Pediatr Cardiol 13:152-158, 1992
Pediatric Cardiology 9 Springer-Verlag New York Inc. 1992
Children with Congenital H e a r t Disease: Probability of Natural Survival Milan ~am~inek Center of Pediatric Cardiologyand CardiovascularSurgery, UniversityHospital Motol, Prague, Czechoslovakia SUMMARY. The age distribution of death in all children with congenital heart disease (CHD), who died in a 27-year period in Central Bohemia (population of 1.2 million), and the data on the incidence of CHD in children born in Bohemia (population of 6.3 million) in 1980 were used to calculate the probability of survival of a child born with CHD. Eighty-six percent of these children survived to the first month of life--mostly those with pulmonary stenosis (PS, 99%), aortic stenosis (AS, 95%), ventricular septal defect (VSD, 92%), and atrioventricular septai defect (AVSD, 91%). Seventy-one percent of patients survived the first year of life--mostly those with PS (97%), AS (91%), atrial septal defect (ASD, 89%), VSD (80%), and persistent ductus arteriosus (PDA, 78%). In total, 67% of CHD patients can be expected to survive childhood. The highest survival rates were found in PS (94%), AS and ASD (84%), VSD and PDA (70-80%), and coarctation of the aorta (COA, 68%). The survival rate for the remaining forms of CHD was less than 50%. The highest mortality rate (10% of all children born with CHD) can be expected in the first postnatal week. The lowest survival in the first week was found among those with hypoplastic left heart (HLHS, 39%), double-outlet right ventricle (DORV, 50%), truncus arteriosus (TrA, 57%), pulmonary atresia (PA, 70%), and transposition of the great arteries (TGA, 83%). In addition, total anomalous pulmonary venous connection (TAPVC) and single ventricle had the highest risk of death in the first year of life. KEY WORDS: Mortality rate - - Congenital heart defect - - Infant mortality - - Natural survival
In Central Bohemia, a territory with over l million inhabitants, all children who died underwent autopsies, all autopsies being performed at the same Department of Pathological Anatomy by a pathologist specializing in heart malformations. Congenital heart disease (CHD) was involved in 7.4% of all deaths of liveborn children [16]. The most common CHD in liveborn children and those who died later were ventricular septal defect (VSD, 21.1%), hypoplastic left heart (HLHS, 10.5%), and transposition of the great arteries (TGA, 10.3%). In more than 5% of children with CHD who died, the diagnosis was tetralogy of Fallot (TF, 7.2%), coarctation of the aorta (COA, 6.9%), or complete atrioventricular septal defect (AVSD, 6.0%). CHD was more than 10 times more frequent in stillborn infants than in Prof. Dr. Milan ~amfinek, Kardiocentrum, UniversityHospitalMotol, 150 18 Prague5, Czechoslovakia.
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liveborns, and the distribution of types of defect was different. The most common was VSD (31.7%), followed by TF (8.5%). Hypoplastic left heart, pulmonary atresia (PA), and double-outlet right ventricle (DORV) were each found in 7.3% and complete AVSD in 6.1% of stillborns with CHD. In this paper the data on the age distribution of deaths in these children [18] over a 27-year period (which ended just before the introduction of openheart surgery as a routine procedure) as well as our data on CHD incidence [19] are used to calculate the probability of natural survival of a child born with CHD.
Materials and Methods
The death certificates and autopsy protocols were available for all children among 468,733 liveborns who died--before they were 15 years of age--either in a hospital or at home, in Central Bohemia.The regionhas a populationof 1.2 millionwith negligi-
Samgnek: Survival in Congenital Heart Disease
T a b l e
153
1. Survival of children born with CHD in first month of life
CHD
Incidence/1000 livebirths
Ventricular septal defect
2.015
Atrial septal defect
0.729
Aortic stenosis
0.490
Pulmonary stenosis
0.457
Coarctation of the aorta
0,370
Transposition of the great arteries
0,348
Patent ductus arteriosus
0.305
Actuarial survival rate (% of liveborns with CHD) 1 week
2 weeks
3 weeks
4 weeks
95 94-95 98 97-98 96 96-97 99 98-99 88 86-90 83 79-85
94 93-94 --95 95-96 --84 81-86 77 73-80
- -
- -
93 92-93 97 96-97 95 94-96 --80 76-83 72 67-76 99 99-99 91 89-93 26 9-40 86 83-89 60 47-68 88 94-91 47 21-60 33 0-50 --59 27-72 91 89-92 87 86-87
92 91-92 96 95-96 95 94-96 --78 74-81 69 64-74 97 96-98 --22 0-38 84 80-87 --86 81-90 40 13-56 25 0-50 87 79-91 50 9-66 91 -86 85-86
- -
Atrioventricular septal defect
0.261
Hypoplastic left heart syndrome
0.261
Tetralogy of Faltot
0.229
Pulmonary atresia
0.152
Single ventricle
0.098
Truncus arteriosus
0.076
Double-outlet right ventricle
0.076
Tricuspid atresia
0.054
Total anomalous pulmonary venous drainage Others
0.044
All
6.415
0.450
ble migration. Both industrial and agricultural areas are included in the region. The population of the largest town did not exceed 70,000, CHD was reliably diagnosed in 946 children between 1952 and 1979, a period during which cardiac surgery was already significantly affecting the mortality data [18]. Some necropsy diagnoses were reclassified to comply with present nomenclature. The malformation which dominated the hemodynamic abnormality was the one used in classifying complex forms of CHD [I6]. All 91,823 children born in 1980 in a territory of 6.3 million inhabitants were examined at least four times during infancy. All those suspected of having heart disease were referred to a pediatric cardiologist and finally examined at the Centre of Pediatric Cardiology and Cardiovascular Surgery [19J. Two-dimensional and Doppler echocardiography was performed in all patients. The shunt volume was measured by the radionuclide technique. Cardiac catheterization and angiocardiography were undertaken as indicated. Surgical and autopsy protocols were available for all those children born in 1980 who were operated on or had died.
97 96-97 39 26-50 88 85-90 70 61-76 98 97-98 57 38-69 50 25-63 95 73-97 95 91-97 93 92-94 90 90-91
-95 94-96 29 10-42 87 84-89 64 51-72 93 91-95 50 25-63 33 0-50 91 86-94 84 72-90 92 90-93 88 88-89
The number of children born with a heart defect in the 27year period was estimated using the Poisson distribution method. The survival estimate with the maximal limitation of an interval in percent for a given period of time was calculated by the pickup rate method [1]. Actuarial analysis of the survival data was used [5].
