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Ann Thorac Surg 1998;66:1551-1559
© 1998 The Society of Thoracic Surgeons

Surgical repair of the congenitally malformed mitral valve in infants and children

^a Divisions of Cardiovascular-Thoracic Surgery and Cardiology, The Children’s Memorial Hospital, and Departments of Surgery and Pediatrics, Northwestern University Medical School, Chicago, Illinois, USA

Accepted for publication May 12, 1998.

Address reprint requests to Dr Mavroudis, Division of Cardiovascular-Thoracic Surgery, Mail Code #22, Children’s Memorial Hospital, 2300 Children’s Plaza, Chicago, IL 60614
e-mail: (c-mavroudis{at}nwu.edu)

Presented at the Second World Congress of Pediatric Cardiology and Cardiac Surgery, Honolulu, Hawaii, May 11–15, 1997.

	Abstract

Top Abstract Introduction Material and methods Results Comment References

 
Background. Mitral valve remodeling techniques were applied to 26 infants and children (mean age, 6.0 years, range, 0.4 to 15.9 years) with various forms of congenital mitral valve disease over a 7-year period. Patients with atrioventricular canal, L-transposition and single ventricle were excluded. Intraoperative transesophageal echocardiography (TEE) was utilized to assess the repair and guide the need for immediate intervention.

Methods. Twenty-one patients had mitral regurgitation: 10 with cleft anterior mitral leaflet, 7 with annular dilatation, 1 with normal leaflets with an obstructing cord, 2 with prolapsed leaflets and elongated cords, and 1 with restricted leaflet motion, normal papillary muscles, and shortened cords. Of the 5 mitral stenosis patients, 3 had supravalvular mitral ring, 1 had midvalvular mitral ring, and 1 had a parachute valve. Three of the mitral stenosis patients had additional stenotic lesions. Concurrent repair of associated lesions was performed in 21 patients (78%).

Results. Operative mortality was 3.8% (n = 1). There were no late deaths. Immediate rerepair in 4 patients resulted in improved function. All mitral stenosis patients improved. A total of 20 mitral regurgitation patients (95%) improved; 1 required mitral valve replacement. Mean follow-up is 31 months (range, 2 to 81 months). All patients are in New York Heart Association functional class I or II.

Conclusions. Mitral valve repair can be successfully performed in infants and children with excellent short- and midterm results. Assessment using transesophageal echocardiography can guide the necessity for immediate rerepair to achieve improved function.

	Introduction

Top Abstract Introduction Material and methods Results Comment References

 
Mitral valve repair using remodeling valvuloannular techniques has developed into a reproducible, reliable and preferable alternative to prosthetic mitral valve replacement in adults [1]. Reconstructive techniques for infants and children have evolved more slowly due to the great variability of mitral disease and the uncertain effect that growth will have on the mitral apparatus [2–13]. The purpose of this paper is to review our experience with various mitral valve remodeling techniques utilizing intraoperative transesophageal echocardiography (TEE) in 26 infants and children with congenital lesions of mitral insufficiency and stenosis.

	Material and methods

Top Abstract Introduction Material and methods Results Comment References

 
From 1989 to 1997, 26 infants and children underwent mitral valve repair at our institution. The mean age was 6.0 ± 4.5 years (range, 0.7 to 16.0 years). Patients with atrioventricular canal defects, single ventricle, and L-transposition were excluded. Included in our analysis are 2 patients with congenital heart abnormalities leading to mitral valve disease: 1 with Marfan’s syndrome and mitral insufficiency, and another with annular dilatation and ventricular dysfunction due to severe congenital left main coronary artery stenosis.

Twenty-one patients had congenital mitral valve regurgitation (MR) and 5 had congenital mitral valve stenosis (MS). The patients were grouped according to Carpentier’s pathophysiological classification [5] (Table 1). We referred to the mitral valve leaflets as anterior and posterior, although we acknowledge the preference of others [4] to use the terms aortic and mural leaflets, respectively. For those patients with multiple abnormalities, the predominant lesion was used for classification. All patients had preoperative echocardiography. Mitral regurgitation was moderate or severe in 16 (77%) of 21 patients and MS was moderate or severe in 4 of 5 patients (80%).

