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Surgical correction of coarctation of the aorta
J
THORAC CARDIOVASC SURG
85:532-536, 1983
Surgical correction of coarctation of the aorta Influence of age on late results The influence of age at operation on long-term results was studied in three different age .groups of patients with coarctation of the aorta. The groups consisted of 19 children, 19 younger adults, and 20 older adults: 5 to 15, 16 to 31, and 35 to 62 years of age, respectively, at operation. Four patients died early of postoperative complications. The surviving patients were followed up for a mean period of 28, 29,15 years, respectively. Twelve patients had died during the follow-up period. Three of them died of unrelated diseases and nine died of cardiovascular complications. At follow-up, hypertension was diagnosed in only one patient operated upon in childhood (9%). The incidence of hypertension was significantly higher (46%) in patients operated upon at older ages. All of the patients operated upon in childhood were in Functional Class I (NYHA): 10 of 26 older patients were in Class II or III. All of the children had a normal heart size, whereas seven of 26 patients in the two older groups had cardiac enlargement. Six of these seven patients had aortic valvular disease. A peak systolic pressure difference of 20 mm Hg or more across the anastomosis was measured at catheterization in only two of 29 patients. Aortic valvular disease was diagnosed in 37% of patients operated upon in childhood, in 58% of the younger adults, and 45% of the older adults. In light of the high late mortality and morbidity rates, surgical repair should no longer be considered as curative. Patients with coarctation of the aorta should be operated upon before school age in order to prevent hypertension. All patients having coarctation repair must be followed up carefully in order to detect aortic valvular disease.
Leif Bergdahl, M.D., Viking Olof Bjork, M.D., and Rune Jonasson, M.D., Stockholm, Sweden
Different opmions exist as to when patients with coarctation of the aorta should undergo operation. Recently, Patel, Singh, Abrams, and Roberts! suggested that, in order to prevent persistent hypertension, resection of the coarctation should be done at 1 year of age. Mustard, Rowe, Keith, and Sirek" recommended operation between 3 and 6 years of age. Noordijk, Oey, and van den Boogaard'' reported persistent hypertension in only two of 36 patients operated upon before 12 years of age. In most reports, recommendations regarding the optimum age for operation vary between 4 and 12 years." " This report analyzes the long-term results in three different age groups of patients operated upon for coarctation of the aorta. From the Thoracic Surgical Clinic and the Department of Clinical Physiology, Thoracic Clinics, Karolinska sjukhuset, Stockholm, Sweden. Received for publication March 16, 1982. Accepted for publication June 7, 1982. Address for reprints: Leif Bergdahl, M.D., Thoracic Surgical Clinic, Karolinska sjukhuset, S-I04 01 Stockholm, Sweden.
532
Patients The first 19 consecutive Swedish children (range 5 to 15 years, mean 10 years) and the first 19 consecutive Swedish adult patients operated upon by Crafoord during the period from October, 1944, to January, 1953, were all followed up. The first patient 35 years of age or more was operated upon in 1957. Twenty consecutive patients in this age group operated upon during the following 9 years were also included in this study. Symptoms The different preoperative symptoms in the three age groups are listed in Table I. Seven children (Group I) and one patient 35 years of age or more (Group III) had no symptoms. All patients were hypertensive. The criterion for hypertension in children was a blood pressure of more than 130/80 mm Hg measured by a cuff on the right arm; in the two other groups the criterion was 160190 mm Hg measured in the same way. Rib notching was found in 13 of 19 children and in 18 of 20 older adults (Group III). The most prominent symptoms were dyspnea and fatigue. The symptoms were clearly more pronounced in older patients than in children.
