CONGENITAL MITRAL STENOSIS

(1)

Larry P. Elliott, M.D., Ray C. Anderson, M.D., Ph.D., Kurt Amplatz, M.D.,

C. Walton Lillehel, M.D., and Jesse E. Edwards, M.D.

Departments of Pediatrics, Radiology, Surgery, and Pathology, University of Minnesota, Minneapolis, Minnesota, and the Department of Pathology, Charles T. Miller Hospital, St. Paul, Minnesota

This study was supported by grants ( H 5694 and H 830) from the National heart Institute, United

States Public health Service, and from The Life Insurance s1edical Research Fund.

ADDRESS: (J.E.E.) The Charles T. Miller Hospital, 125 West College Avenue, St. Paul 2, Minnesota.

PEDIAmIc5, October 1962

CONGENITAL

MITRAL

STENOSIS

552

C

ONGENITAL MITRAL STENOSIS falls within

the category of anomalies that are

characterized functionally by causing

pul-monary venous obstruction. According

to

general experience this malformation often

seems to he associated with other cardiac

anomolies, particularly those of the aortic

valve and aortic arch. This association adds

to tile difficulties in making a clinical

diag-nosis of congenital mitral stenosis, a

condi-tion for which some surgical reparative

p0-tential exists.

The purpose of this study was to

deter-mine whether or not a diagnostic clinical

profile could be identified in congenital

mi-tral stenosis. The source of material

in-cluded 57 cases reported in the literature

and 4 cases observed at the University of

Minnesota Hospitals.

The details of the latter four cases are

re-viewed herein and the findings compared

\Vitil those in tile existing literature.

FOUR CASES OBSERVED

The clinical data of the four cases of

con-genital mitral stenosis observed at the

Uni-versity of Minnesota Hospitals are

pre-sented ilere in composite form to emphasize

the features common to all four cases

(Ta-ble I).

Among tile four patients, three were

fe-male (Cases 1, 3, and 4) and one was male

(

Case 2). The ages were 2, 3, 4, and 8 years,

respectively, at the time of initial

observa-tion. The maternal and prenatal histories had not been remarkable. One patient

(Case 4), weighed only 3 lb 11 oz (1,763

gm) at birth, and subsequently exhibited a

pattern of poor gain of weight. The

re-maining tilree patients seemed to be

nor-mal in the first month of life, but exercise

intolerance and deficiency in the pattern of

weight-gain soon developed. Recurrent

in-fections of the lower respiratory tract

(pos-sibly episodes of pulmonary edema) were

recorded for all four patients.

In each of three cases, a murmur was

de-tected during the first year of life, and in

the fourth patient, a murmur was first

rec-ognized at the age of 3 years (Case 3).

Cy-anosis was present in only one patient (Case

3) and this was noticed to be episodic. One

patient (Case 4) was occasionally observed

to assume the knee-chest position, a

re-sponse usually associated with attacks of

cyanosis or respiratory infection or both.

By history it was ascertained that in three

of the four patients, congestive cardiac

fail-ure had developed within the first year of

life: Case 1, 6 weeks; Case 2, 3 months; and

Case 4, 10 months. Initial response to

digi-talization was estimated to have been fair.

Physical examination showed that the

blood pressures were abnormal in only one

patient (Case 1, with flush arm pressure of

120 mm Hg and flush leg pressure of 70

mm Hg). In general appearance all patients

were described as thin, small, and poorly

developed. A left precordial bulge and an

apical thrill were present in each, the thrill

being systolic in three patients (Cases 1, 2,

and 4) and diastolic in one patient (Case 3).

In the patient with an associated

ventricu-lar septal defect

(Case

2), an additional

systolic thrill was located along the left

ster-nal border at the fourth left intercostal

space. Each of the four cases revealed a

(2)

Case

I

Case

3 Case

4 mLJ

aVL

L4

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Case

2

!i

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ARTICLES

apex. Accentuation of the second sound at

the pulmonary area was also noted in each

l)ttien t.

