Scandinavian Journal of Thoracic and Cardiovascular Surgery
ISSN: 0036-5580 (Print) (Online) Journal homepage: http://www.tandfonline.com/loi/icdv19
Congenital Heart Disease in the First Month of Life H. Bækgaard Laursen & P. Lomholt To cite this article: H. Bækgaard Laursen & P. Lomholt (1979) Congenital Heart Disease in the First Month of Life, Scandinavian Journal of Thoracic and Cardiovascular Surgery, 13:2, 111-118, DOI: 10.3109/14017437909100975 To link to this article: http://dx.doi.org/10.3109/14017437909100975
Published online: 12 Jul 2009.
Submit your article to this journal
Article views: 2
View related articles
Full Terms & Conditions of access and use can be found at http://www.tandfonline.com/action/journalInformation?journalCode=icdv19 Download by: [University of California, San Diego]
Date: 20 April 2016, At: 22:04
Scand J Thor Cardiovasc Surg 13: I 11-1 18, 1979
CONGENITAL HEART DISEASE IN THE FIRST MONTH OF LIFE H. Bzkgaard Laursen and P. Lomholt From the Depurtrnents of Curdiology ctnd Poedicctrics, Universify Hospital, t h u s , Denmark
Downloaded by [University of California, San Diego] at 22:04 20 April 2016
(Submitted for publication May 22, 1978)
Abstract. During the years 1963-73, 276 children with congenital heart disease were admitted to this hospital during their first month of life. Ventricular septal defect was the most common cardiac anomaly and this lesion, together with transposition of the great arteries, comprised 35 ‘30of all cardiovascular malformations. Extracardiac malformations were found in 86 patients. The cumulative survival rate for all patients was 66% in the first month of life and 33% in the first year. Forty-three patients were operated upon, but it is estimated from necropsy reports and available clinical data that another 74 patients, who died without operation, would have been suitable candidates for total corrective surgery.
The present study, including infants with congenital heart disease hospitalized during the first month of life, was undertaken: I ) to describe the nature of heart defects found in this period of life, 2) to determine the mortality and causes of death, and 3) to estimate whether cardiac surgery could have been performed in those children who died of heart disease. Present study The clinical study includes 276 infants hospitalized during the first month of life from a total of 1504 children with congenital heart disease in the age group 0-15 years. The patients were hospitalized in the Department of Paediatrics, and heart investigations were performed in the Department of Cardiology, University Hospital, Arhus, Denmark, during the years 1963-73. Table I shows that congenital heart disease was the main reason for admission to hospital in 64% of the cases. In some of the remainder, congenital heart disease might have been present, but other illness obscured the cardiac anomaly. Heart catheterization was performed in 101 patients and 127 cases were classified by necropsy
without previous heart catheterization (Table 11). Forty-eight patients underwent only clinical investigation. Natrire and frequency of‘ cardiac mutformutions The incidence of various major cardiovascular abnormalities is shown in Table 111. The largest group is ventricular septal defect (19.2%), followed by transposition of the great arteries (15.2%), coarctation of the aorta (12.7%) and persistent ductus arteriosus (8.3 %). Those four malformations made up 55.4%. In most cases the major cardiac malformations were accompanied by other cardiac lesions, as shown in Table IV. Extructrrdiuc mrrlformutions Additional congenital malformations were encountered in 89 patients. Minor anomalies, such as small naevi, strabism and small umbilical herniae, were disregarded. Table V summarizes the extracardiac abnormalities. Sixty-eight patients had 1 malformation each, 19 patients had 2, and 2 patients had 3 malformations each. Thirty cases of chiomosomal abnormalities included 24 cases of Down’s syndrome, 4 cases of Trisomy E (16-18), one case of Turner’s syndrome and one Cri-du-Chat syndrome. Mortality Life expectancy tables up to 10 years were made for all patients (Fig. 1 and Table VI). Fifty-nine per cent of all the patients died within the first 3 months of life and after 12 months the cumulative survival was 33 %. Only a few deaths occurred after the first year and none were recorded after the age of 6 years. Ventricular septal defect, transposition of the great arteries and complicated coarctation of the
112
H . Bakgaard Laursrn and P . Lornliolt
Table I. Reasons for admissions to hospital n
Congenital heart disease Neonatal icterus
Cyanosis
Malnutrition, vomiting
Downloaded by [University of California, San Diego] at 22:04 20 April 2016
Chromosomal anomaly Extracardiac congenital malformation Pneumonia Other causes
(%I
179 (64) 10 (4) 16 (6) 5 (2) 19 (7) 22 (8) 1 1 (4) 14 ( 5 ) 276 (100)
aorta are responsible for 40% of all deaths during the first year of life.
Causes ofdeath The causes of death are summarized in Table VIII. Most patients died of cardiac failure. In some of the other cases, cardiac failure was probably a contributory factor.
catheterization was postponed in most cases. Seventy-four patients were reckoned to be suitable for complete corrective surgery, without other illness rendering operation irnadvisable (Table IX). Operation was not considered in 44 patients because of other illness (Table IX). Thus, in 25 cases, death was caused by infections and in 18 cases either by extracardiac congenital malformations or by complications to surgery of defects in the gastro-intestinal tract. One of the 44 infants suffering from coarcation of the aorta had a birth weight below 1400 g and died on the second day of life. Patients with hypoplastic left heart syndrome, tricuspid atresia and cardiomyopathia were considered inoperable. In addition, several patients with otherwise corrective lesions were found inoperable because of complicating heart defects (Table 1X). Two patients with coarctation of the aorta proved to be inoperable, one because of additional aortic stenosis and fibro-elastosis, the other because of a hypoplastic left ventricle. One patient with Tetralogy of Fallot was not considered for surgery because of very hypoplastic pulmonary arteries in association with atresia of the pulmonary valve. Another patient with Tetralogy of Fallot had an ectopic heart placed partially in an omphalocele. In one patient a common atrioventricular canal was combined with a hypoplastic left ventricle and aortic stenosis. Three patients with truncus arteriosus were considered inoperable because of combination with single ventricle. None of the patients with aortic stenosis were considered operable, 3 because of severe fibro-elastosis, in one combined with small, thickened aortic cusps. The fourth was complicated by a hypoplastic mitral valve and a ventricular septal defect. The one patient with double outlet right ventricle had a hypoplastic aorta. Patients with single ventricle were considered inoperable when the atrioventricular valves were defective or one of the main arteries was hypoplastic.
