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J Thorac Cardiovasc Surg 1994;108:525-531
© 1994 Mosby, Inc.
SURGERY FOR CONGENITAL HEART DISEASE |
Groningen, The Netherlands
From the Division of Cardiothoracic Surgery, University Hospital Groningen, Groningen, The Netherlands.
Received for publication Nov. 12, 1993. Accepted for publication April 13, 1994. Address for reprints: René M. H. J. Brouwer, MD, Division of Cardiothoracic Surgery, University Hospital Groningen, Oostersingel 59, 9700 RB Groningen, The Netherlands.
Abstract
The optimal age for elective repair of aortic coarctation is controversial. The optimal age should be associated with a minimal risk of recoarctation, late hypertension, and other cardiovascular disorders. The purpose of this retrospective study is to determine the actuarial survival after aortic coarctation repair 25 years or more after operation and to calculate the optimal age for elective aortic coarctation repair. From 1948 to 1966, 120 consecutive patients underwent aortic coarctation repair. Eighty-seven were male (72.5%). The mean age at operation was 15.5 years (SD ± 9.1 years). Resection and end-to-end anastomosis was performed in 103 patients (85.8%). Early mortality occurred in 6 patients as a result of surgical problems, whereas late mortality in 15 patients was predominantly caused by cardiac causes. The mean follow-up period was 32 years (range 25 to 44.2 years). Ninety-two patients (96.8%) were in New York Heart Association class I. The probability of survival 44 years after operation was 73%. Patients younger than 10 years at operation had the highest probability of survival at 97%. Multivariate analysis produced age at operation as the only incremental risk factor for the occurrence of recoarctation, of late hypertension, and of premature death. So that these sequelae can be avoided, elective aortic coarctation repair should be performed around 1.5 years of age. At that age, the probability of recoarctation will have decreased to less than 3%, and the probability of upper body normotension and long-term survival will be optimal. (J THORAC CARDIOVASC SURG 1994;108:525-31)
Unrepaired aortic coarctation is known to have a detrimental effect on survival. Survival is jeopardized by heart failure, rupture of the aorta, intracranial hemorrhage, endocarditis, hypertension, or coronary artery disease. Ninety percent of untreated patients die of these complications before the age of 50 years. 
1-3 The first clinical surgical repair of this anomaly was reported by Craaford and Nylin 4 in 1945. Ever since, different studies have revealed early success of the repair in terms of decreased mortality and lowering of blood pressure. However, the success of elective repair diminishes with older age at repair. 5-14
On the other hand, aortic coarctation repair in infancy carries an increased risk of recoarctation. 15-19 Therefore, to calculate the optimal age for elective repair, the surgeon should steer a course between the risk of recoarctation and the risk of late hypertension and other cardiovascular disorders.
The purpose of this retrospective study is to determine the actuarial survival after aortic coarctation repair in patients with a follow-up of more than 25 years and to calculate the optimal age for elective aortic coarctation repair.
PATIENTS AND METHODS
From 1948 to 1966, 120 consecutive patients underwent surgical repair of an aortic coarctation at the Division of Cardiothoracic Surgery of the University Hospital of Groningen. The group comprised 87 male (72.5%) and 33 female patients. The mean age at operation was 15.5 years (SD* ± 9.1 years, range 0.5 year to 48.0 years). Thirty-seven patients (30.8%) were less than 10 years old at operation (group 1), 57 patients (47.5%) were between 10 and 20 years old at operation (group 2), and 26 patients (21.7%) were older than 20 years at operation (group 3). Preoperatively, 61 patients (50.8%) had one symptom or more (Table I). In the other 59 patients, the aortic coarctation was found by chance during a medical examination. On auscultation, 108 patients (90%) had a systolic murmur over the precordium. The mean systolic blood pressure in the left arm was 169 mm Hg (SD ± 120 to 240 mm Hg), and the mean systolic blood pressure in the left leg was 37 mm Hg (SD ± 56 mm Hg, range 0 to 170 mm Hg). Femoral pulsations were found to be absent in 77 patients (64.1%). Left ventricular hypertrophy was found on the electrocardiogram in 78 patients (65.5%). Rib notching was seen on the chest roentgenogram in 33 patients (27.5%) and a dilated ascending aorta in 9 patients (7.5%). In all patients, the aortic coarctation was confirmed by angiography. Aortic valve stenosis as a concomitant anomaly was seen in 5 patients (4.1%), aortic valve regurgitation in 5 other patients, ventricular septal defect in 4 patients (3.3%), and mitral valve regurgitation was found in another 2 patients (1.6%).