Electrocardiograph ic abnormalities

oc-curred in all four cases (Fig. 1). First

de-gree A-V i)lock ill ne patient was tile only

conduction disturbance (Case 4). The

A-Q

RS axis in the frontal pitie was of the

“right axis deviation” type in all four

pa-tients, Vitil values ranging fronl 100

de-grees to + 130 degrees. The P waves were

abnormal in all patients, displaying

fea-tures of enlargement of either the left atriuni

(

Cases 1 and 2) or both atria (Cases 3 and

IV4

L

:::::;L

L1W _

&vR1*

m17

*vIi1 +

&vrv.4.td

FIG. 1. Electrocardiograms in the four cases of congenital mitral stenosis reporte(l here. Case I. 1itr:tl st(’IlOSis and coarctation of aorta; age 2 years. Left atrial enlargement is slo\n lw IlOtChe(l, broad P wave in Lead II and late inversion of P ave in Lead V. Right ventricular

lly)ertrOphy of the systolic (pressure) overload type is indicated by the tall R wave in Lead V

(N/2 indicates half standardization). Case 2. Mitral stenosis and ventricular septal defect; age

3 years. Left atrial enlargement is shown by a broad, notched P wave in Lead I and late

inver-sion of P wave in Lead V3. A pattern of rSR’ indicating right ventricular overload is shown in

Lead Vi. Additionally, signs of left ventricular “overload” are indicated by the deep

Q

wave in

Lead V6 and the tall and peaked T waves in Leads II, III, and aVF. Case 3. Mitral stenosis

and patent ductus ateriosus; age 4 years. Left atriai enlargement is indicated b the notched P

vaves in Leads II, V and V,. Right atriai enlargement is additionally suggested by the tall, broad 1 wave ill Lead I and the narrow, tall P wave in Lead V. Case 4. Mitral stenosis and coar(tation

of the aorta; age 8 years. Right atriai enlargement is indicated by the tall, peaked P waves in

(3)

(‘ase Age (yr) Sex .1.s.socialed Malformations Onset Congestive (‘ardiac Failure (age) Murni sir .lpicai (Grade)* Syst. Diast. Age Onset Apical l’lsrill (Tune) folio,,’- iijt ‘2/6

1 ‘2 F

‘2 3 M

3 I F

4 8 F

Coaretation of tile

aorta

elItricUlar septal (lefect

Patent (lUctUs

arteriosus

(‘oarctation of the

aorta

Systolic ()perated 1 956 (closed

te(lllliqIle) :flu

6 wk 3 3 yr 10 mo 6 wk 3 mo 3 yr 10 mo 4/6 4/6 also 4 LICS ‘4/6 4/6 3/6 Systolic (also 3-4 LICS) 4/6 l)iastolic ‘2/6 Systolic

()perated 1956 (ol’lI

te(lIlIi(IUe) ;alive

()perated I 957 ( open

te(lllli(Ill(’) ; (li(’(l K

days postoperatively

No operation; died age 10 years

554 CONGENITAL MITRAL STENOSIS

TABLE I

CLINICAL ()BsKlIv.tTIoNs IN TIlE FOUR CASES OF CONGENITAL %IITRAL STENOSIM OF ‘l’llls STUDY

* SeCOU(l sound in pulmonary area, grade 3 to 4 and split, in each case.

4). Signs of hypertrophy of the right

yen-tricle of the systolic (pressure) overload

type were present in three of the four

pa-tients (Cases 1, 3 and 4). One patient (Case

2) showed signs of combined ventricular

overload,’ as demonstrated by an rsR

pat-tern in Lead V, and a deep

Q

wave in Lead

V6.

Thoracic roentgenograms showed

promi-nence of the right ventricle, enlargement

of the main pulmonary arterial shadow,

and enlargement of the left atrium in each

instance (Fig. 2, upper and left lower).

Addi-tionally, enlargement of the left ventricle

was considered to be present in one patient

(

Case 2). The pulmonary vascularity was

in-terpreted as being increased in two patients

(Cases 1 and 2).

The one angiocardiogram which was

per-formed (Case 1) was of the venous type,

and on the late film it showed delayed

emptying of a large left atrium (Fig. 2,

right lower). Data of the right-sided cardiac

catheterization performed in each case are

summarized in Table II. The systolic

pres-sure in the right ventricle was elevated in

each instance, and in the three cases in which the catheter entered the pulmonary

trunk the pressures in that location were also

elevated. In tile one patient in whom

meas-urement of the pulmonary arterial “wedge”

pressure was accomplished, the valve was

found to be elevated

(

37/20 mm Hg).