Ten patients with normal leaflet motion and a cleft anterior mitral leaflet were treated with suture closure of the cleft without removal of the chordae attached to the edges of the cleft (Fig 1). Further annuloplasty techniques were not necessary in any of these patients since the valve was competent after the repair. Associated lesions were present in 4 patients and were repaired concomitantly.

View larger version (111K): [in this window] [in a new window]   Fig 1. (A) Cleft anterior mitral valve leaflet. (B) The repair is accomplished by simple, interrupted, and inverted sutures. (C) Closure of the cleft will result in a competent valve without added annuloplasty techniques in a great majority of cases.

View larger version (108K): [in this window] [in a new window]   Fig 2. (A) Artist’s representation of an insufficient mitral valve due to annular dilatation. Dotted lines represent the proposed posterior leaflet quadrangular resection and valvular incisions for the sliding plasty. (B) After the quadrangular resection, linear incisions are made in the posterior leaflet to accomplish the sliding valvuloplasty (arrows). (C) The posterior annulus is brought together with care not to injure the circumflex coronary artery. The resultant tightening of the annulus brings the remaining scallops of the posterior leaflets in a position for valvular repair. (D) The repair is strengthened by a semicircular Gore-Tex (W. L. Gore & Assoc, Flagstaff, AZ) strip. The anterior annulus was not included in the annuloplasty technique due to somatic growth considerations.

There were 5 patients with MS. Three had resection of a restrictive supravalvular ring (Fig 3) and 1 had resection of a midvalvular fibrous ring (Fig 4). Care was taken not to injure the valve or the circumflex coronary artery while resecting the fibrous ring. Relief of concomitant stenotic lesions in these patients was undertaken at the same time, except for the double orifice mitral valve which was left intact for fear of creating insufficiency (Fig 5). The fifth patient had repair of a parachute mitral valve by valvular fenestration and papillary muscle incision (Fig 6).

View larger version (30K): [in this window] [in a new window]   Fig 3. Diagrammatic lateral cut-away view of a stenotic mitral valve caused by a restrictive supravalvular fibrous ring. Sharp dissection can free the fibrous ring from the native annulus, frequently resulting in dramatic increases in orifice size.

View larger version (65K): [in this window] [in a new window]   Fig 4. Rarely a fibrous mitral ring can be found within the substance of the mitral valve leaflets causing valve stenosis (midvalvular ring), as well as leaflet restriction. Oftentimes, associated obstructive lesions can be identified which will require therapy, such as excess valvular tissue and fused papillary muscles.

View larger version (89K): [in this window] [in a new window]   Fig 5. Diagrammatic (A) left atrial and (B) cut-away atrioventricular views of a double-orifice mitral valve. More commonly, the smaller of the two orifices is in the right lateral position. The degree of stenosis is variable. Effective orifice enlarging reparative techniques have not been developed.

View larger version (116K): [in this window] [in a new window]   Fig 6. Cut-away atrioventricular view (A) and left atrial views (B, C) of a stenotic parachute mitral valve. (A) A single papillary muscle or fused papillary muscles usually arise from the posterior left ventricular wall. (B) The dotted lines show the areas of proposed leaflet fenestrations and papillary muscle incision to open the ventricular inlet. (C) Leaflet fenestrations are accomplished to maximize unrestricted blood flow into the left ventricle during diastole while preserving enough valvular tissue for effective coaptation during systole. The fused papillary muscle is being incised to facilitate valvular mobility.

	Results

Top Abstract Introduction Material and methods Results Comment References

Of the 20 patients who had MR (including the patient who died after intracerebral hemorrhage) 95% improved by postoperative echocardiogram to trivial or mild regurgitation. One patient remained unchanged with moderate MR, and subsequently underwent mitral valve replacement (number 19 St. Jude mitral prosthesis) 1 week after repair. She required pacemaker insertion for heart block 8 days after valve replacement. Another patient needed a pacemaker insertion after mitral valve repair, and an additional patient was readmitted for a pericardial effusion that was drained.