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20 (end-to-end anastomosis of the left subclavian artery to the descending aorta) in 12 patients (10%) in the period 1951 to 1962, and insertion of a prosthetic vascular graft in 5 patients (4.2%). Thoracic aneurysms were identified during the operation in 6 patients and were resected during the same surgical procedure: 4 aneurysms of an intercostal artery, 1 of the left subclavian artery, and 1 of the descending aorta. Recent follow-up data were collected by two questionnaires—one sent to the patient and one to the family physician. The questionnaires included demographic and current health status data, as well as questions concerning cardiac-related events, symptoms, and medications. Additional information was obtained from the cardiologist or other specialist if necessary. Hypertension was defined when the blood pressure in the right arm exceeded 160/90 mm Hg at regular blood pressure measurements.
The mean follow-up period was 32 years (SD ± 4.8 years, range 25 to 44.2 years). Four patients (3.8%) were lost to follow-up.
Statistical analysis.
All data were summarized in contingency tables. A time-related analysis according to the Kaplan-Meier method was done to depict the probability of survival of the whole group and was stratified into different age groups. Comparisons of all continuous variables between the groups were made by one-way analysis of variance. The null hypothesis, that is, that no difference existed between the groups, was rejected at a p value of 0.05 or less. To determine incremental risk factors for survival, we also performed a stepwise multivariate logistic regression analysis. p Values of 0.05 or less were considered to be significant.
RESULTS
Clinical course.
Twenty-one of 120 patients (17.5%) died in our series. Six patients (5%) died within 48 hours after coarctation repair, 5 due to tear of the suture line and 1 due to an unexplained cause. Late postoperative mortality occurred in 15 patients (12.5%) at a mean of 17.8 years (SD ± 11.7 years) after operation. The mean age at operation of these 15 patients was significantly higher (18.7 years, SD ± 7.4 years) than that of the survivors (14.3 years, SD ± 8.2 years; p = 0.036). The mean age at death was 37 years (SD ± 11.7 years, range 13.2 to 57.5 years). Cardiovascular causes were predominant: myocardial infarction in 5 patients (4.2%), rupture of a true aneurysm of the ascending aorta in 2 patients (3.4%), both cerebrovascular accident and bleeding of the pulmonary artery in 1 patient (1.7%), and sudden death in 6 patients (5.2%).
Late morbidity occurred in an additional 55 patients (45.8%): hypertension in 29 patients (25%) and valvular anomalies in 13 patients (11.2%); aortic valve stenosis necessitated aortic valve replacement in four, and mitral valve regurgitation necessitated mitral valve replacement in 2. Nine patients (7.7%) had angina pectoris, and 4 of them eventually had a myocardial infarction. Recurrent coarctation was found in 2 patients (4.5%) who had undergone initial repair at the ages of 4 and 8 years, respectively: one patient underwent a reoperation 16 years after the initial repair and the other is being treated medically. Finally, a cerebrovascular accident and a carcinoma of the lung both developed in 1 patient (1.7%).
At most recent follow-up, 95 patients (79.2%) were alive with a mean age of 46.6 years (SD ± 10.2 years, range 31 to 79 years). Ninety-two (96.8%) were in New York Heart Association class I, 2 patients in class II, and 1 patient in class III.
Survival.
The probability of survival (Kaplan-Meier) in our series was 93% at 1 year after operation, 89% at 10 years, 88% at 20 years, 81% at 30 years, and 73.8% at 44 years (Fig. 1). However, it appeared that the survival of an age- and era-matched group based on cohort life tables of the normal population after 40 years differed significantly at 93% (p = 0.026). 21
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Table II. The null hypothesis that no difference existed between the groups was rejected for age at operation and the preoperative systolic blood pressure (p = 0.0124 and p = 0.0254, respectively). The multivariate analysis that followed produced age at operation as the only incremental risk factor for survival (logistic coefficient -0.0710, p = 0.03).
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The follow-up of our group of patients for aortic coarctation is one of the longest published until now, with a probability of survival at 44 years after elective coarctation repair of 73%. 5-14,22 These results are in contrast to the survival of patients not undergoing an operation: nearly 90% of those patients die before reaching the age of 50 years. 23 However, the course of the actuarial survival curve shows a striking phenomenon: it declines more from the second postoperative decade onward then does the natural course of a matched population. This phenomenon indicates that despite the early apparent success after elective aortic coarctation repair, not all patients live healthily ever after but remain at some risk of premature death. It must be borne in mind, though, that the age at operation of our group of patients is old compared with current practice.
Premature death long after successful aortic coarctation repair is an alarming event. Seventy-one percent of the mortality in our series was the result of a cardiovascular cause. The explanation for this can be manifold. Left ventricular hyperkinesia and increased ventricular mass are found even in normotensive patients long after successful aortic coarctation repair: increased ventricular mass can provoke rhythm disturbances and may cause an imbalance between muscle and coronary vessel growth with subsequent decreased oxygen reserve. 24-26 Moreover, hypertension and coronary sclerosis as normal aging processes will further accelerate the effects of these hemodynamic changes and may explain the increased likelihood of sudden death and myocardial infarction. 27-29 Therefore, even long after successful elective aortic coarctation repair, patients appear to remain at risk for premature death mainly because of cardiovascular disorders.
