Double Outlet Right Ventricle With Pulmonary Stenosis · Double Outlet Right Ventricle with Pulmonary Stenosis Type 2a. These patients have a supracristal ventricular septal defect

Brit. HeartJ., 1967, 29, 64.

Double Outlet Right Ventricle With PulmonaryStenosis

M. K. A. DAYEM*, L. PREGERt, J. F. GOODWIN, AND R. E. STEINER

From the Departments of Radiodiagnosis and Medicine (Clinical Cardiology), Postgraduate Medical Schooland Hammersmith Hospital, London W.12

This paper reports five patients with the con-genital cardiac anomaly of double outlet rightventricle and pulmonary stenosis, reviews the pre-viously reported cases, and discusses the diag-nostic features of the abnormality, especially withregard to the differential diagnosis from the tetra-logy of Fallot.The term double outlet right ventricle denotes

that both the aorta and pulmonary artery have theirorigin wholly from the right ventricle. There maybe no obstruction to the outflow tract of the rightventricle or there may be pulmonary infindibularor valvar stenosis, as in the five patients to be de-scribed. A ventricular septal defect provides theonly route for blood to leave the left ventricle; thereis therefore a left-to-right shunt at ventricularlevel. Double outlet right ventricle with pul-monary stenosis closely simulates the tetralogy ofFallot but the treatment differs. In the tetralogyof Fallot, radical surgical correction entails closureof the ventricular septal defect, in addition to re-section of the pulmonary stenosis. The formerwould be lethal in double outlet right ventricle,where the ventricular septal defect is the only routeby which the left ventricle can empty. Theappropriate surgical treatment for the condition hasbeen discussed by McGoon (1961) and Kirklin,Harp, and McGoon (1964). They described anoperation in which a hammock-shaped prosthesis ofivalon, teflon, or pericardium is fashioned to seal offthe ventricular septal defect from the right ventriclebut at the same time to act as a conduit for blood topass from the left ventricle via the partly closed

Received March 8, 1966.* Holder of a Scholarship from the Government of the

United Arab Republic.t Holder of a Fellowship of the James Picker Foundation,

in radiological research. Clinical Instructor in Radiology,University of California Hospitals, San Francisco.

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ventricular septal defect to the aorta. If the ven-tricular septal defect is very small, it may needenlargement. Pulmonary valvotomy and/or in-fundibular resection is performed to relieve thepulmonary stenosis.

All cases previously reported with clinical detailsare summarized in Tables I and Ifl. Relatively fewpatients (about 34 in all) with double outlet rightventricle complicated by pulmonary stenosis havebeen previously recorded. Witham (1957) was thefirst to give this condition the name of doubleoutlet right ventricle; he showed that cyanoticpatients with this disorder could be divided intotwo subgroups depending on the site of obstructionto blood flow: those with pulmonary stenosis re-sembled the tetralogy of Fallot, while those with ahigh pulmonary vascular resistance and a right-to-left shunt simulated the Eisenmenger complex.Morgan et al. (1962) reported double outlet rightventricle with and without pulmonary stenosis, andused a similar classification as follows: Type 1.Acyanotic. Type 2. Cyanotic: (2a) Fallot type(with pulmonary stenosis); (2b) Eisenmenger type(without pulmonary stenosis but with increasedpulmonary arteriolar resistance).

Neufeld et al. (1961b) and Carey and Edwards(1965) in an analysis of their patients with doubleoutlet right ventricle without pulmonary stenosismade a classification according to the site of theventricular septal defect.

Type 1. There is an infracristal ventricularseptal defect close to the aortic root and remotefrom the origin of the pulmonary artery. Most ofthe right ventricular blood passes into the pul-monary artery, and most of the left ventricular out-flow passes directly to the aorta. These patientsare thus acyanotic unless their condition is compli-cated by pulmonary stenosis or by severe pulmonaryhypertension.

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Double Outlet Right Ventricle with Pulmonary Stenosis

Type 2a. These patients have a supracristalventricular septal defect which lies near to the pul-monary artery and is relatively remote from theaortic orifice. Flow from the right ventricle ismainly into the aorta, and these patients are cyan-otic from birth.

Type 2b. Here there is a large supracristal ventri-cular septal defect related to both the aortic and pul-monary artery orifices. Flow from the rightventricle is into both great vessels. There was one

patient only in this group who was cyanosed.Neufeld, DuShane, and Edwards (1961a), andCarey and Edwards (1965) have not reported ex-

tensively on patients with double outlet rightventricle with pulmonary stenosis, but in an analysisof six such patients (Neufeld et al., 1961a), all hadan infracristal ventricular septal defect togetherwith infundibular stenosis created by the verticallimb of the crista supraventricularis. All patientswere cyanosed from birth, or soon after.

Carey and Edwards (1965) suggest that a mainfeature of double outlet right ventricle, with or

without pulmonary stenosis, is the separation of theaortic and mitral valve rings by a ridge of musculartissue. In normal patients, or patients with thetetralogy of Fallot and those with simple ventricularseptal defect, the two valve rings are in continuitywith the cardiac fibrous skeleton. These same

authors also state that in double outlet right ven-

tricle with pulmonary stenosis the parietal band ofthe crista supraventricularis is enlarged and very

prominent when seen in angiocardiograms in theantero-posterior projection. They also make sev-

eral observations on the position of the semilunarvalves, the appearance of the crista, and the inclin-ation of the ascending aorta in the lateral projectionon angiocardiography. These further points are

discussed later in the present paper.