Of the patients with MS, all improved postoperatively as demonstrated by echocardiography. The patient with double outlet right ventricle, supravalvular ring and double orifice mitral valve, although improved, remained with mild mitral stenosis.

No embolic episodes were reported in any patient; no hemorrhagic events were reported in our 1 patient receiving anticoagulation for a prosthetic heart valve.

	Comment

Top Abstract Introduction Material and methods Results Comment References

 
The spectrum of congenital mitral valve malformations ranges from the readily reparable cleft anterior mitral leaflet to the restrictive and challenging lesions of MS. Whatever the abnormality, the incidence of associated heart defects is high [3, 6, 8] and can have a significant impact on the eventual clinical outcome.

Because of the significant short- and long-term problems with mechanical and bioprosthetic mitral valves in children [15, 16], considerable attention has been paid to mitral remodeling techniques [6–8, 12] for insufficient and stenotic valves. This trend has lowered the perioperative mortality from as high as 21% to 43% for mitral valve replacement [2, 9, 17] to as low as 0% to 5% for mitral valve repair [6, 7, 9, 12, 13]. Our overall 3.8% operative mortality compares with these trends.

The most common cause of congenital mitral insufficiency in our series was cleft anterior mitral valve leaflet. In the greater percentage of cases, simple cleft closure will result in a competent valve without annuloplasty remodeling techniques and will likely be the only necessary therapy. More challenging is the infant and young child with annular dilatation and mitral insufficiency who will require annular remodeling. Early in our experience we used DeVega [18] annuloplasty techniques, but later converted to Carpentier’s techniques using annular rings in older children. An annular ring will reinforce and improve the repair, but size considerations in a growing child confound the problem. Chauvaud and associates [6] have approached this problem by using one of three solutions based on the patient’s size and degree of annular dilatation. One solution is to increase the size of the anterior leaflet using a gluteraldehyde preserved autologous pericardial patch which will advance the anterior leaflet, increase its mobility, and allow the use of an adult-sized prosthetic ring (number 28 or 30). Our experience with anterior leaflet enlargement is favorable, but confined to patients with complete atrioventricular canal who have insufficient valvular tissue or endocarditis [19, 20]. We would not hesitate to use this technique in appropriate patients with annular dilatation and mitral insufficiency. Another solution is to use a smaller ring (number 26) without anterior leaflet enlargement. The third solution is for babies and young children where annular reduction and remodeling techniques are used, based on Rowlatt’s [21] measurements, without prosthetic rings. Chauvaud’s series of 135 patients under 12 years of age undergoing these techniques for mitral insufficiency is the largest reported experience and showed a remarkable 4% mortality and 5% reoperation rate over an 8.4 year follow-up [6]. They did not enumerate the number of children under 1 year of age. Our experience with infants is small. We chose to use a semicircular Gore-Tex strip (Fig 2) to reinforce the posterior annuloplasty in an 8-month-old with favorable results 2 years postoperatively. Sousa and associates [12] recently reported on 10 patients with mitral insufficiency under 1 year of age who underwent commissural or posterior leaflet annuloplasty techniques without a complete or partial prosthetic ring. They achieved excellent and stable results in 7 patients at a mean follow-up of 5.5 years (3 patients had mitral valve replacement). The overwhelming evidence supports the idea that prosthetic rings, be they complete or partial, are not necessary to achieve favorable results in infants and young children.

