Tricuspid valve endocarditis in two children with normal hearts: Diagnosis and therapy of an unusual clinical entity

Tricuspid valve endocarditis in two children with normal hearts: Diagnosis and therapy of an unusual clinical entity

CLINICAL AND LABORATORY OBSERVATIONS Tricuspid valve endocarditis in two children with normal hearts: Diagnosis and therapy of an unusual clinical en...

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CLINICAL AND LABORATORY OBSERVATIONS

Tricuspid valve endocarditis in two children with normal hearts: Diagnosis and therapy of an unusual clinical entity Norman N. Musewe, M.B., Ch.B., F.R.C.P.(C), Bruce M. Hecht, M.D., Peter S, Hesslein, M.D., F.A.C.C., Vera Rose, M.B.B.S., F.R.C.P.(C), and William G. Williams, M.D., F.R.C.P.(C) From the Divisions of Cardiology and Cardiovascular Surgery, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Ontario, Canada

a u r e u s has become a more i m p o r t a n t p a t h o g e n in both hospital- and c o m m u n i t y - a c q u i r e d infections over the last decade. ~T h e exact incidence of endocarditis in S. a u r e u s septicemia remains controversial, with estimates ranging from as high as 60% in early reports to less t h a n 10% in recent reviews. 2 S. a u r e u s septicemia in childhood and adolescence is particularly devastating when it occurs in previously healthy children, ~,4 in w h o m it usually begins as a "flulike" illness. In association with this entity, endocarditis almost always involves t h e left side of the heart, and occurs in the absence of the classic signs usually associated With subacute bacterial endocarditis, 1-5 m a k i n g diagnosis difficult. S. a u r e u s endocarditis involving the tricuspid valve has been described only once before in a child with a normal heart. 6 W e describe two children (aged 5 a n d 8 years), with previously normal hearts a n d no evidence of immunodeficiency, with tricuspid valve endocarditis. Staphylococcus

CASE REPORTS Patient 1. This previously healthy 8-year-old girl with no history of heart disease or other significant previous illness had a 3-week history of symptoms of upper respiratory tract infection. During the following 2 weeks she had fever (temperature up to 40 ~ C), chills and joint pains, anorexia, and lethargy. There were no preceding intravenous or subcutaneous injections. On examination, she was pale and had tachypnea, a dry cough, and fever (temperature 39 ~ C). Rales were present in both lower zones. The first and second heart sounds were normal. There were no murmurs, but a faint pericardial friction rub was evident. The liver was palpable 2 cm below the right costal margin, and the spleen tip was palpable. Chest radiograph revealed normal heart size and consolidation in a small area in the left lower lobe.

Submitted for publication Dec. 15, 1986; accepted Dec. 31, 1986. Reprint requests: Vera Rose, M.D., Rm. 1503, Division of Cardiology, Department of Pediatrics, The Hospital for Sick Children, Toronto, Ontario, Canada M5G 1X8.

Hemoglobin was 82 g/L; and white blood cell count 10.0 • 106/L with polymorpholeukocyte count 3.0 • 106/L. There was marked thrombocytopenia (20 • 109/L platelets). Blood cultures grew S. aureus on four of four occasions. The combination of anemia, thrombocytopenia, and septicemia suggested a possible malignancy, and a bone marrow examination was carried out. On the basis of the findings, medullary histiocytic reticulosis was strongly suspected. Over the next few days respiratory distress became more marked, with concomitant worsening of clinical and chest radiographic signs of pneumonia and development of a right pleural effusion. Spiking fevers continued, despite in vitro sensitivity of the organism to methicillin, and treatment with 200 mg/kg/d Iv, maintaining bactericidal serum titers of 1 : 32. Two-dimensional echocardiography and Doppler scanning carried out on day 10 to confirm the presence of pericardial effusion revealed a large area of tricuspid valve vegetation (Fig. 1, A) an enlarged right atrium and right ventricle, and minimal tricuspid regurgitation. A pulmonary perfusion scan performed 2 days later revealed almost complete absence of perfusion of the left lung and patchy involvement of the right lung (Fig. 2, A). A subsequent digital subtraction angiogram revealed total occlusion of the left main pulmonary artery (Fig. 2, B). A repeat two-dimensional echocardiogram showed neither a change in size of the tricuspid vegetation nor progression of tricuspid regurgitation, but there was a further increase in the size of the right atrium and ventricle. The patient continued to have significant respiratory distress and spiking fevers, although blood cultures were now negative. Surgical exploration was carried out because of the child's clinical deterioration, sepsis, and right-sided heart failure. With cardiopulmonary bypass, a 2 X 3 cm area of vegetation was excised from the septal leaflet of the tricuspid valve. The majority of the septal leaflet and 1 cm of the anterior leaflet were excised as part of the debridement. Vegetations were teased off the anterior and posterior leaflets, which were left grossly intact. There was no evidence of myocardial abscess formation. A large septic thrombus occluding the left main pulmonary artery was removed. The patient had an uneventful convalescence. Antibiotic therapy was continued for 6 weeks. At discharge 8 weeks after admission, there was a 2/6 ejection systolic murmur at the left sternal border and mild cardiomegaly. The two-dimensional echo-Doppler examination showed no residual vegetation (Fig. 1,

