The Significance of Asplenia in the Recognition of Inoperable Congenital Heart Disease

SPLENIC AGENESIS in association with conigeniital heart disease imnplies the preseniee of multiple anid complex nmalformations of tire heart an1d great vessels,' 3 anid conse(luently inoperable cardiae disease.3 Such was the ease in an extremely eyanotic anid dyspneic 3-monlth-old male iiifant with nio cardiac imiurmur. The electrocardiogramn showed righlt axis deviatiotn, anid suggested right atrial enlargemenit, righlit ventricular hypertrophy, antd abnormal positioninig of the ventrieles. Chest roenitgenog-ranm (fig. 1) disclosed a normal-sized, nornmally located heart and a rigrht-sided gastric air bubble. Selective angiocardiogrraphly (fi(g 2) slboxved tranispositioIi of the great vessels, al SingOle ventricle, a righ1t aortic arel, aid l)robable pulmloniie valvular and subvalIvular stenosis. Neeropsy followinig suddeni deathl reveated additioial malformrlations (figs. 3 antd 4). 'This patient demiionistrates tlhe synodrome of spleniic agenesis, partial situis3 inversus, anid mi:ultiple congenital eardliovascular aliomal ies. Approxinmately 100 patients with this enititv have been reported. All of the more commnoln mnalformriationis of the heart aitid great vessels assoeiate(l withi this syj)nromiie are illustrate(d in the patient describeld hierein. Tlei complexity of the cardiae disorde,rs cani le determinedI only bv initracardiac eatheterization anid ulSUally by angiocardiography. The diagnosis of aspleniia, on the otlher lhanld, frequenitly may, be made simnply by study of the peripheral blood sm-tear. IIowell-Jolly bodies or siderotic granules in the erythrocytes of the periplheral blood for practical purposes indicate an. ab-