Download Post Superior Vena Cava-Right Pulmonary Artery Shunt

BALKOURA-CHRISTOPOULOS, KITTLE
patients with SLE. If the respiratory symptoms d o not
regress or a restrictive pulmonary defect remains, irnmur~osuppressivetherapy would appear to be indicated.
This patient remains well two years after the original
illness.
Post Superior Vena Cava-Right
Pulmonary Artery Shunt; Total
Surgical Correction of Ebstein's
Anomaly with Starr-Edwards
Prosthesis*
Maria H. Balkoura-Christopoulos, M.D. and C. Frederick
Kittle, M.D.
REFERENCES
1 Harvey All, Shulman LE, Tumulty PA, et al: Systemic
lupus erythematosus: review of the literature and clinical
analysis of 138 cases. Medicine 33:291-437, 1954
1 Estes D, Christian CL: The natural history of systemic
lupus erythematosus by prospective analysis. Medicine
50:85-95, 1971
3 Dubois EL, Tuffanelli DL: Clinical manifestations of
systemic lupus erythematosus. JASIA 190:104-111, 1964
4 Levin DC: Proper interpretation of pulmonary roentgen
changes in systemic lupus erythematosus. Am J Roentgen
111:510-517, 1971
5 Gould DhI, Daves ML: Roentgenologic findings in systemic lupus erythematosus: an analysis of 100 cases. J
Chronic Dis 2: 136-145, 1955
6 Huang C, Hennigar GR, Lyons HA: Pulmonary dysfunction in systemic lupus erythematosus. New Eng J Med
272 :288-293, 1965
7 Gold Whl, Jennings DB: Pulmonary function in patients
with systemic lupus erythematosus. An1 Rev Resp Dis
93: 556-567, 1966
8 Rakov HL, Taylor JS: Acute disseminated lupus erythematosus without cutaneous manifestations and with
heretofore undescribed pulmonary lesions. Arch Intern
Iled 70:88-100, 1942
9 Ellman P, Cudkowicz L: Pulmonary manifestations in the
diffuse collagen diseases. Thorax 9:46-57, 1954
10 Hoffbrand BI, Beck ER: Unexplained dyspnea and shrinking lungs in systemic lupus erythematosus. Br hled J 12:
1273-1277, 1965
11 Foldes J : Acute systemic lupus erythematosus. Am J Clin
Path 16:160-173, 1946
12 Rich AR: Hypersensitivity in disease, with especial reference to periarteritis nodosa, rheumatic fever, disseminated lupus and rheumatoid arthritis. In The Harvey
Lrctnres, Lancaster, Pa, The Science Press Printing Co,
1948, p 127
13 blaher JF, Schreiner GE: Treatment of lupus nephritis
with azathioprine. Arch Intern Iled 125:293-298, 1970
14 Drinkard JP, Stanley Tbt, Dornfeld L, et al: Azathioprine
and prednisone in the treatment of adults with lupus
nephritis: clinical, histological and immunological
changes with therapy. Medicine 49:411-432, 1970
15 Iiiescher PA, Paranetto F: Systemic lupus erythematosus.
In Textbook of Immunopathology, Sliescher PA, MullerEberhard HJ ( e d s ) . New York, G n ~ n eand Stratton Inc,
1969, pp 699-700
16 Kahn \IF, Rambouts C, de Seze S: Traitement du lupus
erythemateux dissemine par les immnnodepresseurs. Sem
Hop Paris 47:435-445, 1971
Total surgical correction of Ebstein's anomaly was successfully done in a patient with a previous Glenn shunt
by replacing the rudimentary tricuspid valve with a StarrEdwards milral prosthesis and closing the atrial septal
defect. During the four-year follow-up, the patient has
shown marked improvement in exercise tolerance and
disappearance of clinical cyanosis and polycythemia.
Control of his congestive heart failure and arrhythmias
has been easier. This illuslrates the feasibility of total
correction in patients with Ebstein's anomaly who have
had little benefit from a Glenn shunt.
