Correction of Aortic Coarctation in Neonates: Mortality and Late Results Quentin Macmanus, M.D., Albert Starr, M.D., Louis E.. Lambert, M.S., a i d Gary Grunkemeier, Ph.D. ABSTRACT To determine those factors that affect mortality and to analyze long-term results, the records of 44 infants who underwent repair of aortic coarctationat less than 90 days of age were examined. There were 14 operative and 8 late deaths (mean, 4.3 months postoperatively). Subsequent operation, generally to repair or palliate associated anomalies, was required 22 times in 20 patients and was a source of considerable mortality. There is evidence that earlier total repair of associated anomalies might improve survival. Sufficient data were available on 17 of the 22 survivors to assess long-term results. The outcome was considered excellent in 8 patients, fair in 4, and poor in 5. Revision of the coarctation repair due to growth failure of the anastomosis was required in 1 patient. Aggressive surgical management is recommended in these infants because, despite a high early mortality, a considerable proportion of excellent results can be anticipated in what is otherwise a hopeless situation.
Coarctation of the aorta was one of the earliest congenital cardiovascular lesions to be surgically repaired, and current results in children and young adults are gratifying. Problems persist, however, in the neonatal period, during which coarctation continues to be a lethal anomaly. Among 69 children younger than 5 years of age who underwent coarctation repair at the University of Oregon Health Sciences Center, all but 1 of 23 known deaths have occurred when operation was performed in the first 3 months of life. For those who survive, scant data are currently available regarding long-term prognosis. Therefore we reviewed our surgical experience in this high-risk group to determine those facFrom the Division of Cardiopulmonary Surgery, University of Oregon Health Sciences Center, Portland, OR Accepted for publication Apr 1, 1977. Address reprint requests to Dr. Macmanus, Division of Cardiopulmonary Surgery, University of Oregon Health Sciences Center, Sam Jackson Park Rd, Portland, OR 97201.
544
tors affecting mortality and to analyze long-term results. Materials and Methods The charts of all infants who underwent repair of coarctation of the aorta at less than 90 days of age at the University of Oregon Health Sciences Center from January, 1960, through January, 1976, were examined. The 44 patients so characterized included 27 boys and 17 girls. Average gestational age was 38.7 weeks and average birth weight, 3.5 kg. Forty-three of the patients were operated on for heart failure that was progressive despite aggressive medical management. Average onset of heart failure was 17 days after birth. One child with severe coarctation and a ventricular septa1defect (VSD)underwent coarctation repair because of failure to thrive. Associated cardiac anomalies were frequent, as noted in Table 1. Most common were coarctation with patent ductus arteriosus (PDA), alone or with VSD. As depicted in Figure 1,the majority of patients underwent coarctation repair within the first 30 days of life. Average age at operation was 32 days. Thirty-six patients had preductal coarctation with a PDA, 1 had postductal coarctation with a patent ductus, and 7 had juxtaductal coarctation with a closed ductus. In all cases coarctation repair was performed by way of a left thoracotomy incision. End-to-end anastomosis was done in 40 patients, a plastic repair was utilized in 2, and 2 patients died in the operating room prior to completion of the anastomosis. Silk was used in 32 of the 39 repairs in which the suture type was specified. In 32 instances a posterior continuous row and an anterior interrupted row were placed. In 5 instances separate anterior and posterior continuous rows were used, and interrupted sutures were utilized exclusively in 1patient. The technique of anastomosis was not specified in 4 patien t s.
545 Macmanus et al: Correction of Aortic Coarctation in Neonates
Table 1. Associated Cardiac Anomalies and Outcome in 44 Patients with Coarctation of the Aorta Operative
Anomaly Coarctation without shunts Coarctation with PDA Coarctation with VSD Coarctation with VSD, ASD, and PDA Coarctation with VSD and PDA Coarctation with ASD Coarctation with ASD and PDA Coarctation with PDA and TGV Coarctation with other complex anomalies
Total
Alive
Dead
3 4 2 4 4 1 1 3 0
1 5 0 3 7 0 0 4 2
22
22
and Late Mortality (%) 25 55 0 43 64 0 0 57 100
PDA = patent ductus arteriosus;VSD = ventricularseptal defect; ASD = atrial septal defect; TGV = transpositionof the great vessels.
