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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 8 Photomicrographs of cross-sections of the left subclavian artery (A),
which was anastomosed to the left main pulmonary artery (Blalock-Taussig procedure), and closed ductus arteriosus (B). In A, note the marked intimal brous
proliferation. The intima of the adjoining aorta and main left pulmonary artery,
in contrast, was free of brosis. The brous proliferation on the intimal surface is
believed to be the result of a “jet lesion.” In B, note the large number of elastic bers
(stained black) in the occluded lumen. Verhoeff-van Gieson elastic tissue stain; original magnication of each, × 17.
nodule, which weighed 36 grams, and which showed histologically a pleomorphic
collection of cells which invaded the capsule (Figure9). No metastases, however,
were found.
COMMENTS
This patient represents the longest survival of an individual whose major cardiovascular anomaly was complete transposition of the great vessels. This 21-year-old
man, however, had had a Blalock-Taussig anastomosis at the age of 10years, and
this procedure apparently lengthened his life span. Previous reports record only
4 instances, proven by autopsy, of survival of patients with this malformation to the
age of 10years or longer
1–4
; the oldest of these cases lived for 18years.3 Other patients,
who have been described as having complete transposition and who lived into adulthood, should be classied instead under their major cardiac defect (usually tricuspid atresia or single ventricle).
and Musser
9
probably had corrected transposition of the great vessels, rather than
5–8
The 44-year-old patient reported by Carns, Ritchie,
the complete (“uncorrected”) variety. The 38-year-old patient reported by Messeloff
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Case 15 survIval to adulthood In a patIent wIth CoMplete transposItIon
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Figure 9 A. Photograph of the left adrenal gland containing a well-circumscribed
adrenal-cortical tumor. There is considerable focal hemorrhagic necrosis of the
tumor. B. Photomicrograph of the adrenal tumor shown in A. Original magnication, × 610.
10
and Weaver
also had partial anomalous pulmonary venous connection. Since this
anomaly provided partial physiologic “correction,” this case probably should be
excluded from a consideration of patients with complete transposition of the great
vessels in which the venous connections of the heart are normal.
Of the 5 patients with complete transposition who survived to the age of 10years
or longer, only 2 possessed more than one communication between the 2 circulations.
In addition to the present case, Keith’s patient
defects, as well as pulmonic stenosis. Hanlon and Blalock
of survival in complete transposition is related to the number of defects present,
namely, ventricular and atrial septal defects, patent ductus arteriosus, and collateral
bronchial arterial circulation. In their series, the single compensating abnormality
associated with the longest life expectancy was a ventricular septal defect; an atrial
defect was the next most favorable isolated defect, and the combination of these
2 provided the best prognosis of all.
4
also had ventricular and atrial septal
11
showed that the length
43
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Pulmonic stenosis is also a favorable lesion in patients with complete transposition, since it prevents excessive pulmonary blood ow, allows more left to right
shunting through the intracardiac communications, and, consequently, more saturated blood enters the systemic circuit. In a review of complete transposition of the
great vessels, pulmonic stenosis was found in more than a third of the cases.
Alcalde, and Baffes
13
mention that the pulmonic stenosis in complete transposition
12
Lev,
“is almost, if not always, some distance from the pulmonary orice and may be socalled ‘subpulmonary’ or ‘left infundibular.’ ” The stenosis in the present patient,
however, clearly involved only the valve.
The occurrence of both atrial appendages on the same side of the great vessels
(juxtaposition) is more than an anatomical curiosity. Although this anomaly is of
no functional signicance, it may be interpreted on angiocardiography as an atrial
aneurysm or pathologic diverticulum. During open heart surgery, the absence of a
normally situated right atrial appendage might present the surgeon with considerable technical difculty at cannulation. At least 20 examples of juxtaposition of the
atrial appendages have been recorded; both appendages lay to the left of the great
vessels in 18 (left juxtaposition), and both to the right (right juxtaposition), in 2.
14–16
All 20 had transposition of the great vessels and, frequently, tricuspid atresia or
pulmonic stenosis.
