MORTALITY FROM CONGENITAL CARDIOVASCULAR DISEASE IN OREGON

(1)

MORTALITY

FROM

CONGENiTAL

CARDIOVASCULAR

DISEASE

IN

OREGON

Victor D. Menashe, M.D., Harold T. Osterud, M.D., and Herbert E. Griswold, M.D.

Crippled Chiklren’s Divi.rion, Department of Public Health and Preventive Medicine, and Divirion of

Cardiovascular Renal Diseases, Department of Medicine, University of Oregon Medical School,

Portland, Oregon

(Received May 16, 1966; revision accepted for publication April 28, 1967.)

This investigation was supported by research grant HE-06336 from the National Heart Institute,

Pub-lic Health Service.

ADDRESS: As above, Portland, Oregon 97201.

PEDIATRICS, Vol. 40, No. 3, Pant I, September 1967

C

ONGENITAL MALFORMATIONS are the

tenth leading cause of death in Ore-gon. In the past decade congenital

anoma-lies changed from the third cause to the

leading cause of death under 1 year of age.

This change was due to the decline of

in-fant deaths from prematurity, asphyxia, and atelectasis rather than from an increase in congenital defects. Congenital malforma-tions account for 15% of all deaths under 1 year of age. More than one half of these are

caused by congenital cardiovascular

dis-ease. Recent advances in the medical and

surgical management of congenital heart

disease have created a greater awareness of these conditions. Early diagnosis and treat-ment of these lesions has been stressed by many physicians,1’2 since surgical treatment of neonates and infants has been reported with increasing frequency and with gratify-ing results.6

The distribution of the mortality from

congenital cardiovascular disease

applica-ble to the entire state of Oregon has not

been previously investigated. These investi-gations are a necessary step for a clearer understanding of the disease and its effect

on the population. This epidemiologic

study of mortality from congenital

cardio-vasular disease includes variations in the

fre-quency of the disease in different geographic areas, sex, race, birth weight and gestation, birth order, age at death, parental age, and others. This investigation circumvents a

ma-jor criticism of many mortality studies by

drawing from the entire population of the

state rather than drawing from a single in-stitution.

MATERIALS AND METHODS

The population under investigation

in-cludes all congenital malformations

re-corded on 81,453 death certificates on file at

the Oregon State Board of Health, for the

period 1957 thru 1961. The mortality data from death certificates were supplemented by review of birth certificates, clinical rec-ords, and autopsy reports. Birth certificates were obtained for all resident deaths,

clin-ical records were reviewed in 97.5%, and

autopsy reports were obtained for 78%. The control population included all resident

births and deaths in Oregon during 1957 to

1961.

The cardiac defects have been classified as single, anatomically grouped, and multi-ple. The categories single and multiple are self explanatory. The anatomically grouped

defects include sets of defects which by

custom are commonly included together

(see Appendix). All cardiovascular defects were further classified as those of the heart alone or as associated with malformations

of other systems.

Congenital heart deaths were contrasted to the total population of the state and to all deaths from congenital malformations

other than congenital cardiovascular

dis-ease.8 Contrasts were made with respect

to age at death, sex, race, population dis-tribution, age of parents at the birth, birth weight and gestation period, birth order, prenatal care, and other factors. Data for

these contrasts were not always complete.

Information was obtained from birth

(2)

TABLE I

CLASSIFICATION OF LIVE BIRTH BY

WEIGHT AND GESTATION

,

Group

Birth Weight (to the last ounce)

Ib, 01

. Gestatzon

Weeks

I 3, 8 or less All gestations

II 3,9to5,8 Lessthan87

III 3, 9 to 5, 8 37 or more IV 5, 9 or more Less than 37

V 5, 9 or more 37 or more

of parents, birth order, and prenatal care

were obtained for 97% of these deaths.

Data on birth weight and gestation was not included on all birth certificates; however, birth weight and gestation were obtained for 386 of the 390 deaths with congenital heart disease that were autopsied. In order to correlate the factors of birth weight and

gestation, groupings according to

Yeni-shalmy9 were utilized

(

Table I).

Differences noted in the various

con-trasting populations were tested at the 5%

significance level using appropriate statisti-cal tests.

