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Congenital mitral valve disease associated with coarctation of the aorta
Congenital with
mitral
coarctation
A report
London,
valve of
the
disease
associated
aorta
of 39 cases
England
c
ottgenital mitral valve disease as an isolated lesiori is a rare auotnal~~. \Iost reported experience has been with relatively few cases associated with other congenital cardiac defects.‘,? Mitral stenosis occurs with increased frequency in patent cluctus at-teriosus, aortic stettosis, ventt-irular septal defect, and occasionall>\vith atria1 septal defect (Lutentbacher’s s!mdrottte), and endocardial fibroelastosis. Alitral regurgitatiott (incompetence) is cYmlInoni~ associated with endocardial cushion defects (ostittrrt printurn atria1 sjc[M defects or complete atrioventricular c;tual), and occasionallywith endocardial lil)roelastosis. There are few reported cases of congenital tnitral valve defects associated bvith coarctatiott of the thoracic aorta.“-” This paper draw attention to this rektionship, a11r1 its influence on sttrvival. Methods llit ral valve defects were I,! either cardiac investigation
documented (i.e. cardiac
catheterizatiou and selective attgiocat-diogmph!-), operation, or postmortem exatuination. Cases diagnosed clittically, but without confirmation 1)~. one of the above methods were excluded, as were those children with any evidence suggestive of rheumatic fever, or of subacute bacterial endocarditis. Children with endocardial cushion defects were excluded since a cleft anterior (septal) leaflet of the mitral valve is au integral part of this complex defect. Patients with the hypoplastic left heart syndrome were also excluded.” Two sources of patient ntaterial were available. \;C’e have reviewed the 15 year operative experience from 1953 to 1967 \vith 333 children (179 heiug under one year of age) who had operations for coarctation of the thoracic aorta, and we assessed the coincidence of mitral valve dcfects (Group I). The second source was a separate group of 74 childretl coming to Ijostmorterrt exatnination durittg the same period, who had coarctatiott of the aorta as part of a complex of cotigettital cardiac
From the Thoracic Unit, The Hospital for Sick Children. Great Ormond Street, This study was supported by the British Heart Foundation. Received for publication Seyt. Ii. 1968. *Present address: Cardiac Department. The Hcwital of Sick Children. Toronto. **Giannini Foundation Fellow, Department of Surgery. University oi California
I;01
J-7. .VCI. 6, pp. 743-754
JZOIC, 1969
American
London,
Ilngland.
Ontario, Medical
Canada. Center. San Franci%m.
Heart
Jottrnal
Cnlil.
743
5. M.,
R. I\‘.,
51. F., ll
D. H., F
1‘. J., Xl 5. E., >I
L. L., F
3
1
5
6
7 8
9
E. H., F N. Xl., F .A. F., F
\\‘.
14 15 I6
Ii
G.. XI
P. B., ),I
11
11
s. s.,
10
Xl
F
P. s., 11
2
M
c. n.,
I
Cm-e Patient, *To. i sex
I
.! w-1;.
9 >r.
1 mo.
Age at operation (Conm!.)
I
Juxtalig. Juxtalig. Preductal Juxtaductal Preductal Juxtaductal Justaductal
Juxtaductal
+ 0 + + + + + +
0
0 +
t
0 + -t
0 +
0
+
+
+
+
+ I’reductal
Preductal Juxtalig. Juxtalig.
l’ostductal Juxtaductal
Postlig.
+
+
+’ 3 0 3
Juxtalig.
+
+
Type of coarct.
Juxtalig.
P.H. T.
1
in patients
0
+
C. C.F.
/
data and method of diagnosis
j Age at ! diagnosis ( (Coarc‘t.)
I
I
Table I. Clinical
;\IS
NlR, MS MK
imi, %lR
MR
311~
MR
AlS MK
AIR
$lR
XIR
MR
MS
MR,
MS
31s
MS
Mitral value lesion
undergoing
FDA, VSD EFE, LA EFE, I~.\‘, Subaortic stenosis, ASD:TR PD.%, EFE. LA, .\ortic
PD.1 P D:l
0
.-Is
0
0
0
Other cardiac lesions
coarctation
-
+ i
+
+
+
+ +
+
+
+
+
+
+
, Cardiac , ineesti( gation
1 Method mitral I--
-
-
+ + +
-+
.-
-
-
_-
-
-
+
-
-
&-I u( topsy
-
+
-
Operation
of diagnosis of nralve disease --
resection (Croup I)
,
I ).().‘I-.;
\‘I-
:2live and symptom-free; increasing L\‘H on ECG; awaiting reinvestigation Open mitral valvotom!.at .3’i years: relieved C.C.F. refractory to medical treatment .Uve and well, on digitalis; awaiting mitral annuloplast~ .Xlive; C.C.F. improved; on maintenance digitalis Alive and \vell; not in C.C.F.; on digitalis .llive and well: moderate I,\‘11 on ECG -Alive and well .\live and \\-ell: reconrctation resected at 61;/ \enr~ :Jlive; recoarctation; awaiting rejection -Alive; frequent respiratory infections; awaiting mitral valve repair .1live; mitral valvotomy at 11 years, 3 to i mm. orifice opened fully; ? abnormal chordae .X)&e and well; L\W + on ECG .Uive; recoarctatiou resected at 11 vears DI0.T.: P-1 hemorrhage, \‘F n.0.T. ; VF D.O.T. : VI’
Olclcome
lesions, but who had not had resection of the coarctation. The incidence of nritral valve defects was determined in this group (Group II). Results Gkoup I. Coarctation CLINICAL
DATA
AND
resected. METHOD
OF DIAGNOSIS
hlitral valve defects occurred in 23 of 333 patients in the operative group, an incidence of 7 per cent. Sixteen were male, and 7 were female. The age at diagnosis ranged from one week to 12 years, with a median of 6 weeks. All but one patient presented with hypertension in the arms and absent or weak femoral pulses. (Patient No. 17, with a preductal coarctation, had forceful femoral pulses.) Mitral valve diseasewas suspected preoperativeI>. in only 14 cases (61 per cent). Congestive cardiac failure was present in 78 per cent (18 of 23), at the time of resection of the coarctation. All of the infants were in congestive cardiac failure at the time of operation. The failure was refractory to medical therapy in 12 patients and the coarctation was resected as an emergency procedure. Mitral regurgitation was present in 13 children, mitral stenosis in 5, and 4 had a combined lesion. One child had mitral atresia. .%ssociatedcardiac lesions were common. A patent durtus arteriosus was present in 16 children. In 10 children the patent ductus arteriosus was associated with a preductal coarctation (Table I). Cardiac lesions other than mitral occurred in 11 children (47 per cent), ventricular septal defect being the most common. (TABLE
I).
