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Ann Thorac Surg 2001;71:S336-S339
© 2001 The Society of Thoracic Surgeons


Autografts, allografts, and biological valves in children

Midterm results of the Ross procedure

Renzo Pessotto, MDa, Winfield J. Wells, MDa, Craig J. Baker, MDa, Carlos Luna, MDb, Vaughn A. Starnes, MDa

a Department of Cardiothoracic Surgery, Keck School of Medicine of the University of Southern California, Los Angeles, California, USA
b Division of Pediatric Cardiology, Keck School of Medicine, University of Southern California, Los Angeles, California, USA

Address reprint requests to Dr Wells, Division of Cardiothoracic Surgery, Keck School of Medicine, University of Southern California, Children’s Hospital Los Angeles, 4650 Sunset Blvd MS 66, Los Angeles CA 90027
e-mail: wwells{at}chla.usc.edu

Presented at the VIII International Symposium on Cardiac Bioprostheses, Cancun, Mexico, Nov 3–5, 2000.


    Abstract
 Top
 Abstract
 Introduction
 Material and methods
 Results
 Comment
 References
 
Background. The optimal hemodynamic performance and potential growth of the pulmonary autograft has led to expanded indications for the Ross procedure. We reviewed our institutional experience to assess midterm results with the Ross operation.

Methods. In a 7-year period (1992 to 1999), 111 patients with a median age of 15.7 years (range 2 days to 67 years), underwent the Ross procedure. Ninety-five patients had isolated aortic valve disease and 16 pediatric patients had a more complex left ventricular outflow tract obstruction.

Results. There were 3 early (2.7%) and 3 late deaths over a median follow-up of 3.6 years (range 6 months to 7.6 years). Actuarial survival at 5 years was 94% ± 2%. In pediatric patients, the pulmonary autograft annulus enlarged from 14.7 ± 6.2 mm to 22 ± 6.3 mm. This growth followed the expected increase in pulmonary valve diameter based on body surface area. Eight reoperations were necessary for autograft insufficiency at a median interval of 14 months (range 2 days to 31 months). Freedom from replacement of the pulmonary autograft was 91% ± 3% at 5 years. Three patients developed important obstruction of the pulmonary homograft requiring reoperation at a median of 29 months (range 9 to 31 months).

Conclusions. The Ross procedure can be performed with good midterm results. In pediatric patients, autograft growth has been appropriate. The potential for development of important autograft insufficiency suggests close follow-up through the intermediate and late term.


    Introduction
 Top
 Abstract
 Introduction
 Material and methods
 Results
 Comment
 References
 
Replacement of the aortic valve with the pulmonary autograft (Ross procedure) offers the advantage of excellent hemodynamic performance, freedom from anticoagulation, durability, and, for children, the potential for growth [1, 2]. For these reasons, the pulmonary autograft has become our valve of choice for children and young adults with isolated aortic valve disease or more complex left ventricular outflow tract lesions [3]. The purpose of this study was to review our experience with the Ross procedure.


    Material and methods
 Top
 Abstract
 Introduction
 Material and methods
 Results
 Comment
 References
 
Between July 1992 and July 1999, 111 patients underwent a Ross operation at the Children’s Hospital of Los Angeles or the University of Southern California University Hospital. There were 71 male patients (64%) and 40 female patients (36%) with a median age of 15.7 years (range 2 days to 67 years). Sixty-seven patients (60%) were younger than 18 years of age and 39 (35%) were younger than 10 years. Ninety-five patients had isolated aortic valve disease and 16 pediatric patients had more complex left ventricular outflow tract obstruction (LVOTO). The indications for the Ross procedure are shown in Table 1.


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Table 1. Indications for the Ross Procedure

 
Forty-nine patients (44%) had previously undergone a total of 63 interventions:
Balloon valvuloplasty 22
Surgical valvotomy/valvuloplasty 16
Subaortic stenosis resection 8
Interrupted aortic arch repair 5
Ventricular septal defect closure 5
Aortic valve replacement 3
Aortic coarctation repair 2
Arterial switch 2

The Ross operation was performed as an elective procedure in 104 patients (94%) and as an urgent/emergent procedure in 7 (6%). These included 4 adults with acute bacterial endocarditis and 3 neonates with critical aortic stenosis after failure of balloon valvuloplasty.

