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Original Article

Replacement of the Aortic Root with a Pulmonary Autograft in Children and Young Adults with Aortic-Valve Disease

Nicholas T. Kouchoukos, Victor G. Davila-Roman, Thomas L. Spray, Suzan F. Murphy, and Johanna B. Perrillo

N Engl J Med 1994; 330:1-6January 6, 1994

Abstract

Background

The optimal substitute for severely diseased aortic valves in children and young adults is unknown. The use of a mechanical prosthesis requires permanent treatment of the patient with anticoagulants and is associated with thromboembolic and hemorrhagic complications. Aortic-valve allografts and porcine bioprostheses, which do not necessitate anticoagulant therapy, may deteriorate and have limited durability.

Methods

We therefore evaluated the use of the autologous pulmonary valve (i.e., the patient's own pulmonary valve) and the adjacent pulmonary artery as a replacement for the aortic valve and aortic sinuses in 33 patients. Five of the patients were from 8 to 16 years of age, and 28 were from 20 to 47 years of age. The pulmonary valve and the main pulmonary artery were used to replace the diseased aortic valve and the adjacent aorta. The coronary arteries were detached from the aorta and implanted into the pulmonary artery. The pulmonary valve and artery were replaced with a cryopreserved pulmonary allograft.

Results

There were no deaths during follow-up of up to 48 months (mean, 21 months). There were no episodes of infective endocarditis, and no reoperations on the aortic root were necessary. Also, there was no evidence on echocardiography of progressive dilatation of the autografts. With color-flow Doppler imaging, 22 patients were found to have only trivial regurgitation or none, 9 patients to have mild regurgitation, and no patients to have moderate or severe regurgitation across the autograft at the most recent follow-up visit. The mean peak velocity of flow across the autograft was 1.3 m per second (upper limit of normal, 1.8), indicating the absence of stenosis. One patient required reoperation for stenosis of the pulmonary allograft.

Conclusions

Although the pulmonary-autograft procedure is more complex than simple aortic-valve replacement, it has been safely applied in selected patients, including young adults. Intermediate follow-up indicates satisfactory function of the autografts, with no dilatation or progressive valvular regurgitation. Pulmonary-root autografts may thus be the best available substitute for diseased aortic valves in children and young adults.

Media in This Article

Figure 1Schematic Representation of the Operative Procedure.
Figure 2Serial Echocardiographic Assessments of the Severity of Regurgitation in the Pulmonary Autograft in 31 Patients.
Article

The optimal substitute for a diseased aortic valve in children and young adults has not been identified. Although several mechanical valves have satisfactory hemodynamic characteristics, all necessitate lifelong treatment of the patient with anticoagulants. Thromboembolism, hemorrhage, and prosthetic-valve endocarditis remain important complications1,2. Porcine bioprostheses, which do not necessitate long-term anticoagulation, deteriorate rapidly in young patients and have limited durability3. Aortic-valve allografts have excellent hydraulic function, necessitate no anticoagulant therapy, and are associated with a low incidence of thromboembolic complications. However, they also have limited durability in younger patients4-6. Because aortic-valve allografts are also used for other conditions (such as endocarditis, aneurysms that involve the aortic root, and reconstruction of the right ventricular outflow tract), they are not readily available.

In 1967, Ross7 described the use of the autologous pulmonary valve to replace the aortic valve. The valve was implanted within the aortic root, and the pulmonary valve was replaced with either an aortic or a pulmonary allograft. The viability of pulmonary-valve autografts in the aortic position has been documented, and they are more resistant to deterioration than aortic allografts6,8. The availability of commercially prepared, cryopreserved aortic-valve and pulmonary-valve allografts in recent years and the development of safe techniques for the replacement of the aortic root have broadened the indications for the replacement of the aortic root with either aortic allografts or pulmonary autografts to include patients with isolated aortic-valve disease8-11.