A
superior vena cava to right pulmonary artery shunt
was introduced by GlennlJ in 1954 as a palliative
procedure for the treatment of Ebstein's anomaly.
Hunter a n d Lillehei3 in 1958 proposed a plastic procedure for correction of this anomaly by realigning the
downward displaced hicuspid leaflets to the normal
annulus, which was successfully applied later by Hardy
and associate^.^ Total surgical correction of Ebstein's
anomaly with a prosthetic valve was first described by
Barnard and SchrireS in 1963 a n d nine other patients
have been r e p ~ r t e d . ~ . ~ - ~ ~
The patient is a 21-year-old white man. At age four, he
developed exercise intolerance, dyspnea and aching pain over
his left chest. Clubbing and cyanosis on exertion had been
present since early life. At age 16, the diagnosis of Ebstein's
anomaly was made by cardiac catheterization and inhacardiac electrocardiography ( Table 1 ) .
A superior vena cava-right pulmonary artery anastomosis
was performed in January 1963 when he was 17. He improved initially, but deteriorated slowly during the following
three years. Congestive heart failure, polycythemia, and
many episodes of arrhythmias with blurred vision and
syncope occurred. Therapy consisted of digitalis, diazepam
(Valium) and monthly phlebotomies.
Admission examination revealed a tall, asthenic man with
cyanosis and clubbing of digits. Blood pressure was 108/78
mm Hg, pulse 102/min and regular. The jugular venous
pulse showed a prominent V wave. There was mild pectus
excavatt~ni with a narrow A-P diameter. The heart was
enlarged with the point of maximal impulse at the fifth left
intercostal space, 2 cm outside of the midclavicular line. A
'From the Departments of hfedicine and Surgery, University
of Chicago, Pritzker School of Medicine, Chicago.
Supported by US Public Health Service Grant HE-05793-04.
R&&t sequ&t.s: Dr. Kittle, University of Chicago Ho~l)itab,
950 East 59th Street, Chicago 60637
CHEST, VOL. 63, NO. 1, JANUARY, 1973
Downloaded From: http://publications.chestnet.org/pdfaccess.ashx?url=/data/journals/chest/21552/ on 05/02/2017
POST SUPERIOR VENA CAVA-RIGHT PULMONARY ARTERY SHUNT
FIGURE 1. Postemanterior ( A )
and lateral view ( B ) of the chest
x-ray films prior to surgery, demonstrating moderate cardiomegaly with diminished vascularity
of the left lung, and notching of
the right 6fth and sixth ribs.
4/6 midsystolic murmur with an early systolic click and a
loud third heart sound were present at the left lower sternal
border.
The hemoglobin was 15.2 gm percent, Hct 58 percent,
white cell count and platelets were normal. Pulmonary function studies showed moderately reduced vital capacity with
normal expiratory flow rates. Differential bronchospirometry
showed the ventilation of the right lung to be 58 percent and
of the left 44 percent with oxygen consumption 27 percent
and 73 percent respectively. The electrocardiogram showed
sinus rhythm, complete right bundle branch block, right
atrial hypertrophy and nonspecific T-wave changes. Chest xray picture revealed moderate cardiomegaly, decreased vascularity of the left lung and notching of the fifth and sixth
ribs on the right side ( Fig 1) .
A second cardiac catheterization and angiography in September 1967 ( Table 1 ) demonstrated tricuspid regurgitation
with massive filling of the right atrium and later of the left
atrium. The tricuspid valve was displaced anteriorly and to
the left from its usual position. A superior vena cava injection
demonstrated an open anastomosis between superior vena
cava-right pulmonary artery. Intracardiac ECG showed right
ventricular complexes in the atrialized portion of the right
ventricle.
