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The hemodynamics in ventricular septal defect in childhood
Clinical c ommunic
The hemodynamics ventricular septal
ations
in defect
in childhood
Langford Kidd, M.D., M.R.C.P.Ed.* Vera Rose, B.Sc., M.B., B.S. George Collins, M.B., Ch.B. John Keith, M.D. Toronto, Canada
T
the prognosis in cases of isolated ventricular septal defect, and the indication for surgical closure are subjects for concern and are still matters of considerable doubt. A recent review1 has suggested that the over-all picture is a benign one, that the main hazards to life are congestive heart failure and bacterial endocarditis, and that early surgery to forestall progressive pulmonary vascular obstruction is rarely indicated. This approach has been emphasized by Arcilla,z Lucas,3 and Lynfield4 and their colleagues; on the other hand, Auld5 and Weidman’j and associates have indicated that a progressive increase in pulmonary vascular obstruction will occur early in some cases. A series of 151 infants with ventricular septal defects who were catheterized before their first birthday has recently been reported from this department7: this demonstrated that most of the patients in this age group had large shunts (78 per cent), and serial studies suggested that a proportion of the infants who initially had large shunts developed progressive pulFrom
monary vascular obstruction-the so-called Eisenmenger reaction-early in life. The hemodynamic data from 247 additional patients who were catheterized in this laboratory between the ages of 1 and 16 years have subsequently been analyzed, and the results are reported here. In some places the data from the “infancy” groups are included to obtain an over-all picture of 398 cases from birth to 16 years of age, and an additional 25 cases which have been studied serially are added to the 25 previously reported, A comparison is made of the hemodynamics in the under-2-year and the 2-to-l&year age group. Methods
and
materials
Routine catheterization procedures were used. All but the very young were lightly sedated with chlorpromazine, meperidine, and promethazine (ClVI,).8 Pressures were obtained using Statham P23Db strain gauges at a reference level one third of chest thickness below the sternum, and were recorded on a Sanborn 4-channel direct-writer recorder, and later on an
the Department of Cardiology and the Research Institute, The Hospital for Sick Children, and of Pediatrics, University of Toronto. Toronto, Ontario. Canada. This work was supported by grants from the Department of National Health and Welfare, Ottawa, Heart Foundation. Received for publication Dec. 22, 1964. *Address: The Hospital for Sick Children. 555 University Ave., Toronto 2, Ontario. Canada.
732
the Department and
the Ontario
Hemodynamics
Table
I. Definition
I
Syst./P111m. resistance ratio
<2:1
II III IV
Normal >7:1 5:1-7:l
>2:1 >2:1
<5:1
>2:1 <2:1 Reversed shunt
v VI
septal defect in childhood
733
of hemodynamic grozdpsand their frequency
Pnlm./Syst. $ow ratio
Group
in ventricular
High
PVR
High PVR
Patients 1 to 16 yr.
Patients under 1 yr. (h7idd et al., 1965)
Total
number
of patients 0 to 16 yv.
99 56 29 37 5 21
(29) (29) (9) (0)
123 116 58 66 14 21
247
(151)
398
(24)
(60)
-.
Electronics for Medicine DR8 recorder. Oxygen saturations and contents were assessedusing a Wood oximeter and Van Slyke analysis. Oxygen consumption was calculated in all instances, using a figure of 180 ml. per square meter per minute.g The pulmonary artery was entered in all cases. Total pulmonary vascular resistance was calculated throughout; calculations using pulmonary perfusion pressures in those casesin which it was available showed some alteration in absolute values, but little change in intragroup relationships. In some cases either the systemic arterial pressure or the systemic oxygen saturation was not recorded, or both were not. Cuff pressure S+
(mean : 3)
2D
was used where necessary;
oxygen saturation was assumed to be 95 per cent unless pulmonary hypertension was present, or sedation was noted to be heavy, and systemic desaturation was considered to be a possibility. All patients in whom the hemodynamic data were inadequate or suggested a possible associated lesion were excluded from the study. Data were entered on IBM punch cards, and calculations and analyses were made by the IBM 7094 computer,* using conventional Fick formulae. Results
Data from a total of 247 patients were adjudged to meet satisfactory criteria and were included in the study. The age *At
the Institute
of Computer
Science,
University
of Toronto.
