Prognosis of infants with coarctation of aorta Su-chiung Chen, M.D. Leonard F. Fagan, M.D. Gerard J. F. Mudd, M.D. Vallee L. Willman, M.D. St. Louis, Mo.
Infants with coarctation of the aorta and congestive heart failure in the first six months of life usually represent a mixed group, both pathologically and clinically.1 The clinical course and ' prognosis of infants with coarctation of the aorta are reviewed in relation to their associated cardiac defects and their medical or surgical management.
Material Thirty-six infants with coarctation of the aorta who developed congestive heart failure in the first six months of life were seen between 1960 and 1974. There were 24 males and 12 females. Fourteen were seen within one month of age, ten during the second month, and twelve between the third and sixth month. They were followed from 18 months to 15 years {mean 4.5 years). Ten of the twelve patients referred between the third and sixth month of life survived. Usually they had feeding problems, poor weight gain, dyspnea, tachycardia, cardiomegaly and hepatomegaly. All infants had congestive heart failure. All had absent or weak femoral artery pulsation, and elevation of blood pressure in the upper extremity. Of those who had measurement of blood pressure performed in the upper and lower extremity on the initial examination, two had a systolic gradient of 20 mm. Hg, 16 had a systolic gradient of 20 to 50 mm. Hg, and ten had a systolic gradient of more t h a n 50 mm. Hg. All had cardiomegaly on x-ray examination. Initial From the Department of Pediatrics and the Department of Surgery, St. Louis University School of Medicine, St. Louis, Missouri. Received for publication June 2, 1976. Accepted for publication July 19, 1976. Reprint requests: Su-chiung Chen, M.D., Cardinal Glennon Memorial Hospital for Children, St. Louis, Missouri 63104.
November, 1977, Vol. 94, No. 5, pp. 557-561
electrocardiogram showed 15 patients with combined ventricular hypertrophy, 16 with right ventricular hypertrophy, and five with left ventricular hypertrophy. Twelve patients had right atrial hypertrophy and one patient had biatrial hypertrophy in addition to the ventricular hypertrophy. All of the infants were treated by vigorous medical management, including correction of acidosis, administration of digitalis and diuretics. All but one patient had cardiac catheterization during the initial hospitalization. When clinical improvement was not apparent and there was no evidence of central nervous system damage, surgery was recommended. The operative procedures included relief of coarctation, ligation and division of a patent ductus arteriosus, and banding of the pulmonary artery in the presence of a large ventricular septal defect.
Hemodynamic studies The patients were divided into four groups according to their findings at cardiac catheterization. Group I: Twelve patients with isolated coarctation of the aorta. The coarcted area was at the level distal to the left subclavian artery. The pulmonary artery pressure varied from normal to systemic level. In one it was 15/5 mm. Hg, in eight patients the systolic pulmonary artery pressure was 25 to 50 mm. Hg, and in two patients the systolic pulmonary artery pressure was more than 50 mm. Hg. One patient was not catheterized in infancy. Group II: Five patients with coarctation of the aorta and a patent ductus arteriosus. Three patients had coarctation at the preductal level, and two had coarctation distal to the origin of the
American Heart Journal
557
Chen et al.
T a b l e I. C l i n i c a l d a t a in p a t i e n t s w i t h c o a r c t a t i o n of a o r t a a n d V S D I
Age at catheterization
P,A. pressure (mm. Hg)
8
2mos. l mo.
54/15 105/40
No No
Coarctectomy at 2 yrs Coarctectomy and PA band at 2
11
2 mos.
110/45
Yes
12
2mos.
110/47
Yes
19
6mos.
90/44
No
Coarctectomy, PDA division, PA Died at 7 9/12 yrs., pulmonary hyband at 13 mos., VSD closure and pertension on autopsy removal of PA band at 5 yrs. Coarctectomy, PDA division, PA Living and well, VSD is small band at 3 mos. Coarctectomy, PA band at 8 mos. Died at 3 3/12 yr., small VSD, pulmonary hypertension on au-
26
18 days
82/30
Yes
No
28
4mos.
90/35
No
Coarctectomy, PA band at 5mos.
31 32
l mo. 3mos.
