226 Proceedings ofthe Royal Society ofMedicine

Myxoma of the Left Atrium

Hugh Smellie MD MRCP(for R S Bruce Pearson DM FRCP)

Although myxoma of the left atrium is rare itcomes into a common differential diagnosis, thatof mitral stenosis. Because anaemia, fever andraised sedimentation rate are associated withmyxoma (McGregor & Cullen 1959) it may bereadily confused with subacute bacterial endo-carditis. It is possible only to suspect the presenceof a myxoma on clinical and routine laboratoryevidence, but attacks of unconsciousness, peri-pheral embolism and changing physical signswith changing posture are significant guides.Proof of the diagnosis may be obtained fromangiocardiography. In the present case, the dia-gnosis was suspected clinically, confirmed byangiocardiography and the tumour was success-fully removed with gratifying results.

History: Mrs J J, aged 46, gave a three-monthhistory of breathlessness, both on exertion and onlying down; attacks of fainting; episodes of pro-fuse perspiration, mainly at night; and loss ofweight. The breathlessness had increased to suchan extent that she became dyspnceic while walkingon the level and she had to sleep propped up withfour pillows at night. During the fainting attacks,she became unconscious for about half a minuteand lay pale and still. She always had warning ofthese attacks which had occurred both when shewas standing and lying; once she fainted on turn-ing over in bed to adjust her pillows. During theprevious year she had lost 12-7 kg in weight.There was no history of acute rheumatism. She

had attended hospital seven years previously be-cause of dyspepsia and backache. No abnormalitywas then found on clinical examination of theheart, X-ray of the chest, or barium meal.

On examination: A spare woman weighing 42 kg.Pyrexial to 990 F. A small nodule was present inthe thyroid isthmus. Blood pressure 105/70.Radial pulse regular at 90 per minute and of smallvolume. Venous pulse in the neck just visible, 'a'wave prominent. Apex beat of tapping quality,10 cm to the left in the fifth space. At the apex,the first heart sound was loud, systole silent andthere was a short low-pitched mid-diastolic mur-mur without opening snap. These signs were notaltered by change of posture. At the base, thepulmonary component of the second sound wasloud. Phonocardiogram confirmed these findings.There was doubtful finger clubbing; no otherabnormality was found.

Investigations: Hb 9.5 g/100 ml. M.C.H.C. 28%.

E.S.R. (Wintrobe) 40 mm in one hour. Serumproteins (total 7.7 g/100 ml), a-, - and y-globulins increased. Urine analysis normal.Blood culture sterile. No circulating L.E. cellsfound. Radioactive iodine uptake normal.

Initial X-rays: Chest X-ray, slight cardiac en-largement with a straight left border, main pul-monary arteries enlarged, pulmonary veins en-gorged, Kerley's lines prominent (Fig 1). Bariumswallow, slight left atrial enlargement. Tomogramof left atrium, very faint calcification in region ofmitral valve.

Fig 1 Chest radiograph showing cardiomegaly withstraight left border and Kerley's lines

ECG: Sinus rhythm, P mitrale, partial rightbundle branch block, inverted T in V 1-3.

Cardiac catheterization and dye dilution curveshowed no evidence of shunt. The right atrialpressure was slightly raised and a prominent 'a'wave demonstrated. The pressure in the rightventricle and pulmonary artery was considerablyraised. The pulmonary artery 'wedge' pressurewas very high and the pulmonary vascular resist-ance increased. These findings were interpreted asshowing considerable obstruction to the flow ofblood through the left atrium. Although the like-liest cause of this was mitral stenosis the historyof fainting, the presence of anemia, fever, raisedE.S.R. and abnormal serum proteins and theshort duration of illness suggested an atrialmyxoma as a likely alternative. The relativelysmall size of the left atrium and the absence offibrillation made a ball thrombus most unlikely.

Pre-operative course: In the light of these findings,the decision was made to explore the left atrium

4

Clinical Section

Fig 2 Angiocardiogram - oblique view showing fillingdefect (arrowed) in left atrium and left ventricle

and the patient was transferred to HammersmithHospital for that purpose. Shortly before transfer,the clinical and radiographic features of a pul-monary infarct in the right lower lobe developed,without evidence of thrombosis in the legs.When the right heart was catheterized at

Hammersmith Hospital, the pressures in the pul-monary circuit were found to be higher thanrecorded one month previously. An angiocardio-gram was achieved via the catheter in the pul-monary artery and this demonstrated the leftatrial tumour and showed it protruding throughthe mitral orifice into the left ventricle (Fig 2). Italso appeared to obstruct the flow of blood fromthe right lower lobe pulmonary vein, a point ofinterest in view of the recent infarct.