Evi-dence for a left-to-rigilt shunt was obtained

in Case 2 (associated ventricular septal

de-feet). In Case 3 (associated patent ductus

arteriosus) the oxygen saturation in the

pul-monary artery may have been high as a

re-suit of wedging of the catheter tip;

more-over, the low oxygen saturation in the

fe-moral artery would favor a reversed shunt

through the ductus arteriosus, although this

sample was obtained toward the end of tile

procedure. In Case 4, there is no

estab-lished explanation for the oxygen

desatura-tion of the systemic arterial blood, since

neither the coarctation of the aorta nor

mi-tral stenosis of itself would be responsible for a shunt. It is presumed that the oxygen

desaturation was a result of inadequate

oxy-gen exchange in the lung, although this

point is not established.

SURGICAL CONSIDERATIONS AND

RESULTS

Operation upon the mitral valve was

per-formed in three of the four patients, Case 1

(4)

* Ittt = right atrium ;RV = right ventricle; PA = pulmonary artery; PAC = pulmonary arterial “wedge” pressure;

FA =fenioral artery; MRA=mid-right atrium; NS not simultaneous with PA sample.

t Catheterization performed elsewhere. Pulmonary arterial wedge sample.

555

TABLE II

HEMODYNAMIC DATA IN Fouis C&sEs OF CONGENITAL MITRAL STENOSIS

Pressure*

(mm Jig)

(‘ace

R.1 RI’ PA PAC

Blood Oxygen Saturation*

(%)

-Oxygen Capacity

(Vol%) JVC MIIA SVC

-RV

--PA

1t 5/0 68/31 771,5.5 .. .. 79 70 73 75 9’2 16

4 5/0 85/() 85/50 .. 63 55 67 93 88 95.5 16.9

3

4

.5/-3 110/() 115/70 37/’20 6’2

38/10 Not entered

and Case 3 (January 18, 1957). In one

in-stance a closed technique was used (Case

1), while in tile other two (Cases 2 and 3)

an open technique was employed in

con-junction with cardiopulmonary bypass. In

each instance, prior to manipulation of the

mitral valve, a tilrill was perceptible over

tile anterior portion of the left atrium and

left ventricle. In each patient operated

up-on, a mitral commissurotomy was

per-formed. Following this procedure the

pre-\‘iously observed tilrill disappeared in each

patient.

In Case 1, in which the patient was 2

years old, a closed approach was utilized

tilrougll a left posterolateral thoracotomy.

The coarctation of the aorta (which was

severe Witil a 2-mm orifice) was repaired

and the ductus arteriosus closed. The mitral

valve was then approached through the left

atrium. Tile stenotic valve orifice was

en-larged by dilators.

The open approach utilized in Cases 2

and 3 was carried out by exposing the

mi-tral valve througil a left atriotomy after

cardiopulmonary bypass had been

estab-lished. Tile anterior and posterior

commis-sores were opened out to tile annulus. In

Case 2 (with a co-existing ventricular septal

defect) tile tilOracotomy was done through

an anterior bilateral intercostal approach.

68 7’2 61 88 80 14.5

34 4’2 44 47 8’2 20.3

In Case 3, thought to represent isolated

mi-tral stenosis, the thoracotomy was done

through the bed of the right fifth rib, which

provided excellent exposure of the mitral

valve but precluded any inspection of the

ductus area. Failure to detect an existing

patent ductus arteriosus was undoubtedly

of crucial importance. Of the three patients

on whom operation was performed, one

pa-tient (Case 3) died 8 days after surgical

in-tervention, while the other two patients

(

Cases 1 and 2) survived and are alive at

present.

Opportunity for re-examination in Case 1

presented itself in Marcil, 1961

(

courtesy

of Dr. B. H. Hartman), 5 years after surgical

therapy. The examination showed that,

al-though the patient had remained small and

thin, her exercise tolerance was excellent,

and she had had no episodes of congestive

cardiac failure since the operation. Physical

examination revealed a loud harsh apical

systolic murmur and thrill. No evidence of

cardiac enlargement existed at this time.

Upon examination in May, 1960

(

cour-tesy of Drs. R. B. Logue and E. R. Dorney),

4 years after tile operation, the patient in

Case 2 was free of symptoms. Examination

revealed a grade 2/6 ejection type systolic

murmur at the pulmonary area and

(5)

CONGENITAL MITRAL STENOSTS

Ill two of tile four cases necropsy was Per- Of tile two cases in which neropsv lla(l

,*‘t 6 ‘

., 4’

:

‘

‘ii

1h

536

considered normal for a boy of this age. No

cardiac murmur was identified at tile apex.

The roentgenogram of the thorax revealed

slight residual cardiac enlargement.