120/54 95/30
No No
No No
Case 2
I PDA
Outcome
Surgery
Living and well, VSD is small Died at 4 mos., no autopsy
mos.
topsy
Died at age 5 mos., VSD closed on autopsy Moved, persistent coarctation and VSD Living and well, VSD is small Living and well with VSD and coarctation, awaiting surgery
T a b l e II. M o r t a l i t y classified b y a s s o c i a t e d c a r d i a c d e f e c t s a n d s u r g e r y o r n o n - s u r g e r y
Deaths
Group I
II III IV Total
Catheterization findings Isolated coarctation Coarctation and PDA Coarctation, VSD with or without PDA Coarctation and complicated lesions
Number of cases
Surgery in infancy
after infancy
12 5 9 10 36
0/1 0/3 3/5 3/7 6/16
0/6 0/0 0/1 0/0 0/7
6/23
left subclavian artery. Three had a pulmonary a r t e r y p r e s s u r e o f 80/35-40 m m . H g , t w o , 5 0 / 1 5 - 2 0 m m . Hg. G r o u p I I I : N i n e p a t i e n t s w i t h c o a r c t a t i o n of the aorta and ventricular septal defect, with or without a patent ductus arteriosus. All had e l e v a t e d p u l m o n a r y a r t e r y p r e s s u r e { T a b l e I). T h e m a g n i t u d e o f t h e p u l m o n a r y b l o o d flow w a s difficult t o e v a l u a t e in t h i s g r o u p o f p a t i e n t s because of their unstable conditions; some received oxygen during the catheterization. However, o n c i n e a n g i o g r a p h i c e x a m i n a t i o n a l l h a d l a r g e left t o r i g h t s h u n t a t t h e v e n t r i c u l a r level. Group IV: Ten patients with complex heart l e s i o n s in a d d i t i o n t o t h e c o a r c t a t i o n of t h e a o r t a . There were three patients with transposition of t h e g r e a t vessels, t w o w i t h a t r i o v e n t r i c u l a r c a n a l ,
558
Non-
Total
surgery
deaths
1/5 1/2 1/3 3/3 6/13 (46%)
1/12 1/5 4/9 6/10 12/36' (33.3%)
(26%)
two with hypoplastic aortic arch, ventricular septal defect, and patent ductus arteriosus, one with supravalvular mitral stenosis, one with severe aortic stenosis and mitral insufficiency, and one had an atrial septal defect, patent ductus a r t e r i o s u s , a n d t w o c o a r c t e d a r e a s in t h e t h o r a c i c aorta.
Clinical course, surgical procedures and mortality In general, the coarcted segment was resected and anastomosis was achieved by running sutures at the posterior aspect and interrupted sutures at the anterior aspect, or interrupted mattress sutures entirely. Three patients received grafts at t h e c o a r c t e c t o m y site. The clinical courses varied according to the
November, 1977, Vol. 94, No. 5
Prognosis of infants with aorta coarctation
anatomical defects (Table II). Of the 12 Group I patients with isolated coarctation, only one had surgery in infancy. There were six patients in this group operated upon after infancy without surgical mortality. One infant without surgery died at home, probably due to aspiration of food. Four patients a r e doing well and are awaiting coarctectomy, In five Group II patients who had coarctation and a patent ductus arteriosus, three had coarctect0mY and !igation of the paten t ductus during infancy and all survived. Of the two patients who were not operated upon, one died and one is doing well. In nine Group III patients with coarctation and ventricular septal defect, with or Without a patent ductus arteriosus, five patients were subjected to coarctectomy with a patent ductus arteriosus l~gation and pulmonary artery banding in infancy. One of ~hese died two m o n t h s postoperatively, and t w o patients died several Years later. One died a t 7 years 9 months of age, two years after the closure of the Ventricular septal defect and removal of the pulmonary artery band. One died at 3 years 3 months after probable subacute bacterial endocarditis and lung abscess. One patien t who had coarctectomy at age 2 years is doing well with a small ventricu!ar septal defect. Of the two surviving patients who had coarctectomy and pulmonary artery banding, one is doing well with an apparently non-functioning pulmonary artery band and a small or sponta' neously closed ventricular septal defect, not visualized by cineangiogram. The other, lost to follow-up, had a persistent Coarctation with a 80 mm. Hg systolic gradient by blood pressure measurement and ventricular septal defect. One baby with severe cerebral hypoxia died without surgery. Two patients, o n e age two years, the other, age three years have had n o surgery yet and are doing well. The ventricular septal defect appears to be getting smaller. In Group IV patients, three babies died without surgery in' infancy and seven underwent surgery with three deaths. F o u r surviving patients are: two children w i t h transposition of the great vessels, one child with post-aortic valvulotomy an d mitral valve replacement, and one child with two coarcted areas who had coarctectomy and a patch graft. The Patients with transpositions are awaiting further surgery, and the one with the double Coarctation is s u r ~ v i n g With a residual
