in adult patients with corrected transposition, to fibrous replacement of the conducting tissues connecting the anterior node to the His bundle. In our patient with congenital heart block, the fibrous tissue between the atrioventricular node and His bundle, which may represent a distorted central fibrous body, was presumably present prior to birth and provides an anatomic explanation for heart block. In conclusion, this patient with Kartagener's syndrome had corrected transposition as the manifestation of abnormal laterality. The cardiac conducting system was unusual in that congenital heart block was present and no anterior atrioventricular node was found. Prosthetic valvular surgery was complicated by myocardial infarction from suture injury and thrombosis of the left circumHex coronary artery. REFERENCES

1 Kartagener M: Zur Pathogenese der Bronchiektasien: Bronchiektasien bei Situs viscerum inversus. Beitr Klin Tuberk 83:489-501, 1933 2 Kartagener M, Stucki P: Bronchiectasis with situs inversus. Arch Pediatr 79:193-207, 1962 3 Katsuhara K, Kawamoto S, Wakabayashi T, et al: Situs inversus totalis and Kartagener's syndrome in a Japanese population. Chest 61:56-61, 1972 4 Schiebler GL, Edwards JE, Burchell HB, et al : Congenital corrected transposition of the great vessels : Astudy of 33 cases. Pediatrics 27 ( suppl) :849-888, 1961 5 Anderson RH, Arnold R, Wilkinson JL: The conducting system in congenitally corrected transposition. Lancet 1:1286-1288, 1973 6 Anderson RH, Becker AE, Arnold R, et al: The conducting tissues in congenitally corrected transposition. Circulation 50:911-923, 1974 7 Lev M, Licata RH, May RC: The conduction system in mixed levocardia with ventricular inversion (corrected transposition). Circulation 28:232-237, 1963 8 Holmes LB, Blennerhassett JB, Austen KF: A reappraisal of Kartagener's syndrome. Am J Med Sci 255:13-28, 1968 9 Logan WD Jr, Abbott OA, Hatcher CR Jr: Kartagener's triad. Dis Chest 48:613-616, 1965 10 Overholt EL, Barman DF: Variants of Kartagener's syndrome in the same family. Ann Intern Med 48:574-579, 1958 11 Fomatto EJ, Skolnilc EM, Timpton RH : The triad of Kartagener: Relation of upper to lower respiratory pathology. Laryngoscope 66:1202-1220, 1956 12 Knox G, Murray S, Strang L: A family with Kartagener's syndrome: Linkage data. Ann Hum Genet 24:137-140, 1960 13 Bergstrom WH, Cook CD, Scannell J, et al: Situs inversus, bronchiectasis and sinusitis: Report of a family with two cases of Kartagener's triad and two additional cases of bronchiectasis among six siblings. Pediatrics 6:573-580, 1950 14 Cockayne EA: The genetics of transposition of the viscera. Q JMed 7:479-493, 1938 15 Olsen AM : Bronchiectasis and dextrocardia. Am Rev Tuberc 47 :435-439, 1943 16 McKusick VA: Human Genetics. Englewood Cliffs, NJ, Prentice-Hall Inc, 1964 17 Nora JJ. Fraser FC: Medical Genetics. PhiladeiJ?hia, Lea

880 SCHLOSSBERG, ET AL

and Febiger, 1974, p 251 18 Dickey LB: Kartagener's syndrome in children. Dis Chest 23:657-666, 1953 19 Divekar MV: Kartagener's syndrome: Transposition of viscera associated with bronchiectasis and affection of nasal sinuses: A report of five cases. Indian J Med Sci 10:102-113, 1956 20 Pomerleau D, Gilbert G, Thibert D: Syndrome de Kartagener associe a une tetralogie de Fallot. Union Med Can 101:79-84, 1972 21 de Ia Cruz MV, Anselmi G, Cisneros F, et al: An embryologic explanation for the corrected transposition of the great vessels: Additional description of the main anatomic features of this malformation and its varieties. Am Heart J 57:104-117, 1959 22 Butler JK : An Experimental Analysis of Cardiac Loop Formation in the Chick, thesis. University of Texas, Austin, 1952 23 Manasek FJ, Monroe RG : Early cardiac morphogenesis is independent of function. Dev Bioi 27:584-588, 1972 24 Bjork VO, Book K: Surgical treatment of systemic atrioventricular valve insufficiency in corrected transposition. Scand J Thorac Cardiovasc Surg 7:21-24, 1973

