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Eur J Cardiothorac Surg 2004;25:742-747
© 2004 Elsevier Science NL

Ross and Ross–Konno procedure in children and adolescents: mid-term results

^a Department of Pediatric Cardiac Surgery, University Hospital Hamburg-Eppendorf, Martinistrase 52, 20246 Hamburg, Germany
^b Pediatric Cardiology, University Hospital Hamburg-Eppendorf, Martinistrase 52, 20246 Hamburg, Germany
^c Cardiac Surgery, University Hospital Hamburg-Eppendorf, Martinistrase 52, 20246 Hamburg, Germany
^d Department of Cardiac Surgery, Children's University Hospital, Bratislava, Slovakia
^e Department of Cardiac Surgery, Children's Hospital, Denver, CO, USA

Received 7 October 2003; received in revised form 9 January 2004; accepted 12 January 2004.

^* Corresponding author. Tel.: 49-40-428-03-2835; fax: 49-40-428-03-8275
e-mail: v.hraska{at}uke.uni-hamburg.de

	Abstract

Top Abstract 1. Introduction 2. Materials and methods 3. Results 4. Discussion 5. Conclusions Appendix A. Conference... References

 
Objectives: The aim of the study was to analyze mid-term results of aortic root replacement with pulmonary autograft in children and adolescents in two centers. Methods: From December 1997 through August 2003, a total of 66 patients underwent the Ross procedure in two centers. Indication for Ross procedure was predominantly aortic stenosis in 24 patients and predominantly aortic regurgitation (AR) in 22 patients. Twenty patients with severe left ventricular outflow tract obstruction (LVOTO) underwent Ross–Konno procedure. No patient had a geometric mismatch of more than 5 mm in favor of the aortic annulus. Results: There was no early death. One patient died 3 months after surgery due to bacterial endocarditis. Survival on median follow-up period of 2.4 years was 98.5%. Neo-aortic regurgitation was none in 29 (44%) patients, trivial in 35 (53%) patients and mild in 2 (3%) patients. One patient (1.5%) needed aortic valve replacement because of autograft failure. Actuarial freedom from more than trivial neo-aortic regurgitation, or aortic valve replacement was 95% at 5 years follow-up. There was no patient either with recurrent LVOTO or significant aortic root dilatation. Freedom from redo was 93% at 5 years of follow-up. There had been a significant reduction (P=0.001) and normalization in the left ventricle diastolic diameter index and left ventricle mass index, respectively, within 3–12 months after operation. Sixty-three percent of all operated patients are without medication; no one is on anticoagulation therapy. Conclusions: Our 7 years experience with the Ross and Ross–Konno operation has shown excellent mid-term results, with mortality rate approaching zero in both simple and complex left heart lesions, even in the neonates and infants. It is a procedure of choice in children with severe anomaly of the aortic valve and/or left ventricular outflow tract obstruction. The main concern is dilatation of the neo-aortic root leading to progression of AR, especially in the settings of geometric mismatch of aortic and pulmonary roots and bicuspid, regurgitant aortic valve. The risk of autograft failure in these specific subsets of patients remains to be determined.

Key Words: Congenital heart disease • Aortic valve disease in children • Left ventricular outflow tract obstruction • Ross–Konno procedure

	1. Introduction

Top Abstract 1. Introduction 2. Materials and methods 3. Results 4. Discussion 5. Conclusions Appendix A. Conference... References

 
The decision-making process of operative care in children and young adults with aortic valve disease is still the subject of controversy. Growth, active life style with appropriate activity level and difficulty in medical compliance represent the specific set of requirements posed by this subset of patients. The Ross and Ross–Konno procedure have changed the approach to children with congenital aortic valve disease allowing replacement of stenotic or regurgitant aortic valve even very early in life [1–3]. The pulmonary autograft has proved to be durable and grow in proportion to somatic growth [4,5].

The main concern is dilatation of the neo-aortic root leading to progression of aortic regurgitation (AR), especially in the settings of geometric mismatch of aortic and pulmonary roots and bicuspid aortic valve [6–9].