PATHOLOGIC FEATURES

formed and served as the basis for tue

ana-tOflliC analysis. In tile other two instances

tile patients are living at the time of this

writing, and tile description of the niitral

valve has been based upon surgical

obser-vations.

FIG. 2. Tlioracic roentgenograms in three of the four cases of congenital mitral stenosis. Upper left 071(1 right: Case 2. Anteroposterior and lateral thoracic roentgenograms with barium swallow showing right-Posterior displacement of the esophagus by an enlarged left atrium. Also, enlargernt’iit of cardiac

shadow and of pulmonary arterial segment. The pulmonary vasculature is prominent. Lower left:

Case 3. Posteroanterior roentgenogram showing a “double contour” shadow caused by an enlarged left atrium (LA). Lower right: Case 1. Late phase of venous angiocardiogram (lateral view) showing enlargement and delayed emptying of the left atrium (LA). Also shown is the aorta (A) and its

(6)

:

fr’c

“

--- A

Fic. 3. Case 3. Congenital stenosis of mitral valve. Left: Interior of left ventricle and mitral valve

Vie\VC(i from l)(-low. Mitral valve leaflets are thickened and the chordae are extremely short. Probe in

intrkcdlv narrowe(l mitral orifice. At the tip of the probe is a focus of endocardial thickening of the septal left ventricle representing a jet lesion. This is considered to have resulted from the trauma to this area

by a j(tlikC stream of blood passing through the stenotic mitral orifice. Patent ductus arteriosus was

asso-elated. Rig/it: Lov p\er photomicrograph of posterior leaflet of mitral valve and adjacent portions of the left atrium an(l left ventricle. The valvular tissue is markedly thickened with fibrous tissue

(H & E; X2.5).

iX’eIl 1X’rfOrllle(l, 0110 specimen (from Case

3) is available to us for study and will be

described. Tile remaining patient (Case 4)

(lied at ilOme.#{176}

In Case 3 (Fig. 3) each of tile mitral

leaf-lets was thickened and each commissure

vas fused. Tile chordae were unusually

short, so that PaI)illary muscles an(l valve

leaflets appeared to he in direct continuity.

All these changes converted tile valve into

a funnel type structure vitil considerable

narrowing of tile valvular orifice to an

effec-tive diameter of only about 5 mm.

Wilile

tue

changes are considered to be those associated with congenital mitral

ste-nosis, tile basic alterations are also similar

to tilose oi)served in rheumatic mitral

ste-nosis.

A nurni)er of secondary lesions were

ob-0 Necropsv l1L(l l)(Cl1 1)(fm1(l elsevhere, and the pathologic report submitted to us indicated tile presence of mitral stenosis and coarctation of the aorta. We are ifldel)ted to Drs. E. F. Ilagen and

H. E. Wvland for this information.

served in tile heart. These included a “jet

lesion” at tile apical portion of tile

endocar-dium of the left ventricle, a lesion

consid-ered to have resulted from the trauma of

tile jetlike stream of blood passing the

ste-notic mitral orifice. Dilatation and

ilyper-trophy of tile left atrium were a prominent

feature as was hypertrophy of the right

ventricle.

The remaining valves and the connections

of the great vessels with the heart were

normal. The aorta showed no obstruction

and the ductus arteriosus was patent,

hay-ing a diameter of 5 mm. The only

peculiar-ity of this heart with mitral stenosis was

tile presence of moderate hypertrophy of

tile left ventricle, which measured 1 cm in

thickness.

In the two living patients surgical

ob-servation indicated that (1) the valve cusps

were thickened without calcific deposits

and were pliable and (2) the basis for

vai-vular obstruction was fusion at each of tile

(7)

narrowing of the valve diameter to 3 mm

in one instance and to 4 mm in the other.

Histologic examination of the lungs in

Case 3 revealed features commonly seen in

obstructive pulmonary venous disease (Fig.

4). Clearly evident was medial hypertrophy

of the muscular arteries and of the

mus-cular portion of the arterioles. The

capil-lanes showed moderate engorgement with

some variation in calibre from area to area.

The radicals of the pulmonary veins were

abnormal, showing medial thickening in

some instances and intimal thickening with

collagen and elastic tissue.

The visceral pleura was characterized by

dilatation of venules and of iymphatics. The

lymphatics penetrated into the interiobular

septa as dilated structures.

Focal collections of air spaces contained

hemosiderin-laden macrophages.