The age at operation has a key position in the optimal treatment for elective aortic coarctation repair. In the past era, the ideal age for elective aortic coarctation repair was supposed to be between 4 years and adolescence in an attempt to avoid the high operative mortality in neonates, to minimize the occurrence of recoarctation, and to avoid late cardiovascular complications. 30-33 Gradually the timing for elective aortic coarctation repair has shifted toward infancy. Currently, instead of age at operation, the presence of congestive heart failure in infants with an aortic coarctation plays an important role in the clinical decision whether to operate promptly. If congestive heart failure is present, prompt surgical repair is generally accepted to be mandatory. However, the issue remains when to operate electively on the infant with isolated aortic coarctation who is symptom-free.
To calculate the optimal age for elective aortic coarctation repair, the physician should take into account three potential problems. First, young age at operation and even more strongly low weight at operation are incremental risk factors for the prevalence of recoarctation. In a previous study we demonstrated that in infants less than 2 years of age with an aortic coarctation the prevalence of persisting coarctation or recoarctation was strongly related to low weight at operation: the lower the weight, the higher the probability of recoarctation. 15 The linear correlation between weight and age at operation in that study was almost perfect (r = 0.94, p < 0.001). After exclusion of weight at operation from the multivariate analysis, the subsequent analysis produced age at operation as an incremental risk factor for recoarctation: the younger the patient at operation, the higher the probability of recoarctation. This probability is 22% to 33% when the repair is performed in neonates, about 15% at 6 months of age, and less than 3% when the infant is older than 1.5 years of age. Thus these findings, which concur strongly with those reported by Kirklin and Barratt-Boyes, 23 suggest that the optimal age for elective coarctation repair is beyond 3 years to avoid recoarctation (Fig. 3).
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6,12 Older age at operation, endocrine factors, abnormal compliance of upper body small blood vessels, stiffness of the aorta proximal to the aortic coarctation repair, and persistent recoarctation are associated with late hypertension. 34-40 Currently, less is known about the influence of performing the operation very early in life on the prevalence of late hypertension. Therefore, age at operation, the preoperative systolic blood pressure, and follow-up as independent variables were included in this present study into a multivariate analysis for late hypertension: the analysis produced age at operation as the only incremental risk factor for the prevalence of late hypertension (logistic coefficient = 0.0685, p = 0.016): even if the aortic coarctation repair is performed in neonatal life, the probability for late hypertension is more than 10% (Fig. 4).
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41 in which the risk for late hypertension even in infants less than 6 months of age at repair was significantly higher than that of the normal population. Finally, age at operation for aortic coarctation repair also is an incremental risk factor for premature death: it superimposes a risk factor above the "normal" risk for death found in the normal population. The risk for premature death remains fairly constant up to 9 years of age but increases beyond that age (Fig. 5).
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15 and the present study on late hypertension and premature death. A major problem, though, is that these results come from different patient populations, operated on in different time frames. However, it is impossible to use other data sets, because nobody would now operate electively on patients at an age that was common several decades ago. The composite nomogram, combining the nomograms for the risk for recoarctation, late hypertension, and premature death after aortic coarctation repair, shows the optimal freedom from these sequelae to be around 1.5 years of age. The reason is that the risks for late hypertension and premature death are almost constant in that age range, and the risk for recoarctation drops rapidly and levels off at 1.5 years of age. Therefore, we conclude that the combined risk is lowest at this age (Fig. 6).
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Appendix: APPENDIX
Probability estimates were obtained from the logistic regression equation according to the following formulas:
where e is the base of the natural logarithm, b0 is the intercept of the logistic equation, and b1and bk are logistic regression coefficients associated with the values for the incremental risk factors x1 and xk.
Footnotes
References
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|
|
|
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|
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|
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|
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|
|
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|
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|
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|
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|
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|
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|
|
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|
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|
|
|
|
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|
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|
|
|
|
|
|
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|
|
|
|
|
|
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|
|
|
|
|
|
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|
|
|
|
 |
|
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|
|
|
|
 |
|
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|
|
|
|
|
|
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|
|
|
|
|
|
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|
|
|
|
 |
|
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|
|
|
|
|
|
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|
|
|
|
|
|
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|
|
|
|
|
|
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|
|
|
|
|
|
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|
|
|
|
|
|
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|
|
|
|
|
|
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|
|
|
|
|
|
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|
|
|
|
|
|
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|
|
|
|
|
|
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