The aim of the study was to analyze mid-term results of aortic root replacement with pulmonary autograft in children and adolescents in two centers.

	2. Materials and methods

Top Abstract 1. Introduction 2. Materials and methods 3. Results 4. Discussion 5. Conclusions Appendix A. Conference... References

 
2.1. Patient population
From December 1997 through August 2003, a total of 66 patients underwent a Ross or Ross–Konno procedure in two centers: at the Children's Hospital, Bratislava, Slovakia and at the University Hospital Eppendorf-Hamburg, Germany. The preoperative hemodynamic indication for Ross procedure was predominantly aortic stenosis (AS) in 24 patients, predominantly AR in 22 patients. Twenty patients with severe left ventricular outflow tract obstruction (LVOTO) (19 pts) and/or interruption of aortic arch (IAA) (1 pt) underwent Ross–Konno procedure. Patients with AR were indicated for surgery before a significant mismatch between pulmonary and aortic annulus had developed. No one patient had a geometric mismatch more than 5 mm in favor of the aortic annulus.

Median age at operation was 12.9 years (from 8 days to 23 years) (Fig. 1) . Thirty-seven (56%) patients had previous procedures. Twenty-eight patients underwent balloon valvuloplasty, 10 patients had surgical valvulotomy and/or valvuloplasty, 4 patients needed resection of subaortic area and 3 patients had other miscellaneous procedures. Concomitant procedures were performed in 16 patients (24%). There were 10 plications of the aortic annulus, 8 reduction aortoplasties of the ascending aorta, 4 resections of endomyocardial fibroelastosis, 1 ventricular septal defect closure, 1 reconstruction of the aortic arch and 1 resection of a right ventricular outflow obstruction.

View larger version (14K): [in this window] [in a new window]   Fig. 1. Age distribution of children undergoing the Ross and Ross-Konno procedure. (m, month; y, year).

2.3. Functional outcome and follow-up
The postoperative result was assessed in all patients at discharge. Regular clinical check-up was performed at 3–6 month intervals during the first postoperative year and at 12-month intervals thereafter. All patients underwent two-dimensional echocardiography (ECHO) and Doppler assessment of the intracardiac repair. M-mode measurement of left ventricle diastolic diameter was performed at the level of chordae of mitral valve in the long axis parasternal view and was indexed to body surface area. Follow-up data was complete in all patients.

2.4. Statistical analysis
Data were analyzed using a statistical program (JMP Statistical Analysis, Cary, NC). Descriptive statistics are expressed as median and range. Continuous variables were analyzed with Student's t-test. The Kaplan–Meier method was used to determine event-free survival curves. Subgroups were compared with the use of the log-rank test.

	3. Results

Top Abstract 1. Introduction 2. Materials and methods 3. Results 4. Discussion 5. Conclusions Appendix A. Conference... References

 
There was no early death. The median length of stay in hospital was 7 days (from 5 to 52). Early postoperative complications included re-exploration for bleeding and tamponade in one (1.5%) patient, diaphragm paresis required plication in one (1.5%) patient. There was one patient with temporary complete heart block, no patient required a pacemaker. One patient had junctional ectopic tachycardia immediately postoperatively. One patient needed ECMO support because of intractable ventricular tachycardia on the third postoperative day. He was successfully weaned from ECMO on eighth postoperative day. After discharge, four patients were readmitted for pericardial effusion.

The median follow-up for the entire group was 2.4 years (range 2 months–5.6 years). One patient died 3 months after surgery due to bacterial endocarditis. Considering both early and late events the probability of survival for the entire group from the time of surgery was 98.5% (70% confidence limits [97;100]) after 4 years and remained unchanged during the rest of follow-up. There was no difference in survival between patients with Ross and Ross–Konno procedure.