Pericarditis with Effusion Caused by • • , ••• Ad1nomyces 11rae 11 David Schlossberg, M.D.;•• Donna Franco-Jove, M.D.;t Craig Woodward, M.D.,t and Jonas Shulman, M.D.§

Actinomyces israelii is a rare cause of pericarditis. Infection spreads to pericardium from an intrathoracic focus. Branching gram-positive filaments are seen on microscopic examination of clinical specimens. Cultures should be performed under anaerobic conditions. Both surgical drainage and antibiotics (penicillin) are required for treatment. Nocardiosis may resemble actinomycosis in many respects.

A ctinomyces israelii

infrequently causes disease in man. Pericarditis caused by this organism is even rarer. 1 We recently cared for a patient who had signs and symptoms of pericarditis with effusion and a pulmonary infiltrate resulting from infection with A israelii. CASE REPoRT

A 47-year-old man was admitted to the Atlanta Veterans Administration Hospital because of chest pain and cough. °From the Department of Medicin~1 Division of Infectious Disease, and the Department of Patnology, School of Medicine, Emory University, Atlanta. This investigation was supported by National Institutes of Health grant-in-aid 5 T01 A100447-04. 0 °Fellow, Department of Medicine. Dr. Schlossberg is now with the Department of Medicine, Division of Infectious Disease, Naval Regional Medical Center, Portsmouth, Va. tAssistant Professor of Pathology. tSenior Resident, Department of Medicine. §Professor of Medicine and Chief, Division of Infectious Disease. Reprint requests: Dr. Schlossberg, Naval Regional Medical Center, Portsmouth, Virginia 23708

CHEST, 69: 5, MAY, 1976

The patient had been in good health until eight months before admission, when, he noted the onset of left-sided pleuritic chest pain. After four months, the pain was associated with a persistent nonproductive cough, which lasted until admission. Over this eight-month period, the patient lost 30 pounds. On admission, the patient was afebrile, with a pulse rate of 80 beats per minute, a respiration rate of 20/min, and blood pressure of 132/78 mm Hg, without paradox. Pertinent physical findings included a grade 2/6 early systolic crescendo-decrescendo murmur at the apex, a one-component pleuropericardial rub heard along the left sternal border, and tenderness over the third and fourth ribs in the anterior axillary line. Findings from the remainder of the physical examination were within normal limits. Laboratory data included the following: hemoglobin level, 10.3 gm/100 ml; white blood cell count, 18,000/cu mm with 91 percent neutrophils, 1 percent band cells, 4 percent lymphocytes, and 4 percent monocytes; platelet count, 702,000/cu mm; total serum protein level, 8.4 gm/100 ml; serum albumin level, 3.2 gm/100 ml; serum alkaline phosphatase level, 199 units/100 ml; serum glutamic oxaloacetic transaminase, 70 units/ml; and normal electrolyte levels. The chest x-ray film ( Fig 1 ) revealed a mass in the left upper lung, obscuring the left cardiac border with marked volume loss. The electrocardiogram was normal. Examination of sputum showed no predominant pathogen and no acid-fast organisms on staining. Bronchoscopic examination was performed and demonstrated erythema and mucosal irregularity of the primary segmental left upperlobar bronchus, as well as lingular bronchial compression. Brush biopsy and bronchial washings showed no malignant