Operability and surgery Forty-three patients underwent surgery, with a combined primary and late mortality of 58% (Table IX). The patients operated on for ventricular septa1 defect had a banding operation with a high mortality. The 7 surviving patients with transposition of the great arteries had a Rashkind septostomy in the first days of life in 5 cases followed by a Mustard operation. Two of the 9 patients who died with transposition of the great arteries also had a Mustard operation, while 7 patients were only submitted to the Rashkind procedure. The patients with Tetralogy of Fallot had palliative operations7 had Blalock shunts and one a pulmonary valvulotomy. The high mortality rate was mainly caused by very hypoplastic pulmonary arteries. The patients with pulmonary valvular stenosis underwent valvulotomy, as did the patient with pulmonary valve atresia. No shunt operation was performed in this last-mentioned patient. From necropsy reports and available clinical data, we Table II. Numbers of heart cutheterizations, have tried to determine whether those patients who necropsies and clinical investigations died without operation might have been saved by n % cardiac surgery as is the case today. Only the possibility of complete correction has been consid- Heart catheterization 101 36.6 ered and only patients who were not inoperable for Necropsy without previous heart catherization 127 46.0 other reasons were taken into account. The esti- Clinical investigation 48 17.4 mate of operability has not been tried in the group 276 100.0 of patients surviving without operation, as heart Scand J Thor Cordiovasc Surg 13
Congenital heart disease in neonates
I13
Table 111. Incidence of' curdiovascular mrilformations in 276 children uith cotigenitcil heart di.tea.w cidmitted to hospital in the first month of life
Downloaded by [University of California, San Diego] at 22:04 20 April 2016
Sex Major cardiovascularmalformation
n
Ventricular septal defect Transposition of great arteries Coarctation of the aorta Persistent ductus arteriosus Underdevelopment of left ventricle Tetralogy of Fallot Single ventricle with transposition of great arteries: 2 with normal great arteries: 13 Pulmonary stenosis Pulmonary atresia with intact ventricular septum Common atrioventricular canal Tricuspid valve atresia with transposition of great arteries: 3 with normal great arteries: 3 Truncus arteriosus Atrial septal defect Aortic stenosis Anomalous pulmonary venous drainage Double outlet right ventricle Cardiomyopathia Ebstein's anomaly of tricuspid valve Cardiac tumour Pulmonary artery stenosis Aortopulmonary window No diagnosis
Thus, 117 of 212 patients were either operated on or considered suitable for surgery (those patients alive without operation excluded).
DISCUSSION This series is a selected one and consists of all infants with heart defects, who were hospitalized in the first month of life. Most of the children had symptoms of the cardiac defect, but in some the heart disease was of minor importance. This was particularly true for most of the 45 patients alive, who had not been submitted to heart catheterization-22 diagnosed as ventricular septal defect, 4 as coarctation of the aorta, 3 as persistent ductus arteriosus, 2 as pulmonary stenosis and 14 with uncertain diagnosis. The cardiac anomalies diagnosed in this age group are complicated by other cardiac lesions in most cases (Table IV). In some anomalies co-existing lesions are obligatory, for example abnormal arteriovenous communications in atresia of the 8-7929 18
6
0
53 (19.2) 42 (15.2) 35 (12.7) 23 (8.3) 19 (6.9) 16 (5.8) 15 (5.4)
29 29 17
24
12
13 18 10 4 5 3
14
(5.1)
10
4
6 6 6
(2.1) (2.1) (2.1)
5 4
5 4 4
(1.8) (1.5)
3
(1.1) (0.7) (0.7) (0.4) (0.4) (0.4) (0.4) (6.2)
12
5
276(100)
174
102
(%)
(1.5)
2 2 1 1 1 1
17
13 15
II
I
3
2 3
2 2 2
3 2 2
3 I
1
2
I 1 1
I
tricuspid valve, but also anomalies found as isolated defects in older children are most often complicated in these infants. Among the infants submitted to heart catheterization or necropsy, coarctation of the aorta was not found as an isolated anomaly in a single case in this series, and ventricular septal defect was complicated in 61 c/o of the cases. The fact that several cardiac malformations were often found in the same infant sometimes made classification in major and co-existing cardiovascular malformations difficult. Nevertheless, the incidence in this series with regard to the more common cardiovascular anomalies is in accordance with the incidence in the series of Rowe & Mehrizi (1968) comprising infants hospitalized during the first month of life, and with the incidence in the study by Coleman (1965, 1969), including symptomatic infants with congenital heart disease hospitalized during the first year of life. A series comprising infants submitted to heart catheterization during the first week of life has qiute another distribution of cardiac anomalies (Miller,
114
H . Biekguurd Laursen und P. Lomholt
Table IV. Co-existing cardiovascular malformations in 228 of 276 patients
Downloaded by [University of California, San Diego] at 22:04 20 April 2016
Only those patients in whom the nature of cardiac abnormalities was determined by heart catheterization or necropsy, are included in the Table PDA: Persistent ductus arteriosus, VSD: Ventricular septal defect. ASD: Atrial septal defect. PS: Pulmonary stenosis. AS: Aortic stenosis Major cardiovascular malformation
Num- Isober kdted
Ventricular septal defect Transposition of great arteries Coarctation of the aorta Persistent ductus arteriosus Underdevelopment of left ventricle Tetralogy of Fallot Single ventricle Pulmonary stenosis Pulmonary atresia with intact ventricular septum Common atrioventricular canal Tricuspid valve atresia Truncus arteriosus Atrial septal defect Aortic stenosis Anomalous pulmonary venous drainage Double outlet right ventricle Ebstein's anomaly of tricuspid valve Cardiac tumour Pulmonary artery stenosis Aortopulmonary window Cardiomyopat hia
31 42 31 20 19 16 15 12 6 6 6 5 4 4 3 2 1 1 1
12 8
PDA
VSD
13 21 26
12 11
16
2
5 8
PS
AS
1
2
1 1
1
6 4 2 1
5 7
9 1
8
6
6 6 3 1 2 4 3 2
2
6 10 8 3 11
1 8
17 7 13 2
6
ASD
Other ma.lformations
6 2
4 3
1 1
2
12
5
1 3 1 4 1 1
1 3 2 1
1 1
I 2
I
228
50
1
1
126
42
78
39
1974), representing those congenital heart defects giving serious symptoms in the first days of life, the leading anomaly being transposition of the great arteries, followed by pulmonary atresia with intact ventricular septum or common ventricle, hypoplastic left heart syndrome and coarctation of the aorta. As stated by others (Rowe & Mehrizi, 1968; Carlgren, 1959; Landtman, 1971; Mitchell et al.,
Table V. Extrtrcurdiuc congenital m a ~ o r m u t i o n si n 276 infiint.5 ulith congenital Iieurt diseaw admitted t o liospital during the first month of'lge Chromosomal anomaly Gastro-intestinal tract Urogenital system Lungs Skeleton, muscles, skin Nervous system, sense organs
30 cases 30 17 1
22 12 112
2
1 AGE
2
3
4
5
6
7
8
3 4
5
6
7
8
9 10
9101112
YEARS
Fig. i . Cumulative survival. All 276 patients: 0-0.Patients investigated by heart catheterization (101 cases):
A-A. Patients who died without previous heart catheterization (127 cases): 0-0.