The most common cause of MS in our series was supravalvular mitral ring, associated in most cases with other stenotic lesions such as excess valvular tissue, papillary muscle fusion, and double orifice mitral valve. Others [7, 12] have reported similar findings. We found that effective resection of the supravalvular and midvalvular mitral ring may uncover other stenotic lesions that can be treated by conservative measures such as papillary muscle incision and excess valvular resection. The notable exception to repair is double orifice mitral valve, which has been the subject of anatomic study [22] and speculation that attempted repair would lead to flail leaflets. Most authors have advocated mitral valve replacement when indicated under these circumstances [23]. We encountered 1 patient with parachute mitral valve based on fused papillary muscles originating from the posterior left ventricular wall who underwent papillary muscle incision and leaflet fenestration (Fig 6). While some authors [24] recognize this anatomy as parachute mitral valve, others [11] do not, insisting that the parachute valve moniker be given only when it is connected to a single papillary muscle and not a series of fused ones. The surgical significance of this difference may be in the ease that papillary muscle incision be performed. In any case, close attention must be paid to the papillary muscle incision to prevent acute regurgitation or delayed rupture.

Reoperation after chest closure and transfer to the intensive care unit occurred in 1 patient who underwent prosthetic mitral valve replacement after failed mitral valve repair. This reoperation rate of 3.8% compares favorably to other reports [2, 3, 10, 12] of mitral valve repair in children. The immediate reoperation rate before chest closure on the basis of hemodynamic findings and intraoperative TEE is not widely reported. We performed immediate reoperation and repair in 4 patients (15.4%), resulting in improved mitral valve function in each case. Others [2, 6, 7] have recognized the importance of intraoperative TEE for functional evaluation that can give the surgeon the option for reintervention as deemed necessary.

Most reports dealing with congenital mitral anomalies center on the obvious issues of regurgitation and stenosis. However, we have encountered 3 patients with mitral insufficiency in whom the mitral apparatus complicated the intended cardiac repair by obstruction to flow or impediment to ventricular septation (mitral attachments to the left ventricular outflow tract or straddling cord). These significant problems may or may not be associated with mitral malfunction, but will require mitral reconstruction to achieve the beneficial effects of the intracardiac repair. Two patients (patients 18 and 19) in our series required papillary muscle detachment and reimplantation as part of the mitral and congenital heart repair; 1 to relieve left ventricular outflow tract obstruction in association with correction of transposition of the great arteries and ventricular septal defect closure; and another to transfer a straddling papillary muscle to allow ventricular septal defect closure in association with tetralogy of Fallot repair. The third patient (patient 21) had left ventricular outflow tract obstruction relief by fibromuscular resection and resection of obstructing secondary cords of the anterior leaflet. The 2 surviving patients (patient 18 died of cerebral hemorrhage) have had effective congenital repairs and are without mitral regurgitation in the midterm follow-up. This is a small series to advocate routine papillary muscle transfer, especially in light of previous reports [25, 26] which advocate ventricular septal defect closure around an intact straddling papillary muscle. We submit that in certain circumstances, mitral papillary muscle detachment and reimplantation can be performed in association with congenital heart repairs to relieve left ventricular outflow tract obstruction and simplify ventricular septal defect closure.

Our postoperative morbidity rate is minimal and similar to the experience of other centers [2, 6, 7, 12]. The lack of thromboembolic episodes observed in our patients is also consistent with the findings of others [2, 6, 7, 12] and probably contributes to the low postoperative morbidity. We believe that anticoagulation therapy is not needed for patients with mitral valvuloplasty except for the first 3 months postoperatively when a prosthetic ring is used.

In summary, mitral valve repair in infants and children using Carpentier techniques can be accomplished with excellent short-term and midterm results with a low mortality and low incidence of prosthetic valve replacement. Routine use of TEE improves decision-making, guides the necessity for intraoperative rerepair and decreases the incidence of reoperation after chest closure. Papillary muscle reimplantation can be accomplished in association with congenital heart repairs to relieve left ventricular outflow tract obstruction and facilitate ventricular septal defect closure.

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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): Elias A. Zias Constantine Mavroudis Carl L. Backer Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Zias, E. A. Articles by Rocchini, A. P. Search for Related Content PubMed PubMed Citation Articles by Zias, E. A. Articles by Rocchini, A. P.