Volume 85 Number 4 April,1983
Coarctation of aorta
Table I. Preoperative data in three different age groups of patients
I
Age groups
Group (5-15 yr)
Table DB. Causes of late deaths Age at operation (yr)
III
Group (35-62 yr)
5 6
20 20 18 10 10 8 8 8 4 4 2 5
10 10 23 24 27 31 37 38 46
Cause of death
19 19 13
19 19 15 4 4 3 5 3 4 4 2 2 0
I
3 0 I
2 I
0 2 3
7
I
Table IIA. Causes of early deaths Age at operation (yr)
Cause of death
8 25
4 wk 2 hr
25 46
4 wk 7 wk
Bowel necrosis Hemorrhage from an intercostal artery Rupture of the suture line Rupture of the suture line
Table DI. Early and late deaths in the three age groups Age at operation (yr)
No. of patients
Meanfollow-up period (yr)
Early deaths
Late deaths
No. still alive
5-15 16-31 35-62
19 19 20
28 29 15
I 2 I
5 4
13 13 16
3
Table IV. Functional classes at follow-up
Surgical technique Resection of the coarctation with end-to-end anastomosis was performed in all 38 of the children and patients in the age group of 16 to 31 years (Group II). In his first 20 patients, Crafoord used an over-andover continuous silk suture and he carefully avoided penetrating the intima with the needle. This technique resulted in pseudoaneurysm formation in one patient (who was successfully reoperated upon) and in a fatal rupture of the suture line in another. Crafoord therefore changed to a continuous everting suture both posteriorly and anteriorly in adults. In children (beginning with Child No.6), he used a continuous everting suture line posteriorly and isolated mattress sutures anleriorly to permit growth of the anastomosis. In 13 of the 20 older adults (Group III), a direct end-to-end anastomosis was performed. The anastomoses were sewn with everting continuous silk or Mersilene suture. The whole aortic wall was penetrated with these sutures. In seven Group III patients, resection of the coarctation (including an aortic aneurysm in one patient) and reconstruction of the aorta with a tubular Dacron graft were performed and the anastomoses were sewn with continuous silk or Mersilene sutures.
Barbiturate intoxication Disseminated sclerosis Cardiac failure Tight subvalvular aortic stenosis Cardiac failure Aortic valvular disease Aortic valvular disease Aortic valvular disease Aortic valvular disease Aortic valvular disease Colonic carcinoma Aortic valvular disease
30 25 15 14 16 41 43 40 58 49 56 60
8
No. of patients Systemic hypertension Rib notching Dyspnea Fatigue Palpitation Angina Headache Tired legs Vertigo Cold feet Other symptoms No symptoms
533
Functional class (NYHA)
Age at operation (yr)
Mean age at followup (yr)
No. of patients followed up
5-15 16-31 35-62
37 51 58
II
II
13 13
10 6
IIIIIIIIIIV 0 2 6
0 I
I
0 0 0
Legend: NYHA. New York Heart Association.
Early deaths Four patients died early while in the hospital (Tables I1A and III). One 8-year-old child died of bowel necrosis 4 weeks after the primary operation. Microscopy revealed panarteri tis (periarteritis nodosa) . One 25-year-old patient died 2 hours after the operation because of hemorrhage from the right second intercostal artery. The artery had been divided and the central ligature had cut through the thin wall. Another 25-year-old patient was examined 20 days after operation for a hemothorax on the left. Evacuation of blood and decortication of the upper and lower lobes were performed. However, the clot over the aortic wall was not removed. He died suddenly 4 weeks after the
The Journal of
5 34 Bergdahl. Bjork. Jonasson
Thoracic and Cardiovascular Surgery
Table V. Number of patients with hypertension or cardiac enlargement at follow-up Age at operation (yr)
No. ofpatients operated upon
No. of patients followed up
Mean follow-up period (surviving patients) (yr)
No. with hypertension (>160190)
No. with cardiac enlargement *
5-15 16-31 35-62
19 19 20
II
28 29 15
I (9%)t 6 (46%) 6 (46%)
4 3
13 13
o
*Nonnal values: men <500 ml/rrr' BSA; women <450 ml/m' BSA. tPercentage of patients who were followed up.
Table VI. Aortic valvular disease at follow-up Age at operation (yr)
No. of patients operated upon
Mean follow-up period (surviving patients) (yr)
5-15 16-31 35-62
19 19 20
28 29 15
Aortic valvular disease 7 (37%)* 11 (58%) 9(45%)
*Percentage of patients operated upon (this percentage refers to patients operated upon rather than those followed up. since some patients died of aortic valvular disease during follow-up.)
primary operation following a hemoptysis. Autopsy revealed a 2.5 em rupture of the suture line on the anterior wall of the aorta. The fourth patient, 46 years of age, died during reoperation for rupture of the suture line 7 weeks after the primary operation. The rupture was probably due to infection.
Late deaths Twelve patients died during a follow-up period of 12 to 32 years (mean 24 years) (Tables lIB and III). Three patients, 5, 6, and 38 years of age (at operation), died of unrelated disease. No information about their blood pressure could be found. One patient died of a tight subvalvular aortic stenosis 4 years postoperatively at the age of 14 years. Six patients with repair at ages 23 to 46 years died of aortic valvular disease. No information about their postoperative blood pressures could be obtained. Two patients died in cardiac decompensation at 15 and 16 years of age; no information about their postoperative blood pressures was available and autopsy was not performed.