incidence of the catheterized material is illustrated in Fig. 1. Over 30 patients were investigated in each of the second to the sixth years, and thereafter, the number studied in any one-year group fell below 15. Hemodynamic classification. The material was divided into hemodynamic groupings as set out in Table I. In Group I were patients who had a low pulmonary/ systemic flow ratio (FR) (less than 2:l) with normal pulmonary arterial pressures; the small size of the shunt was determined by the small size of the defect. Ninety-nine of the total 247 patients fell into this group. One hundred and twenty two patients had high pulmonary blood flows (FR > 2:1), and these were classified into Group II, those with low pulmonary vascular resistance (PVR), systemic/puhnonary resistance ratio (RR) greater than 7 :l, Group III, those with normal PVR (RR 5:1-7:1), and Group IV, those with higher PVR (RR < 5:l). There were 56, 29, and 37 patients in these groups, respectively. Group V included those patients with stnall shunts (FR < 2:l) due to increasing pulmonary vascular obstruction (5 patients), and Group VI comprised those 21 patients who at the time of study had clear evidence of shunt reversal due to vascular obstruction. Age incidence in hemodynamic groupings. When the cases were set out in the hemodynamic groups by year of age at study, it became obvious that those patients in the second year of life (28 patients) had a distribution almost identical with those in the first year, whereas the distribution in the
Kidd,
734
Am. Heart .I. I~ercmbev, 1965
Rose, Collins, and Keith
V.S.D. 160
HAEWODYNAMICS AGE INCIDENCE
150
140
Age in Years
Fig.
1. Age distribution
V.S.D. TOTAL
NO.
Percent
: 50
of patients
at time
of cardiac
HAEMODYNAMICS 187
211
30 20 10 0 Under
2 years
2-15
years
Fig. 2. Distribution of hemodynamic groups in age periods, under 2 years and from 2 to 1.5 years.
third and each succeeding year was similar. Fig. 2 illustrates the hemodynamic groupings in the two age periods, under 2 years and 2 to 16 years. It will be seen that, whereas 78 per cent of those catheterized early in life had large shunts, only 40 per cent of those catheterized later fell into these groups. The difference is accounted for by the larger number catheterized later who proved to have small defects (Group I) and those who had re-
catheterization.
versed shunts (Group VI), none of whom were found at early catheterization. The differing indications of catheterization preclude interpretation of these data as demonstrating a trend with age. Serial catheterization. A total of 50 patients have now undergone a second catheterization, 1 to 9 years after the first. In 9 patients the second study was made ofter surgical closure of the defect. Fig. 3 illustrates the change in mean pulmonary arterial pressure, together with the change in pulmonary/systemic flow ratio in 41 patients, 36 of whom were first studied when they were under 2 years of age. It was found that there was functional closure of the defect in 6 patients, all of whom had had large shunts at the time of the first study; in an additional 8 patients a fall in flow ratio, together with a fall in pulmonary arterial pressure, suggested that the defect had decreased in size. Four patients showed a drop in PVR, 15 patients had maintained their status, and 8 patients showed an increased resistance. It should be emphasized that in the large majority of those patients studied twice, the hemodynamic changes were slight. Table II presents the detailed hemodynamic data in the 8 children who demonstrated the Eisenmenger reaction. It will be seen that all cases fell into the high-flow groups at the time of the first study (mean pulmonary/systemic flow ratio 4.2:1range 2.1-6.4). The second study was carried out at varying intervals after the first
Hemodynamics
V.S.D.
Change
HAEMODYNAMICS
in Pul./Syst.
Flow
Ratio
Fig. 3. Change in pulmonary/systemic flow ratio plotted against change in mean pulmonary arterial pressure between first and second cardiac catheterizations. The symbols refer to the hemodynamic group into which the case fell at first study. Solid squares, Group I. Open circles, Group II. Open squares, Group III. Open triangles, Group IV. Solid circles, Group V.