PATIENTS AND METHODSFive patients having double outlet right ventricle with

pulmonary stenosis were studied (Tables I, II, and III).In each case the diagnosis was suspected on clinical,cardiographic, and hemodynamic grounds and con-firmed by angiocardiography. In one patient cardi-otomy was performed, the patient died subsequently andthe diagnosis was proved at necropsy. In addition, allthe published cases of double outlet right ventricle withpulmonary stenosis in which clinical data were reportedare analysed and compared with our series. Twopatients with double right ventricle with pulmonarystenosis who have been previously diagnosed at thishospital and reported by Morgan et al. (1962) are in-cluded.

In order to detect the characteristic angiocardio-graphic signs of double outlet right ventricle with pul-monary stenosis, we reviewed all the recent angio-cardiographic examinations on patients with the clinicaldiagnosis of tetralogy of Fallot in this hospital. Thisseries comprised 120 patients in whom angiocardio-graphy was performed between 1962 and 1965. Thissurvey was performed because patients with doubleoutlet right ventricle with pulmonary stenosis may easilybe confused with patients with the tetralogy of Fallot

TABLE ICLINICAL FINDINGS IN 20 PATIENTS WITH DOUBLE OUTLET RIGHT VENTRICLE WITH PULMONARY STENOSIS

Case Author Age (yr.) Age at onset Dyspnea| Ventricular Systolic Second sound Methods ofNo. and sex of cyanosis hypertrophy thrill diagnosis

1) 18 F 1 year + RV+ NR Single Angiography2 1967 31 M Birth + RV+ + + Fixed split Angiography3 This series M 6 months + RV+ + + - Single Angiography

Thsseis 16 F Birth + RV + + - Single Angiography5) 25 M Birth + NR - Single Angiography,

operation andnecropsy

6 7/12 M 2 months - NR - Single Necropsy7l 3 F 6 months _ NR - Single Necropsy8 13 M 2-5 months + NR + Single Necropsy9 Neufeld et al. 8 M 8 months + NR + Accentuated Necropsy10 (1961a) 3 5 M Birth + NR + Diminished Necropsy11 3 F Birth NR NR NR Single, diminished Necropsy12 Braun et al. 19 M Birth + RV + + Split Necropsy

(1952)13 Levy et al. 7 F 4 years I + NR + Diminished Operation

(1962)14 Lauer et al. 13 M 2-5 years + NR + Single Operation

(1960) and necropsy15 Redo et al. 7 F _ NR NR + Accentuated Operation

(1963)16 Coelho et al. 5 F 3 years NR NR + Accentuated Angiography

(1963)17 22 F Early childhood NR NR _ Single Necropsy18 Mehrizi (1965) 2 M 4 days + _ _ Normal Necropsy19 J 14 M 6 weeks + _ NR Normal Necropsy20 Witham (1957) 3/12 F Birth NR NR Single, soft Necropsy

-, Absent; NR, not recorded; +, present.5

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Dayem, Preger, Goodwin, and Steiner

TABLE II

SUMMARY OF ANGIOCARDIOGRAPHIC FINDINGS IN PATIENTS WITH DOUBLE OUTLET RIGHT VENTRICLE WITHPULMONARY STENOSIS (PRESENT SERIES)

Case Age (yr.) Relation of aorta Parietal band Level of Pulmonary Relative Ratio of First great ProofNo. and sex and pulmonary enlargement aortic and stenosis density of width of vessel to

artery on lateral on antero- pulmonary great pulmonary opacifyview posterior artery valve vessels artery and

angiocardiogram angiocardio- rings aorta ongram antero-

posteriorview

1 18 F Aotra slightly + Same level Infundibular Equal 0 9 Pulmonary Angiocardio-anterior to and valvar artery graphypulmonaryartery

2 31 M Aorta slightly + Same level Infundibular Pulmonary 0 9 Pulmonary Angiocardio-anterior to and valvar artery artery graphypulmonary denserartery than aorta

3 9 M Aorta slightly + Pulmonary Infundibular Equal 0*7 Simul- Angiocardio-posterior to artery and valvar taneous graphypulmonary valveartery 1 cm.

higherthanaorticvalve ring

4 16 F Dextrocardia; + Aortic valve Infundibular Initially 0-8 Simul- Angiocardio-aorta slightly ring 3 cm. equal, on taneous graphyanterior to lower recircula-pulmonary than tion aortaartery pulmon- denser

ary artery than pul-valve ring monary

artery5 25 M Aorta and pul- + Same level Infundibular Pulmonary Could not Could not Necropsy

monary artery artery be beoverlap assessed assessed

with marked aortic override whom they so closely re-

semble.The initial procedure was to select the angiocardio-

grams showing pulmonary stenosis, a ventricular septaldefect, and marked aortic override. This group in-cluded patients with double outlet right ventricle andpulmonary stenosis, and also those patients with thetetralogy of Fallot who most resembled double outlet

right ventricle with pulmonary stenosis. An importantsign required for acceptance of patients as having doubleoutlet right ventricle with pulmonary stenosis was thatthe ascending aorta as seen on the lateral angiocardio-gram had to be directed inferiorly and anteriorly towardsthe right ventricle, and not posteriorly and inferiorlytowards the left ventricle as in normal patients and mostof those with the tetralogy of Fallot. Furthermore, the