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Fig. t. A, Parasternal short axis view of heart in patient 1, showing large (3 X 2 cm) area of vegetation (small arrows) on septal leaflet of tricuspid valve (large arrows). B, Same view of heart after removal of vegetation from tricuspid valve. Doppler ultrasound showed only trivial tricuspid regurgitation. AO, Aorta; LA., left atrium; RA, right atrium; RV, right ventricle.

Fig. 2. A, Pulmonary perfusion scan in patient 1 showing normal perfusion to right lung and markedly diminished perfusion to left lung (arrows indicate perfusion of apical segment of left upper lobe only). B, Digital subtraction angiogram showing almost total occlusion (arrows) of left main pulmonary artery and unobstructed flow to right lung.

B) and trivial tricuspid regurgitation. Pulmonary perfusion scan showed improved, though still abnormal, perfusion of the left lung. On follow-up (3 months) she continued to do well, with no clinical evidence of tricuspid regurgitation or right-sided heart failure. Patient 2. This 5-year-old previously healthy boy had a 2-week history of fever and sore throat, followed by cough, headache, anorexia, and weight loss. He had been given penicillin orally. On admission 4 weeks after onset of symptoms, he was anemic, with fever (temperature 40 ~ C), respiratory rate 50 breaths/min, and heart rate 125 beats/min. Both lung bases were dull to percussion; no rales were heard. A systolic ejection murmur was heard at the lower left sternal border. The liver was palpable 6 cm below the right costal margin, with evidence of ascites, but no splenomegaly. Hemoglobin was 72 g/L; and white blood cell count 17.0 x 106/L with 70% polymorphonuclear leukocytes. The platelet count was

116 • 109/L. Several blood cultures grew S. aureus. The patient was given cloxacillin 300 mg)kg/d Iv. Malignancy was suspected when computed tomography of the abdomen revealed nodular hepatomegaly and a retroperitoneal mass. Two-dimensional echocardiography, carried out on day 2 of admission because of the systolic murmur, revealed a large area of vegetation on the tricuspid valve. The right atrium and ventricle were moderately enlarged. Doppler evaluation was not available. Spiking fever continued, and S. aureus was repeatedly grown on blood cultures despite adequate antibiotic levels. Bilateral pleural effusion and peripheral edema were treated with furosemide and chest tube drainage. After the onset of pleuritic chest pain 4 weeks after admission, a lung perfusion scan showed a defect in the left lower lobe and tingula and patchy changes in the right lung. Two-dimensional echocardiography showed a slightly larger area