Neo-aortic regurgitation at follow-up was none in 29 (44%) patients, trivial in 34 (52%) patients and mild in 2 (3%) patients. Actuarial freedom from more than trivial neo-aortic regurgitation, or aortic valve replacement was 98% [96.5;99.5] at 1 year and 95% [92;98] at 2–5 years of follow-up. There was no patient with either recurrent LVOTO or with significant aortic root dilatation.

Freedom from redo was 95% [92;98] at 1 year and 93% [89;97] at 2–5 years of follow-up. One patient underwent aortic valve replacement with a mechanical valve 2.3 years after Ross procedure because of autograft failure. In this patient a morphologically abnormal pulmonary valve had been used for the Ross procedure. The separation of the commissural attachments of the cusps of the pulmonary valve was noted during the operation. Patient had mild to moderate neo-aortic regurgitation immediately after the operation with gradual progression to severe regurgitation with dilatation of the LV. The pulmonary homograft was replaced in one patient, one patient required a redo because of septal patch aneurysm after a Ross–Konno operation and one had a LIMA graft because of stenosis of LAD. Three patients (4.5%) developed pulmonary homograft stenosis with a peak gradient >40 mmHg and underwent balloon dilatation with success. Overall freedom from homograft re-intervention (redo or balloon dilatation) was 91% [85;97] at 4 years of follow-up.

Event-free interval including redos and re-interventions was 95% [90;100] at 1 year, 90% [81;99] at 3 years and 85% [78;82] at 5 years of follow-up (Fig. 2).

View larger version (10K): [in this window] [in a new window]   Fig. 2. Event-free survival, in years, for whole group (n=66). Numbers of patients at risk are in parentheses.

View larger version (14K): [in this window] [in a new window]   Fig. 3. Normalization of the left ventricle diastolic diameter index (%) after operation. (AS, aortic stenosis; AR, aortic regurgitation; op., operation; m, month).

View larger version (19K): [in this window] [in a new window]   Fig. 4. Normalization of the left ventricle mass index (g/m2) after operation. (AS, aortic stenosis; AR, aortic regurgitation; op., operation; m, month).

	4. Discussion

Top Abstract 1. Introduction 2. Materials and methods 3. Results 4. Discussion 5. Conclusions Appendix A. Conference... References

The preferentially used technique of root replacement demonstrated excellent mid-term functional results with a mortality rate <5% [3,6,10,12]. Our study confirmed that the Ross and Ross–Konno procedure can be performed with a mortality rate approaching zero in both simple and complex left heart lesions, even in neonates and infants. The mid-term survival 98.5% at 5 years follow-up is very encouraging.

Conduction abnormalities and arrhythmias represent the most serious early postoperative complication especially if the patient required the complex reconstruction of LVOTO. Marino et al. [3] reported 28% incidence of ventricular tachycardia in the postoperative period. There was one early death secondary to a fatal arrhythmia in the report of Elkins et al. [4]. In this study one patient had a ventricular tachycardia leading to fibrillation on postoperative day 3 and was successfully resuscitated with ECMO. Apart from the conduction abnormalities the Ross procedure is well tolerated with LOS in hospital approximately 1 week.

The main concern is dilatation of the neo-aortic root leading to progression of AR. Provided that leaflets are normal, neo-aortic valve competence depends on the sinotubular junction and valve annulus. Thus, if the sinotubular junction dilates, the commissures are pulled outward and prevent the leaflets from coapting centrally. Similarly, if the annulus dilates, the base of the leaflets are pulled outward and cause valve incompetence [8,9]. Geometric mismatch in favor of the aortic annulus, which is a typical finding, especially in the settings of a bicuspid, chronically regurgitant aortic valve, can cause aortic insufficiency after the Ross procedure [7,8,13]. The adjustment of the diameter of the aortic annulus and/or the sinotubular junction of the aorta seems to be an important factor in preventing neo-aortic regurgitation after the Ross procedure. The sinotubular junction can be easily adjusted to the appropriate diameter by reducing the diameter of the ascending aorta before autograft-ascending aorta anastomosis is finished. Adjustment of the diameter of the aortic annulus with plication or annular reinforcement should be considered only in older patients (over 15 years old). Bearing this in mind, our policy was to indicate patients with predominantly AR earlier, before significant geometric mismatch in favor of the aortic annulus had developed. Only patients with mismatch less than, or equal to 5 mm were indicated for the Ross procedure and all these patients had plication of the annulus and adjustment of the sinotubular junction.

