Translocation of atrial septum - Thorax · 55 years was with haemoptysis, pleuritic chest pain, inability to speak, and mild right hemiparesis. She was conscious but had a motor aphasia

Thorax (1970), 25, 499.

Translocation of the atrial septumC. R. C. WYNDHAM'

Pathology Department, University of Adelaide, South Australia

Translocation of the atrial septum to the left of the mitral valve is a congenital anomaly: theauthor has not found a similar case previously reported in the English literature. The curiousanomaly described here was associated with deformed mitral and tricuspid valves, a postero-inferior atrial septal defect, a persistent left superior vena cava, a hypoplastic aorta, and a shortinnominate artery. The patient lived to a remarkable age.

CASE REPORT

No details are available of the antenatal history ofthe patient, an unmarried white Australian woman,who was born a 'blue baby'. 'Heart disease' or 'coro-naries' were responsible for the deaths of her motherat the age of 73 years, her father at 67 years, and sixof his siblings between the ages of 40 and 50 years.Since childhood she had had chronic bronchitis andwas prone to infections. She worked hard as a dress-maker's machinist and had no cardiac symptoms untilthe age of 47 years, when she noticed palpitations,nocturnal breathlessness, syncopal attacks, dizziness,and coldness of the extremities of gradual onset.Within two years there were clinical and radiologicalsigns of an enlarging heart (Fig. 1), and there was aprecordial systolic murmur transmitted to the leftaxilla. At the age of 49 years she developed anginaof effort and an electrocardiogram showed S-T seg-ment depression in leads V2 to V4 with aggravationby exercise. During the next seven years she gradu-ally developed mild biventricular failure, but at theage of 53 years she claimed to have been 'climbingmountains' without undue distress while on holiday.Ultimately she had to retire because of moderateexertional dyspnoea.

Her terminal admission to hospital at the age of55 years was with haemoptysis, pleuritic chest pain,inability to speak, and mild right hemiparesis. Shewas conscious but had a motor aphasia and a right-sided pyramidal lesion involving the face, arm, andleg. There was central and peripheral cyanosis andfinger clubbing. The pulse rate was 78 per minute,and there were multiple extrasystoles. The bloodpressure was 120/60 mm. Hg. There was mild eleva-tion of the jugular venous pressure, a laterally dis-placed thrusting apex beat, and a right ventricularimpulse in the left parasternal area. She had a pre-systolic triple rhythm, a systolic murmur at the left

tPresent address: Repatriation General Hospital, Daws Road,Daw Park, South Australia, 5041

sternal edge, and signs of consolidation at both lungbases. Initial investigations disclosed a leukoerythro-blastic blood picture and a reticulocytosis of 5 O%without anaemia, and there was considerable eleva-tion of the serum alkaline phosphatase and glutamic-oxalacetic transaminase.An electrocardiogram showed the mean axis in the

frontal plane to be -136° and there was clockwiserotation in the horizontal plane. It satisfied Milnor'sand Roman's criteria for right ventricular hypertrophy(Fig. 2).

Despite broad-spectrum antibiotic therapy, diuresis,and digitalization, she died after cardiac arrest duringthe passage of a naso-gastric tube on the third day.

NECROPSY

The body weighed 40 kg. and the heart 440 g.From the external aspect, there was a persistentleft superior vena cava draining the left internaljugular and subclavian veins and showing novestige of an innominate vein. The innominateartery was greatly foreshortened to about 0 5 cm.in length and the aorta was uniformly hypoplastic,measuring 13 cm. in diameter. The right atriumreceived all three caval veins and the coronarysinus, and was itself greatly dilated and hyper-trophied. The interatrial septum was placed veryobliquely, being attached superiorly in the normalposition, but inferiorly to the atrioventricular ringat the left of the mitral valve, so that the rightatrium led directly into both atrioventricularcanals (Fig. 3). The left atrium was also hyper-trophied and a little dilated and received all fourpulmonary veins. It communicated with the rightatrium via an oval atrial septal defect, 1 cm. indiameter, 0 5 cm. above the mitral valve ring,posteriorly. Otherwise the atrial septum appearednormal, containing a rather anteriorly placed fossaovalis which exhibited flap-valve probe patency

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C. R. C. Wyndham

FIG. 1. Postero-anterior chest radiograph.

DEFECT

FIG. 2. Electrocardiogram made two days before death. FIG. 3. Schematic representation of the cardiovascularfindings at necropsy.

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Translocation of the atrial septum

FIG. 4. Postero-anterior view of right atrium opened along the lineXYZ. A. Right aspect of the atrial septum, B. Lip of the right superiorvena caval orifice; C. Right atrial appendage; D. Probe-patent foramenovale; E. Postero-inferior atrial septal defect; F. Entry into the rightatrium of the persistent superior vena cava; G. Orifice of the coronarysinus; H. Fibrous trigone-cleft mitral valve to the left and deformedtricuspid valve to the right; J. Chiari net and inferior vena caval orifice.