American Heart Journal
systolic gradient of 25 mm. Hg. The patient who had aortic valvulotomy and mitral valve replacement with a BjSrk-Shiley valve is doing extremely well two years after surgery. The over-all surgical mortality rate was 26 per cent (6/23). The surgical mortality rate for those of less than one year of age was 37.5 per cent (6/16). There was no mortality when surgery was done after one year of age {0/7). In contrast, there were 46 per cent (6/13) deaths in non-operated cases (Table II). Obviously, there was a higher mortality rate in the groups of complicated coarctation of the aorta. Blood pressure in survivors
There were 17 survivors after surgery and seven survivors without operation. Among the 17 patients, ten had coarctectomy at less than one year of age and seven were operated upon between 3 to 8 years of age. There were five patients operated within one month of age, one each a t 2 months and at 3 months, two at 5 months, and one at 8 months. Two of these ten operated in infancy have hypertension in the arms with a systolic gradient of more than 20 mm. Hg between the blood pressures in the arms and that in the legs. There was no gradient of more than 10 mm. Hg between the systolic arm blood pressures and leg blood pressures in patients operated upon a f t e r infancy. However, one patient who was operated at age 3.5 years and is now 16 years of age has mild hypertension of 140/60 ram. Hg to 160/80 mm. Hg. This patient has a very mild residual coarctation on aortogram with a mean gradient of 15 mm. Hg across the coarctation. Extracardiac anomalies
One patient with isolated coarctation developed subarachnoid hemorrhage at age 8 years due to a ruptured aneurysm of the anterior communicating artery. After a bi-frontal Craniotomy with clipping of the aneurysm, she subsequently underwent coarctectomy with success. One patient with isolated coarctation also has agenesis of the right kidney. Autopsy findings
There were ten postmortem examinations among the 12 deaths, five each with and without surgery. Of the two late operative deaths who had what appeared to be functioning pulmonary
559
C h e n et al.
artery banding in infancy, severe pulmonary hypertensive changes were found. One had a Heath-Edwards Grade IV hypertensive change and the other Grade VI hypertensive change. One of these had cardiac catheterization at age 5 years and the right ventricular systolic pressure was noted to be 100 mm, Hg, proximal pulmonary artery pressure was 80/40 mm. Hg, and distal pulmonary a~tery 50/35 ram. Hg. He had surgical closure of the ventricular septal defect and debanding of the pulmonary artery at age 5 years. However, he developed m u r m u r of a pulmonary insufficiency subsequently and had a repeat cardiac catheterization at age 6.5 years. The pulmonary artery pressure was noted to be 110/ 50 mm. Hg and he died a year later suddenly. The other patient came to the hospital at the age of 3 years with clinical findings compatible with subacute bacterial endocarditis and on cardiac catheterization the proximal pulmonary artery pressure was 110/20 mm. Hg and distal pressure was 85/55 mm. Hg. Of the three patients thought to have a large ventricular septal defect clinically, one had a completely closed ventricular septal defect and in the other two the ventricular septal defect was small on postmortem examination. Discussior
The mortality rate among patients with coarctation of the aorta in the first year of life has been variable, '-3 The surgical mortality rate of 33 to 41 per cent in earlier series '-3 has decreased to 14 to 17 per cent in more recent reports. 4' 5 The present series has a surgical mortality rate of 26 per cent (6/23) and non-surgical mortality rate of 46 per cent (6/13). It is difficult to compare the surgical mortality rates in each series since each report deals with different clinical and age groups. The higher non-surgical mortality rate in our series can be explained by the inclusion of patients with severe complicated cardiac lesions. In general, it seems apparent t h a t surgery should be recommended in those patients who show no improvement with medical m a n a g e m e n t . There was virtually no mortality in the groui~ with isolated coarctation, and they had good response to medical management in infancy. In the group with coarct~ition and ventricular septal defect, co~rctectomy and a pulmonary artery banding carried a high risk in our small series (3 out of 5). One died shortly after surgery and two died several years later. It is discouraging t h a t two of