HEMODYNAMIC
FINDINGS
(TABLE
II).
ru’ille-
teen of the 23 patients in the group undergoing coarctation resection had cardiac catheterization. Eleven were investigated before resection of their coarctation, 7 after, and 1 patient both before and after resection. The left ventricle was catheterized in 10 patients, and t-he left atrium in only. 4. pulmonary arHowever, :I salkfactor), terial \veclge pressure \vas obtained in 14. I;ourteen patients had pulmonary hypertension (l~uln~onar~~arter1’ systolic pressure > 40 mm. Hgj and 13 had elevation of the pulmonary wedge pressure. (Man)
746
E~lslhopc
C’l al.
t
-t-t
t
of the patients were in cardiac failure and therefore an elevated pulmonary arterial wedge pressure was not, by itself, an illdication of mitral valve disease. j dNGIOC~~KDIOGRAPHIC
FINDINGS
(TAHLE
Clear angiocardiographic evidence of mitral regurgitation was obtained in 10 patients. Examples are shown (Figs. 1, (No. 2) had evidence 2, and 3). One patient of mitral stenosis. TW:O others (Nos. 1 and 22) had indirect evidence of nlitral stenosis angiographically, but had good clinical, electrocardiographic, and radiographic evidence of this lesion II).
OPEKATI~
RESULTS
(TABLE
I).
1. Hospital deaths: Eight (39 per cent) of the 23 children died in hospital followinK resection of their coarctation (l;ig. 4). Seven of the deaths occurred in infants, 5 being under 3 months of age. Four died at operation with ventricular fibrillation. in Four died within 1 \veeks of resection persistent congestive failure with pulmonary complir-ations. 2. Late deaths: One death occurred 7 months after coarctation resection from an unknown cause. The other late death occurred 36 hours after lnitral an~luloplasty, performed 8 nlonths after resection of the child’s coarctation. 3. Survivors: There are 13 children nou living. The median age at the time of resection was 21 d years compared to a median of 2 nlouths for those who died. The survivors have heen followed for periods of 7 months to 11’ ; \‘ears _ (mean 4 J-ears 7 lllolltlls).
Two children have had successful mitral valvoloniies. One has been followed for S years after a closed mitral valvotomy and IS \f:ell, without evidence of mitral regurgitation. The other had a limited mitral valvotolny under cardiopulmonaq* bypass of a parachute type of mitral valve with relief of persisteiit congestive cardiac failure 6 n1011ths after resection of his coarctation. He is well, 4 months postTUW children lvith unitral operativelv. lilitr;il \~ilve regurgitatinll are ;i\\xiting rep;air. ‘1’hrt.c otht~rs have h;ltl ;I recoarc‘ta1ioii s~ic~c~ssfull~~ r~src~lcd.“” The 6 miminiiig .chiltlre[t III-~> cl&rig u~ell, 1,111 \vill prolxrl~l~~ require Illitral \xlvc~ surgery (valvoto~ii~, repair or repl~~w~lc~~t‘) in the future.
748
Husthope
et-cd.
B
A
-ig. f. Case 10. I,V angiocardiograrn, of aorta. .I, Systole. A, Diastole.
Fig. 2. Case 1. I,V angiocardiogram, aorta resected. -4, Systole. B, Diastole.
POSTMOKTEM
I:INDINGs
(Th1~1~:
anteroposterior
anteroposterior
III).
projection.
projection.
()f
the 0 children in the operative group conling to postmortem cxmnination, 4 hat1 mitral stenosis, 4 had iiiconipetent nlitral valves, and one had mitral atresia. Six had involving the cnclocardial fibroelastosis
left
Severe
Severe
:l(t-itllll
mitral
mitral
;111<1:/or
regurgitatioll:
regurgitation;
left
recoarctatiwb
coarctation
vc’lltric.lt*.
the Q children, a cardiac. anon~aly dition to coarctation of the aorta, tluctiis arteriosus, and niitral valve was present, this being a ventricular defect in 4.
III
of
6 of in XIpater~t
disease septal
Fig. 3. Case 3. LV projection. Severe of .torta.
angiocardiogram, mitral regurgitation;
7‘trbIe I I I. Poshnortem Xitual v&e postmortem
anteroposterior coarctation
data in Group I patients at
Preductal
1.5 16
Mitral stenosis Shortened chordae; valve obviously incompetent
Juxtaligamental Juxtaligamental
17
blitral
stenosis
Preductnl
I8
XIitral
valve
IO
Mitral stenosis; shortened chordae h1 itral stenosis; 0.5 cm. diam. orifice; shortened rhordae attached to single papillary muscle Tethered, thickened chordae; valve obviously incompetent Dilated ring; shortened chordae; deficient posterior cusp
2I
73
resected)
TrPe of
Dilated ring; thickened free edges of cusps
ntresia
(coctrcttrtio~~
Other
coarctation
II
10
Groltp Il. C’ourcttrtiola not resected. C‘LINI<‘,\L DATA (TAnLri IV), nfitral valve defects occurred in 16 of 74 children, an incidence of 22 per cent. These children did not have resection of their coarctation and postmortem examination confirmed or revealed the associated defects. The age at death of these 16 children ranged front 1 day to 4 ).ears (median 7?$ months). Twelve were infants and 6 were under 3 months of age. The nature of the terminal illness is summarized in Table IV. Most children died before the diagnosis was established or while awaiting surgery for the coarctation. Ten children (63 per cent) died within 48 hours of admission to hospital. Earlier in the series, two children died during aortic valvotom). with ventricular fibrillation. Coarctation was not suspected preoperatively as both had palpable femoral pulses associated with a preductal coarctation. One other child died 2 da>-s after ligation of a patent duct us arteriosus. One
Preductnl Preductal Preductal
I’reductal
Predtlctal
cardiac
anomalies
Patent ductus arteriosus; small membranous ventricular septal defect; dilated tricuspid valve ring; endocardial fibroelastosis of left atrium Endocardial fibroelastosis of left atrium Secundum atrial septal defect; subaortic stenosis (muscular); endocardial fibroelastosis of left ventricle; dilated tricuspid valve ring; persistent (left) S.V.C. --) coronary sinus + R.A. Aortic valve atresia; patent ductus arteriosus; endocardial fibroelastosis of left atrium and left ventricle Patent ductus arteriosus; ventricular septal defect (membranous); quadricuspid pulmonary valve Small membranous ventricular septal defect; patent ductus arteriosus; tricuspid septal cusp anomaly Patent ductus arteriosus; persistent (L) S.V.C. ---) coronary sinus -+ R.A.; bicuspid aortic valve; membranous ventricular septal defect; muscular ventricular septal defect; endocardial fibroelastosis of left atrium Patent ductus arteriosus; endocardial fibroelastosis of left ventricle Patent
ductus
arteriosus
l‘uhlr
I \f. Yosttnortenz
!Jmo.
