September
and a vital capacity 58% of predicted. Data obtained at cardiac catheterization are summarized in Table I. Plans were made for combined heart and lung transplantation but she died of pneumonia while waiting. At necropsy, the heart weighed 360 g and the right atrium and ventricle were dilated. The left atrial endocardium was diffusely thickened and the left ventricular endocardium was focally thickened. A dense, calcific bar extended transversely for approximately 4.5 cm in the ventricular septum and protruded approximately 1 cm into the left ventricular cavity [Fig. 3). Several left ventricular trabeculae also were calcified. The valves and coronary arteries were normal. Histologic sections of myocardium disclosed only myocyte hypertrophy. Atherosclerotic plaques were present in the intima of the pulmonary trunk and in the extraparenchymal pulmonary arteries (Fig. 4). Histologic examination of both ascending aorta and pulmonary trunk (stained for elastic tissue) showed normal configuration of the elastic fibers in the pulmonary trunk (adult pattern).l Plexiform lesions were absent in all 26 sections of lung examined. The pulmonary veins were thickened and dilated, the alveolar septae were focally fibrotic and many but not all of the muscular pulmonary arteries had thickened media and in a few the intima was thickened by fibrous tissue. Many hemosiderin laden macrophages were present in alveolar spaces and the alveolar lining cells were cuboidal in shape and increased in number. The interlobular septa were thickened by fluid, fibrous tissue and dilated lymphatic channels. During life this patient was believed to have primary pulmonary hypertension, but at necropsy no plexiform lesions were present in the lungs, a finding essential for this diagnosis.2 The medial thickening of the pulmonary arteries is simply an anatomic manifestation of severe pulmonary hypertension and not an indication of its cause. Study at necropsy of histologic
EchocardiographicFindings in Right Ventricular Myxoma B.L. CHIA, MB, FRACP CHIN-HOCKLIM, MB, FRACP JOSEPHH. SHEARES, MB, FRCS MAURICE H. CHOO, MB, MRCP
A
lthough right ventricular (RV] myxomas are uncommon, these lesions must be detected during life because surgical removal of the tumor frequently results in complete cure.l
From the Department of Medicine, National University of Singapore, Lower Kent Ridge Road, Singapore 0511, and Mount Elizabeth Medical Centre, Singapore. Manuscript received August 26, 1985; revised manuscript received February 25, 1986, accepted February 27,1986.
15.1986
THE AMERICAN
JOURNAL
OF CARDIOLOGY
FIGURE 4. Close-up view of a longitudinal ing a thickened large pulmonary artery. atherosclerosis.
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section of the lung showThe aorta was free of
sections of the wall of the pulmonary trunk indicated an adult configuration of the medial elastic fibers, a finding that indicates that the pulmonary hypertension was acquired rather than having persisted from birth.l The calcific bar in the ventricular septum and left ventricular free wall, of course, is not observed in patients with primary pulmonary hypertension, but again, its cause was not revealed by anatomic study. Thus, both the cause of the severe pulmonary arterial and venous hypertension and of the cardiac ventricular calcific deposit remain unexplained.
References 1. Heath
JJ, Wood EH, DuShane
JW, Edwards
JE. The structure
of the pulmo-
nary trunk at different ages and in cases of pulmonary hypetension and pulmonary
stenosis. J Path Bact
1959:77:443-456.
2. Wagenvoort CA, Wagenvoort N. Primary pulmonary hypertension: a pathologic study of the lung vessels in 156 clinically diagnosed cases. Circulation 1970;42:1163-1184.
A 1%year-old oriental man presented with exertional dyspnea of 2 years duration. A prominent a wave was present in the jugular venous pulse. A 3/6 ejection systolic murmur was loudest in the fourth and fifth left intercostal spaces, just lateral to the sternal edge, followed by a soft diastolic murmur. Blood pressure was 130/85 mm Hg. The chest x-ray was normal and the electrocardiogram showed sinus rhythm and complete right bundle branch block. Two-dimensional, M-mode and pulsed Doppler echocardiography (echo) showed an echo-dense mass, which was attached to the anterior RV wall in the RV outflow tract. This mass was mobile, moving toward the pulmonic valve in systole and toward the RV cavity in diastole [Fig. 1). Pulsed Doppler evaluation, with the sample volume placed in the proximal pulmonary artery trunk, showed systolic turbulence. Cardiac surgery was performed immediately after echo and without cardiac catheterization and angiography. At operation, a large, mobile tumor measuring 4 x 4 cm was
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seen. This tumor was attached by a short, broad stalk to the anterior RV free wall. Histologic exhmination showed that it was consistent with a myxoma. The patient has been followed up for about 6 months and is well. Two-dimensional echo detection of RV myxoma has been rarely reportedS2 In most cases of RV myxomas which have so far been described, the diagnosis during life has been made using cardiac catheterization and angiography. This case illustrates that like intraatrial myxomas, RV myxoma can similarly be diagnosed accurately using &dimensional echo and that surgery can also be achieved successfully without invasive investigations.
References FIGURE 1. Two-dimensional echocardiogram recorded in the parasternal long-axis view. Ao = aorta; LA = left atrium; LV = left ventricle; MYX = myxoma.
1. Snyder SN, Smith DC, Lau FYK, Turner AF. Diagnostic features of right ventricular myxoma. Am Heart 1 1976;91:240-248. 2. Nanda NC, Barold SS, Gramiak R, Ong LS, Heinle RA. Echocardiographic features of right ventricular outflow tumor prolapsing into the pulmonary artery. Am r Cordial 1977;40:272-276.