March I 963 MERIKAS AND OTHERS: Reversible Psychotic Syndrome 155

unaffected when subsequently thyrotoxicosis reap-peared are all strong evidence against the etiologicsignificance of the latter.By excluding therefore other potential causes, the

possible relationship of this syndrome to thepotassium perchlorate, and particularly by amechanism of toxic reaction, becomes more likelysince allergic manifestations were not observed.

REFERENCESBrit. med. J. (I960): Editorial: Potassium Perchlorate in Thyrotoxicosis, i, 517.

(I96I): Editorial: Potassium Perchlorate and Aplastic Aniemia, i, io86.CROOKS, J., and WAYNE, E. J. (I959): Potassium Perchlorate Sensitivity, Brit. med. J., i, 1,2.

,~ (I960): Comparison of Potassium Perchlorate, Methylthiouracil and Carbimazole in Treatment of Thyro-toxicosis, Lancet, i, 401.

FAWCETT, J. B., and CLARKE, C. W. F. (I96I): Aplastic Anxemia Due to Potassium Perchlorate, Brit. med. J., i, 1537.GOODLEY, A. F., and STANBURY, J. B. (I954): Preliminary Experience in the Treatment of Hyperthyroidism with

Potassium Perchlorate, J. Clin. Endocr., 14, 70.HOBSON, Q. J. R. (I96I): Aplastic Aneemia Due to Treatment with Potassium Perchlorate, Brit. med. J3., i, 1368.JOHNSON, R. S., and MooRE, W. G. (I96I): Fatal Aplastic Anamia After Treatment of Thyrotoxicosis with Potassium

Perchlorate, Ibid., i, I369.KREVANS, J. R., ASPER, S. P., and RIENHOFF, W. F. (I962): Fatal Aplastic Aneemia Following Use of Potassium

Perchlorate in Thyrotoxicosis, J. Amer. med. Ass., x8I, I62.LEE, R. E., VERNIER, R. V., and ULSTROM, R. A. (I96I): The Nephrotic Syndrome as a Complication of Perchlorate

Treatment of Thyrotoxicosis, New Engl. J3. Med., 264, 122I.MORGANS, M. E., and TROTTER, W. R. (1954): Treatment of Thyrotoxicosis with Potassium Perchlorate, Lancet, i, 749.

(1960): Potassium Perchlorate in Thyrotoxicosis, Brit. med. J., ii, io86.RIENHOFF, W. F., Jr. (I96I): Total Aplastic Anmemia and other Complications Following So-called 'Antithyroid

Drugs' in Treatment of Thyrotoxicosis, Sth med. Jt. (Bgham, Ala.), 54, 1034.

EISENMENGER SYNDROME WITH RADIOLOGICALCALCIFICATION OF MAIN PULMONARY ARTERIES

K. RAJASURIYA, M.D.(Cey.), M.R.C.P.(Lond.)Professor of Medicine, University of Ceylon

M. A. MACAN MARKAR, M.D.(Lond.), M.R.C.P.(Lond.)Lecturer in Medicine, University of Ceylon

K. M. C. DE SILVA, D.M.R.D., D.M.R.T. V. T. RATNAIKE, M.D.(Cey.)Radiologist, General Hospital, Colombo Registrar, Department of Medicine, University of Ceylon

From the Department of Medicine, Faculty of Medicine, University of Ceylon, Kynsey Road, Colombo, Ceylon,and the General Hospital, Colombo, Ceylon

CALCIFICATION of the main pulmonary artery or ofits left and right branches gross enough to bevisualized radiologically and diagnosed during lifehas not been described, as far as we could find fromthe literature. Atherosclerosis of the pulmonaryarteries is of course common in long-standingpulmonary hypertension, and these atheromatouspatches would naturally get calcified, although notlarge enough to be seen on X-ray examination.Thrombosis-in-situ of the large pulmonary arterieshas been described by Brenner (I93 i), Dimond andJones (1954), Magidson and Jacobson (1955), Ringand Bakke (I955) and by others. Magidson andJacobson (i9g5), describing a series of these caseswith autopsy findings did not mention any grosscalcification of the pulmonary arteries. Closedchest injury has been mentioned as a cause ofpulmonary artery thrombosis (Dimond and Jones,1954; Don Michael, I962, etc.)

Aortic valve cusp damage following non-pene-trating injury of the chest and causing aorticincompetence has been described (Wood, I956).