RESULTS AND DISCUSSION

Death certificates identified 946 residents who died with congenital malformations

out of 81,453 deaths in Oregon during the

5-year period. Congenital anomalies were

the leading cause of death under 1 year of

age, third in the 1 to 4 age group, fourth for ages 5 to 14 and tenth for all age groups.

Congenital cardiovascular disease was

recorded in 496 resident deaths of which

83% were born during the 5-year study

pe-riod. Deaths with anomalies of the heart

and great vessels were identified in 477 of the 496 deaths and other congenital vascu-lar lesions were noted in 19 deaths. Nine of these were associated with congenital heart

disease, leaving only 10 deaths with

con-genital cardiovascular disease apart from that of the heart and great vessels.

Deaths from congenital cardiovascular

disease, contrasted to deaths from

congeni-tal malformations of other major systems

(

Table II and Fig. 1

)

clearly indicate that congenital cardiovascular disease,

essential-1)7 congenital heart disease, is the major

cause of death from malformations.

Con-genital cardiovascular disease was the

underlying cause of death in 96% of the

deaths recorded as having cardiovascular

anomalies. Malformations of no other

sys-tems approach this degree of lethality. Congenital heart disease takes its toll in infancy. Out of the 496 deaths due to

con-genital cardiovascular disease, 73% died

under 1 year of age (43% in the neonatal period

)

,

8% between 1 and 5 years, 9%

be-tween 6 and 19 years, and 10% over 20

years of age

(

Fig. 2). The distribution of

the age at death for all other congenital

malformations was remarkably similar.

These findings are in keeping with other

studies of mortality from congenital heart

disease.’#{176},” It also emphasizes the need for early diagnosis since 32% of those infants who died had single cardiovascular lesions which could be cured or palliated by surgery.

Deaths from congenital cardiovascular

disease by county of residence ranged

TABLE II

I)EATHS FROM CONGENITAL MALFORMATIONS,

RECORDED ON l)EATII CERTIFICATES, CLASSIFIED

BY SYSTEM AS THE 1NDERLYING CAUSE, OR TIlE

CONTRIBUTORY ASSOCIATED CAUSE OF DEATh,

OREGON RESIDENT., 1957 TII1IU 1961

Major Systems Total .Ifaljor-motions . C n(krlying Cause Total %‘ Contributory Associoied Causet Total % Cardi,,vascular Nervous 1)igestive Genitourinary Musculoskeletal Respiratory Skin Unspecified 496 61 175 164 94 49 10 6 477 188 104 96 7 5 3 6 96 7 60 .59 9 SI 30 100 19 73 71 68 66 4 7 -4 8 40 41 71 49 70

-*The percentages are calculated by dividing the totals of the underlying or contributory cause of death by the total malformations in each system.

(3)

DIGESTIVE

GENITOURINARY

MUSC U LOS KELE TAL

RESPIRATORY Underlying

1

Cause

of

Not underlying] Death

336

CARDIOVASCULAR

NERVOUS

SKIN

O 100 200 300 400 500

FIG. 1. Deaths with congenital malformations, classified by system and b

underlying cause of death as recorded on death certificates, Oregon 1957

thru 1961.

from no deaths in two sparsely populated

rural counties to a high of 18 per 1,000

deaths. The rate for the state was 6.09 per 1,000 deaths. When the state was divided into five areas with comparable climatic and economic conditions, no significant dif-ferences were noted as to the geographic occurrence of death. However, deaths from congenital heart disease, tabulated by pop-ulation density, were significantly different (a

priori

= 100.38;

x2.95

= 14.07). The

number of deaths in the Portland and

Eu-gene metropolitan areas and in rural areas

TABLE III

POPULATION DENSITY OF RESIDENCE OF PERSONS

\ViioDIED FROM CONGENITAL HEART

DISEASE, OREGON 1957 THRU 1961

#{149} r

100,000 and more

Ezpreted

Number of

Deaths

41.17 166 198

50,000-99,999 9tI 6 44

40,009-49,999 3.39 9 16

30,000-39,999 - -

-0,009-9.999 .55 9 1

10,000-19,999 5.63 31 7

3,000- 9,999 4.84 4 3

,3O0- 4,999 4.41 34 l

,4!)’) and less 8.8I 106 139

#{149}Expected number of deaths was based on the percent distribution of inhabitants.

was fewer than expected

(

Table III

)

. The

reasons for this are unknown.