sociated anomalies is listed in Table 1, and the outcome as a function of age at operation is illus12F n trated in Figures 1 and 3. The 22 subsequent operations in 20 patients resulted in 10 deaths (9 operative, 1 late). The causes of death are listed in Table 3 and the relationship of outcome to type of later operation in Table 2. Mortality as a function of the age at reoperation is diagrammed in Figure 2, which indicates that 80% of deaths following sub1-10 11-20 21-30 31-40 41-50 51-60 61-70 71-80 sequent operation were produced by reoperaAge a t Coarctation Repair (Days) tion in the first 3 months of life. Two patients Fig 1 . Age at coarctation repair and outcome. underwent 2 subsequent procedures. Overall, 22 of the 44 patients (50%) who required coarctation repair in the first 3 months of Twenty patients required 22 subsequent operations during the follow-up period, usually to life are known to have died following surgical correct or palliate associated anomalies. The repair. An actuarial survival curve is given in type of subsequent operation is listed in Table 2 Figure 4. For statistical comparison, a subgroup and the age at this later operation (mean, 89 of 18 patients surviving at least one year after days) is shown in Figure 2. Four of these patients operation and 21 patients who died during that required revision of their initial coarctation re- first postoperative year was examined. Statistical analysis of possible variables affecting morpair. tality in this group revealed onset of heart failure at less than 2 weeks of age and subsequent operResults ation at less than 90 days of age to be significant Mortality predictors of mortality ( p < 0.025 and 0.005, Twelve of 44 patients (27%) died during or fol- respectively, by chi-square analysis). Gestalowing coarctation repair and prior to sub- tional age, birth weight, and severity of heart sequent operation. These included 9 operative failure as reflected in mean pulmonary artery and 3 late deaths. The causes of death are given and left ventricular end-diastolic pressures did in Table 3. The relationship of deaths to as- not seem to be significant. Alive Dead (operative and late)
546 The Annals of Thoracic Surgery Vol 24 No 6 December 1977
Table 2 . Type of Subsequent Operation and Outcome Operative
Subsequent Operation
Alive
VSD repair VSD and ASD repair Pulmonary artery banding Revision of coarctation repair
Aortic commissurotomy Removal of pulmonary artery band Atrial baffle Thoracotomy for hemorrhage Atrial septectomy
Total
Dead
and Late Mortality
2 2 2 1 1 2 1 1 0
0 1 4 3
0 33 66
1
50
0 0 0
0
1
100
12
10
(YO)
:75
0 0
VSD = ventricular septal defect; ASD = atrial septal defect.
Alive D e a d (operative and late)
Age a t Subsequent Operation
Fig 2 . Age (in months) at subsequent operation and outcome.
Follow-up The duration of follow-up has ranged from 2 months to 15 years 9 months, with a mean of 3.5 years. Of the 22 survivors, 17 were asymptomatic. Two had heart failure under medical control, 2 had failed to thrive, and 1 infant had both heart failure and severe failure to thrive. Percentile height and weight were recorded in 16 and 18 of the 22 survivors, respectively. Average height percentile was 36.3 and average weight percentile, 27.7. Systolic blood pressure averaged 125 mm Hg in 19 patients in whom it was recorded at follow-up. Blood pressure in the lower extremity was recorded in 17 survivors and averaged 99.9
mm Hg. Blood pressure gradient arm-to-leg was recorded in 17 patients and averaged 29.8 mm Hg. Seventeen survivors for whom follow-up and sufficient blood pressure data were available were grouped according to excellent, fair, or poor results; criteria and results are given in Table 4. Follow-up in this subgroup averaged 4.2 years. The number of patients was insufficient to make conclusions regarding technique of anastomosis and blood pressure data at follow-up. Four patients required revision of their anastomosis. Three of them died at 13, 20, and 49 days of age following reoperation for aortic thrombosis at the suture line. In these infants a posterior continuous and anterior interrupted suture technique was utilized. A fourth child
547 Macmanus et al: Correction of Aortic Coarctation in Neonates
Table 3 . Causes of Death (Operative and Late) Coarctation Subsequent Repair Operation
Cause of Death Renal failure Left ventricular failure Arrhythmia
1 8a 2
Sepsis Hemorrhage
0
2 5b 2 0 1
l b
Total
12
10
a T w late ~ deaths. late death.
