Asian Cardiovasc Thorac Ann 2008;16:129-133
© 2008 Asia Publishing EXchange Ltd
Valve Repair in Rheumatic Heart Disease in Pediatric Age Group
Pramod K Reddy, MCh,
Anil K Dharmapuram, MCh,
Sunil K Swain, MCh,
Nagarajan Ramdoss, MD,
Sreekanth S Raghavan, AB,
Kona S Murthy, MCh
Department of Pediatric Cardiac Surgery Apollo Childrens Heart Hospital Hyderabad, India
For reprint information contact: Kona Samba Murthy, MCh, Tel: 91 40 2360 0852, Fax: 91 40 2360 8050, Email: konasmurthy{at}gmail.com, Apollo Childrens Heart Hospital, Jubilee Hills, Hyderabad 500033, India.
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ABSTRACT
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Valve repair in children is technically demanding but more desirable than valve replacement. From April 2004 to September 2005, 1 boy and 8 girls with rheumatic heart disease, aged 2–13 years (median, 9 years), underwent valve repair for isolated mitral regurgitation in 5, combined mitral and aortic regurgitation in 2, mitral stenosis in 1, and mitral regurgitation associated with atrial septal defect in 1. Chordal shortening in 7, annular plication in 6, commissurotomy in 1, reconstruction of commissural leaflets in 7 were performed for mitral valve disease. Plication and reattachment of the aortic cusps was carried out in 2 patients. Annuloplasty rings were not used. All patients survived the operation, 8 had trivial or mild residual mitral regurgitation, and 1 had trivial aortic regurgitation. Mean left atrial pressure decreased from 14 to 7 mm Hg postoperatively. During follow-up of 3–18 months, all children were asymptomatic and enjoyed normal activity. None required reoperation. In addition to chordal shortening and annular plication, reconstruction of the commissural leaflets is considered the most important aspect of valve repair. It can be achieved without annuloplasty rings, giving good early and midterm results.
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INTRODUCTION
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Valve repair is now well established in adults, with long-term follow-up confirming its reliability.1,2 Valve repair is preferred to valve replacement when technically feasible, but experience in children with rheumatic heart disease is limited.3–8 Valve repair offers the advantages of avoidance of thromboembolism, preservation of chordal function, lower operative mortality and continued growth of the valve in young patients.9,10 Thus valve repair appears to be the most desirable option in children; however, it is more challenging and technically demanding. Because of the wide spectrum of lesions, a more customized approach with multiple repair techniques is required.11 We report our experience of valve repair for rheumatic heart disease in the pediatric age group.
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PATIENTS AND METHODS
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From April 2004 to September 2005, 1 boy and 8 girls aged 2–13 years (median, 9 years), with rheumatic heart disease, underwent valve repair. Two were younger than 5 years of age. Five had isolated mitral regurgitation (MR), 2 had combined MR and aortic regurgitation (AR), 1 had mitral stenosis, and 1 had MR associated with an atrial septal defect. Eight patients were in New York Heart Association functional class III and 1 was in class IV at the time of operation. Valve morphology was assessed thoroughly by 2-dimentional echocardiography to determine the cause of valve dysfunction and to customize valve repairs. Preoperative transthoracic echocardiography and intraoperative transesophageal echocardiography were performed in all patients. Echocardiographic assessment included annulus size, leaflet thickness and mobility, commissural and chordal fusion, the presence of calcification, site and amount of the regurgitant jet, thickness of the chordae tendineae and the presence of clots in the left atrium or left atrial appendage in mitral valve disease.8 For aortic valve disease, the regurgitation jet, cusp prolapse, rolling of cusps, degree of coaptation of the valve and condition of the other valves were assessed in a systematic fashion. The mitral valve annulus was dilated in 6 patients. Based on the Carpentier classification, leaflet prolase was present in 7 patients, restricted leaflet motion in 1, and normal leaflet motion in 1.
Surgery was undertaken through a midline sternotomy. Cardiopulmonary bypass was instituted with aortic and bicaval cannulation, with systemic hypothermia of 30°C. Antegrade cold blood cardioplegia and topical ice slush were used for myocardial protection. The mitral valve was exposed through a standard incision in the left atrial wall behind the interatrial groove. The aortic valve was exposed by a transverse incision in the anterior wall of the aorta above the aortic valve. After careful evaluation of the valve apparatus, reconstruction was customized according to valve morphology. The techniques included chordal shortening, annular plication, commissurotomy and reconstruction of commissural leaflets, and plication and reattachment of the aortic cusps. Chordal shortening was performed at the papillary muscle level in most cases, using pledgeted 6/0 or 7/0 polypropylene sutures to plicate the elongated chordae and correct cusp prolapse (Figure 1
).12,13 Modified De Vega annuloplasty was carried out for annular plication, using pledgeted polypropylene sutures. This was accomplished in 2 parts at the commissures, to allow for annular growth (Figure 2
). A leak was found at the commissural leaflet in 7 patients, due to ill-formed commissures with multiple small leaflets. Reconstruction of the commissural leaflets was achieved by suturing the multiple valve leaflets together, passing sutures through individual valve leaflets to form a new commissure. This technique was an important aspect of mitral valve repair in most of our cases (Figure 3
). For aortic valve repair, the redundant noncoronary cusp was plicated at the commissure with the adjacent right coronary cusp, slightly lifted up, and reattached to the commissure using 7/0 polypropylene sutures. This allowed the cusp to coapt with the other cusps (Figure 4
).14 On completion of the repair, mitral valve competence was tested using a 50-cc syringe to inject saline into the left ventricular cavity to observe coaptation of the leaflets. To check for aortic valve competence, water was infused into the aortotomy to detect leakage. Transesophageal echocardiography was also undertaken to assess the repairs. Left atrial pressure was measured both pre- and postoperatively. Transthoracic echocardiography was performed before discharge from the hospital and at regular intervals during follow-up. All patients were given aspirin for 3 months and a long-acting penicillin injection for rheumatic prophylaxis.