Surgical technique
Ninety-six patients (86%) had a root replacement, and in 15 patients (14%) the pulmonary autograft was inserted as a subcoronary implant [3].

Thirty-three additional procedures were performed in 32 patients at the time of the Ross procedure:
Konno procedure 14
Ascending aorta replacement 9
Mitral valve repair 5
Subaortic membrane resection 3
Ventricular septal defect closure 2

Follow-up
Transthoracic M-mode, two-dimensional, color-flow, and Doppler echocardiograms were obtained in all patients before hospital discharge and annually thereafter. The degrees of autograft and allograft regurgitation were quantitated as none/trivial, mild, moderate, and severe [4]. The peak velocity flow across both semilunar valves was also assessed.

To document growth of the pulmonary autograft in children, the early postoperative echocardiographic measurement of the diameter at the neoaortic annulus was compared with the subsequent annular measurements. The changes in the autograft annulus dimension were then compared with the normal aortic and pulmonary annulus growth as a function of somatic growth (body surface area [BSA]) in normal children (Z score) [5].

Statistical analysis
Data were expressed as mean ± standard deviation or as the median value and the range. Continuous variables were compared using a two-tailed, paired Student’s t test. Discrete variables were compared using Fisher’s exact test. Survival analysis and the actuarial estimate of freedom from reoperation were obtained using Kaplan–Meier methods.


    Results
 Top
 Abstract
 Introduction
 Material and methods
 Results
 Comment
 References
 
Hospital mortality
There were 3 early deaths for an operative mortality of 2.7%. A 65-year-old man with aortic insufficiency experienced severe bleeding from the inferior vena cava cannulation site several hours after operation. Despite control of the hemorrhage the patient had a cardiac arrest and could not be resuscitated. Two neonates with critical aortic stenosis underwent emergent Ross–Konno procedure after failed attempt at balloon valvuloplasty. In both cases the postoperative course was characterized by irreversible cardiogenic shock with multisystem organ failure.

Postrepair transesophageal echocardiography
The transesophageal echocardiogram performed in the operative room after the repair revealed no/trivial neoaortic insufficiency (AI) in 90 patients (81%), mild AI in 15 (14%), and moderate AI in 6 (5%). No patient had LVOTO. The pulmonary allograft used to reconstruct the right ventricular outflow tract was competent in 98 patients (88%) and showed mild regurgitation in 13 (12%). No patient had important flow acceleration across the pulmonary homograft.

Morbidity
Early autograft failure occurred in 1 patient (0.9%) who developed severe AI on the first postoperative day. At reoperation disruption of one autograft leaflet was found. The autograft was replaced with an aortic homograft.

Additional morbidity included reexploration for bleeding in 4 patients (3.6%), pericardial effusion requiring drainage in 3 patients (2.7%), postoperative atrial fibrillation requiring pharmacologic intervention in 2 patients (1.8%), and complete heart block requiring permanent pacemaker in 1 (0.9%).

Length of stay
The median hospital stay was 5 days (range 4 to 27 days).

Late mortality
At a median follow-up of 3.6 years (range 6 months to 7.6 years), 3 late deaths (2.8%) were recorded. All occurred in children with complex LVOTO who underwent a Ross–Konno. One infant died of cardiogenic shock after a mitral valve replacement for severe mitral regurgitation 6 months after the Ross procedure. Two other children died suddenly at home, 6 and 15 months postoperatively. The actuarial survival at 5 years was 94% ± 2%.

Echocardiography
The most recent follow-up echocardiogram revealed no/trivial AI in 73 patients (70%), mild AI in 22 (21%), moderate AI in 2 patients (2%), and severe AI in 7 (7%).

Pulmonary autograft failure
Seven patients with severe AI have undergone reoperation. Five adults underwent replacement of the pulmonary autograft 7, 9, 10, 19, and 20 months after the Ross procedure, respectively. In all cases a mechanical prosthesis was inserted.