TABLE IIIHNMODYNAMIC FINDINGS IN 12 PATIENTS WITH DOUBLE OUTLET RIGHT VENTRICLE WITH PULMONARY STENOSIS

Case Authors Right atrium Right ventricle Pulmonary artery Left atrium Left ventricle Systemic arteryNo

Pressure 02 Pressure 02°

Pressure 02 Pressure 02 Pressure 02 Pressure 02(mm. Hg) Satura- (mm. Hg) Satura- (mm. Hg) Satura- (mm. Hg) Satura- (mm. Hg) Satura- (mm. Hg) Satura-

tion % tion % tion % tion %0 tion % tion %

1 ) 5 74 110/5 81-88 130/65 932 I1967 3 67 1130/15 72 I92113 1115/65 8343 Ti7series 3 68 100/0 74-77 9/2 70-72 9/0 98 120/70 834 ~~~~~77 120/0 86 25* 100/10* 1210 83

5 8 140/6 67 112/62 74

Coelho et al. 80/0 57 - - 10/4 92(1963)

Redo et al. -3 97/3 57/34 - 97/61(1963)

Braun et al. 12-13-5 33 - -(1952)

Levy et al. 4/0 68 100/0 77 20/10 85 100/6 90 96/60 91(1962)

Lauer et al. 0-4 63 62/0 73 810 74 62/54 84(1960)

Neufeld et al. 8/5 56 115/7 55 15/7 56 125/71 61(1961a)

Neufeld et al. 8/3 57 116/4 65-67 - - 11/7 79-85 - - 121/63 72(1961a)

* At operation.

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FIG. -Angiocardiogram, right ventricular injection. Antero-posterior and lateral views of a patient (Case 3)with double outlet right ventricle with pulmonary stenosis (infundibular and valvar) *-- (higher arrow)indicates abnormally prominent parietal band of crista supraventricularis. Po indicates high aortic valve

position. Catheter tip lies in the ventricular septal defect to opacify left ventricle (lower arrow *-- indicates

tip of catheter).

non-coronary cusp of the aortic valve had to be in frontof the plane of the ventricular septum. The relationof the walls of the pulmonary artery and aorta, and inaddition the ratio of the width of the pulmonary arteryto the width of the aorta, was measured in the lateralangiocardiogram. The aortic width was measuredabove the level of the sinuses of Valsalva, and the pul-monary artery width was measured beyond an area ofpost-stenotic dilatation where its calibre had becomeuniform. Similar measurements were made in 12patients with a typical appearance of the tetralogy ofFallot, to provide a basis for comparison.Angiocardiograms so selected were then subjected to

an examination of the crista supraventricularis and theposition of the semilunar valves. The patients weredivided into two groups; those in whom the cristasupraventricularis could be more easily identified in thelateral projection and those in whom it could only beidentified in the antero-posterior projection. Theformer group were considered as typical examples of thetetralogy of Fallot, and surgical proof of this diag-nosis was available in the large majority. The lattergroup, following the work of Carey and Edwards(1965), were considered as potential cases of doubleoutlet right ventricle with pulmonary stenosis. Thecrista was identified in the antero-posterior projectionas a non-opacified tongue-like area lying between theaorta and the pulmonary artery in a medial relation with

the infundibular portion of the right ventricular out-flow tract (Fig. 1, 2, 7, 10, and 11). An assessment ofthe position of the semilunar valves relative to each otheron the antero-posterior and lateral projections was made(Fig. 2). The level of the valve ring was chosen ratherthan the valve cusps, since varying degrees of domingof the pulmonary cusps in those patients with valvularstenosis made comparison of the levels of the cuspsdifficult. In the lateral projection the most inferiorlevel of the aortic and pulmonary sinuses was taken as thelevel of the valve rings (A. Rudhe, 1964, personal com-munications). Because of the aortic valve obliquity inthe antero-posterior projection, its mid-point was takenin the comparison of aortic and pulmonary valve levels.The width of the pulmonary artery and aorta was

measured in the antero-posterior projection.In an attempt to assess the degree of shunting, the

relative degree of opacification in the great vessels, andthe time sequence of opacification were noted.

RESULTS

Clinical Findings. rn Table I the main clinicalfindings in 15 patients with double outlet rightventricle with pulmonary stenosis, previously re-ported, are summarized, together with our fivepatients.

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FIG. 2.-Angiocardiogram, right ventricular injection. Antero-posterior and lateral views of Case 1 withdouble outlet right ventricle with pulmonary stenosis (infundibular and valvar). Aortic and pulmonaryvalves lie at the same level in antero-posterior projection. The origin of both great vessels from the opacifiedright ventricle is seen in the lateral projection. (<-) Prominent parietal band is seen in the antero-posteriorprojection. Previous subclavian-pulmonary artery anastomosis. o indicates the level of aortic and pul-

monary valves.

The male-to-female ratio was 1-5: 1 in our casesand in those reported by others, and the agesranged between 3 months and 31 years with a meanof 11-0 years. Shortness of breath was present inall our patients with double outlet right ventricleand pulmonary stenosis, and in 14 of the 15 patientsreported previously. Cyanosis was found at birthor developed during the first year of life in all our 5patients. The majority of the patients previouslyreported had a similar early onset of cyanosis. Theintensity of cyanosis progressively decreased in3 of our patients, being minimal in late child-hood. Four of our own patients and 5 reported inother series had clubbing of the fingers and toes.