Replacement of the aortic root with a pulmonary-root autograft in patients with aortic-valve disease is an alternative to replacement of the aortic valve with a mechanical or bioprosthetic valve or an allograft. This approach should result in optimal hemodynamic function, since the pulmonary valve is approximately the same size as the aortic valve and is implanted with the adjacent sinuses of Valsalva. Also, the use of a pulmonary autograft should, in principle, eliminate thromboembolic complications without the need for anticoagulant therapy. In addition, these viable valves have the potential for growth,6,12 a particular advantage in children and young adults with aortic-valve disease. In this report we describe our experience in replacing the aortic root with a pulmonary autograft in 33 patients with aortic-valve disease and the results of serial echocardiographic studies assessing the function of these autografts and of the pulmonary allografts that were implanted in the right ventricular outflow tract.

Methods

Between June 1989 and May 1993, 33 patients with aortic-valve disease underwent replacement of the aortic root with a pulmonary autograft and replacement of the pulmonary root with a pulmonary allograft. The patients ranged in age from 8 to 47 years (mean, 31.4); 5 patients were from 8 to 16 years of age, and 28 were from 20 to 47 years of age. Twenty-two of the 33 were male. The indications for aortic-valve replacement are shown in Table 1Table 1Indications for Aortic-Root Replacement.. A bicuspid aortic valve, present in 23 patients, was the most common indication for operation. The majority of these valves were heavily calcified; two of these patients also had aneurysms of the ascending aorta, which were replaced with Dacron grafts. Four patients had aortic regurgitation resulting from rheumatic heart disease, three had infective endocarditis, and three had failure of a previously inserted porcine bioprosthesis or mechanical valve. One of the 33 patients had previously undergone balloon valvuloplasty, and 8 had undergone one or more previous operations on the aortic valve. The patients were for the most part healthy and generally had good left ventricular function. The mean preoperative New York Heart Association functional class was 2.2 (on a 4-point scale, with 1 indicating no symptoms and 4 indicating symptoms at rest).

Preoperative Evaluation

All the patients underwent preoperative left-heart catheterization and coronary arteriography to assess the severity of the aortic-valve disease and left ventricular dysfunction, to determine the size and location of the coronary arteries, and to determine the size and number of proximal septal perforating branches of the anterior descending coronary artery. These branches must be protected during the removal of the pulmonary valve and the adjacent right ventricular myocardium in order to prevent infarction of the ventricular septum13. Any large conal branches of the right coronary artery, which may connect with the anterior descending system and which may be injured during the removal of the pulmonary root, were also identified.

Operative Procedure

Intraoperative transesophageal two-dimensional and color-flow Doppler echocardiography was performed in most of the patients before cardiopulmonary bypass began in order to determine the diameter of the annulus and the sinuses of the pulmonary root and to assess the competence of the pulmonary valve. The size of the aortic annulus and the aortic sinuses was also measured. Mismatches in size of more than 2 to 3 mm between the aortic and pulmonary roots or more than trivial regurgitation in the pulmonary valve were not found in any of the patients. Patients with dilatation of the aortic root were not considered candidates for this procedure.

The technique for excision of the pulmonary root and its implantation into the aortic root is shown in Figure 1Figure 1Schematic Representation of the Operative Procedure. 14. The procedure was performed during a period of cardiopulmonary bypass that averaged 199 minutes (range, 105 to 286). During the period of myocardial ischemia, which averaged 134 minutes (range, 70 to 200), the myocardium was protected by the infusion of cold blood (4 °C) or crystalloid cardioplegic solution directly into the coronary ostia or in a retrograde fashion into the coronary sinus. External cooling of the heart with iced crystalloid solution or with a cooling jacket was also used. After the discontinuation of cardiopulmonary bypass, the competence of the pulmonary-autograft valve and of the pulmonary-allograft valve in the right ventricular outflow tract was assessed by transesophageal echocardiography.

Postoperative Evaluation and Management

Transthoracic M-mode, two-dimensional, color-flow, and Doppler echocardiograms were obtained before discharge from the hospital and every 6 to 12 months thereafter. The presence of regurgitation in the autograft was determined by continuous-wave, pulsed-wave, and color-flow Doppler methods. The severity of regurgitation was measured with the method of Perry et al.,15 in which the ratio of the width of the jet of regurgitation to the diameter of the left ventricular outflow tract at the level of the valve annulus is determined. This measurement has been shown to correlate well with the severity of aortic regurgitation as indicated by angiography15. A ratio of less than 0.2 was considered to indicate trivial regurgitation; a ratio of 0.2 to 0.39, mild regurgitation; 0.4 to 0.6, moderate regurgitation; and more than 0.6, severe regurgitation. A numerical grade was assigned to each of these ranges (Table 2Table 2Echocardiographic Assessment of Regurgitation in the Autograft.). Using the method of Roman et al.,16 we measured the three dimensions of the pulmonary-root autograft at end-diastole: the diameter at the annulus, the maximal diameter of the sinuses of Valsalva, and the diameter at the supravalvular ridge. We also measured the peak velocity of the flow across the pulmonary-autograft and pulmonary-allograft valves in order to detect evidence of any obstruction of blood flow.