Early development of significant (moderate) neo-aortic regurgitation is usually associated with using a morphologically abnormal pulmonary valve for the Ross procedure [3]. One of our patients, in whom a morphologically abnormal pulmonary valve was used, needed AVR 2.3 years after operation.

Another potential risk factor for Ross failure is mechanical adaptation phenomena in the wall of the autograft associated with elastic fiber fragmentation [14,15]. Schoof et al. [16], in an experimental study, demonstrated that the architectural differences between the well-organized elastic lamellae of the aortic media and the thinner less-organized elastic lamellae of the pulmonary media of the autograft are retained after operation. The remodeling of the autograft, triggered by increased wall stress and shearing force, was a subtle process restricted to the differentiation of few cell types with the potential to respond to altered mechanical load. Ishizaka et al. [17] demonstrated severe elastin fragmentation in all excised pulmonary autograft walls during the valve spearing aortic root replacement for dilatation of the autograft and aortic regurgitation after the Ross procedure. It is obvious that the postoperative control of blood pressure, decreasing the wall stress, may be critically important. Our protocol includes aggressive postoperative control of blood pressure, usually extended 3–6 months after surgery.

In this study the functional outcome on neo-aortic valve with actuarial 95% freedom from more than trivial neo-aortic regurgitation, or valve replacement at 5 years follow-up, is encouraging. In addition to this there was no patient with either recurrent LVOTO or significant aortic root dilatation. However, our follow-up is short.

As for the failure of Ross procedure, patients with bicuspid valve and chronic AR with dilated aortic root are the most problematic. It is possible that AR could alter the geometry and tissue characteristic of subvalvular LVOT. Another possibility is that these patients have inherent abnormality of the pulmonary annulus, valves with mucocystic degeneration of the stroma. In this time frame the risk of autograft failure in these specific subsets of patients remains to be determined [7,13,15].

Freedom from homograft replacements after the Ross procedure was 90% at 12 years follow-up [1]. Our results are along these lines. One patient needed homograft replacement and three others (5%) had balloon dilatation because of a gradient more than 40 mmHg. Overall freedom from homograft re-intervention (redo or balloon dilatation) is 91% at 4 years follow-up. The results achieved with the reconstruction of RVOT after the Ross procedure seems to be superior to other right-sided reconstruction for other reasons. One potential explanation is that orthotopical placement of over-sized pulmonary homografts provides the ideal hemodynamics and flow pattern. Nevertheless, reoperation will likely be necessary in very young children in whom more accelerated degeneration of the homograft can be expected.

Remodeling of the left ventricle after the Ross procedure, with normalization of the left ventricle diastolic diameter index and left ventricle mass index, can be expected over a 3-12 months period in every patient without the associated lesion.

	5. Conclusions

Top Abstract 1. Introduction 2. Materials and methods 3. Results 4. Discussion 5. Conclusions Appendix A. Conference... References

 
Our 7-year experience with the Ross and Ross–Konno operation has shown excellent mid-term results. The Ross and Ross–Konno procedure can be performed with a mortality rate approaching zero in both simple and complex left heart lesions even in the neonates and infants. The mid-term survival of 98.5% at 5 years follow-up is encouraging. It is the procedure of choice in children with severe anomaly of the aortic valve and/or left ventricular outflow tract obstruction. The main concern is dilatation of the neo-aortic root leading to progression of AR especially in the settings of geometric mismatch of the aortic and pulmonary roots and bicuspid, regurgitant aortic valve. The risk of autograft failure in these specific subsets of patients remains to be determined.

