Interventricular Septal Defects with

Associated Major Vascular Anomalies

Report of Three Cases*

GEORGE ROBINSON . S1 .D., PHILIP GL 'rzER, M .D ., MARVIN GILBERT, 1] .D . . DURls .1 . \V. FSCHER, M .D .,and ELLIOTT S . HURwITT. M .D .

New York, New York

~fIE current literature relating to the cor-rection of interventricular septal defects

makes frequent reference to associated cardio-vascular anomalies, hut little attention hasbeen paid to specific physiologic considerationsand technical problems related to their com-bined management .

Warden et a! .,1 in a series of 87 cases of in-terventrieular septal defects, reported 26 as-sociated defects . The majority of these, in-tracardiac in location, consisted of additionalseptal defects that could be corrected, or lesionsthat could not he corrected such as endocardialfihroelastosis. Extracardiac lesions in 11 casesincluded five left superior venac cavae, fourpatent ductus arteriosus, and two coaretations ofthe aorta . Kirklin2 reported a series of 81interventricular septal defects with 10 as-sociated lesions, of which five were intracardiac .The extracardiac lesions consisted of three leftsuperior venae cavae, one patent ductus ar-teriosus, and one right aortic arch .

In many instances, these associated lesionsare of major significance . They may enhancethe clinical disability, confuse the diagnosticpicture, and complicate the surgeon's task,either in the correction of the septal defect or inthe additive problem of their own repair whenphysiologically indicated .

This paper reports three cases of open heartrepair of interventricular septal defects in whichunusual associated anomalies were simulta-

* From the Surgical and Medical Divisions, The 11ontcliore Hospital, New' York, New York .Aided by a grant from the New York Heart Association .

746

ncously and successfully corrected . Of ourelective repairs of interventricular septal de-fect;, they are the first, third, and fourth pa-tients subjected to operation . Ail patientswere operated upon with an cxuacorporealpump oxygenator of the De\Vall Type . Elec-tive cardiac arrest was performed employingpotassium citrate according to the Melrosetechnique. All lesions were diagnosed pre-operatively except for the anomalous pul-monary venous drainage of the left upper lobedescribed in case 2, and the pateney of theductus arteriosus associated with die knowncoaretation of the aorta in case 3 .

CASE HISTORIESCAST 1 (S . H .1. 'This nine-year-old female was ad-

mitted to Nfonicfiorc Hospital on September 12, 195?, forcorrective surgery of a previously diagnosed inter-ventricular septal defect and patent ductus arteriosus,with known anomaly of the aortic arch. Congenitalheart disease was established at age 3i/e . Developmentwas within normal limits except for failure to gain in',eight . Easy fatigability was present. In February,1956 . she was successfully treated for subacute bacteria[endocardttis.

Physical examination revealed a thin small femalechild appearing younger than the stated age . Therewas a left precordial bulge of nmderate degree . Theheart was enlarged, with a harsh systolic nuirnmrheardalong the left sternal border in the 3rd and 4th inter-costal spaces . P-2 was much louder than A-2 . A classicmurmur of patent ductus arteriosus was heard in the left2nd intercostal space.

X-ray examination revealed a generally enlarged

THE AMERICAN JOURNAL. OF CARDIOLOGY

Robinson, Glotzer, Gilbert, Escher, and Hurwitt

Fi Case I is . 11 . i . X-rays of chest showing cardiac enlargement . increased pitlmonarv vascn-1 : ity . right aortic arch, vascular shadow in left upper mrdiastinum . and retroesophageal duetusarterinsus . isAiP\view; (R)Irftlateralcir,, ;

(Clright)hli :.larsite :

(Dl left ol,itc1tti view .

hear l, inert' vaseolatity of the lung fields, a right-sided aortic arch, a large retroesophageal vessel and avascular shadow to the left of the upper mediastinum(Fig . 1 ). The electrocardiogram showed enlargementof both ventricles. Cardiac catheterization findings arcdetailed in Table I .