560
the three who survived the initial surgery died eventually with pulmonary hypertension despite functioning pulmonary artery band. Perhaps primary closure of the ventricular septal defect under hypothermia in addition to coarctectomy in infancy is an alternative2 It is of interest t h a t in five of the nine Group III patients the ventricular septal defect had closed spontaneously or had become smaller. This suggests t h a t banding of the pulmonary artery at the time of coarctectomy may not be beneficial. Persistent or residual coarctation with a systolic gradient of more than 20 mm. Hg is not a common problem in our series, even in patients operated upon in infancy (2 out of 10). We have Seen no patients with recurrent coarctation, although it is known to occur. 7, s It is possible t h a t the low recurrence is related to the surgical technique of using interrupted sutures as proposed by Mustard2 Several reports indicated a high incidence of hypertension and premature cardiovascular disease in patients operated for coarctation. 1~ 11 Nanton and Olley '2 reported a 15 per cent incidence of unexplained hypertension in children operated between 1 year and 15 years. Although we have observed only one patient with unexplained hypertension, the incidence could possibly increase with prolonged follow-up. Our series is too small for meaningful comparison with others. It is now our feeling t h a t coarctectomy is indicated if there is persistent congestive heart failure in infancy. Infants with isolated coarctation or coarctation with patent ductus arteriosus have a good prognosis. In infants with a large ventricular septal defect and coarctation, three surgical approaches could be considered: pulmonary artery banding and coarctectomy, coarctectomy and primary closure of ventricular septal defect under hypothermia, or coarctectomy alone with the hope t h a t the ventricular septal defect will decrease in size with growth. At the present time it appears t h a t the approach will depend upon the preference and experience of the individual institution. In infants with complicated cardiac lesions in addition to coarctation, palliative or corrective surgery should be attempted. REFERENCES
1, Sinha,S. N., Kardatzke,M. L., Cole, R. B., Muster, A. J., Wessel, H. U., and PaUl,M. H.: Coarctation of the aorta in infancy,Circulation40:385, 1969. 2. Hartman, A. F., Jr., Goldring, D., and Staple, T. W.:
November, 1977, Vol. 94, No. 5
Prognosis of infants with aorta coarctation Coarctation of aorta in infancy, J. Pediatr. 70:95, 1967. 3. Glass, H. I., Mustard, W. T., and Keith, J. D.: Coarctation of aorta in infants, Pediatrics 26:109, 1960. 4. Connors, J. P., Hartman, A. F., Jr., and Weldon, C. S.: Considerations in the surgical management of infantile coarctation of aorta, Am. J. Cardiol. 36:489, 1975. 5. Fishman, N. H., Bronstein, M. H., Berman, W., Jr., Roe, B. B., Edmunds, L. H., Jr., Robinson, S. J., and Rudolph, A. M.: Surgical management of severe aortic coarctation and interrupted aortic arch in neonates, J. Thorac. Cardiovasc. Surg. 71:35, 1976. 6. Barratt-Boyes, B. G., Neutze, J. M., Seelye, E. R., and Simpson, M.: Complete correction of cardiovascular malformations in the first year of life, Progr. Cardiovasc. Dis. 15:229, 1972. 7. Khoury, G. H., and Hawes, C. R.: Recurrent coarctation of the aorta in infancy and childhood, J. Pediatr. 72:801, 1968.
American Heart Journal
8. Hartman, A. F., Goldring, D., Hernandez, A., Behrer, M. R., Schad, N., Ferguson, T., and Burford, T.: Recurrent coarctation of the aorta after successful repair in infancy, Am. J. Cardiol. 25:405, 1970. 9. Mustard, W. T.: Pediatric surgery, ed. 2, Chicago, 1969, Year Book Medical Publishers, Inc., pp. 526-529. 10. Simon, A. B., and Zloto, A. E.: Coarctation of the aorta, longitudinal assessment of operated patients, Circulation 50:456, 1974. 11. Maron, B. J., O'Neal-Humphries, J., Rowe, R. D., and Metlits, E. D.: Prognosis of surgically corrected coarctation of the aorta; a 20 year postoperative appraisal, Circulation 47:119, 1973. 12. Nanton, M. A., and Olley, P. M.: Residual hypertension after coarctectomy in Children, Am. J. Cardiol. 37:769, 1976.