Results
Calculated Overall Survival T h e h i g h e s t m o r t a l i t y r a t e o f 10% w a s c a l c u l a t e d f o r the first p o s t n a t a l w e e k . T h e survival c u r v e dec r e a s e d in t h e s u b s e q u e n t w e e k s a n d d e c l i n e d m o r e g r a d u a l l y d u r i n g i n f a n c y a n d c h i l d h o o d ( T a b l e 1). O f all c h i l d r e n b o r n w i t h C H D , 8 6 % ( 8 5 - 8 6 )
154
Pediatric Cardiology Vol. 13, No. 3, 1992
Table 2. Survival of children born with CHD in the first year of life CHD
Incidence/1000
Actuarial survival rate (% o f liveborns with CHD)
livebirths
Ventricular septal defect Atrial septaI defect
2.015
0.729
Aortic stenosis
0.490
Pulmonary stenosis
0.457
Coarctation o f the aorta Transposition o f the great arteries
0.370 0.348
1 mo
2 mo
3 mo
4 mo
5 mo
6 mo
7 mo
8 mo
92
89
87
84
91-92
88-90
8 6 -8 8 "
83-85
96
95
93
95-96
94-95
95
94
9 mo
10 m o
83
82
81
80
.
82-84
81-83
79-82
79-81
.
--
91
90
--
90
89
--
--
89
92-94
--
90-92
89-91
--
88-91
88-90
--
--
87-90
93
92
91
--
--
91
.
.
I [ mo
.
.
.
.
.
.
.
.
.
12 mo
.
94-96
93-94
92-94
91-93
90-92
--
--
89-92
.
99
--
--
98
98
--
--
97
97
--
.
.
--
.
--
98-99
--
--
98-98
97-98
--
--
97-97
96-97
--
--
--
78
75
74
73
71
--
69
--
--
68
--
--
74-81
71-79
70-78
68-77
66-76
--
63-73
--
--
66-73
--
--
69
55
52
47
45
43
41
40
38
--
--
38
64-74
47-61
24-57
37-54
35-53
33-51
30-49
29-49
27-47
--
--
92
92
83
82
81
79
78
.
Patent ductus arteriosus
0.305
97 96-98
90-93
85-89
80-86
79-86
77 85
75-83
73-81
.
Atrioventricular septal defect
0.261
9I
88
8t
73
67
63
60
58
55
53
--
89-93
85-90
78-85
68-78
62-74
56-69
52 67
49-66
45-65
37-61
--
18
17
--
15
--
14
--
13
--
.
.
.
.
24-45 .
.
.
12
49 35-54 --
Hypoplastic left heart syndrome
0.261
22
Tetralogy o f Fallot
0,229
84
79
77
75
71
69
69
65
64
--
--
--
90-87
73-82
71-81
69-80
64-86
61-75
60-74
56-72
54-70
--
--
--
60
50
47
43
33
31
30
.
47-68
35-61
29-58
25-55
16-49
14-48
12-47
.
86
75
66
63
59
50
48
45
--
40
36
--
81-10
66-81
53-74
50 72
44-69
31-62
28-60
25-59
--
16-53
13 52
--
40
3[
27
19
--
16
.
.
.
.
.
0-46
0-40
--
.
.
.
.
.
0
0
0-38
Pulmonary atresia
0,152
Single ventricle
0,098
Truncus arteriosus
0.076
13-56
0-31
0-48
11
0-30
--
0-29
--
(t-38
0
--
0-23
--
0-28
--
0-27
.
--
. .
0
.
.
0-21
.
.
0-27
. .
. .
.
. .
14 0-35 .
.
Double outlet right ventricle
0.076
25
Tricuspid atresia
0,054
87
78
74
69
--
65
61
57
.
.
.
.
79-9 I
64-85
57-82
50-79
--
43-76
36-73
28-71
.
.
.
.
50
30
I0
0 50
Total anomalous pulmonary venous drainage
0,043
Others
0.450
All
6.415
0-44
0-33
0 38
(I-2 5
--
-
0
--
--
0
--
--
--
--
0 28
--
--
0-24
--
--
81
--
80
79
79
78
78-83
--
77-82
76-82
76-8I
75-81
74
73
72
72
71
--
--
73-75
72-73
71-73
70-73
70-72
--
--
9
89
85
84
83
82
89-92
87-90
93-87
81-86
80-85
79-84
86
82
79
77
75
85-86
81-82
78 80
76-78
74-76
Eight percent of children born with ventricular septal defect (VSD) died in the neonatal period, most of them already in the first postnatal week (Tables 1 and 2). In general, the first 4 months of life were the most critical, beyond this 84% (83-85) survived (Table 2). Probability of survival did not decrease between 8 months and 5 years of life. A further slight decline in the survival curve started at this age, to reach 76% (74-78) survival at the end of childhood (Table 3), In total, 84% (82-87) of children born with atrial septal defect (ASD) reached the age of 15 (Table 3). The steepest decline in the survival curve was
.
(1
0-5t
Survival in Various Forms of CHD
.
0-31
9 66
survived the neonatal period, and 71 (70-72%) survived the first year of life (Table 2). In total, 25% of all children born with CHD die during the first 5 months of life. At the age of 2 years, 70% (69-71) of children were alive: 67% (65-69) survived to the age of 15.
.
--
found immediately after birth. Eighty-nine percent (87-90) survived infancy. Children born with aortic stenosis (AS) had the same chance of reaching the age of 15 as ASD (Table 3). However, a few children born with AS died in the first postnatal week (Table 1). The survival curve stabilized around 95% (94-95) and declined slowly during the second through fifth month of life. There is a 91% chance for all children with AS to survive the first half, as well as the first year of life. The survival curve stabilized between the second and tenth years of life. A slightly more pronounced decline of the survival curve starts later, to reach a calculated survival rate of 84% (80-86) at the age of 15 years. The most favorable course was with pulmonary stenosis (PS). As many as 94% (93-95) of patients can be expected to be alive at the age of 15 (Table 3). Three percent of children with PS die during the first year of life. Relatively safe is the period between the third and tenth year of life. The survival curve for coarctation of the aorta (COA) exhibited the steepest decline in the neonatal
~ a m ~ m e k : S u r v i v a l in C o n g e n i t a l H e a r t D i s e a s e
155
T a b l e 3. S u r v i v a l o f c h i l d r e n b o r n w i t h C H D , 1 - 1 5 y e a r s CHD
Incidence/1000
Actuarial survival rate (% of liveborns with CHD)
livebirths
Ventricular septal defect Atrial septal defect Aortic stenosis Pulmonary stenosis
2.015 0.729 0.490 0.457
Coarctation of the aorta
0.370
Transposition of the great arteries
0.348
Patent ductus arteriosus
0.305
Atrioventricular septal defect
0.261
H y p o p l a s t i c left h e a r t s y n d r o m e
0.261
1 yr
2 yr
5 yr
10 yr
15 yr
80
--
--
79
76
78-81
--
--
78-81
74-78
89
86
85
84
84
87-90
84-87
84-87
83 - 8 7
82-87
91
88
--
87
84
89-92
87-90
--
85-89
80-86
97
96
--
--
94
96-97
95-97
--
--
93-95
68
.