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of vegetation and further increase in size of the right ventricle. With cardiopulmonary bypass support, vegetation on all three leaflets of the tricuspid valve was completely excised. After surgery, recovery was uneventful, and the patient was discharged 8 weeks after admission. Cardiac catheterization t year later showed free tricuspid regurgitation, with equal pressures in the right atrium and ventricle. Two years after surgery, the child had severe right-sided heart failure, with marked exercise intolerance and pulsatile hepatomegaly. Symptomatic episodes of supraventricular tachycardia noted on routine ECG and frequent periods of second-degree heart block on 24-hour Holter monitoring were thought to be secondary to severe right-sided heart failure. At surgery, a porcine bioprosthesis was placed in the tricuspid valve position, with good results. The patient was discharged with sinus rhythm and requiring no cardiac medications. DISCUSSION Acute disseminated staphylococcal septicemia in childhood mimics an influenza-like illness.3,4 The overall incidence of endocarditis in staphylococcal septicemia is now thought to be less than 10%, 2 although specific data pertaining to the illness in childhood is not available. Both of our patients had a prolonged course prior to diagnosis, because of the nonspecific nature of the clinical presentation. The usual signs of subacute bacterial endocarditis are not evident in staphylococcal endocarditis. 3 In common with the only other reported case of tricuspid valve endocarditis in a child with a normal h e a r t : both of our patients had a protracted illness with spiking fever and respiratory symptoms, even after apparent sterilization of the blood in one patient. Major pulmonary embolization involving several lobes occurred in all three patients, and was one of the indications for surgical intervention in our two patients. The presentation and course of tricuspid valve endocarditis in these patients are different from that reported in adult patients 7~~most of whom are intravenous drug abusers, up to 40% of adults respond to medical treatment alone, ~~with the indications for surgical intervention being persistent infection, multivalve involvement, infection with organisms such as Pseudomonas and fungi, and severe right-sided failure: ,~~ Multiple small septic pulmonary emboli occur in up to 80% of patients, but are not predictive of outcome. Two-dimensional echocardiography is necessary for specific diagnosis?." Although the timing of the appearance of vegetation in relation to onset of symptoms is not clear, most areas 0f vegetation are present within 2 weeks of onset of illness) Neither the size nor the persistence of vegetation on two-dimensional echocardiograms have been found to correlate with outcome: ,9 In our two patients, major pulmonary embolization occurred with no detectable change in amount of vegetation. 9 The surgical management of tricuspid valve endocarditis

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in adults remains controversial. Although there is general agreement as to the indication for surgical excision of the tricuspid valve, some authors L2,~3advocate excision without valve replacement as a primary procedure and valve replacement only if right-sided heart failure occurs. This view is supported by the anticipated high risk of reinfection of a prosthesis in intravenous drug abusers, the majority of whom resume their habit after hospital discharge. More recently, however, because of the high incidence of late right-sided heart failure after valve excision alone, tricuspid valve replacement with a prosthesis, as a primary procedure, has been advocated) 4 Cardiac murmurs were unimpressive in both of our patients and the one reported by Goessler et al. 6 Tricuspid valve endocarditis should be considered in a child with major respiratory symptoms in the setting of staphylococcal septicemia. Early two-dimensional echocardiography should be carried out, and is probably merited in every child with acute staphylococcal sepsis and no obvious primary focus, particularly if fever does not abate despite adequate antimicrobial therapy. Pulmonary ventilation and perfusion scanning should be performed when tricuspid endocarditis is confirmed. Major pulmonary embolization may be more common in children with tricuspid valve endocarditis than in adults, and appears to be a reasonable further indication for surgical intervention, in addition to those already established, that is, persistent positive blood cultures, multivalve involvement, infection with unusual organisms, and severe right-sided heart failure. Even with subtotal excision of the tricuspid valve, valve replacement may still be necessary in children, as in the case presented by Goessler et a l : Valve replacement appears to be indicated when total tricuspid valvectomy is performed, to avoid the consequences of right-sided heart failure, but should likely be staged so that eradication of infection is more likely (and therefore contamination of the prosthesis less likely). This is particularly so because intravenous drug abuse is not a factor in younger children, making the risk of future endocarditis of the prosthetic valve lower than in adults who abuse drugs. REFERENCES

1. Wilson WR, Giuliani ER, Danielson GK, Gerfaci JE. General considerations in the diagnosis and treatment of infective endoearditis. Mayo Clinic Proc 1982;57:81. 2. Sheagren JN. Staphylococcus aureus: the persistent pathogen N Engl J Med 1984;310:1368. 3. Shulman ST, Ayoub EM. Severe staphylococcal sepsis in adolescents. Pediatrics 1976;58:59. 4. Hieber JP, Nelson AJ, McCracken GH Jr. Acute disseminated staphylococcal disease in childhood. Am J Dis Child 1977;131:181. 5. Thompson RI. Staphylococcal infective endocarditis. Mayo Clinic Proc 1982;57:106.