The patient underwent cardiac surgery in November 1967
with the use of total cardiopulmonary bypass. The heart was
moderately enlarged with a very large right atrium. An
abnormal tricuspid valve was noted with a sail-like anterior
leaflet and rudimentary septal and posterior leaflets displaced
downward without chordae tendineae. No fenestrations of
the leaflets were present. There was a slit-like opening in the
atrial septum about 1.5 cm in diameter in the region of the
fossa ovalis. The atrial septal defect was closed directly. The
rudimentary tricuspid valve waq excised and a No. 4 mitral
Starr-Edwards prosthesis inserted above the coronary sinus to
avoid damage to the conduction system.
Five months later he underwent a third cardiac catheterization (Table 1). Right atrial cineangiography demonstrated
a small right atrium. The right ventricle was globular in
shape with decreased contractions (Fig 2). Thoracic aortogram revealed dilatation and tortuosity of the fourth and fifth
right intercostal arteries resulting in rib notching (Fig 3, 4 ).
ECC showed right axis deviation with complete right bundle
branch block, but the right atrial hypertrophy was not
present.
Four years after the total correction of Ebstein's anomaly
the patient continues to do well.
T h e treatment of Ebstein's anomaly by a superior vena
cava-right pulmonary artery shunt is a palliative proce-
Table 1 S u m m a r y of Cardiac Catheterisation Data
Preoperative
Site
Dynamic
Mean
Dynamic
Mean
3
6
Right atrium
Right ventricle
15/2
Pulmonary artery
15/8
10
11
Left ventricle
105/5
85
95
Aorta
105/70
3.8
Cardiac output liters/min
Arterial O, mturation yo
88.5
84.0
*All pressures measured in mm Hg with reference to the sternal angle.
Postoperative
Dynamic
Mean
12
Dynamic
Mean
8
20
95
92.0
CHEST, VOL. 63, NO. 1, JANUARY, 1973
Downloaded From: http://publications.chestnet.org/pdfaccess.ashx?url=/data/journals/chest/21552/ on 05/02/2017
94.5
Frcum 4. Close-up view of the markedly tortuous right fifth
intercostal artery, producing rib notching.
FIGURE
2. Postoperative right atrial angiocardiogram, demonstrating a rather small right atrium and a right ventricle of
globular shape. The Starr-Edwards prosthesis is seen at the
trici~spidarea.
dure for small infants and children with severe cyanosis
and polycythemia.'*2,7-0
Elimination of the "atrialized" right ventricle by plication and realigning of the downward displaced tricuspid
leaflets to the normal annulus has been successfully
applied with excellent r e s ~ l t s . ~ . + l ~
Our patient is interesting in that a total surgical
correction of Ebstein's anomaly was done successfully in
FIGURE3. Retrograde thoracic aortogram, demonstrating
marked tortuosity of the right fifth and sixth intercostal arteries. Increased bronchial artery supply to the right lung and
dilatation of the right internal mammary artery is also noted.
a patient who had been subjected to a previous Glenn
procedure. The latter procedure did not provide continued benefit for the patient. There is also evidence
from the differential bronchospirometry done in 1967
that the right lung is well ventilated (56 percent) in
comparison with the left (44 percent), while the oxygen
consumption was only 27 percent in the right and 73
percent in the left lung. This indicates poor perfusion in
the right lung, which may account for the patient's
deterioration of the arterial hypoxemia (from 88.5 percent in 1962 to 84 percent in 1967).
Of further interest is the angiographic demonstration
of rib notching and the transpleural blood vessels, believed secondary to the initial Glenn procedure.