Late results Functional classes. All 12 of the children who were followed up were in New York Heart Association (NYHA) Functional Class I (Table IV). Ten of 13 patients in the younger adult group (Group II) were in Functional Class I, two in Class II, and one in Class III.
All three patients (16 to 35 at repair) who were ultimately classified in NYHA Functional Class II or III had had aortic replacement at operation. The results were worse in the 13 older adult patients (Group III). Only six of them were in Functional Class I. Six of the remaining seven patients had aortic valvular disease. Blood pressure. Blood pressure measured at rest by a cuff on the right arm was higher than 160/90 mm Hg in only one of 11 children (9%) (Table V). Two patients operated upon in childhood did not consent to physical follow-up examination; both of them, however, reported that they were in good health and that the blood pressure had been normal at earlier postoperative examinations. Three patients operated upon at 35 to 62 years of age did not consent to a follow-up examination; they were also in good health but had not had any blood pressure determination in recent years. Forty-six percent of the patients in the other two groups had high blood pressure at follow-up (Table V). Aortic valvular disease. Aortic valvular disease (significant pressure gradient and/or valvular insufficiency) was diagnosed at follow-up or autopsy in 37% of the children, in 58% of the younger adults, and in 45% of the older adults (Table VI). The mean followup period, however, was significantly shorter in the last group of patients. Aortic valve replacement had been performed in four patients. One of these died because of thrombosis of a Bjork-Shiley tilting disc valve 14 months postoperatively. The remaining three patients were in good health with good results on exercise tolerance tests. Heart size. All the children followed up had a normal heart size (Table V). Seven of 26 Group II and III patients had enlarged hearts, six of whom had aortic valvular disease. Catheterization. The heart catheterizations were usually performed at rest and during exercise and included simultaneous pressure measurements proximal and distal to the anastomosis as well as in the left ventricle and ascending aorta. There was a significant (more than 20 mm Hg) pressure difference across the
Volume 85
Coarctation of aorta
Number 4 April,1983
5 35
Q,l/min 15
10
5
"02' ml STPD/min
O-t----r-----,.-----r----""'T"""
o
500
1000
1500
2000
Fig. 1. Cardiac output (Q, L1min) in relation to oxygen uptake (V0 2 ' ml STPD/min) at rest and during supine exercise in nine young (0) and 12 old (e ) patients operated upon for coarctation of the aorta. Thick line = regression of Q on V0 2 in healthy young persons; broken line = - 2 SD. anastomoses in only two of 29 patients at rest (Table VII). The pressure differences usually increased during exercise, and a significant peak systolic pressure difference was found in 11 of 20 patients. An elevated left ventricular end-diastolic pressure was found in 14 of 25 examined patients (Table VII). Oxygen consumption was measured by collecting expired air in Douglas bags and analyzing it for oxygen and carbon dioxide according to the method of Scholander. Gas volumes were measured with a spirometer. Cardiac output (Fick method) in relation to oxygen uptake was measured at the follow-up examination and was normal at rest in all nine investigated patients with operations performed in childhood (Fig. 1). This relation increased normally in six of the seven exercised patients. In contrast, five of the 12 investigated Group III patients had a low cardiac output, four of whom had aortic valvular disease (Fig. 1). Catheterization had been performed in eight of the 13 patients with hypertension at follow-up. In eight patients the catheterization had been performed at rest, and only one of the patients had a pressure difference of 20 mm Hg or more; the remaining seven patients had differences between 0 and 16 mm Hg. In five of the eight patients catheterization had been performed during excercise and in one patient a pressure difference of 12 mm Hg was recorded; the remaining four patients had values between 27 and 70 mm Hg.
