Systolic antegrade tricuspid blood flow—a sign of severe prosthetic valve stenosis

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Fig. 3. Autopsy specimen sliced through its long axis, showing a huge, gray-white, left atria1 tumor. The tumor infiltrates the mitral leaflet, chordae, and papillary muscle and extends to the left ventricular outflow tract. Note metastases in the myocardium and epicardium (arrows). IVS = interventricular septum; APA = anterior papillary muscle; LA = left atrium.

AA, Churg A, Sbarbaro JA, Sheppard JM, Lamberti J. Malignant fibrous histiocytoma of the heart presenting as an atria1 myxoma. Cancer 1978;42:2466. 4. Hamada N, Matsuzaki M, Kusukawa R, Fujii Y, Yamaki R, Masuda M, Tani S. Malignant fibrous histiocytoma of the heart. Jpn Circ J 1980;44:361. 5. Mori K, Itho H, Kanaya H, Onoe T, Ohka T, Lin S, Matsubara F, Ohmura K, Magara T, Tsuchiya K, Iwa T. Malignant fibrous histiocytoma of the heart. Jpn Circ J 3. Shah

1983;47:188.

Eckstein R, Gijssner W, Rienmiiller R. Primary malignant fibrous histioytoma of the left atrium. Br Heart J 1984;52:354. 7. Come PC, Riley MF, Markins JE, Malagold M. Limitation of echocardiographic techniques in evaluation of left atria1 masses. Am J Cardiol 1981;48:947. 8. Panidis IP, Mintz GS, McAllister M. Hemodynamic consequences of left atria1 myxomas as assessed by Doppler ultrasound. AM HEART J 1986;111:927. 6.

Systolic antegrade tricuspid blood flow-a sign of severe prosthetic valve stenosis Barry P. Rosenzweig, M.D., Itzhak Kronzon, M.D., Frederick Feit, M.D., Peter J. Stecy, M.D., Mark S. Nachamie, M.D., and Frank Politzer, M.D. New York, N.Y.

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The characteristic auscultatory findings of tricuspid valve disease have been previously described.l Both the diastolic rumble of tricuspid stenosis and the holosystolic murmur of tricuspid regurgitation typically increase in intensity with inspiration. We recently observed a patient with a malfunctioning bioprosthetic tricuspid valve in whom the murmur of tricuspid regurgitation disappeared upon inspiration. Doppler echocardiography and cardiac catheterization proved this finding to be the result of severe prosthetic tricuspid valve stenosis. A 46-year-old black man, a former intravenous drug abuser, presented with anasarca. Ten years prior to admission, he underwent tricuspid valve replacement with a No. 31 Hancock prosthesis for severe tricuspid regurgitation and endocarditis unresponsive to medical therapy. Physical examination on admission revealed an irregularly irregular pulse at 90 bpm, marked jugular venous distention at 45 degrees without discernible pulsations, hepatomegaly, and anasarca. On cardiac examination, S, and S, were normal and no gallop was noted. A grade 3/4 diastolic rumble was heard at the left sternal border and apex, and a left parasternal diastolic thrill was palpable. Both the murmur and the thrill increased in intensity and duration with inspiration. A grade 216 holosystolic blowing murmur was heard at the lower left sternal border. This murmur, however, disappeared with inspiration. The chest x-ray film showed cardiomegaly suggesting right atria1 enlargement, and no pulmonary venous engorgement. The ECG was remarkable for atria1 fibrillation with a ventricular response of 90 per minute and nonspecific ST and T wave changes. The M-mode and two-dimensional imaging studies showed normal left heart structures and a dilated right

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Fig. 1. Pulsed Doppler echocardiogram. A, Recording with the sample volume in the right ventricle just distal to the tricuspid orifice. The maximum flow velocity is increased as seen with tricuspid stenosis and varies with the respiratory cycle. The systolic periods following diastoles labeled 1 and 6 are associated with failure of the spectral curve to return to the zero kHz baseline, indicating persistent antegrade flow. EXP = expiration; INS = inspiration. B, High-speed (50 mm/set) recording with the sample volume just distal to the tricuspid valve orifice. Note the high peak flow velocity (1.7 to 2.7 m/set), and the prolonged pressure half-time (320 to 600 msec). After the first diastole, during inspiration, antegrade flow continues throughout systole (arrow). C, Recording with the sample volume in the right atrium just proximal to the tricuspid valve reveals tricuspid regurgitation (horizontal arrows). However, when the spectral tracing fails to return to baseline, indicating persistent antegrade flow (vertical arrow), there is no tricuspid regurgitation. EXP = expiration; INS = inspiration; TR = tricuspid regurgitation.

