Echocardiographic appearance of a flail bioprosthetic mitral valve leaflet mimicking vegetation

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Fig. 2. Cardiac NMR short-axis views of the heart. A massof bright signal intensity surrounds the entire heart (solid arrows), is more dominant over the right ventricular wall, and is both intrinsic and extrinsic to the pericardium (closed triangle). The brightness of the signal intensity is comparableto that of the subcutaneousfat of the anterior chest wall (open arrow). LV, Left ventricle.

up to a point, with age. The deposits are largest in the atrioventricular sulci and about the anterior and posterior descendingcoronary arteries3 It has been observed that the amount of adipose tissue surrounding the heart is proportional to the amount of fat present in other body tissues;however, exceptions have been reported involving considerablequantities of subepicardial adiposetissue in personsof normal body weight and vice versa.4Nonspecific symptoms of dyspnea and/or chest discomfort can occur but are dependent upon the size and location of the lipomatous tissue.5s6In this patient, the cardiac NMR images clearly defined and characterized a lipomatous massencircling the heart, suggestingthat the patient’s symptoms were related to this abnormality. Subsequent cardiac catheterization defined the physiologic compressive effects of the mass.The limited surgical intervention of pericardiotomy resulted in marked symptomatic improvement of the patient. The observed symptomatic improvement is postulated to be secondary to partial decompressionof the heart resulting from releaseof the lipoma from the confines of the parietal pericardium. REFERENCES

1. McAllister HA. Primary tumors and cysts of the heart and pericardium. Curr Probl Cardiol 1979;4:1-51. 2. Dixon WT. Simple proton spectroscopic imaging. Radiology 1984;153:189-94.

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Fig. 3. Intraoperative photograph of the heart with the apex pointing downward. The extensive iipoma surrounding the heart is shown.

Roberts WC, Spray TL. Pericardiil heart disease: a study of its causes, consequences, and morphologic features. Cardiovast Clin 1976;7:11-65. Roberts WC, Roberts JD. The floating heart or the heart too fat to sink: analysis of 55 necropsy patients. Am J Cardiol 1983;52:1286-9. Morere P, Ftain JP, Hertzog P, Nouvet G, Denoix C. Les lipomes mediastinaux. A propos d’une observation. Ann Chir Thorac Cardiovasc 1972;11:417-22. Moulton WL, Jaretzki A, Bowman FO, Silverstein EF, Bregman D. Massive lipoma of heart. NY State J Med 1976; 76:1820-5.

Echocardiographic appearance of a flail bioprosthetic mitral valve leaflet mimicking vegetation Charles M. Gross, MD, L. Michael Prisant, MD, Domenic Paolini, MD, and Thomas W. von Dohlen, MD. Augusta, Ga. From the Sections of Cardiology, Hypertension, and Thoracic and Cardiac Surgery, Medical College of Georgia and Veterans Administration Medical Center. Reprint requests: Charles M. Gross, MD, Section of Cardiology, Medical College of Georgia, Augusta, GA 30912.

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Though echocardiography is the noninvasive method of choice for the evaluation of prosthetic heart valves, its ability to differentiate bioprosthetic dysfunction caused by endogenousdegenerationfrom that causedby infective endocarditis remains limited. It has been proposed that antegrade extension of leaflet-associatedechoesbeyond the stent of a bioprosthesisis the only echocardiographic sign that is completely specific for the presence of a vegetation.’ We report our findings in a patient with hioprosthetic disruption but without any grossvegetations

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in whom the echocardiogram demonstrated a falsepositive example of this sign. A 46-year-old woman with a Hancock mitral bioprosthesisimplanted 9 years earlier becauseof mitral stenosis wasreferred to our institution for evaluation of prosthetic valve dysfunction. She had been in good health until 3 months previously when an episodeof “viral pneumonia” was treated with antibiotics. Subsequently, a new or altered heart murmur and progressivesymptoms of pulmonary venous congestiondeveloped. During the 2 to 3

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Fig. 1. A and B, Parasternal long axis view in diastole demonstrating mobile massprotruding into left ventricular outflow tract beyond stent. Depth scale markers are 1 cm apart. C, M-mode echocardiogram obtained with cursor passingthrough tip of mass,just to left of cursor position shownin A. The mass(M) protrudes beyond stent (ST) in diastole and recedesinto it in systole. Note fine fluttering of massin diastole (open arrows). Paper speedis 50 mm/set with depth scale markers at l-second intervals. AO, Aortic root; LA, left atrium; LV, left ventricle; LVOT, left ventricular outflow tract; RVOT, right ventricular outflow tract; ST, valve stent.