Fig. 2. Aortogram of the first patient in the world operated upon for coarctation of the aorta. This study was made 32 years postoperatively and shows the anastomotic diameter to be about 70% that of the aortic arch. No gradient at rest was recorded. Discussion There was a high incidence of aortic valvular disease in these patients; the incidence in age group 16 to 31 years (Group II), for example, was 58%. The frequency of aortic valvular disease was higher in this study than previously reported. This probably is due to the very long follow-up period and to the fact that many patients in this study underwent cardiac catheterization at follow-up. In view of the high frequency of aortic valvular disease and hypertension, patients operated upon for aortic coarctation should be followed up carefully. Our study has also revealed the importance of the aortic valvular disease regarding the prognosis of patients who have had coarctation operations. Nine of the 12 late deaths were due to cardiac diseases and six of seven patients with cardiac enlargement at follow-up had aortic valvular disease. Only two of 20 catheterized patients had a peak systolic pressure difference at rest across the anastomosis of 20 mm Hg or more. The minimum pressure difference at rest necessary to produce hypertension in experimental animals has been shown to be 20 nun Hg.7
The Journal of
5 36 Bergdahl, Bjork, Jonasson
Thoracic and Cardiovascular Surgery
Table VII. Some catheterization data: three different age groups Age at operation (yr) 5-15 16-31 35-62
No. a/patients examined 8 9
12
Significant (>20 mm Hg) PSPD at rest 2
o
o
No. a/patients examined
Significant (>20 mm Hg) PSPD during work
No. a/patients examined
Elevated LVEDP >13 mm Hg
6 4 10
5 3 3
8 5 12
4 4 6
Legend: PSPD, Peak systolic pressure difference. LVEDP, Left ventricular end-diastolic pressure.
However, only one of the two patients with a significant stenosis at rest was hypertensive. It is difficult to evaluate the significance of a pressure difference during work. A pressure difference of 25 to 60 rnrn Hg during work was found in five of six patients operated upon in childhood, and four of these five patients had a normal blood pressure. We also found a pressure difference of 20 to 70 mm Hg during work over the anastomosis (or Dacron graft) in three of 10 exercised patients in Group III, and only two of these three patients were hypertensive. Our study has thus shown that other factors than a residual stenosis can be responsible for postoperative hypertension. We found no difference in the Group III in the results between patients operated upon with end-to-end anastomosis and with a tubular Dacron graft. The pressure difference over the operative site in five of seven patients with end-to-end anastomosis and in one of the five with ~ Dacron graft was small in all and judged to be of no clinical significance. This study has shown that resection of a coarctation with end-to-end anastomosis in childhood gives good long-term results as judged by the blood pressure; only 9% of the patients were hypertensive about 30 years after the operation. Other operative methods should be considered in patients in whom only a narrow anastomosis is possible. In these patients a tubular Dacron graft should be used instead of patch grafting because of a considerable risk of aneurysm formation. 8 In the two older age groups the incidence of high blood pressure was much higher-46%. Even considering the increased prevalence of hypertension with age in the general population, these results speak in favor of early operation. Like us, Maron, Humphries, Rowe, and Mellits? found an increased frequency (37%) of
hypertension in older patients (mean age at operation 22 years) followed up for an average of 18 years. We recommend operation in childhood in order to prevent persistent hypertension. As there are no technical advantages to be gained by waiting until 12 to 15 years of age, the operation can be performed before school age.
2
3
4
5
6
7
8
9
REFERENCES Patel R, Singh SP, Abrams L, Roberts KD: Coarctation of aorta with special reference to infants. Long-term results of operation in 126 cases. Br Heart 1 39: 1246- I253, 1977 Mustard WT, Rowe RD, Keith lD, Sirek A: Coarctation of the aorta with special reference to the first year of life. Ann Surg 141:429-436, 1955 Noordijk lA, Oey FrI, van den Boogaard HM: Late results of the operation for coarctation in the growing children. Arch Dis Child 40:192-196, 1965 Schuster SR, Gross RE: Surgery for coarctation of the aorta. A review of 500 cases. 1 THoRAc CARDIOVASC SURG 43:54-70, 1962 Keith lD, Rowe RD, Vlad P: Heart Disease in Infancy and Childhood, New York, 1967, Macmillan Publishing Co., Inc., p 226 Duffie ER, Wilson lL: Coarctation of the aorta, Heart Disease in Infancy, Childhood and Adolescence, Al Moss, FH Adams, editors, Baltimore, 1968, The Williams & Wilkins Company, p 395 Sykes Bl, Kreuzer W, Schenk WG lr: A new method for preparation of experimental coarctation of the aorta. Surg Gynecol Obstet 135:541 -544, 1972 Bergdahl L, Ljungqvist A: Long-term results after repair of coarctation of the aorta by patch grafting. 1 THoRAc CARDIOVASC SURG 80:177-181, 1980 Maron Bl, Humphries lON, Rowe RD, Mellits ED: Prognosis of surgically corrected coarctation of the aorta. A 20 year postoperative appraisal. Circulation 47: I 19-I26, 1973