Mortality of Coarctation Repair Mortality due to Subsequenl Surgery
Comment Mortality Although Morgagni [lll and Paris [121 described the pathology of coarctation of the aorta in the late 1700s and Maude Abbott [ll provided her classic clinicopathological study of the entity in 1928, it was not until 1944 that a successful repair was performed, by Crafoord and Nylin [51 in Sweden. Progress during the past 30 years has been rapid, and mortality has progressively declined. In 1962, Schuster and Gross 1133 reported an overall 4.1% mortality rate in 487 coarctation repairs. Unfortunately, this low mortality does
0-10 11-20 21-30 31-40 41-50 51-60 61-70
Age at Coarctation Repair (Days)
Fig 3 . Mortality rate versus age a t coarctation repair.
~Cfo~,,n
0
I m o 6 m o lyr
,
(la
2
3
I
(5;
I
Number of Potients at Risk
4
5
6
,
7
I
8
Years Post -op
Fig 4. Actuarial survival curve of44 patients w h o required coarctation repair in the first 3 months oflife.
underwent repair of coarctation and PDA at 24 days of age. At 11years 2 months he had an atrial baffle placed for correction of partial anomalous venous return. At 15 years of age he began to experience dyspnea on exertion, and catheterization revealed recurrent coarctation. He underwent ascending-to-descending aortic bypass grafting. At follow-up 6 months later he reported an increased exercise tolerance; there was no arm-leg blood pressure gradient. Four other patients, currently being followed at 2, 4, 4, and 5 years of age, have high blood pressure and significant arm-leg gradients and will probably need rerepair in the future. One other patient with high blood pressure and a large gradient was lost to follow-up at 6 years of age.
9
10
u II
12
13
548
The Annals of Thoracic Surgery Vol 24 No 6 December 1977
Table 4 . Results of Coarctation Repair in 17 Survivors (Mean Follow-up, 4.2 Years) No. of
Patients
Percent
Excellent BP difference arm-leg 20 mm Hg, hypertension vresent
subsequent operation plays such a prominent role in mortality in these infants has not been stressed before. Shumacker and co-workers [141, in a review of the literature up to 1968, make a convincing argument that mortality is directly related to the presence of associated anomalies, which are very common in these small infants with congestive heart failure [3, 7, 151. The most critical factor in neonates appears to be the age at onset of heart failure and the necessity for early subsequent operation.
Associated Anomalies The management of associated anomalies in these neonates has not previously been explored in depth. Hallman and associates [lo] stressed aHypertension defined as blood pressure at follow-up the importance of banding the pulmonary artery greater than 2 standard deviations above accepted normals at coarctation repair when catheterization or [2, 8, 91 for age. operative findings indicated the presence of a BP = blood pressure. VSD. This was done in 11 of 24 patients with not pertain to infants, in whom associated VSD in their series of infants less than 1 year of anomalies, both cardiac and noncardiac, are age; 6 of these infants were alive at follow-up common and whose small size alone puts them (mean, 3.5 years). In the present series, 7 infants were thought at at a technical disadvantage should repair be required. In the early 1960s, operative mortality the time of coarctation repair to require pulmorates of 90% [71 and 100% [41 were reported for nary artery banding. Palliative pulmonary artery infants who had coarctation and congestive banding was employed subsequently in 6 heart failure. As surgical techniques and life- others. Eight of these 13 patients had VSD with support systems continue to improve, reports or without associated atrial septa1 defect (ASD), are beginning to appear examining the opera- theoretically easily correctable anomalies. Mortive results in large numbers of these very small tality in this group was 62% (5 of 8). In contrast, infants. Tawes and associates [15] recorded a of 5 patients with VSD with or without as57% hospital mortality (71 of 123) in infants sociated ASD who underwent subsequent total younger than 3 months of age, and Chang and repair, only 1 died, a mortality rate of 20%. DifBurrington [3] reported a 56% operative mortal- ferences are not statistically significant due to ity (24 of 43). The most promising report to date, the small number of patients in each