The case we describe below is of a young femalewhohad pulmonary incompetence, with the clinicalpicture of Eisenmenger's syndrome and radiologicalcalcification of the main pulmonary arteries, follow-ing a history of trauma to the chest.

Case ReportA 27-year-old unmarried female patient was admitted

to the General Hospital, Colombo, in October I962with a history of dyspncea on ordinary exertion, such aswalking on the flat, since the age of ten years.At the age of eight years the patient had fallen while

playing and had struck the front of her chest on theedge of a stone step, with another child's weight fallingon her back. For one month following this she had apain in the precordial region. Two years after thechest injury she woke up one morning with a sense ofdyspnea and pain in the chest, and these symptomsincreased as she was made to walk to school. She hada syncopal attack on the way to school and was cyanosedduring the attack.There had been no syncopal attacks since then, but

anginal pain on walking had been experienced off andDl

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156 POSTGRADUATE MEDICAL JOURNAL March I963

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FIG. I.-Aneurysmal dilatation of pulmonary conus andright and left pulmonary arteries, showing hori-zontal streaks of calcification in right hilum andupper part of pulmonary conus.

on. Dyspncea on ordinary effort was persistent fromthe age of ten years, and she was noticed to turn blueafter exertion. She had also suffered from frequentattacks of ' bronchitis ' about five to six times eachyear, since the time when she started getting theeffort dyspncea.When she was warded in 1957, ECG examination

had shown right ventricular preponderance, right axisdeviation with a vertical heart, and pulmonary P-waves.Screening then had shown prominent pulmonary arteryand main branches with exaggerated pulsation, andsome right ventricular enlargement. She was thendiagnosed as an A.S.D. with pulmonary hypertensionand ieversed shunt, after cardiac catheterization. InI959 the patient had a bout of cough and hemoptysislasting for two days.Two months before the present admission to hospital,

her effort intolerance had increased, dyspnea beingbrought on by less than ordinary exertion. Duringthe same time she had frequent attacks of palpitationand pain over the precordium, even at rest. She alsocomplained of dysphagia for solids.The patient was of slender build and poorly nour-

ished. The skin was xerodermic, especially in the legs.There were no deformities of the chest wall. The palatehad a high-arched appearance. There was centralcyanosis, and clubbing of the finger nails. There wasno dependent cedema.The jugular venous pressure was not raised; but a

hepato-jugular reflux was present. Pulse rate was88/min. regular, and of moderate volume. B.P.I20/8o mm. Hg. The apex beat was in the fifth leftintercostal space, 4i in. from the mid-line: it was afairly vigorous 'right ventricular ' tap. A left para-

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FIG. 2.-Left anterior oblique (I5 degrees) view ofchest showing massive dilatation of left pulmonaryartery, with calcification. Screening confirmed theabrupt constriction of the pulmonary arterydistally.

sternal thrust was present, and pulmonary artery pul-sations were felt in the second and third left intercostalspaces. A diastolic thrill was palpable, maximally inthe third left intercostal space. There was percussiondullness in the second left intercostal space. The firstheart sound was normal. There was a pulmonaryejection click and a short soft systolic murmur, bestheard in the third left intercostal space. The secondsound was normal in the aortic area, and very soft andsingle in the pulmonary area. There was a long,moderately loud, rough early diastolic murmur con-tinuing into mid-diastole maximal in the third leftintercostal space, but heard widely over the precordium.The lungs were clinically normal. The liver was

just palpable, the spleen was not palpable.Investigations. Blood: Hb, 13.2 g./ioo ml.; RBC,

6 million/cu. mm.; PCV, 56%. The blood picturewas normal. WBC, 11,200/CU. mm; polymorphs, 56%;lymphocytes, 36%; eosinophils, 8%. Serum calcium,IO mg./ioo ml.; alkaline phosphatase, 7 K.A. units.Blood urea, 40 mg./xoo ml.; blood cholesterol, 242mg./Ioo ml. Blood for Kahn and V.D.R.L. tests,negative.ECG: There was marked right axis deviation, vertical

heart, and clock-wise rotation. P-wave was i mm. inheight in Lead 2 and in Vi, tall R waves with invertedT waves in Lead 3, Lead 2, a VF, and in Vi, denotingright ventricular strain. X- ray of chest showed grossenlargement of pulmonary arteries (right and left) andof pulmonary conus (Fig. i) and calcification in left andright pulmonary arteries (Figs. i and 2).