THORAC CARDIOVASC SURG
85:532-536, 1983
Surgical correction of coarctation of the aorta Influence of age on late results The influence of age at operation on long-term results was studied in three different age .groups of patients with coarctation of the aorta. The groups consisted of 19 children, 19 younger adults, and 20 older adults: 5 to 15, 16 to 31, and 35 to 62 years of age, respectively, at operation. Four patients died early of postoperative complications. The surviving patients were followed up for a mean period of 28, 29,15 years, respectively. Twelve patients had died during the follow-up period. Three of them died of unrelated diseases and nine died of cardiovascular complications. At follow-up, hypertension was diagnosed in only one patient operated upon in childhood (9%). The incidence of hypertension was significantly higher (46%) in patients operated upon at older ages. All of the patients operated upon in childhood were in Functional Class I (NYHA): 10 of 26 older patients were in Class II or III. All of the children had a normal heart size, whereas seven of 26 patients in the two older groups had cardiac enlargement. Six of these seven patients had aortic valvular disease. A peak systolic pressure difference of 20 mm Hg or more across the anastomosis was measured at catheterization in only two of 29 patients. Aortic valvular disease was diagnosed in 37% of patients operated upon in childhood, in 58% of the younger adults, and 45% of the older adults. In light of the high late mortality and morbidity rates, surgical repair should no longer be considered as curative. Patients with coarctation of the aorta should be operated upon before school age in order to prevent hypertension. All patients having coarctation repair must be followed up carefully in order to detect aortic valvular disease.
Leif Bergdahl, M.D., Viking Olof Bjork, M.D., and Rune Jonasson, M.D., Stockholm, Sweden
Different opmions exist as to when patients with coarctation of the aorta should undergo operation. Recently, Patel, Singh, Abrams, and Roberts! suggested that, in order to prevent persistent hypertension, resection of the coarctation should be done at 1 year of age. Mustard, Rowe, Keith, and Sirek" recommended operation between 3 and 6 years of age. Noordijk, Oey, and van den Boogaard'' reported persistent hypertension in only two of 36 patients operated upon before 12 years of age. In most reports, recommendations regarding the optimum age for operation vary between 4 and 12 years." " This report analyzes the long-term results in three different age groups of patients operated upon for coarctation of the aorta. From the Thoracic Surgical Clinic and the Department of Clinical Physiology, Thoracic Clinics, Karolinska sjukhuset, Stockholm, Sweden. Received for publication March 16, 1982. Accepted for publication June 7, 1982. Address for reprints: Leif Bergdahl, M.D., Thoracic Surgical Clinic, Karolinska sjukhuset, S-I04 01 Stockholm, Sweden.
532
Patients The first 19 consecutive Swedish children (range 5 to 15 years, mean 10 years) and the first 19 consecutive Swedish adult patients operated upon by Crafoord during the period from October, 1944, to January, 1953, were all followed up. The first patient 35 years of age or more was operated upon in 1957. Twenty consecutive patients in this age group operated upon during the following 9 years were also included in this study. Symptoms The different preoperative symptoms in the three age groups are listed in Table I. Seven children (Group I) and one patient 35 years of age or more (Group III) had no symptoms. All patients were hypertensive. The criterion for hypertension in children was a blood pressure of more than 130/80 mm Hg measured by a cuff on the right arm; in the two other groups the criterion was 160190 mm Hg measured in the same way. Rib notching was found in 13 of 19 children and in 18 of 20 older adults (Group III). The most prominent symptoms were dyspnea and fatigue. The symptoms were clearly more pronounced in older patients than in children.