.
.
.
.
.
38
.
.
.
.
24-45
.
.
.
.
78
77
--
--
70
73-81
73-85
--
--
60-76
49
.
.
35-54
.
.
12
--
--
--
11
--
--
--
1-30
56
49
23
4
Tetralogy of Fallot
0.229
64 54-70
38-66
Pulmonary atresia
0.152
30
.
.
12-47
.
.
Single ventricle
0.098
36
33
13-52 0.076
14
Double-outlet right ventricle
0.076
0
Tricuspid atresia
0.054
57
0.044
0
0.450
78
venous drainage Others
--
--
75-81 All
6.415
period (Table 2). It dropped to 88% (86-90) already in the first postnatal week. The curve stabilized at the age of 10 months at a survival rate of 68% (6673). There was no further decline beyond infancy. Only 69% (64-74) of children born with one of the various forms of transposition of the great arteries (TGA) survive the neonatal period (Table 1). A steep decrease in the survival rate continued through the fourth month of life (Table 2). At the end of the first year, 38% (24-45) of children can be expected to be alive. No further decrease in the survival rate occurred beyond infancy. The second and fourth months of life were the most dangerous periods for a child born with a persistent ductus arteriosus (PDA) (Table 2), leading to a 70% (60-76) chance of surviving childhood.
.
0-37
.
.
.
. 31 0-50 11 0-36
0-25
-----
0
--
--
--
0-11
--
--
--
42
--
27
43 7-59
0-28
.
7-48 --
28-71
.
31
--
0-21
.
35-59
6-48
0-35
Total anomalous pulmonary
.
63-73
0-27
Truncus arteriosus
.
0-60
0
--
0-14
--
---
--
--
--
--
--
-
-
-
-
-
0-56
--
-
78 72-80
71
70
--
--
67
70-72
69-71
70-73
69-72
65-69
Atrioventricular septal defect (AVSD) mostly endangered life in the first five postnatal months (Table 2). The age of 6 months was reached by only 63% (56-69) of patients. A total of 49% (35-54) of patients survived infancy. Once having survived infancy the rest of the childhood was a relatively safe period. Hypoplastic left heart syndrome (HLHS) had the highest neonatal mortality rate (Table 1). Most patients died in the first postnatal week, leaving a 39% (26-50) probability of survival. Only patients with a less pronounced form of the malformation survived the first few months of life (Tables 2 and 3). The child with tetralogy of Fallot (TF) has two very critical periods of life, the first postnatal week
156
and school age. Twelve percent can be expected to die soon after birth, but 84% (80-87) of patients survive the first month (Table 1), and about 64% reach the age of 1 year. An increase in mortality rate between 5 and 15 years of age gives a chance for only 14% (0-25) of patients to survive childhood (Table 3). The survival curve in pulmonary atresia (PA) (including PA with a VSD) showed a drop in the early postnatal period, leaving only a 60% (47-68) probability of survival at the age of 3 weeks (Table 1). A less pronounced decrease of the survival curve continued during the subsequent 6 months of infancy (Table 2). All patients who survived the seventh month of age had a good chance of surviving to 15 years. Eighty-six percent (81-90) of children born with a single (double inlet) ventricle (DIV) survived the neonatal period and 36% (13-52) the first year of life (Table 2). There was a low mortality rate .among those who survived infancy (Table 3). A steep decline of the survival curve already in the neonatal and early infancy period characterized truncus arteriosus (TrA). Only 16% (0-38) of patients had a chance to survive the first 6 months of life (Tables 1 and 2). Even a more pronounced drop in the survival rate among neonates was found in double-outlet right ventricle (DORV) (Table 1): the survival curve reached 0 at the age of 3 months (Table 2). Tricuspid atresia (TA) endangered life in 87% (79-91) of patients in the neonatal period (Table I). A 30% decrease in survival rate occurred in the first to eighth month of life (Table 2). A total of 57% (2871) of patients can be expected to survive the end of the first year and 27% to the age of 15 (Table 3). A 50% (9-66) survival of the neonatal period can be expected in total anomalous pulmonary venous connection (TAPVC) (Table 1). The survival curve continued to fall very steeply in early infancy, reaching 0 at the age of 4 months (Table 2).
Discussion The chance that a child born with CHD will survive depends on the anatomy and the degree of hemodynamic disturbances caused by the defect(s). The highest survival rate in the neonatal period was found for PS. In addition, 95% or more of children born with a PDA, ASD, or AS are likely to survive the first 4 weeks of life. It can be assumed that more than 90% of children with VSD or AVSD will survive. A low probability of surviving the first postnatal week exists for HLHS (61%), DORV (50%), TrA (43%), and PA (30%). These four forms of CHD
Pediatric Cardiology Vol. 13, No. 3, 1992
remain the most dangerous ones during the second and third weeks after birth. An autopsy finding of CHD in a neonate does not always mean that the CHD was the cause of death [7]. Of 393 neonates, 30% died obviously and 22% probably from a noncardiac cause [17]. The highest incidence of noncardiac causes was proven for the first 24 postnatal hours, after which it decreased rapidly. Starting with the fourth day of life, CHD itself was the most likely cause of a fatal outcome. Noncardiac causes of death can explain the drop in the actuarial survival curve during the neonatal period in some forms of CHD with generally very good prognosis, such as ASD, VSD, and PS. The forms of CHD with the greatest chance of survival during the first 3 months of life, include children with isolated PS and more than 90% of those born with ASD, AS, or PDA. In contrast, a very small number of children with DORV, TAPVC, or hypoplastic left heart survive this period of life. Children with TrA or PA have a calculated survival of only 27 and 47%, respectively, in the first 3 months of life. Children born with PS, AS, and ASD have the best chance to survive the first 6 months of life. More than 80% of those with VSD and PDA wilt survive this age period. Children with the most serious CHD die in the same order of frequency as in the previous period of life. This ranking in the survival rate does not change later in infancy. The probability of reaching age 15 is of the same order as that at the end of the first year. Children born with PS have a greater than 90% chance and those with AS or ASD have a 84% chance of survival. The estimate in patients with VSD and PDA ranges from 70-80%, followed by coarctation, with a 68% chance of survival to age 15. The rest of CHD patients have a lower than 50% chance of surviving childhood. The accuracy of calculated survival rate depends on the reliability of methods used to detect and classify CHD, on the completeness with which the population has been studied, and on the elimination of any kind of selection bias. There can be no doubt about the reliability of detailed autopsy protocols. We selected a limited region of Bohemia in which all autopsies were performed or supervised by a single experienced pathologist specializing in CHD. This approach necessitated a long period of sampling a representative number of cases and retrospective reclassification, in some cases of complex CHD. A very limited life expectancy in DORV could reflect a possible misinterpretation of some autopsy findings. It was sometimes difficult to distinguish retrospectively DORV (with d-malposition of the