cells and revealed no acid-fast organisms on staining. Fungal cultures grew small numbers of Rlwclotonda rubra and Penicillium species. No anaerobic cultures were inoculated, and no Gram's staining was performed on the bronchoscopic specimen. Aspiration of bone marrow revealed no organisms or tumor cells. The patient continued to be afebrile with intermittent pain in the left anterior portion of the chest, which necessitated the administration of narcotics for relief. On the seventh hospital day an electrocardiogram demonstrated changes compatible with pericarditis, but there was no evidence of hemodynamic compromise. On the 14th day an echocardiogram suggested both anterior and posterior pericardia! effusions. Pericardiocentesis was performed on the next day, and 30 ml of sanguinous fluid was aspirated. Gram's staining of this fluid revealed numerous polymorphonuclear leukocytes but no organisms, and cytologic findings were normal. On the following day, thoracotomy was performed, revealing an extensive inflamm~tory mass in the left upper lobe, with extension to the chest wall, left lower lobe, and pericardium. The left upper lobe and pericardium were removed. Sections of both the pericardium and the inflammatory mass demonstrated sulfur granules and branching filaments ( Fig 2). One initial weakly decolorized acid-fast stain of the tissue from the inflammatory mass was positive, but all subsequent stains of the tissue and of cultures were negative with this method. Cultures of the inflammatory mass and of a section of pericardium yielded A israeUi, identification of which was confirmed by the fluorescent-antibody stain. The patient was initially treated with trimethoprim-sulfamethoxazole because of the consideration of infection with Nocardia asteroidea based on the weakly decolorized acidfast stain. The patient showed marked improvement with this medication. When Nocardia organisms failed to grow and A israelii was definitely identified, the patient was then treated with penicillin ( 1.5 gm of the potassium salt of penicillin V [Pen-Vee .K] and 1 gm of probenecid, q.i.d. ). At follow-up, three months after discharge, the patient was gaining weight, felt well, and his chest x-ray films had continued to show improvement. DISCUSSION

FicURE 1. Chest x-ray film on admission.

CHEST, 69: 5, MAY, 1976

Actinomyces israelii is a nonnal inhabitant of man·s oral cavity. The disease caused by this organism is usually chronic, with suppuration and fonnation of

PERICARDmS WITH EFFUSION 881

granulomas, frequently with development of draining sinuses. Involvement of the heart is seen in less than 2 percent of the cases of actinomycosis 1 and typically is secondary to intrathoracic disease. 2 We believe that our patient's disease followed this progression, that is, development of pneumonia with secondary contiguous extension to the pericardia! sac. Pericarditis due to the organism is rare. 8 •4 Three recent reviews 5 - 7 of actinomycotic infection listed no cases of pericarditis. When the pericardium is infected, signs and symptoms of pericarditis, including friction rub, may be missing. Gram's staining of clinical specimens shows branching gram-positive filaments often arranged in tangled masses called sulfur granules. Nocardia organisms may cause clinical disease resembling actinomycosis, and the pathologic and cultural characteristics of these two organisms frequently overlap.8 Both organisms have the same appearance on Gram's staining. Nocardia organisms may even form granules 8 but usually in superficial lesions only. uo Furthermore, N asteroides and N brasiliensis are often acid-fast when an acid-fast stain with weak decolorization is used, 11 while Actinomyces species are negative with this method; however, there is some dispute over this point. 1 o Since Actinomyces organisms are found normally in the mouth, culture of sputum is not helpful in diagnosis of pulmonary infection unless the sputum was obtained from a transtracheal aspirate, Frequently, culture and Gram's staining of the infected tissue itself must be performed. Actinomyces organisms are anaerobic or microaerophilic; and in cases of thoracic disease with pericardia! effusion, both pulmonary tissue and pericardial fluid or biopsy should be cultured under anaerobic conditions. Failure to perform these studies may account for the low reported incidence of pericardia! involvement in thoracic actinomycosis. In our patient the diagnosis was further verified by demonstrating fluorescence of the branching filaments with the fluorescent-antibody technique, which is specific for both recognized serotypes of A israelii, A naeslundii, and Arachnia propionica. 12 The agar-gel double-diffusion test is the most reliable serologic test available; it is most helpful in patients with disseminated disease, but both false-negative and false-positive results occur, the latter especially in patients with tuberculosis.t2 Although rare, infection of the pericardium with A israelii is treatable with penicillin and surgical drainage, and should be considered among the causes of purulent pericarditis. ACKNOWLEDGMENTS: We wish to thank Ms Janet Collins, Supervis?r Microbiology Laboratory, Veterans Administration Hospiw,1 Atlanta, for her help in identification of the organism; Ms Lenore Haley, Chief, Mycology Training Branch, Communicable Disease Center, Atlanta; Ms June Brown, Chief, Developmental Mycology Laboratory, Communicable Disease Center, for performing the fluorescentantibody Test; the Medical Illustration Departments of Grady Memorial Hospital, Atlanta, and of the Veterans Administra-