Volume 85 Number 4 April,1983
Coarctation of aorta
Table I. Preoperative data in three different age groups of patients
I
Age groups
Group (5-15 yr)
Table DB. Causes of late deaths Age at operation (yr)
III
Group (35-62 yr)
5 6
20 20 18 10 10 8 8 8 4 4 2 5
10 10 23 24 27 31 37 38 46
Cause of death
19 19 13
19 19 15 4 4 3 5 3 4 4 2 2 0
I
3 0 I
2 I
0 2 3
7
I
Table IIA. Causes of early deaths Age at operation (yr)
Cause of death
8 25
4 wk 2 hr
25 46
4 wk 7 wk
Bowel necrosis Hemorrhage from an intercostal artery Rupture of the suture line Rupture of the suture line
Table DI. Early and late deaths in the three age groups Age at operation (yr)
No. of patients
Meanfollow-up period (yr)
Early deaths
Late deaths
No. still alive
5-15 16-31 35-62
19 19 20
28 29 15
I 2 I
5 4
13 13 16
3
Table IV. Functional classes at follow-up
Surgical technique Resection of the coarctation with end-to-end anastomosis was performed in all 38 of the children and patients in the age group of 16 to 31 years (Group II). In his first 20 patients, Crafoord used an over-andover continuous silk suture and he carefully avoided penetrating the intima with the needle. This technique resulted in pseudoaneurysm formation in one patient (who was successfully reoperated upon) and in a fatal rupture of the suture line in another. Crafoord therefore changed to a continuous everting suture both posteriorly and anteriorly in adults. In children (beginning with Child No.6), he used a continuous everting suture line posteriorly and isolated mattress sutures anleriorly to permit growth of the anastomosis. In 13 of the 20 older adults (Group III), a direct end-to-end anastomosis was performed. The anastomoses were sewn with everting continuous silk or Mersilene suture. The whole aortic wall was penetrated with these sutures. In seven Group III patients, resection of the coarctation (including an aortic aneurysm in one patient) and reconstruction of the aorta with a tubular Dacron graft were performed and the anastomoses were sewn with continuous silk or Mersilene sutures.
Barbiturate intoxication Disseminated sclerosis Cardiac failure Tight subvalvular aortic stenosis Cardiac failure Aortic valvular disease Aortic valvular disease Aortic valvular disease Aortic valvular disease Aortic valvular disease Colonic carcinoma Aortic valvular disease
30 25 15 14 16 41 43 40 58 49 56 60
8
No. of patients Systemic hypertension Rib notching Dyspnea Fatigue Palpitation Angina Headache Tired legs Vertigo Cold feet Other symptoms No symptoms
533
Functional class (NYHA)
Age at operation (yr)
Mean age at followup (yr)
No. of patients followed up
5-15 16-31 35-62
37 51 58
II
II
13 13
10 6
IIIIIIIIIIV 0 2 6
0 I
I
0 0 0
Legend: NYHA. New York Heart Association.
Early deaths Four patients died early while in the hospital (Tables I1A and III). One 8-year-old child died of bowel necrosis 4 weeks after the primary operation. Microscopy revealed panarteri tis (periarteritis nodosa) . One 25-year-old patient died 2 hours after the operation because of hemorrhage from the right second intercostal artery. The artery had been divided and the central ligature had cut through the thin wall. Another 25-year-old patient was examined 20 days after operation for a hemothorax on the left. Evacuation of blood and decortication of the upper and lower lobes were performed. However, the clot over the aortic wall was not removed. He died suddenly 4 weeks after the
The Journal of
5 34 Bergdahl. Bjork. Jonasson
Thoracic and Cardiovascular Surgery
Table V. Number of patients with hypertension or cardiac enlargement at follow-up Age at operation (yr)
No. ofpatients operated upon
No. of patients followed up
Mean follow-up period (surviving patients) (yr)
No. with hypertension (>160190)
No. with cardiac enlargement *
5-15 16-31 35-62
19 19 20
II
28 29 15
I (9%)t 6 (46%) 6 (46%)
4 3
13 13
o
*Nonnal values: men <500 ml/rrr' BSA; women <450 ml/m' BSA. tPercentage of patients who were followed up.
Table VI. Aortic valvular disease at follow-up Age at operation (yr)
No. of patients operated upon
Mean follow-up period (surviving patients) (yr)
5-15 16-31 35-62
19 19 20
28 29 15
Aortic valvular disease 7 (37%)* 11 (58%) 9(45%)
*Percentage of patients operated upon (this percentage refers to patients operated upon rather than those followed up. since some patients died of aortic valvular disease during follow-up.)
primary operation following a hemoptysis. Autopsy revealed a 2.5 em rupture of the suture line on the anterior wall of the aorta. The fourth patient, 46 years of age, died during reoperation for rupture of the suture line 7 weeks after the primary operation. The rupture was probably due to infection.
Late deaths Twelve patients died during a follow-up period of 12 to 32 years (mean 24 years) (Tables lIB and III). Three patients, 5, 6, and 38 years of age (at operation), died of unrelated disease. No information about their blood pressure could be found. One patient died of a tight subvalvular aortic stenosis 4 years postoperatively at the age of 14 years. Six patients with repair at ages 23 to 46 years died of aortic valvular disease. No information about their postoperative blood pressures could be obtained. Two patients died in cardiac decompensation at 15 and 16 years of age; no information about their postoperative blood pressures was available and autopsy was not performed.