$ndings
in Group
II pdients
(coarddion
not resected)
De&h 24 hr. after admission; congestive cardiac failure Death 24 hr. after admission; acute bronchitis
Mitral stenosis--thickened, rigid cusps, thickened short chordae Mitral stenosis-thickened cusps, partially fused
duxtaligamental
Endocardial fibroelastosis {)f left auricle and left ventricle
Preductal
:1y”.
Death during aortic valvotomy (ventricular fibrillation
Mitral regurgitationdilated ring, short chor&as, abnormal cusps (cartilaginous)
Preductal
2 yi-.
Death 2 days after admission; congestive cardiac failure and bronchopneumonia Death 2 days after division of trachea-esophageal fistula and gastrostomy for esophageal atresia
11 itral regurgitation-+ dilated ring, short chw dae, rigid ant.erior cusp, thickened wit,h cartilage hlitrnl atresin
1’relignment;d
Patent ductus arteriosus; endw cardial fibroelastosis of left auricle and left ventricle Patent ductus arteriosus; ventricular septal defect (membranous); bicuspid (stenosedi aortic valve; subaortic stenosis (muscular) Endocardial fibroelastosis of left auricle and left vent,ricle; bicuspid aortic valve
3y
Death 10 hr. after admission; myocardial infarction
hlitral regurgitation-shortened chordac, thickened cusps
8 mo.
Death during aortic valvotomy (ventricular fibrillation)
Mitral stenosis-narrow ring, diam. 1.2 cm.
I’reductal
3 mo.
Death 2 days after ligation of patent ductus arteriosus (coarctation not resected) Death 2 days after admission; congestive cardiac failure and bronchopneumonia I)eath 24 hr. after admission; congestive cardiac failure
Ntral regurgitation-dilated ring gelatinous vegetations on cusps
auxtaductal
Mitral regurgitationdilated ring; absence of chordae; cartilaginous nodules on cusps Mitral regurgitation-very dilated ring; two main cusps and additional frills
l’reductal
Death 2 weeks after admission; congestive cardiac failure and bronchopneumonia Death 2 days after admission; congestive cardiac failure Death 24 hr. after admission; congestive cardiac failure Death 14 hr. after admission; congestive cardiac failure
Mitral regurgitationdilated ring; gelatinous vegetations on cusps
Preductal
Mitral
regurgitationesshort chordae and papillary muscles Mitral regurgitation short, t.aut ehordae
Preductal
Patent ductus arteriosus
Preductal
Patent ductus arteriosus
hlitral
I’reductal
Patent ductus arteriosus; ventricular septal defect (large, membranous) ; tricuspid valve cusps thickened and short chordae
7 mo.
5 days
1 yr.
10 days
8 mo.
6 wk.
2 wk.
8 days
taut,
I’reductal
l’rrligamental
(‘omplete aortic arch atresia
Patent ductus arteriosus; ventricular septal defect (large, membranous); persistent left superior vena cava j eorw nary sinus Awticstenosis (bicuspid valve); ventricular septnl defect I membranous; occluded by adherent cusp of tricuspid valve); thrombosis left corednary artery Patent ductus arteriosus; hicuspid aortic valve Istrnosed); subaortic stenosis (muscular1 Patent ductus arteriosus (Iigated ) ; ventricular septal defect Imembranous) Patent ductus arteriosus; ventricular septal defect (muscular); dilated tricuspid ring; cartilaginous cusps Patent ductus arteriosus; tricuspid atresia; ventriculal septal defect (membranous); transposition of the great arteries Patent ductus arteriosus; persistent left superior vena wv:t f coronary sinus
tremely
atresia
3s
P
1 day
39
11
7: h mo. Death (ventricular fibrillation) after test injection of contrast material into left ventricle
Imtient tion.
died
POSTMORTEM
Death 12 hr. after admission; congestive cardiac failure
following FINDINGS
rn>-ocardial (TABLE
Ntrsl
atresia
J’reductul
Mitral regurgitationthickened cuspsand short chordae
infarcIV).
111
this second group, the coarctation was preductal in 10 children and in 2 others, preligainental. A patent ductus arteriosus n.as present in 2 others, one of whom had am-tic atresia. Ten had mitral incompetence (regurgitation), 3 had mitral stenosis, and 3 mitral atresia. All 16 children in this postmortenl group had additional cardiac anomalies. \.entricular septal defect occurred in half the cases. Discussion It is clear from this study that the association of congenital mitral vaIve disease with coarctation of the aorta is not rare. The incidence was 7 per cent iu the operative group of 333 children. It would be 10 per cent if 12 other children in this group nho have on14; clinical evidence of mitral valve disease were included. The incidence was 22 per cent in a postmortem group of 74 patients who did not have resection of thy coarctatioil. III most large published series of coarctation of the aorta, mitral valve disease is illfrcquentl~nlentioned.13-1* ILIichaelsson,19 reviewing the experience of the TJniversit?, Hospital, l.Tppsala, Sveden, from 19.52 to 1967, found 111 cases of coarctatioll of the aorta, 11 of whom had congenital mitral valve disease, an incidence of 10 per cent. Bernhard and Norman20 ilotrrl 5 cases of nlitral incompetence among 11 infants with coarctation. i)li the other hand, most reports of con-
Preligamental
Patent ductus arteriosus; right pulmonary veins --f coronary sinus; bicuspid aortic valve: bicuspid right-sided atrioventricular valve Endocardial fibroelastosis of left auricle and left ventricle; fibrous nodules on tricuspid valve cusps
genital mitral valve disease in the literature contaiii a significant proportion of patients with associated coarctation of the aorta.3-11 There were 23 cases of coarctation in Van der Worst and Hastreiter’$ comprehensive review of 122 cases of congenital nlitral stenosis, an incidence of 19 per cent. Fort)- per cent of children with untreated congenital heart disease die in the first )rear of life.21a?3 Infants with severe, mdtiple cardiac lesions usually develop congestive cardiac failure and die. Early death also occurs with coarctation of the aorta associated with lnitral valve disease. We have found that nlitral valve disease is present in a significantly greater number of childreu who die before their coarctation can be resected than in those who come to resection (22 per cent versus 7 per cellt,). We feel that this explains the apparent discrepancy bctweeu the incidence of mitral valve disease iu our experieuce of coarctation of the aorta, and that in previous reports, because a relatively greater proportion of infants is seen at this hospital. Only by effective diagnosis and therap). will many of these infants survive. Resection of the coarctation as an emergenq’ procedure may be lifesaving, often in the presence of other cardiac anomalies, which may then be correctable later. About half of the iufants in persistent coiigestive cardiac failure can be saved by earl) operation.z” AIost die iu early infant\,, whether or not mitral valve disease ;s present (Figs. 4 and 5). The prognosis for these children with
lo r
0
6/52
3112
6/12
lyr.
2
3
1.