group, nor by Fishman and colleagues [6], reveals an opera- should the groups be considered comparable, tive mortality of 26% (8 of 31) in this age group, because those patients who underwent total rewith only a 14% operative mortality (3 of 22) pair tended to be older at reoperation and, in the judgment of the surgeon at coarctation repair, since 1969. Fourteen (31%) in this series of 44 patients presumably better risks. There is no doubt, died within 30 days of coarctation repair. This is however, that palliation-specifically, pulmosimilar to the operative mortality reported by nary artery banding-has been disappointing in Fishman and colleagues [6]. There were 8 late this setting, and the data are at least suggestive deaths following subsequent operation, how- that earlier total repair, perhaps done concomever, for an overall mortality of 50% (22 of 4) mitantly with coarctation repair, might improve over a mean follow-up of 3.5 years. The fact that survival. 5
29
549 Macmanus et al: Correction of Aortic Coarctation in Neonates
Hypertension Few data are available regarding long-term follow-up in these small infants, generally because most series contain large numbers of older infants and adults. Tawes and colleagues [151 studied 209 children after coarctation repair and found 82% with a normal blood pressure and femoral pulse, 8.6% with slightly decreased femoral pulse and slight blood pressure elevation, and 9.5% with high blood pressure and high arm-leg gradients (mean follow-up, 6 years). Hallman and co-workers [lo] followed, for an average of 3.5 years, 42 survivors who had undergone coarctation repair at 1year of age or less. Fifteen (36%) had an excellent result, 22 (52%)had an arm-leg blood pressure gradient of less than 30 mm Hg, and 5 (12%) had an unsatisfactory result, with gradients greater than 30 mm Hg. Our results (see Table 4) are roughly comparable, although they show a higher percentage of poor results in this younger age group. Recurrence of Coarctation VData concerning the necessity for revision of the anastomosis are equally sparse in this age group. Tawes and associates [151 reported that 20 (9.5%) of 209 children of all ages had obvious signs of recoarctation at follow-up (mean, 6 years). Fourteen of these 20 patients had had coarctation repair in the first 3 months of life. Fewer data are available on the actual incidence. In the present series, 3 patients required early revision of their anastomoses at 1,2, and 49 days following initial repair due to technical failures. All these patients died. A fourth patient, who underwent revision of his anastomosis 15.5 years following initial repair, is doing well. Four other patients, representing 22% of the 18 patients followed l year or more, with a mean of 4.2 years, will require anastomotic revision. Fourteen of 52 survivors (27%) in Tawes' series [15] who were operated on when they were younger than 3 months of age underwent rerepair of their anastomosis, although the average follow-up in this subgroup is not discernible. Although the 4.2-year mean follow-up in our series is one of the longest reported in a large
group of neonates with coarctation, it is conceivable-and perhaps likely-that the rate of coarctation recurrence will increase as these patients are followed into maturity. That 1 patient required reoperation at 15 years of age reiterates the importance of close, long-term follow-up in these patients.
References 1. Abbott ME: Coarctation of the aorta of the adult type. Am Heart J 3:392, 1928 2. Allen-Williams GM: Pulse-rate and blood pressure in infancy and early childhood. Arch Dis Child 20:125, 1945 3. Chang JHT, Burrington JD: Coarctation of the aorta in infants and children. J Pediatr Surg 7:127, 1972 4. Cooley DA, Hallman GL: Cardiovascular surgery in the first year of life: experience with 450 consecutive operations. Am J Surg 107:474, 1964 5. Crafoord C, Nylin G: Congenital coarctation of the aorta and its surgical therapy. J Thorac Surg 14:347, 1945 6. Fishman NH, Bronstein MH, Berman W, et al: Surgical management of severe aortic coarctation and interrupted aortic arch in neonates. J Thorac Cardiovasc Surg 71:35, 1976 7. Glass IH, Mustard WT, Keith JD: Coarctation of the aorta in infants: a review of 12 years experience. Pediatrics 26:109, 1960 8. Goodman HG, Cummings GR, Raber MB: Photocell oscillometer for measuring systolic pressure in newborns. Am