Squatting, which has previously been recordedinfrequently in double outlet right ventricle withpulmonary stenosis, was recorded in all our patients,and was frequent in 3. Cyanotic attacks werepresent infrequently.The arterial pulse was usually normal in char-

acter in our patients. In 2, however, the left radialpulse was absent due to a previous subclavian-pulmonary artery anastomosis. Right ventricularhypertrophy was detected in all our, patients. A

systolic thrill was felt in only one patient in ourseries. It was present, however, in more than halfthe patients previously described. All our patientshad grade 3/4 ejection systolic murmurs maximalalong the left sternal border, bbtween the secondand the fourth intercostal spaces. A similar mur-mur was heard in nearly all the cases previously re-ported. This murmur was holosystolic in qualityin the cases reported by Levy et al. (1962) and byRedo et al. (1963). The second heart sound waswell heard and was single in most patients. Onlyone of our cases had duplication of the second pul-monary sound, similar to the patient reported byBraun et al. (1952). The site where the secondheart sound is maximally heard was reported to beof importance in differentiating Fallot's tetralogyfrom transposition of the great vessels (Nadas,1963). This was carefully noted in 4 of ourpatients: in 2 this sound was best heard in thesecond space close to the left sternal edge, as intransposition of the great arteries; in the other 2 thesecond heart sound was heard better in 'the fourthleft intercostal space, as in the tetralogy of Fallot.

Other auscultatory signs were caused by addit-

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ional lesions. For example, a continuous murmurwas heard in our patients who had been previouslysubmitted to a subclavian-pulmonary artery anasto-mosis. An early diastolic murmur was heard in thepatient reported by Levy et al. (1962) who had, inaddition to double outlet right ventricle with pul-monary stenosis, a bicuspid aortic valve and aorticincompetence.The jugular venous pulse was normal in 3 of our

patients and slightly raised in 2.Electrocardiogram. In our 5 patients the elec-

trical axis of the hearts in the frontal plane was+ 1200, + 1200, + 1300, + 1650 respectively in 4,and in the patient with dextrocardia it was + 40°.The P wave was high and peaked in 3 patients,indicating right atrial hypertrophy, and also bifidin one, indicating biatrial hypertrophy. Partialright bundle-branch block was found in one patientand a prolonged P-R interval in another.

Right axis deviation was not common in the casespreviously reported. Seven patients had, instead,left axis deviation (Neufeld et al., 1961a, b; Lauer,DuShane, and Edwards, 1960; Witham, 1957;Mirowski, Mehrizi, and Taussig, 1963), whichaccounts for 18 per cent of all cardiograms reportedin this condition. Mirowski et al. (1963) analysed aseries of 22 cardiograms of patients with doubleoutlet right ventricle with pulmonary stenosis, andnoted the frequency of left ventricular hypertrophy(74%), prolonged P-R interval (grade 1 heartblock) (60%), and partial right bundle-branch

block (63%) Other cases with left ventricularhypertrophy and partial right bundle-branch blockwere reported by Levy et al. (1962) and Braunet al. (1952).

Radiological Examination (plain films). The peri-pheral pulmonary arterial vascular pattern wasnormal in 4 patients in whom a plain film wasavailable, though 2 of them had had a previous sub-clavian-pulmonary artery anastomosis. In 3 pat-ients there was evidence of right ventricular en-largement in the lateral film (Fig. 3). In 2 patientsthe cardiac configuration suggested the tetralogy ofFallot in the postero-anterior projection (Fig. 3and 4). In one (Case 1, Fig. 5) who previously hadhad a subclavian-pulmonary artery anastomosis,there was evidence of generalized cardiac enlarge-ment. In one patient the appearances were normal(Fig. 6).H-modynamics. There have been earlier re-

ports of cardiac catheterization data in 7 patientswith double outlet right ventricle with pulmonarystenosis, and catheterization was performed in allour patients. The findings in the fully reportedcases are shown in Table III. The right atrialpressure and oxygen saturation were generallywithin normal limits. The right ventricularpressure was always raised to systemic levels, andthe mean systolic pressure was 100 mm. Hg for allthe patients. There was an increase in the oxygensaturation in the right ventricle compared with thesaturation in the right atrium in 4. The same

FIG. 3.-Chest radiograph. Postero-anterior and lateral views of Case 3 with double outlet right ventriclewith pulmonary stenosis simulating the tetralogy of Fallot, with large right ventricle and broad aortic sil-

houette but fairly normal peripheral pulmonary arterial pattern.

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FIG. 4.-Chest radiograph. Postero-anterior and lateral views of Case 4, with double outlet right ventriclewith pulmonary stenosis. Cardiomegaly present. Right ventricular outflow tract is normal. Pulmonaryarterial vascular pattern is normal. Previous subclavian-pulmonary artery anastomosis. Dextrocardia.