Co;trctntion
ot’ the
sort;\
with
mitral
valve
Hospital
q
Late
death death
,
L
,
K
I
,
,
t
4
5
6
7
8
9
10
11
Age Fig.
m
I
I
12
13+
at Operation
diheaie:
death?.
70 65 60 55 50 45 Lo 2 2 4o
I
E! 35 “0 k 30 “E 25 2 2C
Deaths
8 ILlJzlJliM 13
15
13
8
1c
5
I
1
( 0
6/52
3/12
7
Age
3
$a
at Operation
8
9
10
11
12
13 +
nlitral valve disease who survive resectiotl of their coarctation is not good. Vau der Horst reported that 20 per cent of childreu with congenital mitral stenosis die iu the neonatal period, 50 per cent within the first year, and only SO per cent live to 3 l-ears of age. Slit& valvotom?~ for congenital tllitral stenosis has a mortalit>rate of 40 per cent to 63 per cent and postoperatively Illitral regurgitation is co~n~llou, because the valve tissue is so abnormal.“~‘” The operative results are somewhat luore encouraging with congenital mitral incolltpeteuce, but depend on the t).pe of almormality-i.e., dilated mitral valve ring, c.left leaflet, fenestration, or ruptured c.]lortlae,“,‘“-“X There is a general reluctance to replace the mitral valve with a prosthesis in a child lmy~use of 3 principal considerations, growth, thro~llboen~bolisnl and durability of tile valve. Ho%mver, Tank and associateszg recently reported that valve replacement proved the only successful form of treatment in their experience \vith 14 children, 2 to f 5 years old with congenital mitral stenosis. 131ood\vell and associates30 recently reported a 28 per cent cumulative mortalit) rate for 1X mitral valve replacements in children. Long-term results following mitral valve replacenlent are not >.et available.
Congenital
23~3.19,
Horst,
I<. L.,
and
Hahtrciter,
.i.
I<.:
1961.
l)aoud, G.. Kaplan, S., I’errin, E. V., I )orht, J. f’., and Edwards, k‘. K.: Congenit;tl Illitrill htenosis. Circulation 27:185. 1963. 10. Elliutt, I.. I’., Anderwn, I<. C., ~\r~lplatz, Ii., Lillehei. C. it.. and Edwards. I. l-1.: Conuenital mitral ~;e,losi~,‘P’edi~ltrics 30:552, 1962. c 11. AtIger. P., and \Vigle, E. I.).: Coarrtntiwl of the aorta aswci;jted with bevere mitral illwfficiellcy, Am. J. Cardiol. 21:190, 1968. 12. Sillha, 5. S., liu~nak. S. I,., Sommer~, H. M.. Cole, I~. B., Muster, A. J., and Paul, ?I. H.: Hypoplastic left ventricle syndrome. Analvsih of thirty altop>> cases in infants with sur$xl con~iderntion~, Am. J. Cardiol. 21:166, 1968. 13. Srhubter, S. Ii.. and Gross, Ii. E.: Surger). fol coarctation of the aorta; n revieiv of j(N) c*tyeh, 14.
J. ‘I‘homcic Pr C:lrdiovas. SLlq. 43:5-k, CIA+. 1. H.. Mustxrd, \‘1’. ‘I‘., md Keith. C~~arctntitm of I:! year1961).
15.
16.
17.
18.
21.
der
J. Cardiol.
9.
20.
l’an
AnI.
Ferencz, C., Johnson, X. l.., and \l’iglesworth, F. IV.: Congenital mitral stenosis, Circulatioll 9:161, 1954. S\van, H., Trapnell, J. XI., and I)enst, J.: Congenital mitral steuoais and s:.stemic right ventricle with associated pulmonary v:~xular changes frustrating surgical repair of patent ductus arteriosus and coarctation of the aorta, AM. HLAIU J. 38:914, 1949. H. K., Shapiro, M., Redillgtwl, J. \*., 6. ‘I‘aji, and iiay, J. I(.: Congenital mitral htenwk, J. ‘I’horaric Sr Cardiovas. Surg. 53:850, 1967. C. i\., Maha, G. I<.. and orgain, 7. Muwoe, E. S. : Congenital mitral stenosis awxiated \vith coarctation of the aorta, bicuspid arxtic valve, hypoplasia of the left ventricle. auricttlar septal defect, and tricuspid valvular IWLIformation, AS!. HI~AI
19.
3.
htenmis:,
4.
Summary i’oltgenital niitral valve defects occurred in 23 (7 per cent) of 333 infants and chiltlrell who had a coarctation of the aorta resected. X 22 per cent incidence was found at postniorteni esamiriatioii in a separate group of 74 patients who had not had operation for coarctation. Reasons for the relatively high incidence of this combinatioli of cardiac anomalies, previously cousideretl rare, are discussed with special refereucc to natural selectioll. The prognosis of those itlfauts and children surviving resectiotl of the coarctatioll is poor.
mitral
20:773, 1967.
~JI the aorta experience,
iu infants; I’ediatrb
1962.
J. 11.: ;L revien 26:lOY.
754
22.
23.
24.
2.5.
26.
I’ustkopc
Pi 111.
Hoffman, J. I. E.: Natural historT\. of congenital heart disease: Problems in its assessment xvith special reference to ventricular septal defects, Circulation 37:97, 1968. Tawes, Ii. I>., Aberdeen, E., 12’aterston, D. J., and Bonhan-Carter, Ii. E.: Coarctation of the aorta in infants and childret]--a review of 333 operative cases including 179 infants, in press. Starkey, C. LV. 13.: Surgical experience in the treatment of congenital mitral stenosis alld mitral insufficiency, J. ‘l‘horncic & Cardiovas. Surg. 38:336, 1959. Creech, O., lxdbetter, .\I. K., a~ld I
27.
28.
29.
.iO.
