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Eur J Cardiothorac Surg 2000;17:426-430
© 2000 Elsevier Science NL

Mitral-valve replacement in children under 6 years of age

Klinik für Herz- und Gefäßchirurgie, Deutsches Herzzentrum, Klinik an der Technischen, Universität München, Lazarettstraße 36, 80636 Munich, Germany

Corresponding author. Tel.: +49-89-1218-4111; fax: +49-89-1218-4113
e-mail: chinfo{at}dhm.mhn.de

	Abstract

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

 
Objective: In patients with congenital mitral-valve disease, reconstructive surgery is the primary goal. However, in cases with severely dysplastic valves or failed repair, mitral-valve replacement (MVR) is the only option. We analyzed, retrospectively, data of 35 patients younger than 6 years of age, who underwent MVR at our institution.Methods: Between 1974 and 1997, 35 children underwent MVR. The ages ranged from 2.7 months to 5.5 years (mean=1.9±1.7 years) and body weight varied between 3.2 and 16.7 kg (mean=8.2±4 kg). The main indication (57%) for valve replacement was severe mitral-valve insufficiency. Eighteen patients (51%) had undergone at least one previous reconstructive operation (mean=1.46±1.86 years) before the MVR. In 29 cases (83%), mechanical prostheses were implanted. Six patients received a bioprosthesis. The size of the prostheses ranged between 14 and 27 mm.Results: The overall hospital mortality was 17.1% (6/35), and decreased from 33 (1974–1985) to 11.5% (1986–1997). Seven children died late. The actuarial survival after 20 years was 51.2±13.3%. Eight patients (23%) required 10 reoperations (8.2%/100 patient-years). Freedom from reoperation at 10 years was 50±22%. Valve-related complications were thrombo-embolism (n=2; 1.6%/100 patient-years), hemorrhage (n=1; 0.8%/100 patient-years), structural deterioration (n=3; 2.5%/100 patient-years) and non-structural dysfunction (n=3; 2.5%/100 patient-years). Follow-up is 96% complete, with a total of 122 patient-years (mean=4.2±4.7 years). Eighty six percent of the patients are in New York Heart Association (NYHA) class I, 95% have sinus rhythm and 59% do not need medication. All survivors, except for those who received a bioprosthesis, were placed on a regimen of Phenprocoumon (Marcumar^®), aiming to maintain the International Normalized Ratio (INR) between 2.5 and 3.5. In one third of these children, self-management of oral anticoagulation was performed either by the patients or their parents.Conclusions: MVR in small children still carries a high risk. In our experience, the long-term results are satisfying. After failed reconstructive surgery, or as a primary procedure, we prefer mechanical prostheses. They are well tolerated and the incidence of anticoagulation-related complications is low.

Key Words: Mitral-valve replacement • Children • Mechanical prostheses

	1. Introduction

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

 
In patients with congenital mitral-valve disease, reconstructive surgery is the primary goal and valve-repair techniques are very effective [1,2]. However, in cases with severely dysplastic valves or after failed repair, mitral-valve replacement (MVR) is the only option. The ideal prosthesis for MVR in children has not yet been found, and some authors even regard valve replacement in pediatric patients as a palliative procedure, as all available prostheses have significant drawbacks [3]. For the present study, we retrospectively analyzed the data of 35 patients younger than 6 years of age, who underwent MVR at our institution.