J Dis Child 103:152, 1962 9. Graham AW, Hines EA, Gage RP: Blood pressure in children between the ages of 5 and 16 years. Am J Dis Child 69:203, 1945 10. Hallman GL, Yashar JJ, Bloodwell RD, et al: Surgical correction of coarctation of the aorta in the first year of life. Ann Thorac Surg 4:106, 1967 11. Morgagni JB: De sedibus et causis morborum. Epist XVIII, Article VI, 1760 12. Paris M: Retrecissement considerable de l'aorte pectorale observe a l'H6tel Dieu de Paris. J Chir Desault 2:107, 1791 13. Schuster SR, Gross RE: Surgery for coarctation of the aorta: a review of 500 cases. J Thorac Cardiovasc Surg 43:54, 1962 14. Shumacker HB, Nahnvold DL, King H, et al: Coarctation of the aorta. Curr Probl Surg2:1,1968 15. Tawes RL, Aberdeen E, Waterston DJ, et al: Coarctation of the aorta in infants and children: a review of 333 operative cases, including 179 infants. Circulation 39,4O:Suppl 1:173, 1969
Coarctation of the aorta was one of the earliest congenital cardiovascular lesions to be surgically repaired, and current results in children and young adults are gratifying. Problems persist, however, in the neonatal period, during which coarctation continues to be a lethal anomaly. Among 69 children younger than 5 years of age who underwent coarctation repair at the University of Oregon Health Sciences Center, all but 1 of 23 known deaths have occurred when operation was performed in the first 3 months of life. For those who survive, scant data are currently available regarding long-term prognosis. Therefore we reviewed our surgical experience in this high-risk group to determine those facFrom the Division of Cardiopulmonary Surgery, University of Oregon Health Sciences Center, Portland, OR Accepted for publication Apr 1, 1977. Address reprint requests to Dr. Macmanus, Division of Cardiopulmonary Surgery, University of Oregon Health Sciences Center, Sam Jackson Park Rd, Portland, OR 97201.
544
tors affecting mortality and to analyze long-term results. Materials and Methods The charts of all infants who underwent repair of coarctation of the aorta at less than 90 days of age at the University of Oregon Health Sciences Center from January, 1960, through January, 1976, were examined. The 44 patients so characterized included 27 boys and 17 girls. Average gestational age was 38.7 weeks and average birth weight, 3.5 kg. Forty-three of the patients were operated on for heart failure that was progressive despite aggressive medical management. Average onset of heart failure was 17 days after birth. One child with severe coarctation and a ventricular septa1defect (VSD)underwent coarctation repair because of failure to thrive. Associated cardiac anomalies were frequent, as noted in Table 1. Most common were coarctation with patent ductus arteriosus (PDA), alone or with VSD. As depicted in Figure 1,the majority of patients underwent coarctation repair within the first 30 days of life. Average age at operation was 32 days. Thirty-six patients had preductal coarctation with a PDA, 1 had postductal coarctation with a patent ductus, and 7 had juxtaductal coarctation with a closed ductus. In all cases coarctation repair was performed by way of a left thoracotomy incision. End-to-end anastomosis was done in 40 patients, a plastic repair was utilized in 2, and 2 patients died in the operating room prior to completion of the anastomosis. Silk was used in 32 of the 39 repairs in which the suture type was specified. In 32 instances a posterior continuous row and an anterior interrupted row were placed. In 5 instances separate anterior and posterior continuous rows were used, and interrupted sutures were utilized exclusively in 1patient. The technique of anastomosis was not specified in 4 patien t s.
545 Macmanus et al: Correction of Aortic Coarctation in Neonates
Table 1. Associated Cardiac Anomalies and Outcome in 44 Patients with Coarctation of the Aorta Operative
Anomaly Coarctation without shunts Coarctation with PDA Coarctation with VSD Coarctation with VSD, ASD, and PDA Coarctation with VSD and PDA Coarctation with ASD Coarctation with ASD and PDA Coarctation with PDA and TGV Coarctation with other complex anomalies
Total
Alive
Dead
3 4 2 4 4 1 1 3 0
1 5 0 3 7 0 0 4 2
22
22
and Late Mortality (%) 25 55 0 43 64 0 0 57 100
PDA = patent ductus arteriosus;VSD = ventricularseptal defect; ASD = atrial septal defect; TGV = transpositionof the great vessels.