I.orig-term results of valvuloplast)for initl-,!I irlwticiency in childrell, J. ‘I‘horncic & (‘:II diovas. Surg. 53:1, 1967. Flege, J. R., Vlad, P., and Ehrenhalt, J. I..: Congenital mitral incompetence, J. Thurwil, ck Cardiovns. Surg. 53:138, 1967. l.evy, M. J., \rarco, I<. I,., Lillehei, C. \\‘.. .111d Ednxds, J. E.: IIitral insufficiency in inlallt-. children, and adolescent.s, J. Thoracic & (‘,I[-diovas. Surg. 55:434, 1963. Talk, I<. S., Bernhard, \V. F., and Gobs, I<. 1.1.: 5 Irgicd ,lll,ltomy of cotlgenitnl mitral stelllbia. ~irrlliatih,l’(S~Ippl. 2) 36:246. 1967. Bluodwell, I(. I)., H~IIIII;LII. (;. I.., :UI~ Cooler, I ). .I.: Cardiac valve replawtneut in childrc~~. Surgery 63:77, 1968.
mitral
coarctation
A report
London,
valve of
the
disease
associated
aorta
of 39 cases
England
c
ottgenital mitral valve disease as an isolated lesiori is a rare auotnal~~. \Iost reported experience has been with relatively few cases associated with other congenital cardiac defects.‘,? Mitral stenosis occurs with increased frequency in patent cluctus at-teriosus, aortic stettosis, ventt-irular septal defect, and occasionall>\vith atria1 septal defect (Lutentbacher’s s!mdrottte), and endocardial fibroelastosis. Alitral regurgitatiott (incompetence) is cYmlInoni~ associated with endocardial cushion defects (ostittrrt printurn atria1 sjc[M defects or complete atrioventricular c;tual), and occasionallywith endocardial lil)roelastosis. There are few reported cases of congenital tnitral valve defects associated bvith coarctatiott of the thoracic aorta.“-” This paper draw attention to this rektionship, a11r1 its influence on sttrvival. Methods llit ral valve defects were I,! either cardiac investigation
documented (i.e. cardiac
catheterizatiou and selective attgiocat-diogmph!-), operation, or postmortem exatuination. Cases diagnosed clittically, but without confirmation 1)~. one of the above methods were excluded, as were those children with any evidence suggestive of rheumatic fever, or of subacute bacterial endocarditis. Children with endocardial cushion defects were excluded since a cleft anterior (septal) leaflet of the mitral valve is au integral part of this complex defect. Patients with the hypoplastic left heart syndrome were also excluded.” Two sources of patient ntaterial were available. \;C’e have reviewed the 15 year operative experience from 1953 to 1967 \vith 333 children (179 heiug under one year of age) who had operations for coarctation of the thoracic aorta, and we assessed the coincidence of mitral valve dcfects (Group I). The second source was a separate group of 74 childretl coming to Ijostmorterrt exatnination durittg the same period, who had coarctatiott of the aorta as part of a complex of cotigettital cardiac
From the Thoracic Unit, The Hospital for Sick Children. Great Ormond Street, This study was supported by the British Heart Foundation. Received for publication Seyt. Ii. 1968. *Present address: Cardiac Department. The Hcwital of Sick Children. Toronto. **Giannini Foundation Fellow, Department of Surgery. University oi California
I;01
J-7. .VCI. 6, pp. 743-754
JZOIC, 1969
American
London,
Ilngland.
Ontario, Medical
Canada. Center. San Franci%m.
Heart
Jottrnal
Cnlil.
743
5. M.,
R. I\‘.,
51. F., ll
D. H., F
1‘. J., Xl 5. E., >I
L. L., F
3
1
5
6
7 8
9
E. H., F N. Xl., F .A. F., F
\\‘.
14 15 I6
Ii
G.. XI
P. B., ),I
11
11
s. s.,
10
Xl
F
P. s., 11
2
M
c. n.,
I
Cm-e Patient, *To. i sex
I
.! w-1;.
9 >r.
1 mo.
Age at operation (Conm!.)
I
Juxtalig. Juxtalig. Preductal Juxtaductal Preductal Juxtaductal Justaductal
Juxtaductal
+ 0 + + + + + +
0
0 +
t
0 + -t
0 +
0
+
+
+
+
+ I’reductal
Preductal Juxtalig. Juxtalig.
l’ostductal Juxtaductal
Postlig.
+
+
+’ 3 0 3
Juxtalig.
+
+
Type of coarct.
Juxtalig.
P.H. T.
1
in patients
0
+
C. C.F.
/
data and method of diagnosis
j Age at ! diagnosis ( (Coarc‘t.)
I
I
Table I. Clinical
;\IS
NlR, MS MK
imi, %lR
MR
311~
MR
AlS MK
AIR
$lR
XIR
MR
MS
MR,
MS
31s
MS
Mitral value lesion
undergoing
FDA, VSD EFE, LA EFE, I~.\‘, Subaortic stenosis, ASD:TR PD.%, EFE. LA, .\ortic
PD.1 P D:l
0
.-Is
0
0
0
Other cardiac lesions
coarctation
-
+ i
+
+
+
+ +
+
+
+
+
+
+
, Cardiac , ineesti( gation
1 Method mitral I--
-
-
+ + +
-+
.-
-
-
_-
-
-
+
-
-
&-I u( topsy
-
+
-
Operation
of diagnosis of nralve disease --
resection (Croup I)
,
I ).().‘I-.;
\‘I-
:2live and symptom-free; increasing L\‘H on ECG; awaiting reinvestigation Open mitral valvotom!.at .3’i years: relieved C.C.F. refractory to medical treatment .Uve and well, on digitalis; awaiting mitral annuloplast~ .Xlive; C.C.F. improved; on maintenance digitalis Alive and \vell; not in C.C.F.; on digitalis .llive and well: moderate I,\‘11 on ECG -Alive and well .\live and \\-ell: reconrctation resected at 61;/ \enr~ :Jlive; recoarctation; awaiting rejection -Alive; frequent respiratory infections; awaiting mitral valve repair .1live; mitral valvotomy at 11 years, 3 to i mm. orifice opened fully; ? abnormal chordae .X)&e and well; L\W + on ECG .Uive; recoarctatiou resected at 11 vears DI0.T.: P-1 hemorrhage, \‘F n.0.T. ; VF D.O.T. : VI’
Olclcome
lesions, but who had not had resection of the coarctation. The incidence of nritral valve defects was determined in this group (Group II). Results Gkoup I. Coarctation CLINICAL
DATA
AND
resected. METHOD
OF DIAGNOSIS
hlitral valve defects occurred in 23 of 333 patients in the operative group, an incidence of 7 per cent. Sixteen were male, and 7 were female. The age at diagnosis ranged from one week to 12 years, with a median of 6 weeks. All but one patient presented with hypertension in the arms and absent or weak femoral pulses. (Patient No. 17, with a preductal coarctation, had forceful femoral pulses.) Mitral valve diseasewas suspected preoperativeI>. in only 14 cases (61 per cent). Congestive cardiac failure was present in 78 per cent (18 of 23), at the time of resection of the coarctation. All of the infants were in congestive cardiac failure at the time of operation. The failure was refractory to medical therapy in 12 patients and the coarctation was resected as an emergency procedure. Mitral regurgitation was present in 13 children, mitral stenosis in 5, and 4 had a combined lesion. One child had mitral atresia. .%ssociatedcardiac lesions were common. A patent durtus arteriosus was present in 16 children. In 10 children the patent ductus arteriosus was associated with a preductal coarctation (Table I). Cardiac lesions other than mitral occurred in 11 children (47 per cent), ventricular septal defect being the most common. (TABLE
I).