Situs inversus.

observation was found in 4 of the patients pre-viously reported. This is due to the total shunt ofthe left ventricular output through the ventricularseptal defect into the right ventricle. The increaseranged from 5 to 14 per cent in our patients andfrom 8 to 10 per cent in the earlier group.The catheter could not be introduced into the

pulmonary artery in 4 of our patients and in 6patients previously reported. In the patients inwhom the pulmonary valve was crossed, the pressuregradient ranged from 40 to 100 mm. Hg with amean of 77 mm. Hg. The pulmonary arterypressure varied from 8/0 to 57/34 mm. Hg. In onepatient reported by Levy et al. there was a furtherincrease in the oxygen saturation in the pulmonaryartery of about 8 per cent: this might be due to theincomplete mixing of the blood derived from thesystemic and pulmonary venous returns in the rightventricle. The arterial pressure was usually equalto the right ventricular pressure, and the arterialoxygen saturation varied between 61 and 93 percent. The left atrium was entered once in ourseries and in the cases reported by Coelho et al.(1963)' and Neufeld et al. (1961a). In our pat-ients the left atrial pressure and oxygen saturationwere normal. In those reported earlier, the leftatrial pressure was normal, but the oxygensaturation was reduced, possibly due to a right-to-left shunt across the atrial septum.

In no case was the left ventricular pressure signi-ficantly higher than the right ventricular pressurewhen both were measured simultaneously.

Angiocardiography. In the 5 patients consideredto have a double outlet right ventricle the angio-cardiogram showed that, in addition to the basicdefect of ventricular septal defect with pulmonarystenosis, the origins of the great vessels overlay eachother and were placed in the same coronal plane.Both the aorta and the pulmonary artery appearedto arise from the right ventricle and were opacifiedfrom this chamber. The essential diagnosticcriteria for double outlet right ventricle were thusfulfilled.The degree of forward displacement of the aorta

was variable: in all patients the right posterior(non-coronary) cusp lay at or anterior to the planeof the interventricular septum. In 2 patients(Cases 1 and 2) the anterior wall of the aorta layslightly anterior to the anterior wall of the pul-monary artery. In one (Case 5) there was super-imposition of the anterior wall of the aorta andpulmonary artery, and in another (Case 4) therewas dextrocardia, with both great vessels arisingfrom the anterior ventricle, the aorta being slightlyanterior to the pulmonary artery (Fig. 4).

In 3 patients (Cases 1, 2, and 5) the semilunarvalves of the aorta and pulmonary artery lay at thesame cross-sectional level as judged on antero-

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FIG. 5.-Chest radiograph. Postero-anterior and lateral view of Case 1, with double outlet right ventriclewith pulmonary stenosis. Cardiomegaly present. Right ventricular outflow tract is normal. Pulmonary

arterial vascular pattern is normal. Previous subclavian-pulmonary artery anastomosis.

posterior and lateral films. In 2 (Cases 3 and 4) the seen prominently in the antero-posterior projection,pulmonary valve lay superior to the aortic valve by but not at all in the lateral projection (Fig. 1, 2, 7,1 cm. and 3 cm., respectively. In all 5 patients the 10, and 11).parietal band of the crista supraventricularis was The sequence of opacification of the great vessels,

FIG. 6.-Chest radiograph. Postero-anterior and lateral view of Case 5, with double outlet right ventriclewith pulmonary stenosis. Appearance is normal.

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FIG. 7.-Angiocardiogram, right ventricular injection via femoral artery and aorta. Antero-posterior andlateral view of Case 4 with double outlet right ventricle with pulmonary stenosis. There is dextrocardiaand transposition ofthe great vessels, the aorta lying anteriorly and to the left ofthe pulmonary artery. Parietalband <- and aortic valve 4 are indicated. Previous pulmonary valvotomy and subclavian-pulmonary artery

anastomosis.

FIG. 8.-Angiocardiogram, left ventricular injection via atrial septal defect. Lateral view of a patient withthe tetralogy of Fallot. Most of aortic root overlies the right ventricle. The inclination of the aortic root is

more anterior than usual, but is not so anterior as in double outlet right ventricle (cf. Fig. 1).

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their relative densities, and the ratio of the width ofthe pulmonary artery to the width of the aorta areshown in Table II. The ratio of the width of thepulmonary artery to the width of the aorta variedfrom 0 7-0 9.

DISCUSSIONProper recognition of the double outlet right

ventricle with pulmonary stenosis is essential forsuccessful surgery, for the surgical treatment of thisentity differs from that required for Fallot's tetra-logy. Failure to recognize this congenital abnorm-ality at the time of operation, or ignorance of itsexistence, has led to inappropriate closure of theventricular septal defect, with fatal results due toobstruction of the only exit from the left ventricle(Redo et al., 1963; Engle et al., 1960).

Differentiation on a clinical basis alone is difficult.The early onset of cyanosis and significant degree ofshortness of breath should, however, draw attentionto the possibility of double outlet right ventricle withpulmonary stenosis. The site of maximum in-tensity of aortic closure in the 2nd intercostal spaceis a valuable clue, but maximum intensity in the 4thspace does not preclude the diagnosis.Another clue to the diagnosis may be derived

from the electrocardiogram. There may be evi-dence of slight left ventricular hypertrophy, pro-longed P-R interval, or right bundle-branch block

FIG. 9.-Angiocardiogram, injection into common ventricle,morphologically left, right ventricle is rudimentary. Lateralview of a patient with transposition of the great vessels.Aortic valve is at high level. * Points to the level of the

aortic valve.~~~~~~~~~~~~~~~~~~~~~..FIG. 10.-Angiocardiogram, right ventricular injection. Antero-posterior and lateral view of Case 2 withdouble outlet right ventricle with pulmonary stenosis. Anterior wall of the aorta (white arrow head) liesslightly anterior to anterior wall of pulmonary artery. Prominent parietal band of the crista supraventric-

ularis ().