The patients were evaluated at 6-to-12-month intervals after surgery for symptoms and signs of valve regurgitation and for other cardiac abnormalities. No patient was lost to follow-up. No anticoagulant or antiplatelet drugs were administered to any of the patients.

Statistical Analysis

The dimensions of the valve autograft were expressed as means ±SD. Repeated-measures analysis of variance was used to compare these measurements and the numerical values for the severity of regurgitation in the autograft at each echocardiographic study for each patient. A two-tailed P value of <0.05 was considered to indicate statistical significance.

Results

Mortality and Morbidity

There were no deaths in the hospital. One patient required early reoperation for bleeding. Twenty-two patients (67 percent) did not require transfusion of homologous blood products during the perioperative period. Fifteen of these patients had predeposited one or two units of autologous blood, which were transfused during or after the operation. In the 11 patients who required homologous blood, a mean of 2.7 units of red cells was transfused. There was no electrocardiographic or echocardiographic evidence of perioperative myocardial infarction in any patient. Seven patients had a pattern indicative of right bundle-branch block after the operation. In one patient, an eight-year-old child who had previously undergone balloon angioplasty of the aortic valve, complete heart block developed and implantation of a permanent pacemaker was required. One patient had partial homonymous hemianopia. This was probably the result of intraoperative embolization of a fragment from the severely diseased aortic valve. The median postoperative stay in the hospital was 7 days (range, 4 to 32).

There have been no deaths during the follow-up period, which at this writing extends to 48 months (mean duration of follow-up, 21 months), nor have there been any episodes of thromboembolism or infective endocarditis. No reoperations have been required on the valve autografts. One 15-year-old patient required reoperation for circumferential stenosis of the pulmonary allograft in the right ventricular outflow tract above the level of the pulmonary valve 16 months after the initial operation. The obstruction was successfully relieved with a pericardial patch. An aortic-root angiogram obtained immediately before the reoperation showed a normal-sized aortic root and no regurgitation in the autograft. The mean New York Heart Association class at the most recent follow-up was 1.0 for the 33 patients.

Echocardiographic Studies

Pulmonary-Autograft Function

The numerical grades for the severity of aortic-autograft regurgitation in 31 patients, as determined by echocardiography, are shown in Figure 2Figure 2Serial Echocardiographic Assessments of the Severity of Regurgitation in the Pulmonary Autograft in 31 Patients.. At the initial postoperative study, performed in the first month after the operation, 29 patients had trivial regurgitation or none, and 4 patients had mild regurgitation. At the most recent follow-up study, 22 patients had only trivial or no regurgitation, 9 patients had mild regurgitation, and no patients had moderate or severe regurgitation across the autograft. Of the 16 patients who were examined 19 to 24 months postoperatively, 11 had trivial regurgitation or none, and 5 had mild regurgitation. For this subgroup, the mean regurgitation score was 0.34 ±0.30 at the initial postoperative examination and 0.52 ±0.44 at the later study (P = 0.3). For the group as a whole, there was no appreciable increase in the severity of regurgitation with time.

Continuous-wave Doppler echocardiography across the pulmonary autograft at the initial postoperative study showed mildly increased flow velocity (from 2.0 to 2.2 m per second) in six patients (18 percent of the group). The upper limit of normal is 1.8 m per second. At the most recent examination, all values for velocity were within the normal range (mean, 1.3 m per second; range, 1.0 to 1.8). The early postoperative increase in velocity probably reflected a hyperdynamic state rather than valvular obstruction.