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6. Goessler MC, Riggs TW, Deleon S, Paul MH. Echocardiographic diagnosis of tricuspid valve endocarditis in a child with a normal heart. Pediatr Cardiol 1982;2:141. 7. Panidis IP, Kotler MN, Mintz GS, Segal BL, Ross JL. Right heart endocarditis: clinical and echocardiographic features. Am Heart J 1984;107:759. 8. Ginzton LE, Siegel RJ, Criley M. Natural history of tricuspid valve endocarditis: a two-dimensional echocardiographic study. Am J Cardiol 1982;49:1853. 9. Stewart JA, Silimperi D, Harris P, Wise NK, Fraker TD, Kisslo JA. Echocardiographic documentation of vegetative lesions in infective endocarditis:clinical implications.Circulation 1980;61:374. 10. Panidis IP, Kotler MN, Mintz GS, Ross J, Wever J. Clinical and echocardiographic correlations in right heart endocarditis. Int J Cardiol 1984;6:17.

The Journal of Pediatrics May 1987 11. Berger M, DelfinLA, Jelveh M, Goldberg E. Two-dimensional echocardiographic findingsin right sided infective endocarditis. Circulation 1980;61:855. 12. Barbour DI, Roberts WC. Valve excision only vs valve excision plus replacement for active infective endocarditis involvingthe tricuspid valve. Am J Cardiol 1986;57:475. 13. Arbulu A, Asfaw I. Tricuspid valvulectomy without prosthetic replacement: ten years of experience. J Thorac Cardiovasc Surg 1981;82:684. 14. Stern H J, Sisto DA, Strom JA, Soeiro R, Jones SR, Frater RWM. Immediate tricuspid valve replacement for endocarditis: indications and results J Thorac Cardiovasc Surg 1986;91:163.

Immunoglobulin E antibodies in young children with possible allergic symptoms N o r m a n Lewiston, M.D., Robert Hindi, M.D., Yao-Pi Hsu, M.S., J a m e s Lewiston, a n d Richard Moss, M.D. From the Allergy Diagnostic Laboratory, Children's Hospital at Stanford, and the Department of Pediatrics, Stanford University School of Medicine, Palo Alto, California

The diagnosis of allergic disease usually includes the demonstration of allergen-specific IgE antibodies by allergy skin testing or the in -r testing of serum. Comprehensive allergy testing by either method is relatively expensive and requires expertise in specific methods, equipment, and interpretation. Skin testing, particularly when intradermal testing is required, is uncomfortable and is often frightening to younger patients and their parents. There has been considerable interest, therefore, in the identification of "markers" of allergic disease that would assist the primary physician in deciding which patients would benefit from a complete allergy workup and those in whom the cost and discomfort of extensive testing may be unnecessary. We report our experience with the use of antigenselective radioallergosorbent testing in young children. Our group has suggested that a possible marker for the presence of allergic antibody in young children was the combination of an elevated serum concentration of IgE or the presence of IgE antibodies to either milk or egg2 We tested this hypothesis in our retrospective analysis.

Submitted for publication Dec. 18, 1985; accepted Dec. 15, 1986. Reprint requests: Norman Lewiston, M.D., Children's Hospital at Stanford, 520 Sand Hill Road, Palo Alto, CA 94304.

METHODS Referring physicians from several Stanford University clinics and from the community send sera to the Allergy Diagnostic Laboratory for in vitro testing for specific IgE antibodies. It is assumed that these physicians select those antigens that history taking and physical examination have suggested might be responsible for allergic disease. Inasmuch as patients are billed for these services, not every patient is tested for every antigen. We reviewed the results of RAST in 726 children younger than 6 years of age for PRIST RAST

Paper radioimmunosorbent test Radioallergosorbent test

allergens selected by their referring physicians. Total serum IgE was measured by Phaedezyme PRIST (Pharmacia Diagnostics, Piscataway, N.J.) and was considered elevated if :the value was higher than the geometric mean + 1 SD for age. 2 Antigen-specific IgE was measured by Phaedezyme RAST (Pharmacia.) A RAST result was considered positive if the results were class 1 or greater, using the scoring standards recommended by the manufacturer. Tests with RAST classes + / - and zero were considered negative? Results are reported by antigen group. Pollen represents