In 1960, Bartter and associates
17
reported a cyanotic 25-year-old man who died
with congenital heart disease and an endocrine tumor. This patient, who presented
with the clinical picture of Cushing’s syndrome, had total anomalous pulmonary
venous connection, pituitary (? chromophobe) adenoma, and bilateral adrenal cortical hyperplasia. Since that time, 4 other patients with heart disease and an endocrine tumor have been autopsied at the Clinical Center. Three of these patients had
unilateral adrenal cortical tumors, which were not apparent clinically. The patient
described in this report had a pleomorphic adrenal cortical carcinoma, which
invaded the capsule but did not metastasize; the other 2 patients, acyanotic men
aged 57 and 48years with isolated calcic aortic stenosis, each had large (4 by 3
centimeters and 2 by 2 centimeters, respectively), histologically uniform adrenal
cortical adenomas which neither invaded the capsule nor metastasized. The fth
patient, a cyanotic 46-year-old man, had congenital heart disease (total anomalous
pulmonary venous connection) and an islet cell tumor of the pancreas, not clinically apparent, which metastasized to the peripancreatic lymph nodes. In summary,
of nearly 300 patients with congenital or rheumatic heart disease autopsied at the
Clinical Center, 5 had associated endocrine tumors. All of these cases were adults;
3 had congenital malformations of the heart and great vessels leading to cyanosis, 2 had isolated calcic aortic stenosis of uncertain etiology, and in 4 the tumors
involved the pituitary-adrenal axis. This association appears to be more than mere
coincidence. Four patients in whom pheochromocytoma was associated with cyanotic congenital heart disease have been observed at The Johns Hopkins Hospital.
18
This association of adrenal tumors with cyanotic congenital heart disease conceivably could be related to the stress produced by prolonged hypoxemia.
SUMMARY
A man is described who lived for 21years with complete transposition of the great
vessels. He is believed to represent the oldest pathologically proven instance of complete transposition. Associated abnormalities included valvular pulmonic stenosis,
ventricular and atrial septal defects, and collateral bronchial arterial circulation.
ABlalock-Taussig anastomosis, in combination with these associated defects, may
have been responsible for the patient’s long survival. Left juxtaposition of the atrial
appendages was an additional anomaly. An unilateral adrenal-cortical carcinoma
was found at autopsy, and the association of endocrine tumors with heart disease,
particularly of the cyanotic variety, may be more than mere coincidence.
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Case 15 survIval to adulthood In a patIent wIth CoMplete transposItIon
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SUMMARIO IN INTERLINGUA
Es describite un homine qui viveva 21 annos con transposition complete del grande
vasos. Es opinate que iste patiente representa le plus perdurative, pathologicamente
vericate caso de transposition complete. Le associate anormalitates includeva stenosis pulmono-valvular, defactos septal ventricular e atrial, e circulation bronchoarterial collateral. Un anastomosis Blalock-Taussig in combination con le mentionate
associate defectos esseva possibilemente responsabile pro le longe superviventia del
patiente. Juxtaposition sinistre del appendice atrial esseva un anormalitate additional. Un unilateral carcinoma adreno cortical esseva trovate al necropsia. Le association de tumores endocrin con morbo cardiac, particularmente morbo cardiac de
typo cyanotic, es possibilemente plus que un coincidentia. Inter quasi 300 patientes
con congenite o rheumatic morbo cardiac qui esseva necropsiate al Centro Clinic,
5 habeva associate tumores endocrin. In omne iste casos il se tractava de adultos.
Tres habeva malformationes congenite del corde o del grande vasos con resultante
cyanosis; 2 habeva isolate calcic stenosis aortic de incerte etiologia; e in 4 le tumores
afceva le axe pituitarioadrenal.
REFERENCES
1. DORNING, J.: Acase of transposition of the aorta and pulmonary artery, with
patent foramen ovale; death at ten years of age. Trans. Amer. Pediat. Soc. 2: 46,
1890.
2. ALEXANDER, F., WHITE, P. D.: Four important congenital cardiac conditions
causing cyanosis to be differentiated from the tetralogy of Fallot: tricuspid atresia, Eisenmenger’s complex, transposition of the great vessels, and a single ventricle. Ann. Intern. Med. 27: 64, 1947.
3. PUNG, S., GOTTSTEIN, W. K., HIRSCH, E. F.: Complete transposition of the
great vessels in a male aged 18years. Amer. J. Med. 18: 155, 1955.
4. KEITH, A.: Six specimens of abnormal heart. J. Anat. Physiol. 46: 211,
1911–1912.
5. NASSE, F.: Leichenöffnungen: zur Diagnostik und Pathologischen Anatomie.
Bildungsfehler des Herzens in Einem Falle von Blauer Krankheit, Adolph Marchus,
Bonn, 1821, pp.162–194.
6. HEDINGER, E.: Transposition der grossen Gefässe bei rudimentärer linker
Herzkammer bei einer 56 jährigen Frau. Zbl. Allg. Path. 26: 529, 1915.
7. MARCHAND: Cited by Hedinger (6).
8. LEWIS, F. T., ABBOTT, M.: Reversed torsion of the human heart. Anat. Rec. 9: 103,
1915.
9. CARNS, M. L., RITCHIE, G., MUSSER, M. J.: An unusual case of congenital heart
disease in a woman who lived for forty-four years and six months. Amer. Heart J.
21: 522, 1941.