(Fig. 4). There was a small Chiari net adjacent tothe inferior vena caval orifice. Both atrioventri-cular valves showed deficiencies of their septalcusps, that of the mitral (anterior cusp) beingrepresented by a thickened arcuate fold concavedownwards, and that of the tricuspid by a smallfibrous tag, 0*5 cm. in diameter, attached to thefibrous trigone. The right ventricle was hyper-trophied to 0 9 cm. in thickness (Fig. 5); the leftventricular cavity was small and the wall measured15 cm. in thickness. There were no demonstrableinterventricular communications, and the ductusarteriosus was closed. The pulmonary and aorticvalves and the main trunks of the coronary arteries

2N

were normal. The pulmonary trunk was of normaldimensions, but recent and old antemortemthrombi filled medium-sized arteries in the leftupper lobe and large and medium-sized arteriesto the lower lobes of both lungs which containedmultiple old and recent infarcts. There wasthrombosis of the para-vaginal plexus and smalladherent thrombi in the right atrial appendage,no doubt accounting for recurrent pulmonaryembolism.The liver and spleen were greatly enlarged and

almost totally infarcted. Thrombo-emboli wereimpacted in the main hepatic and splenic arteriesand in a branch of the superior mesenteric artery,

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C. R. C. Wyndham

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FIG. 5. Ventricular aspect of the tricuspid valve, showingthe deformities, seen through a window cut in the hyper-trophied anterior wall of the right ventricle.

no doubt having travelled paradoxically throughthe right atrium and left ventricle.

Other evidence for paradoxical embolism con-

sisted of: (1) a focal embolic glomerulonephritiswith platelet thrombi in many isolated glomerularloops, with organizing thrombus in an interlobarrenal artery; (2) platelet thrombi in small vesselsin the left corona radiata with histological corticalinfarction ; (3) small, old, gross softenings in the

right lobe of the cerebellum and in the left sub-

thalamic region; and (4) multiple micro-infarcts,mainly perivascular and of varying ages, in the

myocardium of all chambers, associated with

marked endocardial fibro-elastosis and small mural

thrombi. Arteries in the lungs showed organizingand recanalizing thrombi, moderate thickening,fibroelastosis, and patchy atherosclerosis of elasticarteries, prominent bronchial arteries, but little

change in the small muscular arteries andarterioles.

DISCUSSION

The lack of proper cardiovascular investigation inthis case unfortunately prevents any conclusionsas to intracardiac pressure-flow dynamics, but on asuperficial consideration of the complex anomalyinvolved it seems remarkable that the patient livedas long and as symptom-free as she did, and thathistological evidence of pulmonary hypertensionwas so slight, and consistent with recurrentthrombo-embolism per se.The genesis of the cardiac anomaly remains

uncertain but presumably represents some variantof an endocardial cushion defect, with a low atrialseptal defect, cleft anterior mitral valve leaflet, anddeformed septal tricuspid valve leaflet, but with-out ventricular septal defect, whose associationwith deformed septal tricuspid valve leaflet, caus-ing left ventricular-right atrial shunt, is well recog-nized. In the present case any shunt between thesetwo chambers would have been due to the mitraldeformity and the displacement of the atrialseptum. Persistent left superior vena cava has beenreported to enter the left atrium in association witha postero-inferior atrial septal defect and absenceof the coronary sinus, a complex attributed tofailure of development of the left atriovenous fold(Raghib, Ruttenberg. Anderson, Amplatz, Adams,and Edwards, 1965). Although the site of the atrialseptal defect in the present case corresponds totheir description, the left caval vein entered theright atrium and a coronary sinus was present.However, the longevity of this patient may per-haps be attributed to the relatively benign haemo-dynamic situation found in such patients, namelya left-to-right shunt at atrial level and mild arterialoxygen desaturation without pulmonary hyper-tension. In the absence of studies during life thesethoughts are admittedly speculative. Finally, theage of the patient, the presence of a well-formedforamen ovale in the atrial septum, and theabsence of Mongolism dispose of the remote pos-sibility that the anomaly was cor triloculare bi-ventriculare with a separate chamber draining thepulmonary veins.

Whatever the haemodynamic situation presentfor the greater part of the patient's life, cardiacdecompensation was undoubtedly due to a gradualrise in right ventricular pressure secondary torecurrent pulmonary embolism, and death was dueto the combined effect of the latter and hepaticinfarction.

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My thanks are due to Dr. T. D. Finey and to Dr.H. R. Gilmore for permission to publish the clinicalrecord, and to Professor J. S. Robertson and Dr.P. S. Hetzel for their helpful advice and criticism.Miss H. Fuller and Mr. D. N. Caville prepared thedrawings and photographs.

REFERENCES

Raghib, G., Ruttenberg, H. D., Anderson, R. C., Amplatz, K.,Adams, P., and Edwards, J. E. (1965). Termination of leftsuperior vena cava in left atrium, atrial septal defect and absenceof coronary sinus. A developmental complex. Circulation,31, 906.

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Translocation of atrial septum - Thorax · 55 years was with haemoptysis, pleuritic chest pain, inability to speak, and mild right hemiparesis. She was conscious but had a motor aphasia