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Fig. 2. Parasternal long axis view in systole demonstrating leaflet echoesprolapsing into left atrium, indicative of flail leaflet(s). Depth scale markers are 1 cm apart. Abbreviations sameas in Fig. 1.

weeksbefore admissionto the hospital, shebecamefebrile to 101’ to 102.5’ F orally and had drenching night sweats. She had been taking sulfisoxazole on a more or lessdaily basissince valve replacement, presumably for secondary prevention of rheumatic fever in the face of a putative penicillin allergy, but had taken none for approximately 2 weeks before admission. Physical examination demonstrated a blood pressure of 150/70 mm Hg, pulse of 88 bpm, temperature of 99.9’ F orally, and she appeared chronically ill. No peripheral stigmata of infective endocarditis were present. Cardiac examination revealed a heave at the left sternal border, accentuation of the first heart sound and physiologic splitting of the secondheart sound. A grade III/VI holosystolic cooing murmur was heard at the apex and left sternal border with radiation to the axilla, and there were no gallop sounds.The liver and spleen were not palpable. Hemoglobin and hematocrit levels were 13.5 gm/dl and 38.7%, respectively, and red blood cell morphologic features were normal. The white blood cell count was 8100/mm3with a shift to the left and relative lymphopenia. The Westergren erythrocyte sedimentation rate was21 mm/hr. A urinalysis revealed 0 to 5 red cells per high power field but no white cells. The electrocardiogram demonstrated right ventricular enlargement or volume overload and right atria1 enlargement. The chest radiograph showed pulmonary vascular engorgementwithout pulmonary edema. The echocardiogramdemonstrated what appearedto be a 5 mm x 20 mm mobile massattached to the leaflets of the bioprosthesis.In diastole (Fig. l), the massprotruded into the left ventricular outflow tract just beyond the stent

and displayed fine fluttering. In systole (Fig. 2), leaflet echoesprolapsed into the left atrium, indicating a flail leaflet.*13Doppler and color flow mapping studiesdemonstrated moderate to severe mitral regurgitation but no abnormal impediment to antegrade flow through the prosthesis. Trace aortic regurgitation and moderate tricuspid regurgitation were also present. A pulmonary artery systolic pressureof 80 to 90 mm Hg wasestimated from the continuous wave Doppler recording4 and was subsequently confirmed by flow-directed catheterization. Becausethe history strongly suggestedinfective endocarditis and the echocardiogram demonstrated a mass consistentwith a vegetation, four setsof blood samplesfor culture, two of the setscontaining an aerobic resin bottle, were obtained and therapy with vancomycin, rifampin, and gentamicin wasbegun, On the third hospital day, the bioprosthesiswasreplaced with a St. Jude Medical 25M101 mechanical prosthesis. The explanted bioprosthesis(Fig. 3) revealed changes typical of chronic endogenousdegeneration. One of the leaflets wasalmost completely avulsed from the stent and was essentially flail about its remaining point of attachment. Another leaflet had tears along its free edge with loss of integrity of the line of coaptation. On gross pathologic examination, the leaflets were calcified and had a thin coating of fibrin. No evidence of infection was present grossly. On microscopic examination, no organisms were present, although it must be noted that an “exudative area” was not included in the section. The patient continued to receive antibiotics for 2% weeks postoperatively while awaiting the final results of

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Fig. 3. A, Explanted valve viewed from ventricular aspect, simulating diastole. Leaflets demonstrate grosscalcification and thickening, changestypical of chronic endogenousdegeneration. There is leaflet avulsion from the portions of stent at left and in foreground. Note that hail tip of leaflet is even with end of stent and that any further antegrade movement during diastolic inflow would cause it to protrude beyond stent. B, Explanted valve viewed from atria1 aspect. Forceps hold avulsed leaflet in a position simulating systole and demonstrating flail nature of leaflet. Raggedtip of torn leaflet just to left of forceps is sameportion that in diastole protruded antegrade beyond stent.