	2. Material and methods

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

 
Between November 1974 and November 1997, 35 children younger than 6 years of age, underwent MVR at the German Heart Center, Munich. Eighteen were male and 17 were female. The age at the initial MVR ranged from 2.7 months to 5.5 years (mean=1.9±1.7 years) and body weight varied between 3.2 and 16.7 kg (mean=8.2±4 kg). Fifteen patients underwent MVR in the first year of life. The main indication (57%) for valve replacement was severe mitral-valve insufficiency. Seven patients had a complete atrioventricular septal defect (CAVSD, Rastelli type A in six and type C in one) and two had a partial atrioventricular septal defect (PAVSD). Seventeen patients, other than those with atrioventricular septal defect (AVSD), had associated cardio-vascular anomalies, including aortic and subaortic stenosis in four, coarctation of the aorta in four, ventricular septal defect (VSD) in three, Bland–White–Garland syndrome in three, congenital occlusion of the circumflex artery in one, pulmonary atresia and VSD in one, and single ventricle with pulmonary stenosis and VSD in one (Fig. 1). Two patients developed a complete heart block before MVR (one after a previous VSD-closure and one after CAVSD repair) and permanent pacemakers were implanted. All patients underwent at least one cardiac catheterization (Department of Pediatric Cardiology, Director: Professor Dr J. Hess). Most patients showed some degree of pulmonary hypertension. The mean systolic pulmonary artery pressure was 60±20 mmHg (median=56 mmHg). Ten patients (28.6%) were in New York Heart Association (NYHA) functional class IV.

View larger version (25K): [in this window] [in a new window]   Fig. 1. Age distribution of 35 children, who underwent MVR and the corresponding diagnoses.

2.2. Operative data
A median sternotomy was performed in all patients. Thirty three patients (94%) were operated on with continuous extracorporeal circulation and moderate hypothermia (rectal temperature, 24–28°). The mean total bypass time was 108±43 min and mean aortic cross-clamp time was 71±37 min. Deep hypothermic circulatory arrest was used in two patients. The mitral valve was exposed through the right atrium in three, and through the left atrium in the remaining patients. We assessed the left atrioventricular (AV) valve-diameter with a Hegar dilator, applying the nomograms of Rowlatt and colleagues [4]. In 29 cases (83%), mechanical (mainly bileaflet) prostheses were implanted. Six patients received a bioprosthesis. The size of the prostheses ranged from 14 to 27 mm. In all cases, the implantation of a prosthesis greater than or equal to the valve size according to Rowlatt was possible (Fig. 2). In one patient, the prosthesis was sutured above the native mitral valve (supra-annular MVR). In all other patients, the prosthesis was placed within the native annulus. Additional surgical procedures in 14 patients (40%) included the resection of subaortic stenosis (three), aortic valvulotomy (one), VSD and ASD-closure (one), coarctectomy (one), PAVSD repair (one), ligation of patent ductus arteriosus (one), VSD-closure (one), tricuspid valvuloplasty (two), tricuspid-valve replacement (Hancock 29 mm) (one), aortic-valve replacement with a 10-mm aortic homograft (one) and the Rastelli procedure (one).

View larger version (18K): [in this window] [in a new window]   Fig. 2. Relation between size of the prosthesis used at (•), initial MVR; and (), valve size according to Rowlatt.

	3. Results

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

 
The overall hospital mortality was 17.1% (6/35) and decreased from 33 (3/9; 1974–1985) to 11.5% (3/26; 1986–1997). Seven children (20%) died late. The actuarial survival after 20 years was 51.2±13.3% (95% confidence limit; see Fig. 3). Operative and late mortality were mainly due to cardiac failure. The causes of early and late mortality are reported in Table 1. Statistical analysis failed to identify age of <1 year (P=0.698), weight of <5 kg (P=0.135), ECC-time (P=0.776), aortic x-clamp-time (P=0.823), pulmonary artery pressure (P=0.220), or type of prosthesis (P=0.268) as risk factors for early mortality. Patients with AV-septal defect or additional cardiovascular anomalies showed a higher risk for early death (P=0.031).

View larger version (12K): [in this window] [in a new window]   Fig. 3. Kaplan–Meier estimate of survival function after MVR. The actuarial survival at 20 years is 51±13.3% (95% confidence limit).

View larger version (13K): [in this window] [in a new window]   Fig. 4. Kaplan–Meier estimate of freedom from reoperation after MVR. Freedom from reoperation at 10 years is 50±22% (95% confidence limit).

