Pulmonary Stenosis: Cyanosis, lnteratrial

Communication and Inadequate Right Ventricular

Distensibility Following Pulmonary Valvotomy

CARLOS M. de CASTRO, Lt Cal, MC Three patients with persistence of clinical venous-arterial admixture
WILLIAM P. NELSON, Lt Cal, after successful valvotomy for severe pulmonary valvular stenosis
MC, FACC with intact ventricular septum were studied by serial cardiac cath-
ROBERT C. JONES, Cal, MC, FACC eterization. Hemodynamic data after valvotomy documented the pres-
ROBERT J. HALL, Cal, MC, FACC
ence of atrial venous-arterial admixture in the absence of pulmonary
ALAN R. HOPEMAN, Col, MC
hypertension, pulmonary stenosis or tricuspid stenosis in all 3 pa-
EDWARD J. JAHNKE, MD
tients. Inadequate right ventricular distensibility was implicated as
Washington, D. C. the cause of the atrial venous-arterial admixture. The secundum
atrial septal defect was surgically closed to eliminate the potential
hazards of such interatrial communications with significant venous-
arterial admixture. After closure, the inadequately compliant right
ventricle accommodated itself to the reception of the total systemic
venous return without residual evidence of right atrial hypertension.

Right ventricular performance after adequate surgical relief of

isolated pulmonary valvular stenosis may remain compromised
by muscular hypertrophy and fibrosis.l,z Residual right ven-
tricular hypertrophy may be manifested by infundibular pul-
monary stenosis or inadequate distensibility of the right ven-
tricle, or both.2-4 Lack of distensibility of the previously ob-
structed right ventricle has been reported to result in inadequate
right ventricular diastolic filling with persistence of venoarterial
shunting in the presence of an interatrial communication, either
a patent foramen ovale or an atria1 septal defect.2s3,5-7
Three cases are presented that illustrate the persistence of
right atria1 hypertension and shunt reversal with clinical cyano-
sis through atria1 septal defects after adequate surgical relief of
pulmonary valvular stenosis with intact ventricular septum dur-
ing the second year of life. These cases demonstrate the need to
close an interatrial communication associated with significant
isolated pulmonary valvular stenosis. If technically possible,
closure should be performed at the time of the pulmonary valvot-
omy or at a subsequent operation when the size of the child per-
mits safer utilization of cardiopulmonary bypass.
Closure of the atria1 septal defect in these 3 patients not only
From the Cardiology and Thoracic Surgery resulted in the elimination of the clinical cyanosis but also re-
Services, Walter Reed General Hospital, lieved the right atria1 hypertension. The latter, finding is note-
Washington, D. C. Manuscript received worthy since the impairment of right ventricular distensibility
June 18, 1969, accepted August 22, 1969. in these patients was previously believed to be secondary to myo-
Address for reprints: Carlos M. de Cas-
tro, Lt Cal, MC, Cardiology Service, Walter
cardial fibrosis?** associated with the right ventricular hyper-
Reed General Hospitgl, Washington, D. C. trophy ; thus persistent right ventricular failure with increased
20012. central venous pressure would have been expected.