10. MESSELOFF, C. R., WEAVER, J. C.: Acase of transposition of the large vessels in
an adult who lived to the age of 38years. Amer. Heart J. 42: 467, 1951.
11. HANLON, C. R., BLALOCK, A.: Complete transposition of the aorta and the
pulmonary artery. Experimental observations on venous shunts as corrective
procedures. Ann. Surg. 127: 385, 1948.
12. BECKER, M. C., BRILL, R. M.: Complete transposition of the great vessels; report
of three cases and a review of the literature. Arch. Pediat. 65: 249, 1958.
13. LEV, M., ALCALDE, V. M., BAFFES, T. G.: Pathologic anatomy of complete trans-
position of the arterial trunks. Pediatrics 28: 293, 1961.
14. DIXON, A. ST. J.: Juxtaposition of the atrial appendages: two cases of an unusual
congenital cardiac deformity. Brit. Heart J. 16: 153, 1954.
15. SMYTH, N. P. D.: Lateroposition of the atrial appendages. Acase of levoposition
of the appendages. Arch. Path. 60: 259, 1955.
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16. FRAGOYANNIS, S. G., NICHERSON, D.: An unusual congenital heart anomaly.
Tricuspid atresia, aortic atresia, and juxtaposition of atrial appendages. Amer. J.
Cardiol. 6: 678, 1960.
17. BARTTER, F. C., LIDDLE, G. W., BELL, N. H., BRAUNWALD, E., HILBISH, T. G.,
CORNELL, W., HICKLIN, M.: Problem in differential diagnosis: clinical pathological conference at the National Institutes of Health. Ann. Intern. Med. 52: 1289,
1960.
18
. ROSS, R. S., CARPENTER, C. C. J., GLANCY, D. L.: Personal communication.
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Case 16 CoMBIned CongenItal pulMonIC and MItral stenosIs
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Case 16 Combined Congenital
Pulmonic and Mitral Stenosis*
William C. Roberts, MD†, Allan Goldblatt, MD‡, Dean T.
Mason, MD
§
and Andrew G. Morrow, MD
¶
Bethesda, Maryland
IN AN OCCASIONAL patient with rheumatic mitral stenosis tricuspid stenosis is
present as well. In the combined lesion the usual symptoms and signs of mitral
stenosis may be absent since the obstruction in the right side of the heart tends to
prevent pulmonary vascular congestion. The occurrence of a right-sided stenotic
lesion in a patient with congenital mitral stenosis, on the other hand, has not previously been described. Such a case was seen, however, in a three-year-old, poorly
developed girl who fatigued easily and periodically had signs of right-sided cardiac
failure. Examination disclosed a loud (Grade 4 of 6) ejection-type systolic murmur
over the pulmonic area, but no precordial diastolic murmur was either audible or
recordable. An electrocardiogram showed right-axis deviation and hypertrophy of
the right ventricle and atrium, and a roentgenogram of the chest disclosed enlargement of the left atrium as well as the right atrium and ventricle. Cardiac catherization (Figure 1) and a cineangiogram of the right ventricle conrmed the clinical
impression of congenital pulmonic stenosis but also aroused suspicion of a leftsided cardiac lesion since the pressure in the left atrium was elevated (Figure 2).
After direct measurement of the pressure in the left ventricle, which was normal,
a pulmonary valvulotomy was performed. After release of the right-sided obstruction no immediate change was observed in the already elevated pressure in the left
atrium (Figure 2), but the pulmonary arterial systolic pressure was noted to be considerably elevated (Figure 3). The patient died suddenly one day after operation,
and at autopsy the mitral valve was also found to be congenitally stenotic (Figure 4).
This case (Figure 5) demonstrates the possible consequences of releasing
the right-sided cardiac obstruction without also relieving the left-sided valvular stenosis. The lungs are suddenly overlled with blood since the runoff from
the lungs is unable to keep pace with the inow to the lungs. In a review of 43
patients with congenital mitral stenosis none had an associated pulmonic stenosis, and only 1 lived for more than three years.
1
The right-sided cardiac obstruc-
tion in the patient described above appears to have allowed considerably longer
* From the Pathologic Anatomy Department, Clinical Center and Cardiology Branch and
Clinic of Surgery, National Heart Institute, National Institutes of Health, United States Public
Health Service.
†
Assistant resident, Osler Medical Service, Johns Hopkins Hospital, Baltimore, Maryland;
formerly, resident, Pathologic Anatomy Department, Clinical Center, National Institutes of
Health, Bethesda, Maryland.
‡
Resident, Children’s Hospital Medical Center, Boston, Massachusetts; formerly, pediatric
associate, Cardiology Branch, National Heart Institute, Bethesda, Maryland.
§
Clinical associate, Cardiology Branch, National Heart Institute.
¶
Chief, Clinic of Surgery, National Heart Institute.
