Association of pulmonary valvular stenosis and muscular ventricular septal defect

Case Reports Association

of Pulmonary

and Muscular Report RICHARD

D

Ventricular

ERSONS

\ ears age

having

lesions

rarely

SAUTTER,

tetralogy survive

In 1964, 62 years,

MD

found

only

viving

beyond

the age

reported

survivor

ously The ported though

27

upon

, DEAN A EMANUEL,

not

a survival patients

The

oldest

was 69 years m

the

a tetralogy fulfill

all

of

to sur-

prevl-

old s

case

to

be

of Fallot, the

real-

anatomic

criteria CASE REPORT The patient was first seen at age 63 years and finally at age 75 She was admitted to St Joseph’s Hospital on Ott 29, 1952, and agam on Ott 29, 1953, because of weakness and tarry stools She also complained of shortness of breath with exertion and stated that she had been cyanotic her entire hfe She had been told m early life that she had some form of congenital heart disease She felt, however, that her heart condltlon had been better m recent years than it was m her childhood She also stated that she had a nephew who was a blue baby On physzcal e\anunat2on she stood 4 ft 11 m m height and weighed 89 lb The blood pressure was 136/80 and her pulse was 76 at rest There Mas cvanosls of her nail-beds, nose, hps and mucous membranes The distal phalanges of the fingers and toe3 showed changes consistent with pulmonary osteoarthropathy There was a grade 5 pansvstohc murmur transmitted over the entire precordmm but heard best over the left sternal border and pulmonary area A thrill was present over the pulmonary area and over the left sternal border There was no dlas* From the Marshfield VOLUME

16,

NOVEMBER

1365

and

KARL

H

DOEGE,

MD

tolic murmur The lungs were clear to auscultatlon and percussIon Except for some mild eplgastllc tenderness, the remainder of the phvslcal exammatlon was normal The hemoglobin on the first admlsslon, even tho Igh the patlent had been actlvelv bleeding \Ids 16 4 gm /lOO ml with a red blood cell mass of 5,600 000 cells/‘cu mm Her hemoglobm on the second admlsslon, when again she was actively bleeding, was 18 gm with a red blood cell mass of 5,2OO,OOO/cu mm A radlographlc study of the upper gastromtestmal tract on the first admIssIon confirmed the The ulcer healed dlagnosls of a duodenal ulcer after 15 days of medlcal management on the first admlsslon, and required only SIX days of therapy before discharge on the second admIssIon She was seen as an outpatzent at the Marahfield Clmlc on June 15, 1962, because of dyspnea preclpltated bv chmbmg stairs Physlcal exammatlon \\as essentially unchanged Hemoglobm at this time \\as 20 8 gm with a hematocrlt of 64 per cent and a red blood cell mass of 6,06O,OOO/cu mm She \~ab treated with a phlebotomy of 250 cc of blood Over the next nme months she was treated nlth multiple phlebotomies of 200 to 500 cc of blood which resulted m conslderable improvement m her elerclse tolerance and dyspnea Her final adrnzsszon to St Joseph’s Hospital has on March 2, 1964, after the sudden onset of paralysis of the left side of her face and left arm and leg, ~41th associated aphasia The paralvsls wab flaccid m the arm and spastic m the leg with a posltlve Babmskl sign Except for the neurologlc findings, the physical exammatlon remained unchanged She became comatose shortly after admission and died March 8 An electrocardiogram on this admission was mterpleted as showmg right ventricular hype] trophy

of 40

the literature

reported of 40

lesion

IS dynamically It does

reported

age

M D

*

Wlsconsm

tetralogy-hke the

reviewing

previously

congenital

or

beyond

Memdok’

and

Defect

Septal

of a Case in a Patient Aged 75 Years Marshfield,

P

Stenosis

Valvular

Clmlc and Marshfield 743

Chnlc Foundation,

Marshfield

LVvls

Sautter,

FIG 1 Photografih of trzcuqld leaflet with se\ ere calclficatlon at postmortem exammatlon

Emanuel

pomtmg to The mclslon was made

rlal~le, arrow

Photograph of thepulmonzc valve showmg narrowed 2 The orifice and typical valvular pulmomc stenosis mclslon was made at postmortem exammation FIG