561
Infants with coarctation of the aorta and congestive heart failure in the first six months of life usually represent a mixed group, both pathologically and clinically.1 The clinical course and ' prognosis of infants with coarctation of the aorta are reviewed in relation to their associated cardiac defects and their medical or surgical management.
Material Thirty-six infants with coarctation of the aorta who developed congestive heart failure in the first six months of life were seen between 1960 and 1974. There were 24 males and 12 females. Fourteen were seen within one month of age, ten during the second month, and twelve between the third and sixth month. They were followed from 18 months to 15 years {mean 4.5 years). Ten of the twelve patients referred between the third and sixth month of life survived. Usually they had feeding problems, poor weight gain, dyspnea, tachycardia, cardiomegaly and hepatomegaly. All infants had congestive heart failure. All had absent or weak femoral artery pulsation, and elevation of blood pressure in the upper extremity. Of those who had measurement of blood pressure performed in the upper and lower extremity on the initial examination, two had a systolic gradient of 20 mm. Hg, 16 had a systolic gradient of 20 to 50 mm. Hg, and ten had a systolic gradient of more t h a n 50 mm. Hg. All had cardiomegaly on x-ray examination. Initial From the Department of Pediatrics and the Department of Surgery, St. Louis University School of Medicine, St. Louis, Missouri. Received for publication June 2, 1976. Accepted for publication July 19, 1976. Reprint requests: Su-chiung Chen, M.D., Cardinal Glennon Memorial Hospital for Children, St. Louis, Missouri 63104.
November, 1977, Vol. 94, No. 5, pp. 557-561
electrocardiogram showed 15 patients with combined ventricular hypertrophy, 16 with right ventricular hypertrophy, and five with left ventricular hypertrophy. Twelve patients had right atrial hypertrophy and one patient had biatrial hypertrophy in addition to the ventricular hypertrophy. All of the infants were treated by vigorous medical management, including correction of acidosis, administration of digitalis and diuretics. All but one patient had cardiac catheterization during the initial hospitalization. When clinical improvement was not apparent and there was no evidence of central nervous system damage, surgery was recommended. The operative procedures included relief of coarctation, ligation and division of a patent ductus arteriosus, and banding of the pulmonary artery in the presence of a large ventricular septal defect.
Hemodynamic studies The patients were divided into four groups according to their findings at cardiac catheterization. Group I: Twelve patients with isolated coarctation of the aorta. The coarcted area was at the level distal to the left subclavian artery. The pulmonary artery pressure varied from normal to systemic level. In one it was 15/5 mm. Hg, in eight patients the systolic pulmonary artery pressure was 25 to 50 mm. Hg, and in two patients the systolic pulmonary artery pressure was more than 50 mm. Hg. One patient was not catheterized in infancy. Group II: Five patients with coarctation of the aorta and a patent ductus arteriosus. Three patients had coarctation at the preductal level, and two had coarctation distal to the origin of the
American Heart Journal
557
Chen et al.
T a b l e I. C l i n i c a l d a t a in p a t i e n t s w i t h c o a r c t a t i o n of a o r t a a n d V S D I
Age at catheterization
P,A. pressure (mm. Hg)
8
2mos. l mo.
54/15 105/40
No No
Coarctectomy at 2 yrs Coarctectomy and PA band at 2
11
2 mos.
110/45
Yes
12
2mos.
110/47
Yes
19
6mos.
90/44
No
Coarctectomy, PDA division, PA Died at 7 9/12 yrs., pulmonary hyband at 13 mos., VSD closure and pertension on autopsy removal of PA band at 5 yrs. Coarctectomy, PDA division, PA Living and well, VSD is small band at 3 mos. Coarctectomy, PA band at 8 mos. Died at 3 3/12 yr., small VSD, pulmonary hypertension on au-
26
18 days
82/30
Yes
No
28
4mos.
90/35
No
Coarctectomy, PA band at 5mos.
31 32
l mo. 3mos.