Dimensions of the Autograft

The mean values for the three diameters of the autograft (annulus, sinuses of Valsalva, and supravalvular ridge) soon after the operation and at the most recent follow-up visit (the interval between the two measurements was longer than three months for 31 patients) were as follows: 2.2 ±0.4 as compared with 2.2 ±0.3 cm at the annulus, 3.1 ±0.6 as compared with 3.3 ±0.5 cm at the sinuses of Valsalva, and 2.5 ±0.4 as compared with 2.6 ±0.4 cm at the supravalvular ridge. None of the differences were statistically significant. For the 16 patients who were examined early after surgery and 19 to 24 months thereafter, the mean diameters at the annulus were 2.3 ±0.3 and 2.3 ±0.3 cm, those at the sinuses of Valsalva were 3.3 ±0.5 and 3.4 ±0.6 cm, and those at the supravalvular ridge were 2.6 ±0.4 and 2.6 ±0.4 cm. These differences were also not statistically significant.

Pulmonary-Allograft Function

At the initial postoperative study, there was no regurgitation in the pulmonary-allograft valve in the right ventricular outflow tract in 18 patients and trivial or mild regurgitation in 13 patients. At the time of the most recent study, mild regurgitation had developed in three patients who initially had no pulmonary regurgitation, and three patients who initially had trivial regurgitation or none had no regurgitation.

At the initial study, continuous-wave Doppler echocardiography across the pulmonary allograft showed a mild overall elevation in systolic velocity (mean, 1.1 ±0.4 m per second; range, 0.6 to 2.0). The upper limit of normal for the native pulmonary valve is 0.8 m per second. These velocities returned to normal in later studies in most of the patients, suggesting that the early elevations were due to postoperative hemodynamic changes. During the follow-up period, however, seven patients were found to have moderate pressure gradients (mean, 26 ±14 mm Hg; range, 12 to 45), which were measured at a point distal to the pulmonary-allograft valve, primarily in the vicinity of the anastomosis between the arterial portion of the allograft and the native pulmonary artery. The gradients were confirmed by right-heart catheterization in two patients. In one of the patients, who had a measured gradient of 65 mm Hg at cardiac catheterization, the obstruction was surgically corrected.

Discussion

Although replacement of the aortic root with a pulmonary autograft in patients with isolated aortic-valve disease is a longer and more complex procedure than simple aortic-valve replacement, we and others12,17 have documented the safety of the procedure. In our series, there were no deaths in the hospital and no serious postoperative complications that could be attributed to the operative technique. With the methods of intraoperative myocardial protection that we employed, which included cold cardioplegia and surface cooling of the myocardium, intraoperative and postoperative myocardial dysfunction was minimal despite a duration of myocardial ischemia longer than that required for simple aortic-valve replacement. Only one third of the patients required transfusion of homologous blood products. Although there is the potential for injury to the septal branches of the left anterior descending coronary artery during the removal of the pulmonary root, no evidence of septal myocardial infarction was detected in any of the patients.

The advantages of using the pulmonary root as a substitute for the aortic root in patients with aortic-valve disease include the use of autologous tissue with documented long-term viability,8 the optimal or near-optimal alignment and function of the valve leaflets, since the sinuses of Valsalva are also transplanted, and the absence of substantial transvalvular pressure gradients, thromboembolism, and the need for anticoagulant therapy12. There is also evidence of growth of the autograft, which makes it an attractive option for aortic-valve replacement in infants and children6,12.

Aortic allografts that are implanted in the aortic root have hemodynamic characteristics that are similar to those of pulmonary autografts; they are also associated with a low incidence of thromboembolism and endocarditis, and they do not necessitate the use of anticoagulants or antiplatelet drugs. However, valve degeneration and reoperation because of mechanical failure occur more often in younger than in older patients4-6. In the experience of Gerosa et al.,9 aortic or pulmonary allografts that are implanted in the right ventricular outflow tract to replace the excised pulmonary root have a cumulative rate of freedom from valve failure or reoperation that exceeds 80 percent at 16 years. This freedom from valve failure or reoperation is superior to that for aortic allografts in the left ventricular outflow tract. Mild or moderate allograft stenosis or incompetence is likely to be tolerated better in the low-pressure right ventricular outflow tract than in the left ventricular outflow tract. Thus, the need for a second operation to remove a malfunctioning or degenerated allograft should be substantially reduced or possibly eliminated if the allograft is placed in the right ventricular rather than the left ventricular outflow tract.

