intraventricular throm

vera :

A. Olusegun Fayemi, M.D.** Daniel Malcolm” * * Evalynne V. Braun, M.D.**** Teanech, N. J.

ase report

A 72-year-old man was hospitalized for massive edema of the iegs, increase in abdominal girth of recent onset, weakness, and anorexia. He bad previously been healthy, and he specif ically denied antecedent hypertension or heart disease. A diagnosis of congestive heart failure was made on the basis of findings of cardiomegaly, pitting dependent edema, ascites, hepatomegaly, and bilateral basal pulmonary rdles.

Pertient laboratory data were as follows: hemoglobin 18.4 Gm./dl.; hematocrit 63%; RBC 6.73 million/cu. mm.; WBC 13JOQ/cu. mm. with 48% mature neutrophiils, 6% bands, 18% lymphocytes, 27% eosinophils, and 1% basophils; platelets 3O,OOO/cu. mm. Leukocyte alkaline phosphatase score was 162 (control:76). Total blood volume was 19,500 ml. (predicted value:5,700 ml.).; the red cell mass was 9,000 ml. (predicted value?&550 ml.). The results of serum chemical analysis were as follows: LDH, 590 MU./ml. (laboratory normal 90 to 200 MU./ml.); SGOT, 90 MU./ml. (laboratory normal 10 to 50 MU./ml.); total bihrubin, 3.8 mg./dl.; total protein, 5.8 Gm./ dl.; albumin, 2.94 Gm./dl.; uric acid, 9.5 mg./dl.; BUN, 19 mg./dl.; calcium, 8.1 mg./dl.; and alkaline phosphatase, 90 MU./ml. (laboratory normal 30 to 85 MU./ml.). The serum electrolyte values were within normal limits except for a slightly elevated value for serum sodium of 148 mEq./L. Chest

From the Departments of Pathology and Medicine of the Holy Nzme Hospital, Teaneck, N. J.

Received for publication June 4, 1979.

Accepted for publicatio,? July 19, 1979.

Reprint requests: Dr. Majid Ali, Director of Laboratories, Holy Name Hospital, Teaneck, N. 9. 07666.

*Director of Laboratories, Holy Name Hospital, Teaneck, N. 9.; Ass’t. Professor of Pathology (Adj.), College of Physicians and Surgeons, Columbia University, New York, N. Y.

**Attending Pathologist, Holy Name Hospital, Teaneck, N. 9.; Ass’t. Clinica! Professor of Pathology, The Mount Sinai School of Medicine,

New York, N. Y.

***Attending Physician. Department of Medicine, Holy Name Hospi- tal, Teaneek, N.J.

****Attending Pathologist, Holy Name Hospital, Teaneck, N.J.; Ass’t. Professor of Pathology (Adj.), Fairleigh Dickinson School of Dentistry,

Teaneck, N.J.

roentgenogram demonstrated an enlarged heart and left pleural effusion. Gastrointestinal series and intravenous pyei- ogram studies were normal except for displacements of stom- ach and left kidney by an enlarged spleen. Liver scan with Au’“” showed hepatomegaly without focal intrahepatic lesions. The ECG showed ST-T wave abnormalities. Bone marrow aspiration was dry, but a biopsy showed pronounced erythroid hyperplasia, megakaryocytosis, eosinophiha, and fibrosis.

With the clinical diagnosis of congestive heart failure secondary to arteriosclerotic coronary artery disease, the patient was treated with digitalis, diuretics, and phlebotomies. Lowering of hematocrit to below 50% was achieved by periodic phlebotomies. However, the congestive failure proved to be intractable and he died 6 months later of refractory congestive failure with left pleural effusion, massive ascites, and anasar- ca.

athology

At autopsy, the heart was hypertrophied and weighed 480 grams, the maximum thickness of the right and the left ventricular walls being 0.3 and 1.8 cm., respectiveiy. A massive intramural thrombus covering the endocardial surface of the left ventricle in its entirety was observed. The thrombus extended from the apex of the heart, and reduced the capacity of the left ventricle by about 75% (Fig. 1). Microscopically the thrombus displayed various stages of organization; the surface exposed to the blood flow was well endothelialized. All the major branches of both the right and left coronary arteries showed minimal arteriosclerotic changes. No significant nar- rowing of the arterial lumma was demonstrable. The myocar- dium beneath the intramural thrombus was studied by multi- ple sections. No evidence of any recent or remote infarct was observed.