Operctirc Proeeddn mad Findings: Operation was per-formed on September 17, 1957 thmrgh a bilateralanterior thoraeotomy incision with transeetion of thesternum . A true right-sided aortic arch was found witha retroesophageal patent duetus arteriosus enteringthe left pulmonarv artery (Fig. 2) . The left subclavian

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747

artery arose hom the superior aspect of the duetus arteri-osus and ascended along the left side of the superiormediastinum. Measurcmcnts of the pulmonary arteryand the aorta are indicated in Fig. 2, 'Che duetusarterirnus was divided at its insertion into the left puI-monarv artery . Following division of the duttus, bloodsamples drawn from the right atrium (saturation 54 percrmt) and right ventricle (saturation 71 per cent), con-firmed the presence of a large interventricular shunt .Cardiopulmonary bypass was then established witharterial perfusion through the right femoral artery,follows d by elective cardiac arrest . Right vnotriculot-

748

Ventricular Septal Defects with Associated Anomalies

TABLE I

Physiologic and Operative Data

Age at operation (years)Weight (Ib)

Cardiac Catheterization

SVCIVCRAKVPABAFA

Analysis of reservoir blood attermination of perfusion

Plasma hemoglobin (mg c )Platelets (cu mm)Fibrinogen (mg di ( )

Case 1

24150,000200

only revealed a 2 cm defect in the vcntric dar septumcompletely concealed by the septal leaflet of the tricuspidvalve . The chordal attachments of the septal leaflet tothe ventricular septum were divided to visualize thedefect. The aortic valves were immediately to the leftof the defect and the annulus of the tricuspid valve wason its right rim . The defect was closed with 8 closelyplaced 3-0 silk sutures . The divided chordae tendineacwere repaired . The postoperative course was uneventful .

CASE 2 (K . DT .) . This six-and-one-half-year-oldwhite female child was admitted to Montcfiorc Hospitalon October 31, 1957, for correction of a previously diag-nosed interventricular septal defect . Congenital heartdisease was known since the age of eight months at whichtime a murmur was heard . She was always under-developed and gained weight poorly. There were nosymptoms of cardiac decompensation. She was con-sidered to be somewhat mentally retarded .

Physical examination revealed a very apprehensive,easily excited child, grossly underdeveloped for herchronologie age. The heart was enlarged, with a diffuseapical impulse observed in the 5th . 6th, and 7th inter-costal spaces on the left side . A harsh systolic murmurwas heard best over the left 3rd intercostal space atwhich point a distinct thrill was palpable. The chestx-rays (Fig. 3) demonstrated increased vascular mark-ings in both lung fields with enlargement of the pulmo-

Case 2

Case 3

8

con Kg

mm llg

'46968

7082

70/0

82

70/082

69/54

82

69/39110-160/50-70

99

90/60

50

3040,000

130 .000220

220

n tery and right ventricle . 'f he electrocardiogramwas interpreted as showing a large right sent ride . Car-diac catheterizalion findings are detailed in Table I .

Operative Procedure and Findings : Operation was per-formed on November 4, 1957 . through a bilateral anteriorthoracotomy incision with transection of the sternum .The right ventricle was found to be greatly enlarged .The pulmonary artery was 1 inch in diameter in con-trast to the aorta which was '/, inch in diameter at itsorigin . A large anomalous left upper lobe pulmonaryvein was seen to drain into the left innominate vein .The remainder of the pulmonary venous drainage wasnormal . Cardiopulmonary bypass was established andarterial perfusion performed through the left sidaclavianartery . After elective cardiac arrest was established,right ventriculotomy revealed a 2 cot diameter defectin the outflow tract of the right •e ntricle . 'I 'la marginsof this defect were entirely muscular except fur a smallarea in its upper portion The r],feet was closed withmultiple interrupted 3-0 silk sutures. Cardiac bypasswas terminated after restoration of the heartbeat . Aside-to -side anastomosis was then made between the left atrialappendage and the anomalous left superior pulmonaryvein employing 5-0 atraumatic continuous silk sutures(Fig . 4) . '1'hc anomalous vein was then ligatecl anddivided at its insertion into the left innominate vein .

The postoperative course was aneompiicated exceptIre an a .symptnmatie complete heart block. Over the

'i HE AMLRICAN JOURNAL OF CANn10LOCY

Flow rate of perfusion (cc/min) 900Duration of cardiac arrest (min) 28Total duration of perfusion (min) 42

Robinson, ( ;lotzcr, Gilbert, Rscher, and Ilurwitl.