HEMODYNAMIC
FINDINGS
(TABLE
II).
ru’ille-
teen of the 23 patients in the group undergoing coarctation resection had cardiac catheterization. Eleven were investigated before resection of their coarctation, 7 after, and 1 patient both before and after resection. The left ventricle was catheterized in 10 patients, and t-he left atrium in only. 4. pulmonary arHowever, :I salkfactor), terial \veclge pressure \vas obtained in 14. I;ourteen patients had pulmonary hypertension (l~uln~onar~~arter1’ systolic pressure > 40 mm. Hgj and 13 had elevation of the pulmonary wedge pressure. (Man)
746
E~lslhopc
C’l al.
t
-t-t
t
of the patients were in cardiac failure and therefore an elevated pulmonary arterial wedge pressure was not, by itself, an illdication of mitral valve disease. j dNGIOC~~KDIOGRAPHIC
FINDINGS
(TAHLE
Clear angiocardiographic evidence of mitral regurgitation was obtained in 10 patients. Examples are shown (Figs. 1, (No. 2) had evidence 2, and 3). One patient of mitral stenosis. TW:O others (Nos. 1 and 22) had indirect evidence of nlitral stenosis angiographically, but had good clinical, electrocardiographic, and radiographic evidence of this lesion II).
OPEKATI~
RESULTS
(TABLE
I).
1. Hospital deaths: Eight (39 per cent) of the 23 children died in hospital followinK resection of their coarctation (l;ig. 4). Seven of the deaths occurred in infants, 5 being under 3 months of age. Four died at operation with ventricular fibrillation. in Four died within 1 \veeks of resection persistent congestive failure with pulmonary complir-ations. 2. Late deaths: One death occurred 7 months after coarctation resection from an unknown cause. The other late death occurred 36 hours after lnitral an~luloplasty, performed 8 nlonths after resection of the child’s coarctation. 3. Survivors: There are 13 children nou living. The median age at the time of resection was 21 d years compared to a median of 2 nlouths for those who died. The survivors have heen followed for periods of 7 months to 11’ ; \‘ears _ (mean 4 J-ears 7 lllolltlls).
Two children have had successful mitral valvoloniies. One has been followed for S years after a closed mitral valvotomy and IS \f:ell, without evidence of mitral regurgitation. The other had a limited mitral valvotolny under cardiopulmonaq* bypass of a parachute type of mitral valve with relief of persisteiit congestive cardiac failure 6 n1011ths after resection of his coarctation. He is well, 4 months postTUW children lvith unitral operativelv. lilitr;il \~ilve regurgitatinll are ;i\\xiting rep;air. ‘1’hrt.c otht~rs have h;ltl ;I recoarc‘ta1ioii s~ic~c~ssfull~~ r~src~lcd.“” The 6 miminiiig .chiltlre[t III-~> cl&rig u~ell, 1,111 \vill prolxrl~l~~ require Illitral \xlvc~ surgery (valvoto~ii~, repair or repl~~w~lc~~t‘) in the future.
748
Husthope
et-cd.
B
A
-ig. f. Case 10. I,V angiocardiograrn, of aorta. .I, Systole. A, Diastole.
Fig. 2. Case 1. I,V angiocardiogram, aorta resected. -4, Systole. B, Diastole.
POSTMOKTEM
I:INDINGs
(Th1~1~:
anteroposterior
anteroposterior
III).
projection.
projection.
()f
the 0 children in the operative group conling to postmortem cxmnination, 4 hat1 mitral stenosis, 4 had iiiconipetent nlitral valves, and one had mitral atresia. Six had involving the cnclocardial fibroelastosis
left
Severe
Severe
:l(t-itllll
mitral
mitral
;111<1:/or
regurgitatioll:
regurgitation;
left
recoarctatiwb
coarctation
vc’lltric.lt*.
the Q children, a cardiac. anon~aly dition to coarctation of the aorta, tluctiis arteriosus, and niitral valve was present, this being a ventricular defect in 4.
III
of
6 of in XIpater~t
disease septal
Fig. 3. Case 3. LV projection. Severe of .torta.
angiocardiogram, mitral regurgitation;
7‘trbIe I I I. Poshnortem Xitual v&e postmortem
anteroposterior coarctation
data in Group I patients at
Preductal
1.5 16
Mitral stenosis Shortened chordae; valve obviously incompetent
Juxtaligamental Juxtaligamental
17
blitral
stenosis
Preductnl
I8
XIitral
valve
IO
Mitral stenosis; shortened chordae h1 itral stenosis; 0.5 cm. diam. orifice; shortened rhordae attached to single papillary muscle Tethered, thickened chordae; valve obviously incompetent Dilated ring; shortened chordae; deficient posterior cusp
2I
73
resected)
TrPe of
Dilated ring; thickened free edges of cusps
ntresia
(coctrcttrtio~~
Other
coarctation
II
10
Groltp Il. C’ourcttrtiola not resected. C‘LINI<‘,\L DATA (TAnLri IV), nfitral valve defects occurred in 16 of 74 children, an incidence of 22 per cent. These children did not have resection of their coarctation and postmortem examination confirmed or revealed the associated defects. The age at death of these 16 children ranged front 1 day to 4 ).ears (median 7?$ months). Twelve were infants and 6 were under 3 months of age. The nature of the terminal illness is summarized in Table IV. Most children died before the diagnosis was established or while awaiting surgery for the coarctation. Ten children (63 per cent) died within 48 hours of admission to hospital. Earlier in the series, two children died during aortic valvotom). with ventricular fibrillation. Coarctation was not suspected preoperatively as both had palpable femoral pulses associated with a preductal coarctation. One other child died 2 da>-s after ligation of a patent duct us arteriosus. One
Preductnl Preductal Preductal
I’reductal
Predtlctal
cardiac
anomalies
Patent ductus arteriosus; small membranous ventricular septal defect; dilated tricuspid valve ring; endocardial fibroelastosis of left atrium Endocardial fibroelastosis of left atrium Secundum atrial septal defect; subaortic stenosis (muscular); endocardial fibroelastosis of left ventricle; dilated tricuspid valve ring; persistent (left) S.V.C. --) coronary sinus + R.A. Aortic valve atresia; patent ductus arteriosus; endocardial fibroelastosis of left atrium and left ventricle Patent ductus arteriosus; ventricular septal defect (membranous); quadricuspid pulmonary valve Small membranous ventricular septal defect; patent ductus arteriosus; tricuspid septal cusp anomaly Patent ductus arteriosus; persistent (L) S.V.C. ---) coronary sinus -+ R.A.; bicuspid aortic valve; membranous ventricular septal defect; muscular ventricular septal defect; endocardial fibroelastosis of left atrium Patent ductus arteriosus; endocardial fibroelastosis of left ventricle Patent
ductus
arteriosus
l‘uhlr
I \f. Yosttnortenz
!Jmo.