Screening showed vigorous hilar pulsations and

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March I963 RAJASURIYA: Eisenmenger Syndrome

.7.

FIG. 3.-Lateral view taken at screening with barium-swallow, to show ring-like appearance of calcifica-tion in cross-sectioned view of left pulmonaryartery which is seen to displace the cesophagusbackwards.

sudden narrowing in calibre of vessels (after the mainright and left pulmonary arteries). There was displace-ment of cesophagus by enlargement of left pulmonaryartery. Right ventricle was enlarged. Aorta was noteasily identified, and appeared to be small. The radio-logist gave a probable diagnosis of A.S.D., andaneurysmal dilatation of pulmonary arteries with cal-cification.

DiscussionThe interesting features in this case were the

clear history of crush injury to the chest in child-hood, followed by chest pain, dyspncea and thelater development of the clinical picture of theEisenmenger syndrome and radiological evidenceof gross calcification of the main pulmonaryarteries (Figs. I, 2 and 3).Wood (1958) defines the Eisenmenger syndrome

as pulmonary hypertension with pulmonary vascular

resistance around systemic levels, and with areversed shunt at any level centrally. The caseswith the shunt at atrial level tend to develop reversalof shunt in adult life in 92% of cases, whereas thosewith a reversed shunt through a V.S.D. or a patentductus had the syndrome manifesting in infancy inabout 8o% of cases (Wood, 1958). This late develop-ment of the syndrome in cases with the shuntat atrial level is considered to be due to pulmonaryhypertension in atrial septal defects being a lateracquired characteristic. The two most likelycauses for sudden development of dyspncea andcyanosis in later life with sudden rise of pulmonaryvascular resistance are chronic bronchitis withemphysema, and multiple pulmonary embolism(Wood, I956). Our patient had dyspnea from tenyears of age with cyanosis noticed on exertion,and was first seen and diagnosed as a case of theEisenmenger syndrome with the shunt at atriallevel, when she was 22 years old.The factor that brought about acquired pul-

monary hypertension in our case may well berepeated thrombo-embolism from thrombus formedin the main pulmonary arteries (right and left)following trauma. Mural thrombus followingindirect trauma on a large pulmonary artery wouldget organized and later calcification could occur, asis shown in the ring-like appearance of the calci-fication in Fig. 3.

In cases of the Eisenmenger syndrome withA.S.D. a wide and fixed split of the second soundin the pulmonary area was found in 86% of cases inWood's series and in-no case was the second soundsingle in the pulmonary area (Wood, I958). Thesingle soft second sound in the pulmonary area inour case may either be due to the gross pulmonaryincompetence, shown by the diastolic thrill and thelong loud pulmonary diastolic murmur, or may beexplained by the shunt occurring through a patentforamen ovale rather than an A.S.D. (This mightalso explain the absence of right bundle branchblock in the E.C.G.) However, massive dilatationof pulmonary arteries is more a feature of A.S.D.than of pulmonary hypertension as such (Wood,1958).

SummaryA case of Eisenmenger's syndrome with a history

of crush injury to the chest wall in childhoodfollowed by probable thrombosis in the mainpulmonary arteries, giving rise to a thrombo-embolic type of pulmonary hypertension, andlater calcification of the left and right main pulmo-nary arteries, is described and discussed.

REFRENCESBRENNER, 0. (I93 I): Sclerosis of the Pulmonary Artery with Thrombosis, Lancet, ii, 91.DIMOND, E. G., and JONES, T. R. (1954): Pulmonary Artery Thrombosis Simulating Pulmonic Valve Stenosis with

Patent Foramen Ovale, Amer. Heart J., 47, 105.DON MICHAEL, T. A. (I962): Thrombosis-in-situ of the Main Pulmonary Arteries, Postgrad. med. J., 38, 301.MAGIDSON, O., and JACOBSON, G. (1955): Thrombosis of Main Pulmonary Arteries, Brit. Heart Y., 17, 207.RING, A., and BAKKE, J. R. (1955): Chronic Massive Pulmonary Artery Thrombosis, Ann. intern. Med., 43, 78I.WOOD, P. (1956): ' Diseases of the Heart and Circulation', p. 395. London: Eyre and Spottiswoode.

(1958): The Eisenmenger Syndrome, or Pulmonary Hypertension, with Reversed Central Shunt, Brit. med. Y.,ii, 701, 755.

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