atrium. The porcine tricuspid valve leaflets appeared thickened and their motion was grossly diminished. Pulsed Doppler study with the sample volume just distal to the tricuspid orifice demonstrated a high velocity jet. Instantaneous gradients were calculated by means of the modified Bernoulli equation,s3 P = 4V2, where P is the instantaneous gradient and V is the maximum velocity. The calculated mean tricuspid valve gradient varied from beat to beat, and ranged from 9 to 25 mm Hg (average of the means, 15). In addition, the rate of decay of the instantaneous Doppler flow velocity and therefore of the pressure gradient was slow. (Calculated pressure halftime = 320 to 600 msec, mean = 450 msec. Normal in our

laboratory = 60 to 90 msec). These findings are diagnostic of severe tricuspid stenosis.4 Fig. 1, A demonstrates that both the velocity and duration of antegrade tricuspid flow vary markedly throughout the respiratory cycle. The maximum antegrade flow velocity and therefore pressure gradient is noted during inspiration. During expiration (beats numbered 3, 4, and 5), it appears that antegrade flow persists during early systole. This is suggested by extrapolation of the spectral tracing to the baseline at a point well into the ST segment of each of these beats. Ilowever, during inspiration, flow persists throughout all of systole in an antegrade direction. This is demonstrated by failure of the spectral tracing to reach the zero kHz

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Time (seconds) Fig. 2. Simultaneous right atria1 and right ventricular pressuretracings. Right ventricular systolic and diastolic pressuresboth decreasewith inspiration, while right atria1 pressureremains nearly constant. During inspiration, right ventricular pressure is exceeded by right atria1 pressurethroughout systole. ECG = electrocardiogram; EXP = expiration; INS = inspiration; RA = right atria1 pressure;RV = right ventricular pressure.-

baselineafter beats numbered 1 and 6. Fig. 1, B depicts a high-speed recording in which the prolonged pressure half-time and persistent systolic antegrade tricuspid flow are demonstrated. With the sample volume in the right atrium behind the tricuspid valve, retrograde turbulent flow indicating tricuspid regurgitation was detected and wasshownto be of moderate degreeby mapping.5Fig. 1, C demonstratesthat the tricuspid regurgitation disappears upon inspiration. The left-sided pressures were normal. The cardiac output measured 5.7 L/min. There was mild pulmonary hypertension (42/21 mm Hg) and marked elevation of right atrial pressure (mean = 38 mm Hg). Of note is the only minimal change in right atria1 pressure during the respiratory cycle (Fig. 2). A large diastolic gradient across the tricuspid valve was recorded. This gradient varied greatly through the respiratory cycle. The largest diastolic gradient is seenduring inspiration, when the right ventricular diastolic pressuredecreases(Fig. 2). In addition, the drop in right ventricular systolic pressurewith inspiration brings it to a level lessthan the simultaneousright atria1 pressure. This creates a pressure gradient for antegrade transtricuspid flow during systole. Right ventricular angiography demonstrated mild to moderate tricuspid regurgitation. However, one cannot exclude that someor even all of the regurgitation is catheter-induced. The patient underwent percutaneousballoon dilatation of the tricuspid valve prosthesiswith subsequentimprovement in his clinical status. The details of this technique are reported by Feit et al6 The findings in this patient are unusual and may be related to the marked severity of the transvalvular diastolic gradient. Most patients with tricuspid stenosis have associatedmitral valve disease,an increased pulmonary vascular resistance,and a decreasedcardiac 0utput.l The

normal cardiac output in the presenceof severetricuspid stenosis leads to an extreme elevation of right atria1 pressurein this case.It is not clear why this right atria1 pressuredoesnot vary with the changesof intrathoracic pressureinduced by respiration. We hypothesize that the failure of right atria1 pressureto decreasewith inspiration is the consequenceof simultaneous filling of the right atrium by augmentedsystemicvenous return and a severe impediment to right atria1 emptying. Conversely, right ventricular pressuresdrop during inspiration due to the fall in intrathoracic pressure without a concomitant increase in filling. The net result is a pressure gradient from right atrium to right ventricle throughout systole during inspiration. Curiously, there is some degree of antegrade flow during early systole, even during the expiratory phase of respiration. The time required for right ventricular systolic pressureto exceedthe extremely elevated right atria1 pressureis prolonged, as seenin Fig. 2, allowing for this antegradeflow in early systole.It is also possible that right ventricular systolic dysfunction may contribute to this delay. Doppler echocardiographyis useful in the evaluation of native and prosthetic valvular dysfunction. Accurate measurement of instantaneous peak and mean transvalvular gradients is readily provided and quantitative data concerning regurgitant lesions may be obtained.5,8In this patient, the Doppler study demonstratesthat blood flows from the right atrium to the right ventricle throughout the cardiac cycle during inspiration. Tricuspid regurgitation cannot occur unless the right atria1 pressurefalls below right ventricular pressure.Our Doppler study showsthat during inspiration, tricuspid regurgitation disappears. Thus, this unusualpattern of pressuresand flows explains the disappearanceof the tricuspid regurgitation murmur with inspiration as noted on physical examination. A

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“paradoxical” decrease in the intensity of a tricuspid regurgitation murmur during inspiration may therefore indicate associated severe tricuspid stenosis. REFERENCES 1.