$ndings
in Group
II pdients
(coarddion
not resected)
De&h 24 hr. after admission; congestive cardiac failure Death 24 hr. after admission; acute bronchitis
Mitral stenosis--thickened, rigid cusps, thickened short chordae Mitral stenosis-thickened cusps, partially fused
duxtaligamental
Endocardial fibroelastosis {)f left auricle and left ventricle
Preductal
:1y”.
Death during aortic valvotomy (ventricular fibrillation
Mitral regurgitationdilated ring, short chor&as, abnormal cusps (cartilaginous)
Preductal
2 yi-.
Death 2 days after admission; congestive cardiac failure and bronchopneumonia Death 2 days after division of trachea-esophageal fistula and gastrostomy for esophageal atresia
11 itral regurgitation-+ dilated ring, short chw dae, rigid ant.erior cusp, thickened wit,h cartilage hlitrnl atresin
1’relignment;d
Patent ductus arteriosus; endw cardial fibroelastosis of left auricle and left ventricle Patent ductus arteriosus; ventricular septal defect (membranous); bicuspid (stenosedi aortic valve; subaortic stenosis (muscular) Endocardial fibroelastosis of left auricle and left vent,ricle; bicuspid aortic valve
3y
Death 10 hr. after admission; myocardial infarction
hlitral regurgitation-shortened chordac, thickened cusps
8 mo.
Death during aortic valvotomy (ventricular fibrillation)
Mitral stenosis-narrow ring, diam. 1.2 cm.
I’reductal
3 mo.
Death 2 days after ligation of patent ductus arteriosus (coarctation not resected) Death 2 days after admission; congestive cardiac failure and bronchopneumonia I)eath 24 hr. after admission; congestive cardiac failure
Ntral regurgitation-dilated ring gelatinous vegetations on cusps
auxtaductal
Mitral regurgitationdilated ring; absence of chordae; cartilaginous nodules on cusps Mitral regurgitation-very dilated ring; two main cusps and additional frills
l’reductal
Death 2 weeks after admission; congestive cardiac failure and bronchopneumonia Death 2 days after admission; congestive cardiac failure Death 24 hr. after admission; congestive cardiac failure Death 14 hr. after admission; congestive cardiac failure
Mitral regurgitationdilated ring; gelatinous vegetations on cusps
Preductal
Mitral
regurgitationesshort chordae and papillary muscles Mitral regurgitation short, t.aut ehordae
Preductal
Patent ductus arteriosus
Preductal
Patent ductus arteriosus
hlitral
I’reductal
Patent ductus arteriosus; ventricular septal defect (large, membranous) ; tricuspid valve cusps thickened and short chordae
7 mo.
5 days
1 yr.
10 days
8 mo.
6 wk.
2 wk.
8 days
taut,
I’reductal
l’rrligamental
(‘omplete aortic arch atresia
Patent ductus arteriosus; ventricular septal defect (large, membranous); persistent left superior vena cava j eorw nary sinus Awticstenosis (bicuspid valve); ventricular septnl defect I membranous; occluded by adherent cusp of tricuspid valve); thrombosis left corednary artery Patent ductus arteriosus; hicuspid aortic valve Istrnosed); subaortic stenosis (muscular1 Patent ductus arteriosus (Iigated ) ; ventricular septal defect Imembranous) Patent ductus arteriosus; ventricular septal defect (muscular); dilated tricuspid ring; cartilaginous cusps Patent ductus arteriosus; tricuspid atresia; ventriculal septal defect (membranous); transposition of the great arteries Patent ductus arteriosus; persistent left superior vena wv:t f coronary sinus
tremely
atresia
3s
P
1 day
39
11
7: h mo. Death (ventricular fibrillation) after test injection of contrast material into left ventricle
Imtient tion.
died
POSTMORTEM
Death 12 hr. after admission; congestive cardiac failure
following FINDINGS
rn>-ocardial (TABLE
Ntrsl
atresia
J’reductul
Mitral regurgitationthickened cuspsand short chordae
infarcIV).
111
this second group, the coarctation was preductal in 10 children and in 2 others, preligainental. A patent ductus arteriosus n.as present in 2 others, one of whom had am-tic atresia. Ten had mitral incompetence (regurgitation), 3 had mitral stenosis, and 3 mitral atresia. All 16 children in this postmortenl group had additional cardiac anomalies. \.entricular septal defect occurred in half the cases. Discussion It is clear from this study that the association of congenital mitral vaIve disease with coarctation of the aorta is not rare. The incidence was 7 per cent iu the operative group of 333 children. It would be 10 per cent if 12 other children in this group nho have on14; clinical evidence of mitral valve disease were included. The incidence was 22 per cent in a postmortem group of 74 patients who did not have resection of thy coarctatioil. III most large published series of coarctation of the aorta, mitral valve disease is illfrcquentl~nlentioned.13-1* ILIichaelsson,19 reviewing the experience of the TJniversit?, Hospital, l.Tppsala, Sveden, from 19.52 to 1967, found 111 cases of coarctatioll of the aorta, 11 of whom had congenital mitral valve disease, an incidence of 10 per cent. Bernhard and Norman20 ilotrrl 5 cases of nlitral incompetence among 11 infants with coarctation. i)li the other hand, most reports of con-
Preligamental
Patent ductus arteriosus; right pulmonary veins --f coronary sinus; bicuspid aortic valve: bicuspid right-sided atrioventricular valve Endocardial fibroelastosis of left auricle and left ventricle; fibrous nodules on tricuspid valve cusps
genital mitral valve disease in the literature contaiii a significant proportion of patients with associated coarctation of the aorta.3-11 There were 23 cases of coarctation in Van der Worst and Hastreiter’$ comprehensive review of 122 cases of congenital nlitral stenosis, an incidence of 19 per cent. Fort)- per cent of children with untreated congenital heart disease die in the first )rear of life.21a?3 Infants with severe, mdtiple cardiac lesions usually develop congestive cardiac failure and die. Early death also occurs with coarctation of the aorta associated with lnitral valve disease. We have found that nlitral valve disease is present in a significantly greater number of childreu who die before their coarctation can be resected than in those who come to resection (22 per cent versus 7 per cellt,). We feel that this explains the apparent discrepancy bctweeu the incidence of mitral valve disease iu our experieuce of coarctation of the aorta, and that in previous reports, because a relatively greater proportion of infants is seen at this hospital. Only by effective diagnosis and therap). will many of these infants survive. Resection of the coarctation as an emergenq’ procedure may be lifesaving, often in the presence of other cardiac anomalies, which may then be correctable later. About half of the iufants in persistent coiigestive cardiac failure can be saved by earl) operation.z” AIost die iu early infant\,, whether or not mitral valve disease ;s present (Figs. 4 and 5). The prognosis for these children with
lo r
0
6/52
3112
6/12
lyr.
2
3
1.