Sam~mek: Survival in Congenital Heart Disease
great arteries) from TGA with a VSD. A shift from DORV to TGA would result in an increased survival rate for the former and a higher mortality for children with TGA. The survival curve in transposition would then fit better with that reported by Liebman et al. [8]. All necessary methods--echocardiography, radionuclides, and cardiac catheterization with angiography if indicated--were used to get reliable data on the incidence of CHD in children born in 1980 [19]. Autopsies were performed in all children born in 1980 who died, and cardiac surgery was performed solely in our center. A great advantage of this study was the complete coverage of all patients, dead or alive, in a large well-defined territory and compulsory autopsies of all deceased children up to the age of 15. The selection of the region of study can be a serious limiting factor in the unconditional applicability of the epidemiological findings. We chose a relatively large mixed industrial and agricultural territory in Central Europe with a negligible migration. Differences in CHD incidence have been reported within various ethnic groups [9, 14, 15, 20]. Nonetheless, it seems reasonable to expect that our data on survival probability can be used also for other populations. The probability of survival of children born with CHD was slightly higher in our country than that reported in other studies. A minimal difference was found in the final number of survivors at the age of 15. It was most pronounced in the early postnatal period. The slope of the survival curve after the first week of life matches that reported by others [2, 4, 12, 13], and probably more reliable than some [10, 111. An improvement in general perinatal and infant care may well have influenced favorably the mortality rate of our series compared with earlier studies. The mean neonatal mortality rate in the given region was 15.1 per 1000 livebirths. It decreased from 19.0 in the early 1950s to 11.9 in 1977. The infant mortality was 30 per 1000 livebirths in the first 3 years of the study, dropping to below 20 per 1,000 in 197I. In the last 2 years of the study it was 16.2 and 15.7. The mean value from 1952 through 1978 was 21.0 per I000 livebirths. However, a decrease in overall neonatal mortality from 17.3 per 1000 livebirths in the first 5 years of the study to 13.6 per 1000 in the last 5 years did not influence the mortality rate for children with CHD. The proportion of the cardiac to the total child death rate rose from 4.7% in the first years to 7.8% in the last 5 years of the study. Cardiovascular surgery has profoundly changed the natural course of CHD. We tried to eliminate
157
this effect by choosing a period of study which preceded the surgical era in a given region. However, balloon atrial septostomy, introduced in 1967, could have postponed the fatal outcome of simple TGA and change its survival curve in the early postnatal life. Yet separate calculation of the survival curve before and after the introduction of balloon septostomy did not reveal any significant difference. Surgical intervention could favorably influence the survival curve in PDA. However, the operation was done only sporadically in infants. Thus, the survival curve in infancy reflects more the natural course of the disease than the course in a properly treated child with isolated PDA. A similar, but even less pronounced, influence of surgery on the natural course of the disease holds for TF. Until 1968, anastomosing operations were done only in older children. A small number of children less than 4 years of age had this operation in the last few years of the study. Cardiac surgery has markedly changed the course and outcome of CHD, its worldwide availability has eliminated any further possibility to follow-up the natural course and unconditional prognosis of CHD. Data on unconditional survival, however, remain indispensable in answering the questions posed most frequently by the family of an affected child, and they play an important role in planning the strategy of medical and surgical treatment. Recently, rapid progress in the fetal diagnosis of heart defects has accentuated the need to know the natural course of CHD. Such information is important when discussing possible intervention during pregnancy. It also aids in organizing special care, even before the delivery, and immediately after birth. References 1. And61 J (1978) Mathematical statistics. SNTL, Praha (in Czech) 2. Carlgren LE (1959) The incidence of congenital heart disease in children born in Gothenburg 1941-1950. Br Heart J 21:40 3. Collet RW, Edwards JE. Persistent truncus arteriosus: A classification according to anatomic types. Surg Clin N Am 29:1245
4. Feldt RH, Avasthey P, Yoshimasu F, Kurland LT, Titus JL (1971) Incidence of congenital heart disease in children born to Residents of Olmsted Country, Minnesota, 1950-1969. Mayo Clin Proc 46:794 5. Grunkemeier GL, Starr A (1977) Acturial analysis of surgical results: Rationale and method. Ann Thorac Surg 24:404 6. Hay JD (1966) Population and clinic studies of congenital heart disease in Liverpool. Br Med J 2:661 7. Hoffman JIE, Christianson R (1978) Congenital heart disease in a cohort of 19 502 births with long-term follow-up. Am J Cardiol 42:641 8. Liebman J, Cullum L, Belloc NB (1969) Natural history of
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9.
10.
11.
12. 13.
Pediatric Cardiology Vol. 13, No. 3, 1992
transposition of the great arteries. Anatomy and birth and death characteristics. Circulation 40:237 Lien Wen-Pin, Chen Jin Jer, Chen Jyh-Hong, Lin Jiunu-Lee, Jesuh Yen-Yuw, Wu Teh-Lu, Chu Su-Hsun, Hung Chi-Ren (1966) Frequency of various congenital heart diseases in chinese adults: Analysis of 926 congestive heart diseases in chinese adults: Analysis of 926 congestive patients over 13 years of age. A m J Cardiol 57:840 MacMahon B, McKeown T, Record RG (1952) The incidence and life expectation of children with congenital heart disease. Br J Soc Med 6:121 McKeown T, Record RG (1960) Malformations in a population observe for five years after birth. In: Wolstenholme GEW, O'Connor CM (eds) Ciba Foundation Symposium on congenital malformations. Little, Brown and Co, Boston, p. 2 Mitchel SC, Korones SB, Berendes HW (1971) Congenital heart disease in 56 109 births. Circulation 43:323 Mustacchi P, Sherins RS, Miller MJ (1963) Congenital malformations of the heart and the great vessels. JAMA 183:241
14. Neel JV (1958) Study of major congenital defects in Japanese infants. A m J Hum Genet 10:398 15. Ongley PA. Pediatric Cardiology in Thailand. Circulation 34:1