(mean 3-l/12 years-range l-3/12 to S-3/12 years), and in each instance the systemic/pulmonary resistance ratio had decreased markedly (PVR increased). These ratios are independent of the calculated oxygen consumption, and derive solely from the measured pressures and oxygen saturations. Of the 9 patients who were restudied after surgical closure of the defect, in 4 the pulmonary arterial pressure had risen above preoperative levels. In one of these patients, it continued to rise, and a third study when the child was 7 years old showed a pulmonary arterial systolic pressure of 130 mm. Hg (systemic pressure 94/S mm. Hg), although the defect had been closed when the child was 25 months old. Discussion
The present report brings to 398 the total number of casesof ventricular septal defect with adequate hemodynamic data reported from this laboratory. It should, of course, be realized that this is a selected group of patients, and is not a typical
in ventricular
septal defect in childhood
73.5
cross section of the total population with ventricular septal defects, in approximately 1,200 of whom a diagnosis has been made clinically in this department; these patients were catheterized because of the severity of the lesion, in order to make a diagnosis or to determine the level of the pulmonary vascular resistance. Furthermore, the indications will vary with age; those with large defects tend to get into difficulty early and are catheterized in infancy, whereas those with smaller defects are regarded with less urgency and are catheterized when surgical closure is proposed at a later age. Conclusions as to the course of the hemodynamic changes, therefore, can only be based on serial study; however, the patients who have undergone two catheterizations are even more “selected” and cannot be considered to be representative of the large body of those with clinically diagnosed ventricular septal defects, the majority of whom have not been catheterized because their defects are thought to be small and hemodynamitally insignificant. A clinical, radiographic, and electrocardiographic survey of the whole group is in progress and will be reported subsequently. However, some conclusions can be drawn from the data of this “catheter laboratory population.” Among the points of interest are the large proportion (78 per cent) of children who had large pulmonary blood flows (FR > 2:l) when catheterized before the age of 2 years (Fig. 2). In the 2-to-16 year age group, this proportion (Group II, III, and IV) was much smaller, and casesof unequivocal shunt reversal (Group VI) were found for the first time. It would seem to be unlikely that these patients were in this state from the time of birth, since (a) no caseshave been recognized in a catheterized series of 187 infants, and (b) those with increased resistance (Group V) at first study have been found to have a drop in resistance with time, and those developing the Eisenmenger reaction (Table II) all had high flows previously. If such “congenital” cases exist, they must be exceedingly rare. Since the changes can occur early in life, it is obvious that any study which includes only catheterization data from children surviving infancy or over 3 years of age
736
Kidd,
Am. Heart I. llecmber, 1965
Rose, Collins, and Keith
Table II. Hemodynamic
data from
serial cardiac catheterization of 8 children who demonstrated I
~
d ge (yr. 1
Patient
02 saturations
1
Surface area
iL/ (sa.M.) )
Pressures
(y;,)
_____-MV
PA
~
5-A
Phosic
(mm. Hg) -__-~--
PA I
I 1
.25
55
.68
61
89 69
9.5 86
1 lo/65
.24 .61
53 47
90 76
98 95
40/15 75/25
23
8/12 6-l/12
.26
69 58
81 60
92 73
65/20
45
.54
100/5.5
70
l/12 l-1/12
.22 .36
58 57
88 78
95 97
49/13 58/25
28 40
l-6/12 3-2/12
.46 .60
50 65
77 73
9.5 90
35/10 80/30
54
t/12 l-10/12
.25
55 70
83
92
66/30
.50
83
95
78/28
4.5 50
3/12 4-10/12
2
4/12 4-7/12
3
4
5 6
MV:
Mean
65/30
42 85
50
18
7
3/12 2-s/1 2
.26 .48
72 58
83 71
89 88
72/30 75/48
48 55
8
3/12 3-9/l:!
.27 .68
64 69
86 84
93 97
60/20 80/38
3.5 57
Mixed
venous
monary/systemic
sample-R.A. flow
ratio.
or
RR:
(SVC
+2
IVC)
( 3 1’ Systemic/pulmonary
PA;
Pulmonary resistance
will obtain a distorted picture of the hemodynamics in this condition. Thus, it has been suggested that low flow-high resistance cases are those in which there is risk from the Eisenmenger reaction, if indeed, it is not “congenital. “1,4~10~11However, it is clear that if these patients had been catheterized for the first time in infancy, they might then have demonstrated a high flow and moderate or low resistance phase. If they are studied for the first time at the age of 4 or 5 years, only the end-stages of the evolution may be seen and unjustified conclusions drawn. This is obviously an important point in a consideration of the management of children with this condition. If progressive pulmonary vascular obstruction can develop early-and we have found cases in which there was increasing
artery.
SA:
Systemic
artery--aorta,
brachial.
or
femoral.
ratio.
PVR by the age of 2 or 3 years-then surgery would seem to be indicated, in these cases, at an early age. Even then, it is possible that this may be carried out too late, since evidence is presented of progressive obstruction after closure of the defect. Surgery may lead to an earlier death than would be the case were the defect left unclosed, since the “Eisenmenger” patient may survive for 10 to 30 years, cyanosed but fairly active. We believe that a close watch should be kept on patients with a ventricular septal defect who have a high pulmonary blood flow in infancy, and serial studies should be made when indicated. Those cases in which the pulmonary vascular obstruction is increasing should be defined, and the patient slated for early operation.
Volume Number
70 6
Hemodynamics
in ventricular
737
septal defect in childhood
progressive pulmonary vascular obstruction Pressures
(mm.