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Braunwald E, ed. Heart disease: A textbook of cardiovascular medicine. 2nd ed. Philadelphia: W. B. Saunders Co, 1984:1439. Holen J, Aaslid R, Landmark K, Simonsen S. Determination of pressure gradient in mitral stenosis with a non-invasive ultrasound Doppler technique. Acta Med Stand 1976; 199:455-60. Hatle L, Brubakk AO, Tromsdal A, Angelsen B. Noninvasive assessment of pressure drop in mitral stenosis by Doppler ultrasound. Br Heart J 1978:40:131-40. Miyatake K, Okamoto M, Kinoshita N, Ohta M, Kozuka T, Sakkakibara H, Nimura Y. Evaluation of tricuspid regurgitation by pulsed Doppler and two dimensional echocardiography. Circulation 1982;66:777-89. Veyrat C, Kalmanson D, Farjon M, Manin JP, Abitol G. Noninvasive diagnosis and assessment of tricuspid regurgitation and stenosis using one and two dimensional echo-pulsed Donnler. Br Heart J 1982:47:596-605. FeitF, Stecy P, Nachamie M. Percutaneous balloon valvuloplasty for porcine prosthetic valve stenosis. Am J Cardiol 1986;58:363-4. Smith JA, Levine SA. Clinical features of tricuspid stenosis. AM HEART J 1942;23:739. Hatle L, Angelsen B. Doppler ultrasound in cardiology. Physical principles and clinical applications. 2nd ed. Philadelphia: Lea & Febiger, 198597-205.

Metastatic liposarcoma of the right ventricle with outflow tract obstruction: Restrictive pathophysiology predicts poor surgical outcome Pamela Bartels, M.D., William G. O’Callaghan, M.B., M.R.C.P.I., Robert Peyton, M.D., Gulshan Sethi, M.D., Thomas Maley, M.D. Asheville, N.C.

and

Liposarcoma is an uncommon primary neoplasm that very rarely metastasizes to the heart. In one series of 53 tumors over a 17-year period, no cardiac metastases were 0bserved.l We describe the clinical features, diagnosis, and surgical treatment of a metastatic liposarcoma of the heart that was largely intracavitary. M.C. was a 64-year-old man whose past history was remarkable for incomplete surgical excision of a liposarcoma from the left scapular area in June, 1985, with subsequent radiotherapy. Presenting symptoms on admission were a S-week history of increasing dyspnea and lethargy with associated abdominal distension. Physical From tration Reprint Center,

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examination revealed moderate respiratory distress. There was marked jugular venous distension. Sinus tacbycardia was observed and cardiac auscultation revealed normal heart sounds and an ejection systolic murmur at the pulmonic area that varied in intensity with patient position. A pericardial friction rub was present at the lower left sternal border. Respiratory examination revealed crepitations in the left base only. Examination of the abdomen revealed tender hepatomegaly. Ascites was present, as was moderate presacral edema. Chest x-ray examination showed slight cardiomegaly with no evidence of parenchymal or pleural disease. ECG showed sinus tachycardia with deep T wave inversion and poor R wave progression in the precordial leads. The clinical diagnosis was one of severe right-sided heart failure, and cardiac catheterization was undertaken. Right and left heart catheterization were performed. The mean right atria1 pressure was 25 mm Hg and the phasic pressure trace (Fig. I, A) showed a prominent Y descent and plateau. The right ventricular pressure was elevated at 85/25 mm Hg and showed a square root sign (Fig. 1, B). A 34 mm Hg systolic gradient was present between the right ventricular outflow tract and the pulmonary artery. Right ventricular end-diastolic pressure was 25 mm Hg and left ventricular end-diastolic pressure was 9 mm Hg (Fig. 1, C), Contrast ventriculography of the right ventricle (Fig. 2) showed a polypoid filling defect extending from the right ventricular apex through tbe pulmonic valve to the main and right pulmonary arteries. Coronary arteriography showed a late vascular blush on the right side of the interventricular septum. A diagnosis of right ventricular mass with outflow obstruction and restriction of the right side of the heart was made. The patient was referred for urgent thoracotomy. At surgery, a multilobular polypoid mass was observed to extend from the right ventricular apex through the pulmanic valve to the pulmonary arteries. The mass was resected toward its base, which extensively involved the interventricular septum and free wall. Cardiopulmonary bypass was discontinued without difficulty. However, despite removal of the intracavitary mass, severe rightsided heart failure persisted and the patient died from cardiac failure 14 hours following surgery. The tumor removed at surgery was a liposarcoma that measured 10 X 6 X 3 cm. At autopsy, the right ventricular wall showed residual tumor adhering to the endocardium with focal invasion of the myoeardium. The histology of cardiac tumor was identical to that of the primary tumor. Examination of the lungs revealed numerous tumor emboli in the pulmonary arteries. This case represents a clinical entity that is extremely rare, consisting of largely intracavitary metastases of a relatively rare primary tumor. To our knowledge, there are only two reported cases of surgical excision of an intracavitary right ventricular liposarcoma.2,3 These were both successfully removed, but the published data do not comment on the physiologic features encountered at catheterization. In our case there was evidence of restrictive as well as obstructive physiology on the right-sided cardiac pressure recordings. The disparity between left