ADDRESS: 300 Longwood Avenue, Boston 15, Massachusetts 02115.
Sumpportccl in ptrt by gramits from the National Heart Institutes of the National Institutes of Health.
PEDIATRICS, August 1964
DIAGNOSIS
AND
TREATMENT:
MANAGEMENT
VENTRICULAR
SEPTAL
DEFECT
OF
Alexander S. Nadas, M.D.
S/moron Cardiocascular Unit of the Children’s Hospital %JC(liC(ll Center (111(1 (/id’ Department of Pediatrics, Harvard \Ie(lical Sc/meal, Boston, Mas.saclm ,,sctts
INIHd)DUCTOIIY NOTE: Beginnimig with the jamitmarv, 1964, issue of PEomAinmcs, a brief
(hiscumssiomi of some common problem ooilb regumlarlv appear as the last article preceding
thit’ “Experiemice and Reasomi” section. Usually contributed h a member of the
Eo!i-tonal Board, cacti of these short papers is intendled! to pres(’Iit his cumrrent pruucticc’ in
regard! to diagnosis or therap or 1)0th. Tue Editor ooill ooelcome sumggestions for
olesir-ahile topic’s.
V
ENTBICULAR SEPTAL DEFED, alomie or incombination with other defo)rmities, is the most common congenital cardiac mal-formatiomi imi children. Although in the
neo-natal period solitary ventricular septal
de-fect is overshadowed, in frequency, by
more lethal amid much more complicated
anomiialies, even imi this age period
vemitricu-lar defect, within the framework of these complex (leforniities, is the most common
lesion. Close tO) 20% of our congenital heart
patiemits, comisisting almost exclusively of
imifants amid childiremi, have ventricular defect
as a solitary lesion and at least another 20% have such a defect in association with other cardiac abnormalities (tetrabogy of Falbot, tramisposition, corrected transposition ,
en-docardial cushioii defect, etc.) It is
interest-ing to note that ventrictmlar septal defect is seen much less commonly in centers caring
principally for adults. Figures obtained from stmch imistitutions and from the private offices of cardiologists imidicate a frequency of
soli-tary ventricular defect close to 10%. The reasomi for this discrepancy between adtmlts amid children is not clear; but almost every-one agrees that early fatality is not the
ex-chimsive, or evemi the most important, cause.
Spontamieous closure and change in the clini-cal picture due to) changing liemnodymiamnics
tISO) play important roles.
The statistical imilportamice of a
ventricu-lar (lefect to the pediatrician is onh’ o)ne of
the reasomis why nianagement should he discussed in these pages. The other, amid perhaps more pertinent, purpose of this
presentation is to attempt to clarify the
existing confusion l)’ furnishing some guide lines. These will he laid down with the full kmiowledge that the’ will hot satisfy the
experts \Vh() easily will discover the
imi-herent oversimplification l)ut may l)e O)f
help to the prsctitiomier lost in the fog of conflicting advice emanating from various
vested imiterests.
From the pediatrician’s viewpoint, man-agememit O)f patients with ventricular septal
defect should encompass diagnosis, medical
care, indications for special studies, referral to centers, amid indications for surgery as well as a brief survey of the results of
stir-gery. It should lie imnderstood that only children in whom ventricular defect is the
solitary, or at least dominant, defect will
be discussed.
DIAGNOSIS
Ventricular septal defect seldom is dis-covered within the first day of life. The pressure-volume characteristics of the two ventricles are such that the left-to-right shunt, necessary for the production of a murmur, (loes not usually manifest itself
272 SEPTAL DEFECT
Thims a mimrmtmr discovered at birth is
rarely due to simple ventricular defect. WThen the murmur appears it is usually bud, at least Grade IV out of VI, harsh,
pansystohic, and heard best at the lower left
sternal border, especially at the xinhioid process, and is accompanied by a thrill. The
pulmonic closure, in all hut the largest de-fects with pulmonary vasctmlar obstruction,
is clearly distinguishable from aortic
cbs-tire, i.e., the secomid sound is ovelb split. A low frequency, low imitensitv, mid-diastolic murmur, following the third sound, is ustm-ally heard at the apex. The cardiac impulse
is hyperkinetic in the larger defects and
results in left chest prominence. There is
nothing unusual about the peripheral pulses
and cyanosis is usually absent. If a patient with the cardiac findings described does
have cyanosis it means that either the defect is not uncomplicated (ptmlmonic stenosis, transposition of the great vessels) or that congestive failure is present. Of course, in
later childhood, ventricular defect with
pill-monary vascular obstructive disease may
be accompamiied by cyanosis, but in this in-stance the intemisity of the systolic murmur
is much less. The degree of cardiac enlarge-ment by x-ray is proportionate, on the whole, to the size of the shunt. The electro-cardiographic evidence of right ventricular hypertrophy is proportionate, roughly, to the degree of ptmlmonarv vascular
obstruc-tive disease whereas the left vemitricular
forces reflect the size of the shunt.
