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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