1 Glenn WWL, Patino JF: Circulatory bypass of right
heart. J Biol Med 27: 147,1954
2 Glenn WWL: Circulatory bypass of the right side of the
heart: Shunt between superior vena cava and right pulmonary artery: Report of clinical application. New Eng J
Med 259: 117,1958
3 Hunter SW, Lillehei CW: Ebstein's malformation of the
tricuspid valve: Study of a case together with suggestions
of a new form of surgical therapy. Dis Chest 33297,
1958
4 Hardy KL, May IA, Webster CA, et al: Ebstein's anomaly: A functional concept and successful definitive repair. J
Thorac Cardiovasc Surg 48:927, 1964
5 Barnard CN, Schrire V: Surgical correction of Ebstein's
malformation with prosthetic tricuspid valve. Surgery
54:302,1963
6 Lillehei CW, Kalke BR. Carlson RG: Evolution of corrective surgery for Ebstein's anomaly. Circulation 35 (suppl
1):l-111,1967
7 Reed WA, Kittle CF, Heilbrunn A: Superior vena cava
pulmonary artery anastomosis. Arch Surg 86:87,1963
8 McCredie RM. Oakley C, Mahoney EB, et al: Ebstein's
disease: diagnosis by electrode catheter and treatment by
partial bypass of the right side of the heart. New Eng J
Med 267: 174,1962
9 Scott LP, Dempsey JJ, Tirnmis HH, et al: A surgical
approach to Ebstein's disease. Circulation 27:574, 1963
10 Hardy KL, Roe BB: Ebstein's anomaly: further experience with definitive repair. J Thorac Cardiovasc Surg
58:553,1969
CHEST, VOt. 63, NO. 1, JANUARY, 1973
Downloaded From: http://publications.chestnet.org/pdfaccess.ashx?url=/data/journals/chest/21552/ on 05/02/2017
PEDUNCULATED PERlCARDlAL COELOMIC CYST
11 Cartwright RS, Smeloff EA, Cayler CC, et al: Total
correction of Ebstein's anomaly by means of tricuspid
replacement. J Thorac Cardiovasc Surg 47:755,1964
12 Cueron M, Hirsch M, Stem J, et al: Familial Ebstein's
anomaly with emphasis on the surgical treatment. Am J
Cardiol 18: 105, 1966
13 Kay JH, Tsuji HK, Reddington JV, et al: The surgical
treatment of Ebstein's malformation with right ventricular
aneurysmorrhaphy and replacement of the tricuspid valve
with a disc valve. Dis Chest 51:537,1967
14 Timmis HH, Hardy JD, Watson DC: The surgical management of Ebstein's anomaly: The combined use of
tricuspid valve replacement, atrioventricular plication and
atrioplasty. J Thorac Cardiovasc Surg 53:385.1967
15 Najafi H, Hunter JA, Dye WS, et al: Ebstein's malformation of the tricuspid valve: surgical management. Ann
Thorac Surg 4:334,1967
Pedunculated Pericardial Coelomic
Cyst Manifesting as a Rolling
lntrapleural
ass*
Myung Soo Shin, M.D.,** Edward C. Tymloll, M.D.+ and
Alvaro D. Ronderos, M.D.:
A proved cafe of peduneulated pericardial coelomic
cyst, which presented as an intrapleural rolling mass, is
reported. Pneumothorru made the cyst with long stalk
more visiMe on films.
'From the University of Alabama in Birmingham.
"Associate Professor, De artment of Diagnostic Radiology.
Consultant, Veterans ~BministrationHospital, Birmingham.
+Assistant Instructor in Surgery, University Hos ital.
:Clinical Associate Professor, Department o f Diagnostic
Radiology.
Reprint requests: Dr. Shin, Department of Diagnostic Radiology. 619 South 19th Street, Birmingham 35233
FIGURE1. PA upright chest film revealed lobulated and pedunculated intrapleural lesion with pneumothorax on the
right.
FIGURE2. Lateral chest film revealed lobulated lesion in the
anterior costophrenic sinus area.
T
he mediastinum is an intriguing region of the body
because of the wide variety of interesting pathologic
lesions found in it. The exact diagnosis is often an
enigma until surgical procedure.' No radiographic observation of pedunculated pericardial coelomic cyst
manifesting as a rolling intrapleural mass has been previously reported. LeRow* in a review of world literature, collected 120 cases of pericardial coelomic cyst.
FIGURE3. Planigram reavealing lobulated lesion in the right
cardiophrenic sinus area.
CHEST, VOL. 63, NO. 1, JANUARY, 1973
Downloaded From: http://publications.chestnet.org/pdfaccess.ashx?url=/data/journals/chest/21552/ on 05/02/2017