blood cultures. Once thesecultures and the culture of the prosthesis itself proved to be negative, and since no organismshad beendetected on the microscopicexamination, the antibiotics were discontinued. The patient was dischargedfrom the hospital 23 days after admission. Of the six reports’,3.5-8 that have touched on the ability of echocardiography to distinguish bioprosthetic valve dysfunction as a result of endogenousdeterioration from that causedby infective endocarditis, only one’ has proposed specific criteria that enable this distinction to be made. Antegrade extension of leaflet-associated echoes beyond the stent of a bioprosthetic valve wassaid to have a predictive accuracy and specificity of 100% for the presenceof a large vegetation, but it wasspeculatedthat a very large cuspaltear might allow leaflet tissuealone to be detected beyond the stent during antegrade flow. In one other paper a passingreference is madeto visualization of mitral leaflet tissue outside the stent in diastole in the setting of endogenousdegeneration5but no specific comment is made regarding the significance of this finding. One additional report, in describing a mitral bioprosthesis with a flail leaflet caused by endogenousdegeneration, presents an M-mode recording showing fine diastolic fluttering of what is probably the disrupted leaflet outside the stent.g Again, however, this finding is not commented on either in the text or in the figure legend. Last, echocardiographic visualization of leaflet tissue beyond the bounds of the stent may occasionally be a normal finding, at least in the caseof the Ionescu-Shiley bovine pericardial bioprosthesisin the mitral position.7T lo To our knowledge, this is the first reported case in which extension of leaflet-associatedechoesbeyond the

stent of a disrupted bioprosthetic mitral valve represented leaflet tissue only without a large superimposedvegetation. Thus a patient presenting with this solitary echocardiographic sign alone, in the absenceof any suggestionof infection, should not necessarily be assumedto have endocarditis. However, when there is even a suggestionof concomitant infection, asin our patient, prompt initiation of therapy for infective endocarditis on a presumptive basisis clearly indicated. REFERENCES

1. Effron MK, Popp RL. Two-dimensional echocardiographic assessment of bioprosthetic valve dysfunction and infective endocarditis. J Am Co11 Cardiol 1983;2:597-606. 2. Alam M, Madrazo AC, Magilligan DJ, Goldstein S. M mode

and two dimensionalechocardiographic featuresof porcine 3.

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valve dvsfunction. Am J Cardiol 1979:43:502-g. Schapira JN, Martin RP, Fowles RE; Rakowski H, Stinson EB, French JW, Shumway NE, Popp RL. Two dimensional echocardiographic assessment of patients with bioprosthetic valves. Am J Cardiol 1979;43:510-9. Skjmrpe T, Hatle L. Noninvasive estimation of pulmonary artery pressure by Doppler ultrasound in tricuspid regurgitation. In: Spencer MP, ed. Cardiac Doppler diagnosis.volume I. Dordrecht: Martinus Niihoff. 1984:247-54. Alam M, Lakier JB, Pick”ard SD, Goldstein S. Echocardiographic evaluation of porcine bioprosthetic valves: experience with 309 normal and 59 dysfunctioning valves. Am J Cardiol 1983;52:309-15. Grenadier E, Salm DJ, Roche AHG, Valdes-Crux. LM, Copeland JG. Goldberg SJ, Allen HD. Detection of deterioration or infection of homograft and porcine xenograft bioprosthetic valves in mitral and aortic positions by two-dimensional echocardioeranhic examination. J Am Co11 Cardiol 1983; 2:452-g. - Solana LG, Pechacek LW, DeCaatro CM, Klima T, Cooley DA. Two-dimensional echocardiographic assessment of com-

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plications involving the Ionescu-Shiley pericardial valve in the mitral position. J Am Co11 Cardiol 1984;3:328-33. Nagata S, Park Y-D, Nagae K, Beppu S, Kawaxoe K, Fujita T, Sakakibara H, Nimura Y. Echocardiographic features of bioprosthetic valve endocarditis. Br Heart J 1984;51:263-6. Alam M, Rosman HS, Lakier JB, Kemp S, Khaja F, Hautamaki K, Magilligan DJ Jr, Stein PD. Doppler and echocardiographic features of normal and dysfunctioning bioprosthetic valves. J Am Co11 Cardiol 1987;10:851-8, Figure 3. Pechacek LW, Solana LG, DeCastro CM, Edelman SK, Garcia E, Hall RJ. Noninvasive assessment of the IonescuShiley pericardial xenograft in the mitral position: preliminary experience. Tex Heart Inst J 1982;9:275-84.