As on all previous admlsslons, she agam suggestlons of cardiac catheterlzatlon POSTMORTEM

resisted

and Doege

Photograph of bzcuspzd aortzc valve arrow pomtmg FIG 3 The halve leaflet to cusp not dlrlded at postmortem was quite pliable, contammq no calcium

FIG 4 ascendmg

Photograjh

showrng

the w&nor

of

leftventrzcle

Right arrow points to blcuspld Left arrow pomts to muscular \entrlcular valte m which a hemostat has been placed aorta

and

aortlc defect

all

EXAMINATION

The heart was markedly enlarged with hyperThe right trophy of both the right and left ventricles ventricular myocardmm measured 1 5 cm and the

The mommate, left common left, 2 cm m thickness carotid and subclavlan arteries had normal ongms from the aortlc arch The hgamentum arterlosum The mam pulmonary artery measwas not patent ured 3 cm and the ascending aorta 3 5 cm m dlameter THE

AMERICAN

JOURNAL

OF CARDIOLOGY

Pulmonary

Steno?ls

and Ventricular

I ht tricuspid valve was stenotlc with rlgldlty of all three leaflets due to fibrous thickening and a small amount of irregularly distributed calclficatlon (Fig 1 he valve opemng was slit-like and measured 1) 1 5 cm m Its greatest diameter The pulmomc valve was stenotic, the orifice measuring 0 6 cm m its grtattst diameter (Fig 2) Muscular hypertrophy m the right ventricular outflow tract was marked Just beneath the pulmomc valve The aortlc valve was blcuspld, however, the leaflets were quite flexible and showed only mmlmal atherosclerotic thickening (Fig 3) The mitral valve was normally formed and contained only a mmlmal fatty mfiltratlon In the lo\\er muscular portion of the ventricular septum was a defect commumcatmg between the right and left ventncles, It was irregular, ovoid and approximately 8 mm m its greatest diameter (Fig 4) The ventricular septum was markedly thickened There were areas of myocardlal fibrosis wlthm the left

ventricular myocardlum, however, multiple transverse sections of the mam branches of the coronary arteries showed them to be of good caliber without Exammatlon of the lungs was not unusual clots Exammatlon of the brain was not permitted The jinal anatomx dzagnoszs mvolvmg the cardlovascular system was (1) valvular pulmomc stenosis, (2) ventricular septal defect, (3) trlcuspld stenosis, calclfic, and (4) blcuspld aortlc valve COMMENTS

It 1s of interest that this patient, although survlvmg 75 years, at no time had any symptoms to suggest bacterial endocardltls or cerebral abscess, both of which, as reported by Edwards and Fontana, are frequent m this type of cardiac disease The terminal event, almost certainly a cerebrovascular accident, was probably directly related to polycythemla (exammatlon of the brain at autopsy was not allowed) It 1s then of interest to speculate on the patient’s longevity,

VOLUME 16, NOVEMBER 1965

Septal

Defect

745

had the relatlvelb simple caldlac plocedur e of pulmomc \ al\ uloplasty been pel fol med This would have, m all likelihood, lelle\ ed he1 of cy anosls and polvo themla, and thereb, most of her cardiac symptoms It 1s also of interest that this patient had a blcuspld aortlc valve which showed no slqns of calc&icatlon after 75 years Also related to this 1s presence of a calclfic and stenotlc trlcuspld valve which may have been related to an mcrease m the right ventricular pressul e (cardiac catheterization was not permitted) fhJMM4RY

This case demonstrates unusual longevity with congemtal cyanotic heart disease The assoclatlon of a muscular ventricular septal defect with valvular pulmomc stenosis is unusual, for valvular pulmomc stenosis 1s usually associated with an intact ventricular septum It 1s of interest that while there was calclfic stenosis of the tricuspid valve, the blcuspld aortlc valve had no calclficatlon ACKNOWLEDGMENT The author would hke to acknowledge the work of Drs R D Neubecker and C I Bowerman for the postmortem study of the heart REFERENCES 1 MEINDOK, H Longevity m tetraloqy of Fallot Thorax, 19 12, 1964 2 BAIN, G 0 Tetralogy of Fallot, survival to 70th year Report of a case Arch Path , 58 176, 1954 3 EDWARDS, J E and FONTANA, R S Congemtal 4 Reblew of 357 Cases Studled Cardiac Disease Pathologlcally, p 83 Phlladelphla, 1962 W B Saunders Co