The frequency of deaths from congenital cardiovascular disease was stable from year to year, with no significant differences noted between any of the 5 years

investi-gated (contingency f = 1.715;

9.49). No significant variations were

ob-served for seasons, but the month of

No-vember was consistently higher than other

months for deaths with congenital heart

anomalies and for those dying with other

congenital defects (contingency y = 5.98;

= 3.84). Clinical records revealed that respiratory infections in patients in Oregon,

with congenital heart disease were most

prevalent during November. A review of

the immediate causes of death, as shown

on the death certificates, revealed that 47

of the November deaths were due to

car-diovascular causes and only 4 were the

result of pulmonary complications.

How-ever, neither death certificates nor autopsy reports identified the respiratory infections, as recorded on clinical records, as a

precipi-tating cause of illness. Analyzing these

deaths, by date of birth, revealed no differ-ences by year, season, or month.

Congenital malformations in Oregon are

(4)

#{149}Cardiovascular

All other

malformations

8 -27d

2-7 d

I- 24 hr

under I hr

CARDIOVASCULAR

NERVOUS

DIG ESTIVE

GENITOURINARY

MUSCULOSKELETAL

RESPIRATORY Male

UNSPECIFIED Female

SKIN

O 100 200 300

FIG. 3. Deaths with congenital malformations

clas-sified by sex and system as recorded on death

certificates, Oregon 1957 thru 1961.

ARTICLES

age, and congenital heart disease accounts for 50% of these. Deaths from congenital

malformations occur more frequently in

males than in females

(

Fig. 3). This sex

difference is noted for all systems, with

ex-ception of the musculoskeletal system

and unspecified malformations. Congenital

heart disease, most commonly fatal in

in-fancy, had a male to female ratio of 1.34.

No differences were noted in the life span

between males and females who died of

congenital heart disease.

No racial differences were observed

be-tween white and nonwhite congenital

car-diovascular deaths (contingency y. 0.377; y.95 = 3.84) . Oregon is predominantly

Caucasian, with only 2% of its population

classified as nonwhites (Negroes, American

Indians, and Orientals

)

. Only 23 nonwhites

died of congenital malformations-14 from

congenital cardiovascular disease-too few

in number to draw any conclusions

regard-ing the role of race.

\Vhile 1 out of 15 infants born in the

state was of low birth weight (5 lb, 8 oz or

less

)

,

1 otlt of every 4 infants who died

with congenital heart disease was of low

birth weight. The significance of low

birth weight in 386 deaths with congenital

heart disease was further studied using

Yenishalmy’s groupings of immature

in-fants. This method correlates the factors of

birth weight and gestation. The most

sig-nificant difference occurred in Group III,

the infants of greater than 37 weeks

gesta-tion between 3 Ib, 8 oz and 5 lb, 8 oz

(con-tingency y. = 113.31; y..95 = 3.84). The

percentage of infants in Group III who

died with congenital cardiovascular

dis-ease was approximately four times greater

than the percentage of all live births in

Ore-gon in this group (Fig. 4).

Naeye also noted a high proportion of

low birth weight infants among those who

(lied with congenital heart disease.1#{176} He hy-pothesized that the diminished cell number in the various organs was a probable cause of the low birth weight. Naeye’s findings suggest that the small size of these infants is due to faulty embryogenesis.

12#{176}/oDEATHS WERE OVER 20 YRS. 60-69yr

50-59yr

40-49yr ::.

30-39 yr

20- 29 yr

16% DEATHS WERE BETWEEN I- 19 YRS.

15-l9yr

l0-l4yr

6-9yr

l-5yr

72%DEATHS WERE UNDER IYR. 28d-Iyr

0 50 100 50 200 250

FIG. 2. Deaths with congenital cardiovascular

mal-formations contrasted to deaths from all other

mal-formations by age as recorded on death

certifi-cates, Oregon 1957 thru 1961.