sociated anomalies is listed in Table 1, and the outcome as a function of age at operation is illus12F n trated in Figures 1 and 3. The 22 subsequent operations in 20 patients resulted in 10 deaths (9 operative, 1 late). The causes of death are listed in Table 3 and the relationship of outcome to type of later operation in Table 2. Mortality as a function of the age at reoperation is diagrammed in Figure 2, which indicates that 80% of deaths following sub1-10 11-20 21-30 31-40 41-50 51-60 61-70 71-80 sequent operation were produced by reoperaAge a t Coarctation Repair (Days) tion in the first 3 months of life. Two patients Fig 1 . Age at coarctation repair and outcome. underwent 2 subsequent procedures. Overall, 22 of the 44 patients (50%) who required coarctation repair in the first 3 months of Twenty patients required 22 subsequent operations during the follow-up period, usually to life are known to have died following surgical correct or palliate associated anomalies. The repair. An actuarial survival curve is given in type of subsequent operation is listed in Table 2 Figure 4. For statistical comparison, a subgroup and the age at this later operation (mean, 89 of 18 patients surviving at least one year after days) is shown in Figure 2. Four of these patients operation and 21 patients who died during that required revision of their initial coarctation re- first postoperative year was examined. Statistical analysis of possible variables affecting morpair. tality in this group revealed onset of heart failure at less than 2 weeks of age and subsequent operResults ation at less than 90 days of age to be significant Mortality predictors of mortality ( p < 0.025 and 0.005, Twelve of 44 patients (27%) died during or fol- respectively, by chi-square analysis). Gestalowing coarctation repair and prior to sub- tional age, birth weight, and severity of heart sequent operation. These included 9 operative failure as reflected in mean pulmonary artery and 3 late deaths. The causes of death are given and left ventricular end-diastolic pressures did in Table 3. The relationship of deaths to as- not seem to be significant. Alive Dead (operative and late)
546 The Annals of Thoracic Surgery Vol 24 No 6 December 1977
Table 2 . Type of Subsequent Operation and Outcome Operative
Subsequent Operation
Alive
VSD repair VSD and ASD repair Pulmonary artery banding Revision of coarctation repair
Aortic commissurotomy Removal of pulmonary artery band Atrial baffle Thoracotomy for hemorrhage Atrial septectomy
Total
Dead
and Late Mortality
2 2 2 1 1 2 1 1 0
0 1 4 3
0 33 66
1
50
0 0 0
0
1
100
12
10
(YO)
:75
0 0
VSD = ventricular septal defect; ASD = atrial septal defect.
Alive D e a d (operative and late)
Age a t Subsequent Operation
Fig 2 . Age (in months) at subsequent operation and outcome.
Follow-up The duration of follow-up has ranged from 2 months to 15 years 9 months, with a mean of 3.5 years. Of the 22 survivors, 17 were asymptomatic. Two had heart failure under medical control, 2 had failed to thrive, and 1 infant had both heart failure and severe failure to thrive. Percentile height and weight were recorded in 16 and 18 of the 22 survivors, respectively. Average height percentile was 36.3 and average weight percentile, 27.7. Systolic blood pressure averaged 125 mm Hg in 19 patients in whom it was recorded at follow-up. Blood pressure in the lower extremity was recorded in 17 survivors and averaged 99.9
mm Hg. Blood pressure gradient arm-to-leg was recorded in 17 patients and averaged 29.8 mm Hg. Seventeen survivors for whom follow-up and sufficient blood pressure data were available were grouped according to excellent, fair, or poor results; criteria and results are given in Table 4. Follow-up in this subgroup averaged 4.2 years. The number of patients was insufficient to make conclusions regarding technique of anastomosis and blood pressure data at follow-up. Four patients required revision of their anastomosis. Three of them died at 13, 20, and 49 days of age following reoperation for aortic thrombosis at the suture line. In these infants a posterior continuous and anterior interrupted suture technique was utilized. A fourth child
547 Macmanus et al: Correction of Aortic Coarctation in Neonates
Table 3 . Causes of Death (Operative and Late) Coarctation Subsequent Repair Operation
Cause of Death Renal failure Left ventricular failure Arrhythmia
1 8a 2
Sepsis Hemorrhage
0
2 5b 2 0 1
l b
Total
12
10
a T w late ~ deaths. late death.
Mortality of Coarctation Repair Mortality due to Subsequenl Surgery
Comment Mortality Although Morgagni [lll and Paris [121 described the pathology of coarctation of the aorta in the late 1700s and Maude Abbott [ll provided her classic clinicopathological study of the entity in 1928, it was not until 1944 that a successful repair was performed, by Crafoord and Nylin [51 in Sweden. Progress during the past 30 years has been rapid, and mortality has progressively declined. In 1962, Schuster and Gross 1133 reported an overall 4.1% mortality rate in 487 coarctation repairs. Unfortunately, this low mortality does
0-10 11-20 21-30 31-40 41-50 51-60 61-70
Age at Coarctation Repair (Days)
Fig 3 . Mortality rate versus age a t coarctation repair.
