선천성 승모판막 폐쇄부전증이 있는 소아에서승모판막 성형술에 대한 임상적 고찰 = Mitral Valve Repair for Congenital Mitral Regurgitation in Children

국문 초록 (Abstract)

배경: 소아 환자의 승모판막 질환에 대한 외과적 치료는 그 모양의 다양성과 동반된 선천성 심장 기형 등으로 인해 기술적, 임상적으로 어렵다. 본 연구는 선천성 승모판막 폐쇄부전증 환아에서 본원에서 시행한 승모판막 성형 수술의 결과를 평가하였다. 대상 및 방법: 1997년 4월부터 2007년 10월까지 승모판막 폐쇄부전증으로 승모판막 수술을 시행한 소아 환자 22명을 대상으로 후향적으로 분석하였다. 수술 당시 환아의 평균 연령은 5.4세였고, 이 중 12개월 미만의 영아는 4명이었다. 동반된 심기형으로는 심실 중격결손이 13예로 가장 많았고, 그 외에 심방 중격결손과 판막 상부 대동맥 판막 협착증(supravalvar aortic valve stenosis)이 각각 1예 있었으며, 승모판막 폐쇄부전증만 단독으로 있는 경우는 7예가 있었다. 수술 전 승모판막 폐쇄부전증의 정도는 Ⅱ, Ⅲ, Ⅳ가 각각 4예, 15예, 3예였고, 그 원인으로 판막 탈출증(leaflet prolapse)이 12예, 판막륜 확장(annular dilatation)이 4예, 제한된 판막 움직임(restrictive leaflet motion)이 5예 있었다. 수술 전후의 폐쇄부전증 정도와 승모판막륜의 Z-value를 비교함으로써 그 결과를 평가하였다. 결과: 모든 환자에서 판막 성형술이 가능했고, 수술 후 조기 사망과 만기 사망 모두 없었다. 19예에서 판막륜 축소술(reduction annuloplasty)을 시행하였고, 18예에서 건삭 축소술(shortening of chordae)이나 판막열 봉합술(cleft closure) 등의 판막 성형술(valvuloplasty)은 추가로 시행하였다. 수술 직후 승모판막 폐쇄부전증의 정도는 줄어들었고(0=10, I=5, Ⅱ=5, Ⅲ=2), 승모판막륜의 Z-value도 감소하였다(2.2±2.1 vs 0.7±2.3, p＜0.01). 3.68년의 추적기간 동안 3명의 환아가 승모판막 폐쇄부전증으로 재수술을 받았고, 한명은 재성형술을, 나머지 2명은 승모판막 치환술을 받았으며, 퇴원 시 3명의 환아에서 수술 직 후 보다 폐쇄부전증이 좀 더 진행되었다. 결론: 소아의 승모판막 폐쇄부전증에서 일차 수술방법으로 적극적인 승모판막 성형술은 효과적이고 믿을만한 방법이다. 좀 더 좋은 수술 결과를 얻기 위해서는 수술 전 환아의 승모판막의 모양과 기능을 면밀히 파악하고, 다양한 수술적 기법을 적용하는 노력이 필요하겠다.

다국어 초록 (Multilingual Abstract)

Background: Surgery for mitral valve disease in children carries both technical and clinical difficulties that are due to both the wide spectrum of morphologic abnormalities and the high incidence of associated cardiac anomalies. The purpose of this study is to assess the outcome of mitral valve surgery for treating congenital mitral regurgitation in children. Material and Method: From 1997 to 2007, 22 children (mean age: 5.4 years) who had congenital mitral regurgitation underwent mitral valve repair. The median age of the patients was 5.4 years old and four patients (18%) were under 12 months of age. 15 patients (68%) had cardiac anomalies. There were 13 cases of ventricular septal defect, 1 case of atrial septal defect and 1 case of supravalvar aortic stenosis. The grade of the preoperative mitral valve regurgitation was Ⅱ in 4 patients, Ⅲ in 15 patients and Ⅳ in 3. The regurgitation was due to leaflet prolapse in 12 patients, annular dilatation in 4 patients and restrictive leaflet motion in 5 patients. The preoperative MV Z-value and the regurgitation grade were compared with those obtained at follow-up. Result: MV repair was possible in all the patients. 19 patients required reduction annuloplasty and 18 patients required valvuloplasty that included shortening of the chordae, papillary muscle splitting, artificial chordae insertion and cleft closure. There were no early or late deaths. The mitral valve regurgitation after surgery was improved in all patients (absent=10, grade I=5, Ⅱ=5, Ⅲ=2). MV repair resulted in reduction of the mitral valve Z-value (2.2±2.1 vs. 0.7±2.3, respectively, p＜0.01). During the mid-term follow-up period of 3.68 years, reoperation was done in three patients (one with repair and two with replacement) and three patients showed mild progression of their mitral regurgitation. Conclusion: Our experience indicates that mitral valve repair in children with congenital mitral valve regurgitation is an effective and reliable surgical method with a low reoperation rate. A good postoperative outcome can be obtained by preoperatively recognizing the intrinsic mitral valve pathophysiology detected on echocardiography and with the well-designed, aggressive application of the various reconstruction techniques.

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