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FIG. 11.-Angiocardiogram, right atrial injection. Antero-posterior and lateral view of Case 5 with doubleoutlet right ventricle with pulmonary stenosis. There is reflux into superior vena cava * 4. Prominent

parietal band of crista is seen -*. There is complete overlap of vessels in the_lateral projection.

in double outlet right ventricle with pulmonarystenosis. These findings are rare in the tetralogyof Fallot.The plain chest radiograph in our patients with

double outlet right ventricle with pulmonarystenosis does not provide any specific criteria to aidin the diagnosis. The pronounced right ventricularenlargement in 3 patients is to be expected. The"normal" peripheral pulmonary arterial vascu-lature in 3 patients who had not had a previous sub-clavian-pulmonary artery anastomosis was anunexpected finding. Our experience is at variancewith the larger group of patients reported byMehrizi (1965). All 18 of the patients with doubleoutlet right ventricle with pulmonary stenosis re-ported by him had pulmonary oligxmia. In ad-dition, 4 had a right-sided aorta. All our patientshad a left-sided aorta. His series, however, in-cludes very few angiocardiograms, and the reportstresses the necropsy findings. The wide spectrumof appearances of the plain chest film in the tet-ralogy of Fallot is well known. It is not surprising,therefore, that the chest radiographs in our patientsdid not yield any specific points to aid in the differ-entiation of double outlet right ventricle with pul-monary stenosis from the tetralogy of Fallot.The clinical criteria, cardiographic, or radiological

findings discussed above should demand furtherinvestigation of the condition by cardiac catheter-ization and angiocardiography. On cardiaccatheterization the suggestive points are: an increasein the oxygen saturation in the right ventricle ascompared with the right atrium in a cyanosedpatient, and inability to introduce the catheter intothe pulmonary artery. The increase in oxygensaturation from right atrium to right ventricle,though noted in some acyanotic patients with thetetralogy of Fallot, does not commonly occur incyanotic patients with the tetralogy of Fallot.Angiocardiography has an important place in thediagnosis of double outlet right ventricle withpulmonary stenosis and in its differentiation fromthe tetralogy of Fallot. It became apparent onreviewing the 120 patients with the tetralogy ofFallot that the more specific angiocardiographicappearances associated with tetralogy of Fallot arereadily identifiable. Nevertheless, the patientswith tetralogy of Fallot in whom there was markedaortic override of the ventricular septum could beconfused with the condition of double outlet rightventricle with pulmonary stenosis (Fig. 8).Even the necropsy diagnosis may be difficult

(Neufeld et al., 1961a, b). The discontinuity of themitral and aortic valves described in necropsy

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specimens of double outlet right ventricle withpulmonary stenosis (Carey and Edwards, 1965)cannot be depicted angiocardiographically.We have used several angiocardiographic criteria

in the diagnosis of double outlet right ventricle withpulmonary stenosis and its differentiation fromtetralogy of Fallot. That both great vessels shouldopacify directly from the right ventricle is self-evident. Following the description of the parietalband by Carey and Edwards (1965), we confirmedthat the parietal band of the crista supraventri-cularis was enlarged and clearly identifiable in theantero-posterior view in the 5 patients consideredto have double outlet right ventricle with pulmonarystenosis (Fig. 1, 2, 7, 10, and 11); it is seen as a widetongue of non-opacified tissue lying medial to theinfundibulum of the right ventricle. Despite itssize, it could not be visualized on the lateral pro-jection. By contrast, in the anterior view theparietal band is usually a slender tongue of non-opacified tissue in a typical example of the tetralogyof Fallot.

It might be expected that the parietal band wouldbe more prominent during right ventricular systolebut this was not always the case. Although it wasa striking feature throughout the cardiac cycle inCase 3 (Fig. 1), in others this structure was some-times seen better in one or other phase of the cardiaccycle.A relatively high position of the aortic semilunar

valve was seen in 4 out of 5 patients (see Table II).Although 3 out of 5 (Cases 1, 2, and 5) had theaortic and pulmonary semilunar valves in the samehorizontal plane in the antero-posterior and lateralprojection, this localization is somewhat of anapproximation, since the levels of the valve ringsvary during the cardiac cycle. Also, valve ringlevels are not always of value in differentiatingdouble outlet right ventricle with pulmonarystenosis from the tetralogy of Fallot, since we haveseen several patients with the latter condition andrelatively high position of the aortic valves, butwithout extreme aortic override. This aspect hasbeen previously discussed by Carey and Edwards(1965) in double outlet right ventricle with andwithout pulmonary stenosis. The fact that theaorta overrides the septum to such a degree that itarises wholly from the right ventricle may be theexplanation. Perhaps the high position is related tothe fact that the right ventricle has a distinct out-flow tract and so raises the root of the aortic valve.In support of this view is the fact that the aorticvalve is high in transposition and corrected trans-position of the great arteries (Fig. 9).The ascending aorta was seen to point inferiorly