16. 5)amfmek M, Goetzov~i J, Bene~ov~t D (1985) Distribution of congenital heart malformations in an autopsied child population. Int J Cardiol 8:235 17. 5]am~inek M, Goetzov~l J, Benegovfi D (1986) Causes of death in neonates born with a heart malformation. Int J Cardiol 11:63 18. ~am~inek M, Benegovfi D, Goetzovfi J, Hrycejovfi I (1988) Distribution of age at death in children with congenital heart disease who died before the age of 15. Br Heart J 59:581 19. ~am~mek M, Slavfk Z, Zbo~ilovfi B, Hrobofiovft V, Vo~f~kov~ M, ~kovrgmek J (1989) Prospective study on incidence, treatment and outcome of heart diseases in 91 823 live born children. Pediatr Cardiol 10:580 20. Wallooppillai NJ, Jayasinghe MdeS (1970) Congenital heart disease in Ceylon. Br Heart J 32:304
Pediatric Cardiology 9 Springer-Verlag New York Inc. 1992
Children with Congenital H e a r t Disease: Probability of Natural Survival Milan ~am~inek Center of Pediatric Cardiologyand CardiovascularSurgery, UniversityHospital Motol, Prague, Czechoslovakia SUMMARY. The age distribution of death in all children with congenital heart disease (CHD), who died in a 27-year period in Central Bohemia (population of 1.2 million), and the data on the incidence of CHD in children born in Bohemia (population of 6.3 million) in 1980 were used to calculate the probability of survival of a child born with CHD. Eighty-six percent of these children survived to the first month of life--mostly those with pulmonary stenosis (PS, 99%), aortic stenosis (AS, 95%), ventricular septal defect (VSD, 92%), and atrioventricular septai defect (AVSD, 91%). Seventy-one percent of patients survived the first year of life--mostly those with PS (97%), AS (91%), atrial septal defect (ASD, 89%), VSD (80%), and persistent ductus arteriosus (PDA, 78%). In total, 67% of CHD patients can be expected to survive childhood. The highest survival rates were found in PS (94%), AS and ASD (84%), VSD and PDA (70-80%), and coarctation of the aorta (COA, 68%). The survival rate for the remaining forms of CHD was less than 50%. The highest mortality rate (10% of all children born with CHD) can be expected in the first postnatal week. The lowest survival in the first week was found among those with hypoplastic left heart (HLHS, 39%), double-outlet right ventricle (DORV, 50%), truncus arteriosus (TrA, 57%), pulmonary atresia (PA, 70%), and transposition of the great arteries (TGA, 83%). In addition, total anomalous pulmonary venous connection (TAPVC) and single ventricle had the highest risk of death in the first year of life. KEY WORDS: Mortality rate - - Congenital heart defect - - Infant mortality - - Natural survival
In Central Bohemia, a territory with over l million inhabitants, all children who died underwent autopsies, all autopsies being performed at the same Department of Pathological Anatomy by a pathologist specializing in heart malformations. Congenital heart disease (CHD) was involved in 7.4% of all deaths of liveborn children [16]. The most common CHD in liveborn children and those who died later were ventricular septal defect (VSD, 21.1%), hypoplastic left heart (HLHS, 10.5%), and transposition of the great arteries (TGA, 10.3%). In more than 5% of children with CHD who died, the diagnosis was tetralogy of Fallot (TF, 7.2%), coarctation of the aorta (COA, 6.9%), or complete atrioventricular septal defect (AVSD, 6.0%). CHD was more than 10 times more frequent in stillborn infants than in Prof. Dr. Milan ~amfinek, Kardiocentrum, UniversityHospitalMotol, 150 18 Prague5, Czechoslovakia.
Address offprint requests to:
liveborns, and the distribution of types of defect was different. The most common was VSD (31.7%), followed by TF (8.5%). Hypoplastic left heart, pulmonary atresia (PA), and double-outlet right ventricle (DORV) were each found in 7.3% and complete AVSD in 6.1% of stillborns with CHD. In this paper the data on the age distribution of deaths in these children [18] over a 27-year period (which ended just before the introduction of openheart surgery as a routine procedure) as well as our data on CHD incidence [19] are used to calculate the probability of natural survival of a child born with CHD.
Materials and Methods
The death certificates and autopsy protocols were available for all children among 468,733 liveborns who died--before they were 15 years of age--either in a hospital or at home, in Central Bohemia.The regionhas a populationof 1.2 millionwith negligi-
Samgnek: Survival in Congenital Heart Disease
T a b l e
153
1. Survival of children born with CHD in first month of life
CHD
Incidence/1000 livebirths
Ventricular septal defect
2.015
Atrial septal defect
0.729
Aortic stenosis
0.490
Pulmonary stenosis
0.457
Coarctation of the aorta
0,370
Transposition of the great arteries
0,348
Patent ductus arteriosus
0.305
Actuarial survival rate (% of liveborns with CHD) 1 week
2 weeks
3 weeks
4 weeks
95 94-95 98 97-98 96 96-97 99 98-99 88 86-90 83 79-85
94 93-94 --95 95-96 --84 81-86 77 73-80
- -
- -
93 92-93 97 96-97 95 94-96 --80 76-83 72 67-76 99 99-99 91 89-93 26 9-40 86 83-89 60 47-68 88 94-91 47 21-60 33 0-50 --59 27-72 91 89-92 87 86-87
92 91-92 96 95-96 95 94-96 --78 74-81 69 64-74 97 96-98 --22 0-38 84 80-87 --86 81-90 40 13-56 25 0-50 87 79-91 50 9-66 91 -86 85-86
- -
Atrioventricular septal defect
0.261
Hypoplastic left heart syndrome
0.261
Tetralogy of Faltot
0.229
Pulmonary atresia
0.152
Single ventricle
0.098
Truncus arteriosus
0.076
Double-outlet right ventricle
0.076
Tricuspid atresia
0.054
Total anomalous pulmonary venous drainage Others
0.044
All
6.415
0.450
ble migration. Both industrial and agricultural areas are included in the region. The population of the largest town did not exceed 70,000, CHD was reliably diagnosed in 946 children between 1952 and 1979, a period during which cardiac surgery was already significantly affecting the mortality data [18]. Some necropsy diagnoses were reclassified to comply with present nomenclature. The malformation which dominated the hemodynamic abnormality was the one used in classifying complex forms of CHD [I6]. All 91,823 children born in 1980 in a territory of 6.3 million inhabitants were examined at least four times during infancy. All those suspected of having heart disease were referred to a pediatric cardiologist and finally examined at the Centre of Pediatric Cardiology and Cardiovascular Surgery [19J. Two-dimensional and Doppler echocardiography was performed in all patients. The shunt volume was measured by the radionuclide technique. Cardiac catheterization and angiocardiography were undertaken as indicated. Surgical and autopsy protocols were available for all those children born in 1980 who were operated on or had died.
97 96-97 39 26-50 88 85-90 70 61-76 98 97-98 57 38-69 50 25-63 95 73-97 95 91-97 93 92-94 90 90-91
-95 94-96 29 10-42 87 84-89 64 51-72 93 91-95 50 25-63 33 0-50 91 86-94 84 72-90 92 90-93 88 88-89
The number of children born with a heart defect in the 27year period was estimated using the Poisson distribution method. The survival estimate with the maximal limitation of an interval in percent for a given period of time was calculated by the pickup rate method [1]. Actuarial analysis of the survival data was used [5].