120/54 95/30
No No
No No
Case 2
I PDA
Outcome
Surgery
Living and well, VSD is small Died at 4 mos., no autopsy
mos.
topsy
Died at age 5 mos., VSD closed on autopsy Moved, persistent coarctation and VSD Living and well, VSD is small Living and well with VSD and coarctation, awaiting surgery
T a b l e II. M o r t a l i t y classified b y a s s o c i a t e d c a r d i a c d e f e c t s a n d s u r g e r y o r n o n - s u r g e r y
Deaths
Group I
II III IV Total
Catheterization findings Isolated coarctation Coarctation and PDA Coarctation, VSD with or without PDA Coarctation and complicated lesions
Number of cases
Surgery in infancy
after infancy
12 5 9 10 36
0/1 0/3 3/5 3/7 6/16
0/6 0/0 0/1 0/0 0/7
6/23
left subclavian artery. Three had a pulmonary a r t e r y p r e s s u r e o f 80/35-40 m m . H g , t w o , 5 0 / 1 5 - 2 0 m m . Hg. G r o u p I I I : N i n e p a t i e n t s w i t h c o a r c t a t i o n of the aorta and ventricular septal defect, with or without a patent ductus arteriosus. All had e l e v a t e d p u l m o n a r y a r t e r y p r e s s u r e { T a b l e I). T h e m a g n i t u d e o f t h e p u l m o n a r y b l o o d flow w a s difficult t o e v a l u a t e in t h i s g r o u p o f p a t i e n t s because of their unstable conditions; some received oxygen during the catheterization. However, o n c i n e a n g i o g r a p h i c e x a m i n a t i o n a l l h a d l a r g e left t o r i g h t s h u n t a t t h e v e n t r i c u l a r level. Group IV: Ten patients with complex heart l e s i o n s in a d d i t i o n t o t h e c o a r c t a t i o n of t h e a o r t a . There were three patients with transposition of t h e g r e a t vessels, t w o w i t h a t r i o v e n t r i c u l a r c a n a l ,
558
Non-
Total
surgery
deaths
1/5 1/2 1/3 3/3 6/13 (46%)
1/12 1/5 4/9 6/10 12/36' (33.3%)
(26%)
two with hypoplastic aortic arch, ventricular septal defect, and patent ductus arteriosus, one with supravalvular mitral stenosis, one with severe aortic stenosis and mitral insufficiency, and one had an atrial septal defect, patent ductus a r t e r i o s u s , a n d t w o c o a r c t e d a r e a s in t h e t h o r a c i c aorta.
Clinical course, surgical procedures and mortality In general, the coarcted segment was resected and anastomosis was achieved by running sutures at the posterior aspect and interrupted sutures at the anterior aspect, or interrupted mattress sutures entirely. Three patients received grafts at t h e c o a r c t e c t o m y site. The clinical courses varied according to the
November, 1977, Vol. 94, No. 5
Prognosis of infants with aorta coarctation
anatomical defects (Table II). Of the 12 Group I patients with isolated coarctation, only one had surgery in infancy. There were six patients in this group operated upon after infancy without surgical mortality. One infant without surgery died at home, probably due to aspiration of food. Four patients a r e doing well and are awaiting coarctectomy, In five Group II patients who had coarctation and a patent ductus arteriosus, three had coarctect0mY and !igation of the paten t ductus during infancy and all survived. Of the two patients who were not operated upon, one died and one is doing well. In nine Group III patients with coarctation and ventricular septal defect, with or Without a patent ductus arteriosus, five patients were subjected to coarctectomy with a patent ductus arteriosus l~gation and pulmonary artery banding in infancy. One of ~hese died two m o n t h s postoperatively, and t w o patients died several Years later. One died a t 7 years 9 months of age, two years after the closure of the Ventricular septal defect and removal of the pulmonary artery band. One died at 3 years 3 months after probable subacute bacterial endocarditis and lung abscess. One patien t who had coarctectomy at age 2 years is doing well with a small ventricu!ar septal defect. Of the two surviving patients who had coarctectomy and pulmonary artery banding, one is doing well with an apparently non-functioning pulmonary artery band and a small or sponta' neously closed ventricular septal defect, not visualized by cineangiogram. The other, lost to follow-up, had a persistent Coarctation with a 80 mm. Hg systolic gradient by blood pressure measurement and ventricular septal defect. One baby with severe cerebral hypoxia died without surgery. Two patients, o n e age two years, the other, age three years have had n o surgery yet and are doing well. The ventricular septal defect appears to be getting smaller. In Group IV patients, three babies died without surgery in' infancy and seven underwent surgery with three deaths. F o u r surviving patients are: two children w i t h transposition of the great vessels, one child with post-aortic valvulotomy an d mitral valve replacement, and one child with two coarcted areas who had coarctectomy and a patch graft. The Patients with transpositions are awaiting further surgery, and the one with the double Coarctation is s u r ~ v i n g With a residual