Congenital heart disease in neonates
115
Downloaded by [University of California, San Diego] at 22:04 20 April 2016
Table VI. Life expectancy table f o r 276 children with congenital heurt diseuse admitted to hospital in the first month of life (patients operated upon also included) Age interval (months)
Alive at start of interval
0-1
276 183 127 112 I04 100 97 91 89 89 85 82 81 59 52 38 32 26 19 14 6
1-2 2-3 3 4 4-5 5-6 6-7 7-8 8-9 9-10 10-1 1 11-12 12-24 24-36 36-48 48-60 60-72 72-84 8496 9 6 I on 108-120
Live withdrawals during interval
I 0 4 0 0 0 2 1
16 7 11
6 4 7 5 8 2
At risk during interval
Deaths during interval
Death rate
Survival rate
Cumulative survival rate
276.0 182.0 126.5 111.5 103.5 100.0 95.0 91.0 89.0 89.0 84.5 81.5 73.0 55.5 46.5 35.0 30.0 22.5 16.5 10.0 5.0
93 54 14 7 3 3 2 2 0 4 I 0 6 0 3 0 2 0 0 0 0
0.34 0.30 0.11 0.06 0.03 0.03 0.02 0.02 0.00 0.05 0.01 0.00 0.08 0.00 0.07 0.00 0.07 0.00 0.00 0.00 0.00
0.66 0.70 0.89 0.94 0.97 0.97 0.98 0.98 1.00 0.95 0.99 1.00 0.92 1.00 0.93 1.00 0.93 1.00 1.00 1.00
I .oo 0.66 0.46 0.41 0.39 0.38 0.37 0.36 0.35 0.35 0.33 0.33 0.33 0.30 0.30 0.28 0.28 0.26 0.26 0.26 0.26
1.00
Table VII. The incidence of congenitd cardiovascular mrilformations and corresponding deuth rates .for the first year qf life f o r 276 children with congenital heart disease admitted ro hospital during the ,first month of life Death rates according to Campbell (1973) describing the natural history are shown for comparison Major cardiovascular malformation Ventricular septal defect Transposition of great arteries Coarctation of the aorta Persistent ductus arteriosus Underdeveloped left ventricle Tetralogy of Fallot Single ventricle Pulmonary stenosis Pulmonary atresia with intact ventricular septum Common atrioventricular canal Tricuspid atresia Truncus arteriosus Atrial septal defect Aortic stenosis Anomalous pulmonary venous drainage Double outlet right ventricle Ebstein’s anomaly Cardiac turnour Pulmonary artery stenosis Aortopulmonary window Cardiomyopathia No diagnosis
Death rate
Campbell (1973)
53 42 35 23 19 16 15 14 6 6 6 5 4 4 3 2
0.37 0.83 0.83 0.69 I .oo 0.63 I .OO 0.35 I .oo 1.OO
0.36 0.89 0.61 0.23
1 1 1 1
0.00 1.oo 0.00 0.00 1 .OO 0.19
Number
2 17 276
1
0.53 0.24
.oo
0.80 0.75
1 .oo 1 .oo 1
.oo
0.86 0.36 0.29
I 16
H . Bzhgcicird Lciirrsrn cind P . Loiirliolt
Table VIII. Cciirsrs cfrleatli in 194 neontitrs ,fi.oiii 276 infrints l t 3 i i congenital iieart clisrcise h o s pitrilized during the.fir.st month oflfo Cardiac failure Pulmonary infections Other infections Thrombo-embolism Operation Extracardiac congenital malformations Other causes
131
29 10 1
8 10
5
Downloaded by [University of California, San Diego] at 22:04 20 April 2016
194
1971), extracardiac congenital malformations are very common in children showing cardiac symptoms in the first months of life. In this study 32% had one or more other malformations (Table V ) . Children with congenital heart disease usually show a male preponderance (Landtman, 1971: h e n a s h e et al., 1967) and our results confirm earlier observa-
tions (Table 111). Like others, we found transposition of the great arteries (Campbell, 1968) and Tetralogy of Fallot (Rowge & Mehrizi, 1968: Campbell, 1968) most common among boys. Coarctation of the aorta usually shows a male preponderance (Campbell & Polani, 1961), but in this series the sex distribution was almost equal. Persistent ductus arteriosus dominated in males among our patients, contrary to the findings in many earlier investigations (Rowe & Mehrizi, 1968; Keith et al., 1958). Published figures on mortality vary with the selection of patients. Death rates in the first year of life ranged from 8 6 % in a necropsy study based on 777 children dying in hospital (Landtman, 1971) to 2 4 % for 450 children in a population study (Carlgren, 1969). Rowe & Mehrizi (1968) found that 34 % of 100 consecutive neonates with congenital heart disease died in the first month of life between 1960 and 1963 in New Zealand, while 52% died within the first year. The corresponding figures
Table IX. Assrssnietit of oprrnhilitv The first column shows the figures for those infants who died without operation and who might have been totally corrected by surgery as is the case today. The next two columns illustrate those infants who died and were not considered amenable to radical surgery (the possibility of surgery in those infants alive without operation is not taken into account, last column)
Major cardio-vascular malformation
Operable
Operation not indicated because of other illness
Ventricular septal defect I0 Transposition of great arteries 23 Coarctation of the aorta 17 Persistent ductus arteriosus 3 Underdeveloped left ventricle Tetralogy of Fallot 3 Single ventricle 4 Pulmonary stenosis 2 Pulmonary atresia with intact ventricular septum 5 Common atrioventricular canal 2 Tricuspid atresia Truncus arteriosus Atrial septal defect 1 Aortic stenosis Anomalous pulmonary venous drainage 3 Double outlet right ventricle 1 Ebstein's anomaly Cat-diactumour Pulmonary artery stenosis Cardiomyopathia Aortopulmonary window No diagnosis
11
14
44
2 8 12
Alive with-
Operation undertaken Inoperable
out
alive
dead
2 7
4
operation
9 2
26 1 6 6
1
7
3
5
1
4
2 2 19
2 11
2
1
3
1
1
5 3
1 1
2
1
4 1 1
1 1
2 1
3
14
51
18
25
64
Downloaded by [University of California, San Diego] at 22:04 20 April 2016
Congenital heart disease in neonates
from our study are 34 % for the first month and 67 % for the first year. Almost all deaths occur in the first year of life and death rates for this period are shown in Table VII. The death rates would have been still higher if this study had included only patients with symptomatic heart disease, but we have also included children with congenital heart disease hospitalized in the first month of life for other illness. Campbell (1973) summarized the percentage mortality, mainly during the first year of life, for different cardiac malformations based on four population series (Carlgren, 1959, 1969; MacMahon et al., 1953; Hay, 1966). Campbell’s figures are shown for comparison in Table VII and he emphasizes that his figures express the natural history of cardiac malformations, contrary to this series which is a selected clinical material including only patients hospitalized during the first month of life. The estimate that 117 of 212 infants would have been suitable for corrective surgery as judged by present-day criteria is of course attended by uncertainty, but the possibilities of surgery in small infants are growing (Edmunds et al., 1972; Hazan et al., 1974: Hayes et al., 1974; Castanedaet al., 1974: Malm et al., 1974; Edie et al., 1973; Ionescu et al., 1973). The mortality for banding operation in ventricular septal defect is stated to be 30-23% (Hunt et al., 1971; Olsen, 1972), but as high as 50% in the first month of life (Hunt et al., 1971). We found a higher mortality in our patients. In several centres complete correction of ventricular septal defect in small infants is performed with a smaller mortality (Castaneda et al., 1974; Weidman & DuShane, 1974). The outlook for infants with complete transposition of the great arteries has improved considerably since the introduction of septostomy and Mustard’s operation (Castaneda et al., 1974; Breckenridge et al., 1972). Miller (1974) found that the survival rate during the first year of life for transposition of the great arteries improved from 0.45 in 1970 to 0.80 in 1972. The high mortality in our series can probably be explained by a belated second operation; the primary improvement after Rashkind’s procedure is not followed fast enough by either a Blalock-Hanlon or a Mustard’s operation if the condition of the child is deteriorating. The mortality for coarctation of the aorta causing symptoms in the first months of life is very high. Surgery has been performed in this age group with a mortality of 27-30% (Hazan et al.,
117
1974; Connors et al., 1974). Surgical treatment of persistent ductus arteriosus has been performed with a slight risk, even in premature infants suffering from respiratory distress (Edmunds et al., 1972: Hazan et al.. 1974). Thus the four heart diseases which comprise the bulk of all cardiac lesions in the first month of life are amenable to surgery in early infancy with a mortality which, though high, is yet less than that without operation. Concerning the other, more rare cardiac anomalies, there are reports of improving surgical results (Hayes et al., 1974; Malm et al., 1974; Higashino et al., 1974; Poirier et al., 1975; Edie et al., 1973; Ionescu et al., 1973). In this series, heart catheterization was performed in an increasing number of patients during the period of investigation, but still only in a minority of patients because of a somewhat pessimistic attitude to the surgical possibilities. Fig. 1 shows that the investigation was performed particularly in those patients with the best outlook, while seriously ill infants and those suffering from extracardiac anomalies were excluded from heart catheterization to a great extent. With the improved surgical possibilities, a more aggressive attitude would seem justified, especially as heart catheterization can be performed in such infants with few complications in experienced hands.