Hg)
__PBF/M.2 (L./min.)
SA
Z’BF
SBF/M.Z (L./min.)
TP VR/M.2 (dynes sec. C?iC6)
TS VR/M.z (dynes sec. cm.-5)
Pul?n./Syst. FR
Sysl./Pz1lm. RR
Phasic
Mean
75/50 110/75
58 90
16.94 4.62
2.75 4.87
197 1,472
1,694 1,478
6.2 .95
8.6 1.0
80/50 100/75
60 83
16.83 5.57
2.65 2.86
109 733
6,110 2,322
6.4 1.9
16.6 3.2
80/45 100/70
55 85
8.14 2.40
3.89 5.56
442 2,331
1,129 1,221
2.1 .43
89/41 104/60
54 7.5
10.60 6.43
2.0 3.05
211 497
2,152 1,965
5.3 2.0
10.2 3.9
90/55 120/80
67 90
5.92 6.34
2.37 4.44
242 680
2,260 1,619
2.5 1.4
9.1 2.4
70,‘42 105/50
51 68
12.04 9.91
2.93 4.66
299 403
1,392 1,166
4.1 2.0
4.7 2.9
90/50 102/52
63 69
18.06 7.39
6.19 4.19
212 595
813 1,318
2.9 1.8
3.8 2.2
70/45 90/54
5.5 67
16.74 9.47
4.33 4.50
167 481
1,015 1,190
3.9 2.1
6.1 2.5
and
SBF:
Pulmonary
and
systemic
blood
flow.
TPVR
and
Summary
(1) Hemodynamic groups are defined, and the frequency in a “catheter laboratory population” of isolated ventricular septal defects is described. The cases of 247 patients 1 to 16 years old are analyzed and considered, together with 151 previously presented cases of patients under 1 year of age. (2) A natural history is suggested; the majority of septal defects are benign; some defects get smaller or close; a few patients develop progressive pulmonary vascular obstruction. (3) The pulmonary vascular obstruction may progress after surgical closure of the defect. (4) No “congenital Eisenmengers” were found. (5) Those who developed progressive pulmonary vascular obstruction had greatly increased pulmonary blood flow early. (6) It is recommended that a careful watch
TSVR:
Total
pulmonarp
and
systemic
2.6 32
vascular resistance. FR: pul-
be kept on “high-flow” patients in the first few years of life, and that they be restudied early to pick out the developing Eisenmenger reaction, with a view to prompt operation. REFERENCES Bloomfield, D. K.: The natural history of ventricular septal defect in patients surviving infancy, Circulation 29:914, 1964. Arcilla, R. A., Agustsson, M. H., Bicoff, J. P., Lynfield, J., Weinberg, M., Fell, E. H., and Gasul, B. M.: Further observations on the natural history of isolated ventricular septal defects in infancy and childhood. Serial cardiac catheterization studies in 75 patients, Circulation 28:560, 1963. Lucas, R. V., Adams, P., Anderson, R. C., Mayne, N. G., Lillehei, C. W., and Varco, R. L. : The natural history of ventricular septal defect. A serial physiologic study, Circulation 24:1372, 1961. Lynfield, J., Gasul, B. M., Arcilla, R., and Luan,
738
Kidd,
Am. Heart I. December, 1965
Rose, Collins, and Keith
L.: The natural history of ventricular septal defects based on serial catheterization studies, Am. J. Med. 30:357, 1961. 5. Auld, P. A. M., Johnson, A. B., Gibbons, J. E., and MacGregor, M.: Changes in pulmonary vascular resistance in infants and children with left-to-right intracardiac shunts, Circulation 27:257, 1963. 6. Weidman, W. H., DuShane, J. W., and Kincaid, 0. W. : Observation concerning progressive pulmonary vascular obstruction in children with ventricular septal defects, AM. HEART J. 65:148, 1963. 7. Kidd, L., Rose, V., Collins, G., and Keith, J.:
8.
9.
10.
11.
Ventricular septal defect in infancy: A haemodynamic study, AM. HEART J. 69:4, 1965. Smith, C., Rose, R. D., and Vlad, P.: Sedation of children for cardiac catheterization with an ataractic mixture, Canad. Anaesthet. Sot. J. 5:35, 1958. Rudolph, A. M., and Cayler, G. G.: Cardiac catheterization in infants and children, Pediat. Clin. North America 5:907, 1958. Nadas, A. S., Rudolph, A. M., and Gross, R. E.: Pulmonary arterial hypertension in congenital heart disease, Circulation 22:1041, 1960. Wood, P.: The Eisenmenger syndrome, Brit. M. J. 2:701 and 75.5, 1958.