and forward in all 5 patients considered to have

double outlet right ventricle with pulmonarystenosis. This was seen in a sufficient number ofpatients with the tetralogy of Fallot to be of doubt-ful differential value by itself.The ratio of the width of the pulmonary artery to

the width of the aorta varied from 0 7 to 0 9. Thiscontrasted with a group of 12 patients typical ofthe tetralogy of Fallot, in whom the mean ratio was0 5 on the antero-posterior projections. Smithet al. (1965) gave similar values in 21 patients withthe tetralogy of Fallot who had had surgical cor-rection. Of 31 patients, 21 had a ratio of pul-monary artery width to aortic width varying be-between 0 3 and 06, as measured at operation.The ratio for the remainder was between 07 and1.0. The greater relative width of the aorta to thepulmonary artery in the tetralogy of Fallot is wellknown. It is uncertain whether this ratio will proveto be of value for diagnosing double outlet rightventricle with pulmonary stenosis.The aorta never opacified prior to the pulmonary

artery after right ventricular injection, but therelative sequence of great vessel opacification ismerely a reflection of the higher resistance offered bythe systemic circulation over that offered by thepulmonary stenosis.

Engle et al. (1963) has classified the double outletright ventricle according to whether the ventricularseptal defect was subaortic or subpulmonary, butexact timing of great vessel opacification was of nohelp in making this subdivision. In only one ofour series (Case 3) could the site of the ventricularseptal defect be exactly identified: the catheterpassed from the right ventricle through the ven-tricular septal defect which could, therefore, beseen to be infracristal (Fig. 1).

In double outlet right ventricle the left ventriclecan only empty its blood into the right ventricle viathe ventricular septal defect. There is, therefore, aleft-to-right shunt at ventricular level, which ispresent irrespective of the presence of severe pul-monary artery obstruction. This situation isfundamentally different from the tetralogy of Fallotwith cyanosis at rest, where the dominant shunt isright to left. In only one patient was the leftventricle opacified initially (before recirculation),against the current of the left-to-right shunt. Inthis patient the catheter had been passed from theright ventricle to lie in the ventricular septal defectpointing into the left ventricle. The expected areaof negative contrast in the right ventricle due tounopacified blood coming from the left ventricle wasnot seen in any patient. This is not entirely unex-pected, since the ventricular septal defect opens highinto the outflow tract of the right ventricle involvedby pulmonary stenosis. There was no patient in

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this small group with right-sided aorta, thoughMehrizi (1965) described 4 such cases.

Steinberg and Engle (1965) strongly support theuse of left ventricular angiocardiography to differ-entiate double outlet right ventricle with pul-monary stenosis from the tetralogy of Fallot.These authors feel that in the left anterior obliqueprojection the origin of the aorta from the rightventricle can be differentiated from the aorticoverride of the tetralogy of Fallot, since the ventric-ular septum is seen end-on. Since double outletright ventricle with pulmonary stenosis clinicallysimulates the tetralogy of Fallot, and as the tetralogyof Fallot is by far the commoner anomaly it seemslikely that most patients will continue to be studiedby right ventricular catheterization and angiocardio-graphy. In the tetralogy of Fallot the essentialinformation needed is the internal anatomy andhaemodynamics of the right ventricle. A diagnosisof double outlet right ventricle with pulmonarystenosis can be made from a right-sided angio-cardiographic study alone. The additional infor-mation to be gained from a further left-sided angio-cardiographic examination would have to be anindividual and clinical decision for each patient.Left ventricular angiocardiography would furtherentail a transseptal or transaortic approach, since acatheter cannot always be made to pass through theventricular septal defect. This entails an additionalhazard. Carey and Edwards (1965) used the pos-ition of catheters in the right and left ventricle toshow that both aortic and pulmonary artery valveslie in the same coronal plane. Although our seriesdoes not include any left ventricular angiocardio-gram, in one patient the catheter passed from theright ventricle through the ventricular septal defectto opacify the left ventricle (Fig. 1).Our series did not include any patients with

moderator band hypertrophy. Cheng (1962) de-scribed the necropsy findings in a patient withdouble outlet right ventricle without pulmonarystenosis, in whom the right ventricle was partlydivided into two compartments by a hypertrophiedmoderator band projecting into the cavity of theright ventricle from. below, while a thickenedmuscle mass (presumably the parietal band) did sosimilarly from above. Since this patient diedduring angiocardiography, adequate films to showthe moderator band were not available.

CONCLUSIONDouble outlet right ventricle with pulmonary

stenosis and the tetralogy of Fallot may be similarclinically and angiocardiographically, but theirsurgical treatment differs. An important ana-tomical difference, the discontinuity of aortic and