Late results Functional classes. All 12 of the children who were followed up were in New York Heart Association (NYHA) Functional Class I (Table IV). Ten of 13 patients in the younger adult group (Group II) were in Functional Class I, two in Class II, and one in Class III.
All three patients (16 to 35 at repair) who were ultimately classified in NYHA Functional Class II or III had had aortic replacement at operation. The results were worse in the 13 older adult patients (Group III). Only six of them were in Functional Class I. Six of the remaining seven patients had aortic valvular disease. Blood pressure. Blood pressure measured at rest by a cuff on the right arm was higher than 160/90 mm Hg in only one of 11 children (9%) (Table V). Two patients operated upon in childhood did not consent to physical follow-up examination; both of them, however, reported that they were in good health and that the blood pressure had been normal at earlier postoperative examinations. Three patients operated upon at 35 to 62 years of age did not consent to a follow-up examination; they were also in good health but had not had any blood pressure determination in recent years. Forty-six percent of the patients in the other two groups had high blood pressure at follow-up (Table V). Aortic valvular disease. Aortic valvular disease (significant pressure gradient and/or valvular insufficiency) was diagnosed at follow-up or autopsy in 37% of the children, in 58% of the younger adults, and in 45% of the older adults (Table VI). The mean followup period, however, was significantly shorter in the last group of patients. Aortic valve replacement had been performed in four patients. One of these died because of thrombosis of a Bjork-Shiley tilting disc valve 14 months postoperatively. The remaining three patients were in good health with good results on exercise tolerance tests. Heart size. All the children followed up had a normal heart size (Table V). Seven of 26 Group II and III patients had enlarged hearts, six of whom had aortic valvular disease. Catheterization. The heart catheterizations were usually performed at rest and during exercise and included simultaneous pressure measurements proximal and distal to the anastomosis as well as in the left ventricle and ascending aorta. There was a significant (more than 20 mm Hg) pressure difference across the
Volume 85
Coarctation of aorta
Number 4 April,1983
5 35
Q,l/min 15
10
5
"02' ml STPD/min
O-t----r-----,.-----r----""'T"""
o
500
1000
1500
2000
Fig. 1. Cardiac output (Q, L1min) in relation to oxygen uptake (V0 2 ' ml STPD/min) at rest and during supine exercise in nine young (0) and 12 old (e ) patients operated upon for coarctation of the aorta. Thick line = regression of Q on V0 2 in healthy young persons; broken line = - 2 SD. anastomoses in only two of 29 patients at rest (Table VII). The pressure differences usually increased during exercise, and a significant peak systolic pressure difference was found in 11 of 20 patients. An elevated left ventricular end-diastolic pressure was found in 14 of 25 examined patients (Table VII). Oxygen consumption was measured by collecting expired air in Douglas bags and analyzing it for oxygen and carbon dioxide according to the method of Scholander. Gas volumes were measured with a spirometer. Cardiac output (Fick method) in relation to oxygen uptake was measured at the follow-up examination and was normal at rest in all nine investigated patients with operations performed in childhood (Fig. 1). This relation increased normally in six of the seven exercised patients. In contrast, five of the 12 investigated Group III patients had a low cardiac output, four of whom had aortic valvular disease (Fig. 1). Catheterization had been performed in eight of the 13 patients with hypertension at follow-up. In eight patients the catheterization had been performed at rest, and only one of the patients had a pressure difference of 20 mm Hg or more; the remaining seven patients had differences between 0 and 16 mm Hg. In five of the eight patients catheterization had been performed during excercise and in one patient a pressure difference of 12 mm Hg was recorded; the remaining four patients had values between 27 and 70 mm Hg.