Two children required reoperation at 3 and 31 months. An 8-year-old girl who underwent a Ross procedure for severe AI, had autograft regurgitation, which progressed from moderate to severe in 3 months. At reoperation, the dilated autograft annulus was reduced in size with restoration of normal function. The second patient was an 11-month-old infant who had undergone a Ross procedure for severe subaortic stenosis after neonatal repair of an interrupted aortic arch and ventricular septal defect. He developed progressive autograft regurgitation and required replacement with a mechanical valve. The actuarial freedom from reoperation for autograft insufficiency was 91% ± 3% at 5 years.

Pulmonary homograft status
Three patients (2.7%) required reoperation for significant obstruction of the pulmonary homograft. Two children required reoperation at 29 and 31 months postoperatively. One adult had the homograft replaced at 15 months for progressive stenosis.

Two additional patients in the pediatric group have developed a pulse Doppler gradient of 40 mm Hg or more across the pulmonary homograft and intervention is planned.

Pulmonary autograft growth
Echocardiographic measurements revealed autograft annulus enlargement from 14.7 ± 6.2 mm to 22 ± 6.3 mm, in the pediatric population (p < 0.001). When compared with the expected increase in annulus diameter based on somatic growth (BSA), this increase was compatible with growth of the normal pulmonary valve (Fig 1). The pulmonary valve is larger than the aortic valve and thus the observed autograft diameters were bigger than the expected aortic size based on BSA [5].



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Fig 1. Comparison of the autograft annulus diameter with the expected aortic and pulmonary diameter based on the body surface area. (NS = not significant.)

 

    Comment
 Top
 Abstract
 Introduction
 Material and methods
 Results
 Comment
 References
 
The Ross procedure is now considered a better alternative to mechanical, biological, and homograft valves in the treatment of aortic valve disease, in both children and young adults [1, 3]. As in other centers, we have extended its use to patients with more complex disease including active bacterial endocarditis, complex LVOTO, and patients with associated aneurysms of the ascending aorta [13, 68]. Despite the inclusion of high-risk patients, the procedure can be performed with a low overall mortality (2.7%) [8]. Two of our hospital deaths occurred in neonates with critical aortic stenosis who underwent an emergent Ross–Konno procedure after a failed balloon valvuloplasty. Both patients had a moderately hypoplastic left ventricle with endocardial fibroelastosis and continued to be in cardiogenic shock after the Ross procedure. This subgroup is exceptionally difficult to treat, with high-expected mortality and no valid alternative therapeutic options [7].

This study confirms that excellent midterm functional results can be achieved in most patients who are candidates for this procedure. In fact, more than 90% of our patients had no or mild autograft regurgitation at a median follow-up of 3.6 years.

David and colleagues [9] reported that the pulmonary autograft dilates in certain adults with bicuspid and other congenital aortic valve anomalies. In our series, late reoperation was required in 7 patients, 5 of whom had bicuspid aortic valve with severe aortic regurgitation. All developed severe autograft insufficiency after root replacement. At reoperation the AI was found to be due to annular dilation with failed coaptation of the valve leaflets.

The Ross procedure performed as a subcoronary implant seems to prevent dilation and failure of the pulmonary autograft [9]. However, this technique is more difficult because it requires perfect alignment of the commissures to prevent AI [10]. We have used this technique in 15 adults with no incidence of severe regurgitation. For this reason, we now favor a subcoronary implant in patients older than 14 years of age when growth of the pulmonary autograft is not necessary.

Traditionally, children with complex LVOTO constitute a difficult group with high incidence of residual and recurrent lesions [3, 7]. Our experience confirms that the Ross procedure combined with ventriculoplasty is an excellent therapeutic option for these children. This procedure offers lasting relief of the LVOTO with no patients requiring reoperation for recurrent lesions at midterm.