The hemodynamics ventricular septal
ations
in defect
in childhood
Langford Kidd, M.D., M.R.C.P.Ed.* Vera Rose, B.Sc., M.B., B.S. George Collins, M.B., Ch.B. John Keith, M.D. Toronto, Canada
T
the prognosis in cases of isolated ventricular septal defect, and the indication for surgical closure are subjects for concern and are still matters of considerable doubt. A recent review1 has suggested that the over-all picture is a benign one, that the main hazards to life are congestive heart failure and bacterial endocarditis, and that early surgery to forestall progressive pulmonary vascular obstruction is rarely indicated. This approach has been emphasized by Arcilla,z Lucas,3 and Lynfield4 and their colleagues; on the other hand, Auld5 and Weidman’j and associates have indicated that a progressive increase in pulmonary vascular obstruction will occur early in some cases. A series of 151 infants with ventricular septal defects who were catheterized before their first birthday has recently been reported from this department7: this demonstrated that most of the patients in this age group had large shunts (78 per cent), and serial studies suggested that a proportion of the infants who initially had large shunts developed progressive pulFrom
monary vascular obstruction-the so-called Eisenmenger reaction-early in life. The hemodynamic data from 247 additional patients who were catheterized in this laboratory between the ages of 1 and 16 years have subsequently been analyzed, and the results are reported here. In some places the data from the “infancy” groups are included to obtain an over-all picture of 398 cases from birth to 16 years of age, and an additional 25 cases which have been studied serially are added to the 25 previously reported, A comparison is made of the hemodynamics in the under-2-year and the 2-to-l&year age group. Methods
and
materials
Routine catheterization procedures were used. All but the very young were lightly sedated with chlorpromazine, meperidine, and promethazine (ClVI,).8 Pressures were obtained using Statham P23Db strain gauges at a reference level one third of chest thickness below the sternum, and were recorded on a Sanborn 4-channel direct-writer recorder, and later on an
the Department of Cardiology and the Research Institute, The Hospital for Sick Children, and of Pediatrics, University of Toronto. Toronto, Ontario. Canada. This work was supported by grants from the Department of National Health and Welfare, Ottawa, Heart Foundation. Received for publication Dec. 22, 1964. *Address: The Hospital for Sick Children. 555 University Ave., Toronto 2, Ontario. Canada.
732
the Department and
the Ontario
Hemodynamics
Table
I. Definition
I
Syst./P111m. resistance ratio
<2:1
II III IV
Normal >7:1 5:1-7:l
>2:1 >2:1
<5:1
>2:1 <2:1 Reversed shunt
v VI
septal defect in childhood
733
of hemodynamic grozdpsand their frequency
Pnlm./Syst. $ow ratio
Group
in ventricular
High
PVR
High PVR
Patients 1 to 16 yr.
Patients under 1 yr. (h7idd et al., 1965)
Total
number
of patients 0 to 16 yv.
99 56 29 37 5 21
(29) (29) (9) (0)
123 116 58 66 14 21
247
(151)
398
(24)
(60)
-.
Electronics for Medicine DR8 recorder. Oxygen saturations and contents were assessedusing a Wood oximeter and Van Slyke analysis. Oxygen consumption was calculated in all instances, using a figure of 180 ml. per square meter per minute.g The pulmonary artery was entered in all cases. Total pulmonary vascular resistance was calculated throughout; calculations using pulmonary perfusion pressures in those casesin which it was available showed some alteration in absolute values, but little change in intragroup relationships. In some cases either the systemic arterial pressure or the systemic oxygen saturation was not recorded, or both were not. Cuff pressure S+
(mean : 3)
2D
was used where necessary;
oxygen saturation was assumed to be 95 per cent unless pulmonary hypertension was present, or sedation was noted to be heavy, and systemic desaturation was considered to be a possibility. All patients in whom the hemodynamic data were inadequate or suggested a possible associated lesion were excluded from the study. Data were entered on IBM punch cards, and calculations and analyses were made by the IBM 7094 computer,* using conventional Fick formulae. Results
Data from a total of 247 patients were adjudged to meet satisfactory criteria and were included in the study. The age *At
the Institute
of Computer
Science,
University
of Toronto.