	4. Discussion

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

 
Patients with congenital mitral-valve disease present a great variability in valve anomalies, and mitral-valve surgery is a great challenge, especially in small children. Valve-repair techniques can be accomplished with good results and are considered the procedure of choice [1,2]. Valve replacement is reserved for patients with severely dysplastic valves or after failed repair. Patients who require MVR within the first few years of life pose several problems, including the small size of the mitral annulus, left atrium and left ventricle; frequent association with other cardiovascular anomalies; limited valve selection; obstructive hemodynamics of small prostheses; and difficulty with anticoagulant therapy [6,7]. The problem of somatic growth is another matter of concern, because in this particular age group, small prostheses have to be used and later replacement is unavoidable. MVR in small children is generally associated with a high operative and late mortality. In our series, overall operative mortality was 17% and could be reduced from 33 (1974–1985) to 11.5% (1986–1997). Our results compare favorably with those of other authors, who report an operative mortality between 13 and 36% [6–9]. Various risk factors for operative mortality have been questioned. Zweng and associates, analyzing the data of 19 patients who underwent MVR within the first 5 years of life, found no correlation between early and late mortality and various risk factors (age, weight, sex, previous or simultaneous operation, underlying cardiac lesion, prosthesis size, or type of valve) [6]. Kadoba and co-workers, analyzing the anatomic subgroups of 25 patients with MVR within the first year of life, report the highest mortality in patients with AV-septal defect [7]. Seven of our patients (20%) had complete AV-septal defect. None of them underwent primary valve replacement. Hospital mortality in this subgroup was 43%. Schaffer and colleagues state that annular placement of a mitral-valve prosthesis is critical to good outcome. In their study, seven out of eight patients with supra-annular implantation died [10]. In our series, supra-annular MVR was performed only in one patient; in all the other cases, the annular implantation of a prosthesis greater than or equal to the valve size according to Rowlatt was possible [4]. The choice of the prosthetic-valve type remains controversial. All available prostheses have significant drawbacks. Glutaraldehyde-treated porcine heterografts in this particular age group show early calcification [6,11,12]. Zweng and colleagues report, that four of ten patients with bioprostheses in their study had to be reoperated on within 17–47 months [6]. In our series, six patients underwent MVR with a bioprosthesis. Two of them required repeated valve replacement because of structural degeneration after an average of 2.5 years. Three of our patients required repeated valve replacement because they had outgrown the previously-implanted mechanical prostheses after 6.7 years on average. In all of them, a valve two sizes larger than the original prosthesis could be inserted. Prosthetic-valve replacement was not associated with any operative deaths in our series. Nudelmann and colleagues demonstrated that the annulus of the mitral valve will grow even when fixed to a prosthetic sewing ring, and thus, a larger-sized prosthesis can be implanted at the time of re-replacement [13]. The advantages of mechanical prostheses include better hemodynamic flow characteristics in small sizes, low profile with less potential for left ventricular outflow tract obstruction and longer durability [6]. Anticoagulation therapy is a matter of concern in children with mechanical-valve replacement. Some authors suggest that neither aspirin nor antiplatelets or warfarin are needed in children with mechanical prostheses in the mitral or aortic position [9,14]. Other investigators, however, report that anticoagulation with warfarin-derivatives is warranted [6,7,15]. Bradley and associates state that Coumadin and the combination of aspirin plus dipyridamole provides similar protection against anticoagulation-related complications [16]. All of our patients who underwent MVR with a mechanical prosthesis are on a regimen of Phenprocoumon (Marcumar^®), aiming to maintain an INR between 2.5 and 3.5. In 30%, self-management of oral anticoagulation is performed by the patients or their parents. One patient suffered an episode of cerebral bleeding 8 months after MVR. Prothrombin-time in this case was far below the normal range.

The results of this series demonstrate that MVR in this particular age group is associated with a considerable operative and late mortality. If valve replacement is unavoidable, we prefer the implantation of a bileaflet-mechanical prosthesis. Anticoagulation with Phenprocoumon (Marcumar^®) is well tolerated in this age group, and the incidence of anticoagulation-related complications is low.

	Footnotes

 
Presented at the 13th Annual Meeting of the European Association for Cardio-thoracic Surgery, Glasgow, Scotland, UK, September 5–8, 1999.