%a t)

_ : Cam . CarotidEsophagus

Trachea

A

Fig . 2 . Case I . (S . H.) (Aj Anterior view of aortic arch anomaly ; (B) posterior view .

subsequent six months, this patient has shown progressiveimprovement in growth and no svnrptoms ascribable tothe heart block . '1'lic postoperative pulse rate has variedbetween 55 -110 with an average of $0 per minute .

Case 3 (M. P.) This six-year-old white female wasadmitted to Montefiore Hospital on November 14, 1957 .for correction of a previously diagnosed coaretation ofthe aorta and an interventricular septal defect . A heart

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Era tusArteriUSns

Rt Sc*acso an

Fig, 3. Case 2 (K . M.) . X-rays of chest showing cardiac erdargement especially of the right vcn-triele, and increased pulmonary vaseularity . (A) PA vine ; (B) right lateral view .

imnnutr was noted at birth and a diagnosis of congenitalheart disease was established or the age of two months .A recent complaint was the miset of coldness mid pares-thcsias .of the feet as well as easy fatigue of the legs .

Physical examination revealed a fairly well-developedand well-nourished child . There was a systolic thrilland murmur in the 3rd left intercostal space . The aortic2nd sound was well heard but the pubisonic 2nd soundwas distorted by the murmur . ['he blood pressure

7:0

4C

Ventricular Septal Defects with Associated Anomalies

LtI u.n u .nrnnte

+II Arum . tcustt looe~ rt :

Lt itrtalappendage

B

B

Fig . 4. Case 2 (K . M.) . (A) Anomalous left upperlobe vein entering the tcft innutninate vein . Venousdrainage, of left lower lobe was normal . (B) Method ofcorrection : Partial occlusion clamps placed on the sideof the pulmonary vein and across the left atrial append-age in preparation for anastmnwis. (C) Anastomosiscompleted and anomalot's vein heated and divided atthe innominate vein .

Fig . 5 . (idea') Case 3 (M . P .) . X-rays of chest show-ing cardiac nilargement, especially of the left ventricle,and increased pulmonary vascularity . (A) PA view ;(B) lateral view : (C) left oblique view .

C

'IIIK AMERICAN JOURNAL OF CARDIOLOGY

was 160/70 in the arms. and the femoral pulses werenot palpable . Chest fluoroscopy and x-ray demon-strated a large left ventricle and increased pulmonaryvascular markings (rig- 5) . The electrocardiogramshowed enlargement of the left ventricle with possiblecombined ventricular strain pattern . Cardiac cath-eterization findings are detailed in Table I .

Operntits Pacedvre and binding.,: Operation was per-formed on November 26 . 1957, through a left pnstaro-lateral thoraeotoinv incision . The findings were thoseof a typical enaretation of the aorta with patent diemsarteriosus . I 5 e ductus was divided and the conretationwas excised. An end-to-end anastomosis was then per-famed between the divided ends of the aorta . U puncompletion of (his procedure, blood drawn from the rightatrium and right ventricle confirmed a significant intcr-ventricular shunt .

]'[I(- incision was then extendedtransversely across the right chest with transection of thesternum . Cardiopulmonary bypass was establishedwith arterial perfusion performed through the left sob-( lavian artery. Ventriculotmny revealed a 7 urn defectin the inflma aaet of the right ventricle, partially con-cealed by the septal leaflet of the tricuspid valve . Thiswas closed with intcrupted 3-0 silk sutures . The sub-clavian artery was repaired following removal of thecatlit ter . 'Illr pasloperative enurse was uneventful .

Disc :essION

Patent Ductus arteriosus or Persistent Left Su-perior Venn Coca : When preoperative diagnosticexaminations reveal associated cardiovascularanomalies, consideration may he given to thesequence of correction of all significant lesionsand whether surgical repair may be done atone or more operations . Often, however,they are a urprise finding at thoracotomv re-quiring rapid and versatile management . Inthe case of patent ductus arteriosus, car persist-ence of the left superior vena cava draining intodoe right or left atriuur, the associated lesionmust he controlled prior' to the establishmentof cardiopulmonai% co pass . Failure to ac-complish this result' in considerable loss ofblood from the interior of the heart during thecardiotomy. It is therefore accepted practiceto explore for these lesions in all cases . A pro-phylactic ligation of the ligamentum arteriosumis performed when found . The presence of aleft superior vena cava is obvious upon in-spection of the superior mediastinum, and canhe managed by cannulation, occlusion, orligation, depending upon the ultimate site ofdrainage, collateral communications, and thephysiologic significance of the lesion_