REFERENCES Breckenridge, I . M . , Stark, J . , Bonham-Carter, R. E., Oelert, H., Graham, G. R. & Waterston, D. J . 1972. Mustard’s operation for transposition of the great arteries. Luncet i , 1140. Campbell, M. 1968. The incidence and later distribution of malformations of the heart. in Paediutric Cardiology (ed. Hamish Watson). Lloyd-Luke, London. - 1973. Incidence of cardiac malformations at birth and later. Brit Heart J 3 5 , 189. Campbell, M. & Polani, P. E. 1961. The aetiology of coarctation of the aorta. Lancet i , 463. Carlgren, L. E. 1959. The incidence of congenital heart disease in Gothenberg, 1941-50. Brit Heurt J 2 1 , 40. - 1969. The incidence of congenital heart disease in Gothenberg. Proc Ass European Paediat Cardiol5, 2. Castaneda, A. R., Lamberti, J., Sade, R. M., Williams, R. G. & Nadas, A . 1974. Open-heart surgery during the first three months of life. J Tliorac Cardiovusc Surg 68, 719.
Coleman, E. C. 1965. Serious congenital heart disease in infancy. Brit Heart J 27, 42. - 1969. Progress report on infants with serious cardiac malformations. Brit Heart J 3 / , 441, S c m d J Tlior Curdiovasc Sirrg 13
Downloaded by [University of California, San Diego] at 22:04 20 April 2016
118
H . Bzkgciurd Lrrirrsrti
Litid
P . Lomliolt
Connors, J. P., Hartman, A . F. & Weldon, C. S . 1974. Considerations in the surgical management of infantile coarctation of the aorta. Amer J Cardiol36, 489. Edie, R. N., Ellis, K., Gersony, W. M., Krongrad, E., Bowman, F. 0. & Malm, J. R. 1973. Surgical repair of single ventricle. J Thorcic Cardiovasc Surg 66, 350. Edmunds, L. H., Fishman, N. H., Gregory, G. A . , Heyman, M. A., Hoffman, J. I . E., Robinson, S. J., Roe, B. B., Rudolph, A . M. & Stanger, A . M. 1972. Cardiac surgery in infants less than 6 weeks of age. Circulation 66, 250. Hay, J. D. 1966. Population and clinic studies of congenital heart disease in Liverpool. Brit Med J i i , 661. Hayes, C. L., Gersony, W. M., Griffiths, S. P., Steeg, C. N . , Bowman, F. 0. & Malm, J . R. 1974. Results of correction of total anomalous pulmonary venous connections in infancy. Adv Cardiol 1 1 , 36. Hazan, E., Lessana, A . , Parrot, A . M., Louville, Y., Rioux, CI., Chetochine, F., Neveux, J. Y . , Lemoine, G. & Mathey, J. 1974. La chirurgie cardiaque dans le premier mois de la vie. La Nouvelle Presse Medicnle 3, 1135. Higashino, S. M., Shaw, G. G., May, I. A . & Ecker, R. R. 1974. Total anomalous pulmonary venous drainage below the diaphragm.J Thorac Cardiovasc Surg 68, 71 I . Hunt, C. E., Formanek, G., Levine, M. A , , Castaneda, A . & Moller, J. H. 1971. Banding of the pulmonary artery. Circulation 63, 395. Ionescu, M. I., Fergus, J., MacCartney, F. V. & Wooler, G. H . 1973. Intracardiac repair of single ventricle with pulmonary stenosis. J Thorac Cardiowsc Surg 65. 602.
Keith, J. D., Rowe, R. D. & Vlad, P. 1958. Heart Diseuse in Infancy and Childhood. Macmillan, New York. Landtman, B. 1971. Clinical and morphological studies in congenital heart disease. Acta Paediut Srand, Suppl. 213, 3 . MacMahon, B., McKeown, T. & Record, R. G. 1953. The incidence and life expectation of children with congenital heart disease. Brit Hear? J 15, 121. Malm, J . R., Bowman, F. O . , Hayes, C. J., Gersony, W. M. & Ellis, K. 1974. Results of surgical treatment of pulmonary atresia with intact ventricular septum. Ad\, Cardiol t i , 18. Menashe, V . D., Osterud, H. T. & Griswold, H . E. 1967. Mortality from congenital heart disease in Oregon. Pediatrics 40. 334. Miller, G. A. H. 1974. Congenital heart disease in the first week of life. Brit Heart J 36, 1160. Mitchell, S . C., Korones, S. B. & Berendes, H. W. 1971. Congenital heart disease in 56 109 births, incidence and natural history. Circulation 43, 323. Olsen, J. E. 1972. Pulmonary artery banding. A pre- and postoperative catheterization study. Srand J Thor Crtrdiovasr Surg 6 , 13. Pokier, R. A , , Berman, M. A . & Stansel, H. C. 1975. Current status of the surgical treatment of truncus arteriosus. J Thorac Cardiovasc Surg 69, 169. Rowe, R. D. & Mehrizi, A. 1968. The Neonate with Congenital Heart Disease. Saunders, PhiladelphiaLondon - Toronto. Weidman, W. H. & DuShane, J. W. 1974. Course of pulmonary hypertension following surgical closure of ventricular septa1 defects. Adv Cardiol I I , 131.