COMMENT

Congenital mitral stenosis, either isolated

or combined with other malformations, is a

rare condition. In 1954 Ferencz et al.,2 in a

compreilensive report, reviewed 34 cases

\Vilich had appeared in the literature since

1846. To this group these authors added

nine cases studied in The Children’s

Me-morial Hospital, Montreal, since 1939. Since

tile time of that review, additional

re-ports’’1 have appeared, bringing the total

number of reported cases of congenital

mi-tral stenosis, as far as we know, to 57.

Among patients with congenital mitral

stenosis, association of other cardiac

anom-alies is extremely common; thus, the 57

reported cases included only 10 instances

of isolated mitral stenosis. The associated

malformations strongly tend to involve the

aortic valve and the aortic arch in the form

of aortic stenosis, patent ductus arteriosus,

coarctation of the aorta, singly or more than

one together. Patent ductus arteriosus is the

most common. Congenital mitral stenosis

associated with malformations of the

yen-tricular complex is rare. Ventricular septal

defect occurred in one patient in our series

(

Case 2) and, in this patient, involved the

muscular septum.

Among the clinical manifestations of

con-genital mitral stenosis, cyanosis is not

fun-damental. No patient in our series exhibited

persistent cyanosis. When present, cynaosis

was associated with congestive cardiac

fail-ure or pneumonia. Murmurs tend to be

heard early in life. In three of our patients

(

Cases 1, 2, and 4) murmurs were noted

be-fore the first year and in the fourth (Case

3), at 3 years of age. Some of these

mur-murs may have been related to associated

defects. The time of onset of symptoms

may vary from about 6 weeks of age to

be-yond 2 years of age, though symptoms

usri-ally develop within the first year of life.

In most reported cases congestive cardiac

failure developed within tile first year of life

and was often the primary clue to the

dis-covery of cardiac disease. Recurrent

pul-monary infections, exercise intolerance, and

failure to gain weight properly are

corn-monly associated. The occurrence of systolic

murmurs in association with mitral stenosis

may suggest accompanying mitral

insuffi-ciency. In the cases herein reported, mitral

insufficiency was ruled out by observations

made during operation. In the absence of

mitral insufficiency tile opinion is held that

the systolic murmur was generated by an

associated malformation, since, on occasion,

and particularly in infants, tile

malforma-tions wilich are of the types associated with

tile cases here reported may, even when

oc-curring as isolated conditions, cause systolic

murmurs that are ileard maximally in the

apical area.

Patients with patent ductus arteriosus

as-sociated with congenital mitral stenosis may

reveal normal systemic blood pressures

in-stead of the wide pulse pressure usually

found in isolated patent ductus arteriosus.

This hemodynamic paradox can be

cx-plained by the elevation of pulmonary

ar-terial pressure incident to tile mitral

ste-nosis. As the total pulmonary resistance

in-creases, a bidirectional or reversed shunt

through tile patent ductus arteriosus could

theoretically occur, resulting in differential

cyanosis of the feet. Established examples

(8)

.-* .- . . - ,.

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;‘ ‘‘. #{149}

I

&

Fic. 4. Case 3. Representative histologic changes seen in the pulmonary vessels. Upper left: Medial hypertrophy of a muscular artery (H & E; x450). Upper right: Wall of a pulmonary vein showing medial thickening (Elastic tissue stain; x450). Lower left: Pulmonary vein and small tributary showing lung. Dilatation of lymphatics in pleura and in interlobular septum. Dilatation of pleural veins slight intimal thickening of each (Elastic tissue stain; x600). Lower right: Visceral pleura and underlying

(9)

arteriosus in cases associated with

congeni-tal mitral stenosis ilave been reported by

Swan,1’ Ferencz,2 Carvalho Azevedo, and

their respective associates.

Though no one finding is pathognomic,

certain indications in the physical

examina-tion of the infant or child suggest mitral

stenosis as a diagnostic possibility. These

include (1) a left precordial bulge; (2)

either an isolated systolic or diastolic thrill

located entirely or in part at the apex; (3)

accentuation of the pulmonic component of

a split second sound (change in splitting

with respiration was not commented on in

most descriptions) and (4) murmurs

(sys-tolic or diastolic or both) with maximal

in-tensity at the cardiac apex.

Electrocardiographically, certain features

were commonly noted in published

electro-cardiograms and in each of our four cases.