Based on the results of the clinical
oh-servations briefly discussed above,
vemitricu-bar defect may be divided into three cate-gories: (1) Small ventricular septal defect
with characteristic at:scultatory findings
and normal x-rays, electrocardiograms, and
a pulmonary flow which is less than twice
the size of the systemic flow. (2) Large
yen-tricular septal defect with large (more than
bar defect with severe ptulmnonarv vascular obstrtmctive disease with cyanosis, low
in-tensity systolic murmur, slight to moderate cardiac enlargement amid pure right yen-tricular hypertrophv b’i electrocardiogram.
MEDICAL MANAGEMENT
Patients belonging to category one are
asymptomatic and neeo! mio special treat-mont except for tile administration of anti-biotics for dental extraction amid at times of febrile tipper respirators’ imifections last-ing more than two to three (lays. It max’ be advisable at the age of 6 \ears, or there-abouts, to refer such a child to a pediatric
cardiologist, hut surely this is not
manda-tory. If he concurs with the clinical
diag-nosis there is no real reason to suggest
car-diac catheterization.
Infants with large ventricular septal de-fect, as evidenced by x-ray and/or electro-cardiogram, often show evidences of con-gestive heart failure between 1 and 6 months of age. As a matter of fact, it is unlikely that congestive failure will
mani-fest itself during childhood if it had not appeared by the age of 1 year. These babies tend to be underdeveloped amid scrawny, their respiratory and pulse rates are high. Cardiac catheterization is indicated in symptomatic infants with large ventricular defect principally in order to exclude a patent ductus arteriosus hut also to assess the possibility of ventricular defect
stir-gery. Physiologic studies in relatively
asymptoniatic youngsters with large o’en-tricular defects may he 1)OStpOfled as far as two to five years of age depending on the individimal circumstances. In the symp-tomatic children , anticoiigestive measures,
through mo)nths amid years, are indicated. Medical management of patients in group
three is mnost difficult. Cardiac
man(la-ARTICLES 273
or surgical treatmnent for these youngsters.
They are usually in their teemis, more than slightly cyanotic, qumite short of breath, and
have a tendemicy to) svncope. The most a
I)e(1i1tricia1i can dlO for such a patient is to give encouragement amid moral support.
They should be led to accept a relatively
restricted, even sedentary, life. Rest periods (luring the clay, early bedtime, a winter vacation in warni climate, air condition in the hot humid summer, avoidance of alti-tiide climates, and avoidance of infections
are the general principals of care. Most
im-portant of all is recognitiomi of the fact that with the scrupulous avoidance of
adventur-ous surgery these patiemits usually live to their 20’s amid 30’s amid may enjoy a
rela-tively useful and satisfyimig existence.
Con-gestive failure, a late manifestation, should
be treated by comiventional methods. I have
110 good experience with anticoagulamits but
phlebotomy, judiciously applied, may give symptomatic relief if the hematocrit is in the tmpper 70’s.
SURGERY
At the lresemit time, patients belonging
to groimp one do not need operative repair.
I know of no good evidence to indicate that,
with the exception of those contracting
subacute bacterial endocarditis, these
pa-tients should have a limited life expectancy. Furthermore, evidence to indicate that the closure of the defect eliminates the danger
of endocarditis is also missing. In addition, although the risks of surgery are quite low,
I canmiot consider them to be as negligible as
those associated with a patent ductus oper-ation.
Patients with large ventricular defect
tmsuallv need surgical help. If the sympto-matic individual cannot be tided over, by medical means, to a time when the defect may be closed with acceptable risks in the particimlar institution, pulmonary artery handing is recommended. This palliative operation diminishes the tendency to con-gestive failure, promotes satisfactory weight gain, and protects the pulmonary arterioles at a risk of 15% in small, very sick infants.
Closure of large ventricular defect with large pulmonary flow is indicated in all
cases at the earliest age compatible with
the lowest mortality. In our institution we like to wait for definitive repair until 4 or 5
years of age, or 30 to 35 lb in weight.
Earlier operation may be perfectly accept-able if the risks may be kept at the 5 to 10% range. The results of surgery are good, the heart usually returns to normal size, after some months and the residual murmur, if the defect was completely closed, should not be louder than Grade II in intensity. The electrocardiogram probably will show complete right bundle branch block.
As disctmssed above, patients in category three should not be subjected to operation.
SUMMARY
Intelligent management of a ventricular septal defect necessitates full analysis of the size of the defect, the shunt across it
and the status of the pulmonary vascuha-ture. Only patients with moderate or large defects with appreciably increased
pulmo-nary blood flow deserve surgery. Those with