ISSN: 0036-5580 (Print) (Online) Journal homepage: http://www.tandfonline.com/loi/icdv19
Congenital Heart Disease in the First Month of Life H. Bækgaard Laursen & P. Lomholt To cite this article: H. Bækgaard Laursen & P. Lomholt (1979) Congenital Heart Disease in the First Month of Life, Scandinavian Journal of Thoracic and Cardiovascular Surgery, 13:2, 111-118, DOI: 10.3109/14017437909100975 To link to this article: http://dx.doi.org/10.3109/14017437909100975
Published online: 12 Jul 2009.
Submit your article to this journal
Article views: 2
View related articles
Full Terms & Conditions of access and use can be found at http://www.tandfonline.com/action/journalInformation?journalCode=icdv19 Download by: [University of California, San Diego]
Date: 20 April 2016, At: 22:04
Scand J Thor Cardiovasc Surg 13: I 11-1 18, 1979
CONGENITAL HEART DISEASE IN THE FIRST MONTH OF LIFE H. Bzkgaard Laursen and P. Lomholt From the Depurtrnents of Curdiology ctnd Poedicctrics, Universify Hospital, t h u s , Denmark
Downloaded by [University of California, San Diego] at 22:04 20 April 2016
(Submitted for publication May 22, 1978)
Abstract. During the years 1963-73, 276 children with congenital heart disease were admitted to this hospital during their first month of life. Ventricular septal defect was the most common cardiac anomaly and this lesion, together with transposition of the great arteries, comprised 35 ‘30of all cardiovascular malformations. Extracardiac malformations were found in 86 patients. The cumulative survival rate for all patients was 66% in the first month of life and 33% in the first year. Forty-three patients were operated upon, but it is estimated from necropsy reports and available clinical data that another 74 patients, who died without operation, would have been suitable candidates for total corrective surgery.
The present study, including infants with congenital heart disease hospitalized during the first month of life, was undertaken: I ) to describe the nature of heart defects found in this period of life, 2) to determine the mortality and causes of death, and 3) to estimate whether cardiac surgery could have been performed in those children who died of heart disease. Present study The clinical study includes 276 infants hospitalized during the first month of life from a total of 1504 children with congenital heart disease in the age group 0-15 years. The patients were hospitalized in the Department of Paediatrics, and heart investigations were performed in the Department of Cardiology, University Hospital, Arhus, Denmark, during the years 1963-73. Table I shows that congenital heart disease was the main reason for admission to hospital in 64% of the cases. In some of the remainder, congenital heart disease might have been present, but other illness obscured the cardiac anomaly. Heart catheterization was performed in 101 patients and 127 cases were classified by necropsy
without previous heart catheterization (Table 11). Forty-eight patients underwent only clinical investigation. Natrire and frequency of‘ cardiac mutformutions The incidence of various major cardiovascular abnormalities is shown in Table 111. The largest group is ventricular septal defect (19.2%), followed by transposition of the great arteries (15.2%), coarctation of the aorta (12.7%) and persistent ductus arteriosus (8.3 %). Those four malformations made up 55.4%. In most cases the major cardiac malformations were accompanied by other cardiac lesions, as shown in Table IV. Extructrrdiuc mrrlformutions Additional congenital malformations were encountered in 89 patients. Minor anomalies, such as small naevi, strabism and small umbilical herniae, were disregarded. Table V summarizes the extracardiac abnormalities. Sixty-eight patients had 1 malformation each, 19 patients had 2, and 2 patients had 3 malformations each. Thirty cases of chiomosomal abnormalities included 24 cases of Down’s syndrome, 4 cases of Trisomy E (16-18), one case of Turner’s syndrome and one Cri-du-Chat syndrome. Mortality Life expectancy tables up to 10 years were made for all patients (Fig. 1 and Table VI). Fifty-nine per cent of all the patients died within the first 3 months of life and after 12 months the cumulative survival was 33 %. Only a few deaths occurred after the first year and none were recorded after the age of 6 years. Ventricular septal defect, transposition of the great arteries and complicated coarctation of the
112
H . Bakgaard Laursrn and P . Lornliolt
Table I. Reasons for admissions to hospital n
Congenital heart disease Neonatal icterus
Cyanosis
Malnutrition, vomiting
Downloaded by [University of California, San Diego] at 22:04 20 April 2016
Chromosomal anomaly Extracardiac congenital malformation Pneumonia Other causes
(%I
179 (64) 10 (4) 16 (6) 5 (2) 19 (7) 22 (8) 1 1 (4) 14 ( 5 ) 276 (100)
aorta are responsible for 40% of all deaths during the first year of life.
Causes ofdeath The causes of death are summarized in Table VIII. Most patients died of cardiac failure. In some of the other cases, cardiac failure was probably a contributory factor.
catheterization was postponed in most cases. Seventy-four patients were reckoned to be suitable for complete corrective surgery, without other illness rendering operation irnadvisable (Table IX). Operation was not considered in 44 patients because of other illness (Table IX). Thus, in 25 cases, death was caused by infections and in 18 cases either by extracardiac congenital malformations or by complications to surgery of defects in the gastro-intestinal tract. One of the 44 infants suffering from coarcation of the aorta had a birth weight below 1400 g and died on the second day of life. Patients with hypoplastic left heart syndrome, tricuspid atresia and cardiomyopathia were considered inoperable. In addition, several patients with otherwise corrective lesions were found inoperable because of complicating heart defects (Table 1X). Two patients with coarctation of the aorta proved to be inoperable, one because of additional aortic stenosis and fibro-elastosis, the other because of a hypoplastic left ventricle. One patient with Tetralogy of Fallot was not considered for surgery because of very hypoplastic pulmonary arteries in association with atresia of the pulmonary valve. Another patient with Tetralogy of Fallot had an ectopic heart placed partially in an omphalocele. In one patient a common atrioventricular canal was combined with a hypoplastic left ventricle and aortic stenosis. Three patients with truncus arteriosus were considered inoperable because of combination with single ventricle. None of the patients with aortic stenosis were considered operable, 3 because of severe fibro-elastosis, in one combined with small, thickened aortic cusps. The fourth was complicated by a hypoplastic mitral valve and a ventricular septal defect. The one patient with double outlet right ventricle had a hypoplastic aorta. Patients with single ventricle were considered inoperable when the atrioventricular valves were defective or one of the main arteries was hypoplastic.