Co;trctntion
ot’ the
sort;\
with
mitral
valve
Hospital
q
Late
death death
,
L
,
K
I
,
,
t
4
5
6
7
8
9
10
11
Age Fig.
m
I
I
12
13+
at Operation
diheaie:
death?.
70 65 60 55 50 45 Lo 2 2 4o
I
E! 35 “0 k 30 “E 25 2 2C
Deaths
8 ILlJzlJliM 13
15
13
8
1c
5
I
1
( 0
6/52
3/12
7
Age
3
$a
at Operation
8
9
10
11
12
13 +
nlitral valve disease who survive resectiotl of their coarctation is not good. Vau der Horst reported that 20 per cent of childreu with congenital mitral stenosis die iu the neonatal period, 50 per cent within the first year, and only SO per cent live to 3 l-ears of age. Slit& valvotom?~ for congenital tllitral stenosis has a mortalit>rate of 40 per cent to 63 per cent and postoperatively Illitral regurgitation is co~n~llou, because the valve tissue is so abnormal.“~‘” The operative results are somewhat luore encouraging with congenital mitral incolltpeteuce, but depend on the t).pe of almormality-i.e., dilated mitral valve ring, c.left leaflet, fenestration, or ruptured c.]lortlae,“,‘“-“X There is a general reluctance to replace the mitral valve with a prosthesis in a child lmy~use of 3 principal considerations, growth, thro~llboen~bolisnl and durability of tile valve. Ho%mver, Tank and associateszg recently reported that valve replacement proved the only successful form of treatment in their experience \vith 14 children, 2 to f 5 years old with congenital mitral stenosis. 131ood\vell and associates30 recently reported a 28 per cent cumulative mortalit) rate for 1X mitral valve replacements in children. Long-term results following mitral valve replacenlent are not >.et available.
Congenital
23~3.19,
Horst,
I<. L.,
and
Hahtrciter,
.i.
I<.:
1961.
l)aoud, G.. Kaplan, S., I’errin, E. V., I )orht, J. f’., and Edwards, k‘. K.: Congenit;tl Illitrill htenosis. Circulation 27:185. 1963. 10. Elliutt, I.. I’., Anderwn, I<. C., ~\r~lplatz, Ii., Lillehei. C. it.. and Edwards. I. l-1.: Conuenital mitral ~;e,losi~,‘P’edi~ltrics 30:552, 1962. c 11. AtIger. P., and \Vigle, E. I.).: Coarrtntiwl of the aorta aswci;jted with bevere mitral illwfficiellcy, Am. J. Cardiol. 21:190, 1968. 12. Sillha, 5. S., liu~nak. S. I,., Sommer~, H. M.. Cole, I~. B., Muster, A. J., and Paul, ?I. H.: Hypoplastic left ventricle syndrome. Analvsih of thirty altop>> cases in infants with sur$xl con~iderntion~, Am. J. Cardiol. 21:166, 1968. 13. Srhubter, S. Ii.. and Gross, Ii. E.: Surger). fol coarctation of the aorta; n revieiv of j(N) c*tyeh, 14.
J. ‘I‘homcic Pr C:lrdiovas. SLlq. 43:5-k, CIA+. 1. H.. Mustxrd, \‘1’. ‘I‘., md Keith. C~~arctntitm of I:! year1961).
15.
16.
17.
18.
21.
der
J. Cardiol.
9.
20.
l’an
AnI.
Ferencz, C., Johnson, X. l.., and \l’iglesworth, F. IV.: Congenital mitral stenosis, Circulatioll 9:161, 1954. S\van, H., Trapnell, J. XI., and I)enst, J.: Congenital mitral steuoais and s:.stemic right ventricle with associated pulmonary v:~xular changes frustrating surgical repair of patent ductus arteriosus and coarctation of the aorta, AM. HLAIU J. 38:914, 1949. H. K., Shapiro, M., Redillgtwl, J. \*., 6. ‘I‘aji, and iiay, J. I(.: Congenital mitral htenwk, J. ‘I’horaric Sr Cardiovas. Surg. 53:850, 1967. C. i\., Maha, G. I<.. and orgain, 7. Muwoe, E. S. : Congenital mitral stenosis awxiated \vith coarctation of the aorta, bicuspid arxtic valve, hypoplasia of the left ventricle. auricttlar septal defect, and tricuspid valvular IWLIformation, AS!. HI~AI
19.
3.
htenmis:,
4.
Summary i’oltgenital niitral valve defects occurred in 23 (7 per cent) of 333 infants and chiltlrell who had a coarctation of the aorta resected. X 22 per cent incidence was found at postniorteni esamiriatioii in a separate group of 74 patients who had not had operation for coarctation. Reasons for the relatively high incidence of this combinatioli of cardiac anomalies, previously cousideretl rare, are discussed with special refereucc to natural selectioll. The prognosis of those itlfauts and children surviving resectiotl of the coarctatioll is poor.
mitral
20:773, 1967.
~JI the aorta experience,
iu infants; I’ediatrb
1962.
J. 11.: ;L revien 26:lOY.
754
22.
23.
24.
2.5.
26.
I’ustkopc
Pi 111.
Hoffman, J. I. E.: Natural historT\. of congenital heart disease: Problems in its assessment xvith special reference to ventricular septal defects, Circulation 37:97, 1968. Tawes, Ii. I>., Aberdeen, E., 12’aterston, D. J., and Bonhan-Carter, Ii. E.: Coarctation of the aorta in infants and childret]--a review of 333 operative cases including 179 infants, in press. Starkey, C. LV. 13.: Surgical experience in the treatment of congenital mitral stenosis alld mitral insufficiency, J. ‘l‘horncic & Cardiovas. Surg. 38:336, 1959. Creech, O., lxdbetter, .\I. K., a~ld I
27.
28.
29.
.iO.
I.orig-term results of valvuloplast)for initl-,!I irlwticiency in childrell, J. ‘I‘horncic & (‘:II diovas. Surg. 53:1, 1967. Flege, J. R., Vlad, P., and Ehrenhalt, J. I..: Congenital mitral incompetence, J. Thurwil, ck Cardiovns. Surg. 53:138, 1967. l.evy, M. J., \rarco, I<. I,., Lillehei, C. \\‘.. .111d Ednxds, J. E.: IIitral insufficiency in inlallt-. children, and adolescent.s, J. Thoracic & (‘,I[-diovas. Surg. 55:434, 1963. Talk, I<. S., Bernhard, \V. F., and Gobs, I<. 1.1.: 5 Irgicd ,lll,ltomy of cotlgenitnl mitral stelllbia. ~irrlliatih,l’(S~Ippl. 2) 36:246. 1967. Bluodwell, I(. I)., H~IIIII;LII. (;. I.., :UI~ Cooler, I ). .I.: Cardiac valve replawtneut in childrc~~. Surgery 63:77, 1968.