Results
Calculated Overall Survival T h e h i g h e s t m o r t a l i t y r a t e o f 10% w a s c a l c u l a t e d f o r the first p o s t n a t a l w e e k . T h e survival c u r v e dec r e a s e d in t h e s u b s e q u e n t w e e k s a n d d e c l i n e d m o r e g r a d u a l l y d u r i n g i n f a n c y a n d c h i l d h o o d ( T a b l e 1). O f all c h i l d r e n b o r n w i t h C H D , 8 6 % ( 8 5 - 8 6 )
154
Pediatric Cardiology Vol. 13, No. 3, 1992
Table 2. Survival of children born with CHD in the first year of life CHD
Incidence/1000
Actuarial survival rate (% o f liveborns with CHD)
livebirths
Ventricular septal defect Atrial septaI defect
2.015
0.729
Aortic stenosis
0.490
Pulmonary stenosis
0.457
Coarctation o f the aorta Transposition o f the great arteries
0.370 0.348
1 mo
2 mo
3 mo
4 mo
5 mo
6 mo
7 mo
8 mo
92
89
87
84
91-92
88-90
8 6 -8 8 "
83-85
96
95
93
95-96
94-95
95
94
9 mo
10 m o
83
82
81
80
.
82-84
81-83
79-82
79-81
.
--
91
90
--
90
89
--
--
89
92-94
--
90-92
89-91
--
88-91
88-90
--
--
87-90
93
92
91
--
--
91
.
.
I [ mo
.
.
.
.
.
.
.
.
.
12 mo
.
94-96
93-94
92-94
91-93
90-92
--
--
89-92
.
99
--
--
98
98
--
--
97
97
--
.
.
--
.
--
98-99
--
--
98-98
97-98
--
--
97-97
96-97
--
--
--
78
75
74
73
71
--
69
--
--
68
--
--
74-81
71-79
70-78
68-77
66-76
--
63-73
--
--
66-73
--
--
69
55
52
47
45
43
41
40
38
--
--
38
64-74
47-61
24-57
37-54
35-53
33-51
30-49
29-49
27-47
--
--
92
92
83
82
81
79
78
.
Patent ductus arteriosus
0.305
97 96-98
90-93
85-89
80-86
79-86
77 85
75-83
73-81
.
Atrioventricular septal defect
0.261
9I
88
8t
73
67
63
60
58
55
53
--
89-93
85-90
78-85
68-78
62-74
56-69
52 67
49-66
45-65
37-61
--
18
17
--
15
--
14
--
13
--
.
.
.
.
24-45 .
.
.
12
49 35-54 --
Hypoplastic left heart syndrome
0.261
22
Tetralogy o f Fallot
0,229
84
79
77
75
71
69
69
65
64
--
--
--
90-87
73-82
71-81
69-80
64-86
61-75
60-74
56-72
54-70
--
--
--
60
50
47
43
33
31
30
.
47-68
35-61
29-58
25-55
16-49
14-48
12-47
.
86
75
66
63
59
50
48
45
--
40
36
--
81-10
66-81
53-74
50 72
44-69
31-62
28-60
25-59
--
16-53
13 52
--
40
3[
27
19
--
16
.
.
.
.
.
0-46
0-40
--
.
.
.
.
.
0
0
0-38
Pulmonary atresia
0,152
Single ventricle
0,098
Truncus arteriosus
0.076
13-56
0-31
0-48
11
0-30
--
0-29
--
(t-38
0
--
0-23
--
0-28
--
0-27
.
--
. .
0
.
.
0-21
.
.
0-27
. .
. .
.
. .
14 0-35 .
.
Double outlet right ventricle
0.076
25
Tricuspid atresia
0,054
87
78
74
69
--
65
61
57
.
.
.
.
79-9 I
64-85
57-82
50-79
--
43-76
36-73
28-71
.
.
.
.
50
30
I0
0 50
Total anomalous pulmonary venous drainage
0,043
Others
0.450
All
6.415
0-44
0-33
0 38
(I-2 5
--
-
0
--
--
0
--
--
--
--
0 28
--
--
0-24
--
--
81
--
80
79
79
78
78-83
--
77-82
76-82
76-8I
75-81
74
73
72
72
71
--
--
73-75
72-73
71-73
70-73
70-72
--
--
9
89
85
84
83
82
89-92
87-90
93-87
81-86
80-85
79-84
86
82
79
77
75
85-86
81-82
78 80
76-78
74-76
Eight percent of children born with ventricular septal defect (VSD) died in the neonatal period, most of them already in the first postnatal week (Tables 1 and 2). In general, the first 4 months of life were the most critical, beyond this 84% (83-85) survived (Table 2). Probability of survival did not decrease between 8 months and 5 years of life. A further slight decline in the survival curve started at this age, to reach 76% (74-78) survival at the end of childhood (Table 3), In total, 84% (82-87) of children born with atrial septal defect (ASD) reached the age of 15 (Table 3). The steepest decline in the survival curve was
.
(1
0-5t
Survival in Various Forms of CHD
.
0-31
9 66
survived the neonatal period, and 71 (70-72%) survived the first year of life (Table 2). In total, 25% of all children born with CHD die during the first 5 months of life. At the age of 2 years, 70% (69-71) of children were alive: 67% (65-69) survived to the age of 15.
.
--
found immediately after birth. Eighty-nine percent (87-90) survived infancy. Children born with aortic stenosis (AS) had the same chance of reaching the age of 15 as ASD (Table 3). However, a few children born with AS died in the first postnatal week (Table 1). The survival curve stabilized around 95% (94-95) and declined slowly during the second through fifth month of life. There is a 91% chance for all children with AS to survive the first half, as well as the first year of life. The survival curve stabilized between the second and tenth years of life. A slightly more pronounced decline of the survival curve starts later, to reach a calculated survival rate of 84% (80-86) at the age of 15 years. The most favorable course was with pulmonary stenosis (PS). As many as 94% (93-95) of patients can be expected to be alive at the age of 15 (Table 3). Three percent of children with PS die during the first year of life. Relatively safe is the period between the third and tenth year of life. The survival curve for coarctation of the aorta (COA) exhibited the steepest decline in the neonatal
~ a m ~ m e k : S u r v i v a l in C o n g e n i t a l H e a r t D i s e a s e
155
T a b l e 3. S u r v i v a l o f c h i l d r e n b o r n w i t h C H D , 1 - 1 5 y e a r s CHD
Incidence/1000
Actuarial survival rate (% of liveborns with CHD)
livebirths
Ventricular septal defect Atrial septal defect Aortic stenosis Pulmonary stenosis
2.015 0.729 0.490 0.457
Coarctation of the aorta
0.370
Transposition of the great arteries
0.348
Patent ductus arteriosus
0.305
Atrioventricular septal defect
0.261
H y p o p l a s t i c left h e a r t s y n d r o m e
0.261
1 yr
2 yr
5 yr
10 yr
15 yr
80
--
--
79
76
78-81
--
--
78-81
74-78
89
86
85
84
84
87-90
84-87
84-87
83 - 8 7
82-87
91
88
--
87
84
89-92
87-90
--
85-89
80-86
97
96
--
--
94
96-97
95-97
--
--
93-95
68
.