Operability and surgery Forty-three patients underwent surgery, with a combined primary and late mortality of 58% (Table IX). The patients operated on for ventricular septa1 defect had a banding operation with a high mortality. The 7 surviving patients with transposition of the great arteries had a Rashkind septostomy in the first days of life in 5 cases followed by a Mustard operation. Two of the 9 patients who died with transposition of the great arteries also had a Mustard operation, while 7 patients were only submitted to the Rashkind procedure. The patients with Tetralogy of Fallot had palliative operations7 had Blalock shunts and one a pulmonary valvulotomy. The high mortality rate was mainly caused by very hypoplastic pulmonary arteries. The patients with pulmonary valvular stenosis underwent valvulotomy, as did the patient with pulmonary valve atresia. No shunt operation was performed in this last-mentioned patient. From necropsy reports and available clinical data, we Table II. Numbers of heart cutheterizations, have tried to determine whether those patients who necropsies and clinical investigations died without operation might have been saved by n % cardiac surgery as is the case today. Only the possibility of complete correction has been consid- Heart catheterization 101 36.6 ered and only patients who were not inoperable for Necropsy without previous heart catherization 127 46.0 other reasons were taken into account. The esti- Clinical investigation 48 17.4 mate of operability has not been tried in the group 276 100.0 of patients surviving without operation, as heart Scand J Thor Cordiovasc Surg 13
Congenital heart disease in neonates
I13
Table 111. Incidence of' curdiovascular mrilformations in 276 children uith cotigenitcil heart di.tea.w cidmitted to hospital in the first month of life
Downloaded by [University of California, San Diego] at 22:04 20 April 2016
Sex Major cardiovascularmalformation
n
Ventricular septal defect Transposition of great arteries Coarctation of the aorta Persistent ductus arteriosus Underdevelopment of left ventricle Tetralogy of Fallot Single ventricle with transposition of great arteries: 2 with normal great arteries: 13 Pulmonary stenosis Pulmonary atresia with intact ventricular septum Common atrioventricular canal Tricuspid valve atresia with transposition of great arteries: 3 with normal great arteries: 3 Truncus arteriosus Atrial septal defect Aortic stenosis Anomalous pulmonary venous drainage Double outlet right ventricle Cardiomyopathia Ebstein's anomaly of tricuspid valve Cardiac tumour Pulmonary artery stenosis Aortopulmonary window No diagnosis
Thus, 117 of 212 patients were either operated on or considered suitable for surgery (those patients alive without operation excluded).
DISCUSSION This series is a selected one and consists of all infants with heart defects, who were hospitalized in the first month of life. Most of the children had symptoms of the cardiac defect, but in some the heart disease was of minor importance. This was particularly true for most of the 45 patients alive, who had not been submitted to heart catheterization-22 diagnosed as ventricular septal defect, 4 as coarctation of the aorta, 3 as persistent ductus arteriosus, 2 as pulmonary stenosis and 14 with uncertain diagnosis. The cardiac anomalies diagnosed in this age group are complicated by other cardiac lesions in most cases (Table IV). In some anomalies co-existing lesions are obligatory, for example abnormal arteriovenous communications in atresia of the 8-7929 18
6
0
53 (19.2) 42 (15.2) 35 (12.7) 23 (8.3) 19 (6.9) 16 (5.8) 15 (5.4)
29 29 17
24
12
13 18 10 4 5 3
14
(5.1)
10
4
6 6 6
(2.1) (2.1) (2.1)
5 4
5 4 4
(1.8) (1.5)
3
(1.1) (0.7) (0.7) (0.4) (0.4) (0.4) (0.4) (6.2)
12
5
276(100)
174
102
(%)
(1.5)
2 2 1 1 1 1
17
13 15
II
I
3
2 3
2 2 2
3 2 2
3 I
1
2
I 1 1
I
tricuspid valve, but also anomalies found as isolated defects in older children are most often complicated in these infants. Among the infants submitted to heart catheterization or necropsy, coarctation of the aorta was not found as an isolated anomaly in a single case in this series, and ventricular septal defect was complicated in 61 c/o of the cases. The fact that several cardiac malformations were often found in the same infant sometimes made classification in major and co-existing cardiovascular malformations difficult. Nevertheless, the incidence in this series with regard to the more common cardiovascular anomalies is in accordance with the incidence in the series of Rowe & Mehrizi (1968) comprising infants hospitalized during the first month of life, and with the incidence in the study by Coleman (1965, 1969), including symptomatic infants with congenital heart disease hospitalized during the first year of life. A series comprising infants submitted to heart catheterization during the first week of life has qiute another distribution of cardiac anomalies (Miller,
114
H . Biekguurd Laursen und P. Lomholt
Table IV. Co-existing cardiovascular malformations in 228 of 276 patients
Downloaded by [University of California, San Diego] at 22:04 20 April 2016
Only those patients in whom the nature of cardiac abnormalities was determined by heart catheterization or necropsy, are included in the Table PDA: Persistent ductus arteriosus, VSD: Ventricular septal defect. ASD: Atrial septal defect. PS: Pulmonary stenosis. AS: Aortic stenosis Major cardiovascular malformation
Num- Isober kdted
Ventricular septal defect Transposition of great arteries Coarctation of the aorta Persistent ductus arteriosus Underdevelopment of left ventricle Tetralogy of Fallot Single ventricle Pulmonary stenosis Pulmonary atresia with intact ventricular septum Common atrioventricular canal Tricuspid valve atresia Truncus arteriosus Atrial septal defect Aortic stenosis Anomalous pulmonary venous drainage Double outlet right ventricle Ebstein's anomaly of tricuspid valve Cardiac tumour Pulmonary artery stenosis Aortopulmonary window Cardiomyopat hia
31 42 31 20 19 16 15 12 6 6 6 5 4 4 3 2 1 1 1
12 8
PDA
VSD
13 21 26
12 11
16
2
5 8
PS
AS
1
2
1 1
1
6 4 2 1
5 7
9 1
8
6
6 6 3 1 2 4 3 2
2
6 10 8 3 11
1 8
17 7 13 2
6
ASD
Other ma.lformations
6 2
4 3
1 1
2
12
5
1 3 1 4 1 1
1 3 2 1
1 1
I 2
I
228
50
1
1
126
42
78
39
1974), representing those congenital heart defects giving serious symptoms in the first days of life, the leading anomaly being transposition of the great arteries, followed by pulmonary atresia with intact ventricular septum or common ventricle, hypoplastic left heart syndrome and coarctation of the aorta. As stated by others (Rowe & Mehrizi, 1968; Carlgren, 1959; Landtman, 1971; Mitchell et al.,
Table V. Extrtrcurdiuc congenital m a ~ o r m u t i o n si n 276 infiint.5 ulith congenital Iieurt diseaw admitted t o liospital during the first month of'lge Chromosomal anomaly Gastro-intestinal tract Urogenital system Lungs Skeleton, muscles, skin Nervous system, sense organs
30 cases 30 17 1
22 12 112
2
1 AGE
2
3
4
5
6
7
8
3 4
5
6
7
8
9 10
9101112
YEARS
Fig. i . Cumulative survival. All 276 patients: 0-0.Patients investigated by heart catheterization (101 cases):
A-A. Patients who died without previous heart catheterization (127 cases): 0-0.