Operation: Mr W P Cleland approached the leftatrium via the right atrium and interatri-al septum,the circulation meanwhile being maintained by apump oxygenator. The tumour protruded throughthe incision as the septum was opened (Fig 3) andit was excised together with a short pedicleattached to the septum.

Operation specimen: The tumour was about 8 cmlong with a constriction about halfway along itsaxis presumably due to the mitral valve. The cutsurface was of gelatinous appearance with scat-tered areas of macroscopic hvmorrhage and onesmall area of calcification. Microscopically therewas a covering of endothelial cells, a mucinous

matrix with groups of elongated cells scatteredthroughout, and numerous capillaries.

Post-operative course: Further episodes of hmmo-ptysis occurred shortly after operation despiteprophylactic phenindione therapy. Chest X-raysshowed pulmonary infarcts in both lower lobes.The patient's condition rapidly improved and inthe six months since operation she has gained 10kg in weight, has had no more fainting attacksand is able to move about freely without dyspncea.Signs of pulmonary hypertension persist but thesigns of mitral valve obstruction have gone. Sheis afebrile, the E.S.R. and serum proteins arenormal, and the hxemoglobin is 12 g/100 ml.

Comment: In recent years left atrial myxoma hasbeen encountered occasionally at attemptedmitral valvotomy. In one such case, described byChin & Ross (1957), the heart was closed and thetumour was removed at a later date using an'open heart' technique, as there is grave risk ofsystemic embolism if the tumour is manipulatedwhile the circulation is intact. Ashman et al.(1960) described a patient with a right atrialmyxoma and collected 19 other atrial myxomas(mainly left) from the literature. Operative re-moval of the tumour had been attempted in allthese patients. The high mortality of just over

Fig 3 Left atrial myxoma as seen at operation. Theapproach is through the right atrium

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228 Proceedings ofthe Royal Society ofMedicine 6

50% was partly due to early attempts using closedheart surgery.Why fever, anemia and raised sedimentation

rate develop in this condition is unknown; thesefeatures disappeared in our patient when thetumour was removed. Suggested causes includemultiple micro-embolism or degeneration in thetumour. A search was made by gel diffusion andhemagglutination techniques for auto-antibodiesto this tumour but none was found.

Acknowledgments: I thank Dr J F Goodwin andProfessor R E Steiner for permission to show theangiocardiogram, Dr A Hollman for the photo-graph taken at operation and Dr M B Bishop fordetails of the specimen.REFERENCESAshman H, Zaroff L I & Baronfsky 1 (1960) Amer. J. Med. 28,487Chin E F & RossDN (1957) Brit. med. J. i, 1447McGregor G A & CuDlen R A (1959) Brit. med. J. ii, 991

Mucoviscidosis with Hepatosplenomegalyin a Boy of 17

T St M Norris MD MRCP DPH

Mucoviscidosis or hereditary exocrine dyscrasiais being diagnosed in increasing numbers ofpatients beyond infancy. Of 550 cases seen be-tween 1939 and 1958 at the Babies Hospital, NewYork, 106 lived over ten years and 3 are recordedas surviving to twenty years (di Sant' Agnese &Anderson 1959). It is probable also that, likeWilson's disease, the subclinical condition existsin some members of families carrying the trait.When the pancreas is only slightly affected it hasbeen suggested that respiratory disease is the pre-dominant feature (Karlish & Tarnoky 1961) butthere seems no reason why, in those who havepassed infancy, the disease may not present withclinical features related to any of the organswhich may be involved.

In 1957 I presented to this Section the case of aschoolgirl aged 14 with mucoviscidosis who hadattended the Whittington Hospital for hkematem-

esis due to portal hypertension from biliarycirrhosis (Norris 1957). Three years after I firstsaw her she died from antibiotic-resistant pul-monary infection and hepatic failure, with ob-structive jaundice. The pancreas, liver, spleen andlungs show the characteristic macroscopicchanges of mucoviscidosis (Fig IA, B, C).At a meeting of the Section of PRdiatrics Dr

S D V Weller (1960) showed a boy aged 9 withmucoviscidosis on whom a porta-caval shunt hadbeen performed to relieve portal hypertension.Dr Weller tells me that this boy now suffers fromrecurrent pulmonary infection requiring neo-mycin but he has had no further hmmatemesis.

Fig lA .....~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~

Fig 1A

Fig IB

Fig IcFig 1 Mucoviscidosis. A, pancreas with severe fibrosis and cysts up to 3 cm diameter. B, coarsefibrosis ofliver;-enlargement of spleen. c, severe bronchopneumonia and generalized bronchiectasis in right lung