.
.
.
.
.
38
.
.
.
.
24-45
.
.
.
.
78
77
--
--
70
73-81
73-85
--
--
60-76
49
.
.
35-54
.
.
12
--
--
--
11
--
--
--
1-30
56
49
23
4
Tetralogy of Fallot
0.229
64 54-70
38-66
Pulmonary atresia
0.152
30
.
.
12-47
.
.
Single ventricle
0.098
36
33
13-52 0.076
14
Double-outlet right ventricle
0.076
0
Tricuspid atresia
0.054
57
0.044
0
0.450
78
venous drainage Others
--
--
75-81 All
6.415
period (Table 2). It dropped to 88% (86-90) already in the first postnatal week. The curve stabilized at the age of 10 months at a survival rate of 68% (6673). There was no further decline beyond infancy. Only 69% (64-74) of children born with one of the various forms of transposition of the great arteries (TGA) survive the neonatal period (Table 1). A steep decrease in the survival rate continued through the fourth month of life (Table 2). At the end of the first year, 38% (24-45) of children can be expected to be alive. No further decrease in the survival rate occurred beyond infancy. The second and fourth months of life were the most dangerous periods for a child born with a persistent ductus arteriosus (PDA) (Table 2), leading to a 70% (60-76) chance of surviving childhood.
.
0-37
.
.
.
. 31 0-50 11 0-36
0-25
-----
0
--
--
--
0-11
--
--
--
42
--
27
43 7-59
0-28
.
7-48 --
28-71
.
31
--
0-21
.
35-59
6-48
0-35
Total anomalous pulmonary
.
63-73
0-27
Truncus arteriosus
.
0-60
0
--
0-14
--
---
--
--
--
--
--
-
-
-
-
-
0-56
--
-
78 72-80
71
70
--
--
67
70-72
69-71
70-73
69-72
65-69
Atrioventricular septal defect (AVSD) mostly endangered life in the first five postnatal months (Table 2). The age of 6 months was reached by only 63% (56-69) of patients. A total of 49% (35-54) of patients survived infancy. Once having survived infancy the rest of the childhood was a relatively safe period. Hypoplastic left heart syndrome (HLHS) had the highest neonatal mortality rate (Table 1). Most patients died in the first postnatal week, leaving a 39% (26-50) probability of survival. Only patients with a less pronounced form of the malformation survived the first few months of life (Tables 2 and 3). The child with tetralogy of Fallot (TF) has two very critical periods of life, the first postnatal week
156
and school age. Twelve percent can be expected to die soon after birth, but 84% (80-87) of patients survive the first month (Table 1), and about 64% reach the age of 1 year. An increase in mortality rate between 5 and 15 years of age gives a chance for only 14% (0-25) of patients to survive childhood (Table 3). The survival curve in pulmonary atresia (PA) (including PA with a VSD) showed a drop in the early postnatal period, leaving only a 60% (47-68) probability of survival at the age of 3 weeks (Table 1). A less pronounced decrease of the survival curve continued during the subsequent 6 months of infancy (Table 2). All patients who survived the seventh month of age had a good chance of surviving to 15 years. Eighty-six percent (81-90) of children born with a single (double inlet) ventricle (DIV) survived the neonatal period and 36% (13-52) the first year of life (Table 2). There was a low mortality rate .among those who survived infancy (Table 3). A steep decline of the survival curve already in the neonatal and early infancy period characterized truncus arteriosus (TrA). Only 16% (0-38) of patients had a chance to survive the first 6 months of life (Tables 1 and 2). Even a more pronounced drop in the survival rate among neonates was found in double-outlet right ventricle (DORV) (Table 1): the survival curve reached 0 at the age of 3 months (Table 2). Tricuspid atresia (TA) endangered life in 87% (79-91) of patients in the neonatal period (Table I). A 30% decrease in survival rate occurred in the first to eighth month of life (Table 2). A total of 57% (2871) of patients can be expected to survive the end of the first year and 27% to the age of 15 (Table 3). A 50% (9-66) survival of the neonatal period can be expected in total anomalous pulmonary venous connection (TAPVC) (Table 1). The survival curve continued to fall very steeply in early infancy, reaching 0 at the age of 4 months (Table 2).
Discussion The chance that a child born with CHD will survive depends on the anatomy and the degree of hemodynamic disturbances caused by the defect(s). The highest survival rate in the neonatal period was found for PS. In addition, 95% or more of children born with a PDA, ASD, or AS are likely to survive the first 4 weeks of life. It can be assumed that more than 90% of children with VSD or AVSD will survive. A low probability of surviving the first postnatal week exists for HLHS (61%), DORV (50%), TrA (43%), and PA (30%). These four forms of CHD
Pediatric Cardiology Vol. 13, No. 3, 1992
remain the most dangerous ones during the second and third weeks after birth. An autopsy finding of CHD in a neonate does not always mean that the CHD was the cause of death [7]. Of 393 neonates, 30% died obviously and 22% probably from a noncardiac cause [17]. The highest incidence of noncardiac causes was proven for the first 24 postnatal hours, after which it decreased rapidly. Starting with the fourth day of life, CHD itself was the most likely cause of a fatal outcome. Noncardiac causes of death can explain the drop in the actuarial survival curve during the neonatal period in some forms of CHD with generally very good prognosis, such as ASD, VSD, and PS. The forms of CHD with the greatest chance of survival during the first 3 months of life, include children with isolated PS and more than 90% of those born with ASD, AS, or PDA. In contrast, a very small number of children with DORV, TAPVC, or hypoplastic left heart survive this period of life. Children with TrA or PA have a calculated survival of only 27 and 47%, respectively, in the first 3 months of life. Children born with PS, AS, and ASD have the best chance to survive the first 6 months of life. More than 80% of those with VSD and PDA wilt survive this age period. Children with the most serious CHD die in the same order of frequency as in the previous period of life. This ranking in the survival rate does not change later in infancy. The probability of reaching age 15 is of the same order as that at the end of the first year. Children born with PS have a greater than 90% chance and those with AS or ASD have a 84% chance of survival. The estimate in patients with VSD and PDA ranges from 70-80%, followed by coarctation, with a 68% chance of survival to age 15. The rest of CHD patients have a lower than 50% chance of surviving childhood. The accuracy of calculated survival rate depends on the reliability of methods used to detect and classify CHD, on the completeness with which the population has been studied, and on the elimination of any kind of selection bias. There can be no doubt about the reliability of detailed autopsy protocols. We selected a limited region of Bohemia in which all autopsies were performed or supervised by a single experienced pathologist specializing in CHD. This approach necessitated a long period of sampling a representative number of cases and retrospective reclassification, in some cases of complex CHD. A very limited life expectancy in DORV could reflect a possible misinterpretation of some autopsy findings. It was sometimes difficult to distinguish retrospectively DORV (with d-malposition of the