American Heart Journal
systolic gradient of 25 mm. Hg. The patient who had aortic valvulotomy and mitral valve replacement with a BjSrk-Shiley valve is doing extremely well two years after surgery. The over-all surgical mortality rate was 26 per cent (6/23). The surgical mortality rate for those of less than one year of age was 37.5 per cent (6/16). There was no mortality when surgery was done after one year of age {0/7). In contrast, there were 46 per cent (6/13) deaths in non-operated cases (Table II). Obviously, there was a higher mortality rate in the groups of complicated coarctation of the aorta. Blood pressure in survivors
There were 17 survivors after surgery and seven survivors without operation. Among the 17 patients, ten had coarctectomy at less than one year of age and seven were operated upon between 3 to 8 years of age. There were five patients operated within one month of age, one each a t 2 months and at 3 months, two at 5 months, and one at 8 months. Two of these ten operated in infancy have hypertension in the arms with a systolic gradient of more than 20 mm. Hg between the blood pressures in the arms and that in the legs. There was no gradient of more than 10 mm. Hg between the systolic arm blood pressures and leg blood pressures in patients operated upon a f t e r infancy. However, one patient who was operated at age 3.5 years and is now 16 years of age has mild hypertension of 140/60 ram. Hg to 160/80 mm. Hg. This patient has a very mild residual coarctation on aortogram with a mean gradient of 15 mm. Hg across the coarctation. Extracardiac anomalies
One patient with isolated coarctation developed subarachnoid hemorrhage at age 8 years due to a ruptured aneurysm of the anterior communicating artery. After a bi-frontal Craniotomy with clipping of the aneurysm, she subsequently underwent coarctectomy with success. One patient with isolated coarctation also has agenesis of the right kidney. Autopsy findings
There were ten postmortem examinations among the 12 deaths, five each with and without surgery. Of the two late operative deaths who had what appeared to be functioning pulmonary
559
C h e n et al.
artery banding in infancy, severe pulmonary hypertensive changes were found. One had a Heath-Edwards Grade IV hypertensive change and the other Grade VI hypertensive change. One of these had cardiac catheterization at age 5 years and the right ventricular systolic pressure was noted to be 100 mm, Hg, proximal pulmonary artery pressure was 80/40 mm. Hg, and distal pulmonary a~tery 50/35 ram. Hg. He had surgical closure of the ventricular septal defect and debanding of the pulmonary artery at age 5 years. However, he developed m u r m u r of a pulmonary insufficiency subsequently and had a repeat cardiac catheterization at age 6.5 years. The pulmonary artery pressure was noted to be 110/ 50 mm. Hg and he died a year later suddenly. The other patient came to the hospital at the age of 3 years with clinical findings compatible with subacute bacterial endocarditis and on cardiac catheterization the proximal pulmonary artery pressure was 110/20 mm. Hg and distal pressure was 85/55 mm. Hg. Of the three patients thought to have a large ventricular septal defect clinically, one had a completely closed ventricular septal defect and in the other two the ventricular septal defect was small on postmortem examination. Discussior
The mortality rate among patients with coarctation of the aorta in the first year of life has been variable, '-3 The surgical mortality rate of 33 to 41 per cent in earlier series '-3 has decreased to 14 to 17 per cent in more recent reports. 4' 5 The present series has a surgical mortality rate of 26 per cent (6/23) and non-surgical mortality rate of 46 per cent (6/13). It is difficult to compare the surgical mortality rates in each series since each report deals with different clinical and age groups. The higher non-surgical mortality rate in our series can be explained by the inclusion of patients with severe complicated cardiac lesions. In general, it seems apparent t h a t surgery should be recommended in those patients who show no improvement with medical m a n a g e m e n t . There was virtually no mortality in the groui~ with isolated coarctation, and they had good response to medical management in infancy. In the group with coarct~ition and ventricular septal defect, co~rctectomy and a pulmonary artery banding carried a high risk in our small series (3 out of 5). One died shortly after surgery and two died several years later. It is discouraging t h a t two of