540 The American Journal of CARDIOLOGY

 PULMONARY STENOSIS

TABLE I
Cardiac Catheterization Data in Case 1

1962 January 1964 June 1964

02 02
Pressures Sat Pressures Sat Pressures
Site (mm Hg) (o/o) (mm Hg) (o/o) (mm Hg)
PA lo-14/? 1817 65 23112
0.7VPC .‘.
step-up
“2 “2 RV 20-24/? ... 3514-10 60 27/10
Figure 1. Case 1. Electrocardiograms (leads II and V,) show- RA RA and LA . . . a 11; 57 10
ing diminished P wave amplitude after closure of the atrial with equal v8;m8
septal defect. A, January 2, 1964; B, July 31, 1964. pressures;
large a
wave
svc ... ... ... 54 ...
Case Reports IVC ... ... ... 64 . . .
LA ... 82 all; 80 ...
Case 1: This young girl had been cyanotic since
v8;m8
birth. In March 1953 at age 16 months she underwent PV _. . 98 . . .
. . . 96
successful transventricular pulmonary valvotomy for
relief of the severe pulmonary valvular stenosis (Chil- a = a wave; IVC = inferior vena cava; LA = left atrium;
dren’s Memorial Hospital, Chicago, Ill.). Because of LV = left ventricle; m = mean; O2 Sat = oxygen saturation;
the persistence of mild cyanosis and the presence of a PA = pulmonary artery; PV = pulmonary vein; RA = right
continuous murmur suggestive of a patent ductus atrium; RV = right ventricle; SVC = superior vena cava;
arteriosus, cardiac catheterization was performed in v = v wave; VPC = 0, volumes percent.
1962 at age 10 years (Kansas Medical Center). Hemo-
dynamic data (Table I) revealed no significant pul- revealeu absence of cyanosis, regression of the large a
monary outflow obstructive gradient; a small oxygen waves in the jugular venous pulse to normal and loss
saturation step-up in the pulmqnary artery secondary of the right atria1 enlargement noted in the preopera-
to the patent ductus arteriosus; and an interatrial tive electrocardiogram (Fig. 1). A mild pulmonic ejec-
communication, demonstrated by catheter passage, with tion systolic murmur and a low-pitched diastolic mur-
right to left shunting identified by oxygen saturation mur of pulmonary valvular insufficiency persist on clin-
measurements and indicator-dilution studies. Cardiac ical examination.
catheterization was repeated in January 1964 with sim-
ilar hemodynamic findings (Table I). Angiocardiogra- Case 2: This young girl, cyanotic from birth, un-
phy revealed an adequately sized right ventricle. She derwent cardiac catheterization at age 4 months in
was operated on in June 1964 at age 12 with closure of September 1956 with inconclusive findings (Table II).
a 2 cm secundum atria1 septal defect and ligation of a Repeat catheterization and selective right ventricular
small patent ductus arteriosus under cardiopulmonary angiography (Table II) in June 1958 established the
bypass. Subsequent clinical follow-up study at age 16 diagnosis of severe pulmonary valvular stenosis with

TABLE II
Cardiac Catherterization Data in Case 2

September 1956 June 1958 November 1959 June 1966 April 1967

02 02 02 02 02
Pressures Sat Pressures Sat Pressures Sat Pressures Sat Pressures Sat
Site (mm Hg) (%I (mm Hg) 1%) (mm Hg) (%I (mm Hg) (%I (mm Hg) (%I

PA ... ... ... ... 1617 79 22110 75 1816 . . .

Outflow 76 74
RV lob;;-10 -46’ 8;/o -4-i 17;2-7 66 22jiO ... 20;;~9 :: :
a 9; a 15; a 9;
RA (m 10) 44 ... 39
v5;m6 ” v 10
56
v5;m7 ...
WC ... 33 ... 36 ... 63 ... 57 ... ...
IVC ... 43 ... 34 ... ... ... 51 ... ...
a 5; a 12; a 8;
LA (m10) .. . ... ... 74
v8;m4 *-’
v5;m4 74 v 12
LV 98/O-10 63 ... .,. 70/O-5 80 70/o-10 74 120/O-5 85

Abbreviations as in Table I_

VOLUME 26, NOVEMBER 1970 541

DE CASTRO ET AL.

intact ventricular septum. Shortly thereafter, trans-

arterial pulmonary valvotomy was successfully per-
formed under direct vision utilizing inflow occlusion
and hypothermia. Because of the persistence of much
milder cyanosis and the presence of a continuous mur-
mur consistent with patent ductus arteriosus, repeat
hemodynamic assessment was performed in November
1959 when the child was 31/, years old (Table II). No
right ventricular outlet gradient was identified. A small
oxygen saturation step-up in the pulmonary artery was
consistent with the clinical diagnosis of patent ductus
arteriosus. An atria1 septal defect was identified, with
right to left shunting shown by oxygen saturation mea-
surements in the left ventricle and indicator-dilution
curves. Corrective surgery was discussed but not rec-
Figure 2. Case 2. A, preoperative electrocardiogram (Oc- ommended. The patient again underwent catheteriza-
tober 14, 1966) demonstrating right atrial enlargement in tion in June 1966 at age 10 with findings similar to
leads II and VI. B, normal P wave after surgery (July 21, those of the 1959 study (Table II). Selective cineangi-
1967). ography revealed pulmonary valvular insufficiency and
an adequately sized right ventricle without tricuspid in-
sufficiency. A small patent ductus arteriosus was surgi-
cally closed in August 1966 without subsequent change
in the cyanosis or right atria1 hypertension. Catheteri-
zation findings in April 1967 were unchanged except
for the loss of the oxygen saturation step-up in the
pulmonary artery (Table II). A few days later, a
secundum atria1 septal defect was closed under cardio-
pulmonary bypass. The patient has had a good post-
operative course, with absence of cyanosis, normal
jugular venous pulse and loss of electrocardiographic
evidence of right atria1 enlargement at subsequent eval-
uation at age 12 (Fig. 2).
Case 3: Because of cyanosis from birth, this young
girl underwent cardiac catheterization in February
1965 at age 11/2 years (Table III). A diagnosis of
severe pulmonary stenosis with intact ventricular sep-
tum and an interatrial communication was established.
Transventricular pulmonary valvotomy was success-
fully performed (Cardinal Glennon Memorial Hospital
for Children, St. Louis, MO.). Because of the persistence
of mild cyanosis aggravated by exercise, slight club-
bing, increased jugular venous pulse with prominent a
waves and electrocardiographic evidence of right atria1
Figure 3. Case 3. Cardiogreens indicator-dilution curve
enlargement, repeat cardiac catheterization was per-
demonstrating right to left shunting through the atrial septal
formed in April 1968 (Table III). No significant right
defect.
ventricular outflow systolic gradient was observed. An