	Appendix A. Conference discussion

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

 
Dr T. Tlaskal (Prague, Czech Republic): I would like to ask two questions. What is your currently preferred prosthesis for valve replacement, and do you try to preserve some part of the mitral-valve apparatus during replacement?

Dr Guenther: As I said, we prefer mechanical prostheses, and we have used different types of mechanical prostheses.

Dr Tlaskal: I mean a type, I suppose that is bileaflet. I think that a very important possibility is to rotate the valve after placement.

Dr Guenther: We currently use bileaflet prostheses.

Dr B. Messmer (Aachen, Germany): Just for curiosity, where and when did you get a 14-mm valve which is rather special? And the second thing, just a trick which is certainly used by others too: for MVR and especially in small infants, I generally take an aortic valve and use it upside down. By this method you generally get at least one size bigger than you would normally implant.

Dr Guenther: In two patients, a stentless bioprosthesis was used, one of these with a 14-mm diameter because the annulus did not accept the smallest-available mechanical bioprosthesis. This patient had to be reoperated within 5.5 months, because the stentless bioprosthesis was extremely calcified. A mechanical prosthesis was implanted and the patient is doing well.

Dr A. Corno (Lausanne, Switzerland): I was quite surprised to see that you decided to implant a substantial number of biological prostheses in this age group, when it is well known that because of the accelerated calcium metabolism you have an earlier occurrence of calcification of the valve, as you have seen. So could you explain why you decided to employ a substantial number of biological prostheses, particularly when you have a mechanical prosthesis with better hemodynamic performance for the external–internal ratio and the same external diameter?

And since you have seen that even at this age the anticoagulation is very well tolerated, then why, once you implanted a biological prosthesis, did you still decide to use anticoagulation in biological prostheses, with the exception of two patients?

Dr Guenther: We only had six patients who received a bioprosthesis. Since 1984, we don't implant bioprostheses any more.

Dr P. Burczynski (Warsaw, Poland): I have one comment concerning indication for reoperations. In your series, you had one case where you had to reoperate because of thrombus complications. In our experience of I think around 30 patients, children who underwent MVR, we had two cases with thrombus complications, and in both cases, as a first step of treatment we used thrombolytic treatment, I mean Actylise, and in both cases we achieved very good results with very good mobilization of the artificial mitral-valve leaflets, and in those two cases we avoided operations.

Dr Guenther: The patient mentioned developed valve thrombosis and was readmitted in very poor condition; he underwent emergency surgery and the thrombus was removed.

Dr Burczynski: Our two patients were also admitted in very bad condition, they needed to be ventilated, and we immediately started Actylise, intravenous infusion, and this thrombus disappeared after a few h, and after a few days we achieved very good mitral-valve mobilization. This is our very early experience and not too many cases, but this is the first step in our choice.

Dr R. Guerrero (Newcastle-Upon-Tyne, UK): It is 30 years of experience. In many of the countries in the Third World you have probably the highest incidence because of rheumatic fever or endocarditis; you have 95% of patients who remained in sinus rhythm, and I am curious to know if you could comment about your technical approach in the mitral valve for the left atrium? Was it trans-septal, through the groove, or through the roof? Could you comment on that?

Dr Guenther: Excuse me, I didn't understand the last part.

Dr Guerrero: Right. It is, if you can comment about your current technique approaching the mitral valve. Was it biatrial, trans-septal, or standard LA atriotomy, or through the roof of the LA?

Dr Guenther: Usually, the mitral valve was exposed through the interatrial groove, and only in three patients the mitral valve was exposed through the right atrium. These patients had additional anomalies.

Dr B. Maruszewski (Warsaw, Poland): I didn't understand from your presentation, what is your recent policy? Do you still replace a lot of mitral valves or is there a tendency of reduction of this type of operation towards the mitral-valve repair in recent years, because it is a 23-years series?

Dr Guenther: We don't advocate MVR. We always try a reconstructive procedure as far as it is possible.

	References

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

 

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This Article Abstract Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Domenico Mazzitelli Sung-Un Paek Hans Meisner Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Günther, T. Articles by Lange, R. Search for Related Content PubMed PubMed Citation Articles by Günther, T. Articles by Lange, R.