There were no differences in the life

spans between low-birth-weight and

nor-mal-birth-weight infants who died with

congenital heart disease (contingency 12 =

4.40; y2.95 = 7.81). This would appear to

(5)

TABLE IV

BIRTH ORDER OF CONGENITAL HEART DISEASE DEATHS

CONTRASTED TO BIRTH ORDER OF ALL BIRTHS,

OREGON 1957 THRU 1961

Birth Order 2 3 4 5 6 and 7

8+

Total

Percentage Birth, Order of .4!! Oregon Births 24.5 23.2 19.8 13.8 8.3 7.1 3.5 100.0 Congenital [(earl Deaths 86 102 91 53 24 46 14 416 Expected Congenital Heart Dealhs* 102 96 82 57 35 30 15 417 TABLE V

AUTOI#{149}SIED DEAThS WITH CONGENITAL HEART DISEASE, BY SEX, FOR SINGLE, ANATOMICALLY (;ROuI,ED,*

AND MULTIPLE HEART ANOMALIES; CLASSIFIED AS ASSOCIATED OR NOT ASSOCIATED WITIt

MALFORMATIONS OF OTHER SYSTEMS, OREGON RESIDENTS, 1957 THRU 1961

Congenital Heart Anomalies

Single anomalies of the heart

Not associated with malformations of other systems

Associated with malformations of other systems

Subtotal

Percent of Total Number of Persons Male Fern ale

22.6

9.7 32.3

Anatomically grouped anomalies5 of the heart

Associated with Inalformations of other systems

Subtotal

15.6

5.4

21.0

Multiple anomalies of the heart

Associated with malformations of other systems Subtotal 88 38 126 61 2! 82 131 51 182 Total 44 23 67 29 14 43 86 31 117 227 44 1.5 59 32 39 4.5 20 65 163 33.6 13.1 46.7

100 .0 390

* Anatomically grouped anomalies refer to grouped defects commonly accepted as an entity, for example,

tetralogy of Fallot (see Appendix).

* The expected congenital heart deaths were based on

percentage birth order for all births.

The incidence of deaths from congenital cardiovascular disease was studied in twins.

Congenital cardiovascular deaths were

identified for one member in each of 15

pairs of twins. In no case was congenital

cardiovascular disease found in both mem-hers of a pair of twins. Lamy13 found 22 in-stances of multiple births among 1,188 indi-viduals with congenital heart disease, a sta-tistic he interpreted to be no different from the general population.

The distribution of deaths from congeni-tal heart disease, on the basis of birth order, as shown in Table IV, differs significantly from the birth order for all Oregon infants. For example, deaths from congenital heart

disease were noted less frequently among

the first born than expected on the basis of birth order for all Oregon-born infants. There were 86 infants who were first-born

and who died from congenital heart

dis-ease; based on the percentage birth order

in the state, one would expect 102. The

question as to whether they might represent an environmental or a genetic fault has yet

to be answered. The difference might be

the reflection of a higher percentage of first

born in more favorable socioeconomic

(6)

GROUP

LO%

1.9

II

2.8

All single births

a

congenital_vascularSingle births_diseasecardio-with

11.6

Iv

3.5

V.

L::.

.:..:::::: :.:. .::::.::..::. 92.9

77.1

I I I I I I I I I I I I

0 5 10 15 60 70 80 90 100

Percent Births

FIc. 4. Birth weight and gestation period for single births dying with

con-genital cardiovascular disease contrasted to the distribution for all single

births in the state, Oregon 1957 thru 1961.

The risk of death from congenital

car-diovascular disease in infants born during

the 5-year study period was small, with a

ratio of two such deaths to every 1,000 live

births. The mothers of infants who were

born during the 1957 thru 1961 period and

died from congenital cardiovascular

dis-ease during that period were significantly

older when compared to the ages of

moth-ers for all Oregon births in the same time period (a priori y2 = 23.688; 7295 = 11.07).

The same relationship was observed for

fathers, with a mean age of 31.5 years

(me-(han 29.4) for the congenital cardiovascular

group compared to a mean of 28.8 years

(median 28.4) for all fathers in the state.

An increased incidence of cardiovascular

defects in these infants is noted with ad-vancing parental age (Fig. 5). An attempt to correlate these infants with young mothers and older fathers, or the reverse, was

unsuc-cessful, as the number of such marriages

was too few to be reliable. The elevated

ma-ternal age, exclusive of mothers of children

with Down’s syndrome differs from the

stu(lies of Polanil2 and Lamy. It must be

stressed, again, that their studies involved

living children; therefore, the population

of their study is not entirely comparable to this mortality study.