These include evidence of (1) right axis

de-viation (2) hypertrophy of the right

ventri-cle, (3) enlargement of the left atrium or of

both atria, as well as the finding of (4) a

typical QRS frontal vector loop as derived

from the scaiar electrocardiogram.

Combined ventricular overload was seen

in one patient (Case 2), an instance with

co-existing ventricular septal defect. This

electrocardiographic finding was not

ob-served in reported cases of mitral stenosis,

although there was one electrocardiogram

reproduced in which a ventricular septai

defect coexisted.h1 In no cases with

co-existing patent ductus arteriosus was

biven-tricular hypertrophy with diastolic overload

evident. This supports the view that when

a patent ductus co-exists with congenital

mitral stenosis, a left-to-right shunt, if

pres-ent, is not large; the shunt, in fact, may be

predominantly right-to-left.

Evidence of hypertrophy of the right

yen-tricle is more common than are alterations

characteristic of enlargement of the left

atrium. Signs of left or combined atrial

en-largement existed in each of our four cases.

Nevertheless, normal P waves were present

in three of the six patients whose

electro-cardiograms were published by Ferencz

et al.2 in one of two cases of Braudo et

al.,8

and in both cases of Mata et al.h1

In analyzing tile frontal plane of tIle QRS

spatial vector derived from tile scalar

dcc-trocardiogram, OflC finds a similar

configura-tion in each of our patients. Tue clockwise

direction of inscription of tile QRS vector

loop with tile main body of tile lOOn

orien-tated abnormally to tile right is considered

the basis for tile abnormally tall R waves

seen in Lead aVR. A similar QRS vector

loop may be demonstrated in tile tracings

published by Carvaiho Azevedo and by

Braudo,S and their respective associates.

Thoracic roentgenograms show

cardio-megaly. Enlargement of specific chambers

appears to depend on the alterations in

function caused by associated

malforma-tions. The pulmonary vasculature was

prominent only in those patients with an

associated left-to-right shunt or in tilose

with congestive cardiac failure. The typical

roentgenologic pattern of pulmonary venous

obstruction was observed in only one of the

patients in our series (Case 1). The

roent-genogram in each of our patients showed

tile characteristic “double contour” shadow

characteristic of enlargement of the left

atrium. An enlarged left atrium iiav be

ob-served in eacil of the associated

malforma-tions when they occur as isolated

condi-tions. The degree of left atrial enlargement,

however, observed in patients with

co-exist-ing mitral stenosis appears to be greater

than in any of the other conditions alone.

Barium swallow was of additional aid in

demonstrating sucil enlargement in our

ma-terial, altilough Ferencz et a!. reported that

in none of their cases did tilis test reveal

deviation of the esophagus. Enlargement of

the left atrium may be less conspicuous in

the infant age group than in adolescents or

adults with mitral stenosis.

Venous angiocardiography may be

help-ful in demonstrating enlargement and

de-layed emptying of the left atrium. This test

is particularly important in distinguishing

mitral stenosis from most other forms of

congenital pulmonary venous obstruction.

Hypothetically, the only lesion that could

give rise to confusion would be

(10)

561

The differential diagnosis of congenital

mitral stenosis involves distinguishing

UIflOIl many conditions which cause

pul-illonary VenOuS obstruction. These include

stenosis of individual pulmonary veins, cor

triatriatum, supravalvular stenosing ring of

tue left atrium, mitral insufficiency, left

ventricular failure from any cause

(particu-iarly primary endocardial sclerosis), and

con(litionS involving left-to-rigilt shunts

Wi til pulmonary hypertension.

The diagnosis should be suspected in any

infant with (1) a history of recurrent

pul-monary problems suggesting pulmonary

edema, (2) systolic or diastolic murmurs

oc-curring together or alone and heard loudest

at tile apex, and (3) electrocardiographic

and roentgenographic evidence of

enlarge-ment of tile left atrium or left and right

atria and of the right ventricle (particularly

vithout evidence of enlargement of the left

ventricle). Hypertrophy of the left ventricle

suggests an associated malformation.

Tile diagnosis may be aided by (1)

dem-onstration by a pertinent type of cardiac

catileterization of an elevated pulmonary

artery “wedge” pressure, (2) demonstration

of a pressure gradient across the mitral

valve, and (3) demonstration by

angiocardi-ography of an obstructive lesion at the

mi-tral valve as evidenced by late emptying

of an enlarged left atrium.