~Cfo~,,n
0
I m o 6 m o lyr
,
(la
2
3
I
(5;
I
Number of Potients at Risk
4
5
6
,
7
I
8
Years Post -op
Fig 4. Actuarial survival curve of44 patients w h o required coarctation repair in the first 3 months oflife.
underwent repair of coarctation and PDA at 24 days of age. At 11years 2 months he had an atrial baffle placed for correction of partial anomalous venous return. At 15 years of age he began to experience dyspnea on exertion, and catheterization revealed recurrent coarctation. He underwent ascending-to-descending aortic bypass grafting. At follow-up 6 months later he reported an increased exercise tolerance; there was no arm-leg blood pressure gradient. Four other patients, currently being followed at 2, 4, 4, and 5 years of age, have high blood pressure and significant arm-leg gradients and will probably need rerepair in the future. One other patient with high blood pressure and a large gradient was lost to follow-up at 6 years of age.
9
10
u II
12
13
548
The Annals of Thoracic Surgery Vol 24 No 6 December 1977
Table 4 . Results of Coarctation Repair in 17 Survivors (Mean Follow-up, 4.2 Years) No. of
Patients
Percent
Excellent BP difference arm-leg 20 mm Hg, hypertension vresent
subsequent operation plays such a prominent role in mortality in these infants has not been stressed before. Shumacker and co-workers [141, in a review of the literature up to 1968, make a convincing argument that mortality is directly related to the presence of associated anomalies, which are very common in these small infants with congestive heart failure [3, 7, 151. The most critical factor in neonates appears to be the age at onset of heart failure and the necessity for early subsequent operation.
Associated Anomalies The management of associated anomalies in these neonates has not previously been explored in depth. Hallman and associates [lo] stressed aHypertension defined as blood pressure at follow-up the importance of banding the pulmonary artery greater than 2 standard deviations above accepted normals at coarctation repair when catheterization or [2, 8, 91 for age. operative findings indicated the presence of a BP = blood pressure. VSD. This was done in 11 of 24 patients with not pertain to infants, in whom associated VSD in their series of infants less than 1 year of anomalies, both cardiac and noncardiac, are age; 6 of these infants were alive at follow-up common and whose small size alone puts them (mean, 3.5 years). In the present series, 7 infants were thought at at a technical disadvantage should repair be required. In the early 1960s, operative mortality the time of coarctation repair to require pulmorates of 90% [71 and 100% [41 were reported for nary artery banding. Palliative pulmonary artery infants who had coarctation and congestive banding was employed subsequently in 6 heart failure. As surgical techniques and life- others. Eight of these 13 patients had VSD with support systems continue to improve, reports or without associated atrial septa1 defect (ASD), are beginning to appear examining the opera- theoretically easily correctable anomalies. Mortive results in large numbers of these very small tality in this group was 62% (5 of 8). In contrast, infants. Tawes and associates [15] recorded a of 5 patients with VSD with or without as57% hospital mortality (71 of 123) in infants sociated ASD who underwent subsequent total younger than 3 months of age, and Chang and repair, only 1 died, a mortality rate of 20%. DifBurrington [3] reported a 56% operative mortal- ferences are not statistically significant due to ity (24 of 43). The most promising report to date, the small number of patients in each group, nor by Fishman and colleagues [6], reveals an opera- should the groups be considered comparable, tive mortality of 26% (8 of 31) in this age group, because those patients who underwent total rewith only a 14% operative mortality (3 of 22) pair tended to be older at reoperation and, in the judgment of the surgeon at coarctation repair, since 1969. Fourteen (31%) in this series of 44 patients presumably better risks. There is no doubt, died within 30 days of coarctation repair. This is however, that palliation-specifically, pulmosimilar to the operative mortality reported by nary artery banding-has been disappointing in Fishman and colleagues [6]. There were 8 late this setting, and the data are at least suggestive deaths following subsequent operation, how- that earlier total repair, perhaps done concomever, for an overall mortality of 50% (22 of 4) mitantly with coarctation repair, might improve over a mean follow-up of 3.5 years. The fact that survival. 5
