heart
J 81:720, 1971.
3. Jeresaty RM: Atrial septal defect and myxomatous mitral-valve prolapse. N Engl J
Med 290:1088, 1974.
4. Leachman RD, Cokkinos DV, Cooley DA: Association of ostium secundum atrial
septal defects with mitral valve prolapse. Am J Cardiol 38:167, 1976.
5. Rippe JM, Sloss LJ, Angoff G, Alpert JS: Mitral valve prolapse in adults with con-
genital heart disease. Am Heart J 97:561, 1979.
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Case 435 Ebstein’s Anomaly in the Elderly
Henry Scott Cabin, MD, Thomas P. Wood, MD, J. Orson
Smith, MD, and William C. Roberts, MD, F.C.C.P.
Although the average survival with Ebstein’s anomaly, when isolated, (except for
a defect in the atrial septum) is about 25years, at least seven patients surviving
for 70 years or longer have been reported.
patient with Ebstein’s anomaly and analyze factors allowing such survival.
CASE REPORT
A 72-year-old man who died on October2, 1979 had paroxysmal supraventricular
tachycardia since infancy without associated symptoms of cardiac dysfunction. He
was asymptomatic until February, 1975 when at age 68 he had several episodes of
near-syncope. The electrocardiogram showed a prolonged PR interval and right
bundle branch block, and a pericardial friction rub was heard. In July, 1975, chest
pain and palpitations appeared and the electrocardiogram disclosed atrial utter with 2:1 atrioventricular block. After cardioversion, Holter monitor disclosed
episodes of ventricular tachycardia unassociated with symptoms of cardiac dysfunction and he was treated with procainamide. In July, 1979, atrial utter again
appeared and he was again cardioverted to sinus rhythm and then begun on quinidine sulfate. He again did well until seven days before death when severe dyspnea
and substernal chest pain with minimal exertion occurred, which was relieved by
nitroglycerin. Examination three days later disclosed large V waves in the jugular
venous pulse. The rst and second heart sounds split widely. Midsystolic clicks, a
third heart sound, and a soft systolic murmur (at the lower left sternal border) were
heard. Pitting edema in the legs was present. The chest radiograph (Figure1), electrocardiogram (Figure2) and echocardiogram were all compatible with Ebstein’s
anomaly.
7–10
The nal four days were characterized by recurrent episodes of chest
pain, dyspnea and nally electromechanical dissociation.
At necropsy, the parietal and visceral pericardia were everywhere adherent
to one another by brous adhesions. The heart weighed 560 grams. The amount
of downward displacement into the right ventricle of the basal attachments of the
septal and posterior tricuspid valve leaets was severe (Figure 3). The interior
circumference of the right atrioventricular junction, ie, the site where the tricuspid valve leaets should have had their basal attachments, was 16cm (normal ≤
11cm). The mitral valve anulus measured 11cm in circumference. The lumina of
the left anterior descending and right coronary arteries were narrowed 76–95percent, and the left circumex coronary artery from 51–75percent in cross-sectional
area by atherosclerotic plaques (Figure4). Atransmural scar was present in the
1–6
Herein we describe an elderly
From the Pathology Branch, National Heart, Lung and Blood Institute, National Institutes
of Health, Bethesda, Maryland and from the Departments of Pathology and Medicine,
Tallahassee Memorial Regional Medical Center, Tallahassee, Florida.
Reprint requests: Dr. Roberts, National Institutes of Health, Bldg 10A, Room 3E30, Bethesda
20205
180 DOI: 10.1201/9781003409342-25
Case 435 ebstein’s anomaly in the elDerly
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Figure 1 Posteroanterior chest radiograph one day before death showing a very
large cardiac silhouette.
Figure 2 Electrocardiogram four days before death showing complete right bundle branch block, left axis deviation and a supraventricular rhythm.
anterobasal wall of left ventricle. Avalvular competent patent foramen ovale was
present.
COMMENTS
Of 121 previously reported necropsy patients with Ebstein’s anomaly in whom the
ages at death and the status of the atrial septa were described,
died during the rst year of life and 84 (69percent) by age 20 (Figure5). The mean
age of the 93 patients surviving past the rst year of life was 26years. To determine
anatomic factors that might affect prognosis, we examined the relationship, if any,
of survival to presence of an atrial septal defect or patent foramen ovale (Figure5) or
1–5, 11–16
28 (23percent)
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