The high incidence of congenital heart

disease in mongolism is well-known.1 In

this series 43 mongoloids died, 33 from

congenital heart disease. The mean age of

mothers of mongoloid children dying of

congenital cardiovascular disease was 30.7 years, significantly greater than the mean age of all mothers in the state. Mothers of infants dying with congenital heart disease, excluding mongoloids, were still

signifi-cantly older compared to all mothers in

the state (a priori 72 = 11.254; 2.95 =

11.07).

During the study period, there were six

deaths with congenital cardiovascular dis-ease per 1,000 deaths in the state; of these, 1.5 per 1,000 had single heart anomalies, 1.0 per 1,000 had grouped anomalies, and 2.2 per 1,000 had multiple anomalies (Table V

and Fig. 6). The cardiovascular defects

(7)

Congenital heart Anomaly

Occnrring as Primary Defect

underlying death Occurring

as a Co-existant Defectf Total Defecte Total Deaths Single or Grouped5 i!ultiple

\entricular septal defect

Patent ductus arteriosus

Transposition of great arteries5 Tetralogy of FaIlot*

Coarctation of aorta Hypoplastic left heart5 Atrial septal defect Tricuspid atresia5 Cor triloculare5 Atrioventricular canal5 Pulmonary stenosis Ilypoplastic aorta Truncus arteriosus5 Fibroelastosis

Hypoplastic left ventricle Aortic stensosis

Pulmonary artery stensosis

Anomalous pulmonary veins5

Bicuspid aortic valve

Aortico-pulmonary window Ebstein’s

Sinus of valsalva, rupture

Anomalous systemic veins5

Anomalous pulmonary artery, origin aorta

Mitral stenosis

I)eformity of coronary artery

Hypoplastic right ventricle Dextrocardia, situs inversus Hypertrophy right ventricle Cor hiloculare 68 39 38 36 32 28 22 17 17 15 13 10 8 8 7 7 4 4 4 3 3 3 2 2 0 0 0 0 0 0 36 26 9 25 11 22 21 4 5 8 8 1 5 6 0 4 I 2 4 2 3 3 2 0 0 0 0 0 0 0 32 13 29 11 21 6 13 12 7 5 9 3 2 7 3 3 2 0 0 0 0 2 0 0 0 0 0 0 52 75 5 1 8 I 71 I 2 0 32 2 4 0 1 10 16 2 7 0 0 0 1 1 11 5 3 3 0 1 120 114 43 37 40 29 93 18 19 15 45 12 12 8 8 17 20 6 11 3 3 3 3 3 11 5 3 3 2 TABLE VI

390 DEATHS WITH CONGENITAL HEART DISEASE CLASSIFYING TIlE HEART DEFECTS AS TIlE PRIMARY

UNDERLYING CAUSE OF DEATH OR AS CO-EXISTING DEFECTS WHICH MAY OR MAY NOT HAVE

CONTRIBUTED TO DEATH, OREGON 1957 THRU 1961

Total 390 208 182 317 707

* Anatomically grouped anomalies refer to grouped defects commonly accepted as an entity.

t Co-existing defects may have contributed to death or may be simply associated with the underlying cause.

Deaths with malformations of the

car-diovascular system alone or when

associ-ated with malformations of other systems are contrasted by sex for the three groups

(Fig. 6). Congenital heart anomalies,

as-sociated with other malformations,

oc-curred significantly more often for males

than for females (contingency 2 = 8.45;

yI.95 = 3.84). Deaths with multiple heart

anomalies were twice as frequent for males

as for females (contingency 2 = 12.102;

y2.95 = 5.99). The average age at death

for individuals dying with single heart de-fects was 10 years (range: birth to 59 years)

contrasted to 3 years (range: birth to 41

years) for grouped heart defects and 2

(8)

00

50

I0

5

0

‘a ‘a

‘a _Births

WIth congenItal cord,ovosculor

disease

I I I I I I

--F

::L45b0c WIth Malformations

Not rother Systems

II

Ir

.lJa5SOC. WIthj

‘a

50

‘a

0

ARTICLES

birth weight for either sex were noted for

the three groups (contingency 72 0.07;

j95 = 5.99 ). The age of parents did not

appear to he a factor between the three

groups as no differences were noted. Individuals with single heart lesions lived significantly longer than those with ana-tomically grouped or multiple

cardiovas-cular lesions (contingency 2 99.00;

y295 = 3.84

)