TABLE III
Cardiac Catheterization Data in Case 3

February 25,1965 April 16,1968

02 02
Pressure Sat Pressure Sat
Site (mm Hg) (%I (mm Hg) (%I

PA 18/? 2317 73
RV 187/12-21 ‘67 2915-8 ...
a 24; a io;
RA 67 73
v 15: m 14.5 v8;m6
a 20; a 7;
LA 74 91
v 15; m 14 v8
Figure 4. Case 3. Electrocardiograms comparing P waves LV ... ... 10817 91
in leads II and VI obtained before (A, April 15, 1968) and
after (B, June 10, 1968) cardiac surgery. Abbreviations as in Table I.

542 The American Journal of CARDIOLOGY

 PULMONARY STENOSIS

atria1 septal defect was identified by catheter passage, right atria1 hypertension after closure of the atria1
with right to left shunt demonstrated by indicator-dilu- septal defect remains unexplained, it is evident
tion curves (Fig. 3). Pulmonary valvular insufficiency that the right ventricle was capable of successfully
was diagnosed after pulmonary arterial and right ven- accommodating itself to the total systemic venous
tricular diastolic pressures were determined to be iden-
return in each of these cases. Indeed, under this
tical. In May 1968 the patient underwent successful
closure of a 14 mm secundum atria1 septal defect under
stress, the right ventricle adjusted to this load at a
cardiopulmonary bypass. At age 6 she is no longer more normal filling pressure. Impairment of right
cyanotic, and the jugular venous pulse is normal with ventricular distensibility solely as a consequence of
loss of the electrocardiographic evidence of right atria1 myocardial fibrosis in these patients must be ques-
enlargement (Fig. 4). On auscultation, a pulmonary tioned. The loss of right atria1 hypertension pro-
ejection systolic murmur is present with a low-pitched vides evidence that inadequate right ventricular
diastolic murmur of pulmonary valvular insufficiency. distensibility is the major consequence of a “mus-
cle-bound” right ventricle rather than a fibrotic
Discussion myocardium. It is apparent that the right ventricu-
The association of shunt reversal and cyanosis lar restrictive defect may persist despite early sur-
with an atria1 septal defect after adequate surgical gery (during the second year of life) for the relief
valvotomy for severe pulmonary valvular stenosis of severe isolated pulmonary valvular stenosis.
with intact ventricular septum was observed in 3 These 3 case histories emphasize the importance
patients. Serial hemodynamic measurements sub- of surgical closure of an interatrial communication
stantiated the adequacy of the pulmonary valvot- (either patent foramen ovale or atria1 septal de-
omy and the absence of pulmonary hypertension or fect) associated with significant pulmonary steno-
tricuspid stenosis to ’ explain the persistence of sis with an intact ventricular septum. The poten-
venous-arterial admixture through the atria1 sep- tial hazards of atria1 right to left shunting such as
tal defect. The pulmonary valvulai insufficiency paradoxical embolization and secondary polycy-
noted in all 3 patients could not have contributed themia can be eliminated. The right ventricle can
significantly to the atria1 shunt reversal since there accommodate itself to the total systemic venous re-
was adequate right ventricular function after clo- turn without any evidence of right atria1 hyperten-
sure of the atria1 septal defect with normalization sion. If closure of the interatrial communication at
of right ventricular filling pressure. the time of initial surgery for severe pulmonary
In the presence of an atria1 communication, in- stenosis is not technically feasible, a second opera-
adequate distensibility of the right ventricle is pre- tion under cardiopulmonary bypass for correction
sumed to be the cause of continued right atria1 hy- of the atria1 defect with shunt reversal due to right
pertension and venoarterial shunting after ade- ventricular malfunction is both justified and de-
quate pulmonary valvotomy. Although the relief of sirable.

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