Congenital heart disease in neonates
115
Downloaded by [University of California, San Diego] at 22:04 20 April 2016
Table VI. Life expectancy table f o r 276 children with congenital heurt diseuse admitted to hospital in the first month of life (patients operated upon also included) Age interval (months)
Alive at start of interval
0-1
276 183 127 112 I04 100 97 91 89 89 85 82 81 59 52 38 32 26 19 14 6
1-2 2-3 3 4 4-5 5-6 6-7 7-8 8-9 9-10 10-1 1 11-12 12-24 24-36 36-48 48-60 60-72 72-84 8496 9 6 I on 108-120
Live withdrawals during interval
I 0 4 0 0 0 2 1
16 7 11
6 4 7 5 8 2
At risk during interval
Deaths during interval
Death rate
Survival rate
Cumulative survival rate
276.0 182.0 126.5 111.5 103.5 100.0 95.0 91.0 89.0 89.0 84.5 81.5 73.0 55.5 46.5 35.0 30.0 22.5 16.5 10.0 5.0
93 54 14 7 3 3 2 2 0 4 I 0 6 0 3 0 2 0 0 0 0
0.34 0.30 0.11 0.06 0.03 0.03 0.02 0.02 0.00 0.05 0.01 0.00 0.08 0.00 0.07 0.00 0.07 0.00 0.00 0.00 0.00
0.66 0.70 0.89 0.94 0.97 0.97 0.98 0.98 1.00 0.95 0.99 1.00 0.92 1.00 0.93 1.00 0.93 1.00 1.00 1.00
I .oo 0.66 0.46 0.41 0.39 0.38 0.37 0.36 0.35 0.35 0.33 0.33 0.33 0.30 0.30 0.28 0.28 0.26 0.26 0.26 0.26
1.00
Table VII. The incidence of congenitd cardiovascular mrilformations and corresponding deuth rates .for the first year qf life f o r 276 children with congenital heart disease admitted ro hospital during the ,first month of life Death rates according to Campbell (1973) describing the natural history are shown for comparison Major cardiovascular malformation Ventricular septal defect Transposition of great arteries Coarctation of the aorta Persistent ductus arteriosus Underdeveloped left ventricle Tetralogy of Fallot Single ventricle Pulmonary stenosis Pulmonary atresia with intact ventricular septum Common atrioventricular canal Tricuspid atresia Truncus arteriosus Atrial septal defect Aortic stenosis Anomalous pulmonary venous drainage Double outlet right ventricle Ebstein’s anomaly Cardiac turnour Pulmonary artery stenosis Aortopulmonary window Cardiomyopathia No diagnosis
Death rate
Campbell (1973)
53 42 35 23 19 16 15 14 6 6 6 5 4 4 3 2
0.37 0.83 0.83 0.69 I .oo 0.63 I .OO 0.35 I .oo 1.OO
0.36 0.89 0.61 0.23
1 1 1 1
0.00 1.oo 0.00 0.00 1 .OO 0.19
Number
2 17 276
1
0.53 0.24
.oo
0.80 0.75
1 .oo 1 .oo 1
.oo
0.86 0.36 0.29
I 16
H . Bzhgcicird Lciirrsrn cind P . Loiirliolt
Table VIII. Cciirsrs cfrleatli in 194 neontitrs ,fi.oiii 276 infrints l t 3 i i congenital iieart clisrcise h o s pitrilized during the.fir.st month oflfo Cardiac failure Pulmonary infections Other infections Thrombo-embolism Operation Extracardiac congenital malformations Other causes
131
29 10 1
8 10
5
Downloaded by [University of California, San Diego] at 22:04 20 April 2016
194
1971), extracardiac congenital malformations are very common in children showing cardiac symptoms in the first months of life. In this study 32% had one or more other malformations (Table V ) . Children with congenital heart disease usually show a male preponderance (Landtman, 1971: h e n a s h e et al., 1967) and our results confirm earlier observa-
tions (Table 111). Like others, we found transposition of the great arteries (Campbell, 1968) and Tetralogy of Fallot (Rowge & Mehrizi, 1968: Campbell, 1968) most common among boys. Coarctation of the aorta usually shows a male preponderance (Campbell & Polani, 1961), but in this series the sex distribution was almost equal. Persistent ductus arteriosus dominated in males among our patients, contrary to the findings in many earlier investigations (Rowe & Mehrizi, 1968; Keith et al., 1958). Published figures on mortality vary with the selection of patients. Death rates in the first year of life ranged from 8 6 % in a necropsy study based on 777 children dying in hospital (Landtman, 1971) to 2 4 % for 450 children in a population study (Carlgren, 1969). Rowe & Mehrizi (1968) found that 34 % of 100 consecutive neonates with congenital heart disease died in the first month of life between 1960 and 1963 in New Zealand, while 52% died within the first year. The corresponding figures
Table IX. Assrssnietit of oprrnhilitv The first column shows the figures for those infants who died without operation and who might have been totally corrected by surgery as is the case today. The next two columns illustrate those infants who died and were not considered amenable to radical surgery (the possibility of surgery in those infants alive without operation is not taken into account, last column)
Major cardio-vascular malformation
Operable
Operation not indicated because of other illness
Ventricular septal defect I0 Transposition of great arteries 23 Coarctation of the aorta 17 Persistent ductus arteriosus 3 Underdeveloped left ventricle Tetralogy of Fallot 3 Single ventricle 4 Pulmonary stenosis 2 Pulmonary atresia with intact ventricular septum 5 Common atrioventricular canal 2 Tricuspid atresia Truncus arteriosus Atrial septal defect 1 Aortic stenosis Anomalous pulmonary venous drainage 3 Double outlet right ventricle 1 Ebstein's anomaly Cat-diactumour Pulmonary artery stenosis Cardiomyopathia Aortopulmonary window No diagnosis
11
14
44
2 8 12
Alive with-
Operation undertaken Inoperable
out
alive
dead
2 7
4
operation
9 2
26 1 6 6
1
7
3
5
1
4
2 2 19
2 11
2
1
3
1
1
5 3
1 1
2
1
4 1 1
1 1
2 1
3
14
51
18
25
64
Downloaded by [University of California, San Diego] at 22:04 20 April 2016
Congenital heart disease in neonates
from our study are 34 % for the first month and 67 % for the first year. Almost all deaths occur in the first year of life and death rates for this period are shown in Table VII. The death rates would have been still higher if this study had included only patients with symptomatic heart disease, but we have also included children with congenital heart disease hospitalized in the first month of life for other illness. Campbell (1973) summarized the percentage mortality, mainly during the first year of life, for different cardiac malformations based on four population series (Carlgren, 1959, 1969; MacMahon et al., 1953; Hay, 1966). Campbell’s figures are shown for comparison in Table VII and he emphasizes that his figures express the natural history of cardiac malformations, contrary to this series which is a selected clinical material including only patients hospitalized during the first month of life. The estimate that 117 of 212 infants would have been suitable for corrective surgery as judged by present-day criteria is of course attended by uncertainty, but the possibilities of surgery in small infants are growing (Edmunds et al., 1972; Hazan et al., 1974: Hayes et al., 1974; Castanedaet al., 1974: Malm et al., 1974; Edie et al., 1973; Ionescu et al., 1973). The mortality for banding operation in ventricular septal defect is stated to be 30-23% (Hunt et al., 1971; Olsen, 1972), but as high as 50% in the first month of life (Hunt et al., 1971). We found a higher mortality in our patients. In several centres complete correction of ventricular septal defect in small infants is performed with a smaller mortality (Castaneda et al., 1974; Weidman & DuShane, 1974). The outlook for infants with complete transposition of the great arteries has improved considerably since the introduction of septostomy and Mustard’s operation (Castaneda et al., 1974; Breckenridge et al., 1972). Miller (1974) found that the survival rate during the first year of life for transposition of the great arteries improved from 0.45 in 1970 to 0.80 in 1972. The high mortality in our series can probably be explained by a belated second operation; the primary improvement after Rashkind’s procedure is not followed fast enough by either a Blalock-Hanlon or a Mustard’s operation if the condition of the child is deteriorating. The mortality for coarctation of the aorta causing symptoms in the first months of life is very high. Surgery has been performed in this age group with a mortality of 27-30% (Hazan et al.,
117
1974; Connors et al., 1974). Surgical treatment of persistent ductus arteriosus has been performed with a slight risk, even in premature infants suffering from respiratory distress (Edmunds et al., 1972: Hazan et al.. 1974). Thus the four heart diseases which comprise the bulk of all cardiac lesions in the first month of life are amenable to surgery in early infancy with a mortality which, though high, is yet less than that without operation. Concerning the other, more rare cardiac anomalies, there are reports of improving surgical results (Hayes et al., 1974; Malm et al., 1974; Higashino et al., 1974; Poirier et al., 1975; Edie et al., 1973; Ionescu et al., 1973). In this series, heart catheterization was performed in an increasing number of patients during the period of investigation, but still only in a minority of patients because of a somewhat pessimistic attitude to the surgical possibilities. Fig. 1 shows that the investigation was performed particularly in those patients with the best outlook, while seriously ill infants and those suffering from extracardiac anomalies were excluded from heart catheterization to a great extent. With the improved surgical possibilities, a more aggressive attitude would seem justified, especially as heart catheterization can be performed in such infants with few complications in experienced hands.