DOI: 10.1201/9781003409342-5
47
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Figure 1 Simultaneously recorded pressure pulses in the right ventricle (R.V.)
and brachial artery (R.B.A.), demonstrating ventricular hypertension secondary to
pulmonic-valve stenosis. The pulmonary trunk could not be entered by the catheter.
Figure 2 Preoperative pressure tracing of the left atrium (L.A.). The pressure by
direct puncture after the pulmonic valvulotomy was identical to this preoperative
recording; the mean pressure is 25mm. of mercury.
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Case 16 CoMBIned CongenItal pulMonIC and MItral stenosIs
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Figure 3 Continuous pressure tracing of the pulmonary artery (P.A.) and right
ventricle (R.V.) by direct puncture after operation. A residual pulmonic-valve
systolic gradient is evident, as well as pulmonary hypertension.
Figure 4 Diagram of the heart in the patient described. The mitral-valve leaets are
brotic, thickened and shortened and insert at times directly into the papillary muscles.
The remaining chordae tendineae are fused, thickened and shortened. The left atrium is
dilated, and its endocardium diffusely thickened. The pulmonic valve is stenotic. Three
distinct pulmonic-valve cusps are identied. Each is thickened and brotic, but the commissures are not fused. The orice of the pulmonic valve is centrally located. (It is unusual
for a tricuspid pulmonic valve to produce stenosis, but this clearly occurred in the present
patient; in most patients with valvular pulmonic stenosis the valve has a dome shape
without distinct commissures.) The wall of the right ventricle is greatly hypertrophied,
being thicker than that of the left ventricle. The aortic and tricuspid valves are normal.
RA=right atrium; RV=right ventricle; LA=left atrium; and LV=left ventricle.
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Figure 5 Photomicrographs (Verhoeff—Van Gieson elastic-tissue stains; original
magnication X6 for A and X5 for B) of sections through the pulmonic (A) and mitral
valves (B). The leaets of each valve are markedly thickened by brous proliferation.
The arrow (left) points to a jet lesion on the intima of the pulmonary trunk (P.T.) just
above the pulmonicvalve cusp (P.V.). The posterior leaet of the mitral valve (M.V.)
inserts directly into the papillary muscle (P.M.). There is secondary endocardial broelastosis of the left atrium (L.A.).
survival than would have resulted if the stenosis of the mitral valve had been an
isolated lesion.
REFERENCE
1. Ferencz, C., Johnson, A. L., and Wiglesworth, F. W. Congenital mitral stenosis.
Circulation 9:161–179, 1954.
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Case 17 the sIgnIfICanCe of asplenIa In CongenItal heart dIsease
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Case 17 The Significance of Asplenia in the
Recognition of Inoperable Congenital Heart Disease
William C. Roberts, MD, William B. Berry, MD, and Andrew G. Morrow, MD
SPLENIC AGENESIS in association with congenital heart disease implies the presence of multiple and complex malformations of the heart and great vessels,
consequently inoperable cardiac disease.
3
Such was the case in an extremely cya-
1–3
and
notic and dyspneic 3-month-old male infant with no cardiac murmur. The electrocardiogram showed right axis deviation, and suggested right atrial enlargement,
right ventricular hypertrophy, and abnormal positioning of the ventricles. Chest
roentgenogram (Figure 1) disclosed a normal-sized, normally located heart and
a right-sided gastric air bubble. Selective angiocardiography (Figure 2) showed
transposition of the great vessels, a single ventricle, a right aortic arch, and probable pulmonic valvular and subvalvular stenosis. Necropsy following sudden death
revealed additional malformations (Figures 3 and 4).
This patient demonstrates the syndrome of splenic agenesis, partial situs inversus, and multiple congenital cardiovascular anomalies. Approximately 100 patients
with this entity have been reported. All of the more common malformations of the
heart and great vessels associated with this syndrome are illustrated in the patient
described herein. The complexity of the cardiac disorders can be determined only
by intracardiac catheterization and usually by angiocardiography. The diagnosis of
asplenia, on the other hand, frequently may be made simply by study of the peripheral blood smear. Howell-Jolly bodies or siderotic granules in the erythrocytes of
the peripheral blood for practical purposes indicate an absent spleen.
3
The presence of situs inversus, which often suggests that asplenia also exists, can usually
be determined by routine radiographic examination. The gastric air bubble on the
right means situs inversus. The upright abdominal roentgenogram is extremely
helpful in determining the presence or absence of the spleen and will frequently
demonstrate the anteroinferior margin of the liver on the left, and also attening
of this margin.
4
From the Pathologic Anatomy Department, Clinical Center, and the Clinic of Surgery,
National Heart Institute, National Institutes of Health, Bethesda, Maryland.
DOI: 10.1201/9781003409342-6
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