. Infants with single heart

le-sions and no other anomalies lived to a

mean age of 12 years, contrasted to 53

years for individuals with single

cardiovas-cular defects associated with malformations

of other systems. In contrast, infants with

anatomically grouped or multiple

cardio-vascular defects lived to a mean age of 2%

years with no differences noted for deaths

from defects of the cardiovascular system

alone or when associated with

malforma-tions of other systems.#{176}

No particular patterns of malformations

associated with congenital cardiovascular

disease were noted. Malformations of the

digestive and genitourinary systems

ac-counted for one half of all malformations

associated with congenital cardiovascular

disease (25% each). The nervous system

was involved in 22% of the deaths,

muscu-loskeletal 12%, respiratory 6%, and all

others 10%. Lamy13 found 18% of his index

cases to have associated malformations,

whereas, approximately one of three indi-viduals dying with congenital heart disease in this series had malformations of other systems.

Congenital cardiovascular disease was

recorded as the underlying cause of death

in 477 out of the 496 cases in which

con-genital heart anomalies were identified. Specific cardiovascular lesions were iden-tified and ranked for 390 deaths with con-genital heart disease (Table VI).

Ventricu-lar septal defects occurred most often as

the primary defect underlying death. Of the

This study deais only with mortality. A

mor-bidity study of over 4,000 persons from the

con-genital heart clinic, Crippled Children’s Division,

University of Oregon Medical School, is currently

underway.

10-14 15-19 20-24 25-29 30-34 35-39 40-44 45-49

Age of Parents

FIG. 5. Frequency of congenital heart disease by

parental age at time of birth, classified by rates

per 1,000 births, Oregon 1957 thru 1961.

68 deaths with ventricular septal defects, 36 were single lesions; 21 were under 1 year of age and only one of these had surgical

in-tervention. Patent ductus arteriosus, the

second most frequently identified anomaly with 39 deaths, was identified as a single lesion in 26 deaths; 22 were under 1 year of

SINGLE GROUPED MULTIPLE

ANOMALIES ANOMALIES ANOMALIES

FIG. 6. Deaths with congenital heart disease

clas-sified as single, grouped, or multiple anomalies

and as associated or not associated with

malforma-tions of other systems are contrasted by sex, Oregon

(9)

age and only one had surgical correction.f

Single heart lesions accounted for 32% of

the 390 deaths compared to 68% for the

ana-tomically grouped and multiple heart

de-fects. Deaths from single heart defects are still too high, considering that surgery offers

the most beneficial treatment for this

group.

COMMENT

This study underestimates mortality from

congenital heart disease. For example,

6.2% of all Oregon births, over 2,200 per

year, are premature (5 lb, 8 oz or less ) .

Ap-proximately 18% of these 2,200 premature

births (396) were recorded on death

certificates as “death due to prematurity”. Unfortunately, only 20% were autopsied4

Undoubtedly, some of the unautopsied

deaths had unrecognized congenital heart

disease.

It is difficult to compare epidemiologic

studies of this nature as methods of data

collecting differ, diagnostic variations

oc-cur, and differences in coding or in

certification are noted. However, it is

rele-vant to compare some of these data with

that in the literature.

I. T. T. Higgins,15 in his excellent review, states that less than half the cases of

con-genital heart disease are diagnosed at birth.

In this study, only one out of four infants who died of congenital heart disease were

recognized at birth and recorded on the

birth certificate, reaffirming the difficulty of birth certificate studies and the underesti-mation of congenital heart disease from this source.

In classifying abnormalities of the hearts of adults dying of heart disease, the possi-bility of congenital defects is infrequently

considered. It has become apparent from

discussions with our surgeons that many in-dividuals with valvular heart disease,

pre-viously classified as rheumatic, in truth

This patient died 34 hours postoperatively

from a thrombosis of the right common carotid

artery.

t

Vital Statistics Section, Oregon State Board

of Health.