mitral valve rings in double outlet right ventricle,is not apparent on angiocardiograms. Angiocardio-graphy is an essential preliminary to surgery incyanosed patients, and in order to give to thecardiologists and surgeons prior warning of thecondition the following scheme is suggested. Allpatients with a ventricular defect, pulmonary sten-osis, complete overlapping of the proximal ascend-ing aorta and pulmonary artery, and an ascendingaorta pointing inferiorly and forward in the lateralprojection, should be considered as potential ex-amples of double outlet right ventricle. In thosein whom there is prominence of the parietal bandin the antero-posterior view but failure to visualizea crista in the lateral view, the diagnosis should beregarded as one of double outlet right ventricle withpulmonary stenosis until disproved. If this turnsout to be correct at cardiotomy, the mistake ofclosure of the ventricular septal defect as part of thetotal correction of tetralogy of Fallot will not havebeen made. Instead, the appropriate sling oper-ation (McGoon, 1961; Kiklin et al., 1964; Redoet al., 1963) can be performed. If exploratorycardiotomy, however, shows the anatomy to be thatof the tetralogy of Fallot, then total correction withclosure of the ventricular septal defect can becarried out safely. In our view, patients withdouble outlet right ventricle with pulmonarystenosis who have only mild cyanosis and fewsymptoms should not have radical surgical correc-tion of this defect, because of the difficulties in-volved. By contrast, patients with the tetralogy ofFallot usually require radical correction. In thepresence of severe cyanosis due to double outletright ventricle with pulmonary stenosis, closedinfundibular resection or a systemic pulmonaryartery shunt operation may be useful palliativeprocedures.

SUMMARY

Earlier reports on patients with double outletright ventricle and pulmonary stenosis are re-viewed, and the findings in five additional patientswith this condition are reported.Double outlet right ventricle with pulmonary

stenosis is an uncommon lesion. Patients withthis condition are with difficulty differentiated fromthose with tetralogy of Fallot. The differentiationis important since the surgical treatment of the twodisorders is very different. In double outlet rightventricle with pulmonary stenosis the second soundmay be maximal at the second space along the leftsternal border. In tetralogy of Fallot this sound isloudest at the fourth space. A left-to-right ventric-ular shunt is infrequent in cyanosed patients with

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tetralogy of Fallot but is essential in patients withdouble outlet right ventricle with pulmonarystenosis. Angiocardiography shows that both greatvessels originate from the right ventricle, and lie inthe same coronal plane. The aortic valve is higherthan normal and points downwards and anteriorlyrather than downwards and posteriorly, as seen inmost patients with tetralogy of Fallot. Theparietal band of the crista supraventricularis isprominent in the antero-posterior projection butobscured in the lateral projection; this is in con-trast to the findings in the tetralogy of Fallot.

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Feldman, J., and Schorr, S. (1952). Complete dextro-position of the aorta, pulmonary stenosis, interven-tricular septal defect, and patent foramen ovale. Amer.HeartJ., 43, 773.

Carey, L. S., and Edwards, J. E. (1965). Roentgenographicfeatures in cases with origin of both great vessels fromthe right ventricle without pulmonary stenosis. Amer.J. Roentgenol., 93, 269.

Cheng, T: 0. (1962). Double outlet right ventricle: diagnosisduring life. Amer.J. Med., 32,637.

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Engle, M. A., Holswade, G. R., Campbell, W. G., and Gold-berg, H. P. (1960). Ventricular septal defect withtransposition of aorta masquerading as acyanoticventricular septal defect. Circulation, 22, 745.

, Steinberg, I., Lukas, D. S., and Goldberg, H. P.(1963). Acyanotic ventricular septal defect with bothgreat vessels from the right ventricle. Amer. Heart J.,66, 755.

Kirklin, J. W., Harp, R. A., and McGoon, D. C. (1964).Surgical treatment of origin of both vessels from rightventricle, including cases of pulmonary stenosis. J.thorac. cardiovasc. Surg., 48, 1026.

Lauer, R. M., DuShane, J. W., and Edwards, J. E. (1960).Obstruction of left ventricular outlet in associationwith ventricular septal defect. Circulation, 22, 110.

Levy, M. J., DeWall, R., Elliott, L. P., and Cuello, L. (1962).Origin of both great arteries from the right ventricleand pulmonary stenosis. Apropos case successfullycorrected. Dis. Chest, 42, 372.

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Mirowski, M., Mehrizi, A., and Taussig, H. B. (1963). Theelectrocardiogram in patients with both great vesselsarising from the right ventricle combined with pul-monary stenosis. Circulation, 28, 1116.

Morgan, J., Pitman, R., Goodwin, J. F., Steiner, R. E., andHollman, A. (1962). Anomalies of the aorta and pul-monary arteries complicating ventricular septal defect.Brit. Heart J., 24, 279.

Nadas, A. S. (1963). Pediatric Cardiology, 2nd ed., p. 703.Saunders, Philadelphia.

Neufeld, H. N., DuShane, J. W., and Edwards, J. E. (1961a).Origin of both great vessels from the right ventricle.II. With pulmonary stenosis. Circulation, 23, 603.

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Redo, S. F., Engle, M. A., Holswade, G. R., and Goldberg,H. P. (1963). Operative correction of ventricularseptal defect with origin of both great vessels from theright ventricle. J. thorac. cardiovasc. Surg., 45, 526.

Smith, D. R., Effat, H., Hamed, M. A., and Omeri, M. Al(1965). Radiological and surgical anatomy in tetralogyof Fallot and the effect on surgical prognosis. Brit.Heart_J., 27, 604.

Steinberg, I., and Engle, M. A. (1965). Angiocardiographicdiagnosis of both great vessels originating from rightventricle: report of thirteen acyanotic patients. Amer.Y. Roentgenol., 94, 45.

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Double Outlet Right Ventricle With Pulmonary Stenosis · Double Outlet Right Ventricle with Pulmonary Stenosis Type 2a. These patients have a supracristal ventricular septal defect

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