REFERENCES Breckenridge, I . M . , Stark, J . , Bonham-Carter, R. E., Oelert, H., Graham, G. R. & Waterston, D. J . 1972. Mustard’s operation for transposition of the great arteries. Luncet i , 1140. Campbell, M. 1968. The incidence and later distribution of malformations of the heart. in Paediutric Cardiology (ed. Hamish Watson). Lloyd-Luke, London. - 1973. Incidence of cardiac malformations at birth and later. Brit Heart J 3 5 , 189. Campbell, M. & Polani, P. E. 1961. The aetiology of coarctation of the aorta. Lancet i , 463. Carlgren, L. E. 1959. The incidence of congenital heart disease in Gothenberg, 1941-50. Brit Heurt J 2 1 , 40. - 1969. The incidence of congenital heart disease in Gothenberg. Proc Ass European Paediat Cardiol5, 2. Castaneda, A. R., Lamberti, J., Sade, R. M., Williams, R. G. & Nadas, A . 1974. Open-heart surgery during the first three months of life. J Tliorac Cardiovusc Surg 68, 719.
Coleman, E. C. 1965. Serious congenital heart disease in infancy. Brit Heart J 27, 42. - 1969. Progress report on infants with serious cardiac malformations. Brit Heart J 3 / , 441, S c m d J Tlior Curdiovasc Sirrg 13
Downloaded by [University of California, San Diego] at 22:04 20 April 2016
118
H . Bzkgciurd Lrrirrsrti
Litid
P . Lomliolt
Connors, J. P., Hartman, A . F. & Weldon, C. S . 1974. Considerations in the surgical management of infantile coarctation of the aorta. Amer J Cardiol36, 489. Edie, R. N., Ellis, K., Gersony, W. M., Krongrad, E., Bowman, F. 0. & Malm, J. R. 1973. Surgical repair of single ventricle. J Thorcic Cardiovasc Surg 66, 350. Edmunds, L. H., Fishman, N. H., Gregory, G. A . , Heyman, M. A., Hoffman, J. I . E., Robinson, S. J., Roe, B. B., Rudolph, A . M. & Stanger, A . M. 1972. Cardiac surgery in infants less than 6 weeks of age. Circulation 66, 250. Hay, J. D. 1966. Population and clinic studies of congenital heart disease in Liverpool. Brit Med J i i , 661. Hayes, C. L., Gersony, W. M., Griffiths, S. P., Steeg, C. N . , Bowman, F. 0. & Malm, J . R. 1974. Results of correction of total anomalous pulmonary venous connections in infancy. Adv Cardiol 1 1 , 36. Hazan, E., Lessana, A . , Parrot, A . M., Louville, Y., Rioux, CI., Chetochine, F., Neveux, J. Y . , Lemoine, G. & Mathey, J. 1974. La chirurgie cardiaque dans le premier mois de la vie. La Nouvelle Presse Medicnle 3, 1135. Higashino, S. M., Shaw, G. G., May, I. A . & Ecker, R. R. 1974. Total anomalous pulmonary venous drainage below the diaphragm.J Thorac Cardiovasc Surg 68, 71 I . Hunt, C. E., Formanek, G., Levine, M. A , , Castaneda, A . & Moller, J. H. 1971. Banding of the pulmonary artery. Circulation 63, 395. Ionescu, M. I., Fergus, J., MacCartney, F. V. & Wooler, G. H . 1973. Intracardiac repair of single ventricle with pulmonary stenosis. J Thorac Cardiowsc Surg 65. 602.
Keith, J. D., Rowe, R. D. & Vlad, P. 1958. Heart Diseuse in Infancy and Childhood. Macmillan, New York. Landtman, B. 1971. Clinical and morphological studies in congenital heart disease. Acta Paediut Srand, Suppl. 213, 3 . MacMahon, B., McKeown, T. & Record, R. G. 1953. The incidence and life expectation of children with congenital heart disease. Brit Hear? J 15, 121. Malm, J . R., Bowman, F. O . , Hayes, C. J., Gersony, W. M. & Ellis, K. 1974. Results of surgical treatment of pulmonary atresia with intact ventricular septum. Ad\, Cardiol t i , 18. Menashe, V . D., Osterud, H. T. & Griswold, H . E. 1967. Mortality from congenital heart disease in Oregon. Pediatrics 40. 334. Miller, G. A. H. 1974. Congenital heart disease in the first week of life. Brit Heart J 36, 1160. Mitchell, S . C., Korones, S. B. & Berendes, H. W. 1971. Congenital heart disease in 56 109 births, incidence and natural history. Circulation 43, 323. Olsen, J. E. 1972. Pulmonary artery banding. A pre- and postoperative catheterization study. Srand J Thor Crtrdiovasr Surg 6 , 13. Pokier, R. A , , Berman, M. A . & Stansel, H. C. 1975. Current status of the surgical treatment of truncus arteriosus. J Thorac Cardiovasc Surg 69, 169. Rowe, R. D. & Mehrizi, A. 1968. The Neonate with Congenital Heart Disease. Saunders, PhiladelphiaLondon - Toronto. Weidman, W. H. & DuShane, J. W. 1974. Course of pulmonary hypertension following surgical closure of ventricular septa1 defects. Adv Cardiol I I , 131.