have congenital heart disease. Many

exam-ples of this could be cited but the most

striking is that of calcific aortic stenosis, previously almost entirely considered to be due to rheumatic heart disease; but, when it occurs as a single lesion, it is most often the result of congenital aortic stenosis. This calcification, as discussed by Edwards,16

probably occurs from increased stress on a

bicuspid aortic valve, resulting in either aortic stenosis, aortic insufficiency, or both. Deaths from “mild” congenital heart dis-ease are poorly defined. This might be an-other reason for underestimating mortality from this condition. It is common clinical opinion that mild lesions are not lethal. However, Bloomfield,17 in his recent study of the natural history of ventricular septal defects, states, “It is commonly assumed that patients with a small defect, maladie de Roger, have a normal life expectancy,

barring infective endocarditis. For two rca-sons this is probably not true : ( 1) As

pre-viously reported, the author has collected nine cases of minor defects of the ventricu-lar septum in three living and six necrop-sied patients in whom there was evidence of left ventricular myopathic change. (2)

The patient with a small defect who

sur-vives beyond the age of 50 uncommonly

presents in congestive heart failure. This suggests that the trivial defect of youth be-gins to take its toll as the heart ages or in the presence of any unrelated condition that compromises cardiac function, e.g., hy-pertension or coronary artery disease.” The

“benignity” of “mild” congenital heart

dis-ease, therefore, needs further study and

definition.

Sudden, unexpected death due to

con-genital heart disease was rarely encoun-tered. Only 7 persons from this series of 496 deaths were not under the care of a

physi-cian at time of death. This would imply

that most cases were recognized and

mani-fested cardiac symptoms prior to death. Of

these seven, three deaths were from

(10)

only 25% of the 358 infants who died of

this cause during the study period. This

may be indicative of the difficulty in early diagnosis of this condition.

If any significant reduction in mortality from congenital heart disease is to be made, the reduction will come primarily from sal-vage of neonates and infants under 1 year

of age. The identification of 41 infants

dying under 1 year of age from

uncompli-cated ventricular septal defects or patent ductus arteriosus, without surgery, clearly illustrates the importance of early diagnosis and early definitive care. It is important that physicians be aware of the remarkable progress of the last decade in the diagnosis and care of infants with congenital heart disease as well as other congenital anoma-lies.

SUMMARY

Over one half of the deaths from all con-genital malformations were due to congeni-tal cardiovascular disease. There were 496 individuals who died of congenital cardio-vascular disease in Oregon during 1957 thru

1961, and three fourths of the deaths

oc-curred in infants under 1 year of age.

More males were affected by congenital cardiovascular disease than females, but, when congenital cardiovascular disease was present, the length of survival, as measured

by life span, did not differ by sex.

One out of every four infants dying of

congenital cardiovascular disease was of

low birth weight; of these, 60% were over

37 weeks’ gestation. However, there was no

difference in the life span of low birth

weight and normal birth weight infants

who died with congenital cardiovascular

disease. This would imply that the

sig-nificant factor of death in these infants

was the cardiac malformation rather than

the low birth weight. One out of every

three infants who died with congenital

car-diovascular disease had malformations of

other systems.

Fewer deaths than expected were

iden-tified among first born. Fetal deaths were

noted more frequently in the population of

mothers of children with congenital heart disease than in the overall population. The parental age in this group was significantly

higher than in the general population and

death rates of infants with congenital car-diovascular disease increased with advanc-ing parental age. Thirty-two percent of the infants who died with congenital cardiovas-cular disease had single lesions.

Early diagnosis and treatment is to be

stressed if mortality is to be reduced.

REFERENCES

1. McNamara, D. C. : Acyanotic congenital heart

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APPENDIX

ANATOMICALLY GROUPED ANOMALIES OF THE HEART

Tetralogy ofFallot

Tricuspid atresia

Hypoplastic leftheart syndrome

Atrioventricular canal defects (any combination)

Truncus arteriosus

Transposition of the great arteries

Ventricular septal defect Infundibular stenosis

Pulmonary valvular stenosis

Dextroposition aorta

Tricuspid atresia Ventricular septal defect

(Hypoplastic pulmonary artery)

Mitral valve atresia

Hypoplasia of left ventricle Hypoplasia of ascending aorta

Ostium primum Atrial septal defect Ventricular septal defect Cleft mitral valve Cleft tricuspid valve

Common arterial trunk Ventricular septal defect

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1967;40;334

Pediatrics

Victor D. Menashe, Harold T. Osterud and Herbert E. Griswold

OREGON