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the three who survived the initial surgery died eventually with pulmonary hypertension despite functioning pulmonary artery band. Perhaps primary closure of the ventricular septal defect under hypothermia in addition to coarctectomy in infancy is an alternative2 It is of interest t h a t in five of the nine Group III patients the ventricular septal defect had closed spontaneously or had become smaller. This suggests t h a t banding of the pulmonary artery at the time of coarctectomy may not be beneficial. Persistent or residual coarctation with a systolic gradient of more than 20 mm. Hg is not a common problem in our series, even in patients operated upon in infancy (2 out of 10). We have Seen no patients with recurrent coarctation, although it is known to occur. 7, s It is possible t h a t the low recurrence is related to the surgical technique of using interrupted sutures as proposed by Mustard2 Several reports indicated a high incidence of hypertension and premature cardiovascular disease in patients operated for coarctation. 1~ 11 Nanton and Olley '2 reported a 15 per cent incidence of unexplained hypertension in children operated between 1 year and 15 years. Although we have observed only one patient with unexplained hypertension, the incidence could possibly increase with prolonged follow-up. Our series is too small for meaningful comparison with others. It is now our feeling t h a t coarctectomy is indicated if there is persistent congestive heart failure in infancy. Infants with isolated coarctation or coarctation with patent ductus arteriosus have a good prognosis. In infants with a large ventricular septal defect and coarctation, three surgical approaches could be considered: pulmonary artery banding and coarctectomy, coarctectomy and primary closure of ventricular septal defect under hypothermia, or coarctectomy alone with the hope t h a t the ventricular septal defect will decrease in size with growth. At the present time it appears t h a t the approach will depend upon the preference and experience of the individual institution. In infants with complicated cardiac lesions in addition to coarctation, palliative or corrective surgery should be attempted. REFERENCES
1, Sinha,S. N., Kardatzke,M. L., Cole, R. B., Muster, A. J., Wessel, H. U., and PaUl,M. H.: Coarctation of the aorta in infancy,Circulation40:385, 1969. 2. Hartman, A. F., Jr., Goldring, D., and Staple, T. W.:
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Prognosis of infants with aorta coarctation Coarctation of aorta in infancy, J. Pediatr. 70:95, 1967. 3. Glass, H. I., Mustard, W. T., and Keith, J. D.: Coarctation of aorta in infants, Pediatrics 26:109, 1960. 4. Connors, J. P., Hartman, A. F., Jr., and Weldon, C. S.: Considerations in the surgical management of infantile coarctation of aorta, Am. J. Cardiol. 36:489, 1975. 5. Fishman, N. H., Bronstein, M. H., Berman, W., Jr., Roe, B. B., Edmunds, L. H., Jr., Robinson, S. J., and Rudolph, A. M.: Surgical management of severe aortic coarctation and interrupted aortic arch in neonates, J. Thorac. Cardiovasc. Surg. 71:35, 1976. 6. Barratt-Boyes, B. G., Neutze, J. M., Seelye, E. R., and Simpson, M.: Complete correction of cardiovascular malformations in the first year of life, Progr. Cardiovasc. Dis. 15:229, 1972. 7. Khoury, G. H., and Hawes, C. R.: Recurrent coarctation of the aorta in infancy and childhood, J. Pediatr. 72:801, 1968.
American Heart Journal
8. Hartman, A. F., Goldring, D., Hernandez, A., Behrer, M. R., Schad, N., Ferguson, T., and Burford, T.: Recurrent coarctation of the aorta after successful repair in infancy, Am. J. Cardiol. 25:405, 1970. 9. Mustard, W. T.: Pediatric surgery, ed. 2, Chicago, 1969, Year Book Medical Publishers, Inc., pp. 526-529. 10. Simon, A. B., and Zloto, A. E.: Coarctation of the aorta, longitudinal assessment of operated patients, Circulation 50:456, 1974. 11. Maron, B. J., O'Neal-Humphries, J., Rowe, R. D., and Metlits, E. D.: Prognosis of surgically corrected coarctation of the aorta; a 20 year postoperative appraisal, Circulation 47:119, 1973. 12. Nanton, M. A., and Olley, P. M.: Residual hypertension after coarctectomy in Children, Am. J. Cardiol. 37:769, 1976.
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