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Figure 1. (A) Elongated cords causing incompetence. (B) Plication of elongated chords with pledgeted polypropylene suture.
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Figure 3. (A) Leakage at the commissures due to ill-formed commissures with multiple small leaflets; (B) Suturing the multiple valve leaflets together to form a new commissure; (C) The reconstructed commissural leaflets.
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Figure 4. The redundant noncoronary cusp is plicated at the commissure with the adjacent right coronary cusp, slightly lifted up, and reattached to the commissure.
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RESULTS
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There was no operative mortality. The mean aortic cross clamp time was 35 min (range, 30–44 min). The cardiopulmonary bypass time was 75 min (range, 60–85 min) for patients who underwent mitral valve repair. In the 2 cases of double-valve repair, aortic cross clamp times were 85 and 101 min, and bypass times were 116 and 136 min. The mean left atrial pressure was 14 mm Hg (range, 10–34 mm Hg) preoperatively and it reduced to 7 mm Hg (range, 5–13 mm Hg) postoperatively. The median duration of mechanical ventilation was 26 hours (range, 12–48 hours). Mean hospital stay was 10 days (range, 7–18 days). The patients were followed up for 3–18 months (mean, 8.9 months). One child had rheumatic carditis with moderate AR and MR. After medical treatment, AR and MR were reduced to mild, with less cardiomegaly. Two patients had pericardial effusion; one presented on the 9th postoperative day, and the other at 2 months after surgery. None needed reoperation for valve repair or replacement. There were no thromboembolic or hemorrhagic events, and no infective endocarditis. Three patients had residual trivial MR, 5 had mild MR (all had severe MR preoperatively; Figure 5A and B
), and 1 each had mild and trivial AR (Figure 6A and B
). All patients were asymptomatic and in New York Heart Association functional class I. None required valve replacement.

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Figure 5. (A) Preoperative 2-dimensional echocardiogram showing severe mitral regurgitation; (B) Postoperative 2-dimensional echo showing mild mitral regurgitation.
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Figure 6. (A) Preoperative 2-dimensional echocardiogram showing severe aortic regurgitation; (B) Postoperative 2-dimensional echo showing mild aortic regurgitation.
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DISCUSSION
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In developing countries, rheumatic heart disease is the most common acquired cardiovascular disease affecting young people.15 Both repair and replacement of the regurgitant rheumatic valve have been reported.2,5,6,13 Valve replacement is not the best option in children because of higher mortality compared to adults, the small size of the implanted valve, problems with long-term anticoagulation, degeneration of bioprostheses, higher risks of endocarditis and suboptimal ventricular function, and reduced long-term survival. Thus valve repair is the procedure of choice in the pediatric age group.7,9,10
Experience with valve repair remains scant because it is technically more difficult and associated with a high failure rate in this age group.5,10,16,17 The reoperation rate after valve surgery in children is 5%–27% compared to 8%–10% in adults at 5-year follow-up.8,11 Factors contributing to a high rate of reoperation after mitral valve replacement include: continued growth of the child; tissue overgrowth of the replaced valve, causing stenosis; and early calcified degeneration of bioprosthetic valves, reported to be 9% per patient-year.18 Mitral valve repair has a lower reoperation rate: Carpentier and colleagues3 reported an actuarial risk of reoperation of 13.4% at 5 years, and 21% at 10 years; Okita and colleagues19 achieved actuarial freedom from reoperation of 89% at 10 years; and Kirklin20 assessed the freedom from reoperation at 10 years to be 75%.
We customized our approach to reconstruction according to valve morphology, using a number of techniques to repair the valve. Chordal shortening is simple in rheumatic patients because the elongated chordae are usually quite thickened and capable of holding the suture. Annular plication was carried out in cases of annular dilatation. We found leakage at the mitral valve commissural area in most of our patients. Reconstruction of the commissural leaflets using sutures passed through these small leaflets corrected MR. The rate of thromboembolic complications after valve repair compares favorably with the results of mechanical or bioprosthetic valve replacement.2 In our series, no patient suffered a thromboembolic or hemorrhagic event. Valve repair avoids the need for lifelong anticoagulation. In addition to chordal shortening and annular plication, reconstruction of the commissural leaflets was the most important aspect of valve repair in our experience. Valve repair can be performed without annuloplasty rings, which buys some time until the child grows. This is very important in this subset of patients. Early and midterm results are good, although long-term results are still awaited.
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ACKNOWLEDGMENTS
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We thank Dr. Vincent O. (Cardiac Surgeon), Dr. Rohit Agarwal (Anesthesiologist), Dr. Sanjay Bhalerao (Pediatrician) and Mr. Dhanabalan (Artist) for their help in preparing the manuscript.
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