	Acknowledgments

 
We wish to thank Mrs. Elizabeth Grundy for her help in preparing the manuscript.

	Footnotes

 
Presented at the joint 17th Annual Meeting of the European Association for Cardio-thoracic Surgery and the 11th Annual Meeting of the European Society of Thoracic Surgeons, Vienna, Austria, October 12–15, 2003.

	Appendix A. Conference discussion

Top Abstract 1. Introduction 2. Materials and methods 3. Results 4. Discussion 5. Conclusions Appendix A. Conference... References

 
Dr V. Tsang (London, England): There are two comments I would like to make. First, in your conclusion you said mortality approaching zero even in the neonates and the small infants. How many neonates and how many infants in your series?

The second point, what is the optimum timing for children with aortic regurgitation in whom you are prepared to do a Ross? For me that is a very important issue. Quite often they are asymptomatic but with a big LV. When is the right time to do it?

Dr Hraska: There were 2 newborns and 5 infants, so altogether 7 patients less than one year of age. That was the limitation of the study. It's obvious that we didn't have enough newborns and infants to be absolutely sure, but still I think that this procedure can be performed with a pretty low mortality in this age group.

As for your second question, this is difficult to answer. Our policy was to perform the Ross earlier rather later. These patients, the majority of them, are asymptomatic. The indication criteria were progressive left ventricle dilatation, with or without dysfunction of left ventricle. On top of that, there were morphological criteria regard to the geometric mismatch of pulmonary and aortic valve. We proceeded with the operation, especially in the group of patients with long-term aortic regurgitation and bicuspid aortic valve, rather sooner before the significant geometric mismatch was noted on the echo. The geometric mismatch less than 5 mm in diameter was required to proceed with this type of operation. If it was more than 5 mm, we didn't proceed with the Ross procedure. But I agree that it is really very difficult to define the proper timing of the operation, especially in the group of patients with AR.

Dr Z. Prodan (Budapest, Hungary): Would you perform this operation in a neonate with a diagnosis of aortic stenosis after unsuccessful balloon dilatation as a rescue procedure in a poor-condition patient?

Dr Hraska: I think this is the other very good application of the Ross–Konno. This operation allows so-called rescue procedure. Of course, the risk of surgery in this setting is much higher. But after the failing of balloon, I think the second step should be the Ross, because otherwise, and we had one patient like that, you can end up with a situation of progressing fibroelastosis of the left ventricle. Fibroelastosis is a very bad background for further operation and procedures.

Dr Prodan: Do you have any idea about patients at risk for endocarditis?

Dr Hraska: After the Ross?

Dr Prodan: Yes. We had a series of 40 patients and we had one.

Dr Hraska: We lost one patient because of endocarditis, but I have no idea what the risk really is after the Ross. I don't think that there are enough data to be absolutely sure.

Dr G. Wernovsky (Philadelphia, PA, USA): Could you provide any information on the dilation of the neoaortic root that we have seen with some of these patients, as with some of the other operations where the pulmonary valve is used in a systemic circulation?

Dr Hraska: I think the follow-up is too short to answer this question, but there is a bunch of data, I think, that support the idea that some subset of patients, and basically we don't know which one, is prone to dilatation of the neoaortic root. I think in particular the patients with aortic regurgitation and a bicuspid valve belong to this category.

Dr H. Najm (Riyadh, Saudi Arabia): You had one septal aneurysm. Can you elaborate on what kind of patch material you use, or do you use the autograft with an RV outflow tract muscle?

Dr Hraska: Attached right ventricle anterior wall harvested with an autograft was used for patching ventriculoplasty incision. That is our technique. We haven't used artificial material for patching.

Dr Najm: So would you recommend against the use of this muscle extension and rather use an artificial patch in the conal incision?

Dr Hraska: For reconstruction we do not use artificial material.

	References

Top Abstract 1. Introduction 2. Materials and methods 3. Results 4. Discussion 5. Conclusions Appendix A. Conference... References

 

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