Patrick Hallali, MD, Bernard Iung, MD, Alain Davido, MD, Jean-Paul Binet, MD, Henri Leriche, MD, and Pierre Corone, MD. France

Congenital cardiac diverticula, first describedby O’Bryan, are rare, their incidence being estimated at 0.013%.l The present report concernstwo casesof congenital muscular diverticula arising from the baseof the right ventricle, one associatedwith tetralogy of Fallot, the other with ventricular septal defect. Case No. 1. A Z-month-old boy wasreferred for the first time becauseof cyanosis while crying. Clinical examination was consistent with the diagnosis of tetralogy of Fallot, but chest x-ray films showedcardiomegaly (cardiothoracic ratio 0.73) with significant prominence of the right cardiac border. Electrocardiogram (ECG) showed right bundle branch block. Echocardiography revealed a large perimembranousseptal defect associatedwith pulFrom the Department of Medicine and the Department of Cardiac Lannelongue, Le Plessis-Robinson. Reprint requests: Patrick Hallali, Mbdecine, 83 Blvd. de l’H8pital,

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and Cardiology, HBpital la Pitie, Paris; Surgery, Centre Chirurgical MarieMD, HBpital 75651 Paris

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stenosis, suggesting a diagnosis of tetralogy of Fallot. The radiologic and electrical findings, however, were atypical. Cyanosis became more marked over the subsequent17 months, at which time catheterization and angiocardiography were performed (Table I), confirming an atypical tetralogy of Fallot with a large perimembranousventricular septal defect with a right-to-left shunt, a tight infundibular and valvular pulmonary stenosis,and the existence of a large diverticulum arising from the base of the right ventricle anterior to the tricuspid ring compressing the right atrium and displacing the right coronary artery, two branches of which encircled the baseof

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and the intraluminal pressureswere equal (Fig. 1). After a further period of 2 years, increasing cyanosisand polycythemia indicated that surgical treatment was necessary. At operation (Fig. Z), the diverticulum was large (diameter 50 mm) and wassituated on the anterior wall of the right ventricle in front of the tricuspid annulus, opposite the ventricular septal defect. Surgical repair included ventricular septal defect closurewith a patch, pulmonary valvotomy, infundibular enlargementwith a patch, resection of most of the diverticulum leaving only 15 mm, and closure with a Teflon patch. The microscopic examination of the resectedspecimenrevealed an absent epicardium replaced by a fibrous shell, a hypertrophic myocardium with an atypical plexiform structure causing a deformity of the endocardium, and a fibrous and thickened endocardium without thrombosis. The postoperative course was uneventful and at 6 months postoperatively the child was asymptomatic and acyanotic. Case No. 2. A 3-week-old boy with a cardiac murmur wasreferred becauseof failure to thrive. Clinical examination suggestedthe presenceof a ventricular septal defect. Chest x-ray films demonstrated cardiomegaly (cardiothoracic ratio 0.64) with increasedpulmonary vascular markings. ECG showedright bundle branch block. Echocardiography showed a large ventricular septal defect in the muscular portion of the septum. The findings at cardiac catheterization at the age of 5 months are detailed in Table I. Angiography confirmed a large ventricular septal defect and demonstrated a large contractile and trabeculated diverticulum of the anterolateral wall of the right

I. Cardiac catheterization data Case No. 2

CaseNo. 1 Pressure (mm Hg) Right atrium (mean) Right ventricle Pulmonary artery Pulmonary artery wedge (mean) Left atrium Left ventricle

6 8612-g la/8 iz 8 8413-8

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the diverticulum. Contraction of the wall of the diverticulum was vigorous and synchronous with the right ventricle

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Oxygen saturation (%o) 60 63 58 97 89

Pressure (mm Hd 4 8010 78128 = 12 82/O

Oxygen saturation (%) 54 89 89 96