The chief uncertainty regarding the use of pulmonary-root autografts in the aortic position is the potential for dilatation of the wall of the pulmonary artery and the development of progressive valvular regurgitation as a result of continued exposure to systemic pressure. Experimental studies by Gorczynski et al.18 have demonstrated that the leaflets of the pulmonary valve have a tensile strength that equals or exceeds that of aortic-valve leaflets. In an in vitro model of the circulation, Weerasena et al.19 observed a smaller drop in pressure across the pulmonic valve at systemic pressures than at pressures and flows that occur in the pulmonary artery. Furthermore, an increase in pressure had a limited effect on regurgitation in this model, and at the highest pressures most pulmonary valves closed completely, producing a competent valve19. Inward movement of the elastic arterial wall during diastole absorbs energy and reduces dynamic loading during closure19. These conditions may not exist when only the pulmonary valve or an aortic-allograft valve is implanted inside the aortic root, without the sinuses of Valsalva. Furthermore, the aortic wall to which a free pulmonary-valve autograft or aortic-valve allograft is attached may be noncompliant and may prevent the optimal functioning of the valve leaflets. There may also be misalignment of the valve commissures and cusps with a free graft, which will place greater stress on the valve components. These findings argue for the use of the entire pulmonary root rather than the pulmonary valve as a substitute for a diseased aortic valve.

Serial echocardiographic measurements of the three dimensions of the pulmonary-root autografts in 31 patients demonstrated no increase in the size of the autograft for up to 48 months after valve replacement (mean follow-up, 21 months). To our knowledge, this is the largest series of patients with pulmonary-root autografts in whom sequential measurements of these dimensions and semiquantitative estimates of the degree of aortic regurgitation have been performed.

Sievers et al.17 observed no significant increase in the diameter of seven pulmonary-root autografts at the commissural level in adult patients a mean of 12.5 ±7 months after surgery. Elkins et al.12 made sequential measurements of autografts in 13 of their 22 patients, all of whom were children. The mean duration of follow-up for their 21 surviving patients was approximately 21 months. Three patients were followed for more than 48 months, the longest follow-up being 58 months. Since growth occurred in many of these patients, it was not possible to differentiate clearly the growth of the pulmonary-root autograft from dilatation. The absence of progressive dilatation of the autograft in our patients was associated with an absence of progression of aortic regurgitation. Similar findings were reported by Sievers et al.17. Longer follow-up will be necessary to determine whether late changes will occur in the pulmonary arterial wall or in the pulmonary leaflets and whether such changes result in dilatation of the root and valvular regurgitation in the autograft.

Although replacement of the aortic root with a pulmonary root is a more complex operation than aortic-valve replacement in patients with aortic-valve disease, it has been used safely in a selected group of children and young adults with no early mortality or graft-related morbidity. Follow-up extending for as long as four years indicated excellent function of the autografts, with no evidence of dilatation or progressive valvular regurgitation. There have been no infectious or thromboembolic complications and no late deaths. If longer follow-up shows no evidence of the progression of regurgitation in the implanted valve, a pulmonary autograft may be the optimal substitute for a diseased aortic valve in children and young adults.

We are indebted to Drs. Donald Ross, Ronald Elkins, and John W. Kirklin for their advice and encouragement; to Dr. Thomas Wareing, who operated on one of the patients; and to Pamela Pigg and Nikki Gratigny for assistance in the preparation of the manuscript.

Source Information

From the Divisions of Cardiothoracic Surgery (N.T.K., T.L.S., S.F.M., J.B.P.) and Cardiology (V.G.D.-R.), Washington University School of Medicine, St. Louis.

Address reprint requests to Dr. Kouchoukos at the Department of Surgery, Jewish Hospital at Washington University Medical Center, 216 S. Kingshighway Blvd., St. Louis, MO 63110.

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    C. Schmidtke, G. Dahmen, H.-H. Sievers. (2006) Geschlechtsspezifische Aspekte nach pulmonalem Autograft (Ross-Operation). Zeitschrift für Herz-,Thorax- und Gefäßchirurgie 20:6, 229-235
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