As the great majority of reported

exam-pies of congenital mitral stenosis have had

the diagnosis made at necropsy, judgement

as to prognosis in the untreated case may

perhaps be colored by the type of material

studied. In reported cases the age at death

has in general been low. Of the 43 cases

re-viewed by Ferencz et al.2 only one patient

was older than 3 years at the time of

death. Since that review, Mata et al.h1 have

reported seeing patients of 5 and 16 years

of age. One of our patients (Case 4)

sur-viyed to the age of 10 years without

surgi-cal intervention.

Beyond intensive medical management

for congestive cardiac failure, prompt

diag-nosis and surgical treatment in spite of its

high risk seems the only hope for long

sur-vival in this condition.

In general, the results of surgical therapy

in the past have been discouraging. Among

tile seven surgically treated patients

previ-ously described in the literature, only two

had survived. Bower et reported the

first successful surgical treatment, which

in-volved a child aged 9 months; 7 months

after valvotomy the patient was reported to

be alive and well. The second patient for

whom successful surgical treatment of

con-genital mitral stenosis was reported was a

3-month-old patient of Braudo et al.8 This

patient was reported to be alive 16 months

after surgical intervention.

The high surgical mortality seems to be

related to the complex deformites of the

valve and chordae which often preclude complete surgical correction of the obstruc-five element. (The undetected patent

duc-tus arteriosus may have contributed to the

death of our Case 3.) In each of our two

surviving patients an associated malforma-tion (coarctation of the aorta in one and

ventricular septal defect in the second) was

corrected during the same operation as the

mitral valvotomy but prior to it.

The indications for an open as opposed to

a closed approach to these operations

can-not be categorized on the basis of this very

small surgical experience but it would

ap-pear to us that the open approach offers

some definite technical advantages

includ-ing, in selected cases, the opportunity for

replacing the mitral valve.

SUMMARY AND CONCLUSIONS

The clinicopathologic findings were

studied in four cases of congenital mitral

stenosis, all occurring with associated

mal-formations; these included coarctation of

the aorta (two instances), ventricular septal

defect (one case), and patent ductus

arterio-sus (one case). A high incidence of

associ-ated malformations of the aortic arch

sys-tern was also common in cases reported in

the literature. Our four cases of congenital

mitral stenosis and most of the 57 cases

re-ported in the literature presented a clinical

profile which may lead the clinician to

sus-pect congenital mitral stenosis, either alone

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malfor-562 CONGENITAL MITRAL STENOSIS

mation. These features include (1) a history

of recurrent pulmonary problems

suggest-ing pulmonary edema; (2) systolic or

dia-stolic murmurs, occurring together or

in-dividually, heard loudest at the apex; and

(3) electrocardiographic and roentgenologic

evidence of enlargement of left atrium or

left and right atria in combination, and

hy-pertrophy of the right ventricle. (Left

yen-tricular hypertrophy suggests an additional

malformation.) The diagnosis can be

strengthened further by (1) demonstration

by cardiac catheterization of an elevated

pulmonary arterial “wedge” pressure; (2)

demonstration of a pressure gradient across

the mitral valve; and (3) evidence obtained

by selective angiocardiography of an

ob-structive lesion at the mitral valve.

Left-sided cardiac catheterization is advisable

to identify or exclude co-existing anomalies.

A surgical potential exists for patients with

congenital mitral stenosis.

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Cir-culation, 23:225, 1961.

2. Ferencz, C., Johnson, A. L., and Wigglesworth,

F. W. : Congenital mitral stenosis. Circula-tion, 9:161, 1954.

S. Bover, B. D., et al.: Two cases of congenital

mitral stenosis treated by vaivotomy. Arch.

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4. Carvalho Azevedo, A., et a!.: Patent ductus

arteriosus and congenital mitral stenosis.

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5. Maxwell, C. M., and Young, W. P. : Isolated

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heart J., 48:787, 1954.

6. Kjellberg. S. R., et al.: Diagnosis of

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7. Nadas, A. S. : Pediatric Cardiology.

Philadel-phia and London, Saunders, 1957. p. 423.

8. Braudo, J. L., et a!.: Isolated congenital mitral

stenosis: report of two cases with mitral

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10. Edwards, J. E. : In Gould, S. E. : Pathology

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:318, 1960.

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1962;30;552

Pediatrics

Larry P. Elliott, Ray C. Anderson, Kurt Amplatz, C. Walton Lillehei and Jesse E. Edwards