Sam~mek: Survival in Congenital Heart Disease
great arteries) from TGA with a VSD. A shift from DORV to TGA would result in an increased survival rate for the former and a higher mortality for children with TGA. The survival curve in transposition would then fit better with that reported by Liebman et al. [8]. All necessary methods--echocardiography, radionuclides, and cardiac catheterization with angiography if indicated--were used to get reliable data on the incidence of CHD in children born in 1980 [19]. Autopsies were performed in all children born in 1980 who died, and cardiac surgery was performed solely in our center. A great advantage of this study was the complete coverage of all patients, dead or alive, in a large well-defined territory and compulsory autopsies of all deceased children up to the age of 15. The selection of the region of study can be a serious limiting factor in the unconditional applicability of the epidemiological findings. We chose a relatively large mixed industrial and agricultural territory in Central Europe with a negligible migration. Differences in CHD incidence have been reported within various ethnic groups [9, 14, 15, 20]. Nonetheless, it seems reasonable to expect that our data on survival probability can be used also for other populations. The probability of survival of children born with CHD was slightly higher in our country than that reported in other studies. A minimal difference was found in the final number of survivors at the age of 15. It was most pronounced in the early postnatal period. The slope of the survival curve after the first week of life matches that reported by others [2, 4, 12, 13], and probably more reliable than some [10, 111. An improvement in general perinatal and infant care may well have influenced favorably the mortality rate of our series compared with earlier studies. The mean neonatal mortality rate in the given region was 15.1 per 1000 livebirths. It decreased from 19.0 in the early 1950s to 11.9 in 1977. The infant mortality was 30 per 1000 livebirths in the first 3 years of the study, dropping to below 20 per 1,000 in 197I. In the last 2 years of the study it was 16.2 and 15.7. The mean value from 1952 through 1978 was 21.0 per I000 livebirths. However, a decrease in overall neonatal mortality from 17.3 per 1000 livebirths in the first 5 years of the study to 13.6 per 1000 in the last 5 years did not influence the mortality rate for children with CHD. The proportion of the cardiac to the total child death rate rose from 4.7% in the first years to 7.8% in the last 5 years of the study. Cardiovascular surgery has profoundly changed the natural course of CHD. We tried to eliminate
157
this effect by choosing a period of study which preceded the surgical era in a given region. However, balloon atrial septostomy, introduced in 1967, could have postponed the fatal outcome of simple TGA and change its survival curve in the early postnatal life. Yet separate calculation of the survival curve before and after the introduction of balloon septostomy did not reveal any significant difference. Surgical intervention could favorably influence the survival curve in PDA. However, the operation was done only sporadically in infants. Thus, the survival curve in infancy reflects more the natural course of the disease than the course in a properly treated child with isolated PDA. A similar, but even less pronounced, influence of surgery on the natural course of the disease holds for TF. Until 1968, anastomosing operations were done only in older children. A small number of children less than 4 years of age had this operation in the last few years of the study. Cardiac surgery has markedly changed the course and outcome of CHD, its worldwide availability has eliminated any further possibility to follow-up the natural course and unconditional prognosis of CHD. Data on unconditional survival, however, remain indispensable in answering the questions posed most frequently by the family of an affected child, and they play an important role in planning the strategy of medical and surgical treatment. Recently, rapid progress in the fetal diagnosis of heart defects has accentuated the need to know the natural course of CHD. Such information is important when discussing possible intervention during pregnancy. It also aids in organizing special care, even before the delivery, and immediately after birth. References 1. And61 J (1978) Mathematical statistics. SNTL, Praha (in Czech) 2. Carlgren LE (1959) The incidence of congenital heart disease in children born in Gothenburg 1941-1950. Br Heart J 21:40 3. Collet RW, Edwards JE. Persistent truncus arteriosus: A classification according to anatomic types. Surg Clin N Am 29:1245
4. Feldt RH, Avasthey P, Yoshimasu F, Kurland LT, Titus JL (1971) Incidence of congenital heart disease in children born to Residents of Olmsted Country, Minnesota, 1950-1969. Mayo Clin Proc 46:794 5. Grunkemeier GL, Starr A (1977) Acturial analysis of surgical results: Rationale and method. Ann Thorac Surg 24:404 6. Hay JD (1966) Population and clinic studies of congenital heart disease in Liverpool. Br Med J 2:661 7. Hoffman JIE, Christianson R (1978) Congenital heart disease in a cohort of 19 502 births with long-term follow-up. Am J Cardiol 42:641 8. Liebman J, Cullum L, Belloc NB (1969) Natural history of
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transposition of the great arteries. Anatomy and birth and death characteristics. Circulation 40:237 Lien Wen-Pin, Chen Jin Jer, Chen Jyh-Hong, Lin Jiunu-Lee, Jesuh Yen-Yuw, Wu Teh-Lu, Chu Su-Hsun, Hung Chi-Ren (1966) Frequency of various congenital heart diseases in chinese adults: Analysis of 926 congestive heart diseases in chinese adults: Analysis of 926 congestive patients over 13 years of age. A m J Cardiol 57:840 MacMahon B, McKeown T, Record RG (1952) The incidence and life expectation of children with congenital heart disease. Br J Soc Med 6:121 McKeown T, Record RG (1960) Malformations in a population observe for five years after birth. In: Wolstenholme GEW, O'Connor CM (eds) Ciba Foundation Symposium on congenital malformations. Little, Brown and Co, Boston, p. 2 Mitchel SC, Korones SB, Berendes HW (1971) Congenital heart disease in 56 109 births. Circulation 43:323 Mustacchi P, Sherins RS, Miller MJ (1963) Congenital malformations of the heart and the great vessels. JAMA 183:241
14. Neel JV (1958) Study of major congenital defects in Japanese infants. A m J Hum Genet 10:398 15. Ongley PA. Pediatric Cardiology in Thailand. Circulation 34:1
16. 5)amfmek M, Goetzov~i J, Bene~ov~t D (1985) Distribution of congenital heart malformations in an autopsied child population. Int J Cardiol 8:235 17. 5]am~inek M, Goetzov~l J, Benegovfi D (1986) Causes of death in neonates born with a heart malformation. Int J Cardiol 11:63 18. ~am~inek M, Benegovfi D, Goetzovfi J, Hrycejovfi I (1988) Distribution of age at death in children with congenital heart disease who died before the age of 15. Br Heart J 59:581 19. ~am~mek M, Slavfk Z, Zbo~ilovfi B, Hrobofiovft V, Vo~f~kov~ M, ~kovrgmek J (1989) Prospective study on incidence, treatment and outcome of heart diseases in 91 823 live born children. Pediatr Cardiol 10:580 20. Wallooppillai NJ, Jayasinghe MdeS (1970) Congenital heart disease in Ceylon. Br Heart J 32:304