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->I

In the first case (S . H .), the preoperativediagnosis was complete and correct . Theductus was large and was a major contributing)actor to the 1 ulusmiary flow and pulmonaryhypertension . Occlusion of the ductus priorto its division resulted in a considerable rise fitsystemic blood pressure and a significant bradv-eardia . It is beyond the scope of this paper todescribe the multiple aortic arch anomalies .'When aortic arch anomalies are associated withmterveotricular septal defects, specific attenltonmust he paid to the possibility of an associatedcoarctattoo of the aorta. The problems rel-evant to coaretation are discussed in relationto case 3 . It should he noted that in the greatvariety of anomalies of the aortic arch, theductus arteriosus (nay he found entefing eitherthe left or right pulmonary artery, and diligentsearch must he made for the vessel before start-ing arterial perfusion . The aortic ring lesionin this patient gas -c no symptoms of tracheo-esophageal compression .

dnmnalouc Pulmonary I'enouc Drainage : Iuthe second case (K . M .), the diagnostic cardiacentheterization was performed through theright aria, and therefore no screening samplingwas clone alone the innominate vein . '1 hesuperior vena caval oxygen saturation of 74 percent as contrasted with an inferior vena cavilsaturation of 69 per cent is not a diagnosticdiscrepancy, particularly in an anesthetizedpatient . The anomalous pulmonary- venousdrainage was, therefore not diagnosed pre-operatively_

Partial anomalous pulmonary venous drain-age physiologically resembles interatrial septaldefect with which it is so frequently associated .It constitutes an additional left-to-right shunt,rccirculating oxygenated blood in the pul-uronary heed_ The anomalies of venous returnof the right lung differ from those of the leftlung . On the right side, the anomalous veinsmay enter the right atrium, superior or inferiorvena cava . On the left side, ae in the casedescribed, the commonest site of drainage isinto the left innominate vein. Other sites fordrainage are into a left superior vena eava orthe coronarysimts . Bilateral partial anomalousvenous drainage occurs rarely . A variety ofdiagnostic techniques may he employed to es-

752

Ventricular Septal Defects with Associated Anomalies

tablish the diagnosis of anomalous pulmonaryvenous drainage, including conventional x-raysof the chest, cardiac cathete_rization, tornog-raphy, angiocardiography, and selective dyedilution studies . Preoperative diagnosis ofanomalous veins is difficult and frequently thelesion is recognized only at operation . Anom-alous veins draining into the superior venacava or inferior vena cava are u .ually easier todiagnose than those entering the right atrium,

The surgical management of this lesion varieswith the size of the lesion, size and length ofthe draining vessel, and the presence or ah-sence of associated atrial septal defect . Avariety of procedures may he performed tocorrect the anomaly and specific preferencewould depend upon many factors .' , Theselesions do not interfere with intracardiac sur-gery during cardiopulmonary bypass . Theircorrection may await the repair of the inter-ventricular defect, or may be performed priorto ca.rdiotomy, depending upon the apparentsignificance of the lesion . When anastomoticprocedures are performed on the pulmonaryveins, caution must be exercised to preventedema of the lung . The artery to the involvedlobe or lung must be occluded if the vein is to betotally occluded during the performance of theanastorrtosis . This step may be omitted if thevein is only partially occluded, as in the casedescribed above . Caution must also be ex-ercised in the occlusion of a major pulmonaryartery in the presence of severe pulmonaryhypertension .

Coaretation of Aorta : In case 3, clinical andlaboratory methods of investigation establishedthe preoperative diagnosis of coarctation of the.aorta and interventricular septal defect . Thepatency of the ductus artcriosus was not de-tected by sampling in the main and rightpulmonary during catheterization . The leftpulmonary artery was not entered .

The diagnostic features of coarctation of theaorta associated with interventricular septaldefects do not differ from those of the isolatedlesion . The incidence of patent ductus artcri-osus in coarctation of the aorta is 10 per cent,but the incidence when further associated withinterventricular septal defects is not known .The hypertension of the upper half of the body

is likely to be less severe and pulmonary hyper-tension more severe when additional left-to-right shunts such as interventricular septaldefect and patent ductus arteriosus are pres-ent, since these function as channels for "runoff" of the blood proximal to the point of coarc-tation . Similarly, the left-to-right shunt throughthe interventricular septal defect or patentductus artcriosus is more voluminous whenunder the influence of a hypertensive ventric-ular or aortic pressure .