incidence of the catheterized material is illustrated in Fig. 1. Over 30 patients were investigated in each of the second to the sixth years, and thereafter, the number studied in any one-year group fell below 15. Hemodynamic classification. The material was divided into hemodynamic groupings as set out in Table I. In Group I were patients who had a low pulmonary/ systemic flow ratio (FR) (less than 2:l) with normal pulmonary arterial pressures; the small size of the shunt was determined by the small size of the defect. Ninety-nine of the total 247 patients fell into this group. One hundred and twenty two patients had high pulmonary blood flows (FR > 2:1), and these were classified into Group II, those with low pulmonary vascular resistance (PVR), systemic/puhnonary resistance ratio (RR) greater than 7 :l, Group III, those with normal PVR (RR 5:1-7:1), and Group IV, those with higher PVR (RR < 5:l). There were 56, 29, and 37 patients in these groups, respectively. Group V included those patients with stnall shunts (FR < 2:l) due to increasing pulmonary vascular obstruction (5 patients), and Group VI comprised those 21 patients who at the time of study had clear evidence of shunt reversal due to vascular obstruction. Age incidence in hemodynamic groupings. When the cases were set out in the hemodynamic groups by year of age at study, it became obvious that those patients in the second year of life (28 patients) had a distribution almost identical with those in the first year, whereas the distribution in the
Kidd,
734
Am. Heart .I. I~ercmbev, 1965
Rose, Collins, and Keith
V.S.D. 160
HAEWODYNAMICS AGE INCIDENCE
150
140
Age in Years
Fig.
1. Age distribution
V.S.D. TOTAL
NO.
Percent
: 50
of patients
at time
of cardiac
HAEMODYNAMICS 187
211
30 20 10 0 Under
2 years
2-15
years
Fig. 2. Distribution of hemodynamic groups in age periods, under 2 years and from 2 to 1.5 years.
third and each succeeding year was similar. Fig. 2 illustrates the hemodynamic groupings in the two age periods, under 2 years and 2 to 16 years. It will be seen that, whereas 78 per cent of those catheterized early in life had large shunts, only 40 per cent of those catheterized later fell into these groups. The difference is accounted for by the larger number catheterized later who proved to have small defects (Group I) and those who had re-
catheterization.
versed shunts (Group VI), none of whom were found at early catheterization. The differing indications of catheterization preclude interpretation of these data as demonstrating a trend with age. Serial catheterization. A total of 50 patients have now undergone a second catheterization, 1 to 9 years after the first. In 9 patients the second study was made ofter surgical closure of the defect. Fig. 3 illustrates the change in mean pulmonary arterial pressure, together with the change in pulmonary/systemic flow ratio in 41 patients, 36 of whom were first studied when they were under 2 years of age. It was found that there was functional closure of the defect in 6 patients, all of whom had had large shunts at the time of the first study; in an additional 8 patients a fall in flow ratio, together with a fall in pulmonary arterial pressure, suggested that the defect had decreased in size. Four patients showed a drop in PVR, 15 patients had maintained their status, and 8 patients showed an increased resistance. It should be emphasized that in the large majority of those patients studied twice, the hemodynamic changes were slight. Table II presents the detailed hemodynamic data in the 8 children who demonstrated the Eisenmenger reaction. It will be seen that all cases fell into the high-flow groups at the time of the first study (mean pulmonary/systemic flow ratio 4.2:1range 2.1-6.4). The second study was carried out at varying intervals after the first
Hemodynamics
V.S.D.
Change
HAEMODYNAMICS
in Pul./Syst.
Flow
Ratio
Fig. 3. Change in pulmonary/systemic flow ratio plotted against change in mean pulmonary arterial pressure between first and second cardiac catheterizations. The symbols refer to the hemodynamic group into which the case fell at first study. Solid squares, Group I. Open circles, Group II. Open squares, Group III. Open triangles, Group IV. Solid circles, Group V.