In the gross examination of the various viscera, severe passive venous congestion was observed, especially in the lungs and the liver. The liver weighed 2,400 grams, and on cut section showed a nutmeg appearance. The large pulmonary arteries showed prominent arteriosclerotic plaques. The spleen was massively enlarged, weighed 1,200 grams and, on serial sections, displayed many areas of infarction. Histologi- cally, pronounced extramedullary hematopoiesis and diffuse

520 October, 1980, Vol. 100, No. 4 0002-8703/80/100520 + 03$00.30/O 0 1980 The C. V. Mosby Co.

Intrauentricular thrombosis in polycythemia uera

Fig. 1. Massive intraventricular thrombus. The thrombus surface is smooth and well-endothelialized (AA); the underlying myocardium shows no evidence of recent or old infarction (BB,J.

sinusoidal proliferation was observed in the spleen; the splenic infarcts showed vaious stages of organization. Extramedullary hematopoiesis was also observed in the liver, the lungs, and the retroperitoneal and mediastinal lymph nodes. In the lungs, muscular hyperplasia of peripheral, subpleural arteri- oles was observed. The marrow displayed pronounced hyper- plasia involving all the marrow elements; megakaryocytes and eosinophils were especially abundant.

Discussion

Cardiac function and cardiac output are usual- ly normal in polyeythemia Vera.*. i The clinical course of the patient reported in this paper was highly unusual; while the diagnosis of polycy- themia vera was readily made by the bematologic studies and blood volume determinations, the true nature of its relationship with congestive heart failure remained masked. The probability of the cardiac failure being secondary to arterioscle- rotic coronary artery disease or the existing hematologic disorder was clinically suspected, though the electrocardiographic evidence for this was lacking. At autopsy, the clinical picture of refractory heart failure was related to the pres- ence of the left ventricular thrombus per se. Reference to the literature on intracardiac tumors and thrombP1” shows that tumors in the right heart are associated with weakness, edema, hepatomegaly, and ascites. Space occupying lesions in the left heart are associated with dysp- nea, orthopnea, and with emboli to the central nervous system, renal, mesenteric, and peripheral arteries. In view of the closer correspondence of this patient’s clinical manifestations to those of

patients with compromised right ventricular function, we offer the hypothesis that this patient’s thrombus either interfered with mitral valve function or so gradually but progressively interfered with left ventricular filling that the process became a physiological equivalent of mitral stenosis with the subsequent development of pulmonary hypertension and right ventricular failure. From a functional standpoint, the patho- genetic mechanism for the intractable heart fail- ure seen in this patient is closely analogous to the hemodynamic impairment observed in patients with endomyocardial fibrosis.” In this entity, found primarily among Africans, the fibrotic pro- cess with or without thrombus formation may so seriously reduce the ventricular capacity as to prove fatal.

Rheologic factors are known to play an impor- tant role in the local propagation of clotting. It is likely that this clot formed slowly by accretion on a small starting nidus. The large size of the clot, the many stages of organization seen in the thrombus, .the completely endothelialized sur- face, the absence of an acute arterial occlusive event, and the prolonged (6 month) course of this patient are all consistent with this hypothesis. The nature of the event initiating the intraven- tricular clotting was obscured by the reparative processes within the clot.

Had echolcardiographic studies been performed, the clot might have been detected.‘” Its removal might have restored the left ventricular capacity and relieved “intractable” congestive failure.

American Heart Journal 521

Ali et al.

urrence of thrombotic events is central to the course of polyeythemia Vera.‘-” cerebral, peripheral, and pulmonary infarctions are frequent and are consequences of thromboses in small and medium caliber arteries. Thrombosis in large caliber arteries is a rare event. Thrombo- sis within the chambers of the heart has not been hitherto reported.

This report documents the occurrence of mas- sive left ventricular thrombosis In a patient with polycytbemia Vera. The thrombus reduced the left ventricular capacity by about 75% and caused intractable congestive heart failure.

The help of Gertrude Martin is gratefully acknowledged in preparation of the manuscript.

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