Fig. 2. Aortogram of the first patient in the world operated upon for coarctation of the aorta. This study was made 32 years postoperatively and shows the anastomotic diameter to be about 70% that of the aortic arch. No gradient at rest was recorded. Discussion There was a high incidence of aortic valvular disease in these patients; the incidence in age group 16 to 31 years (Group II), for example, was 58%. The frequency of aortic valvular disease was higher in this study than previously reported. This probably is due to the very long follow-up period and to the fact that many patients in this study underwent cardiac catheterization at follow-up. In view of the high frequency of aortic valvular disease and hypertension, patients operated upon for aortic coarctation should be followed up carefully. Our study has also revealed the importance of the aortic valvular disease regarding the prognosis of patients who have had coarctation operations. Nine of the 12 late deaths were due to cardiac diseases and six of seven patients with cardiac enlargement at follow-up had aortic valvular disease. Only two of 20 catheterized patients had a peak systolic pressure difference at rest across the anastomosis of 20 mm Hg or more. The minimum pressure difference at rest necessary to produce hypertension in experimental animals has been shown to be 20 nun Hg.7
The Journal of
5 36 Bergdahl, Bjork, Jonasson
Thoracic and Cardiovascular Surgery
Table VII. Some catheterization data: three different age groups Age at operation (yr) 5-15 16-31 35-62
No. a/patients examined 8 9
12
Significant (>20 mm Hg) PSPD at rest 2
o
o
No. a/patients examined
Significant (>20 mm Hg) PSPD during work
No. a/patients examined
Elevated LVEDP >13 mm Hg
6 4 10
5 3 3
8 5 12
4 4 6
Legend: PSPD, Peak systolic pressure difference. LVEDP, Left ventricular end-diastolic pressure.
However, only one of the two patients with a significant stenosis at rest was hypertensive. It is difficult to evaluate the significance of a pressure difference during work. A pressure difference of 25 to 60 rnrn Hg during work was found in five of six patients operated upon in childhood, and four of these five patients had a normal blood pressure. We also found a pressure difference of 20 to 70 mm Hg during work over the anastomosis (or Dacron graft) in three of 10 exercised patients in Group III, and only two of these three patients were hypertensive. Our study has thus shown that other factors than a residual stenosis can be responsible for postoperative hypertension. We found no difference in the Group III in the results between patients operated upon with end-to-end anastomosis and with a tubular Dacron graft. The pressure difference over the operative site in five of seven patients with end-to-end anastomosis and in one of the five with ~ Dacron graft was small in all and judged to be of no clinical significance. This study has shown that resection of a coarctation with end-to-end anastomosis in childhood gives good long-term results as judged by the blood pressure; only 9% of the patients were hypertensive about 30 years after the operation. Other operative methods should be considered in patients in whom only a narrow anastomosis is possible. In these patients a tubular Dacron graft should be used instead of patch grafting because of a considerable risk of aneurysm formation. 8 In the two older age groups the incidence of high blood pressure was much higher-46%. Even considering the increased prevalence of hypertension with age in the general population, these results speak in favor of early operation. Like us, Maron, Humphries, Rowe, and Mellits? found an increased frequency (37%) of
hypertension in older patients (mean age at operation 22 years) followed up for an average of 18 years. We recommend operation in childhood in order to prevent persistent hypertension. As there are no technical advantages to be gained by waiting until 12 to 15 years of age, the operation can be performed before school age.
2
3
4
5
6
7
8
9
REFERENCES Patel R, Singh SP, Abrams L, Roberts KD: Coarctation of aorta with special reference to infants. Long-term results of operation in 126 cases. Br Heart 1 39: 1246- I253, 1977 Mustard WT, Rowe RD, Keith lD, Sirek A: Coarctation of the aorta with special reference to the first year of life. Ann Surg 141:429-436, 1955 Noordijk lA, Oey FrI, van den Boogaard HM: Late results of the operation for coarctation in the growing children. Arch Dis Child 40:192-196, 1965 Schuster SR, Gross RE: Surgery for coarctation of the aorta. A review of 500 cases. 1 THoRAc CARDIOVASC SURG 43:54-70, 1962 Keith lD, Rowe RD, Vlad P: Heart Disease in Infancy and Childhood, New York, 1967, Macmillan Publishing Co., Inc., p 226 Duffie ER, Wilson lL: Coarctation of the aorta, Heart Disease in Infancy, Childhood and Adolescence, Al Moss, FH Adams, editors, Baltimore, 1968, The Williams & Wilkins Company, p 395 Sykes Bl, Kreuzer W, Schenk WG lr: A new method for preparation of experimental coarctation of the aorta. Surg Gynecol Obstet 135:541 -544, 1972 Bergdahl L, Ljungqvist A: Long-term results after repair of coarctation of the aorta by patch grafting. 1 THoRAc CARDIOVASC SURG 80:177-181, 1980 Maron Bl, Humphries lON, Rowe RD, Mellits ED: Prognosis of surgically corrected coarctation of the aorta. A 20 year postoperative appraisal. Circulation 47: I 19-I26, 1973