(mean 3-l/12 years-range l-3/12 to S-3/12 years), and in each instance the systemic/pulmonary resistance ratio had decreased markedly (PVR increased). These ratios are independent of the calculated oxygen consumption, and derive solely from the measured pressures and oxygen saturations. Of the 9 patients who were restudied after surgical closure of the defect, in 4 the pulmonary arterial pressure had risen above preoperative levels. In one of these patients, it continued to rise, and a third study when the child was 7 years old showed a pulmonary arterial systolic pressure of 130 mm. Hg (systemic pressure 94/S mm. Hg), although the defect had been closed when the child was 25 months old. Discussion
The present report brings to 398 the total number of casesof ventricular septal defect with adequate hemodynamic data reported from this laboratory. It should, of course, be realized that this is a selected group of patients, and is not a typical
in ventricular
septal defect in childhood
73.5
cross section of the total population with ventricular septal defects, in approximately 1,200 of whom a diagnosis has been made clinically in this department; these patients were catheterized because of the severity of the lesion, in order to make a diagnosis or to determine the level of the pulmonary vascular resistance. Furthermore, the indications will vary with age; those with large defects tend to get into difficulty early and are catheterized in infancy, whereas those with smaller defects are regarded with less urgency and are catheterized when surgical closure is proposed at a later age. Conclusions as to the course of the hemodynamic changes, therefore, can only be based on serial study; however, the patients who have undergone two catheterizations are even more “selected” and cannot be considered to be representative of the large body of those with clinically diagnosed ventricular septal defects, the majority of whom have not been catheterized because their defects are thought to be small and hemodynamitally insignificant. A clinical, radiographic, and electrocardiographic survey of the whole group is in progress and will be reported subsequently. However, some conclusions can be drawn from the data of this “catheter laboratory population.” Among the points of interest are the large proportion (78 per cent) of children who had large pulmonary blood flows (FR > 2:l) when catheterized before the age of 2 years (Fig. 2). In the 2-to-16 year age group, this proportion (Group II, III, and IV) was much smaller, and casesof unequivocal shunt reversal (Group VI) were found for the first time. It would seem to be unlikely that these patients were in this state from the time of birth, since (a) no caseshave been recognized in a catheterized series of 187 infants, and (b) those with increased resistance (Group V) at first study have been found to have a drop in resistance with time, and those developing the Eisenmenger reaction (Table II) all had high flows previously. If such “congenital” cases exist, they must be exceedingly rare. Since the changes can occur early in life, it is obvious that any study which includes only catheterization data from children surviving infancy or over 3 years of age
736
Kidd,
Am. Heart I. llecmber, 1965
Rose, Collins, and Keith
Table II. Hemodynamic
data from
serial cardiac catheterization of 8 children who demonstrated I
~
d ge (yr. 1
Patient
02 saturations
1
Surface area
iL/ (sa.M.) )
Pressures
(y;,)
_____-MV
PA
~
5-A
Phosic
(mm. Hg) -__-~--
PA I
I 1
.25
55
.68
61
89 69
9.5 86
1 lo/65
.24 .61
53 47
90 76
98 95
40/15 75/25
23
8/12 6-l/12
.26
69 58
81 60
92 73
65/20
45
.54
100/5.5
70
l/12 l-1/12
.22 .36
58 57
88 78
95 97
49/13 58/25
28 40
l-6/12 3-2/12
.46 .60
50 65
77 73
9.5 90
35/10 80/30
54
t/12 l-10/12
.25
55 70
83
92
66/30
.50
83
95
78/28
4.5 50
3/12 4-10/12
2
4/12 4-7/12
3
4
5 6
MV:
Mean
65/30
42 85
50
18
7
3/12 2-s/1 2
.26 .48
72 58
83 71
89 88
72/30 75/48
48 55
8
3/12 3-9/l:!
.27 .68
64 69
86 84
93 97
60/20 80/38
3.5 57
Mixed
venous
monary/systemic
sample-R.A. flow
ratio.
or
RR:
(SVC
+2
IVC)
( 3 1’ Systemic/pulmonary
PA;
Pulmonary resistance
will obtain a distorted picture of the hemodynamics in this condition. Thus, it has been suggested that low flow-high resistance cases are those in which there is risk from the Eisenmenger reaction, if indeed, it is not “congenital. “1,4~10~11However, it is clear that if these patients had been catheterized for the first time in infancy, they might then have demonstrated a high flow and moderate or low resistance phase. If they are studied for the first time at the age of 4 or 5 years, only the end-stages of the evolution may be seen and unjustified conclusions drawn. This is obviously an important point in a consideration of the management of children with this condition. If progressive pulmonary vascular obstruction can develop early-and we have found cases in which there was increasing
artery.
SA:
Systemic
artery--aorta,
brachial.
or
femoral.
ratio.
PVR by the age of 2 or 3 years-then surgery would seem to be indicated, in these cases, at an early age. Even then, it is possible that this may be carried out too late, since evidence is presented of progressive obstruction after closure of the defect. Surgery may lead to an earlier death than would be the case were the defect left unclosed, since the “Eisenmenger” patient may survive for 10 to 30 years, cyanosed but fairly active. We believe that a close watch should be kept on patients with a ventricular septal defect who have a high pulmonary blood flow in infancy, and serial studies should be made when indicated. Those cases in which the pulmonary vascular obstruction is increasing should be defined, and the patient slated for early operation.
Volume Number
70 6
Hemodynamics
in ventricular
737
septal defect in childhood
progressive pulmonary vascular obstruction Pressures
(mm.