29
549 Macmanus et al: Correction of Aortic Coarctation in Neonates
Hypertension Few data are available regarding long-term follow-up in these small infants, generally because most series contain large numbers of older infants and adults. Tawes and colleagues [151 studied 209 children after coarctation repair and found 82% with a normal blood pressure and femoral pulse, 8.6% with slightly decreased femoral pulse and slight blood pressure elevation, and 9.5% with high blood pressure and high arm-leg gradients (mean follow-up, 6 years). Hallman and co-workers [lo] followed, for an average of 3.5 years, 42 survivors who had undergone coarctation repair at 1year of age or less. Fifteen (36%) had an excellent result, 22 (52%)had an arm-leg blood pressure gradient of less than 30 mm Hg, and 5 (12%) had an unsatisfactory result, with gradients greater than 30 mm Hg. Our results (see Table 4) are roughly comparable, although they show a higher percentage of poor results in this younger age group. Recurrence of Coarctation VData concerning the necessity for revision of the anastomosis are equally sparse in this age group. Tawes and associates [151 reported that 20 (9.5%) of 209 children of all ages had obvious signs of recoarctation at follow-up (mean, 6 years). Fourteen of these 20 patients had had coarctation repair in the first 3 months of life. Fewer data are available on the actual incidence. In the present series, 3 patients required early revision of their anastomoses at 1,2, and 49 days following initial repair due to technical failures. All these patients died. A fourth patient, who underwent revision of his anastomosis 15.5 years following initial repair, is doing well. Four other patients, representing 22% of the 18 patients followed l year or more, with a mean of 4.2 years, will require anastomotic revision. Fourteen of 52 survivors (27%) in Tawes' series [15] who were operated on when they were younger than 3 months of age underwent rerepair of their anastomosis, although the average follow-up in this subgroup is not discernible. Although the 4.2-year mean follow-up in our series is one of the longest reported in a large
group of neonates with coarctation, it is conceivable-and perhaps likely-that the rate of coarctation recurrence will increase as these patients are followed into maturity. That 1 patient required reoperation at 15 years of age reiterates the importance of close, long-term follow-up in these patients.
References 1. Abbott ME: Coarctation of the aorta of the adult type. Am Heart J 3:392, 1928 2. Allen-Williams GM: Pulse-rate and blood pressure in infancy and early childhood. Arch Dis Child 20:125, 1945 3. Chang JHT, Burrington JD: Coarctation of the aorta in infants and children. J Pediatr Surg 7:127, 1972 4. Cooley DA, Hallman GL: Cardiovascular surgery in the first year of life: experience with 450 consecutive operations. Am J Surg 107:474, 1964 5. Crafoord C, Nylin G: Congenital coarctation of the aorta and its surgical therapy. J Thorac Surg 14:347, 1945 6. Fishman NH, Bronstein MH, Berman W, et al: Surgical management of severe aortic coarctation and interrupted aortic arch in neonates. J Thorac Cardiovasc Surg 71:35, 1976 7. Glass IH, Mustard WT, Keith JD: Coarctation of the aorta in infants: a review of 12 years experience. Pediatrics 26:109, 1960 8. Goodman HG, Cummings GR, Raber MB: Photocell oscillometer for measuring systolic pressure in newborns. Am J Dis Child 103:152, 1962 9. Graham AW, Hines EA, Gage RP: Blood pressure in children between the ages of 5 and 16 years. Am J Dis Child 69:203, 1945 10. Hallman GL, Yashar JJ, Bloodwell RD, et al: Surgical correction of coarctation of the aorta in the first year of life. Ann Thorac Surg 4:106, 1967 11. Morgagni JB: De sedibus et causis morborum. Epist XVIII, Article VI, 1760 12. Paris M: Retrecissement considerable de l'aorte pectorale observe a l'H6tel Dieu de Paris. J Chir Desault 2:107, 1791 13. Schuster SR, Gross RE: Surgery for coarctation of the aorta: a review of 500 cases. J Thorac Cardiovasc Surg 43:54, 1962 14. Shumacker HB, Nahnvold DL, King H, et al: Coarctation of the aorta. Curr Probl Surg2:1,1968 15. Tawes RL, Aberdeen E, Waterston DJ, et al: Coarctation of the aorta in infants and children: a review of 333 operative cases, including 179 infants. Circulation 39,4O:Suppl 1:173, 1969