Careful consideration has been given to thepriority of repair of these lesions when found inassociation . In any case, dissection for pos-sible patent ductus arteriosus must be done withligation when present, prior to cardiupulmonarybypass or to correction of aortic coarctation .As regards the sequence of events in relation tothe coarctation and interventricular defect, ourexperience is too limited to recommend a singleoperative attack as performed above . I-Iow-ever, whether correction is done in one stage ortwo, the coarctation should be approachedfirst for the following reasons. If the ven-tricular septal defect and patent ductus artc-riosus are corrected prior to resection of thecoarctation, the proximal aortic segment be-comes increasingly- hypcrtensivc by the elimina-tion of the "run off" through the two shuntingdefects . This hypertension subjects a ventric-ular defect suture line to additional stress .Prior correction of a coarctation, conversely,decreases the volume of either an intra- or extra-cardiac shunt and may materially benefit apoor risk cardiac patient awaiting further cor-rective surgery' . With cardiac bypass andarterial perfusion through a femoral artery, thepresence of a coarctation serves to obstructretrograde flow to the head vessels . Perfusionthrough a subclavian artery presents a similarproblem in relation to ischemia of the abdom-inal viscera . Under such circumstances thedisproportionate venous return flow through thecannulated venac cavac tray- also complicatethe perfusion unless provision can he made foradequate-sized cannulae . The chance ofpostoperative bleeding in the heparinized pa-tient is decreased by the prior correction of thecoarctation, as collateral circulation in thechest wall diminishes . Subsequent rcopera-

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Robinson, Glotzer . Gilbert. Escher, and Hurwitt

tion in the same area for coaretation is renderedsnore difficult by the previous dissection of theductus required for the bypass .

It is our feeling that performance of the aorticanastoinosis prior to systemic heparinizationincident to bypass in a one-stage procedure willnot significantly increase the risk of bleedingfrom this suture tine . The incidence of bleed-ing as a complication may he decreased bymeticulous bemosta,is, proper oxygenation ofthe patient at alt times, and by low levels ofhentoh-sis in the pump-oxygenator system .The patient must also oe adequatelyheparinizedto prevent deposition of fibrin in the extra-corporeal circuit .

In the selection of cases for this series, sub-jects presenting severe physiologic and clinicalfeature were deliberately chosen for earlyoperation . Perhaps, for this reason, the in-cidence of associated anomalies was dispropor-tionately high in our initial group of cases .

SUMMARY

Three cases of ventricular septal defect arc

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I

3

reported in «hick associated anomalies weresimultaneously and successfully corrected htopen-heart surgery . These included patentductus arteriosus, anomaly of the aortic arch,anomalous left upper lobe pulmonary vein drain-ing into the left innominate vein, and coarcas-tinn of the aorta .

REFERENCES

I . WARDEN . H. E ., DFWm.t, R. A., CouvN, M. . l'Atzco .R . L ., and Lu LrHEI, C . W . : A surgical patholog-ical classification for isolated intrrcentricutar scptaldefer is and for those in Fatlot s tetra logy based onobservations madr on 120 patients during itpairunder direct vision . J . 'I hureu c Sang . 33 : 21, 1957 .

2 . KiRsLLS, .1 . W ., HARSnRARGFR, H. G . . DONALU .D . I . . . and Ent;ARns, .1 . E . : Surgical correctionul vtotriattar scptal defect : Anatomic and tech-nical considerations . J . Thoracic Sarg. 33 : 45 . 19;' .

3 . CRoss . R. E . : The Surgery of Llfaen cad Chij toad.

Saunders . Philadelphia, 1953-4. Gicuax, R . .1 . . SBowRoN, C. A., Musscit, B . (3. . and

BAtrer. C . P . : Partial anomalous venous drainage .A" . J. Am,, 94 : 688, 195? .I cLw, J . WV . . I'LLts, F . H ., and WooD, L . 11 . .

rt of anomalous pulmonary venous con-nections in association with interatrial communica-tions . .Sae„rnv 39 : 389, 1956 .