Hg)
__PBF/M.2 (L./min.)
SA
Z’BF
SBF/M.Z (L./min.)
TP VR/M.2 (dynes sec. C?iC6)
TS VR/M.z (dynes sec. cm.-5)
Pul?n./Syst. FR
Sysl./Pz1lm. RR
Phasic
Mean
75/50 110/75
58 90
16.94 4.62
2.75 4.87
197 1,472
1,694 1,478
6.2 .95
8.6 1.0
80/50 100/75
60 83
16.83 5.57
2.65 2.86
109 733
6,110 2,322
6.4 1.9
16.6 3.2
80/45 100/70
55 85
8.14 2.40
3.89 5.56
442 2,331
1,129 1,221
2.1 .43
89/41 104/60
54 7.5
10.60 6.43
2.0 3.05
211 497
2,152 1,965
5.3 2.0
10.2 3.9
90/55 120/80
67 90
5.92 6.34
2.37 4.44
242 680
2,260 1,619
2.5 1.4
9.1 2.4
70,‘42 105/50
51 68
12.04 9.91
2.93 4.66
299 403
1,392 1,166
4.1 2.0
4.7 2.9
90/50 102/52
63 69
18.06 7.39
6.19 4.19
212 595
813 1,318
2.9 1.8
3.8 2.2
70/45 90/54
5.5 67
16.74 9.47
4.33 4.50
167 481
1,015 1,190
3.9 2.1
6.1 2.5
and
SBF:
Pulmonary
and
systemic
blood
flow.
TPVR
and
Summary
(1) Hemodynamic groups are defined, and the frequency in a “catheter laboratory population” of isolated ventricular septal defects is described. The cases of 247 patients 1 to 16 years old are analyzed and considered, together with 151 previously presented cases of patients under 1 year of age. (2) A natural history is suggested; the majority of septal defects are benign; some defects get smaller or close; a few patients develop progressive pulmonary vascular obstruction. (3) The pulmonary vascular obstruction may progress after surgical closure of the defect. (4) No “congenital Eisenmengers” were found. (5) Those who developed progressive pulmonary vascular obstruction had greatly increased pulmonary blood flow early. (6) It is recommended that a careful watch
TSVR:
Total
pulmonarp
and
systemic
2.6 32
vascular resistance. FR: pul-
be kept on “high-flow” patients in the first few years of life, and that they be restudied early to pick out the developing Eisenmenger reaction, with a view to prompt operation. REFERENCES Bloomfield, D. K.: The natural history of ventricular septal defect in patients surviving infancy, Circulation 29:914, 1964. Arcilla, R. A., Agustsson, M. H., Bicoff, J. P., Lynfield, J., Weinberg, M., Fell, E. H., and Gasul, B. M.: Further observations on the natural history of isolated ventricular septal defects in infancy and childhood. Serial cardiac catheterization studies in 75 patients, Circulation 28:560, 1963. Lucas, R. V., Adams, P., Anderson, R. C., Mayne, N. G., Lillehei, C. W., and Varco, R. L. : The natural history of ventricular septal defect. A serial physiologic study, Circulation 24:1372, 1961. Lynfield, J., Gasul, B. M., Arcilla, R., and Luan,
738
Kidd,
Am. Heart I. December, 1965
Rose, Collins, and Keith
L.: The natural history of ventricular septal defects based on serial catheterization studies, Am. J. Med. 30:357, 1961. 5. Auld, P. A. M., Johnson, A. B., Gibbons, J. E., and MacGregor, M.: Changes in pulmonary vascular resistance in infants and children with left-to-right intracardiac shunts, Circulation 27:257, 1963. 6. Weidman, W. H., DuShane, J. W., and Kincaid, 0. W. : Observation concerning progressive pulmonary vascular obstruction in children with ventricular septal defects, AM. HEART J. 65:148, 1963. 7. Kidd, L., Rose, V., Collins, G., and Keith, J.:
8.
9.
10.
11.
Ventricular septal defect in infancy: A haemodynamic study, AM. HEART J. 69:4, 1965. Smith, C., Rose, R. D., and Vlad, P.: Sedation of children for cardiac catheterization with an ataractic mixture, Canad. Anaesthet. Sot. J. 5:35, 1958. Rudolph, A. M., and Cayler, G. G.: Cardiac catheterization in infants and children, Pediat. Clin. North America 5:907, 1958. Nadas, A. S., Rudolph, A. M., and Gross, R. E.: Pulmonary arterial hypertension in congenital heart disease, Circulation 22:1041, 1960. Wood, P.: The Eisenmenger syndrome, Brit. M. J. 2:701 and 75.5, 1958.