882 LEWINSOHN, BRUDERMAN, BOHADANA

tion Hospital for reproduction of the visual material presented; and Ms Louise Denman for secretarial assistance. REFERENCES

1 Dutton WP, Inclan AP: Cardiac actinomycosis. Dis Chest 54:463-465, 1968 2 Datta JS, Raff MJ: Actinomycotic pleuropericarditis. Am Rev Respir Dis 110:338-341, 1974 3 Mohan K, Dass SI, Kemble EE: Actinomycosis of pericardium. JAMA 229:321, 1974 4 Coodley EL: Actinomycosis: Clinical diagnosis and management. Postgrad Med 46:73-78, 1969 5 Prather JR, Eastridge CE, Hughes FA Jr, et al: Actinomycosis of the thorax: Diagnosis and treatment. Ann Thorac Surg 9:307-312, 1970 6 Eastridge CE, Prather JR, Hughes FA, et al: Actinomycosis: A 24-year experience. South Med J 65 :839-843, 1972 7 Tomm KE, Raleigh JW, Guinn GA: Thoracic actinomycosis. Am J Surg 124:46-48, 1972 8 Graybill JR, Silverman BD: Sulfur granules. Arch Intern Med 123:430-432, 1969 9 Conant NF, Smith DT, Baker RD, et al: Manual of Clinical Mycology. Philadelphia, WB Saunders Co, 1971 10 Robboy SJ, Vickery AL Jr: Tinctorial and morphologic properties distinguishing actinomycosis and nocardiosis. N Engl J Med 282:593-596, 1970 11 Brown JR: Human actinomycosis. Hum Pathol4:319-330, 1973 12 Buechner HA, Seabury JH, Campbell CC, et al: The current status of serologic, immunologic and skin tests in the diagnosis of pulmonary mycoses: Report of the Committee on Fungus Diseases and Subcommittee on Criteria for Clinical Diagnosis-American College of Chest Physicians. Chest 63:259-270, 1973

Primary DiHuse Pulmonary Amyloidosis with Monoclonal Gammopathy* Gavriel Lewinaohn, M .D .; 00 Israel Bruderman, F.C.C.P.; and Abraham Bohadana, M.D.

M.D.,

A rare case of primary diffuse amyloidosis of the 111111 with an abnormal monoclonal protein is described. The diagnosis was confirmed by an open-lung biopsy. The different classifications of amyloidosis as weD as the relationship between the lmmunoglobuHns and the pathogenesis of amyloid disease is discussed. amyloidosis is a systemic disease, pulmonary A lthough amyloidosis of the diffuse variety is very rare, and only 15 cases have been reported so far in the literature. 1 • 2 The recent discovery that the amyloid •From the Department of Chest Disease and Pulmonary Research Laboratory, Meir Hospital, Kfar Saba, Israel, and Tel-Aviv University School of Medicine, Tel-Aviv, Israel. 0 • Presently at Asaph Harofeh Hospital, Zerifln, Israel Reprint requests: Dr. Bruderman, Meir Hospital, Kfar Saba, Israel

CHEST, 69: 5, MAY, 1976