Potential growth of the pulmonary autograft has always been viewed as a theoretical advantage in children [13]. Elkins and coworkers [1] found that the intraaortic implants allow pulmonary autograft growth, whereas the root replacements are associated with dilation [11]. More recently, Puntel and colleagues [12] found that the autograft annulus size increases more than expected after a Ross procedure with root replacement. We compared the early postoperative autograft annulus diameters with those at the most recent follow-up echocardiogram. The obtained measurement was compared with the expected value based on patient’s somatic growth (BSA) and the result was expressed as the Z score. Because the pulmonary valve is larger than the aortic valve, the obtained diameters were normalized for both the aortic and the pulmonary annulus. The mean postoperative Z value was +1.3 ± 2 and the late postoperative mean was +2.9 ± 2.6 when compared with the normal aortic annulus (p = 0.0002). However, when the obtained data were compared with the normal pulmonary annulus diameters, the early postoperative mean Z score was +0.4 ± 0.9 and the late postoperative mean Z score was +0.7 ± 1.2 (p = NS) (Fig 1). These data suggest that the pulmonary autograft continues to grow as a normal pulmonary root even when placed in the aortic position.

In conclusion, the Ross procedure has shown good midterm results. In children, growth of the pulmonary autograft has been appropriate to the increase in BSA and paralleled the growth of the normal pulmonary valve. Although pulmonary autograft combined with ventriculoplasty is the best alternative for infants with complex LVOTO, the procedure carries substantial early and late mortality. The potential for development of important autograft insufficiency suggests close follow-up through the intermediate and late term.


    References
 Top
 Abstract
 Introduction
 Material and methods
 Results
 Comment
 References
 

  1. Elkins R.C., Knott-Craig C.J., Ward K.E., McCue C., Lane M.M. Pulmonary autograft in children: realized growth potential. Ann Thorac Surg 1994;57:1387-1394.[Abstract]
  2. Kouchoukos N.T., Davila-Roman V.G., Spray T.L., Murphy S.F., Perrillo J.B. Replacement of the aortic root with a pulmonary autograft in children and young adults with aortic valve disease. N Engl J Med 1994;330:1-6.[Abstract/Free Full Text]
  3. Starnes V.A., Luciani G.B., Wells J.W., Allen R.B., Lewis A.B. Aortic root replacement with the pulmonary autograft in children with complex left heart obstruction. Ann Thorac Surg 1996;62:442-449.[Abstract/Free Full Text]
  4. Roman M.J., Devereux R.B., Kramer-Fox R., O’Loughlin J., Spitzer M., Robins J. Two-dimensional echocardiographic aortic root dimensions in normal children and adults. Am J Cardiol 1989;64:507-512.[Medline]
  5. Capps S.B., Elkins R.C., Fronk D.M. Body surface area as a predictor of aortic and pulmonary valve diameter. J Thorac Cardiovasc Surg 2000;119:975-982.[Abstract/Free Full Text]
  6. Reddy V.M., McElhinney D.B., Phoon C.K., Brook M.M., Hanley F.L. Geometric mismatch of pulmonary and aortic annuli in children undergoing the Ross procedure: implications for surgical management and autograft valve function. J Thorac Cardiovasc Surg 1998;115:1255-1263.[Abstract/Free Full Text]
  7. Reddy V.M., Rajasinghe H.A., Teitel D.F., Haas G.S., Hanley F.L. Aortoventriculoplasty with the pulmonary autograft: the "Ross–Konno" procedure. J Thorac Cardiovasc Surg 1996;111:158-167.[Abstract/Free Full Text]
  8. Marino B.S., Wernovsky G., Rychik J., Bockoven J.R., Godinez R.I., Spray T.L. Early results of the Ross procedure in simple and complex left heart disease. Circulation 1999;100(Suppl):II162-II166.
  9. David T.E., Omran A., Ivanov J., et al. Dilation of the pulmonary autograft after the Ross procedure. J Thorac Cardiovasc Surg 2000;119:210-220.[Abstract/Free Full Text]
  10. Elkins R.C., Lane M.M., McCue C. Pulmonary autograft reoperation: incidence and management. Ann Thorac Surg 1996;62:450-455.[Abstract/Free Full Text]
  11. Solymar L., Sudow G., Holmgren D. Increase in size of the pulmonary autograft after the Ross operation in children: growth or dilation?. J Thorac Cardiovasc Surg 2000;119:4-9.[Abstract/Free Full Text]
  12. Puntel R.A., Webber S.A., Ettedgui J.A., Tacy T.A. Rapid enlargement of neoaortic root after the Ross procedure in children. Am J Cardiol 1999;84:747-749.[Medline]



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